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Sample records for fibrose radio-induite superficielle

  1. Radiation retinopathy; Les retinopathies radio-induites

    Energy Technology Data Exchange (ETDEWEB)

    Lumbroso, L.; Desjardins, L. [Institut Curie, Serv. d' Ophtalmologie, 75 - Paris (France); Dendale, R.; Fourquet, A. [Institut Curie, Serv. de Radiotherapie, 75 - Paris (France)

    2002-09-01

    Radiation retinopathy is a retinal micro-angiopathy, observed after irradiation of the eye. It can rarely lead to neo-vascular glaucoma and enucleation due to pain. It is due to a progressive retinal capillary then vascular occlusion. Total irradiation dose, dose fraction, and surface of the irradiated retina seem to be strong predictive factors for radiation retinopathy. Patients who underwent an irradiation near the eye (skull base tumors, nasal and paranasal tumors, or brain tumors) should be followed by periodic ophthalmologic examination to detect and treat when necessary the non perfusion areas. (authors)

  2. Autoantibodies in cryptogenic fibrosing alveolitis

    Directory of Open Access Journals (Sweden)

    du Bois Ron

    2001-02-01

    Full Text Available Abstract The pathogenesis of cryptogenic fibrosing alveolitis (CFA involves injury, an immune/inflammatory response and fibrosis. The cause of the injury is unknown, but the identification of serum autoantibodies makes an autoimmune aetiology attractive. The core study on which this commentary is based used novel cloning and serum screening technologies in order to identify new public and private autoantibodies in sera from 12 patients with CFA. Largely negative conclusions were drawn from that study. However, we suggest that the prevalence of autoantibodies may have been underestimated, that the study was timely and that this approach is worth pursuing further.

  3. Frontal fibrosing alopecia treatment options.

    Science.gov (United States)

    Fertig, Raymond; Tosti, Antonella

    2016-11-01

    Frontal fibrosing alopecia (FFA) is a rare dermatologic disease that causes scarring and hair loss and is increasing in prevalence worldwide. FFA patients typically present with hair loss in the frontal scalp region and eyebrows which may be associated with sensations of itching or burning. FFA is a clinically distinct variant of lichen planopilaris (LPP) that affects predominantly postmenopausal women, although men and premenopausal women may also be affected. Early diagnosis and prompt treatment are necessary to prevent definitive scarring and permanent hair loss. Data from retrospective studies indicate that 5-alpha-reductase inhibitors (5aRIs) are effective in stabilizing the disease. In our clinical experience, we have seen optimal results treating FFA patients with oral finasteride in conjunction with hydroxychloroquine, topical calcineurin inhibitors (tacrolimus) and excimer laser in patients with signs of active inflammation.

  4. Radioinduced intestinal fibrosis: from molecular mechanisms to therapy applications. Contribution of the TGF--{beta}1, of the CTGF and of the transduction pathway of the Rho/ROCK signal; La fibrose intestinale radio-induite: des mecanismes moleculaires aux applications therapeutiques. Roles du TGF-{beta}1, du CTGF et de la voie de transduction du signal Rho/ROCK

    Energy Technology Data Exchange (ETDEWEB)

    Haydont, V

    2006-12-15

    Delayed radiation enteritis is an intestinal fibrosis induced by accidental or therapeutic radiation for pelvic and abdominal cancer treatments. Studies of molecular mechanisms involved in the development and maintenance of fibrosis have showed the respective contribution of CTGF, low TGF-{beta}1 concentrations and Rho/ROCK pathway. Thus, based on the relationship between CTGF, TGF-{beta}1 and Rho pathway, 2 therapeutics strategies have been develop. First, a pravastatin curative gift leads to a fibro-lysis involving an inhibition of Rho and in cascade a reduction of CTGF expression and extracellular matrix deposition. The data suggest that reversal of established radiation fibrosis in the gut is possible. Second, a pravastatin prophylactic gift prevents the installation of a chronic fibrosis but does not protect the tumor. On the base of these results, the radiation therapy department of the Institut Gustave Roussy will soon initiate 2 clinical trials. (author)

  5. Molecular Targets for the Treatment of Fibrosing Cholangiopathies

    NARCIS (Netherlands)

    Maillette de Buy Wenniger, L. J.; Oude Elferink, R. P.; Beuers, U.

    2012-01-01

    Emerging pathophysiologic insights are leading to novel approaches to treating fibrosing cholangiopathies. The current treatment, using ursodeoxycholic acid (UDCA), may slow the progression of some chronic cholangiopathies but cannot heal them. Apart from immunosuppressive interventions aimed at

  6. Tuberculosis-associated Fibrosing Mediastinitis: Case Report and Literature Review

    Directory of Open Access Journals (Sweden)

    Ronald Tan

    2016-01-01

    Full Text Available Fibrosing mediastinitis is a rare condition defined by the presence of fibrotic mediastinal infiltrates that obliterate normal fat planes. It is a late complication of a previous granulomatous infection, such as histoplasmosis or tuberculosis (TB. Due to its rarity, fibrosing mediastinitis is often under-recognized, and the clinical presentation is variable and dependent on the extent of infiltration or encasement of structures within the mediastinum. We present a case of fibrosing mediastinitis in a man with a prior history of TB, who presented with progressive dyspnea and was found to have chronic mediastinal soft tissue opacities and pulmonary hypertension. His diagnosis was delayed due to the lack of recognition of this clinical/radiographic entity. Fibrosing mediastinitis is a rare entity usually caused by granulomatous disease. Most cases develop as a late complication of histoplasmosis or TB. The presence of calcified mediastinal soft tissue infiltrates on advanced chest imaging can be diagnostic of fibrosing mediastinitis in patients with a prior history of a granulomatous infection once active processes such as malignancy are excluded.

  7. Tuberculosis-associated Fibrosing Mediastinitis: Case Report and Literature Review.

    Science.gov (United States)

    Tan, Ronald; Martires, Joanne; Kamangar, Nader

    2016-01-01

    Fibrosing mediastinitis is a rare condition defined by the presence of fibrotic mediastinal infiltrates that obliterate normal fat planes. It is a late complication of a previous granulomatous infection, such as histoplasmosis or tuberculosis (TB). Due to its rarity, fibrosing mediastinitis is often under-recognized, and the clinical presentation is variable and dependent on the extent of infiltration or encasement of structures within the mediastinum. We present a case of fibrosing mediastinitis in a man with a prior history of TB, who presented with progressive dyspnea and was found to have chronic mediastinal soft tissue opacities and pulmonary hypertension. His diagnosis was delayed due to the lack of recognition of this clinical/radiographic entity. Fibrosing mediastinitis is a rare entity usually caused by granulomatous disease. Most cases develop as a late complication of histoplasmosis or TB. The presence of calcified mediastinal soft tissue infiltrates on advanced chest imaging can be diagnostic of fibrosing mediastinitis in patients with a prior history of a granulomatous infection once active processes such as malignancy are excluded.

  8. Genetics Home Reference: hereditary fibrosing poikiloderma with tendon contractures, myopathy, and pulmonary fibrosis

    Science.gov (United States)

    ... Hereditary fibrosing poikiloderma with tendon contractures, myopathy, and pulmonary fibrosis Printable PDF Open All Close All Enable Javascript ... Fibrosing Poikiloderma with Tendon Contractures, Myopathy, and Pulmonary ... Lung, and Blood Institute (NHLBI): Pulmonary Function Tests National ...

  9. Fibrosing Mediastinitis: Successful Stenting of the Pulmonary Artery

    Directory of Open Access Journals (Sweden)

    Rennae Thiessen

    2008-01-01

    Full Text Available Fibrosing mediastinitis is a rare benign condition, which can cause compression of the pulmonary or systemic vessels, tracheobronchial tree, coronary arteries or esophagus, leading to disabling clinical symptoms and even death. The case of a 26-year-old woman who presented with dyspnea is described. She was found to have 80% stenosis of the right pulmonary artery secondary to fibrosing mediastinitis. The stenosis was managed successfully with an endovascular Palmaz-Schatz stent, and the patient remains symptom-free 10 years later.

  10. Idiopathic fibrosing pancreatitis: A cause of obstructive jaundice in childhood

    International Nuclear Information System (INIS)

    Atkinson, G.O. Jr.; Wyly, J.B.; Gay, B.B. Jr.; Ball, T.I.; Winn, K.J.

    1988-01-01

    Idiopathic fibrosing pancreatitis is a chronic process of unknown etiology characterized by extensive infiltration of the pancreatic parenchyma by fibrous tissue. This disease process is uncommon in the pediatric patient and is consequently rarely considered in the differential diagnosis of abdominal pain and jaundice in the child. The sonographic demonstration of a dilated biliary tree and common bile duct compressed by an enlarged pancreas may be the first suggestion of this entity. Two patients with idiopathic fibrosing pancreatitis and obstructive jaundice are reported with a review of the clinical, radiographic, and pathologic findings. (orig.)

  11. Idiopathic fibrosing pancreatitis: A cause of obstructive jaundice in childhood

    Energy Technology Data Exchange (ETDEWEB)

    Atkinson, G.O. Jr.; Wyly, J.B.; Gay, B.B. Jr.; Ball, T.I.; Winn, K.J.

    1988-01-01

    Idiopathic fibrosing pancreatitis is a chronic process of unknown etiology characterized by extensive infiltration of the pancreatic parenchyma by fibrous tissue. This disease process is uncommon in the pediatric patient and is consequently rarely considered in the differential diagnosis of abdominal pain and jaundice in the child. The sonographic demonstration of a dilated biliary tree and common bile duct compressed by an enlarged pancreas may be the first suggestion of this entity. Two patients with idiopathic fibrosing pancreatitis and obstructive jaundice are reported with a review of the clinical, radiographic, and pathologic findings.

  12. Localization fibrosing mediastinitis causing pulmonary infraction: A case report

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    Sur, Young Keun; Kim, Eun Young; Kang, Doo Kyoung; Park, Kyung Joo; Koh, Young Wha; Sun, Joo Sung [Ajou University School of Medicine, Suwon (Korea, Republic of)

    2016-06-15

    A 44-year-old female patient visited our emergency room for hemoptysis and refractory chest wall pain of 2 months duration. She had no history of smoking or other medical conditions. Chest CT scan showed homogenously enhancing soft tissue mass without calcification at the left pulmonary hilum. Encasing and compression of the left lower pulmonary artery by the mass had resulted in pulmonary infarction in the left lower lobe. Laboratory tests for tuberculosis, fungus, and vasculitis were all negative. The patient underwent surgical biopsy and resection of infarcted left lower lobe that was histopathologically confirmed as fibrosing mediastinitis. Herein, we reported a rare case of surgically confirmed and treated localized fibrosing mediastinitis causing pulmonary infarction.

  13. Ventilation perfusion radionuclide imaging in cryptogenic fibrosing alveolitis

    International Nuclear Information System (INIS)

    Bourke, S.J.; Hawkins, T.; Keavey, P.M.; Gascoigne, A.D.; Corris, P.A.

    1993-01-01

    There is increasing interest in ventilation perfusion (V/Q) imaging in cryptogenic fibrosing alveolitis because of the data these scans provide on the dynamic V/Q relationships in such patients undergoing single lung transplantation. We analysed the V/Q scans of 45 consecutive patients with advanced cryptogenic fibrosing alveolitis being considered for single lung transplantation. Scans were classified according to the presence, severity and degree of matching of defects in ventilation and perfusion images and the results were compared with the data obtained from lung function tests. Ventilation images showed defects in 13 (29%) and ''washout delay'' in 15 (33%) patients; 10 (22%) patients had asymmetric distribution of ventilation with one lung receiving >60% of total ventilation. Perfusion images showed normal perfusion in 8 (18%), mild defects in 18 (40%) and major defects in 19 (42%) patients. The distribution of perfusion between lungs was significantly asymmetric in 20 (45%) patients. V/Q images were matched in 15 (33%), mildly mismatched in 15 (33%) and severely mismatched in 15 (33%) patients, but the degree of V/Q mismatch did not show a relationship to KCO, PaO 2 or A-aO 2 gradient. The appearances were atypical of pulmonary embolism in eight patients. (Author)

  14. Cryptogenic fibrosing alveolitis and lung cancer: the BTS study.

    Science.gov (United States)

    Harris, J M; Johnston, I D A; Rudd, R; Taylor, A J Newman; Cullinan, P

    2010-01-01

    The risk of lung cancer is often reported to be increased for patients with cryptogenic fibrosing alveolitis (CFA). Vital status was sought for all 588 members of the British Thoracic Society (BTS) cryptogenic fibrosing alveolitis (CFA) study 11 years after entry to the cohort. Observed deaths due to lung cancer were compared with expected deaths using age-, sex- and period-adjusted national rates. The roles of reported asbestos exposure and smoking were also investigated. 488 cohort members (83%) had died; 46 (9%) were certified to lung cancer (ICD9 162). The standardised mortality ratio (SMR) was 7.4 (95% CI 5.4 to 9.9). Stratified analysis showed increased lung cancer mortality among younger subjects, men and ever smokers. Using an independent expert panel, 25 cohort members (4%) were considered to have at least moderate exposure to asbestos; the risk of lung cancer was increased for these subjects (SMR 13.1 (95% CI 3.6 to 33.6)) vs 7.2 (95% CI 5.2 to 9.7) for those with less or no asbestos exposure). Ever smoking was reported by 448 (73%) of the cohort and was considerably higher in men than in women (92% vs 49%; p<0.001). Most persons who died from lung cancer were male (87%), and all but two (96%) had ever smoked. Ever smokers presented at a younger age (mean 67 vs 70 years; p<0.001) and with less breathlessness (12% smokers reported no breathlessness vs 5% never smokers; p = 0.02). These findings confirm an association between CFA and lung cancer although this relationship may not be causal. The high rate of smoking and evidence that smokers present for medical attention earlier than non-smokers suggest that smoking could be confounding this association.

  15. Aerobic fitness in patients with fibrositis. A controlled study of respiratory gas exchange and 133-xenon clearance from exercising muscle

    International Nuclear Information System (INIS)

    Bennett, R.M.; Clark, S.R.; Goldberg, L.; Nelson, D.; Bonafede, R.P.; Porter, J.; Specht, D.

    1989-01-01

    Aerobic fitness was evaluated in 25 women with fibrositis, by having them exercise to volitional exhaustion on an electronically braked cycle ergometer. Compared with published standards, greater than 80% of the fibrositis patients were not physically fit, as assessed by maximal oxygen uptake. Compared with matched sedentary controls, fibrositis patients accurately perceived their level of exertion in relation to oxygen consumption and attained a similar level of lactic acidosis, as assessed by their respiratory quotient and ventilatory threshold. Exercising muscle blood flow was estimated by 133-xenon clearance in a subgroup of 16 fibrositis patients and compared with that in 16 matched sedentary controls; the fibrositis patients exhibited reduced 133-xenon clearance. These results indicate a need to include aerobic fitness as a matched variable in future controlled studies of fibrositis and suggest that the detraining phenomenon may be of relevance to the etiopathogenesis of the disease

  16. Corrosion inhibition of magnesium heated in wet air, by surface fluoridation; Inhibition de la corrosion du magnesium chauffe dans l'air humide, par fluoruration superficielle

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    Caillat, R; Darras, R; Leclercq, D [Commissariat a l' Energie Atomique, Saclay (France).Centre d' Etudes Nucleaires

    1960-07-01

    The maximum temperature (350 deg. C) of magnesium corrosion resistance in wet air may be raised to 490-500 deg. C by the formation of a superficial fluoride film. This can be obtained by two different ways: either by addition of hydrofluoric acid to the corroding medium in a very small proportion such as 0,003 mg/litre; at atmospheric pressure, or by dipping the magnesium in a dilute aqueous solution of nitric and hydrofluoric acids at room temperature before exposing it to the corroding atmosphere. In both cases the corrosion inhibition is effective over a very long time, even several thousand hours. (author) [French] La temperature limite (350 deg. C) de resistance du magnesium a la corrosion par l'air humide, peut etre elevee jusque 490-500 deg. C par la formation d'une couche fluoruree superficielle. Deux procedes permettent d'obtenir ce resultat: l'atmosphere corrodante peut etre additionnee d'acide fluorhydrique a une concentration aussi faible que 0,003 mg/litre, a la pression atmospherique, ou bien le magnesium peut etre traite a froid, avant exposition a la corrosion, dans une solution aqueuse diluee d'acides nitrique et fluorhydrique. Dans les deux cas, la protection est assuree, meme pour de tres longues durees d'exposition: plusieurs milliers d'heures. (auteur)

  17. Corrosion inhibition of magnesium heated in wet air, by surface fluoridation; Inhibition de la corrosion du magnesium chauffe dans l'air humide, par fluoruration superficielle

    Energy Technology Data Exchange (ETDEWEB)

    Caillat, R.; Darras, R.; Leclercq, D. [Commissariat a l' Energie Atomique, Saclay (France).Centre d' Etudes Nucleaires

    1960-07-01

    The maximum temperature (350 deg. C) of magnesium corrosion resistance in wet air may be raised to 490-500 deg. C by the formation of a superficial fluoride film. This can be obtained by two different ways: either by addition of hydrofluoric acid to the corroding medium in a very small proportion such as 0,003 mg/litre; at atmospheric pressure, or by dipping the magnesium in a dilute aqueous solution of nitric and hydrofluoric acids at room temperature before exposing it to the corroding atmosphere. In both cases the corrosion inhibition is effective over a very long time, even several thousand hours. (author) [French] La temperature limite (350 deg. C) de resistance du magnesium a la corrosion par l'air humide, peut etre elevee jusque 490-500 deg. C par la formation d'une couche fluoruree superficielle. Deux procedes permettent d'obtenir ce resultat: l'atmosphere corrodante peut etre additionnee d'acide fluorhydrique a une concentration aussi faible que 0,003 mg/litre, a la pression atmospherique, ou bien le magnesium peut etre traite a froid, avant exposition a la corrosion, dans une solution aqueuse diluee d'acides nitrique et fluorhydrique. Dans les deux cas, la protection est assuree, meme pour de tres longues durees d'exposition: plusieurs milliers d'heures. (auteur)

  18. PET scanning of macrophages in patients with scleroderma fibrosing alveolitis

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    Branley, Howard M. [Imperial College London, Hammersmith Campus, London (United Kingdom)], E-mail: Howard.Branley@whittington.nhs.uk; Bois, Roland M. du; Wells, Athol U. [Royal Brompton Hospital, London (United Kingdom); Jones, Hazel A. [Imperial College London, Hammersmith Campus, London (United Kingdom)

    2008-11-15

    Rationale: Assessment of disease activity in fibrosing alveolitis due to systemic sclerosis (FASSc) is difficult without using invasive investigation. A repeatable noninvasive method of assessing disease at a cellular level such as with positron emission tomography (PET) could be of great value in evaluating high-resolution changes in the pathological process. Objectives: To investigate whether the level of inflammatory cell traffic and lung density in FASSc, imaged in vivo by PET, is different to controls and whether they are associated with changes in pulmonary function indices. Methods: We used PET to measure lung density and tissue uptake of {sup 11}C-[R]-PK11195, a ligand that binds to receptors found in abundance in macrophages. Fifteen patients with FASSc were compared to seven controls. Results: A trend of reduced uptake of {sup 11}C-[R]-PK11195 was observed in FASSc patients (P=.09) and correlated inversely with lung density (r=-.62; P<.05), which was significantly elevated in FASSc [0.35{+-}0.02 vs. 0.23{+-}0.02 g/cc (mean{+-}S.E.M.); P<.005]. Conclusion: These results demonstrate that inflammatory cell traffic and lung density can be imaged in vivo in FASSc using PET, and that this approach might be of potential value in understanding, in situ, components of pathogenesis that may have value for prognosis.

  19. PET scanning of macrophages in patients with scleroderma fibrosing alveolitis

    International Nuclear Information System (INIS)

    Branley, Howard M.; Bois, Roland M. du; Wells, Athol U.; Jones, Hazel A.

    2008-01-01

    Rationale: Assessment of disease activity in fibrosing alveolitis due to systemic sclerosis (FASSc) is difficult without using invasive investigation. A repeatable noninvasive method of assessing disease at a cellular level such as with positron emission tomography (PET) could be of great value in evaluating high-resolution changes in the pathological process. Objectives: To investigate whether the level of inflammatory cell traffic and lung density in FASSc, imaged in vivo by PET, is different to controls and whether they are associated with changes in pulmonary function indices. Methods: We used PET to measure lung density and tissue uptake of 11 C-[R]-PK11195, a ligand that binds to receptors found in abundance in macrophages. Fifteen patients with FASSc were compared to seven controls. Results: A trend of reduced uptake of 11 C-[R]-PK11195 was observed in FASSc patients (P=.09) and correlated inversely with lung density (r=-.62; P<.05), which was significantly elevated in FASSc [0.35±0.02 vs. 0.23±0.02 g/cc (mean±S.E.M.); P<.005]. Conclusion: These results demonstrate that inflammatory cell traffic and lung density can be imaged in vivo in FASSc using PET, and that this approach might be of potential value in understanding, in situ, components of pathogenesis that may have value for prognosis

  20. Prevalence of asymptomatic coronary disease in fibrosing idiopathic interstitial pneumonias

    International Nuclear Information System (INIS)

    Cassagnes, Lucie; Gaillard, Vianney; Monge, Emmanuel; Faivre, Jean-Baptiste; Delhaye, Cédric; Molinari, Francesco; Petyt, Grégory; Hossein-Foucher, Claude; Wallaert, Benoit; Duhamel, Alain; Remy, Jacques; Remy-Jardin, Martine

    2015-01-01

    Background: Because of growing body of interest on the association between fibrosing idiopathic interstitial pneumonias (f-IIP) and ischaemic heart disease, we initiated this prospective study to evaluate the prevalence of asymptomatic coronary artery disease (CAD) in patients with f-IIP. Methods: Forty-two patients with f-IIP underwent noninvasive screening for CAD that included (a) a chest CT examination enabling calculation of the coronary artery calcium (CAC) score, then depiction of coronary artery stenosis; and (b) stress myocardial perfusion scintigraphy (MPS). Patients with significant coronary abnormalities, defined by a CAC score >400 or coronary artery stenosis >50% at CT and/or perfusion defect >5% at MPS, were referred to the cardiologist. Coronary angiography was indicated in presence of a perfusion defect >10% at MPS or significant left main or proximal left anterior descending stenosis whatever MPS findings. Results: Combining CT and MPS, significant abnormalities were detected in 32/42 patients (76%). The cardiologist: (a) did not consider further investigation in 21 patients (CT abnormalities but no ischaemia at MPS: 12/21; false-positive findings at MPS: 3/21; poor respiratory condition: 6/21); (b) proceeded to coronary angiography in 11 patients which confirmed significant stenoses in 5 patients (5/42; 12%). In the worst-case-scenario (i.e., inclusion of 6 patients with significant coronary artery abnormalities who were not investigated due to poor respiratory condition), the prevalence of CAD reached 26% (11/42). Conclusion: In the studied population of patients with f-IIP, asymptomatic CAD ranged between 12% and 26%

  1. Measures of the inflammatory response in cryptogenic fibrosing alveolitis

    International Nuclear Information System (INIS)

    Pantin, C.F.; Valind, S.O.; Sweatman, M.; Lawrence, R.; Rhodes, C.G.; Brudin, L.; Britten, A.; Hughes, J.M.; Turner-Warwick, M.

    1988-01-01

    Cryptogenic fibrosing alveolitis (CFA) is characterized by interstitial fibrosis and parenchymal inflammation. Eleven patients with CFA (10 proved by lung biopsy) were followed over 2 yr using clinical symptoms, radiographic change, and pulmonary function tests to adjust their treatment. Lung lavage, positron camera (PET) measurements of regional extravascular lung density (Dev), pulmonary blood volume (Vb), and the metabolic rate for 18F-deoxyglucose (MRglc), clearance of 99mTc-diethylenetriaminepentacetate (99mTc-DTPA) aerosol, and lung uptake of 67Ga were measured initially and at the end of the first year to give a profile of the inflammatory response. Compared with normal subjects, there was an increased percentage of neutrophils and eosinophils in the lung lavage, increased Dev (p less than 0.002) with no significant difference in Vb, increased MRglc (p less than 0.02), 99mTc-DTPA clearance (p less than 0.002), and 67Ga uptake (p less than 0.02). The smallest increases in Dev were seen in the two patients with most destruction shown by lung biopsy. There were inverse correlations between Dev and both FVC and TLC, but a direct correlation between Vb and transfer factor. 99mTc-DTPA clearance changed concordantly with clinical status and radiographic and respiratory function changes during the first year. If glucose utilization (MRglc) remained in the normal range between the initial and first yearly assessment, the patient improved or remained stable during the second year as shown by clinical status and radiographic and respiratory function measurements. If it rose or remained high, the patient's condition deteriorated

  2. Cicatricial organising pneumonia mimicking a fibrosing interstitial pneumonia.

    Science.gov (United States)

    Churg, Andrew; Wright, Joanne L; Bilawich, AnaMaria

    2018-04-01

    Organising pneumonia (OP) is composed of loose granulation tissue plugs in distal airspaces; these disappear with steroid treatment. Recently a variant labelled 'cicatricial' OP has been described in which the granulation tissue organised to much denser fibrous tissue but still retained the usual pattern of OP. Here we report 10 patients thought to have an interstitial lung disease, and who on biopsy had a variant of cicatricial OP characterised by linear bands or small nodular masses of dense fibrous tissue that does not resemble ordinary OP. The bands/nodules were usually distributed randomly but occasionally resembled fibrotic non-specific interstitial pneumonia in local areas. Small foci of loose granulation tissue at the edge of the fibrotic bands sometimes mimicked fibroblast foci. Recognisable conventional OP was always present, but often in very small amounts. Four cases, including one patient with Ehlers-Danlos syndrome, showed formation of bone in the fibrotic bands and nodules. On computerised tomography (CT) scan of the chest some cases looked like typical OP, but some demonstrated only irregularly distributed linear opacities, sometimes with associated calcification. Follow-up imaging on six cases showed that the process either markedly improved or remained stable over time; no case had progressive disease. Cicatricial OP with this pathological pattern represents an uncommon form of OP that appears to be a generally benign process which may have persisting linear opacities on CT scan but that does not progress; however, it can be confused on biopsy and CT with a fibrosing interstitial pneumonia. © 2017 John Wiley & Sons Ltd.

  3. Prevalence of asymptomatic coronary disease in fibrosing idiopathic interstitial pneumonias

    Energy Technology Data Exchange (ETDEWEB)

    Cassagnes, Lucie; Gaillard, Vianney [Department of Thoracic Imaging (EA 2694), Hospital Calmette, CHRU and Univ Lille 2 Nord de France, F-59000 Lille (France); Monge, Emmanuel [Department of Pulmonology, Center of Competence for Rare Pulmonary Diseases, Hospital Calmette, CHRU and Univ Lille 2 Nord de France, F-59000 Lille (France); Faivre, Jean-Baptiste [Department of Thoracic Imaging (EA 2694), Hospital Calmette, CHRU and Univ Lille 2 Nord de France, F-59000 Lille (France); Delhaye, Cédric [Department of Cardiology, Cardiology Hospital, CHRU and Univ Lille 2 Nord de France, F-59000 Lille (France); Molinari, Francesco [Department of Thoracic Imaging (EA 2694), Hospital Calmette, CHRU and Univ Lille 2 Nord de France, F-59000 Lille (France); Petyt, Grégory; Hossein-Foucher, Claude [Department of Nuclear Medicine, Hospital Salengro, CHRU and Univ Lille 2 Nord de France, F-59000 Lille (France); Wallaert, Benoit [Department of Pulmonology, Center of Competence for Rare Pulmonary Diseases, Hospital Calmette, CHRU and Univ Lille 2 Nord de France, F-59000 Lille (France); Duhamel, Alain [Department of Medical Statistics (EA 2694), Univ Lille Nord de France, F-59000 Lille (France); Remy, Jacques [Department of Thoracic Imaging (EA 2694), Hospital Calmette, CHRU and Univ Lille 2 Nord de France, F-59000 Lille (France); Remy-Jardin, Martine, E-mail: martine.remy@chru-lille.fr [Department of Thoracic Imaging (EA 2694), Hospital Calmette, CHRU and Univ Lille 2 Nord de France, F-59000 Lille (France)

    2015-01-15

    Background: Because of growing body of interest on the association between fibrosing idiopathic interstitial pneumonias (f-IIP) and ischaemic heart disease, we initiated this prospective study to evaluate the prevalence of asymptomatic coronary artery disease (CAD) in patients with f-IIP. Methods: Forty-two patients with f-IIP underwent noninvasive screening for CAD that included (a) a chest CT examination enabling calculation of the coronary artery calcium (CAC) score, then depiction of coronary artery stenosis; and (b) stress myocardial perfusion scintigraphy (MPS). Patients with significant coronary abnormalities, defined by a CAC score >400 or coronary artery stenosis >50% at CT and/or perfusion defect >5% at MPS, were referred to the cardiologist. Coronary angiography was indicated in presence of a perfusion defect >10% at MPS or significant left main or proximal left anterior descending stenosis whatever MPS findings. Results: Combining CT and MPS, significant abnormalities were detected in 32/42 patients (76%). The cardiologist: (a) did not consider further investigation in 21 patients (CT abnormalities but no ischaemia at MPS: 12/21; false-positive findings at MPS: 3/21; poor respiratory condition: 6/21); (b) proceeded to coronary angiography in 11 patients which confirmed significant stenoses in 5 patients (5/42; 12%). In the worst-case-scenario (i.e., inclusion of 6 patients with significant coronary artery abnormalities who were not investigated due to poor respiratory condition), the prevalence of CAD reached 26% (11/42). Conclusion: In the studied population of patients with f-IIP, asymptomatic CAD ranged between 12% and 26%.

  4. Frontal fibrosing alopecia and lichen planus pigmentosus: diagnosis and therapeutic challenge.

    Science.gov (United States)

    Mulinari-Brenner, Fabiane Andrade; Guilherme, Marina Riedi; Peretti, Murilo Calvo; Werner, Betina

    2017-01-01

    Frontal fibrosing alopecia is a variant of lichen planopilaris with marginal progressive hair loss on the scalp, eyebrows and axillae. We report a case of frontal fibrosing alopecia and lichen planus pigmentosus in a postmenopausal woman, that started with alopecia on the eyebrows and then on the frontoparietal region, with periocular and cervical hyperpigmentation of difficult management. The condition was controlled with systemic corticosteroid therapy and finasteride. Lichen planus pigmentosus is an uncommon variant of lichen planus frequently associated with frontal fibrosing alopecia in darker phototipes. It should be considered in patients affected by scarring alopecia with a pattern of lichen planopilaris and areas of skin hyperpigmentation revealing perifollicular hyperpigmentation refractory to multiple treatments. This case illustrates diagnostic and therapeutic challenge in face of scarring alopecia and perifollicular hyperpigmentation.

  5. Controvérsias na fibrose cística: do pediatra ao especialista

    Directory of Open Access Journals (Sweden)

    Ribeiro Jose Dirceu

    2002-01-01

    Full Text Available Objetivos: nos últimos 70 anos, a fibrose cística emergiu da obscuridade para o reconhecimento como a mais importante doença hereditária, potencialmente letal, incidente na raça branca. Embora seja uma doença genética, na qual o defeito básico acomete células de vários órgãos, nem todos os indivíduos expressam respostas clínicas na mesma intensidade. Várias manifestações clínicas, principalmente pulmonares e digestivas, podem ocorrer durante a vida dos pacientes fibrocísticos. O objetivo deste artigo é propiciar ao pediatra geral uma visão atualizada dos principais assuntos referentes à fibrose cística. Fontes dos dados: revisão sistemática e atualizada em fonte de dados oficial (Medline. Síntese dos dados: foram revisados 79 artigos sobre fibrose cística, de periódicos internacionais, colocando, de modo atual e crítico, os principais eventos relacionados com a incidência, a fisiopatogenia, as manifestações clínicas, o diagnóstico e o tratamento da fibrose cística. Conclusões: apesar de não existir cura para essa doença, muitos conhecimentos novos sobre a etiologia e a fisiopatologia, adquiridos nas duas últimas décadas, propiciaram uma nova abordagem para o tratamento da fibrose cística. A compreensão dos mecanismos básicos da doença pulmonar, bem como das manifestações digestivas na fibrose cística, decorrente dos conhecimentos de pesquisas recentes, tem sido a chave para o aumento da sobrevida e a melhora da qualidade de vida dos pacientes.

  6. Idiopathic fibrosing pancreatitis: a rare cause of chronic obstructive jaundice in childhood

    International Nuclear Information System (INIS)

    Park, Hye Seong; Kim, Hyun Sook; Kim, Hack Hee; Kim, Ok Hwa; Kim, Choon Yul; Bahk, Yong Whee

    1992-01-01

    We report a 14-months-old infant who had obstructive jaundice caused by idiopathic fibrosing pancreatitis. Ultrasonography and abdominal computed tomography showed dilatation of the intrahepatic ducts, common bile duct, and the pancreatic duct. Diffuse swelling of the pancreas was also noted on CT. At laparotomy, the head portion of the pancreas revealed a stony hard consistency, and proliferation of fibrotic tissue was confirmed pathologically. Idiopathic fibrosing pancreatitis is a very rare disease entity in childhood, but should be considered in the differential diagnosis of obstructive jaundice in children who demonstrate bile duct and pancreatic duct dilatation and/or diffuse pancreas swelling

  7. Successful Treatment of Fibrosing Organising Pneumonia Causing Respiratory Failure with Mycophenolic Acid.

    Science.gov (United States)

    Paul, Christina; Lin-Shaw, Ammy; Joseph, Mariamma; Kwan, Keith; Sergiacomi, Gianluigi; Mura, Marco

    2016-01-01

    Organising pneumonia (OP) is usually promptly responsive to corticosteroid treatment. We describe a series of 3 cases of severe, progressive, biopsy-proven fibrosing OP causing respiratory failure. All cases presented with peribronchial and subpleural consolidations, had a fibro-inflammatory infiltrative component in the alveolar septa, and only had a partial and unsatisfactory response to corticosteroids. However, they responded to mycophenolic acid (MPA) treatment with resolution of respiratory failure as well as clinical and functional improvement. MPA as an additional treatment option for aggressive forms of fibrosing OP and interstitial lung disease needs to be further explored. © 2016 S. Karger AG, Basel.

  8. Radiation-induced pemphigus vulgaris of the breast; Pemphigus vulgaire radio-induit du sein

    Energy Technology Data Exchange (ETDEWEB)

    Vigna-Taglianti, R.; Russi, E.G. [Department of radiotherapy, Santa Croce e Carle general hospital, Via M. Coppino, 12100 Cuneo (Italy); Denaro, N. [Oncology department, university of Messina, Via consolare Valeria no 1, 98100 Messina (Italy); Numico, G. [Department of medical oncology, U. Parini hospital, 11100 Aosta (Italy); Brizio, R. [Department of histopathology, Santa Croce e Carle general hospital, Via M. Coppino, 12100 Cuneo (Italy)

    2011-07-15

    Pemphigus vulgaris is a rare autoimmune muco-cutaneous bullous disease. Patients with a history of pemphigus vulgaris - who need radiotherapy - may show a long lasting bullous cutaneous manifestation, typical of pemphigus, within radiation fields. The literature describes fewer than 20 radio-induced cases. While systematic corticosteroid therapy has proven to be useful, topical treatment used in association with corticosteroid therapy is rarely described. To our knowledge the use of modern dressing products has never been described. We report our experience in a case in which modern dressing products were usefully associated to systemic therapy. (authors)

  9. Fibrose quística em adultos

    Directory of Open Access Journals (Sweden)

    C. Damas

    2007-05-01

    Full Text Available Resumo: Os autores efectuaram uma revisão de doentes adultos com fibrose quística (FQ, seguidos na consulta de Pneumologia no período de 1994-2004 (n=8: cinco mulheres e três homens, com idades compreendidas entre 20 e 34 anos (mediana=27 anos, cuja idade de diagnóstico variou entre os 18 meses e os 31 anos. O diagnóstico foi obtido por prova de suor (positiva em seis doentes e estudo genético (homozigotia para a mutação ΔF508 em quatro doentes. O atingimento respiratório traduziu-se por sinusite e bronquiectasias, acompanhando-se o envolvimento pulmonar por alterações funcionais e também gaso-métricas na maioria dos doentes, ainda que de gravidade variável e não relacionada com a idade de diagnóstico. Verificou-se colonização da árvore brônquica em cinco doentes: Pseudomonas aeruginosa em quatro e Staphilococcus aureus em quatro (verificando-se coloni-zação concomitante por estes agentes em três. As principais causas de exacerbação foram infecções res-piratórias e hemoptises. Quanto ao atingimento não respiratório, quatro doentes apresentavam envolvimento digestivo (com cirrose hepática num caso, um insuficiência renal em hemo-diálise, e em apenas um foi efectuado espermograma para documentação de infertilidade. Quatro doentes tinham osteopenia documentada por densitometria óssea. As medidas terapêuticas mais utilizadas foram a cine-siterapia, os broncodilatadores, a alfa-dornase, muco-líticos, suplementos vitamínicos e enzimáticos, antibio-terapia e oxigenoterapia. Relativamente à evolução, uma doente abandonou a consulta, uma doente faleceu, um doente aguardava transplante pulmonar e os restantes mantiveram as suas características clínicas habituais. Neste grupo, a gravidade da doença pulmonar não se relacionou com o diagnóstico mais tardio, o que se pode dever à diversidade de apresenta

  10. Incidence and prevalence of cryptogenic fibrosing alveolitis in a Norwegian community

    DEFF Research Database (Denmark)

    von Plessen, C; Grinde, O; Gulsvik, A

    2003-01-01

    This study assesses the incidence and prevalence of cryptogenic fibrosing alveolitis (CFA) in a well-defined and stable Norwegian population of 250,000 inhabitants during a period of 15 years. We conducted a file survey of all patients (n = 376) aged 16 years or older with a clinician's diagnosis...

  11. Dispneia expiratória restritiva em um gato com fibrose pulmonar idiopática: relato de caso

    OpenAIRE

    Pereira, C.O.; Costa, F.V.A.; Gomes, D.C.; Kasper, P.N.; Vieira, A.B.; Driemeier, D.

    2017-01-01

    RESUMO As doenças pulmonares intersticiais constituem um grupo de doenças difusas do parênquima pulmonar, no qual a fibrose pulmonar intersticial está incluída. Histologicamente, esta se caracteriza por hiperplasia de pneumócitos tipo II, hiperplasia ou hipertrofia de músculo liso e fibrose. Embora a patogenia da fibrose pulmonar intersticial não esteja bem elucidada, devido às semelhanças microscópicas encontradas nos pneumócitos tipo II em felinos e na forma familiar da doença em humanos, a...

  12. Failure to wean caused by cryptogenic fibrosing pleuritis and bilateral lung trapping: case report Falência do desmame em paciente com fibrose pleural idiopática e trapping pulmonar bilateral: relato de caso

    Directory of Open Access Journals (Sweden)

    Elsemiek Verweel

    2007-12-01

    Full Text Available BACKGROUND AND OBJECTIVES: Cryptogenic fibrosing pleuritis is an extremely rare disease, which can affect both lungs from a very young age. The most common finding is severe lung restriction resulting in both hypoxemic and ventilatory failure. CASE REPORT: Male patient, 26 year old with acute deterioration of chronic respiratory failure. Following admission prolonged mechanical ventilation was necessary. An atypical clinical presentation made the diagnosis difficult, but eventually cryptogenic fibrosing pleuritis and lung fibrosis were established. CONCLUSIONS: The prognostic outcome of patients with the final diagnosis of cryptogenic fibrosing pleuritis is extremely poor, especially in an advanced phase of this disease. We recommend early treatment with corticosteroids or surgical pleural decortication.JUSTIFICATIVA E OBJETIVOS: Fibrose pleural idiopática é uma doença rara e pode afetar ambos pulmões já desde uma idade precoce. O achado mais comum na fibrose pleural idiopática é uma restrição pulmonar grave que pode levar a um quadro de falência respiratória e hipoxemia. RELATO DO CASO: Paciente do sexo masculino, 26 anos, internado com reagudização de insuficiência respiratória crônica e submetido à ventilação mecânica prolongada. Após intensa investigação e uma apresentação clínica atípica, foi estabelecido o diagnóstico de fibrose pleural idiopática associado à fibrose pulmonar. CONCLUSÕES: O prognóstico de pacientes com fibrose pleural idiopática é extremamente ruim, particularmente em fase avançada da doença. Recomenda-se o tratamento precoce com corticosteróides ou decorticação pleural cirúrgica.

  13. Prática fisioterapêutica no tratamento da fibrose cística

    Directory of Open Access Journals (Sweden)

    Carolina Lazzarim de Conto

    2014-08-01

    Full Text Available A fibrose cística (FC, denominada também de mucoviscidose, é uma doença genética do tipo autossômica recessiva que acomete principalmente crianças e indivíduos de raça branca. O objetivo deste estudo foi propiciar uma visão sobre a FC, assim como as possíveis formas de tratamento fisioterapêutico. A pesquisa da literatura foi realizada nas bases de dados SciELO e PubMed, no período de abril de 2002 a março de 2013, através da seleção de artigos científicos referentes à FC, utilizando como palavras-chave: “fibrose cística”, “fisioterapia em fibrose cística” e “fisioterapia respiratória”. Por meio desta revisão, pôde-se observar que, apesar da complexidade da doença, a prática fisioterapêutica, com o uso das técnicas: tapotagem, vibração, drenagem postural (DP, huffing, oscilação oral de alta frequência (OOAF, drenagem autógena (DA e o ciclo ativo da respiração (CAR, apresenta eficácia tanto no tratamento como na manutenção da qualidade de vida dos pacientes com FC.

  14. Isometric and isokinetic muscle strength in patients with fibrositis syndrome. New characteristics for a difficult definable category of patients

    DEFF Research Database (Denmark)

    Jacobsen, Søren; Danneskiold-Samsøe, B

    1987-01-01

    A common complaint among patients with fibrositis syndrome is exhaustion and fatique. It was therefore felt desirable to evaluate the muscle strength of these patients compared with normal subjects. Maximum isometric and isokinetic strength of knee extension was measured in 15 patients and 15...... healthy matched subjects, using an isokinetic dynamometer (Cybex II). Maximum isometric strength at various knee extension angles (90 degrees, 60 degrees and 30 degrees degrees) was significantly (p less than 0.001) lower in the fibrositis group than in controls, a reduction of approximately 58......-66%. Maximum isokinetic strength at various knee extension velocities (30-240 degrees per second) was also significantly (p less than 0.01) lower in the fibrositis group than in controls, the reduction being approximately 41-51%. In conclusion, isometric and isokinetic muscle strength is found to be lower...

  15. O perfil dos cuidadores de pacientes pediátricos com fibrose cística

    OpenAIRE

    Alves, Stella Pegoraro; Bueno, Denise

    2018-01-01

    Resumo O objetivo desse estudo foi identificar o perfil do cuidador principal de pacientes pediátricos com Fibrose Cística (FC). Estudo transversal, descritivo e prospectivo, no qual foi realizado, durante consulta farmacêutica, entrevista com cuidadores de pacientes fibrocísticos acompanhados em um centro de referência de um Hospital Universitário do Sul do Brasil. Foram obtidas informações gerais sobre os cuidadores, sobre o entendimento da doença, os medicamentos em uso do paciente e a din...

  16. Fibrosing mediastinitis and thrombosis of superior vena cava associated with Behcet's disease

    International Nuclear Information System (INIS)

    Harman, Mustafa; Sayarlioglu, Mehmet; Arslan, Halil; Ayakta, Hayati; Harman, Ece

    2003-01-01

    We present CT, MRI and venography findings in 13-year boy with mediastinal fibrosis and superior vena cava (SVC) thrombosis associated with Behcet's disease. Fibrosing mediastinitis is an excessive fibrotic reaction that occurs in the mediastinum and may lead to compression of mediastinal structures (especially vascular). This condition is usually idiopathic, though many (and perhaps most) cases in the USA are thought to be caused by an abnormal immunologic response to Histoplasma capsulatum infection. SVC syndrome secondary to extrinsic compression by mediastinal fibrosis combined with Behcet's disease has rarely been described. Radiological investigations of this syndrome are necessary to avoid a useless anticoagulant therapy

  17. Lethal fibrosing mediastinitis in a child possibly due to Mycobacterium tuberculosis.

    Science.gov (United States)

    Goussard, Pierre; Gie, Robert P; Janson, Jacque

    2018-04-10

    Fibrosing mediastinitis (FM) is a rare benign disease resulting from an excessive fibro-inflammatory reaction in the mediastinum that can compress and occlude mediastinal structures. There is a granulomatous and a diffuse non-granulomatous form of FM. We present a case of granulomatous FM following possible tuberculosis in a young child who presented with severe compression of the airways, pulmonary artery and the superior vena cava (SVC), unresponsive to treatment, resulting in death. Bronchoscopic findings included concentric narrowing and mucosal hyperaemia of the tracheobronchial airways. This case raises awareness of this rare complication and the limited treatment options available. © 2018 Wiley Periodicals, Inc.

  18. Bilateral Breast Enlargement: An Unusual Presentation of Superior Vena Cava Obstruction in a Hemodialysis Patient with Fibrosing Mediastinitis

    International Nuclear Information System (INIS)

    Goo, Dong Erk; Kim, Yong Jae; Choi, Deuk Lin; Kwon, Kui Hyang; Yang, Seung Boo

    2011-01-01

    A 67-year-old woman with end-stage renal disease presented with profound edema of both breasts. The presence of a patent hemodialysis basilic transposition fistula and superior vena cava obstruction (SVC), due to fibrosing mediastinitis, was demonstrated by the use of fistulography. Endovascular treatment with a balloon and stent caused immediate resolution of the breast edema.

  19. Multifocal fibrosing thyroiditis: report of 55 cases of a poorly recognized entity.

    Science.gov (United States)

    Fellegara, Giovanni; Rosai, Juan

    2015-03-01

    During the course of our consultation activity, we have recognized a peculiar form of thyroiditis in which multiple foci of fibrosis, most of which were associated with reactive atypia of the surrounding follicles, are present. We have referred to this condition, both in our consultation reports and in the third series of A.F.I.P. Fascicle on Tumors of the Thyroid Gland, as "multifocal fibrosing thyroiditis" or (less frequently) "multifocal sclerosing thyroiditis," which are descriptive terms that highlight the benign/inflammatory nature of the process, its multiplicity, and its unknown pathogenesis. The aim of this study is to better define the morphologic features of this process and correlate it with some clinical data. With this purpose, the consultation files of one of the authors (J.R.) were searched for cases coded as multifocal fibrosing thyroiditis or multifocal sclerosing thyroiditis in a 20-year period ranging from January 1989 to December 2009. A total of 55 cases were identified that displayed the above-listed features. There were 51 (93%) female and 4 (7%) male patients (F/M=12.75), with ages ranging between 15 and 71 years (mean age, 47.03 y; median age, 44.5 y). Microscopically, multiple foci of fibrosis were identified in all cases, their number ranging from 2 to 51 per case (mean number, 16), with a mean diameter of 3 mm (range: 0.36 to 15.1 mm). Although heterogenous in shape and size, the individual foci were rather similar to each other in composition, being characterized by a fibrotic poorly cellular center that merged with a cellular peripheral zone. Some of the follicular structures present at the periphery of the scar and-to a lesser extent-those entrapped inside it underwent complex reactive and regenerative (atypical) changes that simulated malignancy. We discuss the differential diagnosis with other benign and malignant thyroid conditions and speculate about its pathogenesis and possible relationship with papillary thyroid microcarcinoma.

  20. Exacerbação aguda da fibrose pulmonar idiopática

    Directory of Open Access Journals (Sweden)

    Natália Melo

    2009-03-01

    Full Text Available Resumo: Alguns doentes com fibrose pulmonar idiopática (FPI apresentam durante a sua evolução fases de agravamento clínico sem causa conhecida, designadas como “exacerbação aguda” ou “fase acelerada” da doença (EA. Caracterizam-se pelo agravamento marcado da dispneia, hipoxemia e pelo aparecimento de novas opacidades pulmonares ou pelo agravamento das já existentes no estudo imagiológico. Os achados histológicos típicos são o dano alveolar difuso (DAD sobreposto a alterações de pneumonia intersticial usual (UIP. Esta entidade clínica associa-se a uma mortalidade elevada, não havendo até ao momento nenhuma terapêutica de comprovada eficácia.Os autores descrevem os casos clínicos de cinco doentes que apresentaram alterações clínicas, funcionais e radiológicas sugestivas de EA-FPI, assim como o tratamento efectuado e a evolução observada, enquadrando-os na discussão das características normalmente apresentadas por esta entidade. Abstract: Some patients with Idiopathic Pulmonary Fibrosis (IPF have disease accelerated deterioration without identifiable cause referred as “acute exacerbation” or “accelerated stage”. It is characterized by severe worsening of dyspnea, hypoxemia and new or progressive opacities on imaging studies. The typical histological findings are diffuse alveolar damage in addition to the features of usual interstitial pneumonia pattern. Mortality in this clinical entity is very high and no efficacious therapeutic have been described.The authors describe the clinical, functional and radiological features, treatment and evolution of five patients with IPF acute exacerbation. A discussion will be carry out concerning the IPF acute exacerbation usual features comparing with the alterations noticed in those patients. Palavras-chave: Fibrose pulmonar idiopática, exacerbação aguda, Key words: Idiopathic

  1. Fibrose quística: Revisão

    Directory of Open Access Journals (Sweden)

    C. Damas

    2008-01-01

    Full Text Available Resumo: A fibrose quística (FQ é a doença autossómica recessiva mais frequente na raça caucasiana. Caracteriza-se por mutações na CFTR, uma proteína transmembranar responsável pelo transporte de cloretos. Esta proteína tem uma ampla distribuição epitelial, o que dá um carácter sistémico a esta doença e consequentemente múltiplas manifestações clínicas de gravidade variável. A melhoria dos cuidados de saúde, associada ao desenvolvimento do arsenal terapêutico, permitiu um aumento da sobrevida destes doentes, de tal forma que a FQ já não pode ser abordada como doença da idade pediátrica. Também a evolução técnica na transplantação abriu novas perspectivas quanto ao tratamento desta afecção.Assim, cada vez mais esta patologia implica um envolvimento multidisciplinar no qual a pneumologia tem uma parte preponderante. Abstract: Cystic Fibrosis (CF is the recessive autossomic disease more frequent in the caucasian race. The main characteristic is a mutation in CFTR, a trans-membranar protein, responsible for chlorates transportation. This protein has a diffuse epithelial distribution, which gives a multissistemic involvement to this disease, with clinical manifestations with variable degrees of severity. The development of better health care associated with new therapeutic options became responsible for an increase of survival, so CF is no longer a paediatric disease. Lung transplantation also has an important role, bringing new treatment perspectives.So, this pathology has an increased multi-disciplinary involvement in which the pulmonologist have a preponderant role. Palavras-chave: Fibrose quística, revisão, Key-words: Cystic fibrosis, review

  2. Traction bronchiectasis in cryptogenic fibrosing alveolitis: associated computed tomographic features and physiological significance

    International Nuclear Information System (INIS)

    Desai, Sujal R.; Wells, Athol U.; Bois, Roland M. du; Rubens, Michael B.; Hansell, David M.

    2003-01-01

    Our objective was to evaluate the associated CT features and physiological consequences of traction bronchiectasis in patients with cryptogenic fibrosing alveolitis (CFA). The CT scans of 212 patients with CFA (158 men, 54 women; mean age 62.2±10.6 years) were evaluated independently by two observers. The extent of fibrosis, the proportions of a reticular pattern and ground-glass opacification and the extent of emphysema were scored at five levels. The predominant CT pattern, coarseness of a reticular pattern and severity of traction bronchiectasis were graded semiquantitatively. Physiological indices were correlated with CT features. There was traction bronchiectasis on CT in 202 of 212 (95%) patients. Increasingly severe traction bronchiectasis was independently associated with increasingly extensive CFA (p CO (p 2 (p<0.0005), but not indices of air-flow obstruction. In CFA, traction bronchiectasis increases with more extensive disease, a lower proportion of ground-glass opacification and a coarser reticular pattern, but it decreases with concurrent emphysema. Increasingly severe traction bronchiectasis is associated with additional physiological impairment for a given extent of pulmonary fibrosis and emphysema. (orig.)

  3. Multifocal fibrosing thyroiditis and its association with papillary thyroid carcinoma using BRAF pyrosequencing.

    Science.gov (United States)

    Frank, Renee; Baloch, Zubair W; Gentile, Caren; Watt, Christopher D; LiVolsi, Virginia A

    2014-09-01

    Multifocal fibrosing thyroiditis (MFT) is characterized by numerous foci of fibrosis in a stellate configuration with fibroelastotic and fibroblastic centers entrapping epithelial structures. MFT has been proposed as a risk factor for papillary thyroid carcinoma (PTC) development. We attempted to identify whether MFT showed such molecular changes and could possibly be related to PTC. We identified seven cases of PTC with MFT in our institutional pathology database and personal consult service of one of the authors (VAL) for the years 1999 to 2012. Areas of PTC, MFT, and normal tissue were selected for BRAF analysis. Macro-dissection, DNA extraction and PCR amplification, and pyrosequencing were performed to detect BRAF mutations in codon 600. All of the MFT lesions and normal thyroid tissue were negative for BRAF mutations. Of the seven PTCs analyzed, five (71 %) were negative for BRAF mutations, while two cases were positive. In our study, none of the MFT lesions harbored BRAF mutations, whereas 29 % (two of seven) PTCs in the same gland were positive. Hence, in this small study, we found no evidence that the MFT lesion is a direct precursor to PTC. It is likely an incidental bystander in the process and a reflection of the background thyroiditis.

  4. Traction bronchiectasis in cryptogenic fibrosing alveolitis: associated computed tomographic features and physiological significance

    Energy Technology Data Exchange (ETDEWEB)

    Desai, Sujal R. [Department of Radiology, King' s College Hospital, Denmark Hill, SE5 9RS, London (United Kingdom); Wells, Athol U.; Bois, Roland M. du [Interstitial Lung Disease Unit, Royal Brompton Hospital, Emmanuel Kaye Building, Manresa Road, Fulham, SW6 6LR, London (United Kingdom); Rubens, Michael B.; Hansell, David M. [Department of Radiology, Royal Brompton Hospital, Sydney Street, SW3 6NP, London (United Kingdom)

    2003-08-01

    Our objective was to evaluate the associated CT features and physiological consequences of traction bronchiectasis in patients with cryptogenic fibrosing alveolitis (CFA). The CT scans of 212 patients with CFA (158 men, 54 women; mean age 62.2{+-}10.6 years) were evaluated independently by two observers. The extent of fibrosis, the proportions of a reticular pattern and ground-glass opacification and the extent of emphysema were scored at five levels. The predominant CT pattern, coarseness of a reticular pattern and severity of traction bronchiectasis were graded semiquantitatively. Physiological indices were correlated with CT features. There was traction bronchiectasis on CT in 202 of 212 (95%) patients. Increasingly severe traction bronchiectasis was independently associated with increasingly extensive CFA (p<0.0005), a coarser reticular pattern (p<0.001), a lower proportion of ground-glass opacification (p<0.005) and less extensive emphysema (p<0.0005). Increasingly severe traction bronchiectasis was independently related to depression of DL{sub CO} (p<0.005), FVC (p=0.02) and pO{sub 2} (p<0.0005), but not indices of air-flow obstruction. In CFA, traction bronchiectasis increases with more extensive disease, a lower proportion of ground-glass opacification and a coarser reticular pattern, but it decreases with concurrent emphysema. Increasingly severe traction bronchiectasis is associated with additional physiological impairment for a given extent of pulmonary fibrosis and emphysema. (orig.)

  5. Histopathologic Overlap between Fibrosing Mediastinitis and IgG4-Related Disease

    Directory of Open Access Journals (Sweden)

    Tobias Peikert

    2012-01-01

    Full Text Available Fibrosing mediastinitis (FM and IgG4-related disease (IgG4-RD are two fibroinflammatory disorders with potentially overlapping clinical and radiological features. In this paper, we looked for histopathologic features of IgG4-RD and enumerated infiltrating IgG4-positive plasma cells within mediastinal tissue biopsies from FM patients. We identified 15 consecutive FM surgical mediastinal tissue biopsies between 1985 and 2006. All patients satisfied the clinical and radiological diagnostic criteria for FM. All patients had either serological or radiological evidence of prior histoplasmosis or granulomatous disease, respectively. Formalin-fixed paraffin-embedded tissue sections of all patients were stained for H&E, IgG, and IgG4. Three samples met the predefined diagnostic criteria for IgG4-RD. In addition, characteristic histopathologic changes of IgG4-RD in the absence of diagnostic numbers of tissue infiltrating IgG4-positive plasma cells were seen in a number of additional cases (storiform cell-rich fibrosis in 11 cases, lymphoplasmacytic infiltrate in 7 cases, and obliterative phlebitis/arteritis in 2 cases. We conclude that up to one-third of histoplasmosis or granulomatous-disease-associated FM cases demonstrate histopathological features of IgG4-RD spectrum. Whether these changes occur as the host immune response against Histoplasma or represent a manifestation of IgG4-RD remains to be determined. Studies to prospectively identify these cases and evaluate their therapeutic responses to glucocorticoids and/or other immunosuppressive agents such as rituximab are warranted.

  6. Morphologic and molecular study of lung cancers associated with idiopathic pulmonary fibrosis and other pulmonary fibroses.

    Science.gov (United States)

    Guyard, Alice; Danel, Claire; Théou-Anton, Nathalie; Debray, Marie-Pierre; Gibault, Laure; Mordant, Pierre; Castier, Yves; Crestani, Bruno; Zalcman, Gérard; Blons, Hélène; Cazes, Aurélie

    2017-06-15

    Primitive lung cancers developed on lung fibroses are both diagnostic and therapeutic challenges. Their incidence may increase with new more efficient lung fibrosis treatments. Our aim was to describe a cohort of lung cancers associated with idiopathic pulmonary fibrosis (IPF) and other lung fibrotic disorders (non-IPF), and to characterize their molecular alterations using immunohistochemistry and next-generation sequencing (NGS). Thirty-one cancer samples were collected from 2001 to 2016 in two French reference centers for pulmonary fibrosis - 18 for IPF group and 13 for non-IPF group. NGS was performed using an ampliseq panel to analyze hotspots and targeted regions in 22 cancer-associated genes. ALK, ROS1 and PD-L1 expressions were assessed by immunohistochemistry. Squamous cell carcinoma was the most frequent histologic subtype in the IPF group (44%), adenocarcinoma was the most frequent subtype in the non-IPF group (62%). Forty-one mutations in 13 genes and one EGFR amplification were identified in 25 samples. Two samples had no mutation in the selected panel. Mutations were identified in TP53 (n = 20), MET (n = 4), BRAF (n = 3), FGFR3, PIK3CA, PTEN, STK11 (n = 2), SMAD4, CTNNB1, DDR2, ERBB4, FBXW7 and KRAS (n = 1) genes. No ALK and ROS1 expressions were identified. PD-L1 was expressed in 10 cases (62%) with only one (6%) case >50%. This extensive characterization of lung fibrosis-associated cancers evidenced molecular alterations which could represent either potential therapeutic targets either clues to the pathophysiology of these particular tumors. These findings support the relevance of large molecular characterization of every lung fibrosis-associated cancer.

  7. Fibrosing variant of Hashimoto thyroiditis is an IgG4 related disease.

    Science.gov (United States)

    Deshpande, Vikram; Huck, Amelia; Ooi, Esther; Stone, John H; Faquin, William C; Nielsen, G Petur

    2012-08-01

    Hashimoto thyroiditis (HT) and the fibrosing variant of Hashimoto thyroiditis (FVHT) are immune-mediated tumefactive lesions of the thyroid. Immunoglobulin G4-related disease (IgG4-RD) is now a widely recognised multi-organ system disease characterised by elevated serum and tissue concentrations of IgG4. In this study, the authors address several unresolved questions pertaining to the relationship between HT and FVHT, and the association of each of these diseases with IgG4-RD. The authors evaluated 28 consecutive cases of HT and nine cases of FVHT. The clinical, demographic and serological data were recorded. The slides were stained immunohistochemically using antibodies to IgG4 and IgG and the quantitative analysis was recorded. Data on thyroid function tests were available on seven cases of FVHT and 14 cases of HT. Based on the availability of data, hypothyroidism was noted in 62% (9/14) of HT and 86% of FVHT (6/7). FVHT demonstrated an exaggerated lobular pattern with lobules separated by cellular storiform-type fibrosis, resembling fibrosis seen in other forms of IgG-RD. The median IgG4 counts per high power field (×40) in HT and FVHT were 2.3 and 22, respectively. The median IgG4:IgG ratios in HT and FVHT were 0.11 and 0.58, respectively. The authors propose that FVHT belongs to the spectrum of IgG4-RD. Although a proportion of cases of HT show elevated numbers of IgG4 positive plasma cells, these cases lack the histological features typically associated with IgG4-RD, and thus the relationship between HT and IgG4-RD remains unproven.

  8. ESTUDO BIOQUÍMICO DO POTENCIAL DA MEMBRANA AMNIÓTICA NA FIBROSE HEPÁTICA INDUZIDA EM RATOS

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    Shaine Silva Maciel

    2016-11-01

    Full Text Available A fibrose hepática causada por obstrução do ducto biliar induz alterações tanto na estrutura como na função do fígado, e pode levar a cirrose e insuficiência hepática, quando não tradada. A membrana amniótica humana (MA devido às suas propriedades anti-inflamatórias e anti-fibróticas pode ser uma terapia alternativa. A proposta desse trabalho é analisar as alterações nos níveis séricos das principais enzimas celulares e da albumina, na fibrose hepática induzida pela ligadura do ducto biliar (LDB e após o tratamento com a MA. Dez ratos foram divididos nos grupos LDB e LDB+MA, e eutanasiados após 9 semanas da LDB. As amostras de sangue foram processadas bioquimicamente para análise da albumina (ALB, fosfatase alcalina (FA e transaminases (TGP e TGO.  Comparando os grupos experimentais verificou-se que no grupo LDB+MA houve a diminuição da ALB e TGP e no grupo LDB houve a diminuição da FA e TGO, entretanto sem apresentar diferença significante entre os grupos. Conclui-se que a MA, quando aplicada ao fígado no mesmo momento da indução da fibrose, parece não ter exercido efeito significativo na função hepática.

  9. Fibrose idiopática do mediastino: A propósito de um caso clínico

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    Maria João Canotilho

    2005-03-01

    Full Text Available Resumo: Descreve-se o caso clínico de um doente do sexo masculino com uma massa mediastínica de crescimento lento. Após terem-se efectuado vários exames complementares, foi através de cirurgia que se confirmou o diagnóstico de fibrose idiopática do mediastino. A remoção parcial da massa permitiu uma estabilização transitória das suas dimensões. Cerca de quatro anos depois, verificou-se um crescimento marcado com repercurssões importantes, através da inevitável compressão de estruturas vitais, que conduziram a hipertensão pulmonar grave. A fibrose idiopática do mediastino é uma entidade extremamente rara, estando descritas associações a outras patologias, mais frequentemente às auto-imunes, mas apresenta sempre um curso fatal quando não é possível a cirurgia.Rev Port Pneumol 2005; XI (2: 155-163 Abstract: We herein report a case of a male patient, who presented a mediastinal mass that had a slow growth over the years. After surgery, that made the diagnosis of idiopathic mediastinal fibrosis, the growth stabilized. Four years latter it was observed a substantial increase which lead to the inevitable compression of vital structures which, in turn, led to pulmonary hypertension. Idiopathic mediastinal fibrosis is an extremely rare pathology. There are same cases in which it is associated with other pathologies but has always a fatal prognosis when surgery is not an option.Rev Port Pneumol 2005; XI (2: 155-163 Palavras-chave: Fibrose do mediastino, síndroma da veia cava superior, hipertensão pulmonar, mediastinite fibrosante, Key words: Mediastinal fibrosis, superior vena cava syndrome, pulmonary hypertension, fibrosis mediastinites

  10. Síndrome mieloproliferativa transitória associada à trissomia do 21 e fibrose hepática

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    Sant'Anna Anna L.

    2002-01-01

    Full Text Available Recém-nascidos com Síndrome de Down (SD podem apresentar uma proliferação transitória de células imaturas no sangue periférico e medula óssea. A leucometria pode estar muito elevada, impossibilitando o diagnóstico diferencial com leucemia mielóide aguda (LMA. Em contraste com a LMA, a SMT regride espontaneamente em quatro a oito semanas. Objetivo: Apresentar uma criança com SD, SMT e fibrose hepática, que resultou num prognóstico desfavorável. Relato do Caso: D.M.S., masculino, estigmas da SD, hepatoesplenomegalia, sopro sistólico. Hemograma: 95.000 leucócitos/mm³, 19% blastos, 170.000 plaquetas/mm³, hemoglobina 16,2g/dL. Bilirrubina total 35,86 mg/dL, GOT 184 UI, GPT 122 UI. Ecocardiograma: canal átrio-ventricular total, hipertensão pulmonar, persistência do canal arterial. Sorologias negativas. Biópsia hepática: colestase, fibrose portal e sinusoidal, elementos mielóides imaturos. Após normalização da leucometria, manteve plaquetopenia e disfunção hepática. No 50º dia de vida, quimioterapia com Daunoblastina e Citarabina. Evoluiu com pneumonia e insuficiência renal. Óbito no 61ºdia. Comentários: A história natural da SMT gera questões intrigantes a respeito de sua origem, evolução e desenvolvimento de leucemia subseqüente. A disfunção hepática e a fibrose têm impacto prognóstico. Em relato anterior, de cada oito casos de SMT com disfunção hepática, seis evoluíram para óbito, sendo que fibrose hepática difusa, associada a eritropoese extra-medular, foi encontrada em quatro casos. Acredita-se que a lesão hepática resulta da produção de citocinas pelos megacariócitos, por ser o fígado o segundo órgão hematopoético de células anormais, após a medula óssea.

  11. Fibrose cística: uma abordagem clínica e nutricional Cystic fibrosis: a clinical and nutritional approach

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    Fernanda Ribeiro Rosa

    2008-12-01

    Full Text Available A fibrose cística é a doença genética letal mais comum em populações caucasianas e é caracterizada por infecções crônicas e recorrentes do pulmão, insuficiência pancreática e elevados níveis de cloro no suor. É uma doença de herança autossômica recessiva causada pela mutação no gene do Regulador da Condutância Transmembrana da Fibrose Cística, que induz o organismo a produzir secreções espessas e viscosas que obstruem os pulmões, o pâncreas e o ducto biliar. Muitos pacientes apresentam insuficiência pancreática, que leva a má-absorção de nutrientes especialmente de proteínas e lipídeos e a complicações gastrintestinais tais como prolapso retal, síndrome da obstrução intestinal, constipação e cirrose hepática. A fibrose cística normalmente é diagnosticada na infância, pelos programas de triagem neonatal ou pelo teste do suor. Devido aos vários sistemas envolvidos e à variabilidade e cronicidade da doença, uma abordagem multidisciplinar é essencial para auxiliar o paciente e sua família a compreenderem a doença e aderirem ao tratamento. A terapia atual da fibrose cística inclui a manutenção do estado nutricional, a remoção das secreções das vias aéreas com fisioterapia e mucolíticos, o uso de antibióticos para prevenção e tratamento de infecções, a prescrição de suplementos energéticos, dietas hiperlípidicas e hiperprotéicas, bem como a suplementação de minerais e vitaminas lipossolúveis. O objetivo deste trabalho foi realizar breve revisão de literatura sobre os aspectos clínicos e nutricionais da fibrose cística.Cystic fibrosis is the most common, lethal, genetic disease in Caucasian populations and is characterized by chronic and recurrent lung infections, pancreatic insufficiency and high sweat chloride levels. It is an autosomal recessive hereditary disease caused by a mutation in the cystic fibrosis transmembrane conductance regulator gene which induces the body to

  12. Fibrose pulmonar idiopática: uma década de progressos Idiopathic pulmonary fibrosis: a decade of progress

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    Jeffrey J. Swigris

    2006-06-01

    Full Text Available Embora diagnósticos de fibrose pulmonar idiopática continuem sendo devastadores, avanços recentes têm melhorado nossa compreensão a respeito de muitas das facetas desta doença. Estas descobertas, juntamente com o aumento da disponibilidade geral de ensaios terapêuticos, encerram a promessa de um futuro mais promissor para pacientes com fibrose pulmonar idiopática. Por exemplo, nós temos agora uma compreensão mais abrangente a respeito dos critérios diagnósticos e da história natural da doença. Vários estudos têm mostrado que a mensuração simples da fisiologia pulmonar ou troca gasosa pode ser usada para prever a sobrevida do paciente. Através da identificação de várias vias moleculares que têm papéis importantes na patogênese da fibrose pulmonar idiopática, os pesquisadores têm produzido uma lista crescente de possíveis novos alvos terapêuticos para a doença. Vários ensaios terapêuticos prospectivos e controlados têm sido realizados. Outros estão em andamento ou ainda estão em fase de planejamento. Estes esforços têm avançado nosso conhecimento atual sobre fibrose pulmonar idiopática e levantado novas questões importantes, assim como têm gerado o interesse e o impulso necessários para avançar terreno na luta contra esta doença desafiadora. Este artigo oferece ao leitor um panorama dos avanços recentes nas pesquisas sobre fibrose pulmonar idiopática, tendo como foco a história natural, patogênese e tratamento.Although idiopathic pulmonary fibrosis remains a devastating diagnosis, recent advances have improved our understanding of many facets of this disease. These breakthroughs, combined with the increased general availability of therapeutic trials, hold the promise of a brighter future for idiopathic pulmonary fibrosis patients. For example, we now have a more comprehensive understanding of the diagnostic criteria and natural history of the disease. Several studies have shown that simple

  13. C14. Um caso de polimiosite, fibrose pulmonar e cancro do pulmão

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    Carlos Lopes

    2003-11-01

    Full Text Available Os autores apresentam o caso clínico de um doente do sexo masculino, de 67 anos, fumador (50 UMA. Trabalhou na marinha durante 30 anos, onde esteve exposto a fibras de asbestos.Aparentemente estável até aos 62 anos, altura em que refere início de quadro clínico de dispneia de esforço, de agravamento progressivo. Um ano depois episódio de febre, tosse produtiva e mialgias.Ao exame objectivo eram evidentes hipocratismo digital e fervores subcrepitantes em ambas as bases, na auscultação torácica.Os aspectos vizualizados na TC Torácica eram compatíveis com Pneumonia Intersticial Usual e o lavado broncoalveolar excluiu outras doenças.O estudo da função respiratória mostrou um síndrome restritivo com diminuição da capacidade de difusão do CO.Foi administrada terapêutica com Deflazacort, acetilcisteína e azatioprina. Devido a agravamento clínico e funcional, azatioprina foi substituída por ciclofosfamida. Dois anos depois foi iniciada terapêutica com Interferão Gamma 1b, com melhoria clínica e funcional, durante um ano.Foi então feito o diagnóstico de Polimiosite e recebeu terapêutica com imunoglobulina.Doze meses depois foi internado com quadro clínico de febre intermitente, tosse seca, dispneia de esforço de agravamento progressivo e mialgias. A TC Torácica revelou pulmão em favo e bronquiectasias. No lobo inferior esquerdo foi detectado um nódulo periférico. O doente foi submetido a biópsia aspirativa transtorácica, o padrão imunohistoquímico do tecido revelou Cancro do Pulmão de Pequenas Células. No estadiamento foram identificadas adenopatias hilares e mediastínicas e múltiplas metástases hepáticas. Apenas foi efectuada uma administração de quimioterapia (carboplatinum e etoposido, sem qualquer tipo de resposta. O doente faleceu duas semanas depois.Está documentada a associação de Polimiosite com várias Neoplasias, neste caso clínico, o doente desenvolveu fibrose pulmonar antes de ter sido feito

  14. Two different trichoscopic patterns of mid-frontal scalp in patients with frontal fibrosing alopecia and clinical features of androgenetic alopecia

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    Adriana Rakowska

    2017-03-01

    Full Text Available Introduction . Frontal fibrosing alopecia is a primary lymphocytic cicatricial alopecia with progressive frontotemporal hairline recession. In some cases, hair loss in the mid-frontal scalp, similar to female pattern hair loss, may be observed. Objective. Assessment of the trichoscopic pattern of mid-frontal scalp hair loss in patients diagnosed with frontal fibrosing alopecia. Material and methods. The retrospective analysis included 31 women diagnosed with frontal fibrosing alopecia and hair loss in the mid-frontal scalp and 36 women diagnosed with female pattern hair loss. Results . In patients with frontal fibrosing alopecia two different trichoscopic patterns in the mid-frontal scalp were identified. In 68% of patients (21/31 we observed a diffuse fibrotic pattern. It was characterized by irregular arrangement of follicular units with small areas with loss of follicular units, an increased percentage of follicular units with one hair and a decreased percentage of follicular units with three hairs, normal hair shaft thickness and presence of mild perifollicular scaling. The androgenetic alopecia pattern was present in 32% of patients (10/31. It was characterized by hair shaft thickness diversity (20% or more, a percentage of vellus hairs higher than 10%, presence of yellow dots, an increased percentage of follicular units with one hair and a decreased percentage of follicular units with three hairs. Conclusions. In patients with frontal fibrosing alopecia and coexisting mid-frontal scalp hair loss, we identified two different patterns of this area in trichoscopy: the diffuse fibrotic pattern (more common and the androgenetic alopecia pattern. This observation may have therapeutic and prognostic implications.

  15. Fibrose quística em adultos Cystic fibrosis in adults

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    C Damas

    2007-05-01

    Full Text Available Os autores efectuaram uma revisão de doentes adultos com fibrose quística (FQ, seguidos na consulta de Pneumologia no período de 1994-2004 (n=8: cinco mulheres e três homens, com idades compreendidas entre 20 e 34 anos (mediana = 27 anos, cuja idade de diagnóstico variou entre os 18 meses e os 31 anos. O diagnóstico foi obtido por prova de suor (positiva em seis doentes e estudo genético (homozigotia para a mutação ÄF508 em quatro doentes. O atingimento respiratório traduziu-se por sinusite e bronquiectasias, acompanhando-se o envolvimento pulmonar por alterações funcionais e também gasométricas na maioria dos doentes, ainda que de gravidade variável e não relacionada com a idade de diagnóstico. Verificou-se colonização da árvore brônquica em cinco doentes: Pseudomonas aeruginosa em quatro e Staphilococcus aureus em quatro (verificando-se colonização concomitante por estes agentes em três. As principais causas de exacerbação foram infecções respiratórias e hemoptises. Quanto ao atingimento não respiratório, quatro doentes apresentavam envolvimento digestivo (com cirrose hepática num caso, um insuficiência renal em hemodiálise, e em apenas um foi efectuado espermograma para documentação de infertilidade. Quatro doentes tinham osteopenia documentada por densitometria óssea. As medidas terapêuticas mais utilizadas foram a cinesiterapia, os broncodilatadores, a alfa-dornase, mucolíticos, suplementos vitamínicos e enzimáticos, antibioterapia e oxigenoterapia. Relativamente à evolução, uma doente abandonou a consulta, uma doente faleceu, um doente aguardava transplante pulmonar e os restantes mantiveram as suas características clínicas habituais. Neste grupo, a gravidade da doença pulmonar não se relacionou com o diagnóstico mais tardio, o que se pode dever à diversidade de apresentação fenotípica da FQ na idade adulta.The authors reviewed adult cystic fibrosis patients followed in the Pulmonology

  16. Estado nutricional em pacientes atendidos por um programa de adultos para fibrose cística

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    Bruna Ziegler

    2008-02-01

    Full Text Available Introdução: Na fibrose cística (FC, o estado nutricional está associado com o crescimento, função pulmonar e índices de so-brevida. Objetivo: Avaliar o estado nutricional em adultos com FC e correlacionar com escore clínico, escore radiológico, pressões respiratórias estáticas máximas, capacidade submáxima de exercício e função pulmonar. Metodologia: O estudo realizado foi transver-sal e prospectivo, em pacientes (16 anos ou mais, atendidos em um programa para adultos com FC. Os pacientes foram submetidos a uma avaliação nutricional e clínica, ao teste de caminhada de seis minutos (TC6, à medida das pressões respiratórias máximas, a espirometria e exame radiológico do tórax. Resultados: O estudo incluiu 41 pacientes com media de idade de 23,7 ± 6,5 anos e média de índice de massa corporal (IMC de 20,2 ± 2,2 Kg/m2. Vinte e seis pacientes (63,4% foram classificados como bem nutridos (IMC = 21,7 ± 2,0 Kg/m2, 6 (14,6% como risco nutricional (IMC = 19,4 ± 0,5 Kg/m2 e 9 (22% como desnutridos (IMC = 17,6 ± 0,8 Kg/m2. Não houve correlação significativa entre IMC e a idade (r = 0,24; P = 0,13, idade do diagnóstico (r = -0,04; P = 0,81, escore clínico (r = 0,13; P = 0,40 e radiográfico (r = -0,22; P = 0,17, distância percorrida no TC6 (r = 0,20; P = 0,20, VEF1 % (r = 0,11; P = 0,50 e CVF % (r = 0,06; P = 0,72. Também não houve associação entre o declínio do estado nutricional e essas variáveis. Conclusão: Este estudo mos-trou que a maioria dos pacientes com FC (16 anos ou mais tem um adequado estado nutricional (63,4%, mas uma porcentagem signi-ficante (36,6% tem depleção nutricional. Não houve associação entre o estado nutricional e a função pulmonar, pressões respiratórias máximas, escore clínico e radiográfico e a capacidade submáxima de exercício.

  17. Uterine prolapse with endometrial eversion in association with an unusual diffuse, polypoid, fibrosing perimetritis and parametritis in a cat.

    Science.gov (United States)

    Valentine, Matthew J; Porter, Susan; Chapwanya, Aspinas; Callanan, John J

    2016-01-01

    This case describes a young non-pregnant cat that presented with uterine prolapse in association with an unusual diffuse, polypoid, fibrosing perimetritis and parametritis. Following ovariohysterectomy the cat recovered fully. No intra-abdominal complications were seen on ultrasound examination 3 months postsurgery. At the time of writing, the cat remains healthy. Uterine prolapse in the cat is relatively rare and usually associated with the periparturient period. Inflammatory polypoid perimetritis and parametritis have not previously been documented in cats, and in dogs have only been reported in association with the administration of oestrogenic compounds. The polypoid inflammation affecting the uterus and parametrium may have contributed to increased laxity of the uterine ligaments and predisposed to the development of uterine prolapse.

  18. Recurrent Pneumonia due to Fibrosing Mediastinitis in a Teenage Girl: A Case Report with Long-Term Follow-Up

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    Avigdor Hevroni

    2018-01-01

    Full Text Available A teenage girl was evaluated for recurrent right pneumonia. The evaluation revealed a calcified mediastinal mass that compressed the right intermediate and middle lobar bronchi, as well as the right pulmonary artery and veins. The clinical picture together with imaging studies and borderline positive serology testing suggested a diagnosis of fibrosing mediastinitis associated with histoplasmosis. This rare condition is characterized by the local proliferation of invasive fibrous tissue within the mediastinum due to a hyperimmune reaction to Histoplasma capsulatum. Antifungal and anti-inflammatory therapies are usually ineffective, and surgical intervention contains a high morbidity risk. Palliative surgery and stenting of the compressed airway have been suggested. In the past, the prognosis was thought to be poor, but recent studies demonstrate a more positive outcome. Our patient had been radiologically and functionally stable under follow-up for over thirteen years and has married and delivered two healthy children, both following an uneventful pregnancy.

  19. Relação entre Fibrose e Arritmias Ventriculares na Cardiopatia Chagásica sem Disfunção Ventricular

    OpenAIRE

    Eduardo Marinho Tassi; Marcelo Abramoff Continentino; Emília Matos do Nascimento; Basílio de Bragança Pereira; Roberto Coury Pedrosa

    2014-01-01

    Fundamento: Pacientes com doença de Chagas com alteração segmentar apresentam pior prognóstico independentemente da fração de ejeção ventricular esquerda. A ressonância magnética cardíaca é atualmente o melhor método para detecção de alteração segmentar e para avaliação de fibrose miocárdica. Objetivo: Quantificar a fibrose, por meio do realce tardio, pela ressonância magnética cardíaca, em pacientes com doença de Chagas com fração de ejeção ventricular esquerda preservada ou minimamente ...

  20. O luto pela perda da saúde: vivências de ser mãe de uma criança com Fibrose Cística

    OpenAIRE

    ALMEIDA, Nancy Limeira de

    2012-01-01

    Este estudo tem por objetivo compreender como as mães da criança com Fibrose Cística (FC) vivenciam o luto pela perda da saúde do seu filho, considerando que esta ocorrência representa uma ameaça de morte continua à vida da criança, quando da ausência da adesão do tratamento. A Fibrose Cística é uma doença crônica, genética, sem cura e potencialmente letal, com prognóstico reservado, que demanda tratamento de alto impacto e intenso cuidado. A estratégia metodológica fundamen...

  1. Diabetes melito: uma importante co-morbidade da fibrose cística Diabetes mellitus in patients with cystic fibrosis

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    Crésio de Aragão Dantas Alves

    2007-04-01

    Full Text Available Diabetes melito relacionado à fibrose cística (DMFC é a principal complicação extrapulmonar da fibrose cística. Atualmente, ele afeta 15-30% dos adultos com fibrose cística e sua prevalência tende a aumentar com o aumento da expectativa de vida desses pacientes. Esse trabalho tem por objetivo rever a fisiopatologia, morbidade, manifestações clínicas, diagnóstico e tratamento do DMFC. Uma pesquisa bibliográfica utilizou os bancos de dados Medline e Literatura Latino-Americana e do Caribe em Ciências da Saúde, selecionando artigos publicados nos últimos vinte anos. A insulinopenia secundária à destruição de células beta pancreáticas é o principal mecanismo causal, embora a resistência insulínica também possa estar presente. O DMFC apresenta características do diabetes melito tipo 1 e tipo 2 e tem início, em média, aos 20 anos de idade. Ele pode cursar com hiperglicemia em jejum, pós-prandial ou intermitente. As alterações do metabolismo glicêmico agravam o estado nutricional, aumentam a morbidade, diminuem a sobrevida e pioram a função pulmonar. As complicações microvasculares estão presentes, porém raramente observam-se as macrovasculares. A triagem para o DMFC deve ser anual, a partir dos 10 anos de idade, através do teste de tolerância oral à glicose e, em qualquer faixa etária, se houver perda ponderal inexplicada ou sintomatologia de diabetes. Pacientes hospitalizados também devem ser investigados e receber terapia insulínica se a hiperglicemia em jejum persistir além de 48 h. A insulina é o tratamento de escolha para o diabetes com hiperglicemia em jejum. Não existe consenso quanto ao tratamento do diabetes intermitente ou sem hiperglicemia de jejum. Não há orientações de restrições alimentares. O acompanhamento deve ser multidisciplinar.Cystic fibrosis-related diabetes (CFRD is the principal extra-pulmonary complication of cystic fibrosis, occurring in 15-30% of adult cystic fibrosis

  2. Relação entre Fibrose e Arritmias Ventriculares na Cardiopatia Chagásica sem Disfunção Ventricular

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    Eduardo Marinho Tassi

    2014-06-01

    Full Text Available Fundamento: Pacientes com doença de Chagas com alteração segmentar apresentam pior prognóstico independentemente da fração de ejeção ventricular esquerda. A ressonância magnética cardíaca é atualmente o melhor método para detecção de alteração segmentar e para avaliação de fibrose miocárdica. Objetivo: Quantificar a fibrose, por meio do realce tardio, pela ressonância magnética cardíaca, em pacientes com doença de Chagas com fração de ejeção ventricular esquerda preservada ou minimamente comprometida (> 45% e detectar padrões de dependência entre fibrose, alteração segmentar e fração de ejeção ventricular esquerda na presença de arritmia ventricular. Métodos: Foram realizados eletrocardiograma, teste ergométrico, Holter e ressonância magnética cardíaca em 61 pacientes, separados em três grupos: (1 eletrocardiograma normal e ressonância magnética cardíaca sem alteração segmentar; (2 eletrocardiograma alterado e ressonância magnética cardíaca sem alteração segmentar; e (3 ressonância magnética cardíaca com alteração segmentar independentemente de alteração no eletrocardiograma. Resultados: O número de pacientes com arritmia ventricular em relação ao número total de pacientes em cada grupo, a porcentagem de fibrose e a fração de ejeção ventricular esquerda foram, respectivamente: no primeiro grupo, 4/26, 0,74% e 74,34%; no segundo grupo, 4/16, 3,96% e 68,5%; e no terceiro grupo, 11/19, 14,07% e 55,59%. Arritmia ventricular foi encontrada em 31,1% dos pacientes. Aqueles com e sem arritmia ventricular apresentaram fração de ejeção ventricular esquerda média de 59,87% e 70,18%, respectivamente, e fibrose de 11,03% e 3,01%, respectivamente. Das variáveis alteração segmentar, grupos, idade, fração de ejeção ventricular esquerda e fibrose, a última foi a única significativa para a presença de arritmia ventricular, com ponto de corte de 11,78% para massa fibrosada (p < 0

  3. Frontal fibrosing alopecia: possible association with leave-on facial skin care products and sunscreens; a questionnaire study.

    Science.gov (United States)

    Aldoori, N; Dobson, K; Holden, C R; McDonagh, A J; Harries, M; Messenger, A G

    2016-10-01

    Since its first description in 1994, frontal fibrosing alopecia (FFA) has become increasingly common, suggesting that environmental factors are involved in the aetiology. To identify possible causative environmental factors in FFA. A questionnaire enquiring about exposure to a wide range of lifestyle, social and medical factors was completed by 105 women with FFA and 100 age- and sex-matched control subjects. A subcohort of women with FFA was patch tested to an extended British standard series of allergens. The use of sunscreens was significantly greater in the FFA group compared with controls. Subjects with FFA also showed a trend towards more frequent use of facial moisturizers and foundations but, compared with controls, the difference in frequencies just failed to reach statistical significance. The frequency of hair shampooing, oral contraceptive use, hair colouring and facial hair removal were significantly lower in the FFA group than in controls. Thyroid disease was more common in subjects with FFA than controls and there was a high frequency of positive patch tests in women with FFA, mainly to fragrances. Our findings suggest an association between FFA and the use of facial skin care products. The high frequency of sunscreen use in patients with FFA, and the fact that many facial skin care products now contain sunscreens, raises the possibility of a causative role for sunscreen chemicals. The high frequency of positive patch tests in women with FFA and the association with thyroid disease may indicate a predisposition to immune-mediated disease. © 2016 British Association of Dermatologists.

  4. A intersubjetividade no contexto da família de pessoas com fibrose cística

    Directory of Open Access Journals (Sweden)

    Geisa Santos Luz

    2012-04-01

    Full Text Available Trata-se de uma investigação qualitativa, com aproximação fenomenológica, que objetivou desvelar os processos intersubjetivos no contexto da família de pessoas com Fibrose Cística (FC. Desenvolveu-se entrevista com 14 famílias com portadores de FC, cadastrados na Associação Paranaense de Assistência à Mucoviscidose (AAMPR, residentes nas regiões Norte e Noroeste do estado do Paraná (PR, Brasil. Das suas falas foram apreendidas unidades de significado, agrupadas em três categorias: Família, portador de FC e o outro; Aspectos religiosos na vivência da FC na família; Portador de FC na vida escolar, afetiva e profissional. Os resultados evidenciaram que a experiência das famílias compartilhada com o outro foi crucial para o enfrentamento da doença. Conhecer os processos intersubjetivos que perpassam o âmago da família de pessoas acometidas pela FC possibilita um novo modo de atuar em Enfermagem.

  5. Role of endothelium in radiation-induced normal tissue damages; Role de l'endothelium dans les dommages radio-induits aux tissus sains

    Energy Technology Data Exchange (ETDEWEB)

    Milliat, F

    2007-05-15

    More than half of cancers are treated with radiation therapy alone or in combination with surgery and/or chemotherapy. The goal of radiation therapy is to deliver enough ionising radiation to destroy cancer cells without exceeding the level that the surrounding healthy cells can tolerate. Unfortunately, radiation-induced normal tissue injury is still a dose limiting factor in the treatment of cancer with radiotherapy. The knowledge of normal tissue radiobiology is needed to determine molecular mechanisms involved in normal tissue pathogenic pathways in order to identify therapeutic targets and develop strategies to prevent and /or reduce side effects of radiation therapy. The endothelium is known to play a critical role in radiation-induced injury. Our work shows that endothelial cells promote vascular smooth muscle cell proliferation, migration and fibro-genic phenotype after irradiation. Moreover, we demonstrate for the first time the importance of PAI-1 in radiation-induced normal tissue damage suggesting that PAI-1 may represent a molecular target to limit injury following radiotherapy. We describe a new role for the TGF-b/Smad pathway in the pathogenesis of radiation-induced damages. TGF-b/Smad pathway is involved in the fibro-genic phenotype of VSMC induced by irradiated EC as well as in the radiation-induced PAI-1 expression in endothelial cells. (author)

  6. Role of endothelium in radiation-induced normal tissue damages; Role de l'endothelium dans les dommages radio-induits aux tissus sains

    Energy Technology Data Exchange (ETDEWEB)

    Milliat, F

    2007-05-15

    More than half of cancers are treated with radiation therapy alone or in combination with surgery and/or chemotherapy. The goal of radiation therapy is to deliver enough ionising radiation to destroy cancer cells without exceeding the level that the surrounding healthy cells can tolerate. Unfortunately, radiation-induced normal tissue injury is still a dose limiting factor in the treatment of cancer with radiotherapy. The knowledge of normal tissue radiobiology is needed to determine molecular mechanisms involved in normal tissue pathogenic pathways in order to identify therapeutic targets and develop strategies to prevent and /or reduce side effects of radiation therapy. The endothelium is known to play a critical role in radiation-induced injury. Our work shows that endothelial cells promote vascular smooth muscle cell proliferation, migration and fibro-genic phenotype after irradiation. Moreover, we demonstrate for the first time the importance of PAI-1 in radiation-induced normal tissue damage suggesting that PAI-1 may represent a molecular target to limit injury following radiotherapy. We describe a new role for the TGF-b/Smad pathway in the pathogenesis of radiation-induced damages. TGF-b/Smad pathway is involved in the fibro-genic phenotype of VSMC induced by irradiated EC as well as in the radiation-induced PAI-1 expression in endothelial cells. (author)

  7. Interest of blood markers in predicting radiation-induced toxicity; Interet des marqueurs sanguins dans la prediction de la toxicite radio-induite

    Energy Technology Data Exchange (ETDEWEB)

    Lacombe, J. [Departement de cancerologie radiotherapie, CRLC Val-d' Aurelle-Paul-Lamarque, rue Croix-Verte, 34298 Montpellier cedex 5 (France); Universite de Montpellier I, 5, boulevard Henri-IV, CS 19044, 34967 Montpellier cedex 2 (France); Laboratoire d' oncoproteomique clinique, CRLC Val-d' Aurelle-Paul-Lamarque, rue Croix-Verte, 34298 Montpellier cedex 5 (France); Solassol, J. [Laboratoire de biologie cellulaire et hormonale, hopital Arnaud-de-Villeneuve, CHU de Montpellier, 371, avenue du Doyen-Gaston-Giraud, 34295 Montpellier cedex 5 (France); Laboratoire d' oncoproteomique clinique, CRLC Val-d' Aurelle-Paul-Lamarque, rue Croix-Verte, 34298 Montpellier cedex 5 (France); Coelho, M. [Inserm U896, institut de recherche en cancerologie de Montpellier, CRLC Val-d' Aurelle-Paul-Lamarque, rue Croix-Verte, 34298 Montpellier cedex 5 (France); Ozsahin, M. [Service de radio-oncologie, centre hospitalier universitaire Vaudois, 1011 Lausanne (Switzerland); Azria, D. [Departement de cancerologie radiotherapie, CRLC Val-d' Aurelle-Paul-Lamarque, rue Croix-Verte, 34298 Montpellier cedex 5 (France); Universite de Montpellier I, 5, boulevard Henri-IV, CS 19044, 34967 Montpellier cedex 2 (France); Inserm U896, institut de recherche en cancerologie de Montpellier, CRLC Val-d' Aurelle-Paul-Lamarque, rue Croix-Verte, 34298 Montpellier cedex 5 (France)

    2011-08-15

    The oncologic outcome and the total dose are highly correlated with the treatment by ionizing radiation. The dose increase (total or per fraction) may provoke late-side effects that are potentially irreversible. The radiation-induced CD8 lymphocyte apoptotic value and the molecular modifications within the lymphocyte are capable of predicting the level of risk of developing late-side effects after curative intent radiotherapy. In this review, we present the different blood assays in this setting and discuss the current possibilities of researches, namely those involving the proteomic process. (authors)

  8. Mesenchymal stem cells favour healing of the cutaneous radiation syndrome; Les cellules souches mesenchymateuses favorisent la cicatrisation des lesions cutanees radio induites

    Energy Technology Data Exchange (ETDEWEB)

    Francois, S.; Mouiseddine, M.; Mathieu, N.; Semont, A.; Monti, P.; Dudoignon, N.; Sache, A.; Boutarfa, A.; Thierry, D.; Voisin, P.; Gourmelon, P.; Chapel, A. [IRSN, Dir. de radioprotection de l' Homme, 92 - Fontenay-aux-Roses (France)

    2006-10-15

    It has been suggested that human Mesenchymal Stem Cells (hMSC) could be used to repair numerous injured tissues. We have studied the potential use of hMSC in order to limit radiation-induced skin lesions. Immuno-deficient NOD/SCID mice were locally irradiated to the leg (30 Gy, dose rate 2.7 Gy/mn) using a {sup 60} Co source in order to induce a severe skin lesion. Cultured bone marrow hMSC were delivered intravenously to the mice. The irradiated skin samples were studied for the presence of the human cells, the severity of the lesions and the healing process. Macroscopic analysis and histology results showed that the lesions were evolving to a less severe degree of radiation dermatitis following hMSC transplant when compared to irradiated non-transplanted controls. Clinical scores for the studied skin parameters of treated mice were significantly improved. A faster healing was observed when compared to untreated mouse. Immuno-histology and Polymerase Chain reaction (PCR) analysis provided evidence that the human cells were found in the irradiated area. These results suggest a possible use of hMSC for the treatment of the early phase of the cutaneous radiation syndrome. A successful transplant of stem cells and subsequent reduction in radiation-induced complication may open the road to completely new strategies in cutaneous radiation syndrome therapy. (authors)

  9. Treatment of radioinduced skin burns by adult stem cells;Traitement des brulures cutanees radio-induites par cellules souches adultes

    Energy Technology Data Exchange (ETDEWEB)

    Lataillade, J.J.; Prat, M. [Hopital Militaire Percy, Centre de Transfusion Sanguine des Armees, Dept. Recherches et Therapies Cellulaires, 92 - Clamart (France); Gourmelon, P. [Institut de Radioprotection et de Surete Nucleaire (IRSN), 92 - Fontenay-aux-Roses (France); Bey, E. [Hopital Militaire Percy, Service de Chirurgie Plastique, 92 - Clamart (France)

    2009-12-15

    In the area of skin injuries caused by ionizing radiations; the recent opportunity give us the the possibility to manage victims of radiation accidents with skin acute symptoms of irradiation. The approach of cell therapy developed for these patients could be in a second time, used for other types of injuries as thermal burns and the injuries linked to overexposure in radiotherapy. In spite of the diversity of approaches of allogeneic and autologous transplantation, the prognosis of deep and extended radioinduced burns is not completely satisfying because of inflammatory recurrences, origin of graft failures. The stem cells have been used in association. The stem cells were got from sampling of autologous bone marrow after an expansion in vitro from 15 to 17 days. The cultures were realised in medium with 8% of platelet lysate (clinical grade). The cells have been given by injection in complement of epidermis auto graft. A spectacular effect was noticed the day after the injection, but disappearing in some days encouraging to realize supplementary injections. An effect of the quickness and the quality of the graft success is appeared significant too. no necrosis recurrence for the patients after four years for the first one of them. We think that the stem cells participate to the local control of inflammation. (N.C.)

  10. O impacto da fibrose cística no perfil imunológico de pacientes pediátricos

    Directory of Open Access Journals (Sweden)

    Daniela M. Bernardi

    2013-02-01

    Full Text Available OBJETIVO: Comparar o estado imunológico de 44 pacientes pediátricos com fibrose cística (FCa umgrupo-controle formado por 16 indivíduos saudáveis. MÉTODOS: Foram selecionados para o estudo pacientes com FC com idade entre 3 e 12 anos, apresentando um escore clínico moderado e bom. Foram avaliados a glutationa eritrocitária, a produção de espécies reativas de oxigênio, citocinas (TNF-α, IFN-γ, IL-8, IL-6, IL-10 em culturas de células mononucleares do sangue periférico em condições espontâneas e estimuladas por BCG ou PHA, a concentração sérica de TGF-β2, IgA, IgG, IgM, IgE e IgA salivar. RESULTADOS :A produção espontânea de TNF-α, IL-6 e IL-10, a produção de IL-6 estimulada por PHA e TGF-β2, IgA e IgG séricas aumentaram em amostras de pacientes com FC. Indivíduos saudáveis tiveram uma produção mais elevada de TNF-α em resposta a BCG. CONCLUSÃO: Apesar de os pacientes com FC parecerem clinicamente estáveis, os resultados de seus exames de sangue periférico mostraram que houve um impacto sobre o sistema imunológico.

  11. Exacerbação aguda da fibrose pulmonar idiopática Acute exacerbation of idiopathic pulmonary fibrosis

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    Natália Melo

    2009-03-01

    Full Text Available Alguns doentes com fibrose pulmonar idiopática (FPI apresentam durante a sua evolução fases deagravamento clínico sem causa conhecida, designadas como “exacerbação aguda” ou “fase acelerada” da doença (EA. Caracterizam -se pelo agravamento marcado da dispneia, hipoxemia e pelo aparecimento de novas opacidades pulmonares ou pelo agravamento das já existentes no estudo imagiológico. Os achados histológicos típicos são o dano alveolar difuso (DAD sobreposto a alterações de pneumonia intersticial usual (UIP. Esta entidade clínica associa -se a uma mortalidade elevada, não havendo até ao momento nenhuma terapêutica de comprovada eficácia. Os autores descrevem os casos clínicos de cinco doentes que apresentaram alterações clínicas, funcionais e radiológicas sugestivas de EA-FPI, assim como o tratamento efectuado e a evolução observada, enquadrando-os na discussão das características normalmente apresentadas por esta entidade.Some patients with Idiopathic Pulmonary Fibrosis (IPF have disease accelerated deterioration without identifiable cause referred as “acute exacerbation” or “accelerated stage”. It is characterized by severe worsening of dyspnea, hypoxemia and new or progressive opacities on imaging studies. The typical histological findings are diffuse alveolar damage in addition to the features of usual interstitial pneumonia pattern. Mortality in this clinical entity is very high and no efficacious therapeutic have been described. The authors describe the clinical, functional and radiological features, treatment and evolution of five patients with IPF acute exacerbation. A discussion will be carry out concerning the IPF acute exacerbation usual features comparing with the alterations noticed in those patients.

  12. Condutividade e teste quantitativo coulométrico na triagem neonatal para fibrose cística

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    Mouseline Torquato Domingos

    2015-12-01

    Full Text Available Resumo Objetivo Comparar os resultados obtidos no teste do suor pelo método da condutividade e a dosagem coulométrica de cloreto no suor em recém0nascidos (RN suspeitos da triagem neonatal para fibrose cística (FC. Métodos O teste do suor foi feito simultaneamente pelos dois métodos em crianças com e sem FC. Os valores de corte para confirmar FC foram na condutividade > 50 mmol/L e no teste coulométrico > 60 mmol/L. Resultados Fizeram o teste do suor por condutividade e dosagem coulométrica simultaneamente 444 RN sem FC (185 do sexo masculino, 234 do feminino e 24 não informado e obtiveram resultado mediano de 32 mmol/L e 12 mmol/L respectivamente. Para os noventa RN com FC os valores medianos de condutividade e dosagem coulométrica foram 108 mmol/L e 97 mmol/L respectivamente. O índice de falso positivo para condutividade foi de 16,7% e em todos os pacientes FC foi superior a 50 mmol/L, o que confere ao método 100% de sensibilidade (IC 95% = 93,8 a 97,8, especificidade de 96,2% (IC 95% = 93,8 a 97,8, valor preditivo positivo 83,3 (IC 95% = 74,4 a 91,1, valor preditivo negativo 100% (IC 95% = 90,5 a 109,4 e acurácia 9,8%. A correlação entre os métodos foi de r = 0,97 (p > 0,001.O melhor valor de corte sugerido foi de 69,0 mmol/L, coeficiente de kappa = 0,89. Conclusão O teste da condutividade apresentou excelente correlação com o quantitativo coulométrico, alta sensibilidade e especificidade e pode ser usado no diagnóstico da FC em crianças detectadas pela triagem neonatal.

  13. Stigma and cystic fibrosis Estigma y fibrosis cística Estigma e fibrose c��stica

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    Tainá Maues Peluci Pizzignacco

    2010-02-01

    Full Text Available Cystic Fibrosis (CF, also known as Mucoviscidosis, is a chronic disease of autosomal recessive origin and so far incurable. This analysis considers some characteristics of patients and family members that indicate it is a stigmatizing disease. The CF stigma’s impact on the lives of children and adolescents can affect treatment adherence, socialization, family relationships and the formation of their life histories, with direct consequences on their quality of life.La fibrosis cística (FC, también conocida como mucoviscidosis, es una enfermedad crónica de origen autosómica recesiva y, hasta el momento, incurable. La presente reflexión presenta consideraciones a respecto de algunas características que acompañan a pacientes y familiares, permitiendo comprenderla como enfermedad que estigmatiza. Las repercusiones del estigma en la vida de niños y adolescentes con FC pueden interferir en la adhesión al tratamiento, en el proceso de socialización, en la relación con los familiares y en la formación de su biografía, con reflejo directo en la calidad de vida.A fibrose cística (FC, também conhecida como mucoviscidose, é doença crônica de origem autossômica recessiva e, até o momento, incurável. A presente reflexão traz considerações a respeito de algumas características que acompanham pacientes e familiares, permitindo compreendê-la como doença estigmatizante. As repercussões do estigma na vida de crianças e adolescentes com FC podem implicar na adesão ao tratamento, no processo de socialização, na relação com os familiares e na formação de sua biografia, com reflexo direto em sua qualidade de vida.

  14. Fibrose cardíaca associada à intoxicação por Amorimia septentrionalis em bovinos

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    Samuel S.C. Albuquerque

    2014-05-01

    Full Text Available Amorimia septentrionalis contém monofluoracetato de sódio e quando consumida por ruminantes provoca morte súbita. Este estudo teve o objetivo de relatar a epidemiologia, os sinais clínicos e patológicos de surtos de morte súbita em bovinos provocadas por Amorimia septentrionalis nos Estados de Pernambuco e Paraíba. Para isso, realizaram-se visitas técnicas em diversas propriedades nas Microrregiões do Médio Capibaribe/PE e Itabaiana/PB. Oito bovinos foram necropsiados. Coletaram-se tecidos das cavidades abdominal e torácica, além do encéfalo e medula espinhal. As alterações clínicas consistiram em lentidão, decúbito esternal prolongado, relutância em se movimentar quando em estação, cansaço, taquipneia, taquicardia e pulso venoso positivo. Os bovinos que foram forçados a se movimentar apresentaram instabilidade, tremores musculares e queda repentina seguida de vocalizações, movimentos de pedalagem e morte súbita em cerca de 5 a 7 minutos. As principais alterações macroscópicas consistiram em edema pulmonar, coração com aspecto globular com áreas esbranquiçadas, petéquias e equimoses no epicárdio, miocárdio e músculos papilares. À microscopia observou-se aumento da eosinofilia do citoplasma dos cardiomiócitos, núcleos picnóticos, cariorrexia, cariólise, perda das estriações, edema intersticial, infiltrado inflamatório intersticial mononuclear e áreas multifocais de fibrose cardíaca. Nos rins, constatou-se degeneração hidrópico vacuolar e necrose das células epiteliais em túbulos contorcidos. Os sinais clínicos foram semelhantes aos sinais clínicos já descritos em bovinos por plantas que contém MFA. As lesões macro e microscópicas descritas no coração e rins são de grande valor diagnóstico. A. septentrionalis é a principal planta tóxica de interesse pecuário nas microrregiões do Médio Capibaribe e Itabaiana devido às perdas econômicas diretas e indiretas que provoca na pecu

  15. Fibrose Cística: Análise da Coorte de 10 Anos de um Programa para Adultos

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    Paulo de Tarso Roth Dalcin

    2011-07-01

    Full Text Available Introdução: A fibrose cística (FC que já foi considerada doença da criança é agora doença do adulto e requer programa para adultos. Objetivo: definir características clínicas de uma coorte de 10 anos de um programa para adultos com FC e determinar as características associadas com desfechos clínicos. Métodos: coorte retrospectiva de pacientes com FC (idade ≥ 10 anos atendida pelo Programa para Adultos do Hospital de Clínicas de Porto Alegre, de outubro de 1998 a outubro de 2008. Foram coletados dados demográficos, dados clínicos, status nutricional, função pulmonar, testes laboratoriais e microbiologia do escarro. Foram definidos como desfechos clínicos: sobrevivência, sobrevivência com transplante pulmonar e óbito. Resultados: foram atendidos 94 pacientes pelo programa para adultos. A média de idade foi 24,0±7,4 anos e a média do volume expiratório forçado no primeiro segundo (VEF1 foi 56,4±28,8%. Setenta e sete pacientes foram sobreviventes, 6 sobreviventes com transplante pulmonar e 11 morreram. Na análise univariada, etnia caucasiana (p=0,016, mutação F508del (p=0,04, escore clínico (p<0,001, índice de massa corporal (p<0,001,  oxigenoterapia (p<0,001, capacidade vital forçada (p=0,023 e VEF1 (p<0,001 se associaram com os desfechos clínicos. A análise de regressão logística identificou fatores associados com desfecho precário: VEF1 (RC=0,.72, ICI=0,54-0,94, p=0,017 e escore clínico  (RC = 0,70, IC=0,50-0,97, p = 0,034. Insuficiência respiratória crônica exacerbada por infecção respiratória aguda foi causa imediata de morte na maioria dos casos. Conclusão: Este estudo descreveu uma coorte de 94 pacientes com FC atendidos por um programa de adultos. VEF1 e escore clinico se associaram com os desfechos clínicos.

  16. Fibrose cística no adulto: aspectos diagnósticos e terapêuticos Cystic fibrosis in adults: diagnostic and therapeutic aspects

    Directory of Open Access Journals (Sweden)

    Paulo de Tarso Roth Dalcin

    2008-02-01

    Full Text Available A fibrose cística, que já foi considerada uma doença da infância, é agora também uma doença do adulto. O aumento da longevidade resultou em mais problemas médicos relacionados com a idade e com a própria doença. O crescente número de adultos com fibrose cística resultou em aumento da necessidade de cuidados médicos. Essa necessidade tem sido suprida por um crescente número de pneumologistas de adultos e outros especialistas. O objetivo dessa revisão é sumarizar o conhecimento corrente sobre o diagnóstico e tratamento no adulto com fibrose cística. Na maioria dos casos, o diagnóstico é sugerido por manifestações de doença sinopulmonar crônica e insuficiência pancreática exócrina, e, então, confirmado por um teste do suor positivo. Pacientes adultos podem, entretanto, apresentar suficiência pancreática e características clínicas atípicas, às vezes, associadas com teste do suor normal ou limítrofe. Em tais casos, a possibilidade de realizar pesquisa de mutações para fibrose cística e de medir a diferença de potencial nasal pode ser de utilidade diagnóstica. A abordagem terapêutica padrão para a doença pulmonar inclui: antibióticos, higiene das vias aéreas, exercício, mucolíticos, broncodilatadores, oxigênio, agentes anti-inflamatórios e suporte nutricional. A utilização adequada dessas terapias resulta em mais pacientes com fibrose cística sobrevivendo na vida adulta com uma aceitável qualidade de vida.Once considered a childhood disease, cystic fibrosis is now also a disease of adults. Increased longevity has resulted in the aging of the cystic fibrosis population. The consequent age-related medical problems among adults with cystic fibrosis have increased medical care needs. These needs are being met by a growing number of non-pediatric pulmonologists and other non-pediatric specialists. The objective of this review was to summarize the current knowledge about diagnosis and treatment in adult

  17. Republished: Fibrosing organising pneumonia.

    Science.gov (United States)

    Beardsley, Brooke; Rassl, Doris

    2014-08-01

    Organising pneumonia (otherwise referred to as bronchiolitis obliterans organising pneumonia) is characterised histologically by plugs of granulation tissue, which are present predominantly within small airways, alveolar ducts and peri-bronchiolar alveoli. This pattern is not specific for any disorder or cause, but is one type of inflammatory response to pulmonary injury, which may be seen in a wide variety of clinical conditions. Typically, organising pneumonia responds very well to corticosteroid treatment; however, a small percentage of patients appear to develop progressive fibrosis. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.

  18. Fibrosing organising pneumonia.

    Science.gov (United States)

    Beardsley, Brooke; Rassl, Doris

    2013-10-01

    Organising pneumonia (otherwise referred to as bronchiolitis obliterans organising pneumonia) is characterised histologically by plugs of granulation tissue, which are present predominantly within small airways, alveolar ducts and peri-bronchiolar alveoli. This pattern is not specific for any disorder or cause, but is one type of inflammatory response to pulmonary injury, which may be seen in a wide variety of clinical conditions. Typically, organising pneumonia responds very well to corticosteroid treatment; however, a small percentage of patients appear to develop progressive fibrosis.

  19. Acroqueratodermia aquagénica associada a uma mutação do gene da fibrose quística

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    V. Coelho-Macias

    2013-05-01

    Full Text Available Resumo: Descrita em 1996, a acroqueratodermia aquagénica é uma entidade rara, caracterizada pelo aparecimento de pápulas edematosas palmares após contacto com água. Múltiplas associações foram enumeradas mas, recentemente, a associação a mutações do gene da fibrose quística foi demonstrada.Descreve-se o caso de uma mulher de 18 anos, saudável, com prurido e edema palmar após imersão em água. O exame objetivo inicial não mostrava alterações mas, 5 min após imersão em água, observavam-se múltiplas pápulas esbranquiçadas palmares. O estudo do gene da fibrose quística (CFTR revelou uma mutação F508del num dos alelos. A doente negava outras queixas ou história familiar relevante.A acroqueratodermia aquagénica é uma entidade provavelmente subdiagnosticada que poderá constituir uma manifestação de mutações do gene CFTR, o que possibilitaria a identificação de portadores e aconselhamento genético. Abstract: Reported for the first time in 1996, aquagenic keratoderma is a rare condition which is characterized by edematous flat-topped papules appearing on palmar skin after water immersion. Multiple anecdotal associations have been described but, recently, the association with cystic fibrosis gene mutations (CFTR has been highlighted.The authors describe an 18 year-old female, with one-month complaints of pruritus and swelling of palmar skin after water immersion. On examination, palmar skin was unremarkable but, 5 minutes after water immersion, multiple whitish papules became apparent. CFTR genotype study showed a F508del mutation in one alelle. She had no other symptoms and no relevant family history.Aquagenic keratoderma is probably an under-diagnosed entity that might represent a manifestation of CFTR mutations, making carrier state identification and genetic counseling possible. Palavras-chave: Acroqueratodermia aquagénica, Fibrose quística, Aconselhamento genético, Keywords: Aquagenic keratoderma, Cystic

  20. Achados histológicos e sobrevida na fibrose pulmonar idiopática Histological features and survival in idiopathic pulmonary fibrosis

    Directory of Open Access Journals (Sweden)

    Ester Nei Aparecida Martins Coletta

    2003-12-01

    Full Text Available INTRODUÇÃO: A fibrose pulmonar idiopática foi recentemente redefinida como pneumonia intersticial usual de etiologia desconhecida. O valor prognóstico dos achados histológicos deve ser reavaliado. OBJETIVO: Neste estudo foram correlacionados os achados histológicos e alguns dados clínicos e funcionais (duração dos sintomas, capacidade vital forçada, idade, sexo, hábito de fumar com a sobrevida. MÉTODO: Foram estudados 51 pacientes portadores de fibrose pulmonar idiopática. A média de idade foi de 66 ± 8 anos. Vinte e um pacientes eram do sexo feminino; 26 eram fumantes ou ex-fumantes. Todos apresentavam quadro de pneumonia intersticial usual na histologia. Grau de faveolamento, fibrose estabelecida, descamação, celularidade, espessamento vascular miointimal e focos fibroblásticos foram graduados por método semiquantitativo. RESULTADOS: A mediana do tempo de sintomas foi de 12 meses e a capacidade vital forçada inicial foi de 72 ± 21%. Por análise de risco proporcional de Cox, a sobrevida correlacionou-se, de maneira significativa (p BACKGROUND: Idiopathic pulmonary fibrosis was recently redefined as usual interstitial pneumonia of unknown etiology. Consequently, the prognostic value of histological findings needs to be reassessed. OBJECTIVE: To correlate clinical, functional and histological findings with survival in patients with idiopathic pulmonary fibrosis. METHOD: Patients (n = 51; mean age: 66 ± 8 years; gender: 21 females/30 males were evaluated. Of the 51, 26 were smokers or ex-smokers. Duration of symptoms, forced vital capacity and smoking habits were recorded. All patients presented usual interstitial pneumonia verified through histology. Degree of honeycombing, established fibrosis, desquamation, cellularity, myointimal thickening of blood vessels and number of fibroblastic foci were graded according to the semiquantitative method. RESULTS: Median duration of symptoms was 12 months and initial forced vital

  1. Fatores prognósticos em fibrose pulmonar idiopática Prognostic factors in idiopathic pulmonary fibrosis

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    ADALBERTO SPERB RUBIN

    2000-09-01

    Full Text Available Com o objetivo de avaliar quais fatores prognósticos são significativos na sobrevida de pacientes com fibrose pulmonar idiopática (FPI, foram estudados 117 casos com comprovação histológica da doença, internados no Pavilhão Pereira Filho entre 1970 e 1996. Todos os pacientes realizaram radiologia convencional de tórax, espirometria e preencheram um questionário padronizado no momento da internação. Foram também realizados medida da difusão (34 casos, capacidade pulmonar total (28, gasometria (106, lavado broncoalveolar (39, provas reumatismais (45 e tomografia computadorizada de tórax (24. Para uma análise mais específica, os pacientes foram dividos em dois grupos: grupo A (sobrevida até dois anos - 55 pacientes e grupo B (sobrevida maior que cinco anos - 24 pacientes, sendo estas características também analisadas de acordo com a significância em relação à sobrevida. Foram considerados fatores indicativos de pior prognóstico a idade avançada, a duração prolongada de sintomas, a gravidade da dispnéia, a redução da CVF, da DCO, da PaO2 e da SaO2, a intensidade do faveolamento e o grau de profusão do padrão reticular na TCAR. Estiveram também associados à menor sobrevida os valores menores de VEF1 e CPT. A utilização destes critérios de gravidade que apresentaram significância estatística, quando utilizados em conjunto, pode determinar uma avaliação prognóstica mais apurada em pacientes portadores de FPI, com implicações terapêuticas e sociais relevantes para seu manejo e acompanhamento.In order to evaluate which prognostic factors were significant to the survival of patients with idiopathic pulmonary fibrosis (IPF, 121 histologically confirmed cases of the disease were studied at the Pereira Filho Hospital from 1970 to 1996. All patients were submitted to a standard thorax X-ray and spirometry and answered a standardized questionnaire when admitted to hospital. They also underwent diffusion tests (34

  2. Expanding the clinical spectrum of hereditary fibrosing poikiloderma with tendon contractures, myopathy and pulmonary fibrosis due to FAM111B mutations.

    Science.gov (United States)

    Mercier, Sandra; Küry, Sébastien; Salort-Campana, Emmanuelle; Magot, Armelle; Agbim, Uchenna; Besnard, Thomas; Bodak, Nathalie; Bou-Hanna, Chantal; Bréhéret, Flora; Brunelle, Perrine; Caillon, Florence; Chabrol, Brigitte; Cormier-Daire, Valérie; David, Albert; Eymard, Bruno; Faivre, Laurence; Figarella-Branger, Dominique; Fleurence, Emmanuelle; Ganapathi, Mythily; Gherardi, Romain; Goldenberg, Alice; Hamel, Antoine; Igual, Jeanine; Irvine, Alan D; Israël-Biet, Dominique; Kannengiesser, Caroline; Laboisse, Christian; Le Caignec, Cédric; Mahé, Jean-Yves; Mallet, Stéphanie; MacGowan, Stuart; McAleer, Maeve A; McLean, Irwin; Méni, Cécile; Munnich, Arnold; Mussini, Jean-Marie; Nagy, Peter L; Odel, Jeffrey; O'Regan, Grainne M; Péréon, Yann; Perrier, Julie; Piard, Juliette; Puzenat, Eve; Sampson, Jacinda B; Smith, Frances; Soufir, Nadem; Tanji, Kurenai; Thauvin, Christel; Ulane, Christina; Watson, Rosemarie M; Khumalo, Nonhlanhla P; Mayosi, Bongani M; Barbarot, Sébastien; Bézieau, Stéphane

    2015-10-15

    Hereditary Fibrosing Poikiloderma (HFP) with tendon contractures, myopathy and pulmonary fibrosis (POIKTMP [MIM 615704]) is a very recently described entity of syndromic inherited poikiloderma. Previously by using whole exome sequencing in five families, we identified the causative gene, FAM111B (NM_198947.3), the function of which is still unknown. Our objective in this study was to better define the specific features of POIKTMP through a larger series of patients. Clinical and molecular data of two families and eight independent sporadic cases, including six new cases, were collected. Key features consist of: (i) early-onset poikiloderma, hypotrichosis and hypohidrosis; (ii) multiple contractures, in particular triceps surae muscle contractures; (iii) diffuse progressive muscular weakness; (iv) pulmonary fibrosis in adulthood and (v) other features including exocrine pancreatic insufficiency, liver impairment and growth retardation. Muscle magnetic resonance imaging was informative and showed muscle atrophy and fatty infiltration. Histological examination of skeletal muscle revealed extensive fibroadipose tissue infiltration. Microscopy of the skin showed a scleroderma-like aspect with fibrosis and alterations of the elastic network. FAM111B gene analysis identified five different missense variants (two recurrent mutations were found respectively in three and four independent families). All the mutations were predicted to localize in the trypsin-like cysteine/serine peptidase domain of the protein. We suggest gain-of-function or dominant-negative mutations resulting in FAM111B enzymatic activity changes. HFP with tendon contractures, myopathy and pulmonary fibrosis, is a multisystemic disorder due to autosomal dominant FAM111B mutations. Future functional studies will help in understanding the specific pathological process of this fibrosing disorder.

  3. Estado nutricional e distribuição de gordura corporal em crianças e adolescentes com Fibrose Cística

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    Célia Regina Moutinho de Miranda Chaves

    2015-11-01

    Full Text Available O objetivo deste estudo é avaliar o estado nutricional e a distribuição da gordura corporal em crianças e adolescentes com fibrose cística. Foram realizadas avaliação da distribuição de gordura corporal por meio da absorciometria de duplo feixe de energia, do estado nutricional por estatura/idade e índice de massa corporal/idade e a ingestão dietética pelo recordatório alimentar de 24horas, em 56 pacientes com idade entre 8 e 18 anos. Aproximadamente 50% da amostra apresentou estado nutricional adequado. A maioria apresentou a ingestão calórica e de lipídios inadequadas. O IMC/I foi o indicador nutricional que melhor evidenciou o aumento do percentual de gordura do tronco, razão androide/ginecoide e razão gordura tronco/gordura total. Os pacientes com Insuficiência Pancreática e os eutróficos apresentaram razão mediana androide/ginecoide maior. O aumento da adiposidade abdominal foi evidenciado pela DXA. O IMC/I não identificou a diminuição da massa magra corporal, mas quando aumentado foi significativo para adiposidade abdominal. Pacientes com fibrose cística devem associar a avaliação antropométrica à composição corporal e à distribuição de gordura corporal para um diagnóstico mais precoce de desnutrição e fatores de risco cardiometabólico.

  4. Adsorption studies at ionized surface layers by means of hot atoms; Etude, a l'aide d'atomes excites, de l'adsorption dans des couches superficielles ionisees; Issledovanie pri pomoshchi goryachikh atomov adsorbtsii na ionizirovannykh poverkhnostnykh sloyakh; Estudios, con ayuda de atomos excitados, de la adsorcion en capas superficiales ionizadas

    Energy Technology Data Exchange (ETDEWEB)

    Avrahami, Menashe; Steiger, Naftali H [Department of Chemistry, Technion - Israel Institute of Technology, Haifa (Israel)

    1962-03-15

    directement si l'on utilise des atomes excites. Des quantites extremement faibles d'ions radioactifs appropries, ou d'ions capables de subir in situ des transformations nucleaires induites, sont ajoutees a ces solutions, en remplacement de certains contre-ions coadsorbes sur les ions organiques adsorbes initialement. Des atomes excites dont l'energie va de 100 keV a quelques electrons volts seulement ont dans l'eau un parcours compris entre 1000A et des valeurs correspondant aux couches monomoleculaires. On peut donc s'en sarvir pour des etudes tres sensibles sur les couches superficielles. Les atomes excites qui sont expulses de la surface sont separes et comptes. Pour obtenir des particules de recul par desintegration {alpha}, le systeme {sup 212}Bi/{sup 208}Tl s'est revele satisfaisant. En ameliorant cette methode, on a pu obtenir des rensaignernents utiles sur les conditions d'adsorption en couches superficielles de dodecyl-sulfate de sodium. On a etudie la cinetique de cette adsorption en suivant dans le temps l'activite des particules de recul formees par adsorption d'ions Bi. On a mesure les isothermes d'adsorption des ions Bi en fonction de la concentration globale de la substance tensioactive sous differentes conditions de force ionique, de pH et d'activite {sup 212}Bi. En comparant ces isothermes avec ceux obtenus par mesure de la tension superficielle des solutions et en calculant l'exces de surface a l'aide d'un isotherme de Gibbs convenablement modifie, on a pu determiner l'adsorption de Bi{sup +++} et de Pb{sup ++} par rapport a celle de Na{sup +} et de H{sub 3}O{sup +} et, a partir de ces donnees, l'importance de l'echange ionique dans la couche adsorbee. Etant donne que cette methode permet de mesurer l'adsorption des especes ionisees, on a pu suivre les reactions de surface transformant les anions organiques primaires adsorbes en un etat non ionique. On a pu en tirer des conclusions sur la formation de savons acides non ionises en surface et sur certaines

  5. Fibrose cística em adultos: aspectos clínicos e espirométricos Cystic fibrosis in adults clinical and spirometric aspects

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    Antônio Carlos M. Lemos

    2004-02-01

    Full Text Available INTRODUÇÃO: A fibrose cística é diagnosticada usualmente na infância. No Brasil, poucos estudos abordam seu diagnóstico na idade adulta. OBJETIVO: Descrever as características demográficas, clínicas e os achados de espirometria dos pacientes com fibrose cística diagnosticados na idade adulta, na Bahia (Brasil. MÉTODO: Foram avaliados 28 pacientes com fibrose cística diagnosticada na idade adulta no Centro de Referência de Fibrose Cística do Estado da Bahia. As variáveis de interesse foram: idade, gênero, cor, índice de massa corpórea (IMC, cultivo do escarro, porcentagem do previsto da capacidade vital forçada (% CVF, porcentagem do previsto do volume expiratório forçado no primeiro segundo (% VEF1 e resposta ao broncodilatador. RESULTADOS: A média de idade dos pacientes foi de 31,1±12,4 anos. A proporção de negros e mulatos foi de 53,7%, e a média de IMC foi 18,7±3,0Kg/m2. Em doze pacientes (43% foi confirmada P. aeruginosa no escarro. As médias ±DP dos percentuais do previsto da CVF e do VEF1 foram de 58,9±21,6% e 44,1±23% respectivamente. No grupo colonizado por P. aeruginosa as médias dos parâmetros espirométricos foram inferiores às do grupo não colonizado. Entretanto, somente em relação à CVF esta diferença alcançou significância estatística (p= 0,007. CONCLUSÃO: Concordante com a literatura, este estudo reforça que o diagnóstico de fibrose cística deve ser investigado em pacientes com infecções respiratórias de repetição, sinusite e bronquiectasias, mesmo na idade adulta. Os valores dos percentuais da CVF e VEF1 em relação ao previsto foram menores nos pacientes colonizados por P aeruginosa, evidenciando uma maior deterioração da função pulmonar.INTRODUCTION: Cystic Fibrosis is usually diagnosed in childhood. In Brazil, few studies have approached CF diagnosed in adulthood. OBJECTIVE: The aim of this study was to describe demographic and clinical characteristics and spirometric

  6. A longitudinal study of whole body, tissue, and cellular physiology in a mouse model of fibrosing NASH with high fidelity to the human condition.

    Science.gov (United States)

    Krishnan, Anuradha; Abdullah, Tasduq Sheikh; Mounajjed, Taofic; Hartono, Stella; McConico, Andrea; White, Thomas; LeBrasseur, Nathan; Lanza, Ian; Nair, Sreekumaran; Gores, Gregory; Charlton, Michael

    2017-06-01

    The sequence of events that lead to inflammation and fibrosing nonalcoholic steatohepatitis (NASH) is incompletely understood. Hence, we investigated the chronology of whole body, tissue, and cellular events that occur during the evolution of diet-induced NASH. Male C57Bl/6 mice were assigned to a fast-food (FF; high calorie, high cholesterol, high fructose) or standard-chow (SC) diet over a period of 36 wk. Liver histology, body composition, mitochondrial respiration, metabolic rate, gene expression, and hepatic lipid content were analyzed. Insulin resistance [homeostasis model assessment-insulin resistance (HOMA-IR)] increased 10-fold after 4 wk. Fibrosing NASH was fully established by 16 wk. Total hepatic lipids increased by 4 wk and remained two- to threefold increased throughout. Hepatic triglycerides declined from sixfold increase at 8 wk to threefold increase by 36 wk. In contrast, hepatic cholesterol levels steadily increased from baseline at 8 wk to twofold by 36 wk. The hepatic immune cell population altered over time with macrophages persisting beyond 16 wk. Mitochondrial oxygen flux rates of FF mice diet were uniformly lower with all the tested substrates (13-276 pmol·s -1 ·ml -1 per unit citrate synthase) than SC mice (17-394 pmol·s -1 ·ml -1 per unit citrate synthase) and was accompanied by decreased mitochondrial:nuclear gene copy number ratios after 4 wk. Metabolic rate was lower in FF mice. Mitochondrial glutathione was significantly decreased at 24 wk in FF mice. Expression of dismutases and catalase was also decreased in FF mice. The evolution of NASH in the FF diet-induced model is multiphasic, particularly in terms of hepatic lipid composition. Insulin resistance precedes hepatic inflammation and fibrosis. Mitochondrial dysfunction and depletion occur after the histological features of NASH are apparent. Collectively, these observations provide a unique overview of the sequence of changes that coevolve with the histological evolution of

  7. Avaliação do uso do captopril na fibrose peritoneal induzida em ratos pelo uso de solução de glicose a 4,25%

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    Adriana Fátima Menegat Schuinski

    2013-12-01

    Full Text Available INTRODUÇÃO: A Insuficiência Renal Crônica (IRC tem incidência alarmante neste século. A diálise peritoneal, uma das modalidades de terapia renal pode ter complicações, e entre estas a fibrose peritoneal, que ocorre com o decorrer dos anos nestes pacientes. Sua forma mais grave é a chamada peritonite esclerosante encapsulante, levando à mudança de terapia dialítica. OBJETIVO: Estudar a influência do uso do captopril na fibrose peritoneal induzida em ratos pelo uso de solução de glicose a 4,25 %. MÉTODOS: Estudo prospectivo controlado, em ratos Wistar não urêmicos. Foram estudados 20 animais. Os animais foram submetidos diariamente à punção abdominal, sendo infundida solução de diálise peritoneal com glicose a 4,25% na dose de 10 ml/100 g de peso. Os animais foram divididos em 2 grupos: experimental e controle. O grupo experimental recebeu captopril na dose de 30 mg/kg/dia por gavagem. O grupo controle não recebeu nenhuma droga. Foram acompanhados por 21 e 49 dias. Ao final do período foram submetidos à procedimento cirúrgico para retirada de peritônio parietal e visceral. As amostras obtidas foram analisadas histologicamente, usando-se coloração Hematoxilina - Eosina e Sirius Red, para avaliação do grau de fibrose. RESULTADOS: A análise mostrou que a intensidade da fibrose, a espessura do peritônio e o número de células não atingiram diferença estatisticamente significante entre os grupos experimental e controle. CONCLUSÃO: O estudo mostrou que o uso do captopril não foi capaz de alterar a intensidade da fibrose peritoneal induzida pelo uso de solução de diálise em ratos.

  8. Expression and distribution of connexin 32 in rat liver with experimentally induced fibrosis Expressão e distribuição da conexina 32 em fígados de ratos com fibrose induzida experimentalmente

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    Alexandro dos S. Rodrigues

    2009-04-01

    Full Text Available The connexin 32 (Cx32 is a protein that forms the channels that promote the gap junction intercellular communication (GJIC in the liver, allowing the diffusion of small molecules through cytosol from cell-to-cell. Hepatic fibrosis is characterized by a disruption of normal tissue architeture by cellular lesions, and may alter the GJIC. This work aimed to study the expression and distribution of Cx32 in liver fibrosis induced by the oral administration of dimethylnitrosamine in female Wistar rats. The necropsy of the rats was carried out after five weeks of drug administration. They presented a hepatic fibrosis state. Sections from livers with fibrosis and from control livers were submitted to immunohistochemical, Real Time-PCR and Western-Blot analysis to Cx32. In fibrotic livers the Cxs were diffusely scattered in the cytoplasm, contrasting with the control livers, where the Cx32 formed junction plaques at the cell membrane. Also it was found a decrease in the gene expression of Cx32 without reduction in the protein quantity when compared with controls. These results suggest that there the mechanism of intercellular communication between hepatocytes was reduced by the fibrotic process, which may predispose to the occurrence of a neoplastic process, taken in account that connexins are considered tumor suppressing genes.A conexina 32 (Cx32 é uma proteína que constitui os canais que promovem as comunicações intercelulares via junções comunicantes (CIJC no fígado, permitindo difusão de pequenas moléculas citoplasmáticas de uma célula à outra. A fibrose hepática caracteriza-se pela alteração da arquitetura normal do fígado e podem alterar as CIJCs. O objetivo deste trabalho foi estudar a expressão e distribuição de Cx32 na fibrose hepática. O objetivo do presente trabalho foi estudar a expressão e distribuição da Cx32 em fígados com fibrose induzida pela administração oral de dimetilnitrosamina em fêmeas de ratos Wistar. A

  9. Perfil de citocinas da polipose nasossinusal na Fibrose Cística comparado com indivíduos sem doenças nasossinusais Cytokine profile in subjects with Cystic Fibrosis and nasal polyposis compared to patients with no nasal disorders

    Directory of Open Access Journals (Sweden)

    Flávio Barbosa Nunes

    2010-02-01

    Full Text Available Embora o perfil das citocinas na polipose nasossinusal seja bem documentado, pouco se sabe sobre estas proteínas quando associadas à Fibrose Cística. OBJETIVOS: Avaliar a expressão das citocinas IL¬4, IL¬5, IL¬6, IL¬8, GM¬C-SF e IFN--y analisada pela RT¬-PCR, nos pólipos de pacientes com Fibrose Cística. MATERIAL E MÉTODO: Estudo transversal, prospectivo, de 24 pacientes, 13 com Fibrose Cística e polipose nasossinusal (Grupo Fibrose Cística e 11 com exame otorrinolaringológico normal (Grupo Controle. A média de idade foi de 21 anos (3¬-57, 12 eram do sexo masculino e 12 do sexo feminino. O perfil das citocinas foi pesquisado nos fragmentos de mucosa (Grupo Controle ou pólipo nasal (Grupo Fibrose Cística através da RT-¬PCR. Foram estudadas as transcrições para as citocinas IL¬4, IL¬5, IL¬6, IL¬8, IFN¬y e GM¬-CSF ajustadas pelo valor da β¬ actina. RESULTADOS: As interleucinas 5, 6, 8 e GM¬-CSF foram semelhantes nos dois grupos (p>0,05. Menores valores de IFNy¬ (p=0,03 e forte tendência de aumento de IL¬4 (p=0,06 foram observados no grupo Fibrose Cística. CONCLUSÃO: As células inflamatórias e estruturais podem produzir RNA mensageiro para IL¬4, bloqueando a produção de outras citocinas com IFN-y¬, sugerindo a participação destes mecanismos na formação dos pólipos da Fibrose Cística.Although the cytokine profile in nasal polyposis is well documented, little is known about cytokines associated to cystic fibrosis. AIM: Assess the expression of cytokines IL¬4, IL¬5, IL¬6, IL¬8, GM¬-CSF and IFN¬-y, analyzed through RT-PCR, in the polyps of patients with cystic fibrosis. MATERIALS AND METHODS: A cross-sectional, prospective study was carried out with 24 patients, 13 of whom had cystic fibrosis and nasal polyposis (Cystic Fibrosis Group and 11 had normal otorhinolaryngological exams (Control Group. The average age was 21 years (3¬57; 12 participants were males and 12 were females. The cytokine

  10. Associação entre postura, função pulmonar e capacidade funcional na fibrose quística

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    J.T.S. Penafortes

    2013-01-01

    Full Text Available Resumo: Objetivos: Avaliar as correlações entre função pulmonar, capacidade funcional e postura em pacientes adultos portadores de fibrose quística (FQ. Como segundo objetivo, avaliar a correlação entre a qualidade de vida e as variáveis obtidas na avaliação postural destes pacientes. Métodos: Foi realizado um estudo transversal em que 14 portadores de FQ se submeteram à avaliação da análise postural (software de avaliação postural e provas de função pulmonar (espirometria, pletismografia de corpo inteiro e medição da capacidade de difusão do CO e capacidade funcional (teste da caminhada de 6 min. Todos os pacientes responderam ao Questionário de Fibrose Quística com Versão Revisada (QFQ-R. Resultados: A maioria dos pacientes era do sexo masculino (57%, com mediana da idade de 24,5 anos (22-34 anos. Foram observadas correlações significantes de volume expiratório máximo no primeiro segundo, distância da caminhada dos 6 min, capacidade pulmonar total e resistência de vias aéreas com o alinhamento vertical do tronco (ρ = −0,57, p < 0,05; ρ = −0,65, p < 0,01; ρ = 0,54, p < 0,05; e ρ = 0,67, p < 0,01, respetivamente. Foram observadas correlações estatisticamente significantes entre o domínio «físico» do QFQ-R e o alinhamento vertical do tórax (ρ = −0,74, p < 0,01, e entre o domínio «limitações» do QFQ-R e o ângulo do quadril (ρ = −0,55, p < 0,05. Conclusões: O presente trabalho mostra que as anormalidades na função pulmonar e na capacidade funcional se associam às alterações posturais em adultos portadores de FQ. Entretanto, a gravidade das anormalidades posturais não influenciam negativamente os domínios do QFQ-R. Abstract: Aim: The purpose of this study was to evaluate the

  11. Seguimento nutricional de pacientes com fibrose cística: papel do aconselhamento nutricional Nutritional follow-up of cystic fibrosis patients: the role of nutrition education

    Directory of Open Access Journals (Sweden)

    Fabíola V. Adde

    2004-12-01

    Full Text Available OBJETIVO: Avaliar o estado nutricional de um grupo de pacientes com fibrose cística e analisar a repercussão do aconselhamento nutricional através de um estudo comparativo pré- e pós-intervenção. MÉTODOS: Todos os pacientes com fibrose cística em seguimento regular no ambulatório de pneumologia do Instituto da Criança no período de 1996-99 foram prospectivamente acompanhados durante 3,5 anos. Em quatro etapas (I = inicial, II = 7 meses, III = 13 meses, IV = 43 meses, foi realizada uma avaliação nutricional que consistia de medidas de peso, estatura/comprimento, circunferência do braço e pregas cutâneas, e cálculos de escores z para peso/idade, estatura/idade, peso/estatura, circunferência do braço e da prega cutânea tricipital, porcentagem de peso/estatura e porcentagem de gordura corpórea. Era feita verificação do uso das enzimas pancreáticas e do uso de suplementos nutricionais. Aconselhamento nutricional verbal e através de uma cartilha explicativa foi realizado em todos os pacientes. RESULTADOS: Foram avaliados 74 pacientes, 38 do sexo feminino e 36 do masculino, com idades de 6 meses a 18,4 anos. Na etapa inicial, os dados antropométricos revelaram: porcentagem de peso/estatura = 94±13, porcentagem de gordura corpórea = 15±7,1, escore z peso/idade = -1,13±1,3, escore z estatura/idade = -0,94±1,2, escore z peso/estatura = -0,69±,1, escore z circunferência do braço = -1,35±1,3, escore z prega cutânea tricipital = -0,74±0,9. A aderência ao uso de enzimas e suplementos melhorou durante o estudo. Houve um aumento significativo no escore z de peso/idade e da prega tricipital e na porcentagem de gordura corpórea durante todo o período de estudo. Dividindo-se os pacientes em três grupos etários, a melhora antropométrica só foi significativa nos menores de 5 anos. CONCLUSÕES: Desnutrição leve estava presente nesse grupo de pacientes com fibrose cística. O aconselhamento nutricional realizado

  12. Fibrose quística – Caracterização clínica de uma amostra de doentes portugueses

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    Micaela Guardiano

    2005-07-01

    Full Text Available Resumo: Apesar dos rápidos avanços na compreensão dos determinantes moleculares da fibrose quística, esta continua a ser uma das doenças recessivas letais mais comuns na população caucasiana em todo o mundo. A realidade dos doentes portugueses continua a ser pouco conhecida, pela escassez de trabalhos publicados referentes à nossa população.Este trabalho tem como objectivos: fazer uma avaliação clínica de um grupo de doentes portugueses com fibrose quística, com 2 mutações identificadas e comparar a apresentação clínica de um grupo de doentes homozigóticos para a mutação F508del com doentes não homozigóticos para esta mutação.Para tal, seleccionou-se um grupo de doentes em seguimento na Consulta de Pneumologia Pediátrica do HSJ que foram caracterizados do ponto de vista fenotípico e classificados de acordo com critérios de gravidade.Todos os doentes desta amostra apresentaram mutações de classe I e/ou II, que se associam, classicamente, a fenótipo mais grave. Em conformidade com a gravidade genotípica, todos os doentes apresentaram um fenótipo de insuficiência pancreática mas com maior variabilidade de manifestações pulmonares.Não se encontraram variações significativas em termos de idade de diagnóstico, formas de apresentação e gravidade da doença entre doentes homozigóticos F508del e restantes doentes. Em contrapartida, doentes com o mesmo genótipo (homozigóticos F508del apresentaram diferentes espectros de manifestações clínicas e de gravidade fenotípica. Tanto ou mais do que a caracterização genotípica, o tempo de evolução da doença e os factores externos (nomeadamente a sujeição a estímulos infecciosos deverão interferir na gravidade do fenótipo num dado momento.Rev Port Pneumol 2005; XI (4: 381-406 Abstract: Even though there have been rapid advances in

  13. Capnografia volumétrica como meio de detectar obstrução pulmonar periférica precoce em pacientes com fibrose cística

    Directory of Open Access Journals (Sweden)

    Maria Ângela G. O. Ribeiro

    2012-12-01

    Full Text Available OBJETIVO: Comparar a espirometria e a capnografia volumétrica (CapV para determinar se os valores amostrados pela capnografia acrescentam informações sobre doenças pulmonares precoces em pacientes com fibrose cística (FC. MÉTODOS: Este foi um estudo do tipo corte transversal envolvendo pacientes com FC: Grupo I (42 pacientes, 6-12 anos de idade e Grupo II (22 pacientes, 13-20 anos de idade. Os grupos controle correspondentes eram formados por 30 e 50 indivíduos saudáveis, respectivamente. A capacidade vital forçada (CVF, o volume expiratório forçado no primeiro segundo (VEF1 e a relação VEF1/CVF foram determinados pela espirometria. Através da CapV, medimos a saturação periférica de oxigênio (SpO2, a frequência respiratória (FR, o tempo inspiratório (TI, o tempo expiratório (TE e o slope da fase III normalizado pelo volume corrente (slope da fase III/Vc. RESULTADOS: Em comparação com os grupos controle, todos os pacientes com FC apresentaram valores de slope da fase III/Vc (p < 0,001 mais altos independentemente do estágio de doença pulmonar. O slope da fase III/Vc foi significantemente mais alto nos 24 pacientes que tiveram resultados normais de espirometria (p = 0,018. Os pacientes do Grupo II apresentaram valores de CVF, VEF1, VEF1/CVF (p < 0,05 e SpO2 (p < 0,001 mais baixos que os pacientes do Grupo I. Os pacientes do Grupo II, comparados com os do Grupo Controle II, apresentaram FR (p < 0,001 mais alta e valores de TI e TE (p < 0,001 mais baixos. CONCLUSÕES: Todos os pacientes com FC mostraram ter valores mais altos de slope da fase III/Vc quando comparados com os pacientes dos grupos controle. A CapV identificou a heterogeneidade da distribuição da ventilação nas vias aéreas periféricas dos pacientes com FC que apresentaram espirometria normal.

  14. Colonização por Staphylococcus aureus resistente à meticilina: Que impacto na morbilidade de doentes pediátricos com fibrose quística?

    Directory of Open Access Journals (Sweden)

    Joana Fermeiro

    2010-07-01

    Full Text Available Resumo: Introdução: Ao Staphylococcus aureus resistente à meticilina (MRSA é classicamente reconhecido um papel patogénico no âmbito da fibrose quística (FQ.Objectivos: Avaliação da evolução da prevalência e incidência da colonização por MRSA, impacto clínico no ano após o primeiro isolamento, factores de risco e padrão de resistência antimicrobiana.Métodos: Estudo retrospectivo dos doentes pediátricos colonizados por MRSA seguidos no centro de FQ do Hospital de Santa Maria de 2003 a 2007.Resultados: O MRSA foi isolado em secreções respiratórias de 12 dos 60 doentes seguidos durante este período (colonização crónica em 3 doentes. A idade média à data do primeiro isolamento foi de 9 anos e 10 meses e o tempo médio entre o diagnóstico de FQ e a aquisição de MRSA de 5 anos e 7 meses.Verificou-se um aumento da prevalência e incidência de colonização por MRSA, com um máximo atingido em 2007 (prevalência 14,3% e incidência 8,9%. Quatro doentes cumpriram antibioticoterapia profiláctica antiestafilocócica com flucloxacilina.No ano após o primeiro isolamento de MRSA, constatou-se um aumento do número de dias de internamento em 4 doentes (2 com colonização crónica e deterioração da função pulmonar em 5, incluindo a totalidade dos doentes com colonização crónica. Apenas um doente apresentou diminuição de percentil de índice de massa corporal.As resistências mais frequentemente encontradas foram à rifampicina e à clindamicina.Conclusões: Este estudo revelou ocorrência de deterioração clínica relevante em doentes com colonização crónica por MRSA, reforçando a importância da implementação de estratégias eficazes e precoces de erradicação.Rev Port Pneumol 2010; XVI (4: 527-542 Abstract: Background: Methicillin-resistant Staphylococcus aureus (MRSA plays a well-recognised pathogenic role in cystic fibrosis (CF.Aims: To evaluate the prevalence and incidence of colonisation by MRSA

  15. The radioinduced membranes injuries as biological dose indicators: mechanisms of studies and practical applications; Les dommages membranaires radio-induits comme bio-indicateurs de dose: etudes des mecanismes et applications pratiques

    Energy Technology Data Exchange (ETDEWEB)

    Vincent-Genod, Lucie

    2001-10-15

    After an accidental overexposure, the assessment of the received dose in biological dosimetry is performed by a method based on the effects of irradiation on the DNA molecule. But this technique shows some limitations; therefore we tried to find new bio-sensors of radiation exposure. We have pointed out that membrane is a critical target of ionising radiation after an in vitro and in vivo overexposure. In vitro, these modifications were involved in the radio-induced apoptotic pathway. The measure of membrane fluidity allowed us to obtain an overall view of cellular membrane. Moreover, in vivo, by changing the lipid nutritional status of animals, our results displayed the important role played by membrane lipid composition in radio-induced membrane alterations. Besides, membrane effects were adjusted by the extracellular physiological control, and in particular by the damages on membrane fatty acid pattern. Finally, we have tested the use of membrane fluidity index as a bio-sensor of radiation exposure on in vivo models and blood samples from medical total body irradiated patients. The results achieved on animal models suggested that the membrane fluidity index was a bio-sensor of radiation exposure. Nevertheless, the observations realised on patients highlight that the effect of the first dose fraction of the radiotherapy treatment had some difficulties to be noticed. Indeed, the combined treatment: chemotherapy and radiotherapy disturbed the membrane fluidity index measures. To conclude, whereas this parameter was not a bio-sensor of irradiation exposure usable in biological dosimetry, it may allow us to assess the radio-induced damages and their cellular but also tissue impacts. (author)

  16. Importance of local skin treatments during radiotherapy for prevention and treatment of radio-induced epithelitis; Interet des applications cutanees en cours de radiotherapie pour la prevention et le traitement des epitheliites radio-induites

    Energy Technology Data Exchange (ETDEWEB)

    Chargari, C.; Fromantin, I.; Kirova, Y.M. [Institut Curie, Dept. de Radiotherapie Oncologique, 75 - Paris (France); Chargari, C. [Hopital d' Instruction des Armees du Val-de-Grace, Service d' Oncologie Radiotherapie, 75 - Paris (France)

    2009-07-15

    Radio-epithelitis represents a common problem, for which treatments are characterized by a great heterogeneity. The present review of literature focuses on data referenced in Pub med/Medline and published in French/English. Despite a real preclinical rationale, aloe vera and trolamine failed to demonstrate any benefit in the prophylactic settings. In a prospective assessment phase III assessment, Calendula officinalis was shown to be superior to trolamine for the prevention of radio-epithelitis. In the curative settings, sucrafalte failed to demonstrate any benefit. The benefit of dermo-corticoids was suggested in terms of erythema and itching. Promising clinical results are available with hyaluronic acid (M.A. S065D and Ialugen) and silver leaf may reduce the intensity of cutaneous radio-induced side effects. Data from the literature are conflicting, making real the difficulty to adopt from clinical trials any proof-of-principle strategy. Considering these uncertainties, several strategies are allowed. New topics are under investigation. Present data from the literature highlight the need for further trials, in order to propose evidence-based treatments and to harmonize clinical practice. (authors)

  17. Radioinduced optic nerve neuropathy after treatment for nasopharynx cancer. About two cases and literature review; Neuropathie radio-induite du nerf optique apres traitement pour cancer du nasopharynx. A propos de deux cas et revue de la litterature

    Energy Technology Data Exchange (ETDEWEB)

    Mnejja, W.; Siala, W.; Daoud, J. [Centre Hospitalier Universitaire Habib-Bourguiba, Service de Radiotherapie Carcinologique, Sfax (Tunisia); Fki, J. [Centre Hospitalier Universitaire Habib-Bourguiba, Service d' Ophtalmologie, Sfax (Tunisia); Ghorbel, M. [Centre Hospitalier Universitaire Habib-Bourguiba, Service d' ORL, Sfax (Tunisia); Frikha, M. [Centre Hospitalier Universitaire Habib-Bourguiba, Service de Carcinologie Medicale, Sfax (Tunisia)

    2007-11-15

    The radioinduced neuropathy of the optic nerve is a rare and delayed complication. Its incidence is difficult to evaluate in the literature. It depends on the whole irradiation dose, fractionation and irradiated volume. Currently, it does not exist any efficient treatment, only the prevention play an important part on avoiding the high doses, and the broad irradiation volumes. The innovating techniques using conformal radiotherapy with or without modulated intensity could contribute to reduce this toxicity incidence. (N.C.)

  18. Detection, characterization and measure of a new radiation-induced damage in isolated and cellular DNA; Detection, caracterisation et mesure d'un nouveau dommage radio-induit de l'ADN isole et cellulaire

    Energy Technology Data Exchange (ETDEWEB)

    Regulus, P

    2006-10-15

    Deoxyribonucleic acid (DNA) contains the genetic information and chemical injury to this macromolecule may have severe biological consequences. We report here the detection of 4 new radiation-induced DNA lesions by using a high-performance liquid chromatography coupled to tandem mass spectrometry (HPLC-MS/MS) approach. For that purpose, the characteristic fragmentation of most 2'-deoxy-ribo nucleosides, the loss of 116 Da corresponding to the loss of the 2-deoxyribose moiety, was used in the so-called neutral loss mode of the HPLC-MS/MS. One of the newly detected lesions, named dCyd341 because it is a 2'-deoxycytidine modification exhibiting a molecular weight of 341 Da, was also detected in cellular DNA. Characterization of this modified nucleoside was performed using NMR and exact mass determination of the product obtained by chemical synthesis. A mechanism of formation was then proposed, in which the first event is the H-abstraction at the C4 position of a 2-deoxyribose moiety. Then, the sugar modification produced exhibits a reactive aldehyde that, through reaction with a vicinal cytosine base, gives rise to dCyd341. dCyd341 could be considered as a complex damage since its formation involves a DNA strand break and a cross-link between a damaged sugar residue and a vicinal cytosine base located most probably on the complementary DNA strand. In addition to its characterization, preliminary biological studies revealed that cells are able to remove the lesion from DNA. Repair studies have revealed the ability of cells to excise the lesion. Identification of the repair systems involved could represent an interesting challenge. (author)

  19. Efficacy of ondansetron in the management of radiotherapy induced emesis: a review. Efficacite de l'ondansetron dans les nausees et vomissements radio-induits: revue de la litterature

    Energy Technology Data Exchange (ETDEWEB)

    Levy, E. (Hopital Henri-Mondor, 94 - Creteil (France)); Paillarse, J.M.; Votan, B. (Laboratoires Glaxo, 75 - Paris (France))

    1994-01-01

    Radiotherapy-induced emesis depends on the site of irradiation, the field size and the dose per fraction and is generally less intense than chemotherapy-induced emesis. Established anti-emetic drugs offer only limited symptom control (50%). Ondansetron, a 5HT[sub 3] receptors antagonist, had proven a complete or a major control efficacy (0-2 emetic episodes) of 68 to 95% in three pilot studies (fractionated, single-dose and total body irradiations). In controlled studies, ondansetron efficacy was significantly higher than placebo, metoclopramide and prochlorperazine. The treatment was well tolerated in the different studies. (authors). 23 refs., 2 figs., 1 tab.

  20. Recovery of ovarian function after postradiation menopause. Do FSH levels have a definitive prognostic value?; Recuperation de fonctionnement ovarien apres menopause radio-induite. L`hormone folliculostimulante (FSH) a-t-elle une valeur pronostique definitive?

    Energy Technology Data Exchange (ETDEWEB)

    Letur-Konirsch, H. [Hopital Necker-Enfants-Malades, 75 - Paris (France); Guis, F. [Hopital Antoine-Beclere, Clamart (France)

    1996-08-01

    Menopause, conventionally defined as the permanent cessation as a result of loss of ovarian follicular activity, is biologically expressed by the collapse of plasma levels and increased plasma levels of the gonadotrophins FSH (follicle stimulating hormone) and LH (luteinizing hormone). At present, estimation of the ovarian follicle reserve is based on endocrine capacity tests of the ovaries, with increased FSH representing the first sign of exocrine ovarian failure. We report the case of one of our amenorrhoeic patients after chemotherapy, total body radiation and allogeneic bone marrow transplantation for acute immuno-blastic leukaemia. This patient was included in an in vitro fertilization with oocyte donation (IVF-OD) programme for iatrogenic premature ovarian failure with increased FSH levels. Instead of high levels of gonadotrophins, this young woman recovered spontaneous follicular developments, benefited from standard IVF with her own oocytes and brought a twin pregnancy to term. This observation shows that a high FSH level is not a definitive prediction of ovarian exocrine capacity. In young women of child-bearing age such as these wanting a child and showing signs of endogenous estrogen impregnation, evaluation of the existence and quality of follicular development is an important factor. (author). 13 refs.

  1. New experimental approach to treatment of radiation-induced bone marrow aplasia: ex vivo expansion of hematopoietic cells; Nouvelle approche experimentale du traitement de l`aplasie medullaire radio-induite

    Energy Technology Data Exchange (ETDEWEB)

    Herodin, F.; Mathieu, J.; Drouet, M.; Grenier, N.; Grange, L.; Bourin, P.; Vetillard, J.; Thierry, D.; Mestries, J.C.

    1995-12-31

    The management of bone marrow aplasia secondary to accidental exposure to high doses of ionizing radiations requires new therapeutic protocols in addition to cytokine therapy. The in vitro incubation of hematopoietic stem and progenitor cells from irradiated nonhuman primates with negative and positive regulators of hematopoiesis may lead to helpful products of transfusion. (author).

  2. A musculatura epaxial e a fibrose epidural na compressão medular em cães submetidos à laminectomia dorsal modificada

    Directory of Open Access Journals (Sweden)

    Diego V. Beckmann

    2010-02-01

    Full Text Available O objetivo deste experimento foi isolar a musculatura epaxial da medula espinhal de cães submetidos à laminectomia dorsal modificada (LDM e averiguar se os músculos influenciaram na formação da fibrose epidural, na compressão medular e no aparecimento dos sinais neurológicos. Para isso, dez cães hígidos foram submetidos à LDM entre as vértebras T13 e L1 e distribuídos aleatoriamente em dois grupos denominados controle (I onde a medula espinhal permaneceu exposta sem a presença de implante, e tratado (IIonde foi colocado um im-plante a base de alumínio entre a musculatura epaxial adjacente e a medula espinhal exposta pela LDM. As avaliações constaram de exames neurológicos diários até 180 dias de pós-operatório (PO; mielografia, decorridos 15, 30 e 60 dias de PO; e avaliação macroscópica mediante a reintervenção cirúrgica. Não houve diferença durante as avaliações neurológicas. Aos 15 dias de PO, foi verificado na mielografia, que o grau de compressão da linha de contraste foi maior no grupo tratado (PThe purpose of this study was to isolate the adjacent epaxial musculature from exposed spinal cord by modified dorsal laminectomy in dogs with aluminum implant and to verify whether the muscles contribute to form epidural fibrosis, spinal cord compression, and development of neurological signs. Ten dogs were submitted to modified dorsal laminectomy between T13 and L1 and then distributed along two groups. Dogs in the group 1 remained with the spinal cord exposed without the implant; dogs in the group 2 had an aluminum implant inserted between the epaxial muscles and the exposed spinal cord. Neurological examination was daily performed until 180 days post surgery. Additionally, myelography at 15, 30, and 60 days post surgery and macroscopic evaluation of the implant at six months post surgery were done. There was no difference between groups in the neurological examination. A statistical difference in the degree of

  3. Estado de Nutrição, Hábitos Alimentares e Dispêndio Energético em Crianças com Fibrose Quística

    OpenAIRE

    Guerra, Paula; Almeida, João; Rego, Carla; Nunes, Teresa; G. Vaz, Luisa; Silva, Diana; Lourenço, Susana; Ribeiro, Laura; M. Guerra, António J.

    2014-01-01

    O presente trabalho tem como objectivo avaliar o estado de nutrição, a composição corporal e a função respiratória de um grupo de crianças com fibrose quística sem evidência clínica ou laboratorial de infecção recente, assim como o de conhecer os seus hábitos alimentares e determinar o valor do dispêndio energético de repouso medido por calorimetria indirecta e o avaliado pelas equações de Schofield, da Organização Mundial de Saúde e de Fleish.População e métodos: a população é constituída po...

  4. Os pacientes invisíveis: transtorno de estresse pós-traumático em pais de pacientes com fibrose cística The invisible patients: posttraumatic stress disorder in parents of individuals with cystic fibrosis

    Directory of Open Access Journals (Sweden)

    Mariana Cabizuca

    2010-01-01

    Full Text Available CONTEXTO: Apesar do crescente reconhecimento da relevância do transtorno de estresse pós-traumático (TEPT secundário a doenças médicas, ainda não existem estudos em fibrose cística. OBJETIVO: Verificar a prevalência de TEPT e dos três grupos de sintomas de estresse pós-traumático em pais de pacientes com fibrose cística. MÉTODOS: Pais de pacientes com fibrose cística (idade média: 2 a 33 anos foram recrutados da Associação Carioca de Mucoviscidose. Neste estudo transversal, os pais preencheram um questionário sociodemográfico e foram entrevistados por meio do módulo de TEPT do Structured Clinical Interview for DSM-IV. RESULTADOS: A amostra era composta de 62 indivíduos (46 mães e 16 pais. A prevalência atual de TEPT foi 6,5% e de TEPT parcial, de 19,4%. Os pais com e sem sintomas de TEPT diferiram significativamente em dois aspectos psicossociais: os primeiros relataram mais problemas emocionais (p = 0,001 e reconheceram mais frequentemente a necessidade de tratamento psiquiátrico ou psicológico (p = 0,002 que os últimos. Entretanto, somente 6,3% dos pais com sintomas de TEPT estavam em tratamento psiquiátrico/psicológico. CONCLUSÕES: Este estudo preliminar demonstrou que a frequência dos sintomas de TEPT é bem elevada em pais de pacientes com fibrose cística e, apesar de esses pais reconheceram que tem problemas emocionais e precisam de tratamento psiquiátrico/psicológico, seu sofrimento permanece invisível para o sistema médico, levando ao subdiagnóstico e ao subtratamento.BACKGROUND: Besides the growing acknowledgment of the relevance of posttraumatic stress disorder (PTSD related to medical illness, there is no study in cystic fibrosis yet. OBJECTIVE: To assess the prevalence of PTSD and the three clusters of posttraumatic stress symptoms (PTSS in parents of patients with cystic fibrosis. METHODS: Parents of patients with cystic fibrosis (age range: 2 to 33 years were drawn from the Cystic Fibrosis

  5. Avaliação de parâmetros histológicos na pneumonia intersticial usual (fibrose pulmonar idiopática Evaluation of the histological parameters in usual interstitial pneumonia (idiopathic pulmonary fibrosis

    Directory of Open Access Journals (Sweden)

    RIMARCS GOMES FERREIRA

    2000-12-01

    Full Text Available A fibrose pulmonar idiopática (alveolite fibrosante criptogênica é uma doença pulmonar intersticial progressiva de etiologia desconhecida, morfologicamente reconhecida como pneumonia intersticial usual. Desde a publicação de Hamman e Rich (1944 até os dias atuais, uma das grandes preocupações foi a tentativa de encontrar um marcador histológico para correlacionar com prognóstico e resposta terapêutica. A busca desta situação não tem sido muito alentadora, pois existem vários pontos duvidosos na patogênese desta doença. Admite-se que a resposta terapêutica desta entidade se relaciona com a celularidade e fibrose presentes no tecido. A proposta deste estudo é descrever os resultados de método semiquantitativo segundo a avaliação independente de dois patologistas, das alterações exsudativo-inflamatórias, reparativo-fibróticas e de vias aéreas, em 24 pacientes com diagnóstico de fibrose pulmonar idiopática, submetidos à biópsia a céu aberto. Foram analisados 14 parâmetros histológicos segundo escala de 0 a 5 para as alterações intersticiais e de 0 a 2 para o comprometimento de vias aéreas e de espaços aéreos. Da análise independente realizada pelos dois observadores constatou-se concordância significante em todas as variáveis histológicas com Kw (teste de Kappa indo de 0,47 a 0,92. Apenas na análise da intensidade da inflamação septal as discordâncias também foram significantes, sugerindo que para este parâmetro a percentagem de comprometimento tecidual deve ser previamente discutida entre os observadores. O método utilizado demonstrou ser rápido e eficiente.Idiopathic pulmonary fibrosis (cryptogenic fibrosing alveolitis is a progressive interstitial pulmonary disease of unknown etiology. Since Hamman's and Rich's (1944 reports, many studies have tried to find a histological marker for the correlation between prognosis and response to therapy. However, there are many doubts regarding pathogenesis

  6. Fibrose pulmonar idiopática simultânea a enfisema em pacientes tabagistas Idiopathic pulmonary fibrosis and emphysema in smokers

    Directory of Open Access Journals (Sweden)

    Denise Rossato Silva

    2008-10-01

    Full Text Available OBJETIVO: Descrever os achados clínicos e funcionais de pacientes com enfisema em lobos superiores e fibrose pulmonar idiopática (FPI em lobos inferiores, recentemente descrita na literatura. MÉTODOS: Um grupo de 11 pacientes com a presença simultânea de enfisema e FPI foi identificado retrospectivamente. Todos os pacientes realizaram tomografia computadorizada de tórax com alta resolução e provas de função pulmonar. RESULTADOS: Entre os 11 pacientes identificados, havia 8 homens e 3 mulheres, com média de idade de 70,7 ± 7,2 anos (variação, 61-86 anos. Todos os pacientes eram tabagistas (carga tabágica, 61,5 ± 43,5 anos-maço. As médias da capacidade vital forçada (CVF, do volume expiratório forçado no primeiro segundo (VEF1 e da relação VEF1/CVF foram 72,1% ± 12,7%, 68,2% ± 11,9% e 74,4 ± 10,8, respectivamente. Os volumes pulmonares foram normais em 7 pacientes. Um padrão restritivo foi observado em 3 pacientes e hiperinsuflação estava presente em um. A capacidade de difusão pulmonar apresentou redução moderada a grave em todos os pacientes (média, 27,7% ± 12,9% do previsto. No teste da caminhada de seis minutos, realizado por 10 pacientes, a distância caminhada média foi de 358,4 ± 143,1 m, ocorrendo dessaturação >4% em 9 pacientes. Achados ecocardiográficos sugestivos de hipertensão pulmonar estavam presentes em 4 pacientes (média da pressão sistólica da artéria pulmonar, 61,8 mmHg; variação, 36-84 mmHg. CONCLUSÕES: A presença simultânea de enfisema e FPI causa alterações características nas provas de função pulmonar. O achado mais importante é a discrepância entre a capacidade de difusão e a espirometria.OBJECTIVE: To describe the clinical and functional findings recently reported in the medical literature for patients diagnosed with emphysema involving the upper lobes and idiopathic pulmonary fibrosis (IPF involving the lower lobes. METHODS: Eleven patients with emphysema and IPF

  7. Valor preditivo de marcadores séricos de fibrose hepática em pacientes portadores de hepatite crônica viral C Predictive value of serum markers of hepatic fibrosis in patients with chronic hepatitis C

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    Leila Maria Soares Tojal de Barros Lima

    2008-06-01

    Full Text Available INTRODUÇÃO: Os marcadores séricos têm sido empregados na avaliação da fibrose hepática em pacientes portadores de hepatite crônica C (HCC. OBJETIVOS: Avaliar a capacidade do índice aspartato aminotransferase (AST/alanina aminotransferase (ALT, dos níveis séricos de gama-glutamiltransferase (GGT, contagem de plaquetas, do índice AST/plaquetas (APRI e do ácido hialurônico (AH em predizer a intensidade da fibrose hepática na HCC e a variação desses marcadores após tratamento com interferon. PACIENTES E MÉTODOS: Em 72 pacientes portadores de hepatite C determinamos no soro o índice AST/ALT, GGT, plaquetas, índice APRI (obtido pelo quociente AST/plaquetas e o AH, que foram comparados ao estadiamento histológico, segundo os critérios de METAVIR. Receberam tratamento com interferon e ribavirina 65 pacientes. Os indivíduos que concluíram o tratamento (n = 33 realizaram nova dosagem dos marcadores séricos de fibrose para comparar com os níveis pré-tratamento. RESULTADOS: Observamos que a GGT, a contagem de plaquetas, o índice APRI e o AH se correlacionaram com estádio de doença hepática (p INTRODUCTION: Serum markers have been used in the assessment of liver fibrosis in patients with chronic hepatitis C (CHC. AIMS: We evaluated the capacity of aspartate aminotransferase (AST/alanine aminotransferase (ALT ratio, gama-glutamyltransferase (GGT levels, platelet count, the AST to platelet ratio index (APRI and serum hyaluronic acid (HA to predict the intensity of hepatic fibrosis in patients with CHC and the variation of these markers after therapy with interferon. PATIENTS AND METHODS: In 72 patients with hepatitis C, AST/ALT ratio, GGT levels, platelet count, the APRI index (calculated as the ratio of AST to platelets and serum HA concentration were determined and compared to histological staging according to the scoring system of METAVIR. Sixty-five patients received interferon and ribavirin therapy. The individuals that

  8. Avaliação e recomendações nutricionais para crianças e adolescentes com fibrose cística Nutritional assessment and recommendations for children and adolescents with cystic fibrosis

    Directory of Open Access Journals (Sweden)

    Célia Regina M. M. Chaves

    2012-01-01

    Full Text Available OBJETIVO: Revisar e discutir evidências sobre a avaliação do estado nutricional e as recomendações para o tratamento nutricional de crianças e adolescentes com fibrose cística. FONTES DE DADOS: Bancos de dados MEDLINE (versão PubMed e Latin American and Caribbean Center on Health Sciences Information (LILACS, entre 1984 a 2009. Aplicou-se a combinação dos seguintes descritores: fibrose cística, estado nutricional, criança e adolescente - em inglês e português. SÍNTESE DOS DADOS: A fibrose cística é uma doença genética, de evolução crônica, progressiva e fatal. Resulta do defeito na proteína reguladora transmembrana que regula a condução de cloro e, consequentemente, o fluxo de sódio e água através da membrana apical das células epiteliais. Pacientes fibrocísticos são vulneráveis à desnutrição, que resulta do desequilíbrio entre ingestão alimentar, gasto e perdas energéticas. Novos conhecimentos sobre a fisiopatologia da doença, adquiridos nas últimas décadas, resultaram em mudanças significativas nas atuais recomendações energéticas e principalmente de lipídeos. A importância da nutrição no bem-estar e sobrevida dos fibrocísticos está bem estabelecida, assim como a associação entre a desnutrição e a deterioração da função pulmonar. Existem múltiplos fatores inter-relacionados que afetam o estado nutricional, tais como, mutação genética, insuficiência pancreática, ressecção intestinal, perda de sais e ácidos biliares, refluxo gastroesofágico, inflamação e infecções pulmonares, diabetes e condições emocionais. CONCLUSÕES: O monitoramento nutricional e o aconselhamento dietético são elementos chave no manejo de crianças e adolescentes com fibrose cística com o intuito de controlar a sintomatologia e a progressão da doença, proporcionando melhor qualidade de vida.OBJECTIVE: To review and discuss evidence on the nutritional status assessment and recommendations for

  9. Forma acelerada da fibrose pulmonar idiopática no pulmão nativo após transplante pulmonar unilateral Accelerated form of interstitial pulmonary fibrosis in the native lung after single lung transplantation

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    Rogério Rufino

    2007-12-01

    Full Text Available Relatamos o caso de um paciente de 56 anos submetido a transplante pulmonar unilateral esquerdo em decorrência de fibrose pulmonar idiopática (FPI. No pós-operatório imediato, sob intensa imunossupressão, houve progressão rápida da FPI no pulmão nativo direito, confirmada pela biópsia pulmonar videotoracoscópica, necessitando de ventilação mecânica durante 104 dias até a realização de outro transplante pulmonar à direita. Obteve alta hospitalar após o 26º dia do segundo pós-operatório.We report the case of a 56-year-old patient who underwent left single lung transplantation for idiopathic pulmonary fibrosis (IPF. Despite the high level of immunosuppression after the surgery, there was rapid progression to IPF in the native (right lung as demonstrated by thoracoscopic lung biopsy. After 104 days on mechanical ventilation (MV, the patient underwent right lung transplant and was discharged from the hospital on postoperative day 26.

  10. EVOLUÇÃO DA FIBROSE BILIAR SECUNDÁRIA EM RATOS TRATADOS MEDIANTE DERIVAÇÃO BÍLIO-DUODENAL OU BÍLIO-JEJUNAL COM AL��A DE ROUX MEDINDO 5, 10 E 15 CM

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    Pandolfi Jr. H.

    2001-01-01

    Full Text Available A modalidade de derivação bílio-digestiva empregada no tratamento da colestase extra-hepática crônica pode influenciar na reparação das lesões hepáticas. Avaliou-se o desempenho das derivações bílio-duodenal e bílio-jejunal em Y de Roux com alça exclusa de diferentes comprimentos na reparação das lesões morfológicas e funcionais do fígado de ratos com fibrose biliar secundária. Foram utilizados ratos Wistar, com 15 dias de obstrução biliar, alocados em 5 grupos de 6 animais. O grupo OB caracterizou as alterações da fibrose biliar. Os animais remanescentes foram tratados mediante derivação com o duodeno (grupo DBD, e com o jejuno, em alça exclusa de 5cm (grupo DBJ5, 10cm (grupo DBJ10 e 15cm (grupo DBJ15, sendo reavaliados 3 meses depois. Outros 6 animais foram submetidos à intervenção simulada e considerados grupo controle (IS. Todos animais foram submetidos à avaliação morfométrica do fígado, análise bioquímica do sangue e microbiológica da bile, estudo da função mitocondrial hepática e verificação do peso úmido do fígado e do baço. Na análise estatística adotou-se o nível de significância de 5%. Houve aumento significativo do peso estimado, em g/Kg de peso corporal, dos ductos biliares, da fibrose e dos hepatócitos nos animais do grupo OB (medianas de 1,30; 10,03 e 37,0 em relação aos animais controles (IS (medianas de 0,03; zero e 29,37. Após tratamento, ocorreu regressão significativa do peso estimado dos ductos biliares e da fibrose, com valores medianos de 0,22 e 0,22 para o grupo DBD, 0,45 e 3,31 para o grupo DBJ5 e 0,22 e 5,0 para o grupo DBJ15. Houve regressão significativa do peso estimado dos hepatócitos apenas nos grupos derivados com o jejuno, com valores medianos de 31,93; 24,46 e 28,52. Ocorreu aumento significativo do peso úmido do fígado e do baço no grupo OB (medianas em g/Kg de peso corporal de 49,85 e 5,71 em relação ao grupo IS (30,0 e 3,04. Houve regress

  11. Prevalência de doença mineral óssea em adolescentes com fibrose cística Prevalence of bone mineral disease among adolescents with cystic fibrosis

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    Reinaldo José do Amaral Caldeira

    2008-02-01

    Full Text Available OBJETIVO: Avaliar a prevalência de doença mineral óssea em adolescentes com fibrose cística e associar os achados com as variáveis estudadas. MÉTODOS: Foram selecionados 37 adolescentes, dos quais foram avaliados: estado nutricional pelos índices de altura/idade e massa corporal/idade; densidade mineral óssea da coluna lombar e corpo inteiro por densitometria com emissão de raio X de dupla energia; ingestão dietética diária pelo registro alimentar de 3 dias; e prova de função pulmonar pelo volume expiratório forçado no primeiro segundo. RESULTADOS: A média de idade foi de 13,2 (±2,8 anos. O estado nutricional adequado foi de 70,3 e 75,7% pelos índices de altura/idade e de massa corporal/idade, respectivamente; 54,1% dos pacientes apresentaram redução da densidade mineral óssea para coluna lombar e 32,5% para corpo inteiro. Houve correlação positiva entre densidade mineral óssea e índice de massa corporal (p = 0,04. A doença pulmonar e a insuficiência pancreática apresentaram correlação com a alteração da densidade mineral óssea. O inquérito alimentar revelou percentuais de adequação para o cálcio, fósforo e calorias, de acordo com a recomendação nutricional preconizada pelo Consenso Europeu de Fibrose Cística. Essas variáveis não se mostraram estatisticamente significantes na análise multivariada. CONCLUSÃO: A prevalência de doença mineral óssea é alta na adolescência. O estado nutricional adequado, a reposição de enzimas pancreáticas e o controle da doença pulmonar podem ter efeito protetor para a massa óssea.OBJECTIVE: To evaluate the prevalence of bone mineral disease among adolescents with cystic fibrosis and to relate the findings with the variables studied. METHODS: The study enrolled 37 adolescents who were assessed for: nutritional status according to height/age and body mass/age ratios; bone mineral density of the lumbar spine and entire body by densitometry with dual emission X

  12. Fibrose pulmonar secundária à amiodarona – A propósito de um caso clínico

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    Marta N. Silva

    2006-11-01

    Full Text Available Resumo: A amiodarona é um eficaz fármaco anti-arrítmico usado para tratar arritmias ventriculares e supra-ventriculares, mas não isento de reacções adversas. A toxicidade pulmonar é uma delas, relativamente rara (5 a 10% dos casos, e pode ser fatal. Há vários factores que aumentam a susceptibilidadade para esta toxicidade, tais como a idade avançada e a doença pulmonar preexistente.Apresentamos um caso clínico de toxicidade pulmonar induzida pela amiodarona (fibrose pulmonar num doente do sexo masculino, de 81 anos. Estava medicado com este fármaco, desde há 5 anos, com uma dose diária de 200 mg.Após a suspensão do fármaco e tratamento com corticosteróides sistémicos, houve melhoria clínica, funcional e radiológica.Este caso clínico realça a necessidade de uma monitorização e diagnóstico precoce dos efeitos adversos do tratamento com a amiodarona e a dificuldade no seu diagnóstico, devido à inespecificidade dos sintomas, das manifestações clínicas e dos resultados dos exames complementares de diagnóstico.Rev Port Pneumol 2006; XII (6: 725-730 Abstract: Amiodarone is an antiarrhytmic drug and it is used to treat supraventricular or ventricular rhythm disturbances. Nevertheless it is not free of side effects. Amiodarone-induced pulmonary toxicity is one of them and is relatively rare (5 to 10% of cases and can be fatal. There are several cumulative factors that may enhance susceptibility to pulmonary toxicity, such as advanced age and pre-existing pulmonary dysfunction.We present a case study of amiodarone-induced toxicity (pulmonary fibrosis in an 81-year-old man. The patient had been treated with amiodarone for five years in daily dose 200 mg/day.After withdrawing the drug and systemic corticoste-roid therapy, clinical, functional and radiological improvement was observed.This case confirms the needs of a vigilant monitoring and early diagnosis of

  13. Fibrose maciça progressiva em trabalhadores expostos à sílica: achados na tomografia computadorizada de alta resolução Progressive massive fibrosis in silica-exposed workers: high-resolution computed tomography findings

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    Ângela Santos Ferreira

    2006-12-01

    Full Text Available OBJETIVO: Avaliar as características radiológicas das massas conglomeradas pela tomografia computadorizada de alta resolução de tórax. MÉTODOS: Foram selecionados 75 pacientes silicóticos, a maioria jateadores de areia, portadores de fibrose maciça progressiva, atendidos no Hospital Universitário Antônio Pedro entre 1986 e 2004. Os pacientes foram submetidos a avaliação clínica, radiografia simples de tórax e tomografia computadorizada de alta resolução. RESULTADOS: Mais da metade dos pacientes com silicose complicada mostrou na radiografia de tórax grandes opacidades dos tipos B e C, denotando a gravidade da doença nesses pacientes. Dos 75 casos, apenas um apresentou massa unilateral simulando câncer de pulmão. Quarenta e quatro pacientes realizaram tomografia computadorizada de alta resolução do tórax. As massas predominaram nos terços superiores e posteriores (88,6%. Broncograma aéreo e calcificações no interior das massas foram observados em 70,4% e 63,8% dos casos, respectivamente. História de tuberculose foi relatada em 52% dos pacientes estudados. CONCLUSÃO: Na grande maioria dos casos as massas eram bilaterais, predominando nas regiões póstero-superiores dos pulmões, com broncogramas aéreos e calcificações de permeio. Associação com calcificações linfonodais foi um achado freqüente. A exposição a elevadas concentrações de poeira e a tuberculose foram consideradas fatores de risco para o desenvolvimento da fibrose maciça progressiva.OBJECTIVE: To evaluate the radiological characteristics of conglomerate masses using high-resolution computed tomography of the chest. METHODS: From among the patients treated between 1986 and 2004 at the Antonio Pedro University Hospital, 75 patients with silicosis and massive fibrosis, most working in the field of sandblasting, were selected for study. These patients were submitted to a clinical evaluation, chest X-ray and high-resolution computed tomography of

  14. Kwashiorkor e distúrbio de coagulação: apresentação atípica de fibrose cística Kwashiorkor and coagulation disturbance: atypical presentation of cystic fibrosis

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    Michelle de Oliveira T. Sundell

    2012-01-01

    Full Text Available OBJETIVO: Enfatizar a apresentação clínica precoce da fibrose cística (FC em lactente com Kwashiorkor e distúrbio de coagulação, decorrente de hipovitaminose K. DESCRIÇÃO DO CASO: Paciente com três meses e meio, sexo feminino, nascida a termo, peso de 2655g, estatura de 46cm, foi encaminhada para investigação de lesões perineais associadas à monilíase de difícil controle, refratária a diversos antifúngicos e corticoides. Quadro geral caracterizado por baixo ganho ponderal, edema e diarreia. Admissão hospitalar para investigação com hipótese diagnóstica de Kwashiorkor de origem primária ou secundária. Paciente mantida em aleitamento materno exclusivo, sendo observadas perda ponderal e persistência da diarreia. Na internação, foi iniciado tratamento de infecção do trato urinário. A paciente evoluiu com hemorragia digestiva alta e sangramento pela flebotomia em safena direita, sendo identificada coagulopatia responsiva à vitamina K e plasma fresco congelado. Na evolução, foi confirmada esteatorreia e hipoalbuminemia; as sorologias para sífilis, toxoplasmose, mononucleose, citomegalovírus, rubéola, HIV e hepatite B, apresentaram resultado negativo e a pesquisa da mutação ∆F508 heterozigoto para FC foi positiva. A paciente apresentou piora do estado geral com sinais de sepse, evoluindo para óbito. O laudo necroscópico evidenciou elementos característicos de choque séptico com infecção pulmonar, sinais acentuados de desnutrição e fibrose cística do pâncreas. COMENTÁRIOS: A FC pode manifestar-se com quadro de Kwashiorkor e distúrbio de coagulação por deficiência de vitamina K. Os profissionais de saúde devem estar atentos à possibilidade de FC no diagnóstico diferencial dessa situação.OBJECTIVE: To address the clinical presentation of cystic fibrosis (CF in an infant presenting Kwashiorkor along with coagulation disturbance due to vitamin K deficiency. CASE DESCRIPTION: A female baby aged

  15. Nebulizadores: fonte de contaminação bacteriana em pacientes com fibrose cística? Nebulizers in cystic fibrosis: a source of bacterial contamination in cystic fibrosis patients?

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    Lorena Xavier Costa Brzezinski

    2011-06-01

    Full Text Available OBJETIVO: Determinar se os nebulizadores de pacientes com fibrose cística são fonte de contaminação microbiana e verificar se a técnica e a frequência de desinfecção dos nebulizadores é apropriada. MÉTODOS: Estudo de corte transversal observacional, sem grupo controle. Foram coletadas amostras de 28 pacientes com fibrose cística, no domicílio do paciente, sem aviso prévio sobre o motivo da visita. Foram colhidas três amostras por paciente: do reservatório do nebulizador, da máscara/bocal e do próprio paciente (swab da orofaringe/escarro. As amostras foram acondicionadas adequadamente e levadas para análise. Os pacientes, seus pais ou responsáveis preencheram um questionário sobre métodos de limpeza e desinfecção dos nebulizadores. RESULTADOS: Foram obtidas 84 amostras dos 28 pacientes. Destes, 15 (53,5% eram do gênero masculino. A mediana de idade foi de 11 anos (variação: 1-27 anos. Dos 28 pacientes, 15 apresentaram culturas de escarro/orofaringe positivas. As bactérias encontradas com maior frequencia foram Streptococcus aureus (8/15 e Pseudomonas aeruginosa (4/15. A cultura obtida dos nebulizadores identificou diversos patógenos, sem nenhum predominante. Não houve associações entre os resultados das culturas obtidas dos nebulizadores e aquelas dos pacientes em 27 casos (96,7%. A limpeza e a desinfecção não eram realizadas de forma adequada em 22 casos (78,6%. CONCLUSÕES: Nesta amostra de pacientes, apesar das técnicas de desinfecção inadequadas, os nebulizadores não foram uma fonte de contaminação microbianaOBJECTIVE: To determine whether nebulizers are a source of microbial contamination in patients with cystic fibrosis, as well as whether the technique and frequency of disinfection of these devices is appropriate. METHODS: This was a cross-sectional, uncontrolled observational study. Samples were collected from 28 patients with cystic fibrosis. Samples were collected at the homes of the patients, who

  16. Estudo de mutações do gene CFTR e da concentração sérica da lectina ligante de manose (MBL) em crianças com fibrose cística identificadas pela triagem neonatal

    OpenAIRE

    Ribas, Danieli Isabel Romanovitch

    2014-01-01

    Orientador: Prof. Dr. Nelson Augusto Rosário Filho Co-orientadora: Profª. Drª. Lilian Pereira Ferrari Tese (doutorado) - Universidade Federal do Paraná, Setor de Ciências da Saúde, Programa de Pós-Graduação em Medicina Interna. Defesa : Curitiba, 19/12/2014 Inclui referências Resumo: A fibrose cística é uma doença hereditária autossômica recessiva, causada por mutações no gene CFTR (Cystic Fibrosis Transmembrane Conductance Regulator). Apresenta grande variação clínica, mesm...

  17. Perfil epidemiológico das infecções bacterianas do aparelho respiratório em doentes com fibrose quística

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    Sofia Quintas

    2003-07-01

    Full Text Available RESUMO: Com o intuito de caracterizar a evolução do perfil epidemiológico das infecções bacterianas do aparelho respiratório dos doentes com Fibrose Quística (FQ, os autores realizaram um estudo retrospectivo da prevalência e incidência das mesmas em 78 doentes com FQ seguidos no Centro Especializado de FQ da Clínica Universitária de Pediatria do Hospital de Santa Maria, Lisboa durante um período de 5 anos (1995-1999.A Pseudomonas aeruginosa foi a bactéria mais frequentemente isolada nos três primeiros anos do estudo (60-73%, sendo ultrapassada nos dois anos seguintes pelo Staphylococcus aureus. No entanto, a Pseudomonas aeruginosa constituiu sempre o principal agente de colonização crónica (44-59%, com um pico de início da mesma entre os 0 e os 5 anos (34%. Verificou-se ao longo dos 5 anos um aumento significativo da prevalência de colonização intermitente e crónica por Staphylococcus aureus (48% para 83% e 32% para 54%. A prevalência de isolamentos de Staphylococcus aureus resistente à meticilina e de Burkholderia cepacia quase que duplicou neste período. As taxas de isolamento e de colonização crónica por Alcaligenes xylosoxidans aumentaram bruscamente a partir de 1997 (de 3% e 0% em 1996 para 7% e 5% em 1997 e 10% e 7% em 1999. A colonização crónica por Haemophilus influenzae manteve uma prevalência média de 22%, apesar dum aumento dos isolamentos (de 42% para 61%. Em 55% dos doentes observou-se colonização crónica por dois ou mais agentes.Em função destes resultados são discutidos os esquemas terapêuticos e as medidas de prevenção de contágios que têm sido preconizados nos doentes com FQ do nosso centro.REV PORT PNEUMOL 2003; IX (4: 337-352 ABSTRACT: With the aim of characterizing the evolution of the epidemiological profile of respiratory bacterial infections of patients having Cystic Fibrosis (CF, the authors

  18. Biofilm ved kronisk rhinosinuitis og cystisk fibrose

    DEFF Research Database (Denmark)

    Fisker, Jacob; Buchwald, Christian von; Johansen, Helle Krogh

    2011-01-01

    Microbial biofilms are known to cause persistent foreign-body infections and have recently been acknowledged as involved in more than 65% of all human infections. Microbial biofilms have been detected in chronic rhinosinusitis, and chronic rhinosinusitis is mandatory in patients with cystic...

  19. Idiopatisk pulmonal fibrose er en overset sygdom

    DEFF Research Database (Denmark)

    Bendstrup, Elisabeth; Kronborg-White, Sissel; Prior, Thomas Skovhus

    2017-01-01

    Idiopathic pulmonary fibrosis is a chronic, progressive and fatal disease which primarily occurs in male patients over 60 years with a smoking history. Cryobiopsy is a new promising method for obtaining lung tissue for histologic analysis with fewer complications than surgical lung biopsy...

  20. Imaging pulmonary fibrosis; Imagerie des fibroses pulmonaires

    Energy Technology Data Exchange (ETDEWEB)

    Brauner, M.W.; Rety, F.; Naccache, J.M.; Girard, F.; Valeyre, D.F. [Hopital Avicenne, 93 - Bobigny (France). Service de radiologie et de pneumologie

    2001-02-01

    Localized fibrosis of the lung is usually scar tissue while diffuse pulmonary fibrosis is more often a sign of active disease. Chronic infiltrative lung disease may be classified into four categories: idiopathic pneumonitis, collagen diseases, granulomatosis (sarcoidosis), and caused by known diseases (pneumoconiosis, hypersensitivity pneumonitis, drug-induced lung disease, radiation). (authors)

  1. Morphometric study of the fibrosis and mast cell count in the circular colon musculature of chronic Chagas patients with and without megacolon Estudo morfométrico da fibrose e do número de mastócitos na muscular circular do cólon de chagásicos crônicos com e sem megacólon

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    Simone Wanderley Pinheiro

    2003-07-01

    Full Text Available A morphometric study of the circular colon musculature was performed, in which the mast cell count was determined and the connective fibrous tissue in this layer was measured. The objective was to gain better understanding of Chagas megacolon morphology and contribute towards the knowledge of fibrosis pathogenesis in Chagas megas. An evaluation was made of 15 distal sigmoid rings from Chagas patients with megacolon (MCC, 15 without megacolon (CSMC and 15 non-Chagas patients (NC. The rings were fixed in formol, embedded in paraffin, and 7mm thick sections were cut and stained using Azan-Heidenhain and Giemsa. The mast cell count and fibrosis were greater in the MCC group than in the CSMC and NC groups (p Com os objetivos de conhecer melhor a morfologia do megacólon chagásico e contribuir para o conhecimento da patogênese da fibrose dos megas, realizou-se estudo morfométrico na muscular circular do cólon, contando-se o número de mastócitos e medindo o conjuntivo fibroso nessa camada. Foram avaliados anéis do sigmóide distal de 15 chagásicos com megacólon (MCC, 15 sem megacólon (CSMC e 15 não chagásicos (NC. Os anéis foram fixados em formol, incluídos em parafina, cortados com 7mm de espessura e corados por Azan-Heidenhain e Giemsa. O número de mastócitos e a fibrose foram maiores no grupo com MCC em relação ao CSMC e NC (p < 0,05; teste de Kruskal-Wallis; não houve diferença significante entre os dois últimos grupos. Diante destes achados, é possível, que haja relação entre mastocitose e fibrose no megacólon chagásico, como já se demonstrou em outras doenças.

  2. Incidência e evolução da polipose nasal em crianças e adolescentes com fibrose cística Incidence and evolution of nasal polyps in children and adolescents with cystic fibrosis

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    Silke Anna Thereza Weber

    2008-02-01

    Full Text Available A polipose nasal é manifestação clínica de alerta para investigação de fibrose cística (FC. OBJETIVO: Avaliar incidência de pólipos nasais em crianças e adolescentes com FC, sua associação com idade, sexo, sintomas clínicos, achados laboratoriais e genótipo, e sua evolução com corticoterapia tópica. CASUÍSTICA E MÉTODOS: Foram avaliados sintomas clínicos, níveis de cloro no suor e mutações genéticas de 23 pacientes com FC. A polipose nasal foi investigada por exame endoscópico e quando presente, o paciente recebeu 6 meses de tratamento com corticosteróide tópico e foi realizada nova endoscopia depois. Para análise estatística utilizou-se média, desvio padrão e Teste de Fisher. RESULTADOS: 39,1% dos pacientes apresentaram polipose nasal (cinco bilateral, quatro unilateral, todos com mais de seis anos, 82,6%, pneumonias recorrentes, 87%, insuficiência pancreática e 74%, desnutrição. Não houve associação entre polipose e nível de cloro no suor, genótipo, fenótipo clínico e sintomas nasais. Houve melhora da polipose com tratamento clínico em sete pacientes, com regressão completa em seis. CONCLUSÃO: O estudo mostrou elevada incidência de polipose em crianças com FC, sendo encontrada em todos os espectros de gravidade clínica, mesmo na ausência de sintomas nasais. O tratamento com corticosteróide tópico mostrou-se eficaz. A interação de pneumopediatra e do otorrinolaringologista é fundamental para diagnóstico e seguimento.Nasal polyps are a clinical sign of alert for investigating Cystic Fibrosis (CF. AIMS: To study the incidence of nasal polyps in children and adolescents with cystic fibrosis, its possible association with age, gender, clinical manifestations, genotype and sweat chlorine level, and its evolution with topical steroid therapy. METHODS: Clinical symptoms, sweat chlorine level and genotype were studied in 23 cystic fibrosis patients. Nasal polyps were diagnosed by nasal endoscopy

  3. Prevalência da doença do refluxo gastroesofágico em pacientes com fibrose pulmonar idiopática Prevalence of gastroesophageal reflux disease in patients with idiopathic pulmonary fibrosis

    Directory of Open Access Journals (Sweden)

    Cristiane Dupont Bandeira

    2009-12-01

    Full Text Available OBJETIVO: Determinar a prevalência da doença do refluxo gastroesofágico (DRGE e sua apresentação clínica, além do perfil funcional do esôfago em pacientes com fibrose pulmonar idiopática (FPI. MÉTODOS: Foram avaliados prospectivamente 28 pacientes com FPI. Os pacientes foram submetidos à esofagomanometria estacionária, pHmetria de 24 h e testes de função pulmonar, assim como responderam a questionários sobre sintomas e qualidade de vida em DRGE. RESULTADOS: A prevalência de DRGE foi de 35,7%. Os pacientes foram divididos em dois grupos: grupo DRGE+ (pHmetria anormal; n = 10, e grupo DRGE- (pHmetria normal; n = 18. No grupo DRGE+, 77,7% dos pacientes apresentavam pelo menos um sintoma típico de DRGE. Nesse grupo de pacientes, 8 (80% apresentaram refluxo em posição supina, e 5 (50% apresentaram refluxo exclusivamente nessa posição. Nos grupos DRGE+ e DRGE-, respectivamente, 5 (50,0% e 7 (38,8% dos pacientes apresentaram hipotonia do esfíncter esofágico inferior, assim como 7 (70,0% e 10 (55,5% apresentaram hipomotilidade esofágica. Não houve diferenças significativas entre os grupos quanto a características demográficas, função pulmonar, apresentação clínica ou achados manométricos. CONCLUSÕES: A prevalência de DRGE nos pacientes comOBJECTIVE: To determine the prevalence of gastroesophageal reflux disease (GERD and to evaluate its clinical presentation, as well as the esophageal function profile in patients with idiopathic pulmonary fibrosis (IPF. METHODS: In this prospective study, 28 consecutive patients with IPF underwent stationary esophageal manometry, 24-h esophageal pH-metry and pulmonary function tests. All patients also completed a symptom and quality of life in GERD questionnaire. RESULTS: In the study sample, the prevalence of GERD was 35.7%. The patients were then divided into two groups: GERD+ (abnormal pH-metry; n = 10 and GERD- (normal pH-metry; n = 18. In the GERD+ group, 77.7% of the patients

  4. Padrões ventilatórios na espirometria em pacientes adolescentes e adultos com fibrose cística Respiratory patterns in spirometric tests of adolescents and adults with cystic fibrosis

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    Bruna Ziegler

    2009-09-01

    Full Text Available OBJETIVO: Avaliar os padrões dos distúrbios ventilatórios observados na espirometria em pacientes com fibrose cística (FC e suas relações com a gravidade funcional e com o comportamento dos fluxos máximos expiratórios a baixos volumes. MÉTODOS: Estudo transversal e retrospectivo, incluindo pacientes adolescentes e adultos com FC. Todos os pacientes foram submetidos à espirometria. Os pacientes foram classificados como tendo função ventilatória preservada, distúrbio ventilatório obstrutivo (DVO, DVO com CVF reduzida, sugestivo de distúrbio ventilatório restritivo (DVR ou distúrbio ventilatório combinado (DVC. Os fluxos máximos expiratórios a baixos volumes foram avaliados utilizando-se FEF25-75%, FEF75%e FEF75%/CVF. O grupo controle incluiu 65 indivíduos normais, também submetidos à espirometria. RESULTADOS: Foram incluídos 65 pacientes no grupo de estudo: 8 (12,3% com função pulmonar preservada, 18 (27,7% com DVO, 24 (36,9% com DVO com CVF reduzida, 5 (7,7% com padrão sugestivo de DVR e 10 (15,4% com DVC. O VEF1 foi significativamente menor nos grupos DVO com CVF reduzida e DVC, comparados com os outros grupos (p OBJECTIVE: To evaluate spirometric patterns of respiratory disorders and their relationship with functional severity and maximal expiratory flows at low lung volumes in patients with cystic fibrosis (CF. METHODS: A retrospective cross-sectional study including adolescents and adults with CF. All of the patients were submitted to spirometry. Patients were classified as having preserved respiratory function, obstructive lung disease (OLD, OLD with reduced FVC, presumptive restrictive lung disease (RLD or mixed obstructive and restrictive lung disease (MORLD. Maximal expiratory flows at low lung volumes were assessed using FEF25-75%, FEF75% and FEF75%/FVC. We included 65 normal subjects, also submitted to spirometry, as a control group. RESULTS: The study group included 65 patients: 8 (12.3% with preserved lung

  5. Percepção da gravidade da doença em pacientes adultos com fibrose cística Perception of disease severity in adult patients with cystic fibrosis

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    Paulo de Tarso Roth Dalcin

    2009-01-01

    Full Text Available OBJETIVO: Avaliar a percepção da gravidade da doença em pacientes com fibrose cística (FC, investigando sua relação com escore clínico, escore radiológico, testes de função pulmonar, adesão ao tratamento e percepção de autocuidado. MÉTODOS: Estudo transversal, prospectivo, envolvendo pacientes com FC atendidos em um programa para adultos com FC. A percepção da gravidade da doença, a adesão ao tratamento e o relato de autocuidado foram avaliados por questionários. Foram obtidos de todos os pacientes dados clínicos, escore clínico de Shwachman-Kulczycki, escore radiológico de Brasfield e espirometria. RESULTADOS: De 38 pacientes estudados, 3 (7,9% relataram percepção de sua saúde como muito abaixo da média; 5 (13,2%, como abaixo da média; 15 (39,5%, como na média; 10 (26.3%, como acima da média; e 5 (13,2%, como muito acima da média. A percepção da gravidade da doença correlacionou-se significativamente com o escore clínico (r = 0,43, p = 0,007, CVF (r = 0,34, p = 0,034, VEF1 (r = 0,38, p = 0,019 e com relato de autocuidado (r = 0,33, p = 0,044, mas não com o grau de adesão (r = -0,03, p = 0,842 e escore radiológico (r = 0,33, p = 0,51. CONCLUSÕES: A percepção da gravidade da doença se relacionou com medidas objetivas de gravidade da doença (escore clínico e testes de função pulmonar e com relato de autocuidado, mas não com a adesão ao tratamento.OBJECTIVE: To evaluate the perception of disease severity in patients with cystic fibrosis (CF, investigating its relationship with clinical score, radiographic score, respiratory function tests, adherence to treatment and perception of self-care practices. METHODS: Prospective, cross-sectional study involving CF patients treated in a program for adults with CF. The perception of disease severity, adherence to treatment and reported self-care practices were evaluated by means of questionnaires. Clinical data, Shwachman-Kulczycki clinical score, Brasfield

  6. Análise descritiva dos pacientes com fibrose cística em acompanhamento na Unidade de Pneumologia Pediátrica de um hospital universitário em Porto Alegre-RS = Descriptive analysis of cystic fibrosis patients followed by the Pediatric Pulmonology Unit of a university hospital in Porto Alegre-RS

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    Chakr, Valentina Coutinho Baldoto Gava

    2006-01-01

    Full Text Available Objetivos: Descrever o perfil de pacientes acompanhados num centro de tratamento de médio porte, especializado em fibrose cística, e comparar os dados obtidos com os encontrados na literatura atual. Métodos: Foram analisados e correlacionados dados de importância para o seguimento clínico dos pacientes com fibrose cística obtidos no banco de dados de acompanhamento dos mesmos pacientes. Os resultados foram expressos em médias ± desvio padrão (DP ou medianas e amplitude interquartil 25-75%, conforme a distribuição das variáveis. Resultados: Foram estudados 26 pacientes do sexo masculino e 22 do feminino. A idade mediana do diagnóstico foi de 0,98 anos. Dos 48 pacientes, 89,6% apresentam insuficiência pancreática e 29,1% mostraram pelo menos uma cultura positiva para Pseudomonas aeruginosa nos últimos 6 meses. Apresentavam redução dos parâmetros de função pulmonar o grupo com insuficiência pancreática (84 versus 112%; p = 0,005 e os portadores de infecção crônica por P. aeruginosa (75 versus 85%; p = 0,006. Conclusões: Os dados obtidos em nosso estudo mostram que este grupo de pacientes se apresenta de maneira concordante aos dados disponíveis na literatura quanto às características analisadas da doença. Contudo, há uma exceção marcante quanto ao diagnóstico da mesma, ainda muito tardiamente realizado no Brasil

  7. Características funcionais pulmonares e uso de broncodilatador em pacientes com fibrose cística Pulmonary function parameters and use of bronchodilators in patients with cystic fibrosis

    Directory of Open Access Journals (Sweden)

    Lucia Harumi Muramatu

    2013-02-01

    Full Text Available OBJETIVO: Analisar as características funcionais pulmonares, a resposta farmacodinâmica a um broncodilatador e sua prescrição em pacientes com diagnóstico de fibrose cística (FC. MÉTODOS: Estudo de coorte retrospectivo de pacientes (6-18 anos com diagnóstico de FC acompanhados em um centro de referência, capazes de realizar testes de função pulmonar (TFP entre 2008 e 2010. Foram analisados CVF, VEF1 e FEF25-75%, em percentual do previsto, antes e após prova broncodilatadora (pré-BD e pós-BD, respectivamente de 312 TFP. Foram realizadas ANOVA para medidas repetidas e comparações múltiplas. RESULTADOS: Foram incluídos no estudo 56 pacientes. Desses, 37 e 19, respectivamente, tinham resultados de TFP entre 2008 e 2010 e apenas em 2009-2010, formando dois grupos. No grupo com TFP nos três anos estudados, houve redução significativa em VEF1 pós-BD em 2008-2010 (p = 0,028 e 2009-2010 (p = 0,036 e em FEF25-75% pré-BD e pós-BD em todas as comparações múltiplas (2008 vs. 2009; 2008 vs. 2010; e 2009 vs. 2010. No grupo com TFP apenas em 2009-2010, não houve diferenças significativas em nenhuma das comparações das variáveis estudadas. Dos 312 TFP, somente 24 (7,7% apresentaram resposta significativa ao broncodilatador e pertenciam a pacientes sem prescrição de broncodilatador durante o período estudado. CONCLUSÕES: Houve perda funcional, com indicação de doença pulmonar progressiva, nos pacientes com FC estudados. Houve maiores alterações no FEF25-75%, sugerindo o comprometimento inicial de vias aéreas menores.OBJECTIVE: To analyze pulmonary function parameters and pharmacodynamic response to a bronchodilator, as well as the prescription of bronchodilators, in cystic fibrosis (CF patients. METHODS: This was a retrospective cohort study involving patients 6-18 years of age, diagnosed with CF, and followed at a referral center between 2008 and 2010. We evaluated only those patients who were able to perform pulmonary

  8. Prevalência da mutação ΔF508 no gene cystic fibrosis transmembrane conductance regulator em pacientes com fibrose cística em um centro de referência no Brasil Prevalence of ΔF508 mutation in the cystic fibrosis transmembrane conductance regulator gene among cystic fibrosis patients from a Brazilian referral center

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    Andréia Marisa Bieger

    2012-12-01

    Full Text Available OBJETIVO: Verificar a presença da mutação ΔF508 no gene cystic fibrosis transmembrane conductance regulator na população de pacientes com fibrose cística, diagnosticados pelo teste de sódio e cloro no suor, em acompanhamento no Ambulatório de Pneumologia Pediátrica da Universidade Estadual de Campinas, centro de referência no tratamento da fibrose cística. MÉTODOS: Foram analisadas 167 amostras de DNA de pacientes com fibrose cística. O genótipo dos pacientes foi determinado pela técnica de reação da polimerase e realizado cálculo para a frequência dos alelos e genótipos da mutação ΔF508. RESULTADOS: A frequência genotípica encontrada foi, respectivamente, para os genótipos -/-, ΔF508/- e ΔF508/ΔF508: 43,7% (73 pacientes, 32,9% (55 pacientes e 23,4% (39 pacientes. Do total de 334 alelos analisados, foi observada a frequência de 201 (60,18% alelos para a ausência da mutação ΔF508 e de 133 (39,82% para a presença da mutação ΔF508. O cálculo do equilíbrio de Hardy-Weinberg foi realizado, e obtivemos o valor de qui-quadrado = 16,34 (p OBJECTIVE: To verify the presence of ΔF508 mutation in the cystic fibrosis transmembrane conductance regulator gene among patients with cystic fibrosis diagnosed by the sweat test for sodium and chlorine and followed at the Pediatric Pneumology Outpatient Clinic of Universidade Estadual de Campinas, Brazil, a referral center for the treatment of cystic fibrosis. METHODS: The study analyzed 167 DNA samples from cystic fibrosis patients. Patients' genotype was determined by polymerase chain reaction, and allele and genotype frequencies of ΔF508 mutation were calculated. RESULTS: The genotype frequencies found for -/-, ΔF508/-, and ΔF508/ΔF508 genotypes were respectively: 43.7% (73 patients, 32.9% (55 patients, and 23.4% (39 patients. Of the 334 alleles analyzed, we observed a frequency of 201 (60.18% alleles for the absence of ΔF508 mutation and of 133 (39.82% for the

  9. Exercício aeróbico, treinamento de força muscular e testes de aptidão física para adolescentes com fibrose cística: revisão da literatura Exercise testing, aerobic and strength training for adolescents with cystic fibrosis: a literature review

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    Celia Regina Moutinho de Miranda Chaves

    2007-09-01

    Full Text Available São reconhecidos os benefícios da prática do exercício físico regular para pacientes com fibrose cística. Entretanto, poucos estudos envolvem pacientes adolescentes. O objetivo deste artigo foi revisar os efeitos da prática regular de exercícios aeróbicos e de força e resistência muscular para adolescentes com fibrose cística. Os principais testes de aptidão física para esta faixa etária e a importância deles para melhora do prognóstico e tratamento da doença também foram avaliados. As informações foram coletadas a partir de livro-texto e artigos publicados na literatura nacional e estrangeira nas seguintes bases de dados: LILACS, MEDLINE/PubMed, Biblioteca Cochrane e SciELO, abrangendo o período de 1994 a 2004. Foram utilizados os termos "exercise" e "cystic fibrosis" para seleção dos artigos. Esta pesquisa demonstrou que a prática de exercício aeróbico e treinamento de força muscular melhoram a desobstrução da árvore brônquica, diminuem a queda progressiva da função pulmonar, aumentam a massa muscular e a resistência ao exercício, promovem o desenvolvimento ósseo e melhoram a auto-estima e a qualidade de vida. Os melhores resultados foram obtidos com os programas de treinamento assistido, devido a sua maior regularidade.The benefits of regular physical exercises for cystic fibrosis patients are well known. Nevertheless, few studies involve adolescent patients. The objective of this article was to review the effects of regular practice of aerobic exercises, strength exercises and muscular exercises in adolescents with cystic fibrosis. The main physical fitness tests for this age bracket and their value in improving prognosis and treatment were assessed as well. Information was collected from text books and articles published in the national and foreign literature in the following databases: LILACS, MEDLINE/PubMed, Cochrane Library and SciELO, comprising the period of 1994 to 2004. The terms "exercise" and

  10. Study of human mesenchymal stem cells plasticity into radiation injured tissues in a N.O.D./S.C.I.D. mouse model: therapeutic approach of the multiple organ dysfunction; Etude de la capacite plastique des Cellules Souches Mesenchymateuses humaines (CSM) apres irradiation du tissu receveur: approche therapeutique de l'atteinte multiorgane radio-induite

    Energy Technology Data Exchange (ETDEWEB)

    Francois, S

    2006-01-15

    The therapeutic potential of bone marrow-derived human mesenchymal stem cells (h.M.S.C.) has recently been brought into the spotlight of many fields of research. One possible application of the approach is the repair of injured tissues arising from side effects of radiation treatments and accidents. The first challenge in cell therapy is to assess the quality of the cell and the ability to retain their differentiation potential during the expansion process. Efficient delivery to the sites of intended action is also necessary. We addressed both questions using h.M.S.C. cultured and then infused to Non Obese Diabetes/Severe Combined Immunodeficiency (N.O.D./S.C.I.D.) mice submitted to total body irradiation. Further, we tested the impact of additional local irradiation superimposed to total body irradiation (T.B.I.), as a model of accidental irradiation. Our results showed that the h.M.S.C. used for transplant have been expanded without significant loss in their differentiation capacities. After transplantation into adult unconditioned mice, h.M.S.C. not only migrate in bone marrow but also into other tissues. Total body irradiation increased h.M.S.C. implantation in bone marrow and muscle and further led to engraftment in brain, heart, and liver. Local irradiation, in addition to T.B.I., increased both specific homing of injected cells to the injured tissues and to other tissues outside the local irradiation field. M.S.C. may participate to restoration of intestinal homeostasis 3 days post abdominal irradiation. This study suggests that using the potential of h.M.S.C. to home to various organs in response to tissue injuries could be a promising strategy to repair the radiation induced damages. (author)

  11. In vitro modulation of radiation-induced FAS-related apoptosis in CD34{sup +} progenitor cells by combination cytokines; Reduction de l'apoptose radio-induite impliquant le recepteur FAS au niveau des cellules hematopoietiques CD34{sup +} par une combinaison de cytokines

    Energy Technology Data Exchange (ETDEWEB)

    Drouet, M.; Mathieu, J.; Grenier, N.; Soutif, A.; Herodin, F

    1998-07-01

    Combination cytokines such as SCF, Flt-3 ligand, IL-3 and thrombopoietin can modulate Fas mRNA expression by in vitro irradiated CD34{sup +} cells which results in a moderate decrease of apoptotic ratio and an improved rate of clonogenicity of the irradiated progenitors. (authors)

  12. Radio-induced neuropathology: from early effects to late sequelae. Rat behavioural and metabolic studies after sublethal total body irradiation; Neuropathologie radio-induite: des effets precoces aux sequelles tardives. Etudes comportementales et metaboliques chez le rat apres irradiation globale subletale

    Energy Technology Data Exchange (ETDEWEB)

    Martigne, A.P.

    2010-05-15

    The radioresistance dogma of Central Nervous System (CNS) is now obsolete. Recent progress in neuroscience allow us to reconsider the radiation-induced cognitive dysfunctions observed after radiation therapy or after a nuclear accident, and to devise appropriate diagnostic and therapeutic means. We have developed a Rat model to study the effects of total body irradiation at a sublethal dose (4.5 Gy). This leads to impaired learning and memory of a task being acquired during the first month - which is prevented by administration of a radioprotector (amifostine) - while it does not appear to affect retrograde memory. Early, an apoptotic wave occurs in the sub-ventricular zone, 5 to 9 hours after exposure, while neuro-genesis is suppressed. Two days after irradiation, the metabolic study conducted by NMR HRMAS (High Resolution Magic Angle Spinning) suggests the presence of cerebral oedema and the study of brain lipids in liquid NMR confirms the membrane damages (elevated cholesterol and phospholipids). The lipid profile is then normalized while a gliosis appears. Finally, 1 month post-irradiation, the elevation of GABA, an inhibitory neurotransmitter, in 2 separate brain structures, occurs simultaneously with a taurine decrease in the hippocampus that lasts 6 months. Our integrated model allows validating bio-markers measurable in vivo NMR spectroscopy - the next experimental stage - and testing new radiation-protective agents. (author)

  13. Radio-induced breast cancers exhibiting aggressive anatomo-pathological characteristics: retrospective study of the long-term follow-up committee of the French Society of Child Cancers; Cancers du sein radio-induits presentant des caracteristiques anatomopathologiques agressives: etude retrospective du comite de suivi a long terme de la Societe francaise des cancers de l'enfant

    Energy Technology Data Exchange (ETDEWEB)

    Demoor, C.; Mahe, M.A.; Supiot, S. [ICO Rene-Gauducheau, Nantes (France); Vathaire, F. de [Inserm UMRS 1018, institut de cancerologie Gustave-Roussy, Villejuif (France); Oberlin, O. [Institut de cancerologie Gustave-Roussy, Villejuif (France); Noel, G. [Centre Paul-Strauss, Strasbourg (France); Brillaud, V. [Institut Bergonie, Bordeaux (France); Bernier, V. [Centre Alexis-Vautrin, Nancy (France); Laprie, A. [Institut Claudius-Regaud, Toulouse (France); Claude, L. [Centre Leon-Berard, Lyon (France)

    2011-10-15

    The authors report an analysis of clinical-pathological characteristics of radio-induced breast cancers registered in six French centres. 82 breast cancers concerning 75 women have been analyzed in terms of patient age, cancer type, interval between both cancers. It appears that radio-induced cancers exhibited significantly more aggressive characteristics. The screening of young women at risk is therefore recommended for an early diagnosis and treatment. Short communication

  14. Study of human mesenchymal stem cells plasticity into radiation injured tissues in a N.O.D./S.C.I.D. mouse model: therapeutic approach of the multiple organ dysfunction; Etude de la capacite plastique des Cellules Souches Mesenchymateuses humaines (CSM) apres irradiation du tissu receveur: approche therapeutique de l'atteinte multiorgane radio-induite

    Energy Technology Data Exchange (ETDEWEB)

    Francois, S

    2006-01-15

    The therapeutic potential of bone marrow-derived human mesenchymal stem cells (h.M.S.C.) has recently been brought into the spotlight of many fields of research. One possible application of the approach is the repair of injured tissues arising from side effects of radiation treatments and accidents. The first challenge in cell therapy is to assess the quality of the cell and the ability to retain their differentiation potential during the expansion process. Efficient delivery to the sites of intended action is also necessary. We addressed both questions using h.M.S.C. cultured and then infused to Non Obese Diabetes/Severe Combined Immunodeficiency (N.O.D./S.C.I.D.) mice submitted to total body irradiation. Further, we tested the impact of additional local irradiation superimposed to total body irradiation (T.B.I.), as a model of accidental irradiation. Our results showed that the h.M.S.C. used for transplant have been expanded without significant loss in their differentiation capacities. After transplantation into adult unconditioned mice, h.M.S.C. not only migrate in bone marrow but also into other tissues. Total body irradiation increased h.M.S.C. implantation in bone marrow and muscle and further led to engraftment in brain, heart, and liver. Local irradiation, in addition to T.B.I., increased both specific homing of injected cells to the injured tissues and to other tissues outside the local irradiation field. M.S.C. may participate to restoration of intestinal homeostasis 3 days post abdominal irradiation. This study suggests that using the potential of h.M.S.C. to home to various organs in response to tissue injuries could be a promising strategy to repair the radiation induced damages. (author)

  15. Part of the oxidative stress in the development of radio-induced cell effects at cutaneous level: application to accidental localised irradiations; Role du stress oxydatif dans le developpement des effets cellulaires radio-induits au niveau cutane: application aux irradiations localisees accidentelles

    Energy Technology Data Exchange (ETDEWEB)

    Carine, Laurent

    2005-10-15

    The objective of our study was to answer to the following questions: does the initial radio-induced oxidative stress lead to the accumulation of DNA damages in the low renewal cells (fibroblasts, endothelial cells) that could be responsible of delayed effects; does it exist delayed oxidative phenomena and era they implied in the delayed effects arising; does it exist a phenomenon of premature senescence; does it exist a premature senescence phenomenon that could lead to an accumulation of damages before the cell death; what are the action mechanisms of the association pentoxifylline/{alpha}-tocopherol. (N.C.)

  16. Context of surveillance of underground and surface waters; Contexte de la surveillance des eaux souterraines et superficielles

    Energy Technology Data Exchange (ETDEWEB)

    NONE

    2010-07-01

    This document briefly describes the evolutions of regulations on site liquid effluents and of guideline values concerning radioactive wastes, briefly presents the surveillance of underground and surface waters of CEA sites, comments the guideline values of the radiological quality of waters aimed at human consumption, and gives an overview of information which are brought to public's attention. Then, for different CEA sites (Cadarache, Marcoule, Saclay, Grenoble, Fontenay-aux-Roses, Valduc, DIF), this document proposes a presentation of the hydrological context, regulatory context, the surface and underground water surveillance process and values, the storing zones of old wastes.

  17. Validação do escore ultra-sonográfico de Williams para o diagnóstico da hepatopatia da fibrose cística Validation of the Williams ultrasound scoring system for the diagnosis of liver disease in cystic fibrosis

    Directory of Open Access Journals (Sweden)

    Eleonora D. T. Fagundes

    2004-01-01

    Full Text Available OBJETIVOS: Descrever as alterações hepáticas observadas ao exame ultra-sonográfico de fibrocísticos do Ambulatório de Fibrose Cística do Hospital das Clínicas da UFMG, comparar os achados ultra-sonográficos com critérios clínicos e bioquímicos e validar o escore de Williams para o diagnóstico de hepatopatia associada à fibrose cística. MÉTODOS: Setenta fibrocísticos foram acompanhados prospectivamente e submetidos a exame clínico, bioquímico e ultra-sonográfico. Os achados ultra-sonográficos foram comparados com os resultados do exame clínico e bioquímico. Para a validação do escore ultra-sonográfico de Williams, os critérios clínicos e bioquímicos foram utilizados como padrão-ouro. Foram calculados sensibilidade, especificidade, valor preditivo positivo e valor preditivo negativo do escore de Williams. Para os cálculos, os pacientes foram divididos em dois grupos: normal ao exame ultra-sonográfico (escore = 3 ou alterado (escore > 3. RESULTADOS: Dez pacientes preencheram os critérios clínicos e/ou bioquímicos para hepatopatia (14,3%. Todos os pacientes hepatopatas segundo os critérios clínicos e/ou bioquímicos apresentavam alguma alteração ao exame ultra-sonográfico. As alterações do parênquima hepático, borda hepática e fibrose periportal foram encontradas mais freqüentemente entre os hepatopatas, com diferença estatisticamente significativa. O escore de Williams apresentou alta especificidade (91,7%; IC 80,9-96,9, mas baixa sensibilidade (s = 50%; IC 20,1-79,9 para o diagnóstico da hepatopatia. CONCLUSÕES: O escore de Williams não constituiu um bom exame de triagem quando comparado ao exame clínico e bioquímico. Uma vez que ainda não há nenhum teste que, utilizado isoladamente, apresente sensibilidade adequada, é recomendável a utilização conjunta dos exames clínico, bioquímico e ultra-sonográfico no diagnóstico da hepatopatia associada à fibrose cística, sempre em avalia

  18. Fibrose miocárdica em pacientes com cardiomiopatia hipertrófica com alto risco para morte súbita cardíaca Fibrosis miocárdica en pacientes con cardiomiopatía hipertrófica con alto riesgo para muerte súbita cardíaca Myocardial fibrosis in patients with hypertrophic cardiomyopathy and high risk for sudden death

    Directory of Open Access Journals (Sweden)

    Afonso Akio Shiozaki

    2010-04-01

    Full Text Available FUNDAMENTO: A estratificação de risco para morte súbita na cardiomiopatia hipertrófica (CMH, continua a ser um verdadeiro desafio devido à grande heterogeneidade da sua apresentação, em que a maioria dos indivíduos permanecem assintomáticos por toda sua vida e outros apresentam a morte súbita como primeiro sintoma. Recentes trabalhos vêm sugerindo que a fibrose miocárdica pode constituir-se em um importante substrato para as arritmias ventriculares malignas, responsáveis pela morte súbita nesta doença. OBJETIVO: Avaliação da prevalência e quantificação da fibrose miocárdica (FM, em pacientes com CMH com alto risco ou recuperados de morte súbita, portadores de cardiodesfibrilador implantável (CDI. MÉTODOS: Vinte e oito pacientes com CMH portadores de CDI foram submetidos à tomografia computadorizada com múltiplos detectores, para realização da técnica de realce tardio, e avaliação da fibrose miocárdica. RESULTADOS: 96% dos pacientes apresentavam fibrose miocárdica (20,38 ± 15,55 gramas correspondendo a 15,96 ± 10,20% da massa miocárdica total. A FM foi significativamente mais prevalente que os demais fatores de risco clássicos para morte súbita. CONCLUSÃO: Concluímos que existe uma alta prevalência de fibrose miocárdica em pacientes com cardiomiopatia hipertrófica de alto risco ou recuperados de morte súbita, como neste grupo - portadores de cardiodesfibrilador implantável. A maior prevalência da fibrose miocárdica comparada aos fatores de risco de pior prognóstico levantam a hipótese de que a fibrose miocárdica possa ser um importante substrato potencialmente necessário na gênese das arritmias desencadeadoras da morte súbita.FUNDAMENTO: La estratificación de riesgo para muerte súbita en la cardiomiopatía hipertrófica (CMH, sigue siendo un verdadero reto debido a la gran heterogeneidad de su presentación, cuya mayoría de los individuos permanecen asintomáticos por toda su vida y otros

  19. O que é ser mãe de uma criança com fibrose cística Qué significa ser madre de un niño con fibrosis quística What is being a mother of a child with cystic fibrosis

    Directory of Open Access Journals (Sweden)

    Keila Okuda Tavares

    2010-12-01

    Full Text Available Conhecer o que é ser mãe de uma criança que apresenta fibrose cística é fundamental para o cuidado desses indivíduos, pois geralmente ela divide as responsabilidades relacionadas ao tratamento com o filho que apresenta essa doença grave e sem cura. Nesse sentido, este estudo teve como objetivo conhecer a vivência de ser mãe de uma criança com fibrose cística. Trata-se de um estudo descritivo-exploratório, qualitativo, de caráter fenomenológico. Foram entrevistadas 14 mulheres e a coleta de dados foi realizada por meio de uma entrevista semiestruturada. A análise das falas transcritas na íntegra seguiu direcionamentos propostos pela fenomenologia. Essa é uma experiência que a tristeza, angústia, dúvidas, sofrimento e medo estão presentes, levando essas mulheres a refletirem sobre suas vidas e a de seus filhos, promovendo mudanças em seu mundo vida e uma reorganização de toda a família.Conocer o que es ser madre de un niño con fibrosis quística es esencial para el cuidado de estas personas, porque generalmente ella divide las responsabilidades relacionadas con el tratamiento, con el niño que tiene esta enfermedad grave y sin cura. Este estudio tuvo como objetivo conocer la vivencia de ser madre de un niño con fibrosis quística. Se trata de un estudio descriptivo-exploratorio, cualitativo, fenomenológico. Fueron entrevistadas 14 mujeres y la colecta de datos se realizó por medio de una entrevista semiestructurada. El análisis de las elocuciónes transcriptas en la íntegra siguió direcciones propuestas por la fenomenologia. Esta es una experiencia donde la tristeza, angustia, dudas, sufrimiento y el temor están presentes, haciendo que esas mujeres reflexionen a respecto de sus vidas y la de sus hijos, promoviendo cambios en su mundo vida y una reorganización de toda familia.Knowing what is being a mother of a child with cystic fibrosis is essential to the care of these individuals, because she usually divides the

  20. Análise descritiva dos pacientes com fibrose cística em acompanhamento na Unidade de Pneumologia Pediátrica de um hospital universitário em Porto Alegre-RS =Descriptive analysis of cystic fibrosis patients followed by the Pediatric Pulmonology Unit of a university hospital in Porto Alegre-RS

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    Chakr, Valentina Coutinho Baldoto Gava et al.

    2006-01-01

    Full Text Available Objetivos: Descrever o perfil de pacientes acompanhados num centro de tratamento de médio porte, especializado em fibrose cística, e comparar os dados obtidos com os encontrados na literatura atual. Métodos: Foram analisados e correlacionados dados de importância para o seguimento clínico dos pacientes com fibrose cística obtidos no banco de dados de acompanhamento dos mesmos pacientes. Os resultados foram expressos em médias ± desvio padrão (DP ou medianas e amplitude interquartil 25-75%, conforme a distribuição das variáveis. Resultados: Foram estudados 26 pacientes do sexo masculino e 22 do feminino. A idade mediana do diagnóstico foi de 0,98 anos. Dos 48 pacientes, 89,6% apresentam insuficiência pancreática e 29,1% mostraram pelo menos uma cultura positiva para Pseudomonas aeruginosa nos últimos 6 meses. Apresentavam redução dos parâmetros de função pulmonar o grupo com insuficiência pancreática (84 versus 112%; p = 0,005 e os portadores de infecção crônica por P. aeruginosa (75 versus 85%; p = 0,006. Conclusões: Os dados obtidos em nosso estudo mostram que este grupo de pacientes se apresenta de maneira concordante aos dados disponíveis na literatura quanto às características analisadas da doença. Contudo, há uma exceção marcante quanto ao diagnóstico da mesma, ainda muito tardiamente realizado no Brasil. Aims: To provide a descriptive analysis of patients followed in a local cystic fibrosis treatment center and compare the collected data with those found in the literature. Methods: Relevant data for clinical follow-up of cystic fibrosis patients were used for analysis and correlation. Results: Twenty six male and twenty two female patients were studied. From 48 patients, 89,6% presented with pancreatic insufficiency and 29,1% had at least one positive culture to Pseudomonas aeruginosa in the last 6 months. The patients colonized by P. aeruginosa and the ones with pancreatic insufficiency presented with

  1. A hospitalização e o adoecimento pela perspectiva de crianças e jovens portadores de fibrose cística e osteogênese imperfeita The hospitalization and the process of becoming ill through the children's and adolescents' perspective with cystic fibrosis and osteogenesis imperfecta

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    Daniele Borges de Mello

    2010-03-01

    Full Text Available O presente artigo visa discutir os resultados de uma pesquisa realizada em um hospital localizado no município do Rio de Janeiro, considerado referência em saúde da criança, do adolescente e da mulher. Analisamos os significados da hospitalização e do adoecimento crônico na infância e adolescência pela perspectiva de crianças e adolescentes com fibrose cística e osteogênese imperfeita durante suas internações hospitalares com vistas a explorar suas vivências e suas possibilidades de expressão enquanto sujeitos de conhecimento. Para tanto, privilegiamos a observação e a construção de suas produções mediadas pelo suporte lúdico, utilizando o desenho e/ou história como relevantes vias de acesso aos conteúdos infanto-juvenis. Os dados advindos desse estudo apontam para a capacidade de aquisição e produção de conhecimento que crianças e jovens possuem acerca de sua situação de adoecimento.The present article intends to discuss the results of a study completed in a hospital located in the municipal district of Rio de Janeiro, considered most prominent for child, adolescent and woman's health. We analyzed the meanings of hospitalization and chronic illness in childhood and adolescence through the perspective of children and adolescents with cystic fibrosis and osteogenesis imperfecta during their hospitalizations in order to explore their experience and communicative possibilities as knowledgeable informants. Hence, we privileged the observation and the construction of their productions through games, using drawings and/or story-telling as a relevant approach to childhood and adolescence contents. The data collected signify the acquisition and knowledge production capacity of children and adolescents concerning their illness processes.

  2. Programa de triagem neonatal para fibrose cística no estado do Paraná: avaliação após 30 meses de sua implantação Neonatal cystic fibrosis screening program in the state of Paraná: evaluation 30 months after implementation

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    Grégor P. Chermikoski Santos

    2005-06-01

    Full Text Available OBJETIVOS: Apresentar e analisar os resultados da implantação do Programa Nacional de Triagem Neonatal em Fibrose Cística, no Paraná, após 30 meses do seu início. MÉTODOS: Foi realizado um estudo descritivo com análise dos dados da triagem feita em aproximadamente 98% dos recém-nascidos entre setembro de 2001 e abril de 2004 no laboratório do Serviço de Triagem Neonatal da Fundação Ecumênica de Proteção ao Excepcional do Paraná. As amostras de sangue para o "teste do pezinho" foram coletadas na alta hospitalar, idealmente entre o segundo e sexto dias de vida, com o envio dos papéis-filtro para a dosagem de tripsina imunorreativa por imunofluorimetria. Crianças com valores da dosagem de tripsina imunorreativa > 70 ng/ml em duas amostras distintas, nos primeiros 30 dias de vida, foram submetidas à determinação da condutividade do suor pelo método de Wescor. Com o resultado maior que 50 mMol/l procedeu-se à dosagem quantitativa de cloro e/ou sódio no suor (iontoforese por pilocarpina. RESULTADOS: De 456.982 exames realizados, 4.028 (0,9% crianças apresentaram a primeira dosagem de tripsina imunorreativa acima do ponto de corte estabelecido. Dessas, 478 (12,5% tiveram uma segunda amostra de sangue com dosagem de tripsina imunorreativa acima de 70 ng/ml, das quais, após a realização do teste de suor por condutividade, 56 (11,7% crianças foram encaminhadas para ambulatórios especializados com resultado acima de 50 mMol/l e 48 (0,01% do total de triagens tiveram o diagnóstico de fibrose cística confirmado. A incidência foi de 1:9.520 em nosso estado, embora algumas crianças ainda não tenham completado a investigação. CONCLUSÕES: A triagem neonatal para fibrose cística no estado do Paraná, de acordo com as normas do Ministério da Saúde, foi uma iniciativa pioneira no Brasil. Realizou-se o diagnóstico precocemente em muitos pacientes, mesmo os assintomáticos, fato que representa um desafio à melhora no progn

  3. Socialization of children and adolescents with cystic fibrosis: support for nursing care El proceso de socialización de niños y adolescentes con fibrosis quística: apoyo para la atención de enfermería O processo de socialização de crianças e adolescentes com fibrose cística: subsídios para o cuidado de enfermagem

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    Tainá Maués Pelúcio Pizzignacco

    2006-08-01

    salud conozcan esas demandas y las incorporen al plan de cuidados, con vistas a intervenciones efectivas que promuevan el crecimiento y desarrollo infanto-juvenil.O presente estudo tem por objetivo conhecer o dia-a-dia (escola, trabalho, atividades de lazer e interações com a família e amigos da criança e do adolescente com Fibrose Cística (FC, a partir de suas próprias vivências, bem como identificar situações que possam interferir nesse cotidiano. Os sujeitos da pesquisa são crianças e adolescentes portadores de Fibrose Cística em acompanhamento num hospital-escola do interior do estado de São Paulo. Trata-se de pesquisa com abordagem qualitativa, com coleta de dados empíricos realizada mediante análise de prontuários e entrevista aberta. Dos dados, emergiram os seguintes temas: conhecimento equivocado sobre a doença; preocupação com a auto-imagem; busca pelo autocuidado e esperança de melhorias no futuro. Os resultados evidenciam as repercussões da Fibrose Cística no processo de socialização desses pacientes, salientando a importância dos profissionais de saúde conhecerem essas demandas e incorporarem-nas ao plano de cuidados, visando a intervenções efetivas que promovam o crescimento e o desenvolvimento infanto-juvenil.

  4. Análise semiquantitativa de biópsias cirúrgicas de diferentes lobos pulmonares de pacientes com pneumonia intersticial usual/fibrose pulmonar idiopática Semiquantitative analysis of surgical biopsies of distinct lung lobes of patients with usual interstitial pneumonia/idiopathic pulmonary fibrosis

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    José Júlio Saraiva Gonçalves

    2009-07-01

    Full Text Available OBJETIVO: Avaliar as diferenças histológicas da pneumonia intersticial usual entre biópsias cirúrgicas de lobos pulmonares distintos, utilizando um escore semiquantitativo. MÉTODOS: Foram selecionados todos os pacientes com o diagnóstico de fibrose pulmonar idiopática e submetidos à biópsia cirúrgica em dois lobos distintos no Hospital São Paulo e em hospitais afiliados da Universidade Federal de São Paulo, no período entre 1995 e 2005. Foi utilizado um método semiquantitativo na avaliação histológica dos espécimes, com base em estudos prévios, aplicando-se um escore para cada local submetido à biópsia. RESULTADOS: Nenhuma diferença estatisticamente significante foi encontrada nesta amostra de pacientes que viesse alterar o estágio da doença, com base no escore utilizado. Este achado foi independente do local da biópsia (lobo médio ou segmento lingular. CONCLUSÕES: Não foram observadas diferenças histológicas significantes entre os lobos pulmonares estudados. O diagnóstico histológico definitivo de pneumonia intersticial usual não alterou o estágio da doença.OBJECTIVE: To evaluate the differences between surgical biopsies of distinct lung lobes in terms of the histopathological features of usual interstitial pneumonia, using a semiquantitative score. METHODS: We selected all of the patients diagnosed with idiopathic pulmonary fibrosis and submitted to surgical biopsy in two distinct lobes between 1995 and 2005 at the Hospital São Paulo and other hospitals operated by the Federal University of São Paulo. In the histological evaluation of the specimens, we used a semiquantitative method based on previous studies, assigning a score to each of the biopsied sites. RESULTS: In this sample of patients, we found no statistically significant differences that would alter the stage of the disease, based on the score used. This finding was independent of the biopsy site (middle lobe or lingular segment. CONCLUSIONS: No

  5. The vitamin E reduces liver lipoperoxidation and fibrosis in a model of nonalcoholic steatohepatitis A vitamina E reduz a lipoperoxidação hepática e a fibrose em modelo experimental de esteatohepatite não-alcoólica

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    Idilio Zamin Jr

    2010-03-01

    colina, bem como avaliar o papel do metformina, da vitamina E e da sinvastatina na evolução da esteatohepatite não-alcoólica. MÉTODOS: Foram estudados prospectivamente 50 ratos da linhagem Wistar, por um período de 90 dias, sendo os mesmos divididos em cinco grupos de 10 ratos. Um grupo recebeu ração convencional e os demais a dieta deficiente em metionina e colina. Dentre os quatro grupos que receberam a dieta, para um foi administrado soro fisiológico e para os demais o metformina, a vitamina E e a sinvastatina. Após o período de estudo os animais foram mortos, sendo colhido sangue para análise bioquímica e removido o fígado para análise da lipoperoxidação através do teste de substâncias reativas ao ácido tiobarbitúrico e para a realização dos exames histológicos. RESULTADOS: A dieta deficiente em metionina e colina foi capaz de induzir esteatose em 100% dos animais enquanto que esteatohepatite não-alcoólica foi diagnosticada em 27 (69,2%. Os níveis da ALT foram significativamente maiores no grupo da sinvastatina. Os níveis de AST também foram maiores no grupo da sinvastatina, mas apenas foram estatisticamente significantes em relação ao grupo da ração. Quando se compararam os valores de lipoperoxidação, os grupos da ração convencional e da vitamina E apresentaram índices significativamente menores que os demais. A presença de fibrose foi significativamente menor no grupo que recebeu a vitamina E. CONCLUSÕES: A dieta utilizada foi capaz de induzir esteatose e esteatohepatite não-alcoólica, sendo que a vitamina E demonstrou reduzir o estresse oxidativo hepático, bem como o desenvolvimento de fibrose

  6. Diagnóstico de ABPA em pacientes portadores de fibrose cística: utilidade clínica da pesquisa de IgE específica contra alérgenos recombinantes do Aspergillus fumigatus ABPA diagnosis in cystic fibrosis patients: the clinical utility of IgE specific to recombinant Aspergillus fumigatus allergens

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    Marina B. Almeida

    2006-06-01

    Full Text Available OBJETIVO: A aspergilose broncopulmonar alérgica (ABPA é um fator complicador da fibrose cística que pode determinar uma combinação devastadora na evolução da doença pulmonar. A sobreposição de sinais e sintomas das duas enfermidades dificulta o diagnóstico, mesmo aplicando critérios padronizados. O objetivo deste trabalho foi identificar, em grupo de portadores de fibrose cística, os casos de ABPA através da detecção de IgE específica contra os alérgenos recombinantes do Aspergillus fumigatus e confrontar esse método com os critérios preconizados pela Cystic Fibrosis Foundation. MÉTODOS: Cinqüenta e quatro pacientes de 2 a 20 anos, com características que poderiam estar isoladamente presentes na ABPA, foram avaliados sistematicamente, incluindo: dados clínicos, tomografia computadorizada de tórax, teste cutâneo de hipersensibilidade imediata para A. fumigatus; dosagem de IgE sérica total, RAST para A. fumigatus, e IgE sérica específica para alérgenos recombinantes r Asp f1, f2, f3, f4 e f6. RESULTADOS: Foram elegíveis para o estudo 39 pacientes. Destes, 32 foram investigados. Houve sensibilização ao A. fumigatus em 34%. Ambos os métodos, o critério da Cystic Fibrosis Foundation e a pesquisa de IgE específica contra antígenos recombinantes, determinaram três casos de ABPA; entretanto, o diagnóstico foi concordante em apenas dois pacientes. CONCLUSÃO: A detecção de IgE específica contra antígenos recombinantes do A. fumigatus foi ferramenta útil para detecção precoce da sensibilização e diagnóstico de ABPA. No entanto, a confirmação diagnóstica não pôde ser desvinculada da condição clínica, e sua utilização para diagnóstico, detecção de recidivas e critério de cura ainda requer estudos longitudinais, envolvendo maior número de pacientes.OBJECTIVE: Allergic bronchopulmonary aspergillosis (ABPA is a complicating factor of cystic fibrosis which can result in a devastating combination as

  7. Noninvasiv vurdering af fibrose ved kronisk viral hepatitis

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    Risum, Malene; Barfod, Toke Seierøe; Lindhardt, Bjarne Orskov

    2013-01-01

    In chronic viral hepatitis the liver biopsy helps the clinician to decide when to start treatment and plan follow-up. However, the execution of a liver biopsy is associated with discomfort, and sampling error can lead to misinterpretation. Serum markers and transient elastography (TE) are being...

  8. Rekombinant humant deoxyribonuklease til andet end cystisk fibrose

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    Kristensen, Kim

    2010-01-01

    be associated with increased need for supplemental oxygen. In adults with idiopathic bronchiectasis, treatment with rhDNase leads to more pulmonary exacerbations and a greater decline in pulmonary function tests. There are no controlled studies on rhDNase in primary ciliary dyskinesia or atelectasis.......Recombinant human DNase (rhDNase) reduces viscosity of sputum. Effect has been documented in cystic fibrosis and postoperatively in paediatric heart disease. Single dose treatment with rhDNase in paediatric asthma has no effect. In respiratory syncytial virus infection, treatment with rhDNase may...

  9. Rekombinant humant deoxyribonuklease til andet end cystisk fibrose

    DEFF Research Database (Denmark)

    Kristensen, Kim

    2010-01-01

    be associated with increased need for supplemental oxygen. In adults with idiopathic bronchiectasis, treatment with rhDNase leads to more pulmonary exacerbations and a greater decline in pulmonary function tests. There are no controlled studies on rhDNase in primary ciliary dyskinesia or atelectasis....

  10. Prevalência de hipertensão pulmonar avaliada por ecocardiografia Doppler em uma população de pacientes adolescentes e adultos com fibrose cística Prevalence of pulmonary hypertension evaluated by Doppler echocardiography in a population of adolescent and adult patients with cystic fibrosis

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    Paula Maria Eidt Rovedder

    2008-02-01

    Full Text Available OBJETIVO: Determinar a prevalência de hipertensão pulmonar (HP em pacientes com fibrose cística (FC, comparar características clínicas, escore radiológico, função pulmonar e parâmetros ecocardiográficos nos grupos com e sem HP e correlacionar achados ecocardiográficos com características clínicas, escore radiológico e função pulmonar. MÉTODOS: Estudo transversal prospectivo em pacientes clinicamente estáveis (idade > 16 anos atendidos por um programa de adultos para FC. Os pacientes foram submetidos a avaliação clínica, ecocardiografia Doppler, testes de função pulmonar e exame radiológico do tórax. RESULTADOS: Obteve-se a velocidade de regurgitação tricúspide (VRT em 37 dos 40 pacientes estudados. A prevalência de HP foi de 49% com um ponte de corte da VRT de 2,5 m/s (18 pacientes e de 30% com um ponte de corte da VRT de 2,8 m/s (11 pacientes. Os valores de saturação periférica de oxigênio (SpO2 em repouso, escore clínico, volume expiratório forçado no primeiro segundo (VEF1 e capacidade vital forçada (CVF foram significativamente menores no grupo com HP. A VRT correlacionou-se significativamente com SpO2 em repouso (p OBJECTIVE: To determine the prevalence of pulmonary hypertension (PH in patients with cystic fibrosis (CF, to compare clinical characteristics, radiographic scores, pulmonary function, and echocardiographic parameters in patients with and without PH, and to correlate echocardiographic findings with clinical characteristics, radiographic scores, and pulmonary function. METHODS: This was a prospective, cross-sectional study involving clinically stable patients (aged 16 or older enrolled in an adult CF program. The patients were submitted to clinical evaluation, Doppler echocardiography, pulmonary function tests, and chest X-rays. RESULTS: Tricuspid regurgitant jet velocity (TRV was obtained in 37 of the 40 patients studied. The prevalence of PH was 49% with a TRV cut-off of 2.5 m/s (18 patients

  11. Correlação dos achados tomográficos com parâmetros de função pulmonar na fibrose pulmonar idiopática em não fumantes Correlation of tomographic findings with pulmonary function parameters in nonsmoking patients with idiopathic pulmonary fibrosis

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    Agnaldo José Lopes

    2007-12-01

    Full Text Available OBJETIVO: Correlacionar os achados tomográficos com os parâmetros de função pulmonar em portadores de fibrose pulmonar idiopática (FPI. MÉTODOS: Foi realizado um estudo de corte transversal, em que foram avaliados 30 pacientes não tabagistas, portadores de FPI. Utilizando um sistema de escore semiquantitativo, os seguintes achados na tomografia computadorizada de alta resolução (TCAR foram quantificados: extensão total da doença intersticial (Tot, infiltrado reticular e faveolamento (Ret+Fav, e opacidade em vidro fosco (Vif. As variáveis funcionais foram mensuradas através de espirometria, técnica de oscilações forçadas (TOF, método da diluição com hélio e método da respiração única para medir a capacidade de difusão do monóxido de carbono (DLCO. RESULTADOS: Dos 30 pacientes estudados, 18 eram mulheres e 12 eram homens, com média de idade de 70,9 anos. Foram encontradas correlações significativas de Tot e Ret+Fav com as medidas de capacidade vital forçada (CVF, capacidade pulmonar total (CPT, DLCO e complacência dinâmica do sistema respiratório (correlações negativas, e de Vif com volume residual/CPT (correlação positiva. A relação fluxo expiratório forçado entre 25 e 75% da CVF/CVF (FEF25-75%/CVF correlacionou-se positivamente com Tot, Ret+Fav e Vif. CONCLUSÕES: Em portadores de FPI, as medidas de volume, difusão e complacência dinâmica são as variáveis fisiológicas que melhor refletem a extensão da doença intersticial na TCAR.OBJECTIVE: To correlate tomographic findings with pulmonary function parameters in patients with idiopathic pulmonary fibrosis (IPF. METHODS: A cross-sectional study was carried out, in which 30 nonsmoking patients with IPF were evaluated. Using a semiquantitative scoring system, the following high-resolution computerized tomography (HRCT findings were quantified: total interstitial disease (TID, reticular abnormality/honeycombing, and ground-glass opacity (GGO. The

  12. Capnografia volumétrica como meio de detectar obstrução pulmonar periférica precoce em pacientes com fibrose cística Volumetric capnography as a tool to detect early peripheric lung obstruction in cystic fibrosis patients

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    Maria Ângela G. O. Ribeiro

    2012-12-01

    Full Text Available OBJETIVO: Comparar a espirometria e a capnografia volumétrica (CapV para determinar se os valores amostrados pela capnografia acrescentam informações sobre doenças pulmonares precoces em pacientes com fibrose cística (FC. MÉTODOS: Este foi um estudo do tipo corte transversal envolvendo pacientes com FC: Grupo I (42 pacientes, 6-12 anos de idade e Grupo II (22 pacientes, 13-20 anos de idade. Os grupos controle correspondentes eram formados por 30 e 50 indivíduos saudáveis, respectivamente. A capacidade vital forçada (CVF, o volume expiratório forçado no primeiro segundo (VEF1 e a relação VEF1/CVF foram determinados pela espirometria. Através da CapV, medimos a saturação periférica de oxigênio (SpO2, a frequência respiratória (FR, o tempo inspiratório (TI, o tempo expiratório (TE e o slope da fase III normalizado pelo volume corrente (slope da fase III/Vc. RESULTADOS: Em comparação com os grupos controle, todos os pacientes com FC apresentaram valores de slope da fase III/Vc (p OBJECTIVE: To compare spirometry and volumetric capnography (VCap to determine if the capnographic values add more information about early lung disease in cystic fibrosis (CF patients. METHODS: This was a cross-sectional study involving CF patients: Group I (42 patients, 6-12 years of age; and Group II (22 patients, 13-20 years of age. The corresponding control groups were comprised of 30 and 50 healthy subjects, respectively. Forced vital capacity (FVC, forced expiratory volume in one second (FEV1, and the FEV1/FVC ratio was determined by spirometry. Using VCap, we measured peripheral oxygen saturation (SpO2, respiratory rate (RR, inspiratory time (IT, expiratory time (ET, and the phase III slope normalized by expiratory volume (phase III slope/Ve. RESULTS: In comparison with control groups, all CF patients presented higher phase III slope/Ve values (p < 0.001 independent of the pulmonary disease stage. The phase III slope/Ve was significantly

  13. Deve-se extrapolar o tratamento de bronquiectasias em pacientes com fibrose cística para aqueles com bronquiectasias de outras etiologias? Should the bronchiectasis treatment given to cystic fibrosis patients be extrapolated to those with bronchiectasis from other causes?

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    Rodrigo Abensur Athanazio

    2010-08-01

    Full Text Available OBJETIVO: Conhecer o perfil de pacientes adultos com bronquiectasias, comparando portadores de fibrose cística (FC com aqueles com bronquiectasias de outra etiologia, a fim de determinar se é racional extrapolar terapêuticas instituídas em fibrocísticos para aqueles com bronquiectasias de outras etiologias. MÉTODOS: Análise retrospectiva dos prontuários de 87 pacientes adultos com diagnóstico de bronquiectasia em acompanhamento em nosso serviço. Pacientes com doença secundária a infecção por tuberculose corrente ou no passado foram excluídos. Foram avaliados dados clínicos, funcionais e terapêuticos dos pacientes. RESULTADOS: Dos 87 pacientes com bronquiectasias, 38 (43,7% tinham diagnóstico confirmado de FC através de dosagem de sódio e cloro no suor ou análise genética, enquanto 49 (56,3% apresentavam a doença por outra etiologia, 34 (39,0% desses com bronquiectasia idiopática. Os pacientes com FC apresentavam média de idade ao diagnóstico mais baixa (14,2 vs. 24,2 anos; p OBJECTIVE: To profile the characteristics of adult patients with bronchiectasis, drawing comparisons between cystic fibrosis (CF patients and those with bronchiectasis from other causes in order to determine whether it is rational to extrapolate the bronchiectasis treatment given to CF patients to those with bronchiectasis from other causes. METHODS: A retrospective analysis of the medical charts of 87 patients diagnosed with bronchiectasis and under follow-up treatment at our outpatient clinic. Patients who had tuberculosis (current or previous were excluded. We evaluated the clinical, functional, and treatment data of the patients. RESULTS: Of the 87 patients with bronchiectasis, 38 (43.7% had been diagnosed with CF, through determination of sweat sodium and chloride concentrations or through genetic analysis, whereas the disease was due to another etiology in 49 (56.3%, of whom 34 (39.0% had been diagnosed with idiopathic bronchiectasis. The mean

  14. Fibrose pulmonar idiopática: características clínicas e sobrevida em 132 pacientes com comprovação histológica Pulmonary idiopathic fibrosis: clinical findings and survival in 132 histologically-proven patients

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    ADALBERTO SPERB RUBIN

    2000-04-01

    Full Text Available Com o objetivo de avaliar as características clínicas e sobrevida de pacientes portadores de fibrose pulmonar idiopática, foram analisados 132 casos com confirmação histológica, internados no Pavilhão Pereira Filho entre 1970 e 1996. O diagnóstico foi realizado em 120 casos por biópsia a céu aberto e em 12 casos por biópsia transbrônquica. A idade média do grupo estudado foi de 56 anos; 78 eram do sexo masculino; 126 eram brancos e 6, negros. O tabagismo estava presente em 61 casos. A duração média dos sintomas antes do diagnóstico foi de 22,7 meses. O hipocratismo digital esteve presente em 75 pacientes e estertores teleinspiratórios foram verificados em 100 casos. Dispnéia só não foi constatada em dois pacientes e tosse esteve presente em 89 casos. As provas de função pulmonar apresentaram os seguintes valores médios: CVF, 62%; VEF1, 70%; DCO, 43,4%; CPT, 76,7%; PaO2, 67,3mmHg; PaCO2, 39,1mmHg e SaO2, 92,3%. O lavado broncoalveolar apresentou os seguintes valores médios: macrófagos, 83,8%; neutrófilos, 9,1%; linfócitos; 6,1% e eosinófilos, 0,6%. Na radiologia convencional de tórax, foi observado faveolamento em 79 casos, redução da capacidade pulmonar total em 107 e alargamento da traquéia intratorácica em 50. Na TC de tórax, o grau médio de profusão do padrão reticular foi de 42,3% e do padrão de granularidade, de 43,6%. O padrão histológico usual esteve presente em 128 casos, sendo apenas quatro pacientes portadores de padrão descamativo. Em 121 casos foram obtidas informações quanto à sobrevida em dezembro de 1997. A sobrevida média total desta série foi de 28 meses, sendo de 24 meses para os pacientes que foram a óbito. Os pacientes desta série apresentaram características associadas a um estágio avançado da doença. Este fato, mais a presença maciça de pacientes com padrão usual e a rígida seleção de casos muito provavelmente contribuíram para os resultados encontrados quanto

  15. Quadro clínico e nutricional de pacientes com fibrose cística: 20 anos de seguimento no HC-UFMG Clinical and nutritional aspects of a Center of Cystic Fibrosis-HC-UFMG: 20 years of follow-up

    Directory of Open Access Journals (Sweden)

    F. J. C. Reis

    2000-10-01

    Full Text Available OBJETIVOS: A fibrose cística (FC é a doença genética letal, de herança autossômica recessiva, mais comum entre pacientes de cor branca. O presente estudo foi realizado com o objetivo de identificar o quadro clínico e nutricional à admissão dos pacientes no Centro de Tratamento de FC do HC-UFMG e avaliar a sobrevida a longo prazo. PACIENTES E MÉTODOS: Em um período de 20 anos, 127 pacientes portadores de FC foram acompanhados longitudinalmente e submetidos a protocolo previamente estabelecido, após confirmação do diagnóstico pelo teste do suor. Foram obtidos na admissão dados demográficos, da apresentação clínica, nutricionais e laboratoriais. O genótipo foi obtido de 106 pacientes pela técnica do PCR. Os pacientes foram seguidos por mediana de 44 meses. A análise de sobrevida foi realizada utilizando-se o método de Kaplan-Meier. RESULTADOS: A mediana da idade do diagnóstico da FC foi de 33 meses. A manifestação clínica predominante na época do diagnóstico foi a associação de sintomas respiratórios e gastrointestinais crônicos, presentes em 61% das crianças. A mutação mais freqüente encontrada foi a deltaF508: 17 pacientes (16% eram homozigotos para essa mutação e 30 (28% eram heterozigotos. Houve uma prevalência de desnutrição à admissão de 63% tendo sido reduzida para 45% ao final do seguimento. Um total 20 pacientes (15,7% evoluiu para o óbito. A probabilidade estimada de sobrevida nos primeiros 12 meses após o diagnóstico foi de 96% e após 5 anos de seguimento de 80%. CONCLUSÕES: O diagnóstico da FC é ainda tardio em nosso meio e a sobrevida é menor quando comparada a dados internacionais. O seguimento desses pacientes em centro especializado permitiu uma melhora no estado nutricional apesar da inerente evolução da doença.BACKGROUND: Cystic fibrosis (CF is the most common severe autosomal recessive disease in caucasian population. The life expectancy of patients with CF has improved

  16. Densidade mineral óssea, função pulmonar, idade cronológica e idade de diagnóstico em crianças e adolescentes com fibrose cística Bone mineral density, pulmonary function, chronological age, and age at diagnosis in children and adolescents with cystic fibrosis

    Directory of Open Access Journals (Sweden)

    Márcio V.F. Donadio

    2013-04-01

    Full Text Available OBJETIVO: Avaliar a densidade mineral óssea de pacientes com fibrose cística (FC e correlacioná-la com possíveis variáveis intervenientes. MÉTODOS: Foram incluídos crianças e adolescentes com diagnóstico clínico de FC, idade entre seis e dezoito anos, e em acompanhamento ambulatorial. Primeiramente, foram coletados os dados demográficos, para posterior realização do teste espirométrico. Todos os pacientes responderam ao questionário de qualidade de vida em FC (QFC e realizaram o teste de caminhada dos seis minutos (TC6 e o exame de densitometria óssea (DXA. RESULTADOS: Foram incluídos 25 pacientes fibrocísticos, sendo 56% do sexo masculino. A média de idade foi de 12,3 ± 3,4 anos, altura de 149,2 ± 14,4 cm e peso de 44,4 ± 13,9 kg. A maioria dos dados de função pulmonar e de densidade mineral óssea (DMO encontrou-se dentro dos limites de normalidade. A média do volume expiratório forçado no primeiro segundo (VEF1 foi de 92,5 ± 23,6 (% do previsto, capacidade vital forçada (CVF de 104,4 ± 21,3 (% do previsto e o escore z da DMO de 0,1 ± 1,0. A DMO correlacionou-se de forma moderada com o VEF1 (r = 0,43; p = 0,03 e com a CVF (r = 0,57; p = 0,003. Em relação à idade cronológica e à idade de diagnóstico, também foi encontrada uma correlação moderada e inversa (r = -0,55; p = 0,004 /r = -0,57; p = 0,003, respectivamente. Entretanto, não foram encontradas correlações significativas com os dados do QFC, TC6 e índice de massa corporal. CONCLUSÃO: A maioria dos pacientes avaliados apresenta DMO dentro dos limites de normalidade e possui correlação positiva com a função pulmonar e negativa com a idade cronológica e a idade de diagnóstico.OBJECTIVE: To assess bone mineral density in patients with cystic fibrosis (CF, and to correlate it with possible intervening variables. METHODS: Children and adolescents diagnosed with CF, aged 6 to 18 years, followed at the outpatient clinic were included in the study

  17. Magnetic resonance imaging of lesions to the superficial plantar aponevrosis; Imagerie par resonance magnetique des lesions de l`aponevrose plantaire superficielle

    Energy Technology Data Exchange (ETDEWEB)

    Helie, O.; Dubayle, P.; Boyer, B.; Pharaboz, C. [Hopital des Armees Begin, 94 - Saint-Mande (France)

    1995-01-01

    MRI is an efficient imaging modality to establish the diagnosis of plantar fascia tear and planta fasciitis. MRI allows to differentiate recent rupture from scar and fasciitis. (authors). 13 refs., 6 figs.

  18. Deposição pulmonar de tobramicina inalatória antes e após fisioterapia respiratória e uso de salbutamol inalatório em pacientes com fibrose cística colonizados por Pseudomonas aeruginosa Pulmonary deposition of inhaled tobramycin prior to and after respiratory therapy and use of inhaled albuterol in cystic fibrosis patients colonized with Pseudomonas aeruginosa

    Directory of Open Access Journals (Sweden)

    Milena Baptistella Grotta

    2009-01-01

    Full Text Available OBJETIVO: Avaliar se a fisioterapia respiratória seguida do uso de salbutamol inalatório modifica a deposição pulmonar de tobramicina inalatória em pacientes com fibrose cística (FC e se a deposição pulmonar apresenta correlação com a gravidade da doença ou com o genótipo. MÉTODOS: Um estudo prospectivo foi realizado com pacientes com FC maiores de 6 anos e colonizados por Pseudomonas aeruginosa. Os critérios de exclusão foram exacerbação pulmonar, mudança terapêutica entre as fases do estudo e FEV1 OBJECTIVE: To evaluate whether respiratory therapy followed by the use of inhaled albuterol modifies the pulmonary deposition of inhaled tobramycin in patients with cystic fibrosis (CF and whether pulmonary deposition correlates with disease severity or genotype. METHODS: A prospective study was carried out including patients with CF older than 6 years of age and colonized with Pseudomonas aeruginosa. Exclusion criteria were pulmonary exacerbation, changes in therapy between the study phases and FEV1 < 25%. All patients were submitted to pulmonary scintigraphy by means of a scintillation camera equipped with a low energy all purpose collimator in order to evaluate drug penetration following the administration of inhaled 99mTc-tobramycin, as well as to pulmonary perfusion with 99mTc-macroaggregated albumin (phase 1. One month later, the same procedure was performed following respiratory therapy and administration of inhaled albuterol (phase 2. RESULTS: We included 24 patients (12 males aged 5-27 years (mean ± SD: 12.85 ± 6.64 years. The Shwachman score (SS was excellent/good in 8 patients, moderate/fair in 16 and poor in 0. Genotyping revealed that 7 patients were ΔF508 homozygotes, 13 were ΔF508 heterozygotes; and 4 presented other mutations. In all patients, lung deposition of tobramycin decreased in phase 2, especially in those with moderate/fair SS (p = 0.017 and in heterozygotes (p = 0.043. CONCLUSIONS: The use of a

  19. Influência da infecção pregressa pelo vírus da hepatite B na fibrose hepática em portadores de hepatite C crônica: avaliação retrospectiva de uma série de casos Influence of previous hepatitis B virus infection on liver fibrosis in patients with chronic hepatitis C: a retrospective case series evaluation

    Directory of Open Access Journals (Sweden)

    Gaspar Lisboa Neto

    2010-08-01

    Full Text Available INTRODUCÃO: A hepatite C é uma das principais causas de doença hepática em todo mundo. Apresenta um curso evolutivo dinâmico e influenciável por diversos co-fatores. Dentre eles, a infecção pregressa pelo vírus B (anti-HBcAg [+] e HBsAg [-] tem se associado a pior prognóstico histológico e terapêutico. Este trabalho teve como objetivo analisar a associação entre a infecção pregressa pelo vírus B e fibrose hepática em portadores de hepatite C crônica, de maneira independente. MÉTODOS: Foram revistos retrospectivamente prontuários médicos de pacientes infectados cronicamente pelo vírus C, atendidos consecutivamente durante um ano no ambulatório de Doenças Infecciosas e Parasitárias - HC FMUSP, quanto aos dados epidemiológicos, clínicos, laboratoriais e histológicos. A análise de independência do impacto da infecção pregressa pelo vírus B foi realizada através de modelo estatístico de regressão logística multivariado, considerando a detecção do anti-HBcAg como variável de exposição, sendo o desfecho a alteração estrutural histopatológica graus 3 e 4 (septos com formação de nódulos e cirrose.0 RESULTADOS: 145 indivíduos foram avaliados pelo estudo, 47.2% com anti-HBcAg (+. O fator de risco mais comumente relatado foi transfusão de sangue e hemoderivados (35,9%. Embora necrose em saca-bocado tenha sido encontrada com maior frequência no grupo de infecção pregressa, a sorologia anti-HBcAg (+ não se associou à fibrose hepática avançada. CONCLUSÕES: A infecção pregressa pelo vírus B não parece acentuar a lesão estrutural desencadeada pela hepatite C crônica, após controle estatístico para outros co-fatores sabidamente capazes de influenciar a história natural desta infecção.INTRODUCTION: Hepatitis C is a major cause of liver disease worldwide. Its evolutionary course is dynamics and may be influenced by several cofactors. Among them, previous hepatitis B virus infection (anti

  20. O cotidiano da família com filhos portadores de fibrose cística: subsídios para a enfermagem pediátrica El cotidiano de la familia con hijos portadores de fibrosis quística: aportes para la enfermería pediátrica The routine of families with children bearing cystic fibrosis: subsidies for pediatric nursing

    Directory of Open Access Journals (Sweden)

    Maria Cândida de Carvalho Furtado

    2003-02-01

    Full Text Available A fibrose cística é uma doença genética caracterizada pelo aumento na produção de muco que, depositado em alguns órgãos, causa: doença pulmonar obstrutiva crônica, insuficiência pancreática e nível elevado de eletrólitos no suor. Pretende-se, neste estudo, descrever o cotidiano de famílias com filhos portadores de fibrose cística nos aspectos relacionados à repercussão da doença crônica na dinâmica familiar. Para tanto, realizou-se um estudo qualitativo, com coleta de dados empíricos a partir de entrevistas com 14 famílias de pacientes menores de 18 anos em tratamento no Hospital das Clínicas da Faculdade de Medicina de Ribeirão Preto, da Universidade de São Paulo. Dos dados emergiram os seguintes temas: envolvimento da família, aceitação/entendimento da doença e enfrentando desafios. Identificamos uma vida de dependência relacionada à doença, com desgaste físico e emocional, tanto da criança quanto da família. Implicações para a enfermagem: constituição de modelos assistenciais que tenham a família como foco de atenção, considerando meio ambiente, estilo de vida e promoção à saúde como seus fundamentos básicos.La fibrosis quística es una enfermedad genética caracterizada por el aumento en la producción de moco que es depositado en algunos órganos causando: enfermedad pulmonar obstructiva crónica, insuficiencia pancreática y nivel elevado de electrolitos en el sudor. El objetivo de este estudio es describir el cotidiano de las familias con hijos portadores de fibrosis quística en los aspectos relacionados a la repercusión de la enfermedad crónica en la dinámica familiar. Por lo tanto, se realizó un estudio cualitativo, a través de la recolección de datos empíricos a partir de entrevistas con 14 familias de pacientes menores de 18 años en tratamiento en el Hospital Clínicas de la Facultad de Medicina de Ribeirão Preto de la Universidad de São Paulo. De los datos emergieron los

  1. USING OF MSC WITH DIFFERENT ONTOGENETIC MATURITY FOR CORRECTION OF CHRONIC FIBROSING LIVER DAMAGE

    Directory of Open Access Journals (Sweden)

    M. Y. Shagidulin

    2013-01-01

    Full Text Available Aim. To compare the effectiveness of MSC with different degree of ontogenetic maturity (MSC bone marrow – MSC BM and MSC umbilical cord – MSC UC on regenerative processes in injured liver. Methods. In 4 groups of experiments on Wistar rats (n = 80 with a model of fibrotic toxic liver damage (FLD it was studied the effect of MSCs with different degree of ontogenetic maturity on recovery processes at the regeneration of damaged liver: 1 gr. – Control, 2 gr. and 3 gr. introduction of MSC BM, included in Sphero®GEL-long in doses of 2.5 ×106 and 5.0 x 106 cells, respectively, and 4 gr. – introduction of MSC UC in the form of cell-spheroids (8–10 × 105 cells. The cells were injected into the damaged liver in 7 days after the end of FDL-modeling. The effect of cell therapy was studied during 180 days. The effectiveness of corrective therapy was evaluated by the results of functional and morphological investigations of livers (histological control of parenchymal and nonparenchy- mal liver tissue. Results. MSC BM in both doses and MSC UC contributed to a more rapid normalization of liver enzyme indices compared with the control (1 gr., but the differences in the rate of recovery of disturbed enzymatic liver functions between groups 2, 3 and 4 – were absent. In 90 days after the cell application it was determined a more pronounced recovery activity of cells in groups 3 and 4; in 180 days the more pronounced activation of recovery processes was observed in group 3; but in group 4 the sclerotic processes were more pro- nounced in this period. Conclusion. For the induction of recovery processes in damage liver it is advisable not to use the MSC UC, but to use MSC BM in the Sphero®GEL, because MSC BM exert not only local but also systemic immune-regulatory effect, increasing the pool of T-reg. cells, which are additional carriers of regenera- tion information in organism. 

  2. Catheter-based intervention for pulmonary vein stenosis due to fibrosing mediastinitis: The Mayo Clinic experience

    Directory of Open Access Journals (Sweden)

    Shiva P. Ponamgi

    2015-09-01

    Conclusions: We describe the largest reported case series of catheter-based intervention for PV stenosis in FM. Although catheter-based therapy improved hemodynamics, short-term vascular patency, and patient symptoms, the rate of life-threatening complications, restenosis, and mortality associated with these interventions was found to be high. Despite these associated risks, catheter-based intervention is the only palliative option available to improve quality of life in severely symptomatic patients with PV stenosis and FM. Patients with PV stenosis and FM (especially those with bilateral disease have an overall poor prognosis in spite of undergoing these interventions due to the progressive and recalcitrant nature of the disease. This underscores the need for further innovative approaches to manage this disease.

  3. Hermansky-Pudlak syndrome type 2 manifests with fibrosing lung disease early in childhood

    NARCIS (Netherlands)

    Hengst, M. (Meike); Naehrlich, L. (Lutz); P. Mahavadi (Poornima); Grosse-Onnebrink, J. (Joerg); Terheggen-Lagro, S. (Suzanne); Skanke, L.Hø. (Lars Høsøien); Schuch, L.A. (Luise A.); Brasch, F. (Frank); A. Guenther (Andreas); S. Reu (Simone); Ley-Zaporozhan, J. (Julia); Griese, M. (Matthias)

    2018-01-01

    textabstractBackground: Hermansky-Pudlak syndrome (HPS), a hereditary multisystem disorder with oculocutaneous albinism, may be caused by mutations in one of at least 10 separate genes. The HPS-2 subtype is distinguished by the presence of neutropenia and knowledge of its pulmonary phenotype in

  4. Prova do Suor no Diagnóstico Laboratorial da Fibrose Quistica

    OpenAIRE

    Costa, Alcina; Batalha, Lídia; Almeida, Suza; Vilares, Arminda; Pacheco, Paula; Silva, Conceição; Miranda, Armandina

    2014-01-01

    Objectivo: Apresentar a casuística da Prova do Suor, no período de 2009 a 2013 da UDR do DPS do INSA, I.P. Lisboa , e o estudo do gene CFTR, efetuado na Unidade de Genética Molecular, Departamento de Genética Humana, INSA, I.P. Lisboa.

  5. IMPACT OF ANTIVIRAL THERAPY FOR CHRONIC HEPATITIS C ON CYTOKINE SYNTHESIS AND HEPATIC FIBROSING PROCESSES

    Directory of Open Access Journals (Sweden)

    V. V. Shchekotov

    2015-01-01

    Full Text Available Objective: to estimate the time course of changes in the levels of tumor necrosis factor-α (TNF-α, interleukin-4 (IL-4, IL-6, and the hepatic fibrosis indicators hyaluronic acid (HA and liver elasticity index during combined antiviral therapy (AVT with interferon alpha-2b and ribavirin in patients with chronic hepatitis C (CHC. Subjects and methods. Fifty patients with CHC were examined. Serum TNF-α, IL-6, IL-4, and HA were estimated using an enzyme immunoassay. The stage of hepatic fibrosis was determined by fibroelastography with the liver elastic index being measured; the time course of changes in the indicators was assessed in 20 patients at the end of AVT. A virological response was monitored at therapy completion and 6 months later. Results. The patients with CHC in the reactivation phase were found to have enhanced TNF-α, IL-6, and IL-4 activities in 84, 60, and 100 % of the cases, respectively (р < 0.001, р = 0.01, р < 0.001, respectively. The median serum concentration of HA in CHC was 1.8-fold higher than that in the control group (p = 0.03; the liver elastic index averaged 6.5 kPa. TNF-α and IL-6 levels correlated with viremia, transaminases, and hepatic fibrosis indicators. At combined AVT completion, the virological response rate was as high as 85 %, which was attended by a considerable reduction in cytolysis, HA concentrations, and liver density index to 5.4 kPa (3.6–6.8 kPa (p < 0.04, and in the activity of the examined cytokines. The sustained virological response rate was 80 %. Only IL-4 levels decreased and TNF-α and IL-6 concentration remained at the baseline level in patients unresponsive to AVT. Conclusion. It is expedient to monitor TNF-α, IL-4, IL-6, and HA to evaluate the severity of liver involvement in CHC and to predict the efficiency of AVT.

  6. La structure, morphologie, et texture superficielle des dépôts d'avalanche de débris : cartographie de terrain, par télédétection et par modélisation analogique

    OpenAIRE

    Paguican, Engielle Mae

    2012-01-01

    Flank collapse generates avalanches and large landslides that significantly change the shape of a volcano and alter the surrounding landscape. Most types of volcanoes experience flank collapse at some point during their development. In the Philippines, for example, the numerous volcanoes with breached edifices belong to the cone, subcone, and massif morphometric classes. Debris avalanches occur frequently on both volcanic and non-volcanic terrains making it an important geologic event to cons...

  7. Unusual {sup 210}Po/{sup 210}Pb ratios in the surface water of the Gulf of Lions; Rapports {sup 210}Po/{sup 210}Pb inhabituels dans l`eau superficielle du golfe du Lion

    Energy Technology Data Exchange (ETDEWEB)

    Radakovitch, O.; Heussner, S. [Universite de Perpignan, 66 (France). Laboratoire de Sedimentologie et Geochimie Marines]|[Cerege, Europole de l`Arbois, 13 - Aix-en-Provence (France); Cherry, R.D.; Heyraud, M. [Cape Town Univ. (South Africa). Dept. of Physics

    1998-05-01

    Concentrations of {sup 210}Po and {sup 210}Pb have been measured in sea water collected in the Grand Rhone Canyon. Concentrations of {sup 210}Pb are at similar levels to those found in other Mediterranean sea water samples, and are at the levels which would be expected in comparison with global sea water data. The same applies to the {sup 210}Po concentrations in samples from below 100 m depth. Surface samples from above 100 m have {sup 210}Po at levels rather higher than expected, and their {sup 210}Po/{sup 210}Pb ratios are about 1 or more as compared with the usual open ocean ratio 0.5. These data can be interpreted as indicating another source of supply of {sup 210}Po to the surface layer in addition to the normal atmospheric input. We suggest that an episodic advective particulate input, probably originating in the output of the Rhone river and perhaps involving resuspension of fine particulates from the bottom sediments as a result of storm conditions, is he most likely candidate for this additional source. (authors) 47 refs.

  8. Economical incineration of volatile organic compounds (VOC) using oxide catalysts with optimized superficial properties; Incineration economique de composes organiques volatils (COV) a l'aide des catalyseurs d'oxydes aux proprietes superficielles optimisees

    Energy Technology Data Exchange (ETDEWEB)

    Evstratov, A. [Ecole Nationale Superieure des Techniques Industrielles et des Mines d' Ales, ENSTIMA, Centre LGEI, 30 - Ales (France)

    2001-07-01

    This study aims at presenting the existing possibilities of improvement of the technological parameters of the incineration processes for VOC-bearing industrial gases. Two different approaches are considered. One is based on the preliminary accumulation of the compounds to be degraded on catalytic surfaces having important acid-base and redox capabilities; the formation of the deposits is followed by the in situ catalytic incineration. The other is based on the application of catalysts with optimized acidities in order to limit the acid-base interactions and to maintain the catalytic surfaces in a stationary state at reduced temperatures. The first approach is applied to reactive VOC (unsaturated and polar compounds), while the other can be useful for the economical treatment of any type of VOC-bearing effluent. (J.S.)

  9. Fibrosing gastrointestinal leiomyositis as a cause of chronic intestinal pseudo-obstruction in an 8-month-old dog.

    Science.gov (United States)

    Johnson, C S; Fales-Williams, A J; Reimer, S B; Lotsikas, P J; Haynes, J S

    2007-01-01

    An 8-month-old, female, mixed-breed dog presented to the Iowa State University Veterinary Teaching Hospital with a 1-month history of vomiting and diarrhea. An exploratory laparotomy was performed revealing markedly distended and fluid-filled small and large intestines that were not obstructed. The clinical condition of the dog did not improve subsequent to exploratory surgery, and it was euthanized. At necropsy, both the small and large intestines were distended (approximately 4 cm in diameter) and fluid-filled, and the wall was thin. The abdominal cavity contained approximately 500 ml of a brownish clear fluid. Microscopic lesions of the intestines were confined to the intestinal tunica muscularis and muscularis mucosae and consisted of locally extensive-to-diffuse replacement of the smooth muscle by fibrous tissue and multifocal infiltration by a moderately dense mononuclear inflammatory infiltrate. A unique finding was the presence of similar microscopic lesions in the tunica muscularis of the urinary bladder and stomach.

  10. Oxidative Stress Markers in Exhaled Breath Condensate in Lung Fibroses Are Not Significantly Affected by Systemic Diseases

    Czech Academy of Sciences Publication Activity Database

    Pelclová, D.; Fenclová, Z.; Syslová, K.; Vlčková, Š.; Lebedová, J.; Pecha, O.; Běláček, J.; Navrátil, Tomáš; Kuzma, Marek; Kačer, P.

    2011-01-01

    Roč. 49, č. 6 (2011), s. 746-754 ISSN 0019-8366 Institutional research plan: CEZ:AV0Z40400503; CEZ:AV0Z50200510 Keywords : 8-iso-prostaglandin F-2 alpha * 4-hydroxy-trans-2-nonenale * Malondialdehyde Subject RIV: CF - Physical ; Theoretical Chemistry Impact factor: 0.940, year: 2011

  11. Fibrose hépatique : lien force mécanique et génotoxicité

    OpenAIRE

    Langouët , Sophie

    2014-01-01

    En 2012, en France, il y a eu 6.867 nouveaux cas de cancer du foie chez l’homme et 1.586 chez la femme. On soupçonne que les maladies du foie sont associées à un dysfonctionnement sévère des fonctions hépatiques et susceptibles d’entraîner une sensibilité accrue des patients vis-à-vis des contaminants environnementaux. Comment évaluer cette susceptibilité individuelle au cours de maladies chroniques, qui se prolongent dans le temps ? L’objectif du projet Mécagénotox est de caractériser et mod...

  12. Malignant transformation of a rat fibroma by the treatment with an anti-fibrosing drug: CY-168F (Plastenan

    Directory of Open Access Journals (Sweden)

    Sonia G. Andrade

    1981-09-01

    Full Text Available Fifteen albino (Sprague Dawley rats with subcutaneous transplanted fibromas was used in the present study. The tumour was formed by typical fibroblasts in a dense collagen matrix and was provenient from a fibroma that appeared spontaneously in an albino rat of the same strain. Ultrastructurally collagen disclosed normal periodicity and the fibroblasts showed irregular notched nuclei with irregular distribution of chromatin, that suggests transitional aspects to fibrosarcoma. The 15 animals, from different passage groups, were divided into: 8 animals submitted to treatment with the drug acexamic acid (CY-168F - N acetyl-amino-6-hexanoic acid (plastenan and 7 untreated control animals. Three of the treated animals showed a malignant transformation to fibrosarcoma. transitional histological features from typical fibroma to highly indifferentiated fibrosarcoma could be detected in come animal subjected to repeated biopsies. Ultrastructural study disclosed nuclear alterations and hyperactive ergastoplasm and collagen containing inclusions into the cytoplasm of fibroblasts. In the group of 7 untreated naimals, no malignant transformation could be detected histologically. Two aspects deserve attention: the malignant potential of a typical fibroma and the apparent effect of an antifibrosing drug in inducing malignization of this tumour.Foram utilizados no presente estudo, quinze ratos albinos (Sprague Dawley portadores de fibronas transplantados no tecido subcutâneo da parede abdominal. O tumor era constituído por fibroblastos típicos, em uma densa matriz colagênica e eram provenientes de um fibroma que apareceu espontaneamente em um rato albino da mesma linhagem. Ultraestruturalmente o colágeno mostrava periodicidade normal e os fibroblastos mostravam núcleo irregularmente denteado, com cromatina irregularmente distribuída, sugerindo aspectos transicionais para um fibrossarcoma. Os quinze animais, de diferentes grupos de passagem do tumor, foram divididos em: oito animais submetidos a tratamento com a droga CY-168F (Plastenan e sete animais controles não tratados. Três dos animais tratados mostraram uma transformação malígna para um fibrossarcoma. Em um dos animais emque foram feitas biópsias sucessivas, foram observados aspectos histológicos transicionais de um fibroma típico até um fibrossarcoma altamente indiferenciado. O estudo ultraestrutural demonstrou alterações nucleares, ergastoplasma hiperativo e inclusções contendo colágeno, no citoplasma dos fibroblastos. No grupo dos sete animais não tratados, não foi verificada transformação malígna do tumor. Dois aspectos merecem atenção neste estudo; o potencial malígno de um fibroma típico e o aparente efeito de um antifibrosante na indução da malignização deste tumor.

  13. Aberrant immune response with consequent vascular and connective tissue remodeling - causal to scleroderma and associated syndromes such as Raynaud phenomenon and other fibrosing syndromes?

    Science.gov (United States)

    Durmus, Nedim; Park, Sung-Hyun; Reibman, Joan; Grunig, Gabriele

    2016-11-01

    Scleroderma and other autoimmune-induced connective tissue diseases are characterized by dysfunctions in the immune system, connective tissue and the vasculature. We are focusing on systemic sclerosis (SSc)-associated pulmonary hypertension, which remains a leading cause of death with only a 50-60% of 2-year survival rate. Much research and translational efforts have been directed at understanding the immune response that causes SSc and the networked interactions with the connective tissue and the vasculature. One of the unexpected findings was that in some cases the pathogenic immune response in SSc resembles the immune response to helminth parasites. During coevolution, means of communication were developed which protect the host from over-colonization with parasites and which protect the parasite from excessive host responses. One explanation for the geographically clustered occurrence of SSc is that environmental exposures combined with genetic predisposition turn on triggers of molecular and cellular modules that were once initiated by parasites. Future research is needed to further understand the parasite-derived signals that dampen the host response. Therapeutic helminth infection or treatment with parasite-derived response modifiers could be promising new management tools for autoimmune connective tissue diseases.

  14. Comparative study of Candida in oral submucous fibrosis and healthy individuals = Estudo comparativo de Candida em fibrose submucosa oral e indivíduos saudáveis

    Directory of Open Access Journals (Sweden)

    Anila, K.

    2011-01-01

    Conclusão: A incidência e intensidade de Candida (primariamente C. albicans foi maior nos pacientes com FSO que nos sujeitos controle, mas estes achados estavam dentro dos limites normais (3-47%. Portanto, Candida pode não ser um fator etiológico na transformação maligna. Entretanto, ainda há controvérsias se o hábito de mascar ‘betel’ em casos de FSO teria um efeito inibidor ou promoveria a aderência e invasão de Candida

  15. Comparative study of Candida in oral submucous fibrosis and healthy individuals = Estudo comparativo de Candida em fibrose submucosa oral e indivíduos saudáveis

    Directory of Open Access Journals (Sweden)

    Anila, K.

    2011-01-01

    Conclusão: A incidência e intensidade de Candida (primariamente C. albicans foi maior nos pacientes com FSO que nos sujeitos controle, mas estes achados estavam dentro dos limites normais (3-47%. Portanto, Candida pode não ser um fator etiológico na transformação maligna. Entretanto, ainda há controvérsias se o hábito de mascar ‘betel’ em casos de FSO teria um efeito inibidor ou promoveria a aderência e invasão de Candida.

  16. Nephrogenic systemic fibrosis after application of gadolinium-based contrast agents - a status paper; Nephrogene systemische Fibrose nach Anwendung gadoliniumhaltiger Kontrastmittel - ein Statuspapier zum aktuellen Stand des Wissens

    Energy Technology Data Exchange (ETDEWEB)

    Heinrich, M.; Uder, M. [Erlangen-Nuernberg Univ., Erlangen (Germany). Inst. fuer Radiologie

    2007-06-15

    Recently the association of a rare disease named ''nephrogenic systemic fibrosis'' (NSF) with the administration of gadolinium-containing contrast media, especially gadodiamide (Omniscan, GE-Healthcare), was described. NSF is a scleroderma-like disease characterised by widespread tissue fibrosis. Until now, NSF cases were observed only in patients with kidney disease. Almost all patients were suffering from chronic renal insufficiency, 90 % of them required renal replacement therapy. The true incidence of the disease is unknown. First retrospective analyses of selected collectives of patients with end-stage renal disease showed 2 - 5 % cases of NSF after administration of Gadolinium-containing contrast agents with an odds ratio of 20 - 50 in comparison to non-exposed controls. NSF is a serious adverse reaction, which may result in severe disabilities and even death. Therefore all radiologists applying gadolinium-based contrast agents should be informed about this disease and the recent recommendations for its prevention. On the basis of the published data, Omniscan should not be used in patients with severe renal impairment (GFR < 30 ml/min/1.73 m{sup 2}) and those who have had or are undergoing liver transplantation. In neonates and infants up to 1 year of age, Omniscan should only be used after careful consideration. Also the other gadolinium-based contrast agents should be used in high-risk patients only after careful consideration using the lowest dose possible.

  17. Photobiomodulation therapy alleviates tissue fibroses associated with chronic Graft-Versus-Host Disease : Two case reports and putative anti-fibrotic roles of TGF-β

    NARCIS (Netherlands)

    Epstein, J.B.; Raber-Durlacher, J.E.; Huysmans, M.C.; Schoordijk, M.C.E.; Cheng, J.E.; Bensadoun, R.J.; Arany, P.R.

    2018-01-01

    Objective: Patients who receive allogeneic hematopoietic stem cell transplantation may experience oral complications due to chronic graft-versus-host disease (cGVHD). The manifestations may include progressive sclerosis-like changes that may involve various body sites, including the oropharynx.

  18. Photobiomodulation Therapy Alleviates Tissue Fibroses Associated with Chronic Graft-Versus-Host Disease: Two Case Reports and Putative Anti-Fibrotic Roles of TGF-

    NARCIS (Netherlands)

    Epstein, Joel B.; Raber-Durlacher, Judith E.; Huysmans, Marie-Charlotte; Schoordijk, Maria C. E.; Cheng, Jerry E.; Bensadoun, Rene-Jean; Arany, Praveen R.

    2018-01-01

    Objective: Patients who receive allogeneic hematopoietic stem cell transplantation may experience oral complications due to chronic graft-versus-host disease (cGVHD). The manifestations may include progressive sclerosis-like changes that may involve various body sites, including the oropharynx.

  19. Connective tissue growth factor in renal development and injury

    NARCIS (Netherlands)

    Ito, Y.

    2011-01-01

    Langdurige weefselbeschadiging leidt vaak tot functieverlies van het betreffende orgaan door het ontstaan van veel littekens (fibrose). Yasuhiko Ito ontdekte dat genexpressie van de factor CTGF sterk is verhoogd bij veelvoorkomende nierziekten waarbij fibrose optreedt. De hoeveelheid CTGF in de

  20. Attenuating fibrosis : Modulation of myofibroblast formation and extracellular matrix synthesis

    NARCIS (Netherlands)

    Mia, Md Masum

    2016-01-01

    Fibrose behelst het verlittekenen van weefsels en organen. Daardoor kunnen vitale functies uitvallen, met de dood tot gevolg. Fibrose is in veel gevallen dan ook een zeer ernstige aandoening. Toch bestaat er geen enkel effectief medicijn tegen. Hét kenmerk van fibrose is de excessieve productie van

  1. Identificação de bactérias não fermentadoras isoladas de pacientes com fibrose cística e em hemoculturas de pacientes internados no HC da Unicamp

    OpenAIRE

    Élio Barreto de Carvalho Filho

    2015-01-01

    Resumo: Introdução: Bacilos gram-negativos não fermentadores (BGN-NFs) são microrganismos que se caracterizam pela incapacidade de utilizar a glicose como fonte de energia pela fermentação, degradando-a pela via oxidativa. A identificação dos BGN-NFs continua sendo um desafio para os laboratórios de rotina em microbiologia pela dificuldade de identificação, em virtude, da baixa ocorrência em amostras ambulatoriais, assim como, pela falta de recursos rápidos, eficientes e pela complexidade e ...

  2. Fibrose congênita de reto inferior atípica com hipotonia muscular generalizada: relato de um caso Atipical inferior rectus congenital fibrosis with generalizated muscular hypotonia: case report

    Directory of Open Access Journals (Sweden)

    Ezon Vinicius Alves Pinto Ferraz

    1999-10-01

    Full Text Available O caso descrito apresentava uma hipotropia pouco severa com acuidade visual normal e binocularidade em infra-versão; além de uma hipotonia muscular generalizada, ao contrário da severa hipotropia e ambliopia encontrada nessa afecção. O procedimento cirúrgico levou estes fatos em consideração, tendo-se optado por um retrocesso pequeno do reto inferior para que se corrigisse o torcicolo sem interferir na posição de leitura. Os resultados pós-operatórios foram satisfatórios.A case report of an atipical inferior rectus congenital fibrosis associated with a generalized muscular hypotonia is presented. It shows mild hipotropia with a normal visual acuity and binocularity in infraversion position contrasting with the severe hipotropia and ambliopy found. The surgical procedure took these facts into consideration, so we performed a small retrocess of the muscle with the objective to correct the head position without changes in the reading position. The postoperative results were satisfactory.

  3. Application of extracellular gadolinium-based MRI contrast agents and the risk of nephrogenic systemic fibrosis; Anwendung von extrazellulaeren gadoliniumhaltigen MR-Kontrastmitteln und Risiko der Nephrogenen Systemischen Fibrose

    Energy Technology Data Exchange (ETDEWEB)

    Heverhagen, J.T. [Univ. Hospital Bern (Switzerland). Inst. of Diagnostic, Interventional and Pediatric Radiology, Inselspital; Krombach, G.A. [Justus Liebig Univ. Hopsital Giessen (Germany). Diagnostic and Interventional Radiology; Gizewski, E. [Medical Univ. Innsbruck (Austria). Dept. of Neuroradiology

    2014-07-15

    Nephrogenic systemic fibrosis (NSF) is a serious, sometimes fatal disease. Findings in recent years have shown that a causal association between gadolinium containing contrast media and NSF is most likely. Therefore, the regulatory authorities have issued guidelines on the use of gadolinium-containing contrast media which have reduced the number of new cases of NSF to almost zero. However, it is for precisely this reason that the greatest care must still be taken to ensure that these guidelines are complied with. The most important factors are renal function, the quantity of gadolinium administered and coexisting diseases such as inflammation. All of these factors crucially influence the quantity of gadolinium released from the chelat in the body. This free gadolinium is thought to be the trigger for NSF. Other important factors are the stability of the gadolinium complex and furthermore the route of its elimination from the body. Partial elimination via the liver might be an additional protective mechanism. In conclusion, despite the NSF risk, contrast-enhanced MRI is a safe diagnostic procedure which can be used reliably and safely even in patients with severe renal failure, and does not necessarily have to be replaced by other methods.

  4. Diagnóstico ecodopplercardiográfico da fibrose crônica da válvula mitral em cão Echodopplercardiographic diagnostic of mitral chronic valvular fibrosis in dog

    Directory of Open Access Journals (Sweden)

    R.A.L. Muzzi

    1999-12-01

    Full Text Available A ten year-old male Poodle dog, weighing 3kg, was referred to the Veterinary Hospital of UFMG due to nocturnal coughing, exercise intolerance, weakness and weight loss. Physical examination revealed pale mucous membranes, prolonged capillary refill time, holosystolic regurgitant murmur at left apex and signs of congestive heart failure. M-mode, two-dimensional, and Doppler echocardiography revealed mitral chronic valvular disease

  5. MRI-based flow measurements in the main pulmonary artery to detect pulmonary arterial hypertension in patients with cystic fibrosis; MRT-basierte Flussmessungen im Truncus pulmonalis zur Detektion einer pulmonal-arteriellen Hypertonie in Patienten mit zystischer Fibrose

    Energy Technology Data Exchange (ETDEWEB)

    Wolf, T.; Anjorin, A.; Abolmaali, N. [TU Dresden (Germany). OncoRay, Biologisches und Molekulares Imaging; Posselt, H. [Frankfurt Univ. (Germany). Klinik fuer Paediatrie I, Muskoviszidoseambulanz; Smaczny, C. [Frankfurt Univ. (Germany). Medizinische Klinik I, Pneumologie und Allergologie; Vogl, T.J. [Frankfurt Univ. (Germany). Inst. fuer Diagnostische und Interventionelle Radiologie

    2009-02-15

    Development of pulmonary arterial hypertension (PH) is a common problem in the course of patients suffering from cystic fibrosis (CF). This study was performed to evaluate MRI based flow measurements (MR{sub venc}; Velocity ENCoding) to detect signs of an evolving PH in patients suffering from CF. 48 patients (median age: 16 years, range: 10 - 40 years, 25 female) suffering from CF of different severity (mean FEV1: 74 % {+-} 23, mean Shwachman-score: 63 {+-} 10) were examined using MRI based flow measurements of the main pulmonary artery (MPA). Phase-contrast flash sequences (TR: 9.6 ms, TE: 2.5 ms, bandwidth: 1395 Hertz/Pixel) were utilized. Results were compared to an age- and sex-matched group of 48 healthy subjects. Analyzed flow data where: heart frequency (HF), cardiac output (HZV), acceleration time (AT), proportional acceleration time related to heart rate (ATr), mean systolic blood velocity (MFG), peak velocity (Peak), maximum flow (Fluss{sub max}), mean flow (Fluss{sub mitt}) and distensibility (Dist). The comparison of means revealed significant differences only for MFG, Fluss{sub max} and Dist, but overlap was marked. However, using a scatter-plot of AT versus MFG, it was possible to identify five CF-patients demonstrating definite signs of PH: AT = 81 ms {+-} 14, MFG = 46 {+-} 11 cm/s, Dist = 41 % {+-} 7. These CF-patients where the most severely affected in the investigated group, two of them were listed for complete heart and lung transplantation. The comparison of this subgroup and the remaining CF-patients revealed a highly significant difference for the AT (p = 0.000001) without overlap. Screening of CF-patients for the development of PH using MR{sub venc} of the MPA is not possible. In later stages of disease, the quantification of AT, MFG and Dist in the MPA may be useful for the detection, follow-up and control of therapy of PH. MR{sub venc} of the MPA completes the MRI-based follow-up of lung parenchyma damage in patients suffering from CF. (orig.)

  6. {sup 18}F-F.D.G. PET imaging of infection and inflammation: intestinal, prosthesis replacements, fibrosis, sarcoidosis, tuberculosis..; La TEP au {sup 18}F-FDG dans la pathologie inflammatoire et infectieuse: intestinale, prothetique, fibrose, sarcoidose, tuberculose..

    Energy Technology Data Exchange (ETDEWEB)

    Fernandez, A.; Cortes, M.; Caresia, A.P.; Juan, R. de; Vidaller, A.; Mana, J.; Martinez-Yelamos, S.; Gamez, C. [Hospital Universitari de Bellvitge, Service TEP-Centre IDI, Services de Medecine Interne, Barcelone (Spain)

    2008-10-15

    Nuclear medicine plays an important role in the evaluation of infection and inflammation. A variety of diagnostic methods are available for imaging this inflammation and infection, most notably computed tomography, {sup 68}Ga scintigraphy or radionuclide labeled leucocytes. Fluorine 18 fluorodeoxyglucose ({sup 18}F-F.D.G.) is a readily available radiotracer that offers rapid, exquisitely sensitive high-resolution images by positron emission tomography (PET). Inflammation can be acute or chronic, the former showing predominantly neutrophilic granulocyte infiltrates, whereas in the latter, macrophages predominate. F.D.G. uptake in infection is based on the fact that mononuclear cells and granulocytes use large quantities of glucose by way of the hexose monophosphate shunts. {sup 18}F-F.D.G. PET accurately helps diagnose spinal osteomyelitis, diabetic foot and in inflammatory conditions such as sarcoidosis and tuberculosis.(it appears to be useful for defining the extent of disease and monitoring response to treatment). {sup 18}F-F.D.G. PET can also help localize the source of fever of undetermined origin, thereby guiding additional testing. {sup 18}F-F.D.G. PET may be of limited usefulness in postoperative patients and in patients with a failed joint prosthesis or bowel inflammatory disease. In this review, we will focus on the role of {sup 18}F-F.D.G. PET in the management of patients with inflammation or suspected or confirmed infection.

  7. Diagnosis of chronic rhino sinusitis in patients with cystic fibrosis: correlation between anamnesis, nasal endoscopy and computed tomography; Diagnostico de rinossinusite cronica em pacientes com fibrose cistica: correlacao entre anamnese, nasofibroscopia e tomografia computadorizada

    Energy Technology Data Exchange (ETDEWEB)

    Boari, Leticia; Castro Junior, Ney Penteado de [Faculdade de Ciencias Medicas da Santa Casa de Sao Paulo, SP (Brazil)]. E-mail: curyboari@uol.com.br

    2005-11-15

    The sinonasal involvement is one of the most common manifestations in cystic fibrosis. Data show a high incidence of chronic rhino sinusitis in these patients. Although it has been found radiographic opacification of the sinus in more than 90% of cases, few are symptomatic. So that, it is difficult to recognize nasossinusal disease in patients with cystic fibrosis. Questionnaire, nasal endoscopy and CT-scan are very important methods in this approach. Aim: to evaluate the diagnosis of chronic rhino sinusitis in patients with cystic fibrosis by anamnesis, nasal endoscopy and CT-scan and compare those results.Study Design: Clinical prospective. Material and method: evaluation of 34 patients - older than 6 years and with a confirmed diagnoses of cystic fibrosis - by anamnesis (questionnaire), nasal endoscopy (score Lund-Kennedy) and CT-scan (score Lund-Mackay). Results: chronic rhino sinusitis was confirmed in: 20,58% of cases by the questionnaire, 73,52% of the cases by the nasal endoscopy and in 93,54% of the cases by the CT-scan. The results showed significant differences. The correlation between nasal endoscopy score (Lund-Kennedy score) and CT-scan score (Lund-Mackay score) was statistically significant. Conclusion: the diagnosis of chronic rhino sinusitis was statistically different between the three methods. It was higher in imaging analysis and lower in questionnaire. The nasal endoscopy is an excellent method to evaluate nasossinusal disease in cystic fibrosis. (author)

  8. Contamination métallique de l'eau et du sédiment d'Oued Bouhamra ...

    African Journals Online (AJOL)

    d'identifier les origines industrielle, agricole et domestique de cette pollution. Mots-clés : éléments traces métalliques- rejets urbains en mer – eau superficielle – sédiment - Golfe d'Annaba. Abstract ...... thermique des déchets). Ed. TEC & DOC ...

  9. Mesure de la vitesse d'infiltration des eaux dans le sol : Cas des sols ...

    African Journals Online (AJOL)

    C'est donc une infiltration superficielle qui ne peut pas modifier la composition des eaux de l'aquifère. Ces sols sont alors favorables à une telle agriculture. Mots clés: mesure, vitesse, infiltration, sols, pollutions, eau, Niari, Congo. English Title: Measuring the speed of the water infiltration into the soil: case of the soil of the ...

  10. Contribution à la connaissance du genre Asellus en Yougoslavie et en Turquie

    NARCIS (Netherlands)

    Deeleman-Reinhold, C.L.

    1965-01-01

    INTRODUCTION En 1962, 1963 et 1964, mon mari, Monsieur P. Robert Deeieman et moi, nous avons entrepris des voyages en Yougoslavie, dont le but principal fut la capture d'animaux cavernicoles. Dans les eaux souterraines et superficielles nous avons récolté cinq espèces du genre Asellus Geoffroy, dont

  11. 1665-IJBCS-Article -Majdouline Larif

    African Journals Online (AJOL)

    hp

    Duncan montre un effet hautement significatif (p<0,001) entre la localisation des stations et les saisons. Il nous permet de ... affectent les eaux souterraines mais aussi superficielles ..... des eaux : courants, turbulence et action des marées. On.

  12. Disease: H01906 [KEGG MEDICUS

    Lifescience Database Archive (English)

    Full Text Available H01906 Poikiloderma, hereditary fibrosing, with tendon contractures, myopathy, and... pulmonary fibrosis Poikiloderma, hereditary fibrosing, with tendon contractures, myopathy, and pulmonary fi...ies especially on the face and sun-exposed areas. Scalp hair, eyelashes, and eyebrows are typically sparse. Tendon contract

  13. Sarcoidosis and Histoplasmosis: Is One a Consequence of the Other? A Case Report and Review of the Literature

    Directory of Open Access Journals (Sweden)

    Anupam Bansal

    2015-01-01

    Full Text Available Sarcoidosis involves abnormal collections of inflammatory cells (granulomas which may form as nodules in multiple organs. 90% of affected patients have respiratory tract abnormalities. We present a 61-year-old male with sarcoidosis who was admitted for respiratory distress. Fibrosing mediastinitis was seen in the chest computograph. Management was conservative and included steroids, antibiotics, and oxygen therapy. Sarcoidosis and fibrosing mediastinitis are rare. Fibrosing mediastinitis is more commonly seen with histoplasmosis. We explore the clinical similarities between histoplasmosis and sarcoidosis. We also explore the potential cause and effect relationship and workup for each disease entity.

  14. Illustrations

    OpenAIRE

    2015-01-01

    Fig. 5 – Détail de la carte des formations superficielles du plateau insulaire de la Guadeloupe(Augris et al., 1992) Fig. 6 – Détail de la carte des formations superficielles du plateau insulaire de la Martinique(Augris et al., 2000) Baie échancrée, récif frangeant, récif barrière, et mangrove sur la côte est de la Martinique.© Ifremer/A. Guillou Mangrove à Rhizophora mangle dans le Grand Cul-de-Sac Marin (Gùadeloupe).© M. Louis Pente externe d'un récif corallien en bon état de santé (Mar...

  15. The Emerging Role of Soluble Adenylyl Cyclase in Primary Biliary Cholangitis

    NARCIS (Netherlands)

    Chang, Jung-Chin; Beuers, Ulrich; Oude Elferink, Ronald P. J.

    2017-01-01

    Primary biliary cholangitis (PBC; previously referred to as primary biliary cirrhosis) is a chronic fibrosing cholangiopathy with the signature of an autoimmune disease and features of intrahepatic cholestasis. Immunosuppressing treatments are largely unsuccessful. Responsiveness to ursodeoxycholic

  16. ETUDE DE LA DISTRIBUTION DES CATIONS ECHANGEABLES

    African Journals Online (AJOL)

    SEI Joseph

    pollution. L'objectif du présent travail est l'étude de la qualité des eaux superficielles et souterraines pour évaluer le degré de pollution provenant des lixiviats de la décharge et des cours d'eaux dans lesquels les ..... Les métaux lourds dosés ont montré une pollution métallique des eaux souterraine par rapport à la norme.

  17. The invisible patients: posttraumatic stress disorder in parents of individuals with cystic fibrosis

    OpenAIRE

    Cabizuca, Mariana; Mendlowicz, Mauro; Marques-Portella, Carla; Ragoni, Celina; Coutinho, Evandro Silva Freire; Souza, Wanderson de; Mari, Jair de Jesus [UNIFESP; Figueira, Ivan

    2010-01-01

    CONTEXTO: Apesar do crescente reconhecimento da relevância do transtorno de estresse pós-traumático (TEPT) secundário a doenças médicas, ainda não existem estudos em fibrose cística. OBJETIVO: Verificar a prevalência de TEPT e dos três grupos de sintomas de estresse pós-traumático em pais de pacientes com fibrose cística. MÉTODOS: Pais de pacientes com fibrose cística (idade média: 2 a 33 anos) foram recrutados da Associação Carioca de Mucoviscidose. Neste estudo transversal, os pais preenche...

  18. Evaluation du risque de déstabilisation des infrastructures de haute montagne engendré par le réchauffement climatique dans les Alpes françaises

    OpenAIRE

    Duvillard, Pierre-Allain; Ravanel, Ludovic; Deline, Philip

    2015-01-01

    Dans le contexte du changement climatique actuel, les milieux de haute montagne sont soumis à de profonds bouleversements tels que la fonte des glaciers ou la dégradation du permafrost, à l’origine de mouvements de versant au sein des parois rocheuses comme des formations superficielles. Ces processus impliquent des risques directs de déstabilisation pour les infrastructures (refuges, remontées mécaniques, etc.). Dans un but de prévention de ces risques, un recensement des infrastructures de ...

  19. Stratégie d'échantillonnage pour la campagne nationale de recherche des résidus de médicaments dans l'eau

    OpenAIRE

    Py , Jean-Sébastien; Rosin , Christophe; Hollard , Caroline; Clavos , Marie-Christelle; Rimlinger , Nicole; Piat , Adeline; Garnier , Alexandra; Munoz , Jean-François

    2009-01-01

    National audience; De nombreuses études nationales et internationales ont mis en évidence la présence dans l'eau des rivières de médicaments à l'état de traces, et leurs effets biologiques sur les écosystèmes. En France, des campagnes exploratoires de mesure de la contamination des ressources superficielles (eaux de surface et eaux souterraines) utilisées pour la production d'eaux destinées à la consommation humaine (EDCH) sur trois bassins hydrographiques du territoire français ont montré la...

  20. Vers une esthétique de la science-fiction

    OpenAIRE

    Russ, Joanna

    2013-01-01

    Les outils critiques élaborés en ayant à l’esprit une littérature entièrement différente ne fonctionnent pas quand ils sont appliqués à la science-fiction. Dans cet article, j’avance la proposition suivante : que la science-fiction est didactique. Malgré des similitudes superficielles avec la fiction moderne naturaliste (ou autre), les personnages de science-fiction sont toujours des personnes collectives, jamais individuelles. Je suggère que la critique littéraire contemporaine n’est pas l’...

  1. A moderate response to plasmapheresis in nephrogenic systemic fibrosis

    Directory of Open Access Journals (Sweden)

    Pelin Ustuner

    2011-11-01

    Full Text Available Nephrogenic systemic fibrosis (NSF is a recently identified idiopathic cutaneous fibrosing disorder that occurs in the setting of renal failure. The disease initially called nephrogenic fibrosing dermopathy is closely linked to exposure to gadolinium-based contrast media used during magnetic resonance imaging in patients with renal insufficiency. Although little is known about the pathogenesis of this disease, the increased expression of transforming growth factor-beta has been demonstrated recently. Herein, we present a case of NSF was partially treated due to a moderate and temporary response to plasmapheresis with no recurrence for 6 months, but returned at the end of 6th month.

  2. Protease-activated receptor (PAR)-2 is required for PAR-1 signalling in pulmonary fibrosis

    NARCIS (Netherlands)

    Lin, Cong; von der Thüsen, Jan; Daalhuisen, Joost; ten Brink, Marieke; Crestani, Bruno; van der Poll, Tom; Borensztajn, Keren; Spek, C. Arnold

    2015-01-01

    Idiopathic pulmonary fibrosis is the most devastating diffuse fibrosing lung disease of unknown aetiology. Compelling evidence suggests that both protease-activated receptor (PAR)-1 and PAR-2 participate in the development of pulmonary fibrosis. Previous studies have shown that bleomycin-induced

  3. Pharmacological targeting of protease-activated receptor 2 affords protection from bleomycin-induced pulmonary fibrosis

    NARCIS (Netherlands)

    C. Lin (Cong); J. von der Thusen (Jan); J. Daalhuisen (Joost); M. Ten Brink (Marieke); B. Crestani (Bruno); T. van der Poll (Tom); K. Borensztajn (Keren); C. Arnold Spek (C.)

    2015-01-01

    textabstractIdiopathic pulmonary fibrosis is the most devastating diffuse fibrosing lung disease that remains refractory to therapy. Despite increasing evidence that protease-activated receptor 2 (PAR-2) contributes to fibrosis, its importance in pulmonary fibrosis is under debate. We addressed

  4. Pharmacological Targeting of Protease-Activated Receptor 2 Affords Protection from Bleomycin-Induced Pulmonary Fibrosis

    NARCIS (Netherlands)

    Lin, Cong; von der Thüsen, Jan; Daalhuisen, Joost; ten Brink, Marieke; Crestani, Bruno; van der Poll, Tom; Borensztajn, Keren; Spek, C. Arnold

    2015-01-01

    Idiopathic pulmonary fibrosis is the most devastating diffuse fibrosing lung disease that remains refractory to therapy. Despite increasing evidence that protease-activated receptor 2 (PAR-2) contributes to fibrosis, its importance in pulmonary fibrosis is under debate. We addressed whether PAR-2

  5. The emerging mysteries of IgG4-related disease

    NARCIS (Netherlands)

    Smit, Wouter; Barnes, Eleanor

    2014-01-01

    IgG4-related disease (IgG4-RD) is increasingly recognised in Western societies as a multi-system, inflammatory, fibrosing disease of unknown aetiology that typically, though not exclusively, presents in older men. The clinical manifestations are diverse and almost any organ may be affected. The

  6. Case Presentation at a Clinico-Pathological Conference*

    African Journals Online (AJOL)

    of low-strength mannitoi to reduce possible cerebral oede- ma. His convulsions were ..... No vascular lesions were noted but a few degenerate muscle fibres were ... The liver necrosis appears to have been a terminal event. Cases of fibrosing ...

  7. Nephrogenic systemic fibrosis: risk factors suggested from Japanese published cases

    DEFF Research Database (Denmark)

    Tsushima, Y; Kanal, E; Thomsen, H S

    2010-01-01

    The aim of this article is to review the published cases of nephrogenic systemic fibrosis (NSF) in Japan. The Japanese medical literature database and MedLine were searched using the keywords NSF and nephrogenic fibrosing dermopathy (January 2000 to March 2009). Reports in peer-reviewed journals ...

  8. Case series

    African Journals Online (AJOL)

    abp

    1 oct. 2013 ... conduit, 23 patients ont eu une atteinte pulmonaire rhumatoïde à type de pneumonie infiltrante diffuse, de nodules rhumatoïdes pulmonaires ou de fibrose pulmonaire, 38 patients avaient un syndrome sec, dont 7 souffraient d'un goujerot sjogren retenu selon les critères de l'American European Consensus ...

  9. Dupuytren diathesis and genetic risk

    NARCIS (Netherlands)

    Dolmans, Guido H; de Bock, Geertruida H; Werker, Paul M

    2012-01-01

    PURPOSE: Dupuytren disease (DD) is a benign fibrosing disorder of the hand and fingers. Recently, we identified 9 single nucleotide polymorphisms (SNPs) associated with DD in a genome-wide association study. These SNPs can be used to calculate a genetic risk score for DD. The aim of this study was

  10. Potential novel targets: Protease-activated receptors in idiopathic pulmonary fibrosis

    NARCIS (Netherlands)

    Lin, C.

    2015-01-01

    Idiopathic pulmonary fibrosis (IPF) is the most devastating diffuse fibrosing lung disease of unknown etiology. IPF patients suffer from severe breathlessness caused by decreasing lung compliance eventually leading to respiratory failure and death. The prognosis of IPF is devastating: there is only

  11. Relationship between serum concetrations of type III procollagen, hyluronic acid and histopathological findings in the liver of HCV-positive blood donors Relação entre concentrações séricas de procolágeno tipo III, ácido hialurônico com achados histopatológicos do fígado em doadores de sangue anti-HCV positivos

    Directory of Open Access Journals (Sweden)

    Vera Regina Rodrigues Camacho

    2007-06-01

    Full Text Available BACKGROUND: Serologic markers have been proposed for monitoring hepatic fibrosis in chronic liver disease. Among fibrosis markers, type III procollagen (PIIIP and hyaluronic acid have been studied in these patients. AIM: To evaluate the association between these serum markers with histological findings. METHODS: A prospective cross-sectional study was carried out with HCV-positive blood donors. The studied population included men and women whose age ranged from 18 to 60 years, with elevated liver function tests [ALT levels > 1.5 times the normal value and alterations of two or more of the following: any changes in the levels of ALT, aspartate aminotransferase, conjugated bilirrubin, gammaglobulin, gammaglutamyltranspeptidase, albumin, platelet count; alkaline phosphatase levels >1.5 times the normal value, or prothrombin time below 70% and above 60%]. Fourty-nine patients were submitted to liver biopsy, blood analysis of PIIIP, hyaluronic acid, besides liver function tests. RESULTS: Liver function tests were not associated with tissular fibrosis, as assessed by ALT (>1.5 times above normal, fibrosis risk=18.8%; RACIONAL: Marcadores sorológicos têm sido propostos para monitorar fibrose hepática em doença crônica do fígado. Dentre os marcadores de fibrose, ácido hialurônico e procolágeno tipo III têm sido estudados nestes pacientes. OBJETIVO: Avaliar a associação de marcadores séricos de fibrose com achados histológicos. MÉTODOS: Foi realizado estudo transversal prospectivo em doadores de sangue anti-HCV positivos. A população estudada incluiu homens e mulheres com idade entre 18-60 anos com provas de função hepática alteradas (níveis de alanina aminotransferase >1.5 vezes do normal e alterações de dois ou mais dos seguintes: qualquer alteração nos níveis de alanina aminotransferase, aspartato aminotransferase, bilirrubina conjugada, gamaglobulina, gamaglutamiltranspeptidase, albumina, plaquetas, níveis de fosfatase

  12. Elevação dos níveis séricos de laminina na cirrose hepática induzida pelo tetracloreto de carbono

    Directory of Open Access Journals (Sweden)

    Neves Lindalva Batista

    2003-01-01

    Full Text Available RACIONAL: Laminina sérica tem sido correlacionada com a hipertensão portal e a capilarização dos sinusóides na doença hepática crônica. Pouco é conhecido sobre sua dinâmica nas doenças hepáticas. OBJETIVO: Estudar os níveis séricos de laminina e correlacioná-los com graus de fibrose hepática e hipertensão portal em ratos tratados com tetracloreto de carbono. MATERIAL E MÉTODO: Quarenta e nove ratos albinos Wistar foram mantidos como controles (n = 16 ou tratados com solução de tetracloreto de carbono a 8% (n = 23. Entre a 6ª e 16ª semana de experimento animais foram sacrificados após cateterização da veia porta e medida da pressão portal. Fragmentos de fígado foram fixados em formol para microscopia óptica. A fibrose hepática foi classificada em perivenular, septal incompleta e completa e cirrose. Concentração de laminina circulante foi determinada pela técnica de ensaio imunoenzimático com anticorpo desenvolvido a partir de laminina isolada de tumor Engelbreth-Holm-Swarm e purificado em coluna de afinidade. RESULTADOS: A pressão portal correlacionou-se com grau de fibrose hepática (r = 0,82; n = 45 e seus níveis nos grupos fibrose septal (10,8 ± 1,2 cm H2O e cirrose (13,6 + 3,1 cm H2O foram estatisticamente superiores aos observados no grupo-controle (7,9 + 1,5 cmH2O e no grupo com fibrose perivenular (9,1 + 0,8 cmH2O. A concentração de laminina no sangue periférico dos cirróticos (40,0 + 18,7 µg/dL foi significativamente superior aos grupos-controle (13,8 + 12,1 µg/dL, fibrose perivenular (19,1 + 15,5 e fibrose septal (22,2 + 27,0 µg/dL. Laminina circulante correlacionou-se de maneira significativa com o grau de fibrose hepática (r = 0,59; n = 49 e com os níveis de pressão portal (r = 0,29; n = 45. CONCLUSÕES: Na intoxicação crônica experimental pelo tetracloreto de carbono, níveis circulantes de laminina correlacionam-se mais com o grau de fibrose hepática do que com os níveis de hipertens

  13. Recent Developments in the Theory of Mechanisms in Radiation Chemical Processes; Developpements recents dans la theorie des mecanismes des reactions radiochimiques; Poslednie dostizheniya v teorii mekhanizmov radiatsionno-khimicheski kh protsessov; Adelantos recientes en la interpretacion teorica del mecanismo de los procesos radioquimicos

    Energy Technology Data Exchange (ETDEWEB)

    Roberts, R. [U.K.A.E.A., Wantage Research Laboratory, Berks (United Kingdom)

    1963-11-15

    Recent developments in the mechanisms of radiation-initiated chemical reactions are reviewed. The role of ion molecule processes is reviewed, with particular reference to the radiation chemistry of methane. In this system, the existence of reactions of excited molecules, in addition to ionic processes is deduced. It is shown that, in the radiolysis of methane, unsaturated hydrocarbons play a considerable part in the mechanism of reaction. Developments in ionic polymerization and also polymerization under heterogenous conditions are reviewed. The importance of reactant purity, and also cleanliness of reaction vessels is discussed. The effect of an applied external potential in solid state polymerization is briefly reviewed. The importance of free-radical processes to radiation chemists is considered in the light of the Dow process for the production of ethyl bromide. (author) [French] L'auteur examine les resultats recents obtenus dans l'etude des mecanismes des reactions chimiques radio-induites. Il etudie l'importance des reactions ion-molecule, en considerant tout particulierement la radio-chimie due methane. Il en deduit qu'il existe dans ce systeme, outre les phenomenes ioniques, des reactions de molecules excitees. il montre que les hydrocarbures non satures jouent un role considerable dans la radiolyse du methane. Il passe en revue les resultats obtenus dans la polymerisation ionique ainsi que dans la polymerisation dans des conditions heterogenes. Il insiste sur l'importance de la purete des reactifs ainsi que sur la proprete des recipients. En meme temps, il examine brievement l'effet d'un potentiel exterieur sur la polymerisation a l'etat solide. Il signale l'importance radiochimique des reactions fondees sur des radicaux liebre, libres sous l'angle du procede Dow pour la production de bromure d'ethyle. (author) [Spanish] El autor examina los recientes progresos realizados en el estudio de los mecanismos de las reacciones quimicas radioinducidas. Sedala la

  14. Utilisation de l'essai comete et du biomarqueur gamma-H2AX pour detecter les dommages induits a l'ADN cellulaire par le 5-bromodeoxyuridine post-irradiation

    Science.gov (United States)

    La Madeleine, Carole

    Ce memoire est presente a la Faculte de medecine et des sciences de la sante de l'Universite de Sherbrooke en vue de l'obtention du grade de maitre es sciences (M.Sc.) en radiobiologie (2009). Un jury a revise les informations contenues dans ce memoire. Il etait compose de professeurs de la Faculte de medecine et des sciences de la sante soit : Darel Hunting PhD, directeur de recherche (departement de medecine nucleaire et radiobiologie), Leon Sanche PhD, directeur de recherche (departement de medecine nucleaire et radiobiologie), Richard Wagner PhD, membre du programme (departement de medecine nucleaire et radiobiologie) et Guylain Boissonneault PhD, membre exterieur au programme (departement de biochimie). Le 5-bromodeoxyuridine (BrdU), un analogue halogene de la thymidine reconnu depuis les annees 60 comme etant un excellent radiosensibilisateur. L'hypothese la plus repandue au sujet de l'effet radio sensibilisant du BrdU est qu'il augmente le nombre de cassures simple et double brin lorsqu'il est incorpore dans l'ADN de la cellule et expose aux radiations ionisantes. Toutefois, de nouvelles recherches semblent remettre en question les observations precedentes. Ces dernieres etudes ont confirme que le BrdU est un bon radiosensibilisateur, car il augmente les dommages radio-induits dans l'ADN. Mais, c'est en etant incorpore dans une region simple brin que le BrdU radiosensibilise l'ADN. Ces recherches ont egalement revele pour la premiere fois un nouveau type de dommages produits lors de l'irradiation de l'ADN contenant du BrdU : les dimeres interbrins. Le but de ces travaux de recherche est de determiner si la presence de bromodeoxyuridine dans l'ADN augmente l'induction de bris simple et / ou double brin chez les cellules irradiees en utilisant de nouvelles techniques plus sensibles et specifiques que celles utilisees auparavant. Pour ce faire, les essais cometes et la detection des foci H2AX phosphorylee pourraient permettre d'etablir les effets engendres par

  15. Inertial currents in the Indian Ocean derived from satellite tracked surface drifters

    Digital Repository Service at National Institute of Oceanography (India)

    Saji, P.K.; Shenoi, S.S.C.; Almeida, A.M.; Rao, L.V.G.

    tropical. Largue´s a`15me`tres de profondeur, ces flotteurs pre´sentent une vitesse de de´rive infe´rieure a` 0,1 % de celle du vent. Le spectre de rotation des composantes de la vitesse superficielle souligne l’importance des courants d’inertie. Ceux...-ci sont circulaires (coefficient de rotation \\0.5), tre`s intermittents et ils contribuent environ a` 46 % de l’e´nergie cine´tique totale du champ de courants superficiels. Les e´ve´nements de l’activite´ inertielle, qu’ils soient provoque´s par le...

  16. La représentation de la sexualité dans la littérature féminine du nouveau millénaire : le cas de Catherine Millet

    OpenAIRE

    Nathalie Dumas

    2010-01-01

    Frédéric Beigbeder, dans son dernier roman intitulé Windows on the World, en vient à la conclusion que, de nos jours, le Play-boy international n’est en fait plus qu’un célibataire en quête de jouissance, ne chassant que la femme superficielle ou la prostituée. Selon l’auteur, aujourd’hui le Play-boy international est une femme. Une Bridget Jones ou une Carrie Bradschaw de Sex and the City (Beigbeder, 178 et 231). Le type de femme qui fait peur, qui assume et expose sa féminité et son indépen...

  17. Alexandre de Humboldt et le Marquis de Laplace

    OpenAIRE

    Eberhard Knobloch

    2014-01-01

    Pierre-Simon Marquis de Laplace joua un rôle éminent dans la vie scientifique d’Alexandre de Humboldt. Humboldt avait fait la connaissance du savant français qui avait vingt ans de plus que lui-même à Paris en 1798. Si l’on n’étudie pas seulement leurs œuvres, mais aussi leurs correspondances et le journal américain d’Humboldt, on en conclut qu’il faut réviser l’impression superficielle qui se dégage des publications d’Humboldt. L’estime réciproque entre les deux hommes ne se mit en place que...

  18. Clinical and histological findings in nephrogenic systemic fibrosis

    International Nuclear Information System (INIS)

    Cowper, Shawn E.; Rabach, Morgan; Girardi, Michael

    2008-01-01

    Nephrogenic systemic fibrosis (NSF) is a relative newcomer to the world of medicine. NSF was introduced just over 10 years ago as nephrogenic fibrosing dermopathy, but with further investigation, its systemic nature was determined. The strict adherence to a definition requiring both clinical and pathological concordance has allowed for careful separation of this entity from other fibrosing disorders, leading eventually to the realization that gadolinium-based contrast agents were closely associated with its onset. As planned prospective studies get underway, it is of paramount importance that researchers and clinicians realize that NSF remains a very challenging diagnosis, and that both clinical and histopathological criteria must be employed to reach the most accurate diagnosis possible

  19. Nature et évolution de la microflore du bois de chêne au cours de son séchage et de son vieillissement a l'air libre

    Directory of Open Access Journals (Sweden)

    Pascal Chatonnet

    1994-09-01

    Full Text Available Pendant le séchage naturel et le vieillissement à l'air libre du bois de chêne débité en merrain, on assiste à l'envahissement progressif du bois par différents micro-organismes. On a identifié dans ce travail 19 genres et 27 espèces de champignons, 2 genres et au moins 4 espèces de bactéries. La très grande majorité des micro-organismes est localisée dans les couches superficielles du bois. Seuls certains champignons sont retrouvés au coeur du merrain ayant vieilli 5 ans et plus sur parc. La déshydratation rapide du bois semble limiter le développement et l'envahissement en profondeur par les moisissures. Un champignon basidiomycète à arthroconidies, non formellement identifié, qui produit une pourriture blanche, a été isolé dans des merrains stockés 10 ans à l'air libre. Excepté ce champignon particulier, qui grâce à un équipement enzymatique adapté est susceptible d'attaquer simultanément tous les constituants pariétaux, les autres micro-organismes identifiés sont essentiellement cellulolytiques et ne peuvent pas altérer profondément la composition ni la structure du bois. En revanche, ils devraient pouvoir augmenter notablement la perméabilité et l'accessiblité aux liquides des couches superficielles du merrain et faciliter l'implantation ultérieure de basidiomycètes lignolytiques.

  20. Imaging appearances of synovial plicae syndrome of the knee

    OpenAIRE

    Osama Abdalla Mabrouk Kheiralla

    2016-01-01

    Synovial plicae are synovial folds that may be found as intraarticular structures within the knee joint. They are remnants of incomplete resorption of mesenchymal tissue during fetal development. Synovial plicae, if present, are supposed to be non-pathological and asymptomatic, however if they are exposed to special events like direct trauma or repeated activities, they may be inflamed and become fibrosed and rigid and irritates the synovium of the underlying femoral condyle resul...

  1. Paracoccidioidomycosis: high-resolution computed tomography - anatomo-pathological correlation; Paracoccidioidomicose: correlacao da tomografia computadorizada de alta resolucao com a anatomopatologia

    Energy Technology Data Exchange (ETDEWEB)

    Marchiori, Edson; Muniz, Maria Angelica Soares; Santos, Maria Lucia de Oliveira [Universidade Federal Fluminense, Niteroi, RJ (Brazil). Inst. de Radiologia; Moraes, Heleno Pinto de [Universidade Federal Fluminense, Niteroi, RJ (Brazil). Inst. de Patologia; Capone, Domenico [Universidade do Estado, Rio de Janeiro, RJ (Brazil). Inst. de Pneumologia

    2000-12-01

    We reviewed the high-resolution computed tomography scans of 13 patients with paracoccidioidomycosis and correlated the findings with the anatomo-pathological findings of 5 patients. The most frequent findings observed were thickening of the interlobular septa, emphysema, ground glass areas, thickening of bronchial walls, tracheal dilatation, nodules, cavities and evidence of fibrosing disease such as architectural distortion, parenchymatous bands, spicular pleural thickening, intralobular reticulate and thickening with distortion off the axial interstitium. (author)

  2. Research

    African Journals Online (AJOL)

    ebutamanya

    15 mars 2016 ... Selon les marqueurs évolutifs, 112 patients (78,3%) avaient un AgHBe négatif. Quant à la charge virale, 106 patients (74,2%) avaient une virémie inférieure à 2000UI/ml et une fibrose minime inférieure à 7kpa selon le FibroScan. Parmi eux, 13 malades avaient un ADN du VHB indétectable (<20UI/ml).

  3. Scleroderma Mimickers

    Science.gov (United States)

    Morgan, Nadia D.; Hummers, Laura K.

    2017-01-01

    Opinion statement Cutaneous fibrosing disorders encompass a diverse array of diseases united by the presence of varying degrees of dermal sclerosis. The quality and distribution of skin involvement, presence or absence of systemic complications and unique associated laboratory abnormalities often help to distinguish between these diseases. It is imperative that an effort is made to accurately differentiate between scleroderma and its mimics, in order to guide long-term management and facilitate implementation of the appropriate treatment modality where indicated. PMID:28473954

  4. The diffuse interstitial lung disease - with emphasis in the idiopathic interstitial pneumonias

    International Nuclear Information System (INIS)

    Bustillo P, Jose G; Pacheco, Pedro M; Matiz, Carlos; Ojeda, Paulina; Carrillo B, Jorge A.

    2003-01-01

    The term diffuse interstitial lung disease, it refers to those diseases that commit the interstice basically, the space between the membrane basal epithelial and endothelial, although the damage can also commit the outlying air spaces and the vessels; the supplement is centered in the diffuse interstitial lung illness of unknown cause; well-known as idiopathic interstitial pneumonias, making emphasis in the more frequents, the pulmonary fibrosis idiopathic or cryptogenic fibrosant alveolitis

  5. Morphea: Evidence-based recommendations for treatment

    OpenAIRE

    Nicole M Fett

    2012-01-01

    Morphea is a rare fibrosing disorder of the skin. Evidence-based treatment strategies in morphea are lacking. This review summarizes the available data on morphea treatment and provides therapeutic strategies based on morphea subtypes. The Cochrane Library, Medline and Embase from inception until May of 2011 were searched using the key words "morphea" and "morphea treatment." Reference lists of the resultant articles, as well as relevant reviews, were also searched. This review focuses on ran...

  6. Coexisting Morphea and Bullous Lichen Sclerosus Et Atrophicus: A Case Report

    OpenAIRE

    Nurcan Metin; Mahizer Yaldız; Teoman Erdem; Şahin Erdem

    2015-01-01

    Morphea and lichen skleroatrofikans (LSA)are localized fibrosing diseases characterized by well-defined fibrotic plaques. Blisters rarely occur on morphea plaque, and these blisters are mostly considered as bullous morphea which is a rare type of morphea. The cases of bullous LSA growing on plaque morphea are rarer. In this case report, we presented a patient has bullous lesions compatible with of lichen skleroatrofikans clinically and histopathologically on morphea lesions, to draw attention...

  7. Einfluss von Hypothyreose und Mukoviszidose auf das Ruhe- und Belastungs-EKG

    OpenAIRE

    Gerlach, Julia

    2009-01-01

    Bei Menschen, die an cystischer Fibrose oder auch an einer Hypothyreose erkrankt sind, ist die Beteiligung des Herz-Kreislauf-Systems eine bekannte Komplikation. Ziel dieser Arbeit war, möglicherweise bislang unbekannte Auswirkungen dieser Erkrankungen auf das Belastungs-EKG von Kindern und Jugendlichen zu identifizieren und ggf. zu deuten. Dadurch sollte die Beurteilbarkeit des Belastungs-EKGs bei der Untersuchung der genannten Patientengruppen verbessert werden. Es wurden insgesamt 64 Patie...

  8. Inflammatory Pseudotumor of the Pituitary Gland Mimicking a Pituitary Adenoma: A Case Report

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Da Mi; Yu, In Kyu [Dept. of Radiology, Eulji University Hospital, Daejeon (Korea, Republic of); Kim, Han Kyu [Dept. of Neurosurgery, Eulji University Hospital, Daejeon (Korea, Republic of); Son Hyun Jin [Dept. of Pathology, Eulji University Hospital, Daejeon (Korea, Republic of)

    2011-04-15

    A 38-year-old man was admitted to our hospital with diplopia. The patient had a relatively well-defined pituitary mass with high cellularity as well as weaker enhancement on imaging modalities including computed tomography (CT) and magnetic resonance imaging (MRI), than a typical pituitary adenoma. The distinction between a pseudotumor and an invasive neoplasm is very difficult before biopsy. In this case report, we discuss the characteristic imaging features of a fibrosing inflammatory pseudotumor of the pituitary gland

  9. Riedel thyroiditis: Fine needle aspiration findings of a rare entity.

    Science.gov (United States)

    Weidner, Anna-Sophie; Molina, David; DeSimone, Robert A; Cohen, Marc A; Giorgadze, Tamar; Scognamiglio, Theresa; Hoda, Rana S

    2015-09-01

    Riedel thyroiditis is a rare fibrosing disorder characterized by extension of the fibroinflammatory process beyond the thyroid capsule. Due to the nature of this lesion, fine-needle aspiration often yields scant material and may be interpreted as non-diagnostic. In this report, we describe cytologic features that allow the cytopathologist to favor a diagnosis of Riedel thyroiditis, thereby guiding appropriate further work-up and management. © 2015 Wiley Periodicals, Inc.

  10. PROGRESSION OF LIVER FIBROSIS IN MONOINFECTED PATIENTS BY HEPATITIS C VIRUS AND COINFECTED BY HCV AND HUMAN IMMUNODEFICIENCY VIRUS

    Directory of Open Access Journals (Sweden)

    Cristiane Valle TOVO

    2013-03-01

    Full Text Available Context The progression of liver fibrosis in patients coinfected by hepatitis C virus and human immunodeficiency virus (HCV/HIV has been increasingly studied in the past decade. Studies made before the highly active antiretroviral therapy suggest that HIV can change the natural history of the HCV infection, leading to a faster progression of the liver fibrosis. Objective To evaluate and compare the fibrosis progression in two groups of patients (HCV/HIV coinfected and HCV monoinfected Methods Seventy patients HCV monoinfected and 26 patients HCV/HIV coinfected who had not undertaken HCV treatment and were submitted to serial percutaneous liver biopsies were retrospectively evaluated. There was no difference in the fibrosis progression between the two groups. Conclusion The fibrosis grade evolution was not worse in the coinfected patients. The immunosuppression absence and the shortest time period between the biopsies in the coinfected group are possible explanations. Contexto A progressão da fibrose hepática em pacientes coinfectados pelos vírus da hepatite C (VHC e da imunodeficiência humana (VHC/HIV tem sido mais estudada na última década. Estudos realizados antes da terapia antiretroviral de alta potência (HAART sugerem que o HIV pode mudar a história natural da infecção pelo VHC, levando a uma progressão mais rápida da fibrose hepática. Objetivo Avaliar e comparar a progressão de fibrose em duas populações de pacientes (coinfectados VHC/HIV e monoinfectados VHC. Métodos Foram avaliados retrospectivamente 70 pacientes monoinfectados VHC e 26 coinfectados VHC/HIV nunca tratados para o VHC e que haviam realizado duas biopsias hepáticas seriadas. Não houve diferença na progressão de fibrose entre os dois grupos. Conclusão A evolução do grau de fibrose não foi pior nos pacientes coinfectados. A ausência de imunodepressão e o menor intervalo de tempo entre as biopsias no grupo de coinfectados são poss

  11. Hipertensão arterial pulmonar: uso do realce tardio miocárdico pela ressonância magnética cardíaca na avaliação de risco

    Directory of Open Access Journals (Sweden)

    Luiz Gustavo Pignataro Bessa

    2013-10-01

    Full Text Available FUNDAMENTO: A hipertensão arterial pulmonar é uma doença grave e progressiva. O maior desafio clínico é seu diagnóstico precoce. OBJETIVO: Avaliar a presença e a extensão do realce tardio miocárdico pela ressonância magnética cardíaca bem como verificar se o percentual da massa de fibrose miocárdica é indicador de gravidade. MÉTODOS: Estudo transversal com 30 pacientes com hipertensão arterial pulmonar dos grupos I e IV, submetidos às avaliações clínica, funcional e hemodinâmica, e à ressonância magnética cardíaca. RESULTADOS: A média de idade dos pacientes foi de 52 anos, com predomínio do gênero feminino (77%. Dentre os pacientes, 53% apresentavam insuficiência ventricular direita ao diagnóstico, e 90% encontravam-se em classe funcional II/III. A média do teste de caminhada de 6 minutos foi de 395 m. No estudo hemodinâmico com o cateterismo direito, a média da pressão arterial pulmonar foi de 53,3 mmHg, do índice cardíaco de 2,1 L/min.m², e a mediana da pressão atrial direita foi de 13,5 mmHg. Realce tardio do miocárdio pela ressonância magnética cardíaca foi encontrado em 28 pacientes. A mediana da massa de fibrose foi 9,9 g e do percentual da massa de fibrose de 6,17%. A presença de classe funcional IV, insuficiência ventricular direita ao diagnóstico, teste de caminhada de 6 minutos 15 mmHg, com índice cardíaco < 2,0 L/min.m², teve associação significativa com maior percentual de fibrose miocárdica. CONCLUSÃO: O percentual da massa de fibrose miocárdica mostra-se um marcador não invasivo com perspectivas promissoras na identificação do paciente portador de hipertensão pulmonar com fatores de alto risco.

  12. New advances in the therapy of non-cystic fibrosis bronchiectasis

    Directory of Open Access Journals (Sweden)

    A. Amorim

    2013-11-01

    Full Text Available Non-cystic fibrosis bronchiectasis remains a common and important respiratory disease to date. It is a chronic pathology and consequently the patients usually require continuous treatment.In recent decades therapies that do not have scientific evidence of their benefits have been commonly used in non-cystic fibrosis bronchiectasis. Cystic fibrosis has provided the experience to extrapolate therapeutic approaches to other bronchiectasis patients. Finally, in the last few years some trials have been carried out specifically in non-cystic fibrosis bronchiectasis which aim to assess the efficacy of some of the treatments which are commonly used but sometimes without clear indication.This review will discuss the recent results from these trials, namely mucoactive, anti-inflammatory and antibiotic therapy. Several trials are ongoing and we hope they will be able to add clarification to the management of these patients. Resumo: As bronquiectasias não-fibrose quística continuam a ser uma doença respiratória comum e importante. Trata-se de uma patologia crónica e, consequentemente, os doentes geralmente precisam de um tratamento contínuo.Nas últimas décadas, tratamentos sem evidência científica dos seus benefícios foram comumente usadas nas bronquiectasias não-fibrose quística. A fibrose quística serviu de experiência para extrapolar a abordagem terapêutica para outros doentes com bronquiectasias. Finalmente, nos últimos anos, foram realizados alguns ensaios bronquiectasias não-fibrose quística que visam avaliar a eficácia de alguns dos tratamentos que são comummente usados mas por vezes sem uma clara indicação.Nesta revisão serão apresentados os resultados recentes destes ensaios, nomeadamente sobre o tratamento mucoactivo, anti-inflamatório e antibiótico. Diversos estudos estão a decorrer e esperamos que estes venham a esclarecer a abordagem mais adequada destes doentes. Keywords: Non-cystic fibrosis bronchiectasis, Advances

  13. Doença intersticial pulmonar em doentes com artrite reumatóide: comparação com a alveolite fibrosante criptogénica

    Directory of Open Access Journals (Sweden)

    B.A. Rajasekaran

    2002-09-01

    Full Text Available RESUMO: Os autores efectuaram um estudo prospectivo longitudinal com o objectivo de comparar a história natural da alveolite fibrosante criptogénica (AFC com a doença do interstício pulmonar (DIP relacionada com a artrite reumatóide (AR.Seleccionaram 1 grupo de 18 doentes com AFC e um grupo de 18 doentes com AR e doença do interstício pulmonar. Ambos os grupos apresentavam idade, sexo e duração da doença semelhantes, tendo todos os doentes sido submetidos a uma avaliação clínica, estudo funcional respiratório e TAC torácica de alta resolução.Os resultados revelaram que, em termos clínicos, a dispneia apresentava uma evolução média de 30 meses até ao diagnóstico nos 2 grupos, sendo mais frequente o hipocratismo digital nos doentes apenas com AFC. A presença de Factor Reumatóide foi superior nos doentes com AR e, segundo estes autores, estava relacionada com um pior prognóstico a nível articular mas parecia ser um factor protector contra a fibrose pulmonar neste grupo.A nível de estudo funcional respiratório, os resultados foram praticamente sobreponíveis quer a nível do VEMS, capacidade vital e capacidade de difusão (DLCO nos 2 grupos de doentes.Radiologicamente, nos doentes com artrite reumatóide, a TAC revelou uma maior percentagem de alveolite (vidro despolido em 4 casos, tendo os restantes 14 doentes fibrose pulmonar estabelecida, tal como no outro grupo estudado. Os doentes com AR apresentavam ainda uma distribuição mais periférica das lesões pulmonares, ao contrário dos outros doentes, em que predominavam as alterações basais.Uma diminuição mais acentuada da DLCO correlacionou-se com a existência de fibrose em favo na TAC em ambos os grupos. COMENTÁRIO: A alveolite fibrosante criptogénica, sinónimo de fibrose pulmonar idiopática, possui um mau prognóstico, com uma sobrevida média aos 5 anos após o diagnóstico de cerca de 50%, e aos 10 anos de aproximadamente 20%.A incidência de doença do

  14. Effets de l'interaction avec l'oxygène sur le comportement de couches semi-conductrices de ZnO, SnO{2} et CdSe

    Science.gov (United States)

    Ain-Souya, A.; Ghers, M.; Haddad, A.; Tebib, W.; Rehamnia, R.; Messsalhi, A.; Bounouala, M.; Djouama, M. C.

    2005-05-01

    Les propriétés superficielles des matériaux solides diffèrent de celles du volume. A la surface, des défauts de différentes natures peuvent être présents. Ils permettent à la surface d'être interactive avec le milieu ambiant. Les multiples interactions entre les états de surface et des éléments du milieu extérieur peuvent modifier les propriétés superficielles. Ce travail étudie la régénération de couches semi-conductrices après adsorption isotherme d'oxygène à différentes températures effectuées entre 20 ° C et 300 ° C. Les matériaux qui ont servi à l'étude sont des couches de ZnO, SnO{2} et CdSe. Celles de CdSe ont été obtenues par co-évaporation, sous vide, de cadmium et de sélénium. Les échantillons de ZnO et SnO{2} ont été élaborés par oxydation, à des températures respectives de 450 ° C et 200 ° , de Zn et Sn déposés par électrolyse et par évaporation sous vide. Les matériaux évaporés ont été déposés sur des plaquettes en verre, les autres ont été électrodéposés sur des substrats métalliques. Les variations des propriétés électriques des couches ont été suivies par mesure de leur résistance électrique superficielle R. Les courbes LogR = f (103 /T (K)), relevées sous vide à différentes températures, sont caractéristiques d'un comportement de semi-conducteur. Des essais d'adsorption d'O{2} à différentes températures montrent des variations considérables de R. En effet, la chimisorption forte d'un gaz par une surface semi-conductrice est telle que l'échange électronique entre adsorbant et adsorbat provoque la formation d'une zone de charge d'espace modifiant la conduction superficielle. Les résultats mettent en évidence des domaines de température de plus haute sensibilité à l'oxygène. Pour le CdSe, certaines désorptions isothermes ont été suffisantes pour une régénération totale des échantillons. Les couches de ZnO ont souvent nécessité des désorptions programm

  15. A utilização da Pfaffia glomerata no processo de cicatrização de feridas da pele Pfaffia glomerata in the process of healing of skin wounds

    Directory of Open Access Journals (Sweden)

    Marileide Inacio da Silva

    2010-12-01

    Full Text Available RACIONAL: Quando ocorre perda tecidual na pele surge a necessidade de reconstituir-se o tecido lesionado e atualmente cada vez mais surgem artifícios que se propõem neoformá-lo. O uso de fitoterápicos, como a Pfaffia glomerata, tem a finalidade de buscar nestes produtos princípios ativos que desempenhem efetivo papel no processo de cicatrização. OBJETIVO: Avaliar os resultados da utilização da Pfaffia glomerata na cicatrização de feridas cirúrgicas em ratos. MÉTODOS: Foram utilizados 40 ratos, nos quais realizaram-se feridas cirúrgicas com punch de 3 mm de diâmetro no dorso da parte superior direita onde nenhuma substância foi aplicada e nesses mesmos animais foram também realizadas feridas cirúrgicas na região inferior, onde foi aplicado o extrato do fitoterápico. Foram divididos em quatro subgrupos de 48 horas, uma, duas e três semanas em relação ao sacrifício. Foram tomadas as medidas na circunferência para analisar a contração da ferida macroscopicamente. Microscopicamente os resultados foram analisados utilizando-se a coloração de Hematoxilina-Eosina, para verificar o processo inflamatório; imunohistoquímica, fator VIII, para observar a densidade vascular; e tricrômio de Masson para estudar a fibrose. RESULTADOS: Macroscopicamente o grupo planta obteve resultados superiores ao grupo controle. A análise da variável fator VIII mostrou significância estatística no grupo de uma semana do fitoterápico. Na variável fibrose, constatou-se que no período de 48 horas o grupo controle apresentou 70% de casos com fibrose mínima, ao passo que o da planta, 90%. Em uma semana, o grupo controle apresentou 10% de casos com ausência de fibrose , 60% com fibrose mínima e 30% com fibrose moderada, enquanto que o grupo planta apresentou 70% de casos com fibrose mínima e 30% com fibrose moderada. Já no período de duas semanas, o grupo controle manteve 60% dos casos com fibrose mínima e aumentou para 40% os com

  16. Pengaruh Pemberian Valsartan Dan Kurkumin Terhadap Pembentukan Fibrosis Di Tubulus Proksimal Ginjal Akibat Obstruksi Ureter Unilateral pada Tikus Wistar.

    Directory of Open Access Journals (Sweden)

    Lubis M

    2013-01-01

    . Perbedaan bermakna terbentuKata kunci: Obstruksi ureter, Valsartan, Kurkumin, Fibrosis, Degenerasi hidrofilik, AtrofiAbstractIntroduction: ureter obstruction is a condition where is an obstacle for urine flow from renal to blast (vesica urinaria. The obstruction in ureter will decrease glomerulus filtration flow and it destroys renal parenchym. Fibroses in obstructed renal present through two mediators, there are necrotizing tumor factors-α (TNF-α and angiotension-II. Obstruction of this two mediators will decrease fibroses grading in proximal tubules of renal caused by obtruction. One of TNF-α inhibitors is curcumene and angiotension-II will be obstructed by valsartan. Methods: this experiment is kind of experimental type using animal experiment (Wistar Mice. Wistar Mice are divided into two groups, each group consist of 15 mice, so the total are 30 mice. This animals tighted with at proximal ureter The first group is control one, given valsartan. The second group is given valsartan and curcumene. Oral route and dilution before given. Medicine is given use 1 cc spuit. Giving action in 14 days. The fifteenth day, we take renal of Wistar and do histology examination. Significant difference between fibroses forming in proximal tubulus analyzed by Chi Square Statistic Test with correction of Yates and T-Test, beside that, hydofic degeneration and atrophy in proximal tubulus analyzed by T-Test Statistic Test. Result: there is significant difference in forming of fibroses in proximal tubules of renal between action group and controlled group (Chi Square with p ≤ 0.0001 and T-Test with p ≤ 0.000. In hydrophilic degeneration forming in proximal tubules gotten significant difference between two groups ( T-Test with p ≤ 0.000. In atrophy forming in proximal tubules, there is important difference between two groups (T-Test with p ≤ 0.000.Concultion. There is an effect in giving valsartan and curcumene to fibroses forming in proximal tubules of renal. There is significant

  17. Miopatia por propoxifeno: relato de 2 casos com histoquimica de músculos

    Directory of Open Access Journals (Sweden)

    Lineu Cesar Werneck

    1981-03-01

    Full Text Available São relatados os casos de dois pacientes sofredores de dores crônicas (um com gota e outro com lombalgia, que desenvolveram o vício pelo dextro-propo-xifeno, administrado por via intramuscular. Isto determinou alterações importantes na musculatura onde eram aplicado as injeções, com endurecimento da pele, edema e diminuição da mobilidade do membro afetado. A investigação revelou miopatia crônica, com intensa fibrose do tecido conjuntivo perimisial e endomisial, com infiltração por células linfomonocitárias; havia também atrofia de fibras musculares nas regiões próximas e fibrose com atividade muito aumentada para a fosfatase alcalina no local das lesões. Eletromiografia em um dos casos sugeria denervação e, no outro, envolvimento muscular primário. A retirada da medicação determinou regressão dos sintomas e sinais, mas o retorno de ambos os pacientes ao hábito, fêz recrudescer o processo. São discutidos os achados clínico-patológicos comparando com outras medicação e agressões físicas que acometendo o tecido muscular, acabam levando o musculo à fibrose. Possivelmente as injeções intramusculares repetidas, a irritação pelo cloridrato de dextro-propoxifeno e a redução da drenagem linfática sejam as causas dos sintomas e alterações patológicas.

  18. O Projeto Queixadinha: a morbidade e o controle da esquistossomose em área endêmica no nordeste de Minas Gerais, Brasil

    Directory of Open Access Journals (Sweden)

    José Roberto Lambertucci

    1996-04-01

    Full Text Available Nos últimos cinco anos, em uma área endêmica para esquistossomose no nordeste de Minas Gerais, 561 indivíduos submeteram-se a exames clínico, laboratoriais, ultra-sonografia abdominal e dopplerecocardtografia visando definir a morbidade da doença antes e após o tratamento. Revelaram-se altas a prevalência de esquistossomose (66,3% e de formas graves (9,5% com baço palpável. A prevalência de indivíduos sem fibrose hepática e com fibrose teve, moderada e intensa ao ultra-som foi de 46,0%, 19,6%, 27,6% e 6,8%, respectivamente. Vinte um (39,6%: de 53 indivíduos com baço palpável não apresentavam fibrose periportal ao ultrasom. Linfonodos periportais foram identificados em 33,8% dos indivíduos examinados e anticorpos anti-KLH no soro de 40,7%,. Observaram-se alterações urinárias compatíveis com glomerulopatia esquistossamótica em 4,5% da população e 11,7% apresentavam achados dopplerecocardiográficos de hipertensão pulmonar. Doze meses após o tratamento da esquistossomose, a prevalência da doença reduziu-se de 66,3% para 25,0%. Em Queixadinha, um perfil da morbidade da doença e de sua evolução após o tratamento começa a ser delineado.

  19. Influence des défauts de la structure du verre sur la résistance mécanique des fibres optiques

    Science.gov (United States)

    Chmel, A.; Baptizmanski, V. V.; Kharshak, A. A.

    1992-12-01

    For silica (pure and doped) optical fibers prepared from preforms irradiated by thermal neutrons and Ar^+ ions, the measurements of lifetime under transverse stress were carried out. It is found that the neutron bombardment leads to decrease of the lifetime and the strength of fiber while the ion implantation results in increasing of these parameters. The influence of particle irradiation on the glass strength is explained by the generation of structural defects which were observed by the infrared an Raman spectroscopy methods. The direction of the change of mechanical properties of fibers is determined by the type of defects and their distribution in the cross-section of fiber. Des préformes de fibres optiques de silice ont été exposées à un bombardement par des faisceaux de neutrons thermiques et d'ions Ar^+ à énergie de 40 keV afin d'engendrer des défauts de la structure respectivement dans le volume ou dans la couche superficielle des échantillons. Les fibres obtenues des préformes irradiées et non irradiées ont été mises en charge de flexion. On a déterminé leur temps de rupture sous diverses contraintes de traction sur la surface extérieure de la fibre en flexion. On a observé une diminution du temps de rupture des fibres après le bombardement neutronique et une augmentation après l'implantation ionique. L'analyse de la nature des défauts en faisant appel à la spectroscopie infrarouge et Raman a fait ressortir que l'irradiation aux ions entraînait essentiellement des liaisons chimiques dans une fine couche superficielle de l'échantillon alors que l'irradiation aux neutrons occasionnait une déformation des zones du réseau silicique dans toute la section de la préforme et de la fibre. L'amélioration de la résistance mécanique s'explique par une augmentation de la mobilité des éléments structuraux de la matrice de verre par suite d'une rupture partielle des liaisons chimiques et sa diminution par l'apparition de concentrateurs

  20. Contribution to the study of the oxidation of cobalt and its protoxide in air at high temperatures; Contribution a l'etude de l'oxydation du cobalt et de son protoxyde dans l'air aux temperatures elevees

    Energy Technology Data Exchange (ETDEWEB)

    Vallee, M G [Commissariat a l' Energie Atomique, Saclay (France). Centre d' Etudes Nucleaires

    1964-01-15

    The surface oxidation process of cobalt in air follows, a parabolic law and is characterized between 750 and 1350 deg. C by an activation energy of 41,000 cals/mole. Between 400 and 900 deg. C the oxide film is made up of two layers: CoO next to the metal and Co{sub 3}O{sub 4} on the surface. Above 900 deg. C only CoO remains. The morphological properties of these films have been studied; growth anisotropy, crystallization facies, oxide grain growth, texture, nucleation of Co{sub 3}O{sub 4} on a CoO base round about 900 deg. C. The oxidation of cobalt protoxide between 700 and 910 deg. C results in the building up, on the outside surface of CoO discs, of a usually continuous layer of Co{sub 3}O{sub 4}. Under certain conditions this surface reaction is accompanied by a reaction along the longitudinal symmetry plane of the sample where a layer of Co{sub 3}O{sub 4} of very irregular thickness builds up. (author) [French] Le processus d'oxydation superficielle du cobalt dans l'air obeit a une loi parabolique et peut etre caracterise, entre 750 et 1350 deg. C, par une energie d'activation de 41 000 calorie s /mole. Entre 400 et 900 deg. C, la pellicule d'oxyde est constituee de deux couches superposees de CoO, au contact du metal et de Co{sub 3}O{sub 4} en surface. Au-dessus de 900 deg. C, seul subsiste CoO. On a etudie les proprietes morphologiques de ces pellicules: anisotropie de croissance, facies de cristallisation, croissance des grains d'oxyde, texture, germination de Co{sub 3}O{sub 4} sur un support de CoO au voisinage de 900 deg. C. L'oxydation du protoxyde de cobalt entre 700 et 910 deg. C se traduit par l'edification, sur la surface exterieure des plaquettes de CoO, d'une couche en general continue de Co{sub 3}O{sub 4}. Dans certaines conditions, a cette reaction superficielle se superpose parfois une reaction suivant le plan de symetrie longitudinal de l'echantillon ou s'edifie alors une couche de Co{sub 3}O{sub 4} d'epaisseur tres irreguliere. (auteur)

  1. Study of the effect of positive ions impinging sensitive emulsions in mass spectrography; Etude de l'action des ions positifs sur les emulsions sensibles en spectrographie de masse

    Energy Technology Data Exchange (ETDEWEB)

    Cavard, A [Commissariat a l' Energie Atomique, Grenoble (France). Centre d' Etudes Nucleaires

    1969-08-01

    Experimental relationships have been established between the blackening of emulsions by impinging ions and the following parameters: number, mass and energy of impinging particles. Mean energy ions (about twenty keV) give rise to a latent image probably made of small specks of metallic silver located at the surface or in the bulk of the silver halide grain. A specific developer for ion sensitive emulsion was perfected. Sensitivity and detection threshold are increased by a factor of two or three, compared with values observed using a classical developer. Low energy particles sputtered from superficial layers of the emulsion by the impinging twenty keV ions, produce a latent image close to the surface of the silver halide grain. An oxidizing process bleaches superficial latent image and then reduces background fog. The improved signal over background ratio allows to observe lines undetectable when the plates are developed by usual process. (author) [French] On a etabli les relations experimentales entre le noircissement resultant de l'action des ions sur l'emulsion sensible et les parametres suivants: nombre de particules incidentes, masse et energie de ces particules. L'effet sur les grains d'halogenure d'argent a ete examine: les ions d'energie moyenne (de l'ordre de 20 keV) creent une image latente vraisemblablement constituee d'argent, situee a la fois a la surface et a l'interieur du grain. Un revelateur specifique pour le developpement des emulsions sensibles exposees aux ions a ete mis au point: il accroit la sensibilite d'un facteur deux a trois et le facteur de contraste par rapport a un revelateur usuel. Les particules de faible energie, emises par pulverisation des couches superficielles de l'emulsion au cours de l'enregistrement des spectres de masse des ions d'energie moyenne, creent essentiellement dans les grains d'halogenure d'argent, une image latente superficielle, responsable du fond. Un traitement oxydant est propose pour eliminer cette image en

  2. Nephrogenic systemic fibrosis: risk factors suggested from Japanese published cases

    DEFF Research Database (Denmark)

    Tsushima, Y; Kanal, E; Thomsen, H S

    2010-01-01

    The aim of this article is to review the published cases of nephrogenic systemic fibrosis (NSF) in Japan. The Japanese medical literature database and MedLine were searched using the keywords NSF and nephrogenic fibrosing dermopathy (January 2000 to March 2009). Reports in peer-reviewed journals...... and meeting abstracts were included, and cases with biopsy confirmation were selected. 14 biopsy-verified NSF cases were found. In seven of eight patients reported after the association between gadolinium-based contrast agent (GBCA) and NSF was proposed, GBCA administration was documented: five received only...

  3. Realce Tardio miocárdico por Ressonância Magnética Cardíaca pode identificar risco para Taquicardia Ventricular na Cardiopatia Chagásica Crônica

    OpenAIRE

    Ronaldo Peixoto de Mello; Gilberto Szarf; Paulo Roberto Schvartzman; Edson Minoru Nakano; Mariano Martinez Espinosa; Denis Szejnfeld; Verônica Fernandes; João A. C. Lima; Claudio Cirenza; Angelo A.V. De Paola

    2012-01-01

    FUNDAMENTO: Testes invasivos e não invasivos têm sido usados para identificar risco para Taquicardia Ventricular (TV) em pacientes com Cardiopatia Chagásica Crônica (CCC). Ressonância Magnética Cardíaca (RMC) pela técnica do Realce Tardio (RT) pode ser útil para selecionar pacientes com disfunção ventricular global ou segmentar, com alto grau de fibrose e maior risco para TV clínica. OBJETIVO: Melhorar a identificação de elementos preditivos de TV em pacientes com CCC. MÉTODO: Quarenta e um p...

  4. Comorbidities in interstitial lung diseases

    Directory of Open Access Journals (Sweden)

    George A. Margaritopoulos

    2017-01-01

    Full Text Available Fibrosing lung disorders include a large number of diseases with diverse behaviour. Patients can die because of the progression of their illness, remain stable or even improve after appropriate treatment has been instituted. Comorbidities, such as acute and chronic infection, gastro-oesophageal reflux, pulmonary hypertension, lung cancer, cardiovascular diseases, and obstructive sleep apnoea, can pre-exist or develop at any time during the course of the disease and, if unidentified and untreated, may impair quality of life, impact upon the respiratory status of the patients, and ultimately lead to disease progression and death. Therefore, early identification and accurate treatment of comorbidities is essential.

  5. A molecular imaging approach to cystic fibrosis

    OpenAIRE

    Ferreira, Vera Filipa Cerqueira

    2013-01-01

    Tese de mestrado em Bioquímica, apresentada à Universidade de Lisboa, através da Faculdade de Ciências, 2013 A Fibrose Quística (FQ) é a doença autossómica recessiva letal mais comum na população caucasiana. É caracterizada por um mau funcionamento ao nível pulmonar, pancreático, gastrointestinal e reprodutivo, embora a principal causa de morbilidade e mortalidade se deva à progressiva disfunção pulmonar. A elevada concentração de electrólitos no suor constitui também uma das principais ca...

  6. Pulmonary arterial hypertension secondary to chronic thromboemboli

    International Nuclear Information System (INIS)

    Crysikopoulos, H.; Forrest, J.V.; Olson, L.K.; Roberts, A.C.

    1989-01-01

    The authors report their experience in 150 patients with pulmonary arterial hypertension secondary to chronic pulmonary embolism (CPE). Plain film findings are variable, commonly nonspecific. Occasionally nonuniformity of pulmonary perfusion or truncation of central pulmonary arteries suggest CPE. Multiple, large, unmatched perfusion defects are the most common scintigraphic observations. CT can exclude conditions mimicking CPE, particularly fibrosing mediastinitis. Pulmonary arteriography depicts the location and distribution of emboli. CPE is becoming an increasingly important entity to recognize because of available surgical treatment. Thromboendarterectomy may improve functional status and survival. The diagnosis should be considered in any patient with pulmonary arterial hypertension

  7. Estudo histológico do tecido conjuntivo areolar perifascial implantado em pregas vocais de coelhos

    OpenAIRE

    Adriana Hachiya

    2009-01-01

    Apesar do grande avanço da laringologia nas últimas décadas, o tratamento da rigidez da prega vocal continua sendo um desafio. A rigidez da prega vocal pode estar associada a alterações estruturais mínimas como no sulco vocal profundo ou decorrente da fibrose cicatricial como nos casos de cicatriz pós-operatória. Em ambos os casos, há perda dos elementos da matriz extracelular da camada superficial da lâmina própria (Espaço de Reinke) que se encontra substituída por tecido cicatricial. O obje...

  8. CT appearance of amiodarone-induced pneumonitis

    International Nuclear Information System (INIS)

    Nicholson, A.A.; Hayward, C.

    1989-01-01

    Basal peripheral pleuroparenchymal opacities are described on CT of early cryptogenic fibrosing alveolitis, asbestosis and bleomycin pneumonitis. These diseases may be caused by free radical effects on phospholipid metabolism causing cell wall damage. Amiodarone hydrochloride alters phospholipid synthesis metabolically. Amiodarone pneumonitis might be expected to show similar CT appearances. Sixteen patients who have developed new respiratory symptoms while taking amiodarone have been scanned prone and supine and at inspiration and expiration by means of a scanner with 2-mm sections at 1-cm intervals. All have been previously healthy nonsmokers with no relevant occupational history. Previous chest radiographs have been normal. Results are presented

  9. Eighty cases of nape muscular fasciae inflammation treated with puncture at Hégǔ(合谷LI 4) and Sānjiān(三间 LI 3)%针刺合谷、三间穴治疗项背肌筋膜炎80例

    Institute of Scientific and Technical Information of China (English)

    孙化斌; ZHANG Nan

    2010-01-01

    @@ Nape muscular fasciae inflammation refers to a chronic disease manifesting pain in soft tissues such as fascia and muscle on nape caused by aseptic inflammation, and it is named as "dorsal muscles fibrositis" as well.It is characterized as pain, stiffness and motor limitation in back and nape, involving trapezius, rhomboids and levator scapulae due to coldness, moist, chronic injury and incorrect positions.The author has treated 80 cases of nape muscular fasciae inflammation by puncturing at Hégǔ(合谷LI4)and Sānjiān(三间LI 3), and it is reported as follows.

  10. Epithelial-mesenchymal transition: An emerging target in tissue fibrosis

    Science.gov (United States)

    Li, Meirong; Luan, Fuxin; Zhao, Yali; Hao, Haojie; Zhou, Yong; Han, Weidong

    2016-01-01

    Epithelial-mesenchymal transition (EMT) is involved in a variety of tissue fibroses. Fibroblasts/myofibroblasts derived from epithelial cells contribute to the excessive accumulation of fibrous connective tissue in damaged tissue, which can lead to permanent scarring or organ malfunction. Therefore, EMT-related fibrosis cannot be neglected. This review highlights the findings that demonstrate the EMT to be a direct contributor to the fibroblast/myofibroblast population in the development of tissue fibrosis and helps to elucidate EMT-related anti-fibrotic strategies, which may enable the development of therapeutic interventions to suppress EMT and potentially reverse organ fibrosis. PMID:26361988

  11. Nephrogenic systemic fibrosis: A brief review

    Directory of Open Access Journals (Sweden)

    Rajesh Waikhom

    2011-01-01

    Full Text Available Nephrogenic systemic fibrosis is a fibrosing disorder of the skin that develops in patients with advanced renal failure. It mostly presents with progressive hardening or induration of the skin of the extremities. Systemic involvement is also known to occur in this entity. Exposure to gadolinium contrast for radiological evaluation has been identified as the offending agent. The condition is progressive and can be seriously disabling. Therapeutic options are limited and not rewarding in majority of the cases. Awareness of this entity is important so that proper precautionary measures can be taken at the earliest to ameliorate the condition.

  12. Systemic involvement in localized scleroderma/morphea.

    Science.gov (United States)

    Gorkiewicz-Petkow, Anna; Kalinska-Bienias, Agnieszka

    2015-01-01

    Localized scleroderma (LoSc), also known as morphea, is a rare fibrosing disorder of the skin and underlying tissues. Sclerosis is mainly limited to the skin, but subcutaneous tissue, fascia, and underlying muscles and bone may also be involved. In some cases, systemic manifestation with visceral abnormalities may occur. Several publications have focused on significant aspects of LoSc: genetics, immunity, epidemiology, scoring systems, and unification of classifications. Clinical studies featuring large cohorts with the disease published by various international study groups have been of great value in furthering the diagnostic and therapeutic management of LoSc. Copyright © 2015 Elsevier Inc. All rights reserved.

  13. Update on diagnosis and treatment of idiopathic pulmonary fibrosis

    Science.gov (United States)

    Baddini-Martinez, José; Baldi, Bruno Guedes; da Costa, Cláudia Henrique; Jezler, Sérgio; Lima, Mariana Silva; Rufino, Rogério

    2015-01-01

    Idiopathic pulmonary fibrosis is a type of chronic fibrosing interstitial pneumonia, of unknown etiology, which is associated with a progressive decrease in pulmonary function and with high mortality rates. Interest in and knowledge of this disorder have grown substantially in recent years. In this review article, we broadly discuss distinct aspects related to the diagnosis and treatment of idiopathic pulmonary fibrosis. We list the current diagnostic criteria and describe the therapeutic approaches currently available, symptomatic treatments, the action of new drugs that are effective in slowing the decline in pulmonary function, and indications for lung transplantation. PMID:26578138

  14. [Nonspecific interstitial pneumonitis: a clinicopathologic entity, histologic pattern or unclassified group of heterogeneous interstitial pneumonitis?].

    Science.gov (United States)

    Morais, António; Moura, M Conceição Souto; Cruz, M Rosa; Gomes, Isabel

    2004-01-01

    Nonspecific interstitial pneumonitis (NSIP) initially described by Katzenstein and Fiorelli in 1994, seems to be a distinct clinicopathologic entity among idiopathic interstitial pneumonitis (IIP). Besides different histologic features from other IIP, NSIP is characterized by a better long-term outcome, associated with a better steroids responsiveness than idiopathic pulmonar fibrosis (IPF), where usually were included. Thus, differentiating NSIP from other IIP, namely IPF is very significant, since it has important therapeutic and prognostic implications. NSIP encloses different pathologies, namely those with inflammatory predominance (cellular subtype) or fibrous predominance (fibrosing subtype). NSIP is reviewed and discussed by the authors, after two clinical cases description.

  15. PATHOMORPHOLOGICAL FEATURES OF BONE LESIONS AND CORRELATION OF CLINICAL, LABORATORY AND MORPHOMETRIC CRITERIA IN PATIENTS WITH LATENT SCLEROSING HEMATOGENOUS OSTEOMYELITIS (GARRÉ

    Directory of Open Access Journals (Sweden)

    V. V. Grigorovsky

    2018-01-01

    Full Text Available Introduction. Sclerosing hematogenous osteomyelitis of Garré (SHO holds a significant place among cases of latent hematogenous osteomyelitis. Pathomorphological studies of sclerosing hematogenous osteomyelitis are needed to improve differentiated diagnostics, to prognosticate morphology specifics of nidus and to choose the optimal treatment tactics.Purpose of the study — to identify statistical differences between manifestation patterns of various disease types and correlation between clinical, laboratorial and morphometric criteria of bone lesions in patients with sclerosing hematogenous osteomyelitis.Material and methods. The authors studied tissue fragments of affected bones of 25 patients with SHO which was diagnosed by clinical, laboratorial, visualizing and morphological methods. Gradation morphometric criteria were used to reflect condition of nidus tissues. The authors made the analysis of qualitative characters and correlation analysis of dependencies between clinical and laboratorial criteria on the one hand, and with morphometric criteria — on the other, and identified association factor. Results. Pathomorphological study of SHO nidus demonstrated variances of bone lesions in separate disease cases despite the overall similarity of morphological manifestation. About 2/3 of all cases correspond to fibrosing type whereby even small exudative inflammation sites are absent. In about 1/3 of all cases apart from fibrosis, osteosclerosis and remodeling, the osteomyelitis niduses contain microabscesses indicative of suppurative inflammation as well as the authors observed small sequestration. The maximum differences in patients with various types of SHO were identified in such parameters as share of stab microphages and erythrocyte sedimentation rate (ESR. In cases of long term morbidity the fibrosing disease type is prevailing, in cases of short term lesion (1–2 years — a fibrosing type with microabscesses formation.A series of

  16. Update on diagnosis and treatment of idiopathic pulmonary fibrosis

    Directory of Open Access Journals (Sweden)

    José Baddini-Martinez

    2015-10-01

    Full Text Available Idiopathic pulmonary fibrosis is a type of chronic fibrosing interstitial pneumonia, of unknown etiology, which is associated with a progressive decrease in pulmonary function and with high mortality rates. Interest in and knowledge of this disorder have grown substantially in recent years. In this review article, we broadly discuss distinct aspects related to the diagnosis and treatment of idiopathic pulmonary fibrosis. We list the current diagnostic criteria and describe the therapeutic approaches currently available, symptomatic treatments, the action of new drugs that are effective in slowing the decline in pulmonary function, and indications for lung transplantation.

  17. Idiopathic pulmonary fibrosis in a Staffordshire bull terrier with hypothyroidism.

    Science.gov (United States)

    Corcoran, B M; Dukes-McEwan, J; Rhind, S; French, A

    1999-04-01

    Radiographic evidence of chronic interstitial lung changes, usually believed to be attributable to lung fibrosis, is readily recognised in canine practice. Furthermore, there is a body of anecdotal evidence suggesting that a specific clinical entity consistent with chronic lung fibrosis occurs in specific breeds of terrier dogs. However, there is little pathological data to confirm these radiographic and clinical findings and, therefore, chronic interstitial lung disease of dogs is poorly characterised. In this report, a case of chronic pulmonary fibrosis is described in which histopathological confirmation was possible, and suggested that the condition might be analogous to idiopathic pulmonary fibrosis (cryptogenic fibrosing alveolitis) in humans.

  18. Fibromyalgia syndrome: definition and diagnostic aspects

    Directory of Open Access Journals (Sweden)

    A. Alciati

    2011-09-01

    Full Text Available Although the term “fibromyalgia” (FM is relatively new, the condition characterised by chronic musculoskeletal pain that is accompanied by numerous extra-skeletal symptoms has been described in the medical literature for many years under different names. The term “fibrositis,” which was originally used in 1904 by Sir William Gowers to define a type of lumbalgia, became a synonym for diffuse musculoskeletal pain until 1976 (1. In the mid-1970s, Smythe and Moldofsky used the term “fibrositic syndrome” to describe the presence of tender points (TPs, sleep disturbances and other accompanying symptoms such as asthenia...

  19. Morphea: Evidence-based recommendations for treatment

    Directory of Open Access Journals (Sweden)

    Nicole M Fett

    2012-01-01

    Full Text Available Morphea is a rare fibrosing disorder of the skin. Evidence-based treatment strategies in morphea are lacking. This review summarizes the available data on morphea treatment and provides therapeutic strategies based on morphea subtypes. The Cochrane Library, Medline and Embase from inception until May of 2011 were searched using the key words "morphea" and "morphea treatment." Reference lists of the resultant articles, as well as relevant reviews, were also searched. This review focuses on randomized controlled trials, prospective interventional trials without controls and retrospective reviews with greater than five subjects.

  20. Chronic hypersensitivity pneumonitis.

    Science.gov (United States)

    Pereira, Carlos Ac; Gimenez, Andréa; Kuranishi, Lilian; Storrer, Karin

    2016-01-01

    Hypersensitivity pneumonitis (HSP) is a common interstitial lung disease resulting from inhalation of a large variety of antigens by susceptible individuals. The disease is best classified as acute and chronic. Chronic HSP can be fibrosing or not. Fibrotic HSP has a large differential diagnosis and has a worse prognosis. The most common etiologies for HSP are reviewed. Diagnostic criteria are proposed for both chronic forms based on exposure, lung auscultation, lung function tests, HRCT findings, bronchoalveolar lavage, and biopsies. Treatment options are limited, but lung transplantation results in greater survival in comparison to idiopathic pulmonary fibrosis. Randomized trials with new antifibrotic agents are necessary.

  1. The importance of mdx mouse in the pathophysiology of Duchenne's muscular distrophy

    OpenAIRE

    Seixas, Sandra Lopes; Lagrota-Cândido, Jussara; Savino, Wilson; Quirico-Santos, Thereza

    1997-01-01

    O camundongo mdx desenvolve distrofia muscular recessiva ligada ao cromossoma X (locus Xp21.1) e não expressa distrofina. Embora não apresente intensa fibrose do tecido muscular e acúmulo de tecido adiposo, é considerado o modelo animal mais adequado da distrofia muscular de Duchenne. As alterações estruturais no tecido muscular associadas à mionecrose e presença do infiltrado inflamatório com predomínio de linfócitos e monócitos/macrófagos sugerem uma participação do sistema imunológico nest...

  2. Metrizamide in experimental selective renal angiography

    International Nuclear Information System (INIS)

    Pokorny, L.; Kelemen, J.

    1981-01-01

    Forty-seven dogs or seventy-four kidneys, respectively, were used in studies into the activity of metrizamide (Amipaque). The angiograms were of good quality; as compared with contrast media tested before, the incidence of abnormalities was somewhat lower, and the symptoms (spasm, inhomogeneous nephrogram) were less important. Histological examination showed that plenty of homogeneous pink proteinlike substance had accumulated in many tubules as well as in the lumen of the Bowman's capsules; fibroses were seen in several parts after 7 and 28 days, respectively. However, the evaluation of the renal function showed no considerable changes in the laboratory values. (orig.) [de

  3. Epanchement pleuro-péricardique liquidien révélant un lymphome lymphoblastique

    Science.gov (United States)

    N'goran, Yves N'da Kouakou; Soya, Kossa Esaïe; Beossin, Sylvanus Koui; Angoran, Ines; Traore, Fatou; Tano, Micesse; Afassinou, Yaovi; Ekou, Arnaud; Koffi, Florent; Yao, Hermann; Kramoh, Euloge Kouadio; Kakou, Maurice Guikahue

    2014-01-01

    L'atteinte cardiaque au cours des lymphomes lymphoblastiques est rare. Il s'agissait d'un patient de 17 ans de race noire qui a été admis pour une douleur médiothoracique. Le patient avait des signes d'insuffisance cardiaque droite; un syndrome d’épanchement pleural liquidien gauche; des adénopathies superficielles et une splénomégalie de type IV de Hackett. La biopsie ganglionnaire a été réalisée pour la confirmation du diagnostic par analyse histologique et immuno-histochimique. Des ponctions pleurale et péricardique ont été effectuées. Le patient a été confié au service d'oncologie pédiatrique pour la chimiothérapie. L’évolution a été marquée par le décès du patient 18 jours après. La manifestation cardiaque est une entité rare et très souvent méconnue.la confirmation histologique est parfois difficile à obtenir du vivant de la plupart des patients. Le pronostic est souvent réservé à court terme. PMID:25419294

  4. Chaînes d'exploits scénarios de hacking avancés et prévention

    CERN Document Server

    Whitaker, Andrew; Voth, Jack B

    2009-01-01

    Un pirate informatique s'appuie rarement sur une unique attaque, mais utilise plutôt des chaînes d'exploits, qui impliquent plusieurs méthodes et attaques coordonnées, pour atteindre sa cible et arriver à ses fins. Ces chaînes d'exploits sont généralement complexes et difficiles à prévenir. Or la plupart des ouvrages de sécurité ne les couvrent pas, ou sinon de manière superficielle. Cet ouvrage présente en profondeur les principales chaînes d'exploits qui sévissent actuellement. À travers des exemples basés sur des stratégies d'attaques réelles, utilisant les outils actuels les plus courants et visant des cibles importantes comme des données bancaires ou de sécurité sociale, vous découvrirez le spectre complet des attaques, des réseaux sans-fil à l'accès physique en passant par l'ingénierie sociale. Dans chaque scénario, les exploits sont décortiqués un à un en vue d'expliquer la chaîne qui va conduire à l'attaque finale. Les mesures de prévention à appliquer pour éviter...

  5. Computational and Experimental Study of Bubble Size in Bubble Columns

    DEFF Research Database (Denmark)

    Hansen, Rolf

    sammenklumpning og opbrydning af bobler, hvorved en Sauter gennemsnitsdiameter (SMD) beregnes i domænet. Boblestørrelsesfordelingen måles i en kvadratisk boblekolonne med ”Interferometric Particle Imaging” (IPI), som er en ikke-indtrængende optisk laserbaseret metode. Boblehastigheden måles samtidig med ”Particle...... Tracking Velocimetry” (PTV). Målingerne udføres for tre superficielle gashastigheder i en plan i midten af boblekolonnen. IPImålingerne viser en SMD på omkring 6.0mm for hele måleområdet, hvilket også er observeret visuelt. Lokale boblestørrelseshistogrammer præsenteres for at få data, der kan sammenlignes...... med beregningerne. Den eksperimentelle SMD synes at være konstant lodret gennem boblekolonnen, men faldende fra midten ind mod væggen horisontalt. Den eksperimentelle SMD synes også at falde med stigende superficiel gashastighed.  En beregningsanalyse af flowmønstret i den kvadratiske boblekolonne er...

  6. Analytical methods used by the geochemical section: water; Methodes d'analyses utilisees par la section de geochimie: les eaux

    Energy Technology Data Exchange (ETDEWEB)

    Berthollet, P; Cavalier, G [Commissariat a l' Energie Atomique, Fontenay-aux-Roses (France). Centre d' Etudes Nucleaires

    1971-07-01

    The authors describe the analytical methods used by the C.E.A. Geochemical Section to determine the chemical composition of natural waters encountered during the prospecting of uraniferous deposits or in the course of mining operations. Because of the diversity of the samples and the different items of information requested, methods were selected and adapted to answer to the demands peculiar to mining research. Methods and know-how concerning the quantitative analysis of natural water to find out the concentration of the following chemicals are reviewed: carbonates and bicarbonates, calcium, magnesium, chlorides, sodium and potassium, sulfates, nitrates, silica, phosphates, iron, manganese, aluminium, fluorides, dissolved oxygen, CO{sub 2}, SH{sub 2} and sulphur, and uranium. (authors) [French] Les auteurs decrivent les methodes d'analyses utilisees par la Section de Geochimie du C.E.A., pour la determination de la composition chimique des eaux naturelles rencontrees au cours de prospections de gites uraniferes ou de travaux miniers. La diversite des echantillons et les differents renseignements demandes a l'analyse les ont conduit a selectionner et a adapter des methodes afin qu'elles repondent aux exigences particulieres de la recherche miniere. Les methodes concernant le dosage dans les eaux superficielles et eaux souterraines des elements qui suivent sont presentees : carbonates and bicarbonates, calcium, magnesium, chlorures, sodium et potassium, sulfates, nitrates, silice, phosphates, fer ferreus et ferrique, manganese, aluminium, fluorures, oxygene dissous, CO{sub 2} libre, SH{sub 2} et soufre total, et uranium. (auteurs)

  7. La représentation de la sexualité dans la littérature féminine du nouveau millénaire : le cas de Catherine Millet

    Directory of Open Access Journals (Sweden)

    Nathalie Dumas

    2010-05-01

    Full Text Available Frédéric Beigbeder, dans son dernier roman intitulé Windows on the World, en vient à la conclusion que, de nos jours, le Play-boy international n’est en fait plus qu’un célibataire en quête de jouissance, ne chassant que la femme superficielle ou la prostituée. Selon l’auteur, aujourd’hui le Play-boy international est une femme. Une Bridget Jones ou une Carrie Bradschaw de Sex and the City (Beigbeder, 178 et 231. Le type de femme qui fait peur, qui assume et expose sa féminité et son indépendance ainsi que sa sexualité. Catherine Millet, rédactrice en chef du magazine Art Press, est l’une d’elles. Dans La vie sexuelle de Catherine M., publié en avril 2001, elle expose au grand public, d’une manière peu conventionnelle, les détails de sa vie sexuelle allant souvent au-delà de l’écriture dite féminine.

  8. Use of cadmium in solution in the EL 4 reactor moderator irreversible fixing of cadmium on the metallic surfaces; Utilisation du cadmium en solution dans le moderateur du reacteur EL 4 - fixation irreversible du cadmium sur les surfaces metalliques

    Energy Technology Data Exchange (ETDEWEB)

    Croix, O; Paoli, O; Lecomte, J; Dolle, L; Gallic, Y [Commissariat a l' Energie Atomique, Saclay (France). Centre d' Etudes Nucleaires

    1964-07-01

    In the framework of research into the poisoning of the EL-4 reactor by cadmium sulphate, measurements have been made by two different methods of the residual amounts of cadmium liable to be fixed irreversibly on the surfaces in contact with the heavy water. A marked influence of the pH has been noticed. The mechanism of the irreversible fixing is compatible with the hypothesis of an ion-exchange in the surface oxide layer. In a sufficiently wide range of pH the cadmium thus fixed causes very little residual poisoning. The stability of the cadmium sulphate solutions is however rather low in the conditions of poisoning. (authors) [French] Dans le cadre des etudes sur l'empoisonnement du reacteur EL-4 par le sulfate de cadmium, les quantites residuelles de cadmium susceptibles de se fixer irreversiblement sur les parois que mouillerait l'eau lourde, ont ete mesurees experimentalement par deux methodes differentes. On observe une influence nette du pH. Le mecanisme de la fixation irreversible est compatible avec l'hypothese d'un echange d'ions dans la pellicule d'oxyde superficielle. Dans des limites suffisamment larges de pH, la cadmium ainsi fixe n'occasionne pas d'empoisonnement residuel important. La stabilite des solutions de sulfate de cadmium dans les conditions de l'empoisonnement est cependant mediocre. (auteurs)

  9. Enseigner la Francophonie dans les cours de Français Langue Seconde au niveau universitaire : expériences et défis

    Directory of Open Access Journals (Sweden)

    Sébastien Sacré

    2013-11-01

    Full Text Available En cette époque de mondialisation, la mise en valeur des cultures minoritaires est, dans les milieux universitaires, de plus en plus importante. Cependant, ce développement d’une conscience culturelle est bien moins évidents dans le cadre de cours de langue, dont objectif principal n’est pas l’apprentissage d’une culture, mais celui d’une langue-cible. Ainsi, les langues et leurs cultures associées ont beau être indissociables par nature, il est cependant les enseigner séparément. Enseigner la langue en ne parlant de culture que superficiellement n’est cependant pas sans conséquences et il n’est pas rare de remarquer, dans manuels de langue par exemple, une représentation superficielle, voire stéréotypée du monde. Comment peut-on conjuguer l’apprentissage d’une langue à celui de ses richesses culturelles ? Basé sur des expériences d’enseignement et sur de récents manuels d’apprentissage, cet article se proposera d’analyser les difficultés et les défis de l’intégration d’éléments culturels dans des cours de type Français Langue Seconde.

  10. Neurorétinite unilatérale: une manifestation rare du syndrome post streptococcique

    Science.gov (United States)

    Iferkhass, Said; Elkhoyaali, Adil; Elasri, Fouad; Reda, Karim; Laktaoui, Abdelkader; Oubaaz, Abdelbar

    2014-01-01

    La streptocoque β hémolytique est responsable de plusieurs complications d'origine immunitaire, de localisation cardiaque, articulaire, dermatologique, rénale, cérébrale et oculaire. Nous rapportons le premier cas au Maroc d'une uvéite postérieure unilatérale post- streptococcique présentant une vascularite rétinienne avec œdème papillaire et des hémorragies rétiniennes en tache profondes et superficielles. Notre patient âgé de 56 ans, ayant comme antécédents pathologiques une notion d'angine et d’ érythème noueux à répétition d'origine streptococcique. Le bilan étiologique d'uvéite est revenu normal. En revanche, le titrage des anticorps antistreptococciques était significativement élevé: 430 IU /ml. Le patient a été mis sous amoxicilline protégée à une dose de 2 g par jour pendant 10 jours, associée à une corticothérapie à dose régressive sur un mois. L’évolution était favorable avec récupération totale de l'acuité visuelle (AV) et disparition des lésions neurorétiniennes PMID:25745528

  11. Renal dysplasia in a Limousin calf Displasia renal em um bezerro Limousin

    Directory of Open Access Journals (Sweden)

    M.B. Castro

    2007-04-01

    Full Text Available Descreve-se um caso de displasia renal em um bezerro Limousin, macho, de quatro meses. O animal apresentava perda de peso, baixa taxa de crescimento, anorexia, apatia, diarréia escura fétida e uremia, sem melhora apesar de tratamento. O bezerro foi sacrificado para interromper o sofrimento adicional e submetido à necropsia. Ao exame foi observado que os rins estavam pequenos, firmes, pálidos e com superfície rugosa. A cápsula estava aderida à uma superfície subcapsular irregular. O córtex tinha aparência difusamente pálida e fibrosa e apresentava múltiplos focos brancos de fibrose. A junção córtico-medular estava indistinta. O exame histologico dos rins revelou múltiplos glomérulos imaturos com núcleo periférico, capilares inaparentes e padrão arbóreo. O córtex renal apresentou túbulos primitivos com epitélio cubóide ou cilíndrico envolvido por mesênquima corado somente pelo alcian blue e não pelo tricrômico de Masson. Foi também observada leve fibrose intersticial na medula renal. Os rins mostravam estruturas em estágio inapropriado de desenvolvimento ou anômalas.

  12. IL18 and IL18R1 polymorphisms, lung CT and fibrosis: a longitudinal study in coal miners

    Energy Technology Data Exchange (ETDEWEB)

    Nadif, R.; Mintz, M.; Marzec, J.; Jedlicka, A.; Kauffmann, F.; Kleeberger, S.R. [INSERM, U780, Villejuif (France)

    2006-12-15

    It has been suggested that interleukin (IL)-18 plays a role in the development of inflammatory and fibrosing lung diseases. Associations of polymorphisms in the genes coding for IL-18 (108 /G-656T, C-607A, G-137C, T113G, C127T) and its receptor (IL8R1/C-69T) with coal workers' pneumoconiosis (CWP) were studied in 200 miners who were examined in 1990, 1994 and 1999. Coal-dust exposure was assessed according to job history and ambient measures. The main health outcome was lung computed tomography (CT) score in 1990. Internal coherence was assessed by studying CT score in 1994, 4-yr change in CT score and CWP incidence and prevalence. CT score in 1990 was a good predictor of radiographic grade in 1999 and, therefore, an appropriate subclinical quantitative trait. The IL18 -137C allele was associated with lower CT score in 1990 and 1994 (11.24 versus 1.69 and 1.57 versus 2.46, respectively), slower progression of CT score between 1990 and 1994 and lower pneumoconiosis prevalence in 1999 relative to the G allele (0.33 versus 0.77 and 8.2 versus 19.6%, respectively). Smoking- or dust-adjustment, and stratification on IL18R1 genotype and adjustment for haplotype effects did not change the conclusions. In conclusion, the results of the present study suggest a role for IL18 in reducing the development of this fibrosing lung disease.

  13. The genetic basis of strain-dependent differences in the early phase of radiation injury in mouse lung

    International Nuclear Information System (INIS)

    Franko, A.J.; Sharplin, J.; Ward, W.F.; Hinz, J.M.

    1991-01-01

    Substantial differences between mouse strains have been reported in the lesions present in the lung during the early phase of radiation injury. Some strains show only classical pneumonitis, while other strains develop substantial fibrosis and hyaline membranes which contribute appreciably to respiratory insufficiency, in addition to pneumonitis. Other strains are intermediate between these extremes. These differences correlate with intrinsic differences in activities of lung plasminogen activator and angiotensin converting enzyme. The genetic basis of these differences was assessed by examining histologically the early reaction in lungs of seven murine hybrids available commercially after whole-thorax irradiation. Crosses between fibrosing and nonfibrosing parents were uniformly nonfibrosing, and crosses between fibrosing and intermediate parents were uniformly intermediate. No evidence of sex linkage was seen. Thus the phenotype in which fibrosis is found is controlled by autosomal recessive determinants. Strains prone to radiation-induced pulmonary fibrosis and hyaline membranes exhibited intrinsically lower activities of lung plasminogen activator and angiotensin converting enzyme than either the nonfibrosing strains or the nonfibrosing hybrid crosses. The median time of death of the hybrids was genetically determined primarily by the longest-lived parent regardless of the types of lesions expressed

  14. Estudo comparativo das reações teciduais à implantação de silicone e politetrafluoroetileno no dorso de ratos

    Directory of Open Access Journals (Sweden)

    Kafejian Andréa Paula

    1997-01-01

    Full Text Available A importância das biopróteses na medicina abrange diversas áreas cirúrgicas. Com o objetivo de comparar a reação tecidual do implante de silicone, um dos mais utilizados, com o implante de politetrafluoroetileno expandido (PTFE-E, de uso mais recente, nos propusemos a realizar este estudo. Foram utilizados trinta ratos (Rattus norvegicus albinus machos, distribuídos em três grupos iguais, com implantes de fragmentos discóides dos materiais citados, no dorso de cada rato. Os grupos diferiram entre si quanto ao período de eutanásia: três, sete e trinta dias. Com base no modelo experimental e utilizando metodologia morfométrica, do ponto de vista histológico não houve reação inflamatória aguda importante que se pudesse correlacionar aos materiais de implantes. A proliferação vascular e a presença de fibrose foram prolongadas em relação à cicatrização normal. A irregularidade do PTFE-E, provavelmente relaciona-se à maior quantidade de vasos e de fibrose tardia constatada neste material, quando comparado ao implante de silicone.

  15. Clinical effects of non-ablative and ablative fractional lasers on various hair disorders: a case series of 17 patients.

    Science.gov (United States)

    Cho, Suhyun; Choi, Min Ju; Zheng, Zhenlong; Goo, Boncheol; Kim, Do-Young; Cho, Sung Bin

    2013-04-01

    Both ablative and non-ablative fractional lasers have been applied to various uncommon hair disorders. The purpose of this study was to demonstrate the clinical effects of fractional laser therapy on the course of primary follicular and perifollicular pathologies and subsequent hair regrowth. A retrospective review of 17 patients with uncommon hair disorders - including ophiasis, autosomal recessive woolly hair/hypotrichosis, various secondary cicatricial alopecias, pubic hypotrichosis, frontal fibrosing alopecia, and perifolliculitis abscedens et suffodiens - was conducted. All patients had been treated with non-ablative and/or ablative fractional laser therapies. The mean clinical improvement score in these 17 patients was 2.2, while the mean patient satisfaction score was 2.5. Of the 17 subjects, 12 (70.6%) demonstrated a clinical response to non-ablative and/or ablative fractional laser treatments, including individuals with ophiasis, autosomal recessive woolly hair/hypotrichosis, secondary cicatricial alopecia (scleroderma and pressure-induced alopecia), frontal fibrosing alopecia, and perifolliculitis abscedens et suffodiens. Conversely, patients with long-standing ophiasis, surgical scar-induced secondary cicatricial alopecia, and pubic hypotrichosis did not respond to fractional laser therapy. Our findings demonstrate that the use of non-ablative and/or ablative fractional lasers promoted hair growth in certain cases of uncommon hair disorders without any remarkable side effects.

  16. Fibred confocal fluorescence microscopy in the diagnosis of interstitial lung diseases.

    Science.gov (United States)

    Meng, Peng; Tan, Gan Liang; Low, Su Ying; Takano, Angela; Ng, Yuen Li; Anantham, Devanand

    2016-12-01

    Accurate diagnosis is critical to both therapeutic decisions and prognostication in interstitial lung diseases (ILD). However, surgical lung biopsies carry high complication rates. Fibred confocal fluorescence microscopy (FCFM) offers an alternative as it can visualize lung tissue in vivo at the cellular level with minimal adverse events. We wanted to investigate the diagnostic utility, and safety of using FCFM for patients with ILD. In patients with suspected ILD, FCFM images were obtained from multiple bronchopulmonary segments using a miniprobe inserted through the working channel of a flexible bronchoscope. The procedure was performed under moderate sedation in an outpatient setting. Morphometric measurements and fibre pattern analyses were co-related with computed tomography (CT) findings and patients' final diagnoses based on multi-disciplinary consensus. One hundred and eighty four segments were imaged in 27 patients (18 males) with a median age of 67 years (range, 24-79 years). They were grouped into chronic fibrosing interstitial pneumonia (16 patients) and other ILDs. Six distinct FCFM patterns were observed: normal, increased fibres, densely packed fibres, hypercellular, thickened fibres and others/non-specific. The pattern resembling densely packed fibres was seen in at least one segment in 68.8% patients with chronic fibrosing interstitial pneumonia, but only 36.4% in other ILD (P=0.097). An association between inflammatory patterns on CT and a hypercellular pattern on FCFM was also found (P<0.001). Our study shows the potential of FCFM in classifying ILD, but its role in further diagnosis remains limited.

  17. Aspergilose broncopulmonar alérgica com imagem radiológica em "dedo de luva" Allergic bronchopulmonary aspergillosis presenting a glove-finger shadow in radiographic images

    Directory of Open Access Journals (Sweden)

    Marta Elizabeth Kalil

    2006-10-01

    Full Text Available A aspergilose broncopulmonar alérgica é uma doença pulmonar que ocorre em pacientes com asma ou fibrose cística, desencadeada pela reação de hipersensibilidade à presença do fungo Aspergilus fumigatus nas vias aéreas. Relatamos aqui um caso em que uma paciente com quadro clínico sugestivo de asma apresentou critérios clínicos, laboratoriais e radiológicos compatíveis com o diagnóstico de aspergilose broncopulmonar alérgica. A importância de tais achados deve-se ao fato de que quanto mais precocemente for feito o diagnóstico, menores serão os riscos de agravamento do quadro respiratório e de aparecimento de fibrose.Allergic bronchopulmonary aspergillosis is a lung disease occurring in patients with asthma or cystic fibrosis, triggered by a hypersensitivity reaction to the presence of Aspergillus fumigatus in the airways. We report herein the case of a patient presenting a clinical profile suggestive of asthma and meeting the clinical, laboratory testing and radiological criteria for a diagnosis of allergic bronchopulmonary aspergillosis. The importance of such findings is that early diagnosis can reduce the risk of respiratory exacerbations and fibrosis.

  18. Microencapsulation of Lefty-secreting engineered cells for pulmonary fibrosis therapy in mice.

    Science.gov (United States)

    Ma, Hongge; Qiao, Shupei; Wang, Zeli; Geng, Shuai; Zhao, Yufang; Hou, Xiaolu; Tian, Weiming; Chen, Xiongbiao; Yao, Lifen

    2017-05-01

    Idiopathic pulmonary fibrosis (IPF) is a progressive disease that causes unremitting deposition of extracellular matrix proteins, thus resulting in distortion of the pulmonary architecture and impaired gas exchange. Associated with high morbidity and mortality, IPF is generally refractory to current pharmacological therapies. Lefty A, a potent inhibitor of transforming growth factor-β signaling, has been shown to have promising antifibrotic ability in vitro for the treatment of renal fibrosis and other potential organ fibroses. Here, we determined whether Lefty A can attenuate bleomycin (BLM)-induced pulmonary fibrosis in vivo based on a novel therapeutic strategy where human embryonic kidney 293 (HEK293) cells are genetically engineered with the Lefty A-associated GFP gene. The engineered HEK293 cells were encapsulated in alginate microcapsules and then subcutaneously implanted in ICR mice that had 1 wk earlier been intratracheally administered BLM to induce pulmonary fibrosis. The severity of fibrosis in lung tissue was assessed using pathological morphology and collagen expression to examine the effect of Lefty A released from the microencapsulated cells. The engineered HEK293 cells with Lefty A significantly reduced the expression of connective tissue growth factor and collagen type I mRNA, lessened the morphological fibrotic effects induced by BLM, and increased the expression of matrix metalloproteinase-9. This illustrates that engineered HEK293 cells with Lefty A can attenuate pulmonary fibrosis in vivo, thus providing a novel method to treat human pulmonary fibrotic disease and other organ fibroses. Copyright © 2017 the American Physiological Society.

  19. Ultrasound and magnetic resonance imaging findings in Schistosomiasis mansoni: expanded gallbladder fossa and fatty hilum signs Achados da ultrassonografia e da ressonância magnética na esquistossomose mansônica: sinais da expansão da fossa da vesícula e do hilo gorduroso

    Directory of Open Access Journals (Sweden)

    Luciana Cristina dos Santos Silva

    2012-08-01

    esquistossomose mansônica. Todos apresentavam US indicativa de fibrose hepática esquistossomótica, e foram avaliados com imagens por RM, realizadas com uma unidade magnética supercondutora de 1,5-T(Sigma. RESULTADOS: Quarenta e sete (87% entre 54 pacientes com sinais ultrassonográficosde fibrose periportal esquistossomótica tiveram este diagnóstico confirmado pela RM. Nos sete(13% casos discordantes, a RM revelou tecido adiposo preenchendo o espaço periportal hilaronde a US indicava espessamento isolado da parede da veia porta em seu ponto de entrada no fígado. Este achado foi nomeado sinal do hilo gorduroso. Um dos 47 pacientes com evidência de fibrose periportal RM era colecistectomizado. Trinta e quatro (76,1% dos 46 pacientes restantes apresentavam expansão da fossa da vesícula, que se encontrava preenchida portecido adiposo. Nos outros sete, a RM revelou sinais de fibrose pericolecística. CONCLUSÕES: Os espessamentos ecogênicos central da parede da veia porta, e da parede da vesícula biliar, até o momento, atribuídos à fibrose, foram frequentemente identificados como tecido adiposopela RM. Entretanto, o espessamento da parede da vesícula identificado pela US (expansão da fossa da vesícula na RM é provavelmente secundário a alterações morfológicas hepáticas na esquistossomose, e representa comprometimento grave do fígado.

  20. Aquagenic keratoderma associated with a mutation of the cystic fibrosis gene

    Directory of Open Access Journals (Sweden)

    V. Coelho-Macias

    2013-05-01

    Full Text Available Reported for the first time in 1996, aquagenic keratoderma is a rare condition which is characterized by edematous flat-topped papules appearing on palmar skin after water immersion. Multiple anecdotal associations have been described but, recently, the association with cystic fibrosis gene mutations (CFTR has been highlighted.The authors describe an 18-year-old female, with one-month complaints of pruritus and swelling of palmar skin after water immersion. On examination, palmar skin was unremarkable but, 5 min after water immersion, multiple whitish papules became apparent. CFTR genotype study showed a F508del mutation in one allele (nonhomozygous. She had no other symptoms and no relevant family history.Aquagenic keratoderma is probably an under-diagnosed entity that might represent a manifestation of CFTR mutations, making carrier state identification and genetic counseling possible. Resumo: Descrita em 1996, a acroqueratodermia aquagénica é uma entidade rara, caracterizada pelo aparecimento de pápulas edematosas palmares após contacto com água. Múltiplas associações foram enumeradas mas, recentemente, a associação a mutações do gene da fibrose quística foi demonstrada.Descreve-se o caso de uma mulher de 18 anos, saudável, com prurido e edema palmar após imersão em água. O exame objetivo inicial não mostrava alterações mas, 5 min após imersão em água, observavam-se múltiplas pápulas esbranquiçadas palmares. O estudo do gene da fibrose quística (CFTR revelou uma mutação F508del num dos alelos. A doente negava outras queixas ou história familiar relevante.A acroqueratodermia aquagénica é uma entidade provavelmente subdiagnosticada que poderá constituir uma manifestação de mutações do gene CFTR, o que possibilitaria a identificação de portadores e aconselhamento genético. Keywords: Aquagenic keratoderma, Cystic fibrosis, Genetic counseling, Palavras-chave: Acroqueratodermia aquagénica, Fibrose qu

  1. Pneumonite intersticial não específica: entidade clínico-patológica, padrão histológico ou apenas grupo heterogéneo de pneumonites intersticiais não classificadas?

    Directory of Open Access Journals (Sweden)

    António Morais

    2004-07-01

    Full Text Available RESUMO: A pneumonite intersticial não específica (NSIP, descrita pela primeira vez em 1994 por Katzenstein e Fiorelli, apresenta-se como uma eventual entidade clínico-patológica individualizável dentro do grupo das pneumonites intersticiais idiopáticas (IIP. Aliada a alterações histológicas distintas, apresentauma evolução clínica mais favorável, com consequente melhor prognóstico do que a fibrose pulmonar idiopática (IPF, grupo onde geralmente estas alterações eram incluídas. Estas diferenças levam a que, apesar das dúvidas que ainda envolvem a NSIP, o seu reconhecimento seja importante, uma vez que as atitudes clínicas e terapêuticas se diferenciam da IPF. A NSIP engloba padrões histológicos diferentes, dividindo-se nomeadamente em casos de predomínio inflamatório (tipo celular ou de predomínio fibroso (tipo fibrosante, com os últimos a evidenciarem uma maior gravidade e pior prognóstico do que os primeiros. Os autores descrevem dois casos de NSIP, seguidos de uma revisão e discussão do tema.REV PORT PNEUMOL 2004; X (4: 331-345 ABSTRACT: Nonspecific interstitial pneumonitis (NSIP initially described by Katzenstein and Fiorelli in 1994, seems to be a distinct clinicopathologic entity among idiopathic interstitial pneumonitis (IIP. Besides different histologic features from other IIP, NSIP is characterized by a better long-term outcome, associated with a better steroids responsiveness than idiopathic pulmonar fibrosis (IPF, where usually were included. Thus, differentiating NSIP from other IIP, namely IPF is very significant, since it has important therapeutic and prognostic implications. NSIP encloses different pathologies, namely those with inflammatory predominance (cellular subtype or fibrous predominance (fibrosing subtype. NSIP is reviewed and discussed by the authors, after two clinical cases description.REV PORT PNEUMOL 2004; X (4: 331-345 Palavras chave: pneumonites intersticiais idiopáticas, pneumonite

  2. The effect of prophylactic dose of a low molecular weight heparin on skin wound healing of rats Efeito da dose profilática de heparina de baixo peso molecular na cicatrização de feridas na pele de ratos

    Directory of Open Access Journals (Sweden)

    Ozdamar Fuad Oken

    2009-12-01

    Full Text Available PURPOSE: To investigate the effect of prophylactic dose of a low molecular weight heparin, enoxaparin, on skin wound healing of rats. METHODS: Forty rats were used for the study. Rats were randomly assigned to two equal groups. Experimental group received prophylactic dose of enoxaparin. Physiologic saline was administered to the control group. Parameters of wound healing of experimental and control groups were compared. For comparison of the groups in terms of fibrosis, vascularization, inflammation, epithelization, and tensile strength test (Newton. Mann-Whitney-U test was used because variables were categorical data (fibrosis, vascularization, inflammation and epithelization. Differences between groups were analyzed with independent samples t-test (tensile strength. Significance was set at pOBJETIVO: Investigar o efeito de dose profilática da heparina de baixo peso molecular, enoxaparina, na cicatrização de feridas na pele de ratos. MÉTODOS: Quarenta ratos foram utilizados para o estudo. Ratos foram distribuídos aleatoriamente a dois grupos iguais. O grupo experimental recebeu profilática de enoxaparina. Solução salina fisiologica foi administrada ao grupo controle. Foram comparados parâmetros de cicatrização dos grupos experimental e controle.Os grupos foram comparados em termos de fibrose, vascularização, inflamação, epitelização e força tensil (teste de Newton. Foi realizado o teste de Mann-Whitney-U para variáveis com dados categóricos (fibrose, cicatrização, inflamação e epitelização. Diferenças entre os grupos foram analisadas como amostras independentes pelo t-teste (força tensil. Significância foi fixada para p < 0,05. RESULTADOS: A ferida do grupo experimental apresentou força tensil diminuída significativamente (p < 0,001, o exame histopatológico revelou um significativo (p < 0,001 retardo na epitelização e diminuição na fibrose, cicatrização, inflamação (p < 0,001 no grupo experimental

  3. Variaciones de la temperatura superficial del mar en Puerto Chicama y del índice de oscilación del Sur: 1925-1992

    Directory of Open Access Journals (Sweden)

    1993-01-01

    Full Text Available VARIATIONS DE LA TEMPÉRATURE SUPERFICIELLE DE LA MER À PUERTO CHICAMA ET DE L'INDICE DE L'OSCILLATION AUSTRALE : 1925-1992. Ce travail présente une description des principales variations de la Température Superficielle de la Mer (TSM dans le Port de Chicama (07°42'S-79 °27'O et de l'Indice d'Oscillation Australe entre 1925 et 1992. Il est d'abord précisé que les informations de TSM de Chicama (à présent renommé Puerto Malabrigo constituent la série la plus longue et confiable du pays elle part de décembre 1924 avec 2 mesures quotidiennes jusqu'en 1969, date à partir de laquelle 3 mesures quotidiennes ont été faites. On décrit ensuite, sur l'échelle du temps, la présence d'anomalies de températures par groupes d'années et très rarement an cours d'une même année. On définit le début et la durée de l'événement en fonction des déviations standard mensuelles et annuelles, pour pouvoir ainsi définir des événements chauds ou froids et les comparer avec les IOS. El propósito del presente trabajo es presentar una descripción de las principales variaciones de la Temperatura Superficial del Mar (TSM en el Puerto de Chicama (07° 42'S -79° 27'W y del índice de Oscilación del Sur durante el periodo de 1925 a 1992. Este trabajo empieza precisando, que los datos de TSM de Chicama, (actualmente renominado Puerto Malabrigo constituyen la serie más larga y confiable del país se inició en Diciembre de 1924 con dos mediciones diarias hasta 1969 y a partir de esta fecha se realizan tres mediciones diarias. Luego se describe en la escala de tiempo, la presencia de anomalías de temperatura en grupos de años y escasas veces en un solo año y se define el inicio y duración del evento en función de las desviaciones estandard mensuales y anuales, para después caracterizar eventos cálidos o fríos y compararlos con los IOS. PUERTO CHICAMA SEA SURFACE TEMPERATURE AND THE SOUTHERN OSCILLATION INDEX VARIATIONS: 1925-1992. The aim

  4. Preliminary studies of vanadium-base alloys intended for use in fabrication of cans for fast reactors; Etudes preliminaires sur les alliages a base de vanadium envisages pour la fabrication de gaines de reacteurs rapides

    Energy Technology Data Exchange (ETDEWEB)

    Conte, M [Commissariat a l' Energie Atomique, Fontenay-aux-Roses (France). Centre d' Etudes Nucleaires

    1967-03-15

    1050 deg. C sous vide montrent que d'une facon generale, les elements d'additions ameliorent les proprietes mecaniques du vanadium. Apres des traitements de corrosion dans une boucle a sodium liquide purifie par piege froid, les alliages sont fragilises a la temperature ambiante. Seule la nuance V-20 pour cent Ti conserve des proprietes plastiques. Ces alliages sont recouverts d'une couche superficielle de carbure de vanadium VC. Apres des traitements dans une boucle a sodium liquide purifie par piege chaud, tous les alliages conservent de bonnes caracteristiques mecaniques. La couche superficielle qui les recouvre est constituee de deux carbures de vanadium VC et {sub {gamma}}VC et d'un sous-oxyde de vanadium VO{sub 0,9}. (auteur)

  5. MÉTODO DE INDUÇÃO DE CIRROSE BILIAR SECUNDÁRIA COM USO DE PRÓTESE DE SILICONE METHOD FOR THE EXPERIMENTAL INDUCTION OF SECUNDARY BILIARY CIRRHOSIS IN WISTAR RATS

    Directory of Open Access Journals (Sweden)

    A. A. L. de Araujo Lima

    2001-01-01

    Full Text Available O objetivo deste experimento foi o desenvolvimento de um modelo de obstrução do ducto biliar comum através da interposição de uma prótese de silicone extrínseca ao ducto com única ligadura sem secção. Desenvolveu-se um modelo experimental alternativo, em ratos Wistar, que provoca a interrupção do fluxo bílio-duodenal com resultado satisfatório, pois houve distorção da arquitetura hepática, caracterizada por fibrose e proliferação ductal além de indicadores bioquímicos da colestase.

  6. Novel approach for identification of left ventricle geometry in patients with chronic heart failure, AH and IHD in combination with COPD

    Directory of Open Access Journals (Sweden)

    Potabashniy V.A.

    2016-05-01

    Full Text Available The aim of this study was to examine the direction of change of left ventricle (LV geometry in patients with chronic heart failure (CHF, arterial hypertension (AH and ischemic heart disease (IHD in combination with chronic obstructive pulmonary disease (COPD in dependence on severity of clinical signs of CHF and COPD based on recommendation of American Society of Echocardiography and European Association of Cardiovascular Images (2015. We examined 67 patients with CHF, associated with AH and stable IHD and stable COPD. By the results of this study there were determined different types of left ventricle geometry: concentric LV hypertrophy (LVH, eccentric LVH, mixed LVH, dilated LVH, dependent on blood pressure level, fibrosic and ischemic myocardial changes,, primary predominant disease – AH, IHD or COPD.

  7. Sindrome de Guillain-Barré como manifestação do lupus eritematoso sistêmico: registro de um caso

    Directory of Open Access Journals (Sweden)

    Pedro F. Moreira Filho

    1980-06-01

    Full Text Available É relatado caso de paciente jovem do sexo feminino que apresentou, de modo agudo, quadro de polirradiculoneurite com acometimento de vários nervos cranianos e dissociação albumino-citológica no líquido cefalorraqueano. Os exames complementares mostraram tratar-se de lupus eritematoso sistêmico. A biópsia de nervo periférico revelou diminuição de fibras mielínicas, fibrose do endo e perineuro e hipertrofia da camada média dos vasa-nervorum. Os autores chamam a atenção para a raridade do acometimento do sistema nervoso periférico na vigência da doença lúpica. Acreditam que a síndrome de Guillain-Barré provocada pelo lupus eritematoso sistêmico seria devido a um mecanismo imuno-alérgico.

  8. Early aggressive intra-venous pulse cyclophosphamide therapy for interstitial lung disease in a patient with systemic sclerosis. A case report.

    LENUS (Irish Health Repository)

    Peshin, R

    2009-06-01

    Interstitial lung disease is an important cause of mortality and morbidity in patients with systemic sclerosis (SSc). There are currently no recommended guidelines for management of these patients. This is probably due to the rarity of this condition, as well as clinical trials with only a small number of cases. There are published case report and case series along with the two main trials, viz. Scleroderma Lung Study and the Fibrosing Alveolitis Study, but again, there is no consensus on treatment protocols. In this report, we present a case of aggressive interstitial lung disease in a patient with SSc, which improved dramatically on treatment with intra-venous cyclophosphamide and high dose prednisolone therapy.

  9. Diffuse scarring alopecia in a female pattern hair loss distribution.

    Science.gov (United States)

    Fergie, Bonnie; Khaira, Gurpreet; Howard, Vicki; de Zwaan, Sally

    2018-02-01

    We describe three cases of hair loss in a female pattern hair loss (FPHL) distribution with histologic features of lichen planopilaris (LPP). All patients had a history of diffuse, gradual hair loss in a Christmas tree pattern that clinically presented as FPHL on gross and dermoscopic examination. Notably, there were no characteristic clinical signs of LPP and no histologic features of FPHL. These cases are most consistent with cicatricial pattern hair loss (CPHL). This relatively new entity is similar to fibrosing alopecia in a pattern distribution (FAPD) in that they are both scarring alopecias confined to a FPHL distribution, but CPHL lacks the clinical signs of perifollicular erythema and perifollicular keratosis seen in FAPD. These three cases may present an early, subtle form of CPHL and will be of interest to clinicians and histopathologists alike. © 2017 The Australasian College of Dermatologists.

  10. [Drug treatment of alopecia].

    Science.gov (United States)

    Wolff, H

    2015-10-01

    Alopecia is the term used to describe hairless areas of the scalp. They can follow a specific pattern, be diffuse or circumscript. Androgenetic alopecia (AGA) follows a pattern: in men thinning of temples and vertex up to total baldness; in women thinning of the midline or parietal area. Lack of iron or cytostatic drugs cause diffuse alopecia, while in autoimmune diseases such as alopecia areata or lichen planus bizarre shapes of hairless areas are observed. For therapy, the following medications are used: topical minoxidil solution for AGA of men and women; systemic finasteride 1 mg for men with AGA; topical diphencyprone immunotherapy for alopecia areata; systemic antimycotic agents for tinea capitis; antibiotics such as clindamycin and rifampicin for folliculitis decalvans; systemic corticosteroids and isotretinoin for folliculitis et perifolliculitis capitis abscedens et suffodiens; topical corticosteroids for lichen planus and Kossard's frontal fibrosing alopecia.

  11. Pulmonary Arterial Hypertension: Use of Delayed Contrast-Enhanced Cardiovascular Magnetic Resonance in Risk Assessment

    OpenAIRE

    Bessa, Luiz Gustavo Pignataro; Junqueira, Flávia Pegado; Bandeira, Marcelo Luiz da Silva; Garcia, Marcelo Iorio; Xavier, Sérgio Salles; Lavall, Guilherme; Torres, Diego; Waetge, Daniel

    2013-01-01

    FUNDAMENTO: A hipertensão arterial pulmonar é uma doença grave e progressiva. O maior desafio clínico é seu diagnóstico precoce. OBJETIVO: Avaliar a presença e a extensão do realce tardio miocárdico pela ressonância magnética cardíaca bem como verificar se o percentual da massa de fibrose miocárdica é indicador de gravidade. MÉTODOS: Estudo transversal com 30 pacientes com hipertensão arterial pulmonar dos grupos I e IV, submetidos às avaliações clínica, funcional e hemodinâmica, e à ressonân...

  12. Hipertensão arterial pulmonar: uso do realce tardio miocárdico pela ressonância magnética cardíaca na avaliação de risco

    OpenAIRE

    Bessa, Luiz Gustavo Pignataro; Junqueira, Flávia Pegado; Bandeira, Marcelo Luiz da Silva; Garcia, Marcelo Iorio; Xavier, Sérgio Salles; Lavall, Guilherme; Torres, Diego; Waetge, Daniel

    2013-01-01

    FUNDAMENTO: A hipertensão arterial pulmonar é uma doença grave e progressiva. O maior desafio clínico é seu diagnóstico precoce. OBJETIVO: Avaliar a presença e a extensão do realce tardio miocárdico pela ressonância magnética cardíaca bem como verificar se o percentual da massa de fibrose miocárdica é indicador de gravidade. MÉTODOS: Estudo transversal com 30 pacientes com hipertensão arterial pulmonar dos grupos I e IV, submetidos às avaliações clínica, funcional e hemodinâmica, e à ressonân...

  13. Individual pharmacokinetic variation leads to underdosing of ciprofloxacin in some cystic fibrosis patients

    DEFF Research Database (Denmark)

    Schultz, Anders Nikolai Ørsted; Høiby, N; Nielsen, X C

    2017-01-01

    BACKGROUND: Ciprofloxacin (CIP) is frequently used when treating cystic fibrose (CF) patients with intermittent Pseudomonas aeruginosa (P. aeruginosa) lung colonization. However, approximately 20% of the patients progress to chronic infection despite early intervention. The aim of this study......, was to investigate the pharmacokinetics of CIP, to evaluate if CYP3A4-related metabolism is involved and to find the optimal dose needed to eradicate intermittently colonizing bacteria in the lungs of CF patients. Methods An open-label, prospective pharmacokinetic study was performed. Twenty-two adult CF......-patients were each given 500 mg CIP orally. One blood sample was taken at t = 0, and the following 12 hr, nine blood samples were collected. The optimal dose and interval was then calculated by Monte Carlo simulation. CYP3A4-activity was mesured using the Erythromycin Breath Test (ERMBT). Results A 14-fold...

  14. Changes of bronchoalveolar cell pattern and lecithin content in experimental interstitial pneumonia

    International Nuclear Information System (INIS)

    Manabe, Hideki; Yasuoka, Susumu; Tsubura, Eiro

    1978-01-01

    The pathogenesis of diffuse interstitial fibrosing pneumonitis (DIFP) was studied by histological observations and analysis of the cells and lecithin content of bronchoalveolar lavage of rats with cyclophosphamide (CY)-induced pneumonitis or irradiation pneumonitis. The rats developed diffuse interstitial pneumonitis one week after the last of 5 intraperitoneal injections of 50 mg/kg of CY and gradually recovered in the next 14 weeks. The number of alveolar macrophages and the lecithin content in the bronchoalveolar lavage from these rats corresponded to the degree of inflammatory change of the lung tissue. The results of cell counts and analysis of the bronchoalveolar lavage from rats with irradiated pneumonitis were similar to those on rats with CY-induced pneumonitis, except that in irradiated rats the lecithin content of the lavage decreased with increase in severity of pulmonary fibrosis. These results indicate that the cell number and lecithin content of bronchoalveolar lavage are good parameters for use in diagnosis of DIFP. (auth.)

  15. Study of late effects of lung irradiation with telecobalt on lung function

    International Nuclear Information System (INIS)

    Wolkewitz, J.

    1980-01-01

    Two groups of patients, one with malignant chest tumors before radiation and one more than nine months after radiation treatment of their tumors, are examined by assessment of different lung function parameters. In addition, four patients can be examined both before and more than nine months after radiation treatment for chest tumors. It is seen that the patients whose X-rays show signs of localized radiation fibrosis do not have the typical lung function problems noted in diffuse radiation fibroses. In patients with radiographic signs of localized radiation fibrosis, combined ventilation disturbances of obstruction and restriction type are more frequent and accompanied by a slight increase in the mean pulmonary arterial pressure. These modifications of the lung function cannot solely be attributed to the radiation treatment, as the lung function is also influenced by concomitant pulmonary affections such as chronic bronchitis or renewed growth of the irradiated tumor. (orig.) [de

  16. [Farmer's lung--a form of exogenous allergic alveolitis].

    Science.gov (United States)

    Sambale, M; Liebetrau, G

    1990-11-15

    Exogenic allergic alveolitides are caused by organic dusts which contain bacteria, moulds or vegetable and animal antigens. The farmer's lung as a form of the exogenic allergic alveolitis is a rare disease. The uncharacteristic symptomatology in the initial phase and in particular the retarded beginning of the symptom after several hours handicap the timely recognition in an early phase of the disease so that curative therapeutic measures are rarely possible. The cases of the disease are found only at the chronic stage, at the stage of the pulmonary fibrosis. Then the prognosis is unfavourable. In the Central Clinic for Heart and Lung Diseases Bad Berka 1,110 patients with alveolitides and lung fibroses were diagnosed in the period from 1975 to 1988. 306 of them could be clarified as exogenic allergic alveolitis, 61 of them (19.8%) were farmer's lungs.

  17. Estudo necroscópico de um caso da forma crônica da doença de Chagas com eletrocardiograma e raio-X de tórax normais: referência especial ao sistema excito-condutor do coração

    Directory of Open Access Journals (Sweden)

    Edison Reis Lopes

    1988-06-01

    Full Text Available Descrevem-se as alterações anatomopatológicas observadas na necropsia de chagásico crônico, provavelmente, portador da forma indeterminada da doença. O coração mostrou lesões dos três folhetos e do sistema excito-condutor, traduzidas especialmente por pequenos focos inflamatórios. Havia discreta fibrose do miocárdio contrâtil, nódulo átrio-ventricular e origem do ramo esquerdo do feixe de His. Tais alterações, embora de leve intensidade, assemelham-se, qualitativamente, às relatadas nas demais formas crônicas da doença de Chagas e representam lesões ativas, com potencial evolutivo. No esôfago, observou-se entre outros fatos, despopulação neuronal acentuada.

  18. Uso de macrolídeos em doenças pulmonares: controvérsias da literatura recente

    Directory of Open Access Journals (Sweden)

    Luiz Vicente Ribeiro Ferreira da Silva Filho

    2015-12-01

    Full Text Available Resumo Objetivo: Revisar os mecanismos de ação de macrolídeos em doenças respiratórias pediátricas e as suas indicações clínicas. Fonte de dados: Revisão na base de dados Pubmed, compreendendo os termos em inglês referentes ao tema básico. Síntese dos dados: O seu espectro de ação estende-se desde a produção de mediadores inflamatórios até o controle da hipersecreção de muco e a modulação de mecanismos de defesa do hospedeiro. O potencial benefício dos antibióticos macrolídeos foi estudado em doenças pulmonares como a fibrose cística, as bronquiectasias, a asma, a bronquiolite aguda e as bronquiectasias não ligadas à fibrose cística. Diversos estudos avaliaram os benefícios dos macrolídeos na asma resistente a terapia, porém os resultados são controversos e as indicações devem ser limitadas a fenótipos específicos. Na bronquiolite viral não há benefícios consistentes nos quadros agudos, embora dados recentes mostrem um efeito na prevenção de sibilância recorrente. Em pacientes com fibrose cística os resultados também são contraditórios, mas o consenso é de que há um pequeno benefício clínico, especialmente para os pacientes infectados por P. aeruginosa. Também não foi observada ação positiva dos macrolídeos em pacientes com bronquiolite obliterante pós-infecciosa. Crianças com bronquiectasias não relacionadas à fibrose cística parecem ter claros benefícios em relação ao uso de macrolídeos, os quais mostraram vantagens clínicas, de proteção ao parênquima e na função pulmonar. Conclusões: O uso em longo prazo de macrolídeos deve ser limitado a situações altamente selecionadas, especialmente em pacientes com bronquiectasias. Avaliação cuidadosa dos benefícios e potenciais danos são ferramentas para indicação em grupos específicos.

  19. Bioavailability of very finely distributed metallic platinum in the lungs and first orienting studies on effects. Part 2; Bioverfuegbarkeit von feinstverteiltem metallischem Platin in der Lunge und erste orientierende Wirkungsuntersuchungen (VPT 09; 2. Teil)

    Energy Technology Data Exchange (ETDEWEB)

    Emmendoerffer, A [Fraunhofer-Institut fuer Toxikologie und Aerosolforschung, Hannover (Germany)

    1998-12-31

    Platinum is emitted from automotive catalytic converters in particulate form. The first organ to be exposed to it after its uptake by the organism is the lung. Lung macrophages therefore constitute the first barrier of the immune system which can to react to platinum. In this study the production of nitrogen oxides and cytokines was studied. These parameters play an important role in the initiation of inflammation and in the maintenance of chronic inflammatory processes that can lead to fibrosis of the lung. [Deutsch] Platin wird partikulaer aus Automobilabgaskatalysatoren emittiert. Das erste Zielorgan fuer eine Aufnahme im Organismus ist deshalb die Lunge. Die Lungenmakrophagen stellen also die erste Barriere des Immunsystems dar, das auf Platin reagieren kann. In vitro wurde die Produktion von Stickoxiden und Zytokinen untersucht. Diese Parameter spielen eine Rolle bei der Initiation von Entzuendungen und auch bei der Aufrechterhaltung chronisch entzuendlicher Prozesse, die in der Lunge zu einer Fibrose fuehren koennen. (orig.)

  20. Renal function, nephrogenic systemic fibrosis and other adverse reactions associated with gadolinium-based contrast media.

    Science.gov (United States)

    Canga, Ana; Kislikova, Maria; Martínez-Gálvez, María; Arias, Mercedes; Fraga-Rivas, Patricia; Poyatos, Cecilio; de Francisco, Angel L M

    2014-01-01

    Nephrogenic systemic fibrosis is a fibrosing disorder that affects patients with impaired renal function and is associated with the administration of gadolinium-based contrast media used in MRI. Despite being in a group of drugs that were considered safe, report about this potentially serious adverse reaction was a turning point in the administration guidelines of these contrast media. There has been an attempt to establish safety parameters to identify patients with risk factors of renal failure. The close pharmacovigilance and strict observation of current regulations, with special attention being paid to the value of glomerular filtration, have reduced the published cases involving the use of gadolinium-based contrast media. In a meeting between radiologists and nephrologists we reviewed the most relevant aspects currently and recommendations for its prevention.

  1. Musculoskeletal manifestations of diabetes mellitus.

    Science.gov (United States)

    Merashli, M; Chowdhury, T A; Jawad, A S M

    2015-11-01

    The prevalence of Type 1 and Type 2 diabetes are increasing significantly worldwide. Whilst vascular complications of diabetes are well recognized, and account for principle mortality and morbidity from the condition, musculoskeletal manifestations of diabetes are common and whilst not life threatening, are an important cause of morbidity, pain and disability. Joints affected by diabetes include peripheral joints and the axial skeleton. Charcot neuroarthropathy is an important cause of deformity and amputation associated with peripheral neuropathy. A number of fibrosing conditions of the hands and shoulder are recognized, including carpal tunnel syndrome, adhesive capsulitis, tenosynovitis and limited joint mobility. People with diabetes are more prone to gout and osteoporosis. Management of these conditions requires early recognition and close liaison between diabetes and rheumatology specialists. © The Author 2015. Published by Oxford University Press on behalf of the Association of Physicians. All rights reserved. For Permissions, please email: journals.permissions@oup.com.

  2. Hyperfibrotic myelodysplasia: case report with response to steroid therapy Mielodisplasia hiperfibrótica: relato de caso com resposta à terapia com corticosteróides

    Directory of Open Access Journals (Sweden)

    Maura Romeo

    2002-01-01

    Full Text Available Context: Bone marrow fibrosis is observed in different clonal hematological disorders including myeloproliferative diseases, acute leukemias and myelodysplastic syndromes. In myelodysplastic syndrome a new clinical-pathological entity with significant increase in reticulin fibers has been suggested, and the term hyperfibrotic myelodysplasia was used to define it. Bone marrow biopsy shows increased reticulin fibers, megakaryocytic hyperplasia and dysplasia. Differential diagnosis with primary myelofibrosis may be difficult and hybrid cases may occur. Patients with hyperfibrotic myelodysplastic syndrome responding to treatment with steroids have been reported. In the majority of cases there was only hematological remission, although resolution of fibrosis occurred in one patient. Design: Case report. Case report: A 62-year old male presented in June 95 with a 6-month history of lethargy and dispnea. On examination he was pale without hepato-splenomegaly. Hemoglobin concentration was 3g/dL with marked anisocytosis without teardrop cells. Bone marrow aspirates resulted in dry tap. Bone marrow biopsy showed hypercellularity with increased fibrosis (grade IV obliterating the normal marrow architecture. Megakaryocytes were increased in number, with abnormal morphology. Monoclonal antibodies against factor VIII and CD31 revealed that both were expressed in megakaryocytes. Prednisone (1mg/Kg was introduced in June 1996, after what his symptoms lessened and hemoglobin increased. Bone marrow fibrosis decreased (grade IV to grade II. He has become transfusion independent till Jan/1999, when hemoglobin fell to 6g/dL and prednisone was reintroduced with a prompt rise in hemoglobin concentration.Contexto: A fibrose de medula óssea é encontrada em algumas doenças hematológicas clonais, incluindo síndromes mieloproliferativas, leucemias agudas e síndromes mielodisplásicas. Nas síndromes mielodisplásicas, uma nova entidade clinicopatológica com

  3. Efeitos da derivação ileocólica e da exclusão total do íleo terminal na morfologia e nas funções de fígado, rins e íleo terminal de ratos submetidos a ligadura do ducto hepático comum

    OpenAIRE

    Evandro Luis de Oliveira Costa

    2011-01-01

    As doenças colestáticas e sua evolução para fibrose e cirrose são muito conhecidas. Apesar do incremento de terapias, como o transplante hepático, esse grupo de afecções, ainda representa desafio na prática médica, desde o diagnóstico até o tratamento, além de estarem ligadas a morbidade e letalidade altas, com custos de tratamento elevados. Novas condutas e busca dos mecanismos fisiopatológicos envolvidos são ampliados a cada dia. Este trabalho tem por objetivos avaliar a influência e os efe...

  4. Efeitos da derivação ileocólica e da exclusão do íleo terminal na morfologia e nas funções de fígado, rins e íleo terminal de ratos submetidos a ligadura do ducto hepático comum

    OpenAIRE

    Evandro Luis de Oliveira Costa

    2013-01-01

    As doenças colestáticas e sua evolução para fibrose e cirrose são muito conhecidas. Apesar do incremento de terapias, como o transplante hepático, esse grupo de afecções, ainda representa desafio na prática médica, desde o diagnóstico até o tratamento, além de estarem ligadas a morbidade e letalidade altas, com custos de tratamento elevados. Novas condutas e busca dos mecanismos fisiopatológicos envolvidos são ampliados a cada dia. Este trabalho tem por objetivos avaliar a influência e os efe...

  5. Morphea in Childhood: An Update.

    Science.gov (United States)

    Aranegui, B; Jiménez-Reyes, J

    2018-05-01

    Morphea is an inflammatory, fibrosing skin disorder. When it occurs in childhood, it is also known as localized juvenile scleroderma. It is more common in girls and typically appears around the age of 5 to 7 years. According to a recent classification system, morphea is divided into 5 types: circumscribed (plaque), linear, generalized, pansclerotic, and mixed. Approximately 40% of patients present extracutaneous manifestations. Childhood morphea is treated with phototherapy, oral or topical calcitriol, topical tacrolimus 0.1%, methotrexate, topical or systemic corticosteroids, mycophenolate mofetil, bosentán, and topical imiquimod 5%. A variety of measuring tools are used to monitor response to treatment. Few prognostic studies have been conducted, but findings to date suggest that the disease tends to run a chronic or intermittent-recurrent course and frequently causes sequelae. Copyright © 2017 AEDV. Publicado por Elsevier España, S.L.U. All rights reserved.

  6. Análise morfológica dos cotos neurais de ratos Wistar após segmentectomia do fascículo lateral do plexo braquial direito e treino aeróbico

    OpenAIRE

    Fernando Soares Camelier

    2011-01-01

    A lesão isolada do plexo braquial não apresenta alta mortalidade, porém são marcantes as limitações funcionais nesta patologia traumática. As sequelas graves da lesão estão associadas a diversos fatores, tais como, o padrão anatômico das raízes nervosas, seus ramos difusamente distribuídos, o intenso desenvolvimento de tecido fibroso ao redor dos cotos neurais lesados e o tratamento cirúrgico, que promove a formação de fibrose e prejudica a regeneração neural. Neste trabalho, provocamos a les...

  7. Free epidural fat-grafting after lumbar laminectomy using MRI

    International Nuclear Information System (INIS)

    Ohtsubo, Yoshimasa; Fujita, Masaaki; Motokawa, Satoru; Nakamura, Satoshi; Narabayashi, Yoko

    1994-01-01

    Free epidural fat-grafting was performed to prevent adhesion between scar tissue of the epidural space and dura and nerve root after lumbar laminectomy. The results were evaluated using MRI. Fifteen cases were operated upon with an average follow-up period of 7.5 months. MRI was recorded and the findings were classified into four types as follow: I, equally high signal case; II, slight fibrosis case; III, severe fibrosis case; IV, high signal disappeared case. The results showed 3 cases of type I, 7 cases of type II, 3 cases of type III, and 2 cases of type IV. According to MRI findings, free epidural fat is supposed to be still viable and alive, although slightly fibroses but the dural side remains a high signal area. Free epidural fat-grafting was useful for preventing adhesion around the epidural space. There was high relationship to age and sex with regard to viability of the free fat graft. (author)

  8. Lesions in nerves and plexus after radiotherapy

    International Nuclear Information System (INIS)

    Vees, W.

    1978-01-01

    Apart from the typical, radiation-induced changes in the skin, common secondary findings were oedemas, radiation-induced ulceration, fibroses of the mediastinum and lungs, pleura adhesions, and osteoradionecroses. In one patient with radiogenic paresis of the plexus brachialis, irradiation of the spinal cord because of epidural metastases of a mammary carcinoma resulted in radiation myelopathy which was verified by laminectomy. Observations of radiogenic lesions of the plexus brachialis show that the usual site of the lesion in the vasomotoric nerve bundle is the axilla. The lesion is assumed to be caused mainly by an overlapping of the axillary, infraclavicular and supraclavicular fields of irradiation which results in a dose peak in the axilla. (orig./AJ) 891 AJ/orig.- 892 MKO [de

  9. Cytomegalovirus pneumonitis complicated by a central peribronchial pattern of organising pneumonia.

    Science.gov (United States)

    Cuadrado, Maria M; Ahmed, Asia; Carpenter, Ben; Brown, Jeremy S

    2017-01-01

    We present five cases of cytomegalovirus (CMV) pneumonitis occurring in patients after recent T cell deplete allogeneic stem cell transplantation (AlloHSCT). These cases were complicated by an organising pneumonia (during the recovery period) with a predominantly central peribronchial pattern. All patients presented with evidence of active CMV pneumonitis which was treated successfully with anti-viral therapy but was followed by persistent severe dyspnoea, cough and hypoxia. High resolution computed tomography (HRCT) imaging showed widespread central peribronchial consolidation with traction bronchiectasis. There was a marked clinical and physiological improvement after treatment with systemic corticosteroids. However, in all patients the lung function remained abnormal and in some cases imaging revealed a fibrosing lung disease. These cases represent a previously undescribed central peribronchial pattern of organising pneumonia complicating CMV pneumonitis that can result in chronic lung damage.

  10. Long-term success of endovascular treatment of benign superior vena cava occlusion with chylothorax and chylopericardium

    Energy Technology Data Exchange (ETDEWEB)

    Veroux, Pierfrancesco; Veroux, Massimiliano; Bonanno, Maria Giovanna; Tumminelli, Maria Giuseppina [Department of Surgery and Transplantation, University Hospital, Via S. Sofia, 78, 95123 Catania (Italy); Baggio, Elda [Department of Surgery and Gastroenterological Sciences, University Hospital of Verona (Italy); Petrillo, Giuseppe [Department of Radiology, University Hospital, Via S. Sofia, 78, 95123 Catania (Italy)

    2002-07-01

    The most likely etiology of benign obstruction of the superior vena cava (SVC) include fibrosing mediastinitis and iatrogenic etiologies such as sclerosis and obstruction caused by pacemakers and central venous catheter. Percutaneous stenting of SVC has been used with success both in malignant and benign superior vena cava syndrome; however, long-term follow-up of endovascular procedures is not well known. We present a case of a patient with complete occlusion of SVC of benign etiology, presenting dramatically with bilateral chylothorax and chylopericardium with cardiac tamponade, who underwent successful vena caval revascularization with thrombolytic therapy and placement of self-expanding metallic stent. The 42-month follow-up could encourage endovascular procedures even in SVC syndrome of benign etiology. (orig.)

  11. Opacidades em vidro fosco nas doenças pulmonares difusas: correlação da tomografia computadorizada de alta resolução com a anatomopatologia Ground-glass opacity in diffuse lung diseases: high-resolution computed tomography-pathology correlation

    Directory of Open Access Journals (Sweden)

    Maria Lúcia de Oliveira Santos

    2003-12-01

    Full Text Available Opacidade em vidro fosco é achado freqüentemente visto na tomografia computadorizada de alta resolução do tórax e se traduz pelo aumento do coeficiente de atenuação dos pulmões, mas sem apagar as marcas broncovasculares. Por sua inespecificidade, a associação com outros achados radiológicos, clínicos e anatomopatológicos deve ser considerada para uma interpretação diagnóstica mais correta. Neste trabalho foram analisados 62 exames tomográficos de pacientes com doenças pulmonares difusas, de 14 etiologias diferentes, em que opacidades em vidro fosco foram o achado único ou predominante, e feita correlação anatomopatológica por meio de biópsias ou necropsias. Na pneumocistose as opacidades em vidro fosco corresponderam, histologicamente, à ocupação alveolar por material espumoso contendo parasitos; no carcinoma bronquíolo-alveolar, a espessamento dos septos alveolares e ocupação de sua luz por muco e células tumorais; na paracoccidioidomicose, a espessamento dos septos alveolares, áreas de fibrose e alvéolos contendo exsudato broncopneumônico; na sarcoidose, a fibrose ou a acúmulo de granulomas; na fibrose pulmonar idiopática, a espessamento dos septos alveolares por fibrose; na bronquiolite obliterante com pneumonia em organização, a pneumonia intersticial com áreas de organização intra-alveolar. A ocupação alveolar por sangue foi observada nos casos de leptospirose, hemossiderose idiopática, metástases de tumor renal e na aspergilose invasiva; por vacúolos de gordura na pneumonia lipídica; por material protéico e lipoprotéico na silicoproteinose e na proteinose alveolar; e por líquido de edema na insuficiência cardíaca congestiva.Ground-glass opacity is a finding frequently seen in high-resolution computed tomography examinations of the chest and is characterized by hazy increased attenuation of lung, however without blurring of bronchial and vascular margins. Due to its unspecificity

  12. Role of matrix metalloproteinases in the pathophysiology of idiopathic pulmonary fibrosis

    Directory of Open Access Journals (Sweden)

    Bhattacharyya P

    2007-01-01

    Full Text Available Idiopathic pulmonary fibrosis (IPF, a progressive fibrosing lung condition is a ther-apeutic medical challenge. The etiopathogenesis of IPF is complicated and hitherto not adequately understood. However, the concept, of late, is shifting from fibrosis as a result of inflammation to a mechanism of primarily dysregulated fibrogenesis. A class of enzymes called matrix metallo proteinases (MMPs appear important in the pathogenesis of IPF. The heightened activity of MMPs are derived out of an imbalance between them (MMPs and their tissue inhibitors (TIMPs. This leads to breakdown of interstitial matrix and triggering of certain growth factors which play an important mechanistic role in the pathogenesis of IPF. Understanding of the role of MMPs in pathogenesis of IPF may open up a new horizon of therapeutic intervention of the desease.

  13. Pathogenesis of Idiopathic Pulmonary Fibrosis

    Science.gov (United States)

    Wolters, Paul J.; Collard, Harold R.; Jones, Kirk D.

    2014-01-01

    Idiopathic pulmonary fibrosis (IPF) is a fibrosing interstitial lung disease associated with aging that is characterized by the histopathological pattern of usual interstitial pneumonia. Although an understanding of the pathogenesis of IPF is incomplete, recent advances delineating specific clinical and pathologic features of IPF have led to better definition of the molecular pathways that are pathologically activated in the disease. In this review we highlight several of these advances, with a focus on genetic predisposition to IPF and how genetic changes, which occur primarily in epithelial cells, lead to activation of profibrotic pathways in epithelial cells. We then discuss the pathologic changes within IPF fibroblasts and the extracellular matrix, and we conclude with a summary of how these profibrotic pathways may be interrelated. PMID:24050627

  14. Chronic infection of cystic fibrosis patient airways by a single clone of Burkholderia cepacia: replacement of non-mucoid to mucoid morphotype Infecção pulmonar crônica por um único clone de Burkholderia cepacia: substituição do morfotipo não mucóide por mucóide

    Directory of Open Access Journals (Sweden)

    Ana Paula D'Alincourt Carvalho

    2003-11-01

    Full Text Available Mucoid Burkholderia cepacia morphotype emerged within a nine year follow-up of a cystic fibrosis patient. Clinical data suggested a linkage between the mucoid phenotype isolation and the deterioration of the patient's condition. Despite of the phenotypic variation, molecular typing showed that the patient was chronically infected with B. cepacia complex isolates belonging to a same genetic clone.O presente trabalho descreve a emergência de cepas mucoides do complexo B. cepacia em um paciente com Fibrose Cística dentro de um acompanhamento bacteriológico prospectivo de nove anos. Os dados clínicos sugerem a associação entre o isolamento do morfotipo mucoide e a deterioração clínica do paciente. Apesar da variação fenotípica, os testes moleculares mostraram que o paciente manteve-se cronicamente infectado por cepas de mesma origem clonal.

  15. Guidelines for the medical treatment of idiopathic pulmonary fibrosis.

    Science.gov (United States)

    Xaubet, Antoni; Molina-Molina, María; Acosta, Orlando; Bollo, Elena; Castillo, Diego; Fernández-Fabrellas, Estrella; Rodríguez-Portal, José Antonio; Valenzuela, Claudia; Ancochea, Julio

    2017-05-01

    Idiopathic pulmonary fibrosis is defined as chronic fibrosing interstitial pneumonia limited to the lung, with poor prognosis. The incidence has been rising in recent years probably due to improved diagnostic methods and increased life expectancy. In 2013, the SEPAR guidelines for the diagnosis and treatment for idiopathic pulmonary fibrosis were published. Since then, clinical trials and meta-analyses have shown strong scientific evidence for the use of pirfenidone and nintedanib in the treatment of idiopathic pulmonary fibrosis. In 2015, the international consensus of 2011 was updated and new therapeutic recommendations were established, prompting us to update our recommendation for the medical treatment of idiopathic pulmonary fibrosis accordingly. Diagnostic aspects and non-pharmacological treatment will not be discussed as no relevant developments have emerged since the 2013 guidelines. Copyright © 2017 SEPAR. Publicado por Elsevier España, S.L.U. All rights reserved.

  16. HRCT of the lung in collagen vascular diseases

    International Nuclear Information System (INIS)

    Diederich, S.; Roos, N.; Schmitz-Linneweber, B.; Gaubitz, M.; Peters, P.E.

    1996-01-01

    Collagen vascular diseases, representing systemic soft tissue disorders, may cause a broad spectrum of pathologic changes of the respiratory tract. The type and extent of manifestations can vary considerably among individuals and entities. This survey describes the chest radiographic and, in particular, high-resolution computed tomographic and, in particular, high-resolution computed tomographic (HRCT) findings of individual lesions of the respiratory tract. It includes fibrosing alveolitis (alveolitis, interstitial pneumonia, pulmonary fibrosis) and bronchial (bronchitis/bronchiolitis, bronchiectasis), pleural and vascular manifestations, as well as lymphadenopathy and abnormalities related to therapy. We present typical patterns of changes in progressive systemic sclerosis (PSS, scleroderma), systemic lupus erythematosus (SLE), mixed connective tissue disease (MCTD, Sharp syndrome), Sjoegren syndrome, overlap syndrome and rheumatoid arthritis (RA). Furthermore, we describe findings which are specific for individual entities such as esophageal involvement in PSS, acute pneumonitis and pulmonary hemorrhage in SLE, lymphoproliferative disease in Sjoegren syndrome and necrobiotic nodules in RA. (orig.) [de

  17. Guidelines for the diagnosis and treatment of idiopathic pulmonary fibrosis. Sociedad Española de Neumología y Cirugía Torácica (SEPAR) Research Group on Diffuse Pulmonary Diseases.

    Science.gov (United States)

    Xaubet, Antoni; Ancochea, Julio; Bollo, Elena; Fernández-Fabrellas, Estrella; Franquet, Tomás; Molina-Molina, Maria; Montero, Maria Angeles; Serrano-Mollar, Anna

    2013-08-01

    Idiopathic pulmonary fibrosis is defined as a chronic fibrosing interstitial pneumonia limited to the lung, of unknown cause, with poor prognosis and few treatment options. In recent years there has been an increase in their prevalence, probably due to the optimization of diagnostic methods and increased life expectancy. The ATS/ERS Consensus (2000) established the diagnostic criteria and recommendations for the assessment of the disease course and treatment. Later studies have helped to redefine diagnostic criteria and treatment options. In 2011, an international consensus was published, establishing diagnostic criteria and new treatment strategies. These guidelines have been updated with the newest aspects of diagnosis and treatment of idiopathic pulmonary fibrosis. A level of evidence has been identified for the most relevant questions, particularly with regard to treatment options. Copyright © 2012 SEPAR. Published by Elsevier Espana. All rights reserved.

  18. Quantitative analysis of cardiac lesions in chronic canine chagasic cardiomyopathy Análise quantitativa das lesões cardíacas na cardiomiopatia chagásica crônica canina

    Directory of Open Access Journals (Sweden)

    Marcelo Vidigal CALIARI

    2002-10-01

    úsculo cardíaco, fibrose e substituição por tecido adiposo. No miocárdio atrial dos cães infectados pelas cepas Be78 e Be62 foram observados 34 e 50% de músculo cardíaco, 28 e 32% de fibrose e, 38 e 18% de tecido adiposo, respectivamente. A fibrose observada era tanto difusa quanto focal e, principalmente intrafascicular interrompendo total ou parcialmente o percurso dos feixes musculares. Tais alterações histológicas provavelmente contribuiram para o surgimento dos distúrbios eletrocardiográficos verificados em 10 dos 11 cães estudados e que são comuns na cardiopatia chagásica crônica humana. De todos os achados microscópicos encontrados, a fibrose foi a mais importante por produzir rearranjos na fibras colágenas em relação aos miocardiócitos, modificando a fisionomia anatômica e o comportamento mecânico do miocárdio. Tais anormalidades estruturais podem contribuir para o surgimento à disfunção cardíaca, arritmias e à insuficiência cardíaca congestiva como verificado em dois cães analisados. A cepa Be78 produziu uma destruição de músculo cardíaco atrial estatisticamente superior à induzida pela cepa Be62.

  19. New Treatments for Hair Loss.

    Science.gov (United States)

    Vañó-Galván, S; Camacho, F

    2017-04-01

    The treatment of hair loss is an important part of clinical dermatology given the prevalence of the problem and great impact on patients' quality of life. Many new treatments have been introduced in recent years. This review summarizes the main ones in 4 groups: a) For androgenetic alopecia, we discuss new excipients for oral minoxidil, dutasteride, and finasteride as well as new forms of topical application; prostaglandin agonists and antagonists; low-level laser therapy; and regenerative medicine with Wnt signaling activators and stem cell therapy. b) For alopecia areata, Janus kinase inhibitors are reviewed. c) For frontal fibrosing alopecia, we discuss the use of antiandrogens and, for some patients, pioglitazone. d) Finally, we mention new robotic devices for hair transplant procedures and techniques for optimal follicular unit extraction. Copyright © 2016 AEDV. Publicado por Elsevier España, S.L.U. All rights reserved.

  20. Protocol of the 2nd Oberaudorf talks on October 12th - 14th, 1975, on prophylaxis and therapy of late radiation effects in carcinomas of women

    Energy Technology Data Exchange (ETDEWEB)

    1976-06-01

    The chapter 'prophylaxis and therapy of radiation effects' summarizes the papers on this topic read during the Oberaudorf talks. It is found that a clear definition must be made first of all. Depending on the disease and its stage, certain side-effects must be put up with. Modern methods have helped tp avoid some radiation injuries but not fibroses in the broadest sense. Apart from the technique and dosage used radiation effects also depend on a local and/or general accompanying treatment. In radiotherapy of cancer of the breast, some early changes such as erythemas are unavoidable while others can be avoided. In the treatment of relapses, severe radiation injuries are sometimes unavoidable. Special regard must be paid to changes in the neighbouring organs. The earlier assumption that previous irradiations are a contraindication to surgical treatment at a later stage is no larger generally valid.

  1. Chronic hypersensitivity pneumonitis

    Directory of Open Access Journals (Sweden)

    Pereira CA

    2016-09-01

    Full Text Available Carlos AC Pereira,1 Andréa Gimenez,2 Lilian Kuranishi,2 Karin Storrer 2 1Interstitial Lung Diseases Program, 2Pulmonology Postgraduate, Federal University of São Paulo, São Paulo, Brazil Abstract: Hypersensitivity pneumonitis (HSP is a common interstitial lung disease resulting from inhalation of a large variety of antigens by susceptible individuals. The disease is best classified as acute and chronic. Chronic HSP can be fibrosing or not. Fibrotic HSP has a large differential diagnosis and has a worse prognosis. The most common etiologies for HSP are reviewed. Diagnostic criteria are proposed for both chronic forms based on exposure, lung auscultation, lung function tests, HRCT findings, bronchoalveolar lavage, and biopsies. Treatment options are limited, but lung transplantation results in greater survival in comparison to idiopathic pulmonary fibrosis. Randomized trials with new antifibrotic agents are necessary. Keywords: interstitial lung diseases, extrinsic allergic alveolitis, diffuse lung disease, lung immune response, HRCT, farmers lung

  2. Use of /sup 197/HgCl/sub 2/ in the study of pseudo-tumoral pulmonary afflictions

    Energy Technology Data Exchange (ETDEWEB)

    Biagini, C; Centi Colella, A; Pigorini, F [Rome Univ. (Italy)

    1975-01-01

    The utility of examinations with /sup 197/HgCl/sub 2/ has been shown in the study of pseudo-tumoral pulmonary afflictions. The following points seem to be the most important. The real possibility of differential diagnosis between benign and malignant tumors if the uptake of radio-mercury is related to the volume of the lesion. The possibility of differential diagnosis between cancers and the after-effects of more inflammatory TBC and non-TBC processes (inactive tuberculomas, fibroses). In sub-acute and chronic inflammatory lesions, some indication is furnished by the behavior of the uptake ratio at 24 and 72 hours. The topographical definition of the tumoral lesion in the case where there are associated secondary phenomena (atelectasis, a small quantity of pleural effusion). Individualization of extrapulmonary metastases which are situated at the level of the brain and the thoracic skeleton. Evaluation of the modifications of proliferative activities of cancers during radiologic or medical treatment.

  3. Occipital neuralgia: anatomic considerations.

    Science.gov (United States)

    Cesmebasi, Alper; Muhleman, Mitchel A; Hulsberg, Paul; Gielecki, Jerzy; Matusz, Petru; Tubbs, R Shane; Loukas, Marios

    2015-01-01

    Occipital neuralgia is a debilitating disorder first described in 1821 as recurrent headaches localized in the occipital region. Other symptoms that have been associated with this condition include paroxysmal burning and aching pain in the distribution of the greater, lesser, or third occipital nerves. Several etiologies have been identified in the cause of occipital neuralgia and include, but are not limited to, trauma, fibrositis, myositis, fracture of the atlas, and compression of the C-2 nerve root, C1-2 arthrosis syndrome, atlantoaxial lateral mass osteoarthritis, hypertrophic cervical pachymeningitis, cervical cord tumor, Chiari malformation, and neurosyphilis. The management of occipital neuralgia can include conservative approaches and/or surgical interventions. Occipital neuralgia is a multifactorial problem where multiple anatomic areas/structures may be involved with this pathology. A review of these etiologies may provide guidance in better understanding occipital neuralgia. © 2014 Wiley Periodicals, Inc.

  4. Bioavailability of very finely distributed metallic platinum in the lungs and first orienting studies on effects. Part 2; Bioverfuegbarkeit von feinstverteiltem metallischem Platin in der Lunge und erste orientierende Wirkungsuntersuchungen (VPT 09; 2. Teil)

    Energy Technology Data Exchange (ETDEWEB)

    Emmendoerffer, A. [Fraunhofer-Institut fuer Toxikologie und Aerosolforschung, Hannover (Germany)

    1997-12-31

    Platinum is emitted from automotive catalytic converters in particulate form. The first organ to be exposed to it after its uptake by the organism is the lung. Lung macrophages therefore constitute the first barrier of the immune system which can to react to platinum. In this study the production of nitrogen oxides and cytokines was studied. These parameters play an important role in the initiation of inflammation and in the maintenance of chronic inflammatory processes that can lead to fibrosis of the lung. [Deutsch] Platin wird partikulaer aus Automobilabgaskatalysatoren emittiert. Das erste Zielorgan fuer eine Aufnahme im Organismus ist deshalb die Lunge. Die Lungenmakrophagen stellen also die erste Barriere des Immunsystems dar, das auf Platin reagieren kann. In vitro wurde die Produktion von Stickoxiden und Zytokinen untersucht. Diese Parameter spielen eine Rolle bei der Initiation von Entzuendungen und auch bei der Aufrechterhaltung chronisch entzuendlicher Prozesse, die in der Lunge zu einer Fibrose fuehren koennen. (orig.)

  5. Radiological-morphological synopsis of radiation-induced lung fibrosis

    International Nuclear Information System (INIS)

    Bublitz, G.

    1977-01-01

    As delayed radiation damage after treatment of bronchial carcinoma and mamma carcinoma, fibroses occur as a reaction of the tissues. They have become a clinical-functional syndrome because of their uniform clinicaL-radiological symptomatology and pathophysiology. Pulmonary fibrosis as delayed radiation damage has a special importance with its two different radiation effects on connective tissue: a) on existing structures, b) delayed alterations of the connective tissue. As seen from experiments on lungs of men and rats, radiation-induced alterations can be measured by testing the different solubilities of the collagen types. In addition to the pathologically disordered collagen production, 9 weeks after the irradiation the radiation fibrosis leads to an isolated increase of insoluble collagen corresponding to the formation of metabolism-resistant fibrils. (MG) [de

  6. The use of 197HgCl2 in the study of pseudo-tumoral pulmonary afflictions

    International Nuclear Information System (INIS)

    Biagini, C.; Centi Colella, A.; Pigorini, F.

    1975-01-01

    The utility of examinations with 197 HgCl 2 has been shown in the study of pseudo-tumoral pulmonary afflictions. The following points seem to be the most important. The real possibility of differential diagnosis between benign and malignant tumors if the uptake of radio-mercury is related to the volume of the lesion. The possibility of differential diagnosis between cancers and the after-effects of more inflammatory TBC and non-TBC processes (inactive tuberculomas, fibroses). In sub-acute and chronic inflammatory lesions, some indication is furnished by the behavior of the uptake ratio at 24 and 72 hours. The topographical definition of the tumoral lesion in the case where there are associated secondary phenomena (atelectasis, a small quantity of pleural effusion). Individualization of extrapulmonary metastases which are situated at the level of the brain and the thoracic skeleton. Evaluation of the modifications of proliferative activities of cancers during radiologic or medical treatment [fr

  7. Study, with the help of nuclear indicators (HTO, Urea {sup 14}C and {sup 24}Na), of the renewal of water, urea and sodium in different parts of the rat kidney in aqueous diuresis and of the gerbil kidney in osmotic diuresis or in oliguria; Etude, a l'aide d'indicateurs nucleaires (HTO, UREE {sup 14}C et {sup 24}Na), du renouvellement de l'eau, de l'uree et du sodium dans les differentes regions du rein de rat en diurese aqueuse et de merion en diurese osmotique ou en oligurie

    Energy Technology Data Exchange (ETDEWEB)

    Lechene, C P [Commissariat a l' Energie Atomique, Saclay (France). Centre d' Etudes Nucleaires

    1965-06-01

    In the kidney of the gerbil in oliguria there exists a cortico-papillary gradient in the concentration of the sodium and of the urea; this gradient disappears in the gerbil kidney in osmotic diuresis or in the rat kidney in aqueous diuresis; in these three states of diuresis there is no significant difference in the potassium concentration between the surface and interior regions of the kidneys. Kinetic studies using tritiated water, urea {sup 14}C and {sup 24}Na show that water and urea in the interior regions of the kidney are only renewed very slowly (1 h) whereas the sodium is rapidly changed (1 mn). These results can be explained using WIRZ's theory concerning the mechanism of the counter-current concentration of urine. Furthermore, the evolution of the specific radioactivity of urea {sup 14}C is favorable for a cortical synthesis of urea. (author) [French] II existe dans le rein de Merions en oligurie un gradient cortico papillaire de la concentration du sodium et de l'uree; ce gradient est aboli dans le rein de Merions en diurese osmotique ou de rats en diurese aqueuse; dans ces 3 etats de diurese il n'y a pas de difference significative de la concentration du potassium entre les regions superficielles et profondes des reins. Des etudes cinetiques a l'aide d'eau tritiee, d'uree {sup 14}C et de {sup 24}Na montrent que l'eau et l'uree des regions profondes des reins ne sont renouveles que tres lentement (1 h), alors que le sodium est tres vite renouvele (1 mn). Ces resultats s'expliquent par la theorie de WIRZ sur le mecanisme de la concentration de l'urine par contre-courant. En outre, l'evolution renale de la radioactivite specifique de l'uree {sup 14}C est en faveur d'une synthese corticale de l'uree. (auteur)

  8. Iodine-131 production by a dry method using reactor-irradiated elementary tellurium. Part 1 - Conditions for obtaining iodine emanation and its capture. Part 2 - comparative study of preparation conditions using Pyrex, stainless steel and alumina equipment. Part 3 - production on a semi-industrial scale; Production de l'iode 131 par voie seche a partir de tellure elementaire irradie a la pile. 1ere partie - Etudes des conditions pour obtenir l'emanation de l'iode et le capter. 2eme partie - Etude comparee des conditions pour effectuer cette preparation avec des appareils en Pyrex, en acier inoxydable et en alumine. 3eme partie - production a l'echelle semi-industrielle

    Energy Technology Data Exchange (ETDEWEB)

    Bardy, A; Beydon, J; Murthy, T S; Doyen, J B; Lefrancois, J [Commissariat a l' Energie Atomique, Saclay (France). Centre d' Etudes Nucleaires

    1967-04-15

    A previous report has described how iodine 131 can be prepared from elementary tellurium by a dry method which consists in treating irradiated tellurium at 400 degrees in argon. The possibility of carrying out this treatment in a stainless steel or alumina apparatus has been considered. The behavior of gaseous iodine 131 towards these materials has thus been studied. If the adsorption of iodine on stainless steel is superficial desorption is rapid at 250 degrees in oxygen or 400 degrees in argon. If the adsorption is chemical in nature it becomes necessary to heat to higher temperatures. Adsorption of iodine on alumina is very weak and the iodine can be desorbed rapidly. With these materials tests have been carried out on 300 gms of tellurium containing 41 curies of iodine 131; the yields were very satisfactory ( 98 per cent). (author) [French] La methode de preparation de l iode 131 par voie seche a partir de tellure elementaire decrite dans un precedent rapport consiste a traiter le tellure irradie a 400 degres sous argon. Nous avons examine la possibilite d effectuer ce traitement dans un appareil en acier inoxidable ou en alumine. Le comportement de l iode 131 gazeux vis a vis de ces materiaux a donc ete etudie. Si l adsorption de l iode sur l acier inoxidable est superficielle la desorption est rapide a 250 degres sous oxygene ou 400 degres sous argon. Si la fixation est de nature chimique il est necessaire de chauffer a des temperatures plus elevees. L adsorption de l iode sur l alumine est res faible et l iode peut etre desorbe rapideemnt. En employant ces materiaux des essais ont ete obtenus sur 300 g de tellure contenant 41 curies d iode 131 avec un bon rendement (98 pour cent). (auteur00.

  9. Vers une esthétique de la science-fiction

    Directory of Open Access Journals (Sweden)

    Joanna Russ

    2013-04-01

    Full Text Available Les outils critiques élaborés en ayant à l’esprit une littérature entièrement différente ne fonctionnent pas quand ils sont appliqués à la science-fiction. Dans cet article, j’avance la proposition suivante : que la science-fiction est didactique. Malgré des similitudes superficielles avec la fiction moderne naturaliste (ou autre, les personnages de science-fiction sont toujours des personnes collectives, jamais individuelles. Je suggère que la critique littéraire contemporaine n’est pas l’outil idéal pour traiter de fiction didactique, ou pour évaluer une littérature nouvelle et différente. Appliquer les critères et les méthodes auxquels nous sommes habitués ne peut mener qu’à trois résultats : le rejet de toute science-fiction comme n’étant pas de la littérature, une préférence pour certains genres restreints de science-fiction (parce qu’on peut les comprendre au moins partiellement selon la manière habituelle, ou une conception et une perception erronées des textes que l’on essaie précisément de comprendre. La troisième catégorie est restée rare jusqu’ici, parce que l’intérêt universitaire pour la science-fiction était rare, mais elle pourrait devenir bien trop courante si la popularité croissante des cours d’université sur ce sujet ne s’accompagne pas d’une critique adaptée à son sujet.

  10. Geochemistry and ore prospecting; Geochimie et prospection miniere

    Energy Technology Data Exchange (ETDEWEB)

    Le Caignec, R [Commissariat a l' Energie Atomique, Saclay(France). Centre d' Etudes Nucleaires

    1954-07-01

    Applied geochemistry is a new technique which helps the geologist in detecting ore deposits. Some deposits, even when they are covered with rather thick surface structures, form around these zones where the infinitesimal content of some elements of soils or waters is notably different. These 'anomalies' may be contemporaneous to the deposit-structure (primary dispersion) or may have occurred later (secondary dispersion). Various factors rule these anomalies: ore-stability, soil homogeneity, water conditions, topography, vegetation, etc... Applied geochemistry is in fact the study of analysis techniques of metal traces in soils as well as the geological interpretation of observed anomalies. This report gives practical data on sampling methods, yields, costs and also on special problems of uranium geochemistry. (author) [French] La geochimie appliquee est une nouvelle technique qui vient aider le geologue dans la detection des gisements de minerais. Certains gisements, meme lorsqu'ils sont recouverts par des formations superficielles relativement epaisses, creent autour d'eux des zones ou la teneur infinitesimale des sols ou des eaux en certains elements varie notablement. Ces ''anomalies'' peuvent etre contemporaines de la formation du gisement (dispersion primaire) ou posterieures a celle-ci (dispersion secondaire). De nombreux facteurs gouvernent ces anomalies: stabilite du minerai, homogeneite du sol, regime des eaux, topographie, vegetation, etc... L'etude des techniques d'analyse des traces de metaux dans les sols et l'interpretation geologique des anomalies obtenues constituent la geochimie appliquee. Ce rapport contient, en outre, des donnees pratiques sur les procedes d'echantillonnage, le rendement, les prix de revient, ainsi que sur quelques problemes particuliers a la geochimie de l'uranium. (auteur)

  11. Geochemistry and ore prospecting; Geochimie et prospection miniere

    Energy Technology Data Exchange (ETDEWEB)

    Le Caignec, R. [Commissariat a l' Energie Atomique, Saclay(France). Centre d' Etudes Nucleaires

    1954-07-01

    Applied geochemistry is a new technique which helps the geologist in detecting ore deposits. Some deposits, even when they are covered with rather thick surface structures, form around these zones where the infinitesimal content of some elements of soils or waters is notably different. These 'anomalies' may be contemporaneous to the deposit-structure (primary dispersion) or may have occurred later (secondary dispersion). Various factors rule these anomalies: ore-stability, soil homogeneity, water conditions, topography, vegetation, etc... Applied geochemistry is in fact the study of analysis techniques of metal traces in soils as well as the geological interpretation of observed anomalies. This report gives practical data on sampling methods, yields, costs and also on special problems of uranium geochemistry. (author) [French] La geochimie appliquee est une nouvelle technique qui vient aider le geologue dans la detection des gisements de minerais. Certains gisements, meme lorsqu'ils sont recouverts par des formations superficielles relativement epaisses, creent autour d'eux des zones ou la teneur infinitesimale des sols ou des eaux en certains elements varie notablement. Ces ''anomalies'' peuvent etre contemporaines de la formation du gisement (dispersion primaire) ou posterieures a celle-ci (dispersion secondaire). De nombreux facteurs gouvernent ces anomalies: stabilite du minerai, homogeneite du sol, regime des eaux, topographie, vegetation, etc... L'etude des techniques d'analyse des traces de metaux dans les sols et l'interpretation geologique des anomalies obtenues constituent la geochimie appliquee. Ce rapport contient, en outre, des donnees pratiques sur les procedes d'echantillonnage, le rendement, les prix de revient, ainsi que sur quelques problemes particuliers a la geochimie de l'uranium. (auteur)

  12. DICTIONARIES LOOKUP STRATEGIES FOR ARABIC-ENGLISH TRANSLATION

    Directory of Open Access Journals (Sweden)

    Mohammad Ahmad THAWABTEH

    2013-05-01

    Full Text Available Bien que les dictionnaires ont une extrême importance dans les cours de traduction, on les considère comme une épée à double tranchant. De grotesques traductions indiquent clairement que les étudiants utilisent les dictionnaires d’une façon incompétente. Les données de cette étude représentent les traductions d’une vingtaine d’étudiants de premier cycle d’Anglais à l’université d’Al-quds et l’université de Palestine Ahlyia. Les traductions sont examinées à base des stratégies de recherche en dictionnaire. Les résultats de l’étude montrent que les pièges des sorties de la traduction que les étudiants font sont dus au: 1-la dépendance exagérée du dictionnaire. 2- le manque de compréhension du texte dans sa langue d’origine. 3- le fait de choisir le premier sens. 4- le peu d’attention accordée aux exemples illustrés dans le dictionnaire. 5- favoriser la lecture attentive sur la lecture superficielle. 6- ignorance totale des collocations, des idioms, et des proverbes. 7- l’incapacité de comprendre le sens du contexte. Quelques stratégies de recherche dans le dictionnaire qui peuvent aider les étudiants de traduction à effectuer leur travail rapidement et efficacement sont proposées.

  13. Spectral content of seismic movements produced by underground nuclear explosions; Contenu spectral des mouvements seismiques dus aux explosions nucleaires souterraines

    Energy Technology Data Exchange (ETDEWEB)

    Albaret, A; Duclaux, F [Commissariat a l' Energie Atomique, Bruyeres-le-Chatel (France). Centre d' Etudes

    1969-07-01

    After a summary of available data, both theoretical and experimental, concerning the spectral content of seismic movements, a description is given of the experiments carried out during the French nuclear explosions in the Sahara, and of the results obtained on the volume waves. A comparison is then made with certain American results. A new method is described for studying the amplitude spectra; it has made it possible to show that the amount of low frequencies in the spectrum increases with the power of the explosion, and decreases with the distance to the zero point and with the filtering effect of the weathered zone. A calculation is then made of the low cut-off ground filter, this giving a better representation of the initial seismic phenomenon. (authors) [French] Apres avoir resume les connaissances disponibles, aussi bien theoriques qu'experimentales, sur le contenu spectral des mouvements seismiques, on decrit les experiences effectuees a l'occasion des explosions nucleaires francaises du Sahara et les resultats obtenus sur les ondes de volume. Puis on les compare avec certains resultats americains. On decrit une nouvelle methode d'etude des spectres d'amplitudes qui montre que le spectre est d'autant plus riche en basses frequences que la puissance de l'explosion est grande, que la distance au point zero est faible et qu'il est moins filtre par la zone alteree superficielle. Puis on calcule le filtre terrain coupe-bas qui permet de donner une representation plus fidele du phenomene seismique initial. (auteurs)

  14. Polarographic determination of uranium dioxide stoichiometry; La determination polarographique de la stoechiometrie du dioxyde d'uranium

    Energy Technology Data Exchange (ETDEWEB)

    Viguie, J.; Uny, G. [Commissariat a l' Energie Atomique, Centre d' Etudes Nucleaires de Grenoble, 38 (France)

    1966-10-01

    The method described allows the determination of small deviations from stoichiometry for uranium dioxide. It was applied to the study of surface oxidation of bulk samples. The sample is dissolved in phosphoric acid under an argon atmosphere; U(VI) is determined by polarography in PO{sub 4}H{sub 3} 4.5 N - H{sub 2}SO{sub 4} 4 N. U(IV) is determined by potentiometry. The detection limit is UO{sub 2,0002}. The accuracy for a single determination at the 95% confidence level is {+-}20 per cent for samples with composition included between UO{sub 2,001} and UO{sub 2,01}. (authors) [French] La methode decrite permet de determiner les faibles ecarts a la stoechiometrie du dioxyde d'uranium. Elle a ete appliquee a l'etude de l'oxydation superficielle des echantillons. La mise en solution s'effectue dans l'acide phosphorique concentre sous atmosphere d'argon; U(VI) est dose par polarographie dans le milieu PO{sub 4}H{sub 3} 4,5 N et H{sub 2}SO{sub 4} 4 N; U(IV) est dose par potentiometrie. La limite de detection est UO{sub 2,0002}. La precision obtenue pour une determination au taux de certitude 0,95 est de l'ordre de 20 pour cent pour des echantillons dont la teneur est comprise entre UO{sub 2,001} et UO{sub 2,01}. (auteurs)

  15. AVALIAÇÃO DO POTENCIAL TERAPÊUTICO DE BACCHARIS DRACUNCULIFOLIA E PEUMUS BOLDUS NO PROCESSO FIBROSANTE HEPÁTICO

    Directory of Open Access Journals (Sweden)

    Caio Mateus da Silva

    2017-03-01

    Full Text Available As doenças do fígado são consideradas um problema de saúde mundial e ainda terapias eficientes não foram desenvolvidas para a maioria dessas patologias. Mais especificamente, a fibrose hepática é característica comum do início de várias das anomalias no órgão. Dentro desse contexto, a busca por novos fármacos é necessária e o uso de plantas medicinais tem ganhado destaque. Neste trabalho, foi avaliado os efeitos dos extratos de Baccharis dracunculifolia e Peumus boldus em culturas de células hepáticas estreladas LX-2 em relação as alterações na morfologia dos filamentos de actina do citoesqueleto e no núcleo celular em ensaios de microscopia e na avaliação do padrão de expressão de genes relacionados à processos de apoptose e marcadores clássicos da fibrose hepática. Nos resultados, para as concentrações testadas o extrato de P. boldus altera o perfil de expressão de genes relacionados a apoptose causando maior estresse celular e quebrando a homeostase celular quando observados os filamentos de actina. Enquanto que a planta B. dracunculifolia apresentou uma grande melhora na reorganização do citoesqueleto e diminuição do estresse celular.

  16. Successful treatment of pediatric IgG4 related systemic disease with mycophenolate mofetil: case report and a review of the pediatric autoimmune pancreatitis literature

    Directory of Open Access Journals (Sweden)

    Cron Randy Q

    2011-01-01

    Full Text Available Abstract Autoimmune pancreatitis is frequently associated with elevated serum and tissue IgG4 levels in the adult population, but there are few reports of pediatric autoimmune pancreatitis, and even fewer reports of IgG4 related systemic disease in a pediatric population. The standard of care treatment in adults is systemic corticosteroids with resolution of symptoms in most cases; however, multiple courses of corticosteroids are occasionally required and some patients require long term corticosteroids. In these instances, steroid sparing disease modify treatments are in demand. We describe a 13-year-old girl with IgG4 related systemic disease who presented with chronic recurrent autoimmune pancreatitis resulting in surgical intervention for obstructive hyperbilirubinemia and chronic corticosteroid treatment. In addition, she developed fibrosing medianstinitis as part of her IgG4 related systemic disease. She was eventually successfully treated with mycophenolate mofetil allowing for discontinuation of corticosteroids. This is the first reported use of mycophenolate mofetil for IgG4 related pancreatitis. Although autoimmune pancreatitis as part of IgG4 related systemic disease is rarely reported in pediatrics, autoimmune pancreatitis is also characterized as idiopathic fibrosing pancreatitis. All pediatric autoimmune pancreatitis cases reported in the world medical literature were identified via a PUBMED search and are reviewed herein. Twelve reports of pediatric autoimmune pancreatitis were identified, most of which were treated with corticosteroids or surgical approaches. Most case reports failed to report IgG4 levels, so it remains unclear how commonly IgG4 related autoimmune pancreatitis occurs during childhood. Increased evaluation of IgG4 levels in patients with autoimmune pancreatitis may shed further light on the association of IgG4 with pancreatitis and the underlying pathophysiology.

  17. The antiviral drug tenofovir, an inhibitor of Pannexin-1-mediated ATP release, prevents liver and skin fibrosis by downregulating adenosine levels in the liver and skin.

    Directory of Open Access Journals (Sweden)

    Jessica L Feig

    Full Text Available Fibrosing diseases are a leading cause of morbidity and mortality worldwide and, therefore, there is a need for safe and effective antifibrotic therapies. Adenosine, generated extracellularly by the dephosphorylation of adenine nucleotides, ligates specific receptors which play a critical role in development of hepatic and dermal fibrosis. Results of recent clinical trials indicate that tenofovir, a widely used antiviral agent, reverses hepatic fibrosis/cirrhosis in patients with chronic hepatitis B infection. Belonging to the class of acyclic nucleoside phosphonates, tenofovir is an analogue of AMP. We tested the hypothesis that tenofovir has direct antifibrotic effects in vivo by interfering with adenosine pathways of fibrosis using two distinct models of adenosine and A2AR-mediated fibrosis.Thioacetamide (100mg/kg IP-treated mice were treated with vehicle, or tenofovir (75mg/kg, SubQ (n = 5-10. Bleomycin (0.25U, SubQ-treated mice were treated with vehicle or tenofovir (75mg/kg, IP (n = 5-10. Adenosine levels were determined by HPLC, and ATP release was quantitated as luciferase-dependent bioluminescence. Skin breaking strength was analysed and H&E and picrosirus red-stained slides were imaged. Pannexin-1expression was knocked down following retroviral-mediated expression of of Pannexin-1-specific or scrambled siRNA.Treatment of mice with tenofovir diminished adenosine release from the skin of bleomycin-treated mice and the liver of thioacetamide-treated mice, models of diffuse skin fibrosis and hepatic cirrhosis, respectively. More importantly, tenofovir treatment diminished skin and liver fibrosis in these models. Tenofovir diminished extracellular adenosine concentrations by inhibiting, in a dose-dependent fashion, cellular ATP release but not in cells lacking Pannexin-1.These studies suggest that tenofovir, a widely used antiviral agent, could be useful in the treatment of fibrosing diseases.

  18. Pleuroparenchymal fibroelastosis: report of two cases in Brazil.

    Science.gov (United States)

    Gomes, Paula Silva; Shiang, Christina; Szarf, Gilberto; Coletta, Ester Nei Aparecida Martins; Pereira, Carlos Alberto de Castro

    2017-01-01

    Pleuroparenchymal fibroelastosis (PPFE) is a rare lung disease. It can be idiopathic or associated with any one of various conditions. To our knowledge, this is the first report of two cases of PPFE in Brazil. Our first patient presented with pleural and subpleural fibrosis in the upper lobes; a spiculated nodule in the left upper lobe; and a mild reticular pattern in the lower lobes. Surgical lung biopsy revealed PPFE in the upper lobes, including the nodule, and unclassified interstitial pneumonia in the left lower lobe. Our second patient had a history of exposure to domestic birds, indicating a risk of hypersensitivity pneumonitis, and presented with advanced lung disease, predominantly in the upper lobes, together with subpleural fibrosis.That patient underwent lung transplantation. In the explant specimen, PPFE and granulomas were identified, suggesting hypersensitivity pneumonitis as an associated cause. RESUMO A fibroelastose pleuroparenquimatosa (FEPP) é uma doença pulmonar rara, podendo ser idiopática ou associada a diversas condições. Pelo que sabemos, este é o primeiro relato de dois casos de FEPP no Brasil. Nosso primeiro paciente apresentava fibrose pleural e subpleural nos lobos superiores, um nódulo espiculado no lobo superior esquerdo e um padrão reticular discreto nos lobos inferiores. A biópsia pulmonar cirúrgica demonstrou FEPP nos lobos superiores, incluindo no nódulo, e pneumonia intersticial não classificada no lobo inferior esquerdo. Nosso segundo paciente tinha história de exposição a aves domésticas, indicando um risco de pneumonite de hipersensibilidade, e doença pulmonar avançada predominando em lobos superiores, com fibrose subpleural. Esse paciente foi submetido a transplante pulmonar. No espécime do explante, FEPP e granulomas foram identificados, sugerindo pneumonite de hipersensibilidade como causa associada.

  19. Esclerose sistêmica difusa com hipertensão pulmonar isolada: relato de caso Diffuse systemic sclerosis with isolated pulmonary hypertension: a case report

    Directory of Open Access Journals (Sweden)

    SILMÉIA GARCIA ZANATI

    2000-12-01

    Full Text Available A esclerose sistêmica (ES é uma doença incomum, caracterizada por alterações vasculares e fibrose em pele e vários órgãos. O envolvimento pulmonar é freqüente na ES; entretanto, hipertensão pulmonar (HP na ausência de fibrose intersticial é raramente observada. Os autores descrevem um caso de ES difusa com HP isolada em paciente do sexo feminino, de 49 anos. O diagnóstico de HP pôde ser sugerido pelo exame físico e confirmado por ecodopplercardiograma, que permitiu estimar a pressão sistólica em artéria pulmonar em 80mmHg. Tratamento foi iniciado com nifedipina; entretanto, devido a efeitos colaterais da droga, não foi possível o aumento da dose além de 30mg ao dia; nesta dosagem, a nifedipina não acarretou redução da pressão arterial pulmonar.Systemic sclerosis (SS is an uncommon disease characterized by small blood vessel vasculopathy and increased connective tissue in the skin and in other organs. The pulmonary involvement is common in SS; however, pulmonary hypertension without interstitial fibrosis is rare. The authors present a case of the diffuse form of SS with isolated pulmonary hypertension. The diagnosis of pulmonary hypertension was suggested by physical examination and confirmed by doppler-echocardiography which allowed for the estimation of the pulmonary artery systolic pressure in 80 mmHg. The authors started treatment with nifedipine; however, as the patient presented side effects, it was not possible to increase the dose beyond 30 mg/day. In this dosage, nifedipine did not decrease the pulmonary arterial pressure.

  20. Atualização na etiopatogênese da esclerose sistêmica

    Directory of Open Access Journals (Sweden)

    Adriana Fontes Zimmermann

    2013-12-01

    Full Text Available A Esclerose Sistêmica (ES é uma doença autoimune de etiologia multifatorial, desencadeada pela combinação de fatores genéticos e ambientais. Sua variada expressão clínica resulta da complexa interação fisiopatogênica de três elementos principais: a vasculopatia proliferativa, a desregulação imunológica e a deposição e remodelamento anormais da matriz extracelular (MEC, da qual resulta a fibrose característica da doença. Eventos fisiopatogênicos precoces parecem ser a lesão endotelial e o desequilíbrio no reparo vascular, com a ativação de células endoteliais, do sistema imune e das plaquetas, com a liberação de múltiplos mediadores, como as citocinas proinflamatórias TH2 e os fatores de crescimento, desencadeando uma sequência de eventos simultâneos ou em cascata que envolve diversas vias de sinalização intracelular. O resultado mais importante desses eventos é a hiperativação dos fibroblastos, as principais células efetoras da fibrose, as quais passam a produzir grandes quantidades de constituintes da MEC e a secretar múltiplos fatores de crescimento e citocinas que perpetuam o processo. Neste artigo apresentamos uma revisão dos principais fatores potencialmente implicados na etiologia da ES e revisitamos os conhecimentos atuais sobre os mais importantes mecanismos envolvidos no desenvolvimento das lesões características da doença. O melhor entendimento desses mecanismos fisiopatogênicos possibilita identificar potenciais alvos terapêuticos, o que pode resultar em avanços no manejo dessa complexa e debilitante doença.

  1. Nephrogenic systemic fibrosis associated with gadolinium based contrast agents: A summary of the medical literature reporting

    International Nuclear Information System (INIS)

    Broome, Dale R.

    2008-01-01

    Nephrogenic systemic fibrosis (NSF) is a systemic fibrosing disorder that principally affects the skin, but can involve virtually any tissue in the human body and result in significant disability and even death. Since 2006 numerous retrospective case reports and case series have reported a very strong association of this disease with exposure to gadolinium-based contrast agents (Gd-CA) for MR imaging in the setting of severe or end-stage renal disease. The purpose of this report is to summarize the medical literature reporting of biopsy-proven NSF cases in which the authors specifically investigated patient exposure to Gd-CA. A Pub Med MEDLINE search was performed using the key words-nephrogenic systemic fibrosis and nephrogenic fibrosing dermopathy. All case reports and case series of NSF were reviewed to determine if patients had a preceding exposure to Gd-CA and which specific Gd-CA was involved. If the original reports did not clarify the specific Gd-CA, I reviewed follow-up letters to the editors or contacted the authors to clarify which specific Gd-CA were linked to the NSF cases. If several reports originated from the same institution, clarification was also obtained to avoid redundant reporting. As of February 1, 2008 there have been 190 biopsy-proven cases of NSF published in the peer-reviewed literature with the following associations: 157 gadodiamide (Omniscan, GE Healthcare), 8 gadopentetate (Magnevist, Bayer Healthcare), 3 gadoversetamide (OptiMARK, Covidien), and 18 unspecified Gd-CA, and 4 confounded cases with more than one Gd-CA. Five cases of NSF were unassociated with Gd-CA

  2. STAT4 is a genetic risk factor for systemic sclerosis having additive effects with IRF5 on disease susceptibility and related pulmonary fibrosis.

    Science.gov (United States)

    Dieudé, P; Guedj, M; Wipff, J; Ruiz, B; Hachulla, E; Diot, E; Granel, B; Sibilia, J; Tiev, K; Mouthon, L; Cracowski, J L; Carpentier, P H; Amoura, Z; Fajardy, I; Avouac, J; Meyer, O; Kahan, A; Boileau, C; Allanore, Y

    2009-08-01

    Systemic sclerosis (SSc) belongs to the group of connective tissue disorders (CTDs), among which are several disorders characterized by a type I interferon (IFN) signature. The recent identification of an association between IRF5 and SSc further highlights a key role for IFN. STAT4, which encodes STAT-4, contributes to IFN signaling, and its genetic variants were found to be associated with CTDs. The aim of this study was to determine whether the STAT4 rs7574865 single-nucleotide polymorphism is associated with SSc, and whether it interacts with IRF5. Both the STAT4 rs7574865 and IRF5 rs2004640 polymorphisms were genotyped in 1,855 individuals of French Caucasian origin comprising a discovery set of 440 patients with SSc and 485 control subjects and a replication set of 445 patients with SSc and an additional 485 control subjects. STAT4 rs7574865 was shown to be associated with SSc (P=0.001, odds ratio [OR] 1.29, 95% confidence interval [95% CI] 1.11-1.51). This association was not restricted to a particular phenotype. An additive effect of the STAT4 rs7574865 T allele and the IRF5 rs2004640 T allele was observed, resulting in a multiple increased 1.28-fold risk of SSc. The OR for SSc was 2.72 (95% CI 1.86-3.99) for combinations of genotypes with >or=3 risk alleles. An additive effect was also detected for fibrosing alveolitis: carriage of at least 3 risk alleles appeared to be an independent risk factor (P=2.2x10(-4), OR 1.97, 95% CI 1.28-3.04). Our results establish STAT4 rs7574865 as a new SSc genetic susceptibility factor. STAT4 and IRF5 act with additive effects in terms of susceptibility to both SSc and SSc-related fibrosing alveolitis.

  3. Effects of iloprost on bleomycin-induced pulmonary fibrosis in rats compared with methyl-prednisolone

    Directory of Open Access Journals (Sweden)

    Z.A. Aytemur

    2012-11-01

    Full Text Available Objective: Prostacyclin (PGI2 has been shown to inhibit the expression of pro-inflammatory and pro-fibrotic mediators in pulmonary fibrosis. In this study, we aimed to test the preventive effects of intraperitoneally administered iloprost, a stable PGI2 analog, on bleomycin-induced pulmonary fibrosis in rats and to compare the effects of iloprost with the effects of methyl-prednisolone, a traditional therapy. Methods: Rats were randomly allocated into four groups: 1. Saline alone (n = 6; 2. Bleomycin + placebo (n = 7; 3. Bleomycin + methyl-prednisolone (n = 7; 4. Bleomycin + iloprost (n = 7. Fibrotic changes in the lungs were demonstrated by analyzing the cellular composition of bronchoalveolar lavage fluid, histological evaluation and lung hydroxyproline content. Results: Fibrosis was made in the lungs of rats by bleomycin experimentally. Fibrosis scores in the methyl-prednisolone and the iloprost groups were significantly lower than in the placebo group (p < 0.05. Furthermore, the score of the iloprost group was significantly lower than the score of the methyl-prednisolone group. The hydroxyproline content was significantly less in the methyl-prednisolone and the iloprost groups (p < 0.05. In the placebo group, the neutrophil percentage in bronchoalveolar lavage was significantly higher than in the other groups, whereas the macrophage percentage in placebo group was significantly lower (p < 0.05. Conclusion: Iloprost has protective effect on the pulmonary fibrosis induced by bleomycin and it may be more effective in decreasing fibrotic changes than methyl-prednisolone. Resumo: Objetivo: A prostaciclina (PGI2 é conhecida por inibir a expressão de mediadores pró-inflamatórios e pró-fibróticos na fibrose pulmonar. Neste estudo, procurou-se testar os efeitos preventivos do iloprost administrado por via intraperitoneal, um análogo estável do PGI2, na fibrose

  4. Endomiocardiofibrose: resultados do tratamento cirúrgico com conservação das valvas atrioventriculares Endomyocardial fibrosis: results of the surgical treatment with atrioventricular valve preservation

    Directory of Open Access Journals (Sweden)

    Sérgio Almeida de Oliveira

    1991-04-01

    Full Text Available Entre abril de 1988 e janeiro de 1991, foram operados 25 pacientes consecutivos, para correção de endomiocardiofibrose, com técnica conservadora das valvas atrioventriculares. Dezenove pacientes eram do sexo feminino e seis do masculino. As idades variaram de 11 a 59 anos, com média de 40,6 anos. Dezesete pacientes apresentavam acometimento biventricular, seis com comprometimento do ventrículo esquerdo e apenas dois pacientes com lesão isolada do ventrículo direito. Todos estavam em grupos avançados de insuficiência cardíaca, sendo 19 em grupo IV da NYHA e seis no grupo III. No pré-operatório, 24 apresentavam insuficiência da valva tricúspide e 22 apresentavam insuficiência da valva mitral. Todos os pacientes foram operados com o auxílio de circulação extracorpórea e hipotermia sistêmica de 28ºC e pinçamento aórtico intermitente, para melhor exposição das cavidades ventriculares. A fibrose ventricular direita foi sempre ressecada através da valva tricúspide, enquanto que a fibrose do ventrículo esquerdo foi removida através de pequena ventriculotomia esquerda apical. Em todos os pacientes com insuficiência tricúspide, foi feita plástica anular do tipo DeVega. Dos pacientes com insuficiência mitral, sete tiveram correção espontânea após a ressecção da fibrose, e os outros 15 receberam anuloplastia. Houve apenas um (4% óbito no pós-operatório imediato e nenhum óbito tardio. A recuperação funcional foi boa, estando atualmente 16 pacientes em grupo funcional I e 8 em grupo II.Between April 1988 and January 1991 we operated on 25 consecutive patients with endomyocardial fibrosis (EMF with an approach to preserve the atrioventricular valves. Nineteen patients were females and six males. Their ages ranged from 11 to 5 years, with an average of 40.6 years. Seventeen patients had biventricular involvement, six had involvement of the left ventricle and only two patients had an isolated lesion of the right

  5. Rinite crônica em portadores do HTLV-1: estudo histopatológico Chronic rhinitis in HTLV-1 carriers: a histopathologic study

    Directory of Open Access Journals (Sweden)

    Fernando P. Gaspar Sobrinho

    2012-04-01

    Full Text Available A histopatologia nasal de portadores do HTLV-1 com rinite crônica é desconhecida. OBJETIVO: Descrever aspectos histopatológicos de portadores do HTLV-1 com rinite crônica. CASUÍSTICA E MÉTODOS: Amostras de mucosa nasal de 10 portadores do HTLV-1 com rinite crônica, sendo oito com rinite alérgica e dois com rinite não alérgica, foram estudadas por microscopia de luz. Amostras de 10 pacientes com rinite alérgica não infectados pelo HTLV-1 serviram como controle. RESULTADOS: Fibrose subepitelial foi maior nos pacientes com rinite alérgica infectados pelo HTLV-1 (p=0,01, enquanto o espessamento da membrana basal foi maior nos controles (p=0,03. Houve tendência a menor eosinofilia e edema entre os infectados pelo HTLV-1, sem significância estatística (p=0,2. Para o infiltrado linfocítico, não houve diferença entre os pacientes com rinite alérgica infectados e não infectados (p=1,0. Fibrose subepitelial com infiltrado linfocítico de intensidade leve a moderada foram os achados encontrados nos dois portadores do HTLV-1 com rinite não alérgica. CONCLUSÕES: O estudo sugere que a infecção pelo HTLV-1 pode modificar a histopatologia da rinite alérgica, sobretudo por maior fibrose, e pode estar relacionada a uma rinite crônica não alérgica com infiltrado linfocítico.The nasal histopathology of HTLV-1 carriers with chronic rhinitis is unknown. OBJECTIVE: To describe the histopathological features of HTLV-1 carriers with chronic rhinitis. MATERIALS AND METHODS: Biopsies of nasal mucosa of ten HTLV-1 carriers with chronic rhinitis (eight patients with allergic rhinitis and two patients with non-allergic rhinitis were studied using a light microscope. Samples from ten patients with allergic rhinitis not infected with HTLV-1 were used as controls. RESULTS: Subepithelial fibrosis was more pronounced in patients with allergic rhinitis infected with HTLV-1 (p=0.01, while the basement membrane thickness was greater in controls (p=0

  6. According to Hepatitis C Virus (HCV) Infection Stage, Interleukin-7 Plus 4-1BB Triggering Alone or Combined with PD-1 Blockade Increases TRAF1low HCV-Specific CD8+ Cell Reactivity.

    Science.gov (United States)

    Moreno-Cubero, Elia; Subirá, Dolores; Sanz-de-Villalobos, Eduardo; Parra-Cid, Trinidad; Madejón, Antonio; Miquel, Joaquín; Olveira, Antonio; González-Praetorius, Alejandro; García-Samaniego, Javier; Larrubia, Juan-Ramón

    2018-01-15

    Hepatitis C virus (HCV)-specific CD8 + T cells suffer a progressive exhaustion during persistent infection (PI) with HCV. This process could involve the positive immune checkpoint 4-1BB/4-1BBL through the loss of its signal transducer, TRAF1. To address this issue, peripheral HCV-specific CD8 + T cells (pentamer-positive [pentamer + ]/CD8 + T cells) from patients with PI and resolved infection (RI) after treatment were studied. The duration of HCV infection and the liver fibrosis progression rate inversely correlated with the likelihood of detection of peripheral pentamer + /CD8 + cells. In PI, pentamer + /CD8 + cells had impaired antigen-specific reactivity that worsened when these cells were not detectable ex vivo Short/midduration PI was characterized by detectable peripheral PD-1 + CD127 low TRAF1 low cells. After triggering of T cell receptors (TCR), the TRAF1 level positively correlated with the levels of CD127, Mcl-1, and CD107a expression and proliferation intensity but negatively with PD-1 expression, linking TRAF1 low to exhaustion. In vitro treatment with interleukin-7 (IL-7) upregulated TRAF1 expression, while treatment with transforming growth factor-β1 (TGF-β1) did the opposite, suggesting that the IL-7/TGF-β1 balance, besides TCR stimulation, could be involved in TRAF1 regulation. In fact, the serum TGF-β1 concentration was higher in patients with PI than in patients with RI, and it negatively correlated with TRAF1 expression. In line with IL-7 increasing the level of TRAF1 expression, IL-7 plus 4-1BBL treatment in vitro enhanced T cell reactivity in patients with short/midduration infection. However, in patients with long-lasting PI, anti-PD-L1, in addition to the combination of IL-7 and 4-1BBL, was necessary to reestablish T cell proliferation in individuals with slowly progressing liver fibrosis (slow fibrosers) but had no effect in rapid fibrosers. In conclusion, a peripheral hyporeactive TRAF1 low HCV-specific CD8 + T cell response

  7. Fístula pancreática após duodenopancreatectomia: correlação dos aspectos intra-operatórios e histologicos do pâncreas

    Directory of Open Access Journals (Sweden)

    Fernando Leal Pereira

    Full Text Available OBJETIVO: Avaliar a relação entre a ocorrência de fístula pancreática pós-anastomose pancreatojejunal, em doentes submetidos à duodenopancreatectomia por neoplasia maligna periampolar, com aspectos histológicos de fibrose e inflamação encontrados no tecido pancreático e com o calibre do ducto pancreático principal. MÉTODOS: Estudo retrospectivo interessando doentes que foram submetidos ao tratamento com ressecção cirúrgica. Verificou-se o índice de fístulas pancreáticas encontradas. Classificou-se de acordo com a histologia da fibrose e da inflamação pancreática. RESULTADOS: Identificaram-se 77 doentes, com média de idade de 57,6 anos, sendo 62,4% do sexo masculino. De acordo com o tipo de operação realizada, 66,3% constituíram-se em gastroduodenopancreatectomia e 33,7% em duodenopancreatectomia com preservação do piloro. Em relação ao número de fístulas diagnosticadas, identificou-se que 23,4% doentes apresentaram tal complicação pós-operatória, sendo que em 66,7% a causa era neoplasia de papila. Achados macroscópicos intraoperatórios, identificou-se classificação da textura pancreática, tida como normal, em 85,8% e, quanto ao calibre do ducto principal pancreático, foi encontrada média de 4,9mm. Houve relação importante entre a consistência endurecida do coto pancreático e a ausência da fístula. Nos doentes com tecido normal ou amolecido, o índice de fístula foi 25,4%. Quanto ao diâmetro ductal, identificou-se (p <0,05 maior número de deiscências da anastomose pancreática na ausência de dilatação ductal. Evidenciou-se que doentes com valores médios do diâmetro de ducto de 5,4mm (76,7% não mostraram esta complicação. CONCLUSÃO: A presença de fibrose e de dilatação ductal habitualmente coexistem e estão relacionadas à menor porcentagem de fístulas enteropancreáticas.

  8. Nonalcoholic fatty liver disease: histopathological evaluation and interobserver agreement Doença hepática gordurosa não-alcoólica: avaliação histopatológica e concordância interobservador

    Directory of Open Access Journals (Sweden)

    Cynthia Koeppel Berenstein

    2008-02-01

    Full Text Available BACKGROUND: Nonalcoholic fatty liver disease comprises a spectrum of lesions ranging from steatosis to cirrhosis, with nonalcoholic steatohepatitis being the progressive form of the disease. Alcohol intake, viral hepatitis and other liver diseases must be excluded. Liver biopsy is the gold standard for diagnosis of the disease and is the only method able to differentiate nonalcoholic steatohepatitis from simple steatosis, to grade inflammation and to stage fibrosis. AIMS: To analyze the histopathological findings and evaluate interobserver agreement in biopsies previously diagnosed as steatosis or steatohepatitis. METHODS: Seventy needle biopsies were analyzed according to Brunt et al.(4, with modifications in the grading and staging components. Clinical data of patients were collected. Interobserver agreement was calculated based on histopathological findings. RESULTS: Mild nonalcoholic steatohepatitis (grade 1 was the most common form. If fibrosis was detected, stage 1 was the most frequent. Interobserver agreement was very good for macrovesicular steatosis (K W = 0,82 and good for lobular inflammation (K W = 0,68 and fibrosis (K W = 0,73. CONCLUSIONS: The classification of Brunt et al., with modifications, can be applied to diagnosis not only of nonalcoholic steatohepatitis but also of nonalcoholic fatty liver disease, representing a reliable method for use in the daily practice of pathologists.INTRODUÇÃO: A doença hepática gordurosa não-alcoólica compreende um espectro de lesões que variam da esteatose à cirrose, sendo a esteatoepatite não-alcoólica a forma progressiva da doença. Uso de álcool, hepatites virais e outras doenças hepáticas devem ser excluídos. A biópsia hepática é o padrão-ouro para o diagnóstico da doença, sendo o único método capaz de diferenciar a esteatoepatite da esteatose, graduar a inflamação e estadiar a fibrose. OBJETIVOS: Analisar os achados histopatológicos e avaliar a concord

  9. Early morphological and histochemical alterations in rats subjected to ileocystoplasty Alterações morfológicas e histoquímicas precoces em ratos submetidos à ileocistoplastia

    Directory of Open Access Journals (Sweden)

    Edinaldo Gonçalves de Miranda

    2009-10-01

    I (controle biópsia intestinal no momento da cirurgia; Grupo II - anastomose íleo-ileal; Grupo III - anastomose íleo-vesical e Grupo IV - segmento intestinal da neobexiga. Os parâmetros avaliados foram: displasia, metaplasia, processo inflamatório agudo e crônico, fibrose, atrofia, hipertrofia, conteúdo total de mucinas, sialomucinas e sulfomucinas. Utilizou-se os testes não-paramétricos de Wilcoxon e Mann-Whitney para estudo estatístico. RESULTADOS: Não houve displasia. Processo inflamatório agudo e atrofia ocorreram nos grupos II, III e IV, sem significância estatística. Metaplasia com significância estatística ocorreu somente no grupo III (p=0.012. Processo inflamatório crônico, fibrose e hipertrofia foram significantes nos grupos II, III e IV. Observou-se aumento significante no conteúdo total de mucinas no grupo IV (p=0.014 e redução no grupo III (p=0.013. Aumento significante de sialomucinas foi observado nos grupos III (p=0.003 e IV (p=0.002 e redução significante das sulfomucinas nos grupos III (p=0.013 e IV (p=0.008. CONCLUSÃO: Nas ileocistoplastias em ratos fêmea observou-se metaplasia escamosa, processo inflamatório crônico, fibrose, hipertrofia, aumento do conteúdo de sialomucinas, redução das sulfomucinas e alterações no conteúdo total de mucinas com significância estatística, bem como atrofia e processo inflamatório agudo em menor intensidade.

  10. Chronic myocardial damage in experimental T. cruzi infection of a new world primate, Cebus sp. monkey Lesões miocárdicas crônicas na infecção experimental pelo T. cruzi no macaco (Cebus apella

    Directory of Open Access Journals (Sweden)

    Carlos Alberto Falasca

    1990-06-01

    áficos foram anteriormente publicados15 e são semelhantes àqueles vistos no homem. O achado eletrocardiográfico mais freqüente foi o bloqueio do ramo direito. Seis animais, escolhidos ao acaso, foram sacrificados. Aqueles sacrificados 20 a 25 meses após a primeira inoculação mostraram acúmulos focais de leucócitos com miocitólise. Focos de fibrose intersticial difusa com pequeno infiltrado de leucócitos entre as fibras foram observados em animais sacrificados 36 a 47 meses após a inoculação. Não foram encontrados parasitas. As lesões foram mais proeminentes nas paredes ventriculares e no septo. O achado de infiltrados predominantemente, nos animais sacrificados em tempo mais curto em relação à primeira inoculação e a fibrose mais severa naqueles sacrificados após um tempo maior sugere que existe uma progressão das lesões infiltrativas até a fibrose, com atividade leucocítica indicativa de fase crônica. Estas lesões são semelhantes àquelas descritas na doença de Chagas crônica humana. Este modelo, portanto, é útil na avaliação do progresso e conhecimento da patogênese da doença, assim como de sua imunologia, imunogenesis e da ação da quimioterapia, tanto na sua fase crônica como indeterminada.

  11. Intoxicação natural por Senecio brasiliensis em equinos

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    Welden Panziera

    Full Text Available RESUMO: Intoxicações por plantas do gênero Senecio representam uma importante causa de morte em animais de produção, sendo Senecio brasiliensis a espécie mais envolvida nos casos de intoxicação espontânea. Nesse trabalho, são descritos os aspectos epidemiológicos, clínicos e anatomopatológicos de um surto de intoxicação natural por Senecio brasiliensis em equinos. Dois equinos, de um total de dezoito, foram afetados. Os casos ocorreram em uma propriedade rural no município de São Martinho da Serra, Rio Grande do Sul, Brasil. Grande quantidade de exemplares de Senecio brasiliensis em estágio de brotação foi encontrada na área onde os equinos estavam. Os animais eram da raça Crioula, sendo uma égua com quatro anos e um potro com cinco meses. A égua apresentou evolução aguda da doença com duração de três dias e o potro evolução crônica de dois meses. As principais manifestações clínicas observadas em ambos os equinos incluíam apatia, anorexia, emagrecimento, desidratação e sinais neurológicos. Além disso, havia moderado edema subcutâneo na região ventral do abdômen e marcada icterícia nas mucosas ocular, oral e vulvar da égua e palidez das mucosas no potro. Na avaliação bioquímica do potro, havia acentuado aumento da atividade sérica da gamaglutamil transferase (119 U/L, aumento da fosfatase alcalina (434 U/L e hipoalbuminemia (2,1g/dL. No hemograma do mesmo, observou-se leve anemia normocítica e normocrômica (30% de hematócrito; VCM: 39,5 fL; HCM: 15,3pg. As alterações presentes em ambas as necropsias foram observadas principalmente no fígado, que estava difusamente aumentado de tamanho, escuro e firme. Na superfície de corte, observaram-se acentuação do padrão lobular e hemorragia. Histologicamente, no fígado dos equinos, havia graus variados de necrose coagulativa, hemorragia, fibrose, proliferação de ductos biliares, hepatomegalocitose e bilestase. Nas diferentes seções analisadas

  12. Study, with the help of nuclear indicators (HTO, Urea {sup 14}C and {sup 24}Na), of the renewal of water, urea and sodium in different parts of the rat kidney in aqueous diuresis and of the gerbil kidney in osmotic diuresis or in oliguria; Etude, a l'aide d'indicateurs nucleaires (HTO, UREE {sup 14}C et {sup 24}Na), du renouvellement de l'eau, de l'uree et du sodium dans les differentes regions du rein de rat en diurese aqueuse et de merion en diurese osmotique ou en oligurie

    Energy Technology Data Exchange (ETDEWEB)

    Lechene, C.P. [Commissariat a l' Energie Atomique, Saclay (France). Centre d' Etudes Nucleaires

    1965-06-01

    In the kidney of the gerbil in oliguria there exists a cortico-papillary gradient in the concentration of the sodium and of the urea; this gradient disappears in the gerbil kidney in osmotic diuresis or in the rat kidney in aqueous diuresis; in these three states of diuresis there is no significant difference in the potassium concentration between the surface and interior regions of the kidneys. Kinetic studies using tritiated water, urea {sup 14}C and {sup 24}Na show that water and urea in the interior regions of the kidney are only renewed very slowly (1 h) whereas the sodium is rapidly changed (1 mn). These results can be explained using WIRZ's theory concerning the mechanism of the counter-current concentration of urine. Furthermore, the evolution of the specific radioactivity of urea {sup 14}C is favorable for a cortical synthesis of urea. (author) [French] II existe dans le rein de Merions en oligurie un gradient cortico papillaire de la concentration du sodium et de l'uree; ce gradient est aboli dans le rein de Merions en diurese osmotique ou de rats en diurese aqueuse; dans ces 3 etats de diurese il n'y a pas de difference significative de la concentration du potassium entre les regions superficielles et profondes des reins. Des etudes cinetiques a l'aide d'eau tritiee, d'uree {sup 14}C et de {sup 24}Na montrent que l'eau et l'uree des regions profondes des reins ne sont renouveles que tres lentement (1 h), alors que le sodium est tres vite renouvele (1 mn). Ces resultats s'expliquent par la theorie de WIRZ sur le mecanisme de la concentration de l'urine par contre-courant. En outre, l'evolution renale de la radioactivite specifique de l'uree {sup 14}C est en faveur d'une synthese corticale de l'uree. (auteur)

  13. The geology of uranium in the Saint-Sylvestre granite district (Limousin, Massif Central, France); La geologie de l'uranium dans le massif granitique de Saint-Sylvestre (Limousin - Massif Central Francais)

    Energy Technology Data Exchange (ETDEWEB)

    Marquaire, C.; Moreau, M.; Barbier, J.; Ranchin, G.; Carrat, H.G.; Coppens, R.; Senecal, J.; Koszotolanyi, C.; Dottin, H

    1969-07-01

    This report concerns the geology of uranium in Limousin, more particularly in the St-Sylvestre massif, and the related phenomena: regional geology, petrographic and geochemical zonal distribution observed in various granite massifs, uranium movement in connection with surface alteration, geochronology of uranium ore. The work is made up of six articles covering the various scientific aspects listed above. Each article is headed with an abstract. The paper comprises the following chapters: Foreword by Marcel ROUBAULT. 1. Ch. MARQUAIRE, M. MOREAU Outline of geological conditions in Northern Limousin and distribution of uraniferous occurrences. 2. J. BARBIER, G. RANCHIN, H. G. CARRAT and R. COPPENS Geology of the St-Sylvestre Massif and uranium geochemistry - Introduction to laboratory studies - Problems of methodology. 3. J. BARBIER and G. RANCHIN Petrographical and geochemical zones in the St-Sylvestre granite massif (Limousin - French 'Massif Central'). 4. J. BARBIER and G. RANCHIN Uranium geochemistry in the St-Sylvestre Massif (Limousin - French 'Massif Central') - Occurrences of primary geochemical uranium and replacement processes. 5. J. SENEGAL Monograph of the Brugeaud orebody. 6. R. COPPENS, Ch. KOSZTOLANYI and H. DOTTIN Geochronological study of the Brugeaud mine. 1969. (authors) [French] Ce memoire est consacre a la geologie de l'uranium dans le Limousin, plus specialement dans le massif de St-Sylvestre, et aux phenomenes qui s'y rattachent: geologie regionale, phenomenes de zonalite petrographique et geochimique dans certains massifs granitiques, mouvements de l'uranium lies a l'alteration superficielle, geochronologie du minerai d'uranium. L'ouvrage comprend six articles qui recouvrent les differents aspects scientifiques enumeres. Chacun de ces six articles est precede d'un resume. La composition du memoire st la suivante: Marcel ROUBAULT, Preface. 1. Ch. MARQUAIRE, M. MOREAU Esquisse geologique du

  14. El olivar andaluz y sus transformaciones recientes

    Directory of Open Access Journals (Sweden)

    Sánchez Martínez, José Domingo

    2011-06-01

    Full Text Available The olive grove in Andalusia suffered critical moments during the 20th century, and it was considered like a «culture problem» in the decades preceding the incorporation of Spain in the current European Union. Since then olive grove has known an exceptional economic conjuncture, like express his enormous superficial expansion or his notable productivity. In this work we approach the reasons that explain the consolidation of one of the most typical monocultures of southern Spain, indicating the diversity of situations that coexist under a seemingly monotonous landscape and analyzing some cases that can well illustrate the set of changes experienced since 1986.

    El olivar andaluz sufrió momentos críticos a lo largo del siglo XX, hasta el punto de ser considerado como un «cultivo problema» durante las décadas que precedieron a la incorporación de España en la actual Unión Europea. Desde entonces ha conocido una coyuntura económica excepcional, como atestiguan su enorme expansión superficial y el notable incremento de sus rendimientos. En este trabajo nos acercamos a las causas que explican la consolidación de uno de los monocultivos más característicos del sur peninsular, señalando la diversidad de situaciones que conviven bajo un paisaje aparentemente monótono y analizando algunos casos que pueden ilustrar bien el conjunto de cambios experimentados desde 1986. [fr] La culture de l’olivier andalou a connu des moments critiques tout au long du XXème siècle, au point d’être considérée comme une «culture problème» pendant les décennies qui ont précédé l’entrée de l’Espagne dans l’Union européenne. Dès lors, l’oliveraie a vécu une conjoncture économique exceptionnelle, sa forte expansion superficielle et l’augmentation remarquable de ses rendements en sont témoins. Cet article explique les causes de la consolidation d’une des monocultures les plus caractéristiques du sud de la péninsule Ib

  15. Structure of two-phase air-water flows. Study of average void fraction and flow patterns; Structure des ecoulements diphasiques eau-air. Etude de la fraction de vide moyenne et des configurations d'ecoulement

    Energy Technology Data Exchange (ETDEWEB)

    Roumy, R [Commissariat a l' Energie Atomique, Grenoble (France). Centre d' Etudes Nucleaires

    1969-07-01

    This report deals with experimental work on a two phase air-water mixture in vertical tubes of different diameters. The average void fraction was measured in a 2 metre long test section by means of quick-closing valves. Using resistive probes and photographic techniques, we have determined the flow patterns and developed diagrams to indicate the boundaries between the various patterns: independent bubbles, agglomerated bubbles, slugs, semi-annular, annular. In the case of bubble flow and slug flow, it is shown that the relationship between the average void fraction and the superficial velocities of the phases is given by: V{sub sg} = f(<{alpha}>) * g(V{sub sl}). The function g(V{sub sl}) for the case of independent bubbles has been found to be: g(V{sub sl}) = V{sub sl} + 20. For semi-annular and annular flow conditions; it appears that the average void fraction depends, to a first approximation only on the ratio V{sub sg}/V{sub sl}. (author) [French] Ce rapport est une etude experimentale d'un melange diphasique eau-air dans des tubes verticaux de differents diametres. Nous avons mesure la fraction de vide moyenne dans une portion de canal de longueur 2 m, au moyen d'un systeme de vannes a fermeture rapide et simultanee. Au moyen de sondes resistives et de photographies nous avons determine la configuration de l'ecoulement et trace des cartes donnant les frontieres entre les differentes configurations d'ecoulement: bulles independantes, bulles agglomerees, bouchons, semi-annulaire, annulaire. Nous montrons que pour les regimes a bulles et a bouchons, une equation de la forme V{sub sg} = f(<{alpha}>) * g(V{sub sl}) relie la fraction de vide moyenne aux vitesses superficielles de chacune des phases. Nous avons pu determiner la fonction g(V{sub sl}) dans le cas du regime a bulles independantes, et nous trouvons g(V{sub sl}) = V{sub sl} + 20. Pour les regimes semi-annulaire et annulaire, il semble qu'en premiere approximation, la fraction de vide moyenne ne depende que

  16. Electromagnetic Gauge Study of Laser-Induced Shock Waves in Aluminium Alloys

    Science.gov (United States)

    Peyre, P.; Fabbro, R.

    1995-12-01

    The laser-shock behaviour of three industrial aluminum alloys has been analyzed with an Electromagnetic Gauge Method (EMV) for measuring the velocity of the back free surface of thin foils submitted to plane laser irradiation. Surface pressure, shock decay in depth and Hugoniot Elastic Limits (HEL) of the materials were investigated with increasing thicknesses of foils to be shocked. First, surface peak pressures values as a function of laser power density gave a good agreement with conventional piezoelectric quartz measurements. Therefore, comparison of experimental results with computer simulations, using a 1D hydrodynamic Lagrangian finite difference code, were also in good accordance. Lastly, HEL values were compared with static and dynamic compressive tests in order to estimate the effects of a very large range of strain rates (10^{-3} s^{-1} to 10^6 s^{-1}) on the mechanical properties of the alloys. Cet article fait la synthèse d'une étude récente sur la caractérisation du comportement sous choc-laser de trois alliages d'aluminium largement utilisés dans l'industrie à travers la méthode dite de la jauge électromagnétique. Cette méthode permet de mesurer les vitesses matérielles induites en face arrière de plaques d'épaisseurs variables par un impact laser. La mise en vitesse de plaques nous a permis, premièrement, de vérifier la validité des pressions d'impact superficielles obtenues en les comparant avec des résultats antérieurs obtenus par des mesures sur capteurs quartz. Sur des plaques d'épaisseurs croissantes, nous avons caractérisé l'atténuation des ondes de choc en profondeur dans les alliages étudiés et mesuré les limites d'élasticité sous choc (pressions d'Hugoniot) des alliages. Les résultats ont été comparés avec succès à des simulations numériques grâce à un code de calcul monodimensionnel Lagrangien. Enfin, les valeurs des pressions d'Hugoniot mesurées ont permis de tracer l'évolution des contraintes d

  17. In-Situ Survey System of Resistive and Thermoelectric Properties of Either Pure or Mixed Materials in Thin Films Evaporated Under Ultra High Vacuum

    Science.gov (United States)

    Lechevallier, L.; Le Huerou, J.-Y.; Richon, G.; Sarrau, J.-M.; Gouault, J.

    1995-04-01

    The study of thermoelectric and resistive in situ behaviours depending on temperature for thin films of either pure or composite materials obtained under ultra-high vacuum, is very interesting, since they can be used as strain gauges or superficial resistances. However, studies become particularly difficult when the measurements generate very low-level electrical signals. Indeed, these turn out to be hardly detectable because of the perturbations brought by the experimental environment. The apparatus described below allows for the measurement of resistance with a relative uncertainty of 2×10^{-4}, resistance variation with an absolute uncertainty of 2 mΩ and thermoelectric e.m.f. of about 2 μV. Films studied in the laboratory generally exhibit resistances lower than 100 Ω and resistance variations due to temperature variations of about a few ohms. So this device has sufficient technical characteristics for our studies. It can be connected to a PC, which allows for easy data collection and treatment. L'étude des comportements résistif et thermoélectrique in situ en fonction de la température de couches minces de matériaux simples ou composites obtenus en milieu raréfié s'avére intéressante en vue d'applications comme jauge de contrainte ou résistance superficielle mais particulièrement délicate lorsque les mesures donnent naissance à des signaux électriques de très faible amplitude. Ces derniers deviennent en effet difficilement décelables en raison des perturbations apportées par l'environnement expérimental. Le système qui est décrit ici permet de mesurer des résistances avec une certitude relative de 2×10^{-4} et d'apprécier des variations de résistance de 2 mΩ et des f.e.m. thermoélectriques de l'ordre de 2 μV. Les couches étudiées au laboratoire présentent généralement des résistances inférieures à 100 Ω et des variations de résistance dues aux variations de température de l'ordre de quelques Ω. Le dispositif de mesure

  18. Development of mercury porosimeter. Application to nuclear graphite studies (1961); Mise au point d'un porosimetre a mercure. Application a l'etude des graphites nucleaires (1961)

    Energy Technology Data Exchange (ETDEWEB)

    Bocquet, M; Genisson, J; Sailleau, J [Commissariat a l' Energie Atomique, Saclay (France). Centre d' Etudes Nucleaires

    1961-07-01

    A mercury porosimeter, model IFP-CEA, has been developed for application to nuclear graphite studies. The apparatus is based on the capillary depression phenomenon. The relationship between the radius of a pore and the pressure at which mercury fills it is pr = -2 {sigma} cos {theta} ( {sigma} is the surface tension, {theta} the angle of contact of the mercury). After some theoretical consideration, the apparatus is described. The mercury pressure is increased step-wise from 0 to 1000 kg/cm{sup 2} thus yielding the complete distribution of pores from 92 {mu} to 75 A. Results are, then presented concerning nuclear graphites which show the evolution of the porous structure under the effect of bitumen impregnation. In general, the volume of the large pores decreases while that of the small pores increases. The structure of impregnated products appears to depend to a certain extent on that of the starting materials. It has also been possible to study other products with this porosimeter; the range of measurements possible is such that it may be used for the study of the majority of porous materials. (authors) [French] Un porosimetre a mercure modele IFP-CEA a ete mis au point en vue de son application a l'etude de graphites nucleaires. Le fonctionnement de l'appareil repose sur le phenomene de depression capillaire. On etablit la relation existant entre le rayon r d'un pore et la pression p pour laquelle le mercure peut y penetrer: pr = -2 {sigma} cos {theta} ( {sigma} tension superficielle, {theta} angle de contact du mercure). Apres quelques considerations theoriques, l'appareil utilise est decrit. Il permet de faire varier par palier la pression du mercure entre 0 et 1000 kg/cm{sup 2} et d'etablir ainsi la distribution complete des rayons de pores compris entre 92 {mu} et 75 A. Les resultats d'une etude faite sur des graphites nucleaires sont alors presentes faisant apparaitre l'evolution de la structure poreuse sous l'effet des impregnations au brai. D'une facon

  19. Oxidation of iron and steels by carbon dioxide under pressure (1962); Oxydation du fer et des aciers par l'anhydride carbonique sous pression (1962)

    Energy Technology Data Exchange (ETDEWEB)

    Colombie, M [Commissariat a l' Energie Atomique, Saclay (France). Centre d' Etudes Nucleaires

    1962-07-01

    After having developed one of the first thermo-balances to operate under pressure, we have studied the influence of the pressure on the corrosion of iron and steels by carbon dioxide. The corrosion was followed by three different methods simultaneously: by the oxidation kinetics, by micrographs, and by radiocrystallography. We have been able to show that the influence of the pressure is not negligible and we have provided much experimental evidence: oxidation kinetics, micrographic aspects, surface precipitation of carbon, metal carburization, the texture of the magnetite layer. All these phenomena are certainly modified by changes in the carbon dioxide pressure. In order to interpret most of our results we have been led to believe that the phenomenon of corrosion by CO{sub 2} depends on secondary reactions localised at the oxide-gas interface. This would constitute a major difference between the oxidation by CO{sub 2} and that by oxygen. (author) [French] Apres avoir etudie et mis au point une des premieres thermobalances fonctionnant sous pression, nous avons etudie l'influence de la pression sur la corrosion du fer et des aciers par l'anhydride carbonique. Notre etude a ete conduite simultanement sur trois plans differents: etude des cinetiques d'oxydation, etude micrographique et etude radiocristallographique. Nous avons pu montrer que l'influence de la pression n'etait pas negligeable et nous en avons fourni un faisceau de preuves experimentales important: cinetiques d'oxydation, aspect micrographique, precipitation superficielle de carbone, carburation du metal, texture de la couche de magnetite. Tous ces phenomenes sont sans aucun doute modifies par une variation de pression du gaz carbonique. Pour interpreter la plupart de nos resultats, nous avons ete conduits a penser que le phenomene de corrosion par CO{sub 2} etait tributaire de reactions secondaires localisees a l'interface oxyde-gaz. Ce serait la une des differences fondamentales entre l'oxydation par

  20. Decontamination of material in the Marcoule plutonium producing centre (1961); La decontamination du materiel dans le centre de production du plutonium de Marcoule (1961)

    Energy Technology Data Exchange (ETDEWEB)

    Rodier, J; Bouzigues, H; Boutot, P [Commissariat a l' Energie Atomique, Centre de Production de Plutonium, Marcoule (France). Centre d' Etudes Nucleaires

    1961-07-01

    The decontamination of material in an atomic centre is an essential operation in view of the cost and the difficulties of replacement. It also makes it possible to reduce the storage of radio-active materials always an expensive task. Surfaces are contaminated by retention of radioactive products and the mechanism of the bounding forces can be explained in terms of chemical, mechanical, electrostatic and surface tension phenomena. The methods used for decontamination are either physical (section, abrasion, steam, ultrasonics) or chemical (acids, alkalis, detergents, reducing and oxidising agents). At Marcoule, chemical methods of treatment are used. This is effected in tanks, the exact composition of the liquids being regulated from a control panel. Working experience has shown that the fact of operating in humid conditions eliminates all problems of atmospheric contamination; as a result it is possible for the personnel to work without any special protective devices. Almost all the material can be re-utilized and the operations produce only a small volume of liquid waste. The decontamination workshop is operated by a small number of workers. The working costs, including capital repayment and treatment of the waste, do not exceed 15 per cent of the value of the apparatus treated. (authors) [French] La decontamination du materiel dans un centre atomique est une operation indispensable etant donne le cout et les difficultes du remplacement. Elle contribue aussi a diminuer les stockages toujours onereux des materiaux radioactifs. Les surfaces se contaminent par retention de corps radioactifs, l'origine des forces de liaison devant etre recherchee dans les phenomenes chimiques, mecaniques, electro-statiques ou de tensions superficielles. Les methodes de decontamination peuvent etre physiques (aspiration, abrasion, vapeur, ultra-sons) ou chimiques (acides, alcalins, detergents, reducteurs, oxydants). Sur le centre de Marcoule, la methode des traitements chimiques a ete

  1. La lectura en FLE por y para el aprendizaje

    Directory of Open Access Journals (Sweden)

    Daniela Ventura

    2017-04-01

    Full Text Available The learning process of mother tongue depends on proficiency in comprehension and interpreting. Results from PISA 2009 and PISA 2012 Assessment Framework (OECD, measuring the knowledge, skills and attitudes of 15-year-olds over the last ten years, show that Spain performs below the average of OECD countries in reading literacy. This low performance can be extended to second language learning. The results obtained in reading literacy by Spanish pupils studying French as a foreign language highlight a problem concerning text deep comprehension and inner text organization. In this paper, we will show how the inattentiveness, the cursory reading and, at the same time, the basic lack of proficiency in cognitive and some discursive abilities may be the main cause of reading incomprehension. Le processus d’acquisition de la langue maternelle passe par la maîtrise des compétences de compréhension et d’interprétation. Selon les enquêtes Pisa 2009 et PISA 2012 de la OCDE, menée avec un échantillon d’élèves de quinze ans, l’Espagne accuse un score inférieur à la moyenne des pays de l’OCDE en compréhension de l’écrit. Mauvaise performance que l’on retrouve au niveau de l’apprentissage d’une langue étrangère (LE. Dans ce cadre, nous allons apporter les résultats obtenus en FLE qui montrent qu’il existe, chez l’apprenant, un problème au niveau de la compréhension profonde du texte ainsi qu’au niveau de son organisation interne. Nous allons démontrer qu’à l’origine de l’incompréhension en lecture en FLE il y a, d’une part, l’inattention et la lecture superficielle, et de l’autre, une carence de fond dans le maniement de certaines habiletés cognitivo-discursives.

  2. Prediction du profil de durete de l'acier AISI 4340 traite thermiquement au laser

    Science.gov (United States)

    Maamri, Ilyes

    Les traitements thermiques de surfaces sont des procedes qui visent a conferer au coeur et a la surface des pieces mecaniques des proprietes differentes. Ils permettent d'ameliorer la resistance a l'usure et a la fatigue en durcissant les zones critiques superficielles par des apports thermiques courts et localises. Parmi les procedes qui se distinguent par leur capacite en terme de puissance surfacique, le traitement thermique de surface au laser offre des cycles thermiques rapides, localises et precis tout en limitant les risques de deformations indesirables. Les proprietes mecaniques de la zone durcie obtenue par ce procede dependent des proprietes physicochimiques du materiau a traiter et de plusieurs parametres du procede. Pour etre en mesure d'exploiter adequatement les ressources qu'offre ce procede, il est necessaire de developper des strategies permettant de controler et regler les parametres de maniere a produire avec precision les caracteristiques desirees pour la surface durcie sans recourir au classique long et couteux processus essai-erreur. L'objectif du projet consiste donc a developper des modeles pour predire le profil de durete dans le cas de traitement thermique de pieces en acier AISI 4340. Pour comprendre le comportement du procede et evaluer les effets des differents parametres sur la qualite du traitement, une etude de sensibilite a ete menee en se basant sur une planification experimentale structuree combinee a des techniques d'analyse statistiques eprouvees. Les resultats de cette etude ont permis l'identification des variables les plus pertinentes a exploiter pour la modelisation. Suite a cette analyse et dans le but d'elaborer un premier modele, deux techniques de modelisation ont ete considerees, soient la regression multiple et les reseaux de neurones. Les deux techniques ont conduit a des modeles de qualite acceptable avec une precision d'environ 90%. Pour ameliorer les performances des modeles a base de reseaux de neurones, deux

  3. The geology of uranium in the Saint-Sylvestre granite district (Limousin, Massif Central, France); La geologie de l'uranium dans le massif granitique de Saint-Sylvestre (Limousin - Massif Central Francais)

    Energy Technology Data Exchange (ETDEWEB)

    Marquaire, C; Moreau, M; Barbier, J; Ranchin, G; Carrat, H G; Coppens, R; Senecal, J; Koszotolanyi, C; Dottin, H

    1969-07-01

    This report concerns the geology of uranium in Limousin, more particularly in the St-Sylvestre massif, and the related phenomena: regional geology, petrographic and geochemical zonal distribution observed in various granite massifs, uranium movement in connection with surface alteration, geochronology of uranium ore. The work is made up of six articles covering the various scientific aspects listed above. Each article is headed with an abstract. The paper comprises the following chapters: Foreword by Marcel ROUBAULT. 1. Ch. MARQUAIRE, M. MOREAU Outline of geological conditions in Northern Limousin and distribution of uraniferous occurrences. 2. J. BARBIER, G. RANCHIN, H. G. CARRAT and R. COPPENS Geology of the St-Sylvestre Massif and uranium geochemistry - Introduction to laboratory studies - Problems of methodology. 3. J. BARBIER and G. RANCHIN Petrographical and geochemical zones in the St-Sylvestre granite massif (Limousin - French 'Massif Central'). 4. J. BARBIER and G. RANCHIN Uranium geochemistry in the St-Sylvestre Massif (Limousin - French 'Massif Central') - Occurrences of primary geochemical uranium and replacement processes. 5. J. SENEGAL Monograph of the Brugeaud orebody. 6. R. COPPENS, Ch. KOSZTOLANYI and H. DOTTIN Geochronological study of the Brugeaud mine. 1969. (authors) [French] Ce memoire est consacre a la geologie de l'uranium dans le Limousin, plus specialement dans le massif de St-Sylvestre, et aux phenomenes qui s'y rattachent: geologie regionale, phenomenes de zonalite petrographique et geochimique dans certains massifs granitiques, mouvements de l'uranium lies a l'alteration superficielle, geochronologie du minerai d'uranium. L'ouvrage comprend six articles qui recouvrent les differents aspects scientifiques enumeres. Chacun de ces six articles est precede d'un resume. La composition du memoire st la suivante: Marcel ROUBAULT, Preface. 1. Ch. MARQUAIRE, M. MOREAU Esquisse geologique du Nord Limousin et repartition des mineralisations

  4. Comportement des fondations et des ancrages de structures marines sous l'effet de sollicitations cycliques Behavior of Foundations and Anchors for Marine Structures under the Effect of Cyclic Stresses

    Directory of Open Access Journals (Sweden)

    Le Tirant P.

    2006-11-01

    Full Text Available L'exploration et l'exploitation des hydrocarbures en haute mer conduisent à la mise en place d'ouvrages fixes ou flottants de dimensions de plus en plus colos sales, par des profondeurs d'eau sans cesse croissantes, atteignant actuellement 200 à 300 mètres en production, un millier de mètres en exploration. Les fondations des ouvrages pétroliers de production comprennent essentiel-lement- les pieux ou groupes de pieux, battus ou forés; - les fondations superficielles à embase poids. Le comportement des fondations ou ancrages de structures sous l'action des sollicitations cycliques est étudié, en fonction de la nature des terrains, à partir - de mesures et d'observations sur des structures réelles; d'expérimentations sur des modèles en semi-grandeur ou de simulations sur modèles réduits. Les exemples donnés situent la diversité des problèmes rencontrés pour l'instal-lation des ouvrages en haute mer et! l'ampleur des travaux nécessaires pour décrire plus correctement les phénomènes d'intéraction sols-structures mannes sous l'effet des chargements cycliques et transitoires et, par suite, mieux optimiser le dimensionnement des fondations et des ancrages. Exploration and production of hydrocarbons in the high seas lead ta the installation of fixed or floating structures having more and more colossal sizes at constantly increasing water depths which now attain 200 ta 300 meters for production and 1000 meters for exploration. The foundations of ail production structures mainly consist of - pilings or groups of pilings, either driven or drilled; superficial gravity foundations the behovior of foundations or anchors for such structures under the effect of cyclic stresses is examined as a function of the nature of formations, on the basis of - measurements and observations on actual structures, experiments with semi-full-sized models or by simulations with scale models. The examples given illustrate the diversity of the problems

  5. Liver cirrhosis and hepatic stellate cells Cirrose hepática e células estreladas do figado

    Directory of Open Access Journals (Sweden)

    Daniel Ferracioli Brandão

    2006-01-01

    Full Text Available The cirrhosis represents the final stage of several chronic hepatic diseases and it is characterized by the presence of fibrosis and morphologic conversion from the normal hepatic architecture into structurally abnormal nodules. In the evolution of the disease there is loss of the normal vascular relationship and portal hypertension. There are also regenerative hepatocelular alterations that become more prominent with the progression of the disease. The liver transplantation continues to be the only therapeutic option in cases of disease in terminal phase. The hepatic stellate cells (HSC are perisinusoidal cells that store vitamin A and produce growth factors, citocins, prostaglandins and other bioactive substances. They can suffer an activation process that convert them to cells with a phenotype similar to myofibroblasts. When activated, they present increased capacity of proliferation, mobility, contractility and synthesis of collagen and other components of extracelular matrix. They possess cytoplasmic processes adhered to sinusoids and can affect the sinusoidal blood flow. HSC are important in pathogenesis of fibrosis and portal hypertension.A cirrose representa o estágio final de diversas doenças hepáticas crônicas e é caracterizada pela presença de fibrose e conversão da arquitetura hepática normal em nódulos estruturalmente anormais. Na evolução da doença ocorre perda da relação vascular normal e hipertensão portal. Há também alterações regenerativas hepatocelulares que se tornam mais proeminentes com a progressão da doença. O transplante hepático permanece como a única opção terapêutica nos casos de doença em fase terminal. As células estreladas hepáticas (CEH são células perisinusoidais que armazenam vitamina A e produzem fatores de crescimento, citocinas, prostaglandinas e outras substâncias bioativas. Podem sofrer um processo de ativação para um fenótipo semelhante a miofibroblastos. Quando ativadas

  6. Primary cutaneous marginal zone lymphoma associated with juxta-articular fibrotic nodules in a teenager.

    Science.gov (United States)

    Ghatalia, Pooja; Porter, Joanne; Wroblewski, Danielle; Carlson, John Andrew

    2013-05-01

    Primary cutaneous marginal zone lymphoma (PCMZL) has rarely been reported in teenagers and is occasionally associated with Borrelia burgdorferi infection. Juxta-articular fibrotic nodules represent a unique, localized fibrosing response to spirochete infections, namely Borreliosis. Herein, we report a 15-year-old healthy boy who presented with a 4-year history of progressive acquisition of asymptomatic, erythematous nodules, ≤ 3 cm, beginning with his right forearm (3), then right arm (1) and lastly his right inner thigh (1). Biopsy showed PCMZL in three of five samples, and inflamed, fibrotic nodules, near the elbow in two. The bottom heavy lymphomatous nodules consisted of mostly small CD20+ CD43+ lymphocytes, some with plasmacytoid features. Mature plasma cells were lambda light chain restricted by in situ hybridization. The juxta-articular fibrotic nodules were located in the deep dermis and subcutis, had peripheral plasma cell-rich infiltrates, and showed nodular sclerosis (morphea profunda-like) in one, and lamellar and angiocentric sclerosis in the other reminiscent of quiescent lesions of chronic localized fibrosing leukocytoclastic vasculitis. Immunohistochemistry for B. burgdorferi revealed rare positive organisms; however, polymerase chain reaction (PCR) and serology were negative for B. burgdorferi as were serologic and/or PCR assays for Bartonella henselae, Ba. quintana, Ehrlichia chaffeensis, Treponema pallidum, Helicobacter pylori and Babesia microti. No evidence of extracutaneous disease was found by the review of systems and imaging studies. A 4-week trial of doxycycline therapy failed, whereas intralesional (IL) corticosteroid therapy induced rapid regression of his nodules. After two local recurrences, also treated with IL corticosteroids, he is well, without cutaneous disease, 20 months later. A literature review of 19 pediatric cases PCMZL reveals a similar natural history as adult PCMZL. Despite negative serology and PCR for B. burgdorferi

  7. Cirrose experimental induzida pela inalação de tetracloreto de carbono: adaptação da técnica e avaliação da peroxidação lipídica

    Directory of Open Access Journals (Sweden)

    CREMONESE Ricardo Viégas

    2001-01-01

    Full Text Available Racional - Administração a longo prazo de tetracloreto de carbono é modelo experimental para produzir fibrose hepática. O estresse oxidativo parece ser o mecanismo envolvido na hepatoxicidade por tetracloreto de carbono, onde as espécies ativas de oxigênio têm importante papel na patogênese da fibrose hepática. Objetivos - Avaliar a eficácia de um modelo experimental de cirrose hepática induzida pela inalação de tetracloreto de carbono em ratos, bem como avaliar a peroxidação lipídica e as características do líquido de ascite neste modelo. Material e Métodos - Inicialmente, acompanhou-se a evolução dos achados histológicos, através da técnica de hematoxilina e eosina, mediante o uso de tetracloreto de carbono inalatório ao longo das diferentes semanas (5ª, 7ª, 9ª, 12ª. Posteriormente, ao final da 15ª semana de estudo, os ratos, então divididos em três grupos (controle; controle + fenobarbital; e tetracloreto de carbono + fenobarbital, foram avaliados em sua histologia hepática, peroxidação lipídica e as características do líquido de ascite. Para as análises de peroxidação lipídica utilizaram-se as técnicas de substâncias reativas ao ácido tiobarbitúrico e de quimiluminescência. No líquido de ascite avaliaram-se a citologia e a bacteriologia. Resultados - Observou-se entre a 12ª e 15ª semanas de inalação o estabelecimento de cirrose em 100% dos animais submetidos a inalação com tetracloreto de carbono, acompanhada de um aumento significante na peroxidação lipídica no fígado dos ratos inalados com tetracloreto de carbono. Evidenciou-se a presença de infecção do líquido de ascite em um dos sete casos nos quais esta estava presente. Conclusão - O método inalatório desenvolvido é eficaz na indução de cirrose hepática e formação de ascite, sendo o estresse oxidativo um dos principais mecanismos da indução de cirrose pelo tetracloreto de carbono.

  8. Adenocarcinoma do pulmão em doente com esclerodermia: Um caso clínico

    Directory of Open Access Journals (Sweden)

    João Bento

    2009-01-01

    Full Text Available Resumo: A esclerodermia é uma doença do tecido conjuntivo de etiologia desconhecida, que tem sido associada a um risco acrescido de malignidade. O cancro do pulmão é a neoplasia mais frequente, nestes doentes.Apresenta-se o caso clínico de uma mulher de 42 anos, não fumadora, com esclerodermia, que desenvolveu agravamento progressivo do seu estado geral e derrame pleural com características de exsudado, sem evidência de infecção ou malignidade. A TAC torácica mostrou zonas de fibrose, a broncofibroscopia, os lavados brônquico e broncoalveolar foram normais. Foi excluída neoplasia extrapulmonar. Na pleuroscopia, observaram-se formações nodulares, cujas biópsias revelaram tratar-se de adenocarcinoma pulmonar. Iniciou quimioterapia, desenvolvendo ao 48.° dia neutropenia febril e sépsis, vindo a morrer 12 dias depois.Salientamos este caso pela relação, apesar de rara, entre a esclerodermia e o cancro do pulmão e pela importância de uma vigilância pulmonar cuidadosa, em indivíduos com esta doença reumatológica, pelo risco acrescido de neoplasia. Abstract: Systemic sclerosis (scleroderma is a connective tissue disorder of unknown aetiology characterised by immune abnormalities, which has been related to an increased risk of malignancy. Lung cancer is the most prevalent among these patients.We present a clinical case of a 42 years old non smoker female with systemic sclerosis. She presented progressive general health status worsening and an exudative pleural effusion, with no evidence of infection or malignancy. Chest high resolution computed tomography revealed pulmonary fibrosis. Bronchoscopy, bronchial and bronchoalveolar lavage were normal. Extrapulmonary malignancies were excluded. Pleural nodularities were found on pleuroscopy and the biopsy was compatible with lung adenocarcinoma. Chemotherapy was then started, which complicated with febrile neutropenia, sepsis and patient death 12 days after.The purpose of this case

  9. Endoprótese revestida de jugular preservada de bovino: estudo comparativo da resposta tecidual em aorta torácica descendente e veia cava inferior de suínos

    Directory of Open Access Journals (Sweden)

    Celso Luiz Muhlethaler Chouin

    Full Text Available OBJETIVO: Avaliar e comparar a resposta tecidual de uma endoprótese biosintetica implantada na aorta torácica descendente e veia cava inferior de suínos. MÉTODO: Foi implantada uma endoprótese auto-expansível composta de aço inoxidável, revestida por veia jugular de bovino, processada pelo método L-hydro, com auxilio de uma bainha de liberação Taheri-Leonhardt (Flórida, EUA na aorta torácica descendente, e a veia cava infra-renal de 10 suínos. Sessenta dias após, as endopróteses foram retiradas e analisadas sob o ponto de vista macro e microscópicos. Foram observados: perviedade, grau de incorporação a parede do vaso, tipo de reação inflamatória, e local de maior resposta, tanto em relação a camada do vaso quanto ao local de contato com o anel de aço RESULTADOS: Todas as endopróteses encontravam-se pérvias, e incorporadas à parede. No setor venoso, seis apresentaram traves fibrosas em sua luz, e quatro apresentaram fibrose perivascular. No setor arterial somente uma prótese apresentou discreta estenose, sem fibrose perivascular. A reação inflamatória crônica tipo corpo estranho ocorreu em 100% das peças, a camada média foi a mais acometida no setor venoso, enquanto a íntima foi mais constante na artéria, o grau de incorporação foi mais firme na veia em comparação a artéria. A reação tecidual mostrou maior tendência nas áreas em intimo contato com o anel de aço (intra-anelar, mais intensa na artéria do que na veia. CONCLUSÃO: A prótese apresentou baixa trombogenicidade em ambos os sistemas, houve maior reação tecidual e baixa biocompatibilidade no setor venoso.

  10. Adenocarcinoma do pulmão em doente com esclerodermia: Um caso clínico

    Directory of Open Access Journals (Sweden)

    João Bento

    2009-01-01

    Full Text Available Resumo: A esclerodermia é uma doença do tecido conjuntivo de etiologia desconhecida, que tem sido associada a um risco acrescido de malignidade. O cancro do pulmão é a neoplasia mais frequente, nestes doentes.Apresenta-se o caso clínico de uma mulher de 42 anos, não fumadora, com esclerodermia, que desenvolveu agravamento progressivo do seu estado geral e derrame pleural com características de exsudado, sem evidência de infecção ou malignidade. A TAC torácica mostrou zonas de fibrose, a broncofibroscopia, os lavados brônquico e broncoalveolar foram normais. Foi excluída neoplasia extrapulmonar. Na pleuroscopia, observaram-se formações nodulares, cujas biópsias revelaram tratar-se de adenocarcinoma pulmonar. Iniciou quimioterapia, desenvolvendo ao 48.º dia neutropenia febril e sépsis, vindo a morrer 12 dias depois.Salientamos este caso pela relação, apesar de rara, entre a esclerodermia e o cancro do pulmão e pela importância de uma vigilância pulmonar cuidadosa, em indivíduos com esta doença reumatológica, pelo risco acrescido de neoplasia.Rev Port Pneumol 2009; XV (1: 93-99 Abstract: Systemic sclerosis (scleroderma is a connective tissue disorder of unknown aetiology characterised by immune abnormalities, which has been related to an increased risk of malignancy. Lung cancer is the most prevalent among these patients.We present a clinical case of a 42 years old non smoker female with systemic sclerosis. She presented progressive general health status worsening and an exudative pleural effusion, with no evidence of infection or malignancy. Chest high resolution computed tomography revealed pulmonary fibrosis. Bronchoscopy, bronchial and bronchoalveolar lavage were normal. Extra-pulmonary malignancies were excluded. Pleural nodularities were found on pleuroscopy and the biopsy was compatible with lung adenocarcinoma. Chemotherapy was then started, which complicated with febrile neutropenia, sepsis and patient death 12

  11. Myocardial Viability on Cardiac Magnetic Resonance.

    Science.gov (United States)

    Souto, Ana Luiza Mansur; Souto, Rafael Mansur; Teixeira, Isabella Cristina Resende; Nacif, Marcelo Souto

    2017-05-01

    The study of myocardial viability is of great importance in the orientation and management of patients requiring myocardial revascularization or angioplasty. The technique of delayed enhancement (DE) is accurate and has transformed the study of viability into an easy test, not only for the detection of fibrosis but also as a binary test detecting what is viable or not. On DE, fibrosis equal to or greater than 50% of the segmental area is considered as non-viable, whereas that below 50% is considered viable. During the same evaluation, cardiac magnetic resonance (CMR) may also use other techniques for functional and perfusion studies to obtain a global evaluation of ischemic heart disease. This study aims to highlight the current concepts and broadly emphasize the use of CMR as a method that over the last 20 years has become a reference in the detection of infarction and assessment of myocardial viability. Resumo O estudo de viabilidade miocárdica é de grande importância para a orientação e manejo de pacientes que necessitam de cirurgia de revascularização miocárdica ou angioplastia. A técnica de realce tardio (RT) é precisa e transformou o estudo de viabilidade em um teste fácil, não só para a detecção de fibrose, mas também como um modelo binário para a detecção do que é ou não é viável. Uma fibrose identificada pelo RT é considerada como não viável quando igual ou maior do que 50% da área segmentar e como viável quando menor que 50%. A ressonância magnética cardíaca (RMC) também pode lançar mão de outras técnicas para estudo funcional e de perfusão para uma avaliação global da doença isquêmica do coração no mesmo exame. Este estudo tem como objetivo destacar os conceitos atuais e enfatizar amplamente o uso da RMC como um método que nos últimos 20 anos se tornou referência na detecção de infarto e avaliação de viabilidade miocárdica.

  12. Realce Tardio miocárdico por Ressonância Magnética Cardíaca pode identificar risco para Taquicardia Ventricular na Cardiopatia Chagásica Crônica

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    Ronaldo Peixoto de Mello

    2012-05-01

    Full Text Available FUNDAMENTO: Testes invasivos e não invasivos têm sido usados para identificar risco para Taquicardia Ventricular (TV em pacientes com Cardiopatia Chagásica Crônica (CCC. Ressonância Magnética Cardíaca (RMC pela técnica do Realce Tardio (RT pode ser útil para selecionar pacientes com disfunção ventricular global ou segmentar, com alto grau de fibrose e maior risco para TV clínica. OBJETIVO: Melhorar a identificação de elementos preditivos de TV em pacientes com CCC. MÉTODO: Quarenta e um pacientes com CCC foram pesquisados, sendo 30 (72% do sexo masculino, com média de idade de 55,1 ± 11,9 anos. Vinte e seis pacientes apresentavam histórico de TV (grupo TV, e 15 não apresentavam TV (grupo NTV. Todos os pacientes incluídos tinham RT e disfunção segmentar ventricular. Volume, porcentagem de comprometimento da espessura da parede ventricular em cada segmento, e distribuição de RT foi determinado em cada caso. RESULTADOS: Não houve diferença estatística em termos de volume de RT entre os dois grupos: grupo TV = 30,0 ± 16,2%; grupo NTV = 21,7 ± 15,7%; p = 0,118. A probabilidade de TV foi maior se duas ou mais áreas contíguas de fibrose transmural estivessem presentes, sendo um fator preditor de TV clínica (RR 4,1; p = 0,04. A concordância entre os observadores foi de 100% nesse critério (p < 0,001. CONCLUSÃO: A identificação de dois ou mais segmentos de RT transmural por RMC está associado com a ocorrência de TV clínica em pacientes com CCC. Portanto, a RMC melhora a estratificação de risco na população estudada. (Arq Bras Cardiol. 2012; [online].ahead print, PP.0-0

  13. Patogênese, sinais clínicos e patologia das doenças causadas por plantas hepatotóxicas em ruminantes e eqüinos no Brasil Pathogenesis, clinical signs and pathology of diseases caused by hepatotoxic plants in ruminants and horses in Brazil

    Directory of Open Access Journals (Sweden)

    Julio Cesar A. Santos

    2008-01-01

    Full Text Available Plantas que causam lesões hepáticas em ruminantes e eqüinos constituem um grupo importante de plantas tóxicas no Brasil. Em geral essas plantas podem ser divididas em três grandes grupos: plantas que causam necrose hepática aguda; plantas que causam fibrose hepática; e plantas que causam fotossensibilização. Em algumas dessas plantas os princípios tóxicos já foram identificados. Das plantas que causam necrose hepática aguda, os carboxiatractilosídeos estão presentes em Cestrum parqui e Xanthium cavanillesi. Os alcalóides pirrolizidínicos estão presentes nas plantas que causam fibrose hepática (Senecio spp., Echium plantagineum, Heliotropum spp. e Crotalaria spp.. Das plantas que causam fotossensibilização hepatógena são conhecidos os furanossesquiterpenos em Myoporum spp., triterpenos em Lantana spp., e saponinas esteroidais em Brachiaria spp. e Panicum spp. O quadro clínicopatológico dessas intoxicações e o mecanismo geral da insuficiência hepática, incluindo meios de diagnóstico, são descritos neste artigo de revisão.Plants causing hepatic lesions in ruminants and horses constitute one important group of poisonous plants in Brazil. These plants can be placed in three major groups: plants causing acute liver necrosis; plants causing liver fibrosis; and plants causing hepatogenous photosensitization. For some of these plants the toxic principles are known. Cestrum parqui and Xanthium cavanillesi that cause acute liver necrosis contain carboxy-atractylosides. Senecio spp., Crotalaria spp., and Echium plantagineum that cause liver fibrosis contain pyrrolizidine alkaloids. As for the group of plants causing hepatogenous photosensibilization, Myoporum spp. contain furanosesquiterpenes, Lantana spp contain triterpenes, and Brachiaria spp. and Panicum spp. contain steroidal saponins. The clinical and pathologic features of the toxicosis caused by these phytotoxins, general mechanisms of production for the production of

  14. Hiperplasia de mastócitos na oxalose óssea

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    Duarte M.E.L.

    1999-01-01

    Full Text Available OBJETIVOS: Avaliar através de técnicas de histomorfometria a incidência de hiperplasia de mastócitos na medula óssea de pacientes portadores de oxalose e insuficiência renal crônica. MATERIAL E MÉTODOS: Foram estudados 18 indivíduos divididos em 3 grupos: 6 (4 homens e 2 mulheres com média de idade de 26.31±2.5 anos portadores de oxalose óssea e insuficiência renal crônica (IRC ; 6 (5 mulheres e 1 homem com idade média de 22.1±3.56 anos portadores de IRC e 6 indivíduos saudáveis (5 homens e 1 mulher com idade média de 23±2.78 anos. A análise do tecido ósseo foi realizada em biópsias de crista ilíaca, incluídas em resina, sem descalcificação prévia e coradas pela técnica do Azul de Toluidina. A contagem dos mastócitos foi feita utilizando-se sistema analisador de imagem e os valores (média±DP foram expressos sob a forma de células por mm² de tecido. RESULTADOS: O número de mastócitos foi significativamente maior nos portadores de oxalose óssea, 32.67±9.59, ao se comparar com os pacientes portadores de IRC sem oxalose (20.84±5.04,p<0.05 e nos indivíduos do grupo controle (3.26±1.03,p<0.001 CONCLUSÕES: A oxalose óssea está associada com um aumento substancial do número de mastócitos na medula óssea. Esta alteração não está relacionada com a IRC per se e não parece representar uma resposta inespecífica à fibrose medular. O acúmulo anormal de mastócitos deve, de alguma forma, contribuir para o desenvolvimento da fibrose de medula óssea que acompanha esta condição.

  15. Influence of Low Molecular Weight Polymers on the Rheology of Bentonite Suspensions Influence de polymères de faible masse moléculaire sur la rhéologie de suspensions de bentonite

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    Rossi S.

    2006-12-01

    Full Text Available In this communication we demonstrate how relatively low molecular weight synthetic polymers significantly alter the rheological properties of Bentonite/water suspensions depending upon the polymer dosage and/or degree of surface coverage on the clay particle. The behaviour of two types of nonionic polymers are reported; a series of nonylphenol poly(propylene oxide poly(ethylene oxide polymers with varying EO chain length and a series consisting of an ABA block copolymer of poly(propylene oxide-poly(ethylene oxide with varying EO chain length. Adsorption isotherms of dilute clay suspensions showed a higher uptake of the lowest molecular weight polymer (in µmol/m² for each series. The adsorbed amount G increases in the order NPE > PEO > PE. The oscillatory shear and steady state shear stress-shear rate measurements of concentrated clay suspensions (3-6% w/v facilitate the interpretation of the particle-particle interactions. The yield stress, plastic viscosity and elastic modulus showed a maximum at about 50% surface coverage by the NPE polymers, and a subsequent decrease due to stabilisation of the suspension. For the PE polymers these parameters continously decreased as the surface of the clay particle was covered. Scaling of the elastic modulus with increasing clay content allows the determination of the exponent n from a power-law fit G = Kon which qualitatively describes the degree of dispersion for the different polymers. Preliminary results of the effect of temperature and pressure on Bentonite/polymer suspensions are also presented. Dans cette communication, nous démontrons comment les polymères synthétiques de masse moléculaire relativement faible modifient notablement les propriétés rhéologiques de suspensions de bentonite dans l'eau en fonction de polymère et/ou du degré de couverture superficielle de la particule d'argile. Les comportements de deux types de polymères non ioniques sont décrits: une série de polymères

  16. Radiation accidents

    International Nuclear Information System (INIS)

    Saenger, E.L.

    1986-01-01

    It is essential that emergency physicians understand ways to manage patients contaminated by radioactive materials and/or exposed to external radiation sources. Contamination accidents require careful surveys to identify the metabolic pathway of the radionuclides to guide prognosis and treatment. The level of treatment required will depend on careful surveys and meticulous decontamination. There is no specific therapy for the acute radiation syndrome. Prophylactic antibodies are desirable. For severely exposed patients treatment is similar to the supportive care given to patients undergoing organ transplantation. For high-dose extremity injury, no methods have been developed to reverse the fibrosing endarteritis that eventually leads to tissue death so frequently found with this type of injury. Although the Three Mile Island episode of March 1979 created tremendous public concern, there were no radiation injuries. The contamination outside the reactor building and the release of radioiodine were negligible. The accidental fuel element meltdown at Chernobyl, USSR, resulted in many cases of acute radiation syndrome. More than 100,000 people were exposed to high levels of radioactive fallout. The general principles outlined here are applicable to accidents of that degree of severity

  17. Asbestosis and other pulmonary fibrosis in asbestos-exposed workers: high-resolution CT features with pathological correlations

    Energy Technology Data Exchange (ETDEWEB)

    Arakawa, Hiroaki [Dokkyo Medical University, Department of Radiology, Mibu, Tochigi (Japan); Kishimoto, Takumi [Okayama Rosai Hospital, Asbestos Research Center, Okayama (Japan); Ashizawa, Kazuto [Nagasaki University Graduate School of Biomedical Sciences, Department of Clinical Oncology, Nagasaki (Japan); Kato, Katsuya [Kawasaki Medical School, Department of Diagnostic Radiology 2, Okayama (Japan); Okamoto, Kenzo [Hokkaido Chuo Hospital, Department of Pathology, Iwamizawa, Hokkaido (Japan); Honma, Koichi [Dokkyo Medical University, Department of Pathology, Mibu, Tochigi (Japan); Hayashi, Seiji [National Hospital Organization Kinki-Chuo Chest Medical Center, Osaka (Japan); Akira, Masanori [National Hospital Organization Kinki-Chuo Chest Medical Center, Department of Radiology, Osaka (Japan)

    2016-05-15

    The purpose was to identify distinguishing CT features of pathologically diagnosed asbestosis, and correlate diagnostic confidence with asbestos body burden. Thirty-three workers (mean age at CT: 73 years) with clinical diagnoses of asbestosis, who were autopsied (n = 30) or underwent lobectomy (n = 3), were collected. Two radiologists independently scored high-resolution CT images for various CT findings and the likelihood of asbestosis was scored. Two pathologists reviewed the pathology specimens and scored the confidence of their diagnoses. Asbestos body count was correlated with CT and pathology scores. Pathologically, 15 cases were diagnosed as asbestosis and 18 cases with various lung fibroses other than asbestosis. On CT, only the score of the subpleural curvilinear lines was significantly higher in asbestosis (p = 0.03). Accuracy of CT diagnosis of asbestosis with a high confidence ranged from 0.73 to 0.79. Asbestos body count positively correlated with CT likelihood of asbestosis (r = 0.503, p = 0.003), and with the confidence level of pathological diagnosis (r = 0.637, p < 0.001). Subpleural curvilinear lines were the only clue for the diagnosis of asbestosis. However, this was complicated by other lung fibrosis, especially at low asbestos body burden. (orig.)

  18. Results of radiotherapy of Duplay's syndrome

    International Nuclear Information System (INIS)

    Kreisfeldt, E.

    1979-01-01

    The observed patient group comprised 185 persons who underwent treatment during 1970 and 1976. The sexual distribution within this group is normal. The age peak ranges between 50 and 60 years. In a late inquiry 31.9% of the patients can indicate the freedom from pain. 29.2% tell about a good, 18.4% about moderate success. In about one half of the treated persons roentgenologically visible calcareous deposits were detected. In both groups their treatment led to an equally good result. However, an accumulation of diseases with calcifications is found in patients who were younger than 50 years. In acute cases without previous treatment the achieved result was better than in those who had been treated already elsewhere. Acute cases respond considerably better to the treatment than the chronicaal ones. For the acute cases also a notably inferior recidivity rate was found. In 38% of all cases recidivation was observed. For the acute cases this rate is only 16%. Although investigations exist which do not hint at an autonomous course of this disease, a therapeutic treatment is not considered as superfluous. Considering the known risks due to ionizing irradiation, radiotherapy of periarticular fibrositis is recommended particularly for patients older than 40 years. (orig./MG) [de

  19. Characteristics of calcaneal bone infarction: an MR imaging investigation

    Energy Technology Data Exchange (ETDEWEB)

    Abrahim-Zadeh, R.; Klein, R.M.; Leslie, D.; Norman, A. [Department of Radiology, New York Medical College, Macy Pavillion, Valhalla, NY 10595 (United States)

    1998-06-01

    Objective. Bone infarction (BI) of the calcaneus is an uncommon entity which has received little mention in the recent literature. In this paper, we review the MR images of six calcanei with BI, which demonstrate a pattern of presentation that may explain the etiology of BI at this unusual location. Design. A retrospective review was performed of the transcribed reports of the foot or ankle MR examinations at our institution. MR images of examinations with any marrow signal abnormality were reviewed for presence of BI and its distribution. Patients. Based on MRI criteria, four patients had calcaneal BI (none biopsy proven); they ranged in age from 37 to 51 years old. Two patients were diagnosed with systemic lupus erythematosus, one with fibrositis, and another with polymyositis. All were treated with corticosteroids. Results. Six calcanei (in four patients) contained a region of calcaneal BI. In five of the six, the lesions were entirely or predominantly located in the posterior half of the calcaneus. Conclusion. Two theories are proposed which may explain why BI predominantly occurs in the posterior half of the calcaneus. First, the convergence of the recurrent intraosseous calcaneal vessels may occasionally produce the equivalent of a single dominant vessel that is more prone to vascular accidents. Secondly, the region between the recurrent and the epiphyseal vessels may act as a watershed zone, increasing its susceptibility to ischemia. (orig.) With 4 figs., 1 tab., 8 refs.

  20. The key role of exudative lesions and their encapsulation: lessons learned from the pathology of human pulmonary tuberculosis.

    Science.gov (United States)

    Cardona, Pere-Joan

    2015-01-01

    A review of the pathology of human pulmonary TB cases at different stages of evolution in the pre-antibiotic era suggests that neutrophils play an instrumental role in the progression toward active TB. This progression is determined by the type of lesion generated. Thus, exudative lesions, in which neutrophils are the major cell type, are both triggered by and induce local high bacillary load, and tend to enlarge and progress toward liquefaction and cavitation. In contrast, proliferative lesions are triggered by low bacillary loads, mainly comprise epithelioid cells and fibroblasts and tend to fibrose, encapsulate and calcify, thus controlling the infection. Infection of the upper lobes is key to the progression toward active TB for two main reasons, namely poor breathing amplitude, which allows local bacillary accumulation, and the high mechanical stress to which the interlobular septae (which enclose secondary lobes) are submitted, which hampers their ability to encapsulate lesions. Overall, progressing factors can be defined as internal (exudative lesion, local bronchogenous dissemination, coalescence of lesions), with lympho-hematological dissemination playing a very limited role, or external (exogenous reinfection). Abrogating factors include control of the bacillary load and the local encapsulation process, as directed by interlobular septae. The age and extent of disease depend on the quality and speed with which lesions liquefy and disseminate bronchially, the volume of the slough, and the amount and distribution of the sloughing debris dispersed.

  1. Colchicine in therapy. State of the art and new perspectives for an old drug.

    Science.gov (United States)

    Famaey, J P

    1988-01-01

    Colchicine is the most specific treatment in acute gouty attacks. In several European countries, oral colchicine is still used for routine treatment of acute gout. Its selectivity is used as a diagnostic tool. It is also active in the treatment of acute crises of chondrocalcinosis and more occasionally of other arthritic crises (e.g. sarcoidosis). Colchicine appears to be the necessary adjuvant prophylactic drug when starting a hypouricemic treatment with uricosuric or uricolytic drugs for avoiding acute gouty crisis due to sudden mobilisation of the uric acid pool. Besides gout, colchicine is the drug of choice for treating familial mediterranean fever. It appears to be helpful in the treatment of Behçet's disease. It seems also useful for treating fibrosing conditions such as liver cirrhosis and scleroderma. As an adjuvant therapy, it helps treating dermatological disorders which are associated with leucocyte migration as an essential pathogenic factor (e.g. psoriasis, dermatitis herpetiformis, necrotising vasculitis ...). It has been advocated as an adjuvant therapy in malignant diseases as a support in radiotherapy and as an useful drug in various other diseases where it has been tried occasionally (e.g. Paget's disease of the bone, idiopathic thrombocytopenic purpura, disc syndrome). This very old drug remains a modern therapeutic agent.

  2. Efeitos da intoxicação crônica com o etanol na evolução da Tripanosomíase cruzi experimental no camundongo

    Directory of Open Access Journals (Sweden)

    Nildete das Graças Lima Gomes

    1989-12-01

    Full Text Available Dois experimentos foram realizados para estudar o efeito da intoxicação crônica com o etanol (solução a 7% como única fonte de líquido sobre a evolução da infecção pelo T. cruzi em camundongos: (1 animais após 60 dias de infecção com cepa miotrópica do T. cruzi foram submetidos à intoxicação crônica com o etanol durante 6 meses; (2 animais cronicamente intoxicados com etanol durante 5 meses foram infectados com a mesma cepa do T. cruzi e, continuando a ingestão do etanol, foram acompanhados até 45 dias após a infecção. Os animais infectados e tratados com etanol apresentaram, em relação aos que não ingeriram álcool etílico: (a mortalidade semelhante nos dois experimentos; (b parasitemia mais alta na fase aguda e parasitemia patente mais freqüente na fase crônica; (b miocardite com exsudato inflamatório menos intenso e fibrose miocárdica mais extensa na fase crônica; (c no músculo esquelético, miosite menos intensa e arterite com trombose hialina menos freqüente.

  3. Magnetic resonance imaging and ultrasound in hepatosplenic schistosomiasis mansoni Ressonância magnética e ultrassonografia na esquistossomose mansoni hepatoesplênica

    Directory of Open Access Journals (Sweden)

    José Roberto Lambertucci

    2004-08-01

    Full Text Available We report the findings of abdominal ultrasound and magnetic resonance imaging observed in a patient with advanced schistosomiasis mansoni. A 25-year-old man with hepatosplenic schistosomiasis and variceal bleeding confirmed by upper endoscopy was submitted to abdominal ultrasound and magnetic resonance imaging. During surgery for portal hypertension, a liver biopsy was taken and the diagnosis of Symmers' fibrosis was confirmed. magnetic resonance imaging scans gave more precise information about the gallbladder, periportal thickening and abdominal venous system than did the ultrasound.Relatamos os achados ultrassonográficos e à ressonância magnética intra-abdominais observados em um paciente com esquistossomose mansoni grave. Um homem de 25 anos de idade com esquistossomose hepatoesplênica e sangramento digestivo de varizes esofagianas, com diagnóstico confirmado pela endoscopia, foi submetido à ultrasonografia abdominal e ressonância magnética. Durante a cirurgia de hipertensão porta, um fragmento de fígado foi obtido e o exame histológico confirmou o diagnóstico de fibrose de Symmers. A ressonância magnética forneceu informações mais precisas sobre a vesícula biliar, espessamento periportal e sistema venoso abdominal do que a ultrassonografia.

  4. Chronic pancreatitis with secondary diabetes mellitus treated by use of insulin in an adult California sea lion.

    Science.gov (United States)

    Meegan, Jenny M; Sidor, Inga F; Steiner, Jörg M; Sarran, Delphine; Dunn, J Lawrence

    2008-06-01

    A 21-year-old neutered male captive California sea lion developed chronic polyuria; polydipsia; polyphagia; accelerated development of existing cataracts; and frequent episodes of gastrointestinal upset including anorexia, signs of abdominal discomfort, diarrhea, and vomiting. Chronic hypercholesterolemia, hypertriglyceridemia, hyperglycemia, and glucosuria were identified. During episodes of gastrointestinal abnormalities, transient hyperbilirubinemia and increased serum J-glutamyltransferase activities developed. Clinical findings strongly suggested chronic pancreatitis with secondary diabetes mellitus and intermittent cholestasis. Multiple diagnostic tests, including abdominal ultrasonography, serial hematologic and serum biochemical analyses, fecal examinations, urinalyses and bacteriologic culture of urine, measurement of serum fructosamine and insulin concentrations, and evaluation of thyroid and adrenal function, did not reveal any specific parasitic, endocrine, hepatic, or neoplastic etiologies. For 1.5 years, the sea lion received once-daily administration of glargine insulin, gastrointestinal protectants, and a strict high-protein, low-fat diet. Daily monitoring of glucose regulation was achieved by training the sea lion to submit to blood and urine sampling. Glucose regulation ranged from fair to good, and clinical signs of diabetes mellitus lessened. Episodes of gastrointestinal upset still occurred, although the frequency and severity decreased. Ultimately, a severe episode developed, associated with diabetic ketoacidosis and sepsis, and the sea lion died. Severe fibrosing pancreatitis with exocrine and endocrine atrophy and abscesses arising from ectatic pancreatic ducts were found. Peripancreatic fibrosis caused stricture of the common bile duct, resulting in gallbladder distension without cholecystitis. Diabetes mellitus can occur secondary to chronic pancreatitis in California sea lions and insulin therapy should be considered.

  5. BK virus encephalopathy and sclerosing vasculopathy in a patient with hypohidrotic ectodermal dysplasia and immunodeficiency.

    Science.gov (United States)

    Darbinyan, Armine; Major, Eugene O; Morgello, Susan; Holland, Steven; Ryschkewitsch, Caroline; Monaco, Maria Chiara; Naidich, Thomas P; Bederson, Joshua; Malaczynska, Joanna; Ye, Fei; Gordon, Ronald; Cunningham-Rundles, Charlotte; Fowkes, Mary; Tsankova, Nadejda M

    2016-07-13

    Human BK polyomavirus (BKV) is reactivated under conditions of immunosuppression leading most commonly to nephropathy or cystitis; its tropism for the brain is rare and poorly understood. We present a unique case of BKV-associated encephalopathy in a man with hypohidrotic ectodermal dysplasia and immunodeficiency (HED-ID) due to IKK-gamma (NEMO) mutation, who developed progressive neurological symptoms. Brain biopsy demonstrated polyomavirus infection of gray and white matter, with predominant involvement of cortex and distinct neuronal tropism, in addition to limited demyelination and oligodendroglial inclusions. Immunohistochemistry demonstrated polyoma T-antigen in neurons and glia, but expression of VP1 capsid protein only in glia. PCR analysis on both brain biopsy tissue and cerebrospinal fluid detected high levels of BKV DNA. Sequencing studies further identified novel BKV variant and disclosed unique rearrangements in the noncoding control region of the viral DNA (BKVN NCCR). Neuropathological analysis also demonstrated an unusual form of obliterative fibrosing vasculopathy in the subcortical white matter with abnormal lysosomal accumulations, possibly related to the patient's underlying ectodermal dysplasia. Our report provides the first neuropathological description of HED-ID due to NEMO mutation, and expands the diversity of neurological presentations of BKV infection in brain, underscoring the importance of its consideration in immunodeficient patients with unexplained encephalopathy. We also document novel BKVN NCCR rearrangements that may be associated with the unique neuronal tropism in this patient.

  6. Update on the efficacy of extracorporeal shockwave treatment for myofascial pain syndrome and fibromyalgia.

    Science.gov (United States)

    Ramon, Silvia; Gleitz, Markus; Hernandez, Leonor; Romero, Luis David

    2015-12-01

    Chronic muscle pain syndrome is one of the main causes of musculoskeletal pathologies requiring treatment. Many terms have been used in the past to describe painful muscular syndromes in the absence of evident local nociception such as myogelosis, muscle hardening, myalgia, muscular rheumatism, fibrositis or myofascial trigger point with or without referred pain. If it persists over six months or more, it often becomes therapy resistant and frequently results in chronic generalized pain, characterized by a high degree of subjective suffering. Myofascial pain syndrome (MPS) is defined as a series of sensory, motor, and autonomic symptoms caused by a stiffness of the muscle, caused by hyperirritable nodules in musculoskeletal fibers, known as myofascial trigger points (MTP), and fascial constrictions. Fibromyalgia (FM) is a chronic condition that involves both central and peripheral sensitization and for which no curative treatment is available at the present time. Fibromyalgia shares some of the features of MPS, such as hyperirritability. Many treatments options have been described for muscle pain syndrome, with differing evidence of efficacy. Extracorporeal Shockwave Treatment (ESWT) offers a new and promising treatment for muscular disorders. We will review the existing bibliography on the evidence of the efficacy of ESWT for MPS, paying particular attention to MTP (Myofascial Trigger Point) and Fibromyalgia (FM). Copyright © 2015 IJS Publishing Group Limited. Published by Elsevier Ltd. All rights reserved.

  7. Clinical application of dynamic lung imaging by the single breath measurement with carbon-11-labeled CO/sub 2/, CO and nitrogen-13-labeled N/sub 2/

    Energy Technology Data Exchange (ETDEWEB)

    Kimura, K; Rikitake, T; Hasegawa, S [Tsukuba Univ., Sakura, Ibaraki (Japan); Matsumoto, T; Tateno, Y

    1979-09-01

    The single breath measurement with /sup 11/CO/sub 2/, /sup 11/CO and /sup 13/N-N composed of inhalation system and a coincidence positron camera interfaced with an on-line computer system has been used to evaluated regional pulmonary function in two normal volunteers and four patients with chronic obstructive pulmonary disease (COPD). In serial images of normal subjects after single inhalation of /sup 11/CO/sub 2/, the appearance time of the heart was within about 10 - 20 seconds and the radioactivity at heart area remained relatively high compared with lung fields. However in some patients with COPD, the heart appearance time was delayed and the hypo-ventilated lung area became gradually hot during the wash-out phase. The heart appearance time after an inspiration of /sup 11/CO was within 10 - 20 seconds in normal subject. In contrast, the appearance time was remarkably prolonged in the patients with disturbance of diffusion capacity. Immediately after single breath of /sup 13/N-N in the patient with COPD the well ventilated lung area was revealed as a region of high radioactivity and the distribution of the slow space was showed in the late phase of wash out. These findings should have patho-physiological diagnostic usefulness, especially for the patients with COPD and fibrosing lung disease.

  8. Diagnostic imaging of digestive tract involvement in cystic fibrosis. Part 2: pancreatic and gastrointestinal disease

    International Nuclear Information System (INIS)

    Berrocal, T.; Prieto, C.; Miralles, M.; Pozo, G. del; Martinez, A.; Manzanares, J.

    1998-01-01

    Cystic fibrosis (CF) is the most common fatal, autosomal recessive disease among the white population. Although recurrent pulmonary infections and pulmonary insufficiency are the major causes of morbidity and mortality, gastrointestinal symptoms generally present earlier and may suggest the diagnosis in the newborn or even prior to birth. The changes are attributed to the secretion of an abnormally thick mucous into the intestinal lumen, leading to the hallmark of diseases of the digestive tract: obstruction. This can be detected at birth in the form of mecanium ileus, ileal atresia, mecanium peritonitis and mecomiun plug, or present later on in childhood and adolescence as distal bowel obstruction syndrome or fibrosing colonopathy. This thick mucous can also trigger intussusception or acute appendicitis. Pancreatic insufficiency or pancreatic enzyme replacement therapy is the direct cause of most of these disorders. Plain radiography is of the utmost utility in assessing the digestive tract in CF. When the disease is detected in a newborn, the recommended approach is to perform plain abdominal X-ray, followed by barium enema, always accompanied by ultrasound. In older children and adolescents, enema and ultrasound are usually sufficient, although computed tomography and magnetic resonance may sometimes be necessary. (Author) 52 refs

  9. Síndrome de Erasmus: silicose e esclerose sistêmica

    Directory of Open Access Journals (Sweden)

    Aryádine Allinne Machado de Miranda

    2013-06-01

    Full Text Available A silicose é a pneumoconiose mais frequente, decorrente da inalação de sílica ou de poeiras minerais contendo silicatos, caracterizada principalmente pela fibrose pulmonar de caráter irreversível. Está associada com o desenvolvimento de outras doenças, incluindo tuberculose pulmonar, câncer pulmonar e doenças autoimunes. A doença do tecido conjuntivo posterior à exposição à sílica ocorre geralmente após 15 anos do início da exposição. A síndrome de Erasmus refere-se ao desenvolvimento de esclerose sistêmica em indivíduos previamente expostos à sílica e que apresentavam ou não silicose. Os autores relatam dois casos de pacientes com diagnóstico de silicose que desenvolveram esclerose sistêmica.

  10. How to combine hormonotherapy and radiation treatment in adjuvant breast cancer?

    International Nuclear Information System (INIS)

    Azria, D.; Llacer Moscardo, C.; Lemanski, C.; Ozsahin, M.; Gligorov, J.; Zaman, K.; Jacot, W.; Belkacemi, Y.

    2008-01-01

    Combined radiation and hormone therapies have become common clinical practice in recent years for locally-advanced prostate cancers. The use of such concomitant therapy in the treatment of breast disease has been infrequently reported in the literature, but seems justified given the common hormonal dependence of breast cancer and the potential synergistic effect of these two treatment modalities. As adjuvant therapy, two strategies are used in daily clinical practice: up front aromatase inhibitors or sequentially after a variable delay of tamoxifen. These molecules may, thus, interact with radiotherapy. Retrospective studies recently published did not show any differences in terms of locoregional recurrences between concurrent or sequential radio hormonotherapy. Lung and skin fibroses due to concurrent treatment are still under debate. Nevertheless, late side effects appeared to be increased by such a treatment, particularly in hypersensitive patients identified at risk by the lymphocyte predictive test. Concurrent radio hormonotherapy should, thus, be delivered cautiously at least for these patients. This article details the potent advantages and risks of concurrent use of adjuvant hormonotherapy and radiotherapy in localized breast cancers. (authors)

  11. Single stage circumferential lingual mucosal graft urethroplasty in near obliterative bulbar urethra stricture: A novel technique.

    Science.gov (United States)

    Sharma, Umesh; Yadav, Sher Singh; Tomar, Vinay; Garg, Amit

    2016-01-01

    This is a prospective study of the use and efficacy of a novel technique of circumferential tubularised lingual mucosal graft (LMG) in obliterative and near obliterative bulbar urethral stricture of >2 cm where excisional and augmented anastomotic urethroplasty are not feasible. The stenotic urethral segment was opened dorsally in midline and fibrosed urethra was excised taking care to preserve the healthy spongiosum tissue. LMG (av. Length 3 cm) was placed from one end of corporal body towards spongy tissue in a circumferential manner. Another LMG was placed in similar manner to deal with longer stricture. The urethra was tubularised over 14 Fr silicone catheter. A total of 12 men, of mean age 47 years underwent this procedure. The mean follow up period was 11 months starting from July 2014 till manuscript submission. Follow up included voiding cystourethrogram at 3 weeks, cystoscopy at 3 months (one patient didn't turned up) and subsequent follow up. Mean stricture length was 4.66 cm (range, 3-8.5 cm) and mean operative time was 195 min. (range, 160 to 200 min.). The technique was successful (normal voiding with no need for any post-operative procedure) in 11(91.6%) patients. One patient developed early recurrence at 4 month of surgery and had anastomotic stricture which was successfully managed by direct visual internal urethrotomy. Single stage circumferential tubularised graft urethroplasty is an excellent technique for strictures that include segments of obliterative and near obliterative diseased urethra. It provide a wider neourethra than patch graft urethroplasty.

  12. Pulmonary venous thrombosis secondary to radiofrequency ablation of the pulmonary veins

    Directory of Open Access Journals (Sweden)

    Raquel López-Reyes

    Full Text Available Background: Pulmonary Vein Thrombosis (PVT is a rare and underdiagnosed entity produced by local mechanical nature mechanisms, vascular torsion or direct injury to the vein. PVT has been described in clinical cases or small multicenter series mainly in relation to pulmonary vein stenosis, metastatic carcinoma, fibrosing mediastinitis, as an early surgical complication of lung transplantation lobectomy and radiofrequency ablation performed in patients with atrial fibrillation, although in some cases the cause is not known. Case: We report the case of a 57 years old male with history of atrial fibrillation treated by radiofrequency ablation who was admitted in our center because of a two-week history of consistent pleuritic pain in the left hemithorax and low-grade hemoptysis and a lung consolidation treated as a pneumonia with antibiotic but not responding to medical therapy. In view of the poor evolution of the patient, computed tomography angiography was performed with findings of PVT and secondary venous infarction and anticoagulation therapy was optimized. At the end, pulmonary resection was performed due to hemorrhagic recurrence. Conclusion: PVT remains a rare complication of radiofrequency ablation and other procedures involving pulmonary veins. Clinical suspicion and early diagnosis is crucial because is a potentially life-threatening entity. Keywords: Venous thrombosis, Atrial fibrilation, Radiofrequency ablation, Hemoptysis, Lung consolidation, Lung infarction, Lung resection surgery

  13. Pulmonary venous thrombosis secondary to radiofrequency ablation of the pulmonary veins.

    Science.gov (United States)

    López-Reyes, Raquel; García-Ortega, Alberto; Torrents, Ana; Feced, Laura; Calvillo, Pilar; Libreros-Niño, Eugenia Alejandra; Escrivá-Peiró, Juan; Nauffal, Dolores

    2018-01-01

    Pulmonary Vein Thrombosis (PVT) is a rare and underdiagnosed entity produced by local mechanical nature mechanisms, vascular torsion or direct injury to the vein. PVT has been described in clinical cases or small multicenter series mainly in relation to pulmonary vein stenosis, metastatic carcinoma, fibrosing mediastinitis, as an early surgical complication of lung transplantation lobectomy and radiofrequency ablation performed in patients with atrial fibrillation, although in some cases the cause is not known. We report the case of a 57 years old male with history of atrial fibrillation treated by radiofrequency ablation who was admitted in our center because of a two-week history of consistent pleuritic pain in the left hemithorax and low-grade hemoptysis and a lung consolidation treated as a pneumonia with antibiotic but not responding to medical therapy. In view of the poor evolution of the patient, computed tomography angiography was performed with findings of PVT and secondary venous infarction and anticoagulation therapy was optimized. At the end, pulmonary resection was performed due to hemorrhagic recurrence. PVT remains a rare complication of radiofrequency ablation and other procedures involving pulmonary veins. Clinical suspicion and early diagnosis is crucial because is a potentially life-threatening entity.

  14. Congenital anomalies, hereditary diseases of the pancreas, acute and chronic pancreatitis; Entwicklungsstoerungen, angeborene Erkrankungen des Pankreas, akute und chronische Pankreatitis

    Energy Technology Data Exchange (ETDEWEB)

    Brambs, Hans-Juergen; Juchems, Markus [Universitaetsklinikum Ulm (Germany). Abt. fuer Diagnostische und Interventionelle Radiologie

    2011-06-15

    The most important congenital anomalies include pancreas divisum, annular pancreas and ectopic pancreas. Patients with pancreas divisum may be more susceptible to acute or chronic pancreatitis and patients with an annular pancreas may develop duodenal stenosis. In pancreas divisum the key finding is the visualization of the main duct draining into the duodenum via the small papilla, separated from the common bile duct. Annular pancreas may show as a well defined ring of pancreatic tissue that encircles the duodenum. Ectopic pancreas is usually asymptomatic but may give rise to abdominal complaints and may be confused with submucosal tumors. Acute pancreatitis is classified as mild or severe. In mild forms ultrasound is the imaging modality of choice whereas in severe forms with extensive pancreatic and peripancreatic necroses computed tomography is the favored method. It is crucial to identify signs and criteria that come along with an increased risk of infection of the necroses. MRI plays an inferior role in the assessment of acute pancreatitis. Chronic pancreatitis is a longstanding inflammatory and fibrosing process causing pain and loss of function. Cross-section imaging is particularly in demand for the detection of complications and the differentiation from pancreatic cancer. Autoimmune pancreatitis is a unique form of chronic pancreatitis characterized by lymphoplasmacytic infiltration and fibrosis, and favourable response to corticosteroid treatment. (orig.)

  15. Experimental Chagas' disease in dogs

    Directory of Open Access Journals (Sweden)

    Marta de Lana

    1992-03-01

    Full Text Available This paper describes the development of experimental Chagas' disease in 64 out-bred young dogs. Twenty-nine animals were inoculated with the Be-62 and 35 with Be-78 Trypanosoma cruzi strains. Twenty-six were infected with blood trypomastigotes by different inoculation routes and 38 with metacyclic trypomastigotes from the vector via the conjunctival route. Twenty of the 26 dogs infected with blood trypomastigotes were autopsied during the acute phase. Eleven died spontaneously and nine were sacrificed. Six remained alive until they died suddenly (two or were autopsied (four. Twelve of the 38 dogs infected with metacyclic trypomastigotes evolved naturally to the chronic phase and remained alive for 24-48 months. The parasitemia, clinical aspects and serology (IgM and IgG as well as electrocardiogram, hemogram and heart anatomo-histopathologic patterns of acute and chronic cardiac forms of Chagas' disease as seen in human infections, were reproduced. The most important finding is the reproductibility of diffuse fibrosing chronic chagasic cardiopathy in all dogs infected with Be-78 T. cruzi strain autopsied between the 90th and 864th days of infection. Thus, the dog can be considered as a suitable experimental model to study Chagas' disease according to the requisites of the World Health Organization (1984. Futhermore the animal is easily obtained and easy to handle and maintain in experimental laboratory conditions.

  16. Cystic Shape Cemento-Ossifying Fibroma of Ethmoid Sinus.

    Science.gov (United States)

    Shejbal, Dražen; Vonsović, Gabrijela; Baudoin, Tomislav; Vagic, Davor

    2015-06-01

    Cemento-ossifying fibromas are a group of rarely occurring benign tumours, developing from the periodontal membrane and varying considerably in appearance and in the progress of the disease. Their common feature is higher or lower production of cemental tissue. In most cases the tumours are small because their cementoma mature quickly and become inactive, which causes the tumour to stop growing. They develop most frequently in the mandible and also in the maxilla. Other sites, such as paranasal cavities, soft tissues and bones of the head, are extremely rare. The case of a cemento-fibrosing tumour with psammoma infiltrations, developing from the ethmoid sinus in a nine-year-old girl is reported. Due to frontal headaches and sight defects as well as impaired vision on the right side, NMR was done, which showed a mucocele of the front and rear ethmoid with destruction of the orbital wall and a breakthrough into the orbit. The right maxillary sinus showed a visible retention and a thickened mucous membrane. A rhinoscopy revealed a ball-shaped spherical mass in the medial nasal meatus, which was defined as concha bullosa. An endoscopic examination showed that the tumour protruded in front of the medium nasal concha into the right nasal cavity, softened the ethmoid roof, penetrated toward the base of the skull, adhered and pushed the orbit. It was removed by FESS technique, and PHD revealed subsequently that it was not a mucocele but a cemento-ossifying fibroma.

  17. Factors Promoting Development of Fibrosis in Crohn’s Disease

    Directory of Open Access Journals (Sweden)

    Gerhard Rogler

    2017-07-01

    Full Text Available The concepts on the pathophysiology of intestinal fibrosis in Crohn’s disease (CD have changed in recent years. Some years ago fibrosis was regarded to be a consequence of long-standing inflammation with subsequent destruction of the gut wall matrix followed by scar formation and collagen deposition. Fibrosis in CD patients appeared to be an irreversible process that could hardly be influenced. Therefore, the main target in CD therapy was to control inflammation to avoid fibrosis development. Many of these assumptions seem to be only partially true. Inflammation may be a necessary prerequisite for the initiation of fibrosis. However, when the pathophysiologic processes that lead to fibrosis in CD patients have been initiated fibrosis development may be independent of inflammation and may continue even when inflammation is under good medical control. Fibrosis in CD also may be reversible. After strictureplasty local collagen deposits decrease or even disappear. With new animal models for intestinal fibrosis on the horizon, we need to spend more efforts on understanding the factors influencing fibrosis in CD patients to finally find specific therapies. In this context, it will be as important to find markers and quantitative imaging tools to have reliable endpoints for clinical trials in fibrosing CD.

  18. Consensus document for the diagnosis and treatment of idiopathic pulmonary fibrosis: Joint Consensus of Sociedade Portuguesa de Pneumologia, Sociedade Portuguesa de Radiologia e Medicina Nuclear e Sociedade Portuguesa de Anatomia Patológica.

    Science.gov (United States)

    Robalo Cordeiro, C; Campos, P; Carvalho, L; Campainha, S; Clemente, S; Figueiredo, L; Jesus, J M; Marques, A; Souto-Moura, C; Pinto Basto, R; Ribeiro, A; Serrado, M; Morais, A

    2016-01-01

    Idiopathic pulmonary fibrosis is a rare interstitial lung disease included in the Idiopathic Interstitial Pneumonias group. Although several potential risk factors have been described, it is a progressive fibrosing disease of unknown cause affecting mainly adults over 50 years and associated with a poor prognosis, reflected in a median survival of 2-3 years after diagnosis. The concept of a multidisciplinary working group for the diagnosis of idiopathic pulmonary fibrosis is based on the need to have experienced pulmonologists, radiologists and pathologists in the evaluation and correct treatment of the disease, and requires the use of all available data about individual patients, standardized (largely through High Resolution Computed Tomography and pathology when needed) as well as non-standardized data (laboratory, serology and biomarkers). This approach helps to increase diagnostic accuracy and is an internationally accepted recommendation. In regard to therapy, the situation has changed radically since the publication of the ATS/ERS/JRS/ALAT 2011 guidelines on the diagnosis and management of idiopathic pulmonary fibrosis where it was stressed that no proven therapy exists for this disease. Currently besides non-pharmacological treatment, therapy of complications and comorbidities and palliative care, nintedanib and pirfenidone, two compounds with pleiotropic mechanisms of action, are to date, the two drugs with confirmed efficacy in slowing functional decline and disease progression in idiopathic pulmonary fibrosis patients. Copyright © 2016 Sociedade Portuguesa de Pneumologia. Published by Elsevier España, S.L.U. All rights reserved.

  19. Imaging appearances of synovial plicae syndrome of the knee

    Directory of Open Access Journals (Sweden)

    Osama Abdalla Mabrouk Kheiralla

    2016-08-01

    Full Text Available Synovial plicae are synovial folds that may be found as intraarticular structures within the knee joint. They are remnants of incomplete resorption of mesenchymal tissue during fetal development. Synovial plicae, if present, are supposed to be non-pathological and asymptomatic, however if they are exposed to special events like direct trauma or repeated activities, they may be inflamed and become fibrosed and rigid and irritates the synovium of the underlying femoral condyle resulting in secondary mechanical synovitis and chondromalacia leading to what is known as plica syndrome of the knee. Inspite plica syndrome is always suspected on clinical bases and can be clearly visualized by arthroscopic application, still diagnostic imaging by MRI, CT scan and Sonography play important role in the evaluation and diagnosis of this pathological condition. The aim of this article is to provide an overview of the imaging appearances of synovial plicae syndrome of the knee on ultrasound, magnetic resonance imaging (MRI and computerized tomography scan (CT scan.

  20. The study of the lung accumulation of I-123 IMP by the broncho-alveolar lavage

    International Nuclear Information System (INIS)

    Itasaka, Miyoko; Ikeda, Hideki; Yakuwa, Naoshi; Kato, Shuichi; Takahashi, Keiji; Yasui, Shoji

    1989-01-01

    We studied the accumulated portion and the movement of I-123 IMP in the lung. Ten subjects were studied. They were four patients with fibrosing lung disease, two with lung cancer, and four with other lung disease. They underwent the broncho-alveolar lavage (BAL) for the diagnosis of their diseases. l.5 mCi of I-123 IMP was injected into the ante-cubital vein. The BAL examination was carried out about 40 minutes after the injection of I-123 IMP. The subjects' blood was sampled at the same time. The total BAL liquid (BAL-T) was divided into the fluid component (BAL-F) and the cell component (BAL-C) by centrifugation. The radioactivities in BAL-T, BAL-F, BAL-C, and serum (B-S) were measured by the well-counter. The average of BAL-T/B-S, BAL-F/B-S and BAL-C/B-S were 6.86, 4.26 and 2.71 respectively. It was confirmed that I-123 IMP was transported from the pulmonary capillary to the alveolar space and was taken up by the alveolar cells. It was considered that the analysis of the I-123 IMP release from the lung showed not only the endothelial cell uptake function but also the interstitial and material cells' amine transport and uptake function. (author)

  1. Nephrogenic systemic fibrosis: chronic imaging findings and review of the medical literature

    International Nuclear Information System (INIS)

    Weigle, Jeffrey P.; Broome, Dale R.

    2008-01-01

    Nephrogenic systemic fibrosis (NSF) is a systemic fibrosing disorder which has been strongly associated with exposure to gadolinium-based contrast media (GBCM) in the setting of renal insufficiency. Although this disorder primarily affects the skin, it can result in severe joint contractures, disabilities and even death. However, to date, there have been no published studies reporting chronic imaging findings of NSF. In this report we present three biopsy-proven cases of NSF with the associated chronic MRI, radiographic and bone scintigraphy findings. Two of the patients had been exposed to gadodiamide, and one had been exposed to gadopentetate dimeglumine prior to the onset of NSF. Two are newly reported cases. One patient's subacute imaging findings have previously been reported, but significant chronic images will now be presented. This patient became severely disabled from contractures and developed long bone smooth periosteal reaction, extensive intra-articular and periarticular calcifications, musculotendinous heterotopic ossification and ankylosis of several joints. One of the patients underwent renal transplantation 6 months after GBCM exposure, with near complete resolution of the skin fibrosis. The third patient had persistent MRI findings of skin thickening, with low T1 and high T2 signal intensity 5 years after exposure to gadodiamide. A review of the medical literature is provided, emphasizing the association of NSF with various GBCM. These cases broaden our understanding of the long-term imaging findings and complications of NSF and the stratified risk of NSF with various GBCM. (orig.)

  2. Clinical Update in Aspects of the Management of Autoimmune Thyroid Diseases

    Directory of Open Access Journals (Sweden)

    Duncan J. Topliss

    2016-12-01

    Full Text Available Aspects of autoimmune thyroid disease updated in this review include: immunoglobulin G4 (IgG4-related thyroid disease (Riedel's thyroiditis, fibrosing variant of Hashimoto's thyroiditis, IgG4-related Hashimoto's thyroiditis, and Graves' disease with elevated IgG4 levels; recent epidemiological studies from China and Denmark indicating that excess iodine increases the incidence of Hashimoto's thyroiditis and hypothyroidism; immunomodulatory agents (ipilimumab, pembrolizumab, nivolumab activate immune response by inhibiting T-cell surface receptors which down-regulate immune response, i.e., cytotoxic T-lymphocyte antigen 4 and programmed cell death protein 1 pathways; alemtuzumab is a humanised monoclonal antibody to CD52 which causes immune depletion and thyroid autoimmune disease especially Graves' hyperthyroidism; small molecule ligand (SML agonists which activate receptors, SML neutral antagonists, which inhibit receptor activation by agonists, and SML inverse agonists which inhibit receptor activation by agonists and inhibit constitutive agonist independent signaling have been identified. SML antagonism of thyroid-stimulating hormone-receptor stimulatory antibody could treat Graves' hyperthyroidism and Graves' ophthalmopathy; and thyroxine treatment of subclinical hypothyroidism can produce iatrogenic subclinical hyperthyroidism with the risk of atrial fibrillation and osteoporosis. The increased risk of harm from subclinical hyperthyroidism may be stronger than the potential benefit from treatment of subclinical hypothyroidism.

  3. Pulmonary manifestations of Sjögren's syndrome

    Directory of Open Access Journals (Sweden)

    Thomas Flament

    2016-06-01

    Full Text Available In 9–20% of cases, Sjögren's syndrome is associated with various respiratory symptoms. The most typical manifestations are chronic interstitial lung disease (ILD and tracheobronchial disease. The most common manifestation of ILD is nonspecific interstitial pneumonia in its fibrosing variant. Other types of ILD, such as organising pneumonia, usual interstitial pneumonia and lymphocytic interstitial pneumonitis, are rare. Their radiological presentation is less distinctive, and definitive diagnosis may require the use of transbronchial or surgical lung biopsy. Corticosteroid therapy is the mainstay of ILD treatment in Sjögren's syndrome, but the use of other immunosuppressive drugs needs to be determined. ILD is a significant cause of death in Sjögren's syndrome. Tracheobronchial disease is common in Sjögren's syndrome, characterised by diffuse lymphocytic infiltration of the airway. It is sometimes responsible for a crippling chronic cough. It can also present in the form of bronchial hyperresponsiveness, bronchiectasis, bronchiolitis or recurrent respiratory infections. The management of these manifestations may require treatment for dryness and/or inflammation of the airways. Airway disease has little effect on respiratory function and is rarely the cause of death in Sjögren's syndrome patients. Rare respiratory complications such as amyloidosis, lymphoma or pulmonary hypertension should not be disregarded in Sjögren's syndrome patients.

  4. Inflammatory/granulomatous diseases of the lung

    International Nuclear Information System (INIS)

    Ivancevic, V.; Munz, D.L.

    1998-01-01

    The term 'inflammatory' and 'granulomatous' lung disease represents a pool of many etiologically different diseases, the pathologic mechanisms of which are characterized by inflammatory reactions of varying intensity and cell composition. In sarcoidosis and other granulomatous diseases as well as in lung fibroses, gallium scintigraphy allows reliable non-invasive estimation of alveolitis activity and is suitable for therapy monitoring. Granulomatous diseases seem to be detectable sensitively by means of somatostatin receptor scintigraphy as well. It is yet uncertain, whether positron emission tomography with F-18 fluordeoxyglucose will play a role in quantitative assessment of disease activity in sarcoidosis. Gallium scintigraphy is very useful in the early detection of pulmonary complications in AIDS patients. Pneumocystis carinii pneumonia, which is important in this patient population, can also be detected by both Tc-99m and In-111 labelled polyclonal human immunoglobulin, and in future possibly with a monoclonal antibody fragment against Pneumocystis carinii as well. The significance of primary bacterial pneumonias has decreased and nuclear medicine procedures for diagnosing inflammation are needed only exceptionally in this indication. (orig.) [de

  5. Cholesterol crystallization within hepatocyte lipid droplets and its role in murine NASH.

    Science.gov (United States)

    Ioannou, George N; Subramanian, Savitha; Chait, Alan; Haigh, W Geoffrey; Yeh, Matthew M; Farrell, Geoffrey C; Lee, Sum P; Savard, Christopher

    2017-06-01

    We recently reported that cholesterol crystals form in hepatocyte lipid droplets (LDs) in human and experimental nonalcoholic steatohepatitis. Herein, we assigned WT C57BL/6J mice to a high-fat (15%) diet for 6 months, supplemented with 0%, 0.25%, 0.5%, 0.75%, or 1% dietary cholesterol. Increasing dietary cholesterol led to cholesterol loading of the liver, but not of adipose tissue, resulting in fibrosing steatohepatitis at a dietary cholesterol concentration of ≥0.5%, whereas mice on lower-cholesterol diets developed only simple steatosis. Hepatic cholesterol crystals and crown-like structures also developed at a dietary cholesterol concentration ≥0.5%. Crown-like structures consisted of activated Kupffer cells (KCs) staining positive for NLRP3 and activated caspase 1, which surrounded and processed cholesterol crystal-containing remnant LDs of dead hepatocytes. The KCs processed LDs at the center of crown-like structures in the extracellular space by lysosomal enzymes, ultimately transforming into lipid-laden foam cells. When HepG2 cells were exposed to LDL cholesterol, they developed cholesterol crystals in LD membranes, which caused activation of THP1 cells (macrophages) grown in coculture; upregulation of TNF-alpha , NLRP3, and interleukin 1beta ( IL1β ) mRNA; and secretion of IL-1beta. In conclusion, cholesterol crystals form on the LD membrane of hepatocytes and cause activation and cholesterol loading of KCs that surround and process these LDs by lysosomal enzymes.

  6. Esclerose sistêmica e sarcoidose Scleroderma and sarcoidosis

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    Fernanda Guidolin

    2005-10-01

    Full Text Available Os autores descrevem o caso de uma paciente com esclerose sistêmica (ES - forma limitada - com comprometimento pulmonar tipo fibrose intersticial. Após sete anos sem acompanhamento, foram identificados gânglios mediastinais e esplenomegalia. A biópsia de linfonodos mostrou granuloma não caseoso sugestivo de sarcoidose. Estamos mostrando, neste caso, a associação de ES e sarcoidose, para chamar a atenção para esse fato e enfatizar que a sarcoidose deve ser lembrada no diagnóstico diferencial das complicações pulmonares da esclerodermia.The authors describe the case of a patient with limited scleroderma and interstitial lung disease. Follow-up was lost for seven years, when patient returned presenting nodular mediastinal enlargement and splenomegaly. Lymph node biopsy showed granulomatous lesions without caseum suggestive of sarcoidosis. This case is being presented to remind the association of scleroderma and sarcoidosis as a possible differential diagnosis of scleroderma pulmonary complications.

  7. Detection of pericardial inflammation with late-enhancement cardiac magnetic resonance imaging: initial results

    International Nuclear Information System (INIS)

    Taylor, Andrew M.; Dymarkowski, Steven; Bogaert, Jan; Verbeken, Eric K.

    2006-01-01

    To examine the value of late-enhancement cardiac magnetic resonance imaging (MRI) for detection of pericardial inflammation. Late-enhancement cardiac MRI was performed in 16 patients with clinical suspicion of pericardial disease. Pericardial effusion, pericardial thickening and pericardial enhancement were assessed. MRI findings were compared with those of definitive pericardial histology (n=14) or microbiology (n=2). A control group of 12 patients with no clinical evidence of pericardial disease were also imaged with the same MRI protocol. Sensitivity and specificity for late-enhancement MRI detection of pericardial inflammation was of 100%. There was MRI late enhancement of the pericardial layers in all five patients with histological/microbiological evidence of inflammatory pericarditis. MRI demonstrated no pericardial thickening and no MRI late enhancement with or without a pericardial effusion in any of the five patients with histological evidence of a normal pericardium. MRI detected pericardial thickening in the absence of both pericardial effusion and late enhancement in all six patients with histological evidence of chronic fibrosing pericarditis. The 12 control subjects showed no evidence of pericardial MRI late enhancement. These findings demonstrate that MRI late enhancement can be used to visualize pericardial inflammation in patients with clinical suspicion of pericardial disease. (orig.)

  8. Aspectos raciais dos "megas" e da cardiopatia na doença de Chagas crônica

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    Lineu José Miziara

    1981-06-01

    Full Text Available O estudo de 724 pacientes chagásicos crônicos mostrou que a insuficiência cardíaca congestiva é mais freqüente e de aparecimento mais precoce nos pacientes de raça negra do que nos brancos. A ocorrência de "megas" foi ligeiramente inferior nos chagásicos negros não sendo estatisticamente significativa a diferença observada. A maior freqüência de insuficiência cardíaca nos pretos parece estar relacionada a características biológicas do tecido conjuntivo que condicionam uma resposta fibrosante mais acentuada no miocârdio agredido pela Tripanossomose. Estas observações estariam de acordo com outros estudos sobre a doença de Chagas que admitem ser a denervação o fator mais importante para o aparecimento dos "megas" e a inflamação com fibrose miocârdica acentuada um elemento básico para explicar a insuficiência cardíaca.

  9. Paralisia do nervo ulnar na lepra sem alterações cutâneas: biópsia do ramo superficial do nervo ulnar na mão

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    FREITAS MARCOS R. G. DE

    1998-01-01

    Full Text Available A lepra constitui causa frequente de acometimento de nervos periféricos, em nosso meio. O sistema nervoso periférico é acometido por vezes sem que haja alterações cutâneas: é a chamada forma neurítica pura. Nessa variante, o nervo mais afetado é o ulnar. Nos casos de acometimento isolado de nervos periféricos somente a feitura de biópsia de nervo conduzirá ao diagnóstico. Assim, resolvemos realizar biópsia do ramo sensitivo superficial do nervo ulnar na mão em 17 pacientes com paresia ou paralisia desse nervo e espessamento do mesmo na altura do cotovelo. Os principais achados foram: redução do número de fibras mielínicas em 14 casos, infiltrado inflamatório em 13, fibrose em 12, desmielinização e remielinização em 9, presença de granuloma em 6 e visualização do Mycobacterium leprae em 5. Concluímos que a biópsia do ramo sensitivo superficial do nervo ulnar na mão é um bom meio diagnóstico de lepra em pacientes com acometimento desse nervo

  10. Sonographic Findings of Hashimoto's Thyroiditis and Associated Nodular Lesions

    International Nuclear Information System (INIS)

    Kang, Bong Joo; Park, Young Ha; Jung, So Lyung; Chung, Soo Kyo

    2007-01-01

    To evaluate the sonographic findings of Hashimoto's thyroiditis and associated nodular lesions. We retrospectively reviewed the sonographic findings of twenty patients who had surgically confirmed Hashimoto's thyroiditis between 1 March 2005, and 26 November 2005. In these patients, we reviewed the sonographic findings of the associated focal nodular lesion. Assessed were size, homogeneity, and echogenicity of the diseased thyroid gland and shape, echogenicity, margin, rim, microcal cification of the associated nodules. Without knowledge of the pathological diagnosis of the nodular lesions, based on the sonographic criteria, the nodules were classified as either malignant or benign. Hashimoto's thyroiditis demonstrates a variety of sonographic findings for size, homogeneity, and echogenicity. Among the nineteen nodules that were sonographically diagnosed and pathologically confirmed, nine papillary cancers, seven nodular hyperplasias, two Huthle cell adenomas, and one focal hyalinized fibrosing nodule were included. All of the nine papillary cancers showed more than one malignant finding such as marked hypoechogenicity, an irregular shape, a taller than wide shape, a spiculated margin, or microcalcifications that were classified as malignant nodulea, and all of the ten benign nodules showed no malignant findings. Circumscribed isoechoic, hyperechoic, or hypoechoic nodules without calcification were classified as bending nodules. Hashimoto's thyroiditis demonstrates various findings on a sonographic examination,and associated various benign and malignant lesions. Moreover, a sonographic examination is helpful to differentiate between malignant and benign lesions in Hashimoto's thyroiditis as in the normal thyroid

  11. Fatores relacionados ao prognóstico da atresia biliar pós-portoenterostomia Factors related to the post-portoenterostomy prognosis of biliary atresia

    Directory of Open Access Journals (Sweden)

    Jorge Luiz dos Santos

    2002-01-01

    Full Text Available Objetivo: o estudo está envolvido com o prognóstico da atresia biliar pós-portoenterostomia, presença de anomalias congênitas associadas à doença e de malformação de placa ductal, área de fibrose hepática e, sobretudo, com a idade dos pacientes por ocasião da cirurgia. O presente estudo verificou numa amostra de atresia biliar as implicações prognósticas destes fatores. Métodos: foram avaliados 47 pacientes com atresia biliar, em estudo de corte transversal. O material histológico dos casos foi marcado com anticorpo anticitoqueratina 19 e CAM 5.2 por método imunoistoquímico, para o estudo das estruturas biliares, e corado com picrossírius para avaliação da área de fibrose. O estudo das estruturas biliares foi realizado por dois patologistas e pelo primeiro autor deste estudo, "cegos" quanto à evolução dos casos. A mensuração da área de fibrose foi quantitativa. Os dados dos pacientes em relação à idade, ocorrência de óbito ou realização de transplante hepático foram pesquisados nos prontuários. Resultados: a idade por ocasião da portoenterostomia variou entre 24 e 251 dias de vida (90,4 + 44,8 dias, e em 32 casos (72% a evolução pôde ser acompanhada. Os 9 casos (19% com anomalias congênitas extra-hepáticas associadas não diferiram quanto ao prognóstico em relação ao restante da amostra. A idade por ocasião da portoenterostomia influenciou o prognóstico (p=0,016. A área de fibrose foi diferente entre pacientes operados com menos de 60 dias de vida e os operados com mais de 90 dias (p= 0,023, mas não influenciou a evolução dos casos. Tampouco a presença de malformação de placa ductal influiu no prognóstico. Conclusões: a idade por ocasião da portoenterostomia foi o único fator que afetou o prognóstico dos casos de atresia biliar. É necessário maior número de pacientes para avaliar a influência da presença de anomalias congênitas extra-hepáticas associadas sobre a evolução p

  12. HFE gene variants and iron-induced oxygen radical generation in idiopathic pulmonary fibrosis.

    Science.gov (United States)

    Sangiuolo, Federica; Puxeddu, Ermanno; Pezzuto, Gabriella; Cavalli, Francesco; Longo, Giuliana; Comandini, Alessia; Di Pierro, Donato; Pallante, Marco; Sergiacomi, Gianluigi; Simonetti, Giovanni; Zompatori, Maurizio; Orlandi, Augusto; Magrini, Andrea; Amicosante, Massimo; Mariani, Francesca; Losi, Monica; Fraboni, Daniela; Bisetti, Alberto; Saltini, Cesare

    2015-02-01

    In idiopathic pulmonary fibrosis (IPF), lung accumulation of excessive extracellular iron and macrophage haemosiderin may suggest disordered iron homeostasis leading to recurring microscopic injury and fibrosing damage. The current study population comprised 89 consistent IPF patients and 107 controls. 54 patients and 11 controls underwent bronchoalveolar lavage (BAL). Haemosiderin was assessed by Perls' stain, BAL fluid malondialdehyde (MDA) by high-performance liquid chromatography, BAL cell iron-dependent oxygen radical generation by fluorimetry and the frequency of hereditary haemochromatosis HFE gene variants by reverse dot blot hybridisation. Macrophage haemosiderin, BAL fluid MDA and BAL cell unstimulated iron-dependent oxygen radical generation were all significantly increased above controls (pHFE allelic variants was markedly higher in IPF compared with controls (40.4% versus 22.4%, OR 2.35, p=0.008) and was associated with higher iron-dependent oxygen radical generation (HFE variant 107.4±56.0, HFE wild type (wt) 59.4±36.4 and controls 16.7±11.8 fluorescence units per 10(5) BAL cells; p=0.028 HFE variant versus HFE wt, p=0.006 HFE wt versus controls). The data suggest iron dysregulation associated with HFE allelic variants may play an important role in increasing susceptibility to environmental exposures, leading to recurring injury and fibrosis in IPF. Copyright ©ERS 2015.

  13. Periostin differentially induces proliferation, contraction and apoptosis of primary Dupuytren's disease and adjacent palmar fascia cells

    International Nuclear Information System (INIS)

    Vi, Linda; Feng, Lucy; Zhu, Rebecca D.; Wu, Yan; Satish, Latha; Gan, Bing Siang; O'Gorman, David B.

    2009-01-01

    Dupuytren's disease, (DD), is a fibroproliferative condition of the palmar fascia in the hand, typically resulting in permanent contracture of one or more fingers. This fibromatosis is similar to scarring and other fibroses in displaying excess collagen secretion and contractile myofibroblast differentiation. In this report we expand on previous data demonstrating that POSTN mRNA, which encodes the extra-cellular matrix protein periostin, is up-regulated in Dupuytren's disease cord tissue relative to phenotypically normal palmar fascia. We demonstrate that the protein product of POSTN, periostin, is abundant in Dupuytren's disease cord tissue while little or no periostin immunoreactivity is evident in patient-matched control tissues. The relevance of periostin up-regulation in DD was assessed in primary cultures of cells derived from diseased and phenotypically unaffected palmar fascia from the same patients. These cells were grown in type-1 collagen-enriched culture conditions with or without periostin addition to more closely replicate the in vivo environment. Periostin was found to differentially regulate the apoptosis, proliferation, α smooth muscle actin expression and stressed Fibroblast Populated Collagen Lattice contraction of these cell types. We hypothesize that periostin, secreted by disease cord myofibroblasts into the extra-cellular matrix, promotes the transition of resident fibroblasts in the palmar fascia toward a myofibroblast phenotype, thereby promoting disease progression.

  14. Monitoring the sensory quality of canned white asparagus through cluster analysis.

    Science.gov (United States)

    Arana, Inés; Ibañez, Francisco C; Torre, Paloma

    2016-05-01

    White asparagus is one of the 30 vegetables most consumed in the world. This paper unifies the stages of their sensory quality control. The aims of this work were to describe the sensory properties of canned white asparagus and their quality control and to evaluate the applicability of agglomerative hierarchical clustering (AHC) for classifying and monitoring the sensory quality of manufacturers. Sixteen sensory descriptors and their evaluation technique were defined. The sensory profile of canned white asparagus was high flavor characteristic, little acidity and bitterness, medium firmness and very light fibrosity, among other characteristics. The dendrogram established groups of manufacturers that had similar scores in the same set of descriptors, and each cluster grouped the manufacturers that had a similar quality profile. The sensory profile of canned white asparagus was clearly defined through the intensity evaluation of 16 descriptors, and the sensory quality report provided to the manufacturers is in detail and of easy interpretation. AHC grouped the manufacturers according to the highest quality scores in certain descriptors and is a useful tool because it is very visual. © 2015 Society of Chemical Industry. © 2015 Society of Chemical Industry.

  15. Comparing ultraviolet light A photo(chemo)therapy with Methotrexate protocol in childhood localized scleroderma: Evidence from systematic review and meta-analysis approach.

    Science.gov (United States)

    Marrani, Edoardo; Foeldvari, Ivan; Lopez, Jordi Anton; Cimaz, Rolando; Simonini, Gabriele

    2018-03-14

    Localized scleroderma is a skin fibrosing disorder that, if untreated, may result in severe disability. The purpose of this systematic review is to compare the present evidence concerning the effectiveness of Methotrexate versus phototherapy, alone or associated with Psoralen, in childhood localized scleroderma. A systematic search between January 1996 and May 2017 was performed to identify studies investigating the efficacy of Methotrexate (MTX) or phototherapy (UVA) for treating localized scleroderma with onset ≤18 years. Due to a lack of validated clinical criteria, four clinical response criteria were used to assess the treatment efficacy as primary outcome. We determined a combined estimate of the proportion of children responding to MTX and UVA. A total of 19 studies was included (8 MTX; 11 UVA). In the methotrexate group, 193 children were included in the analysis; in the phototherapy group, a total of 48 treated children. For both groups age, disease subtype, glucocorticoids (GCs) use, and side effects of treatment were also analyzed. The meta-analysis suggested that UVA and MTX protocols have both a favorable effect in active lesions of childhood localized scleroderma. However, MTX resulted significantly superior to UVA, with or without Psoralen. Our study supports the combination of MTX and GCs in patients with a high risk of complication. Phototherapy with UVA1 could represent a therapeutic option in patients with limited scleroderma, where lesions do not cross joints and they do not lead to potential cosmetic changes. Copyright © 2018 Elsevier Inc. All rights reserved.

  16. Supernatants from culture of type I collagen-stimulated PBMC from patients with cutaneous systemic sclerosis versus localized scleroderma demonstrate suppression of MMP-1 by fibroblasts.

    Science.gov (United States)

    Brown, Monica; Postlethwaite, Arnold E; Myers, Linda K; Hasty, Karen A

    2012-06-01

    Systemic sclerosis (SSc) is a chronic fibrosing disease characterized by vasculopathy, autoimmunity, and an accumulation of collagen in tissues. Numerous studies have shown that compared to healthy or diseased controls, the peripheral blood mononuclear cells (PBMC) from patients with SSc produce a variety of cytokines or proliferate when cultured with solubilized type I collagen (CI) or constituent α1(II) and α2(I) polypeptide chains. The purpose of this study was to determine whether PBMC isolated from patients with SSc and cultured in vitro with soluble CI elaborated soluble mediators that inhibit the production of collagenase (i.e., matrix metalloproteinase, MMP-1) by fibroblasts. Supernatants of CI-stimulated PBMC from juvenile and adult diffuse cutaneous (dc)SSc patients significantly reduced MMP-1 production by SSc dermal fibroblasts, while supernatants of CI-stimulated PBMC from patients with localized scleroderma (LS) did not. CI-stimulated PBMC culture supernatants from patients with dcSSc in contrast to patients with LS exhibited increased levels of platelet-derived growth factor (PDGF)-AA, PDGF-BB, TNF-α, IL-13, and EGF. Prolonged culture of SSc dermal fibroblasts with recombinant PDGF-BB or IL-13 inhibited the induction of MMP-1 in response to subsequent TNF-α stimulation. These data suggest that therapies aimed at reducing these cytokines may decrease collagen accumulation in SSc, preventing the development of chronic fibrosis.

  17. Scleroderma: nomenclature, etiology, pathogenesis, prognosis, and treatments: facts and controversies.

    Science.gov (United States)

    Fett, Nicole

    2013-01-01

    Scleroderma refers to a heterogeneous group of autoimmune fibrosing disorders. The nomenclature of scleroderma has changed dramatically in recent years, with morphea (localized scleroderma), limited cutaneous systemic sclerosis, diffuse cutaneous systemic sclerosis, and systemic sclerosis sine scleroderma encompassing the currently accepted disease subtypes. Major advances have been made in the molecular studies of morphea and systemic sclerosis; however, their etiologies and pathogenesis remain incompletely understood. Although morphea and systemic sclerosis demonstrate activation of similar inflammatory and fibrotic pathways, important differences in signaling pathways and gene signatures indicate they are likely biologically distinct processes. Morphea can cause significant morbidity but does not affect mortality, whereas systemic sclerosis has the highest disease-specific mortality of all autoimmune connective tissue diseases. Treatment recommendations for morphea and systemic sclerosis are based on limited data and largely expert opinions. Current collaborative efforts in morphea and systemic sclerosis research will hopefully lead to better understanding of the etiology and pathogenesis of these rare and varied diseases and improved treatment options. Published by Elsevier Inc.

  18. Possible involvement of gadolinium chelates in the pathophysiology of nephrogenic systemic fibrosis: A critical review

    International Nuclear Information System (INIS)

    Idee, Jean-Marc; Port, Marc; Medina, Christelle; Lancelot, Eric; Fayoux, Emmanuelle; Ballet, Sebastien; Corot, Claire

    2008-01-01

    Nephrogenic systemic fibrosis (NSF) is a recently described, highly debilitating scleroderma-like disease occurring in patients with severe or end-stage renal failure. NSF is characterized by cutaneous papules and coalescing plaques ('peau d'orange' appearance) and a wooden consistency. It may ultimately cause disabling contractures of several joints, thus making many patients wheelchair-dependent. NSF has been associated to prior administration of gadolinium chelates (GC) used as contrast agents for magnetic resonance imaging. The best available treatment option at the present time is renal transplantation. The mechanism of NSF has not been fully elucidated. Several hypotheses have been proposed so far and are critically discussed in the present review article. Gadolinium has been found in skin biopsy samples of patients. The most widely accepted hypothesis is related to dechelation of less stable GC, progressively releasing free Gd 3+ which may subsequently lead to the attraction of CD34+, CD45+, pro-collagen+ circulating fibrocytes via the release of chemokines, thereby inducing systemic fibrosing disorders. Pre-existing renal failure may facilitate the process by delaying the excretion of GC. A complex interplay between gadolinium and co-factors (pro-inflammatory status, vascular injury, high dose of erythropoietin, high levels of calcium, phosphorus, etc.) may occur in patients with impaired renal function. This and other hypotheses remain to be investigated, as well as the role and independence of co-factors

  19. Treatment of idiopathic pulmonary fibrosis in Australia and New Zealand: A position statement from the Thoracic Society of Australia and New Zealand and the Lung Foundation Australia.

    Science.gov (United States)

    Jo, Helen E; Troy, Lauren K; Keir, Gregory; Chambers, Daniel C; Holland, Anne; Goh, Nicole; Wilsher, Margaret; de Boer, Sally; Moodley, Yuben; Grainge, Christopher; Whitford, Helen; Chapman, Sally; Reynolds, Paul N; Glaspole, Ian; Beatson, David; Jones, Leonie; Hopkins, Peter; Corte, Tamera J

    2017-10-01

    Idiopathic pulmonary fibrosis (IPF) is a fibrosing interstitial lung disease (ILD) of unknown aetiology with a median survival of only 2-5 years. It is characterized by progressive dyspnoea and worsening lung function, ultimately resulting in death. Until recently, there were no effective therapies for IPF; however, with the publication of two landmark clinical trials in 2014, the anti-fibrotic therapies, nintedanib and pirfenidone, have gained widespread approval. This position paper aims to highlight the current evidence for the treatment of IPF, with particular application to the Australian and New Zealand population. We also consider areas in which evidence is currently lacking, especially with regard to the broader IPF severity spectrum and treatment of co-morbid conditions. The utility of non-pharmacological therapies including pulmonary rehabilitation, oxygen as well as symptom management thought to be important in the holistic care of IPF patients are also discussed. © 2017 The Authors. Respirology published by John Wiley & Sons Australia, Ltd on behalf of Asian Pacific Society of Respirology.

  20. Biopsy in idiopathic pulmonary fibrosis: back to the future.

    Science.gov (United States)

    Rossi, Giulio; Spagnolo, Paolo

    2017-09-01

    Idiopathic Pulmonary Fibrosis (IPF) is a relentlessly progressive, fibrosing interstitial pneumonia characterized by a radiologic and/or histologic pattern of usual interstitial pneumonia (UIP). The availability of two effective anti-fibrotic drugs in IPF has encouraged the identification and treatment of patients in early stages in order to maximize clinical benefit. The ability of high-resolution computed tomography (HRCT) to identify a 'definite' UIP pattern is suboptimal, particularly in the absence of honeycombing. Therefore, radiologic criteria for UIP are currently being redefined. Histology represents the major source of information to define a UIP pattern. Novel and less invasive approaches (particularly cryobiopsy) to sample interstitial lung diseases have demonstrated high sensitivity and specificity. In parallel, researchers are focusing on molecular mechanisms underlying IPF with the aim to identify more specific druggable targets. Lung tissue is therefore essential for diagnostic, pathogenetic and therapeutic purposes. Areas covered: We identified and critically reviewed the most relevant recent literature related to the limitations of current radiologic criteria, new lung sampling procedures, and molecular pathways in support of the need of lung tissue to better understand IPF. Expert commentary: The development of truly effective treatments for IPF requires the identification of key pathogenetic molecules and pathways. To this end, the availability of lung tissue is vital.

  1. Ten-Year Survival in Patients with Idiopathic Pulmonary Fibrosis After Lung Transplantation.

    Science.gov (United States)

    ten Klooster, Liesbeth; Nossent, George D; Kwakkel-van Erp, Johanna M; van Kessel, Diana A; Oudijk, Erik J; van de Graaf, Ed A; Luijk, Bart; Hoek, Rogier A; van den Blink, Bernt; van Hal, Peter Th; Verschuuren, Erik A; van der Bij, Wim; van Moorsel, Coline H; Grutters, Jan C

    2015-12-01

    Idiopathic pulmonary fibrosis (IPF) is a progressive and lethal fibrosing lung disease with a median survival of approximately 3 years after diagnosis. The only medical option to improve survival in IPF is lung transplantation (LTX). The purpose of this study was to evaluate trajectory data of IPF patients listed for LTX and to investigate the survival after LTX. Data were retrospectively collected from September 1989 until July 2011 of all IPF patients registered for LTX in the Netherlands. Patients were included after revision of the diagnosis based on the criteria set by the ATS/ERS/JRS/ALAT. Trajectory data, clinical data at time of screening, and donor data were collected. In total, 98 IPF patients were listed for LTX. During the waiting list period, 30 % of the patients died. Mean pulmonary artery pressure, 6-min walking distance, and the use of supplemental oxygen were significant predictors of mortality on the waiting list. Fifty-two patients received LTX with a median overall survival after transplantation of 10 years. This study demonstrated a 10-year survival time after LTX in IPF. Furthermore, our study demonstrated a significantly better survival after bilateral LTX in IPF compared to single LTX although bilateral LTX patients were significantly younger.

  2. Doença pulmonar intersticial associada a bronquiolite respiratória Respiratory bronchilitis-associated interstitial lung disease

    Directory of Open Access Journals (Sweden)

    Sílvia CS. Rodrigues

    2004-12-01

    Full Text Available A doença pulmonar intersticial associada a bronquiolite respiratória faz parte do espectro anatomopatológico das lesões pulmonares infiltrativas difusas induzidas pela fumaça de cigarro. Raramente tem apresentação clínico-funcional exuberante. Descrevemos dois casos diagnosticados por biópsia pulmonar aberta, caracterizados por dispnéia de evolução insidiosa, baqueteamento digital, lesões císticas à tomografia computadorizada e hipoxemia ao exercício. Enfatizamos considerar, em indivíduos tabagistas, a doença pulmonar intersticial associada a bronquiolite respiratória no contexto das pneumopatias intersticiais císticas, juntamente com a linfangioleiomiomatose, o granuloma eosinofílico e a fibrose pulmonar idiopática.Respiratory bronchiolitis-associated interstitial lung disease is one of many within the spectrum of smoking-related diffuse infiltrative lung diseases. The clinical and functional characteristics are typically subtle. Herein, we describe two cases of diagnosed through open-lung biopsy, and characterized by insidious evolution of dyspnea, digital clubbing, cystic lesions on computed tomography scans, and hipoxemia upon exertion. We emphasize that, when smokers are evaluated, it is imprtant to consider a diagnosis of respiratory bronchiolitis-associated interstitial lung disease in the context of interstitial cystitis, as well as in that of lymphangioleiomyomatosis, eosinophilic granuloma and idiophatic pulmonary fibrosis.

  3. Ossificação pulmonar dendriforme Dendriform pulmonary ossification

    Directory of Open Access Journals (Sweden)

    Andrezza Araújo de Oliveira Duarte

    2006-06-01

    Full Text Available A ossificação pulmonar difusa é uma condição rara, de etiologia desconhecida, na qual osso maduro é encontrado no parênquima pulmonar. É quase sempre descoberta como um achado incidental de autópsias. Freqüentemente afeta homens de meia-idade e é assintomática. Relata-se o caso de um paciente de 75 anos, que apresentou uma radiografia torácica com comprometimento pulmonar difuso e cujo diagnóstico foi baseado no exame histopatológico de fragmento pulmonar obtido através da biópsia a céu aberto, o qual demonstrou fibrose intersticial acentuada com ossificação do parênquima pulmonar.Diffuse pulmonary ossification is a rare condition of unknown pathogenesis in which mature bone is found in the pulmonary parenchyma. It is almost invariably discovered as an incidental finding at autopsy. Most commonly, it affects middle-aged men and is asymptomatic. We present the case of a 75-year-old man in which the chest X-ray showed diffuse interstitial infiltrate. Diagnosis was based on histopathological examination by open-lung biopsy, which revealed interstitial fibrosis with pulmonary ossification.

  4. Idiopathic pulmonary fibrosis.

    Science.gov (United States)

    Xaubet, Antoni; Ancochea, Julio; Molina-Molina, María

    2017-02-23

    Idiopathic pulmonary fibrosis is a fibrosing interstitial pneumonia associated with the radiological and/or histological pattern of usual interstitial pneumonia. Its aetiology is unknown, but probably comprises the action of endogenous and exogenous micro-environmental factors in subjects with genetic predisposition. Its diagnosis is based on the presence of characteristic findings of high-resolution computed tomography scans and pulmonary biopsies in absence of interstitial lung diseases of other aetiologies. Its clinical evolution is variable, although the mean survival rate is 2-5 years as of its clinical presentation. Patients with idiopathic pulmonary fibrosis may present complications and comorbidities which modify the disease's clinical course and prognosis. In the mild-moderate disease, the treatment consists of the administration of anti-fibrotic drugs. In severe disease, the best therapeutic option is pulmonary transplantation. In this paper we review the diagnostic and therapeutic aspects of the disease. Copyright © 2016 Elsevier España, S.L.U. All rights reserved.

  5. Doença pulmonar induzida pelas radiações ou pelos fármacos citostáticos

    Directory of Open Access Journals (Sweden)

    Ulrich Costabel

    2000-03-01

    Full Text Available RESUMO: O artigo discute dois temas afins: 1 doença pulmonar induzida pela radioterapia nas suas diferentes apresentações: pneumonite de radiação clássica e fibrose pulmonar e outras duas mais recentemente descritas, BOOP e alveolite linfocitária bilateral São discutidos os mecanismos patoeenicos, clinic:a e tratamento; 2 a patologia intersticial induzida por fármacos citostáticos nas suas várias formas de apresentação.REV PORT PNEUMOL 2000; VI (2: 141-144 ABSTRACT: In this paper two topics art adressed: 1 the radiation induced lung injury in its possible forms: classical radiation pneumonitis and radiation fibrosis or two other newly recoenised sindromes BOOP and bilateral lymphodtic alveolitis. The mecanism and treatment are discussed; 2 the cytotocic drug-induced lung diseases and their pulmonary reaction patterns.REV PORT PNEUMOL 2000; VI (2: 141-144 Palavras-chave: Radioterapia, Citostaticos, BOOP, Alveolite Linfocitica, Key-words: Radiation, Cytotocic Drugs, Lymphocitic Alveolitis

  6. Nursing interventions in monitoring the adolescent with Cystic Fibrosis: a literature review.

    Science.gov (United States)

    Reisinho, Maria da Conceição Marinho Sousa Ribeiro Oliveira; Gomes, Bárbara Pereira

    2016-12-08

    to search for nursing interventions focused on the improvement of quality of life and promotion of self-care of adolescents suffering from the Cystic Fibrosis. literature review. The inclusion criteria were: primary studies and studies with interventions developed by nurses in the adolescent population with Cystic Fibrosis, using Portuguese, Spanish, French and English with no time limit, and supported by the databases Scopus, Web of Science and CINAHL. The search expressions were: nursing AND care AND adolescent AND "Cystic Fibrosis" AND ("quality of life" OR "self-care"). a total of 59 articles was retrieved; 8 matched the criteria chosen. Nursing interventions targeted at adolescents with Cystic Fibrosis and their family members were identified. These interventions were organized according to the nurses' role, namely caregiver, coordinator, counsellor, researcher, trainer and care partner. nursing interventions targeted at following up the adolescent during the entire therapeutic process, involving the presence of parents/significant others, since both the adolescent and family have to be responsible for self-care. Healthcare professionals should be capable of identifying the specific needs of patients with chronic disease and their family, permitting a better understanding and adaptation to the health-disease transition process. buscar intervenções de enfermagem que enfoquem a melhoria da qualidade de vida e a promoção do autocuidado em adolescentes que sofrem de fibrose cística. revisão de literatura. Os critérios de inclusão foram: estudos primários e estudos com intervenções desenvolvidas por enfermeiros na população adolescente com fibrose cística, em português, espanhol, francês e inglês, sem delimitação temporal, nas bases de dados Scopus, Web of Science e CINAHL. Os termos utilizados na busca foram: enfermagem AND cuidado AND adolescente AND "Fibrose Cística" AND ("qualidade de vida" OR "autocuidado"). ao total, 59 artigos foram

  7. Automated scanning electron microscopy and x-ray microanalysis for in situ quantification of gadolinium deposits in skin

    International Nuclear Information System (INIS)

    Thakral, Charu; Abraham, Jerrold L.

    2007-01-01

    Gadolinium (Gd) has been identified as a possible causative agent of an emerging cutaneous and systemic fibrosing disorder, nephrogenic systemic fibrosis (NSF), which can cause serious disability and even death. To date, there are only two known associations with this disorder - renal insufficiency and Gd enhanced magnetic resonance imaging (MRI). We developed an automated quantitative scanning electron microscopy (SEM) and Energy dispersive x-ray spectroscopy (EDS) method for Gd in tissue of NSF patients. Freshly cut paraffin block surfaces examined using the variable pressure mode under standardized conditions and random search of the tissue area allow in situ detection and semiquantitative morphometric (volumetric) analysis of insoluble higher atomic number features using backscattered electron imaging. We detected Gd ranging from 1 to 2270 cps/mm 2 in 57 cutaneous biopsies of NSF. Gd was associated with P, Ca, and usually Na in tissue deposits. Our method reproducibly determines the elemental composition, relative concentration, and spatial distribution of detected features within the tissue. However, we cannot detect features below our spatial resolution, nor concentrations below the detection limit of our SEM/EDS system. The findings confirm transmetallation and release of toxic Gd ions in NSF and allow dose-response analysis at the histologic level. (author)

  8. Osteoporosis associated with pulmonary silicosis in an equine bone fragility syndrome.

    Science.gov (United States)

    Arens, A M; Barr, B; Puchalski, S M; Poppenga, R; Kulin, R M; Anderson, J; Stover, S M

    2011-05-01

    California horses incur a bone fragility syndrome manifested by pathologic fractures. This study investigated gross, radiographic, and histologic features of the disorder as well as relationships with silicosis and levels of heavy metals and trace minerals through a postmortem study of 9 affected and 3 unaffected horses. Bones and soft tissues were evaluated grossly and histologically. Bones, lymph nodes, and lung tissue were evaluated radiographically. Tissues were evaluated for silicon levels, intracytoplasmic crystals, heavy metals, and trace minerals. All 9 affected horses had osteoporosis and clinical or subclinical pulmonary disease due to silicosis (8/9) or pneumoconiosis (1/9). All affected horses had radiographic findings consistent with osteopenia and histologic evidence of osteoporosis characterized by osteopenia, numerous resorption cavities, cement lines, and a mosaic lamellar pattern indicative of multiple remodeling events. Silicosis was characterized by widespread pulmonary granuloma formation with fibrosis; variable tracheobronchiolar and mediastinal granulomatous lymphadenitis; intracellular crystals within lung and lymph node macrophages; and pronounced lymph node fibrosis, focal necrosis, and dystrophic calcification. Crystals in lung (6/9) and lymph node (8/9) tissues were identified as cytotoxic silica dioxide polymorphs. Lung and liver tissue from affected horses had elevated levels of elemental silicon. Osteoporosis was highly correlated (r = 0.8, P horses with bone fragility disorder have systemic osteoporosis associated with fibrosing pulmonary silicosis. The etiopathogenesis of the bone fragility syndrome is unknown; however, this study provides circumstantial evidence for a silicate associated osteoporosis.

  9. Characterizing nanoscale topography of the aortic heart valve basement membrane for tissue engineering heart valve scaffold design.

    Science.gov (United States)

    Brody, Sarah; Anilkumar, Thapasimuthu; Liliensiek, Sara; Last, Julie A; Murphy, Christopher J; Pandit, Abhay

    2006-02-01

    A fully effective prosthetic heart valve has not yet been developed. A successful tissue-engineered valve prosthetic must contain a scaffold that fully supports valve endothelial cell function. Recently, topographic features of scaffolds have been shown to influence the behavior of a variety of cell types and should be considered in rational scaffold design and fabrication. The basement membrane of the aortic valve endothelium provides important parameters for tissue engineering scaffold design. This study presents a quantitative characterization of the topographic features of the native aortic valve endothelial basement membrane; topographical features were measured, and quantitative data were generated using scanning electron microscopy (SEM), atomic force microscopy (AFM), transmission electron microscopy (TEM), and light microscopy. Optimal conditions for basement membrane isolation were established. Histological, immunohistochemical, and TEM analyses following decellularization confirmed basement membrane integrity. SEM and AFM photomicrographs of isolated basement membrane were captured and quantitatively analyzed. The basement membrane of the aortic valve has a rich, felt-like, 3-D nanoscale topography, consisting of pores, fibers, and elevations. All features measured were in the sub-100 nm range. No statistical difference was found between the fibrosal and ventricular surfaces of the cusp. These data provide a rational starting point for the design of extracellular scaffolds with nanoscale topographic features that mimic those found in the native aortic heart valve basement membrane.

  10. Challenging hepatitis C-infected liver transplant patients

    Directory of Open Access Journals (Sweden)

    Oliver M

    2016-01-01

    Full Text Available Madeleine Oliver,1 Christopher Chiodo Ortiz,2 Jorge Ortiz31University of Toledo College of Medicine, Toledo, OH, 2Bucknell University, Lewisburg, PA, 3Department of Transplant Surgery, University of Toledo Medical Center, Toledo, OH, USA Abstract: Caring for liver transplant patients suffering from chronic hepatitis C virus (HCV infection is a challenging task for transplant surgeons and primary physicians alike. HCV is the leading cause of liver transplantation in the USA and comes with a myriad of complications that increase morbidity and mortality. This review focuses on patient follow-up, spanning from before the liver transplant occurs to the patient's long-term health. Pretransplant, both donor and recipient variables, must be carefully chosen to ensure optimal surgical success. Risk factors must be identified and HCV viral load must be reduced to a minimum. In addition to standard transplant complications, HCV patients suffer from additional problems, such as fibrosing cholestatic hepatitis and widespread viremia. Physicians must focus on the balance of immunosuppressive and antiviral medications, while considering possible side effects from these potent drugs. Over the years following surgery, physicians must identify any signs of failing liver health, as HCV-positive patients have an increased risk for cirrhosis and certain life-threatening malignancies. Keywords: liver transplant, hepatitis C virus, postoperative, cirrhosis, donor and recipient variables, viremia

  11. Modifications of the thorax radiography after postoperative irradiation in mammary carcinoma

    Energy Technology Data Exchange (ETDEWEB)

    Scheier, M; Rhomberg, W; Schelling, F

    1987-02-01

    The radiogenic modifications of the thorax radiography were evaluated in 100 patients irradiated postoperatively for mammary carcinoma between January, 1980 and March, 1983. No adjuvant chemotherapy was given. A telecobalt unit was used for the irradiations. A computer-assisted planning (Evados) was applied in order to obtain an individual optimization especially for the fields situated at the thoracic walls. The average focal doses to the thoracic wall and the regional lymph nodes were between 50 and 52 Gy. Taking into consideration all modifications visualized by radiography, 65% of patients had subacute radiogenic reactions in the lungs, especially in the apex fields. In general the modifications had no clinical importance. Only seven patients presented more severe forms of pneumonitis with clinical symptoms. Fibroses were developed only in the apex fields; infraclavicular infiltrations were seen only in exceptional cases. There were no costal necroses, essential pleural reactions, or radiogenic enlargements of the heart shadow. The rate of pulmonary reactions with clinical symptoms is reduced as compared with former techniques, e.g. opposing tangential fields. The knowledge of anamnesis and typical radiographic modifications should help to avoid problems of differentiation between apex reactions and tuberculosis.

  12. A experiência da família ao conviver com a família doença crônica da criança The family experience of living with the child's chronic illness

    Directory of Open Access Journals (Sweden)

    Elaine Buchhorn Cintra Damião

    2001-03-01

    Full Text Available Esta pesquisa objetivou compreender como a família vivencia os períodos de dificuldades impostos pela doença crônica da criança. Utilizou-se como método de pesquisa a Teoria Fundamentada nos Dados e como referencial teórico o Interacionismo Simbólico. Foram entrevistadas famílias de crianças com doença crônica, fibrose cística do pâncreas e diabetes, possibilitando desvelar o processo vivenciado e o significado atribuído pela família a sua experiência. Os dados foram analisados até a fase de categorização dos significados, tendo emergido o fenômeno: "Sendo Difícil Não Ter Controle".This research has focused on the family meaning of having a child with chronic illness. The Grounded Theory and the Symbolic Interactionism were employed as research method and theoretical approach respectively. The interviews involved families with children diagnosed as having a chronic disease, cystic fibrosis and diabetes, aiming to study the way the family copes with this situation, and the meaning giving by the family to this experience. Data were analyzed until the phase of categorizing and the phenomenon disclosed was: "Being hard not to have control".

  13. [Subluxation of hydrophilic acrylate intraocular lenses due to massive capsular fibrosis].

    Science.gov (United States)

    Kramer, S; Schröder, A C; Brückner, K; Jonescu-Cuypers, C; Seitz, B

    2010-05-01

    Compared with other biomaterials, hydrophilic acrylate provides better uveal biocompatibility, lower adhesion rates of bacteria and silicone oil, and less glare. Because of reduced capsular biocompatibility, increased fibrosis may initiate dislocation of the intraocular lens (IOL). In six eyes of four patients, enhanced fibroses led to IOL dislocation, leading to an IOL exchange an average of 40 weeks after implantation of the same hydrophilic acrylate lens type. Predisposing factors were found in 90% of all reported cases of IOL dislocation in the literature, but not in the cases described here. The lens type that was implanted was unable to adapt to the massive fibrosis induced by its hydrophilic biomaterial. The pattern of lens opacification should receive attention when one is choosing an IOL type. Eyes showing pseudoexfoliation syndrome as well as post-uveitis eyes might require a hydrophilic IOL for less cellular reaction, whereas a posterior subcapsular cataract might need a hydrophobic IOL to prevent a massive capsular fibrosis. In the case of increased capsular contraction, unreflected YAG laser capsulotomy may result in IOL subluxation when the lens design cannot handle capsule shrinkage, as demonstrated here.

  14. Circumscribed Morphea and Breast Asymmetry in an Adolescent

    Directory of Open Access Journals (Sweden)

    António Augusto Fernandes Massa

    2014-01-01

    Full Text Available Morphea is a rare fibrosing disorder of the skin and underlying tissues. Circumscribed morphea presents with less than three discrete indurated plaques and breasts are commonly affected in women. We report the case of a 12-year-old female with a right infra-areolar, nontender, brownish patch and asymmetry of the right breast with 2 years of evolution. Skin biopsy showing thickening of the dermal collagen bundles confirmed the clinical diagnosis of morphea. After a 3-year follow-up period without progression of disease, reconstructive surgery is scheduled. Plaque morphea can involve all layers of the skin but associated breast deformity is rare. It can mimic benign and malignant breast disorders justifying the benefit for early tissue biopsy. Breast morphea generally has a good prognosis but hyperpigmentation and breast deformity in young girls have been rarely described. An early diagnosis can possibly lead to a therapeutic intervention with a different outcome, as it can be the source of severe psychological and social issues in a delicate period of development such as adolescence.

  15. Bronchoalveolar lavage in patients with interstitial lung diseases: side effects and factors affecting fluid recovery.

    Science.gov (United States)

    Dhillon, D P; Haslam, P L; Townsend, P J; Primett, Z; Collins, J V; Turner-Warwick, M

    1986-05-01

    One hundred and seventy patients with interstitial lung diseases undergoing bronchoalveolar lavage (BAL), were contrasted with 51 patients undergoing fibreoptic bronchoscopy alone to define the factors which predispose to post-lavage side-effects. Transient post-bronchoscopy fall in the peak expired flow (PEF) greater than or equal to 20% occurred in both groups (24% and 23% respectively), and thus was probably related to the bronchoscopy procedure. Post-lavage pyrexia (greater than or equal to 1 degree C) occurred only in the patients undergoing BAL (26%), p less than 0.001. Only 4% with pyrexia required antibiotics, and only 2% with falls in PEF needed bronchodilator therapy. The only significant clinical association was more frequent pyrexia in patients on treatment with prednisolone, particularly in women (p less than 0.01). Pyrexia was also associated with higher lavage fluid introduction volumes (greater than 240 ml). Side effects did not relate to the percentages of lavage fluid recovered, although smokers had lower recoveries and, recoveries tended to be higher in sarcoidosis than cryptogenic fibrosing alveolitis. Serial lavages in 25 patients caused no significant increase in side effects.

  16. Mechanisms and Implications of Age-Related Changes in the Liver: Nonalcoholic Fatty Liver Disease in the Elderly

    Directory of Open Access Journals (Sweden)

    Lay Gan

    2011-01-01

    Full Text Available Nonalcoholic fatty liver disease (NAFLD is hepatic steatosis associated with metabolic abnormalities such as overweight/central obesity, insulin resistance, type 2 diabetes (T2D, and dyslipidemia. NAFLD is becoming the most common liver disease in contemporary society, with the highest prevalence in those over 60 years. NAFLD pathology ranges from simple steatosis to a necroinflammatory fibrosing disorder called steatohepatitis (SH, the latter associated with high risk of developing cirrhosis, often occuring in the seventh to ninth decades of life. While the main health implications of NAFLD are increased risk of developing T2D, cardiovascular diseases, and common cancers, there is substantantially increased standardized mortality, and deaths from decompensated cirrhosis and hepatocellular carcinoma (HCC. Little is known about the interactive effects of ageing and NAFLD, with most studies focusing on the younger population. This paper summarises the epidemiology, pathogenesis, and clinical course of NAFLD, with particular attention to persons over age 60 years. An approach to the management of NASH and its complications in the elderly, will also be presented here.

  17. Female Pattern Hair Loss

    Science.gov (United States)

    Herskovitz, Ingrid; Tosti, Antonella

    2013-01-01

    Context: Female pattern hair loss (FPHL) also known as female androgenetic alopecia is a common condition afflicting millions of women that can be cosmetically disrupting. Prompt diagnosis and treatment are essential for obtaining optimal outcome. This review addresses the clinical presentation of female pattern hair loss, its differential diagnosis and treatment modalities. Evidence Acquisition: A) Diffuse thinning of the crown region with preservation of the frontal hairline (Ludwig’s type) B) The “Christmas tree pattern” where the thinning is wider in the frontal scalp giving the alopecic area a triangular shaped figure resembling a christmas tree. C) Thinning associated with bitemporal recession (Hamilton type). Generally, FPHL is not associated with elevated androgens. Less commonly females with FPHL may have other skin or general signs of hyperandrogenism such as hirsutism, acne, irregular menses, infertility, galactorrhea and insulin resistance. The most common endocrinological abnormality associated with FPHL is polycystic ovarian syndrome (PCOS). Results: The most important diseases to consider in the differential diagnosis of FPHL include Chronic Telogen Effluvium (CTE), Permanent Alopecia after Chemotherapy (PAC), Alopecia Areata Incognito (AAI) and Frontal Fibrosing Alopecia (FFA). This review describes criteria for distinguishing these conditions from FPHL. Conclusions: The only approved treatment for FPHL, which is 2% topical Minoxidil, should be applied at the dosage of 1ml twice day for a minimum period of 12 months. This review will discuss off-label alternative modalities of treatment including 5-alfa reductase inhibitors, antiandrogens, estrogens, prostaglandin analogs, lasers, light treatments and hair transplantation. PMID:24719635

  18. Pathophysiology of chronic pancreatitis.

    Science.gov (United States)

    Brock, Christina; Nielsen, Lecia Møller; Lelic, Dina; Drewes, Asbjørn Mohr

    2013-11-14

    Chronic pancreatitis (CP) is an inflammatory disease of the pancreas characterized by progressive fibrotic destruction of the pancreatic secretory parenchyma. Despite the heterogeneity in pathogenesis and involved risk factors, processes such as necrosis/apoptosis, inflammation or duct obstruction are involved. This fibrosing process ultimately leads to progressive loss of the lobular morphology and structure of the pancreas, deformation of the large ducts and severe changes in the arrangement and composition of the islets. These conditions lead to irreversible morphological and structural changes resulting in impairment of both exocrine and endocrine functions. The prevalence of the disease is largely dependent on culture and geography. The etiological risk-factors associated with CP are multiple and involve both genetic and environmental factors. Throughout this review the M-ANNHEIM classification system will be used, comprising a detailed description of risk factors such as: alcohol-consumption, nicotine-consumption, nutritional factors, hereditary factors, efferent duct factors, immunological factors and miscellaneous and rare metabolic factors. Increased knowledge of the different etiological factors may encourage the use of further advanced diagnostic tools, which potentially will help clinicians to diagnose CP at an earlier stage. However, in view of the multi factorial disease and the complex clinical picture, it is not surprising that treatment of patients with CP is challenging and often unsuccessful.

  19. Pathophysiology of chronic pancreatitis

    Science.gov (United States)

    Brock, Christina; Nielsen, Lecia Møller; Lelic, Dina; Drewes, Asbjørn Mohr

    2013-01-01

    Chronic pancreatitis (CP) is an inflammatory disease of the pancreas characterized by progressive fibrotic destruction of the pancreatic secretory parenchyma. Despite the heterogeneity in pathogenesis and involved risk factors, processes such as necrosis/apoptosis, inflammation or duct obstruction are involved. This fibrosing process ultimately leads to progressive loss of the lobular morphology and structure of the pancreas, deformation of the large ducts and severe changes in the arrangement and composition of the islets. These conditions lead to irreversible morphological and structural changes resulting in impairment of both exocrine and endocrine functions. The prevalence of the disease is largely dependent on culture and geography. The etiological risk-factors associated with CP are multiple and involve both genetic and environmental factors. Throughout this review the M-ANNHEIM classification system will be used, comprising a detailed description of risk factors such as: alcohol-consumption, nicotine-consumption, nutritional factors, hereditary factors, efferent duct factors, immunological factors and miscellaneous and rare metabolic factors. Increased knowledge of the different etiological factors may encourage the use of further advanced diagnostic tools, which potentially will help clinicians to diagnose CP at an earlier stage. However, in view of the multi factorial disease and the complex clinical picture, it is not surprising that treatment of patients with CP is challenging and often unsuccessful. PMID:24259953

  20. Idiopathic pulmonary fibrosis misdiagnosed as sputum-negative pulmonary tuberculosis.

    Science.gov (United States)

    Isah, Muhammad Danasabe; Abbas, Aminu; Abba, Abdullahi A; Umar, Mohammed

    2016-01-01

    Idiopathic pulmonary fibrosis (IPF), also known as cryptogenic fibrosing alveolitis, is one of a spectrum of idiopathic interstitial pneumonia. IPF is an increasingly common condition which poses many diagnostic and therapeutic challenges leading to misdiagnosis and mismanagement. We presented a 55-year-old male textile trader who was initially managed as sputum-negative pulmonary tuberculosis before histology report. He presented to our clinic with Breathlessness and cough of 3 years and 2.5 years, respectively. He had commenced anti-tuberculosis two months before presentation without significant relief. General Physical examination and vital signs were essentially normal. SPO2 was 96% on room air. Chest Examination revealed end-inspiratory bi-basal velcro-like crackles. Other systemic examinations were normal. Radiological examination by way of chest X- ray and chest CT showed features suggestive of IPF. The patient also had open Lung biopsy for histology and spirometry which demonstrated restrictive ventilatory function pattern. A diagnosis of Interstitial lung disease probably Idiopathic Pulmonary Fibrosis was entertained. He was commenced on Tab prednisolone, Tab Rabeprazole, with minimal improvement. IPF have often been misdiagnosed and treated as pulmonary tuberculosis with unfavorable outcome.

  1. Sonographic Findings of Hashimoto's Thyroiditis and Associated Nodular Lesions

    Energy Technology Data Exchange (ETDEWEB)

    Kang, Bong Joo; Park, Young Ha; Jung, So Lyung; Chung, Soo Kyo [Catholic University of Korea College of Medicine, Seoul (Korea, Republic of)

    2007-12-15

    To evaluate the sonographic findings of Hashimoto's thyroiditis and associated nodular lesions. We retrospectively reviewed the sonographic findings of twenty patients who had surgically confirmed Hashimoto's thyroiditis between 1 March 2005, and 26 November 2005. In these patients, we reviewed the sonographic findings of the associated focal nodular lesion. Assessed were size, homogeneity, and echogenicity of the diseased thyroid gland and shape, echogenicity, margin, rim, microcal cification of the associated nodules. Without knowledge of the pathological diagnosis of the nodular lesions, based on the sonographic criteria, the nodules were classified as either malignant or benign. Hashimoto's thyroiditis demonstrates a variety of sonographic findings for size, homogeneity, and echogenicity. Among the nineteen nodules that were sonographically diagnosed and pathologically confirmed, nine papillary cancers, seven nodular hyperplasias, two Huthle cell adenomas, and one focal hyalinized fibrosing nodule were included. All of the nine papillary cancers showed more than one malignant finding such as marked hypoechogenicity, an irregular shape, a taller than wide shape, a spiculated margin, or microcalcifications that were classified as malignant nodulea, and all of the ten benign nodules showed no malignant findings. Circumscribed isoechoic, hyperechoic, or hypoechoic nodules without calcification were classified as bending nodules. Hashimoto's thyroiditis demonstrates various findings on a sonographic examination,and associated various benign and malignant lesions. Moreover, a sonographic examination is helpful to differentiate between malignant and benign lesions in Hashimoto's thyroiditis as in the normal thyroid

  2. HURTLE CELLS IMMUNOHISTOCHEMICAL ACTIVITIES IN HASHIMOTO THYROIDITIS PARENCHYMA.

    Science.gov (United States)

    Tsagareli, Z; Kvachadze, T; Melikadze, E; Metreveli, L; Nikobadze, E; Gogiashvili, L

    2016-11-01

    The present study was designed to evaluate the participation and utility of Hǘrtle cells morphological requirment and transformation under Hashimoto autoimmune thyroiditis versus Riedel´s struma. Several markers have been evaluated to detect induced activities of Hǘrtle cells. Study subject - specimens (tissue fragments) collected from TG surgery (thyroidectomy) for mollecular (receptor) diagnosis of Hǘrtle cells activities using routine histological and immunohistochemical samples. 89 cases were selected in Hashimoto thyroiditis diagnosis with Hǘrtle cells history (adenoma and adenomatous grouth of oncocytes). Markers as: TSH receptors, TTF-1, S-100 protein, also anti-TPO and anti-TG levels in blood plasm were detected. It was shown that solid cell claster-nests like agregation of oncocytes and adenomatous growth foci in parafollicular areas with anti-TPO and anti-TG antibodies levels arising while Riedel´s struma shown only large intra- and extra glandular inflammatory proliferative fibrosing process. Large positive expression of TTF-1 and S-100 protein and the negative reaction of TSH receptor factor suggest that Thyroid parenchyma disorganization and mollecular biological atypia with Hǘrtle cells are proceses due to hypothyreoidismus, as well as neuroectodermal cells prominent activities in 70% of Hashimoto cases.

  3. Overlapping Morphologic and Immunohistochemical Features of Hashimoto Thyroiditis and IgG4-Related Thyroid Disease.

    Science.gov (United States)

    Raess, Philipp W; Habashi, Arlette; El Rassi, Edward; Milas, Mira; Sauer, David A; Troxell, Megan L

    2015-05-01

    Immunoglobulin G4-related disease (IgG4-RD) is an emerging clinicopathologic entity characterized by both IgG4+ plasma cell infiltration and fibrosis in one or more organs, prototypically pancreas or salivary/lacrimal glands. IgG4-RD in the thyroid (IgG4-RTD) is an area of active study, and the relationship between IgG4-RTD and Hashimoto thyroiditis is not fully delineated due to their overlapping histologic features. Retrospective review was performed of all thyroidectomy cases demonstrating lymphocytic inflammation at a single institution over a 4-year period. Approximately half (23/38) of patients had a clinical diagnosis of Hashimoto thyroiditis (HT). Nine of the 38 patients had increased absolute and relative numbers of IgG4+ plasma cells. Patients with a clinical diagnosis of HT had increased lymphoplasmacytic inflammation, but the relative proportion of IgG4+ plasma cells was not increased compared to patients without HT. There was no correlation between IgG4 levels and the amount of fibrosis in patients with or without HT. Patients identified as having the fibrosing variant of HT were not more likely to have increased levels of IgG4+ plasma cells than those without. There is significant morphologic and immunohistochemical overlap between HT and IgG4-RTD. Future studies to identify specific characteristics of IgG4-RTD involving the thyroid are necessary to accurately define this entity.

  4. Detection of hepatitis C virus sequences in brain tissue obtained in recurrent hepatitis C after liver transplantation.

    Science.gov (United States)

    Vargas, Hugo E; Laskus, Tomasz; Radkowski, Marek; Wilkinson, Jeff; Balan, Vijay; Douglas, David D; Harrison, M Edwyn; Mulligan, David C; Olden, Kevin; Adair, Debra; Rakela, Jorge

    2002-11-01

    Patients with chronic hepatitis C frequently report tiredness, easy fatigability, and depression. The aim of this study is to determine whether hepatitis C virus (HCV) replication could be found in brain tissue in patients with hepatitis C and depression. We report two patients with recurrent hepatitis C after liver transplantation who also developed severe depression. One patient died of multiorgan failure and the other, septicemia caused by Staphylococcus aureussis. Both patients had evidence of severe hepatitis C recurrence with features of cholestatic fibrosing hepatitis. We were able to study samples of their central nervous system obtained at autopsy for evidence of HCV replication. The presence of HCV RNA-negative strand, which is the viral replicative form, was determined by strand-specific Tth-based reverse-transcriptase polymerase chain reaction. Viral sequences were compared by means of single-strand conformation polymorphism and direct sequencing. HCV RNA-negative strands were found in subcortical white matter from one patient and cerebral cortex from the other patient. HCV RNA-negative strands amplified from brain tissue differed by several nucleotide substitutions from serum consensus sequences in the 5' untranslated region. These findings support the concept of HCV neuroinvasion, and we speculate that it may provide a biological substrate to neuropsychiatric disorders observed in patients with chronic hepatitis C. The exact lineage of cells permissive for HCV replication and the possible interaction between viral replication and cerebral function that may lead to depression remain to be elucidated.

  5. Aspergillus serology: Have we arrived yet?

    Science.gov (United States)

    Richardson, Malcolm D; Page, Iain D

    2017-01-01

    Aspergillosis presents in various clinical forms, among them chronic pulmonary aspergillosis, which is a spectrum of disease entities including aspergilloma, chronic cavitary pulmonary aspergillosis, and chronic fibrosing pulmonary aspergillosis. Aspergillus also contributes to fungal allergy and sensitization. Analysis of the immune response to Aspergillus and its antigens is an integral part of the diagnosis of these diseases. Over the past half century, the techniques used to determine antibody titers have evolved from testing for precipitating and agglutinating antibodies by agar gel double diffusion and immunolectrophoresis to enzyme-linked immunosorbent assays using recombinant proteins as capture antigens. A resurgence of interest in the detection of immunoglobulins, primarily Aspergillus-specific IgG, has hinted at the possibility of distinguishing between colonization and invasion in immunocompromised patients with invasive aspergillosis. Even though there appears to be a greater degree of discrimination between the clinical forms of aspergillosis there is still a long way to travel. This review presents illustrative examples of where new diagnostic platforms and technologies have been applied to this intriguing spectrum of diseases. © The Authors 2016. Published by Oxford University Press on behalf of The International Society for Human and Animal Mycology. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.

  6. 020. Coexistence of lung adenocarcinoma and usual interstitial pneumonia: a case report

    Science.gov (United States)

    Baliaka, Aggeliki; Papaemmanouil, Styliani; Spyratos, Dionysis; Zarogoulidis, Paul; Sakkas, Leonidas

    2015-01-01

    Background Usual interstitial pneumonia (UIP)/idiopathic pulmonary fibrosis (IPF) is a chronic fibrosing interstitial pneumonia of unknown cause. The most common symptoms are progressively increased shortness of breath and dry cough. Some studies suggest an association between usual interstitial pneumonia and lung cancer through different pathogenetic mechanisms. Objective The case presentation of a patient with lung adenocarcinoma and UIP. Methods A 66-year-old male presented with persistent dry cough, hemoptysis and dyspnea. The chest radiographs revealed a mass in the lower lobe of the left lung, measuring 3 cm, as well as diffuse interstitial changes in the same lobe. Two partial lobectomies were performed. Results Histological examination of the mass showed moderately differentiated adenocarcinoma, focally with bronchoalveolar pattern (Immunohistochemical detection of EGFR: positive). The rest lung parenchyma presented histological appearance of UIP. Conclusions According to clinicopathological studies, the prevalence of lung cancer among patients with UIP/IPF varies between 4% and 9%. The overall median survival of IPF-Ca patients is seven months in comparison with IPF only patients (14 months).

  7. Nephrogenic systemic fibrosis

    International Nuclear Information System (INIS)

    Samtleben, W.

    2007-01-01

    A scleromyxedema-like disease was recognized in 1997. In 2000 this disorder was first published and termed nephrogenic fibrosing dermopathy because all patients had advanced renal failure. In 2006 it was discovered that the patients had a history of a preceding contrast-enhanced magnetic resonance imaging (MRI). All patients had acute or chronic severe renal insufficiency with a glomerular filtration rate (GFR) 2 . So far a total of about 215 patients with this new skin disorder have been reported to international registries. The skin thickening has a typical histology and begins in the peripheral extremities and progresses proximally, including also the abdominal wall and the head in some patients. NSF involves not only the skin, but also the muscles and other organs (e.g., lungs, heart, eyes) in some patients. Therefore the term nephrogenic systemic fibrosis (NSF) was introduced. Skin fibrosis and sclerosis are usually progressive with disabling contractures of involved joints (knees, hands, feet). NSF may be lethal in up to 28% of patients. Spontaneous remissions are rare. No generally accepted treatment is available. So far, the pathogenesis is not well understood. One hypothesis supposes a role of gadolinium liberated from the contrast agents. As patients with acute or chronic advanced renal failure (GFR 2 ) including those with hepatorenal dysfunctions are at high risk to develop NSF after exposure to gadolinium-based contrast agents, contrast-enhanced MRI should be avoided in this group and alternative diagnostic procedures should be used whenever possible. (orig.) [de

  8. Segmental stiff skin syndrome (SSS): A distinct clinical entity.

    Science.gov (United States)

    Myers, Kathryn L; Mir, Adnan; Schaffer, Julie V; Meehan, Shane A; Orlow, Seth J; Brinster, Nooshin K

    2016-07-01

    Stiff skin syndrome (SSS) is a noninflammatory, fibrosing condition of the skin, often affecting the limb girdles. We present 4 new patients with SSS with largely unilateral, segmental distribution. To date, reported cases of SSS have been grouped based on generally accepted clinical and histopathologic findings. The purpose of this study was to analyze differences in clinical and histopathologic findings between previously reported SSS cases. This is a retrospective review of 4 new cases and 48 previously published cases of SSS obtained from PubMed search. Of 52 total cases, 18 (35%) were segmentally distributed and 34 (65%) were widespread. The average age of onset was 4.1 years versus 1.6 years for segmental versus widespread SSS, respectively. Limitation in joint mobility affected 44% of patients with segmental SSS and 97% of patients with widespread SSS. Histopathologic findings were common between the 2 groups. This was a retrospective study of previously published cases limited by the completeness and accuracy of the reviewed cases. We propose a distinct clinical entity, segmental SSS, characterized by a segmental distribution, later age of onset, and less severe functional limitation. Both segmental SSS and widespread SSS share common diagnostic histopathologic features. Copyright © 2016 American Academy of Dermatology, Inc. All rights reserved.

  9. Far-Infrared Radiation Thermotherapy Improves Tissue Fibrosis in Chronic Extremity Lymphedema.

    Science.gov (United States)

    Li, Ke; Zhang, Zheng; Liu, Ning Fei; Sadigh, Parviz; Evans, Verity Joyce; Zhou, Huihong; Gao, Weiqing; Zhang, Yi Xin

    2017-09-29

    Fibrosis can enhance the exacerbation of lymphedema, which becomes obvious in late stage II-III lymphedema. However, whether far-infrared radiation thermotherapy (FIRT) can cure lymphedema fibrosis is still lack of research. This research was to investigate the therapeutic effect of FIRT on tissue fibrosis in the treatment of Late stage II-III lymphedema. Patients accepted only FIRT for a total of 20 sessions. The treatment session duration was 2 hours, and a stable machine temperature of 42°C was maintained throughout treatments. Clinical evaluation and laboratory evaluation were conducted before and after FIRT. Clinical outcome measures included circumference of affected extremity, skin elasticity, ultrasound, patients' subjective assessment, and quality of life (QOL). Laboratory outcome measures included serum and local lymphedema tissue fluid concentrations of fibrosis associated cytokines, tissue growth factor beta-1 (TGF-β1), interleukin (IL)-1β, IL-4, IL-18, and caspase-1. Between 2015 and 2016, clinical evaluation of 64 patients with late stage II-III lymphedema was conducted. From this group, 12 cases (18.75%) underwent simultaneous laboratory evaluation. Circumferences of affected extremities improved significantly following treatment (p pain, discomfort, and numbness (p effective treatment for lymphedema tissue fibrosis; it reduces the concentration of fibrosis cytokines in local lymphedema tissues. Consequently, this treatment can reduce the density of fibrosed tissue in the affected extremity, increase skin elasticity, significantly improve clinical symptoms, and improve QOL of patients.

  10. Parvovirus B19 Associated Hepatitis

    Science.gov (United States)

    Bihari, Chhagan; Rastogi, Archana; Saxena, Priyanka; Rangegowda, Devraj; Chowdhury, Ashok; Gupta, Nalini; Sarin, Shiv Kumar

    2013-01-01

    Parvovirus B19 infection can present with myriads of clinical diseases and syndromes; liver manifestations and hepatitis are examples of them. Parvovirus B19 hepatitis associated aplastic anemia and its coinfection with other hepatotropic viruses are relatively underrecognized, and there is sufficient evidence in the literature suggesting that B19 infections can cause a spectrum of liver diseases from elevation of transaminases to acute hepatitis to fulminant liver failure and even chronic hepatitis. It can also cause fatal macrophage activation syndrome and fibrosing cholestatic hepatitis. Parvovirus B19 is an erythrovirus that can only be replicate in pronormoblasts and hepatocytes, and other cells which have globosides and glycosphingolipids in their membrane can also be affected by direct virus injury due to nonstructural protein 1 persistence and indirectly by immune mediated injury. The virus infection is suspected in bone marrow aspiration in cases with sudden drop of hemoglobin and onset of transient aplastic anemia in immunosuppressed or immunocompetent patients and is confirmed either by IgM and IgG positive serology, PCR analysis, and in situ hybridization in biopsy specimens or by application of both. There is no specific treatment for parvovirus B19 related liver diseases, but triple therapy regimen may be effective consisting of immunoglobulin, dehydrohydrocortisone, and cyclosporine. PMID:24232179

  11. Female pattern hair loss.

    Science.gov (United States)

    Herskovitz, Ingrid; Tosti, Antonella

    2013-10-01

    Female pattern hair loss (FPHL) also known as female androgenetic alopecia is a common condition afflicting millions of women that can be cosmetically disrupting. Prompt diagnosis and treatment are essential for obtaining optimal outcome. This review addresses the clinical presentation of female pattern hair loss, its differential diagnosis and treatment modalities. A) Diffuse thinning of the crown region with preservation of the frontal hairline (Ludwig's type) B) The "Christmas tree pattern" where the thinning is wider in the frontal scalp giving the alopecic area a triangular shaped figure resembling a christmas tree. C) Thinning associated with bitemporal recession (Hamilton type). Generally, FPHL is not associated with elevated androgens. Less commonly females with FPHL may have other skin or general signs of hyperandrogenism such as hirsutism, acne, irregular menses, infertility, galactorrhea and insulin resistance. The most common endocrinological abnormality associated with FPHL is polycystic ovarian syndrome (PCOS). The most important diseases to consider in the differential diagnosis of FPHL include Chronic Telogen Effluvium (CTE), Permanent Alopecia after Chemotherapy (PAC), Alopecia Areata Incognito (AAI) and Frontal Fibrosing Alopecia (FFA). This review describes criteria for distinguishing these conditions from FPHL. The only approved treatment for FPHL, which is 2% topical Minoxidil, should be applied at the dosage of 1ml twice day for a minimum period of 12 months. This review will discuss off-label alternative modalities of treatment including 5-alfa reductase inhibitors, antiandrogens, estrogens, prostaglandin analogs, lasers, light treatments and hair transplantation.

  12. Entendendo a classificação, a fisiopatologia e o diagnóstico radiológico das bronquiectasias

    Directory of Open Access Journals (Sweden)

    Bruno Hochhegger

    2010-07-01

    Full Text Available Resumo: O termo bronquiectasia é definido como uma dilatação brônquica anormal persistente geralmente associada a inflamação na via aérea e no parênquima pulmonar. A doença continua a ser uma causa comum de morbidade e mortalidade, especialmente quando associada a doenças hereditárias, como a fibrose cística, a discinesia ciliar e a alguns estados de imunodeficiência. A tomografia computadorizada é, actualmente, a modalidade de escolha para o dianóstico e pode também contribuir para o manejo clínico, sugerindo possíveis diagnósticos. Destacamos nesta revisão a classificação, a fisiopatologia e as manifestações radiológicas desta doença. Abstract: Bronchiectasis is defined as an abnormal persistent bronchial dilatation usualy associated with inflammation in the bronchial tree and lung parenchyma. The disease remains a common cause of significant morbidity and mortality, especially when associated with hereditary disorders such as cystic fibrosis, ciliary dyskinesia, and immunodeficiency states. Computed tomography is now the diagnostic modality of choice and may also contribute to clinical management, suggesting some etiologic causes. We highlight developments in classification, physiopathology and radiology of this debilitating disease. Palavras-chave: Tomografia computadorizada espiral, bronquiectasia, radiologia, fisiologia, patologia, Key-words: Tomography, spiral computed, bronchiectasis, radiology, physiology pathology

  13. Entendendo a classificação, a fisiopatologia e o diagnóstico radiológico das bronquiectasias Understanding the classification, physiopathology and the diagnostic radiology of bronchiectasis

    Directory of Open Access Journals (Sweden)

    Bruno Hochhegger

    2010-08-01

    Full Text Available O termo bronquiectasia é definido como uma dilatação brônquica anormal persistente geralmente associada a inflamação na via aérea e no parênquima pulmonar. A doença continua a ser uma causa comum de morbidade e mortalidade, especialmente quando associada a doenças hereditárias, como a fibrose cística, a discinesia ciliar e a alguns estados de imunodeficiência. A tomografia computadorizada é, actualmente, a modalidade de escolha para o dianóstico e pode também contribuir para o manejo clínico, sugerindo possíveis diagnósticos. Destacamos nesta revisão a classificação, a fisiopatologia e as manifestações radiológicas desta doença.Bronchiectasis is defined as an abnormal persistent bronchial dilatation usualy associated with inflammation in the bronchial tree and lung parenchyma. The disease remains a common cause of significant morbidity and mortality, especially when associated with hereditary disorders such as cystic fibrosis, ciliary dyskinesia, and immunodeficiency states. Computed tomography is now the diagnostic modality of choice and may also contribute to clinical management, suggesting some etiologic causes. We highlight developments in classification, physiopathology and radiology of this debilitating disease.

  14. O pulmão na doença inflamatória do cólon: estudo experimental em ratos

    Directory of Open Access Journals (Sweden)

    Biondo-Simões Maria de Lourdes Pessole

    2000-01-01

    Full Text Available Embora a colite ulcerativa seja conhecida desde 1875 e muitas sejam as manifestações extra-intestinais descritas nesta doença, só recentemente chamou-se a atenção para o envolvimento do aparelho respiratório. Severas complicações têm sido descritas em pacientes como: estenose traqueal inflamatória, bronquiolite com pneumonia, pneumonite intersticial, granulomatose de Wegener, bronquite crônica com bronquiectasia, nódulos necrobióticos, vasculites, fibrose e alveolites. O presente estudo visa reconhecer as alterações pulmonares, na fase aguda da doença inflamatória do cólon, induzida em ratos com ácido acético à 10% e comparar com controles normais. Foi possível constatar que 100% dos animais com colite apresentaram reação inflamatória pulmonar (p=0,0210 de intensidade moderada à severa (p=0,0340. Vasculite foi vista em 58,33% dos pulmões (p=0,0060 e em 3 animais detectou-se hemorragia focal, necrose e abscesso. Estes achados permitem atribuir uma forte associação entre a doença inflamatória do cólon e alterações do aparêlho respiratório, durante a fase aguda da doença, em ratos.

  15. Radiological manifestations of pulmonary tuberculosis

    Energy Technology Data Exchange (ETDEWEB)

    Andreu, J. E-mail: andreuj@hg.vhebron.es; Caceres, J.; Pallisa, E.; Martinez-Rodriguez, M

    2004-08-01

    Pulmonary tuberculosis (TB) is a common worldwide lung infection. The radiological features show considerable variation, but in most cases they are characteristic enough to suggest the diagnosis. Classically, tuberculosis is divided into primary, common in childhood, and postprimary, usually presenting in adults. The most characteristic radiological feature in primary tuberculosis is lymphadenopathy. On enhanced CT, hilar and mediastinal nodes with a central hypodense area suggest the diagnosis. Cavitation is the hallmark of postprimary tuberculosis and appears in around half of patients. Patchy, poorly defined consolidation in the apical and posterior segments of the upper lobes, and in the superior segment of the lower lobe is also commonly observed. Several complications are associated with tuberculous infection, such as hematogenous dissemination (miliary tuberculosis) or extension to the pleura, resulting in pleural effusion. Late complications of tuberculosis comprise a heterogeneous group of processes including tuberculoma, bronchial stenosis bronchiectasis, broncholithiasis, aspergilloma, bronchoesophageal fistula and fibrosing mediastinitis. Radiology provides essential information for the management and follow up of these patients and is extremely valuable for monitoring complications.

  16. Evidence of a generalized defect of acinar cell function in Shwachman-Diamond syndrome.

    Science.gov (United States)

    Stormon, Michael O; Ip, Wan F; Ellis, Lynda; Schibli, Susanne; Rommens, Johanna M; Durie, Peter R

    2010-07-01

    : Because the acinar cells of the exocrine pancreas in patients with Shwachman-Diamond syndrome (SDS) are severely depleted, we hypothesized that a similar deficiency may be present in acinar cells of the parotid gland. : We determined serum pancreatic isoamylase and parotid amylase activities in 16 patients with SDS, 13 healthy controls, and 13 disease controls (cystic fibrosis or fibrosing pancreatitis). Parotid amylase and electrolyte concentrations were measured in stimulated parotid gland secretions. Starch digestion was assessed by breath hydrogen testing in patients with SDS (with and without enzyme supplements) and healthy controls. : Serum pancreatic and parotid isoamylase values were lower in the patients with SDS than in the healthy controls (P gland amylase concentration (units per milligram of protein) in patients with SDS was lower than that in the healthy controls (P = 0.04), whereas the disease controls were comparable to the healthy subjects (P = 0.09). Secreted parotid chloride concentration was inversely correlated with amylase concentration in the patients with SDS (P = 0.01), but no correlation was seen in the healthy controls or disease controls. When patients with SDS ingested starch without enzyme supplementation, their breath hydrogen excretion was significantly higher than that in the healthy controls (P = 0.009). Following starch ingestion with enzymes, breath hydrogen in the patients with SDS was lower (P functional abnormality of exocrine acinar cells.

  17. Congenital anomalies, hereditary diseases of the pancreas, acute and chronic pancreatitis

    International Nuclear Information System (INIS)

    Brambs, Hans-Juergen; Juchems, Markus

    2011-01-01

    The most important congenital anomalies include pancreas divisum, annular pancreas and ectopic pancreas. Patients with pancreas divisum may be more susceptible to acute or chronic pancreatitis and patients with an annular pancreas may develop duodenal stenosis. In pancreas divisum the key finding is the visualization of the main duct draining into the duodenum via the small papilla, separated from the common bile duct. Annular pancreas may show as a well defined ring of pancreatic tissue that encircles the duodenum. Ectopic pancreas is usually asymptomatic but may give rise to abdominal complaints and may be confused with submucosal tumors. Acute pancreatitis is classified as mild or severe. In mild forms ultrasound is the imaging modality of choice whereas in severe forms with extensive pancreatic and peripancreatic necroses computed tomography is the favored method. It is crucial to identify signs and criteria that come along with an increased risk of infection of the necroses. MRI plays an inferior role in the assessment of acute pancreatitis. Chronic pancreatitis is a longstanding inflammatory and fibrosing process causing pain and loss of function. Cross-section imaging is particularly in demand for the detection of complications and the differentiation from pancreatic cancer. Autoimmune pancreatitis is a unique form of chronic pancreatitis characterized by lymphoplasmacytic infiltration and fibrosis, and favourable response to corticosteroid treatment. (orig.)

  18. Traction alopecia: the root of the problem

    Directory of Open Access Journals (Sweden)

    Billero V

    2018-04-01

    Full Text Available Victoria Billero, Mariya MitevaDepartment of Dermatology and Cutaneous Surgery, University of Miami School of Medicine, Miami, FL, USAAbstract: Traction alopecia (TA affects one-third of women of African descent who wear various forms of traumatic hairstyling for a prolonged period of time. The risk of TA is increased by the extent of pulling and duration of traction, as well as the use of chemical relaxation. The frequent use of tight buns or ponytails, the attachment of weaves or hair extensions, and tight braids (such as cornrows and dreadlocks are believed to be the highest risk hairstyles. TA can also occur in the setting of religious and occupational traumatic hairstyling. In its later stages, the disease may progress into an irreversible scarring alopecia if traumatic hairstyling continues without appropriate intervention. The most common clinical presentation includes marginal alopecia and non-marginal patchy alopecia. A clue to the clinical diagnosis is the preservation of the fringe sign as opposed to its loss in frontal fibrosing alopecia (FFA. Dermoscopy can be helpful in the diagnosis and can detect the ongoing traction by the presence of hair casts. Histopathology can distinguish TA from alopecia areata, FFA, and patchy central centrifugal cicatricial alopecia. Currently, there is no cure. Therefore, it is imperative that clinicians educate high-risk populations about TA and those practices that may convey the risk of hair loss. Keywords: hair loss, alopecia, dermoscopy, trichoscopy, traction alopecia, African-American 

  19. Traction alopecia: the root of the problem.

    Science.gov (United States)

    Billero, Victoria; Miteva, Mariya

    2018-01-01

    Traction alopecia (TA) affects one-third of women of African descent who wear various forms of traumatic hairstyling for a prolonged period of time. The risk of TA is increased by the extent of pulling and duration of traction, as well as the use of chemical relaxation. The frequent use of tight buns or ponytails, the attachment of weaves or hair extensions, and tight braids (such as cornrows and dreadlocks) are believed to be the highest risk hairstyles. TA can also occur in the setting of religious and occupational traumatic hairstyling. In its later stages, the disease may progress into an irreversible scarring alopecia if traumatic hairstyling continues without appropriate intervention. The most common clinical presentation includes marginal alopecia and non-marginal patchy alopecia. A clue to the clinical diagnosis is the preservation of the fringe sign as opposed to its loss in frontal fibrosing alopecia (FFA). Dermoscopy can be helpful in the diagnosis and can detect the ongoing traction by the presence of hair casts. Histopathology can distinguish TA from alopecia areata, FFA, and patchy central centrifugal cicatricial alopecia. Currently, there is no cure. Therefore, it is imperative that clinicians educate high-risk populations about TA and those practices that may convey the risk of hair loss.

  20. Role of serum angiotensin converting enzyme in sarcoidosis.

    Science.gov (United States)

    Khan, A H; Ghani, F; Khan, A; Khan, M A; Khurshid, M

    1998-05-01

    This study was conducted to determine the role of Serum Angiotensin Converting Enzyme (SACE) as a marker in the differential diagnosis of pulmonary diseases and prognosis of sarcoidosis. A retrospective analysis of 113 medical records of patients at The Aga Khan University Hospital, with laboratory investigation for SACE was performed. Among 113 patients, 51 cases were found to have sarcoidosis, 44 of them had SACE levels greater than 52 IU/L (mean ACE 104.44). SACE levels were also found elevated in other clinical conditions like tuberculosis (mean 58.64 IU/L), but the enzyme level were less (p 0.04) than those found in sarcoidosis (mean (92.97 IU/L). SACE activity was found to be considerably lower in other chronic lung diseases such as, fibrosing alveolitis (mean 43.98 IU/L), interstitial lung disease (mean 42.11 IU/L) and chronic obstructive lung disease (mean 40.85 IU/L). Twenty patients of sarcoidosis, who received steroid treatment subsequently showed a decline in the SACE levels. SACE is a useful marker in differential diagnosis as 37.2% cases of sarcoidosis compared to only 9.09% of tuberculosis had SACE levels greater than 100 IU/L. In addition, our data also suggest that serum ACE is useful for the diagnosis as well as monitoring prognosis in sarcoidosis.

  1. The Technology and Applications of Large Fission Product Beta Sources; Technologie et applications des grandes sources beta de fission; Tekhnologiya i primenenie krupnykh istochnikov beta-izluchenij, ispuskaemykh produktami deleniya; Tecnologia y utilizacion de los productos de fision como fuentes de irradiacion beta de elevada intensidad

    Energy Technology Data Exchange (ETDEWEB)

    Silverman, Joseph [Radiation Applications Incorporated (United States)

    1960-07-15

    test the validity of the calculations. 4. An engineering cost analysis indicates that fission product beta power can be competitive with alternative sources of radiation. (author) [French] Si les emetteurs beta ne sont pas entres en ligne de compte en tant que sources de rayonnement de grandes dimensions, c'est que dans le passe les procedes sous irradiation presentant un interet pour l'industrie etaient bases sur l'utilisation de particules a grand pouvoir de penetration; de la l'importance considerable que l'on accordait aux rayons gamma et aux electrons artificiellement acceleres. Il y a environ quatre ans, il devint evident que de vastes perspectives s'ouvraient aux applications pratiques des rayonnements beta dans le domaine du traitement par irradiation superficielle, par exemple, la modification par copolymerisation greffee de la surface des moulages en matiere plastique et la pasteurisation superficielle des aliments. En effet, l'irradiation en profondeur occasionne dans ces cas des pertes et peut meme presenter des dangers. D'autre part, il y a deux autres domaines dans lesquels l'utilisation des electrons artificiellement acceleres ne semble pas indiquee; ce sont les syntheses chimiques amorcees par irradiation dans des cuves sous pression et certaines categories de reactions en chaine provoquees par des radicaux libres pour lesquels le taux de production par kilowatt decroit en fonction de la racine carree de l'intensite d'irradiation. Dans tous ces domaines, les sources beta de grandes dimensions presentent des avantages potentiels evidents; puisque la fission nucleaire permet d'en obtenir en quantites importantes, elles meritent que leurs- possibilites d'utilisation fassent l'objet d'un examen approfondi. Compte tenu de ces considerations, on a procede - sous l'egide de la Commission de l'energie atomique - a une etude des applications'et de la technologie des sources beta de fission. Cette etude a permis d'aboutir aux conclusions suivantes: 1. Des

  2. Improvements to the properties of uranium by addition of small quantities of other metals; Ameliorations apportees a l'uranium par de faibles additions metalliques

    Energy Technology Data Exchange (ETDEWEB)

    Englander, M [Commissariat a l' Energie Atomique, Saclay (France).Centre d' Etudes Nucleaires

    1960-07-01

    {sup 235}. Dans les conditions optimales de fonctionnement, un meme materiau combustible doit alors fournir un minimum de 3000 MWj/t, soit 72.10{sup 6} kWh par tonne d'uranium naturel, tout en etant porte a une temperature suffisante pour jouer efficacement son role de source de chaleur (entre 350 et 550 deg. C minimum). Or d'une facon assez surprenante, les agregats polycristallins des billettes d'uranium obtenues par coulee sous vide, ou apres filage a haute temperature, se presentent comme un ensemble de grains tres grossiers, a contours dechiquetes et irreguliers, et comportent de nombreuses marques de deformation intragranulaire (macles, lignes de glissement), accompagnees de fortes sous-structures. En plus, le spectre de la dimension des grains s'etend de quelques microns a quelques millimetres, selon les plages micrographiques examinees. Sous irradiation a ces temperatures, l'uranium metallique pur en barreaux cylindriques de diametre de l'ordre du pouce, se deforme: il se produit des fissures dans la masse, des variations de dimensions longitudinales et transversales (qui se traduisent par des peaux d'orange), soit par croissances plus ou moins directionnelles, soit par deformations superficielles qui peuvent provoquer des ruptures du materiau, de sa gaine ou des fleches suffisamment importantes pour obstruer les canaux de refroidissement. Il a ete reconnu par la suite que cette instabilite, sous l'effet des contraintes thermiques d'origine nucleaire, est due a la morphologie heterogene de l'uranium et a sa structure cristalline anisotrope (U{sub {alpha}} ou U{sub {beta}}). (auteur)

  3. Study of the artificial radioactivity of the marine medium using gamma spectrometry (1962-1966); Etude de la radioactivite artificielle du milieu marin par spectrometrie gamma (1962-1966)

    Energy Technology Data Exchange (ETDEWEB)

    Chesselet, R [Commissariat a l' Energie Atomique, Saclay (France). Centre d' Etudes Nucleaires

    1969-07-01

    incidences de cette retombee dans les eaux marines superficielles. Considerant les radioelements etudies comme des 'traceurs' dans le milieu marin, divers processus physiques, chimiques ou biologiques ont ete etudies par spectrometrie gamma a haute sensibilite. Le passage en phase soluble dans l'eau de mer d'une fraction importante (environ 50 pour cent) de l'activite liee aux poussieres radioactives, phenomene qui n'etait pas previsible pour les radioelements etudies, gouverne les processus d'accumulation dans la biomasse planctonique et la diffusion vers les eaux plus profondes. Par ailleurs, une methode de spectrometrie 'in situ' permettant de mesurer directement dans la mer de tres faibles concentrations en certains radionuclides emetteurs gamma est decrite. L'application de cette methode a permis de nombreuses observations dans les eaux de surface de la Mediterranee occidentale et du Golfe de Gascogne. On montre quo la profondeur de melange est etroitement liee a celle de la thermocline. Un processus d'accumulation au niveau de celle-ci est constate. Les coefficients de diffusion sont voisins du coefficient de diffusion thermique turbulente. L'existence de compartiments qui subsistent pendant plusieurs mois dans la masse des eaux de surface du Golfe de Gascogne est mise en evidence. Grace aux bilans effectues, une etude comparative montre que le taux de retombees radioactives sur les surfaces maritimes est toujours deux a trois fois superieur a celui sur les regions continentales voisines. Diverses explications de ce phenomene sont discutees. (auteur)

  4. Anionic Polyelectrolyte-Cationic Surfactant Interactions in Aqueous Solutions and Foam Films Stability Interactions entre polyélectrolytes anioniques et tensioactifs cationiques en solutions aqueuses et stabilité des films de mousses

    Directory of Open Access Journals (Sweden)

    Langevin D.

    2006-12-01

    superficielle (coadsorption à des concentrations en tensioactifs très faibles (10 puissance (-3 à 10 puissance (-1 CMC. À plus fortes concentrations, en particulier supérieures à la concentration critique d'agrégation (CAC, des complexes polymères/tensioactifs se forment en solution et les macromolécules précipitent. Les films minces réalisés à partir de ces solutions sont stables, alors que les films C12TAB sont instables. Les mesures de pression de disjonction sur des films mixtes avec des concentrations en tensioactifs de deux ordres de grandeur en dessous de la CAC montrent l'existence de forces de répulsion à longue portée et une transition discrète d'épaisseur du film. À la valeur de la CAC, nous obtenons des films mixtes avec des réseaux similaires à un gel qui sont fortement affectés par la vitesse d'amincissement du film.

  5. Contribution to the study of the flow of adsorbed phases. Enrichment of binary Gaseous mixtures; Contribution a l'etude de l'ecoulement des phases absorbees. Enrichissement des melanges gazeux binaires

    Energy Technology Data Exchange (ETDEWEB)

    Sapet, J C [Commissariat a l' Energie Atomique, Saclay (France). Centre d' Etudes Nucleaires

    1962-12-15

    The research which makes up this thesis is concerned both with the problems of adsorption an chromatographic columns and of catalysis, which involve the surface properties of these solids, and with problems of isotope separation by gaseous diffusion. In this latter case, the flow in the two-dimensional adsorbed phase is a secondary phenomenon but it is nevertheless necessary to know its influence on the separation factor. Up to the present time very little work has been published on this subject, the importance of which needs no stressing. The author has devised very sensitive techniques making it possible to study the flow of two-dimensional phases in micro-porous bodies. He has demonstrated not only the role of the relative pressure and of the nature of the components (gas and solid) but also the relationship between the mobility of the adsorbate and the thermodynamic characteristics of the adsorption. The author has treated numerous examples involving the diffusion either of pure gases (n-butane, n-pentane, isopentane), or of binary mixtures, and has shown on the one hand that the separative effect of the two-dimensional flow at the interface is a maximum in the neighbourhood of the monolayer, and on the other hand that the mass effect is of the second order with respect to the structural factor of the diffusing molecule. The problem considered is of importance both from a fundamental point of view and from that of its industrial applications. (author) [French] Le sujet de travail de cette these interesse aussi bien les problemes d'adsorption sur colonne de chromatographie et de catalyse, qui font intervenir directement les proprietes superficielles des solides, que ceux concernant la separation isotopique par diffusion gazeuse. Dans ce dernier cas l'ecoulement en phase adsorbee bidimensiormelle est un phenomene secondaire dont i1 faut cependant connaitre l'effet sur le facteur de separation. Pratiquement aucun travail fondamental n'a encore ete publie sur ce

  6. Frontal and band displacement chromatography of the hydrogen isotopes on palladium; Chromatographies frontale et de deplacement de bande des isotopes de l'hydrogene sur palladium

    Energy Technology Data Exchange (ETDEWEB)

    Botter, F.; Menes, J.; Tistchenko, S.; Dirian, G. [Commissariat a l' Energie Atomique, Saclay (France). Centre d' Etudes Nucleaires

    1964-07-01

    {alpha} tres different de 1. Ce modele, celui d'un echange isotopique a contre courant, neglige les diffusions longitudinales en phase gazeuse et laterale en phase adsorbee et ne tient compte que de la resistance superficielle a l'echange entre phases. Il permet de chiffrer l'efficacite d'une colonne de chromatographie en hauteur equivalente a un plateau theorique (HEPT). Les ecarts legers observes entre les chromatogrammes reels et le modele simple justifient d'une part l'etude entreprise d'un modele plus elabore tenant compte des diffusions, d'autre part l'adoption du modele simple pour la comparaison de l'efficacite de plusieurs colonnes. Nous donnons egalement une methode graphique simple et nouvelle de deduire le nombre de plateaux theoriques d'une colonne des chromatogrammes de deplacement de bande et de frontales. Experimentalement, nous donnons essentiellement les criteres de validite du modele utilise, la loi en fonction de la temperature de {sup {alpha}}H{sub 2}-HD, l'etude de la HEPT en fonction de divers parametres sur plusieurs masses palladiees, et les possibilites de l'application a la chromatographie preparative. (auteurs)

  7. The reactions of magnesium and its alloys with moist gases at high temperatures; Les reactions du magnesium et de ses alliages avec les gaz humides aux temperatures elevees

    Energy Technology Data Exchange (ETDEWEB)

    Darras, R [Commissariat a l' Energie Atomique, Saclay (France). Centre d' Etudes Nucleaires

    1963-02-15

    , un changement important d'energie d'activation du processus d'oxydation se produit systematiquement, mais aussi a une temperature variable suivant l'atmosphere consideree. Cependant, dans le cas des alliages, la cinetique d'oxydation lineaire classique est generalement precedee de courtes periodes d'induction, a caractere parabolique. On a ensuite mis en evidence et interprete deux possibilites d'inhibition de ia corrosion du magnesium par la vapeur d'eau: soit a la suite d'une fluoration superficielle partielle, soit lorsque le gaz porteur est l'anhydride carbonique. D'autre part, les conditions extremes de l'oxydation, c'est-a-dire les phenomenes d'inflammation qui surviennent a une temperature bien determinee dans chaque atmosphere, ont fait l'objet d'une etude particuliere. Enfin, on s'est efforce de degager la portee de l'ensemble de ces resultats aux deux points de vue fondamental et pratique. (auteur)

  8. Investigation of the diffusion {proportional_to} brasses using methods depending on the evaporation or condensation of zinc; Etude de la diffusion dans les laitons {proportional_to} au moyen des methodes d'evaporation ou de condensation du zinc

    Energy Technology Data Exchange (ETDEWEB)

    Accary, A [Commissariat a l' Energie Atomique, Saclay (France).Centre d' Etudes Nucleaires; Centre d' Etudes de Chimie Metallurgique du CNRS (France)

    1959-07-01

    sont precises: les traitements mecaniques subis par l'eprouvette avant diffusion, le sens de la diffusion, la nature des eprouvettes. Le coefficient de diffusion D est alors donne par la relation: D = ({pi}/4t){rho}{sup 2}{sub 0} expression dans laquelle t est la duree de diffusion et {rho}{sub 0} la valeur extrapolee de {rho} = ({delta}m)/({delta}C) a variation nulle de concentration; ({delta}m, variation de poids de l'eprouvette par unite de surface; {delta}C, difference entre la concentration superficielle et la concentration initiale de l'eprouvette). Cette methode a ete utilisee pour etudier l'influence du sens de diffusion sur le coefficient de diffusion le coefficient de diffusion avec appauvrissement en zinc est 5 fois plus grand que le coefficient de diffusion avec enrichissement en zinc;une interpretation de ce phenomene, basee sur le mecanisme lacunaire de diffusion a ete proposee. Par une etude ponderale et une etude micrographique, l'auteur a montre que certaines impuretes, telles que le phosphore, l'arsenic, l'antimoine, le silicium et l'aluminium peuvent augmenter notablement la vitesse de diffusion; il a relie a la valence et a la masse atomique des impuretes, l'influence de celles-ci sur le coefficient de diffusion. (auteur)

  9. Study of the catalytic activity of mixed non-stoichiometric uranium-thorium oxides in carbon monoxide oxidation; Etude de l'activite catalytique des oxydes mixtes d'uranium et de thorium non stoechiometriques dans l'oxydation du monoxyde de carbone

    Energy Technology Data Exchange (ETDEWEB)

    Brau, G [Commissariat a l' Energie Atomique, Fontenay-aux-Roses (France). Centre d' Etudes Nucleaires

    1969-06-01

    chimique massique et superficielle, texture, structure, conductivite electrique) ont ete etudiees, ainsi que leurs proprietes d'adsorption vis-a-vis des partenaires gazeux de la reaction catalytique. Cette analyse a permis d'avancer un mecanisme reactionnel fonde sur des oxydations et reductions successives de la surface active par les reactifs. L'etude cinetique de la reaction a confirme l'existence de ce mecanisme par oxydo-reduction, qui n'a lieu que pour les oxydes dont la teneur en uranium depasse 0,0014. Le dioxyde de carbone engendre par la reaction a un role inhibiteur par blocage des sites d'adsorption du monoxyde de carbone. Ces oxydes mixtes non stoechiometriques sont un exemple particulierement net d'une catalyse par echange d'oxygene entre le solide et la phase gazeuse. (auteur)

  10. Study of the artificial radioactivity of the marine medium using gamma spectrometry (1962-1966); Etude de la radioactivite artificielle du milieu marin par spectrometrie gamma (1962-1966)

    Energy Technology Data Exchange (ETDEWEB)

    Chesselet, R. [Commissariat a l' Energie Atomique, Saclay (France). Centre d' Etudes Nucleaires

    1969-07-01

    atmospherique entre 1962 et 1964 et les incidences de cette retombee dans les eaux marines superficielles. Considerant les radioelements etudies comme des 'traceurs' dans le milieu marin, divers processus physiques, chimiques ou biologiques ont ete etudies par spectrometrie gamma a haute sensibilite. Le passage en phase soluble dans l'eau de mer d'une fraction importante (environ 50 pour cent) de l'activite liee aux poussieres radioactives, phenomene qui n'etait pas previsible pour les radioelements etudies, gouverne les processus d'accumulation dans la biomasse planctonique et la diffusion vers les eaux plus profondes. Par ailleurs, une methode de spectrometrie 'in situ' permettant de mesurer directement dans la mer de tres faibles concentrations en certains radionuclides emetteurs gamma est decrite. L'application de cette methode a permis de nombreuses observations dans les eaux de surface de la Mediterranee occidentale et du Golfe de Gascogne. On montre quo la profondeur de melange est etroitement liee a celle de la thermocline. Un processus d'accumulation au niveau de celle-ci est constate. Les coefficients de diffusion sont voisins du coefficient de diffusion thermique turbulente. L'existence de compartiments qui subsistent pendant plusieurs mois dans la masse des eaux de surface du Golfe de Gascogne est mise en evidence. Grace aux bilans effectues, une etude comparative montre que le taux de retombees radioactives sur les surfaces maritimes est toujours deux a trois fois superieur a celui sur les regions continentales voisines. Diverses explications de ce phenomene sont discutees. (auteur)

  11. Cartografía mediante imágenes Landsat de suelos salinos en la tierra de Medina (Valladolid

    Directory of Open Access Journals (Sweden)

    García Rodríguez, M. P.

    2010-07-01

    cartographier les sols salins (solonchaks, solonetzs et luvisoles avec des phases saliques dans la «Tierra de Medina» (Valladolid avec l’aide des senseurs TM et ETM des satellites Landsat. Les signes spectraux de ces sols montrent des valeurs numériques très hautes, ce qui permet de les différencier d’autres types de sols et couvertures superficielles. Les valeurs plus hautes correspondent auxquelles ont efflorescences salines en surface et ceux plus faibles aux sols perturbés par hydromorphisme. Les images obtenues avec le NDVI ne permettent pas une discrimination claire des sols salins, mais d’interpréter les changements dans leur utilisation. Dans ces sols les valeurs de NDVI sont très faibles, en accord avec leur faible végétation. Il y a une diminution remarquable entre les années 1987 et 2001, ce qui implique une détérioration de sa structure et une grande perte de couverture végétale; beaucoup des sols perturbés par salinización ont été cessés de cultiver.

  12. Evolution des composés polyphénoliques du bois de chêne au cours de son séchage. Premiers résultats

    Directory of Open Access Journals (Sweden)

    Pascal Chatonnet

    1994-12-01

    Full Text Available Pendant le séchage naturel à l'air libre du bois de chêne, on assiste à une diminution régulière de la teneur du bois en composés extractibles et notamment en composés phénoliques du type ellagitanins. La diminution de la teneur en ellagitanins provient essentiellement de la disparition des formes oligomères solubles dans l'eau; les tanins polymérisés non extractibles car liés aux parois, évoluent peu au çours du séchage naturel à l'air libre. Les bois séchés rapidement à l'étuve possèdent toujours des quantités plus importantes de polyphénols. Dans le même temps et quel que soit le mode de séchage, on obselVe une diminution des coumarines glucosilées et une augmentation non proportionnelle des formes aglycones. D'un point de vue organoleptique, la diminution de l'ensemble des polyphénols pendant le séchage peut être interprétée comme favorable à l'évolution de la qualité du merrain. En effet, les fractions amères et astringentes du bois de chêne correspondent respectivement .sr certains tanins ellagiques et catéchiques. L'intelVention des micro-organismes isolés de bois de chêne en cours de séchage naturel dans la disparition des composés phénoliques du bois est discutée. La grande majorité des moisissures étudiées est capable de dégrader enzymatiquement les tanins hydrolysables du chêne. Cependant, ces mêmes composés s'hydrolysent et s'oxydent spontanément en présence d'eau et d'oxygène. En conséquence, considérant le profil de colonisation du bois par les micro-organismes et celui des composés phénoliques extractibles, on peut supposer que l'on assiste vraisemblablement pendant le séchage naturel du bois à une dégradation biochimique et chimique des polyphénols dans les couches superficielles du merrain (0 à 4 mm de profondeur et à une dégradation presque' exclusivement chimique au coeur du merrain. La présence d'eau et d'oxygène apparaÎt par conséquent comme un facteur

  13. Bases scientifiques de la préparation des catalyseurs constitués d'oxydes mixtes massiques Scientific Bases for the Preparation of Bulk Mixed Oxide Catalysts

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    Courty P.

    2006-11-01

    Full Text Available Cette étude de la préparation des oxydes mixtes massiques et de leur mise en régime dans le milieu réactionnel concerne les différentes méthodes de synthèse utilisées et précise quelles sont les lois générales qui régissent chaque étape unitaire de la préparation (séparation d'un précurseur hydraté, lavage, transformations hydrothermiques, séchage, activation thermique, addition d'autres éléments, mise en forme. De nombreux exemples révèlent la diversité des transformations observées. L'oxyde mixte activé thermiquement est lui-même le précurseur d'autres oxydes mixtes, de métaux ou d'alliages divisés, de sulfures, de nitrures ou de carbures simples ou mixtes. Ces composés se forment lors de la stabilisation du catalyseur dans le milieu réactionnel, au cours d'une transformation complexe et spécifique du type de réaction envisagé. Divers exemples (oxydation ménagée, déshydrogénation, synthèse d'alcools sont donnés. Une caractérisation systématique et rigoureuse de chaque intermédiaire réactionnel est finalement nécessaire pour préciser les lois plus ou moins empiriques qui régissent l'évolution des propriétés superficielles et massiques de texture, de structure et de composition, au cours des transformations successives du solide ; le procédé de préparation et de mise en régime optimisé qui en résulte doit enfin satisfaire aux impératifs techniques et économiques du procédé catalytique correspondant. This study of the preparation of bulk mixed oxides and of their stabilization into the reaction medium deals with the different synthesis methods used and gives some general laws governing each specific type of preparation (separation of a hydrated precursor, washing, hydrothermal transformations, drying, thermal activation, addition of other elements, shaping. Numerous examples reveal the diversity of the transformations observed. The thermally activated mixed oxide becomes itself the

  14. Lésions bulleuses et purpuriques unilatérales: pathomimie cutanée

    Science.gov (United States)

    Zinoun, Mouna; Chiheb, Soumia; Marnissi, Farida; Kadiri, Nadia; Benchikhi, Hakima

    2015-01-01

    La pathomimie cutanée est une forme particulière de troubles factices relativement rare, et constitue l'un des problèmes les plus complexes pour le dermatologue. Nous rapportons un cas de pathomimie révélée par des lésions cutanées unilatérales, mimant une brûlure. Une jeune femme de 27 ans, était suivie depuis 4 ans pour une dépression. Elle a présenté 15j avant sa 1ère hospitalisation un placard inflammatoire du sein gauche compliqué de lésions bulleuses et d’érosions superficielles. La biopsie cutanée avait montré une dermite non spécifique. Une cicatrisation rapide sous traitement local a été notée. Elle a présenté 10 jours plus tard de nouvelles lésions similaires étagées au membre inférieur gauche, évoluant vers le décollement bulleux spontané. La biopsie cutanée avait montré un décollement bulleux jonctionnel et des foyers de nécrose ischémique. L'IFD était négative. Devant les données anamnestiques, cliniques, la négativité du bilan paraclinique, et la guérison des lésions sous pansements occlusifs seuls, le diagnostic de pathomimie a été évoqué et retenu. La patiente a été adressée en psychiatrie où une thérapie cognitivo-comportementale a été préconisée. Notre observation correspond à un tableau de pathomimie de présentation clinique particulière par sa localisation unilatérale et son caractère bulleux. Chez notre patiente qui est droitière, la localisation unilatérale gauche sur des zones accessibles, l'absence de lésions spécifiques à l'examen histologique, la cicatrisation rapide des lésions sous traitement local occlusif seul et leur récurrence malgré des soins adaptés étaient en faveur d'une pathologie factice. Néanmoins, la localisation au niveau des seins peut être très déroutante. Le caractère bulleux des lésions dans le cadre d'une pathomimie a été rarement rapporté. Dans notre cas, la pathomimie s'associe à des troubles anxieux et dépressifs très importants

  15. Aluminium-nickel-iron alloys resistant to corrosion by water at high temperature. Their basic properties - their improvement; Les alliages aluminium-nickel-fer resistant a la corrosion par l'eau a haute temperature. Bases de leurs proprietes - leur amelioration

    Energy Technology Data Exchange (ETDEWEB)

    Coriou, H; Fournier, R; Grall, L; Hure, J [Commissariat a l' Energie Atomique, Saclay (France).Centre d' Etudes Nucleaires

    1959-07-01

    intergranulaire. Ces ilots distincts de la couche de corrosion superficielle et se formant parfois tres en profondeur dans le metal seraient dus a des heterogeneites de composition de la solution solide constituant la matrice de ces alliages. 2 - Le role des additions de titane et zirconium sur le metal lamine. On etudie systematiquement sur une serie d'alliages ayant des teneurs en titane et zirconium comprises entre 0,05 et 0,15 pour cent. On a pu mettre en evidence en particulier le role favorable du titane. Le zirconium agit dans le meme sens mais moins efficacement. L'amelioration due a ces additions est a rapprocher de leur action sur la repartition des secondes phases qui tendent a s'affirmer et a se repartir de fa n plus homogene. On a etudie l'influence de la soudure sur ces alliages ce qui met en evidence le role des gradients de structure introduite par la fusion. (auteur)

  16. Frontal and band displacement chromatography of the hydrogen isotopes on palladium; Chromatographies frontale et de deplacement de bande des isotopes de l'hydrogene sur palladium

    Energy Technology Data Exchange (ETDEWEB)

    Botter, F; Menes, J; Tistchenko, S; Dirian, G [Commissariat a l' Energie Atomique, Saclay (France). Centre d' Etudes Nucleaires

    1964-07-01

    different de 1. Ce modele, celui d'un echange isotopique a contre courant, neglige les diffusions longitudinales en phase gazeuse et laterale en phase adsorbee et ne tient compte que de la resistance superficielle a l'echange entre phases. Il permet de chiffrer l'efficacite d'une colonne de chromatographie en hauteur equivalente a un plateau theorique (HEPT). Les ecarts legers observes entre les chromatogrammes reels et le modele simple justifient d'une part l'etude entreprise d'un modele plus elabore tenant compte des diffusions, d'autre part l'adoption du modele simple pour la comparaison de l'efficacite de plusieurs colonnes. Nous donnons egalement une methode graphique simple et nouvelle de deduire le nombre de plateaux theoriques d'une colonne des chromatogrammes de deplacement de bande et de frontales. Experimentalement, nous donnons essentiellement les criteres de validite du modele utilise, la loi en fonction de la temperature de {sup {alpha}}H{sub 2}-HD, l'etude de la HEPT en fonction de divers parametres sur plusieurs masses palladiees, et les possibilites de l'application a la chromatographie preparative. (auteurs)

  17. Method of uranium prospecting in a mining division: development and results; La methode de prospection de l'uranium dans une division miniere: sa mise au point - ses resultats

    Energy Technology Data Exchange (ETDEWEB)

    Carrat, G. [Commissariat a l' energie atomique et aux energies alternatives - Service des Recherches a la Division Grury CEA (France)

    1959-07-01

    'expose des diverses techniques qui se relaient pour aboutir aux travaux miniers et a l'individualisation d'un tonnage d'uranium exploitable, est presente l'aspect synthetique de l'operation realisee depuis douze annees. La methode de prospection a fait l'objet de modifications profondes depuis le debut des travaux. Au cours des annees, elle a evolue en fonction de la connaissance progressivement acquise du mode de gisement des indices et des gisements uraniferes. En outre, elle s'est diversifiee en adaptant au terrain la merveilleuse technique nouvelle que nous appelons la radiometrie ou etude des radioactivites superficielles. Elle a egalement utilise un certain nombre de techniques geophysiques ou geochimiques, realisant ainsi sur le terrain une gamme de travaux qui permettent une reconnaissance poussee du sous-sol avant le debut des travaux miniers. Mais a aucun moment elle n'a songe a abandonner la conception classique et fondamentale du leve geologique de terrain au marteau et a la boussole. Dans ce domaine, elle a tente de tirer parti des enseignements fournis par un leve geomorphologique et tectonique precis. La plupart de ces travaux ont ete effectues sur les terrains granitiques du Morvan et les gisements qui sont consideres dans cette etude sont tous typiquement hydrothermaux. Reproduction d'un article publie dans 'Annales des Mines', Mars 1959.

  18. Diffusion along and around dislocations; Diffusion le long et autour des dislocations

    Energy Technology Data Exchange (ETDEWEB)

    Brebec, G [Commissariat a l' Energie Atomique, Saclay (France). Centre d' Etudes Nucleaires

    1965-07-01

    la forme, supposee cylindrique, de la region perturbee autour d'une dislocation. Il s'ensuit que l'equation de Fick est utilisee sous la forme: {partial_derivative}C/{partial_derivative}t=D[{partial_derivative}{sup 2}C/{partial_derivative}r{sup 2}+1/r{partial_derivative}C/{partial_derivative}r+{partial_derivative}{sup 2}C/{partial_derivative}z{sup 2}] pour pouvoir exprimer simplement les conditions aux limites et pour que la solution rende compte de la symetrie de revolution du systeme. Ceci complique beaucoup la forme finale des resultats. Nous donnons a titre indicatif, une solution obtenue en partant des memes hypotheses, et en faisant les memes approximations que celles utilisees par WHIPPLE dans son etude du joint de grain. Malheureusement la forme finale est impropre au calcul numerique. En faisant des approximations plus larges, equivalentes a celles de FISHER, nous pouvons determiner le parametre D/(a{sup 2}D'): on retrouve le meme resultat que pour le joint a savoir que le logarithme de la concentration moyenne varie lineairement avec la penetration, la pente de cette droite est proportionnelle a {radical}(D/(a{sup 2}D')) Enfin nous donnons la solution exacte relative a une feuille d'epaisseur finie en supposant que la diffusion dans le bon cristal est negligeable et que la diffusion superficielle est infiniment rapide. C'est le probleme traite par HENDRICKSON et MACHLIN. Nous arrivons a des conclusions differentes de celles obtenues par ces deux auteurs. (auteur)

  19. Efeito da utilização de um centrifugado de medula óssea no tratamento de lesão muscular: estudo experimental em coelhos Effect of use of bone-marrow centrifugate on muscle injury treatment: experimental study on rabbits

    Directory of Open Access Journals (Sweden)

    Daniel Ferreira Fernandes Vieira

    2011-01-01

    Full Text Available OBJETIVO: O objetivo deste estudo foi de avaliar o efeito de um centrifugado de medula óssea na cicatrização de lesão muscular em coelhos. MÉTODOS: Este estudo experimental envolveu a utilização de 15 coelhos machos, adultos, da raça Nova Zelândia Branco. Em cada animal, realizou-se uma lesão transversal na região média do músculo tibial anterior direito, onde se adicionou uma esponja de colágeno absorvível, embebida em um centrifugado de aspirado de medula óssea do osso ilíaco ipsilateral. O membro posterior esquerdo foi utilizado como controle, sendo submetido à mesma lesão; porém, neste caso, adicionando-se somente a esponja de colágeno absorvível. Após 30 dias, os animais foram sacrificados para o estudo da cicatrização muscular. As áreas de cicatrização muscular foram submetidas à análise histológica com histomorfometria, interessando-se a mensuração do número de células musculares em regeneração por micrômetro quadrado e a proporção de fibrose formada. RESULTADOS: O método de centrifugação utilizado neste estudo resultou em uma concentração média de células nucleadas maior que o número destas células nos aspirados originais, sem provocar destruição celular significativa. A adição do centrifugado de medula óssea não resultou em aumento significante do número de células musculares em regeneração em relação ao grupo controle. Também não houve diferença significante na proporção de fibrose formada em relação ao grupo controle. CONCLUSÃO: A administração de centrifugado de medula óssea utilizada neste estudo não favoreceu a cicatrização de lesão muscular em coelhos.OBJECTIVE: The objective of this study was to evaluate the effect of bone-marrow centrifugate on the healing of muscle injuries in rabbits. METHODS: This experimental study involved use of fifteen adult male New Zealand White rabbits. Each animal received a transverse lesion in the middle of the right

  20. Comparative study between polypropylene and polypropylene/poliglecaprone meshes used in the correction of abdominal wall defect in rats Estudo comparativo entre as telas de polipropileno e polipropileno/poliglecaprone utilizadas na correção de defeito na parede abdominal ventral de ratos

    Directory of Open Access Journals (Sweden)

    Carlos Alberto Lima Utrabo

    2012-04-01

    ós-operatório. Fragmentos da parede abdominal foram submetidos à análise macroscópica, tensiométrica e histológica. RESULTADOS: A tensiometria no subgrupo A30 mostrou tensão média de ruptura de 0,78 Mpa e no subgrupo A60 de 0,66 MPa. No subgrupo B30 foi de 0,84 MPa e no B60 de 1,27 MPa. O escore do processo inflamatório mostrou fase subaguda nos subgrupos A30 e B30 e processo inflamatório crônico no subgrupo A60 e B60. CONCLUSÕES: A resistência à tensão foi maior na parede reparada pela tela de polipropileno/poliglecaprone no 60º dia pós-operatório. Na análise histológica houve maior concentração da fibrose na superfície da tela de polipropileno com tendência ao encapsulamento. Nos subgrupos polipropileno/poliglecaprone a análise histológica mostrou maior fibrose