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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

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    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. 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)

  4. Polysomnography in idiopathic muscle pain syndrome (fibrositis)

    OpenAIRE

    Silva, A. B.; Bertoríni, T. E.; Lemmi, H.

    1991-01-01

    Muscle pain occurs in various neuromuscular disorders with characteristic physiological or biochemical abnormalities. There is, however, a group of patients in whom there is no clear physiological or structural basis for their pains. This syndrome has been called fibrositis or fibromyalgia. Sleep abnormalities have been reported in some of these patients, but have not been confirmed by others. We studied 8 patients with this disorder and found sleep abnormalities that were characterized by no...

  5. Nephrogenic systemic fibrosis; Nephrogene systemische Fibrose

    Energy Technology Data Exchange (ETDEWEB)

    Samtleben, W. [Klinikum Grosshadern der Ludwig-Maximilians-Universitaet Muenchen, Schwerpunkt Nephrologie,Medizinische Klinik und Poliklinik I, Muenchen (Germany)

    2007-09-15

    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) <30 ml/min per 1.73 m{sup 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 <30 ml/min per 1.73 m{sup 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.) [German] Ein neues, Skleromyxoedem-aehnliches Krankheitsbild wurde erstmals 1997 beobachtet und im Jahre 2000 publiziert. Es trat nur bei Patienten mit eingeschraenkter Nierenfunktion (glomerulaere Filtrationsrate [GFR] <30 ml/min/1,73 m{sup 2}) auf, weshalb der Terminus nephrogene fibrosierende Sklerose gewaehlt wurde. Erst 2006 konnte ein

  6. Bacteriologia da Fibrose Cística

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    Larissa Lutz

    2011-07-01

    Full Text Available O exame bacteriológico é um dos principais parâmetros que auxiliam o diagnóstico e manuseio da infecção respiratória dos pacientes com Fibrose Cística (FC. Os microrganismos que colonizam e infectam o paciente fibrocístico determinam o tratamento, a qualidade de vida, as perspectivas para o transplante e a sua sobrevida global. A identificação precisa de patógenos respiratórios é essencial para o tratamento da infecção, seja como guia para o uso adequado de antibióticos por longos períodos para os pacientes com infecção bacteriana crônica ou para a aplicação adequada de medidas de controle de infecção. Embora exista um espectro limitado de patógenos respiratórios classicamente associados à doença respiratória na FC, um número crescente de microrganismos vem sendo reconhecido como potenciais agentes patogênicos. O espectro de patógenos em FC varia com a idade do paciente mas, de uma forma geral, é bem estabelecido na literatura que existem quatro bactérias “clássicas”: Staphylococcus aureus, Haemophilus influenzae, Pseudomonas aeruginosa e o complexo B. cepacia (CBC. A maior sobrevida dos pacientes fibrocísticos os quais são submetidos a ciclos repetidos de antibióticos bem como o uso de novas metodologias de diagnóstico microbiológico contribuíram para o reconhecimento de patógenos emergentes ou “não-clássicos”.

  7. "Efeito da colchicina na fibroses hepatica induzida quimicamente em coelhos"

    OpenAIRE

    Clodomir Garcia Brandão

    1999-01-01

    Resumo: O desenvolvimento de fibrose é um ponto fundamental na formação da cirrose hepática e sua compreensão é essencial para o entendimento da fisiopatologia da cirrose e da conseqüente hipertensão portal. Assim, o desenvolvimento de um modelo experimental adequado de fibrose hepática é útil para o entendimento das enfermidades que podem evoluir para a cirrose hepática. A colchicina tem sido usada em pacientes com cirrose hepática mas efeitos não são totalmente conhecidos. Este estudo teve ...

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

    Energy Technology Data Exchange (ETDEWEB)

    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.

  9. Fuzheng Huayu recipe prevents nutritional fibrosing steatohepatitis in mice

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    Jia Yan-Hong

    2012-03-01

    Full Text Available Abstract Background Fuzheng Huayu recipe (FZHY, a compound of Chinese herbal medicine, was reported to improve liver function and fibrosis in patients with hepatitis B virus infection. However, its effect on nutritional fibrosing steatohepatitis is unclear. We aimed to elucidate the role and molecular mechanism of FZHY on this disorder in mice. Methods C57BL/6 J mice were fed with methionine-choline deficient (MCD diet for 8 weeks to induce fibrosing steatohepatitis. FZHY and/or heme oxygenase-1 (HO-1 chemical inducer (hemin were administered to mice, respectively. The effect of FZHY was assessed by comparing the severity of hepatic injury, levels of hepatic lipid peroxides, activation of hepatic stellate cells (HSCs and the expression of oxidative stress, inflammatory and fibrogenic related genes. Results Mice fed with MCD diet for 8 weeks showed severe hepatic injury including hepatic steatosis, necro-inflammation and fibrosis. Administration of FZHY or hemin significantly lowered serum levels of alanine aminotransferase, aspartate aminotransferase, reduced hepatic oxidative stress and ameliorated hepatic inflammation and fibrosis. An additive effect was observed in mice fed MCD supplemented with FZHY or/and hemin. These effects were associated with down-regulation of pro-oxidative stress gene cytochrome P450 2E1, up-regulation of anti-oxidative gene HO-1; suppression of pro-inflammation genes tumor necrosis factor alpha and interleukin-6; and inhibition of pro-fibrotic genes including α-smooth muscle actin, transforming growth factor beta 1, collagen type I (Col-1 and Col-3. Conclusions Our study demonstrated the protective role of FZHY in ameliorating nutritional fibrosing steatohepatitis. The effect was mediated through regulating key genes related to oxidative stress, inflammation and fibrogenesis.

  10. Fibrose Cística: Diagnóstico e Tratamento

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    Anneliese Hoffmann

    2011-07-01

    Full Text Available Fibrose Cística, também chamada de mucoviscidose, é uma doença crônica, de origem genética, causada por um gene defeituoso herdado do pai e outro da mãe. Não é, portanto, uma doença contagiosa. O defeito genético faz com que todas as glândulas secretoras do corpo sejam afetadas. É por este motivo que o suor é salgado, o muco que reveste as vias aéreas é mais espesso e a bile também é mais viscosa.

  11. Development and validation of the Frontal Fibrosing Alopecia Severity Score.

    Science.gov (United States)

    Saceda-Corralo, David; Moreno-Arrones, Óscar Muñoz; Fonda-Pascual, Pablo; Pindado-Ortega, Cristina; Buendía-Castaño, Diego; Alegre-Sánchez, Adrián; Segurado-Miravalles, Gonzalo; Rodrigues-Barata, Ana Rita; Jaén-Olasolo, Pedro; Vaño-Galván, Sergio

    2018-03-01

    Frontal fibrosing alopecia (FFA) is a scarring alopecia characterized by recession of the frontotemporal hairline and loss of the eyebrows. To design and validate a scoring system to assess the severity of FFA. The Frontal Fibrosing Alopecia Severity Score (FFASS) was developed; criterion validity was assessed by the Investigator's Global Assessment, and construct validity was evaluated by the convergence of other measures of severity (the Patient's Global Assessment], the rest of the clinical features, the Lichen Planopilaris Activity Index, and quality of life measures (Dermatology Life Quality Index and Hospital Anxiety Depression Scale). Intraobserver and interobserver reliability were determined. In total, 103 female patients were included. The FFASS showed significant correlation to the Patient's Global Assessment, occipital involvement, and the Lichen Planopilaris Activity Index. Intraobserver reliability was completed for 31 subjects and showed good correlation (intraclass correlation coefficient, 0.86; 95% confidence interval, 0.7-0.95; P < .001). Interobserver reliability showed excellent correlation (intraclass correlation coefficient, 0.97; 95% confidence interval, 0.95-0.99; P < .001). The study was performed at a single institution, and only female patients were assessed. The FFASS is a statistically validated scale and a reliable measure of FFA severity, and it can be used in clinical practice and future research studies as an assessment tool. Copyright © 2017 American Academy of Dermatology, Inc. Published by Elsevier Inc. All rights reserved.

  12. Contribution à l'étude des formations superficielles de la boutonnière du Bélinois (Sarthe, France

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    Jean-Pierre Larue

    2008-09-01

    Full Text Available L'étude géomorphologique et sédimentologique permet de distinguer plusieurs formations superficielles conservées dans la boutonnière du Bélinois. Les sables fins inférieurs sont issus de l'altération des marno-calcaires jurassiques alors que les sables supérieurs proviennent des sables cénomaniens périphériques et ont été mis en place par le vent lors de la dernière phase froide du Quaternaire. Entre les deux formations, on trouve un horizon plus argileux d'origine pédologique et, localement, des colluvions sablo-argileuses. La rareté des formations superficielles sur les versants et dans le fond des vallons témoigne de l'efficacité du déblaiement par les cours d'eau lors des phases de creusement.The geomorphological and sedimentological study makes it possible to distinguish several superficial formations preserved in the Belinois depression. The lower fine sands come from the alteration of Jurassic marl-limestone whereas the upper sands issue from Cenomanian outlying sands and were brought by wind during the last glaciation period. A clay sand bed coming from pedogenetic process and, in places, from clay sand colluvial materials, lie between the two formations. The thinness of superficial formations on the slopes and in the vale bottoms testifies the efficiency of river erosion during the periods of incision.

  13. DHEA and frontal fibrosing alopecia: molecular and physiopathological mechanisms.

    Science.gov (United States)

    Gaspar, Neide Kalil

    2016-01-01

    The transforming growth factor-beta 1 (TGFβ1) promotes fibrosis, differentiating epithelial cells and quiescent fibroblasts into myofibroblasts and increasing expression of extracellular matrix. Recent investigations have shown that PPAR (peroxisome proliferator-activated receptor*) is a negative regulator of fibrotic events induced by TGFβ1. Dehydroepiandrosterone (DHEA) is an immunomodulatory hormone essential for PPAR functions, and is reduced in some processes characterized by fibrosis. Although scarring alopecia characteristically develops in the female biological period in which occurs decreased production of DHEA, there are no data in the literature relating its reduction to fibrogenic process of this condition. This article aims to review the fibrogenic activity of TGFβ1, its control by PPAR and its relation with DHEA in the frontal fibrosing alopecia.

  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. 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%.

  17. Fisioterapia Respiratória na Fibrose Cística

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    Jefferson Veronezi

    2011-07-01

    Full Text Available A fisioterapia torácica convencional (FTC foi introduzida na década de 1950 como “padrão ouro” nos cuidados dos pacientes com fibrose cística (FC, no entanto há poucas evidências para que seu uso seja mantido na rotina diária. Revisou-se a evolução das condutas fisioterapêuticas em pacientes portadores de FC, bem como as novas opções de tratamento, através de um levantamento bibliográfico nas bases de dados eletrônicas Cochrane, LILACS, MEDLINE e SciELO no período entre 1990 e 2010. Nas últimas décadas a fisioterapia respiratória evoluiu favoravelmente disponibilizando aos pacientes técnicas ativas, realizadas em posições mais confortáveis e tão ou mais efetivas do que as convencionais. Entre elas, destacamos a pressão expiratória positiva (PEP, PEP oscilatória, ciclo ativo da respiração, aumento do fluxo expiratório, drenagem autógena e drenagem autógena modificada. O paciente deve conhecer e eventualmente participar, juntamente com o profissional, da definição da técnica mais apropriada a seu caso. Para tal, uma boa relação fisioterapeuta-paciente é de fundamental importância.

  18. 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...... in patients with fibrositis syndrome than in healthy subjects....

  19. 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.

  20. Controvérsias na fibrose cística: do pediatra ao especialista

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    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.

  1. 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

  2. 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)

  3. Epidemiological data and radiation risk estimates; Donnees epidemiologiques et estimations de risques radio-induits

    Energy Technology Data Exchange (ETDEWEB)

    Cardis, E. [Centre International de Recherche sur le Cancer, Unite Rayonnement et Cancer, 69 - Lyon (France)

    2002-01-01

    The results of several major epidemiology studies on populations with particular exposure to ionizing radiation should become available during the first years of the 21. century. These studies are expected to provide answers to a number of questions concerning public health and radiation protection. Most of the populations concerned were accidentally exposed to radiation in ex-USSR or elsewhere or in a nuclear industrial context. The results will complete and test information on risk coming from studies among survivors of the Hiroshima and Nagasaki atomic bombs, particularly studies on the effects of low dose exposure and prolonged low-dose exposure, of different types of radiation, and environmental and host-related factors which could modify the risk of radiation-induced effects. These studies are thus important to assess the currently accepted scientific evidence on radiation protection for workers and the general population. In addition, supplementary information on radiation protection could be provided by formal comparisons and analyses combining data from populations with different types of exposure. Finally, in order to provide pertinent information for public health and radiation protection, future epidemiology studies should be targeted and designed to answer specific questions, concerning, for example, the risk for specific populations (children, patients, people with genetic predisposition). An integrated approach, combining epidemiology and studies on the mechanisms of radiation induction should provide particularly pertinent information. (author)

  4. 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

  5. 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...

  6. Progressive subretinal fibrosis and multifocal granulomatous chorioretinitis Fibrose sub-retiniana progressiva e corioretinite granulomatosa multifocal

    Directory of Open Access Journals (Sweden)

    Miguel Hage Amaro

    2006-06-01

    Full Text Available We describe a case of progressive subretinal fibrosis and multifocal chorioretinitis along with its findings on both fluorescein and indocyanine green angiography. The progressive subretinal fibrosis syndrome is a severe subset of multifocal choroiditis. The clustering of lesions around the nerve optic head may mean that the disease is spread through the flow in and out of the eye around the optic nerve.Descrevemos um caso de fibrose sub-retiniana progressiva e corrioretinite multifocal granulomatosa junto com os achados de angiografia fluoresceínica e com indocianina verde e propomos uma nova fisiopatologia para a fibrose em anel justa-papilar. A síndrome de fibrose sub-retiniana progressiva é uma doença grave, rara e constitui um subtipo grave da coroidite multifocal. As múltiplas lesões com fibrose circundando o disco óptico podem significar que a doença é oriunda do fluxo do líquido céfalo-raqueano, ao redor do nervo óptico.

  7. Achados Radiológicos Pulmonares da Fibrose Cística

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    Kelly Ribeiro Neves

    2011-07-01

    Full Text Available As radiografias de tórax são essenciais na avaliação pulmonar em pacientes com fibrose cística e a despeito da superioridade técnica da tomografia computadorizada, elas ainda permanecem como a ferramenta diagnóstica primária no seguimento destes pacientes. Demonstramos algumas manifestações radiológicas pulmonares desta entidade diagnóstica e destacamos alguns aspectos da literatura.

  8. Alopecia frontal fibrosante: relato de seis casos Frontal fibrosing alopecia: report of six cases

    Directory of Open Access Journals (Sweden)

    Fabiane Mulinari-Brenner

    2007-10-01

    Full Text Available Alopecia frontal fibrosante é forma progressiva de alopecia cicatricial. Os casos iniciais foram relatados a partir 1994, na Austrália, em pacientes do sexo feminino pós-menopausa. Desde então inúmeros casos foram descritos na literatura sugerindo que ela é mais prevalente do que inicialmente se supunha. Seu curso progressivo se assemelha ao da alopecia androgenética; histologicamente, entretanto, o infiltrado liquenóide é evidente. O artigo relata seis casos brasileiros e discute a alopecia frontal fibrosante dentro do grupo das alopecias cicatriciais, como variante do líquen plano pilar.Frontal fibrosing alopecia is a progressive cicatricial alopecia. The first cases were described in Australia in postmenopausal women, in 1994. Since then, numerous cases were reported, suggesting that frontal fibrosing alopecia is more prevalent than initially thought. Its progressive course in postmenopausal women, clinically resembles androgenetic alopecia; however, histologically, lichenoid infiltrate is evident. This article report six brazilian cases of frontal fibrosing alopecia and discusses them in the context of cicatricial alopecias, as a variant of lichen planopilaris.

  9. 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.

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

    Energy Technology Data Exchange (ETDEWEB)

    Harman, Mustafa E-mail: drmharman@hotmail.com; Sayarlioglu, Mehmet; Arslan, Halil; Ayakta, Hayati; Harman, Ece

    2003-11-01

    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.

  11. 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.

  12. Infecção Respiratória na Fibrose Cística e Tratamento

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    Anneliese Hoffmann

    2011-08-01

    Full Text Available Fibrose Cística é uma doença multissistêmica e complexa, que exige tratamento contínuo. A doença pulmonar é o principal determinante da sua evolução. Alterações na composição da secreção das vias aéreas levam ao desenvolvimento de doença pulmonar progressiva com infecção respiratória crônica por germes peculiares à doença.  Esquemas de antibioticoterapia para tratamento das exacerbações pulmonares, erradicação de bactérias ou de supressão da infecção crônica constituem parte importante do tratamento, com repercussão significativa na melhora dos sintomas, da função pulmonar e da qualidade de vida dos pacientes com Fibrose Cística.

  13. Severe fibrosing mediastinitis with atypical presentation: Effective control with novel therapeutic approach.

    Science.gov (United States)

    Joury, Abdulaziz Uthman; Al Boukai, Ahmad Amer; Kashour, Tarek Seifaw

    2017-01-01

    Fibrosing mediastinitis (FM), also known as sclerosing mediastinitis, is an uncommon but serious disease involving the mediastinal structures. A high index of suspicion is essential to establish the diagnosis of FM and starting the appropriate therapy for patients. Here, we report a case of a young female who presented with chest symptoms and subsequently underwent different laboratory and radiologic investigations and an excisional biopsy. The findings of these investigations were consistent with the diagnosis of idiopathic FM. Her disease was associated with complete occlusion of three pulmonary veins and the left main pulmonary artery. The patient was treated with initial high-dose steroids followed by maintenance steroid and methotrexate therapy with very good long-term disease control. Clinical response, high-sensitivity C-reactive protein, and erythrocyte sedimentation rate were used to monitor disease activity and response to therapy.

  14. Severe fibrosing mediastinitis with atypical presentation: Effective control with novel therapeutic approach

    Directory of Open Access Journals (Sweden)

    Abdulaziz Uthman Joury

    2017-01-01

    Full Text Available Fibrosing mediastinitis (FM, also known as sclerosing mediastinitis, is an uncommon but serious disease involving the mediastinal structures. A high index of suspicion is essential to establish the diagnosis of FM and starting the appropriate therapy for patients. Here, we report a case of a young female who presented with chest symptoms and subsequently underwent different laboratory and radiologic investigations and an excisional biopsy. The findings of these investigations were consistent with the diagnosis of idiopathic FM. Her disease was associated with complete occlusion of three pulmonary veins and the left main pulmonary artery. The patient was treated with initial high-dose steroids followed by maintenance steroid and methotrexate therapy with very good long-term disease control. Clinical response, high-sensitivity C-reactive protein, and erythrocyte sedimentation rate were used to monitor disease activity and response to therapy.

  15. 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...... of pulmonary fibrosis (ICD 8: 517 and ICD 9: 515 and 516). Cases with a history of exposure to fibrogenic agents or with collagen vascular disease were excluded and the remaining 158 cases were defined as CFA. The average annual incidence of hospitalised CFA was 4.3 per 100,000. No change was observed...... with age were also found when the diagnosis of CFA was exclusively based on cases with hospital file records of breathlessness, bilateral crackles and bilateral shadows on chest radiograph....

  16. Induction of heme oxygenase-1 protects against nutritional fibrosing steatohepatitis in mice

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    Kong Li

    2011-02-01

    Full Text Available Abstract Background Heme oxygenase-1 (HO-1, an antioxidant defense enzyme, has been shown to protect against oxidant-induced liver injury. However, its role on liver fibrosis remains unclear. This study aims to elucidate the effect and the mechanism of HO-1 in nutritional fibrosing steatohepatitis in mice. Methods Male C57BL/6J mice were fed with a methionine-choline deficient (MCD diet for eight weeks to induce hepatic fibrosis. HO-1 chemical inducer (hemin, HO-1 chemical inhibitor zinc protoporphyrin IX (ZnPP-IX and/or adenovirus carrying HO-1 gene (Ad-HO-1 were administered to mice, respectively. Liver injury was assessed by serum ALT, AST levels and histological examination; hepatic lipid peroxides levels were determined; the expression levels of several fibrogenic related genes were assayed by real-time quantitative PCR and Western blot. Results MCD feeding mice showed progressive hepatic injury including hepatic steatosis, inflammatory infiltration and fibrosis. Induction of HO-1 by hemin or Ad-HO-1 significantly attenuated the severity of liver injury. This effect was associated with the up-regulation of HO-1, reduction of hepatic lipid peroxides levels, down-regulation of inflammatory factors tumor necrosis factor-alpha, interleukin-6 and suppressor of cytokine signaling-1 as well as the pro-fibrotic genes alpha-smooth muscle actin, transforming growth factor-β1, matrix metallopeptidase-2 and matrix metallopeptidase-9. A contrary effect was observed in mice treated with ZnPP-IX. Conclusions The present study provided the evidence for the protective role of HO-1 in ameliorating MCD diet-induced fibrosing steatohepatitis. Modulation of HO-1 expression might serve as a therapeutic approach for fibrotic steatohepatitis.

  17. Frontal fibrosing alopecia among men: A clinicopathologic study of 7 cases.

    Science.gov (United States)

    Tolkachjov, Stanislav N; Chaudhry, Hafsa M; Camilleri, Michael J; Torgerson, Rochelle R

    2017-10-01

    Frontal fibrosing alopecia (FFA) is a lichen planopilaris-variant scarring alopecia that has rarely been described in men. To characterize the clinicopathologic findings of FFA in men by studying a series of 7 male patients. We conducted a retrospective review of all cases of male patients with FFA at the Mayo Clinic from 1992 to 2016. Seven male patients with FFA were identified. The frontal scalp (in 6 of 7 patients), sideburns (in 4 of 7), and temporal scalp (in 4 of 7) were most frequently involved. Three patients had involvement of the eyebrows. One patient had hair loss of the upper cutaneous lip. All patients had biopsy evidence of lichen planopilaris. None of the patients had associated autoimmune or thyroid disease. Two patients had hypogonadism upon testosterone studies. Limitations include small sample size and varied follow-up. Although most often reported among postmenopausal women, FFA also occurs among men. The clinical and histopathologic characteristics of FFA in men parallel those described in women with FFA. Unique areas of involvement in men include sideburns and facial hair. Concomitant mucocutaneous lichen planus, autoimmune disease, and thyroid disease are infrequent among men with FFA. Distribution of hair loss and associated hormonal abnormalities aid in the recognition of FFA in men. Copyright © 2017 American Academy of Dermatology, Inc. Published by Elsevier Inc. All rights reserved.

  18. Frontal Fibrosing Alopecia and Concomitant Lichen Planus Pigmentosus: A Case Series of Seven African American Women.

    Science.gov (United States)

    Uwakwe, Laura N; Cardwell, Leah A; Dothard, Emily H; Baroudi, Bernice I; McMichael, Amy J

    2018-04-01

    The association of frontal fibrosing alopecia (FFA) and lichen planus pigmentosus (LPPigm) is rare. Prior reports suggest that FFA and LPPigm are on the same spectrum of disease, and a diagnosis of LPPigm may predict the future development of FFA. We aim to further characterize the association between FFA and LPPigm by reviewing the clinical cases of seven African American women. Seven patients with FFA were diagnosed clinically by recession of frontotemporal hairline and confirmed by histopathologic examination showing lymphocyte-mediated cicatricial alopecia. LPPigm was diagnosed by clinical evaluation alone based on the characteristic morphology, color, and distribution of the lesions. It is difficult to distinguish whether halted progression of FFA was due to the success of the treatment regimen or spontaneous stabilization of disease over time. Our case series supports the theory that FFA and LPPigm likely exist on the same spectrum of disease. Our observations demonstrate a likely positive correlation between FFA and LPPigm. J Drugs Dermatol. 2018;17(4):397-400..

  19. 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.)

  20. Bronchoalveolar lavage fluid cell counts in cryptogenic fibrosing alveolitis and their relation to therapy.

    Science.gov (United States)

    Haslam, P L; Turton, C W; Lukoszek, A; Salsbury, A J; Dewar, A; Collins, J V; Turner-Warwick, M

    1980-01-01

    Bronchoalveolar lavage was used to sample inflammatory cells from the lungs of 51 patients with cryptogenic fibrosing alveolitis (CFA) (24 smokers, 12 ex-smokers, and 15 non-smokers). The smokers with CFA have been compared with 15 smoking control subjects in whom there was no radiographic abnormality or clinical evidence of chronic bronchitis. Significantly lower volumes of lavage fluid were recovered from the smokers with CFA (p < 0.001) and the fluid contained lower percentages of macrophages (p < 0.01), reflecting increased percentages of eosinophils (p < 0.001) and neutrophils (p < 0.01). Similar changes were seen in the ex-smokers and non-smokers. There was also an increase in the percentages of lymphocytes when the whole group of CFA patients was compared with the control subjects (p less than or equal to 0.05). No significant differences were found when patients with "lone" CFA were compared with those having associated systemic disease. The only feature distinguishing smokers from non-smokers with CFA was the presence of pigmented cytoplasmic inclusions in the macrophages from the smokers (p < 0.001). However, there were lower numbers of pigmented macrophages in the smoking CFA patients by comparison with the control subjects suggesting either a change in phagocytic capacity or turnover rate in this disease. Profiles of differential cell counts in individual patients showed that increases of eosinophils over 3% or neutrophils over 4% or both with lymphocyte counts of less than 11% related to a poor clinical response to corticosteroids, but lymphocyte percentages greater than 11% related to improvement (p < 0.05). Images PMID:7434282

  1. 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.

  2. Case study: fibrosing alopecia in a pattern distribution localized on alopecia androgenetica areas and unaffected scalp.

    Science.gov (United States)

    Amato, Lauretta; Chiarini, Caterina; Berti, Samantha; Bruscino, Paolo; Fabbri, Paolo

    2004-01-01

    A 54-year-old man with a 24-year history of androgenetic alopecia was referred to the Department of Dermatological Sciences with follicular inflammatory lesions leading to scleroatrophy in the vertex region (Figure 1) of 1-year duration. These lesions appeared a year ago. There was no previous history of this condition. On examination, the patient showed confluent infiltrative follicular lesions on the frontoparietal and occipital scalp (Figure 2). Some lesions evolved into erosions that developed in ivory white scleroatrophy within weeks. These lesions were localized both in and outside of are as affected by alopecia androgenetica and were associated with mild pruritus. Histopathologic examination, performed on an early lesion of the vertex, documented a mild thinning of follicular epithelium associated with an intense lymphohistiocytic perifollicular infiltrate. The damage of the basal cell layer was limited to the follicle, while epidermis was intact. In particular, follicular keratinocytes under the isthmus showed a very intense degeneration exactly where the infiltrate was the most prominent. The damage of the hair sheath was under the isthmus and involved the lower portions of the follicles (including the hair bulbs). The inflammatory infiltrate was exclusively represented by perifollicular lymphohistiocytes. Finally, a connective fibrotic shell with numerous fibroblasts formed a sheath around the atrophic follicle (Figure 3). Results of laboratory investigations (including complete blood cell counts, basal thyroid-stimulating hormone, C-reactive protein, serum ferritin levels, B and C hepatitis markers, antinuclear antibodies, and cultural examinations) were negative.We diagnosed the patient with fibrosing alopecia in a pattern distribution.

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

    Directory of Open Access Journals (Sweden)

    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.

  4. Avaliação do estado de saúde bucal de pacientes com fibrose cística

    OpenAIRE

    Ana Chapper

    2010-01-01

    Esse estudo transversal avaliou a saúde bucal de 36 pacientes com fibrose cística (FC). Um questionário foi aplicado para obter informações sobre autocuidados e outros aspectos que pudessem influenciar os resultados. Os exames, realizados por examinadora treinada e calibrada, foram placa visível (IPV), sangramento gengival (ISG), profundidade de sondagem (PS), perda de inserção (PI), exsudato à sondagem (SS), presença de sítios com cálculo dental, experiência de cárie (ceo/CPO-D + MBA) e núme...

  5. A experiência da doença na fibrose cística: caminhos para o cuidado integral

    Directory of Open Access Journals (Sweden)

    Tainá Pelucio Pizzignacco

    2011-06-01

    Full Text Available A Fibrose Cística é uma doença crônica que grande impacto exerce sobre a vida familiar. A experiência da doença é a maneira pela qual os indivíduos respondem à doença, atribuindo significados e buscando maneiras para lidar com ela no seu cotidiano. O estudo teve como objetivo compreender a experiência da Fibrose Cística a partir do contexto familiar. Estudo de caso etnográfico, realizado com famílias de crianças com a doença atendidas em um hospital escola do interior do estado de São Paulo. Os resultados foram divididos nos temas: passado, presente e futuro e permearam essas fases a busca pelo significado da doença e pelo suporte social, a importância da religião e espiritualidade e a centralidade da socialização da criança. Conhecer a experiência na doença e a rede social torna-se imprescindível para o planejamento de um cuidado integral. Essa abordagem mostrou-se inovadora para o cuidado à doença crônica.

  6. Nephrogenic systemic fibrosis (NSF) - implications for radiology; Nephrogene systemische Fibrose (NSF) - Implikationen fuer die Radiologie

    Energy Technology Data Exchange (ETDEWEB)

    Michaely, H.J.; Schoenberg, S.O. [Klinikum Mannheim der Universitaet Heidelberg, Institut fuer Klinische Radiologie, Mannheim (Germany); Klinikum Grosshadern der Ludwig-Maximilians-Universitaet Muenchen, Institut fuer Klinische Radiologie, Muenchen (Germany); Thomsen, H.S. [Copenhagen University, Hospital at Herlev, Kopenhagen (Denmark); Reiser, M.F. [Klinikum Grosshadern der Ludwig-Maximilians-Universitaet Muenchen, Institut fuer Klinische Radiologie, Muenchen (Germany)

    2007-09-15

    Nephrogenic systemic fibrosis (NSF) is a systemic disease with a 5% mortality which was first described in 1997 and which only occurs in patients with severely impaired renal function (GFR <30 ml/min per 1.73 m{sup 2}) and for which an association with previous administration of several Gd-chelates has been observed. According to retrospective case control studies the odds ratio for a patient with severely impaired renal function to develop NSF was increased by a factor of 22-32 when gadodiamide was administered. At this time there are approximately 250 confirmed cases of NSF of which 177 are associated with the administration of gadodiamide and 78 are associated with gadopentetate dimeglumine. This review article elucidates the postulated pathogenesis of NSF and provides an overview of the published statements and recommendations from international regulatory authorities and from international advisory boards. Even though the pathogenesis is not completely understood at this time, the European Pharmacovigilance Working Party has decided that gadodiamide and gadopentetate dimeglumine must not be used in high-risk patients. Other Gd-containing contrast agents should only be administered after thorough assessment of the indication and with minimized Gd dose. In the USA, the FDA has issued a black box warning for Gd-containing contrast agents. (orig.) [German] Die nephrogene systemische Fibrose (NSF) ist eine 1997 erstmals beschriebene systemische Erkrankung mit einer 5%igen Mortalitaet, die bislang ausschliesslich bei Patienten mit terminaler Niereninsuffizienz (glomerulaere Filtrationsrate [GFR] <30 ml/min/1,73 m{sup 2}) aufgetreten ist und fuer die eine Assoziation mit der vorherigen Verabreichung verschiedener Gadolinium- (Gd-)haltiger MR-Kontrastmittel beobachtet wurde. Laut retrospektiver Fall-Kontroll-Studien war die Odds Ratio fuer die Entwicklung einer NSF bei Patienten mit stark eingeschraenkter Nierenfunktion 22- bis 32-fach hoeher, wenn Gadodiamid

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

    Directory of Open Access Journals (Sweden)

    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

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

    Directory of Open Access Journals (Sweden)

    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.

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

    Directory of Open Access Journals (Sweden)

    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.

  10. Fibrose quística em adultos Cystic fibrosis in adults

    Directory of Open Access Journals (Sweden)

    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

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

    Directory of Open Access Journals (Sweden)

    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.

  12. 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...

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

    Directory of Open Access Journals (Sweden)

    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

  14. 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.

  15. 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.

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

    Directory of Open Access Journals (Sweden)

    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

  17. Pseudomucocele bilateral associada à fibrose cística: relato de caso Cystic Fibrosis-Associated Bilateral Pseudomucocele: case Report

    Directory of Open Access Journals (Sweden)

    Karla Palma Portes

    2007-12-01

    Full Text Available A fibrose cística, também conhecida como mucoviscidose, é um distúrbio monogenético que se apresenta como uma doença multissistêmica. A incidência é de aproximadamente 1:2500 nascidos vivos. O mecanismo fisiopatológico é uma mudança qualitativa em todas as secreções exócrinas do organismo. O aumento da viscosidade dessas secreções leva à estase e obstrução mecânica, prejudicando a função secretora dos órgãos-alvo. O nariz e seios paranasais são freqüentemente envolvidos devido ao clearence mucociliar anormal, responsável pelo desenvolvimento de rinossinusite crônica, polipose nasal e pseudomucocele sinusal. OBJETIVO: É apresentar um caso raro de pseudomucocele bilateral em uma criança portadora de fibrose cística. DESCRIÇÃO DO CASO: M.F.B.R., 2 anos, masculino, apresentava obstrução nasal crônica e infecções pulmonares recorrentes. O exame clínico detectava presença de secreção nasal abundante, com descarga posterior em orofaringe. A tomografia computadorizada dos seios paranasais mostrou imagem sugestiva de pseudomucocele, com velamento dos seios maxilares e etmóides. A dosagem de sódio e cloro no suor apresentou alterações significativas. Optamos por tratamento cirúrgico, após internação do paciente para controle das manifestações pulmonares exacerbadas. A criança evoluiu com melhora do quadro obstrutivo nasal. CONCLUSÕES: A pseudomucocele é uma entidade que começou a fazer partes da rotina de diagnóstico diferencial a partir do momento em que os exames tomográficos tornaram-se parte da semiologia das doenças sinusais. Os pacientes com psedomucocele têm apresentado um aumento importante da sobrevida graças aos tratamentos atuais.Cystic fibrosis, also known as mucoviscidosis, is a monogenetic disorder that is presented as a multisystemic disease. The incidence is approximately 1: 2500 live births. The pathophysiologic mechanism is a qualitative change in all exocrine secretions of

  18. 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)

  19. Cell cycle regulation and radiation-induced cell death; Regulation du cycle cellulaire et de la mort cellulaire radio-induite

    Energy Technology Data Exchange (ETDEWEB)

    Favaudon, V. [Centre Universitaire d' Orsay, Institut Curie, Section de Recherche, Lab. Raymond-Latarjet, Unite 350 Inserm, 91 (France)

    2000-10-01

    Tight control of cell proliferation is mandatory to prevent cancer formation as well as to normal organ development and homeostasis. This occurs through checkpoints that operate in both time and space and are involved in the control of numerous pathways including DNA replication and transcription, cell cycle progression, signal transduction and differentiation. Moreover, evidence has accumulated to show that apoptosis is tightly connected with the regulation of cell cycle progression. In this paper we describe the main pathways that determine checkpoints in the cell cycle and apoptosis. It is also recalled that in solid tumors radiation-induced cell death occurs most frequently through non-apoptotic mechanisms involving oncosis, and mitotic or delayed cell death. (author)

  20. Mechanisms of DNA repair and radio-induced mutagenesis in higher eukaryotes; Mecanismes de reparation et mutagenese radio-induite chez les eucaryotes superieurs

    Energy Technology Data Exchange (ETDEWEB)

    Averbeck, D. [Centre Universitaire d' Orsay, Institut Curie, Section de Recherche, Lab. Raymond-Latarjet, UMR 2027 CNRS, 91 (France)

    2000-10-01

    Cells of higher eukaryotes possess several very efficient systems for the repair of radiation-induced lesions in DNA. Different strategies have been adopted at the cellular level to remove or even tolerate various types of lesions in order to assure survival and limit the mutagenic consequences. In mammalian cells, the main DNA repair systems comprise direct reversion of damage, excision of damage and exchange mechanisms with intact DNA. Among these, the direct ligation of single strand breaks (SSB) by a DNA ligase and the multi-enzymatic repair systems of mismatch repair, base and nucleotide excision repair as well as the repair of double strand breaks (DSB) by homologous recombination or non homologous end-joining are the most important systems. Most of these processes are error-free except the non homologous end-joining pathway used for the repair of DSB. Moreover, certain lesions can be tolerated by more or less accurately acting polymerases capable of performing trans-lesion DNA syntheses. The DNA repair systems are intimately integrated in the network of cellular regulation. Some of their components are DNA damage inducible. Radiation-induced mutagenesis is largely due to unrepaired DNA damage but also involves error-prone repair processes like the repair of DSB by non-homologous end-joining. Generally, mammalian cells are well prepared to repair radiation-induced lesions. However, some questions remain to be asked about mechanistic details and efficiencies of the systems for removing certain types of radiation-damage and about their order and timing of action. The answers to these questions would be important for radioprotection as well as radiotherapy. (author)

  1. 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.)

  2. 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)

  3. Private specificities can dominate the humoral response to self-antigens in patients with cryptogenic fibrosing alveolitis

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    Lake Richard A

    2001-02-01

    Full Text Available Abstract Background The pathogenetic mechanisms that underlie the interstitial lung disease cryptogenic fibrosing alveolitis (CFA may involve an immunological reaction to unidentified antigens in the lung, resulting in tissue damage. Method In order to identify the range of target autoantigens, we used expression cloning, employing serum from an index patient as the probe against an expressed cDNA library that was derived from a tumour cell line. We screened over 5 × 105 recombinants and obtained sequence information on three antigens that had provoked strong responses with immunoglobulin heavy chain class switching, presumably as a consequence of T-cell recognition. Results All of the antigens were identifiable by comparison with sequence data from the US National Center for Biotechnology Information. Alanyl tRNA synthetase (ATS was picked on six occasions; five of these incidences reflected independent recombination events, indicating that the library was not biased. Antibodies to ATS (anti-PL-12 represent the most common reactivity that defines the antisynthetase syndrome, which is typically expressed as polymyositis, dermatomyositis and interstitial lung disease (ILD. The index patient never showed symptoms other than those associated with alveolitis, even though sera obtained from him over a period of 2 years contained antibodies with the same specificity. Autoantibodies to ATS were never detected in serial bleeds from 11 other patients with CFA, and neither did we detect antibodies to the other two antigens identified from the serum of the index patient. Conclusion The humoral response in patients with CFA can be dominated by autoantibodies with private specificities. This suggests that the antibodies are epiphenomenal and are a secondary feature of tissue damage induced by some other mechanism.

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

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    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.

  5. 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.

  6. 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

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

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    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

  8. Pathogenesis of hepatic septal fibrosis associated with Capillaria hepatica infection of rats Patogenia da fibrose septal hepática associada com a infecção por Capillaria hepatica em ratos

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    Antônio Benigno dos Santos

    2001-12-01

    Full Text Available Septal fibrosis is a common form of hepatic fibrosis, but its etiology and pathogenesis are poorly understood. Rats infected with the helminth Capillaria hepatica constitute a good experimental model of such fibrosis. To investigate the pathogenetic contribution of the several parasitic factors involved, the following procedures were performed in rats: a regarding the role of eggs, these were isolated and injected either into the peritoneal cavity or directly into the liver parenchyma; b for worms alone, 15-day-old infection was treated with mebendazole, killing the parasites before oviposition started; c for both eggs and worms, rats at the 30th day of infection were treated with either mebendazole or ivermectin. Eggs only originated focal fibrosis from cicatricial granulomas, but no septal fibrosis. Worms alone induced a mild degree of perifocal septal fibrosis. Systematized septal fibrosis of the liver, similar to that observed in the infected controls, occurred only in the rats treated with mebendazole or ivermectin, with dead worms and immature eggs in their livers. Thus, future search for fibrogenic factors associated with C. hepatica infection in rats should consider lesions with both eggs and worms.A fibrose septal é uma forma comum de fibrose hepática, mas a sua etiologia e patogenia são ainda desconhecidas. Os ratos infectados com o verme Capillaria hepatica representam um bom modelo experimental para tal fibrose. Para verificar a contribuição de cada fator parasitário na patogenia, os seguintes experimentos foram realizados em ratos: a para testar o papel dos ovos, estes foram isolados e injetados seja na cavidade peritoneal ou no interior do parênquima hepático; b para verificar o papel dos vermes, foram tratados com mebendazol, ratos infectados aos 15 dias da inoculação; c para o papel de ovos e vermes conjuntamente, os ratos com infecção de 30 dias foram tratados com mebendazol ou ivermectina. Os ovos injetados no f

  9. Imatinib atenua a fibrose miocárdica em associação com a inibição da atividade do PDGFRα

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    Li-kun Ma

    2012-12-01

    Full Text Available FUNDAMENTO: O Imatinib é um inibidor do receptor tirosina-quinase que foi confirmada como exercendo um efeito inibidor sobre a atividade do receptor do PDGF, fator de crescimento plaquetário (PDGFRα e PDGFRβ. OBJETIVO: Investigar o efeito protetor do Imatinib na fibrose miocárdica em acetato de deoxicorticosterona (DOCA/ratos com hipertensão induzida por sal. MÉTODOS: Sessenta ratos Sprague-Dawley machos, uninefrectomizados foram distribuídos em três grupos: ratos controles (grupo CON: grupo deoxicorticosterona (grupo DOCA; grupo deoxicorticosterona e Imatinib (grupo DOCA IMA. A Pressão Arterial Sistólica (PAS foi medida quinzenalmente. Foi estudada a porção apical do ventrículo esquerdo. Foram empregados: coloração vermelho sirius, coloração de hematoxilina-eosina, imuno-histoquímica e ensaio de western blot. RESULTADOS: A PAS nos grupos DOCA e IMA+DOCA foi maior que no grupo CON nos dias 14 e 28. Os animais do grupo DOCA apresentaram fibrose intersticial e perivascular grave no dia 28, e as expressões de PI, PIII, tenascina-C e fibronectina foram significativamente maiores que nos grupos DOCA+IMA e CON. Quando comparados com o grupo CON, os grupos DOCA e DOCA+IMA apresentaram resposta inflamatória de tecido miocárdico e infiltração de monócitos/macrófagos de diferentes graus. As expressões proteicas do PDGF-A, PDGF-C e PDGFRα foram significativamente maiores nos grupos DOCA e DOCA+IMA que no grupo CON, mas a expressão proteica do p-PDGFRα no grupo DOCA+IMA foi menor que no DOCA. CONCLUSÃO: O Imatinib pode exercer efeitos inibitórios sobre a fibrose miocárdica em ratos com hipertensão induzida por DOCA/sal, os quais podem ser atribuídos à inibição da atividade do PDGFR-α.

  10. Importance des communications intercellulaires en radiopathologie expérimentale : le syndrôme inflammatoire radioinduit

    Science.gov (United States)

    Agay, D.; Clarencon, D.; Multon, E.; Mestries, J.-C.; van der Meeren, A.; Mouthon, M.-A.; Gourmelon, P.

    1998-04-01

    The monolithic image of radiation pathology, the physiopathological mechanisms of which are limited to the conventional concept of specific “target cell" with mitotic and apoptotic cell death, is changing owing to our increasing knowledge about intercellular communications. The cellular radiationbiology is currently enriched by the “humoral" radiationbiology with its concept of “target network" including cells and intercellular messengers. The radiation-induced disorders observed in these networks take shape in the inflammatory reaction, which is largely involved, in the physiopathological development of the acute radiation syndrome. These concepts of a perpetual cascade of cytokines, leading to radiation-induced late effects with no biological latent period, are especially illustrated by the radiation-induced pneumonitis and pulmonary fibrosis. L'image monolithique de la radiopathologie dont les mécanismes physiopathologiques sont réduits aux concepts classiques de “cellule cible" spécifique avec mort cellulaire mitotique et apoptotique, se modifie grâce à l'accroissement de nos connaissances sur les communications intercellulaires. La radiobiologie cellulaire s'enrichit aujourd'hui de la radiobiologie “humorale" avec son concept de “réseau cible" tant au niveau des cellules que des messagers intercellulaires. Les désordres radio-induits observés au niveau de ces réseaux se concrétisent dans la réaction inflammatoire qui est fortement impliquée dans l'évolution physiopathologique du syndrome aigu d'irradiation. Ces concepts d'une cascade perpétuelle de cytokines, sans période de latence biologique et qui amènent aux effets tardifs radio-induits, sont particulièrement illustrés dans la pneumonie et la fibrose pulmonaire radio-induites.

  11. Primary cicatricial alopecia: Lymphocytic primary cicatricial alopecias, including chronic cutaneous lupus erythematosus, lichen planopilaris, frontal fibrosing alopecia, and Graham-Little syndrome.

    Science.gov (United States)

    Bolduc, Chantal; Sperling, Leonard C; Shapiro, Jerry

    2016-12-01

    Both primary and secondary forms of cicatricial alopecia have been described. The hair follicles are the specific target of inflammation in primary cicatricial alopecias. Hair follicles are destroyed randomly with surrounding structures in secondary cicatricial alopecia. This 2-part continuing medical education article will review primary cicatricial alopecias according to the working classification suggested by the North American Hair Research Society. In this classification, the different entities are classified into 3 different groups according to their prominent inflammatory infiltrate (ie, lymphocytic, neutrophilic, and mixed). Part I discusses the following lymphocytic primary cicatricial alopecias: chronic cutaneous lupus erythematosus, lichen planopilaris, frontal fibrosing alopecia, and Graham-Little syndrome. Copyright © 2015 American Academy of Dermatology, Inc. Published by Elsevier Inc. All rights reserved.

  12. A Importância das Associações de Fibrose Cística na Vida dos Pacientes e Familiares

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    Cleci Furian Müller

    2011-07-01

    Full Text Available Na organização da sociedade moderna as associações filantrópicas tornaram-se importantes no cenário mundial.No Brasil após a fundação da ABRAM várias associações regionais se estruturaram para defender os pacientes com Fibrose Cística.No RGS, duas entidades a AGAM   com 237 pacientes e a AMUCORS com 103 pacientes, trabalham para garantir o fortalecimento das equipes, o conhecimento e divulgação da patologia para a  sociedade e o direito ao tratamento correto para os pacientes.

  13. 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.

  14. 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.

  15. Hepatotrophic factors reduce hepatic fibrosis in rats Fatores hepatotróficos reduzem a fibrose hepática em ratos

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    Bruno Cogliati

    2010-03-01

    Full Text Available CONTEXT: Hepatic fibrosis occurs in response to several aggressive agents and is a predisposing factor in cirrhosis. Hepatotrophic factors were shown to stimulate liver growth and to restore the histological architecture of the liver. They also cause an improvement in liver function and accelerate the reversion of fibrosis before it progresses to cirrhosis. OBJECTIVE: To test the effects of hepatic fibrosis solution composed by amino acids, vitamins, glucose, insulin, glucagon and triiodothyronine on hepatic fibrosis in rats. METHODS: Fibrosis was induced in rats by gastric administration of dimethylnitrosamine (10 mg/kg for 5 weeks. After liver biopsy, the rats received either hepatotrophic factors solution (40 mg/kg/day or saline solution for 10 days by intraperitoneal injection. Blood samples and liver fragments were collected for hepatic function analysis, standard histopathology evaluation, and morphometric collagen quantification. RESULTS: Rats in the hepatotrophic factors group showed a decrease of the histopathological components of fibrosis and an increase of their hepatic mass (12.2%. There was no development of neoplasic lesions in both groups. Compared with the saline group, the hepatotrophic factors group also had a decrease of blood levels of hepatic-lesion markers (AST, ALT and a decrease of collagen content in the portal spaces (31.6% and perisinusoidal spaces (42.3%, as well as around the hepatic terminal vein (57.7%. Thus, hepatotrophic factors administration in the portal blood promoted a regenerative hepatic response, with an overall reduction of the volumetric density of collagen, improved hepatic function, and a general improvement in the histopathological aspects of fibrosis. CONCLUSION: Taken together, these results suggest the potential therapeutic use of this hepatotrophic factors solution to treat chronic liver diseases.CONTEXTO: A fibrose hepática ocorre em resposta a diversos agentes agressores e é um fator

  16. Avaliação evolutiva da espirometria na fibrose cística Spirometry evolution assessment of cystic fibrosis

    Directory of Open Access Journals (Sweden)

    ELENARA DA FONSECA ANDRADE

    2001-05-01

    Full Text Available Objetivo do estudo: Avaliar o padrão da evolução dos fluxos e volumes pulmonares dinâmicos dos pacientes com fibrose cística (FC, assim como analisar a relação entre a gravidade do comprometimento ventilatório com a idade, sexo, genótipo e colonização pulmonar. Material e método: Na Unidade de Pneumologia Infantil e Serviço de Pneumologia do Hospital de Clínicas de Porto Alegre, foram avaliadas 243 espirometrias de 52 pacientes portadores de FC maiores de quatro anos realizadas no período de 1987 a 1999. Do prontuário médico foram extraídas informações sobre idade ao diagnóstico, pesquisa genética, colonização brônquica e os valores absolutos e percentuais para fluxos e volumes da melhor espirometria de cada ano. Resultados: A idade dos pacientes variou de quatro a 26 anos (média = 13,04 ± 4,82. Dos 52 pacientes, 49 (94% tiveram pelo menos uma cultura de escarro com P. aeruginosa, 45 (86% com S. aureus e 13 (25% com B. cepacia. Na avaliação inicial dos quatro aos seis anos (n = 40, os valores médios da CVF e do VEF1 foram de 114,24% e 112,25%, respectivamente. A média da CVF manteve-se acima do normal até os 18 anos, quando apresentou queda súbita para 67,2% (p = 0,0002. A média do VEF1 atingiu valores abaixo de 80% aos dez anos, estando em 50% aos 18 anos (p Objectives: To evaluate the evolution pattern of dynamic pulmonary flow and volume in cystic fibrosis patients and analyze the relation between the severity of ventilatory attacks and age, gender, genotype and pulmonary colonization. Methods: At the Pediatric Pulmonary Unit and Pulmonary Service of Hospital de Clínicas of Porto Alegre, 243 spirometries performed between 1987 and 1999 in 52 cystic fibrosis patients older than four years of age were reviewed. From the patients' medical records the following informations were extracted: age at diagnosis, genetic data, bronchial colonization and absolute as well as percent values of the flows and volumes of

  17. Pollution des eaux superficielles et des nappes en milieu urbain ...

    African Journals Online (AJOL)

    ) suite aux apports générés par les activités industrielles et domestiques. Les eaux de surface sont polluées (carbonique organique total, 13,0 mg/l ; turbidité 168 NTU). Une source potentielle de pollution au nickel et en matière organique est ...

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

    Directory of Open Access Journals (Sweden)

    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

  19. 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

  20. 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

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    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

  1. 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

  2. Patogenesis of pipe-stem fibrosis of the liver (experimental observation on murine Schistosomiasis Patogenia da fibrose "pipe-stem" do fígado (observações experimentais na esquistossomose murina

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    Zilton A. Andrade

    1987-09-01

    Full Text Available Mice infected with 30 cercariae of Schistosoma mansoni developed portal and septal fibrosis due to the massive and concentrated deposition of eggs in the periportal areas which occurred following the 16th week after infection. The lesion resembled pipe-stem fibrosis seen in human hepatosplenic schistosomiasis in the following characters: portal fibrosis interconnecting portal spaces as well as portal spaces and central canals; portal inflammation; periovular granulomas; vascular obstruction and telangiectasia. The liver parenchyma maintained its normal architecture. Vascular injection techniques with Indian ink and vinylite revealed that the portal system developed numerous dilated collateral venules coming from the large and medium-sized portal branches, about 10 weeks after schistosome infection. The lodging of schistosome eggs into these collaterals resulted in granulomatous inflammation and fibrosis along all the portal tracts, thus forming the pipe-stem lesion. Although not readily demonstrable grossly, the pipe-stem fibrosis of murine schistosomiasis has many similarities with the human lesion and can be considered to have the same basic pathogenesis.Camundongos infectados com 30 cercárias do Schistosoma mansoni desenvolveram fibrose porta em virtude de um depósito progressivo e concentrado de ovos na região periportal, o que aconteceu a partir da 16ª semana da infecção. Esta fibrose certas características da chamada fibrose "pipe-stem" do homem vista na forma hepatoesplênica da esquistossomose, tais como obstrução das radiculas porta, telangiectasia, conexão fibrosa entre espaços porta e entre estes e veias centrais, além de certo grau de fibrose septal, presença dos granulomas em várias fases evolutivas e reação inflamatória crônica difusa, enquanto o parênquima hepático mantinha a sua estrutura lobular normal. As técnicas de injeção vascular com tinta da China e com vinilite feitas no sistema porta permitiram a

  3. Níveis séricos de globulinas e a intensidade da fibrose hepática em pacientes com esquistossomose mansônica Serum globulin levels and intensity of hepatic fibrosis in patients with mansonic schistosomiasis

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    Henrique S. T. Correia

    2009-09-01

    Full Text Available CONTEXTO: Tem sido descrita correlação entre os níveis séricos de globulinas e o grau de fibrose hepática nas hepatites crônicas, mas não se encontram relatos na esquistossomose mansônica. OBJETIVO: Avaliar os níveis séricos de globulinas e de IgG, e a intensidade da fibrose periportal mensurada pela ultrassonografia em pacientes com esquistossomose mansônica. MÉTODOS: Entre novembro de 2006 e fevereiro de 2007, foram estudados 41 pacientes que preencheram ficha clínica e realizaram dosagens de IgG por imunoturbidimetria e de globulinas indiretamente pelo método do biureto. A ultrassonografia foi realizada por um único pesquisador, seguindo os protocolos do Cairo e de Niamey. RESULTADOS: A média de idade foi 41 anos, sendo 25 pacientes (61% do sexo feminino. Dez dos 41 pacientes (24% apresentaram elevação dos níveis séricos de globulinas e 21 (51% dos de IgG. Conforme a classificação do Cairo, 21 pacientes apresentaram grau I de fibrose, 18 grau II e 2 grau III, e pela classificação de Niamey 8 apresentavam padrão C, 20 D e 13 E. Aqueles com graus II ou III de fibrose tiveram maiores níveis de IgG do que os de grau I (P = 0,047, assim como aqueles que apresentaram padrões D e E em relação ao C (P = 0,011. Não houve associação entre os níveis de globulinas e o grau ou padrão de fibrose. CONCLUSÃO: Em pacientes com esquistossomose mansônica, observou-se elevação dos níveis séricos de IgG de acordo com a progressão do grau e do padrão de fibrose periportal, mas o mesmo não se observou com os níveis de globulinas.BACKGROUND: A correlation between the levels of serum globulins and the hepatic fibrosis degree in chronic hepatitis was described, but reports in schistosomiasis mansoni have not been found. OBJECTIVE: To evaluate the serum globulins and IgG levels, and periportal fibrosis intensity measured by ultrasound in patients with schistosomiasis mansoni. METHODS: Between November, 2006 and February 2007

  4. How useful is GLUT-1 in differentiating mesothelial hyperplasia and fibrosing pleuritis from epithelioid and sarcomatoid mesotheliomas? An international collaborative study.

    Science.gov (United States)

    Husain, Aliya N; Mirza, M Kamran; Gibbs, Allen; Hiroshima, Kenzo; Chi, Yiqing; Boumendjel, Redouane; Stang, Nolwenn; Krausz, Thomas; Galateau-Salle, Francoise

    2014-03-01

    Mesothelial hyperplasia (MH) and fibrosing pleuritis (FP) can be difficult to distinguish from epithelioid (MM-E) and sarcomatoid (MM-S) malignant pleural mesotheliomas. GLUT-1 has shown variable results regarding its sensitivity and specificity when used to evaluate mesothelial proliferations. We evaluated the utility of GLUT-1 immunostaining in differentiating MH and FP from MM-E and MM-S. In this retrospective study, diagnostically well-characterized cases (MH=31, FP=29, MM-E=41, MM-S=29) were collected and manually stained for GLUT-1. All slides were visually scored by 2 pathologists; using the following system: 0%, 1+ 1-25%, 2+ 26-50% and 3+ >51% cells staining. All benign cases (n=60) were negative for GLUT-1 while 45 of 78 (58%) MM [21 of 41 (50%) MM-E, 21 of 29 (72%) MM-S and 3 of 3 biphasic mesothelioma (100%)] had 1+ to 3+ staining. Of the MM-E, 10 had 1+, and 11 had 2+ staining; of the MM-S 3 had 1+, 15 had 2+ and 3 had 3+ staining. Both sarcomatoid and epithelioid components of the 3 biphasic mesotheliomas revealed 1+ staining. All 5 desmoplastic MM were negative. Positive staining with GLUT-1 is helpful since it is present in half of MM-E and three-quarter of MM-S. Although all reactive mesothelial lesions were negative, the absence of immunoreactivity does not exclude the diagnosis of MM. As with all IHC stains used for diagnostic purposes, GLUT-1 has to be a part of a panel, and the results interpreted in the context of clinical, radiological and histological findings. Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.

  5. 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

  6. 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

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

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    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

  8. Role of partial hepatectomy on Capillaria hepatica-induced hepatic fibrosis in rats Papel da hepatectomia parcial sobre a fibrose septal do fígado induzida pela Capillaria hepatica em ratos

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    Carolina Cincurá Silva Santos

    2007-10-01

    Full Text Available It is known that hepatic fibrosis may regress following partial hepatectomy, since the hepatic parenchyma regenerates very rapidly, but not the excess of fibrous tissue. The present study evaluated this hypothesis by observing the behavior of systematized septal fibrosis induced by either 30 or 90-day-old Capillaria hepatica infection, in rats subjected to partial hepatectomy. The results revealed that the morphology of the fibrosis was unaffected, but its relative quantity within the microscope field appeared significantly decreased, as a consequence of the increased liver tissue mass following regeneration.Sabe-se que a fibrose hepática pode sofrer uma redução em seqüência uma hepatectomia parcial, uma vez que o parênquima hepático se regenera muito rápido, mas não o excesso de tecido fibroso. O presente trabalho avalia esta hipótese ao observar como se comporta a fibrose septal sistematizada induzida pela Capillaria hepática no rato, após infecção de 30 ou 90 dias de duração, em animais submetidos à hepatectomia parcial. Os resultados revelaram que a fibrose em si mesma não foi afetada na sua morfologia, mas a sua quantidade relativa apareceu diminuída significativamente no campo microscópico como conseqüência do aumento da massa de tecido hepático pós-regeneração.

  9. Perdas econômicas pela condenação em matadouro frigorífico de fígados de bovino por fibrose causada por ingestão de Braquiaria spp

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    Tatiane C. Faccin

    2015-06-01

    Full Text Available Resumo: A ingestão crônica de braquiária induz lesões hepáticas em bovinos caracterizadas por fibrose, atrofia do lobo esquerdo, hipertrofia compensatória do lobo direito e proliferação de ductos biliares. Tipicamente, essas lesões são associadas com agregados de macrófagos espumosos no parênquima hepático. Nesse trabalho foram estudados fígados com essas lesões num abatedouro frigorífico do Brasil Central e as perdas econômicas causadas pela condenação de tais fígados afetados foram estimadas. Durante o período estudado, 488.476 bovinos foram abatidos nesse matadouro frigorífico, dos quais 5.295 fígados foram condenados devido à fibrose, e 192 com lesão hepática foram estudados. Cálculos econômicos permitiram inferir que essas condenações representaram uma perda de R$ 108.817,60. Conclui-se que a condenação de fígados em razão de fibrose induzida pela ingestão de braquiária causa uma perda significativa para a indústria de carne e produtos bovinos devido à condenação de 23,6 toneladas de fígado em um ano em apenas um frigorífico, com perdas estimadas acima de R$ 100.000,00.

  10. Simultaneous bilateral spontaneous pneumothorax in an adult patient with cystic fibrosis Pneumotórax espontâneo simultâneo bilateral em um paciente adulto com fibrose cística

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    Kamlesh Mohan

    2009-02-01

    Full Text Available Pneumothorax is a common complication in cystic fibrosis and is associated with worsening of lung function. However, bilateral simultaneous pneumothorax in cystic fibrosis is a rare condition. We describe the case of a 17-year-old female with cystic fibrosis who presented with spontaneous pneumothorax. Clinically, she presented right-sided chest pain and progressive breathlessness. The pneumothorax failed to resolve after the initial treatment (chest drainage. However, the patient was later successfully treated with additional chest drainage and talc pleurodesis. We also discuss the etiology and management of pneumothorax in patients with cystic fibrosis, since pneumothorax is associated with increased morbidity and mortality among such patients.O pneumotórax é uma complicação comum na fibrose cística e está associado com a piora da função pulmonar. Entretanto, o pneumotórax simultâneo bilateral na fibrose cística é um achado raro. Nesse artigo é descrito o caso de uma paciente de 17 anos com fibrose cística que cursou com pneumotórax espontâneo simultâneo bilateral. Clinicamente ela apresentou dor torácica à direita e dificuldade respiratória progressiva. Embora o pneumotórax não tenha respondido bem ao tratamento inicial (drenagem torácica, ela foi posteriormente tratada com drenagem e pleurodese com talco, com sucesso. São discutidos também a etiologia e a conduta nesta condição, que está associada com o aumento da morbidade e mortalidade.

  11. Fibrosis in tubularized skin flaps in rats, using silicon catheters with two different degrees of flexibility: experimental model Fibrose em retalhos tubulizados de pele de ratos usando cateteres de diferentes flexibilidades como molde: modelo experimental

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    Antonio Henrique Rodrigues dos Passos

    2008-06-01

    Full Text Available PURPOSE: Microscopically evaluate the intensity of fibrosis in tubularized skin flaps on the back of Wistar rats, using silicon molds with different degrees of flexibility. METHODS: Twenty rats were submitted to three tubularized skin flaps on their backs. In two tubular flaps, we placed, as a mold, silicon catheters with different degrees of flexibility and removed them on the seventh day after the surgery. They were divided into two groups and euthanized, on the seventh and twenty-first days respectively after the surgery for the collection of the pieces, coloration with Masson tricromic, quantification of the area of each sample and comparison among the groups. RESULTS: Fibrosis was less intense on the tubular flaps where a catheter was not used as a mold. No significant difference was verified among the pieces with the silicon catheters, but there was a tendency of less fibrosis on the tubules with the most flexible catheter. CONCLUSION: There was no significant difference among the two catheter types. Fibrosis was less intense in the flaps where the mold was not used.OBJETIVO: Avaliar microscopicamente a intensidade da fibrose em retalhos tubulares de pele do dorso de ratos Wistar em uso de moldes de silicone de diferentes flexibilidades. MÉTODOS: Vinte animais foram submetidos à confecção de três retalhos tubulizados de pele na região dorsal. Em dois túbulos foram colocados, como molde, cateteres de silicone com flexibilidades diferentes e retirados no sétimo dia após a cirurgia. Foram divididos em dois grupos e sacrificados, respectivamente, no sétimo e vigésimo primeiro dia após a cirurgia para a coleta das peças, coloração pelo tricrômico de Masson, quantificação da área de cada amostra e comparação entre os grupos. RESULTADOS: A fibrose foi menos intensa nos retalhos tubulares em que não se usou cateter como molde. Não se verificou diferença significativa entre os retalhos com os cateteres de silicone, mas sim

  12. Efeito da ressecção do íleo terminal na fibrose hepática secundária à ligadura do ducto hepático comum em ratas

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    Evandro Luis de Oliveira Costa

    Full Text Available OBJETIVO: A evolução para fibrose hepática e, posteriormente, para cirrose são fatos bem estabelecidos na colestase extra-hepática prolongada. A despeito dos avanços nos métodos diagnósticos e terapêuticos, essas complicações continuam de difícil solução, principalmente, quando não é possível reverter a causa da colestase. Neste trabalho, procurou-se verificar, em modelo experimental de colestase pela ligadura do ducto hepático comum, se a exclusão do íleo terminal reduziria o desenvolvimento de fibrose hepática. Não houve abordagem direta da causa da colestase, mas atuou-se nos mecanismos de secreção e regulação do fluxo biliar êntero-hepático. MÉTODO: Foram utilizadas trinta e cinco ratas Wistar, distribuídas em três grupos: Grupo 1, apenas laparotomia e laparorrafia; Grupo 2, ligadura do ducto hepático comum; Grupo 3, ligadura do ducto hepático comum associada a ressecção do íleo terminal, com reconstrução do trânsito intestinal, por meio de anastomose íleo-cólon ascendente. Após trinta dias, os animais foram mortos e o fígado de cada rata foi retirado, para a análise histológica. RESULTADOS: Os resultados foram submetidos a análise estatística pelo teste de Kuskal-Wallis, com nível de significância de 95 % (p < 0,05. Verificou-se que houve fibrose hepática nos grupos 2 e 3, porém sem cirrose. O Grupo 3 apresentou fibrose menos acentuada que o Grupo 2. CONCLUSÕES: Conclui-se que a ressecção do íleo terminal associa-se a menor alteração histológica, no fígado de ratas, decorrente de colestase obstrutiva.

  13. Bronchial artery embolization for therapy of pulmonary bleeding in patients with cystic fibrosis; Bronchialarterienembolisation bei rezidivierenden oder akuten pulmonalen Blutungen von Patienten mit zystischer Fibrose

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    Thalhammer, A.; Jacobi, V.; Balzer, J.; Straub, R.; Vogl, T.J. [Frankfurt Univ. (Germany). Inst. fuer Diagnostische und Interventionelle Radiologie

    2002-05-01

    Introduction: Acute pulmonary emergencies in patient with cystic fibrosis (CF) can be found in cases of pneumothorax as well as hemoptysis. If the bleeding cannot be stopped by conservative methods, an embolization of the bronchial arteries should be done. Materials and Method: 11 patients were embolized using a combination of PVA particles and microcoils. Results: From January 1996 to June 2001 17 bronchial arteries in 11 patients were embolized. 7 patients suffered from chronical hemoptysis, 4 patients had an acute hemoptysis. In 4 patients both sides were embolized, in 3 patients only one side. The remaining 4 patients needed a second intervention, embolizing the other side. The primary embolizated bronchial artery was still closed in all 4 patients. In 1 patient the selective catheterization of a bronchial artery was not successful, thus the embolization could not be carried out. 1 patient died 5 days after the intervention due to a fulminant pneumonia (Pseudomonas aeruginosa) without recurrent bleeding. In two patients atypical branches from intercostal arteries feeding the bronchial arteries were detected and successfully embolized. All patients profited from the therapy, as bleeding could be stopped or at least be reduced. 3 patients suffered from back pain during or after intervention. There were no severe complications like neurological deficiencies or necroses. (orig.) [German] Einleitung: Akute pulmonale Notfaelle bei Patienten mit zystischer fibrose (CF) sind neben auftretenden Pneumothoraces, Haemoptysen oder Haemoptoe. Sind die Blutungen unter konservativen Massnahmen nicht zu beherrschen, steht als radiologische Intervention die Bronchialarterienembolisation zur Verfuegung. Material und Methodik: Bei 11 Patienten wurde eine Embolisation der Bronchialarterien mit PVA-Partikeln und Mikrospiralen durchgefuehrt. Ergebnisse: Von Januar 1996 bis Juni 2001 wurden bei 11 Patienten 17 Bronchialarterien embolisiert. 7 Patienten hatten chronisch rezidivierende

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

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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 < 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.

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

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    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

  16. Prevalência da mutação ΔF508 no gene cystic fibrosis transmembrane conductance regulator em pacientes com fibrose cística em um centro de referência no Brasil

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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 < 0,001. A população analisada está fora do equilíbrio. Os valores esperados são, para os respectivos genótipos -/-, ΔF508/- e ΔF508/ΔF508: 32,22% (60,48 pacientes, 47,93% (80,04 pacientes e 15,86% (26,48 pacientes. CONCLUSÕES: Na população analisada, a mutação ΔF508 se mostrou menos prevalente em relação ao alelo sem a mutação. A frequência encontrada neste estudo foi semelhante à de outras regiões do Brasil e do mundo, principalmente devido à origem predominantemente caucasoide da população incluída no estudo.

  17. 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.

  18. 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

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    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)

  19. 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)

  20. 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

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    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)

  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. Radiation-induced sarcoma following radiotherapy for breast cancer: report of eight cases and review of the literature; Sarcomes radio-induits apres cancer du sein. A propos de huit cas et revue de la litterature

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    Kirova, Y.M.; Feuilhade, F.; Calitchi, E.; Otmezguine, Y.; Belembaogo, E.; Le Bourgeois, J.P. [Hopital Henri-Mondor, 94 - Creteil (France)

    1998-07-01

    Retrospective analysis of eight new cases of radiation-induced sarcomas following radiotherapy for breast carcinoma and literature review. Eleven patients presenting with radiation-induced sarcoma after radiotherapy for breast cancer have been treated between 1983 and 1997 at Henri Mondor University Hospital (France). Eight of these patients respected the criteria established by Cahan et al. The others had Stewart-Treves Syndrome and were thus excluded from the analysis. Only one of the eight patients had received chemotherapy. All of the patients at the time of diagnosis of radiation-induced sarcoma were free of breast cancer recurrence. Radiation-induced sarcoma appeared with a latency period of 5 to 18 years (mean: 10.3 years). Patients` ages ranged from 39 to 88 years (mean: 57.6 years) at the time of diagnosis of sarcoma. Three sarcomas occurred in the treated breast, two in the chest wall, one in the pre-clavicular area and two in the axillary region. There were two angiosarcomas, three fibrosarcomas, one osteosarcoma, one malignant fibrous histiocytoma (MFH), and one undifferentiated sarcoma. All patients have received treatment for their sarcoma: all of them underwent surgery, one patient combined radiotherapy and chemotherapy, and three patients chemotherapy. Two patients were alive and free from disease. Six patients died (5-34 months); all six had local and/or metastatic recurrence. Radiotherapy can induce malignancies after a latent period of several years. Radiation-induced sarcomas are associated with poor overall prognosis. The treatment in most of the cases is late and ineffective, therefore careful follow-up is needed. There are still many uncertainties and questions about radiation-induced sarcomas. (authors)

  3. Avaliação endoscópica nasal de crianças e adolescentes com fibrose cística Nasal endoscopic evaluation of children and adolescents with cystic fibrosis

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    Letícia Paiva Franco

    2009-12-01

    Full Text Available As principais manifestações otorrinolaringológicas da Fibrose Cística são a rinossinusite crônica e a polipose nasossinusal, com diferentes apresentações clínicas. OBJETIVO: Caracterizar, do ponto de vista nasossinusal, as crianças e adolescentes com fibrose cística por meio de um questionário, do exame clínico e da endoscopia nasal. FORMA DE ESTUDO: Clínico descritivo transversal. MATERIAL E MÉTODO: Avaliação de 100 crianças e adolescentes com fibrose cística por meio de um questionário específico, exame físico otorrinolaringológico, endoscopia nasal e estadiamento endoscópico dos pólipos nasais. RESULTADOS: Os sintomas mais frequentes foram: tosse (45%, respiração oral (44%, distúrbios do sono (42% e obstrução nasal (37%. Vinte e oito pacientes (28% apresentaram secreção mucopurulenta nasal e 41% apresentaram abaulamento medial da parede lateral do nariz. Os pólipos nasais foram identificados em apenas 14% dos casos, nenhum deles era obliterante. CONCLUSÃO: O questionário, o exame clínico e especialmente a endoscopia nasal permitiram uma avaliação detalhada das características nasais das crianças e adolescentes com fibrose cística. Alguns achados foram discordantes da literatura, principalmente a baixa prevalência encontrada de pólipos nasais, e parecem estar relacionados com características próprias da população estudada. A melhor caracterização desse grupo de pacientes, do ponto de vista otorrinolaringológico, contribui para uma adequada abordagem multidisciplinar.The main otorhinolaryngological manifestations of CF are chronic rhinosinusitis and nasal polyposis, with different clinical presentations. AIM: To characterize children and adolescents with cystic fibrosis through a questionnaire, an ENT clinical examination and nasal endoscopy. STUDY DESIGN: Cross-sectional clinical descriptive. MATERIAL AND METHOD: Assessment of 100 children and adolescents with cystic fibrosis through a specific

  4. Validação lingüística dos questionários de qualidade de vida em fibrose cística Linguistic validation of cystic fibrosis quality of life questionnaires

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    Tatiana Rozov

    2006-04-01

    Full Text Available OBJETIVO: O propósito deste estudo foi validar em português as quatro versões de questionários de qualidade de vida em fibrose cística, desenvolvidos para pacientes com fibrose cística de 6 a 11 anos, de 12 a 13 e mais de 14 anos, e para os pais de pacientes de 6 a 13 anos. MATERIAL E MÉTODOS: A validação das quatro versões de questionários de qualidade de vida em fibrose cística (de 35 e 50 questões, abrangendo nove domínios constou de: versão inglês-português, aplicação-piloto, tradução retrógrada e aprovação da autora da versão inglesa. As quatro versões foram aplicadas a 90 pacientes estáveis (30 de cada grupo etário e aos pais de doentes de 6-13 anos (n = 60, em duas entrevistas, com intervalo de 13-17 dias. Foi avaliada a reprodutibilidade pelo coeficiente de correlação intraclasse (CCI. O estudo foi aprovado pela comissão de ética em pesquisa da instituição. RESULTADOS: A reprodutibilidade foi boa (CCI = 0,62 a 0,99 para as quatro versões, em todos os domínios, exceto o digestivo (CCI = 0,59 e CCI = 0,47 para os grupos etários de 6 a 11 e 12 a 13 anos, respectivamente, e domínio papel social (CCI = -0,19 para o grupo acima de 14 anos. CONCLUSÃO: A tradução e a adaptação à língua e à cultura brasileiras das quatro versões de questionários de qualidade de vida em fibrose cística mostraram-se de fácil entendimento e boa reprodutibilidade.OBJECTIVE: The purpose of this study was to validate the Portuguese translations of four cystic fibrosis quality of life questionnaires (CFQ. The first three were developed for patients with cystic fibrosis aged from 6 to 11 years, from 12 to 13 years and 14 years or more, while the fourth was developed for the parents of patients aged 6 to 13 years. MATERIAL AND METHODS: The four CFQ translations contained from 35 to 50 questions covering nine domains and were validated as follows: translation from English to Portuguese, pilot application, back translation

  5. 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

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    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

  6. Diagnóstico clínico e laboratorial da fibrose cística : métodos clássicos e novas perspectivas = Clinical and laboratorial diagnosis of cystic fibrosis: classical methods and new perspectives

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    Maria de Fátima Corrêa Pimenta Servidoni

    2014-01-01

    Resumo: A Fibrose Cística (FC) é uma doença genética autossômica recessiva, comum em caucasianos. Tem incidência de 1: 2.500 a 1: 6.000 nascidos vivos e 1: 25 em portadores sãos na Europa e EUA e no Brasil a incidência estimada é de 1:10.000 nascidos vivos. É causada pela presença de dois genes CFTR (do inglês Cystic Fibrosis Transmembrane Conductance Regulator) mutados, que codificam uma proteína também denominada CFTR. A CFTR é o principal canal de Cloro (Cl-), é expressa na membrana apical...

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

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    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

  8. Colonização microbiana precoce de pacientes identificados por triagem neonatal para fibrose cística, com ênfase em Staphylococcus aureus Early microbial colonization of cystic fibrosis patients identified by neonatal screening, with emphasis on Staphylococcus aureus

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    Helena A. P. H. M. Souza

    2006-10-01

    Full Text Available OBJETIVOS: Avaliar prospectivamente a colonização bacteriana de pacientes com fibrose cística identificados por triagem neonatal. Avaliar a suscetibilidade a antimicrobianos e caracterizar molecularmente as cepas de Staphylococcus aureus isoladas da orofaringe dos pacientes no período do estudo. MÉTODOS: Foram estudados 25 pacientes com fibrose cística, identificados por tripsina imunorreativa e com diagnóstico confirmado por duas ou mais provas de suor, atendidos regularmente no ambulatório de fibrose cística do Hospital de Clínicas da Universidade Federal do Paraná. Foram coletadas amostras de orofaringe com swab e cultivadas por métodos rotineiros; as colônias bacterianas foram identificadas fenotipicamente e testadas quanto à suscetibilidade a antimicrobianos. Os isolados de S. aureus foram submetidos a tipagem molecular por eletroforese em campo pulsado. RESULTADOS: De um total de 234 amostras de orofaringe, S. aureus foi isolado em maior número (76% dos pacientes, 42% das amostras, seguido de Pseudomonas aeruginosa (36% dos pacientes, 16% das amostras e Haemophilus spp. (76% dos pacientes; 19% das amostras. Dos 19 pacientes colonizados com S. aureus, foram obtidos 73 isolados, 18 oxacilina-resistentes (24,6%, isolados de dois pacientes, com perfis eletroforéticos idênticos ao do clone brasileiro. Os demais isolados oxacilina-sensíveis distribuíram-se entre 18 perfis eletroforéticos distintos. CONCLUSÃO: Observou-se uma maior prevalência de S. aureus, com isolamento mais precoce em relação aos outros patógenos pesquisados. Os isolados multissensíveis distribuíram-se em clones distintos, caracterizando a não transmissibilidade entre as cepas comunitárias. Os S. aureus resistentes a oxacilina isolados apresentaram perfis eletroforéticos idênticos, provavelmente adquiridos no ambiente hospitalar. P. aeruginosa foi pouco freqüente na população estudada.OBJECTIVES: To assess bacterial colonization prospectively

  9. 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

  10. Papel do Enfermeiro na Assistência a Pacientes Pediátricos e Adolescentes com Fibrose Cística no Hospital de Clínicas de Porto Alegre

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    Maria do Carmo da Rocha Laurent

    2011-08-01

    Full Text Available A Fibrose Cística é uma doença genética, sistêmica, manifestando-se principalmente através de alterações no trato respiratório e digestório. O tratamento é diário e rigoroso cujo objetivo é de manutenção da saúde, evitando a exarcebação da doença. Este relato pretende dar visibilidade ao trabalho realizado pelas enfermeiras no Hospital de Clínicas de Porto Alegre no cuidado à criança e adolescente com Fibrose Cística e suas famílias, através de atividades realizadas pelo enfermeiro no ambulatório e internação hospitalar. Entre estas atividades destaca-se o grupo de sala de espera, participação em reuniões multidisciplinares, implementação de rotinas, treinamento da equipe de enfermagem, cuidados na prevenção da infecção cruzada, elaboração da lista de pacientes para a internação  e implementação do tratamento hospitalar. A enfermeira realiza a assistência, visando uma melhor qualidade de vida a estes pacientes e suas famílias, através da implementação de cuidados adequados e de orientações específicas. As intervenções realizadas, pretendem a aceitação da doença, a melhora clínica do paciente, o controle dos sintomas, o conhecimento da doença e do regime terapêutico, o comportamento de adesão ao tratamento e a participação do familiar no cuidado. Acredita-se que a apresentação da dinâmica de trabalho e atividades descritas neste relato, possa contribuir com outros  serviços de enfermagem que atendam esta clientela, na busca das melhores práticas.

  11. Caracterização das Crianças e Adolescentes com Fibrose Cística Atendidos em um Centro de Referência no Sul do Brasil

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    Clarissa Pitrez Abarno

    2011-08-01

    Full Text Available Trata-se de um estudo documental retrospectivo quantitativo, com o objetivo de caracterizar as crianças e adolescentes com Fibrose Cística atendidos no Hospital de Clínicas de Porto Alegre no período de 2007 e 2008. A população deste estudo caracteriza-se por 100% dos pacientes acompanhados pela Equipe da Pneumologia Infantil do Hospital, totalizando 166 sujeitos. A análise de dados utilizada foi através de freqüências absolutas, relativas, valores de média, mediana, desvio padrão e foi utilizado o Teste U de Mann Whitney. Os dados foram coletados no banco de dados eletrônicos e registros da enfermeira responsável pelo atendimento desses pacientes. A mediana de idade dos pacientes é de 10,5 anos; 56% são do sexo masculino. Quase metade da população (48,8% teve o diagnóstico antes do primeiro ano de vida. A bactéria de mais prevalente nessa população foi o Staphylococcus aureus (88,6%., seguida de Pseudomonas aeruginosa mucóide (55,4%, Pseudomonas aeruginosa (39,2%, Complexo Burkholderia cepacia (22,9%, e Staphylococcus aureus resistente à meticilina (20,5%. Os pacientes que necessitaram de hospitalização tiveram mediana de uma internação e apresentaram mediana de 22 dias internados no período de dois anos. Dentre os que compareceram às consultas ambulatoriais, apresentaram uma mediana de sete consultas nesse mesmo período. Foram transferidos para a Equipe de Adultos 15 pacientes. Três foram a óbito. Estudos semelhantes a esse possibilitam o conhecimento das respostas desses pacientes ao tratamento e adequação dos cuidados aos pacientes com Fibrose Cística.

  12. Pico do fluxo expiratório na avaliação da função pulmonar na fibrose cística Peak expiratory flow rate in the management of cystic fibrosis

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    Paulo A.M. Camargos

    2002-02-01

    Full Text Available Objetivo: avaliar o valor do pico de fluxo expiratório, obtido através de medidores portáteis, como método alternativo de acompanhamento da função pulmonar na fibrose cística. Métodos: quarenta e nove pacientes, de 5 a 19 anos, clinicamente estáveis e aptos a realizar a manobra para obtenção do pico do fluxo foram incluídos no estudo. Na mesma visita, pontuou-se o escore de Shwachman-Kulczycki e registrou-se o valor do pico do fluxo expiratório. Analisou-se a correlação entre esses dois parâmetros pela regressão linear, com nível de significância de P = 0,05. Resultados: obteve-se uma correlação discreta, mas estatisticamente significante, entre os valores absolutos e percentuais desse parâmetro funcional com o escore clínico-radiológico (r = 0,31, P = 0,02 e r = 0,30, P = 0,03, respectivamente. Conclusões: conclui-se que a significância estatística observada não corresponde necessariamente a uma relevância clínica e, portanto, a função pulmonar na fibrose cística deve ser avaliada pela espirometria convencional.Objective: to verify the role of peak expiratory flow, measured through portable meters, as an alternative test to assess pulmonary disease in cystic fibrosis. Methods: forty-nine patients aged five to 19 years old in stable health condition and able to perform the peak expiratory flow maneuver were included. In the same visit, Shwachman-Kulczycki score was recorded. Linear regression was used to assess the correlation between the Shwachman-Kulczycki score and the peak expiratory flow rate. A P value of 0.05 was considered to be significant.Results: a slight, but statistically significant correlation between absolute and percent values for this functional parameter and the Shwachman-Kulczycki score (r = 0.31, P = 0.02 and r = 0.30, P = 0.03, respectively was found. Conclusions: although this correlation was statistically significant, these findings are not clinically relevant, i.e., the lung involvement

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

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    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

  14. Síndrome mieloproliferativa transitória associada à trissomia do 21 e fibrose hepática Transient myeloproliferative disorder associated with trisomy 21 and liver fibrosis

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    Anna L. Sant'Anna

    2002-03-01

    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.Neonates with Down's Syndrome (DS occasionally show an excess of blast cells in their peripheral blood and bone marrow. The leukocyte counts are high and just the evolution can discriminate the diagnosis of Transient

  15. 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

  16. 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

  17. 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

  18. Participação das plaquetas no processo de fibrose dos pacientes com esquistossomose mansônica Participation of platelets in the process of fibrosis in patients with mansonic schistosomiasis

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    Sheila Soares

    2007-06-01

    Full Text Available O objetivo deste estudo foi avaliar a ativação plaquetária através da P-selectina e o conteúdo de PDGF-AB e TGFbeta1, nos pacientes com esquistossomose que desenvolveram fibrose (F3, naqueles que não tiveram esta manifestação (F0 e nos controles (C. Os resultados mostraram que a percentagem de P-selectina nas plaquetas sem estímulo de agonistas foi de 10,6% nos F3; 11,1% nos FO, e 6,3% nos C e após a adição de ADP/adrenalina, foi de 44%; 25,3% e 42%, respectivamente. A dosagem do PDGF-AB e TGFbeta1 por plaquetas foi de 11,016ng/dL (F3; 3,172 ng/dL (F0 e 5,01ng/dL (C e, (0,012ng/dL (F3; 5,27ng/dL (F0 e 4,66ng/dL (C, respectivamente. Em relação à P-selectina, concluímos que as plaquetas dos pacientes com esquistossomoses, apesar de estarem pré ativadas, mantiveram-se responsivas aos agonistas. O TFGbeta1 não apresentou diferença entre os três grupos, enquanto o PDGF-AB foi significantemente maior no grupo F3, sugerindo a participação deste no desenvolvimento da fibrose.The aim of this study was to evaluate platelet activation through P-selectin, and PDGF-AB and TGFbeta1 content, in schistosomiasis patients who developed fibrosis (F3 and who did not present this (F0, and in a control group (C. The results showed that the percentage of P-selectin in platelets without agonist stimulation was 10.6% in F3, 11.1% in F0 and 6.3% in C. After the addition of ADP/adrenaline, the percentages were 44%, 25.3% and 42%, respectively. The PDGF-AB and TGFbeta1 contents per platelet were 11,016ng/dl (F3, 3,172ng/dl (F0 and 5.01ng/dl (C and 0,012ng/dl (F3, 5.27ng/dl (F0 and 4.66ng/dl (C, respectively. Concerning the P-selectin, we can conclude that platelets from patients with schistosomiasis continued to be responsive to agonists, despite being pre-activated. There were no differences in TGFbeta1 between the groups, but the PDGF-AB content was significantly higher in F3. This suggests that PDGF-AB may have some participation in the development

  19. Application of expanded inert polytetrafluorethylene membrane in prevention of post laminectomy fibrosis in wistar rats Estudo do uso da membrana de politetrafluoroetileno inerte expandido para a prevenção da fibrose pós laminectomia em ratos wistar

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    Tarcísio Eloy Pessoa Barros Filho

    2003-04-01

    Full Text Available Post laminectomy fibrosis in spine surgery has been responsible for a high rate of failure in a short or long term. Many of this patients develop chronic or recurrent pain after surgery of discal herniation, canal stenosis etc. Although there some doubts about the etiology of this problem, it was known that fibrosis interfere with the normal mobility of roots and spinal cord. This factor is considered as the most important aspect in the genesis of the surgical failures. To avoid postoperative fibrosis, a lot of materials of interposition between posterior spine muscles and dura mater were studied, without good response. This paper studied comparatively post laminectomy fibrosis in Wistar rats with the addition of the following materials to avoid it: control group (without interposition and expanded inert polytetrafluoroethylene membrane (Preclude Spinal Membrane®. It was observed that accurate surgical technique apparently is an important factor to prevent the formation of abundant scar tissue and that the expanded inert polytetrafluoroethylene membrane is an inert material.A fibrose pós laminectomia em cirurgias da coluna vertebral tem sido responsabilizada por um grande percentual das falhas nestas cirurgias, tanto a curto quanto a longo prazo. Muitos dos pacientes desenvolvem sintomas de dor crônica ou recorrente após tratamento cirúrgico de hérnias de disco, estenose de canal, etc. Apesar de ainda existirem dúvidas quanto à fisiopatologia do problema, sabe-se que a fibrose interfere na mobilidade normal das raízes e da medula espinal, fator este responsabilizado como o principal na gênese das falhas cirúrgicas. Para se evitar a fibrose pós-cirúrgica, vários materiais de interposição entre a musculatura posterior da coluna e a dura-máter foram estudados, sem grandes resultados. Neste trabalho foi feito o estudo da membrana de politetrafluoroetileno inerte expandido (Preclude Spinal Membrane® comparando-se a mesma com grupo

  20. Low coronary perfusion pressure is associated with endocardial fibrosis in a rat model of volume overload cardiac hypertrophy A redução da pressão de perfusão coronariana está associada com a fibrose endocárdica no modelo de hipertrofia por sobrecarga de volume em ratos

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    Maria Carolina Guido

    2004-01-01

    Full Text Available Left ventricular hypertrophy following volume overload is regarded as an example of cardiac remodeling without increased fibrosis accumulation. However, infarction is associated with increased fibrosis within the noninfarcted, hypertrophied myocardium, particularly in the subendocardial regions. It is conceivable to suppose that, as also occurs postinfarction, low coronary driving pressure may also interfere with accumulation of myocardial fibrosis following aortocaval fistula. PURPOSE: To investigate the role of acute hemodynamic changes in subsequent deposition of cardiac fibrosis in response to aortocaval fistula. METHOD: Aortocaval fistula were created in 4 groups of Wistar rats that were followed over 4 and 8 weeks: aortocaval fistula 4 and aortocaval fistula 8 (10 rats each and their respective controls (sham-operated controls - Sh, Sh4 and Sh8 (8 rats each. Hemodynamic measurements were performed 1 week after surgery. Hypertrophy and fibrosis were quantified by myocyte diameter and collagen volume fraction at the end of follow up. RESULT: Compared with Sh4 and Sh8, pulse pressure, left ventricular end-diastolic pressure, and +dP/dt were higher in aortocaval fistula 4 and aortocaval fistula 8, but -dP/dt was similar. Coronary driving pressure (mm Hg, used as an estimate of perfusion pressure, was lower in aortocaval fistula 8 (52.6 ± 4.1 than in Sh8 (100.8 ± 1.3, but comparable between aortocaval fistula 4 (50.0 ± 8.9 and Sh4 (84.8 ± 2.3. Myocyte diameter was greater in aortocaval fistula 8, whereas interstitial and subendocardial fibrosis were greater in aortocaval fistula 4 and aortocaval fistula 8. Coronary driving pressure correlated inversely and independently with subendocardial fibrosis (r² = .86, P No remodelamento que se segue às sobrecargas de volume não é descrito o aumento de fibrose miocárdica. Após o infarto, entretanto, há hipertrofia do miocárdio remoto com acúmulo de fibrose, particularmente no subendoc

  1. Qualité hydro chimique des eaux de la nappe superficielle dans la ...

    African Journals Online (AJOL)

    L'interprétation des analyses physico-chimiques montrent une forte conductivité électrique, traduisant une salinité élevée. L'utilisation de la méthode de GOD montre que la nappe phréatique de l'Oued Righ à une vulnérabilité forte à modéré. Les zones urbaines, les chotts et les zones agricoles présentent une vulnérabilité ...

  2. Qualité hydro chimique des eaux de la nappe superficielle dans la ...

    African Journals Online (AJOL)

    forte conductivité électrique, traduisant une salinité élevée. L'utilisation de la méthode de GOD montre que la nappe phréatique de l'Oued Righ à une vulnérabilité forte à modéré. Les zones urbaines, les chotts et les zones agricoles présentent une vulnérabilité forte. Mots clés : nappes aquifères- hydrochimie- vulnérabilité- ...

  3. Diagnóstico de rinossinusite crônica em pacientes com fibrose cística: correlação entre anamnese, nasofibroscopia e tomografia computadorizada Diagnosis of chronic rhinosinusitis in patients with cystic fibrosis: correlation between anamnesis, nasal endoscopy and computed tomography

    Directory of Open Access Journals (Sweden)

    Letícia Boari

    2005-12-01

    Full Text Available O comprometimento nasossinusal é uma das principais manifestações otorrinolaringológicas da fibrose cística. Na literatura, é descrita a alta incidência de rinossinusite crônica nesses pacientes. Apesar de mais de 90% dos casos apresentarem panopacificação dos seios da face em exames de imagem, tais achados são raramente acompanhados de sintomas. Por isso, o profissional tem dificuldade de diagnosticar a doença nasossinusal em pacientes com fibrose cística. Dentre os métodos disponíveis para essa avaliação, o questionário (sintomas, a nasofibroscopia (sinais e a tomografia computadorizada têm grande importância. OBJETIVO: Avaliar o diagnóstico de rinossinusite crônica em pacientes portadores de fibrose cística por meio de questionário (anamnese; nasofibroscopia e tomografia computadorizada de seios da face e comparar os seus achados. FORMA DE ESTUDO: Clínico prospectivo transversal. MATERIAL E MÉTODO: Avaliação de 34 pacientes - maiores de 6 anos de idade e portadores de fibrose cística - por meio de questionário, nasofibroscopia (escore de Lund-Kennedy e tomografia computadorizada de seios da face (escore de Lund-Mackay. RESULTADOS: Observou-se que o diagnóstico de rinossinusite crônica foi positivo em: 20,58% dos casos pelo questionário; 73,52% dos casos pela nasofibroscopia e 93,54% dos casos pela tomografia computadorizada. A diferença entre os resultados foi estatisticamente significante. Verificou-se uma correlação de 55,1% entre as estratificações dos escores de nasofibroscopia e de tomografia computadorizada. CONCLUSÃO: O diagnóstico positivo da rinossinusite crônica foi predominantemente observado pela tomografia computadorizada. O diagnóstico negativo foi predominante na avaliação pelo questionário. Houve diferença estatisticamente significante entre os meios de avaliação. A nasofibroscopia é um excelente recurso que deve ser utilizado na avaliação de rinossinusite crônica em fibrose

  4. C27. Perfil epidemiológico das infecções bacterianas do aparelho respiratório em doentes com fibrose quística

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    Sofia Quintas

    2003-11-01

    Full Text Available 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. : With the aim of characterizing the evolution of the epidemiological profile of respiratory bacterial infections of patients having Cystic Fibrosis (CF, the authors conducted a retrospective analysis about it

  5. 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

  6. 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 fibro...... fibrosis. We believe that a reservoir for a sustained lung infection in these patients might be found in the nasal sinuses, and that the sinuses may act as a reservoir for reinfection after CF-patient lung transplants. Further studies are necessary....

  7. 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

  8. 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

  9. Estudo da freqüência de diabetes mellitus e intolerância à glicose em pacientes com fibrose cística Study of the frequency of diabetes mellitus and glucose intolerance in patients with cystic fibrosis

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    Flávia A.A. de Castro

    2001-08-01

    Full Text Available OBJETIVO: avaliar a freqüência de diabetes mellitus (DM e de intolerância à glicose nos pacientes com fibrose cística seguidos no Serviço de Gastropediatria do HC-FMRP-USP. MÉTODOS: foi realizado estudo analítico transversal de um grupo de 25 pacientes mucoviscidóticos do HC-FMRP-USP. Foram realizados testes orais de tolerância à glicose (OGTT com determinação concomitante da glicemia e da insulinemia. Foram obtidas áreas sob as curvas da glicemia (G e da insulinemia (I e calculada a relação I/G, correlacionando-as com o tempo de doença clinicamente manifesta e função exócrina pancreática. RESULTADOS: cinco pacientes apresentaram alterações: um é diabético e quatro são intolerantes à glicose e/ou hiperinsulinêmicos. Há correlação direta entre a área sob a curva de insulinemia e o tempo de mucoviscidose. Verificou-se também relação inversa estatisticamente significativa entre a área sob a curva de insulinemia e o índice I/G com o número de cápsulas de enzima/kg/dia. CONCLUSÕES: a freqüência de alterações na homeostase glicêmica nos mucoviscidóticos estudados é maior que na população em geral (20% da amostra total e 33% do grupo de pacientes com glicemia e insulinemia no OGTT. Assim, é importante a realização sistemática de testes de tolerância à glicose em mucoviscidóticos, visando à detecção precoce de anormalidades metabólicas para adequado tratamento.ABSTRACT OBJECTIVE: To evaluate the frequency of diabetes mellitus and glucose intolerance in patients with cystic fibrosis treated at the Pediatric Gastroenterology Service of HC-FMRP-USP. METHODS: A cross-sectional analytical study was conducted on a group of 25 patients with mucoviscidosis who were followed up at HC-FMRP-USP. Oral glucose tolerance tests (OGTT were performed, with simultaneous determination of glycemia and insulinemia. Areas under the curve were obtained for glycemia (G and insulinemia (I and the I/G ratio was

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

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    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

  11. 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

  12. 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

  13. Lola tinha uma coisa: construção de um livro educativo para crianças com fibrose cística Lola tenía una cosa: construcción de un libro educativo para niños con fibrosis cística Lola had one thing: building an educational book for children with cystic fibrosis

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    Tainá Maués Pelúcio Pizzignacco

    2012-01-01

    Full Text Available Este trabalho descreve a experiência da construção de um livro de história com o objetivo de auxiliar profissionais de saúde e familiares a contarem para crianças menores de 5 anos sobre seu diagnóstico de Fibrose Cística. O livro traz a história de Lola, uma criança que descobre que tem Fibrose Cística e cria um monstro imaginário como representação da doença. A experiência emergida da prática profissional em um ambulatório multiprofissional de atendimento a esses pacientes, traduz a importância de conciliar teoria e prática, além da interdisciplinaridade para a construção de estratégias inovadoras e criativas no desenvolvimento do cuidado integral.Este trabajo describe la experiencia de la construcción de un libro de historia con el objetivo de auxiliar a profesionales de salud y familiares a contar a los niños menores de 5 años sobre su diagnóstico de Fibrosis Cística. El libro muestra la historia de Lola, un niño que descubre que tiene Fibrosis Cística y crea un monstruo imaginario como representación de la enfermedad. La experiencia emergida de la práctica profesional en un consultorio externo multiprofesional de atención a esos pacientes, traduce la importancia de conciliar la teoría y la práctica, además de la interdisciplinaridad para la construcción de estrategias innovadoras y creativas en el desarrollo del cuidado integral.This work describes the experience of constructing a story book with the objective of helping health professionals and families to share with children less than 5 years about their diagnosis of cystic fibrosis. The book tells the story of Lola, a child who discovers she has cystic fibrosis and creates an imaginary monster as a representation of the disease. The experience emerged out of professional practice in an outpatient multidisciplinary service for these patients, translating the importance of reconciling theory and practice, and interdisciplinarity for the construction of

  14. 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

  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. 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)

  17. 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

  18. 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)

  19. 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)

  20. 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

  1. 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

  2. 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

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    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

  3. Frequency of the deltaF508 mutation in 108 cystic fibrosis patients in São Paulo: comparison with reported Brazilian data Freqüência da mutação deltaF508 em 108 pacientes com fibrose cística de São Paulo: comparação com dados de estudos brasileiros

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    Thelma Suely Okay

    2005-04-01

    Full Text Available PURPOSE: To analyze the frequency of the delta F508 (deltaF508 deletion mutation in 108 unrelated cystic fibrosis patients and compare the results with the previously reported data for Brazilian patients. Cystic fibrosis is the leading cause of genetic disease in Caucasians, and the deltaF508 deletion is the most common mutation associated with the disease. METHOD: The frequency of the deltaF508 mutation was assessed by means of a polymerase chain reaction (PCR followed by detection in 8% silver-stained polyacrylamide gels. RESULTS: Twenty-three of 108 patients (21.3% were homozygous for the deltaF508 deletion, 50 were heterozygous (46.3%, and the remaining 35 (32.4% were non-carriers. In terms of alleles, there were 96 mutated (96/216 or 44.45% and 120 wild-type ones (120/216 or 55.5%. CONCLUSION: The 44.45% of affected alleles that were found is higher than the 33% first described in 1993, but slightly lower than the 48% recently reported. Moreover, our data corroborated the idea that the frequency of the deltaF508 mutation is lower in Brazil in comparison to that found in studies carried out in Europe and North American (circa 70.0%, probably due to increased racial miscegenation. These findings must be taken into account before any genetic screening of the population is proposed in Brazil.OBJETIVO: Analisar a freqüência da mutação delta F508 (deltaF508 em 108 pacientes não aparentados, com fibrose cística e comparou os resultados com os dados de outros estudos brasileiros. A fibrose cística (CF constitui a doença genética mais comum em populações caucasianas, sendo a deltaF508 a mais freqüente dentre as mutações relacionadas à doença. MÉTODO: A freqüência da deltaF508 foi analisada por meio da Reação em Cadeia da Polimerase (PCR seguida de detecção em géis de poliacrilamida a 8%. RESULTADOS: Vinte e três dos 108 pacientes foram homozigotos para a mutação (21,3%, 50 foram heterozigotos (46,3% e os 35 restantes n

  4. 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

    Directory of Open Access Journals (Sweden)

    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.

  5. 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

    Directory of Open Access Journals (Sweden)

    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.

  6. Abdominal ultrasound in the evaluation of fibrosis and portal hypertension in an area of schistosomiasis low endemicity Ultra-sonografia abdominal na avaliação de fibrose e hipertensão portal em área de baixa endemicidade de esquistossomose

    Directory of Open Access Journals (Sweden)

    Maria Cristina Carvalho do Espírito Santo

    2008-04-01

    Full Text Available This study was undertaken in the municipality of Bananal, São Paulo, an endemic area for schistosomiasis with a prevalence under 10% and low parasite load among infected individuals. Our objective was to identify the clinical forms of schistosomiasis among 109 patients in whom the disease had been diagnosed through direct fecal analysis and who had been medicated with oxamniquine at the time of the Plan for the Intensification of Schistosomiasis Control Actions (1998-2000. These patients were submitted to an abdominal ultrasonography and fecal analysis by Kato-Katz method, four years, on average, after the end of the Plan. Five patients, whose abdominal ultrasound images were compatible with either peripheral or central periportal fibrosis and portal hypertension, were identified. None of the 109 patients presented Schistosoma mansoni eggs at fecal analysis. Ultrasonography is a sensitive, noninvasive diagnostic method that allows a better identification of the extent of liver involvement in schistosomiasis cases.Este estudo desenvolveu-se no município de Bananal, São Paulo, uma área endêmica para esquistossomose com prevalência menor que 10% e baixa carga parasitária nos infectados. Teve como objetivo a identificação de formas clínicas da esquistossomose mansoni através do exame ultra-sonográfico, em 109 pacientes diagnosticados parasitologicamente e medicados com oxamniquine, durante a realização do Plano de Intensificação das Ações de Controle da Esquistossomose mansônica (1998-2000. Foram utilizadas a ultra-sonografia abdominal e exames de fezes (Kato-Katz realizados após o término do plano, quatro anos em média. Nesta casuística, foram identificados cinco pacientes com imagens ultra-sonográficas abdominais compatíveis com fibrose periportal periférica ou central e hipertensão portal, além da negatividade de todos os exames parasitológicos nos 109 pacientes. A ultra-sonografia, um método de diagnóstico sens

  7. Percepção de qualidade de vida de pessoas com fibrose cística: um estudo sobre a adequação dos instrumentos de medida Perception of quality of life of persons with cystic fibrosis: a study on the adequacy of rating scales

    Directory of Open Access Journals (Sweden)

    Juliana Bredemeier

    2007-04-01

    Full Text Available INTRODUÇÃO: Avaliações de qualidade de vida (QV na fibrose cística (FC são tão importantes quanto a avaliação da condição clínica do paciente. Porém, há divergências sobre conceitos e teorias de QV. METODOLOGIA: Onze pacientes brasileiros com FC maiores de 18 anos responderam a perguntas sugeridas pela OMS para a exploração conceitual da QV. A pesquisa foi orientada pela abordagem da grounded theory, e os dados sofreram análise qualitativa. RESULTADOS: Apesar da diversidade de domínios valorizados pelos entrevistados estar de acordo com o caráter multidimensional da QV, há domínios ainda não presentes nas escalas. CONCLUSÕES: Nenhuma escala atualmente disponível abrange todos os aspectos valorados pelos pacientes, o que aponta para a importância de questões idiossincráticas e culturais para a QV na FC. O esclarecimento da relação entre saúde e QV ainda depende de pesquisas futuras.INTRODUCTION: Quality of life (QOL assessment in cystic fibrosis is as important as the assessment of the patient's health status. However, there are controversies on QOL concepts and theories. METHOD: Eleven Brazilian patients with cystic fibrosis, 18 years or older, answered questions suggested by the World Health Organization to explore the concept of QOL. The grounded theory approach provided the basis for this study and the data were qualitatively analyzed. RESULTS: Although the diversity of domains valued by the participants is in agreement with the multidimensional nature of QOL, there are some domains missing in the scales. CONCLUSIONS: No currently available scale comprises all aspects ranked as important by the patients. This underscores the importance of idiosyncratic and cultural aspects on QOL in cystic fibrosis. Clarifying the relationship between health and QOL still relies on further research.

  8. Proteinuria in cystic fibrosis: a possible correlation between genotype and renal phenotype Proteinúria na fibrose cística: possível correlação entre genótipo e fenótipo renal

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    Jessica Cemlyn-Jones

    2009-07-01

    Full Text Available OBJECTIVE: To assess proteinuria in patients with cystic fibrosis (CF, and to correlate proteinuria with genotype, CF-related diabetes and disease severity. METHODS: A prospective study was carried out over a six-month period and involving 22 CF patients. After the collection and analysis of 24-h urine samples, the patients were divided into two subgroups: protein excretion 150 mg/day (highproteinuria. Patient charts were reviewed to obtain data on genotype and CF-related diabetes. Disease severity was assessed based on acute exacerbations in the last six months and FEV1 measured during the study period. To assess the correlation between genotype and proteinuria, the two main mutations (ΔF508 and R334W were evaluated. Due to the existence of genotype ΔF508/R334W, two categories were created to enable statistical analysis, ΔF508 being evaluated in category 1 and R334W being evaluated in category 2. RESULTS: The ΔF508 mutation tended to be associated with normal protein excretion: 100% of the low-proteinuria subgroup patients were considered ΔF508 in category 1, compared with 86.7% in category 2. Protein excretion tended to be higher in patients with the R334W mutation: 60.0% of the high-proteinuria subgroup patients were considered R334W in category 1, compared with 80.0% in category 2 (p = 0.009 and p = 0.014, respectively. No significant association was found for any of the other variables. CONCLUSIONS: The results suggest that genotype is associated with renal phenotype, depending on the mechanism by which the genotype alters the function of the cystic fibrosis transmembrane conductance regulator gene.OBJETIVO: Avaliar a proteinúria em pacientes com fibrose cística (FC e correlacioná-la com o genótipo, com a diabetes relacionada à FC e com a gravidade da doença. MÉTODOS: Estudo prospectivo realizado num período de seis meses com 22 pacientes com FC. Efetuada proteinúria de 24 h com a divisão dos pacientes em dois subgrupos

  9. 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

    Directory of Open Access Journals (Sweden)

    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

  10. 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

  11. Noninvasiv vurdering af fibrose ved kronisk viral hepatitis

    DEFF Research Database (Denmark)

    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...... considered as surrogates to the liver biopsy. The noninvasive tests are considered equal in identifying significant fibrosis, but TE is superior in identifying cirrhosis....

  12. Farmacotherapie bij adolescente en volwassen patienten met cystische fibrose

    NARCIS (Netherlands)

    Touw, D.J.; Heijerman, H.G.M.; Bakker, Wim

    1996-01-01

    Cystic fibrosis (CF) is the most common potentially fatal inherited disease in the white population. In patients with CF, abnormal chloride transport across the apical membrane of epithelial cells has been identified, causing inspissated secretions in the airways, pancreas and other organs. Clinical

  13. 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....

  14. 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

    Directory of Open Access Journals (Sweden)

    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

  15. 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

    Directory of Open Access Journals (Sweden)

    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

  16. Crianças hospitalizadas com Fibrose Cística: percepções sobre as múltiplas hospitalizações Niños con Fribrosis Quistica: percepciones sobre las multiples hospitalizaciones Children with Cystic Fibrosis: perceptions about multiple hospital admissions

    Directory of Open Access Journals (Sweden)

    Ruth Irmgard Bärtschi Gabatz

    2007-02-01

    Full Text Available As crianças com fibrose cística (FC geralmente são hospitalizadas inúmeras vezes ao longo de suas vidas. Este estudo objetiva conhecer as percepções destas crianças em relação às múltiplas hospitalizações, bem como identificar o que percebem sobre o tratamento e os cuidados recebidos. Trata-se de um estudo descritivo com abordagem qualitativa. Os sujeitos são sete crianças portadoras de FC, entre 6 e 11 anos. Os dados são coletados em uma unidade pediátrica de um hospital geral, através de uma entrevista focalizada, pouco estruturada, e analisados pelo método da análise compreensiva. Os resultados deste estudo demonstram que as crianças prezam muito sua liberdade, que durante a hospitalização fica restringida, o que pode ser amenizado através da nossa escuta, compreensão e atenção.Los niños con fibrosis quística generalmente son hospitalizados innumerables veces a lo largo de sus vidas. Este estudio objetiva conocer las percepciones de estos niños con relación a las múltiplas hospitalizaciones, así como reconocer lo que se dan cuenta sobre el tratamiento y los cuidados recibidos. Se trata de un estudio descriptivo con abordaje cualitativa. Los sometidos son siete niños portadores de fibrosis quística, entre 6 y 11 anos. Los datos son colectados en una unidad pediátrica de un hospital general, por medio de una entrevista enfocada, poco estructurada y examinados por el método de la análisis comprensiva. Los resultados de este estudio enseñan que los niños estiman mucho su libertad, donde durante la internación se pone restriñida, lo que puede ser amenizado por medio de nuestra escucha, comprensión y atención.Children with cystic fibrosis are usually admitted to the hospital many times through their lives. This study aims to know the perceptions of these children towards the multiple hospital admissions, as well to identify what they realize about the treatment and the care they get. This is a descriptive

  17. 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?

    Directory of Open Access Journals (Sweden)

    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

  18. 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

  19. 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

  20. 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

  1. Relation entre nappes superficielles et aquifère profond dans le bassin de Sfax (Tunisie)Relationship between shallow and deep aquifers in the Sfax basin (Tunisia).

    Science.gov (United States)

    Ahmed Maliki, My; Krimissa, Mohamed; Michelot, Jean-Luc; Zouari, Kamel

    2000-07-01

    The study of the isotopic composition ( 18O and 2H) of groundwater collected in the Sfax basin (Tunisia), helped to understand the behaviour of the different aquifers. It showed that the groundwater in the deep aquifer is old, probably slow moving and recharged under a colder climate than at present. The increasing exploitation of the shallow aquifers probably favoured upward leakage from the deep aquifer. Isotope balance equations allowed us to estimate the contribution of the deep aquifer to the shallow aquifer recharge.

  2. Anévrisme de l'artère fémorale superficielle lié à une infection à ...

    African Journals Online (AJOL)

    Les anévrismes infectieux de l'axe fémoro-poplité sont rares et d'évolution silencieuse. Ils surviennent surtout chez les sujets immunodéprimés. Leur traitement fait appel souvent à l'exclusion chirurgicale associée ou non à un geste de revascularisation. Nous rapportons le cas d'un patient opéré pour un anévrisme de ...

  3. 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

  4. 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

  5. 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. 

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

    Science.gov (United States)

    ... the body, contains a functional region called a peptidase domain. Similar proteins containing such a domain are ... people with POIKTMP result in changes in the peptidase domain, researchers think that the mutations alter the ...

  7. 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.

  8. 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.

  9. Hemolytische anemie en een stollingsstoornis als eerste manifestatie van cystische fibrose bij twee zuigelingen

    NARCIS (Netherlands)

    ter Avest, P. C.; Tytgat, G. A. M.; Westra, M.; Peters, M.; van den Berg, M.

    2005-01-01

    2 infants, a boy aged 8 weeks and a girl aged 5 months, presented with symptoms of fat-soluble vitamin deficiencies. The first infant had frequently voluminous bowel movements, anaemia and was not thriving; he had anaemia due to vitamin-E deficiency. The second infant had multiple haematomas on the

  10. 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.

  11. 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...

  12. 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.)

  13. Análise dos efeitos do método Pilates em pacientes com fibrose cística

    OpenAIRE

    Caroline Buarque Franco

    2011-01-01

    Resumo: Objetivo: A proposta de utilizar o método Pilates teve o objetivo de desenvolver um programa de exercícios, avaliando os efeitos no início e final do acompanhamento, coletando os seguintes dados: tolerância ao esforço físico pelo teste do degrau de 3 minutos (TD3); prova de função pulmonar (PFP); avaliação força muscular respiratória (FMR) pela pressão inspiratória máxima (PImáx) e pressão expiratória máxima (PEmáx) pela manovacuometria; qualidade de vida (QV) pelos questionários de q...

  14. 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.

  15. 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

  16. La fibrose rétropéritonéale: à propos de 12 cas | El Majdoub | Pan ...

    African Journals Online (AJOL)

    échographie qui a montré une obstruction de la voie excrétrice supérieure sans obstacle visible chez tous les malades, et confirmé par la TDM abdominale sans injection du produit de contraste qui objectivait une lésion tissulaire ...

  17. Etude paléopédologique de vertisols dans un système fluviatile (Marnière d'Epéclens, USM du plateau): et Cartographie des formations superficielles du Cirque de St-Sulpice, Jura neuchâtelois, Suisse

    OpenAIRE

    Jeannottat, Simon; Verrecchia, Eric

    2005-01-01

    Ce travail a pour but de comprendre l’évolution des paléosols récents dans la marnière d’Eclépens et de reconstituer les aléoenvironnements dans lesquels les sédiments s’y sont déposés. Pour ce faire, différentes méthodes sont utilisées. Dans le cadre de cette étude, elles ont consisté en l’analyse des éléments majeurs, de la minéralogie totale de la roche, de la minéralogie de la fraction argileuse, et de la granulométrie. Des lames minces ont également été réalisées. La marnière d’Eclépens ...

  18. 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

  19. 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.

  20. 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.

  1. 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

    2016-01-01

    Purpose of review 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% two-year survival rate. Recent findings 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 co-evolution, 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. Summary 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. PMID:27548652

  2. O significado de uma organização de apoio aos portadores e familiares de fibrose cística na perspectiva das famílias

    Directory of Open Access Journals (Sweden)

    Geisa dos Santos Luz

    2011-01-01

    Full Text Available El presente estudio tiene como objetivo comprender el significado de una organización de apoyo a las familias de pacientes con fibrosis quística. Se utilizó como referencia el método cualitativo y fenomenológico. Se hicieron entrevistas a 14 familias que viven en las regiones Norte y Noroeste del Estado de Paraná, Brasil, registradas en la Associação Paranaense de Assistência a Mucoviscidose. Los resultados se agruparon en tres categorías: a Sentir apoyo en el ambiente de la fibrosis quística; b Compartir la vida: los cuidados esenciales a las familias que tienen pacientes con fibrosis quística; c Solidaridad, equidad y empowerment: un trípode de la organización social en la fibrosis quística. Se concluye que compartir la experiencia de las familias es crucial para el enfrentamiento de la enfermedad y que la asociación de pacientes es fundamental para que ellos acepten la enfermedad con tranquilidad teniendo una buena expectativa de calidad en sus vidas.

  3. Estado nutricional e ingestão alimentar de pessoas com fibrose cística Nutritional status and food intake of individuals with cystic fibrosis

    Directory of Open Access Journals (Sweden)

    Giovanna Medeiros Rataichesck FIATES

    2001-08-01

    Full Text Available A mucoviscidose é uma doença que afeta o estado nutricional por interferir na ingestão e absorção adequadas de nutrientes. Este estudo avaliou 22 pacientes mucoviscidóticos atendidos no Ambulatório de Nutrição do Hospital Infantil Joana de Gusmão em Florianópolis, SC, no período de agosto de 1998 a janeiro de 1999. O estado nutricional foi determinado através de medidas antropométricas e dados de consumo alimentar. Quanto ao estado nutricional, 42,9% dos menores de 2 anos e 28,6% dos maiores de 10 anos estavam desnutridos; na faixa etária de 2 a 10 anos não houve nenhuma criança desnutrida. Quanto ao consumo alimentar, 33,3% atingiram a recomendação de energia e 95,2% atingiram a recomendação de proteína. Podemos concluir que a mucoviscidose afeta o estado nutricional das crianças em períodos críticos de crescimento e desenvolvimento, e que a qualidade da dieta destes pacientes pode ser melhorada através de um cuidado nutricional adequado.Cystic fibrosis affects nutritional status because it interferes with adequate ingestion and absorption of nutrients. The present study evaluated 22 fibrocystic patients, who attended ambulatory visits at Hospital Infantil Joana de Gusmão, in the city of Florianópolis, Santa Catarina, Brazil, from August 1998 to January 1999. Nutritional status was determined by anthropometric measurements and food intake data. As to the nutritional status, 42.9% of the children under 2 years old and 28.6% over 10 years old were malnourished. Children between 2-10 years old did not present malnutrition. Evaluation of nutritional intake showed that 33.3% were eating adequate amounts of energy, and 95.2% of protein. It can be concluded that cystic fibrosis affects nutritional status of children in critical periods of growth and development, and that the quality of their diets can benefit from adequate nutritional care

  4. 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

  5. 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.

  6. Proteinúria na fibrose cística: possível correlação entre genótipo e fenótipo renal

    OpenAIRE

    Cemlyn-Jones, Jessica; Gamboa, Fernanda

    2009-01-01

    OBJECTIVE: To assess proteinuria in patients with cystic fibrosis (CF), and to correlate proteinuria with genotype, CF-related diabetes and disease severity. METHODS: A prospective study was carried out over a six-month period and involving 22 CF patients. After the collection and analysis of 24-h urine samples, the patients were divided into two subgroups: protein excretion 150 mg/day (highproteinuria). Patient charts were reviewed to o...

  7. 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.)

  8. 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 ...

  9. 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)

  10. 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

  11. 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

  12. 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 ...

  13. Catalytic wet peroxide oxidation of formic acid in wastewater with ...

    African Journals Online (AJOL)

    2016-07-03

    Jul 3, 2016 ... total reflectance Fourier transform infrared (ATR FTIR) spectra ... ATR FTIR. Hydrogen peroxide decomposition test with the naturally- occurring iron ore added as catalyst. Hydrogen peroxide reaction mixtures of different concentrations ..... la degradation superficielle de FeS2, CuFeS2, ZnS et PbS a.

  14. Embryonic adaptations and nutrition in the viviparous teleost Clinus ...

    African Journals Online (AJOL)

    the process of research in Holland during the war. No. 11,. Amsterdam. CORNISH, D.A. 1983. Reproductive biology and viviparity in the teleost Clinus dorsalis. (Perciformes: Clinidae). M.Sc. dissertation. Zoology Department, University of Stellenbosch. DEP:f;CHE, J. 1973. Infrastructure superficielle de la vesicule vitellaire.

  15. 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

  16. Recherche de nouvelles substances naturelles d'intérêt dans la prévention de la fibrose rénale d'origine médicamenteuse / Research of new natural substances of interest in the prevention of drug induced renal fibrosis

    OpenAIRE

    Bunel, Valérian

    2014-01-01

    Les reins sont les organes cibles de nombreuses molécules toxiques. Les cellules épithéliales du tubule proximal rénal sont particulièrement vulnérables vis-à-vis de xénobiotiques utilisés comme médicaments ou non. Ces agressions peuvent être corrélées à une augmentation du stress oxydatif et induire la mort cellulaire. Elles peuvent également mener à la perte des caractéristiques phénotypiques des cellules épithéliales, initiant leur dédifférenciation en cellules mésenchymateuses et éventuel...

  17. O bloqueio da síntese do óxido nítrico promove aumento da hipertrofia e da fibrose cardíaca em ratos submetidos a treinamento aeróbio Nitric oxide synthesis blockade increases hypertrophy and cardiac fibrosis in rats submitted to aerobic training

    Directory of Open Access Journals (Sweden)

    Hugo Celso Dutra de Souza

    2007-08-01

    Full Text Available OBJETIVO: O presente estudo avaliou as adaptações teciduais cardíacas em ratos submetidos a treinamento aeróbio, após o bloqueio da síntese de óxido nítrico (NO. MÉTODOS: Os animais (n = 48 foram divididos em quatro grupos: sedentários (grupo CONTROLE, hipertensos após administração de Ng-nitro-L-arginina metil éster durante sete dias (grupo L-NAME, treinados por meio de natação durante oito semanas (grupo TREINADO e treinados e tratados com L-NAME na última semana (grupo TREINADO L-NAME. Em todos os animais foi registrada a pressão arterial (PA e realizada a avaliação morfométrica cardíaca. RESULTADOS: Os grupos L-NAME e TREINADO L-NAME apresentaram-se hipertensos em relação aos demais (p OBJECTIVE: The objective of the present study was to evaluate cardiac tissue adaptations in rats submitted to aerobic training after nitric oxide (NO synthesis blockade. METHODS: The animals (n=48 were divided into four groups: sedentary (CONTROL group; hypertensive after administration of NG-nitro-L-arginine methyl ester for 7 days (L-NAME Group; trained for 8 weeks through swimming exercises (TRAINED Group;trained and treated with L-NAME during the last week (L-NAME TRAINED Group. All the animals were submitted to the experiment procedures for blood pressure (BP readings and cardiac morphometric evaluation. RESULTS: In comparison to the other groups, the L-NAME and L-NAME TRAINED groups were hypertensive (p<0.05; however, BP elevation in the L-NAME TRAINED group was significantly lower than the L-NAME group (p<0.05. The heart weight indexes for the TRAINED and L-NAME TRAINED groups were higher than the CONTROL and L-NAME groups (p<0.05. Also they had presented higher rates of macroscopic cardiac area and cardiac fibrosis in relation to the rest (p<0.05; comparisons revealed that the values for the L-NAME TRAINED group were significantly higher (p<0.05 than the others. CONCLUSION: Short term NO synthesis blockade in sedentary animals induced hypertension but did not cause cardiac hypertrophy. In the trained animals, the inhibition of NO synthesis attenuated hypertension, induced cardiac hypertrophy and significantly increased myocardial fibrosis, indicating that NO plays an important role in cardiac tissue adaptations caused by aerobic exercise.

  18. 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.

  19. Etude de la qualité physicochimique et bactériologique de la nappe ...

    African Journals Online (AJOL)

    AKA Boko

    est due à la texture sableuse très filtrante, à l'alimentation de la nappe par les eaux superficielles (pluies, oued Sebou, retour d'eau d'irrigation), et à l'utilisation abusive des fertilisants azotés [3]. Le taux moyen d'azote potentiellement lixiviable au niveau de la zone M'nasra est de l'ordre de 189 Kg N/ha /an [4]. La pollution ...

  20. 2154-IJBCS-Article-Gilbert Tite Layeye

    African Journals Online (AJOL)

    hp

    generale de l'eau Ministère de l'énergie, des recherches pétrolières et minières, de l'eau et du développement des énergies renouvelables (merpmeder), 77 p. Le Barbé L, Alé G, Millet B, Texier H, Borel. Y, Gualde R. 1993. Les Ressources en. Eaux Superficielles de la République du. Bénin. Edition Orstom : Paris ; 540 p.

  1. Research Article

    African Journals Online (AJOL)

    1 mai 2017 ... Le bassin versant de l'Oued Righ comblé de terrains Quaternaires (sables, calcaires, grès, argiles et évaporites) siège de la nappe superficielle (niveaux sableux et argilo-évaporitiques). L'alternance de couches imperméables et perméables d'une part et l'existence d'un fossé de subsidence d'autre part, ...

  2. Case report

    African Journals Online (AJOL)

    abp

    3 juil. 2015 ... A l'examen, la patiente présente des taches café au lait sur pratiquement tout le corps, accentués au niveau du tronc avec présence de nombreux neurofibromes plexiformes au niveau du thorax et de l'abdomen. La tumeur de la région scapulaire était superficielle, mobile par rapport au plan profond, ...

  3. 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.

  4. Modelisation et optimisation de la trempe de contour des pièces ...

    African Journals Online (AJOL)

    Après avoir traité la trempe superficielle par induction des pièces de géométries simples (cylindriques) [1], nous proposons donc de traiter la trempe de contour des pièces dentées de type engrenages, roues dentées, …, et plus précisément ... Key Words: Inductive heating, Quenching of contour, optimal frequency, gearing

  5. 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

  6. 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...

  7. Fibrose cística em um centro de referência no Brasil: características clínicas e laboratoriais de 104 pacientes e sua associação com o genótipo e a gravidade da doença Cystic fibrosis at a Brazilian center of excellence: clinical and laboratory characteristics of 104 patients and their association with genotype and disease severity

    Directory of Open Access Journals (Sweden)

    Alfonso E. Alvarez

    2004-01-01

    Full Text Available OBJETIVO: Estudar as características clínicas, laboratoriais e radiográficas de pacientes fibrocísticos acompanhados na última década do século 20 na UNICAMP e verificar se existe associação com o genótipo e a gravidade da doença medida pelo escore de Shwachman. MÉTODOS: Estudo descritivo, retrospectivo e de corte transversal dos pacientes fibrocísticos acompanhados na UNICAMP, que tiveram atendimento entre julho de 1990 e julho de 2000. RESULTADOS: Foram estudados 104 pacientes: sexo masculino - 53,8%; raça caucasóide - 93,3%; comprometimento pulmonar - 89,4%, comprometimento digestivo - 59,6%; íleo meconial - 5,8%; diabetes melito - 4,8%; mediana da idade de início dos sintomas - 3 meses; mediana da idade no diagnóstico - 2 anos e 4 meses; 69,9 e 56,6% apresentavam peso e estatura abaixo do percentil 10, respectivamente, na época do diagnóstico; dosagem de cloro no suor OBJECTIVE: To identify the clinical, laboratory and radiographic characteristics of the cystic fibrosis patients under care at Universidade Estadual de Campinas (UNICAMP in the last decade of the twentieth century, and to investigate the association of these characteristics with genotype and severity of the disease as measured by the Shwachman score. METHODS: Descriptive, retrospective and cross-sectional study of the patients assisted at UNICAMP hospital's Cystic Fibrosis Clinic from July 1990 to July 2000. RESULTS: One hundred and four patients were studied; 53.8% male; 93.3% Caucasian; 89.4% presented with respiratory symptoms; 59.6% presented with digestive symptoms; 5.8% had meconium ileus ; 4.8% had diabetes. The mean age at onset of symptoms was 3 months, and the mean age at diagnosis was 2 years and 4 months. At diagnosis, 69.9 and 56.6% of the patients had weight and height below 10th percentile, respectively; in 10.6%, sweat chloride was < 60 mEq/l. Staphylococcus aureus was found in 80.2%, Pseudomonas aeruginosa in 76.0%, and Burkholderia cepacia in 5.2%. deltaF508 homozygosis was observed in 18.75%, whereas 62.50% of the patients were deltaF508 heterozygous. A moderate/severe Shwachman score was found in 15.7%. Eighteen patients died in that period (17.3%. The mean age at death was 7 years and 8 months; median survival after diagnosis was 18 years and 4 months. Patients who have at least one deltaF508 mutation have more frequent alterations in fecal fat levels when compared to patients who do not have this mutation (p < 0.05. There were no differences in any parameter between deltaF508 homozygous and heterozygous patients. CONCLUSIONS: The clinical and laboratory characteristics of the 104 patients studied were similar to the characteristics described for patients in other countries. Exceptions are the higher age at diagnosis and lower survival. Our results support the recommendation for early diagnosis and the need for more treatment opportunities in the population of cystic fibrosis patients.

  8. 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...

  9. 833-IJBCS-Article-Jhonn Logbo

    African Journals Online (AJOL)

    DR GATSING

    par les racines sert à dissiper l'énergie solaire non utilisée par la partie aérienne au cours de la photosynthèse. Les besoins en eau de la plante sont directement liés au climat. La présence ou l'absence de l'eau entraine le développement d'un compartiment par rapport à l'autre. En saison sèche, les couches superficielles ...

  10. Détermination de valeurs seuils sécheresse pour les eaux de surface du département de Mayotte - Rapport final

    OpenAIRE

    Nicolle, P.; Lebecherel, L.; Perrin, C.; Delaigue, O.

    2016-01-01

    / Les cours d'eau de Mayotte sont soumis à des pressions croissantes pour l'approvisionnement en eau potable de la population et la satisfaction de différents usages, notamment agricoles. Pour satisfaire ces besoins en eau, les gestionnaires disposent de différentes sources d'approvisionnement, incluant les eaux superficielles (captages en rivières et retenues collinaires), souterraines et le dessalement de l'eau de mer. La tension sur la ressource et les besoins de protection des milieux ont...

  11. RHINORRHEE CEREBROSPINALE

    African Journals Online (AJOL)

    31 mai 2013 ... L'interposition de la greffe était faite selon la procédure overlay dans tous les cas. Deux récidives ont été notées et ont ... une greffe d'aponévrose temporale superficielle dans 1 cas. Une exclusion sinusienne était ... de confirmer l'origine cérébrospinale de la rhinorrhée: la détection électro phorétique de la ...

  12. Calcul des ouvrages géotechniques selon l'Eurocode 7

    OpenAIRE

    BURLON, Sébastien; DESODT, Clément; HABERT, Julien; REIFFSTECK, Philippe

    2017-01-01

    L'Eurocode 7 et ses normes d'application nationale ont introduit ces dernières années de nouvelles procédures et méthodes de calcul des ouvrages géotechniques. Cet ouvrage propose une synthèse et une analyse des principaux changements introduits et met en évidence les différences entre ce nouvel ensemble de normes et celui plus ancien. Chaque chapitre correspond à un type d'ouvrages géotechniques (fondations superficielles, fondations profondes, écrans de soutènement, etc.) et comprend à l...

  13. 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’...

  14. Influence de la maturation des sols de mangrove sur la déflation éolienne et la formation des dunes argileuses dans le delta du fleuve Sénégal

    OpenAIRE

    Barbiéro, Laurent; Ould Mohamedou, A.; Caruba, R.

    1998-01-01

    L'étude des dunes argileuses est largement employée pour la reconstitution des paléo-environnements. L'arrêt de la sédimentation éolienne est généralement attribuée à des modifications climatiques ou hydrologiques. Notre étude, menée dans le delta du fleuve Sénégal, montre que des transformations internes de la couverture pédologique peuvent induire des modifications de la partie superficielle des sols et arrêter la déflation éolienne, sans modifications des conditions environnementales. (Rés...

  15. Images in medicine

    African Journals Online (AJOL)

    ebutamanya

    29 mai 2015 ... cornéenne proche du limbe avec un appel vasculaire cornéen, une infiltration limbique collectée, une kératite ponctuée superficielle. L'examen cutané du visage retrouve une légère éruption vésiculo- pustuleuse (flèche noir) prenant la partie supérieure de l'hémiface droite et le nez. Trois diagnostiques on ...

  16. Role of hepatitis C virus genotype 3 in liver fibrosis progression : a systematic review and meta-analysis ; et, Impact of nurse vaccination program on hepatitis B immunity in a Swiss HIV clinic

    OpenAIRE

    Probst, A.

    2012-01-01

    Rôle du génotype 3 du virus de l'hépatite C dans la progression de la fibrose hépatique, une revue systématique avec méta-analyse. On estime à 170 millions le nombre de personnes atteintes d'hépatite C chronique dans le monde. La principale conséquence de cette maladie est la fibrose du foie, qui évolue plus ou moins rapidement, pour aboutir au développement d'une cirrhose et/ou d'un hépatocarcinome. Certains des facteurs accélérateurs de la fibrose, comme l'âge avancé au moment de l'infec...

  17. Fiabilidade do modelo Lok no diagnóstico da cirrose hepática

    OpenAIRE

    Costa, Jennifer Lima da

    2010-01-01

    Trabalho final de mestrado integrado em Medicina área cientifica de Gastroenterologia, apresentado à Faculdade de Medicina da Universidade de Coimbra Introdução: Marcadores serológicos que permitem avaliar a presença de cirrose hepática têm sido intensamente estudados. Contudo, a biópsia hepática continua a ser o Gold-standard para avaliar o grau de fibrose. O modelo de Lok é um teste indirecto que consiste numa fórmula que analisa marcadores indirectos de fibrose e foi formulado para perm...

  18. The lungs in rheumatoid arthritis - a clinical, radiographic and ...

    African Journals Online (AJOL)

    Five patients had digital clubbing, of whom 2 had fibrosing alveolitis and 1 bronchiectasis. No cause of the ..... Junk AG. Davidsen O. Graudal H. Prevalence of pulmonary involvement in rheumatoid arthrrtis and its relationship to some characterist,cs of the patients. Scand J RhelJmatol1982: 11: 217-224. 21. Hyland RH.

  19. CLINICAL EVALUATION OF THE MANIFESTATIONS OF INTERSTITIAL LUNG INJURYIN SYSTEMIC SCLERODERMA FROM HIGH-RESOLUTION COMPUTER T OMOGRAPHY DATA

    Directory of Open Access Journals (Sweden)

    L P Anan'eva

    2011-01-01

    Conclusion. Chest HRCT reveals the characteristic symptoms of ILI and reflects different phases of a fibrosing process in the lung. It is essential to make an in-depth examination using HRCT in all patients with SDD, irrespective of its clinical form in the earliest periods for the timely detection and treatment of ILI.

  20. ANÁLISE CELULAR E MOLECULAR DO PROCESSO APOPTÓTICO EM CÉLULAS ESTRELADAS HEPÁTICAS TRATADAS COM O PEPTÍDEO ANGIOTENSINA-(1-7.

    Directory of Open Access Journals (Sweden)

    Letícia Ferreira Ramos

    2017-03-01

    Full Text Available O fígado possui funções importantes e danos excessivos neste órgão acarretam em alterações metabólicas que podem induzir o aparecimento da fibrose do órgão. Dependendo da intensidade e persistência do estímulo indutor da fibrose, estes podem levar à cirrose e até mesmo ao hepatocarcinoma. Sabe-se que a necrose e a apoptose podem ocorrer em resposta a danos crônicos, mas também podem reverter o quadro fibrosante. Além disso, durante estímulos negativos, o estresse oxidativo também contribui para o estabelecimento da fibrose.  Uma das células relacionadas com a fibrogênese do órgão são as células estreladas hepáticas. Estas se apresentam quiescentes em fígados saudáveis e ativadas em fígados doentes. Atualmente terapias inovadoras são objetivadas e a Angiotensina-(1-7 [Ang-(1-7], desponta-se como um possível elemento modulador da fibrose. Neste estudo observou-se a atuação da Ang-(1-7 no controle de mecanismos negativos no equilíbrio dessas células.

  1. Conjugation Is Essential for the Anticholestatic Effect of NorUrsodeoxycholic Acid in Taurolithocholic Acid-Induced Cholestasis in Rat Liver

    NARCIS (Netherlands)

    Denk, Gerald U.; Maitz, Silvia; Wimmer, Ralf; Rust, Christian; Invernizzi, Pietro; Ferdinandusse, Sacha; Kulik, Wim; Fuchsbichler, Andrea; Fickert, Peter; Trauner, Michael; Hofmann, Alan F.; Beuers, Ulrich

    2010-01-01

    NorUDCA (24-norursodeoxycholic acid), the C-23-homolog of ursodeoxycholic acid (UDCA), showed remarkable therapeutic effects in cholestatic Mdr2 (Abcb4) (multidrug resistance protein 2/ATP-binding cassette b4) knockout mice with sclerosing/fibrosing cholangitis. In contrast to UDCA, norUDCA is

  2. Nephrogenic systemic fibrosis: More questions and some answers

    DEFF Research Database (Denmark)

    Morcos, S.K.; Thomsen, Henrik S.

    2008-01-01

    Nephrogenic systemic fibrosis (NSF) is a fibrosing disorder that may develop in patients who have advanced reduction in renal function. A causal relation between gadolinium (Gd3+)-based contrast agents (Gd-CA) and NSF is probable and is supported by the accumulating data in the literature. From t...

  3. Anaesthesia for tonsillectomy in HIV-infected children with ...

    African Journals Online (AJOL)

    lymphangioleiomyomatosis, neurofibromatosis, vasculitis. 5.3 Metabolic disorders: glycogen storage disease, Gaucher disease, thyroid disorders. 5.4 Others: tumoral obstruction, fibrosing mediastinitis, chronic renal failure on dialysis. PAH – pulmonary arterial hypertension; BMPR2 – bone morphogenetic protein receptor 2; ...

  4. Stem cell  mediated liver regeneration:

    DEFF Research Database (Denmark)

    Jelnes, Peter

    Leversygdomme er et udbredt sundhedsproblem verden over. Kroniske leversygdomme er karakteriseret ved en kontinuerlig ødelæggelse af leverparenkymet samt fibrose. Den nuværende og foretrukne behandling af terminale leversygdomme er levertransplantation med efterfølgende immunsuppressiv behandling...

  5. 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

  6. 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

  7. 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

  8. 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

  9. Trend Over Time for Cholecystectomy following the Introduction of ...

    African Journals Online (AJOL)

    2018-03-05

    Mar 5, 2018 ... and acalculous cholecystitis. Patients with preoperative diagnosis of common bile duct stones were excluded from this study. There were six conversion to OC due to uncertain anatomy, excessive bleeding and extensively fibrosed gallbladder in two cases each. No mortality was recorded in this series.

  10. Acute coronary syndrome: Role of the telomere dynamic

    African Journals Online (AJOL)

    USER

    2010-05-03

    May 3, 2010 ... SSDB, single-stranded DNA breaks; eNOS, endothelial nitric oxide synthase; RNP ... ignore telomere as a double stranded DNA break point ...... 62(1): 7-12. Bellini A, Mattoli S (2007). The role of the fibrocyte, a bone marrow- derived mesenchymal progenitor, in reactive and reparative fibroses. Lab. Invest.

  11. Hypertension artérielle pulmonaire au cours de la sclérodermie: à ...

    African Journals Online (AJOL)

    Le syndrome de Raynaud était observé chez 8 patients soit 66,67% de nos patients. L'électrocardiogramme montrait des signes de surcharge droite chez 4 malades (33,33%) et la radiographie thoracique en faveur d'une fibrose pulmonaire chez 4 patients. L'échocardiographie-Doppler notait une insuffisance tricuspide ...

  12. MRI in mucoviscidosis (cystic fibrosis); MRT bei Mukoviszidose

    Energy Technology Data Exchange (ETDEWEB)

    Eichinger, M.; Puderbach, M.; Kauczor, H.-U. [Deutsches Krebsforschungszentrum (DKFZ), Abteilung Radiologie, Heidelberg (Germany); Heussel, C.-P. [Universitaetsklinikum Mainz (Germany). Klinik und Poliklinik fuer Radiologie

    2006-04-15

    Cystic fibrosis (CF) is a multi-systemic disease with major impact on the lungs. Pulmonary manifestation is crucial for the prognosis and life expectancy of patients. Imaging modalities and lung function tests reflect the pulmonary status in these patients. The standard imaging modality for diagnosis and follow-up of pulmonary changes is chest x-ray. The gold standard for the detection of parenchymal lung changes remains high resolution computed tomography (HRCT), but this is not used routinely for CF-patients due to radiation exposure. Magnetic resonance imaging (MRI) used to be of no importance in monitoring cystic fibrosis lung disease, as shown in studies from the 1980s and early 1990s. The continuing improvement of MRI techniques, however, has allowed for an adequate application of this non-radiation method in diagnosing the major pulmonary findings in CF, in addition to the assessment of lung function. (orig.) [German] Die Lunge ist Hauptmanifestationsort der Mukoviszidose (zystischen Fibrose, CF) und entscheidend fuer Prognose und Lebenserwartung der Betroffenen. Bildgebende Verfahren spielen in der Diagnostik und Verlaufsbeurteilung der Lungenveraenderungen sowie dem Monitoring pulmonaler Komplikationen bei zystischer Fibrose eine wichtige Rolle. Obwohl die hochaufloesende Computertomographie (HRCT) als Goldstandard zur Beurteilung morphologischer Lungenveraenderungen bei zystischer Fibrose gilt, ist die Roentgenthoraxaufnahme aufgrund der geringeren Strahlenbelastung bei den meist jungen Patienten das derzeit eingesetzte Standardverfahren zur Diagnostik und Verlaufsbeurteilung. Die Magnetresonanztomographie (MRT) spielte bislang in der Diagnostik und Verlaufsbeobachtung der zystischen Fibrose keine Rolle, da Arbeiten aus den 80er und fruehen 90er Jahren keinen sinnvollen Beitrag der MRT zeigen konnten. Durch die kontinuierliche Verbesserung der Technik ist es seit neuestem erstmals moeglich, die MRT als Alternative fuer die Lungenbildgebung bei zystischer

  13. 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

  14. The Emancipation of Mimi ?

    OpenAIRE

    Guibert, Gérôme

    2013-01-01

    Signée chez Sony, puis chez Universal, Mariah Carey est une chanteuse américaine née en 1970 qui a vendu plus de 150 millions d’albums depuis les débuts de sa carrière professionnelle en 1990. Une analyse superficielle pourrait laisser penser qu’il s’agit d’une interprète façonnée par les majors pour interpréter des titres calibrés en fonction de la demande (Guibert, 1998). Pourtant, le fait qu’elle écrive ou coécrive la quasi intégralité de ses chansons et qu’elle soit elle-même productrice ...

  15. POMME - Programme Océan Multidisciplinaire Méso Echelle. CAMPAGNE POMME T0. Rapport de Données Eulériennes (Volume 1)

    OpenAIRE

    Billant, Andre; Branellec, Pierre

    2002-01-01

    Un des éléments essentiels de la circulation de l’océan et de ses interactions avec l’atmosphère est la formation d’eaux modales. Dans l’Atlantique Nord, les courants chauds se dirigent vers le Nord (dérive Nord-Atlantique) cèdent leur chaleur à l’atmosphère et se refroidissent vigoureusement. Il en résulte, l’hiver, la formation de couches superficielles homogènes, dites «couches de mélange», qui peuvent atteindre plusieurs centaines de mètres. Ces masses d’eau étant entraînées par le couran...

  16. Vieillissement thermique d’un jonc composite carbone/époxy

    OpenAIRE

    SERROR, Maéva; COLIN, Xavier; LARCHE, Jean-François

    2013-01-01

    Le vieillissement thermique d’un jonc composite carbone/époxy a été étudié dans l’air entre 160 et 220 °C par tomographie X, microscopie optique et flexion trois points. La thermo-oxydation de la matrice affecte une couche superficielle trop fine pour pouvoir altérer les propriétés mécaniques. La thermolyse de la matrice est donc le principal mode de dégradation. Elle conduit à la formation de longs microcanaux parallèles aux fibres dans les régions les plus riches en matrice du cœur du jonc....

  17. Bainville-sur-Madon – Terre Vaine, carrière COGESUD (tranche 2)

    OpenAIRE

    Brénon, Jean-Charles; Koenig, Marie-Pierre

    2013-01-01

    Identifiant de l'opération archéologique : 137211 Date de l'opération : 2008 (EX) Cette opération de diagnostic archéologique fait suite à la première tranche de 5 ha sondée en 2005 par M.-P. Koenig. Aucun vestige archéologique n'a été mis au jour sur les 7 ha expertisés lors de cette seconde phase. L'absence de vestiges n'est pas surprenante au vu de l'érosion totale de la formation superficielle. De nouvelles anomalies circulaires et curvilignes, identiques à celles observées en photographi...

  18. L'ALTERATION DES VERRES : DONNEES DE L'ANALYSE DE SURFACE

    OpenAIRE

    Thomassin, J.; Baillif, P.; Touray, J.

    1982-01-01

    L'étude de la composition superficielle des verres soumis à altération a été abordée par spectrométrie XPS (ou ESCA), sonde ionique (SIMS) et microscopie électronique (transmission et balayage). Les matériaux choisis sont des verres industriels et naturels ; les milieux de corrosion sont l'eau douce, l'eau de mer et des solutions d'ions métalliques. La température est de 90°C, le milieu est non agité et non renouvelé. Tous les essais ont été réalisés sur des esquilles. Les principaux résultat...

  19. Détermination de la qualité du milieu sur l'inactivation des virus en mer

    OpenAIRE

    Schwartzbrod, L.; Dincher, M.l; Deloince, R.; Crance, J.m

    1992-01-01

    Les virus sont rejetés en quantité considérable dans le milieu aquatique et ce par le biais des eaux usées urbaines fortement chargées en microorganismes. Le traitement de ces eaux n'étant pas d'une efficacité parfaite en ce qui concerne les virus, une quantité non négligeable de ces derniers persiste dans les eaux épurées qui sont rejetées dans les eaux superficielles et en particulier dans les mers. En zones conchylicoles côtières, la présence de virus dans l'eau de mer constitue un risque ...

  20. Détermination expérimentale de la résistance thermique d'interface d'un dépôt métallique submicronique sur son substrat

    OpenAIRE

    Hmina, N.; Scudeller, Y.

    1995-01-01

    Les mécanismes de croissance et l'adhésion d'un dépôt sont étroitement liés aux propriétés physiques et chimiques superficielles du substrat. La détermination de la résistance thermique d'interface peut mettre en lumière certains aspects de ce couplage en caractérisant la présence d'impuretés, de fissures ou de décollements liés au procédé d'élaboration. Une méthode de mesure originale de la résistance d'interface entre un dépôt submicronique et un substrat diélectrique est présentée. Elle es...

  1. 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

  2. QUANDO O DIAGNÓSTICO É TARDIO

    Directory of Open Access Journals (Sweden)

    Ana Lachado

    2016-07-01

    Conclusões/Comentários: A fibrose quística é a doença autossómica recessiva mais frequente na raça caucasiana, com incidência de 1:2000-3000 em determinados países e por vezes ainda subdiagnosticada. A apresentação clínica desta doença é variada. Com este caso clínico, os autores pretendem alertar para a necessidade de pensar em fibrose quística na presença de sintomas mais leves ou menos frequentes, uma vez que precocidade do diagnóstico é essencial para um melhor prognóstico. Realça-se a importância de um exame objetivo pormenorizado e adequada valorização das alterações identificadas.

  3. 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

  4. CT in the diagnosis of interstitial lung disease

    International Nuclear Information System (INIS)

    Bergin, C.J.; Mueller, N.L.

    1985-01-01

    The computed tomographic (CT) appearance of interstitial lung disease was assessed in 23 patients with known interstitial disease. These included seven patients with fibrosing alveolitis, six with silicosis, two with hypersensitivity pneumonitis, three with lymphangitic spread of tumor, two with sarcoidosis, one with rheumatoid lung disease, and two with neurofibromatosis. The CT appearance of the interstitial changes in the different disease entities was assessed. Nodules were a prominent CT feature in silicosis, sarcoidosis, and lymphangitic spread of malignancy. Distribution of nodules and associated interlobular septal thickening provided further distinguishing features in these diseases. Reticular densities were the predominant CT change in fibrosing alveolitis, rheumatoid lung disease, and extrinsic allergic alveolitis. CT can be useful in the investigation of selected instances of interstitial pulmonary disease

  5. Exercise testing, aerobic and strength training for adolescents with cystic fibrosis: a literature review

    OpenAIRE

    Chaves, Celia Regina Moutinho de Miranda; Oliveira, Cristiano Queiroz de; Britto, José Augusto Alves de; Elsas, Maria Ignez Capella Gaspar

    2007-01-01

    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 inform...

  6. Case report

    African Journals Online (AJOL)

    abp

    3 mai 2014 ... l'orientation thérapeutique des plexopathies brachiales chez les femmes présentant un cancer du sein. Pan African Medical Journal. ... Il s'agit d'une femme de 50 ans, suivie depuis quatre ans pour une tumeur du sein gauche pour ... Parfois la discrimination entre métastase et fibrose post-radique est ...

  7. 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)

  8. Author Details

    African Journals Online (AJOL)

    Dhifallah, Maher. Vol 21, No 1 (2015) - Articles La fibrose rétropéritonéale idiopathique: une cause rare de douleurs lombaires chez le sujet âgé. Abstract PDF · Vol 21, No 1 (2015) - Articles Dilatation de bronches séquellaire d'une tuberculose pulmonaire au cours d'un syndrome de Rhupus Abstract PDF. ISSN: 1937-8688.

  9. 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

  10. [Some parameters of bronchoalveolar lavages in alveolitis].

    Science.gov (United States)

    Makhmudova, S Iu

    2003-01-01

    Eighty nine patients with alveolitis [60 with extrinsic allergic alveolitis (EAA) and 29 with idiopathic fibrosing alveolitis (IFA)] were followed up. Cytological and immunological studies of bronchoalveolar lavage revealed that the patients with EAA had elevated counts of lymphocytes, moderately increased neutrophils and eosinophils, decreased alveolar macrophages, elevated SIgA and T lymphocytes. In the patients with IFA, only higher counts of neutrophils were significant.

  11. Charakterisierung inaktivierender posttranslationaler Modifikationen des GC-A-Rezeptors für das atriale natriuretische Peptid (ANP)

    OpenAIRE

    Hartmann, Michael

    2014-01-01

    Das atriale natriuretische Peptid (ANP) wird infolge einer Zunahme des atrialen Drucks aus den Myozyten des Atriums sezerniert. Es spielt lokal eine bedeutende, protektive Rolle und wirkt der Entstehung von Herzhypertrophie und Fibrose entgegen. Darüber hinaus kommt ANP vor allem eine wichtige Rolle als endokrines Hormon zu, das den arteriellen Blutdruck und das Blutvolumen regelt. Diese physiologischen Effekte vermittelt das Herzhormon durch seinen Rezeptor, das Transmembranprotein Guanylatz...

  12. [Ultrastructure of endometrium during low-dose gestagenic anticonception (author's transl)].

    Science.gov (United States)

    Houdek, J; Pelák, Z; Vacek, Z

    1977-02-01

    31 women (average age, 26 years) were administered a modified low dosage gestagen contraceptive, containing .3 of norethisterone for an average period of 10.6 months. Changes in the ultrastructure of the endometrium were studied throughout this period. Changes in both parts of the ultrastructure, the fibrose and epithelial parts, were revealed in comparison to material taken before use of the preparation. Changes in the stromata cells were particularly of a gestagenic character. Epithelial cells also demonstrated a particularly gestagenic stimulation.

  13. Case series

    African Journals Online (AJOL)

    abp

    1 nov. 2017 ... &Corresponding author: Aziz El Majdoub, Service d'Urologie, CHU Hassan II, Fès, Maroc. Mots clés: ... Il s'agissait de dix hommes et deux femmes. ..... deux uretères. -TDM : UHN avec atrophie rénale droite en amont d'une plaque de fibrose rétropéritonéale. - scintigraphie au DMSA/ rein muet droit.

  14. Proceedings of the Army Symposium on Solid Mechanics. Advances in Solid Mechanics for Design and Analysis Held at Newport, Rhode Island on 1-3 October 1984.

    Science.gov (United States)

    1984-10-01

    the differential equation * (16) This is the familiar equation for the nonlinear, simple pendulum . Before presenting the solution, we put the problem...consistent. Lonqitudinal and transverse V-notch charpy impact energy and fibrosty data for the GD material are shown in Table IV. Pased on a l0% fibrosity...specifications. However, it is highly recommended that a V-notch charpy energy requirement be idded to the specification. It is further felt that the

  15. Improved High Strength Armor Steel through Texturing

    Science.gov (United States)

    1979-09-01

    conditions. CHARPY IMPACT AND TRANSITION TEMPERATURE It has been established that cleavage is crystallographic in nature and in iron occurs on the (100...allel to the plate surface or face notched (L-S), (T-S), and (LT-S). Specimens were tested on a 217 ft-lb capacity pendulum -type machine with a striking...velocity of 17 ft/sec. The Charpy transition temperature, the lowest temperature at which 100 percent fibrosity still exists, was determined for each

  16. Primary Sclerosing Cholangitis

    OpenAIRE

    A Stiehl; C Benz; P Sauer

    2000-01-01

    Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease characterized by fibrosing inflammation and obliteration of intra- and/or extrahepatic bile ducts. The disease is one of the most common cholestatic diseases in adults and is diagnosed with increasing frequency. It is very often associated with ulcerative colitis. Patients with PSC have an increased incidence of bile duct carcinomas, and those with ulcerative colitis also have an increased incidence of colonic carcino...

  17. Futures Group Periodic Report 6

    Science.gov (United States)

    1981-06-01

    sclerosis, coeliac disease , or duodenal ulcers). Later-life disease susceptibility could be determined at an early age or at any time. "Knowing what...importantly, how such counseling will be related to identifying pre- disease susceptibility (such as fibrositis, skin and lung cancer, arthritis, multiple...immunologists with the telltale signs of the pre- disposition to different diseases . Parallel and related investigations are 7. Bernard Dixon, "Life

  18. 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).

  19. Pulmonary damage caused by cytostatics and paraquat

    International Nuclear Information System (INIS)

    Fischer, G.; Woeltjen, H.H.; Schauer, A.

    1981-01-01

    Substances which exercise a pulmonary toxic action will first of all produce alveolar and perivascular oedemas followed by fibrosis. Differential diagnosis is explained on the basis of two cases of a fatal mitomycin fibrosis of the lung, as well as the observation of fibrous changes following the administration of Bleomycin and Metothrexat. The course of pulmonary fibroses caused by paraquat is described for two cases of fatal paraquat intoxications. (orig.) [de

  20. 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

  1. 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...

  2. 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...

  3. 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...... of knowledge about the possible association between GBCA and NSF. NSF is found among Japanese end-stage renal failure patients even after examinations using a single dose....

  4. Evaluation and management of alveolitis and interstitial lung disease in scleroderma.

    Science.gov (United States)

    Latsi, Panagiota I; Wells, Athol U

    2003-11-01

    In the fibrosing alveolitis of systemic sclerosis, treatment decisions depend on prognostic evaluation, which continues to excite considerable interest and debate. Advances in the staging of fibrosing alveolitis of systemic sclerosis and recent therapeutic studies are discussed in this review. The decision about whether to start treatment is often the most difficult clinical challenge, because many patients have limited pulmonary fibrosis that will not necessarily progress. The estimation of disease extent (using high-resolution CT) and disease severity (using pulmonary function tests) is pivotal. Factors reducing the threshold for treatment, in addition to severe disease, include evidence of recent deterioration, a short duration of systemic disease, antitopoisomerase antibody positivity, and, in some cases, bronchoalveolar lavage findings (although the role of bronchoalveolar lavage remains contentious). Histologic appearances at surgical biopsy have little prognostic value, with the great majority of patients having nonspecific interstitial pneumonia. Best current initial treatment consists of either oral or intravenous cyclophosphamide, usually administered with low-dose corticosteroid therapy, although the risk of scleroderma renal crisis with low-dose steroid therapy requires further evaluation. Careful prognostic evaluation, including the staging of disease severity and the definition of longitudinal disease behavior (by serial imaging and pulmonary function tests), is central to the formulation of a logical management plan in fibrosing alveolitis of systemic sclerosis. Cyclophosphamide, the best initial treatment currently, is associated with significant toxicity, justifying therapeutic studies of other immunosuppressive agents and a wide range of anticytokine and antifibrotic agents.

  5. 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

  6. 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.

  7. 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

  8. 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

  9. 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

  10. 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

  11. Capillaria hepatica-induced septal fibrosis in rats: a contribution to the study of liver fibrogenesis Contribuição para o estudo da fibrogenesis hepática em ratos infectados com Capillaria hepatica

    Directory of Open Access Journals (Sweden)

    Valter Lucas Chaves Barbosa

    2010-10-01

    Full Text Available INTRODUCTION: Septal fibrosis of the liver regularly develops in rats infected with the nematode Capillaria hepatica. Curative treatment of the infection prevents the development of septal fibrosis when intervention occurs up to postinfection day (PID 15, but not later. The present investigation aimed to demonstrate which parasitic factors are present when the process of septal fibrosis can no longer be prevented by curative treatment. METHODS: Wistar rats were infected with 600 embryonated eggs of C. hepatica administered by gavage and treated with ivermectin and mebendazole in separate groups at PIDs 10, 12, 15, 17 or 20. Rats from each group and their nontreated controls, were killed and examined 40 days after the end of treatment. RESULTS: Findings by PID 15 were compatible with the stage of complete maturation of infection, when worms and eggs were fully developed and a complex host-parasite multifocal necroinflammatory reaction showed greater intensity, but with no signs of septal fibrosis, which appeared from PID 17 onward. CONCLUSIONS: Since the worms spontaneously died by PID 15, not only septal fibrosis production, but also its maintenance and further development appeared dependent on the presence of eggs, which were the only parasitic factor remaining thereafter.INTRODUÇÃO: A fibrose septal do fígado se desenvolve regularmente em ratos infectados pelo nematódeo Capillaria hepatica. O tratamento curativo da infecção, feito antes do 15º dia da infecção, mas não mais tarde, impediu o aparecimento da fibrose septal. O presente trabalho procura verificar qual o estado do parasitismo aos 15 dias da infecção, crucial para patogenia da fibrose septal. MÉTODOS: Ratos foram infectados por via digestiva com 600 ovos embrionados de C. hepatica e tratados com Ivermectina e mebendazol, em grupos separados, aos 10, 12, 15, 17 ou 20 dias após a infecção. O animal de cada grupo e seus respectivos controles foram mortos e examinados aos

  12. 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.

  13. 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.

  14. Intervenção fisioterapêutica na dor e na qualidade de vida em idosos com esclerose sistêmica. Relato de casos

    OpenAIRE

    Jorge, Matheus Santos Gomes; Wibelinger, Lia Mara; Knob, Bruna; Zanin, Caroline

    2016-01-01

    RESUMO JUSTIFICATIVA E OBJETIVOS: A esclerose sistêmica é uma doença crônica, multissistêmica e autoimune, caracterizada por angiopatia disseminada em pequena e microcirculação, fenômeno de Raynoud e fibrose cutânea e de órgãos internos. A fisioterapia tem se mostrado uma alternativa eficaz no combate aos agravos osteomioarticulares causados pela doença. O objetivo deste estudo foi verificar os efeitos de um programa de intervenção fisioterapêutica na dor e na qualidade de vida de dois idoso...

  15. A importância da integração de dados do diagnóstico das hemopatias

    OpenAIRE

    Lorand- Metze,Irene

    2009-01-01

    A análise diagnóstica da medula óssea compreende classicamente a citologia. Mais recentemente, tornou- se rotina o estudo histológico. Desde o início, tentou- se integrar estes dados, pois, enquanto a citologia fornece uma análise mais detalhada das características das células e permite quantificá- las, a biópsia, por analisar o tecido como um todo, permite o estudo da estrutura do tecido hemopoético, seu estroma e a ocorrência de estruturas estranhas à medula, como granulomas, fibrose e metá...

  16. Beyond fibromyalgia: ideas on etiology and treatment.

    Science.gov (United States)

    Bennett, R M

    1989-11-01

    A common syndrome of musculoskeletal pain, currently called fibrositis or fibromyalgia, accounts for 10-30% of all rheumatology consultations in North America. Lacking a distinctive pathophysiological basis the nature of the pain experienced by these patients remains elusive and treatment is not based on sound scientific principles. An hypothesis is advanced which suggests that skeletal muscle is the "end organ" responsible for the pain of fibromyalgia and that previous studies on muscle deconditioning and microtrauma may be relevant to the etiopathogenesis of fibromyalgia syndrome.

  17. Vascularização na cirrose hepática: estudo imunoistoquímico baseado em necropsias

    OpenAIRE

    Maeda,Mariane de Fátima Yukie; Silva,Camilla Duarte; Harima,Leila Suemi; Silva,Luiz Fernando Ferraz da; Ctenas,Bruno; Alves,Venâncio Avancini Ferreira

    2008-01-01

    RACIONAL: O processo patológico mais discutido na gênese da cirrose hepática é a fibrose progressiva, porém alterações na vasculatura do órgão têm sido apontadas como elementos fundamentais na fisiopatologia da doença e de suas complicações, como hipertensão portal, insuficiência hepática e carcinoma hepatocelular. OBJETIVO: Avaliar a densidade microvascular em 35 casos de necropsias de pacientes com cirrose hepática mediante pesquisa imunoistoquímica do marcador endotelial CD34 a fim de comp...

  18. Scleroderma and sarcoidosis

    OpenAIRE

    Guidolin, Fernanda; Esmanhotto, Letícia; Silva, Marilia B.; Mesquita, Lismari; Skare, Thelma L.

    2005-01-01

    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 ...

  19. Génotypes du virus de l'hépatite B et marqueurs évolutifs des ...

    African Journals Online (AJOL)

    Le génotypage, les dosages quantitatifs de l'AgHBe et de l'ADN virale B ont été réalisés au Laboratoire Cerba, Cergy Pontoise, France. L'évaluation de la fibrose était faite par le Fibrotest ou le FibroScan. Résultats: Au total, 143 patients, 52,4% de sexe masculin, âge moyen 38,1 ans ont été inclus. Selon les marqueurs ...

  20. Efeito da icterícia obstrutiva na morfologia e na funçao renal após nefrectomia em rato

    OpenAIRE

    Daniel Xavier Lima

    2007-01-01

    Introdução: O crescimento de um rim em resposta à perda do outro rim recebe o nome de hipertrofia renal compensatória. Durante esse processo, ocorrem alterações na morfologia e na função do rim remanescente, que aumenta sua massa tecidual e o ritmo de filtração glomerular. Tais modificações visam à restauração da função renal, embora, em alguns casos, possam ocorrer glomeruloesclerose, fibrose tubular e outras alterações que levam à disfunção renal. Existem muitos estudos a esse respeito, ...

  1. Influence de la fistule pancréatique et de l'hémorragie post ...

    African Journals Online (AJOL)

    En analyse multi variée, la fibrose était un facteur protecteur tandis qu'une durée opératoire supérieure à 310 minutes était un facteur de risque (p = 0,026). La fistule pancréatique, l'hémorragie post opératoire et la reintervention étaient des facteurs de risque de survenue de décès (p=0,019, p=0,002 et p<0,001).

  2. 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

  3. 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

  4. 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...

  5. Disfunções da coagulação e da fibrionólise em pacientes com esquistossomose mansônica na forma hepatoesplênica

    OpenAIRE

    Leite, Luiz Arthur Calheiros

    2014-01-01

    A esquistossomose hepatoesplênica (HE) é a forma mais grave desta doença, sendo caracterizada por fibrose periportal (FPP), hipertensão portal, esplenomegalia e citopenias. Pacientes com esquistossomose na forma HE podem apresentar disfunções hemostáticas que predispõem a tromboses e hemorragias. Este estudo teve como objetivo avaliar as alterações hepáticas e da coagulação em 55 pacientes com esquistossomose na forma HE, 45 esplenectomizados, 30 pacientes com DHCM, bem como em 30 indivíduos ...

  6. ANTISYNTHETASE SYNDROME IS THE MOST SEVERE SUBTYPE OF POLYMYOSITIS/DERMATOMYOSITIS:DESCRIPTION OF CASES

    Directory of Open Access Journals (Sweden)

    Olga Alekseyevna Antelava

    2009-01-01

    Full Text Available Polymyositis (PM and dermatomyositis (DM are autoimmune skeletal muscle diseases of unknown etiology, which are referred to as systemic connective tissue diseases and united under the common term Tidiopathic inflammatory myopathiesy. The most severe subtype of PM/DM is the antisynthetase syndrome that is characterized by a certain sympathocomplex, including interstitial lung lesion that is one of the most common visceral changes. Of interest are the specific features of the antisynthetase syndrome, its onset, the course and pulmonary manifestations of fibrosing alveolitis, unlike the classical course of PM/DM. Two clinical cases of the antisynthetase syndrome are given.

  7. 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.

  8. Esclerose sistémica juvenil: uma doença incomum na infância

    OpenAIRE

    da Silva, Manuela

    2013-01-01

    A esclerose sistêmica juvenil (ESJ) é uma doença rara, de etiologia autoimune, caracterizada por fibrose tecidual intensa, com acometimento da pele e órgãos internos. O diagnóstico é baseado em critérios clínicos e laboratoriais. A doença é progressiva e limitante e seu tratamento permanece ainda controverso. Neste estudo, relata-se um caso de uma paciente feminina de 14 anos com diagnóstico de ESJ que iniciou quadro com lesões discrômicas de pele, fenômeno de Raynaud, úlceras digitais, escle...

  9. 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...

  10. 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...

  11. 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.

  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. 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.

  14. 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.

  15. 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...

  16. 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)

  17. 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)

  18. Dimensionnement des ouvrages génie civil au calcul sismique

    CERN Document Server

    Poehler, M

    2001-01-01

    L'énergie la plus importante libérée au niveau de la cro te terrestre par un séisme est sous forme d'ondes superficielles, appelées ondes de Rayleigh. Sous l'effet de ces ondes à déplacements verticaux de forme elliptique, le sol et les bâtiments qu'il soutient vibrent dans une gamme de fréquences de 0,1 à 30 hertz. Cette gamme de valeurs est spécialement nocive pour les bâtiments élancés. Les calculs de dimensionnement des ouvrages de génie civil prennent en compte ce type de sollicitation, via des forces horizontales de remplacement. Les différentes normes nationales définissent une accélération nominale due au séisme, au moyen d'une combinaison de classes d'ouvrages et de zones sismiques. De plus, des mesures spéciales de dispositions constructives et de conception architecturale sont prescrites. Les statistiques montrent que 90% des ouvrages ont été construits avant l'introduction, fin des années 80, des normes de construction parasismiques modernes.

  19. 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

  20. 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.

  1. 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

  2. Mediastinal tumors

    International Nuclear Information System (INIS)

    Canizares, Claudio; Araujo, Ivan; Rodriguez, Amparo; Robles, Wilson; Simba, Catalina

    2005-01-01

    In our practice the mediastinal tumors are infrequent. The mediastinum is the portion of the thoracic cavity that contains numerous organs and structures which makes a crossroad for the diagnostic process. Within which congenital cysts, inflammatory and benign tumors, malignant neoplasms may develop. In the superior compartment are found: thymoma and thymic cysts, germ cell tumors, thyroid lesions, parathyroid adenomas, malignant lymphomas, paragangliomas, hemangiomas, lipomas, and inflammatory lesions such as fibrosing mediastinitis. In the middle portion: pericardial cysts, bronchial cysts, malignant lymphomas. In the posterior region: neurogenic tumors such as Shawnomas, neurofibromas, ganglioneuroblastomas, neuroblastomas, paragangliomas, and gastro enteric cysts. We describe two cases. One of a female patient with a prominent tumor in the anterior compartment of the mediastinum, detected by the x-ray films. Initially a cardiac lesion was excluded by echographic, angiographic studies. The biopsy exhibited a prominent fibrosis that suggested fibrosing mediastinitis (sclerosing). Whoever the immunohistochemical phenotype was positive for lambda chains, determining the diagnosis of lymphoma. The other case is of a young male with a thymoma associated to a pure red cell aplasia, which was the initial clinical symptom. Computerized tomography and thyroid scintigraphy was used. (The author)

  3. Mast cell and histamine content of human bronchoalveolar lavage fluid.

    Science.gov (United States)

    Agius, R M; Godfrey, R C; Holgate, S T

    1985-01-01

    Bronchoalveolar lavage was performed in 97 patients including control patients with bronchial carcinoma (24) and patients with sarcoidosis (20), cryptogenic fibrosing alveolitis (9), and asthma (4), and others. Cytocentrifuged slides were stained by two methods: May-Grünwald Giemsa and toluidine blue. In the last 32 subjects the bronchoalveolar lavage fluid was separated into supernatant and cell pellet for the subsequent assay of the performed mast cell mediator, histamine. Comparison of the two methods of staining showed a bias towards toluidine blue. Controls had a differential mean (SE) mast cell count of 0.07% (0.01%). Higher counts were noted in cryptogenic fibrosing alveolitis--0.61% (0.15%) (p less than 0.001)--and in sarcoidosis--0.14% (0.02%) (p less than 0.05). There was a strong correlation between absolute mast cell counts and cell lysate histamine concentration (r = 0.78, p less than 0.001). Less strong, significant, correlations between supernatant histamine concentration and absolute mast cell counts (r = 0.48, p less than 0.01) or cell lysate histamine concentration (r = 0.72, p less than 0.01) were also found. Derived mean values of histamine per mast cell ranged from 3.7 to 10.9 picograms. The mean histamine content of lavage fluid supernatant as a percentage of the total lavage fluid histamine was 24.9% (3.3%). The possible clinical significance of these findings is discussed. Images PMID:4060097

  4. Chronic interstitial pancreatitis and chronic wasting disease caused by Eurytrema coelomaticum in Nelore cow

    Directory of Open Access Journals (Sweden)

    M.A. Rachid

    2011-06-01

    Full Text Available A euritrematose bovina, causada por Eurytrema coelomaticum, tem sido relatada no Brasil. A doença pode afetar animais domésticos, incluindo ruminantes. Estes parasitos geralmente vivem nos ductos pancreáticos e ocasionalmente nos ductos biliares. Este estudo relata um quadro de caquexia em um bovino parasitado por E. coelomaticum proveniente de um rebanho de gado de corte localizado no Estado de Minas Gerais. O animal acometido apresentou perda progressiva de peso, glicosúria e cetonúria. Na necropsia o pâncreas apresentava-se diminuído de tamanho, brancacento, intensa e difusamente firme (fibrose. Vários ductos apresentavam-se dilatados e repletos de Eurytrema. Microscopicamente foi observada destruição extensa do parênquima pancreático e fibrose, ovos e parasitas intralesionais, hiperplasia ductal e inflamação multifocal crônica. Este relato descreve a perda progressiva de peso e pancreatite crônica associada a E. coelomaticum em bovino de corte no Estado de Minas Gerais.

  5. Simultaneous chronic seneciosis and eurytrematosis in a cow/ Simultânea seneciose crônica e euritrematose em uma vaca

    Directory of Open Access Journals (Sweden)

    Ighor Augusto de Almeida

    2004-05-01

    Full Text Available Chronic intoxication associated with the ingestion of Senecio brasiliensis with concomitant interstitial and periductal pancreatitis induced by Eurytrema spp are described in a cow that demonstrated severe progressive emaciation as its principal clinical manifestation. In chronic seneciosis there was severe toxic hepatitis characterized by periportal and bridging fibrosis, megalocytosis, and proliferation of epithelial bile duct cells. Infestation by Eurytrema spp produced marked periductal and interstitial fibrosis associated with parasitic forms within interlobular pancreatic ducts.Descreve-se a intoxicação crônica por Senecio brasiliensis com concomitante pancreatite intersticial e periductal por Eurytrema spp em uma vaca que demonstrou emagrecimento progressivo como sua principal manifestação clínica. Na intoxicação crônica por Senecio spp observou-se hepatite tóxica caracterizada por fibrose periportal e em ponte, megalocitose e proliferação das células epiteliais dos ductos biliares. A infestação por Eurytrema spp induziu fibrose periductal e intersticial acentuada associada a formas interductais do trematódeo.

  6. Dorsal prevertebral lesions in Wegener granulomatosis: report on four cases.

    Science.gov (United States)

    Barreto, Paulo; Pagnoux, Christian; Luca, Luminata; Aouizerate, Jessie; Ortigueira, Isabel; Cohen, Pascal; Muller, Géraldine; Guillevin, Loïc

    2011-01-01

    Retroperitoneal fibrosis has been reported in several patients with Wegener granulomatosis (WG), but only three isolated cases of dorsal prevertebral lesions, closely resembling fibrosing mediastinitis, have been published so far. We describe four new WG patients (two men, two women), 49-59 years old at diagnosis, with dorsal prevertebral lesions, mainly right-sided, and with adjacent pleural thickening in two. These lesions were detected on computed-tomography scans at diagnosis in two patients, and occurred later in the two others. Only one of them had mild back pain. Two patients' lesions were biopsied, revealing granulomatous inflammation. In one patient, the lesion regressed under WG treatment. Lesion size did not change in the remainings. Intralesional calcifications appeared in two. None of the patients had local bone erosion, vascular or neurological complications. These prevertebral lesions might represent a dorsal form of retroperitoneal fibrosis in WG, but usually with a more benign presentation and course. WG should be included in the differential diagnosis of fibrosing mediastinitis (with tuberculosis, neoplastic diseases, sarcoidosis, histiocytosis and inflammatory pseudotumor), which may have a similar radiological appearance. Copyright © 2010 Société française de rhumatologie. Published by Elsevier SAS. All rights reserved.

  7. 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.

  8. 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.

  9. 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.

  10. 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

  11. Quality characterization of celery (Apium graveolens L.) by plant zones and two harvest dates.

    Science.gov (United States)

    Guerra, Natalia; Carrozzi, Liliana; Goñi, María Gabriela; Roura, Sara; Yommi, Alejandra

    2010-08-01

    The aim of this study was to characterize the quality of celery petioles (Apium graveolens L. cv Golden Clause) from the external, middle, and internal zones of each plant. Harvest dates were 93 (HD1) and 124 (HD2) days after transplantation. Total weight (TW), total length (TL), total leaves number (LN), and petiole length of leaves (PL) for the 3 zones were measured. Physicochemical (color, b* and h; texture; total soluble solids, TSS; titratable acidity, TA; water content, WC), nutraceutical (ascorbic acid, AA; total quinones, TQ; browning potential, BP), and sensorial analysis (flavor, fibrosity, crunchiness) were done at harvest on petioles of each zone. No differences between harvesting dates were found in TW, TL, LN, and PL. Middle leaves had the highest PL. Harvest dates were not different in b*, h, TA, AA, and WC. Texture, TSS, BP, and TQ resulted higher in petioles of HD2 than in HD1. Similar TSS and TA were found in leaves of different plant zones. The yellow color of both external and internal zones was significantly higher than in the middle zone. The texture and BP was similar between the external and middle zones but the WC was lower in the external zone. Similarly, the AA content as well as the TQ was also lower than in the middle zone. Harvest delay improved the nutraceutical value in terms of BP and TQ, even though it also resulted in pithiness and fibrosity of the leaves. This study therefore suggests that the petioles of the middle zone when harvested at HD1 are the most suitable for consumption. Practical Application: Celery is a vegetable reduced in calories, has a high nutritional value and its fresh petioles are mainly consumed in salads. The texture and flavor are the most important attributes that define consumers' acceptability. As nutritional value, texture, and flavor may change with plant age and different zones of the plant, harvest date plays an important role on quality. Results indicate that harvest delay improved the nutraceutical

  12. Pierre Curie et le rôle de la symétrie dans les lois physiques

    Science.gov (United States)

    de Gennes, P. G.

    Pierre et Jacques Curie découvraient il y a cent ans le phénomène de piézoélectricité : comment un cristal, de suffisamment basse symétrie, développe une polarization lorsqu'il est soumis à une contrainte mécanique. Pierre Curie n'a alors que 21 ans ! Et pourtant ses notes lapidaires aux Comptes Rendus datées de 1880 et 1881 [1] portent en elles tout l'essentiel du phénomène : charges superficielles proportionnelles à la pression, indépendantes de l'épaisseur du cristal, etc. Lorsque quelques mois plus tard, Lippmann prédit l'effet inverse (déformation du crystal sous champ électrique), les frères Curie imaginent tout de suite un dispositif admirablement simple pour détecter ces très faibles déformations. Ils couplent mécaniquement le cristal étudié avec un deuxième cristal piézoélectrique qui joue le rôle d'un détecteur; ce dernier transforme le signal mécanique du premier cristal en un signal électrique mesuré a l'électromètre ! Les résultats confirment brillamment la prédiction de Lippmann. Et pourtant, les moyens engagés sont très modestes — les champs électriques, par exemple, sont calibrés par la longueur d'une décharge dans l'air…

  13. 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.

  14. 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.

  15. Application de la diffraction des rayons X in situ à haute température pour l'identification d'une nouvelle phase lors de l'oxydation à 900circC de l'acier 304

    Science.gov (United States)

    Riffard, F.; Buscail, H.; Caudron, E.; Cueff, R.; Issartel, C.; El Messki, S.; Perrier, S.

    2004-11-01

    Une nouvelle interprétation du comportement atypique couramment appelé "breakaway" observé lors de l'oxydation à haute température d'alliages chromino-formeurs est proposée grâce à l'utilisation de la diffraction des rayons X in situ à haute température. L'acier chromino-formeur AISI 304 doit établir une couche d'oxyde superficielle généralement dense et majoritairement, constituée de chromine, dont la vitesse de croissance est lente, afin d'assurer sa protection contre la corrosion à haute température. Cette faible vitesse de croissance de la couche d'oxyde est effectivement observée à 1000circC. Elle serait favorisée par l'établissement d'une couche de chromine induite par la présence d'une sous-couche continue de silice à l'interface interne. Cette dernière limiterait la diffusion du fer. Le phénomène du "breakaway" est observé à la température de 900circC après 40 heures d'oxydation. Ce phénomène serait lié à la croissance initiale d'oxydes contenant du fer. L'oxyde Fe{7}SiO{10, }a été identifié{ }pour la première fois grâce à la technique de diffraction des rayons X in situ à haute température. Cet oxyde semble piéger le silicium dans la couche d'oxyde, empêchant son accumulation à l'interface interne et la formation d'une couche continue de silice.

  16. In situ and glancing angle X-ray diffraction of the structure change during and after the high temperature oxidation at 1000 ^{circ}C in air of an yttrium-implanted 304 steel

    Science.gov (United States)

    Riffard, F.; Buscail, H.; Caudron, E.; Cueff, R.; Rabaste, F.; Issartel, C.

    2002-07-01

    A study has been made of the initial stages of oxidation of yttrium ion implanted 304 steel in an attempt to shed some light on the so-called `reactive element effect' observed on chromia forming alloys. In situ XRD was used in order to observe the initial local structural changes during the high temperature oxidation process, while glancing angle XRD was used to monitor the longer range structural transformations taking place near the surface after cooling of oxidised samples. The treatment by ion implantation promotes the intensive establishment of the chromic layer, which leads to the inhibition of the formation of iron rich oxides, by preventing the diffusion of iron through the more compact and more uniform layer initially formed than it would be in the absence of yttrium. However, the identification of yttried phases could not be established. L'effet bénéfique d'un traitement par implantation ionique d'yttrium sur un acier 304 a été mis en évidence par le suivi d'analyses in situ développées par diffraction des rayons X au cours de son oxydation isotherme à 1000^{circ}C sous air. Malgré l'altération superficielle du substrat, le traitement par implantation ionique favorise l'établissement accéléré de la couche de chromine et inhibe la formation au cours du processus d'oxydation des oxydes contenant du fer, réputés peu protecteurs, car faiblement adhérents. Des analyses conduites après refroidissement des échantillons oxydés par diffraction des rayons X sous incidence rasante révèlent la localisation de la structure spinelle au manganèse à l'interface le plus externe. L'identification de phases yttriées n'a cependant pu être établie.

  17. 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.

  18. Polymer-Surfactant Mono and Bilayers Monocouches et bicouches de polymères et de tensioactifs

    Directory of Open Access Journals (Sweden)

    Langevin D.

    2006-12-01

    éthylsiloxane étalé sur des monocouches de tensioactifs de différentes longueurs de chaînes. La pénétration des chaînes de polymères dans la couche de tensioactifs a été étudiée à l'aide de différentes techniques (tension superficielle, ellipsométrie, réflectivité de neutrons. Des exemples de la deuxième situation seront donnés à l'aide de deux polyélectrolytes (polystyrène sulfonate PSS and polyacrylamide sulfonate PAMPS et différents tensioactifs (ioniques et non-ioniques. Les mesures de tension superficielles de solutions diluées de tensioactif non ionique (C10E5 montrent qu'il existe une forte interaction avec le PSS et pas d'interaction avec le PAMPS. Cet effet est probablement dû au caractère hydrophobe plus important du squelette de la chaîne de PSS. Des expériences de diffraction des rayons X et de microscopie électronique sur des phases lamellaires en présence de PSS montrent que les chaînes de polymères sont imbriquées à l'intérieur de la bicouche de tensioactifs. Les interactions entre PSS et PAMPS avec des tensioactifs cationiques (DTAB de charges opposées ont aussi été étudiées par tension superficielle, ellipsométrie et appareil de force de film mince. Un complexe de surface étendu polymère/tensioactif se forme et un phénomène de stratification est observé dans les films minces formés à partir des solutions.

  19. 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.

  20. 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.

  1. Inflammation-adapted liver stiffness values for improved fibrosis staging in patients with hepatitis C virus and alcoholic liver disease.

    Science.gov (United States)

    Mueller, Sebastian; Englert, Stefan; Seitz, Helmut K; Badea, Radu I; Erhardt, Andreas; Bozaari, Bita; Beaugrand, Michel; Lupșor-Platon, Monica

    2015-12-01

    It is well known that inflammation increases liver stiffness (LS) in patients with chronic hepatitis C (HCV) and alcoholic liver disease (ALD) independent of fibrosis stage, but no inflammation-adapted cut-off values have been settled so far. An early identification of rapid fibrosers, however, is essential to decide whom to treat first with the novel but expensive antiviral drugs. Liver stiffness, biopsy-proven fibrosis stages F0-F4 (METAVIR or Kleiner score) and routine laboratory parameters were studied in 2068 patients with HCV (n = 1391) and ALD (n = 677). Among the routine parameters for liver damage, AST correlated best with LS (HCV: r = 0.54, P liver diseases. Especially in HCV, they could help to decide whom to treat first with the novel but expensive antiviral drugs. © 2015 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  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. Neoplasias mieloproliferativas: revisão dos critérios diagnósticos e dos aspectos clínicos

    OpenAIRE

    Chauffaille,Maria de Lourdes L. F.

    2010-01-01

    As síndromes mieloproliferativas crônicas, atualmente denominadas neoplasias mieloproliferativas (NMP), de acordo com a 4ª. edição da classificação da Organização Mundial da Saúde (OMS), são doenças clonais de célula-tronco hematopoética, nas quais há a proliferação aumentada de uma ou mais das séries mieloides (granulocítica, eritrocítica, megacariocítica ou mastocítica) com maturação eficaz. A progressão de todas é caracterizada por fibrose medular ou transformação leucêmica. Pela classific...

  4. [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.

  5. 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.

  6. Estudo comparativo da reação inflamatória e da deposição de fibras colágenas induzidas por biopolímero da cana-de-açucar e polipropileno

    OpenAIRE

    Silveira, Arlon Breno Figueiredo Nunes da

    2014-01-01

    Introdução: O Sling é considerado padrão ouro para o tratamento da Incontinência Urinária de Esforço. Essa técnica cirúrgica utiliza um material sintético no espaço suburetral para prover um arcabouço de fibrose e corrigir a perda urinária. A partir do melaço da cana-de-açúcar, por síntese da bactéria Zoogloea sp., obtivemos o biopolímero da cana-de-açúcar que pode ser processado em forma de membrana. Nosso objetivo é avaliar a ação do biopolímero da cana-de-açúcar comparando-o...

  7. Alterações celulares, moleculares e funcionais de fígados de ratas Wistar tratadas com fatores hepatotróficos

    OpenAIRE

    Thiago Pinheiro Arrais Aloia

    2010-01-01

    Fatores hepatotróficos (FH) possuem a capacidade de promover aumento de massa hepática em ratos e diminuição da fibrose em animais cirróticos. Os FH podem ser importantes nos casos de ressecção e transplantes hepáticos no qual o fígado remanescente necessita de um volume considerável para exercer suas funções após a cirurgia. Objetivou-se neste trabalho avaliar a cinética de uma solução de FH em fígados de animais sadios. Utilizou-se 105 ratos Wistar fêmeas divididos em 7 grupos de 15 animais...

  8. 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

  9. 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...

  10. 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.

  11. 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.

  12. Medical image of the week: superior vena cava syndrome

    Directory of Open Access Journals (Sweden)

    Ateeli H

    2016-08-01

    Full Text Available Superior vena cava (SVC syndrome results from obstruction of blood flow in the SVC. Most cases are secondary to malignancy, the most common being lung cancer or non-Hodgkin lymphoma. Other less encountered etiologies include fibrosing mediastinitis and thrombosis associated with intravascular devices (1,2. Here, we present a case of advanced lung cancer undergoing chemo-radiation therapy who presented with typical symptoms of SVC syndrome including progressive shortness of breath and facial swelling/ fullness over weeks to months. His chest CT scan showed SVC obstruction due to his tumor mass (Figure 1. The patient underwent stenting and improved partially for sometime. However, he returned again with worsening symptoms over a few weeks with discovery of SVC in-stent thrombosis. He was started on therapeutic enoxaparin and his symptoms improved partially with time.

  13. Paracoccidioidomicose pulmonar assintomática e regressiva, com posterior disseminação: relato de um caso

    Directory of Open Access Journals (Sweden)

    Bodo Wanke

    1983-09-01

    Full Text Available É relatado o caso de um homem aparentemente sadio no qual uma abreugrafia de rotina evidenciou lesões pulmonares. Enquanto se buscava a etiologia, as lesões radiológicas foram regredindo espontaneamente até restar apenas fibrose. Por apresentar linhas de precipitação específicas frente à paracoccidioidina na prova de imunodifusão dupla em gel de agar, o paciente foi revisto 3 meses após. Nesta ocasião, persistindo a positividaàe da sorologia e a radiografia de tórax normal, surgiu uma lesão labial, na qual, ao exame microscópico, foi possível demonstrar a presença do Paracoccidioides brasiliensis. Os autores ressaltam as dificuldades diagnosticas do caso e o correlacionam com as formas clinicas da doença.

  14. Bone marrow stroma in idiopathic myelofibrosis and other haematological diseases. An immunohistochemical study

    DEFF Research Database (Denmark)

    Lisse, I; Hasselbalch, H; Junker, P

    1991-01-01

    Bone marrow stroma was investigated immunohistochemically in 31 patients with haematological diseases, mainly idiopathic myelofibrosis (n = 8) and related chronic myeloproliferative disorders (n = 14). The bone marrow from patients with idiopathic myelofibrosis and some CML patients showed marked...... staining reactions with antibodies against type III procollagen (pN collagen), type IV collagen, fragment P1 of laminin and factor VIII. Patients with osteomyelosclerosis had particularly increased collagen content, including both newly deposited type III collagen (pN collagen) and mature collagen fibres....... As in normal bone marrow, argyrophilic fibres and type III collagen displayed a close co-distribution, which was also demonstrated for type IV collagen and laminin. While normal bone marrow sinusoids had discontinuous basement membranes, fibrosing bone marrow was characterized by endothelial cell proliferation...

  15. 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.

  16. Implicações do consumo de fibras alimentares na doença hepática gordurosa não alcoólica de adultos em programa para modificação do estilo de vidaImplicações do consumo de fibras alimentares na doença hepática gordurosa não alcoólica de adultos em programa para modificação do estilo de vida

    OpenAIRE

    Ramos, Fernanda Maria Manzini [UNESP

    2016-01-01

    A Doença hepática gordurosa não alcoólica (DHGNA) se refere ao acúmulo de gordura hepática e pode evoluir para esteatohepatite, fibrose, cirrose e hepatocarcinoma. O principal fator que leva ao acúmulo de gordura hepática é a obesidade, principalmente visceral. Para o controle da doença deve-se focar na mudança do estilo de vida com associação entre prática de exercício físico e intervenção nutricional. Esse estudo teve como objetivo avaliar o consumo adequado de fibras associado a programa s...

  17. Dopplerfluxometria da veia hepática em pacientes com esteatose não alcoólica

    OpenAIRE

    Borges, Valéria Ferreira de Almeida e; Diniz, Angélica Lemos Debs; Cotrim, Helma Pinchemel; Rocha, Haroldo Luis Oliva Gomes; Salomão, Frederico Chaves

    2011-01-01

    OBJETIVO: Avaliar a correlação entre a dopplerfluxometria da veia hepática direita e o grau de esteatose, inflamação e fibrose à biópsia na doença hepática gordurosa não alcoólica. MATERIAIS E MÉTODOS: Foi realizada ultrassonografia com Doppler em 80 pacientes, sendo 40 portadores de doença hepática gordurosa não alcoólica, também submetidos à biópsia. Quarenta controles normais saudáveis, sem fatores risco para doença hepática gordurosa não alcoólica foram submetidos a ultrassonografia com D...

  18. Chemotherapeutic drug induced pneumonitis

    International Nuclear Information System (INIS)

    Schmidt, M.; Brugger, E.

    1981-01-01

    A series of chemotherapeutic drugs is known to induce interstitial lung disease of letal outcome. Diffuse fibrosing interstitial pneumonias are more frequently observed due to Busulfan, Bleomycin, BCNU or Methotrexat therapy. As well literature as our own investigations demonstrate low sensitivity of X-ray controlls in diagnosing beginning changes. Lung function tests including diffusion capacity analysis are more practicable to recognize early phases of disease. Nevertheless, clinical practice shows patients being moust sensitive in decovering beginning decreases of lung function. Exercise induced dyspnea, raw cough and often fever, dyspnea at rest and finally pulmonary insufficiency will be the climax of symptoms. All patients treated with Busulfan, Bleomycin, BCNU and probably Methotrexat should regulary be controlled by lung function analysis. (orig.) [de

  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)

    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.)

  20. 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.

  1. 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.

  2. Evaluation of surgical margins according to the histological type of basal cell carcinoma.

    Science.gov (United States)

    Godoy, Charles Antonio Pires de; Neta, Alice Lima de Oliveira; Leão, Sofia Silveira de Souza; Dantas, Raul Lima; Carvalho, Valeska Oliveira Fonseca; Silva, Samuel Freire da

    2017-01-01

    Basal cell carcinoma is the most common skin cancer in the world. The aim of this study was to evaluate the surgical margin of basal cell carcinoma and correlate this with its histologic subtype. A retrospective analysis of pathology laboratory records from 1990 to 2000 was performed and the following data was collected: age, sex, race, anatomical location, histological type, and state of the excision margins in 1,428 histopathological reports of basal cell carcinoma. Ages ranged from 6 to 99 years, with an average of 57. There was a slight predominance of lesions in white women patients, and the most common histological subtype was the nodular, followed by the superficial. The most common locations were in the head and neck, with highest prevalence appeared in the nose. Surgical margins revealed a lateral involvement of 20.14% and a deep involvement of 12.47%. The fibrosing basal cell carcinoma is the histological type that most often presented positive surgical margins.

  3. Management of the kidney transplant patient with chronic hepatitis C infection.

    Science.gov (United States)

    Tang, Ignatius Y S; Walzer, Natasha; Aggarwal, Nidhi; Tzvetanov, Ivo; Cotler, Scott; Benedetti, Enrico

    2011-01-01

    Chronic Hepatitis C (HCV) infection is an important cause of morbidity and mortality in patients with end-stage renal disease. Renal transplantation confers a survival advantage in HCV-infected patients. Renal transplant candidates with serologic evidence of HCV infection should undergo a liver biopsy to assess for fibrosis and cirrhosis. Patients with Metavir fibrosis score ≤3 and compensated cirrhosis should be evaluated for interferon-based therapy. Achievement of sustained virological response (SVR) may reduce the risks for both posttransplantation hepatic and extrahepatic complications such as de novo or recurrent glomerulonephritis associated with HCV. Patients who cannot achieve SVR and have no live kidney donor may be considered for HCV-positive kidneys. Interferon should be avoided after kidney transplant except for treatment of life-threatening liver injury, such as fibrosing cholestatic hepatitis. Early detection, prevention, and treatment of complications due to chronic HCV infection may improve the outcomes of kidney transplant recipients with chronic HCV infection.

  4. Pentazocine-induced leg ulcers and fibrous papules

    Directory of Open Access Journals (Sweden)

    De Dipankar

    2007-01-01

    Full Text Available We herein describe a case of 55-year-old farmer, who presented with chronic non-healing ulcers over both shins of 4 years duration. Intravenous drug abuse was suspected due to inability to find any venous access and all peripheral veins being found thickened and fibrosed. There were multiple atrophic scars in linear distribution in all limbs as well as in both groins. In addition there were multiple discrete fibrous papules in linear distribution on both hands, which were more obvious on the left side. The patient denied abusing intravenous drugs. However, his relatives confirmed that he abused pentazocine for almost one year before his chronic pain in abdomen was treated by appendicectomy. With subsequent counseling, it was found that he continued to abuse pentazocine at times even after surgery leading to the non-healing of ulcers.

  5. 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

  6. Chemotherapeutic drug induced pneumonitis

    Energy Technology Data Exchange (ETDEWEB)

    Schmidt, M.; Brugger, E.

    1981-09-01

    A series of chemotherapeutic drugs is known to induce interstitial lung disease of letal outcome. Diffuse fibrosing interstitial pneumonias are more frequently observed due to Busulfan, Bleomycin, BCNU or Methotrexat therapy. As well literature as our own investigations demonstrate low sensitivity of X-ray controlls in diagnosing beginning changes. Lung function tests including diffusion capacity analysis are more practicable to recognize early phases of disease. Nevertheless, clinical practice shows patients being moust sensitive in decovering beginning decreases of lung function. Exercise induced dyspnea, raw cough and often fever, dyspnea at rest and finally pulmonary insufficiency will be the climax of symptoms. All patients treated with Busulfan, Bleomycin, BCNU and probably Methotrexat should regulary be controlled by lung function analysis.

  7. 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.

  8. 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

  9. 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.

  10. 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

  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. 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.

  13. Nephrogenic systemic fibrosis

    Energy Technology Data Exchange (ETDEWEB)

    Martin, Diego R. [Emory University Hospital, Department of Radiology, Atlanta, GA (United States)

    2008-01-15

    Nephrogenic systemic fibrosis (NSF) is a dermatological fibrosing disease with potential systemic manifestations resulting in mild to severe symptoms including joint contractures. NSF can be lethal in a subset of cases. It has been recently observed that almost all patients with NSF have had severe renal insufficiency and have received prior administrations of gadolinium-based contrast agents (GBCA). This review will focus on the specific evidence from a retrospective study performed at our center in combination with the general experience available. Conclusions summarize our current understanding of NSF. Recommendations are provided for use of GBCA in clinical MRI practice. Conclusions and recommendations include that the risk of inducing NSF is related to higher-dose administrations of GBCA and that there are differences in risk related to differences in gadolinium-chelate formulation, with the highest relative risk associated with gadodiamide (Omniscan, Nycomed Amersham, GE Healthcare, Piscataway, N.J.). (orig.)

  14. Influência de polimorfismos em genes de citocinas e de receptores de citocinas na resposta ao tratamento e no grau do dano hepático em pacientes portadores de hepatite C crônica

    OpenAIRE

    Moreira, Sara Tatiana

    2012-01-01

    Resumo: O vírus da hepatite C (HCV) é um patógeno capaz de causar infecção crônica em cerca de dois terços dos indivíduos infectados, conseqüência de suas habilidades em driblar tanto a imunidade inata quanto a adquirida. A fibrose é resultado de injúrias repetitivas causadas aos hepatócitos decorrente da infecção pelo HCV e da ação da resposta imunológica, que levam a uma falha no processo regenerativo e deposição de uma quantidade abundante de matriz extracelular. Citocinas regulam a respos...

  15. 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.

  16. Nephrogenic systemic fibrosis: More questions and some answers

    DEFF Research Database (Denmark)

    Morcos, S.K.; Thomsen, Henrik S.

    2008-01-01

    those data, the prevalence of NSF is seen to be significantly higher after exposure to gadodiamide than any other gadolinium-based agent. Gd-CA are either linear or macrocyclic chelates and are available as ionic or non-ionic preparations. The molecular structure, whether cyclic or linear......-macrocyclic chelate Gd-DOTA and the least stable agents are the non-ionic linear chelates gadodiamide and gadoversetamide. The stability of Gd-CA seems to be an important factor in the pathogenesis of NSF. Gd-CA of low stability are likely to undergo transmetallation and release free Gd ions that may deposit......Nephrogenic systemic fibrosis (NSF) is a fibrosing disorder that may develop in patients who have advanced reduction in renal function. A causal relation between gadolinium (Gd3+)-based contrast agents (Gd-CA) and NSF is probable and is supported by the accumulating data in the literature. From...

  17. 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

  18. 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.

  19. Ledipasvir and Sofosbuvir Plus Ribavirin for Treatment of HCV Infection in Patients With Advanced Liver Disease.

    Science.gov (United States)

    Charlton, Michael; Everson, Gregory T; Flamm, Steven L; Kumar, Princy; Landis, Charles; Brown, Robert S; Fried, Michael W; Terrault, Norah A; O'Leary, Jacqueline G; Vargas, Hugo E; Kuo, Alexander; Schiff, Eugene; Sulkowski, Mark S; Gilroy, Richard; Watt, Kymberly D; Brown, Kimberly; Kwo, Paul; Pungpapong, Surakit; Korenblat, Kevin M; Muir, Andrew J; Teperman, Lewis; Fontana, Robert J; Denning, Jill; Arterburn, Sarah; Dvory-Sobol, Hadas; Brandt-Sarif, Theo; Pang, Phillip S; McHutchison, John G; Reddy, K Rajender; Afdhal, Nezam

    2015-09-01

    There are no effective and safe treatments for chronic hepatitis C virus (HCV) infection of patients who have advanced liver disease. In this phase 2, open-label study, we assessed treatment with the NS5A inhibitor ledipasvir, the nucleotide polymerase inhibitor sofosbuvir, and ribavirin in patients infected with HCV genotypes 1 or 4. Cohort A enrolled patients with cirrhosis and moderate or severe hepatic impairment who had not undergone liver transplantation. Cohort B enrolled patients who had undergone liver transplantation: those without cirrhosis; those with cirrhosis and mild, moderate, or severe hepatic impairment; and those with fibrosing cholestatic hepatitis. Patients were assigned randomly (1:1) to receive 12 or 24 weeks of a fixed-dose combination tablet containing ledipasvir and sofosbuvir, once daily, plus ribavirin. The primary end point was sustained virologic response at 12 weeks after the end of treatment (SVR12). We enrolled 337 patients, 332 (99%) with HCV genotype 1 infection and 5 (1%) with HCV genotype 4 infection. In cohort A (nontransplant), SVR12 was achieved by 86%-89% of patients. In cohort B (transplant recipients), SVR12 was achieved by 96%-98% of patients without cirrhosis or with compensated cirrhosis, by 85%-88% of patients with moderate hepatic impairment, by 60%-75% of patients with severe hepatic impairment, and by all 6 patients with fibrosing cholestatic hepatitis. Response rates in the 12- and 24-week groups were similar. Thirteen patients (4%) discontinued the ledipasvir and sofosbuvir combination prematurely because of adverse events; 10 patients died, mainly from complications related to hepatic decompensation. The combination of ledipasvir, sofosbuvir, and ribavirin for 12 weeks produced high rates of SVR12 in patients with advanced liver disease, including those with decompensated cirrhosis before and after liver transplantation. ClinTrials.gov: NCT01938430. Copyright © 2015 AGA Institute. Published by Elsevier Inc

  20. 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

  1. Clearance of inhaled technetium-99m-DTPA as a clinical index of pulmonary vascular disease in systemic sclerosis

    Energy Technology Data Exchange (ETDEWEB)

    Kon, O.M.; Daniil, Z.; Bois, R.M. du [Royal Brompton Hospital, Interstitial Lung Disease Unit, London (United Kingdom); Black, C.M. [Royal Free Hospital, Dept. of Rheumatology, London (United Kingdom)

    1999-01-01

    This study evaluated the utility of the clearance time of inhaled diethylenetriamine pentaacetate (DTPA) to distinguish pulmonary vascular disease from early fibrosing alveolitis (FA) in patients with systemic sclerosis (SSc) It was hypothesized that this would be preserved in patients with vascular disease compared with FA, despite similar gas-transfer deficits and matching lung volumes, because of the preservation of alveolar epithelial integrity. All patients had SSc and were categorized into a control group (C; n=9), pulmonary vascular group (VAS; n=14) or FA group (n=14) dependent on the appearance on a computed tomography (CT) scan and the transfer factor of the lung for carbon monoxide (TL,CO) (VAS and FA {<=}70%, C {>=}80%). All patients had a forced vital capacity (FVC) of >80%. The TL,CO (median) was similar in the VAS (57.5%) and FA (60%) groups. There was a significant difference in median DTPA clearance half-times between FA (21.25 min) and VAS (46.5 min) (p=0.014) and between FA and C (84.5 min) (p=0.0004). No difference was found between VAS and C (p=0.0778). Follow-up data from the VAS group showed no subsequent development of FA on the CT scan and no decrease in FVC (n=13, mean 42 months). These results suggest that clearance of diethylenetriamine pentaacetate is preserved in patients likely to have pulmonary vascular disease and may be useful in distinguishing fibrosing alveolitis from vascular disease in systemic sclerosis. (au) 22 refs.

  2. 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.

  3. Histopathological approach to patterns of interstitial pneumonia in patient with connective tissue disorders.

    Science.gov (United States)

    Nicholson, Andrew G; Colby, Thomas V; Wells, Athol U

    2002-03-01

    It is well established that some patients with connective tissue disorders will suffer from pulmonary disease at some stage in their disease progression. This article concentrates on the interstitial pneumonias, seen in association with most types of connective tissue disorder, particularly in the ligh of non-specific interstitial pneumonia (NSIP) being recognised as a distinct histological pattern. Most published articles on this subject precede recognition of NSIP and, as such, the relative incidence of patterns of interstitial pneumonia, as defined by the International Consensus Classification Committee for Interstitial Lung Disease (ICCILD), as well as the clinical and prognostic significance of these patterns is undergoing further scrutiny. In this review, the recognised histological patterns, namely usual interstitial pneumonia (UIP), non-specific interstitial pneumonia (NSIP), diffuse alveolar damage (DAD), organising pneumonia (OP), reactive pulmonary lymphoid hyperplasia, desquamative interstitial pneumonia (DIP) and respiratory bronchiolitis-associated interstitial lung disease (RBILD) are reviewed systematically in relation to the various subgroups of connective tissue disorders. As yet, there are few published studies, but current evidence suggests that many cases previously classified as fibrosing alveolitis are likely to show a pattern of NSIP rather than UIP, particularly in relation to systemic sclerosis. The histological pattern of usual interstitial pneumonia, the most frequently seen pattern in biopsies from patients with idiopathic pulmonary fibrosis/cryptogenic fibrosing alveolitis, appears to be comparatively rare. Furthermore, any biopsy showing a combination of histological patterns, a pattern of non-specific interstitial pneumonia or a pattern of lymphoid interstitial pneumonia/follicular bronchiolitis should be thoroughly investigated for a background connective tissue disorder, if previously unsuspected. Finally, the recently published

  4. 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

  5. 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

  6. Les zéolithes: supports des complexes des métaux de transition Using Zeolites As Supports for Transition-Metal Complexes

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    Le Van Mao R.

    2006-11-01

    Full Text Available On met en évidence dans cet article les principales caractéristiques structurales et superficielles des zéolithes qui peuvent être avantageusement utilisées comme catalyseurs acides ou bifonctionnels et comme supports présentant une grande dispersion de métal de transition et d'intéressants effets sélectifs. Les résultats plutôt encourageants, obtenus dans l'hétérogénéisation de certains complexes des métaux de transition sur des polymères organiques ou sur la silice, ont servi à mesurer les progrès réalisés dans le domaine de leur introduction dans les zéolithes : l'intérêt scientifique et pratique d'une telle opération augmenterait considérablement si l'on pouvait « encager s directement ces complexes dans les cavités - de dimensions moléculaires - des zéolithes ou des silicates récemment synthétisés et dont la cristallisation est caractérisée par une cinétique très lente. This article describes the leading structural and superficial properties of zeolites which can be advantageously used as acid or bifunctional catalysts and as supports offering a considerable capacity for dispersing transition metal as well as interesting selective effects.The rather encouraging results obtained in heterogenizing various transition-metal complexes on org anic polymers or on silica have been used to measure the advances made in the field of incorporating them in zeolites. The scientific and pratical impor-tance of such an operation would be considerably enhanced if such complexes could be « encaged » in molecular-size cavities in zeolites or in recently-synthetized silicates whose crystallization is characterized by very slow kinetics.

  7. 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

  8. 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

  9. 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)

  10. 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

  11. Caractérisation des effets de l'implantation ionique dans les alliages super-élastiques nickel titane par diffraction des rayons X

    Science.gov (United States)

    Pelletier, H.; Muller, D.; Grob, J. J.; Mille, P.; Cornet, A.

    2002-07-01

    augmentation importante de la dureté superficielle et du module élastique. La formation de zones amorphes est directement liée aux effets balistiques générant une augmentation de la densité de dislocations et de défauts dans la zone affectée par le traitement.

  12. Caractérisation physique et mécanique de l'acier 4140 nitruré et implanté

    Science.gov (United States)

    Benarioua, Y.; Chicot, D.; Lesage, J.

    2005-05-01

    La nitruration est un procédé thermochimique destiné à améliorer les propriétés chimiques et mécaniques des aciers et des fontes. Les procédés les plus courant sont la nitruration gazeuse, la nitruration en bain de sels et la nitruration ionique par plasma. Cette dernière présente certains avantages par rapport aux deux précédentes, elle est moins polluante, consomme moins d'énergie et permet un traitement local. De manière générale, quel que soit le procédé utilisé, le processus de nitruration des aciers se caractérise par la diffusion d'azote à partir de la surface vers le cœur du matériau et par la formation d'une couche de combinaison superficielle après une période d'incubation. Si le temps du traitement est suffisamment long, on constate un ralentissement de la croissance de la couche de combinaison alors que la diffusion de l'azote se poursuivre vers le cœur de la pièce en formant une solution solide d'insertion dans la nitroferrite ou des précipités de nitrures ou de carbonitrures d'éléments alliés. Pour ce travail et en vue d'améliorer le comportement mécanique des échantillons, les aciers nitrurés ioniquement pour deux taux d'activation ont subi un traitement supplémentaire d'implantation d'ions d'azote. La caractérisation, tant du point de vue microstructurale que mécanique, des couches obtenues à partir de la nitruration ionique avec ou sans implantation ionique a été effectué et les moyens utilisés dans cette étude sont la microdureté, diffraction de rayons X, microscopies optique et électronique à balayage.

  13. Analyse de la dégradation thermique du Poly(éther imide)

    Science.gov (United States)

    Courvoisier, Emilie; Bicaba, Yoann; Colin, Xavier

    2018-03-01

    La dégradation thermique du PEI a été étudiée dans de larges intervalles de température (entre 180 et 250 °C) et de pression partielle d'oxygène (entre 0,21 et 50 bars). Tout d'abord, les mécanismes de vieillissement thermique ont été analysés et élucidés par spectroscopie IRTF et par calorimétrie différentielle (DSC) sur des films de PEI suffisamment minces (entre 10 et 60 μm d'épaisseur) pour s'affranchir totalement des effets de la diffusion d'oxygène. Comme attendu, et par analogie avec d'autres polymères aromatiques de structure chimique similaire, l'oxydation se produit préférentiellement sur les groupes méthyle de l'unité isopropylidène du motif bisphenol A, causant la disparition de leur bande d'absorption IR caractéristique à 2970 cm-1 et la croissance d'une nouvelle bande d'absorption IR centrée à 3350 cm-1 et attribuée aux groupes alcool. De plus, l'oxydation conduit successivement à une prédominance relative des coupures de chaîne (diminution de Tg) et de la réticulation (augmentation de Tg). Enfin, les conséquences de l'oxydation sur les propriétés élastiques ont été analysées et élucidées par micro-indentation sur des sections droites préalablement polies de plaquettes de PEI de 3 mm d'épaisseur. Cependant, l'augmentation du module d'Young dans la couche superficielle oxydée est principalement due à un vieillissement physique.

  14. 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

  15. 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.

  16. Liver morphology with emphasis on bile ducts changes and survival analysis in mice submitted to multiple Schistosoma mansoni infections and chemotherapy Alterações morfológicas hepáticas, com especial ênfase nas alterações dos ductos biliares e an��lise de sobrevivência em camundongos submetidos a infecções múltiplas por S. mansoni e a quimioterapia

    Directory of Open Access Journals (Sweden)

    L. C. da Silva

    1990-10-01

    Full Text Available In an attempt to be as close as possible to the infected and treated patients of the endemic areas of schistosomiasis (S. mansoni and in order to achieve a long period of follow-up, mice were repeatedly infected with a low number of cercariae. Survival data and histological variables such as schistosomal granuloma, portal changes, hepatocellular necrosis, hepatocellular regeneration, schistosomotic pigment, periductal fibrosis and chiefly bile ducts changes were analysed in the infected treated and non treated mice. Oxamniquine chemotherapy in repeatedly infected mice prolonged survival significantly when compared to non-treated animals (chi-square 9.24, p = 0.0024, thus confirming previous results with a similar experimental model but with a shorter term follow-up. Furthermore, mortality decreased rapidly after treatment suggesting an abrupt reduction in the severity of hepatic lesions. A morphological and immunohistochemical study of the liver was carried out. Portal fibrosis, with a pattern resembling human Symmers fibrosis was present at a late phase in the infected animals. Bile duct lesions were quite close to those described in human Mansonian schistosomiasis. Schistosomal antigen was observed in one isolated altered bile duct cell. The pathogenesis of the bile duct changes and its relation to the parasite infection and/or their antigens are discussed.Numa tentativa de estar o mais próximo possível a pacientes infectados e tratados nas áreas endêmicas de esquistosomose (S. mansoni e também para obter um período mais longo de seguimento, camundongos foram repetidamente infectados com um número baixo de cercarias. Dados de sobrevivência e variáveis histológicas tais como granuloma esquistosomótico, alterações portais, necrose hepatocelular, regeneração hepática, pigmento esquistosomótico, fi-brose periductal e principalmente, alterações dos ductos biliares foram analisados nos animais infectados tratados e não tratados

  17. Aborto em bovinos devido à intoxicação por Tetrapterys acutifolia (Malpighiaceae

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    Saulo A. Caldas

    2011-09-01

    Full Text Available Esse estudo teve por objetivo demonstrar experimentalmente que Tetrapterys acutifolia Cav. (fam. Malpighiaceae é capaz de provocar aborto em bovinos e caracterizar as alterações clínico-patológicas nas vacas e nos fetos. Estas plantas são responsáveis por significativo número de mortes em bovinos com mais de um ano de idade, especialmente nos Estados de Rio de Janeiro e São Paulo, mas até agora não havia sido comprovado experimentalmente seu efeito abortivo em bovinos. Os experimentos foram realizados no município de Barra do Piraí, RJ. Quatro vacas de descarte receberam brotos e folhas novas frescas de T. acutifolia, coletadas em propriedades vizinhas, nas doses de 2,5g/kg/dia, 5,0g/kg/dia (2 vacas e 10g/kg/dia, até ocorrer o abortamento. O quadro clínico nas vacas caracterizou-se por arritmia cardíaca, tremores musculares, anorexia, ascite, jugular ingurgitada, edema de peito e barbela e aborto (23-76 dias após o início da ingestão da planta; todas as vacas abortaram. Das quatro vacas apenas uma (a que recebeu 10g/kg/dia morreu 36 dias após o abortamento, com sintomas de insuficiência cardíaca. O exame necroscópico dos fetos/natimortos revelou hidrotórax, hidropericárdio, hidroperitônio e congestão hepática; ao corte do miocárdio, verificaram-se áreas pálidas. No exame histológico havia edema intersticial com fibrose incipiente. Na vaca que recebeu a maior dose e foi a óbito, bem como em outra intoxicada naturalmente, os achados de necropsia foram similares aos observados nos fetos, exceto pela dilatação dos vasos da base do coração e mais acentuada palidez do miocárdio. Observaram-se ainda edema subcutâneo nas regiões cervical e esternal, bem como veias jugulares ingurgitadas. Os achados histopatológicos foram necrose e edema intersticial com acentuada fibrose no miocárdio, espongiose da substância branca do encéfalo e, no fígado, congestão e leve fibrose. Adicionalmente, observou-se na vaca

  18. 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.

  19. 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

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

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    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

  1. According to Hepatitis C Virus (HCV) Infection Stage, Interleukin-7 Plus 4-1BB Triggering Alone or Combined with PD-1 Blockade Increases TRAF1lowHCV-Specific CD8+Cell Reactivity.

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    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

  2. Aspectos epidemiológicos e padrões de lesões hepáticas em 35 surtos de intoxicação por Senecio spp. em bovinos no sul do Rio Grande do Sul Epidemiological aspects and hepatic lesions pattern in 35 outbreaks of Senecio spp. poisoning in cattle in southern Brazil

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    Fabiane B. Grecco

    2010-05-01

    Full Text Available O presente estudo teve como objetivo caracterizar os diferentes padrões morfológicos hepáticos em 59 fígados de bovinos intoxicados por Senecio spp. provenientes de 35 surtos observados na área de influência do Laboratório Regional de Diagnóstico da Faculdade de Veterinária da Universidade Federal de Pelotas (LRD de 2000-2009, dos quais havia registro fotográfico das lesões macroscópicas, fazendo uma relação com as variações epidemiológicas observadas no período. Foram analisadas as variações climáticas referentes à precipitação pluviométrica e temperatura média nas diferentes estações do ano ocorridas neste intervalo de tempo. As alterações hepáticas macro e microscópicas foram agrupadas em padrões morfológicos semelhantes. Foi considerada como critérios de classificação macroscópica a coloração das superfícies capsular e de corte e a presença ou não de nódulos; e como critérios para a classificação histológica a quantidade e distribuição da fibrose, o número de megalócitos por campo de maior aumento, o grau estimado de proliferação de ductos biliares e a presença de hepatócitos vacuolizados. O Padrão 1 caracterizou-se por fígado esbranquiçado, fibrose difusa, acentuada proliferação de células de ductos biliares e megalocitose discreta; o Padrão 2 por presença de nódulos correspondentes a grupos de hepatócitos ou lóbulos hepáticos circundados por tecido conjuntivo fibroso, acentuada proliferação de células de ductos biliares e megalocitose de discreta a moderada; o Padrão 3 por superfície de corte com aspecto macronodular correspondente a lóbulos hepáticos circundados por delgado cordão de tecido conjuntivo, acentuada proliferação de ductos biliares e megalocitose moderada; o Padrão 4 por superfície sem nodulações e com aspecto marmorizado e histologicamente por fibrose em ponte, megalocitose e proliferação de células de ductos de moderadas a acentuadas; o Padr

  3. Foliculite queloidiana

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    Maria Carolina Coelho Gozzano

    2015-10-01

    Full Text Available Introdução: Foliculite queloidiana (FQ é uma doença crônica com presença de inflamação,  cicatriz de folículo piloso e posterior desenvolvimento de pápulas, placas queloidianas e alopecia cicatricial; com episódios de agravamento da inflamação. Afeta principalmente couro cabeludo e nuca. Normalmente acomete homens de ascendência africana e fototipos elevados (Fitzpatrick IV-VI na adolescência e é rara após os 50 anos. Sua causa não é definida; aponta-se a irritação crônica ou oclusão dos folículos  -  devido a práticas de corte de cabelo  -, trauma, fricção, predisposição e fator agravante. A inflamação ativa é manifestada por pápulas, pústulas e eritema; acompanhada de prurido ou dor. A inflamação tende a ser menos ativa, mas as pápulas queloidianas e as placas podem persistir se não tratadas. Achados histológicos: infiltrado inflamatório neutrofílico ou linfoplasmocitário perifolicular e intrafolicular, fibrose cutânea, folículos capilares quebrados cercados por inflamação granulomatosa, formação de abscesso perifolicular e dérmica fibrose. Tratamento é difícil, podendo ser utilizados antibióticos e corticoides intralesionais.  Objetivo:  Relatar caso de FQ.  Metodologia:  Paciente diagnosticada com FQ atendido em serviço ambulatorial.  Relato de caso: Masculino, 33 anos, Fitzpatrick III, com lesões em couro cabeludo e nuca há 2 anos, sem atendimento médico anterior. Exame físico: presença de pápulas e placas queloidianas na nuca, substituindo o folículo piloso, com consequente alopecia cicatricial. Sem queixas de dores ou prurido. Hipótese diagnóstica: FQ Conclusão: É uma doença crônica, de diagnóstico clínico e difícil tratamento. Assim, é importante o diagnóstico precoce para um tratamento adequado e acompanhamento regular da doença, visando a melhor qualidade de vida para o paciente.

  4. Cirrose experimental induzida pela inalação de tetracloreto de carbono: adaptação da técnica e avaliação da peroxidação lipídica

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    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.

  5. 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

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

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    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

  7. Endoprótese revestida de jugular preservada de bovino: estudo comparativo da resposta tecidual em aorta torácica descendente e veia cava inferior de suínos

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    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.

  8. 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

  9. 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

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    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

  10. 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.

  11. 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

  12. Liver histology in co-infection of hepatitis C virus (HCV and Hepatitis G virus (HGV

    Directory of Open Access Journals (Sweden)

    STRAUSS Edna

    2002-01-01

    Full Text Available As little is known about liver histology in the co-infection of hepatitis C virus (HCV and hepatitis G virus (HGV, HGV RNA was investigated in 46 blood donors with hepatitis C, 22 of them with liver biopsy: co-infection HCV / HGV (n = 6 and HCV isolated infection (n = 16. Besides staging and grading of inflammation at portal, peri-portal and lobular areas (Brazilian Consensus, the fibrosis progression index was also calculated. All patients had no symptoms or signs of liver disease and prevalence of HGV / HCV co-infection was 15.2%. Most patients had mild liver disease and fibrosis progression index, calculated only in patients with known duration of infection, was 0.110 for co-infection and 0.130 for isolated HCV infection, characterizing these patients as "slow fibrosers". No statistical differences could be found between the groups, although a lesser degree of inflammation was always present in co-infection. In conclusion co-infection HCV / HGV does not induce a more aggressive liver disease, supporting the hypothesis that HGV is not pathogenic.

  13. Liver pathology of hepatitis C, beyond grading and staging of the disease.

    Science.gov (United States)

    Dhingra, Sadhna; Ward, Stephen C; Thung, Swan N

    2016-01-28

    Liver biopsy evaluation plays a critical role in management of patients with viral hepatitis C. In patients with acute viral hepatitis, a liver biopsy, though uncommonly performed, helps to rule out other non-viral causes of deranged liver function. In chronic viral hepatitis C, it is considered the gold standard in assessment of the degree of necroinflammation and the stage of fibrosis, to help guide treatment and determine prognosis. It also helps rule out any concomitant diseases such as steatohepatitis, hemochromatosis or others. In patients with chronic progressive liver disease with cirrhosis and dominant nodules, a targeted liver biopsy is helpful in differentiating a regenerative nodule from dysplastic nodule or hepatocellular carcinoma. In the setting of transplantation, the liver biopsy helps distinguish recurrent hepatitis C from acute rejection and also is invaluable in the diagnosis of fibrosing cholestatic hepatitis, a rare variant of recurrent hepatitis C. This comprehensive review discusses the entire spectrum of pathologic findings in the course of hepatitis C infection.

  14. MR evaluation of retroperitoneal fibrosis

    Energy Technology Data Exchange (ETDEWEB)

    Kamper, L.; Scharwaechter, C.; Haage, P.; Piroth, W. [HELIOS-KLINIK Wuppertal - Klinikum der Privaten Univ. Witten/Herdecke (Germany). Klinik fuer Diagnostische und Interventionelle Radiologie; Brandt, A.S.; Kukuk, S.; Roth, S. [HELIOS-KLINIK Wuppertal - Klinikum der Privaten Univ. Witten/Herdecke (Germany). Klinik fuer Urologie

    2011-08-15

    Purpose: Retroperitoneal fibrosis (RPF) is a rare fibrosing process. The relation of contrast enhancement within the fibrosis and the psoas muscle was suggested for the evaluation of disease activity and response to treatment, but no standardized method for the quantification of the disease activity is currently available. We introduce an MR parameter for the evaluation of RPF. Materials and Methods: 29 patients with untreated idiopathic RPF were examined before medical therapy and after three months of treatment. MR analyses included quotients of T 1 and T 2-signal intensities between the RPF and the psoas muscle. Furthermore, we examined the dynamic contrast enhancement of the fibrous tissue. The respective data were compared with the extent of the RPF after 3 months. Results: In the follow-up examinations we observed a significant regression in 37.9 % (n = 11) of cases, a mild regression in 48.3 % (n = 14) and a stable extent in 13.8 % (n = 4). Patients with a significantly reduced extent showed the highest mean values for the applied MR quotients in the initial examination. The lowest mean values for the respective parameters were found for the group with a stable extent. Only dynamic enhancement quotients showed statistically significant differences (p = 0.011) between the groups of response. Conclusion: We observed a significant reduction in the fibrous tissue after pharmacological treatment in patients with an elevated dynamic enhancement quotient in the initial examination. (orig.)

  15. 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.

  16. Sclérodermie systémique associée à l’exposition à la silice survenant après une exposition professionnelle à la soudure à l’arc

    Science.gov (United States)

    Alaya, Zeineb; Kalboussi, Houda; Osman, Walid; Naouar, Nader; Zeglaoui, Héla; Bouajina, Elyès

    2016-01-01

    La sclérodermie systémique liée à l’exposition à la silice cristalline peut apparaître chez les personnes utilisant la soudure à l’arc. Une forme diffuse de sclérodermie a été diagnostiquée chez un plombier-soudeur de 57 ans, qui présentait des polyarthralgies inflammatoires, un phénomène de Raynaud, une sclérodactylie, une sclérose cutanée diffuse, des télangiectasies, une atteinte œsophagienne, une hypertension artérielle pulmonaire et une fibrose pulmonaire associées à la présence d’anticorps anti-nucléosomes. Au cours de son activité professionnelle, le patient était fréquemment exposé à des concentrations atmosphériques élevées de silice cristalline lors de la soudure à l’arc. Le diagnostic d’un syndrome d’Erasmus avec une association d’une sclérodermie systémique à une silicose pulmonaire était retenu. Une déclaration en maladie professionnelle au titre du tableau n°17 en Tunisie a été réalisée. PMID:28292033

  17. Severe digital ischemia due to systemic sclerosis successfully treated with bosentan: case report Tratamento de isquemia digital severa em paciente com esclerose sistêmica utilizando bosentana: relato de caso

    Directory of Open Access Journals (Sweden)

    Cesar José Guimarães Soares

    2007-09-01

    Full Text Available Systemic sclerosis (SSc is an autoimmune connective tissue disease characterized by cutaneous and visceral fibrosis and widespread vascular pathology. Raynaud's phenomenon is one of the most common manifestations of SSc. It may lead to complications such as digital ulceration and infarction and its treatment remains elusive. We present the case of a female patient with severe digital ischemia secondary to SSc. Treatment using vasodilators and prostacyclin analogues was ineffective, but the patient experienced a remarkable response to bosentan, a dual endothelin receptor antagonist. This report suggests that endothelin antagonists may benefit patients with SSc and vasculopathy as a major feature of the disease, including limb ischemia.Esclerose sistêmica (SSc é uma doença auto-imune do tecido conjuntivo caracterizada por fibrose cutânea e visceral, além de vasculopatia difusa.Ofenômeno deRaynaud é uma das manifestações mais comuns da SSc, que pode levar a complicações como úlceras digitais e infarto. Seu tratamento ainda é indefinido. Apresentamos o caso de uma paciente com isquemia digital grave secundária a SSc. O tratamento incluindo vasodilatadores e análogos de prostaciclina foi ineficaz, mas a paciente apresentou excelente resposta ao bosentan, um antagonista de receptores de endotelina. Este relato sugere que antagonistas de endotelina podem beneficiar pacientes com SSc e vasculopatia como principal característica da doença, incluindo isquemia de extremidades.

  18. Characterization of Novel Non-Invasive Biomarkers for the Evaluation of Extracellular Matrix Remodeling in Renal Fibrosis

    DEFF Research Database (Denmark)

    Rasmussen, Daniel Guldager Kring

    begge har en stigende forekomst i de industrialiserede lande. Patienter med kronisk nyresygdom har en forhøjet risiko for negative hændelser såsom kardiovaskulær sygdom og mortalitet. Raten hvorved sygdommen forværres varierer meget mellem patienter og de forhåndenværende kliniske karakteristika er ikke...... tilstrækkelige for fyldestgørende risiko stratificering. Der er et akut behov for biomarkører, som kan måle sygdomsbyrden og forudsige negative hændelser i patienter med kronisk nyresygdom. Egenskaben til at identificere undergrupper af patienter med kronisk nyresygdom med forskellige hastigheder for forværring...... som surrogat endepunkt for effekt af behandlingen. Uanset den underliggende årsag udvikles kronisk nyresygdom grundet vedvarende sårheling. Dette fører til en ubalanceret omsætning af komponenter fra den extracellulære matrix (ECM) hvilket i sidste ende fører til fibrose i nyrerne og destruktion af...

  19. 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)

  20. Improving imaging of the air-liquid interface in living mice by aberration-corrected optical coherence tomography (mOCT) (Conference Presentation)

    Science.gov (United States)

    Schulz-Hildebrandt, Hinnerk; Sauer, Benjamin; Reinholz, Fred; Pieper, Mario; Mall, Markus; König, Peter; Huettmann, Gereon

    2017-04-01

    Failure in mucociliary clearance is responsible for severe diseases like cystic fibroses, primary ciliary dyskinesia or asthma. Visualizing the mucous transport in-vivo will help to understanding transport mechanisms as well as developing and validating new therapeutic intervention. However, in-vivo imaging is complicated by the need of high spatial and temporal resolution. Recently, we developed microscopy optical coherence tomography (mOCT) for non-invasive imaging of the liquid-air interface in intact murine trachea from its outside. Whereas axial resolution of 1.5 µm is achieved by the spectral width of supercontinuum light source, lateral resolution is limited by aberrations caused by the cylindric shape of the trachea and optical inhomogenities of the tissue. Therefore, we extended our mOCT by a deformable mirror for compensation of the probe induced aberrations. Instead of using a wavefront sensor for measuring aberrations, we harnessed optimization of the image quality to determine the correction parameter. With the aberration corrected mOCT ciliary function and mucus transport was measured in wild type and βENaC overexpressing mice, which served as a model for cystic fibrosis.

  1. 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.)

  2. 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.

  3. [Stenosis of the terminal ileum. Endometriosis as the differential diagnosis of Crohn's disease].

    Science.gov (United States)

    Körber, J; Grammel, S; Lobeck, H; Weidemann, H

    1997-07-25

    A 25-year-old woman had for 5 years complained of lower and mid-abdominal pain, at first monthly but later continuous and gradually increasing in severity. At the same time she had diarrhoea associated with nausea, vomiting and weight loss. Physical examination showed a markedly distended abdomen, diffuse pain on pressure over the whole abdomen, most marked in the left middle and lower part, and high pitched peristalsis, but was otherwise unremarkable. Abdominal radiography indicated small-intestinal ileus, while ultrasound revealed absent peristalsis in the small intestine and a 10 cm stenosis in the terminal ileum. Coloscopy demonstrated a fibrosed stenosis of the terminal ileum but a biopsy showed no specific changes. Under suspicion of Crohn's disease with stenosis of the terminal ileum a partial resection of the ileum was performed. The ileum was on inspection thickened with scar tissue but no inflammation. Histology revealed florid chronic mucosal and submucosal inflammation, haemorrhages and ulcers, as well as numerous islands of endometriosis in the subserosa and muscularis propria. Endometriosis is a rare disease in women of the reproductive age, but should be considered in the differential diagnosis of Crohn's disease of the terminal ileum.

  4. 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

  5. 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.

  6. Periostin differentially induces proliferation, contraction and apoptosis of primary Dupuytren’s disease and adjacent palmar fascia cells

    Science.gov (United States)

    Vi, Linda; Feng, Lucy; Zhu, Rebecca D.; Wu, Yan; Satish, Latha; Gan, Bing Siang; O’Gorman, David B.

    2016-01-01

    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. Periostinwas 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. PMID:19619531

  7. 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

  8. Un cas de textilome renal simulant une tumeur de découverte anatomopathologique

    Directory of Open Access Journals (Sweden)

    K. Doh

    2017-12-01

    Conclusion: Les fibres de textile peuvent déclencher une réaction inflammatoire aiguë avec formation d’un abcès périrénal ou chronique en cas d’asepsie entrainant la constitution de foyers de fibrose, d’enkystement et de calcification. Le textilome rénal pose des difficultés diagnostiques à cause de l’absence de signes cliniques et radiologiques pathognomoniques. La présence d’une masse rénale associée à un antécédent de chirurgie rénale du coté homologue doit faire évoquer en premier un textilome. Le traitement est chirurgical. Une néphrectomie partielle sera le traitement de choix. Le meilleur traitement reste toutefois préventif par le compte minutieux des compresses, et des champs opératoires en début et en fin d’intervention.

  9. 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.

  10. 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

  11. 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.

  12. The insulin response integrates increased TGF-β signaling through Akt-induced enhancement of cell surface delivery of TGF-β receptors

    Science.gov (United States)

    Budi, Erine H.; Muthusamy, Baby Periyanayaki; Derynck, Rik

    2015-01-01

    Increased activity of transforming growth factor β (TGF-β), which binds to and stimulates cell surface receptors, contributes to cancer progression and fibrosis by driving epithelial cells toward a migratory mesenchymal phenotype and increasing the abundance of extracellular matrix proteins. The abundance of TGF-β receptors at the cell surface determines cellular responsiveness to TGF-β, which is often produced by the same cells that have the receptors, and thus serves as an autocrine signal. We found that Akt-mediated phosphorylation of AS160, a RabGAP [guanosine triphosphatase (GTPase)-activating protein] promoted the translocation of TGF-β receptors from intracellular stores to the plasma membrane of mouse embryonic fibroblasts (MEFs) and NMuMG epithelial cells. Consequently, insulin, which is commonly used to treat hyperglycemia and activates Akt signaling, increased the amount of TGF-β receptors at the cell surface, thereby enhancing TGF-β responsiveness. This insulin-induced increase in autocrine TGF-β signaling contributed to insulin-induced gene expression responses, attenuated the epithelial phenotype, and promoted the migration of NMuMG cells. Furthermore, the enhanced delivery of TGF-β receptors at the cell surface enabled insulin to increase TGF-β-induced gene responses. The enhancement of TGF-β responsiveness in response to Akt activation may help to explain the biological effects of insulin, the progression of cancers in which Akt is activated, and the increased incidence of fibroses in diabetes. PMID:26420907

  13. Biopsia hepática em cães: relação entre qualidade da amostra e grau de conclusão do diagnóstico

    Directory of Open Access Journals (Sweden)

    Tostes R.A.

    2002-01-01

    Full Text Available O presente estudo objetivou analisar a qualidade do diagnóstico de biopsias colhidas com agulhas tipo aspirativa e cortante, comparando-as ao exame histológico post-mortem do fígado de cães portadores de afecções hepáticas. Foram utilizados 60 cães, sem distinção de sexo, raça ou idade. As biopsias foram procedidas com agulhas tipo MenghiniÒ (de natureza aspirativa e agulhas tipo Tru-CutÒ (de natureza cortante. As biopsias foram colhidas utilizando-se a técnica percutânea transabdominal. O diagnóstico referente à agulha tipo Tru-CutÒ não diferiu do diagnóstico referente à agulha tipo MenghiniÒ. O referente à agulha tipo Tru-CutÒ foi o que apresentou maior índice de concordância, 88,3% de acertos. A agulha tipo Tru-CutÒ apresentou excelentes resultados independente da natureza das lesões, enquanto que a agulha tipo MenghiniÒ falhou nos casos de fibrose.

  14. The Diagnosis and Treatment of Hair and Scalp Diseases.

    Science.gov (United States)

    Wolff, Hans; Fischer, Tobias W; Blume-Peytavi, Ulrike

    2016-05-27

    Hair loss is caused by a variety of hair growth disorders, each with its own pathogenetic mechanism. This review is based on pertinent articles retrieved by a selective search in PubMed, on the current German and European guidelines, and on the authors' clinical and scientific experience. Excessive daily hair loss (effluvium) may be physiological, as in the postpartum state, or pathological, due for example to thyroid disturbances, drug effects, iron deficiency, or syphilis. Androgenetic alopecia generally manifests itself in women as diffuse thinning of the hair over the top of the scalp, and in men as receding temporal hairlines and loss of hair in the region of the whorl on the back of the head. Alopecia areata is patchy hair loss arising over a short time and involving the scalp, eyebrows, beard, or entire body. The hair loss of alopecia areata is reversible in principle but hard to treat. Folliculitis decalvans is a form of alopecia with scarring, characterized by inflamed papules, pustules, and crusts at the edges of the lesions. Lichen planopilaris generally presents with small patches of baldness, peripilar erythema, and round areas of skin scaling. Kossard's frontal fibrosing alopecia is characterized by a receding hairline and loss of eyebrows. Hair loss is a symptom, not a diagnosis. The pathogenesis of the alopecias involves a range of genetic, endocrine, immune, and inflammatory processes, each of which calls for its own form of treatment.

  15. Leptospira spp. infection in wild ruminants: a survey in Central Italian Alps

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    Elena Andreoli

    2014-12-01

    Full Text Available Leptospirosis is an important zoonotic disease diffused worldwide, and wildlife species are commonly considered to be important epidemiological carriers. Four-hundred and forty‑one serological and 198 renal samples from red deer, roe deer and chamois collected in the Province of Sondrio were analysed using the microscopic agglutination test and histopathologic examination. Positive serological findings were found only in 15 red deer and 19 positive serologic reactions were recorded. The most frequent serovars were Bratislava and Grippotyphosa, followed by Pomona, Hardjo and Copenhagheni. Twenty-two per cent of renal samples from seropositive red deer were affected by mild to moderate multifocal chronic lymphoplasmacytic and fibrosing tubulo-interstitial nephritis, mainly involving the cortical parenchyma. In this study, antibodies to Leptospira spp. were infrequent in wild ruminants, and only red deer seemed to be sensitive to the infection. Given the low presence and the fact that there was no record of Leptospira spp. infections in cattle, sheep, goats and also hunters in area during the study period, wild ruminants in Alpine environments cannot be considered as reservoirs or important sources of Leptospira spp. infection for humans or domestic animals.

  16. A entomoftorose nasal

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    Zilton A. Andrade

    1972-10-01

    Full Text Available São apresentados os dados clínico-patológicos de dois casos de entomoftorose nasal, nova doença humana causada por um ficomiceto - Entomophthora coronata. Os pacientes, uma menina e um homem, com 8 e 44 anos de idade respectivamente, apresentaram doença localizada, com nódulos no nariz e região paranasal, edema e deformação da face. O parasito foi isolado do primeiro caso, mas todas as tentativas para isolá-lo no segundo caso resultaram negativas. Histologicamente, havia reação granulomatosa, fibrose e edema, em torno de hifas não septadas, as quais exibiam envólucro eosinófilo amorfo em torno. Foram demonstrados anticorpos circulantes contra, as hifas do E. coronata no soro de um dos pacientes. O material eosinófilo em torno das hifas continha fibrina e material auto-fluorescente sob luz ultra-violeta, provavelmente lipofuscina, mas não foram demonstrados anticorpos ou antígenos nesta área. A apresentação destes casos, os primeiros a serem descritos no Brasil, é acompanhado, de uma revisão geral do assunto, pois tal poderá vir a ser de interesse para aqueles que estudam os problemas da patologia tropical em nosso meio.

  17. 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.

  18. 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

  19. Lichen planus pigmentosus and its variants: review and update.

    Science.gov (United States)

    Robles-Méndez, Juan Carlos; Rizo-Frías, Paulina; Herz-Ruelas, Maira Elizabeth; Pandya, Amit G; Ocampo Candiani, Jorge

    2018-05-01

    Lichen planus pigmentosus (LPP) is considered a rare variant of lichen planus (LP). It is characterized by acquired dark brown to gray macular pigmentation located on sun-exposed areas of the face, neck, and flexures, commonly found in dark-skinned patients. In patients with LPP, an inflammatory lichenoid response results in marked pigmentary incontinence. It has been associated with hepatitis C virus, sun exposure, and contactants such as mustard oil and nickel. LPP-inversus affects fair and dark skin, predominantly involving flexural and intertriginous areas, while sun-exposed areas are spared; friction is an associated trigger. LPP along Blaschko's lines has been associated with susceptibility to genetic mosaicisms. LPP can present concomitantly with other variants of LP such as frontal fibrosing alopecia, as well as endocrinopathies, and autoimmune diseases. Treatment is difficult and consists of avoidance of triggers and topical and systemic medications in order to stop the inflammatory reaction and reduce pigmentation, improving aesthetic appearance and quality of life. © 2017 The International Society of Dermatology.

  20. 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

  1. Factors Promoting Development of Fibrosis in Crohn’s Disease

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    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.

  2. 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.

  3. Síndrome de Young: infecções respiratórias de repetição e azoospermia

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    Balbani A.P.S.

    2000-01-01

    Full Text Available INTRODUÇÃO: A síndrome de Young é uma variante da discinesia ciliar primária, caracterizada pela ocorrência de infecções respiratórias de repetição e obstrução congênita do epidídimo. APRESENTAÇÃO DO CASO: Os autores apresentam um caso de rinossinusite e pneumonias de repetição em um paciente de 28 anos do sexo masculino. Dosagem de sódio e cloro no suor e pesquisa de imunodeficiências celulares e humorais resultaram negativas. O espermograma revelou azoospermia, embora a espermatogênese estivesse mantida, conforme achado na biópsia de testículo. DISCUSSÃO: O diagnóstico foi de síndrome de Young, sendo este o primeiro caso relatado no Brasil. CONCLUSÃO: Os autores alertam para a importância desse diagnóstico, dadas suas implicações para aconselhamento genético, além do diagnóstico diferencial a ser feito com a fibrose cística.

  4. Pulmonary fibrosis, part II: state-of-the-art patient management.

    Science.gov (United States)

    Meyer, Keith C

    2017-05-01

    While many pharmacologic therapies for the treatment of idiopathic pulmonary fibrosis (IPF) have been evaluated via randomized, placebo-controlled clinical trials (RCTs) conducted over the past two decades, most therapies have been shown to be ineffective or even potentially harmful. However, a number of recently completed RCTs have shown significant efficacy for pirfenidone and nintedanib for the treatment of IPF. Areas covered: This manuscript reviews recent advances in the management of IPF and other forms of fibrosing interstitial lung disease (ILD) with an emphasis on IPF. The material upon which this discussion is based was obtained from various published texts and manuscripts identified via literature searching (e.g. PubMed). Expert commentary: Anti-fibrotic drugs are now available for clinical use and perceived as standard-of-care therapies that have the potential to blunt disease progression for many patients with IPF. However, these agents do not necessarily stop disease progression or have a significant impact on mortality, and more effective pharmacologic therapies are needed for patients with IPF. Additionally, whether anti-fibrotic agents can be effective therapies for other forms of pulmonary fibrosis, which often have radiologic and histopathologic manifestations that mimic IPF, is being evaluated in a number of RCTs.

  5. Histopathology of 460 liver allografts removed at retransplantation: a shift in disease patterns over 27 years.

    Science.gov (United States)

    Neves Souza, Lara; de Martino, Rodrigo Bronze; Sanchez-Fueyo, Alberto; Rela, Mohamed; Dhawan, Anil; O'Grady, John; Heaton, Nigel; Quaglia, Alberto

    2018-02-25

    Approximately 10-19% of liver transplant recipients develop irreversible graft failure requiring retransplantation. We reviewed the histology of failed grafts removed at retransplantation in our centre over 27 years. 276 adults and 118 children underwent retransplantation from 1987-2014, receiving 321 and 139 liver grafts, respectively. We analysed graft histology, recipient demographics, indications for transplantation and retransplantation and time interval to retransplantation. We divided retransplantation in 3 eras: A (1987-1994); B (1995-2001) and C (2002-2014). 3298 adult and 938 paediatric primary liver transplants were conducted in our centre, and 8.4% of adults and 12.6% of children experienced retransplantation. Considering the changes throughout the eras, the proportion of chronic rejection declined, whilst that of unexplained chronic fibrosing hepatitis increased steadily, representing the main reason for retransplantation conducted >10 years after primary transplant in children, and second in adults in the most recent era. This chronic hepatitis of the graft might correspond to a slowly-evolving form of rejection, possibly with a humoral component, associated with progressive graft fibrosis and eventually failure. we observed a shift in histopathology of failed liver grafts, with increasing relevance of chronic idiopathic hepatitis associated with progressive fibrosis and graft failure. This article is protected by copyright. All rights reserved. This article is protected by copyright. All rights reserved.

  6. Is IgG4-Related Disease a Cause of Xerostomia? A Cohort Study of 60 Patients

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    M. Hermet

    2012-01-01

    Full Text Available Objective. Immunoglobulin-G4-(IgG4- related disease (IgG4 RD is a fibrosing process characterized by a significant infiltration of IgG4-secreting plasma cells. IgG4 RD can affect almost all organs including salivary glands. Whether IgG4 RD plays a role in the development of sicca syndrome and particularly dry mouth syndrome remains to be investigated. Methods. We conducted a monocentric cohort study for two years to search for IgG4 RD features in patients with dry mouth syndrome using immunostainings of labial salivary gland specimens with anti-IgG4 antibody. Results. Among 60 patients presenting with dry mouth syndrome who underwent labial salivary gland biopsy, 18 showed positive immunostaining with the anti-IgG4 antibody including 4 patients with typical systemic IgG4 RD. Five also fulfilled criteria for Sjögren's syndrome. Conclusion. These findings suggest that clinical forms of IgG4 RD salivary involvement without salivary swelling may occur. This salivary involvement is probably overlooked in everyday practice and could represent a mild form of IgG4 RD.

  7. Adaptação cultural e propriedades psicométricas iniciais do instrumento DISABKIDS ® – Cystic Fibrosis Module – versão brasileira

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    Danielle Maria de Souza Serio dos Santos

    2013-12-01

    Full Text Available Este estudo objetivou adaptar culturalmente e descrever as propriedades psicométricas iniciais do instrumento de mensuração de qualidade de vida relacionada à saúde DISABKIDS® − Cystic Fibrosis Module para crianças e adolescentes e seus pais/cuidadores. Estudo metodológico de desenvolvimento sequencial, incluindo 126 participantes em quatro estados brasileiros. Envolveu tradução e retrotradução dos itens, equivalência conceitual e semântica, validade de face e descrição das propriedades psicométricas iniciais relacionadas ao construto e fidedignidade . Para equivalência semântica da versão adaptada houve ajustes na redação de um item. O instrumento apresentou consistência interna satisfatória com valores de alfa de Cronbach entre 0,70 e 0,85, validade convergente com valores de correlação acima 0,40 em 85% dos itens e validade divergente com valores de ajuste superiores a 75%. A versão brasileira do DISABKIDS® - CFM certamente se constituirá em um instrumento válido e confiável para a mensuração da qualidade de vida de crianças e adolescentes brasileiros com fibrose cística.

  8. A Rare Case of Non-Small Cell Carcinoma of Lung Presenting as Miliary Mottling

    Directory of Open Access Journals (Sweden)

    Ballaekere Jayaram Subhashchandra

    2013-03-01

    Full Text Available Miliary mottling on chest radiography is seen in miliary tuberculosis, certain fungal infections, sarcoidosis, coal miner’s pneumoconiosis, silicosis, hemosiderosis, fibrosing alveolitis, acute extrinsic allergic alveolitis, pulmonary eosinophilic syndrome, pulmonary alveolar proteinosis, and rarely in hematogenous metastases from the primary cancers of the thyroid, kidney, trophoblasts, and some sarcomas. Although very infrequent, miliary mottling can be seen in primary lung cancers. Herein, we report the case of a 28-year-old female with chest X-ray showing miliary mottling. Thoracic computed tomography (CT features were suggestive of tuberculoma with miliary tuberculosis. CT-guided fine needle aspiration cytology confirmed the diagnosis as lower-lobe, left lung non-small cell carcinoma (adenocarcinoma. It is rare for the non-small cell carcinoma of the lung to present as miliary mottling. The rarity of our case lies in the fact that a young, non-smoking female with miliary mottling was diagnosed with non-small cell carcinoma of the lung.

  9. Indium-doped aluminium oxide as a non-radioactive test aerosol for aerosol experiments

    International Nuclear Information System (INIS)

    Drosselmeyer, E.; Mueller, H.L.; Seidel, A.; Pickering, S.

    1986-01-01

    For testing inhalation facilities it is advantageous to use a non-radioactive, low toxicity test aerosol which can be detected at low concentrations. These criteria are met by a mechanically generated aerosol of indium-doped alumina. Although some cases of lung fibroses have been associated with the inhalation of aluminium compounds in industry, aluminum oxide aerosols are generally considered to be non-toxic. Indium was chosen as a dopant material because (a) it is not normally present in the lung in detectable amounts, (b) it is chemically similar to aluminum and (c) it can be detected in trace amounts by neutron activation analysis (Friberg et al., 1979). Indium aerosols have the same advantages as radioactive tracers for ease of detection, but they are non-toxic during use. This combination of properties offers advantages that could be of use in a wider range of applications than hitherto used, e.g. in inhalation experiments. This paper describes nose-only inhalation experiments on rats using an aerosol of alumina doped with indium. (author)

  10. The key role of exudative lesions and their encapsulation: Lessons learned from the pathology of human pulmonary tuberculosis

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    Pere-Joan eCardona

    2015-06-01

    Full Text Available 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 towards 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 towards 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 towards 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-haematological 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.

  11. ETIOLOGY, PATHOGENESIS AND MORPHOLOGY OF BILIARY ATRESIA

    Directory of Open Access Journals (Sweden)

    O. E. Iryshkin

    2012-01-01

    Full Text Available Biliary atresia is an inflammatory fibrosing process, which invades both extrahepatic and intrahepatic bile ducts. There are two forms of biliary atresia: embryonic form, associated with another visceral malformations, and perinatal form, which is an isolated disease. Biliary atresia is a heterogeinc disease which may appear as a result of different etiological factors, the most important of them are genetics, viral infection, vascular factors, toxins and disregulation of immune system. The characteristic morphological features of biliaryatresia are: intracellular and ductular cholestasis, ductular proliferation, giant cell transformation, portal and perilobular oedema and/or fibrosis. As the patalogical process progresses, ductopenia appears, and fibrosis turns to cirrhosis. Embryonic form mophologically manifests with ductal plate malformation is, what is an important diagnostic criterion. Pro- gnosis of biliary atresia depends on form of disease, age of patient and type of surgical operation. Today surgical treatment includes two steps: palliative portoenterostomia, performed in neonatal period and further liver trans- plantation. However liver transplantation is more preferable as first and single-step treatment. 

  12. [Pulmonary crackles, what does the clinician hear?].

    Science.gov (United States)

    Postiaux, G; Vilaro, J; Charlier, J-L; Marchand, E; Lens, E

    2015-09-01

    The overall duration of a pulmonary crackle is usually less than 20-30 ms but psychoacoustics demonstrates that an acoustical event with a duration of less than 20-40 ms cannot be estimated in terms of pitch and duration. We pose the hypothesis that the main resonant information is contained into the breath sounds following the crackle. Eight patients with COPD, viral pneumonia, bronchiectasis, congestive heart failure, hypoproteinemia and fibrosing alveolitis were recruited for this study. Thirty-six crackles were analyzed in time and frequency domains; 12 in each category of low, medium and high frequencies. The acoustic features of the crackles, their segments (initial deflection width, first cycle duration, two cycles duration, decay segment) and the breath sounds following the crackles were compared. The study confirms the differences between the three crackles categories in time and frequency domains. No statistical differences were found between the decay segments and breath sounds in each category. Breath sounds modified by lung tissue density could be the main resonators determining the fundamental transmission frequencies of crackle signals. Combined acoustic analysis of crackles and breath sounds could replace single analysis of isolated crackles. Copyright © 2014 SPLF. Published by Elsevier Masson SAS. All rights reserved.

  13. Management of the Kidney Transplant Patient with Chronic Hepatitis C Infection

    Directory of Open Access Journals (Sweden)

    Ignatius Y. S. Tang

    2011-01-01

    Full Text Available Chronic Hepatitis C (HCV infection is an important cause of morbidity and mortality in patients with end-stage renal disease. Renal transplantation confers a survival advantage in HCV-infected patients. Renal transplant candidates with serologic evidence of HCV infection should undergo a liver biopsy to assess for fibrosis and cirrhosis. Patients with Metavir fibrosis score ≤3 and compensated cirrhosis should be evaluated for interferon-based therapy. Achievement of sustained virological response (SVR may reduce the risks for both posttransplantation hepatic and extrahepatic complications such as de novo or recurrent glomerulonephritis associated with HCV. Patients who cannot achieve SVR and have no live kidney donor may be considered for HCV-positive kidneys. Interferon should be avoided after kidney transplant except for treatment of life-threatening liver injury, such as fibrosing cholestatic hepatitis. Early detection, prevention, and treatment of complications due to chronic HCV infection may improve the outcomes of kidney transplant recipients with chronic HCV infection.

  14. [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.

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

    Science.gov (United States)

    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 deficiency persistently reduces bleomycin-induced pulmonary fibrosis or merely delays disease progression and whether pharmacological PAR-2 inhibition limits experimental pulmonary fibrosis. Bleomycin was instilled intranasally into wild-type or PAR-2–deficient mice in the presence/absence of a specific PAR-2 antagonist (P2pal-18S). Pulmonary fibrosis was consistently reduced in PAR-2–deficient mice throughout the fibrotic phase, as evident from reduced Ashcroft scores (29%) and hydroxyproline levels (26%) at d 28. Moreover, P2pal-18S inhibited PAR-2–induced profibrotic responses in both murine and primary human pulmonary fibroblasts (p bleomycin-treated wild-type mice but did not further reduce fibrosis in PAR-2–deficient mice. Importantly, P2pal-18S treatment starting even 7 d after the onset of fibrosis limits pulmonary fibrosis as effectively as when treatment was started together with bleomycin instillation. Overall, PAR-2 contributes to the progression of pulmonary fibrosis, and targeting PAR-2 may be a promising therapeutic strategy for treating pulmonary fibrosis. PMID:26147947

  16. Entendendo a classificação, a fisiopatologia e o diagnóstico radiológico das bronquiectasias

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    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

  17. 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.

  18. O pulmão na doença inflamatória do cólon: estudo experimental em ratos

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    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.

  19. Primary Sclerosing Cholangitis

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    A Stiehl

    2000-01-01

    Full Text Available Primary sclerosing cholangitis (PSC is a chronic cholestatic liver disease characterized by fibrosing inflammation and obliteration of intra- and/or extrahepatic bile ducts. The disease is one of the most common cholestatic diseases in adults and is diagnosed with increasing frequency. It is very often associated with ulcerative colitis. Patients with PSC have an increased incidence of bile duct carcinomas, and those with ulcerative colitis also have an increased incidence of colonic carcinomas. In end-stage disease, liver transplantation is the treatment of choice. Immunosuppressive treatment has little effect. Ursodeoxycholic acid (UDCA, which has been shown to improve liver histology and survival in patients with primary biliary cirrhosis, has a beneficial effect in PSC, provided that patients who develop major duct stenoses are treated endoscopically. The aim is to treat patients as early as possible to prevent progression to the advanced stages of the disease. During treatment with UDCA, stenoses of major ducts may develop, and early endoscopic dilation is highly effective. Because UDCA treatment improves but does not cure cholestatic liver diseases, permanent treatment seems to be necessary. Such prolonged treatment with UDCA may be recommended because, until now, no side effects have been reported. In patients with end-stage disease, UDCA is not effective and liver transplantation is indicated.

  20. Experimental Chagas' disease in dogs

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    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.

  1. Exercise reduces airway sodium ion reabsorption in cystic fibrosis but not in exercise asthma.

    Science.gov (United States)

    Schmitt, L; Wiebel, M; Frese, F; Dehnert, C; Zugck, C; Bärtsch, P; Mairbäurl, H

    2011-02-01

    When ventilating large volumes of air during exercise, airway fluid secretion is essential for airway function. Since these are impaired in cystic fibrosis and exercise-induced asthma, it was the aim of this study to determine how exercise affects airway Na(+) and Cl(-) transport and whether changes depend on exercise intensity. Nasal potential was measured in Ringer's solution, with amiloride to block Na(+) transport, and in low chloride-containing isoproterenol to assess Cl(-) channels. Nasal potential was measured at rest and during submaximal and maximal bicycle ergometer exercise in individuals with cystic fibrosis, exercise-induced asthma and controls. At rest, nasal potential was significantly higher in cystic fibroses than in the others. Maximal exercise decreased nasal potentials in cystic fibrosis and controls but not in exercise asthma. Submaximal exercise decreased nasal potentials only in cystic fibrosis. Cl(-) transport was not affected. Our results indicate that nasal potentials and Na(+) transport were decreased by maximal exercise in healthy and cystic fibrosis, whereas submaximal exercise decreased potentials in cystic fibrosis only. Exercise did not affect nasal potentials in asthmatics. Decreased reabsorption during exercise might favour airway fluid secretion during hyperpnoea. This protective effect appears blunted in patients with exercise-induced asthma.

  2. EFEITO DE CÁLCULOS BILIARES HUMANOS NA CAVIDADE PERITONEAL DE RATOS

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    Torres Orlando Jorge Martins

    1998-01-01

    Full Text Available Este estudo tem por objetivo analisar experimentalmente as características macroscópicas e microscópicas de cálculos biliares humanos na cavidade peritoneal de ratos. Foram utilizados 32 ratos Wistar, machos, pesando entre 205 e 268 g. Estes animais foram distribuídos em dois grupos e o procedimento cirúrgico foi realizado em cada grupo: no grupo A (n =16, os animais foram submetidos a manipulação intestinal; no grupo B (n=16, cálculos biliares humanos foram colocados na cavidade peritoneal. Os ratos foram avaliados no 21º e 42º dias do período pós-operatório. Os resultados mostraram que a simples manipulação causou aderências nos animais (n=10. As aderências foram notadas em 11 cálculos no grupo B. Histologicamente não houve fibrose intensa em torno do cálculo, principalmente no 42º dia do período pós-operatório. Não foram observados macro ou micro abscessos e não houve evidências de fluído livre intra-peritoneal. Este estudo demonstra que apesar da baixa incidência de complicações, todas as tentativas devem ser feitas para recuperar cálculos perdidos durante colecistectomia.

  3. Efeitos da intoxicação crônica com o etanol na evolução da Tripanosomíase cruzi experimental no camundongo

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    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.

  4. Persistent lung inflammation and fibrosis in serum amyloid P component (APCs-/- knockout mice.

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    Darrell Pilling

    Full Text Available Fibrosing diseases, such as pulmonary fibrosis, cardiac fibrosis, myelofibrosis, liver fibrosis, and renal fibrosis are chronic and debilitating conditions and are an increasing burden for the healthcare system. Fibrosis involves the accumulation and differentiation of many immune cells, including macrophages and fibroblast-like cells called fibrocytes. The plasma protein serum amyloid P component (SAP; also known as pentraxin-2, PTX2 inhibits fibrocyte differentiation in vitro, and injections of SAP inhibit fibrosis in vivo. SAP also promotes the formation of immuno-regulatory Mreg macrophages. To elucidate the endogenous function of SAP, we used bleomycin aspiration to induce pulmonary inflammation and fibrosis in mice lacking SAP. Compared to wildtype C57BL/6 mice, we find that in Apcs-/- "SAP knock-out" mice, bleomycin induces a more persistent inflammatory response and increased fibrosis. In both C57BL/6 and Apcs-/- mice, injections of exogenous SAP reduce the accumulation of inflammatory macrophages and prevent fibrosis. The types of inflammatory cells present in the lungs following bleomycin-aspiration appear similar between C57BL/6 and Apcs-/- mice, suggesting that the initial immune response is normal in the Apcs-/- mice, and that a key endogenous function of SAP is to promote the resolution of inflammation and fibrosis.

  5. Cystic Shape Cemento-Ossifying Fibroma of Ethmoid Sinus

    Science.gov (United States)

    Shejbal, Dražen; Vonsović, Gabrijela; Baudoin, Tomislav; Vagic, Davor

    2015-01-01

    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. PMID:27688399

  6. 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.)

  7. 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

  8. 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.

  9. A experiência da família ao conviver com a família doença crônica da criança The family experience of living with the child's chronic illness

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    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".

  10. 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.

  11. 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 

  12. Early identification of aortic valve sclerosis using iron oxide enhanced MRI.

    Science.gov (United States)

    Hamilton, Amanda M; Rogers, Kem A; Belisle, Andre J L; Ronald, John A; Rutt, Brian K; Weissleder, Ralph; Boughner, Derek R

    2010-01-01

    To test the ability of MION-47 enhanced MRI to identify tissue macrophage infiltration in a rabbit model of aortic valve sclerosis (AVS). The aortic valves of control and cholesterol-fed New Zealand White rabbits were imaged in vivo pre- and 48 h post-intravenous administration of MION-47 using a 1.5 Tesla (T) MR clinical scanner and a CINE fSPGR sequence. MION-47 aortic valve cusps were imaged ex vivo on a 3.0T whole-body MR system with a custom gradient insert coil and a three-dimensional (3D) FIESTA sequence and compared with aortic valve cusps from control and cholesterol-fed contrast-free rabbits. Histopathological analysis was performed to determine the site of iron oxide uptake. MION-47 enhanced the visibility of both control and cholesterol-fed rabbit valves in in vivo images. Ex vivo image analysis confirmed the presence of significant signal voids in contrast-administered aortic valves. Signal voids were not observed in contrast-free valve cusps. In MION-47 administered rabbits, histopathological analysis revealed iron staining not only in fibrosal macrophages of cholesterol-fed valves but also in myofibroblasts from control and cholesterol-fed valves. Although iron oxide labeling of macrophage infiltration in AVS has the potential to detect the disease process early, a macrophage-specific iron compound rather than passive targeting may be required. (c) 2009 Wiley-Liss, Inc.

  13. 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

  14. Liver and kidney structure and iron content in romanian brown bears (Ursus arctos) before and after hibernation.

    Science.gov (United States)

    Prunescu, Carol- Constantin; Serban-Parau, Nicolae; Brock, Jeremy H; Vaughan, Diane M; Prunescu, Paula

    2003-01-01

    The annual cycle of the brown bear (Ursus arctos) in the Carpathian Mountains (Romania) consists of an active period from April to November, and an inactive period (hibernation) of approximately 4-5 months between November and March. During hibernation, the brown bears sleep continually and do not feed or drink water. Analyses of liver and kidney of male brown bears showed that liver iron content was 3 times higher in bears at the end of hibernation than at the end of the active period. A possible trend towards a decrease in iron content was noted for the kidney. The presence of iron in the liver was confirmed by the presence of the Perls-positive granules in the cytoplasm of Kupffer cells, in other non-parenchymal cells and also in some hepatocytes. The hepatic veins of the bear liver samples obtained in early spring showed narrower lumens with pleated walls, compared to the normal outline of the hepatic veins in the liver from the bears sampled during autumn. Also in the early spring bears, the renal glomeruli were partially fibrosed. Renal glomerular fibrosis was sometimes observed in samples from the prehibernation period. The tissue iron values from the livers and kidneys of brown bears in early spring or autumn might provide useful data on iron metabolism under conditions of hibernation and accompanying starvation.

  15. Função pulmonar na evolução de 35 doentes com paracoccidioidomicose

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    E. P. Campos

    1986-10-01

    Full Text Available Lesões pulmonares observadas na paracoccidioidomicose (pbmicose pela radiologia foram designadas: leve, moderada e grave de acordo com critério estabelecido pelos autores. Lesões infiltrativas intersticiais bilaterais nddulo fibrolineares e cotonosas foram identificadas respectivamente em 34 e em 23 doentes. Formas leve, moderada e grave assinaladas respectivamente em 6 10 e 19 mostraram à análise radiológica evolutiva melhora em 2, piora em 15 e manutenção do padrão da lesão em 18 doentes. Testes de função pulmonar realizados nos doentes durante o retorno ambulatorial evidenciaram: 12 com padrão espirográfico normal, 20 obstrutivos e 3 mistos; 34 doentes estavam hiperventilando e todos apresentaram aumento da diferença alvéolo arterial. Os resultados obtidos permitiram supor que a fibrose residual descrita nos padrões radiológicos; manutenção e piora de 33 deles aliada à doença obstrutiva crônica verificada pelas provas de função pulmonar constituíram subsídios para o desenvolvimento do Cor pulmonale assinalado

  16. Aspectos raciais dos "megas" e da cardiopatia na doença de Chagas crônica

    Directory of Open Access Journals (Sweden)

    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.

  17. 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.

  18. 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.

  19. 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.

  20. Challenging hepatitis C-infected liver transplant patients

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    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

  1. Circumscribed Morphea and Breast Asymmetry in an Adolescent

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    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.

  2. 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)

  3. Les encroûtements sous-marins de Méditerranée orientale : une explication génétique Subsea Incrustations in the Eastern Mediterranean: a Genetic Explanation

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    Allouc J.

    2006-11-01

    Full Text Available Les encroûtements sous-marins ou fonds durcis ne sont pas spécifiques à la Méditerranée, mais ils y sont particulièrement abondants, notamment dans le bassin oriental. Ils tapissent les pentes de reliefs sous-marins et représentent un exemple de diagenèse superficielle liée aux périodes de vacuité sédimentaire. Il existe divers types d'encroûtements qui peuvent être distingués en fonction principalement de l'épaisseur de la partie indurée (appelée calcaire et de la teinte générale du matériau. Le plus souvent le calcaire surmonte une boue encore plus ou moins meuble. L'induration (ou lithification de cette dernière résulte de l'introduction d'un ciment de calcite magnésienne et on peut distinguer différentes étapes du processus d'après la nannostructure du calcaire. Le phénomène semble purement physico-chimique et résulterait de la conjonction de plusieurs facteurs : - présence de courants profonds; - sursaturation suffisamment élevée par rapport à la calcite non magnésienne; - très faibles concentrations en inhibiteurs de croissance cristalline autres que le magnésium. Bien qu'affectant des matériaux d'âges variés, cette forme de diagenèse sous-marine paraît essentiellement quaternaire. D'après les échantillons examinés, les conditions les plus favorables à la genèse des encroûtements apparaîtraient lors des stades climatiques froids (notamment le dernier et plus particulièrement durant leur phase terminale. Subsea incrustations or hardened sea floors are not specific to the Mediterranean, but they are particularly abundant there, especially in the eastern basin. They cover the slopes of subsea relief features and represent an example of surface diagenesis linked to periods of absence of sedimentation. Several types of incrustations can be distinguished, mainly as a function of the thickness of the indurated part (called limestone and the general color of the material. Such limestone usually

  4. Suspension du droit et rétrécissement du politique : apothéose de l’État planétaire

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    Jesús Cuenca De la Rosa

    2003-09-01

    Full Text Available La dernière guerre du Golfe nous aiderait à interpréter mieux que n’importe quel autre signe ce qu’est objectivement ce début de millénaire. Sur le plan utopique (subjectif les idéologies néolibérales sous tutelle américaine tendent à l’universel fixé sur la démocratie et les droits de l’homme partout, simultanément à la planétarisation du marché. Une lecture même superficielle de notre réalité concrète qui tendrait à mettre au clair comment les habitants concrets du monde qui est le nôtre vivent ou survivent – en quoi consiste leur vie d’être vivants, pour reprendre à notre compte l’idée avancée par Michel Foucault à la fin de La volonté de savoir, lorsqu’il affirme que la modernité se caractérise par l’intrusion de la vie naturelle dans le champ politique – ne pourrait qu’infliger à cette belle prétention un ferme démenti. Dans ces conditions, parler toujours de politique en tant que contrat liant librement les pratiques sociales des différents individus des différents pays relève de la fiction.La reciente guerra del Golfo podría proporcionarnos, mejor que ningún otro fenómeno, las claves objetivas para interpretar el comienzo del nuevo siglo. En lo utópico (subjetivo, las variantes ideológicas neoliberales al amparo americano pretenden abarcar lo universal polarizado universalmente en la democracia y los derechos humanos al tiempo que el mercado alcanza una dimensión planetaria.Un análisis, siquiera superficial, de nuestro mundo real con vocación de esclarecer las reales condiciones de vida de los seres reales del mundo que nos ha tocado vivir – en qué consiste su vida como seres vivos, por retomar la idea sugerida por Michel Foucault al final de La voluntad de saber, cuando afirma que la modernidad se caracteriza por la irrupción de la vida natural en el ámbito político – acabaría irremediablemente impugnando con energía tan noble proyecto. En tales condiciones

  5. 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

  6. Analyse des contraintes résiduelles dans les dépôts réalisés par rechargement laser sur alliage d'aluminium

    Science.gov (United States)

    Dubourg, L.; Hlawka, F.; Cornet, A.

    2002-07-01

    Superficial characteristics of a substrate, particularly the hardness, the elastic modulus and the wear resistance can be considerably improved by laser cladding. This high energy process causes thermal stresses, leading to residual stresses and substrate distortions. In this study, laser cladding is carried out onto an aluminium alloy with a Nd:YAG laser and a coaxial injection of Al 50Si powders. Residual stresses are determined by X-ray diffraction and by sample strain measurement. The differences of results between these two ways highlight the influence of the heat-affected zone (HAZ) under the coating. These differences can be explained by a model of three blocks: the coating, the HAZ and the substrate. The experimentation shows an optimal treatment speed, leading to maximum compression stresses interesting for the hardness and the wear resistance. Moreover, at a certain fixed speed, the thermal stresses balance each other in the sample and lead to the no-distortion of the substrate. Par la formation d'un dépôt, le rechargement laser permet d'amé liorer les caractéristiques superficielles d'un substrat, en particulier la dureté, le module d'élasticité et la résistance à l'usure. Ce procédé utilisant un faisceau de haute énergie crée dans le matériau traité des contraintes résiduelles d'origine thermique et des déformations du substrat. Dans cet article, le rechargement laser a été réalisé sur un alliage d'aluminium grâce à un laser Nd YAG et une injection coaxiale de poudres Al 50Si. Les contraintes résiduelles ont été étudiées par diffraction des rayons X et par la méthode de la flèche. Les différences de résultats entre ces deux méthodes ont permis de mettre en évidence l'influence de la zone affectée thermique (ZAT) en dessous du dépôt et ont pu être expliquées à l'aide d'un modèle de blocs correspondant respectivement au dépôt, à la ZAT et au substrat. L'expérimentation a révélé, entre autres, une vitesse de

  7. Muestreo en transecto de formaciones vegetales de fanerófitos y caméfitos (MIFC (II: estudio de los sabinares de la Reserva Biológica de Doñana (RBD (España

    Directory of Open Access Journals (Sweden)

    Cámara Artigas, Rafael

    2013-06-01

    érant des phanérophytes et des chamaephytes (MIFC dans le voisinage des lagunes de Charco del Toro (TCS et Navazo del Toro (NVT. Cinq groupes de genévriers ont été identifiés en fonction de leurs éléments constitutifs et de leurs variables de structure verticale et horizontale (couverture, liés du point de vue géobotanique aux unités de manteaux éoliens de Doñana et au comportement de haute tranmissivité et à la dynamique hydrique du sous-sol des formations superficielles sur lesquelles ils sont distribués. Le groupe considéré comme la formation de genévriers type est situé dans le Haut Manteau Éolien Sec (HMES dans la contrée du genévrier de El Ojillo (Groupe 1 : NVTv 19.

  8. Comparaison de l'altérabilité au brouillard salin des deux pierres calcaires de construction au moyen des mesures pondérales acoustiques et par traitement d'images

    Directory of Open Access Journals (Sweden)

    Birginie, J. M.

    2000-09-01

    Full Text Available Not available

    Se presentan los resultados de un estudio comparativo de la resistencia a la alteración en atmósfera de niebla salina de dos rocas calizas utilizadas en la construcción de monumentos en Francia y en España. Las modificaciones experimentadas por las probetas durante este ensayo se han valorado utilizando tres métodos: la medida de la evolución del peso, la medida de la velocidad de propagación de ultrasonidos y el análisis visual de las modificaciones superficiales por un sistema de barrido mediante luz láser de las superficies (método de triangulación luz láser-cámara y posterior tratamiento de imágenes. La comparación de los resultados obtenidos muestra que los tres métodos ofrecen una información complementaria que permite caracterizar de manera precisa el proceso de alteración y su evolución durante el ensayo. Es, sin embargo, el método de análisis mediante el sistema láser-cámara el que ofrece más posibilidades para describir y comparar de manera objetiva la morfología y la evolución de la desagregación arenosa observada sobre las dos rocas calizas alteradas artificialmente. [fr] Nous présentons les résultats d'une étude comparative de la résistance à l'altération au brouillard salin de deux pierres utilisées dans la construction de monuments en France et en Espagne. Les transformations subies par les échantillons au cours de l'essai de vieillissement ont été évaluées eu utilisant trois méthodes: l'évolution de la masse, la mesure de la vitesse de propagation d'ultrasons et l'analyse visuelle automatisée des désagrégations superficielles au moyen d'un système de balayage par plan laser de la surface (triangulation laser-caméra. La comparaison de l'ensemble de ces résultats démontre que les trois méthodes non destructives fournissent des informations complémentaires qui permettent de caractériser les processus d'altération et leur évolution. C'est néanmoins l

  9. 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 entre os filamentos da tela.

  10. Differences between polydioxanone and poliglactin in intestinal anastomoses – a comparative study of intestinal anastomoses

    Directory of Open Access Journals (Sweden)

    Carlos Henrique Marques dos Santos

    2017-10-01

    laparotomia nos grupos A e B (10 ratos cada, com transecção do íleo a 5 e 10 centímetros proximalmente à válvula ileocecal; no grupo A, a anastomose foi realizada com 4 suturas de mucosa separadas com uso de polidioxanona; no grupo B, a anastomose foi realizada com poliglactina; no grupo C (5 ratos, foi realizada apenas a laparotomia e manipulação do íleo. Transcorridos 21 dias, os ratos foram anestesiados e submetidos à eutanásia. Os espécimes foram enviados para estudo histopatológico e análise de resistência à tração. A análise estatística foi efetuada com a aplicação dos testes de Tukey e de t de Student, com significância de p < 0,05. Resultados: Os resultados demonstraram que, na análise de resistência à tração, não foram observadas diferenças significativas entre os materiais. A análise histológica revelou diferenças significativas entre padrões de cicatrização, em que polidioxanona causou menos fibrose versus poliglactina. Conclusão: : Polidioxanona causou menos fibrose versus poliglactina em anastomoses intestinais realizadas em ratos. Keywords: Anastomosis, surgical, Polydioxanone, Polyglactin 910, Rats, Small intestine, Palavras-chave: Anastomose cirúrgica, Polidioxanona, Poliglactina 910, Ratos, Intestino delgado

  11. Ledipasvir and sofosbuvir plus ribavirin in patients with genotype 1 or 4 hepatitis C virus infection and advanced liver disease: a multicentre, open-label, randomised, phase 2 trial.

    Science.gov (United States)

    Manns, Michael; Samuel, Didier; Gane, Edward J; Mutimer, David; McCaughan, Geoff; Buti, Maria; Prieto, Martín; Calleja, José Luis; Peck-Radosavljevic, Markus; Müllhaupt, Beat; Agarwal, Kosh; Angus, Peter; Yoshida, Eric M; Colombo, Massimo; Rizzetto, Mario; Dvory-Sobol, Hadas; Denning, Jill; Arterburn, Sarah; Pang, Phillip S; Brainard, Diana; McHutchison, John G; Dufour, Jean-François; Van Vlierberghe, Hans; van Hoek, Bart; Forns, Xavier

    2016-06-01

    Treatment options are limited for patients infected by hepatitis C virus (HCV) with advanced liver disease. We assessed the safety and efficacy of ledipasvir, sofosbuvir, and ribavirin in patients with HCV genotype 1 or 4 and advanced liver disease. We did an open-label study at 34 sites in Europe, Canada, Australia, and New Zealand. Cohort A included patients with Child-Turcotte-Pugh class B (CTP-B) or CTP-C cirrhosis who had not undergone liver transplantation. Cohort B included post-transplantation patients who had either no cirrhosis; CTP-A, CTP-B, or CTP-C cirrhosis; or fibrosing cholestatic hepatitis. Patients in each group were randomly assigned (1:1) using a computer-generated randomisation sequence to receive 12 or 24 weeks of ledipasvir (90 mg) and sofosbuvir (400 mg) once daily (combination tablet), plus ribavirin (600-1200 mg daily). The primary endpoint was the proportion of patients achieving a sustained virological response 12 weeks after treatment (SVR12). All patients who received at least one dose of study drug were included in the safety analysis and all patients who received at least one dose of study drug and did not undergo liver transplantation during treatment were included in the efficacy analyses. Estimates of SVR12 and relapse rates and their two-sided 90% CI (Clopper-Pearson method) were provided. This exploratory phase 2 study was not powered for formal comparisons among treatment groups; no statistical hypothesis testing was planned or conducted. The trial is registered with EudraCT (number 2013-002802-30) and ClinicalTrials.gov (number NCT02010255). Between Jan 14, 2014, and Aug 19, 2014, 398 patients were screened. Of 333 patients who received treatment, 296 had genotype 1 HCV and 37 had genotype 4 HCV. In cohort A, among patients with genotype 1 HCV, SVR12 was achieved by 20 (87%, 90% CI 70-96) of 23 CTP-B patients with 12 weeks of treatment; 22 (96%, 81-100) of 23 CTP-B patients with 24 weeks of treatment; 17 (85%, 66-96) of 20 CTP

  12. Anatomopathological study of cardiomyopathy induced by doxorubicin in rats Estudo anatomopatológico da miocardiopatia induzida pela doxorrubicina em ratos

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    José Carlos Dorsa Vieira Pontes

    2010-04-01

    presença de fibrose, vacuolização citoplasmática, necrose e variação do tamanho do núcleo. Os resultados foram submetidos a análise estatística pelo teste t de Student. RESULTADOS: Os corações dos animais do grupo II aumentaram 41% em relação ao peso; 33% no diâmetro interno e 14% no diâmetro externo; e 24% na espessura da parede do VE. Fibrose do tecido miocárdico foi observada em 75% dos animais do grupo II; todos os animais apresentaram vacuolização citoplasmática dos miócitos; Houve necrose miocárdica em 75% dos animais e 87% apresentaram variação no tamanho do núcleo. A presença de células polimorfonucleares também foi observada. CONCLUSÃO: A doxorrubicina foi efetiva na promoção de alterações macro e microscópicas no tecido cardíaco de ratos, possivelmente constituindo-se num modelo experimental para estudo da miocardiopatia.

  13. Tratamento cirúrgico da taquicardia ventricular refratária: nova proposta técnica

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    BRICK Alexandre Visconti

    1997-01-01

    Full Text Available A miocardiopatia provocada pela doença de Chagas cria, freqüentemente, circuitos elétricos de reentrada, possibilitando o desencadeamento de taquicardia ventricular, geralmente refratária (TVR às drogas antiarrítmicas. Muitas vezes, este quadro desorganiza eletricamente os ventrículos, provocando a morte do paciente. Nova proposta técnica para tratamento da (TVR foi empregada em 9 pacientes, portadores da doença de Chagas com esta arritimia, sendo 8 com aneurisma de ponta e 1 da região infero-basal. Na maioria dos casos, o foco da taquicardia encontrava-se fora da borda do aneurisma ou da área de fibrose, geralmente na região basal ou póstero-lateral do ventrículo esquerdo. A idade variou entre 34 e 62 anos, com média de 48. Quatro eram do sexo masculino e 5 do feminino. Todos encontravam-se no grau funcional III e IV e a maioria apresentava episódios freqüentes de síncope, provocados pela taquicardia. Em 2 dos pacientes havia relato de AVC prévio e foi encontrado aneurisma de ponta em 8, aneurisma póstero-basal com extensa fibrose em 1 e trombo intracavitário em 6. Durante o ato cirúrgico foram induzidas as taquicardias clínicas em todos os pacientes. Na região em que o toque do instrumental cirúrgico conseguiu interrompê-las, foram realizadas aplicações de radiofreqüência, através de cateteres de ablação, no centro e nas bordas do foco. Logo após, e no sétimo dia de pós-operatório, nenhuma taquicardia pôde ser induzida com os protocolos de estimulação ventricular programada. A evolução de 13 +/- 7 meses, sem uso de drogas antiarrítmicas, mostra que 8 estão assintomáticos e em classe funcional I e II. Um paciente, com doença pulmonar obstrutiva crônica, faleceu de insuficiência respiratória no pós-operatório tardio (3 meses, sem ter apresentado taquicardia. Em conclusão, esta técnica é facilmente reprodutível, podendo ser realizada com simplicidade, sem necessidade de aparelhagem

  14. Morphologic evaluation of the use of a latex prosthesis in videolaparoscopic inguinoplasty: an experimental study in dogs Avaliação morfológica da utilização de prótese de latex na inguinoplastia videolaparoscópica: estudo experimental em cães

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    Luiz Henrique de Sousa

    2011-01-01

    espessura. Avaliou-se a região inguinal e as peças retiradas macro e microscopicamente, no grupo 1 no 7º, 14º, 21º e 28º dias e nos demais grupos no 28º dia pós-operatório. RESULTADOS: ausência de hematoma, seroma e infecção; presença de tortuosidade; indução de neoformação vascular, reação inflamatória, deposição de colágeno e encistamento total das biomembranas de latex, exceto com poliamida porosa fina que se incorporou apenas parcialmente, com formação de microcistos. Nenhuma biomembrana de latex induziu fibrose como no grupo controle prolene. CONCLUSÕES: As biomembranas mantêm indução do processo de cicatrização sem fibrose, sofrem encistamento e, exceto com poliamida porosa fina, não se incorporam aos tecidos vizinhos. A biomembrana de latex, com e sem poliamida, isoladamente não é recomendada para inguinoplastia pré-peritoneal.

  15. Mucosa-associated lymphoid tissue in individuals with AIDS Tecido linfoide associado à mucosa em indivíduos com AIDS

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    Janainna Grazielle Pacheco Olegario

    2011-06-01

    Full Text Available Vestibular folds (VF protect upper airways, but contain fewer immune cells in AIDS patients, which affects the structure of lymphoid follicles (LF. OBJECTIVE: To characterize fibrosis and immunoglobulin production in vestibular fold lymphoid tissues of AIDS patients with or with no infection and malnutrition. MATERIALS AND METHODS: A retrospective study of 71 adult vestibular fold autopsy specimens. The morphological analysis was done using the picrosirius staining method. Immunohistochemical methods consisted of anti-IgA, anti IgG, and anti IgM antibodies. RESULTS: Fibrosis was less intense in AIDS patients compared to subjects without AIDS; the same applied to patients with infection or malnutrition. IgA and IgG titers were higher in AIDS patients; IgM titers were higher in cases with infection. CONCLUSION: This study helps understand variations in lymphoid follicle components of AIDS patients; it also shows the influence of architectural changes and the effect of associated respiratory infection and malnutrition on lymphoid follicle function.Pregas vestibulares (PV são responsáveis pela proteção das vias aéreas superiores e, nos indivíduos com AIDS, apresenta diminuição das células imunes, o que influencia na estrutura dos folículos linfoides (FL. OBJETIVO: Caracterizar a fibrose e a produção de imunoglobulinas nos FL das PV nos indivíduos com AIDS, com e sem infecções e subnutrição associadas. MATERIAIS E MÉTODOS: Foi realizado um estudo retrospectivo transversal em 71 PV de adultos autopsiados. Para a análise morfológica foi usada a coloração picro-sirius. A imuno-histoquímica foi realizada com os anticorpos: anti-IgA, anti-IgG, anti-IgM. RESULTADOS: Nos pacientes com AIDS, a quantidade de fibrose foi menor quando comparados com aqueles que não possuíam AIDS, o mesmo sendo encontrado nos pacientes com infecções ou subnutrição. As quantidades de IgA e IgG foram maiores nos indivíduos com AIDS, e os valores de Ig

  16. An acqueous extract of Bidens pilosa L. protects liver from cholestatic disease: experimental study in young rats Um extrato aquoso de Bidens pilosa L. protege o fígado da doença colestática: estudo experimental em ratos jovens

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    Marta Izabel Suzigan

    2009-10-01

    Full Text Available PURPOSE: To test the hepatoprotective effect of water extract from Bidens Pilosa L. (BPE in cholestatic liver disease induced by ligature and resection of the common bile ducts (LRBD in young rats. METHODS: We studied four groups of ten 21 days old (P21 Wistar rats, Group SW: sham operation and water; Group SD: sham operation and BPE (160 mg of fresh leaves/100 g of body weight/day; Group LW: LRBD and water and Group LD: LRBD and BPE daily. Pentobarbital sleeping time (PST and serum activities of aspartate aminotransferase (AST and of alanine aminotransferase (ALT were determined after the sacrifice (P70. A Ruwart's score for hepatic fibrosis (RS was given to each animal. Were employed two way ANOVA and the test of Tukey or a non-parametric test for multiple comparisons. RESULTS: There were statistically significant differences between LW and LD in the measurements of the PST ((means LW=390; LD=173, AST (means LW=8, LD=5, ALT (medians LW=2; LD=1 e RS (medians LW=2; LD=1. CONCLUSION: BPE could be used in the phytotherapy of the hepatic damage induced by chronic obstructive cholestasis, because protects liver function, decreases the rate of necrosis and liver fibrosis in cholestatic liver disease.OBJETIVO: Testar o efeito hepatoprotetor do extrato aquoso de Bidens pilosa L. (EBP na doença hepática induzida pela ligadura e ressecção do ducto biliar comum (LRDBC em ratos jovens. MÉTODOS: Estudamos ratos Wistar com 21º. dia de vida (P21 divididos em quatro grupos de 10 animais, Grupo SA: operação simulada e água; Grupo SD: operação simulada e EBP (160mg de folhas frescas/100g de peso corporal/dia; Grupo LA: LRDBC e água e Grupo LD: LRDBC e EBP diariamente. O tempo de sono por pentobarbital (TSP, aspartato (AST e alanina (ALT aminotransferase foram determinadas após o sacrifício (P70. O Score de Ruwart (SR para fibrose hepática foi atribuído para cada animal. Foi realizada análise de variância com dois fatores e pelo teste de Tukey

  17. Intoxicação por Ateleia glazioviana (Leg. Papilionoideae em bovinos Poisoning by Ateleia glazioviana (Leg. Papilionoideae in cattle

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    Aldo Gava

    2001-06-01

    Full Text Available É descrita uma enfermidade caracterizada por manifestações relacionadas aos sistemas nervoso e cardiovascular, que afeta bovinos no oeste de Santa Catarina e noroeste do Rio Grande do Sul. Os animais apresentam "morte súbita" ou edemas de declive, veias jugulares ingurgitadas e pulso venoso positivo, precedida ou não de apatia, letargia e cegueira. A doença atinge bovinos com mais de um ano, principalmente no outono e inverno, com morbidade de 10 a 60% e mortalidade chegando a 95%. As lesões macroscópicas consistem de áreas esbranquiçadas e firmes no miocárdio, principalmente nas proximidades dos vasos coronários e no septo interventricular. Em parte dos animais, o fígado apresenta-se aumentado de volume e com aspecto de noz-moscada. Alterações histológicas incluem tumefação e necrose de miofibras cardíacas, fibrose e infiltrado de macrófagos no interstício do músculo cardíaco e marcada congestão centrolobular e leve fibrose no fígado. No encéfalo dos bovinos com quadro clínico de letargia, a substância branca apresenta degeneração esponjosa (status spongiosus. A doença com manifestações de letargia e cegueira foi reproduzida em bovinos com o fornecimento de folhas de Ateleia. glazioviana no cocho, na dose única de 40 e 50 g/kg e em doses fracionadas de 2,5, 5,0, 7,5 e 10 g/kg. Lesões cardíacas crônicas foram reproduzidas com doses fracionadas de 2,5, 5,0 e 7,5 g/kg por longo período e com dose inicial de 1 g/kg, acrescida de 1g/kg/dia até atingir 15 g/kg, num total de 120 g/kg.A disease affecting cattle from western Santa Catarina and northwestern Rio Grande do Sul, characterized by nervous and caradiovascular manifestations, is described. The animals succumbed by "sudden death" or showed subcutaneous pendant edema and engorged pulsating jugular veins, preceded or not by apathy, lethargy and blindness. The disease affects cattle over 1 year of age and occurs mainly during fall and winter. Morbidity rates

  18. O EFEITO DO TRICLOSAN NO PROCESSO FIBROSANTE DE CÉLULAS HEPÁTICAS LX-2 MEDIANTE A AÇÃO NA SÍNTESE DE ÁCIDO GRAXO DE NOVO

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    Juliana Fabiani Miranda

    2017-02-01

    Full Text Available A fibrose hepática é uma condição clínica presente na maioria das doenças hepáticas, caracterizada pelo aumento na síntese dos componentes da matriz extracelular na tentativa de cicatrização do tecido lesionado. A principal célula responsável pelo desenvolvimento da fibrose hepática é a célula estrelada hepática, localizada no espaço de Disse. Esta apresenta dois fenótipos: o quiescente e o ativado. O metabolismo de lipídeo na célula estrelada está relacionado com o fornecimento de energia para a iniciação e perpetuação do seu estado ativado. Dentro deste contexto a ácido graxo sintase (FASN, proteína chave da síntese de ácido graxo de novo torna-se alvo do estudo, uma vez que o metabolismo de lipídeos está intimamente relacionado ao processo fibrosante em células estreladas hepáticas. Sabe-se que o fármaco triclosan (TCS é inibidor da proteína FASN na síntese de ácido graxo de novo, com potencial alteração do processo fibrosante em células estreladas hepáticas. Sendo assim, avaliamos os aspectos celulares e moleculares do efeito do fármaco TCS na modulação do metabolismo de lipídeo através da inibição da FASN em modelo de células estreladas hepáticas LX-2. Os resultados demonstraram que o TCS na concentração 50 µM em cultura celular LX-2 crescida em 10% de soro bovino fetal (SBF – estado ativado- promove alterações morfofuncionais nas células; tanto pela despolimerização dos filamentos de actina quanto pela formação de estruturas globulares de actina no citoesqueleto celular. Desta maneira, o fármaco TCS apresentou características possivelmente citotóxicas em células LX-2 com 10% de SBF. Por outro lado, o estudo da inibição da proteína FASN demonstrou resultados que sugerem uma transdiferenciação celular correlacionada com a reversão do quadro pró-fibrosante hepático em células LX-2, com o aumento das gotículas de lipídeo no citoplasma e aumento da expressão dos

  19. Tomografia computadorizada de alta resolução na silicose: correlação com radiografia e testes de função pulmonar High-resolution computed tomography in silicosis: correlation with chest radiography and pulmonary function tests

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    Agnaldo José Lopes

    2008-05-01

    Full Text Available OBJETIVO: Correlacionar os parâmetros tomográficos com os de função pulmonar em portadores de silicose, bem como comparar os resultados da tomografia computadorizada de alta resolução (TCAR com os da radiografia de tórax. MÉTODOS: Foi realizado um estudo de corte transversal, em que foram avaliados 44 pacientes não-tabagistas e sem história pregressa de tuberculose. As radiografias foram classificadas de acordo com a proposta da Organização Internacional do Trabalho. Utilizando um sistema de escore semiquantitativo, os seguintes achados na TCAR foram quantificados: extensão total do envolvimento pulmonar, opacidades parenquimatosas e enfisema. Foram realizados espirometria, oscilações forçadas, avaliação dos volumes pulmonares pela técnica de diluição com hélio e medida da diffusing capacity of the lung for carbon monoxide (DLCO, capacidade de difusão do monóxido de carbono. RESULTADOS: Dos 44 pacientes estudados, 41 eram homens, com média de idade de 48,4 anos. Na análise comparativa, 4 pacientes classificados na categoria 0 pela radiografia foram reclassificados na categoria 1 pela TCAR e, enquanto a radiografia diagnosticou 23 casos de fibrose maciça progressiva, a TCAR estabeleceu esse diagnóstico em 33 doentes. Para o escore de opacidades, as maiores correlações foram observadas com as medidas de fluxo, DLCO e complacência. Já o enfisema correlacionou-se negativamente com as medidas de volume, DLCO e fluxo. Nesta amostra com predomínio de grandes opacidades (75% dos indivíduos, a deterioração da função pulmonar associou-se com a extensão das alterações estruturais. CONCLUSÕES: Na silicose, a TCAR é superior à radiografia tanto na detecção precoce da doença quanto na identificação de fibrose maciça progressiva.OBJECTIVE: To correlate tomographic findings with pulmonary function findings, as well as to compare chest X-ray findings with high resolution computed tomography (HRCT findings, in

  20. Efeito da solução de ácido acetilsalicílico e de ácido acético em fígado de coelhos Effect of acetylsalicylic acid and acetic acid solutions in liver of rabbits

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    Rogério Saad-Hossne

    2004-12-01

    Full Text Available Objetivo: Analisar os efeitos das soluções de aspirina e de ácido acético, in vivo, em fígado de coelhos sadios, verificando o efeito histolítico e o resultado anatomo-patológico das lesões e eventuais alterações bioquímicas hepáticas. Métodos: Utilizou-se 80 coelhos, divididos em 2 protocolos experimentais (1 e 2, subdivididos em 5 grupos cada, sendo os mesmos submetidos a laparotomia mediana, com injeção de 0,4 ml da solução de aspirina (2,5 e 5,0%, de ácido acético (2,5 e 5,0% e solução salina; o sacrifício ocorreu apos 24 horas (protocolo1 e 14 dias (protocolo 2; avaliou-se o peso, evolução clinica, dosagens bioquímicas, cavidade abdominal e torácica e microscopia do fígado. Resultados: Não foram observadas alterações na evolução clinica, peso e nas dosagens bioquímicas, apenas elevação da AST e ALT no grupo 24 horas(Protocolo 1. À macroscópica mostrou que nos animais tratados, em ambos os grupos, a presença de lesão hepática localizada na área infiltrada, correspondente a necrose (24 horas e fibrose (14 dias. Conclusão: Ambas as soluções (aspirina e ácido acético acarretaram destruição localizada do órgão substituída por fibrose apos 14 dias.Purpose: In the present work, 2.5% and 5% acetyl salicylic acid solution and acetic acid solutions were tested in vivo. Methods: In all tests control animals were used and given salt solution. 80 male rabbits were employed and randomly distributed among the different experimental groups. Results: When injected in the liver of healthy rabbits (in vivo, both solutions produced localized destruction (coagulative necrosis of the hepatic parenchyma in 24 hours. After 14 days, these lesions either disappeared or were replaced by mild scars (fibrosis. The lesions were not followed by clinical alterations and the only observed biochemical alteration was an increase in the amount of alanine amino transferase after 24 hours. Conclusion: In vivo, the effects of

  1. Adesividade e purulência de secreções respiratórias: implicações no transporte mucociliar em pacientes com bronquiectasias Adhesiveness and purulence of respiratory secretions: implications for mucociliary transport in patients with bronchiectasis

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    Joana Tambascio

    2010-10-01

    Full Text Available OBJETIVO: Analisar e comparar as propriedades de transporte de secreções respiratórias, classificadas através de parâmetros selecionados, de indivíduos com bronquiectasias não secundárias à fibrose cística. MÉTODOS: Foram avaliadas amostras de muco respiratório, classificadas como com propriedades de superfície adesivas ou não adesivas, assim como com aspecto mucoide ou purulento, de 35 participantes com bronquiectasias não secundárias à fibrose cística, quanto a velocidade relativa de transporte (VRT, deslocamento em máquina simuladora de tosse (MST e ângulo de contato (AC. Para as comparações propostas, foram utilizados modelos de ANOVA, com nível de significância estabelecido em 5%. RESULTADOS: Houve uma diminuição significativa no deslocamento em MST, assim como um aumento significativo no AC, das amostras adesivas quando comparadas às não adesivas (6,52 ± 1,88 cm vs. 8,93 ± 2,81 cm e 27,08 ± 6,13º vs. 22,53 ± 5,92º, respectivamente; p OBJECTIVE: To analyze and compare the transport properties of respiratory secretions, classified by selected parameters, in individuals with bronchiectasis unrelated to cystic fibrosis. METHODS: We collected mucus samples from 35 individuals with bronchiectasis unrelated to cystic fibrosis. The samples were first classified by their surface properties (adhesive or nonadhesive, as well as by their aspect (mucoid or purulent. We then tested the samples regarding relative transport velocity (RTV, displacement in a simulated cough machine (SCM, and contact angle (CA. For the proposed comparisons, we used ANOVA models, with a level of significance set at 5%. RESULTS: In comparison with nonadhesive samples, adhesive samples showed significantly less displacement in the SCM, as well as a significantly higher CA (6.52 ± 1.88 cm vs. 8.93 ± 2.81 cm and 27.08 ± 6.13º vs. 22.53 ± 5.92º, respectively; p < 0.05 for both. The same was true in the comparison between purulent and mucoid

  2. Influência do bloqueador de receptor de angiotensina (Losartana potássica na função renal e pressão arterial em cães GRMD Influence of angiotensin receptor blocker of renal function and arterial pression in GRMD dogs

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    Marina Brito Silva

    2009-04-01

    Full Text Available A distrofia muscular de Duchenne (DMD é uma alteração neuromuscular caracterizada por contínua necrose muscular e degeneração, com eventual fibrose e infiltração por tecido adiposo. O aumento progressivo da fibrose intersticial no músculo impede a migração das células miogênicas, necessárias para a formação muscular. O modelo canino constitui-se nas melhores fenocópias da doença em humanos, quando comparados com outros modelos animais com distrofia. O tratamento antifibrose de pacientes DMD, tendo como alvo os mediadores da citocina, TGF-beta, e o tratamento com antiinflamatórios, podem limitar a degeneração muscular e contribuir para a melhora do curso da doença. O presente estudo teve como objetivo observar os possíveis efeitos adversos na fisiologia renal, por meio de avaliação bioquímica sanguínea e da pressão arterial, verificando a viabilidade do uso do Losartan (um inibidor de TGF-beta nos cães afetados pela distrofia muscular. Foram utilizados quatro cães adultos, sendo dois machos e duas fêmeas. Utilizou-se a dose de 50mg de Losartan, administrada via oral, uma vez ao dia. Os exames clínicos, bem como alterações na função renal, o nível do potássio sérico e a pressão arterial não evidenciaram reação adversa durante todo o período do experimento. O uso de Losartan, por um período de 9 semanas, mostrou-se como uma terapia segura para o tratamento antifibrótico em cães adultos, não afetando a função renal ou pressão arterial dos animais.Duchenne muscular dystrophy (DMD is a neuromuscular disorder characterized by a continuous muscle necrosis and degeneration with eventual fibrosis and fatty tissue infiltration. Progressive increase in muscle interstitial fibrosis prevents the movement of myogenic cells, which is necessary for myotube formation. Canine model is the best phenocopies of the disease in humans when comparing with others animal models with dystrophy. Anti-fibrotic treatment of

  3. Complicações no tratamento com laser endovascular em varizes de membros inferiores Complications evidenced in the endovascular laser treatment for varicose veins

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    Jorge Enrique Soracco

    2005-01-01

    Full Text Available OBJETIVO: O objetivo do presente estudo é relatar as complicações no tratamento de varizes em membros inferiores com laser endovascular. MÉTODOS: Foram levantadas, no período de junho de 1999 a dezembro de 2002, algumas complicações, como queimadura de pele, neurite do nervo safeno, hiperpigmentação e fibrose no local da safena em 250 pacientes submetidos a tratamento endovascular com laser em varizes de membros inferiores. O diagnóstico das complicações foi clínico e baseado nos sinais e sintomas. Avaliou-se 196 pacientes do sexo feminino e 54 do sexo masculino, com idades variando entre 25 e 79 anos, no Hospital Militar de Buenos Aires. Foram tratados com laser de diodo de alta potência de 810 nm de longitude de onda mediante um sistema de fibras óticas semi-rígidas de quartzo de 400 e 600 µm e ponta de contato plana em modo cirúrgico contínuo. Para análise estatística, foram calculadas as percentagens. RESULTADOS: Lesões tipo queimadura foram observadas em 3,2%, hiperpigmentação em 9,6%, fibrose no local da safena por mais de 6 meses em 5,6% e neurite do nervo safeno em 4,8%. CONCLUSÃO: Conclui-se que o tratamento com laser endovascular de varizes de membros inferiores não é desprovido de intercorrências e que os fatores que levaram a essas complicações devem ser identificados e reavaliados.OBJECTIVE: The aim of the present study is to report the complications seen in the endovascular laser treatment of varicose veins of the lower limbs. METHODS: From June 1999 to December 2002, 250 patients submitted to the endovascular treatment of varicose veins of the lower limbs using lasers and suffering from complications, such as skin burns, saphenous neuritis, hyperpigmentation and fibrosis along the course of the saphenous vein, were assessed. The diagnosis of the complications was made clinically based on the signs and symptoms evidenced. Of the 250 patients, aged 25-79 years, treated in the Hospital Militar de Buenos

  4. 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

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    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

  5. FECHAMENTO DA PAREDE ABDOMINAL COM AFASTAMENTO PARCIAL DAS BORDAS DA APONEUROSE UTILIZANDO SOBREPOSIÇÃO COM TELAS DE VICRYL OU MARLEX EM RATOS

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    Mazzini Décio Luiz

    1999-01-01

    Full Text Available O presente experimento tem por finalidade estudar o efeito da aplicação de telas absorvíveis e inabsorvíveis, como reforço no fechamento de incisões medianas abdominais em ratos, na situação de aproximação parcial das bordas das aponeuroses. Para isto, foram estudados 45 ratos machos da raça Wistar, divididos em três grupos, nos quais se procedeu da seguinte maneira: Grupo "controle" - incisão mediana abdominal atingindo a cavidade peritoneal, seguida por fechamento apenas da pele; Grupo "vicryl" - incisão mediana abdominal atingindo a cavidade peritoneal, seguida por reforço com tela de vicryl, sobreposta em forma de ponte sobre a aponeurose, mantendo os lábios da aponeurose distantes entre si por 1,0 cm; Grupo "marlex" - procedimento idêntico ao grupo "vicryl", substituindo-se a tela de vicryl por marlex. Após um ano, os animais foram sacrificados e submetidos à avaliação macroscópica quanto à presença de hérnias e aderências às telas; aferição da resistência tênsil da cicatriz cirúrgica através da tração por dinamômetro e estudo histológico dos seguintes fenômenos da cicatrização: reação inflamatória crônica, inflamação granulomatosa tipo corpo estranho, tecido de granulação, hiperplasia fibroblástica e fibrose. Apenas os animais do grupo "controle" desenvolveram hérnias ao final do experimento. Não houve formação de aderências intestinais significativas em nenhum dos grupos estudados. A resistência tênsil foi significativamente maior nos animais em que se aplicaram telas para reforço. A reação inflamatória crônica e a inflamação granulomatosa tipo corpo estranho foram muito mais intensas no grupo em que se utilizou marlex, que nos demais grupos. Quanto ao tecido de granulação e hiperplasia fibroblástica, estavam ausentes em todos os grupos. A fibrose foi mais intensa nos grupos em que as telas foram empregadas. Conclui-se que a utilização de telas nesta situação evita o

  6. Long term evaluation of functional and morphological bladder alterations on alloxan-induced diabetes and aging: experimental study in rats Avaliação funcional e morfológica tardia de alterações na bexiga secundárias ao diabetes induzido por aloxano e no envelhecimento: estudo experimental em ratos

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    Antonio Antunes Rodrigues Jr

    2008-01-01

    Full Text Available PURPOSE: to evaluate structural and functional effects of Alloxan- induced diabetes and aging on bladder of rats. METHODS: evaluations were performed in three groups: A - 8 weeks of age, B - 44 weeks of age, C - 44 weeks of age with alloxan-induced diabetes. Muscle layer thickness, extracellular matrix fibrosis and collagen were quantified on digital images of bladder samples. Cystometric evaluations before surgical vesical denervation (SVD, included maximum cystometric capacity (MCC, maximum bladder pressure (MBP, bladder contraction frequency (VCF, duration of bladder contraction (DC, threshold pressure (TP and bladder compliance (BC. After SVD, maximum cystometric capacity (MCC, BC and maximum urethral closing pressure (MUCP were also measured. RESULTS: Reduced extracellular matrix fibrosis concentration and contraction strength were found in the bladders of group C. Before SVD, bladder compliance was not different between groups. Alterations were observed in MCC after SVD. CONCLUSIONS: We did not notice smooth muscle hypertrophy in Alloxan-induced diabetic rats after 44 weeks. There was alteration in the total and relative amount of fibrosis and collagen. The cystometric studies support the idea that this morphological alterations are important to determine the different bladder functional patterns found in the aging and the Alloxan-induced diabetic animals.OBJETIVOS: avaliar alterações estruturais e funcionais da bexiga de ratos machos, associadas ao diabetes induzido por aloxano e ao envelhecimento. MÉTODOS: três grupos de animais: A - 8 semanas de idade; B- 44 semanas de idade; C - 44 semanas de idade com diabetes induzido por aloxano, foram avaliados. Realizadas medidas de espessura da camada muscular, fibrose de matriz extracelular e quantidade de colágeno, através de análise de imagem digital dos tecidos. Realizados também testes cistométricos, antes da desnervação vesical cirúrgica (DVC, para avaliar capacidade vesical (CV

  7. Mycoplasma pneumoniae and Chlamydia pneumoniae in calcified nodules of aortic stenotic valves Mycoplasma pneumoniae e Chlamydia pneumoniae nos focos de calcificação de valva aórtica estenótica

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    Maria de Lourdes HIGUCHI

    2002-07-01

    ,27 +/- 2,06 nos focos de calcificação; 2,8 +/- 3,33 e 1,78+/- 3,063 nas áreas de fibrose ao redor e 0,21 +/- 0,17 e 0,12 +/- 0,13 nas regiões menos lesadas da válvula. Houve uma maior quantidade de CP e MP nos focos de calcificação e na fibrose ao redor do que nas regiões valvulares mais preservadas. Em conclusão, o fato de haver maior quantidade de CP e MP nos focos de calcificação da EVA favorece a hipótese de que a estenose aórtica não é um processo degenerativo inevitável devido a idade, mas sim uma resposta inflamatória à presença dessas bactérias, em uma morfologia semelhante à detectada na injúria aterosclerótica.

  8. Novel CFTR missense mutations in Brazilian patients with congenital absence of vas deferens: counseling issues Mutações novas no gene CFTR de pacientes brasileiros portadores de agenesia dos vasos deferentes: dificuldades no aconselhamento

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    Patricia de Campos Pieri

    2007-01-01

    Full Text Available PURPOSE: Screening for mutations in the entire Cystic Fibrosis gene (CFTR of Brazilian infertile men with congenital absence of vas deferens, in order to prevent transmission of CFTR mutations to offspring with the use of assisted reproductive technologies. METHOD: Specific polymerase chain reaction (PCR primers were designed to each of the 27 exons and splicing sites of interest followed by single strand conformational polymorphism and Heteroduplex Analysis (SSCP-HA in precast 12.5% polyacrylamide gels at 7ºC and 20ºC. Fragments with abnormal SSCP migration pattern were sequenced. RESULTS: Two novel missense mutations (S753R and G149W were found in three patients (two brothers together with the IVS8-5T allele in hetrozygosis. CONCLUSION: The available screenings for CF mutations do not include the atypical mutations associated to absence of vas deferens and thus, when these tests fail to find mutations, there is still a genetic risk of affected children with the help of assisted reproduction. We recommend the screening of the whole CFTR gene for these infertile couples, as part of the work-up before assisted reproduction.OBJETIVO: Pesquisar mutações em toda a extensão do gene que causa a Fibrose Cística (CFTR de homens brasileiros inférteis por agenesia congênita dos vasos deferentes, com a finalidade de prevenir a transmissão de mutações em CFTR à prole com o uso das tecnologias de reprodução assistida. MÉTODOS: Foram desenhados oligonucleotídeos específicos para realização de reação de polimerização em cadeia (PCR para cada um dos 27 exons e sítios de processamento de interesse no gene CFTR. O PCR foi seguido pela técnica de SSCP-HA (polimorfismos de conformação no DNA de fita simples e na formação de heteroduplexes em géis pré-fabricados de poliacrilamida a 12,5% em duas temperaturas, 7ºC e 20ºC. Os fragmentos com padrão alterado na migração do SSCP foram submetidos a seqüenciamento automatizado

  9. Avaliação do efeito do ibandronato na consolidação de fratura: estudo experimental em coelhos Evaluation of ibandronate effects in bone healing: experimental study in rabbits

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    Roberto Guarniero

    2007-08-01

    Full Text Available OBJETIVO: Determinar a presença de efeito favorável do ibandronato de sódio na consolidação óssea, contribuindo assim para estudos futuros visando sua utilização terapêutica no tratamento de fraturas e pseudartroses. MÉTODOS: Vinte coelhos da raça Nova Zelândia Albino foram submetidos à osteotomia transversa mediodiafisária da fíbula direita e divididos aleatoriamente em dois grupos com 10 animais cada. Os animais do grupo I receberam 2ml de solução contendo ibandronato de sódio e água destilada; os animais do grupo II receberam somente água destilada (grupo controle. Os coelhos foram sacrificados após 30 dias. Os calos ósseos formados no local das osteotomias foram avaliados por densitometria e histomorfometria. Utilizou-se na avaliação estatística dos resultados o teste t para a inferência sobre a diferença das médias de amostras paramétricas e a prova de Mann-Whitney para as amostras não paramétricas. Adotou-se o nível de significância de 5%. RESULTADOS: Observou-se que a quantidade relativa de osso foi maior e a quantidade relativa de fibrose foi menor nos calos ósseos formados no grupo medicado com ibandronato do que no grupo controle. Não houve diferença na quantidade relativa de cartilagem e na densidade mineral dos calos ósseos comparando-se os dois grupos. CONCLUSÃO: Neste experimento a administração do ibandronato de sódio favoreceu a consolidação de osteotomias da fíbula em coelhos, aumentando a quantidade relativa de osso nos calos ósseos formados e diminuindo a quantidade de fibrose.OBJECTIVE: To determine the presence of a favorable sodium ibandronate effect in bone healing, thus contributing for future studies of its clinical use in the treatment of fractures and pseudoarthroses. METHODS: 20 New Zealand white rabbits were submitted to transverse mid-diaphysis osteotomy of the right fibula and divided at random into two groups of 10 animals each. Animals in group I were given 2 ml of a

  10. NAFLD as a Sexual Dimorphic Disease: Role of Gender and Reproductive Status in the Development and Progression of Nonalcoholic Fatty Liver Disease and Inherent Cardiovascular Risk.

    Science.gov (United States)

    Ballestri, Stefano; Nascimbeni, Fabio; Baldelli, Enrica; Marrazzo, Alessandra; Romagnoli, Dante; Lonardo, Amedeo

    2017-06-01

    Nonalcoholic fatty liver disease (NAFLD) spans steatosis through nonalcoholic steatohepatis, cirrhosis, and hepatocellular carcinoma (HCC) associated with striking systemic features and excess cardiovascular and liver-related mortality. The pathogenesis of NAFLD is complex and multifactorial. Endocrine derangements are closely linked with dysmetabolic traits. For example, in animal and human studies, female sex is protected from dysmetabolism thanks to young individuals' ability to partition fatty acids towards ketone body production rather than very low density lipoprotein (VLDL)-triacylglycerol, and to sex-specific browning of white adipose tissue. Ovarian senescence facilitates both the development of massive hepatic steatosis and the fibrotic progression of liver disease in an experimental overfed zebrafish model. Consistently, estrogen deficiency, by potentiating hepatic inflammatory changes, hastens the progression of disease in a dietary model of nonalcoholic steatohepatitis (NASH) developing in ovariectomized mice fed a high-fat diet. In humans, NAFLD more often affects men; and premenopausal women are equally protected from developing NAFLD as they are from cardiovascular disease. It would be expected that early menarche, definitely associated with estrogen activation, would produce protection against the risk of NAFLD. Nevertheless, it has been suggested that early menarche may confer an increased risk of NAFLD in adulthood, excess adiposity being the primary culprit of this association. Fertile age may be associated with more severe hepatocyte injury and inflammation, but also with a decreased risk of liver fibrosis compared to men and postmenopausal status. Later in life, ovarian senescence is strongly associated with severe steatosis and fibrosing NASH, which may occur in postmenopausal women. Estrogen deficiency is deemed to be responsible for these findings via the development of postmenopausal metabolic syndrome. Estrogen supplementation may at

  11. "Fibrous nests" in human hepatocellular carcinoma express a Wnt-induced gene signature associated with poor clinical outcome.

    Science.gov (United States)

    Désert, Romain; Mebarki, Sihem; Desille, Mireille; Sicard, Marie; Lavergne, Elise; Renaud, Stéphanie; Bergeat, Damien; Sulpice, Laurent; Perret, Christine; Turlin, Bruno; Clément, Bruno; Musso, Orlando

    2016-12-01

    Hepatocellular carcinoma (HCC) is the 3rd cause of cancer-related death worldwide. Most cases arise in a background of chronic inflammation, extracellular matrix (ECM) remodeling, severe fibrosis and stem/progenitor cell amplification. Although HCCs are soft cellular tumors, they may contain fibrous nests within the tumor mass. Thus, the aim of this study was to explore cancer cell phenotypes in fibrous nests. Combined anatomic pathology, tissue microarray and real-time PCR analyses revealed that HCCs (n=82) containing fibrous nests were poorly differentiated, expressed Wnt pathway components and target genes, as well as markers of stem/progenitor cells, such as CD44, LGR5 and SOX9. Consistently, in severe liver fibroses (n=66) and in HCCs containing fibrous nests, weighted correlation analysis revealed a gene network including the myofibroblast marker ACTA2, the basement membrane components COL4A1 and LAMC1, the Wnt pathway members FZD1; FZD7; WNT2; LEF1; DKK1 and the Secreted Frizzled Related Proteins (SFRPs) 1; 2 and 5. Moreover, unbiased random survival forest analysis of a transcriptomic dataset of 247 HCC patients revealed high DKK1, COL4A1, SFRP1 and LAMC1 to be associated with advanced tumor staging as well as with bad overall and disease-free survival. In vitro, these genes were upregulated in liver cancer stem/progenitor cells upon Wnt-induced mesenchymal commitment and myofibroblast differentiation. In conclusion, fibrous nests express Wnt target genes, as well as markers of cancer stem cells and mesenchymal commitment. Fibrous nests embody the specific microenvironment of the cancer stem cell niche and can be detected by routine anatomic pathology analyses. Copyright © 2016 Elsevier Ltd. All rights reserved.

  12. Prevalence of nephrogenic systemic fibrosis in renal insufficiency patients: Results of the FINEST study

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    Janus, Nicolas [Department of Nephrology, Pitie-Salpetriere Hospital, 83 boulevard de l' Hopital, 75013 Paris (France)], E-mail: nicolas.janus@psl.aphp.fr; Launay-Vacher, Vincent; Karie, Svetlana [Department of Nephrology, Pitie-Salpetriere Hospital, 83 boulevard de l' Hopital, 75013 Paris (France); Clement, Olivier [Department of Radiology, Georges Pompidou European Hospital, Paris (France); Ledneva, Elena [Department of Nephrology, Pitie-Salpetriere Hospital, 83 boulevard de l' Hopital, 75013 Paris (France); Frances, Camille [Department of Dermatology, Tenon Hospital, Paris (France); Choukroun, Gabriel [Department of Nephrology, Amiens University Hospital, Amiens (France); Deray, Gilbert [Department of Nephrology, Pitie-Salpetriere Hospital, 83 boulevard de l' Hopital, 75013 Paris (France)

    2010-02-15

    Purpose: Nephrogenic systemic fibrosis (NSF) is characterized by widespread tissue fibrosis, mainly affecting the skin. Gadolinium chelates have been implicated in the onset of NSF in patients with renal impairment (RI). The FINEST study (FIbrose Nephrogenique SysTemique) was designed to determine the prevalence of NSF after magnetic resonance imaging (MRI) in French RI patients. Materials and methods: We studied all patients with RI who had at least one MRI examination during a one-year period, with or without gadolinium chelate administration. Data were collected retrospectively from 9 Nephrology Departments in France, and included sex, age, renal function, type of gadolinium administered, and subsequent cutaneous disorders. If a patient presented a cutaneous disorder, a skin biopsy was performed to confirm the diagnostic. Results: The 308 eligible patients had a mean age of 59.9 years, 59% were men, and 54% had stage 5 RI. 75% of those 308 patients received a Gadolinium chelate. Among those patients who received a gadolinium chelate, 76% received gadoterate, 20% gadopentetate, 3% gadodiamide and 1% gadobenate. No cutaneous disorders were recorded after MRI. Conclusion: These results confirm that NSF is a rare disease. Based on a reported frequency, {approx}3.5% in patients with glomerular filtration rate <30 ml/min/1.73 m{sup 2}), some cases should have been observed in our study which included 308 patients. Most patients received gadoterate, a macrocyclic gadolinium chelate for which no case of NSF has been observed worldwide. This suggests that more stable macrocyclic agents may be less likely to induce NSF.

  13. Atividade farmacológica da monocrotalina isolada de plantas do gênero Crotalaria

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    José E. R. Honório Júnior

    Full Text Available Crotalaria retusa é uma planta encontrada no Nordeste brasileiro, pertence ao gênero Crotalaria e à família Leguminosae, e possuem mais de seissentas espécies no mundo e mais de quarenta no Brasil. As variedades tóxicas mais conhecidas são C. spectabilis, C. crispata, C. retusa, C. dura e C. globifera. Plantas do gênero Crotalaria são de interesse porque são usadas na medicina popular. Esses gêneros são ricos em alcaloides pirrolizidínicos (AP, que são as principais toxinas e apresentam efeitos pneumotóxicos, nefrotóxicos, cardiotóxicos, fetotóxicos, carcinogênicos, inflamação, hemorragia e fibrose. A monocrotalina é o principal alcaloide pirrolizidínico encontrado nessas plantas e é ativamente oxidada in vivo pelo citocromo P450 no fígado, formando intermediários altamente reativos tipo pirrólicos que são responsáveis pela ligação cruzada do DNA-DNA e DNA-proteína. O presente trabalho teve como objetivo fazer um levantamento bibliográfico via internet, utilizando bancos de dados, programas de pesquisa científica e pesquisa em livros relacionados, acerca da atividade farmacológica e do mecanismo de ação da monocrotalina extraída de plantas do gênero Crotalaria, ressaltando desde os aspectos botânicos da planta, estrutura química dos alcaloides pirrolizidínicos, exemplos experimentais de toxicidade e provável mecanismo de ação.

  14. Joubert syndrome: large clinical variability and a unique neuroimaging aspect Síndrome de Joubert: grande variabilidade clínica e uma neuroimagem característica

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    Emília Katiane Embiruçu Leão

    2010-04-01

    Full Text Available Joubert syndrome (JS is an autosomal recessive inherited disorder characterized by hypotonia, cerebellar vermis hypoplasia, ocular abnormalities (e.g, pigmentary retinopathy, oculomotor apraxia and nystagmus, renal cysts and hepatic fibrosis. Respiratory abnormalities, as apnea and hyperpnea, may be present, as well as mental retardation. At least seven JS loci have been determined and five genes identified. Herein, we report five children, belonging to independent families, with JS: they shared the same typical MRI abnormality, known as molar tooth sign, but had an otherwise quite variable phenotype, regarding mostly their cognitive performance, visual abilities and extra-neurological compromise.A síndrome de Joubert (SJ é uma doença hereditária, autossômica recessiva, caracterizada por hipotonia, hipoplasia do vermis cerebelar, anormalidades oculares (p.ex., retinite pigmentar, apraxia oculomotora e nistagmo, cistos renais e fibrose hepática. Anormalidades respiratórias tais como apnéia e hiperpnéia podem estar presentes, assim como deficiência mental. Pelo menos sete loci e cinco genes diferentes associados à SJ já foram identificados. Este artigo relata cinco crianças com SJ, pertencentes a diferentes famílias. Todos os pacientes compartilham a mesma anormalidade típica da RM, conhecida como sinal do dente molar, e apresentam ampla variabilidade clínica em relação ao desempenho cognitivo, comprometimento visual e alterações extra-neurológicas.

  15. Um caso raro de discinesia ciliar primária associada a heterotaxia A rare case of primary ciliary dyskinesia with heterotaxy

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    Cátia Quintela

    2009-01-01

    Full Text Available A discinesia ciliar primária é uma doença autossómica recessiva caracterizada pela história de infecções de repetição do aparelho respiratório superior e inferior, rinossinusite e bronquite associada a situs inversus completo ou parcial. Os autores apresentam um doente de 78 anos, eurocaucasiano, com rinossinusites, bronquite crónica e dispneia, otite média com défices auditivos, infertilidade, seguido em consulta de gastrenterologia por dispepsia e obstipação há vários anos. Realizou vários exames que mostraram: agenesia frontal direita, espessamento brônquico, bronquiectasias, cego e cólon ascendente localizados na fossa ilíaca esquerda. Excluiu-se imunodeficiência, alergias, fibrose quística e outros. No decurso da investigação concluímos que se tratava de um caso de discinesia ciliar primária. Pela raridade deste caso, decidimos apresentá-lo.Primary ciliary dyskinesia is an autosomal recessive disease with a clinical history of upper and lowers respiratory infections, rhinosinusitis and bronquitis associated with complete or partial situs inversus. The authors present a 78-year-old male caucasian patient with rhinosinusitis, lower respiratory tract infection and dyspnea, chronic otitis with hearing deficit and infertility followed in Gastroenterology for dyspepsia and constipation. The radiological studies revealed agenesis of right frontal sinus; bronchial wall thickening; bronchiectasis; cecum and ascending colon located on the left and small bowel occupies right side of abdomen. He had no immunodeficiency, allergies, cystic fibrosis and others. We concluded primary ciliary dyskinesia with heterotaxy. For the rarity of this case we decided to present it.

  16. Nephrogenic systemic fibrosis

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    Bhushan Madke

    2011-01-01

    Full Text Available Nephrogenic systemic fibrosis (NSF is a relatively new fibrosing disorder which has caught the attention of various specialities in the past decade. NSF is an extremely disabling and often painful condition, affecting up to 13% of the individuals with chronic kidney disease. The administration of a gadolinium chelate contrast agent has been reported to induce the development of NSF, particularly in patients who have acute or chronic renal disease with a glomerular filtration rate (GFR lower than 30-mL/min/1.73 m 2 and in those with acute renal insufficiency. Mass spectroscopy studies have demonstrated particles of gadolinium in t