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Sample records for pulmonary syndrome central

  1. Isolated pulmonary Goodpasture syndrome.

    Science.gov (United States)

    Harrity, P; Gilbert-Barness, E; Cabalka, A; Hong, R; Zimmerman, J

    1991-01-01

    The case of a 13-year-old girl with Goodpasture syndrome is reported. The presentation at this age with only pulmonary hemorrhage is unusual among Goodpasture syndrome patients. The case illustrates well the diagnostic difficulties in Goodpasture syndrome. The choices of treatment modalities available for this disease and the results and risks of such treatment options are reviewed. A discussion and classification of diffuse pulmonary hemorrhage is presented. The case emphasizes that Goodpasture syndrome should be considered in the differential diagnosis of diffuse pulmonary hemorrhage in spite of a lack of renal abnormalities and that serum anti-glomerular basement membrane antibody testing can be of great help in confirming the diagnosis.

  2. Hantavirus Pulmonary Syndrome

    Centers for Disease Control (CDC) Podcasts

    2011-07-14

    Dr. Adam MacNeil, epidemiologist with Viral Special Pathogens Branch at CDC, discusses hantavirus pulmonary syndrome.  Created: 7/14/2011 by National Center for Emerging Zoonotic and Infectious Diseases (NCEZID).   Date Released: 7/18/2011.

  3. [Pulmonary-renal syndrome].

    Science.gov (United States)

    Risso, Jorge A; Mazzocchi, Octavio; De All, Jorge; Gnocchi, César A

    2009-01-01

    The pulmonary-renal syndrome is defined as a combination of diffuse alveolar hemorrhage and glomerulonephritis. The coexistence of these two clinical conditions is due to diseases with different pathogenic mechanisms. Primary systemic vasculitis and Goodpasture syndrome are the most frequent etiologies. Systemic lupus erythematosus, connective tissue diseases, negative anti neutrophil cytoplasmic antibody vasculitis and those secondary to drugs are far less common causes. An early diagnosis based on clinical, radiologic, laboratory and histologic criteria enables early treatment, thus diminishing its high morbidity-mortality rate. Therapy is based on high doses of corticosteroids, immunosuppressants, tumor necrosis factor inhibitors and plasmapheresis.

  4. Treatment of Hantavirus Pulmonary Syndrome

    Science.gov (United States)

    2007-10-14

    Clinical syndrome Hantaviruses cause a spectrum of vascular-leak syndromes n humans ranging from proteinuria to pulmonary edema and rank hemorrhage...of fatal illness. The illness is haracterized by fever and vascular leakage resulting in noncar- iogenic pulmonary edema followed in severe cases by...cardiopulmonary syndrome (HCPS) o emphasize the important role of cardiogenic shock; among ospitalized patients, death almost invariably results from

  5. Pulmonary manifestations of the antiphospholipid antibody syndrome.

    Science.gov (United States)

    Ford, H James; Roubey, Robert A S

    2010-09-01

    A broad spectrum of pulmonary disease may occur in antiphospholipid antibody syndrome. The most common pulmonary manifestations are pulmonary thromboembolism and pulmonary hypertension. In this article the authors review these manifestations, as well as less common findings including acute respiratory distress syndrome, alveolar hemorrhage, and pulmonary capillaritis.

  6. Central Pain Syndrome

    Science.gov (United States)

    ... or hands. Central pain syndrome often begins shortly after the causative injury or damage, but may be delayed by months or even years, especially if it is related to post-stroke pain. × Definition Central pain syndrome is a neurological ...

  7. Sleep apnea syndrome: central sleep apnea and pulmonary hypertension worsened during treatment with auto-CPAP, but improved by adaptive servo-ventilation.

    Science.gov (United States)

    Ono, Hiroshi; Fujimoto, Hiroyuki; Kobayashi, Yoshinori; Kudoh, Shoji; Gemma, Akihiko

    2010-01-01

    In this 71-year-old man diagnosed as obstructive sleep apnea syndrome initially, the apnea-hypopnea index in polysomnography was 31.3/hour. He started auto-adjusted continuous positive airway pressure (auto-CPAP) treatment in July 2005 but developed congestive heart failure in December 2007. Pulmonary arterial pressure (PAP), estimated by echocardiography, was 71 mmHg. In January 2008, during simplified sleep examination with a breath-movement sensor under auto-CPAP, many central-type apneas were recognized. After replacing auto-CPAP with adaptive servo-ventilation (ASV), the apnea-hypopnea index was 5.3/hour and PAP became 36 mmHg after 3 months. It was thought that the increase of PAP was due to long-term inadequate use of auto-CPAP.

  8. Central Neuropathic Pain Syndromes.

    Science.gov (United States)

    Watson, James C; Sandroni, Paola

    2016-03-01

    Chronic pain is common in patients with neurologic complications of a central nervous system insult such as stroke. The pain is most commonly musculoskeletal or related to obligatory overuse of neurologically unaffected limbs. However, neuropathic pain can result directly from the central nervous system injury. Impaired sensory discrimination can make it challenging to differentiate central neuropathic pain from other pain types or spasticity. Central neuropathic pain may also begin months to years after the injury, further obscuring recognition of its association with a past neurologic injury. This review focuses on unique clinical features that help distinguish central neuropathic pain. The most common clinical central pain syndromes-central poststroke pain, multiple sclerosis-related pain, and spinal cord injury-related pain-are reviewed in detail. Recent progress in understanding of the pathogenesis of central neuropathic pain is reviewed, and pharmacological, surgical, and neuromodulatory treatments of this notoriously difficult to treat pain syndrome are discussed.

  9. Lutembacher Syndrome and Rheumatic Pulmonary Stenosis

    Institute of Scientific and Technical Information of China (English)

    Jufang Chi; Hangyuan Guo; Biao Yang

    2008-01-01

    We discdbed a case of a 55-yr-old woman diagnosed with Lutembacher syndrome and rheumatic pulmonary stenosis.Congenital atrial septal defect was found in age 7 and rheumatic fever in age 34.As the patient developed pulmonary hypertension with calcified mitral valve leaflet and pulmonary stenosis so surgery was not indicated.So the patient was managed by medical therapy alone.

  10. Scimitar syndrome with pulmonary arteriovenous fistulas.

    Science.gov (United States)

    Le Rochais, J P; Icard, P; Davani, S; Abouz, D; Evrard, C

    1999-10-01

    Right abnormal pulmonary venous return into the inferior vena cava associated with abnormal fissure, dextrocardia, and systemic arterial supply of a variable degree, are the characteristics of the scimitar syndrome. We report on a patient in whom this rare syndrome was associated with pulmonary arteriovenous fistulas within the involved lung.

  11. Hemorrhagic fever with renal syndrome and coexisting hantavirus pulmonary syndrome

    Directory of Open Access Journals (Sweden)

    Young Min Hong

    2012-06-01

    Full Text Available Hemorrhagic fever with renal syndrome (HFRS is an acute viral disease with fever, hemorrhage and renal failure caused by hantavirus infection. Hantavirus induces HFRS or hantavirus pulmonary syndrome (HPS. HPS progression to a life-threatening pulmonary disease is found primarily in the USA and very rarely in South Korea. Here, we report a case of HFRS and coexisting HPS.

  12. Hantavirus Pulmonary Syndrome in Manitoba

    Directory of Open Access Journals (Sweden)

    L Robbin Lindsay

    2001-01-01

    Full Text Available The first confirmed case of hantavirus pulmonary syndrome in Manitoba was diagnosed in 1999. To define better the risk of exposure to hantaviruses in this area, the clinical features and epidemiological factors pertaining to this case were described, and a serological survey of rodents collected near the patient's residence was undertaken. Small mammals were collected using live traps, were anesthetized via inhalation of isoflurane and were bled. Human and mouse serologies were undertaken using an ELISA to detect hantavirus-specific immunoglobulin G and/or immunoglobulin M antibodies. In addition, a full medical and epidemiological assessment, as well as individual risk factor and exposure analysis, were conducted. A 27-year-old Manitoba woman presented with severe respiratory distress and diffuse bilateral air space disease radiologically. Despite extremely aggressive measures, including mechanical ventilation, antibiotics, fluid management and inotropic support, the patient's condition rapidly deteriorated, and she died 8 h after admission. Hantavirus pulmonary syndrome was confirmed by the detection of immunoglobulin M and immunoglobulin G antibodies to the Sin Nombre virus (SNV in her sera and by the demonstration of SNV genomic sequences in her lung tissue. Exposure to hantavirus likely occurred in and around the home or in the rural area in which she resided. A total of 252 small mammals, primarily deer mice (Peromyscus maniculatus, were collected from 17 different sites at or near where the patient lived. Antibodies to SNV were detected in 28 of 244 (11.5% deer mice, which were collected within 9 km of the residence of the fatal case, indicating that these rodents are a significant reservoir for SNV in this area.

  13. MRI assessment of bronchial compression in absent pulmonary valve syndrome and review of the syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Taragin, Benjamin H.; Berdon, Walter E. [Children' s Hospital of New York, Department of Radiology, New York, NY (United States); Prinz, B. [Children' s Hospital of New York, Department of Cardiology, New York, NY (United States)

    2006-01-01

    Absent pulmonary valve syndrome (APVS) is a rare cardiac malformation with massive pulmonary insufficiency that presents with short-term and long-term respiratory problems secondary to severe bronchial compression from enlarged central and hilar pulmonary arteries. Association with chromosome 22.Q11 deletions and DiGeorge syndrome is common. This historical review illustrates the airway disease with emphasis on assessment of the bronchial compression in patients with persistent respiratory difficulties post-valvular repair. Cases that had MRI for cardiac assessment are used to illustrate the pattern of airway disease. (orig.)

  14. [Central anticholinergic syndrome].

    Science.gov (United States)

    Fernández Urretavizcaya, P; Cenoz Osinaga, J C; Jáuregui Garía, M L; Gállego Culleré, J

    1991-10-01

    Two new cases of anticolinergic central syndrome are described. The first case, a 8 year old girl, suffered a severe encefalopathy after topical application of mydriatic cholirio as an aid in a rutine study of ocular refraction. The second case, 67 year old man presented a severe neurological picture after accidental intake of a silvester plantground (Atropa belladonna). His neurological condition returned quickly to normal whith administration of physostigmine. Differents aspects of the etiology, clinical picture and diagnosis are discussed with special emphasis in patients with delirium or acute confusional states. Finally, attention is drawn in the necessity of a properly use of anticholinergic drugs overcoat in aged or children.

  15. Serotonin syndrome presenting as pulmonary edema

    Directory of Open Access Journals (Sweden)

    Nilima Deepak Shah

    2016-01-01

    Full Text Available Serotonin syndrome (SS is a potentially life-threatening condition resulting from excessive central and peripheral serotonergic activity. Clinically, it is a triad of mental-status changes, neuromuscular abnormalities, and autonomic disturbances. It can be caused by intentional self-poisoning, overdose, or inadvertent drug interactions. We report the case of a 58-year-old male with type 2 diabetes mellitus and obsessive compulsive disorder who developed pulmonary edema as a possible complication of SS. SS was caused by a combination of three specific serotonin re-uptake inhibitors (fluoxetine, fluvoxamine, and sertraline, linezolid, and fentanyl. The hospital course was further complicated by difficult weaning from the ventilator. SS was identified and successfully treated with cyproheptadine and lorazepam. The case highlights the importance of effective consultation-liaison and prompt recognition of SS as the presentation may be complex in the presence of co-morbid medical illness.

  16. Serotonin syndrome presenting as pulmonary edema

    Science.gov (United States)

    Shah, Nilima Deepak; Jain, Ajay B.

    2016-01-01

    Serotonin syndrome (SS) is a potentially life-threatening condition resulting from excessive central and peripheral serotonergic activity. Clinically, it is a triad of mental-status changes, neuromuscular abnormalities, and autonomic disturbances. It can be caused by intentional self-poisoning, overdose, or inadvertent drug interactions. We report the case of a 58-year-old male with type 2 diabetes mellitus and obsessive compulsive disorder who developed pulmonary edema as a possible complication of SS. SS was caused by a combination of three specific serotonin re-uptake inhibitors (fluoxetine, fluvoxamine, and sertraline), linezolid, and fentanyl. The hospital course was further complicated by difficult weaning from the ventilator. SS was identified and successfully treated with cyproheptadine and lorazepam. The case highlights the importance of effective consultation-liaison and prompt recognition of SS as the presentation may be complex in the presence of co-morbid medical illness. PMID:26997733

  17. Fat embolism syndrome and pulmonary microvascular cytology.

    Science.gov (United States)

    Castella, X; Vallés, J; Cabezuelo, M A; Fernandez, R; Artigas, A

    1992-06-01

    Pulmonary microvascular cytology consists of analysis of capillary blood sampled while a Swan-Ganz catheter is in the wedge position. This technique has proved to be useful in the diagnosis of lymphangitic spread of carcinoma in the lungs and there are case reports of their use in amniotic fluid embolism. Its usefulness in diagnosing fat embolism syndrome has been shown only rarely. We report a new case in which pulmonary microvascular cytologic study allowed a definite diagnosis of fat embolism syndrome. We suggest obtaining routinely samples of capillary blood when a pulmonary catheter is in place and fat embolism is suspected on a clinical basis.

  18. Cowden Syndrome and Concomitant Pulmonary Neuroendocrine Tumor

    DEFF Research Database (Denmark)

    Langer, Seppo W; Ringholm, Lene; Dali, Christine I

    2015-01-01

    Cowden Syndrome is a rare autosomal dominantly inherited disorder. Patients with Cowden Syndrome are at increased risk of various benign and malignant neoplasms in breast, endometrium, thyroid, gastrointestinal tract, and genitourinary system. Neuroendocrine tumors are ubiquitous neoplasms that may...... occur anywhere in the human body. Bronchopulmonary neuroendocrine tumors include four different histological subtypes, among these, typical and atypical pulmonary carcinoids. No association between Cowden Syndrome and neuroendocrine tumors has previously been described. We present two cases of Cowden...

  19. Central Cord Syndrome

    Science.gov (United States)

    ... Craniosynostosis Information Page Creutzfeldt-Jakob Disease Information Page Cushing's Syndrome Information Page Dandy-Walker Syndrome Information Page Deep Brain Stimulation for Parkinson's Disease Information Page Dementia Information ...

  20. Pulmonary agenesis and pulmonary sling anomaly in an infant with Down syndrome.

    Science.gov (United States)

    Takci, S; Yigit, S; Haliloglu, M; Boduroglu, K; Kiper, N

    2013-01-01

    Pulmonary agenesis is a rare congenital anomaly presenting with normal karyotype in most of the cases. Rarely pulmonary agenesis is associated with chromosomal abnormalities and other genetic disorders such as Oculo-auriculo-vertebral spectrum, VACTERL association and velo-cardio-facial syndrome. This report presents a patient with pulmonary agenesis, pulmonary sling anomaly and Down syndrome.

  1. An Emerging Pulmonary Haemorrhagic Syndrome in Dogs: Similar to the Human Leptospiral Pulmonary Haemorrhagic Syndrome?

    Directory of Open Access Journals (Sweden)

    R. Klopfleisch

    2010-01-01

    Full Text Available Severe pulmonary haemorrhage is a rare necropsy finding in dogs but the leptospiral pulmonary haemorrhagic syndrome (LPHS is a well recognized disease in humans. Here we report a pulmonary haemorrhagic syndrome in dogs that closely resembles the human disease. All 15 dogs had massive, pulmonary haemorrhage affecting all lung lobes while haemorrhage in other organs was minimal. Histologically, pulmonary lesions were characterized by acute, alveolar haemorrhage without identifiable vascular lesions. Seven dogs had mild alveolar wall necrosis with hyaline membranes and minimal intraalveolar fibrin. In addition, eight dogs had acute renal tubular necrosis. Six dogs had a clinical diagnosis of leptospirosis based on renal and hepatic failure, positive microscopic agglutination test (MAT and/or positive blood/urine Leptospira-specific PCR. Leptospira could not be cultured post mortem from the lungs or kidneys. However, Leptospira-specific PCR was positive in lung, liver or kidneys of three dogs. In summary, a novel pulmonary haemorrhagic syndrome was identified in dogs but the mechanism of the massive pulmonary erythrocyte extravasation remains elusive. The lack of a consistent post mortem identification of Leptospira spp. in dogs with pulmonary haemorrhage raise questions as to whether additional factors besides Leptospira may cause this as yet unrecognized entity in dogs.

  2. Imaging central pain syndromes.

    Science.gov (United States)

    Veldhuijzen, Dieuwke S; Greenspan, Joel D; Kim, Jong H; Coghill, Robert C; Treede, Rolf-Detlef; Ohara, Shinji; Lenz, Frederick A

    2007-06-01

    Anatomic, functional, and neurochemical imaging studies have provided new investigative tools in the study of central pain. High-resolution imaging studies allow for precise determination of lesion location, whereas functional neuroimaging studies measure pathophysiologic consequences of injury to the central nervous system. Additionally, magnetic resonance spectroscopy evaluates lesion-induced neurochemical changes in specific brain regions that may be related to central pain. The small number of studies to date precludes definitive conclusions, but the recent findings provide information that either supports or refutes current hypotheses and can serve to generate new ideas.

  3. Hantavirus pulmonary syndrome in a postpartum woman

    Science.gov (United States)

    Murthy, Pooja R.; Ucchil, Rajesh; Shah, Unmil; Chaudhari, Dipak

    2016-01-01

    Hantavirus infection, a rare disease diagnosed in India and carries a very high mortality. There are no reports of this infection in association with pregnancy or postpartum period in our country. We present a case of a 30-year-old female diagnosed to have hantavirus pulmonary syndrome in the postpartum period. We intend to create awareness about this infection and consider it in the differential diagnosis of patients presenting with acute respiratory distress syndrome and multiorgan dysfunction in association with pregnancy and postpartum period. PMID:27688634

  4. [Hemophagocytic syndrome secondary to cavitary pulmonary tuberculosis].

    Science.gov (United States)

    Rakotoson, J L; Rakotonirainy, O H; Moroyandsa, F; Rakotoharivelo, H; Rakotomizao, J R; Andrianarisoa, A C F

    2012-01-01

    Hemophagocytic syndrome is due to the activation and nonmalignant proliferation of macrophages and T lymphocytes. The purpose of this report is to describe a 25-year-old man who presented with fever, coughing, and weight loss over the past month. Laboratory findings demonstrated pancytopenia, hyperferritinemia, and cytolysis. The myelogram showed a hemophagocytic syndrome, and tuberculosis bacilli were found in the sputum. Chest radiography and thoracic computed tomography depicted a cavitary lesion suggestive of pulmonary tuberculosis. Treatment of tuberculosis alone, without an immunosuppressant agent, was effective and led to improvement. In a country where tuberculosis is highly endemic, hemophagocytic syndrome should be considered in the differential diagnosis of patients with active tuberculosis complicated by pancytopenia.

  5. Hantavirus Fever without Pulmonary Syndrome in Panama

    Science.gov (United States)

    Armien, Blas; Pascale, Juan M.; Muñoz, Carlos; Mariñas, Jamileth; Núñez, Heydy; Herrera, Milagro; Trujillo, José; Sánchez, Deyanira; Mendoza, Yaxelis; Hjelle, Brian; Koster, Frederick

    2013-01-01

    In Panama, hantavirus pulmonary syndrome (HPS) is caused by Choclo virus, a species phylogenetically related to Andes and Maporal viruses. Up to 60% of the population has been positive for specific serum antibody in community-based surveys, but mortality is very uncommon. In four western Panama clinics, we tested individuals presenting with a severe febrile prodrome for acute hantavirus (HV) infection by immunoglobulin M enzyme-linked immunosorbent assay and reverse transcription polymerase chain reaction as well as clinically similar infections, such as dengue and leptospirosis. From 2006 to 2009, at least 21% of 117 patients diagnosed with HV infection had HV Fever (HF) with no evidence of pulmonary edema (no respiratory distress or radiographic lung infiltrates), and 44% of patients had very mild HPS (radiographic pulmonary edema but no respiratory insufficiency). HV infection caused by Choclo virus in Panama presents often as HF, which contrasts with HV in the Americas but is consistent with the high seroprevalence in endemic regions. PMID:23836565

  6. Pulmonary hypertension associated with thalassemia syndromes.

    Science.gov (United States)

    Fraidenburg, Dustin R; Machado, Roberto F

    2016-03-01

    Chronic hemolytic anemia has increasingly been identified as an important risk factor for the development of pulmonary hypertension (PH). Within the thalassemia syndromes, there are multiple mechanisms, both distinct and overlapping, by which PH develops and that differ among β-thalassemia major or intermedia patients. PH in β-thalassemia major correlates with the severity of hemolysis, yet in patients whose disease is well treated with chronic transfusion therapy, the development of PH can be related to cardiac dysfunction and the subsequent toxic effects of iron overload rather than hemolysis. β-Thalassemia intermedia, on the other hand, has a higher incidence of PH owing to the low level of hemolysis that exists over years without the requirement for frequent transfusions, while splenectomy is shown to play an important role in both types. Standard therapies such as chronic transfusion have been shown to mitigate PH, and appropriate chelation therapy can avoid the toxic effects of iron overload, yet is not indicated in many patients. Limited evidence exists for the use of pulmonary vasodilators or other therapies, such as l-carnitine, to treat PH associated with thalassemia. Here, we review the most recent findings regarding the pathogenic mechanisms, epidemiology, presentation, diagnosis, and treatment of PH in thalassemia syndromes. © 2016 New York Academy of Sciences.

  7. Pulmonary Renal Syndrome After Streptococcal Pharyngitis

    Directory of Open Access Journals (Sweden)

    Gopi Mara-Koosham PhD

    2016-04-01

    Full Text Available Pulmonary renal syndrome is a class of small vessel vasculitides that are characterized by the dual presentation of diffuse alveolar hemorrhage (DAH and glomerulonephritis. Pulmonary renal syndrome has multiple etiologies, but its development has been rarely reported following infection with group A streptococcus. We present the case of a 36-year-old Native American male who was transferred to our facility due to refractory hypoxic respiratory failure. He had been diagnosed with streptococcal pharyngitis 2 weeks prior to admission. Given the presence of hemoptysis, bronchoscopy was performed and was consistent with DAH. Urinalysis demonstrated hematuria and proteinuria, in the setting of elevated creatinine and blood urea nitrogen. Additionally, antistreptolysin O titer was positive. Given the constellation of laboratory findings and history of streptococcal pharyngitis, the patient was diagnosed with PRS secondary to streptococcal infection. High-dose methylprednisolone was initiated with concomitant plasmapheresis. He was extubated successfully after his respiratory status improved and was eventually discharged home after making a full recovery within 2 weeks after admission. This case illustrates the importance of clinically relevant sequelae of streptococcal infection as well as the appropriate treatment of PRS secondary to streptococcal pharyngitis with plasmapheresis and intravenous corticosteroids.

  8. Central perforation of atretic pulmonary valve using coronary microcatheter

    Directory of Open Access Journals (Sweden)

    Saurabh Kumar Gupta

    2017-01-01

    Full Text Available Percutaneous perforation of pulmonary valve, using 0.014” guidewires meant for coronary artery chronic total occlusion (CTO, is increasingly being performed for select cases of pulmonary atresia with intact ventricular septum (PA-IVS. Despite growing experience, procedural failures and complications are not uncommon. Even in infants treated successfully, the orifice created in the atretic pulmonary valve is eccentric. In this report, we present usefulness of coronary microcatheter in alignment of perforating coronary guidewire to the center of atretic pulmonary valve resulting in central perforation.

  9. Pulmonary pathology of acute respiratory distress syndrome.

    Science.gov (United States)

    Tomashefski, J F

    2000-09-01

    Lung morphology in ARDS reflects the rapid evolution from interstitial and alveolar edema to end-stage fibrosis consequent to injury of the alveolocapillary unit. This morphologic progression, termed diffuse alveolar damage, has been subdivided into sequentially occurring exudative, proliferative, and fibrotic phases. Pulmonary lesions correlate with the phase of alveolar damage rather than its specific cause. The pathologic features are consistent with the effects of a host of injurious stimuli and the complex interaction of inflammatory mediators on alveolar epithelial and capillary endothelial cells. Although ARDS frequently culminates in "interstitial" fibrosis, the organization of intraluminal exudate dominates the histologic picture in the proliferative phase and establishes the framework for subsequent fibrous remodeling of the lung. Involvement of the pulmonary vasculature is an important aspect of ARDS, from the initial phase of edema to the terminal stage of intractable pulmonary hypertension. Vascular lesions include thrombotic, fibroproliferative, and obliterative changes that, like the parenchymal lesions, correlate with the temporal phase of DAD. Although ARDS is characterized by extensive bilateral lung involvement, alveolar damage can also affect the lung in a localized fashion. RAD is associated with the same clinical risk factors as DAD, suggesting that there is a spectrum in the extent of lung involvement and disease severity in patients at risk for ARDS. The factors that govern which patients will develop the fulminant syndrome are poorly understood. It must be re-emphasized that the lung is stereotyped in its response to injury and, consequently, descriptive, or even quantitative, studies of lung morphology can only provide clues regarding the initiating factors and pathogenetic mechanisms of ARDS. Progress in understanding the pathogenesis of ARDS and development of rational approaches to therapy will ultimately depend on careful clinical and

  10. Swyer-James-Macleod Syndrome Presenting with Pulmonary Hypertension

    Directory of Open Access Journals (Sweden)

    Sh Hajsadeghi

    2010-09-01

    Full Text Available Swyer–James–MacLeod Syndrome is a rare condition as a result of childhood pulmonary infection, especially bronchiolitis obliterans or viral bronchiolitis/pneumonia. It appears as increased radiolucency on chest Xray, in the absence of obstructing lesions and can be confused for other thoracic disease processes such as a large pulmonary emboli or congenital bronchial and/or pulmonary vasculature malformations. We introduce a 46-year-old male patient presented with symptoms and signs of pulmonary hypertension which was initially misdiagnosed as chronic pulmonary emboli. This case highlights the possibility of pulmonary hypertension to be one of the cardinal manifestations of this syndrome, and outlines the significance of application of computedtomography in confirming the diagnosis of SJMS and in eliminating other diseases.

  11. Signs and Symptoms for Hantavirus Pulmonary Syndrome (HPS)

    Science.gov (United States)

    ... page: About CDC.gov . Hantavirus Share Compartir Signs & Symptoms for Hantavirus Pulmonary Syndrome (HPS) Due to the ... urine, droppings, or saliva of infected rodents. Early Symptoms Early symptoms include fatigue, fever and muscle aches, ...

  12. Successful Pulmonary Endarterectomy in a Patient with Klinefelter Syndrome

    Directory of Open Access Journals (Sweden)

    E. Wierda

    2012-01-01

    Full Text Available Klinefelter syndrome (KS is a frequent genetic disorder due to one or more supernumerary X chromosomes. KS is associated with an increased risk for venous thromboembolic events like deep venous thrombosis and pulmonary embolism. This paper describes a 37-year-old male patient with KS referred to our tertiary center with chronic thromboembolic pulmonary hypertension, and who was successfully treated by pulmonary endarterectomy.

  13. central hospital with acute coronary syndrome

    African Journals Online (AJOL)

    consecutivement admi a l'Hopital Central d'Aseer avec le diag- nostic d'un syndrome ... Unit of Aseer Central Hospital with the diagnosis of acute coro- nary syndrome for 14 .... action: consumer information processing. In: G] IDZ K, Lewis.

  14. [Hantavirus pulmonary syndrome in Buenos Aires, 2009-2014].

    Science.gov (United States)

    Iglesias, Ayelén A; Bellomo, Carla M; Martínez, Valeria P

    2016-01-01

    Andes virus is the causative agent of hantavirus pulmonary syndrome (HPS) in Argentina and neighboring countries. In our country four different areas are affected: Northwest, Southwest, Central and Northeast, where distinct Andes virus genotypes were characterized. Three genotypes were described in Buenos Aires province (Central area): AND-Buenos Aires, AND-Lechiguanas and AND-Plata. In this work, we considered all HPS cases confirmed by ELISA and real time RT-PCR during the period 2009-2014 in Buenos Aires province. The annual distribution, fatality rate and geographic distribution were analyzed. We also analyzed the genotypes involved by RT-PCR and nucleotide sequencing. Finally we evaluated epidemiological data in order to establish the route of transmission. We analyzed 1386 suspect cases of hantavirus infection from Buenos Aires province and we confirmed 88 cases of Hantavirus Pulmonary Syndrome during 2009-2014. The overall average was 14.3 cases per year. The occurrence of a HPS outbreak was confirmed in Buenos Aires province during 2013, showing a 3 fold increase in case number compared to the annual average between 2009 and 2012, tending to normalize during 2014. The overall lethality was 25.6%, with a maximum value of 45.5% in 2011. Genotype analysis was performed in 30.7% of confirmed cases, AND-BsAs show the highest incidence, it was characterized in 72% of the studied cases. Epidemiological data and results of viral genome comparison strongly suggest person-to-person transmission in the three clusters of two cases described in our study.

  15. Pulmonary and extrapulmonary acute respiratory distress syndrome: myth or reality?

    Science.gov (United States)

    Rocco, Patricia R M; Pelosi, Paolo

    2008-02-01

    The pathogenesis of acute respiratory distress syndrome has been explained by the presence of a direct (pulmonary) or indirect (extrapulmonary) insult to the lung parenchyma. Evidence indicates that the pathophysiology of acute respiratory distress syndrome may differ according to the type of the insult. This article presents a brief overview of the differences between pulmonary and extrapulmonary acute respiratory distress syndrome, and discusses the interactions between lung functional, morphological aspects, and response to different therapies, both in experimental models and in patients with acute respiratory distress syndrome. Many researchers recognize that experimental pulmonary and extrapulmonary acute respiratory distress syndrome are not identical when considering morpho-functional aspects, the response to positive end-expiratory pressure and recruitment manoeuvre, prone position and other adjunctive therapies. Contradictory results have been reported in different clinical studies, however, which may be attributed to the difficulty of classifying acute respiratory distress syndrome in one or the other category, and being confident of the onset, the phase and the severity of acute respiratory distress syndrome in all patients. Heterogeneous acute respiratory distress syndrome patients are still considered to suffer from one syndrome, and are treated in the same way. Understanding the range of different pathways that lead to pulmonary dysfunction makes it possible to better target clinical treatment.

  16. Pulmonary overinflation syndrome in an underwater logger.

    Science.gov (United States)

    Rozali, A; Sulaiman, A; Zin, B Mohd; Khairuddin, H; Abd-Halim, M; Sherina, M S

    2006-10-01

    Pulmonary overinflation syndrome (POIS) is a group of barotrauma-related diseases caused by the expansion of gas trapped in the lung, or over-pressurization of the lung with subsequent over-expansion and rupture of the alveolar air sacs. This group of disorders includes arterial gas embolism, tension pneumothorax, mediastinal emphysema, subcutaneous emphysema and rarely pneumopericardium. In the case of diving activities, POIS is rarely reported and is frequently related to unsafe diving techniques. We report a classical case of POIS in an underwater logger while cutting trees for logs in Tasik Kenyir, Terengganu. The patient, a 24-year-old worker, made a rapid free ascent to the surface after his breathing equipment malfunctioned while he was working underwater. He suffered from bilateral tension pneumothoraces, arterial gas embolism giving rise to multiple cerebral and cerebellar infarcts, mediastinal and subcutaneous emphysema as well as pneumopericardium. He was treated in a recompression chamber with hyperbaric oxygen therapy and discharged with residual weakness in his right leg.

  17. A Challenging Twist in Pulmonary Renal Syndrome

    Directory of Open Access Journals (Sweden)

    Rajaie Namas

    2014-01-01

    Full Text Available Case. We report a rare case of hydralazine-induced anti-neutrophil cytoplasmic antibody-associated vasculitis. A 75-year-old African American woman with history of high blood pressure on hydralazine for 3 years presented with acute onset of shortness of breath and hemoptysis. Lab workup revealed a severe normocytic anemia and a serum creatinine of 5.09 mg/dL (baseline 0.9. Bronchoscopy demonstrated active pulmonary hemorrhage. A urine sample revealed red cell casts and a renal biopsy demonstrated pauci-immune, focally necrotizing glomerulonephritis with small crescents consistent with possible anti-neutrophil cytoplasmic antibody-positive renal vasculitis. Serologies showed high-titer MPO-ANCA and high-titer anti-histone antibodies. She was treated with intravenous steroids and subsequently with immunosuppression after cessation of hydralazine. The patient was subsequently discharged from hospital after a rapid clinical improvement. Conclusion. Hydralazine-induced anti-neutrophil cytoplasmic antibody-positive renal vasculitis is a rare adverse effect and can present as a severe vasculitic syndrome with multiple organ involvement. Features of this association include the presence of high titer of anti-myeloperoxidase anti-neutrophil cytoplasmic antibody with multiantigenicity, positive anti-histone antibodies, and the lack of immunoglobulin and complement deposition. Prompt cessation of hydralazine may be sufficient to reverse disease activity but immunosuppression may be needed.

  18. Case Report: Bazex Syndrome Associated With Pulmonary Adenocarcinoma

    OpenAIRE

    Zhao, Jing; Zhang, Xilin; Chen, Zhuo; Wu, Jian-Hua

    2016-01-01

    Abstract Bazex syndrome, a rare paraneoplastic syndrome characterized by psoriasiform eruptions, palmoplantar keratosis, and symmetric onychodystrophy, is most prevalent with squamous cell carcinomas of the upper aerodigestive tract. Here, we reported an uncommon case of Bazex syndrome about an 83-year-old man with pulmonary adenocarcinoma and osseous metastasis, Physical examination found psoriasiform eruptions on the nose, cheeks, ears, knees, and the dorsa of interphalangeal joints, along ...

  19. Pulmonary hypertension due to acute respiratory distress syndrome

    Directory of Open Access Journals (Sweden)

    S.A. Ñamendys-Silva

    2014-10-01

    Full Text Available Our aims were to describe the prevalence of pulmonary hypertension in patients with acute respiratory distress syndrome (ARDS, to characterize their hemodynamic cardiopulmonary profiles, and to correlate these parameters with outcome. All consecutive patients over 16 years of age who were in the intensive care unit with a diagnosis of ARDS and an in situ pulmonary artery catheter for hemodynamic monitoring were studied. Pulmonary hypertension was diagnosed when the mean pulmonary artery pressure was >25 mmHg at rest with a pulmonary artery occlusion pressure or left atrial pressure <15 mmHg. During the study period, 30 of 402 critically ill patients (7.46% who were admitted to the ICU fulfilled the criteria for ARDS. Of the 30 patients with ARDS, 14 met the criteria for pulmonary hypertension, a prevalence of 46.6% (95% CI; 28-66%. The most common cause of ARDS was pneumonia (56.3%. The overall mortality was 36.6% and was similar in patients with and without pulmonary hypertension. Differences in patients' hemodynamic profiles were influenced by the presence of pulmonary hypertension. The levels of positive end-expiratory pressure and peak pressure were higher in patients with pulmonary hypertension, and the PaCO2 was higher in those who died. The level of airway pressure seemed to influence the onset of pulmonary hypertension. Survival was determined by the severity of organ failure at admission to the intensive care unit.

  20. Campylobacter-Associated Hemolytic Uremic Syndrome Associated with Pulmonary-Renal Syndrome.

    Science.gov (United States)

    Bowen, Emily Elizabeth; Hangartner, Robert; Macdougall, Iain

    2016-03-01

    Common causes of pulmonary-renal syndrome include anti-glomerular basement membrane (anti-GBM) disease anti-neutrophil cytoplasmic antibody (ANCA) positive vasculitis, and systemic lupus erythematosus. We describe a case of life-threatening pulmonary hemorrhage associated with Campylobacter hemolytic uremic syndrome (HUS), which we believe is a new disease entity. We hypothesize that the cause of this pulmonary-renal syndrome was an immunological reaction to Campylobacter; and that the initiation of high-dose steroids was responsible for the rapid reversal of the patient's pulmonary and renal impairment. The aim of this article is to raise awareness of this unusual cause of a pulmonary-renal syndrome, guiding physicians to recognize it as a potential complication, and to consider high-dose steroids in managing the condition.

  1. Pulmonary-Renal Syndrome with a Focus on Anti-GBM Disease

    NARCIS (Netherlands)

    Sanders, Jan-Stephan F.; Rutgers, Abraham; Stegeman, Coen A; Kallenberg, Cees G.M.

    Pulmonary-renal syndrome is a potentially life-threatening combination of pulmonary hemorrhage and acute renal failure. Several pathological entities can cause this syndrome. This review discusses the diagnostic strategy required to initiate appropriate therapy. Rapid serological testing and

  2. [Hantavirus pulmonary syndrome in southern Argentina].

    Science.gov (United States)

    Lázaro, M E; Resa, A J; Barclay, C M; Calanni, L; Samengo, L; Martinez, L; Padula, P J; Pini, N; Lasala, M B; Elsner, B; Enria, D A

    2000-01-01

    Andes virus was identified in 1995 as the etiologic agent of Hantavirus Pulmonary Syndrome (HPS) in Southern Argentina. We describe herein the main clinical characteristics of 25 HPS confirmed cases acquired in this area between 1993 and September 1999. The mean age was 34 years (range 11-70), with 72% males. Clinical characteristics were similar to those previously reported for Sin Nombre virus (SNV) cases. However, in this group of patients we also observed conjuntival injection in 10/25 (42%), facial flushing in 8/25 (33%), pharyngeal congestion in 7/25 (29%) and petechiae in 3/25 (12%). On the other hand, BUN was increased in 83% of cases (mean 0.77 g/l range 0.31-2.01). Mean serum creatinine concentration was 26.8 mg/l (range: 8.1-110 mg/l) with serum creatinine being higher than 20 mg/l in 8/15 patients (53%). Urinalysis was abnormal in 12/12 cases and was characterized by presence of proteins, red blood cells and granular casts. Aminotransferases were increased in 90% of cases with levels 5-10 times over normal values in 50% of cases. Serum creatine kinase concentration was elevated in 11/14 cases. Two patients required hemodialysis. Case fatality rate was 44% (11/25) and 10 of these cases died among the first 10 days of illness. Mononuclear myocarditis was observed in two cases, a finding that has not been reported for SNV cases. During the 1996 HPS outbreak in Southern Argentina due to Andes virus, there were epidemiological and molecular evidences of person-to-person transmission, a feature not previously shown for other members of the hantavirus genus. These data would also be indicative of some distinctive clinical characteristics of HPS caused by Andes virus, with more frequent renal involvement than in SNV cases.

  3. Paraneoplastic neurological syndrome as initial presentation of pulmonary adenocarcinoma

    OpenAIRE

    Shahani, Lokesh

    2012-01-01

    Paraneoplastic neurological syndromes are defined as remote effects of cancer that are not caused by the tumour and its metastasis, or by infection, ischaemia or metabolic disruptions. The author reports a 63-year-old Caucasian woman presenting with slowly progressing cognitive deficits. Initial workup failed to reveal any obvious cause of her cognitive deficits. Retrospective medical data review revealed a pulmonary nodule, which on further evaluation revealed pulmonary adenocarcinoma with m...

  4. Nitric Oxide-Sensitive Pulmonary Hypertension in Congenital Rubella Syndrome

    Directory of Open Access Journals (Sweden)

    Francesco Raimondi

    2015-01-01

    Full Text Available Persistent pulmonary hypertension is a very rare presentation of congenital virus infection. We discuss the case of complete congenital rubella syndrome presenting at echocardiography with pulmonary hypertension that worsened after ductus ligation. Cardiac catheterization showed a normal pulmonary valve and vascular tree but a PAP=40 mmHg. The infant promptly responded to inhaled nitric oxide while on mechanical ventilation and was later shifted to oral sildenafil. It is not clear whether our observation may be due to direct viral damage to the endothelium or to the rubella virus increasing the vascular tone via a metabolic derangement.

  5. Hantavirus Pulmonary Syndrome: Report of Four Alberta Cases

    Directory of Open Access Journals (Sweden)

    Ameeta E Singh

    1995-01-01

    Full Text Available Four Alberta cases of hantavirus pulmonary syndrome are reported. Three cases required intensive care, with one experiencing a fulminant course resulting in death. A fourth case with milder illness was identified after epidemiological investigations. Ribavirin was used in one patient who experienced a successful outcome. A recent open label trial has not supported the efficacy of this drug. The epidemiology of Peromyscus maniculatus, the primary rodent host, and the clinical features of this syndrome are summarized.

  6. Pulmonary carcinoid tumor associated with nephrotic syndrome.

    Science.gov (United States)

    DePace, N L; Elquezabal, A; Hardenburg, H C

    1980-04-01

    A patient with carcinoid tumor of the lung associated with nephrotic syndrome was treated. Excision of the tumor resulted in remission of marked proteinuria, hypoalbuminemia, and edema. A review of the literature disclosed many neoplasms associated with the nephrotic syndrome; however, no association of the nephrotic syndrome and a carcinoid tumor of the lung has previously been reported, to our knowledge.

  7. Pulmonary Hypertension Syndrome in Chicken: Peeking Under QTL Peaks

    NARCIS (Netherlands)

    Rabie, T.

    2004-01-01

    Pulmonary hypertension syndrome (PHS) is characterized a cascade of events resulting in cardiac anomalies including an enlarged heart, right ventricular hypertrophy, variable liver changes, and accumulation of fluid in the abdominal cavity (ascites). PHS is not a disease; it is a condition in which

  8. Hantavirus pulmonary syndrome outbreak, Brazil, December 2009-January 2010.

    Science.gov (United States)

    Terças, Ana Cláudia Pereira; Atanaka dos Santos, Marina; Pignatti, Marta Gislene; Espinosa, Mariano Martinez; Via, Alba Valéria Gomes de Melo; Menegatti, Jaqueline Aparecida

    2013-11-01

    An outbreak of hantavirus pulmonary syndrome occurred in the Sobradinho Indian settlement of the Kayabí ethnic group in northern Mato Grosso during December 2009-January 2010. We conducted a retrospective study to clarify the outbreak's epidemiologic and clinical characteristics. Results suggest a relationship between the outbreak and deforestation and farming expansion in indigenous areas.

  9. Pulmonary dysfunction and hepatopulmonary syndrome in cirrhosis and portal hypertension

    DEFF Research Database (Denmark)

    Møller, Søren; Krag, Aleksander; Madsen, Jan L

    2009-01-01

    BACKGROUND: Pulmonary dysfunction including the hepatopulmonary syndrome (HPS) is an important complication to cirrhosis and portal hypertension. However, the precise relation to liver dysfunction and the prevalence of HPS are unclear. AIMS: We therefore aimed to assess (i) the prevalence of HPS......, Pportal hypertension (post-sinusoidal resistance, P

  10. Asthma, chronic obstructive pulmonary disease (COPD), and the overlap syndrome.

    Science.gov (United States)

    Nakawah, Mohammad Obadah; Hawkins, Clare; Barbandi, Farouk

    2013-01-01

    Asthma and chronic obstructive pulmonary disease (COPD) are highly prevalent chronic diseases in the general population. Both are characterized by heterogeneous chronic airway inflammation and airway obstruction. In both conditions, chronic inflammation affects the whole respiratory tract, from central to peripheral airways, with different inflammatory cells recruited, different mediators produced, and thus differing responses to therapy. Airway obstruction is typically intermittent and reversible in asthma but is progressive and largely irreversible in COPD. However, there is a considerable pathologic and functional overlap between these 2 heterogeneous disorders, particularly among the elderly, who may have components of both diseases (asthma-COPD overlap syndrome). The definitions for asthma and COPD recommended by current guidelines are useful but limited because they do not illustrate the full spectrum of obstructive airway diseases that is encountered in clinical practice. Defining asthma and COPD as separate entities neglects a considerable proportion of patients with overlapping features and is largely based on expert opinion rather than on the best current evidence. The presence of different phenotypes or components of obstructive airway diseases, therefore, needs to be addressed to individualize and optimize treatment to achieve the best effect with the fewest side effects for the patient. Although specific interventions vary by disease, the treatment goals of obstructive airway diseases are similar and driven primarily by the need to control symptoms, optimize health status, and prevent exacerbations.

  11. [Pulmonary hypertension associated with congenital heart disease and Eisenmenger syndrome].

    Science.gov (United States)

    Calderón-Colmenero, Juan; Sandoval Zárate, Julio; Beltrán Gámez, Miguel

    2015-01-01

    Pulmonary arterial hypertension is a common complication of congenital heart disease (CHD). Congenital cardiopathies are the most frequent congenital malformations. The prevalence in our country remains unknown, based on birthrate, it is calculated that 12,000 to 16,000 infants in our country have some cardiac malformation. In patients with an uncorrected left-to-right shunt, increased pulmonary pressure leads to vascular remodeling and endothelial dysfunction secondary to an imbalance in vasoactive mediators which promotes vasoconstriction, inflammation, thrombosis, cell proliferation, impaired apotosis and fibrosis. The progressive rise in pulmonary vascular resistance and increased pressures in the right heart provocated reversal of the shunt may arise with the development of Eisenmenger' syndrome the most advanced form de Pulmonary arterial hypertension associated with congenital heart disease. The prevalence of Pulmonary arterial hypertension associated with CHD has fallen in developed countries in recent years that is not yet achieved in developing countries therefore diagnosed late as lack of hospital infrastructure and human resources for the care of patients with CHD. With the development of targeted medical treatments for pulmonary arterial hypertension, the concept of a combined medical and interventional/surgical approach for patients with Pulmonary arterial hypertension associated with CHD is a reality. We need to know the pathophysiological factors involved as well as a careful evaluation to determine the best therapeutic strategy. Copyright © 2014 Instituto Nacional de Cardiología Ignacio Chávez. Published by Masson Doyma México S.A. All rights reserved.

  12. Case Report: Bazex Syndrome Associated With Pulmonary Adenocarcinoma.

    Science.gov (United States)

    Zhao, Jing; Zhang, Xilin; Chen, Zhuo; Wu, Jian-Hua

    2016-01-01

    Bazex syndrome, a rare paraneoplastic syndrome characterized by psoriasiform eruptions, palmoplantar keratosis, and symmetric onychodystrophy, is most prevalent with squamous cell carcinomas of the upper aerodigestive tract.Here, we reported an uncommon case of Bazex syndrome about an 83-year-old man with pulmonary adenocarcinoma and osseous metastasis, Physical examination found psoriasiform eruptions on the nose, cheeks, ears, knees, and the dorsa of interphalangeal joints, along with plantar keratosis and symmetric onychodystrophy involving hands and feet. Imaging analyses pulmonary adenocarcinoma with both local metastatic nodules and osseous metastasis.Symptomatic treatment with topical corticosteroids and oral retinoids showed no improvement. A 4-month follow-up showed that Gefitinib, an epidermal growth factor receptor tyrosine kinase inhibitor, successfully reduced primary tumor size and alleviated cutaneous lesions.Our report here highlighted a potential correlation between pulmonary adenocarcinoma and Bazex syndrome, which is characterized by hallmark nail destruction and preferential involvement of body extremities. Moreover, etiological therapy against underlying malignancy is essential for treating paraneoplastic Bazex syndrome.

  13. Sweet's syndrome: a very rare association with pulmonary tuberculosis.

    Science.gov (United States)

    Karmakar, Partha Sarathi; Sherpa, Pasang Lahmu; Ray, Achintya Narayan; Saha, Bikram Kr; Santra, Tuhin; Saha, Sanchita; Chakrabarti, Indranil

    2013-05-13

    Mycobacterium tuberculosis infection is a common infection in developing countries, including India. It can induce several cutaneous reactions such as erythema nodosum, and erythema induratum; however, association of tuberculosis with Sweet's syndrome (also known as acute febrile neutrophilic dermatosis) is extremely rare. Here we present an interesting case of sputum-positive pulmonary tuberculosis with Sweet's syndrome. A 55-year-old female who was receiving a regimen of four antitubercular drugs (isoniazid, rifampicin, pyrazinamide, ethambutol- HRZE) for six weeks for sputum-positive pulmonary tuberculosis developed new onset high-grade fever for 15 days along with multiple reddish brown plaques and nodules involving the face as well as all four limbs of the body. Histopathology of the skin lesion was suggestive of Sweet's syndrome. The patient responded well to immunosuppressive steroid therapy.

  14. Report of a girl with vacterl syndrome and right pulmonary agenesis.

    Science.gov (United States)

    Avcu, S; Akgun, C; Temel, H; Arslan, S; Akbayram, S; Unal, O

    2009-01-01

    Report of a girl with VACTERL syndrome and right pulmonary agenesis: VACTERL syndrome is a combination of vertebral anomalies (V), anal atresia (A), congenital heart defects (C), tracheo-esophageal fistula (T), esophageal atresia (E), abnormalities of kidneys (renal anomalies, R) and limbs (L). In the present patient right pulmonary agenesis is co-occurring with VACTERL syndrome. We report on this case because the association of right pulmonary agenesis and VACTERL syndrome is rare.

  15. Metabolic syndrome and central retinal artery occlusion

    Directory of Open Access Journals (Sweden)

    Kosanović-Jaković Natalija

    2005-01-01

    Full Text Available Background. The accumulation of risk factors for central retinal artery occlusion can be seen in a single person and might be explained by the metabolic syndrome. Case report. We presented the case of a 52-year-old man with no light perception in his right eye. The visual loss was monocular and painless, fundoscopy showed central retinal artery occlusion and the laboratory investigation showed the raised erythrocyte sedimentation rate of 105 mm/h and the raised C-reactive protein of 22 mg/l. Specific laboratory investigations and fluorescein angiography excluded the presence of vasculitis, collagen vascular diseases, hypercoagulable state and antiphospholipid syndrome. Conclusion. The patient met all the five of the National Cholesterol Education Program (NCEP criteria for the metabolic syndrome: hypertension, abnormal lipid profile, abnormal glucose metabolism, obesity and hyperuricemia. Measurement of C-reactive protein is useful for the assessment of therapeutic systemic effect on any abnormality in the metabolic syndrome. Individual therapy for all risk factors in the metabolic syndrome is necessary to prevent complications such as cardiovascular, retinal vascular diseases and stroke.

  16. Pulmonary renal syndrome in childhood: a report of twenty-one cases and a review of the literature.

    Science.gov (United States)

    von Vigier, R O; Trummler, S A; Laux-End, R; Sauvain, M J; Truttmann, A C; Bianchetti, M G

    2000-05-01

    In adults, the term specific pulmonary renal syndrome describes disorders with pulmonary and glomerular manifestations and includes Wegener's granulomatosis, Goodpasture disease, and systemic lupus erythematosus. Nonspecific pulmonary renal syndrome refers to either pulmonary disease complicating glomerular disease, or glomerular diseases following pulmonary disease. Since little is known regarding pulmonary renal syndrome in childhood, we reviewed the charts of 21 pediatric patients with pulmonary renal syndromes treated by the Department of Pediatrics, University of Bern between 1991 and 1998; we also reviewed the pediatric literature that deals with specific pulmonary renal syndromes. Specific pulmonary renal syndrome was noted in 3 children with systemic vasculitis (Wegener granulomatosis, N = 2; microscopic polyangiitis, N = 1) and 2 with systemic lupus erythematosus. Nonspecific pulmonary renal syndrome was observed in 12 patients with pulmonary edema (N = 9), pulmonary thromboembolism (N = 2), and pulmonary infection (N = 1) complicating the course of a glomerular disease, and in 4 children with a pulmonary disease followed by a glomerular disease. Review of the literature disclosed 52 cases of specific pulmonary renal syndrome other than systemic lupus erythematosus: Wegener granulomatosis (N = 28), Goodpasture disease (N = 13), and Henoch-Schönlein purpura (N = 11). In addition, hemolytic uremic syndrome complicated pneumococcal pneumonia in 32 cases. We conclude that pulmonary renal syndromes need to be looked for in childhood. Apart from Wegener granulomatosis, Goodpasture disease, and systemic lupus erythematosus, Henoch-Schönlein purpura and hemolytic-uremic syndrome occasionally have both pulmonary and renal features. Copyright 2000 Wiley-Liss, Inc.

  17. Pulmonary and extrapulmonary acute respiratory distress syndrome: are they different?

    Science.gov (United States)

    Garcia, Cristiane S N Baez; Pelosi, Paolo; Rocco, Patricia R M

    2008-06-01

    The pathogenesis of acute respiratory distress syndrome (ARDS) has been described by the presence of direct (pulmonary) and/or indirect (extrapulmonary) insult to the lung parenchyma. Evidence indicates that the pathophysiology of ARDS may differ according to the type of primary insult. This article presents a brief overview of differences between pulmonary and extrapulmonary ARDS, and discusses the interactions between morpho-functional aspects and response to differents therapies, both in experimental and clinical studies. This systematic review included clinical and experimental ARDS studies found in MedLine and SciElo databases in the last 20 years. Many researchers acknowledge that experimental pulmonary and extrapulmonary ARDS are not identical with regard to morpho-functional aspects, the response to positive end-expiratory pressure (PEEP), recruitment manoeuvre, prone position and other adjunctive therapies. However, contradictory results have been reported in different clinical studies, which could be attributed to the difficulty of classifying ARDS in one or the other category, and to the assurance regarding the onset, phase and severity of ARDS in all patients. Heterogeneous ARDS patients are still considered as belonging to one syndrome, and are therefore treated in a similar manner. Thus, it is important to understand the pathophysiology of pulmonary and extrapulmonary ARDS in an attempt to better treat these patients.

  18. Beware of the devastating pulmonary aspergillosis syndromes In certain environments

    LENUS (Irish Health Repository)

    Kooblall, M

    2016-02-01

    Nowadays with more stem cell transplants and immunosuppressive therapies there has been a rise in pulmonary aspergillosis syndromes. The following illustrates such a case. A 49 year old man had a past history of ankylosing spondylitis with a bilateral hip replacement. He was also on surveillance for a superficial bladder tumour since 2007. His chest x-ray in 2008 was normal. In 2010 his CXR showed patchy opacification in the right apex. CT thorax confirm fibrotic changes.

  19. Acute Pulmonary Edema in Patients with Cushing’s Syndrome

    OpenAIRE

    Mitra Niafar; Mehrnoush Toufan; Nooshin Milanchian; Farhad Niafar; Kavous Shahsavari Nia

    2015-01-01

    Introduction Dyspnea refers to difficulty in breathing, and short and shallow breaths. This sign is seen in numerous diseases due to pulmonary, cardiac, metabolic and neurological causes. Among cardiac causes, heart failure is considered the main cause of dyspnea. Cardiac failure is a clinical syndrome associated with a set of symptoms (dyspnea, and fatigue) and signs (edema and rales). Common causes of cardiac failure include: myocardial infarction, ischemic heart disease, hypertensi...

  20. A syndrome of severe idiopathic pulmonary parenchymal disease with pulmonary hypertension in Pekingese

    Directory of Open Access Journals (Sweden)

    Köster LS

    2016-02-01

    Full Text Available Liza S Köster,1 Robert M Kirberger2 1Section of Medicine, Department of Clinical Sciences, Integrative Mammalian Research (IMR Center, Ross University School of Veterinary Medicine (RUSVM, Basseterre, St Kitts, West Indies; 2Diagnostic Imaging Section, Department of Companion Animal Clinical Studies, Faculty of Veterinary Science, University of Pretoria, Onderstepoort, South Africa Abstract: This paper describes 35 Pekingese dogs with a syndrome characterized by dyspnea, cyanosis, episodic syncope, soft pulmonary “Velcro” crackles, pulmonary hypertension (PH, and computed tomography and radiographic changes consistent with pulmonary parenchymal disease. The medical data base was searched with the criteria “Pekingese” and “syncope” or “dyspnea” or “tachypnea” or “pulmonary hypertension”, over a 36-month period. Inclusion criteria were echocardiographic changes consistent with noninvasive diagnosis of PH, either subjectively by B-mode or objectively by Doppler. Dogs were excluded (n=106 if there were insufficient or poor-quality radiographic or echocardiographic records or if diseases other than chronic pulmonary disease were found to be the etiology. The records of 35 dogs met these criteria and presented with a respiratory crises preceded by a history of chronic exercise intolerance and episodic syncope. The average age was 14.5 years (range: 7–19 years, with 21 males and 14 females. Most of the dogs had an interstitial lung pattern with radiographic evidence of right heart enlargement. There was a 77% (n=27 mortality and a median survival of 60 days (interquartile range: 9–210 days. This study highlights a cor pulmonale syndrome from PH due to chronic pulmonary parenchymal disease, with a grave prognosis, in middle-aged to geriatric population of Hong Kong Pekingese. Keywords: computed tomography, interstitial lung disease, dog, syncope

  1. Sheehan's syndrome with central diabetes insipidus.

    Science.gov (United States)

    Laway, Bashir Ahmad; Mir, Shahnaz Ahmad; Dar, Mohd Iqbal; Zargar, Abdul Hamid

    2011-03-01

    Sheehan's syndrome refers to the occurrence of hypopituitarism after delivery, usually preceded by postpartum hemorrhage. The condition still continues to be a common cause of hypopituitarism in developing countries like India. The disorder usually presents with anterior pituitary failure with preservation of posterior pituitary functions. Posterior pituitary dysfunction in the form of central diabetes insipidus is rare in patients with Sheehan's syndrome. We describe the clinical course of a young lady who after her sixth childbirth developed severe postpartum hemorrhage followed by development of panhypopituitarism which was confirmed by hormonal investigation and demonstration of empty sella on imaging. In addition, she developed Polyuria. The water deprivation test and response to vasopressin test results indicated central diabetes insipidus. She needed oral desmopressin on a continuous basis to control polyuria.

  2. Neuroleptic malignant syndrome presenting as pulmonary edema and severe bronchorrhea.

    Science.gov (United States)

    Patel, U; Agrawal, M; Krishnan, P; Niranjan, S

    2002-04-01

    Neuroleptic malignant syndrome is a rare (incidence, 0.02%-3.2%) but dangerous complication following the use of neuroleptic drugs. When not promptly recognized, this disease carries a high mortality (10%-20%) and morbidity rate. We report an unusual case of neuroleptic malignant syndrome that presented predominantly with autonomic instability in the form of recurrent episodes of respiratory distress. The respiratory distress was initially caused by pulmonary edema and later was caused by severe bronchorrhea. We propose that aspiration pneumonia resulting in respiratory failure, the leading cause of death in neuroleptic malignant syndrome, may be a result of a combination of altered mental status and bronchorrhea. This has therapeutic implications because early institution of bromocriptine/dantrolene can prevent aspiration pneumonia and, hence, mortality from respiratory failure.

  3. Atypical measles syndrome: unusual hepatic, pulmonary, and immunologic aspects.

    Science.gov (United States)

    Frey, H M; Krugman, S

    1981-01-01

    The atypical measles syndrome is a relatively new disease that was first recognized 15 years ago. Initially, it occurred in children who were exposed to wild measles virus several years after they were immunized with killed measles vaccine. It was characterized by a two- to three-day prodrome of high fever, cough, headache, and myalgia followed by a rash that resembled Rocky Mountain spotted fever, scarlet fever, or varicella and associated with roentgenographic evidence of pneumonia with or without pleural effusion. This report highlights three unusual manifestations of this syndrome: 1) transient hepatitis, 2) persistence of pulmonary lesions for several years, and 3) occurrence of excessively high measles hemagglutination-inhibition antibody titers. Today, this syndrome occurs predominantly in adolescents and young adults.

  4. Cushing Syndrome Secondary to Thymic Carcinoid Synchronous With Tuberculous Lymphadenitis and Pulmonary Tuberculosis: A Case

    OpenAIRE

    F Esfahanian; Sadeghi, A.

    2007-01-01

    Thymic carcinoid is an uncommon neoplasm that can present with Cushing syndrome.We report a 39-year old woman with symptoms of Cushing syndrome secondary to thymic carcinoid and synchronous with tuberculous lymphadenitis and pulmonary tuberculosis. 

  5. Pulmonary manifestations of Sjögren's syndrome.

    Science.gov (United States)

    Flament, Thomas; Bigot, Adrien; Chaigne, Benjamin; Henique, Helene; Diot, Elisabeth; Marchand-Adam, Sylvain

    2016-06-01

    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. 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.

  7. Acute Pulmonary Edema in Patients with Cushing’s Syndrome

    Directory of Open Access Journals (Sweden)

    Mitra Niafar

    2015-01-01

    Full Text Available IntroductionDyspnea refers to difficulty in breathing, and short and shallow breaths. This sign is seen in numerous diseases due to pulmonary, cardiac, metabolic and neurological causes. Among cardiac causes, heart failure is considered the main cause of dyspnea.Cardiac failure is a clinical syndrome associated with a set of symptoms (dyspnea, and fatigue and signs (edema and rales. Common causes of cardiac failure include: myocardial infarction, ischemic heart disease, hypertension, valvular heart diseases, and cardiomyopathy. Among uncommon causes of heart failure, endocrine disorders such as Cushing’s syndrome can be cited. Cushing’s syndrome can present itself in less common forms such as dyspnea due to heart failure. Cushing’s syndrome’s cardiovascular complications usually occur due to hypertension, end organ damage such as left ventricular heart failure, diastolic and ischemic myocardial heart failure, which are rather seen in chronic cases of the disease and are often irreversible.Transient heart failure in patients with Cushing’s syndrome, due to adrenal adenoma, has been reported in a number of patients. In this case report, a patient is introduced who presented to emergency department with severe dyspnea (FC III, and was ultimately diagnosed with Cushing’s syndrome after work up. Three months after treatment of Cushing’s syndrome, dramatic improvement was observed in this patient’s cardiac function.

  8. Earlier Pulmonary Valve Replacement in Down Syndrome Patients Following Tetralogy of Fallot Repair.

    Science.gov (United States)

    Sullivan, Rachel T; Frommelt, Peter C; Hill, Garick D

    2017-08-01

    The association between Down syndrome and pulmonary hypertension could contribute to more severe pulmonary regurgitation after tetralogy of Fallot repair and possibly earlier pulmonary valve replacement. We compared cardiac magnetic resonance measures of pulmonary regurgitation and right ventricular dilation as well as timing of pulmonary valve replacement between those with and without Down syndrome after tetralogy of Fallot repair. Review of our surgical database from 2000 to 2015 identified patients with tetralogy of Fallot with pulmonary stenosis. Those with Down syndrome were compared to those without. The primary outcome of interest was time from repair to pulmonary valve replacement. Secondary outcomes included pulmonary regurgitation and indexed right ventricular volume on cardiac magnetic resonance imaging. The cohort of 284 patients included 35 (12%) with Down syndrome. Transannular patch repair was performed in 210 (74%). Down syndrome showed greater degree of pulmonary regurgitation (55 ± 14 vs. 37 ± 16%, p = 0.01) without a significantly greater rate of right ventricular dilation (p = 0.09). In multivariable analysis, Down syndrome (HR 2.3, 95% CI 1.2-4.5, p = 0.02) and transannular patch repair (HR 5.5, 95% CI 1.7-17.6, p = 0.004) were significant risk factors for valve replacement. Those with Down syndrome had significantly lower freedom from valve replacement (p = 0.03). Down syndrome is associated with an increased degree of pulmonary regurgitation and earlier pulmonary valve replacement after tetralogy of Fallot repair. These patients require earlier assessment by cardiac magnetic resonance imaging to determine timing of pulmonary valve replacement and evaluation for and treatment of preventable causes of pulmonary hypertension.

  9. Efficacy and safety of oral sildenafil in children with Down syndrome and pulmonary hypertension.

    Science.gov (United States)

    Beghetti, Maurice; Rudzinski, Andrzej; Zhang, Min

    2017-07-04

    Despite the increased risk for pulmonary hypertension in children with Down syndrome, the response to treatment with targeted therapies for pulmonary hypertension in these patients is not well characterized. The Sildenafil in Treatment-naive children, Aged 1-17 years, with pulmonary arterial hypertension (STARTS-1) trial was a dose-ranging study of the short-term efficacy and safety of oral sildenafil in children with pulmonary arterial hypertension. We assessed the safety and efficacy of oral sildenafil in children with Down syndrome and pulmonary arterial hypertension. This was a post-hoc analysis of children with Down syndrome and pulmonary arterial hypertension enrolled in the STARTS-1 trial. Mean pulmonary arterial pressure (mPAP), pulmonary vascular resistance index (PVRI), and cardiac index (CI) were assessed at baseline and following 16 weeks of treatment with sildenafil. Of 234 patients randomized and treated in the STARTS-1 trial, 48 (20.5%) had Down syndrome. Although sildenafil produced dose-related reductions in PVRI and mPAP, compared with placebo, in non-Down syndrome patients and children developmentally able to exercise, this was not satisfactorily marked in patients with Down syndrome. The dose-related reductions in PVRI, compared with placebo, occurred in all subgroups, with the exception of the Down syndrome subgroup. Sildenafil appeared to be well tolerated in the Down syndrome subpopulation and the most frequently reported AEs were similar to those reported for the entire STARTS-1 population. Sildenafil treatment for 16 weeks had no effect on PVRI or mPAP in children with Down syndrome and pulmonary arterial hypertension. The results suggest that children with Down syndrome may be less responsive to sildenafil for pulmonary arterial hypertension, but the incomplete work-up for the etiology of pulmonary arterial hypertension may have introduced a potential bias. Study received, September 8, 2005 (retrospectively registered); Study start

  10. Pulmonary-Renal Syndrome with a Focus on Anti-GBM Disease

    NARCIS (Netherlands)

    Sanders, Jan-Stephan F.; Rutgers, Abraham; Stegeman, Coen A; Kallenberg, Cees G.M.

    2011-01-01

    Pulmonary-renal syndrome is a potentially life-threatening combination of pulmonary hemorrhage and acute renal failure. Several pathological entities can cause this syndrome. This review discusses the diagnostic strategy required to initiate appropriate therapy. Rapid serological testing and appropr

  11. Blue Rubber Bleb Nevus Syndrome with Musculo-skeletal Involvement and Pulmonary Stenosis.

    Science.gov (United States)

    Singal, A; Vohra, S; Sharma, R; Bhatt, S

    2016-06-08

    Blue rubber bleb nevus syndrome is a rare clinical entity. A 13-year-old Indian boy presented with characteristic cutaneous lesions, gastrointestinal malformations, skeletal involvement and pulmonary stenosis. Diagnosis was confirmed on skin biopsy, radiographic evaluation, colonoscopy and echocardiography. Echocardiography revealed pulmonary stenosis, an association hitherto undescribed. Detailed evaluation in a patient of blue rubber bleb nerves syndrome is mandatory.

  12. Anesthetic management of a parturient with primary pulmonary hypertension with Eisenmenger's syndrome

    Directory of Open Access Journals (Sweden)

    Kirti N Saxena

    2016-01-01

    Full Text Available Labor and delivery are associated with high mortality in parturients with primary pulmonary hypertension with Eisenmenger's syndrome. Epidural anesthesia during labor has been shown to be effective in reducing the morbidity and mortality. We administered epidural labor analgesia successfully to a parturient with primary pulmonary hypertension with Eisenmenger's syndrome. A team approach for such patients leads to successful outcome.

  13. A Review of Select Centralized Pain Syndromes

    Directory of Open Access Journals (Sweden)

    David R. Spiegel

    2015-01-01

    Full Text Available Pain can be broadly divided into 3 classes, including nociceptive or inflammatory pain (protective, neuropathic (pathological, occurring after damage to the nervous system, or centralized (pathological, due to abnormal function but with no damage or inflammation to the nervous system. The latter has been posited to occur when descending analgesic pathways are attenuated and/or glutamatergic transmission is facilitated. Additionally, this “pain prone phenotype” can be associated with early life trauma and a suboptimal response to opiates. This article will review the relationships between centralized pain syndromes (ie, fibromyalgia, chronic low back pain, childhood sexual abuse, and opiate misuse. Finally, treatment implications, potentially effecting primary care physicians, will be discussed.

  14. A Review of Select Centralized Pain Syndromes

    Directory of Open Access Journals (Sweden)

    David R. Spiegel

    2015-01-01

    Full Text Available Pain can be broadly divided into 3 classes, including nociceptive or inflammatory pain (protective, neuropathic (pathological, occurring after damage to the nervous system, or centralized (pathological, due to abnormal function but with no damage or inflammation to the nervous system. The latter has been posited to occur when descending analgesic pathways are attenuated and/or glutamatergic transmission is facilitated. Additionally, this “pain prone phenotype” can be associated with early life trauma and a suboptimal response to opiates. This article will review the relationships between centralized pain syndromes (ie, fibromyalgia, chronic low back pain, childhood sexual abuse, and opiate misuse. Finally, treatment implications, potentially effecting primary care physicians, will be discussed.

  15. Pulmonary embolism as the primary presenting feature of nephrotic syndrome

    Directory of Open Access Journals (Sweden)

    Pallavi Periwal

    2016-01-01

    Full Text Available A 36-year-old previously healthy male presented with subacute onset of shortness of breath and chest pain. He was diagnosed with bilateral extensive pulmonary embolism (PE. In the absence of any predisposing factors, an extensive workup for unprovoked thrombophilia was done. During the course of his illness, the patient developed anasarca and was diagnosed to be suffering from nephrotic syndrome (NS, secondary to membranous glomerulopathy. Although, thrombotic complications are commonly associated with NS, it is unusual for PE to be the primary presenting feature in these patients.

  16. Hantavirus Pulmonary Syndrome: Report of the First Canadian Paediatric Case

    Directory of Open Access Journals (Sweden)

    Bonita E Lee

    1998-01-01

    Full Text Available Hantavirus pulmonary syndrome (HPS was first recognized as a severe respiratory illness transmitted through rodent excreta in the southwestern United States in 1993. As of November 1997, 175 cases have been reported in the United States. The mortality rate of this disease has been reported to be as high as 52% in the United States, and the majority of the cases (94% involved adults. Twenty-one cases have been recognized in Canada. This paper describes the first Canadian paediatric case and discusses some of the clinical features of this disease.

  17. Increased pulmonary technetium-99m DTPA accumulation in a patient with Goodpasture's syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Oster, Z.H.; Atkins, H.L.

    1985-10-01

    Goodpasture's syndrome is characterized by the triad of glomerulonephritis, intra-alveolar pulmonary hemorrhage, and presence of antiglomerular basement membrane antibodies. It has been shown earlier that intra-alveolar hemorrhage can be detected with radioisotopic techniques. A patient with Goodpasture's syndrome showing increased pulmonary accumulation of Tc-99m DTPA with no evidence of pulmonary hemorrhage is presented.

  18. [Non-cardiogenic pulmonary edema, acute respiratory distress syndrome].

    Science.gov (United States)

    Skalická, Hana; Bělohlávek, Jan

    2015-01-01

    Non-cardiogenic pulmonary edema is a clinical syndrome manifested by rapidly progressive respiratory distress leading, without therapy, to severe respiratory insufficiency and subsequent multiorgan failure. The pathophysiological causes are: the change in the pressure gradients in the pulmonary capillaries, the impaired membrane permeability of the alveolocapillary in the lungs, and impaired lymphatic drainage. Unlike in cardiogenic pulmonary edema, cardiac disease is not a cause, and there is no increase in wedge pressure (< 18 mm Hg). The aetiological base is diverse and includes more clinical pathological factors. The diagnosis and evaluation are usually very difficult due to the rapidly deteriorating clinical condition of the patients. A decisive, quick and comprehensive approach, using all available invasive and non-invasive methods is necessary. The basic steps of treatment are: the use of different types of ventilatory support in order to achieve adequate oxygenation, dealing with possible hemodynamic instability, and, when needed, other specific procedures. It is always important to keep in mind that this is a very serious condition with a high mortality rate. And there is a need for fast and efficient access to the best specialized clinic.

  19. Eisenmenger syndrome and idiopathic pulmonary arterial hypertension: do parenchymal lung changes reflect aetiology?

    Energy Technology Data Exchange (ETDEWEB)

    Griffin, N. [Royal Brompton and Harefield NHS Trust, London (United Kingdom)]. E-mail: nyreegriffin@hotmail.com; Allen, D. [Royal Brompton and Harefield NHS Trust, London (United Kingdom); Wort, J. [Royal Brompton and Harefield NHS Trust, London (United Kingdom); Rubens, M. [Royal Brompton and Harefield NHS Trust, London (United Kingdom); Padley, S. [Royal Brompton and Harefield NHS Trust, London (United Kingdom)

    2007-06-15

    Aim: To document the pulmonary vascular changes on thin-section computed tomography (CT) in patients with Eisenmenger syndrome and idiopathic pulmonary arterial hypertension, and to determine whether there is any correlation with pulmonary arterial pressures or the aetiology of pulmonary hypertension. Material and methods: From the National Pulmonary Hypertension Database, we identified eight patients with idiopathic pulmonary arterial hypertension and 20 patients with Eisenmenger syndrome (secondary to a ventriculoseptal defect) who had also undergone contrast-enhanced thin-section CT. CT studies were reviewed for the presence of centrilobular nodules, mosaicism, neovascularity, and bronchial artery hypertrophy. Haemodynamic data were also reviewed. Results: Centrilobular nodules, mosaicism, and neovascularity were seen in both patient groups (p > 0.05). A significantly higher number of enlarged bronchial arteries were seen in patients with Eisenmenger syndrome. There was no correlation with pulmonary arterial pressures. Conclusion: Patients with idiopathic pulmonary arterial hypertension and Eisenmenger syndrome demonstrated similar pulmonary vascular changes on CT. These changes did not predict the underlying cause of pulmonary hypertension or its severity.

  20. Osteosarcoma of the lumbosacral spine invading the central venous pathways, right-sided cardiac chambers, and pulmonary artery

    Energy Technology Data Exchange (ETDEWEB)

    Hines, Neely; Lantos, George; Hochzstein, Jay [Jacobi Medical Center/Albert Einstein College of Medicine, Department of Radiology, Bronx, NY (United States); Gitig, Alon [Montefiore Medical Center/Albert Einstein College of Medicine, Department of Cardiology, Bronx, NY (United States); DeAnda, Abe [Montefiore Medical Center/Albert Einstein College of Medicine, Department of Cardiothoracic Surgery, Bronx, NY (United States)

    2007-11-15

    We report an unusual case of lumbosacral osteogenic sarcoma with cauda equina syndrome and invasion into the central venous and cardiac system. A 41-year-old Hispanic man presented to the emergency department complaining of severe low back pain, cauda equina syndrome, bilateral lower extremity edema, and an extra heart sound on physical examination. CT of the lumbosacral spine done in the emergency department demonstrated a sclerotic lesion in the sacrum with cortical destruction, extension into the spinal canal and a bulky soft tissue mass containing calcifications. Supplemental MRI demonstrated marrow replacement of L4, L5, and the sacrum, soft tissue extension of the tumor, and invasion iliac veins extending into the IVC; however, the full extent of the intravascular tumor was not seen on this examination. Surgical laminectomy and biopsy of the spinal tumor provided the diagnosis of osteogenic sarcoma. A transthoracic echocardiogram was performed while the patient was recovering due to nonsustained ventricular tachycardia, which showed an echogenic mass within the right atrium and ventricle. CT pulmonary angiogram confirmed the echocardiogram showing a tumor extending through the pulmonary valve into the main pulmonary artery. The patient underwent en bloc resection of the tumor from the venous and cardiac systems. Histologic examination of the tumor confirmed osteogenic sarcoma. While vertebral osteogenic sarcoma is uncommon, invasion of the spinal canal is common in these tumors. However, tumor extending into the central venous and cardiac system is rare. The previously reported cases of central venous and cardiac involvement have been related to distant metastases or primary cardiac osteosarcomas. There is only one other reported case of direct extension into the venous system by an iliac bone osteosarcoma in an adolescent; however, the tumor did not extend into the pulmonary circulation. (orig.)

  1. Effect of pulmonary hyperinflation on central blood volume

    DEFF Research Database (Denmark)

    Mijacika, Tanja; Kyhl, Kasper; Frestad, Daria

    2017-01-01

    (11±7%) above the total lung capacity. All cardiac chambers decreased in volume and despite a heart rate increase of 24±29 bpm (39±50%), pulmonary blood flow decreased by 2783±1820mL (43±20%). The pulmonary transit time remained unchanged at 7.5±2.2s and pulmonary blood volume decreased by 354±176m...

  2. Mounier-Kuhn Syndrome in an Elderly Female with Pulmonary Fibrosis

    Directory of Open Access Journals (Sweden)

    Panagiotis Boglou

    2016-01-01

    Full Text Available Mounier-Kuhn syndrome (MKS, or tracheobronchomegaly, is a rare clinical and radiologic condition characterized by pronounced tracheobronchial dilation and recurrent lower respiratory tract infections. Tracheobronchomegaly presents when the defect extends to the central bronchi. MKS can be diagnosed in adult women when the transverse and sagittal diameters of the trachea, right mainstem bronchus, and left mainstem bronchus exceed 21, 23, 19.8, and 17.4 mm, respectively. Its diagnosis is based on chest radiograph and chest computed tomography (CT. Patients, usually middle-aged men, may be asymptomatic or present with clinical manifestations ranging from minimal symptoms with preserved lung function to severe respiratory failure. Pulmonary function tests (PFTs typically reveal a restrictive pattern. This report presents an elderly woman with previously diagnosed pulmonary fibrosis with symptoms of increased sputum production and haemoptysis. High-resolution chest CT showed tracheal and main stem bronchi dilatation along with bronchial diverticulosis. PFTs indicated a restrictive pattern characteristic of the underlying pulmonary fibrosis. The patient is the oldest, referred to the female gender, at presentation of MKS hitherto reported. This case highlights the need to include MKS in the differential diagnosis of recurrent lower respiratory tract infections, even in older subjects.

  3. Hantavirus pulmonary syndrome in Anajatuba, Maranhão, Brazil

    Directory of Open Access Journals (Sweden)

    MENDES Wellington S.

    2001-01-01

    Full Text Available The authors report a confirmed case of hantavirus pulmonary syndrome in the rural area of the municipality of Anajatuba, state of Maranhão. Two other suspected cases from the same region are also described. The confirmed case involved a previously healthy young woman who died with signs and symptoms of acute respiratory insufficiency 5 days after presenting fever, myalgia and a dry cough. The patient was a student who was helping her parents with work in the fields; it was a habit of the family to store rice inside the house. The suspected cases involved two first-degree relatives working as field hands who died of acute respiratory insufficiency 24 and 48 hours, respectively, after presenting fever, myalgia and a dry cough. Both stored rice and corn inside their home. People living in the region reported massive infestations with rats in the woods and fields.

  4. Understanding asthma-chronic obstructive pulmonary disease overlap syndrome.

    Science.gov (United States)

    Wurst, Keele E; Kelly-Reif, Kaitlin; Bushnell, Greta A; Pascoe, Steven; Barnes, Neil

    2016-01-01

    Asthma-chronic obstructive pulmonary disease overlap syndrome (ACOS) is a loosely-defined clinical entity referring to patients who exhibit characteristics of both asthma and chronic obstructive pulmonary disease (COPD). Clinical definitions and classifications for ACOS vary widely, which impacts our understanding of prevalence, diagnosis and treatment of the condition. This literature review was therefore conducted to characterize the prevalence of ACOS and the effect of different disease definitions on these estimates, as this has not previously been explored. From an analysis of English language literature published from 2000 to 2014, the estimated prevalence of ACOS ranges from 12.1% to 55.2% among patients with COPD and 13.3%-61.0% among patients with asthma alone. This variability is linked to differences in COPD and asthma diagnostic criteria, disease ascertainment methods (spirometry-based versus clinical or symptom-based diagnoses and claims data), and population characteristics including age, gender and smoking. Understanding the reasons for differences in prevalence estimates of ACOS across the literature may help guide decision making on the most appropriate criteria for defining ACOS and aid investigators in designing future ACOS clinical studies aimed at effective treatment.

  5. Hantavirus pulmonary syndrome in a highly endemic area of Brazil.

    Science.gov (United States)

    Oliveira, R C; Sant'ana, M M; Guterres, A; Fernandes, J; Hillesheim, N L F K; Lucini, C; Gomes, R; Lamas, C; Bochner, R; Zeccer, S; DE Lemos, E R S

    2016-04-01

    Hantavirus pulmonary syndrome (HPS) is the most frequently reported fatal rodent-borne disease in Brazil, with the majority of cases occurring in Santa Catarina. We analysed the clinical, laboratory and epidemiological data of the 251 confirmed cases of HPS in Santa Catarina in 1999-2011. The number of cases ranged from 10 to 47 per year, with the highest incidences in 2004-2006. Gastrointestinal tract manifestations were found in >60% of the cases, potentially confounding diagnosis and leading to inappropriate therapy. Dyspnoea, acute respiratory failure, renal failure, increased serum creatinine and urea levels, increased haematocrits and the presence of pulmonary interstitial infiltrate were significantly more common in HPS patients who died. In addition, we demonstrated that the six cases from the midwest region of the state were associated with Juquitiba virus genotype. The case-fatality rate in this region, 19·2%, was lower than that recorded for other mesoregions. In the multivariate analysis increase of serum creatinine and urea was associated with death by HPS. Our findings help elucidate the epidemiology of HPS in Brazil, where mast seeding of bamboo can trigger rodent population eruptions and subsequent human HPS outbreaks. We also emphasize the need for molecular confirmation of the hantavirus genotype of human cases for a better understanding of the mortality-related factors associated with HPS cases in Brazil.

  6. Solitary median maxillary central incisor (SMMCI syndrome

    Directory of Open Access Journals (Sweden)

    Hall Roger K

    2006-04-01

    Full Text Available Abstract Solitary median maxillary central incisor syndrome (SMMCI is a complex disorder consisting of multiple, mainly midline defects of development resulting from unknown factor(s operating in utero about the 35th–38th day(s from conception. It is estimated to occur in 1:50,000 live births. Aetiology is uncertain. Missense mutation in the SHH gene (I111F at 7q36 may be associated with SMMCI. The SMMCI tooth differs from the normal central incisor, in that the crown form is symmetric; it develops and erupts precisely in the midline of the maxillary dental arch in both primary and permanent dentitions. Congenital nasal malformation (choanal atresia, midnasal stenosis or congenital pyriform aperture stenosis is positively associated with SMMCI. The presence of an SMMCI tooth can predict associated anomalies and in particular the serious anomaly holoprosencephaly. Common congenital anomalies associated with SMMCI are: severe to mild intellectual disability, congenital heart disease, cleft lip and/or palate and less frequently, microcephaly, hypopituitarism, hypotelorism, convergent strabismus, oesophageal and duodenal atresia, cervical hemivertebrae, cervical dermoid, hypothyroidism, scoliosis, absent kidney, micropenis and ambiguous genitalia. Short stature is present in half the children. Diagnosis should be made by eight months of age, but can be made at birth and even prenatally at 18–22 weeks from the routine mid-trimester ultrasound scan. Management depends upon the individual anomalies present. Choanal stenosis requires emergency surgical treatment. Short stature may require growth hormone therapy. SMMCI tooth itself is mainly an aesthetic problem, which is ideally managed by combined orthodontic, prosthodontic and oral surgical treatment; alternatively, it can be left untreated.

  7. CT features of pulmonary mycobacterial disease in patients with acquired immunodeficiency syndrome

    Institute of Scientific and Technical Information of China (English)

    朱莹

    2013-01-01

    Objective To study the CT features of pulmonary non-tuberculous mycobacteria (NTM) disease in patients with acquired immunodeficiency syndrome (AIDS) and explore the different CT appearances between AIDS-NTM and AIDS-TB. Methods CT findings of pulmonary NTM

  8. Pulmonary epithelial apoptosis in fetal down syndrome: Not higher than normal

    NARCIS (Netherlands)

    M. Bruijn (Martijn); J. von der Thusen (Jan); C.M. van der Loos (Chris); R.R. de Krijger (Ronald); R.B. van Loenhout (Rhiannon); A.P. Bos (Albert); J.B. van Woensel (Job)

    2012-01-01

    textabstractChildren with Down syndrome (DS) are at high risk for acute lung injury (ALI). Pulmonary epithelial apoptosis is an important factor in the pathophysiology of ALI. Whether the risk of ALI in DS is associated with a high level of pulmonary epithelial apoptosis is not known. We

  9. Pulmonary artery dissection in a patient with Eisenmenger syndrome treated with heart and lung transplantation

    DEFF Research Database (Denmark)

    Tønder, Niels; Køber, Lars; Hassager, Christian

    2004-01-01

    We report the case of a patient with known Eisenmenger syndrome due to congenital ventricular septal defect, who developed pulmonary artery dissection. The patient was successfully treated with heart and lung transplantation.......We report the case of a patient with known Eisenmenger syndrome due to congenital ventricular septal defect, who developed pulmonary artery dissection. The patient was successfully treated with heart and lung transplantation....

  10. PHOX2B mutations in three Chinese patients with congenital central hypoventilation syndrome

    Institute of Scientific and Technical Information of China (English)

    Siu-Fong June Or; Ming-for Tony Tong; Fai-Man Ivan Lo; Chi-Wai Law; Ting-Yat Miu; Delphine Trochet; Tak-Sum Stephen Lam

    2006-01-01

    @@ Congenital central hypoventilation syndrome(CCHS, OMIM #209880) is a rare autosomal dominant disorder of the autonomic nervous system(ANS) characterized by an abnormal autonomic ventilatory response to progressive hypercarbia and sustained hypoxemia. Patients typically present in the newborn period with hypoventilation or apnea asleep, awake, or both, without any associated cardiac, pulmonary, neuromuscular or brainstem lesions. Rarely, some patients may present at a later age and are diagnosed to have late onset central hypoventilation syndrome (LOCHS).1 Other features of ANS dysfunction such as feeding difficulty due to oesophageal dysmotility, severe constipation in the absence of Hirschsprung disease, poor regulation of basal body temperature, episodes of profuse sweating, pupillary and ocular abnormalities,decreased beat-to-beat variability of heart rate,attenuated response of heart rate to exercise,abnormal fluctuation of blood pressure, decreased perception to pain, and decreased perception to anxiety may be variably present but not essential for diagnosis.

  11. Vasculitis Syndromes of the Central and Peripheral Nervous Systems

    Science.gov (United States)

    ... Sheets Vasculitis Syndromes of the Central and Peripheral Nervous Systems Fact Sheet Table of Contents (click to jump ... flow of blood. How does vasculitis affect the nervous system? Vasculitis can cause problems in any organ system, ...

  12. Aortic and Pulmonary Root Aneurysms in a Child With Loeys-Dietz Syndrome.

    Science.gov (United States)

    Rizzo, Stefania; Stellin, Giovanni; Milanesi, Ornella; Padalino, Massimo; Vricella, Luca A; Thiene, Gaetano; Cameron, Duke E; Basso, Cristina; Vida, Vladimiro L

    2016-03-01

    We report the case of an 11-year-old boy with Loeys-Dietz syndrome, with both aortic and pulmonary aneurysms requiring cardiac operation because of progressive valve incompetence resulting from loss of coaptation of the cusps. Arterial medial changes, consisting of disarray of elastic fibers and increased collagen deposition, were observed in surgical specimens from both the aorta and the pulmonary artery of our patient, and the strong pSmad2 nuclear staining of smooth muscle cells of both aortic and pulmonary tunica media are the best evidence of transforming growth factor-β pathway activation in Loeys-Dietz syndrome.

  13. Cushing Syndrome Secondary to Thymic Carcinoid Synchronous With Tuberculous Lymphadenitis and Pulmonary Tuberculosis: A Case

    Directory of Open Access Journals (Sweden)

    F Esfahanian

    2007-05-01

    Full Text Available Thymic carcinoid is an uncommon neoplasm that can present with Cushing syndrome.We report a 39-year old woman with symptoms of Cushing syndrome secondary to thymic carcinoid and synchronous with tuberculous lymphadenitis and pulmonary tuberculosis. 

  14. Viral load of patients with hantavirus pulmonary syndrome in Argentina.

    Science.gov (United States)

    Bellomo, Carla María; Pires-Marczeski, Fanny Clara; Padula, Paula Julieta

    2015-11-01

    Hantavirus causes severe illness including pneumonia, which leads to hospitalization and often death. At present, there is no specific treatment available. The hantavirus pathogenesis is not well understood, but most likely both virus-mediated and host-mediated mechanisms, are involved. The aim of this study was to correlate viral load in samples of hantavirus pulmonary syndrome cases and hantavirus infected individuals, with clinical epidemiological parameters and disease outcome. The variables that could potentially be related with viral load were analyzed. The retrospective study included 73 cases or household contacts, with different clinical evolution. Viral load was measured by reverse-transcription and real time polymerase chain reaction. There was no statistically significant association between blood viral RNA levels and severity of disease. However, viral load was inversely correlated with IgG response in a statistically significant manner. The level of viral RNA was significantly higher in patients infected with Andes virus South lineage, and was markedly low in persons infected with Laguna Negra virus. These results suggest that the infecting viral genotype is associated with disease severity, and that high viral load is associated with a low specific IgG response. Sex, age and disease severity were not related with viral load. Further investigations increasing strikingly the number of cases and also limiting the variables to be studied are necessary.

  15. Pulmonary involvement of hypereosinophilic syndrome : high-resolution CT finding in three patients

    Energy Technology Data Exchange (ETDEWEB)

    Kang, Eun Young; Lee, Mee Ran; Shim, Jae Jeong [Korea Univ. College of Medicine, Seoul (Korea, Republic of)

    1996-09-01

    Hypereosinophilic syndrome is a rare entity of eosinophilic lung disease characterized by idiopathic prolonged eosinophilia of marked degree and variable organ involvement. Pulmonary involvement of hypereosinophilic syndrome occurs in up to 40% of patients. We report HRCT findings of three patients with pulmonary involvement of hypereosinophilic syndrome diagnosed by clinical manifestation, bronchoalveolar lavage and transbronchial lung biopsy. On HRCT, several small nodules were seen in both lungs, especially in peripheral lung areas of the three patients. One had nodules with ground-glass attenuation halo and also focal areas of ground-glass attenuation in this area.

  16. Chronic obstructive pulmonary disease and the metabolic syndrome: Consequences of a dual threat

    OpenAIRE

    Dukhabandhu Naik; Anjali Joshi; Thomas Vizhalil Paul; Nihal Thomas

    2014-01-01

    The metabolic syndrome is found to be more frequent in chronic obstructive pulmonary disease (COPD). The presence of inflammatory markers in circulation, sputum, and broncho-alveolar fluid suggest systemic inflammation is one of the potential mechanisms responsible for both COPD and metabolic syndrome. Physical inactivity, skeletal muscle dysfunction, hypogonadism, and steroid use are also important causes of the metabolic syndrome in COPD. Obesity and insulin resistance is found to be more c...

  17. A Case of Severe Pulmonary Thromboembolism in a Young Male With Klinefelter Syndrome

    OpenAIRE

    Kang, Byung-Soo; Cho, Deok-Kyu; Koh, Won-Jun; Yoo, Seung-Hoon; Won, Ki-Bum; Cho, Yun-Hyeong; Hwang, Eui-Seock; Koh, Jong-Hoon

    2012-01-01

    A young male patient diagnosed with Klinefelter syndrome was admitted to our hospital via the emergency room with chief complaints of acute chest pain and dyspnea. Pulmonary thromboembolism was diagnosed from his chest CT images. His symptoms improved after he underwent thrombolysis and anticoagulation treatment. Klinefelter syndrome has a tendency towards hypercoagulability due to hormonal imbalance and one or more inherited thromophilic factors. Thus, Klinefelter syndrome patients with a pa...

  18. Metabolic syndrome in hospitalized patients with chronic obstructive pulmonary disease

    Directory of Open Access Journals (Sweden)

    Evgeni Mekov

    2015-07-01

    Full Text Available Introduction. The metabolic syndrome (MS affects 21–53% of patients with chronic obstructive pulmonary disease (COPD with a higher prevalence in the early stages of COPD, with results being highly variable between studies. MS may also affect natural course of COPD—number of exacerbations, quality of life and lung function.Aim. To examine the prevalence of MS and its correlation with comorbidities and COPD characteristics in patients with COPD admitted for exacerbation.Material and methods. 152 patients with COPD admitted for exacerbation were studied for presence of MS. All of them were also assessed for vitamin D status and diabetes mellitus type 2 (DM. Data were gathered for smoking status and exacerbations during the last year. All patients completed CAT (COPD assessment test and mMRC (Modified Medical Research Council Dyspnea scale questionnaires and underwent spirometry. Duration of current hospital stay was recorded.Results. 25% of patients have MS. 23.1% of the male and 29.5% of the female patients have MS (p > 0.05. The prevalence of MS in this study is significantly lower when compared to a national representative study (44.6% in subjects over 45 years. 69.1% of all patients and 97.4% from MS patients have arterial hypertension. The presence of MS is associated with significantly worse cough and sleep (1st and 7th CAT questions; p = 0.002 and p = 0.001 respectively and higher total CAT score (p = 0.017. Average BMI is 27.31. None of the patients have MS and BMI <25. There is a correlation between the presence of MS and DM (p = 0.008 and with the number of exacerbations in the last year (p = 0.015. There is no correlation between the presence of MS and the pulmonary function.Conclusion. This study among hospitalized COPD patients finds comparable but relatively low prevalence of MS (25% compared to previously published data (21–53% and lower prevalence compared to general population (44.6%. MS may impact quality of life and the

  19. Fungal infections of the central nervous system: The clinical syndromes

    Directory of Open Access Journals (Sweden)

    Murthy J.M.K

    2007-01-01

    Full Text Available Fungal infections of the central nervous system (CNS are being increasingly diagnosed both in immunocompromised and immunocompetent individuals. Sinocranial aspergillosis is more frequently described from countries with temperate climates, more often in otherwise immunocompetent individuals. The clinical syndromes with which fungal infections of the CNS can present are protean and can involve most part of the neuroaxis. Certain clinical syndromes are specific for certain fungal infections. The rhinocerebral form is the most common presenting syndrome with zygomycosis and skull-base syndromes are often the presenting clinical syndromes in patients with sinocranial aspergillosis. Subacute and chronic meningitis in patients with HIV infection is more likely to be due to cryptococcal infection. Early recognition of the clinical syndromes in an appropriate clinical setting is the first step towards achieving total cure in some of these infections.

  20. [The pulmonary-renal syndrome: a diagnostic and therapeutic emergency for the internist and the intensivist].

    Science.gov (United States)

    Hié, M; Costedoat-Chalumeau, N; Saadoun, D; Azoulay, E

    2013-11-01

    The pulmonary-renal syndrome is a rare and life-threatening condition. It is defined as the association of a diffuse alveolar hemorrhage and a rapidly progressive glomerulonephritis. The characteristic histological lesion common to all underlying diseases is a necrotizing and crescentic glomerulonephritis. The pulmonary-renal syndrome is a diagnostic and therapeutic emergency: any delay in its management will lead to death or serious functional damage as pulmonary and renal impairment. ANCA-associated vasculitis and Goodpasture's disease are the main disorders associated to pulmonary-renal syndrome. More rarely systemic lupus, cryoglobulinaemia, Henoch-Schonlein purpura or subacute endocarditis may induce a pulmonary-renal syndrome. Differential diagnosis can sometimes be difficult, highlighting some ambiguity in the definition of the syndrome. Initial treatment usually associates systemic corticosteroid, cyclophosphamide and plasma exchange. The role of biotherapy as first line therapy remains to be determined. Copyright © 2013 Société nationale française de médecine interne (SNFMI). Published by Elsevier SAS. All rights reserved.

  1. Fused pulmonary lobes is a rat model of human Fraser syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Kiyozumi, Daiji; Nakano, Itsuko [Laboratory of Extracellular Matrix Biochemistry, Institute for Protein Research, Osaka University, 3-2 Yamadaoka, Suita, Osaka 565-0871 (Japan); Takahashi, Ken L.; Hojo, Hitoshi; Aoyama, Hiroaki [Toxicology Division, Institute of Environmental Toxicology, 4321 Uchimoriya, Joso, Ibaraki 303-0043 (Japan); Sekiguchi, Kiyotoshi, E-mail: sekiguch@protein.osaka-u.ac.jp [Laboratory of Extracellular Matrix Biochemistry, Institute for Protein Research, Osaka University, 3-2 Yamadaoka, Suita, Osaka 565-0871 (Japan)

    2011-07-29

    Highlights: {yields} Fused pulmonary lobes (fpl) mutant rats exhibit similar phenotypes to Fraser syndrome. {yields} The fpl gene harbors a nonsense mutation in Fraser syndrome-associated gene Frem2. {yields} Fpl mutant is defined as a first model of human Fraser syndrome in rats. -- Abstract: Fused pulmonary lobes (fpl) is a mutant gene that is inherited in an autosomal recessive manner and causes various developmental defects, including fusion of pulmonary lobes, and eyelid and digit anomalies in rats. Since these developmental defects closely resemble those observed in patients with Fraser syndrome, a recessive multiorgan disorder, and its model animals, we investigated whether the abnormal phenotypes observed in fpl/fpl mutant rats are attributable to a genetic disorder similar to Fraser syndrome. At the epidermal basement membrane in fpl/fpl mutant neonates, the expression of QBRICK, a basement membrane protein whose expression is attenuated in Fraser syndrome model mice, was greatly diminished compared with control littermates. Quantitative RT-PCR analyses of Fraser syndrome-related genes revealed that Frem2 transcripts were markedly diminished in QBRICK-negative embryos. Genomic DNA sequencing of the fpl/fpl mutant identified a nonsense mutation that introduced a stop codon at serine 2005 in Frem2. These findings indicate that the fpl mutant is a rat model of human Fraser syndrome.

  2. Sjogrens Syndrome Presenting with Central Nervous System Involvement

    Directory of Open Access Journals (Sweden)

    Tülay Terzi

    2012-01-01

    Full Text Available Sjogren’s syndrome is a slowly progressive autoimmune disease. Neurological involvement occurs in approximately 20-25% cases in Sjogren’s syndrome. 87% of the neurological involvement is peripheral nervous system, almost 13% in the form of central nervous system involvement. Affected central nervous system may show similar clinical and radiological findings as in multiple sclerosis (MS. In this paper, a 43-year-old patient is discussed who was referred with the complaint of dizziness, there was MS- like lesions in brain imaging studies and was diagnosed with Sjogren’s syndrome. MS- like clinical and radiologic tables can be seen, albeit rarely in Sjogren’s syndrome. In these cases, early diagnosis and early treatment for the sjögren has a great importance for the prognosis of the disease.

  3. Paraneoplastic Syndromes of the Central Nervous System

    NARCIS (Netherlands)

    J.W.B. Moll (Wibe)

    1996-01-01

    textabstractIn recent years a continuous stream of new information on clinical, pathological and immunological aspects of paraneoplastic neurological syndromes has been published. In this survey, we will discuss current opinions on the value of anti-neuronal antibody detection for establishing a dia

  4. Percutaneous transhepatic venous embolization of pulmonary artery aneurysm in Hughes-Stovin syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Kyung Ah; Kim, Man Deuk; Oh, Do Yun; Park, Pil Won [Bundang CHA General Hospital, Pochon CHA University, Seongnam (Korea, Republic of)

    2007-08-15

    Hughes-Stovin syndrome is an extremely rare entity. We present a case of a 42-year-old man, who developed deep vein and inferior vena cava (IVC) thrombosis, repeated internal bleeding and pulmonary artery aneurysms (PAAs). The patient presented with massive hemoptysis and with PAAs of a 2.5 cm maximum diameter. We describe the successful percutaneous transhepatic venous embolization of the PAAs due to occluded common vascular pathways to the pulmonary artery.

  5. Genetic mapping of quantitative trait loci affecting susceptibility in chicken to develop the Pulmonary Hypertension Syndrome (PHS)

    NARCIS (Netherlands)

    Rabie, T.S.K.M.; Crooijmans, R.P.M.A.; Bovenhuis, H.; Vereijken, A.L.J.; Veenendaal, A.; Poel, van der J.J.; Arendonk, van J.A.M.; Pakdel, A.; Groenen, M.A.M.

    2005-01-01

    Pulmonary hypertension syndrome (PHS), also referred to as ascites syndrome, is a growth-related disorder of chickens frequently observed in fast-growing broilers with insufficient pulmonary vascular capacity at low temperature and/or at high altitude. A cross between two genetically different

  6. Subclavian steal syndrome presenting as recurrent pulmonary oedema associated with acute left ventricular diastolic dysfunction.

    Science.gov (United States)

    Mangialavori, Giuseppe; Ballo, Piercarlo; Michelagnoli, Stefano; Ercolini, Leonardo; Barbanti, Enrico; Passuello, Franco; Abbondanti, Alessandro; Consoli, Lorenzo; Chechi, Tania; Fibbi, Veronica; Nannini, Marco; Chiodi, Leandro; Zuppiroli, Alfredo

    2013-01-01

    Subclavian steal syndrome typically presents as angina in patients with internal mammary artery grafts. Atypical clinical presentations have been rarely described. We report an unusual case of subclavian steal syndrome presenting as pulmonary oedema with acute left ventricular diastolic dysfunction and preserved ejection fraction in a patient with internal mammary artery graft and severe stenosis of the proximal left subclavian artery. After successful angioplasty and stenting of subclavian artery, the patient remained asymptomatic for six months, but then experienced acute diastolic dysfunction and recurrent pulmonary oedema associated with critical subclavian in-stent restenosis with stent deformation. This report points out that, in patients with internal mammary-to-LAD grafts, subclavian steal syndrome may present as acute left ventricular diastolic dysfunction and pulmonary oedema even in the presence of normal ejection fraction.

  7. A case of William's syndrome associated peripheral pulmonary arterial stenosis

    Energy Technology Data Exchange (ETDEWEB)

    Jung, Kyung Hwa; Hwang, Mi Soo; Kim, Sun Yong; Chang, Jae Chun; Park, Bok Hwan [College of Medicine, Yeungam University, Daegu (Korea, Republic of)

    1988-06-15

    William's syndrome, in order to more completely delineate the total spectrum of the disorder, indicates that 'infantile hypercalcemia', 'peculiar facies' and 'supravalvular aortic stenosis.' In has other many vascular anomalies, such as peripheral pulmonary arterial stenosis, coronary arterial stenosis, celiac arterial stenosis, and renal aterial stenosis. Only 32% of the patients have evidence of supravalvular aortic stenosis. And it is very rare disease entity that has been reported rarely in Korea. Recently authors experienced a case that was questioned William's syndrome with peripheral pulmonary arterial stenosis, clinically and preliminary radiologically and this case was confirmed by operation. Here we report a case of William's syndrome with peripheral pulmonary arterial stenosis and reviewed literatures.

  8. Acute Pulmonary Edema in an Eclamptic Pregnant Patient: A Rare Case of Takotsubo Syndrome

    Science.gov (United States)

    Karamchandani, Kunal; Bortz, Brandon; Vaida, Sonia

    2016-01-01

    Patient: Female, 35 Final Diagnosis: Takotsubo cardiomyopathy Symptoms: Seizures Medication: — Clinical Procedure: Cesarean section Specialty: Critical Care Medicine Objective: Rare co-existance of disease or pathology Background: Acute pulmonary edema in a pregnant patient is associated with significant morbidity and mortality. Takotsubo syndrome, or stress-induced cardiomyopathy, is a rare cause of acute pulmonary edema in a pregnant patient, especially prior to delivery of the fetus. Case Report: We describe a case of a pregnant patient who presented with acute pulmonary edema and eclampsia and was found to have Takotsubo syndrome. To the best of our knowledge, eclampsia as a precipitating factor for Takotsubo syndrome has not been described in literature. Conclusions: Clinicians taking care of pregnant patients should be aware of the potential link between eclampsia and Takotsubo cardiomyopathy. Prompt correction of the precipitating cause along with supportive management as described is the key to a successful outcome. PMID:27658947

  9. Acute Pulmonary Edema in an Eclamptic Pregnant Patient: A Rare Case of Takotsubo Syndrome.

    Science.gov (United States)

    Karamchandani, Kunal; Bortz, Brandon; Vaida, Sonia

    2016-09-23

    BACKGROUND Acute pulmonary edema in a pregnant patient is associated with significant morbidity and mortality. Takotsubo syndrome, or stress-induced cardiomyopathy, is a rare cause of acute pulmonary edema in a pregnant patient, especially prior to delivery of the fetus. CASE REPORT We describe a case of a pregnant patient who presented with acute pulmonary edema and eclampsia and was found to have Takotsubo syndrome. To the best of our knowledge, eclampsia as a precipitating factor for Takotsubo syndrome has not been described in literature. CONCLUSIONS Clinicians taking care of pregnant patients should be aware of the potential link between eclampsia and Takotsubo cardiomyopathy. Prompt correction of the precipitating cause along with supportive management as described is the key to a successful outcome.

  10. Mycological and serological study of pulmonary aspergillosis in central India

    Directory of Open Access Journals (Sweden)

    Kurhade A

    2002-01-01

    Full Text Available PURPOSE: To study the prevalence and predisposing factors of Aspergillus infection and correlate microscopic, culture and serological findings along with drug sensitivity. METHODS: Sputum samples from 123 patients of pulmonary disease with clinical suspicion of having fungal, especially Aspergillus infections, were examined microscopically and for culture. Minimum inhibitory concentration (MIC of itraconazole was tested against the isolates. Serum samples from these patients were tested for precipitin against Aspergillus antigen using immunodiffusion (ID technique. RESULTS: Aspergillus species were isolated in 20 (16.26% cases and Aspergillus fumigatus was the predominant species isolated in 16 (80% cases. Precipitins were detected in 29 (23.58% cases. Serum samples collected from 50 healthy individuals to serve as controls showed no precipitin against Aspergillus antigen galactomannan. This fungus was found to be sensitive to itraconazole with MIC range 0.125-1µg/mL. CONCLUSIONS: Serological tests have an edge over routine smear and culture methods for the diagnosis of pulmonary aspergillosis. Itraconazole is more effective than amphotericin B and fluconazole in the treatment of aspergillosis.

  11. Pulmonary Complications of Azanucleoside Therapy in Patients with Myelodysplastic Syndrome and Acute Myelogenous Leukemia

    Directory of Open Access Journals (Sweden)

    Manuel Molina

    2015-01-01

    Full Text Available Our primary aim was to identify potential risk factors and clinical outcome of azanucleoside induced pulmonary complications in patients with myelodysplastic syndrome (MDS and Acute Myelogenous Leukemia (AML. We present an 89-year-old female with MDS derived AML who developed fatigability, hypoxemia, and bilateral lung infiltrates indicating interstitial lung disease after 11 cycles of azanucleoside. In addition, we describe a cohort of six MDS patients with fever, cough, dyspnea, and pulmonary infiltrates at early time point during azanucleoside treatment. Early and late onset of pulmonary manifestations suggest different pathogenic mechanisms. Brief azanucleoside discontinuation and steroids led to rapid improvement in symptoms.

  12. Coronary Artery Calcification Is Often Unreported in CT Pulmonary Angiograms in Patients With Suspected Pulmonary Embolism: An Opportunity to Improve Diagnosis of Acute Coronary Syndrome

    OpenAIRE

    Johnson, Patrick Connor

    2015-01-01

    Objective: In patients with suspected pulmonary thromboembolism (PTE), coronary artery calcification (CAC) can be an incidental finding in CT pulmonary angiograms. We evaluated the frequency of unreported CAC and its association with diagnosis of acute coronary syndrome (ACS). Methods: The data of 469 consecutive patients who were referred to the emergency radiology department for CT pulmonary angiography because of suspicion for PTE were reviewed. Radiology reports were rechecked, and pos...

  13. Diaphragm pacers as a treatment for congenital central hypoventilation syndrome.

    Science.gov (United States)

    Chen, Maida Lynn; Tablizo, Mary Anne; Kun, Sheila; Keens, Thomas G

    2005-09-01

    Congenital central hypoventilation syndrome is a rare syndrome present from birth, and is defined as the failure of automatic control of breathing. All patients with congenital central hypoventilation syndrome require life-long ventilatory support during sleep, although approximately a third of patients require ventilatory support 24 h per day. Diaphragm pacers offer a modality of ventilatory support that affords congenital central hypoventilation syndrome patients with maximal mobility for full-time ventilatory patients, and they may allow for a more normal lifestyle in the appropriate patient. They may permit tracheostomy decannulation in those requiring only support during sleep. Diaphragm pacing entails surgical placement of an electrode onto the phrenic nerve, connected to a subcutaneous receiver. There is an external battery-operated transmitter and antenna placed on the skin over the receiver. The transmitter emits energy, similar to radio transmission, which is converted into an electrical current by the receiver. This stimulates the phrenic nerve resulting in a diaphragmatic contraction. Settings on the transmitter include respiratory rate and electrical voltage, and are adjusted to give enough tidal volume to allow for adequate oxygenation and ventilation. Therefore, diaphragm pacing is an attractive alternative mode of mechanically assisted ventilation for many patients with congenital central hypoventilation syndrome.

  14. Pulmonary Arteriovenous Malformation in Cryptogenic Liver Cirrhosis Associated with Turner's Syndrome.

    Science.gov (United States)

    Kim, Ji Hoon; Jung, Young Kul; Jeong, Eun Seok; Seo, Yeon Seok; Yim, Hyung Joon; Yeon, Jong Eun; Shim, Jae Jeong; Byun, Kwan Soo; Lee, Chang Hong

    2010-06-01

    Turner's syndrome is a genetic disorder of the sex chromosomes (e.g., 45,X or 45,X/46,XX) that manifests as various congenital anomalies. Despite its numerous extragonadal manifestations and frequent accompanying abnormalities in liver function tests, liver cirrhosis associated with Turner's syndrome has not been reported in Korea. Moreover, pulmonary arteriovenous malformations (PAVMs) have rarely been reported in association with liver cirrhosis, but there have been no reports of PAVMs occurring in cryptogenic liver cirrhosis associated with Turner's syndrome. We report a case of PAVM that occurred in cryptogenic liver cirrhosis associated with Turner's syndrome.

  15. [Peripheral, central and functional vertigo syndromes].

    Science.gov (United States)

    Strupp, M; Dieterich, M; Zwergal, A; Brandt, T

    2015-12-01

    Depending on the temporal course, three forms of vertigo syndrome can be differentiated: 1) vertigo attacks, e.g. benign paroxysmal positional vertigo (BPPV), Menière's disease and vestibular migraine, 2) acute spontaneous vertigo lasting for days, e.g. acute unilateral vestibulopathy, brainstem or cerebellar infarction and 3) symptoms lasting for months or years, e.g. bilateral vestibulopathy and functional vertigo. The specific therapy of the various syndromes is based on three principles: 1) physical treatment with liberatory maneuvers for BPPV and balance training for vestibular deficits, 2) pharmacotherapy, e.g. for acute unilateral vestibulopathy (corticosteroids) and Menière's disease (transtympanic administration of gentamicin or steroids and high-dose betahistine therapy); placebo-controlled pharmacotherapy studies are currently being carried out for acute unilateral vestibulopathy, vestibular paroxysmia, prophylaxis of BPPV, vestibular migraine, episodic ataxia type 2 and cerebellar ataxia; 3) psychotherapy for functional dizziness.

  16. Leptospirosis-Associated Severe Pulmonary Hemorrhagic Syndrome with Lower Back Pain as an Initial Symptom

    DEFF Research Database (Denmark)

    Søndergaard, Mads Madsen; tursunovic, Amela; Thye-Roenn, Peter

    2016-01-01

    BACKGROUND Leptospirosis is a zoonosis transmitted through urine of infected animals. Symptoms range from mild influenza-like symptoms to severe pulmonary hemorrhagic syndrome (SPHS); the latter are often fatal. The serogroup distribution in Denmark has changed from 1988 to 2012, with Icterohaemo......BACKGROUND Leptospirosis is a zoonosis transmitted through urine of infected animals. Symptoms range from mild influenza-like symptoms to severe pulmonary hemorrhagic syndrome (SPHS); the latter are often fatal. The serogroup distribution in Denmark has changed from 1988 to 2012...

  17. CASE REPORT Acute Compartment Syndrome of the Forearm Following Blood Gas Analysis Postthrombolysis for Pulmonary Embolism.

    Science.gov (United States)

    Bisarya, Kamal; George, Samuel; El Sallakh, Sam

    2013-01-01

    Acute compartment syndrome is an important condition with potentially serious consequences if not diagnosed and treated promptly. This report highlights a case of acute compartment syndrome of the forearm after radial artery blood gas analysis in a patient who had been thrombolyzed for a pulmonary embolus. Methods/Case Report: We present a case of a 54-year-old lady, admitted and treated for a pulmonary embolism with tenecteplase for thrombolysis. As per routine management, she had taken an arterial blood gas sample, which caused hematoma in the wrist and a few hours later developed pain and a tense right forearm being diagnosed with compartment syndrome. She underwent fasciotomies and subsequent split skin grafting. We discuss the different etiologies of compartment syndrome, clinical signs, and available investigations as well as immediate and definitive management options including fasciotomy techniques. We present the latest literature on the subject and extract valuable learning points from this case. With the common use of thrombolysis for the management of a myocardial infarction or pulmonary embolus, compartment syndrome is an uncommon but potentially associated problem. Furthermore, with blood gas sampling being part of daily clinical practice and a potential cause of this condition, the compartment syndrome becomes iatrogenic and potentiates serious litigation. As many junior doctors are performing blood gas analysis postthrombolysis, they need to assess patients adequately and realize the risk of possible sequelae such as compartment syndrome in this group and inform patients of such complications.

  18. A rare association of pulmonary hypertension and dextrocardia with Poland syndrome.

    Science.gov (United States)

    Raval, Jwalant; Nagaraja, Vinayak; Burgess, David; Eshoo, Suzanne; Sadick, Norman; Denniss, A Robert

    2013-09-01

    Poland syndrome is a rare congenital abnormality with an estimated incidence of 1 in 20,000 to 1 in 30,000 live births. We report and discuss this rare combination of pulmonary hypertension and dextrocardia with Poland syndrome. This case report also highlights the underlying pathogenetic mechanisms during foetal development and the potential to misdiagnose cardiac ischaemia in a patient with an anatomic anomaly.

  19. Tricuspid atresia and pulmonary atresia in a child with Rubinstein-Taybi syndrome

    Directory of Open Access Journals (Sweden)

    Rohit S Loomba

    2015-01-01

    Full Text Available Rubinstein-Taybi syndrome is a well-characterized condition causing distinctive physical characteristics, intellectual disability, and multiple congenital malformations. Cardiac abnormalities are found in a third of individuals with this condition and usually consist of isolated septal defects or patent ductus arteriosus, although more complex congenital lesions have been described. We present the first reported case of tricuspid atresia and pulmonary atresia with hypoplasia of the right ventricle in the setting of Rubenstein-Taybi syndrome.

  20. Pulmonary Thromboembolism in Klinefelter%u2019s Syndrome Patient with Deficient of Protein C

    OpenAIRE

    Mehmet Yigit

    2013-01-01

    Klinefelter syndrome (KS) is a common genetic disorder caused by one or more supernumerary X chromosomes. KS poses an increased risk for venous thromboembolic events such as deep venous thrombosis and pulmonary embolism. Klinefelter syndrome is prone to hypercoagulability due to hormonal imbalance and one or more inherited thrombophilic factors. Therefore, patients with KS having a medical history of venous thromboembolism require chest computed tomographic (CT) images and oral anticoagulatio...

  1. Synovitis-acne-pustulosis-hyperostosis-osteitis (SAPHO) syndrome complicated by seven pulmonary emboli in a 15-year old patient.

    Science.gov (United States)

    Coloe, Jacquelyn; Diamantis, Stephanie; Henderson, Frederick; Morrell, Dean S

    2010-02-01

    SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) syndrome represents a spectrum of various dermatologic and musculoskeletal conditions. Thromboses have infrequently been reported in SAPHO syndrome, most often in the subclavian vein. There have been no reported cases of pulmonary emboli associated with SAPHO. We report a case of a young patient with SAPHO syndrome who later presented with extensive iliofemoral deep vein thromboses and seven pulmonary emboli.

  2. Rate and Outcomes of Pulmonary Stenosis and Functional Pulmonary Atresia in Recipient Twins with Twin-Twin Transfusion Syndrome.

    Science.gov (United States)

    Ortiz, Javier U; Masoller, Narcís; Gómez, Olga; Bennasar, Mar; Eixarch, Elisenda; Lobmaier, Silvia M; Crispi, Fàtima; Gratacos, Eduard; Martinez, Josep María

    2017-01-01

    To evaluate the rate of pulmonary stenosis and functional pulmonary atresia (PS/PA) in recipient twins prior to fetal surgery for twin-twin transfusion syndrome (TTTS) and their pre- and postnatal outcomes. We carried out a prospective study including 260 cases of TTTS. Echocardiography was performed before laser surgery to detect the presence of PS/PA. The outcomes of recipients with and without PS/PA were compared. The need of postnatal cardiac interventions and the survival rate at 6 months of age were also evaluated. PS was observed in 16/260 (6.2%) of recipient twins and PA in 12/260 (4.6%). After fetal surgery, 10/28 (35.7%) recipients died, 9/28 (32.1%) showed in utero regression, and 9/28 (32.1%) had persistence of PS/PA. Postnatally, seven recipients underwent percutaneous balloon pulmonary valvuloplasty, one required surgical valvotomy and one palliative surgery. Pregnancies with recipient twins with PS/PA had lower survival of at least one twin (67.9 vs. 83.6%, p = 0.045) and lower overall survival (57.1 vs. 72.8%, p = 0.015) at 6 months of age. PS and PA were observed in 10.8% of recipients. Among these, about one third showed persistence of pulmonary valve pathology after delivery, which stresses the need for strict follow-up. © 2016 S. Karger AG, Basel.

  3. Dental treatment of a child with congenital central hypoventilation syndrome

    NARCIS (Netherlands)

    Boka, V.; Lefkelidou, A.; Athanasiadou, E.

    2016-01-01

    Background: Congenital Central Hypoventilation Syndrome (CCHS) is a rare condition combining respiratory hypoventilation with symptoms of autonomic dysregulation. Management of patients requires both medical and dental expertise to achieve a successful outcome. The aim of this paper is to present th

  4. Chronic eosinophilic pneumonia within the syndrom of 'pulmonary infiltrates with eosinophilia'

    Energy Technology Data Exchange (ETDEWEB)

    Heinrich, J.; Schmidt, M.; Kulke, H.; Daemmrich, J.

    1988-06-01

    Typical diagnostic findings of the chronic eosinophilic pneumonia within the syndrom of 'pulmonary infiltrates with eosinophilia' are described. We demonstrate one case of chronic eosinophilia pneumonia of unknown aetiology investigated clinically, by chest radiography, lung biopsy, bronchial lavage and lung function. The importance of chest radiography in the diagnosis of chronic eosinophilic pneumonia is presented.

  5. A Cluster of Three Cases of Hantavirus Pulmonary Syndrome among Canadian Military Personnel

    Directory of Open Access Journals (Sweden)

    Leighanne O. Parkes

    2016-01-01

    Full Text Available Hantavirus pulmonary syndrome (HPS is a rare illness in eastern Canada. We present three cases of HPS among military personnel in Quebec. The three cases shared a common exposure to mouse excreta while engaged in military training in Alberta, a western province of Canada.

  6. Idiopathic Pulmonary Hemosiderosis in a Child with Recurrent Macrophage Activation Syndrome Secondary to Systemic Juvenile Idiopathic Arthritis

    Science.gov (United States)

    Barut, Kenan; Sahin, Sezgin; Adrovic, Amra

    2017-01-01

    Macrophage activation syndrome, a severe complication of systemic juvenile idiopathic arthritis and other inflammatory diseases, represents one of the most important rheumatological emergencies. Delayed diagnosis could lead to life-threatening complications. Pulmonary hemosiderosis has been classically characterized by a triad of anemia, hemoptysis, and lung infiltrates on chest radiogram. Although the majority of patients of pulmonary hemosiderosis are considered idiopathic, secondary hemosiderosis associated with known diseases could be seen. In this case report, we aimed to present gradually increased pulmonary manifestations due to pulmonary hemosiderosis with recurrent macrophage activation syndrome attacks in a child with systemic juvenile idiopathic arthritis.

  7. Exacerbation of hypereosinophilic syndrome with pulmonary involvement in two consecutive pregnancies: a case report and review of the literature

    Directory of Open Access Journals (Sweden)

    Itai Ghersin

    2014-08-01

    Full Text Available Hypereosinophilic syndrome represents a heterogeneous group of disorders with the common feature of prolonged eosinophilia of unknown cause and organ system dysfunction including the pulmonary system. Hypereosinophilic syndrome, with and without pulmonary involvement, in association with pregnancy is very rare, and to the best of our knowledge only one case of hypereosinophilic syndrome with pulmonary involvement during pregnancy has been previously reported in the medical literature. We describe a case of a patient with previously symptomatic hypereosinophilic syndrome with pulmonary involvement who experienced exacerbations of her disease during two consecutive pregnancies. To the best of our knowledge this is the first report which demonstrates a worsening effect of pregnancy on both eosinophil count and end organ involvement in a patient with previous diagnosis of hypereosinophilic syndrome. [Int J Res Med Sci 2014; 2(4.000: 1774-1776

  8. Down Syndrome with Complete Atrioventricular Septal Defect, Hypertrophic Cardiomyopathy, and Pulmonary Vein Stenosis.

    Science.gov (United States)

    Mahadevaiah, Guruprasad; Gupta, Manoj; Ashwath, Ravi

    2015-10-01

    The prevalence of congenital heart disease in infants with Down syndrome is 40%, compared with 0.3% in children who have normal chromosomes. Atrioventricular and ventricular septal defects are often associated with chromosomal aberrations, such as in trisomy 21, whereas hypertrophic cardiomyopathy is chiefly thought to be secondary to specific gene mutations. We found only one reported case of congenital hypertrophic cardiomyopathy and atrioventricular septal defect in an infant with Down syndrome. Here, we report atrioventricular septal defect, hypertrophic cardiomyopathy, and pulmonary vein stenosis in a neonate with Down syndrome-an apparently unique combination. In addition, we discuss the relevant medical literature.

  9. A case of severe pulmonary thromboembolism in a young male with klinefelter syndrome.

    Science.gov (United States)

    Kang, Byung-Soo; Cho, Deok-Kyu; Koh, Won-Jun; Yoo, Seung-Hoon; Won, Ki-Bum; Cho, Yun-Hyeong; Hwang, Eui-Seock; Koh, Jong-Hoon

    2012-08-01

    A young male patient diagnosed with Klinefelter syndrome was admitted to our hospital via the emergency room with chief complaints of acute chest pain and dyspnea. Pulmonary thromboembolism was diagnosed from his chest CT images. His symptoms improved after he underwent thrombolysis and anticoagulation treatment. Klinefelter syndrome has a tendency towards hypercoagulability due to hormonal imbalance and one or more inherited thromophilic factors. Thus, Klinefelter syndrome patients with a past medical history of venous thromboembolism require continuous oral anticoagulation therapy for a period of at least six months.

  10. Unsuspected pulmonary alveolar proteinosis in a patient with acquired immunodeficiency syndrome: a case report

    Directory of Open Access Journals (Sweden)

    Niazi Masooma

    2011-02-01

    Full Text Available Abstract Introduction Diffuse lung infiltrates are a common finding in patients with acquired immunodeficiency syndrome and causes range from infectious processes to malignancies or interstitial lung diseases. Pulmonary alveolar proteinosis is a rare pulmonary disorder rarely reported in patients infected with human immunodeficiency virus. Secondary pulmonary alveolar proteinosis is associated with conditions involving functional impairment or reduced numbers of alveolar macrophages. It can be caused by hematologic malignancies, inhalation of toxic dust, fumes or gases, infectious or pharmacologic immunosuppression, or lysinuric protein intolerance. Case presentation A 42-year-old African American man infected with human immunodeficiency virus was admitted with chronic respiratory symptoms and diffuse pulmonary infiltrates. Chest computed tomography revealed bilateral spontaneous pneumothoraces, for which he required bilateral chest tubes. Initial laboratory investigations did not reveal any contributory conditions. Histological examination of a lung biopsy taken during video-assisted thoracoscopy showed pulmonary alveolar proteinosis concurrent with cytomegalovirus pneumonitis. After ganciclovir treatment, our patient showed radiologic and clinical improvement. Conclusion The differential diagnosis for patients with immunosuppression and lung infiltrates requires extensive investigations. As pulmonary alveolar proteinosis is rare, the diagnosis can be easily missed. Our case highlights the importance of invasive investigations and histology in the management of patients infected with human immunodeficiency virus and pulmonary disease who do not respond to empiric therapy.

  11. A Rare Cause of Persistent Pulmonary Hypertension Resistant to Therapy in The Newborn: Short-Rib Polydactyly Syndrome

    Directory of Open Access Journals (Sweden)

    Nihat Demir

    2015-01-01

    Full Text Available Short-rib polydactyly syndrome is an autosomal recessively inherited lethal skeletal dysplasia. The syndrome is characterized by marked narrow fetal thorax, short extremities, micromelia, cleft palate/lip, polydactyly, cardiac and renal abnormalities, and genital malformations. In cases with pulmonary hypoplasia, persistent pulmonary hypertension of the newborn can develop. In this paper, we present a term newborn with persistent pulmonary hypertension of the newborn, which has developed secondary to short-rib polydactyly syndrome and was resistant to therapy with inhaled nitric oxide and oral sildenafil.

  12. Masquerade Syndrome of Multicentre Primary Central Nervous System Lymphoma

    Directory of Open Access Journals (Sweden)

    Silvana Guerriero

    2011-01-01

    Full Text Available Purpose. In Italy we say that the most unlucky things can happen to physicians when they get sick, despite the attention of colleagues. To confirm this rumor, we report the sad story of a surgeon with bilateral vitreitis and glaucoma unresponsive to traditional therapies. Methods/Design. Case report. Results. After one year of steroidal and immunosuppressive therapy, a vitrectomy, and a trabeculectomy for unresponsive bilateral vitreitis and glaucoma, MRI showed a multicentre primary central nervous system lymphoma, which was the underlying cause of the masquerade syndrome. Conclusions. All ophthalmologists and clinicians must be aware of masquerade syndromes, in order to avoid delays in diagnosis.

  13. Acute Sheehan's syndrome presenting as central diabetes insipidus.

    Science.gov (United States)

    Robalo, Raquel; Pedroso, Célia; Agapito, Ana; Borges, Augusta

    2012-11-06

    Sheehan's syndrome occurs as a result of ischaemic pituitary necrosis due to severe postpartum haemorrhage. Improvements in obstetrical care have significantly reduced its incidence in developed countries, but postpartum pituitary infarction remains a common cause of hypopituitarism in developing countries. We report a case of severe postpartum haemorrhage followed by headache, central diabetes insipidus and failure to lactate, which prompted us to investigate and identify both anterior and posterior pituitary deficiency compatible with Sheehan's syndrome. A timely diagnosis allowed us to implement an adequate treatment and follow-up plan, which are known to improve clinical status and patient outcome.

  14. Prevalence of acute pulmonary embolism in central and subsegmental pulmonary arteries and relation to probability interpretation of ventilation/perfusion lung scans.

    Science.gov (United States)

    Stein, P D; Henry, J W

    1997-05-01

    The purpose of this investigation is to determine the prevalence of acute pulmonary embolism (PE) limited to subsegmental pulmonary arteries. Contrast-enhanced helical (spiral) and electron-beam CT, in the hands of experienced radiologists who are skillful with this modality, are sensitive for the detection of acute PE in central pulmonary arteries, but have a low sensitivity for the detection of PE limited to subsegmental pulmonary arteries. The potential for CT to diagnose PE, therefore, is partially dependent on the prevalence of PE limited to subsegmental pulmonary arteries. Data are from the Prospective Investigation of Pulmonary Embolism Diagnosis (PIOPED). The largest pulmonary arteries that showed PE, as interpreted by the PIOPED angiographic readers, were identified in 375 patients in PIOPED with angiographically diagnosed PE. Among all patients with PE, 6% (95% confidence interval [CI], 4 to 9%) had PE limited to subsegmental branches of the pulmonary artery. Patients with high-probability ventilation/ perfusion (V/Q) scans had PE limited to subsegmental branches in only 1% (95% CI, 0 to 4%). Among patients with low-probability V/Q lung scans, 17% (95% CI, 8 to 29%) had PE limited to the subsegmental branches. Patients with low-probability V/Q scans and no prior cardiopulmonary disease had PE limited to the subsegmental pulmonary arteries in 30% (95% CI, 13 to 53%), whereas patients with low-probability V/Q scans who had prior cardiopulmonary disease had PE limited to subsegmental pulmonary arteries in 8% (95% CI, 2 to 22%) (p PIOPED, the prevalence of PE limited to subsegmental pulmonary arteries is low, 6%. PE limited to subsegmental pulmonary arteries was most prevalent among patients with low-probability V/Q scans, particularly if they had no prior cardiopulmonary disease.

  15. Generalized status epilepticus associated with massive pulmonary aspiration and transient central diabetes insipidus: case report

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    CARVALHO MAURICIO

    2000-01-01

    Full Text Available Status epilepticus causes significant morbidity and mortality. A case of generalized status epilepticus followed by massive pulmonary aspiration, acute respiratory failure and transient central diabetes insipidus is presented. Seizures were promptly controlled, but the patient required mechanical ventilation and correction of polyuria with desmopressin acetate. During hospitalization mental status improved, diabetes insipidus spontaneously remitted and he was discharged without neurologic sequelae. The clinical and pathophysiological features of this case are discussed.

  16. Inhaled iloprost in preterm infants with severe respiratory distress syndrome and pulmonary hypertension.

    Science.gov (United States)

    Yilmaz, Osman; Kahveci, Hasan; Zeybek, Cenap; Ciftel, Murat; Kilic, Omer

    2014-04-01

    Many vasodilator drugs, including inhaled iloprost, are used to treat insufficient pulmonary vasodilatation, which is the main issue in pulmonary hypertension in newborns. The safety and efficacy of inhaled iloprost for the treatment of pulmonary hypertension were evaluated retrospectively in 15 preterm infants diagnosed with respiratory distress syndrome and pulmonary hypertension. The infants were unresponsive to surfactant and conventional mechanical ventilation and thus were treated with inhaled iloprost. Oxygenation parameters and hypoxemia improved rapidly after treatment. There was no decline in systemic blood pressure, no need for increased doses of vasopressor, and no side effects during treatment. One patient died of sepsis during treatment. In the treatment of severely sick premature babies with pulmonary hypertension, inhaled iloprost has high tolerability and a low incidence of systemic side effects. Based on the benefits of inhaled iloprost in preterm infants with pulmonary hypertension in this case series, further studies are required to evaluate its efficacy and safety in the preterm population. Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

  17. Inhibition of autophagy ameliorates pulmonary microvascular dilation and PMVECs excessive proliferation in rat experimental hepatopulmonary syndrome

    Science.gov (United States)

    Xu, Duo; Chen, Bing; Gu, Jianteng; Chen, Lin; Belguise, Karine; Wang, Xiaobo; Yi, Bin; Lu, Kaizhi

    2016-01-01

    Hepatopulmonary syndrome (HPS) is a defective liver-induced pulmonary vascular disorder with massive pulmonary microvascular dilation and excessive proliferation of pulmonary microvascular endothelial cells (PMVECs). Growing evidence suggests that autophagy is involved in pulmonary diseases, protectively or detrimentally. Thus, it is interesting and important to explore whether autophagy might be involved in and critical in HPS. In the present study, we report that autophagy was activated in common bile duct ligation (CBDL) rats and cultured pulmonary PMVECs induced by CBDL rat serum, two accepted in vivo and in vitro experimental models of HPS. Furthermore, pharmacological inhibition of autophagy with 3-methyladenine (3-MA) significantly alleviated pathological alterations and typical symptom of HPS in CBDL rats in vivo, and consistently 3-MA significantly attenuated the CBDL rat serum-induced excessive proliferation of PMVECs in vitro. All these changes mediated by 3-MA might explain the observed prominent improvement of pulmonary appearance, edema, microvascular dilatation and arterial oxygenation in vivo. Collectively, these results suggest that autophagy activation may play a critical role in the pathogenesis of HPS, and autophagy inhibition may have a therapeutic potential for this disease. PMID:27480323

  18. Right pulmonary artery agenesis with patent ductus arteriosus and Eisenmenger syndrome: a rare case diagnosed during the postpartum period.

    Science.gov (United States)

    Beker-Acay, Mehtap; Ozkececi, Gulay; Unlu, Ebru; Hocaoglu, Elif; Kacar, Emre; Onrat, Ersel

    2014-01-01

    Unilateral absence of a pulmonary artery a very rare congenital disorder. We here present a case of a 22-year-old female patient with agenesis of the right pulmonary artery accompanying patent ductus arteriosus and Eisenmenger syndrome, diagnosed by chest X-ray and multidetector computed tomography 5 days after giving birth.

  19. Imaging Findings of Central Nervous System Vasculitis Associated with Goodpasture's Syndrome: a Case Report

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    Kim, Jee Young; Ahn, Kook Jin; Jung, Jung Im; Jung, So Lyung; Kim, Bum Soo; Hahn, Seong Tae [College of Medicine, The Catholic University of Korea, Seoul (Korea, Republic of)

    2007-12-15

    We report a rare case of CNS vasculitis associated with Goodpasture's syndrome in a 34-year-old man, who presented with a seizure and sudden onset of right sided weakness. He also had recurrent hemoptysis of one month's duration. Goodpasture's syndrome is histologically diagnosed by intense linear deposits of IgG along the glomerular basement membrane in both renal and lung tissues. oodpasture's syndrome is a rare disease, characterized by rapidly progressive glomerulonephritis, diffuse pulmonary hemorrhage and circulating antiglomerular basement membrane antibody (anti-GBM antibody). Central nervous system (CNS) manifestations in Goodpasture's syndrome are extremely rare, with only a few cases having been reported in the literature (8 10). Therefore, we present our imaging findings of CNS vasculitis associated with Goodpasture's syndrome, together with a review of the relevant literature. In summary, CNS vasculitis associated with Goodpasture's syndrome is extremely rare. Awareness of the imaging findings, as well as the clinical significance of CNS vasculitis associated with Goodpasture's syndrome, can be helpful in making the correct diagnosis and subsequent management of this rare condition.

  20. [Tunnelled central venous line-associated infections in patients with pulmonary arterial hypertension treated with intravenous prostacyclin].

    Science.gov (United States)

    Boucly, Athénaïs; O'Connell, Caroline; Savale, Laurent; O'Callaghan, Dermot S; Jaïs, Xavier; Montani, David; Humbert, Marc; Simonneau, Gérald; Sitbon, Olivier

    2016-01-01

    Intravenous prostacyclins are a valuable treatment for patients with severe pulmonary arterial hypertension, leading to improved exercise capacity, haemodynamics, quality of life and survival. Unfortunately, due to the short half-life of these drugs, they need to be administered continuously through central venous catheters. Despite aseptic technique, regular dressing changes, tunneled central venous catheters and patient education, patients are exposed to central venous catheter associated infections. These infections cause significant morbidity and mortality. The clinical presentation, microbiology, consequences and management of these central venous catheter associated infections in pulmonary arterial hypertension patients treated with intravenous prostacyclins are discussed.

  1. Paget-Schroetter syndrome and pulmonary thromboembolism: clinical follow-up over 5 years.

    Science.gov (United States)

    Kobayashi, Hideo; Mimura, Satoshi; Motoyoshi, Kazuo

    2005-09-01

    A scaffold constructor lost consciousness at work, leading to emergency admission to our hospital. He had noted gradual worsening of exertional dyspnea over the previous 1 month. Chest radiography showed localized oligemia and enlarged hilar vessels. Pulmonary perfusion scintigraphy confirmed the existence of multiple perfusion defects, so a diagnosis of pulmonary thromboembolism was made. Upper and lower limb venography disclosed interruption of the right subclavian vein (so-called Paget-Schroetter syndrome). However, there was no difference in appearance between the right and left upper extremities. Five years after starting anticoagulant therapy, his symptoms have resolved, but serial perfusion scintigraphy and upper extremity venography revealed the persistence of abnormalities. In patients with pulmonary thromboembolism, lifestyle factors (especially heavy manual labor) should be considered and the possibility of subclavian vein thrombosis should be kept in mind.

  2. Mechanical characteristics of the pulmonary artery in beagle dogs with hepatopulmonary syndrome and portopulmonary hypertension.

    Science.gov (United States)

    Yan, Guozhen; He, Junfeng; Yu, Yueli; Liu, Yang; Yuan, Yanfen; Guo, Zhiyong

    2016-01-01

    The continuous changes in pulmonary hemodynamic properties in hepatopulmonary syndrome (HPS) and portopulmonary hypertension (PoPH) have not been fully characterized in large animal models of HPS and PoPH. Beagle dog models of HPS and PoPH were induced by chronic common bile duct ligation and Sephadex microspheres, respectively. The model was validated by catheter examination and pathological analyses, and the hemodynamic characteristics of the models were observed. The results revealed that the cross-sectional area of the blood vessel was significantly increased in HPS models, but it was significantly decreased in the PoPH models. Furthermore, the resistance of pulmonary circulation was elevated in models of HPS, but it was decreased in models of PoPH. The present findings renew the traditional view that pulmonary hypertension is due to the enhanced peripheral resistance.

  3. Síndromes hemorrágicas pulmonares Pulmonary hemorrhage syndromes

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    Eduardo da Rosa Borges

    2005-07-01

    Full Text Available As síndromes hemorrágicas pulmonares caracterizam-se por infiltrado pulmonar bilateral, queda dos níveis de hemoglobina e hipoxemia. Dentre as causas de sangramento estão as infecções, vasculites, coagulopatias e doenças do colágeno. A terapêutica consiste do tratamento da doença causal e suporte ventilatório, podendo ser associada a plasmaferese.Pulmonary hemorrhage syndromes are characterized by bilateral pulmonary infiltrates, decreased serum levels of hemoglobin, and hypoxemia. The causes of pulmonary hemorrhage include: infections, vasculitis, coagulopathies and collagen diseases. The therapy consists of treating the underlying disease and providing ventilatory support. In some cases, performing plasmapheresis can be beneficial.

  4. Asthma-chronic obstructive pulmonary disease overlap syndrome (ACOS): current literature review

    Science.gov (United States)

    Papaiwannou, Antonis; Porpodis, Konstantinos; Spyratos, Dionysios; Kioumis, Ioannis; Pitsiou, Georgia; Pataka, Athanasia; Tsakiridis, Kosmas; Arikas, Stamatis; Mpakas, Andreas; Tsiouda, Theodora; Katsikogiannis, Nikolaos; Kougioumtzi, Ioanna; Machairiotis, Nikolaos; Siminelakis, Stavros; Kolettas, Alexander; Kessis, George; Beleveslis, Thomas; Zarogoulidis, Konstantinos

    2014-01-01

    Asthma and chronic obstructive pulmonary disease (COPD) are chronic diseases, very common in general population. These obstructive airway illnesses are manifested with chronic inflammation affecting the whole respiratory tract. Obstruction is usually intermittent and reversible in asthma, but is progressive and irreversible in COPD. Asthma and COPD may overlap and converge, especially in older people [overlap syndrome—asthma-chronic obstructive pulmonary disease overlap syndrome (ACOS)]. Although ACOS accounts approximately 15-25% of the obstructive airway diseases, is not well recognised because of the structure of clinical trials. COPD studies exclude asthma patients and asthma studies exclude COPD patients, respectively. It is crucial to define asthma, COPD and overlap syndrome (ACOS), as notable clinical entities, which they share common pathologic and functional features, but they are characterized from differences in lung function, acute exacerbations, quality of life, hospital impact and mortality. PMID:24672688

  5. [Hantavirus pulmonary syndrome (Rio Mamore virus) in the Peruvian Amazon region].

    Science.gov (United States)

    Casapía, Martín; Mamani, Enrique; García, María P; Miraval, María L; Valencia, Pedro; Quino, Alberto H; Alvarez, Carlos; Donaires, Luis F

    2012-01-01

    Hantavirus infection is a viral zoonotic infection borne by rodents which most letal form clinical is the Hantavirus Pulmonary Syndrome (SPH, Spanish abbreviation). The Mamore River variant originates in South America and was found in rodents without any association to human diseases. Two cases of SPH were identified in the Peruvian Amazon region in November 2011. In both cases, a molecular diagnostic testing was conducted by the Instituto Nacional de Salud from Peru. A phylogenetic analysis of a viral genome fragment and a histopathological evaluation were conducted. Both patients developed adult respiratory distress syndrome and refractory shock. A patient died and another one recovered 12 days later.

  6. Hantavirus Pulmonary Syndrome in Santa Cruz, Bolivia: Outbreak Investigation and Antibody Prevalence Study

    Science.gov (United States)

    2012-10-18

    Ecology and epidemiology of an emerging virus in Latin America]. Medicina (B Aires) 66: 343–356. 22. Weissenbacher MC, Cura E, Segura EL, Hortal M, Baek LJ...et al. (1996) Serological evidence of human Hantavirus infection in Argentina, Bolivia and Uruguay. Medicina (B Aires) 56: 17–22. 23. Pini N (2004...Hantavirus in human and rodent population in an endemic area for hantavirus pulmonary syndrome in Argentina]. Medicina (B Aires) 62: 1–8. 26. Simonsen L

  7. Quantitative computed tomography measurements of emphysema for diagnosing asthma-chronic obstructive pulmonary disease overlap syndrome

    OpenAIRE

    Xie M; Wang W; Dou S; Cui L; Xiao W

    2016-01-01

    Mengshuang Xie, Wei Wang, Shuang Dou, Liwei Cui, Wei Xiao Department of Pulmonary Medicine, Qilu Hospital, Shandong University, Jinan, People’s Republic of China Background: The diagnostic criteria of asthma–COPD overlap syndrome (ACOS) are controversial. Emphysema is characteristic of COPD and usually does not exist in typical asthma patients. Emphysema in patients with asthma suggests the coexistence of COPD. Quantitative computed tomography (CT) allows repeated evalua...

  8. Chest sonography: a useful tool to differentiate acute cardiogenic pulmonary edema from acute respiratory distress syndrome

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    Soldati Gino

    2008-04-01

    Full Text Available Abstract Background Differential diagnosis between acute cardiogenic pulmonary edema (APE and acute lung injury/acute respiratory distress syndrome (ALI/ARDS may often be difficult. We evaluated the ability of chest sonography in the identification of characteristic pleuropulmonary signs useful in the diagnosis of ALI/ARDS and APE. Methods Chest sonography was performed on admission to the intensive care unit in 58 consecutive patients affected by ALI/ARDS or by acute pulmonary edema (APE. Results Ultrasound examination was focalised on finding in the two groups the presence of: 1 alveolar-interstitial syndrome (AIS 2 pleural lines abnormalities 3 absence or reduction of "gliding" sign 4 "spared areas" 5 consolidations 6 pleural effusion 7 "lung pulse". AIS was found in 100% of patients with ALI/ARDS and in 100% of patients with APE (p = ns. Pleural line abnormalities were observed in 100% of patients with ALI/ARDS and in 25% of patients with APE (p All signs, except the presence of AIS, presented a statistically significant difference in presentation between the two syndromes resulting specific for the ultrasonographic characterization of ALI/ARDS. Conclusion Pleuroparenchimal patterns in ALI/ARDS do find a characterization through ultrasonographic lung scan. In the critically ill the ultrasound demonstration of a dyshomogeneous AIS with spared areas, pleural line modifications and lung consolidations is strongly predictive, in an early phase, of non-cardiogenic pulmonary edema.

  9. The relationship between physical activity and metabolic syndrome in people with chronic obstructive pulmonary disease.

    Science.gov (United States)

    Park, Soo Kyung; Larson, Janet L

    2014-01-01

    The prevalence of metabolic syndrome has been reported to be 20% to 50% in people with chronic obstructive pulmonary disease (COPD). Because such people are sedentary and physically inactive, they are at risk of metabolic syndrome. The extent of this problem, however, is not fully understood. This study examined the relationship of sedentary time and physical activity to metabolic syndrome and the components of metabolic syndrome in a population-based sample of people with COPD. This was a secondary analysis of existing cross-sectional data. Subjects with COPD (n = 223) were drawn from the National Health and Nutrition Examination Survey data set (2003-2006). Physical activity was measured by accelerometry. Waist circumference, triglyceride level, high-density lipoprotein cholesterol level, blood pressure, and fasting glucose level were used to describe metabolic syndrome. Descriptive and inferential statistics were used for analysis. Fifty-five percent of the sample had metabolic syndrome. No significant differences in sedentary time and level of physical activity were found in people with COPD and metabolic syndrome and people with COPD only. However, those with a mean activity count of greater than 240 counts per minute had a lower prevalence of metabolic syndrome. Waist circumference and glucose level were significantly associated with the time spent in sedentary, light, and moderate to vigorous physical activity. Metabolic syndrome is highly prevalent in people with COPD, and greater physical activity and less sedentary time are associated with lower rates of metabolic syndrome. This suggests that interventions to decrease the risk of metabolic syndrome in people with COPD should include both reducing sedentary time and increasing the time and intensity of physical activity.

  10. Neuroimaging of Central Sensitivity Syndromes: Key Insights from the Scientific Literature.

    Science.gov (United States)

    Walitt, Brian; Ceko, Marta; Gracely, John L; Gracely, Richard H

    2016-01-01

    Central sensitivity syndromes are characterized by distressing symptoms, such as pain and fatigue, in the absence of clinically obvious pathology. The scientific underpinnings of these disorders are not currently known. Modern neuroimaging techniques promise new insights into mechanisms mediating these postulated syndromes. We review the results of neuroimaging applied to five central sensitivity syndromes: fibromyalgia, chronic fatigue syndrome, irritable bowel syndrome, temporomandibular joint disorder, and vulvodynia syndrome. Neuroimaging studies of basal metabolism, anatomic constitution, molecular constituents, evoked neural activity, and treatment effect are compared across all of these syndromes. Evoked sensory paradigms reveal sensory augmentation to both painful and nonpainful stimulation. This is a transformative observation for these syndromes, which were historically considered to be completely of hysterical or feigned in origin. However, whether sensory augmentation represents the cause of these syndromes, a predisposing factor, an endophenotype, or an epiphenomenon cannot be discerned from the current literature. Further, the result from cross-sectional neuroimaging studies of basal activity, anatomy, and molecular constituency are extremely heterogeneous within and between the syndromes. A defining neuroimaging "signature" cannot be discerned for any of the particular syndromes or for an over-arching central sensitization mechanism common to all of the syndromes. Several issues confound initial attempts to meaningfully measure treatment effects in these syndromes. At this time, the existence of "central sensitivity syndromes" is based more soundly on clinical and epidemiological evidence. A coherent picture of a "central sensitization" mechanism that bridges across all of these syndromes does not emerge from the existing scientific evidence.

  11. The Effects of Aquaporin-1 in Pulmonary Edema Induced by Fat Embolism Syndrome.

    Science.gov (United States)

    Zhang, Yiwei; Tian, Kun; Wang, Yan; Zhang, Rong; Shang, Jiawei; Jiang, Wei; Wang, Aizhong

    2016-07-21

    This study was designed to investigate the role of aquaporin1 (AQP1) in the pathologic process of pulmonary edema induced by fat embolism syndrome (FES) and the effects of a free fatty acid (FFA) mixture on AQP1 expression in pulmonary microvascular endothelial cells (PMVECs). In vivo, edema was more serious in FES mice compared with the control group. The expression of AQP1 and the wet-to-dry lung weight ratio (W/D) in the FES group were significantly increased compared with the control group. At the same time, inhibition of AQP1 decreased the pathological damage resulting from pulmonary edema. Then we performed a study in vitro to investigate whether AQP1 was induced by FFA release in FES. The mRNA and protein level of AQP1 were increased by FFAs in a dose- and time-dependent manner in PMVECs. In addition, the up-regulation of AQP1 was blocked by the inhibitor of p38 kinase, implicating the p38 MAPK pathway as involved in the FFA-induced AQP1 up-regulation in PMVECs. Our results demonstrate that AQP1 may play important roles in pulmonary edema induced by FES and can be regarded as a new therapy target for treatment of pulmonary edema induced by FES.

  12. Effect of high laser output on the central bronchi and pulmonary artery.

    Science.gov (United States)

    Kirschbaum, A; Rexin, P; Bartsch, D K; Quint, K

    2017-05-01

    A diode-pump Nd:YAG high-power laser (wavelength 1320 nm, power 100 W) is routinely used to surgically remove lung metastases. Even pulmonary lesions in central locations are resectable via this method, yet it also carries a potential risk of damaging the larger bronchi and vessels in the vicinity. Studies investigating the safety of using high-power lasers are lacking. We therefore aimed to examine the direct effects of a 100-watt laser on the bronchi and pulmonary artery at a standard working velocity. From freshly slaughtered pigs, we isolated cylindrical specimens of the trachea, the main and lobar bronchi, and the central pulmonary artery from the both lungs. These specimens were fixed consecutively in rows behind each other on a Styrofoam surface in the laboratory. The laser's handle was clamped into a hydraulic feed unit so that the laser was focused at constant distance perpendicular to the tissue and would move at 10 mm/s over the specimens. The Nd:YAG Laser LIMAX® 120 functioned at a consistent power of 100 W during all the experiments. The lasered specimens were examined macroscopically and histologically for tissue damage. None of the trachea or bronchial walls were perforated. Compared to the pulmonary parenchyma, we observed no vaporization effects-only minor superficial coagulation (with a mean depth of 2.1 ± 0.8 mm). This finding was histologically confirmed in each specimen, which revealed mild superficial coagulation and no damage to the cartilage. In the presence of a residual peribronchial fatty tissue, the laser effect was even attenuated. The pulmonary arteries presented no lumen openings whatsoever, merely a discrete trace of coagulation. The vessel wall revealed increased vacuolization without alteration of the remaining vessel wall. In conclusion, laser resection at 100 W of the central lung areas is safe with respect to airways and blood vessels and the laser output does not need to be reduced when treating these areas.

  13. Pulmonary Thromboembolism in Klinefelter%u2019s Syndrome Patient with Deficient of Protein C

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    Mehmet Yigit

    2013-08-01

    Full Text Available Klinefelter syndrome (KS is a common genetic disorder caused by one or more supernumerary X chromosomes. KS poses an increased risk for venous thromboembolic events such as deep venous thrombosis and pulmonary embolism. Klinefelter syndrome is prone to hypercoagulability due to hormonal imbalance and one or more inherited thrombophilic factors. Therefore, patients with KS having a medical history of venous thromboembolism require chest computed tomographic (CT images and oral anticoagulation therapy for a period of at least six months. A 21 year old, male patient diagnosed with Klinefelter syndrome was presented to the emergency department of our hospital with primary complaints of left lower extremity pain lasting for 2 months. Deep venous thromboembosis (DVT was diagnosed via venous doppler ultrasound and pulmonary thromboembolism in his chest CT images. Following anticoagulation treatment, his symptoms recovered. An endocrinologic test should be ordered in patients having klinefelter syndrome with a medical or familial history of venous thromboembolism as well as additional assessment of innate or acquired thrombophilia should be made.

  14. [Airway clearance techniques in chronic obstructive pulmonary syndrome : 2011 update].

    Science.gov (United States)

    Opdekamp, C

    2011-09-01

    For many years the airway clearance techniques used in chest physical therapy were assimilated with the singular technique of postural drainage, percussions and vibrations. However the side effects and counter indications and the lack of scientific proof regarding this technique have forced reflection and development of other techniques more comfortable and without deleterious effects. If all these techniques show a high efficiency in terms of improved mucociliary clearance, the literature is unanimous on how little effect these techniques have in the short and the long-term with regards to lung function and arterial blood gases. In view of the scientific literature, it is clear that the airway clearance techniques don't have the same recognition concerning their efficiency in all obstructive pulmonary diseases. As the cornerstone in the management of cystic fibrosis, the efficiency of the bronchial hygiene techniques are in general poorly documented in the management of the non-cystic fibrosis bronchiectasis, bronchitis or emphysema. The use of the chest physical therapy seems more to do with the interpretation of the imagery and symptomatology. The airway clearance techniques should be individualised according to symptoms, the amount of expectorated mucus and the objectives signs of secretions retention or subjective signs of difficulty expectorating secretions with progression of the disease.

  15. Scimitar syndrome: Anaesthetic management for pulmonary resection of the unaffected lung.

    Science.gov (United States)

    Fernández-Torres, B; Fernández-López, A; Congregado, M

    2017-05-26

    Scimitar syndrome is a rare congenital anomaly characterized by anomalous drainage of the right pulmonary veins in the inferior vena cava, frequently associated with right lung and pulmonary artery hypoplasia, dextrocardia and abnormal systemic arterial supply to the lower lobe. Pulmonary resection surgery on healthy lung is exceptional, and there are no published records of it, as far as we know. A man with scimitar syndrome diagnosed with a lung nodule with malignant features in the contralateral lung. This situation implies huge anaesthetic complexity, mainly for intraoperative ventilation. Although spirometry and stress test did not contraindicate the planned lobectomy, scintigraphy showed a hypoplastic right lung with an uptake of 15%. From an anaesthetic point of view we discarded selective ventilation of the right lung, since the shunt made it functionally non-existent. In consequence we proposed four anaesthetic possibilities. After the placement of an epidural catheter and left selective intubation, thoracoscopy with intermittent apnoeas was our first choice, and we could complete the extirpation and avoid excessive complexity. Copyright © 2017 Sociedad Española de Anestesiología, Reanimación y Terapéutica del Dolor. Publicado por Elsevier España, S.L.U. All rights reserved.

  16. Metabolic Syndrome in Chemical Warfare Patients with Chronic Obstructive Pulmonary Disease

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    Shahrzad M. Lari

    2014-11-01

    Full Text Available   Introduction: Sulfur mustard (SM, a toxic alkylating gas, can cause serious long-term pulmonary complications such as chronic obstructive pulmonary disease (COPD. Metabolic syndrome (MetS is one of the important comorbidities of COPD. This study was designed to evaluate the frequency of metabolic syndrome in Iranian chemical warfare patients (CWPs with COPD. Materials and Methods: Thirty CWPs with a mean age of 46.93± 6.8 were enrolled in this study. The following parameters were studied in: complete pulmonary function tests, health-related quality of life, serum triglycerides (TG, high density lipoprotein (HDL and fasting blood sugar (FBS levels. Additionally, 32 COPD patients and 56 healthy persons were considered as control groups who were matched to CWPs. Results: We found a statistically significant difference in the frequency of MetS between the COPD patients and the healthy control group (p=0.04. Additionally, we observed a statistically significant difference in the mean HDL levels among these groups (p=

  17. [A case of clinical overlap syndrome of rheumatoid arthritis and amyopathic dermatomyositis with multiple pulmonary injuries].

    Science.gov (United States)

    Liu, Shuang; An, Yuan; Jia, Yuan; Li, Zhan-guo

    2014-10-18

    Autoimmune diseases can cause various kinds of lung injuries. Clinical features of a case of overlap syndrome with multiple pulmonary injuries were investigated, and the treatment experiences discussed. The patient suffered from rheumatoid arthritis (RA) at first, and then had a lobectomy surgery due to the rheumatoid nodules in her right lung. A year later her disease was diagnosed as amyopathic dermatomyositis (ADM) with typical Gottron's sign, craftsmen hands and rapid-progressive organizing pneumonia (OP). After a combined treatment with glucocorticoids (GCs) and cyclophosphamide (CTX), her OP became better but lung infections progressed. Her lung infections eventually were cured after we used antibiotics and antifungal treatment while we ceased to use CTX and reduced the dosage of GCs. The clinical feature of the patient was overlap syndrome with a variety of lung injuries, such as pulmonary rheumatoid nodules, OP, secondary bronchopleural fistula and lung infections. Its diagnosis and treatment experiences could improve our understanding of pulmonary manifestations of connective tissue disease and improve our diagnosis and treatment level.

  18. Dilated cardiomyopathy in a patient with antibody-negative Goodpasture′s syndrome and pulmonary relapse

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    Solak Yalcin

    2010-01-01

    Full Text Available The Goodpasture′s syndrome, also konwn as anti-GBM disease, is an uncommon disease, responsible for 20% of all cases of rapidly progressive glomerulonephritis. Anti-GBM antibodies are directed against non-collagenous domain (NC-1 of the alpha-3 chain of type IV collagen. When conventional ELISA assays are used, these antibodies can be detected in almost all the patients. Nevertheless, some reports have described antibody-negative relapsing disease. Some aggravating factors, namely, smoking, pulmonary infection and hypervolemia, may expose embedded antigenic target and may be responsible for the relapse. In addition, these antibody-negative relapses also respond to standard treatment, which comprises of plasma exchange, pulse steroids and cyclophosphamide. Herein, we report a patient who presented at the Selcuk Univer-sity Meram School of Medicine, Meram, Konya, Turkey, with the pulmonary-renal syndrome. He was also found to have idiopathic dilated cardiomyopathy (DCM. To our knowledge, this is the first report describing co-existence of DCM and anti-GBM disease. There is growing evidence showing strong relation of both DCM and anti-GBM disease with HLA. Although not proven, this might have occurred in our patient. In our opinion, volume overload was facilitated by anuria and DCM and led to an antibody-negative pulmonary relapse. The relapse was treated just as the first episode and the patient improved satisfactorily.

  19. Bilateral chylothorax in a patient with chronic central vein thrombosis and chronic thromboembolic pulmonary hypertension

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    Avdhesh Bansal

    2015-01-01

    Full Text Available The chylothorax is not a common presentation, and bilateral chylothorax in patients with chronically high central venous pressure secondary to venous thrombosis is a rare in incidence. We reported a case of bilateral chylothorax in a patient of chronic deep vein thrombosis (DVT in central veins with chronic thromboembolic pulmonary hypertension who presented with 2 weeks history of increased breathlessness, bilateral chest discomfort and weakness. Work-up with chest X-ray and ultrasonography-chest showed gross left sided and mild right sided pleural effusion, thoracocentesis was consistent with chylothorax. Contrast enhanced computed tomography-chest showed multiple collateral formation of left side subclavian vein, venous Doppler showed old DVT in right and left subclavian veins and two-dimensional echocardiogram showed finding of severe pulmonary hypertension. After 24 h of fasting and conservative management, pleural drain became clear and decreased in the amount. Patient′s video assisted thoracoscopic surgery was done, and thoracic duct was ligated and cut down at diaphragmatic level and bilateral talc pleurodesis done. Patient improved clinically and radiologically.

  20. Congenital central hypoventilation syndrome: diagnostic and management challenges

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    Kasi AS

    2016-08-01

    Full Text Available Ajay S Kasi,1 Iris A Perez,1,2 Sheila S Kun,1 Thomas G Keens1,2 1Division of Pediatric Pulmonology and Sleep Medicine, Children’s Hospital Los Angeles, 2Keck School of Medicine of the University of Southern California, Los Angeles, CA, USA Abstract: Congenital central hypoventilation syndrome (CCHS is a rare genetic disorder with failure of central control of breathing and of the autonomic nervous system function due to a mutation in the paired-like homeobox 2B (PHOX2B gene. Affected patients have absent or negligible ventilatory sensitivity to hypercapnia and hypoxemia, and they do not exhibit signs of respiratory distress when challenged with hypercarbia or hypoxia. The diagnosis of CCHS must be confirmed with PHOX2B gene mutation. Generally, the PHOX2B mutation genotype can aid in anticipating the severity of the phenotype. They require ventilatory support for life. Home assisted ventilation options include positive pressure ventilation via tracheostomy, noninvasive positive pressure ventilation, and diaphragm pacing via phrenic nerve stimulation, but each strategy has its associated limitations and challenges. Since all the clinical manifestations of CCHS may not manifest at birth, periodic monitoring and early intervention are necessary to prevent complications and improve outcome. Life-threatening arrhythmias can manifest at different ages and a normal cardiac monitoring study does not exclude future occurrences leading to the dilemma of timing and frequency of cardiac rhythm monitoring and treatment. Given the rare incidence of CCHS, most health care professionals are not experienced with managing CCHS patients, particularly those with diaphragm pacers. With early diagnosis and advances in home mechanical ventilation and monitoring strategies, many CCHS children are surviving into adulthood presenting new challenges in their care. Keywords: congenital central hypoventilation syndrome, PHOX2B, home mechanical ventilation, diaphragm

  1. [Pulmonary contusion and acute respiratory distress syndrome (ARDS) as complications of blunt chest trauma].

    Science.gov (United States)

    Michalska, Agata; Jurczyk, Agnieszka P; Machała, Waldemar; Szram, Stefan; Berent, Jarosław

    2009-01-01

    Blunt chest traumas are common nowadays due to development of motor transport. They are associated with high mortality rates because of serious injuries of internal organs. The mechanisms of injuries are complex and may cause damages ranging from small ones, such as bruises or abrasions, to life-threatening trauma. Among typical injuries there are rib fractures, sternal fractures, pneumothorax, hemothorax, diaphragm lacerations, pulmonary contusions, cardiac tamponade, cardiac rupture and many others. The authors of the article would like to emphasize the pathophysiology and diagnostic difficulties in such blunt chest trauma complications as pulmonary contusions and acute respiratory distress syndrome, for which no causal treatment is available and only early diagnosis and administration of symptomatic treatment may increase the patients' chances to survive. In Forensic Medicine Department, Medical University of Łódź, an opinion was issued on a case which illustrates the clinical problem.

  2. Goldenhar Syndrome with Dextrocardia and Right Pulmonary Hypoplasia: An Unusual Association

    Science.gov (United States)

    Halwai, Hemant Kumar

    2017-01-01

    Goldenhar syndrome (GS), a rare condition, occurring due to defect in development of first and second branchial arches, is characterized by a combination of various anomalies involving face, eyes, ears, vertebrae, heart, and lungs. The etiology of GS is not fully known, although various hypotheses have been proposed along with its genetic association and many other causes. Facial asymmetry and hypoplasia of the mandible are characteristic features of GS along with microtia and preauricular appendages and pits. Dextrocardia or pulmonary hypoplasia in GS has previously been reported separately. We report a 7-year-old female child of GS with combination of anomalies, dextrocardia, and pulmonary hypoplasia, which is a rare association. PMID:28377826

  3. A Case of Pulmonary-Renal Syndrome Leading to the Diagnosis of Legionnaires' Disease.

    Science.gov (United States)

    Sabani, Erasmia; Sarafidis, Pantelis A; Lazaridis, Antonios; Kouloukourgiotou, Theodora; Stylianou, Konstantinos; Pantzaki, Afroditi; Papagianni, Aikaterini; Efstratiadis, Georgios

    2016-01-01

    We report a case of a 51-year-old Caucasian man referred at our department due to acute renal failure (ARF) complicating respiratory failure during hospitalization in a regional hospital. The patient was previously started on steroids due to the suspicion of rapidly progressive glomerulonephritis (RPGN) in the context of Goodpasture syndrome. However, clinical and laboratory findings did not support this diagnosis; instead a careful evaluation limited differential diagnosis of the renal insult to acute tubular necrosis or acute interstitial nephritis (AIN) following respiratory infection. With lung function fully improved but renal function not recovering, a renal biopsy revealed AIN, a finding leading to further diagnostic testing and finally to the diagnosis of Legionnaires' disease as a cause of this patient's pulmonary-renal syndrome. The management consisted of progressive tapering of oral steroids associated with full recovery of the patient's renal function. This is a rare case of Legionnaires' disease causing immune-mediated AIN and highlights the possibility of Legionella infection as a cause of pulmonary-renal syndrome.

  4. A Case of Pulmonary-Renal Syndrome Leading to the Diagnosis of Legionnaires’ Disease

    Directory of Open Access Journals (Sweden)

    Erasmia Sabani

    2016-01-01

    Full Text Available We report a case of a 51-year-old Caucasian man referred at our department due to acute renal failure (ARF complicating respiratory failure during hospitalization in a regional hospital. The patient was previously started on steroids due to the suspicion of rapidly progressive glomerulonephritis (RPGN in the context of Goodpasture syndrome. However, clinical and laboratory findings did not support this diagnosis; instead a careful evaluation limited differential diagnosis of the renal insult to acute tubular necrosis or acute interstitial nephritis (AIN following respiratory infection. With lung function fully improved but renal function not recovering, a renal biopsy revealed AIN, a finding leading to further diagnostic testing and finally to the diagnosis of Legionnaires’ disease as a cause of this patient’s pulmonary-renal syndrome. The management consisted of progressive tapering of oral steroids associated with full recovery of the patient’s renal function. This is a rare case of Legionnaires’ disease causing immune-mediated AIN and highlights the possibility of Legionella infection as a cause of pulmonary-renal syndrome.

  5. Calcified pulmonary thromboembolism in a child with sickle cell disease: value of multidetector CT in patients with acute chest syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Staser, Jonathan A. [Indiana University Medical Center, Department of Radiology, Indianapolis, IN (United States); Alam, Tariq [Medical College of Ohio, Department of Radiology, Toledo, OH (United States); Applegate, Kimberly [Indiana University Medical Center, Sections of Pediatric Radiology and Health Services Research, Indianapolis, IN (United States); Indiana University Medical Center, Department of Radiology, Riley Hospital for Children, Indianapolis, IN (United States)

    2006-06-15

    The incidence of pulmonary embolism in children is not clearly known, but is believed to be low. Risk factors for pulmonary thromboembolism include central venous catheter, malignancy, surgery, infection, trauma, and congenital hypercoagulable disorders. Children with sickle cell disease are prothrombotic and are at an increased risk of thromboembolism. The incidence of this event is unknown because these children are often not thoroughly imaged. We report here a case of a calcified pulmonary thromboembolism in a child with sickle cell disease and emphasize the use of multidetector CT in detection of pulmonary thromboembolism in children with sickle cell disease. (orig.)

  6. Acute central cord syndrome: injury mechanisms and stress features.

    Science.gov (United States)

    Li, Xin-Feng; Dai, Li-Yang

    2010-09-01

    Numerical techniques were used to study the mechanisms of acute central cord syndrome. To analyze the features of stress distribution in the cervical cord under different injury conditions using finite element model of the cervical cord and to improve the understanding of the possible pathogenesis of acute central cord syndrome. Acute central cord spinal injury was initially attributed to hemorrhagic damage to the central portion of the spinal cord, but recent histopathologic studies showed that it was predominantly a white matter injury. The precise anatomic location of neuronal injury and the etiology of the clinical manifestation were poorly understood. Cervical cord injury was simulated using a finite element model of the cervical enlargement described previously, with the model loaded under 3 traumatic postures: neutral, flexion, and extension. Five traumatic conditions were simulated and analyzed: hyperextension with the pinch force directed to the anterior (A) or posterior (B); flexion injuries (C), vertical compression with the pinch force directed to the anterior (D) or posterior (E). After simulation, several representative cross-sections of each traumatic pattern were selected. In each cross-section, the average von Mises stress of 9 regions, such as anterior funiculus, lateral part of the lateral funiculus, medial part of the lateral funiculus, lateral part of the posterior funiculus, medial part of the posterior funiculus, anterior horn, the bottom of anterior horn, the cervix cornu posterioris, the caput cornu posterioris, and the apex cornu posterioris was recorded. High localized stress occurred at the portion under compression injury and the level above it. High localized stress tended to occur at the lateral part of the anterior horn motor neurons innervating the hand muscles in traumatic conditions A and D. Under conditions A, D, and E, the average localized stress at the anterior and posterior horn of the gray matter was higher than that at the

  7. Simple pulmonary eosinophilia

    Science.gov (United States)

    Pulmonary infiltrates with eosinophilia; Loffler syndrome; Eosinophilic pneumonia; Pneumonia - eosinophilic ... A rare complication of simple pulmonary eosinophilia is a severe type of pneumonia called acute idiopathic eosinophilic pneumonia.

  8. Prophylaxis of neonatal respiratory distress syndrome by intra-amniotic administration of pulmonary surfactant

    Institute of Scientific and Technical Information of China (English)

    张建平; 王英兰; 王蕴慧; 张睿; 陈环; 苏浩彬

    2004-01-01

    Background Neonatal respiratory distress syndrome (NRDS) is caused by a deficiency in pulmonary surfactant (PS) and is one of the main reasons of neonatal mortality. This study was conducted to evaluate the efficacy and safety of intra-amniotic administration of pulmonary surfactant for prophylaxis of NRDS.Methods Forty-five pregnant women who were due for preterm delivery and whose fetuses' lungs proved immature were divided into two groups. Fifteen women (study group) were administered one dose of pulmonary surfactant injected into the amniotic cavity and delivered within several hours. Nothing was injected into the amniotic cavity of 30 women of the control group. The proportion of neonatal asphyxia, NRDS, mortality and the time in hospital were analyzed to determine if there was any difference between the two groups. Results There was no significant difference between the two groups for neonatal asphyxia. Foam tests showed that higher proportion of neonates in the study group than in the control group (56.3% vs 13.3%, P<0.05) had lung maturity. A greater number of control neonates (11/30, 32.3%) had NRDS, compared with the neonates given PS via the amniotic cavity before delivery (1/16, 6.3%, P<0.05). The neonates in the study group spent nearly 10 days less in hospital than the control group [(32.4±7.6) days vs (42.0±15.7) days, P<0.05], but the difference in mortality between the two groups was not statistically significant. Conclusions Intra-amniotic administration of pulmonary surfactant can significantly reduce the proportion of NRDS and the time in hospital of preterm neonates. Whether this method can reduce the mortality of preterm neonates needs to be evaluated further. Intra-amniotic administration of pulmonary surfactant provides an additional effectual means for NRDS prophylaxis.

  9. Hantavirus pulmonary syndrome in a Chilean patient with recent travel in Bolivia.

    Science.gov (United States)

    Espinoza, R; Vial, P; Noriega, L M; Johnson, A; Nichol, S T; Rollin, P E; Wells, R; Zaki, S; Reynolds, E; Ksiazek, T G

    1998-01-01

    A case of hantavirus pulmonary syndrome (HPS) was serologically confirmed in a critically ill patient in Santiago, Chile. The patient's clinical course had many similarities to that of other HPS patients in North and South America but was complicated by acute severe renal failure. The patient's history included self-reported urban and probable rural rodent exposure during travel in Bolivia. Comparison of a viral sequence from an acute-phase serum sample with other known hantaviruses showed that the hantavirus nucleic acid sequence from the patient was very similar to a virus recently isolated from rodents associated with HPS cases in Paraguay.

  10. Asthma-Chronic Obstructive Pulmonary Disease Overlap Syndrome: Nothing New Under the Sun.

    Science.gov (United States)

    Putcha, Nirupama; Wise, Robert A

    2016-08-01

    The debate about whether asthma and chronic obstructive pulmonary disease (COPD) are distinct clinical syndromes is not new; there is heightened interest in understanding the group of individuals with obstructive lung disease who seem to have elements of both conditions because recent studies have demonstrated increased risk for respiratory events and exacerbations. We describe the clinical characteristics of this subtype of disease and suggest 4 working definitions of individuals who would fall into the asthma-COPD overlap category. Understanding the mechanisms underlying these subtypes will hopefully lead into a better understanding of therapeutic strategies that can target specific pathobiologic pathways.

  11. Prolonged venovenous extracorporeal membrane oxygenation without anticoagulation: a case of Goodpasture syndrome-related pulmonary haemorrhage.

    Science.gov (United States)

    Herbert, David G; Buscher, Hergen; Nair, Priya

    2014-03-01

    We present a 16-year-old male with severe acute respiratory and renal failure as a result of Goodpasture syndrome, requiring venovenous extracorporeal membrane oxygenation (VV-ECMO) for pulmonary haemorrhage. The patient received no systemic anticoagulation for 25 of 26 ECMO days (20 days consecutively) and suffered no coagulation-related adverse events. The patient had a subtherapeutic anticoagulation profile according to recommended ECMO guidelines during most of this time. The patient made a full recovery without respiratory compromise, ECMO circuit failure, thrombotic events or the need for ongoing haemodialysis.

  12. Catastrophic Antiphospholipid Syndrome Presenting as Bilateral Central Retinal Artery Occlusions

    Directory of Open Access Journals (Sweden)

    Steven S. Saraf

    2015-01-01

    Full Text Available A previously healthy 22-year-old African American woman presented with bilateral vision loss associated with headache. Her ocular examination was significant for bilateral retinal arterial “boxcarring,” retinal whitening, retinal hemorrhages, and cherry red spots. She was diagnosed with bilateral central retinal artery occlusions and was hospitalized due to concomitant diagnosis of stroke and hypercoagulable state. She was also found to be in heart failure and kidney failure. Rheumatology was consulted and she was diagnosed with catastrophic antiphospholipid syndrome in association with systemic lupus erythematosus. Approximately 7 months after presentation, the patient’s vision improved and remained stable at 20/200 and 20/80.

  13. Hippocampal volume reduction in congenital central hypoventilation syndrome.

    Directory of Open Access Journals (Sweden)

    Paul M Macey

    Full Text Available Children with congenital central hypoventilation syndrome (CCHS, a genetic disorder characterized by diminished drive to breathe during sleep and impaired CO(2 sensitivity, show brain structural and functional changes on magnetic resonance imaging (MRI scans, with impaired responses in specific hippocampal regions, suggesting localized injury.We assessed total volume and regional variation in hippocampal surface morphology to identify areas affected in the syndrome. We studied 18 CCHS (mean age+/-std: 15.1+/-2.2 years; 8 female and 32 healthy control (age 15.2+/-2.4 years; 14 female children, and traced hippocampi on 1 mm(3 resolution T1-weighted scans, collected with a 3.0 Tesla MRI scanner. Regional hippocampal volume variations, adjusted for cranial volume, were compared between groups based on t-tests of surface distances to the structure midline, with correction for multiple comparisons. Significant tissue losses emerged in CCHS patients on the left side, with a trend for loss on the right; however, most areas affected on the left also showed equivalent right-sided volume reductions. Reduced regional volumes appeared in the left rostral hippocampus, bilateral areas in mid and mid-to-caudal regions, and a dorsal-caudal region, adjacent to the fimbria.The volume losses may result from hypoxic exposure following hypoventilation during sleep-disordered breathing, or from developmental or vascular consequences of genetic mutations in the syndrome. The sites of change overlap regions of abnormal functional responses to respiratory and autonomic challenges. Affected hippocampal areas have roles associated with memory, mood, and indirectly, autonomic regulation; impairments in these behavioral and physiological functions appear in CCHS.

  14. Clinician gestalt estimate of pretest probability for acute coronary syndrome and pulmonary embolism in patients with chest pain and dyspnea.

    Science.gov (United States)

    Kline, Jeffrey A; Stubblefield, William B

    2014-03-01

    Pretest probability helps guide diagnostic testing for patients with suspected acute coronary syndrome and pulmonary embolism. Pretest probability derived from the clinician's unstructured gestalt estimate is easier and more readily available than methods that require computation. We compare the diagnostic accuracy of physician gestalt estimate for the pretest probability of acute coronary syndrome and pulmonary embolism with a validated, computerized method. This was a secondary analysis of a prospectively collected, multicenter study. Patients (N=840) had chest pain, dyspnea, nondiagnostic ECGs, and no obvious diagnosis. Clinician gestalt pretest probability for both acute coronary syndrome and pulmonary embolism was assessed by visual analog scale and from the method of attribute matching using a Web-based computer program. Patients were followed for outcomes at 90 days. Clinicians had significantly higher estimates than attribute matching for both acute coronary syndrome (17% versus 4%; Psyndrome (r(2)=0.15) and pulmonary embolism (r(2)=0.06). Areas under the receiver operating characteristic curve were lower for clinician estimate compared with the computerized method for acute coronary syndrome: 0.64 (95% confidence interval [CI] 0.51 to 0.77) for clinician gestalt versus 0.78 (95% CI 0.71 to 0.85) for attribute matching. For pulmonary embolism, these values were 0.81 (95% CI 0.79 to 0.92) for clinician gestalt and 0.84 (95% CI 0.76 to 0.93) for attribute matching. Compared with a validated machine-based method, clinicians consistently overestimated pretest probability but on receiver operating curve analysis were as accurate for pulmonary embolism but not acute coronary syndrome. Copyright © 2013 American College of Emergency Physicians. Published by Mosby, Inc. All rights reserved.

  15. Central nervous system and cervical spine abnormalities in Apert syndrome.

    Science.gov (United States)

    Breik, Omar; Mahindu, Antony; Moore, Mark H; Molloy, Cindy J; Santoreneos, Stephen; David, David J

    2016-05-01

    Apert syndrome characterized by acrocephalosyndactyly is a rare autosomal dominant congenital malformation with a prevalence of 1/65,000 births. With an extensive range of phenotypic and developmental manifestations, its management requires a multidisciplinary approach. A variety of craniofacial, central nervous system (CNS), and cervical spine abnormalities have been reported in these patients. This study aimed to determine the incidence of these CNS abnormalities in our case series. Retrospective review of Australian Craniofacial Unit (ACFU) database for Apert patients was performed. Data collected that included demographics, place of origin, age at presentation, imaging performed, and images were reviewed and recorded. Where available, developmental data was also recorded. Ninety-four patients seen and managed at the ACFU had their CNS and cervical spine abnormalities documented. The main CNS abnormalities were prominent convolutional markings (67 %), ventriculomegaly (48 %), crowded foramen magnum (36 %), deficient septum pellucidum (13 %), and corpus callosum agenesis in 11 %. Major C-spine findings were present in 50.8 % of patients and included fusion of posterior elements of C5/C6 (50 %) and C3/4 (27 %). Multilevel fusion was seen in 20 %. Other abnormalities were C1 spina bifida occulta (7 %) and atlanto-axial subluxation (7 %). Multiple CNS and cervical spine (c-spine) abnormalities are common in Apert syndrome. The significance of these abnormalities remains largely unknown. Further research is needed to better understand the impact of these findings on growth, development, and treatment outcomes.

  16. Pulmonary diseases in patients infected with the human immunodeficiency virus in Zimbabwe, Central Africa.

    Science.gov (United States)

    McLeod, D T; Neill, P; Robertson, V J; Latif, A S; Emmanuel, J C; Els, J E; Gwanzura, L K; Trijssenaar, F E; Nziramasanga, P; Jongeling, G R

    1989-01-01

    During the 11 month period up to 30 September 1987, 37 patients (26 male, 11 female, mean age 27 years) with respiratory symptoms who were human immunodeficiency virus (HIV) positive, were studied prospectively on 40 occasions to determine the cause of any pulmonary complications. HIV was heterosexually transmitted. Predominant symptoms were cough (89%), fever (89%), weight loss (83%), and dyspnoea (60%). Transnasal fibre-optic bronchoscopy (with bronchoalveolar lavage, bronchial brushings and transbronchial lung biopsies) was performed on 35 patients, twice on 3 patients. 'Tru-cut' lung biopsies were obtained from 2 patients who died before bronchoscopy. Pulmonary tuberculosis was the commonest disease, being found in one-third of the patients (12 of 37). Mycobacterium tuberculosis was cultured from 4; the remainder of the plates were contaminated. Pneumocystis carinii was present in 8 patients: as the sole pathogen in 3, with Streptococcus pneumoniae in 4, Staphylococcus aureus in 2, and one also had tuberculous lymphadenitis. Endobronchial Kaposi's sarcoma was seen in 6 of 7 patients with skin nodules. Bacterial pathogens isolated included Staph. aureus (5), S. pneumoniae (5), Klebsiella pneumoniae (2), Haemophilus influenzae (2), H. parainfluenzae (1) and Pseudomonas aeruginosa (1). Invading Aspergillus fumigatus was diagnosed by lung biopsy in one. No diagnosis was reached for 8 patients. It is concluded that in Central Africa pulmonary complications in AIDS patients are similar to those in Europe and North America but the incidence of different pathogens depends on the prevalence of pathogens in the community. M. tuberculosis is probably the commonest pathogen. This study has confirmed that P. carinii pneumonia does occur, but occurs less frequently.

  17. Central sleep apnoea syndrome in chronic heart failure : an underestimated and treatable comorbidity

    NARCIS (Netherlands)

    Muller, M.; de Jong, M.; Jaarsma, T.; Voors, A. A.; Nieuwenhuis, J. A.; Wijkstra, P. J.; Koops, A.

    Chronic heart failure is a clinical syndrome with a high mortality and morbidity. Despite optimal therapy, five-year survival is still only 50%. Central sleep apnoea syndrome is seen in approximately 40% of patients with congestive heart failure. Sleep apnoea syndrome can be divided into two forms

  18. Pulmonary hantavirus syndrome: case report and brief review

    Directory of Open Access Journals (Sweden)

    VanHook CJ

    2015-09-01

    Full Text Available No abstract available. Article truncated after 150 words. A 31-year-old white man presented to the emergency department complaining of fever, headache, mild confusion, and muscle aches. Approximately three days earlier he had developed non-quantified fever and diffuse muscle aches and pains. He was employed as a feedlot worker. He had visited an urgent care center one day earlier and had been advised to increase his oral fluid intake and to use non-steroidal anti-inflammatory agents as needed. Upon arrival to the emergency department he was found to have a temperature of 103.6º Fahrenheit, blood pressure of 125/72 mm Hg, respiratory rate of 40 breaths per minute, and room-air oxygen saturation of 84% by pulse oximetry. Auscultation of the chest disclosed diffuse rales. Heart sounds were rapid and regular. Abdominal exam was benign. There was no skin rash. Central nervous exam demonstrated agitation and confusion, but was otherwise non-focal. Laboratory examination revealed a white blood count of 11.7 K/uL, hemoglobin ...

  19. Central neurogenic diabetes insipidus, syndrome of inappropriate secretion of antidiuretic hormone, and cerebral salt-wasting syndrome in traumatic brain injury.

    Science.gov (United States)

    John, Cynthia A; Day, Michael W

    2012-04-01

    Central neurogenic diabetes insipidus, syndrome of inappropriate secretion of antidiuretic hormone, and cerebral salt-wasting syndrome are secondary events that affect patients with traumatic brain injury. All 3 syndromes affect both sodium and water balance; however, they have differences in pathophysiology, diagnosis, and treatment. Differentiating between hypernatremia (central neurogenic diabetes insipidus) and the 2 hyponatremia syndromes (syndrome of inappropriate secretion of antidiuretic hormone, and cerebral salt-wasting syndrome) is critical for preventing worsening neurological outcomes in patients with head injuries.

  20. Simultaneous transcatheter closure of intralobar pulmonary sequestration and patent ductus arteriosus in a patient with infantile Scimitar syndrome.

    Science.gov (United States)

    Aslan, Eyüp; Tanıdır, İbrahim Cansaran; Saygı, Murat; Onan, Sertaç Hanedan; Güzeltaş, Alper

    2015-03-01

    Scimitar syndrome is a rare disease associated with a right lung sequestration vascularised by arteries arising from the abdominal aorta and abnormal venous drainage into the inferior vena cava. The infantile form is generally presented with severe heart failure, pulmonary hypertension and respiratory distress. It may be associated with various intracardiac defects, including atrial septal defects, ventricular septal defects, patent ductus arteriosus or more complicated structural congenital heart defects. Here, we present a 2-month-old girl with Scimitar syndrome whose pulmonary arterial pressure decreased after transcatheter patent ductus arteriosus closure and embolization of the anomalous systemic arterial supply.

  1. A novel dermato-pulmonary syndrome associated with MDA-5 antibodies: report of 2 cases and review of the literature.

    Science.gov (United States)

    Chaisson, Neal F; Paik, Julie; Orbai, Ana-Maria; Casciola-Rosen, Livia; Fiorentino, David; Danoff, Sonye; Rosen, Antony

    2012-07-01

    Melanoma differentiation-associated protein 5 (MDA-5) is a novel autoantibody frequently characterized by interstitial lung disease and a distinct cutaneous phenotype with palmar papules, ulceration, and rash. Virtually all patients have underlying dermatomyositis, but many lack the characteristic clinical myopathy associated with it. In the setting of amyopathic disease, the absence of clinically available biomarkers or clear pathologic diagnosis can complicate effective prognostic and therapeutic intervention. Until recently the presence of MDA-5 antibody associated dermato-pulmonary syndrome was described only in Asian populations. We present 2 cases of MDA-5-associated dermato-pulmonary syndrome and provide a comprehensive review of available literature.

  2. [Severe hypoxemic respiratory insufficiency in a patient with hepato-pulmonary syndrome coexisting with interstitial lung disease of unknown etiology].

    Science.gov (United States)

    Gorzkowska-Pasik, Katarzyna; Wiatr, Elżbieta; Burakowska, Barbara; Nowicka, Urszula; Kober, Jarosław; Malong, Przemysław; Pasik, Piotr; Folcik, Krystyna

    2013-01-01

    The coexistence of the interstitial lung disease and respiratory failure is rarely associated with extrapulmonary pathology. In patients with liver cirrhosis, hypoxemia may develop in the course of hepato-pulmonary syndrome (HPS), but radiological pathology seen in the course of HPS is of vascular origin, and thus typically not classified as interstitial lung disease. We present a patient with severe hypoxemic respiratory insufficiency in whom hepato-pulmonary syndrome coexisted with interstitial lung disease of unknown etiology. The mechanisms of hypoxemia in the course of hepatic diseases and reasons of possible coincidence of lung and hepatic pathology are discussed.

  3. Pulmonary vasculitis.

    Science.gov (United States)

    Lally, Lindsay; Spiera, Robert F

    2015-05-01

    Pulmonary vasculitis encompasses inflammation in the pulmonary vasculature with involved vessels varying in caliber from large elastic arteries to capillaries. Small pulmonary capillaries are the vessels most commonly involved in vasculitis affecting the lung. The antineutrophil cytoplasmic antibody-associated vasculitides, which include granulomatosis with polyangiitis (formerly Wegener granulomatosis), microscopic polyangiitis, and eosinophilic granulomatosis with polyangiitis (formerly Churg-Strauss syndrome), are the small vessel vasculitides in which pulmonary vasculitis is most frequently observed and are the major focus of this review. Vasculitic involvement of the large pulmonary vessels as may occur in Behçet syndrome and Takayasu arteritis is also discussed.

  4. Hantavirus pulmonary syndrome in Santa Cruz, Bolivia: outbreak investigation and antibody prevalence study.

    Directory of Open Access Journals (Sweden)

    Joel M Montgomery

    Full Text Available We report the results of an investigation of a small outbreak of hantavirus pulmonary syndrome in 2002 in the Department of Santa Cruz, Bolivia, where the disease had not previously been reported. Two cases were initially reported. The first case was a physician infected with Laguna Negra virus during a weekend visit to his ranch. Four other persons living on the ranch were IgM antibody-positive, two of whom were symptomatic for mild hantavirus pulmonary syndrome. The second case was a migrant sugarcane worker. Although no sample remained to determine the specific infecting hantavirus, a virus 90% homologous with Río Mamoré virus was previously found in small-eared pygmy rice rats (Oligoryzomys microtis trapped in the area. An antibody prevalence study conducted in the region as part of the outbreak investigation showed 45 (9.1% of 494 persons to be IgG positive, illustrating that hantavirus infection is common in Santa Cruz Department. Precipitation in the months preceding the outbreak was particularly heavy in comparison to other years, suggesting a possible climatic or ecological influence on rodent populations and risk of hantavirus transmission to humans. Hantavirus infection appears to be common in the Santa Cruz Department, but more comprehensive surveillance and field studies are needed to fully understand the epidemiology and risk to humans.

  5. Hantavirus Pulmonary Syndrome in Santa Cruz, Bolivia: Outbreak Investigation and Antibody Prevalence Study

    Science.gov (United States)

    Montgomery, Joel M.; Blair, Patrick J.; Carroll, Darin S.; Mills, James N.; Gianella, Alberto; Iihoshi, Naomi; Briggiler, Ana M.; Felices, Vidal; Salazar, Milagros; Olson, James G.; Glabman, Raisa A.; Bausch, Daniel G.

    2012-01-01

    We report the results of an investigation of a small outbreak of hantavirus pulmonary syndrome in 2002 in the Department of Santa Cruz, Bolivia, where the disease had not previously been reported. Two cases were initially reported. The first case was a physician infected with Laguna Negra virus during a weekend visit to his ranch. Four other persons living on the ranch were IgM antibody-positive, two of whom were symptomatic for mild hantavirus pulmonary syndrome. The second case was a migrant sugarcane worker. Although no sample remained to determine the specific infecting hantavirus, a virus 90% homologous with Río Mamoré virus was previously found in small-eared pygmy rice rats (Oligoryzomys microtis) trapped in the area. An antibody prevalence study conducted in the region as part of the outbreak investigation showed 45 (9.1%) of 494 persons to be IgG positive, illustrating that hantavirus infection is common in Santa Cruz Department. Precipitation in the months preceding the outbreak was particularly heavy in comparison to other years, suggesting a possible climatic or ecological influence on rodent populations and risk of hantavirus transmission to humans. Hantavirus infection appears to be common in the Santa Cruz Department, but more comprehensive surveillance and field studies are needed to fully understand the epidemiology and risk to humans. PMID:23094116

  6. Leptospirosis-Associated Severe Pulmonary Hemorrhagic Syndrome with Lower Back Pain as an Initial Symptom

    Science.gov (United States)

    Søndergaard, Mads Madsen; Tursunovic, Amela; Thye-Rønn, Peter; Bang, Jacob Christian; Hansen, Inger Marie Jensen

    2016-01-01

    Patient: Female, 45 Final Diagnosis: Leptospirosis Symptoms: Back pain • fever • headache • Hemopthysis • nausea • sepsis Medication: — Clinical Procedure: — Specialty: Infectious Diseases Objective: Rare disease Background: Leptospirosis is a zoonosis transmitted through urine of infected animals. Symptoms range from mild influenza-like symptoms to severe pulmonary hemorrhagic syndrome (SPHS); the latter are often fatal. The serogroup distribution in Denmark has changed from 1988 to 2012, with Icterohaemorrhagiae and Sejroe now being predominant. Case report: A 45-year-old Danish woman living in an area endemic for Hanta virus, without prior medical history, was admitted because of lower back pain radiating to the left hip, fever, headache, nausea, and malaise. Two weeks before admission she had been bitten by a mouse or a rat. Blood tests revealed raised white cells and CRP, electrolyte imbalances, raised creatinine, low thrombocytes, and a slightly decreased clotting factor (II, VII, and X). Treatment with broad-spectrum intravenous antibiotics and supporting therapy was initiated very quickly. Eight hours after admission she died from respiratory failure where severe hemoptysis was observed. Leptospiral DNA was later detected in a urine sample. Conclusions: This case represents leptospirosis with severe pulmonary hemorrhagic syndrome. In spite of immediate treatment with broad-spectrum antibiotics, the patient died a few hours after hospital admission. PMID:27881835

  7. No additive effects of inhaled iloprost and prone positioning on pulmonary hypertension and oxygenation in acute respiratory distress syndrome.

    Science.gov (United States)

    Senturk, E; Cakar, N; Ozcan, P E; Basel, A; Sengul, T; Telci, L; Esen, F; Nahum, A; Strang, C M; Winterhalter, M

    2012-09-01

    In acute respiratory distress syndrome (ARDS), pulmonary hypertension is associated with a poor prognosis. Prone position is effective to improve oxygenation whereas inhaled iloprost can treat pulmonary hypertension. However, combination of these interventions has not been examined before. The hypothesis was that this combination had additive effects on oxygenation and pulmonary hemodynamics as compared with each intervention alone. In a prospective, randomized cross-over study, ten pigs were anesthetized, intubated and ventilated with volume controlled ventilation. Carotid, jugular venous and pulmonary artery catheters were inserted. ARDS was induced with oleic acid (0.20 mL/kg). Measurements were repeated in randomized different sequences of prone or supine positions with or without iloprost inhalation (220 ng/kg/min) (four combinations). Systemic and pulmonary arterial pressures; arterial and mixed venous blood gases; and Qs/Qt and the resistances were recorded. Iloprost decreased pulmonary artery pressures (for MPAP: P=0.034) in both supine (37±10 vs. 31±8 mmHg; Piloprost application (331±112 vs. 167±117 mmHg, Piloprost reduced pulmonary arterial pressures, and prone positioning improved oxygenation; there are no additive effects of the combination of both interventions on both parameters. To treat both pulmonary hypertension and hypoxemia, application of iloprost in prone position is suggested.

  8. Double-positive Goodpasture′s syndrome with concomitant active pulmonary tuberculosis

    Directory of Open Access Journals (Sweden)

    Waqar Kashif

    2013-01-01

    Full Text Available Anti-glomerular basement membrane (anti-GBM disease usually presents as rapidly progressive glomerulonephritis, and, when accompanied with pulmonary hemorrhage, it is called Goodpasture′s syndrome. Anti-neutrophilic cytoplasmic antibodies (ANCA may co-exist with anti-GBM antibodies. In most of these "double positive" cases, ANCA is specific for myeloperoxidase (p-ANCA. We report a rare case of a critically ill patient c-ANCA-associated double-positive Goodpasture′s syndrome with concomitant tuberculosis that was successfully treated with immunosuppression, plasmapheresis and anti-tuberculous therapy (ATT. A 32-year-old gentleman with a 15 pack-year smoking history presented with massive hemoptysis, respiratory failure and oliguria. Laboratory investigation revealed anemia, elevated creatinine and active urinary sediment. Chest X-ray revealed bilateral pulmonary infiltrates. Broad-spectrum antibiotics and intravenous corticosteroids were started. Bronchoscopy showed alveolar hemorrhage and smears from bronchial lavage from both lungs were positive for acid fast bacillus (AFB. Vasculitis work-up revealed high titers of c-ANCA and anti-GBM antibodies. Kidney biopsy revealed crescents in >50% glomeruli on light microscopy. Immunofluorescence showed linear deposition of IgG and C3. The patient received pulse methylprednisone for three days followed by oral prednisone and ATT. In addition, he also underwent nine sessions of plasmapheresis. Oral Cyclophosphamide was added on Day 10. The patient showed remarkable recovery as his lung fields cleared and his kidney function got stabilized. Cyclophosphamide was continued for three months and then switched to azathioprine. At six months, the creatinine is 1.2 mg/dL, with minimal proteinuria and a normal chest X-ray. To the best of our knowledge, this is the only reported case of double-positive Goodpasture′s syndrome (c-ANCA and anti GBM with active tuberculosis treated successfully.

  9. [Goodpasture syndrome and (idiopathic) immune complex glomerulonephritis with pulmonary hemorrhage: 2 different syndromes?].

    Science.gov (United States)

    Kuhn, M; Gartmann, J; Grob, P J; Widder, W; Hartmann, G

    1984-06-23

    While Goodpasture syndrome was previously defined purely clinically by the combination of pneumorrhagia and glomerulonephritis, today the following immunologic criteria must also be satisfied: evidence, provided by immunofluorescent investigation of the kidneys and lungs, of antibasement membrane antibodies in the serum and linear deposits of immunoglobulins, due to direct apposition of antibasement membrane antibodies. Cases where the lesions are caused by immune complexes should no longer be designated as Goodpasture syndrome. In the light of one of our own cases of immune complex glomerulonephritis with pneumorrhagia, the question is raised whether this subdivision by means of immunologic investigations is meaningful for the clinician.

  10. Childhood Lung Function Predicts Adult Chronic Obstructive Pulmonary Disease and Asthma-Chronic Obstructive Pulmonary Disease Overlap Syndrome.

    Science.gov (United States)

    Bui, Dinh S; Burgess, John A; Lowe, Adrian J; Perret, Jennifer L; Lodge, Caroline J; Bui, Minh; Morrison, Stephen; Thompson, Bruce R; Thomas, Paul S; Giles, Graham G; Garcia-Aymerich, Judith; Jarvis, Debbie; Abramson, Michael J; Walters, E Haydn; Matheson, Melanie C; Dharmage, Shyamali C

    2017-07-01

    The burden of chronic obstructive pulmonary disease (COPD) is increasing, yet there are limited data on early life risk factors. To investigate the role of childhood lung function in adult COPD phenotypes. Prebronchodilator spirometry was performed for a cohort of 7-year-old Tasmanian children (n = 8,583) in 1968 who were resurveyed at 45 years, and a selected subsample (n = 1,389) underwent prebronchodilator and post-bronchodilator spirometry. For this analysis, COPD was spirometrically defined as a post-bronchodilator FEV1/FVC less than the lower limit of normal. Asthma-COPD overlap syndrome (ACOS) was defined as the coexistence of both COPD and current asthma. Associations between childhood lung function and asthma/COPD/ACOS were examined using multinomial regression. At 45 years, 959 participants had neither current asthma nor COPD (unaffected), 269 had current asthma alone, 59 had COPD alone, and 68 had ACOS. The reweighted prevalence of asthma alone was 13.5%, COPD alone 4.1%, and ACOS 2.9%. The lowest quartile of FEV1 at 7 years was associated with ACOS (odds ratio, 2.93; 95% confidence interval, 1.32-6.52), but not COPD or asthma alone. The lowest quartile of FEV1/FVC ratio at 7 years was associated with ACOS (odds ratio, 16.3; 95% confidence interval, 4.7-55.9) and COPD (odds ratio, 5.76; 95% confidence interval, 1.9-17.4), but not asthma alone. Being in the lowest quartile for lung function at age 7 may have long-term consequences for the development of COPD and ACOS by middle age. Screening of lung function in school age children may identify a high-risk group that could be targeted for intervention. Further research is needed to understand possible modifiers of these associations and develop interventions for children with impaired lung function.

  11. Unilateral pulmonary artery stenosis and late-onset cataract in an adult: a case of suspected congenital rubella syndrome

    Institute of Scientific and Technical Information of China (English)

    LIU Yang; GUO Jun; ZHAO Rui-fu; WANG Lin

    2012-01-01

    Congenital rubella syndrome (CRS) is characterized by the triad of deafness,cataract and cardiovascular malformations.1 The great majority of the cases in the literature have been usually diagnosed in infancy and childhood because of various defects at birth.However,we report a rare case of suspected CRS in an adult with unilateral pulmonary artery stenosis and late-onset cataract.

  12. Pulmonary Specific Ancillary Treatment for Pediatric Acute Respiratory Distress Syndrome : Proceedings From the Pediatric Acute Lung Injury Consensus Conference

    NARCIS (Netherlands)

    Tamburro, Robert F.; Kneyber, Martin C. J.

    Objective: To provide an overview of the current literature on pulmonary-specific therapeutic approaches to pediatric acute respiratory distress syndrome to determine recommendations for clinical practice and/or future research. Data Sources: PubMed, EMBASE, CINAHL, SCOPUS, and the Cochrane Library

  13. Postobstructive Pulmonary Edema following Tonsillectomy/Adenoidectomy in a 2-Year-Old with Poland-Moebius Syndrome

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    Tanisha Powell

    2016-01-01

    Full Text Available A 2-year-old male with Poland-Moebius syndrome was transferred from a local hospital to the Pediatric ICU at Children’s Hospital of Georgia for suspected postobstructive pulmonary edema (POPE after tonsillectomy/adenoidectomy (T&A. The patient’s respiratory status ultimately declined and he developed respiratory failure. Imaging suggested pulmonary edema as well as a left-sided pneumonia. Echocardiogram showed pulmonary hypertension and airway exam via direct fiberoptic bronchoscopy revealed tracheomalacia and bronchomalacia. He developed acute respiratory distress syndrome (ARDS and remained intubated for ten days. This case highlights the association between congenital upper body abnormalities with cranial nerve dysfunction and the development of POPE with delayed resolution of symptoms. Patients with upper body abnormalities as above are at great risk of postoperative complications and should therefore be managed in a tertiary-care facility.

  14. Postobstructive Pulmonary Edema following Tonsillectomy/Adenoidectomy in a 2-Year-Old with Poland-Moebius Syndrome

    Science.gov (United States)

    Powell, Tanisha; Sharma, Nirupma; McKie, Kathleen T.

    2016-01-01

    A 2-year-old male with Poland-Moebius syndrome was transferred from a local hospital to the Pediatric ICU at Children's Hospital of Georgia for suspected postobstructive pulmonary edema (POPE) after tonsillectomy/adenoidectomy (T&A). The patient's respiratory status ultimately declined and he developed respiratory failure. Imaging suggested pulmonary edema as well as a left-sided pneumonia. Echocardiogram showed pulmonary hypertension and airway exam via direct fiberoptic bronchoscopy revealed tracheomalacia and bronchomalacia. He developed acute respiratory distress syndrome (ARDS) and remained intubated for ten days. This case highlights the association between congenital upper body abnormalities with cranial nerve dysfunction and the development of POPE with delayed resolution of symptoms. Patients with upper body abnormalities as above are at great risk of postoperative complications and should therefore be managed in a tertiary-care facility. PMID:26942029

  15. Platelet abnormalities in adults with severe pulmonary arterial hypertension related to congenital heart defects (Eisenmenger syndrome).

    Science.gov (United States)

    Remková, Anna; Šimková, Iveta; Valkovičová, Tatiana; Kaldarárová, Monika

    2016-12-01

    Patients with severe pulmonary arterial hypertension suffer from life-threatening thrombotic and bleeding complications. The aim of this study was to compare selected platelet, endothelial, and coagulation parameters in healthy volunteers and patients with severe pulmonary arterial hypertension because of congenital heart defects. The study included healthy volunteers (n = 50) and patients with cyanotic congenital heart defects classified as Eisenmenger syndrome (n = 41). We investigated platelet count, mean platelet volume, and platelet aggregation - spontaneous and induced by various concentrations of five agonists. Von Willebrand factor (vWF), fibrinogen, factor VIII and XII, plasminogen activator inhibitor, antithrombin, D-dimer, and antiphospholipid antibodies were also investigated. We found a decreased platelet count [190 (147-225) vs. 248 (205-295) 10 l, P < 0.0001], higher mean platelet volume [10.9 (10.1-12.0) vs. 10.2 (9.4-10.4) fl, P < 0.0001], and significantly decreased platelet aggregation (induced by five agonists, in various concentrations) in patients with Eisenmenger syndrome compared with controls. These changes were accompanied by an increase of plasma vWF antigen [141.6 (108.9-179.1) vs. 117.4 (9.2-140.7) IU/dl, P = 0.022] and serum anti-β2-glycoprotein [2.07 (0.71-3.41) vs. 0.47 (0.18-0.99) U/ml, P < 0.0001]. Eisenmenger syndrome is accompanied by platelet abnormalities. Thrombocytopenia with increased platelet size is probably due to a higher platelet turnover associated with platelet activation. Impaired platelet aggregation can reflect specific platelet behaviour in patients with Eisenmenger syndrome. These changes can be related both to bleeding and to thrombotic events. A higher vWF antigen may be a consequence of endothelial damage in Eisenmenger syndrome, but the cause for an increase of anti-β2-glycoprotein is unknown.

  16. Is obstructive sleep apnea syndrome a risk factor for pulmonary thromboembolism?

    Institute of Scientific and Technical Information of China (English)

    Kezban Ozmen Suner; Ali Nihat Annakkaya; Umran Toru; Talha Dumlu; Ege Gulec Balbay; Peri Arbak; Leyla Yilmaz Aydin; Hasan Suner

    2012-01-01

    Background In many studies,obstructive sleep apnea (OSA) has been shown to be an independent risk factor for cardiovascular disease.Conversely,there are few reports establishing possible relation between OSA and venous thromboembolism (VTE).In this study,the aim is to evaluate OSA via polysomnography in patients with pulmonary embolism and drawing the attention of clinicians to the presence of obstructive sleep apnea syndrome (OSAS) may be a risk factor for pulmonary embolism.Methods Fifty consecutive patients who were diagnosed with pulmonary embolism (PE) were evaluated prospectively for OSAS.Polysomnographic examination was conducted on 30 volunteer patients.The frequency of OSAS in PE was determined and PE cases were compared to each other after being divided into two groups based on the presence of a major risk factor.Results The study consisted of a total of 30 patients (14 females and 16 males).In 56.7% of the patients (17/30),OSAS was determined.The percent of cases with moderate and severe OSAS (apnea hipoapnea index>15) was 26.7%(8/30).Patients who had pulmonary thromboembolism (PTE) without any known major VTE risk (n=20),were compared to patients with VTE risk factors (n=10),and significantly higher rates of OSAS were seen (70% and 30% respectively;P=-0.045).The mean age of the group with major PE risk factors was lower than the group without major PE risk factors (52 years old and 66 years old,respectively; P=0.015),however,weight was greater in the group with major PE risk factors (88 kg and 81 kg,respectively; P=0.025).By multivariate Logistic regression analysis,in the group without any visible major risk factors,the only independent risk factor for PE was OSAS (P=0.049).Conclusions In patients with PTE,OSA rates were much higher than in the general population.Moreover,the rate for patients with clinically significant moderate and severe OSA was quite high.PTE patients with OSA symptoms (not syndromes) and without known major risk factor

  17. Unilateral right pulmonary artery agenesis and congenital cystic adenomatoid malformation of the right lung with Ortner′s syndrome

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    Jane Jackie David

    2016-01-01

    Full Text Available We report a 2.5-year-old girl who presented with hoarseness of voice since 3 months of age and failure to thrive. Chest X-ray showed cardiomegaly with a deviation of the trachea and mediastinum to the right side. Two-dimensional echocardiography showed decreased flow across the right pulmonary artery, a small atrial septal defect (ASD with a right-to-left shunt, and a dilated right atrium and right ventricle with severe tricuspid regurgitation suggestive of severe pulmonary hypertension. A silent large patent ductus arteriosus was also seen. Multiple detector computerized tomography aortogram confirmed the findings of absent right pulmonary artery and hypoplastic right lung with small cystic lesions suggestive of congenital cystic adenomatoid malformation in the right lower lobe. Hoarseness of voice was due to the left vocal cord palsy probably secondary to severe pulmonary hypertension (Ortner′s syndrome.

  18. Unilateral right pulmonary artery agenesis and congenital cystic adenomatoid malformation of the right lung with Ortner's syndrome.

    Science.gov (United States)

    David, Jane Jackie; Mohanlal, Smilu; Sankhe, Punam; Ghildiyal, Radha

    2016-01-01

    We report a 2.5-year-old girl who presented with hoarseness of voice since 3 months of age and failure to thrive. Chest X-ray showed cardiomegaly with a deviation of the trachea and mediastinum to the right side. Two-dimensional echocardiography showed decreased flow across the right pulmonary artery, a small atrial septal defect (ASD) with a right-to-left shunt, and a dilated right atrium and right ventricle with severe tricuspid regurgitation suggestive of severe pulmonary hypertension. A silent large patent ductus arteriosus was also seen. Multiple detector computerized tomography aortogram confirmed the findings of absent right pulmonary artery and hypoplastic right lung with small cystic lesions suggestive of congenital cystic adenomatoid malformation in the right lower lobe. Hoarseness of voice was due to the left vocal cord palsy probably secondary to severe pulmonary hypertension (Ortner's syndrome).

  19. Dental treatment of a child with congenital central hypoventilation syndrome.

    Science.gov (United States)

    Boka, V; Lefkelidou, A; Athanasiadou, E

    2016-06-01

    Congenital Central Hypoventilation Syndrome (CCHS) is a rare condition combining respiratory hypoventilation with symptoms of autonomic dysregulation. Management of patients requires both medical and dental expertise to achieve a successful outcome. The aim of this paper is to present the dental management of a child diagnosed with CCHS, without pharmacological measures and in cooperation with medical expertise. A 7-year-old girl was referred to a private dental practice with the chief complaints being pain and poor aesthetics. The child had been diagnosed with CCHS since infancy and had undergone several courses of medication. Although the patient was uncooperative, the paediatric pulmonologist advised against general anaesthesia. As a result, she was treated in the operating theater (OT) without sedatives, being monitored throughout the entire procedure. A total of eight primary teeth needed dental treatment (3 were restored, 4 were extracted and 1 was restored with a preformed metal crown). A mandibular lingual holding arch was placed, two weeks later. The patient was seen after 6 months and 1 year. Her oral hygiene had improved significantly and her mother reported that the child ate better, brushed her teeth on a daily basis and was careful with dietary habits. The collaboration between medical experts and a paediatric dentist was of crucial importance. The use of basic behaviour management techniques in conjunction with monitoring the patient's vital signs led to a successful outcome and an improvement in the behaviour of the patient.

  20. Pulmonary hypertension and metabolic syndrome: Possible connection, PPARγ and Caveolin-1.

    Science.gov (United States)

    Mathew, Rajamma

    2014-08-26

    A number of disparate diseases can lead to pulmonary hypertension (PH), a serious disorder with a high morbidity and mortality rate. Recent studies suggest that the associated metabolic dysregulation may be an important factor adversely impacting the prognosis of PH. Furthermore, metabolic syndrome is associated with vascular diseases including PH. Inflammation plays a significant role both in PH and metabolic syndrome. Adipose tissue modulates lipid and glucose metabolism, and also produces pro- and anti-inflammatory adipokines that modulate vascular function and angiogenesis, suggesting a close functional relationship between the adipose tissue and the vasculature. Both caveolin-1, a cell membrane scaffolding protein and peroxisome proliferator-activated receptor (PPAR) γ, a ligand-activated transcription factor are abundantly expressed in the endothelial cells and adipocytes. Both caveolin-1 and PPARγ modulate proliferative and anti-apoptotic pathways, cell migration, inflammation, vascular homeostasis, and participate in lipid transport, triacylglyceride synthesis and glucose metabolism. Caveolin-1 and PPARγ regulate the production of adipokines and in turn are modulated by them. This review article summarizes the roles and inter-relationships of caveolin-1, PPARγ and adipokines in PH and metabolic syndrome.

  1. Cyanosis in atrial septal defect without pulmonary hypertension: a case of platypnea-orthodeoxya syndrome.

    Science.gov (United States)

    Di Bella, Isidoro; Pasquino, Stefano; Da Col, Uberto; Ragni, Temistocle

    2005-02-01

    Cyanosis in atrial septal defect typically occurs when pulmonary hypertension develops. Platypnea-orthodeoxya is an uncommon syndrome, still under debate, characterized by breathlessness and arterial oxygen desaturation exacerbated in the upright position. An interatrial communication is a common finding in this syndrome, but the absence of a right to left pressure gradient complicates the physiopathological picture. To explain the right to left shunt, it is generally advocated a concomitant condition that alternates the sterical relationship between inferior vena cava orifice and the atrial septal defect. A case of a 58-year-old male with platypnea-orthodeoxya syndrome related to a fenestrated redundant interatrial septum without any additional pathologic condition is reported. Possibly, this isolated anatomical abnormality could lead to a right to left shunt in the absence of other coexisting predisposing factors. It is reasonable to hypothesize the septum secundum bulging like a 'spinnaker' into the right atrium, so that it deviates the inferior vena cava venous blood towards the left atrium. Echocardiographic evaluation is mandatory to achieve a correct diagnosis and to decide the therapeutic strategy.

  2. Overlap Syndrome in Respiratory Medicine: Asthma and Chronic Obstructive Pulmonary Disease

    Directory of Open Access Journals (Sweden)

    Alexandru Corlateanu

    2014-02-01

    Full Text Available Asthma and chronic obstructive pulmonary disease (COPD are highly prevalent chronic diseases in the general population. Both are characterized by similar mechanisms: airway inflammation, airway obstruction, and airway hyperresponsiveness. However, the distinction between the two obstructive diseases is not always clear. Multiple epidemiological studies demonstrate that in elderly people with obstructive airway disease, as many as half or more may have overlapping diagnoses of asthma and COPD. A COPD-Asthma overlap syndrome is defined as an airflow obstruction that is not completely reversible, accompanied by symptoms and signs of increased obstruction reversibility. For the clinical identification of overlap syndrome COPD-Asthma Spanish guidelines proposed six diagnostic criteria. The major criteria include very positive bronchodilator test [increase in forced expiratory volume in one second (FEV1 ≥15% and ≥400 ml], eosinophilia in sputum, and personal history of asthma. The minor criteria include high total IgE, personal history of atopy and positive bronchodilator test (increase in FEV1 ≥12% and ≥200 ml on two or more occasions. The overlap syndrome COPD-Asthma is associated with enhanced response to inhaled corticosteroids due to the predominance of eosinophilic bronchial inflammation.The future clinical studies and multicenter clinical trials should lead to the investigation of disease mechanisms and simultaneous development of the novel treatment.

  3. Pulmonary hypertension and metabolic syndrome: Possible connection, PPARγ and Caveolin-1

    Institute of Scientific and Technical Information of China (English)

    Rajamma; Mathew

    2014-01-01

    A number of disparate diseases can lead to pulmonary hypertension(PH), a serious disorder with a high morbidity and mortality rate. Recent studies suggest that the associated metabolic dysregulation may be an important factor adversely impacting the prognosis of PH. Furthermore, metabolic syndrome is associated with vascular diseases including PH. Inflammation plays a significant role both in PH and metabolic syndrome. Adipose tissue modulates lipid and glucose metabolism, and also produces pro-and anti-inflammatory adipokines that modulate vascular function and angiogenesis, suggesting a close functional relationship between the adipose tissue and the vasculature. Both caveolin-1, a cell membrane scaffolding protein and peroxisome proliferator-activated receptor(PPAR) γ, a ligandactivated transcription factor are abundantly expressed in the endothelial cells and adipocytes. Both caveolin-1 and PPARγ modulate proliferative and anti-apoptotic pathways, cell migration, inflammation, vascular homeostasis, and participate in lipid transport, triacylglyceride synthesis and glucose metabolism. Caveolin-1 and PPARγ regulate the production of adipokines and in turn are modulated by them. This review article summarizes the roles and inter-relationships of caveolin-1,PPARγ and adipokines in PH and metabolic syndrome.

  4. Pulmonary cryptococcosis in rheumatoid arthritis (RA) patients: Comparison of imaging characteristics among RA, acquired immunodeficiency syndrome, and immunocompetent patients

    Energy Technology Data Exchange (ETDEWEB)

    Yanagawa, Noriyo, E-mail: noriyo_yana@ybb.ne.jp [Departments of Radiology, Tokyo Metropolitan Cancer and Infectious Diseases Center Komagome Hospital, 3-8-22, Honkomagome, Bunkyo-ku, Tokyo 113-8677 (Japan); Sakai, Fumikazu [Department of Diagnostic Radiology, Saitama Medical University International Medical Center, 1397-1 Yamane, Hidaka-shi, Saitama 350-1298 (Japan); Takemura, Tamiko [Department of Pathology, Japanese Red Cross Medical Center, 4-1-22 Hiroo, Shibuya-ku, Tokyo 150-8935 (Japan); Ishikawa, Satoru [Department of Respiratory Medicine, National Hospital Organization Chiba-East-Hospital, 673 Nitona-cho, Chuo-ku, Chiba-shi, Chiba 260-8712 (Japan); Takaki, Yasunobu [Departments of Radiology, Tokyo Metropolitan Cancer and Infectious Diseases Center Komagome Hospital, 3-8-22, Honkomagome, Bunkyo-ku, Tokyo 113-8677 (Japan); Hishima, Tsunekazu [Department of Pathology, Tokyo Metropolitan Cancer and Infectious Diseases Center Komagome Hospital, 3-8-22, Honkomagome, Bunkyo-ku, Tokyo 113-8677 (Japan); Kamata, Noriko [Departments of Radiology, Tokyo Metropolitan Cancer and Infectious Diseases Center Komagome Hospital, 3-8-22, Honkomagome, Bunkyo-ku, Tokyo 113-8677 (Japan)

    2013-11-01

    Purpose: The imaging characteristics of cryptococcosis in rheumatoid arthritis (RA) patients were analyzed by comparing them with those of acquired immunodeficiency syndrome (AIDS) and immunocompetent patients, and the imaging findings were correlated with pathological findings. Methods: Two radiologists retrospectively compared the computed tomographic (CT) findings of 35 episodes of pulmonary cryptococcosis in 31 patients with 3 kinds of underlying states (10 RA, 12 AIDS, 13 immunocompetent), focusing on the nature, number, and distribution of lesions. The pathological findings of 18 patients (8 RA, 2 AIDS, 8 immunocompetent) were analyzed by two pathologists, and then correlated with imaging findings. Results: The frequencies of consolidation and ground glass attenuation (GGA) were significantly higher, and the frequency of peripheral distribution was significantly lower in the RA group than in the immunocompetent group. Peripheral distribution was less common and generalized distribution was more frequent in the RA group than in the AIDS group. The pathological findings of the AIDS and immunocompetent groups reflected their immune status: There was lack of a granuloma reaction in the AIDS group, and a complete granuloma reaction in the immunocompetent group, while the findings of the RA group varied, including a complete granuloma reaction, a loose granuloma reaction and a hyper-immune reaction. Cases with the last two pathologic findings were symptomatic and showed generalized or central distribution on CT. Conclusion: Cryptococcosis in the RA group showed characteristic radiological and pathological findings compared with the other 2 groups.

  5. Central versus peripheral cardiovascular risk in metabolic syndrome

    Directory of Open Access Journals (Sweden)

    Heather eEdgell

    2012-02-01

    Full Text Available Individuals with metabolic syndrome (MetS; i.e. 3 of 5 of the following risk factors (RFs: elevated blood pressure, waist circumference, triglycerides, blood glucose or reduced HDL are thought to be prone to serious cardiovascular disease and there is debate as to whether the disease begins in the peripheral vasculature or centrally. This study investigates hemodynamics, cardiac function/morphology, as well as mechanical properties of the central (heart, carotid artery and peripheral (total peripheral resistance, forearm vascular bed vasculature in individuals without (1-2 RFs; n=28, or with (≥3 RFs; n=46 MetS. After adjustments for statin and blood pressure medication use, those with MetS had lower mitral valve E/A ratios (<3 RFs: 1.24±0.07; ≥3 RFs: 1.01±0.04; P=0.025, and higher total peripheral resistance index (<3 RFs: 48±2 mmHg/L/min/m2; ≥3 RFs: 53±2 mmHg/L/min/m2; P=0.04. There were no differences in heart size, carotid artery measurements, cardiovagal baroreflex sensitivity, pulse wave velocity, stroke volume index, or cardiac output index due to MetS after adjustments for statin and blood pressure medication use. In a separate analysis, the use of statins was associated with increased inertia in the brachial vascular bed, increased HbA1c and decreased LDL cholesterol. The independent use of anti-hypertensive medication was associated with decreased predicted VO2max, triglycerides, diastolic blood pressure, interventricular septum thickness, calculated left ventricle mass, left ventricle posterior wall thickness, and left ventricle pre-ejection period, but increased carotid stiffness, HDL cholesterol, and heart rate. These data imply that both a central cardiac effect and a peripheral effect of vascular resistance are expressed in MetS. These data also indicate that variance in between-group responses due to pharmacological treatments are important factors to consider in studying cardiovascular changes in these individuals.

  6. Prenatal diagnosis of Pallister-Killian syndrome associated with pulmonary stenosis and right ventricular dilatation.

    Science.gov (United States)

    Park, In Yang; Shin, Jong Chul; Kwon, Ji Young; Koo, Bo Kyung; Kim, Myungshin; Lim, Jihyang; Kim, Yonggoo; Han, Kyungja

    2009-08-01

    Pallister-Killian syndrome (PKS) is a rare disorder characterized cytogenetically by tetrasomy 12p for isochromosome of the short arm of chromosome 12. PKS is diagnosed by prenatal genetic analysis through chorionic villous sampling, genetic amniocentesis, and cordocentesis, or by chromosomal analysis of skin fibroblasts, but is not usually detected by chromosomal analysis of peripheral blood cells. Herein, we report a case of a gravida at 23 weeks gestation with pulmonary stenosis and right ventricular dilation of the heart which were detected by sonography. Fluorescence in situ hybridization and a multicolor banding technique were performed to verify the diagnosis as 47,XX, +mar.ish i(12)(p10)(TEL++)[16]/46,XX[4], and an autopsy confirmed the cardiac anomalies detected on antenatal sonography.

  7. Effectiveness of recorded messages to communicate the risk of acquiring hantavirus pulmonary syndrome.

    Science.gov (United States)

    Predy, G; Carney, B; Edwards, J

    1997-01-01

    To determine the effectiveness of a recorded information line in communicating health risk during the emergence of a new disease, hantavirus pulmonary syndrome (HPS), in the Edmonton area and to study the accuracy of recall of information about hantavirus among the general public. A random telephone survey of residents five months after a death from HPS had occurred. The number of residents who received their information from the recorded line was quite low (approximately 2%), and more people remembered receiving their information through the news media, particularly television (74%) and newspaper (57%). An information line by itself will not communicate risk effectively during an outbreak or other emergent health situation. However, an information line used in conjunction with news media proved effective in providing ongoing, accurate information and allaying public fears in a low-risk situation.

  8. Síndrome pulmonar e cardiovascular por hantavírus Hantavirus pulmonary and cardiovascular syndrome

    Directory of Open Access Journals (Sweden)

    Mariangela Pimentel Pincelli

    2003-10-01

    Full Text Available A síndrome pulmonar e cardiovascular por hantavírus é uma doença de conhecimento relativamente recente e freqüentemente fatal, apresentando-se como síndrome do desconforto respiratório agudo. No Brasil, desde o primeiro surto, relatado em novembro/dezembro de 1993, em Juquitiba, 226 casos já foram registrados pela Fundação Nacional da Saúde. A doença afeta indivíduos previamente hígidos, apresentando-se com pródromo febril e sintomas semelhantes aos de um resfriado comum, podendo rapidamente evoluir para edema pulmonar, insuficiência respiratória aguda e choque. A hemoconcentração e a plaquetopenia são comuns da síndrome pulmonar e cardiovascular por hantavírus, e o quadro radiológico típico é de um infiltrado intersticial bilateral difuso, que progride rapidamente para consolidações alveolares, paralelamente à piora do quadro clínico. A mortalidade inicial era em torno de 75% e declinou para aproximadamente 35%, nos últimos anos. Os pacientes que sobrevivem geralmente recuperam-se completamente, cerca de uma semana após o estabelecimento do quadro respiratório. O agente causal, não reconhecido até há pouco, foi identificado como um hantavírus, cujo reservatório natural são animais roedores da família Muridae, subfamília Sigmodontinae. O tratamento específico antiviral ainda não é bem estabelecido, estando em estudo a eficácia de ribavirina. Cuidados de terapia intensiva como ventilação mecânica e monitoramento hemodinâmico invasivo são necessários nas formas mais graves da doença. Essas medidas, se instituídas precocemente, podem melhorar o prognóstico e a sobrevida dos pacientes com síndrome pulmonar e cardiovascular por hantavírus.Hantavirus pulmonary and cardiovascular syndrome is a recently identified and often fatal disease, which presents as acute respiratory distress syndrome (ARDS. Since the first outbreak, in Nov/Dec 1993, in Juquitiba, Brazil, 226 cases have been registered by

  9. Endomyocardial fibrosis as a cause of Budd-Chiari syndrome and fatal pulmonary embolism.

    Science.gov (United States)

    Bestetti, Reinaldo B; Silva, Carolina M P D C; Ardito, Sabrina Q; Theodoropoulos, Tatiana A D; Cury, Patrícia M; Corbucci, Hélio A R

    2010-01-01

    A 24-year-old-man had right-sided heart failure of 3 months' duration. A Doppler echocardiogram revealed atrium and right ventricular enlargement, obliteration of the right ventricular apex, and a mass with an echolucent center measuring 20x21 mm in the right ventricular outlet. He died of pulmonary embolism. At autopsy, a huge organized thrombus obliterating the right ventricular apex passing through the tricuspid valve to the right atrium and then extending to the inferior vena cava up to the suprahepatic veins was seen. Histologically, an intense fibrotic thickening of the endomyocardium extending into the myocardium was observed. Cardiac thrombosis associated with endomyocardial fibrosis should be added to the list of causes of Budd-Chiari syndrome.

  10. Occult pulmonary mucosa-associated lymphoid tissue lymphoma presenting as catastrophic antiphospholipid antibody syndrome.

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    Regunath, Hariharan; Shortridge, James; Raza, Shahzad; Nistala, Puja; Huffman, Brandon M; Wang, Michael X; Xiang, Dong

    2013-11-01

    Catastrophic antiphospholipid antibody syndrome (CAPS) is characterized by fulminant thrombosis of the arterial and venous beds of multiple organ systems over a relatively short period of time and with a high mortality rate. Mucosa-associated lymphoid tissue (MALT) lymphoma of the lung has never been reported as a causative or precipitating factor for CAPS in the CAPS registry database. The present study describes a rare case of pulmonary MALT lymphoma of the lung that presented as CAPS. A 19-year-old Hispanic female presented with shortness of breath and abdominal pain. Computed tomography (CT) scans of the chest and abdomen revealed multiple portal vein thromboses and bilateral pulmonary nodules. Within one week of presentation, the patient developed a straight sinus thrombosis and upper extremity deep vein thrombosis, which led to shortness of breath. A biopsy of the lung nodule revealed MALT lymphoma. The present case illustrates a rarely reported pulmonary MALT lymphoma presenting as CAPS in a young female. The patient was successfully treated with 90 mg/m(2) bendamustine on days one and two and rituximab 375 mg/m(2) on day one of each 28-day cycle. Complete remission of the lung nodules was observed following three cycles of treatment, as visualized by positron emission tomography (PET)/CT scan. Fondaparinux was identified as a feasible anticoagulation drug of choice for this case. At seven months post-treatment, the patient continues to be stable with no further evidence of thrombosis and is currently undergoing rituximab maintenance therapy every six months for two years. A repeat lupus anticoagulant antibody assay turned and remained negative during the clinical follow-up period. A prompt diagnosis and early aggressive treatment is potentially curative and may dramatically decrease the mortality risk. Future studies should explore the role of rituximab in the management of CAPS-associated B-cell lymphoid malignancies.

  11. Pulmonary hypertension in adults with congenital heart disease and Eisenmenger syndrome: current advanced management strategies.

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    D'Alto, Michele; Diller, Gerhard-Paul

    2014-09-01

    The presence of pulmonary arterial hypertension (PAH) increases morbidity and reduces survival in patients with congenital heart disease (CHD). PAH-CHD is a heterogeneous condition, depending on the type of the underlying defect and previous repair strategies. There is growing evidence of the benefits of PAH-specific therapy in the PAH-CHD population, but despite recent advances mortality rates remain relatively high. In the last years, an increasing focus has been placed on patients with PAH-CHD and net left-to-right shunt. Currently, there are limited data to guide the management of these patients and uncertainty on the cut-off values for eventual defect closure. Pregnancy conveys significant risks in PAH-CHD patients: appropriate counselling and care, including psychological support and a multidisciplinary team, should be part of the routine management of women with PAH-CHD of reproductive age. Some subgroups, such as patients with Down's syndrome, Fontan circulation and 'segmental' pulmonary hypertension, present particular challenges in terms of management and therapy. The current review focuses on contemporary treatment strategies in PAH-CHD patients with particular emphasis on challenging patient groups and conditions.

  12. Laguna Negra Virus Infection Causes Hantavirus Pulmonary Syndrome in Turkish Hamsters (Mesocricetus brandti).

    Science.gov (United States)

    Hardcastle, K; Scott, D; Safronetz, D; Brining, D L; Ebihara, H; Feldmann, H; LaCasse, R A

    2016-01-01

    Laguna Negra virus (LNV) is a New World hantavirus associated with severe and often fatal cardiopulmonary disease in humans, known as hantavirus pulmonary syndrome (HPS). Five hamster species were evaluated for clinical and serologic responses following inoculation with 4 hantaviruses. Of the 5 hamster species, only Turkish hamsters infected with LNV demonstrated signs consistent with HPS and a fatality rate of 43%. Clinical manifestations in infected animals that succumbed to disease included severe and rapid onset of dyspnea, weight loss, leukopenia, and reduced thrombocyte numbers as compared to uninfected controls. Histopathologic examination revealed lung lesions that resemble the hallmarks of HPS in humans, including interstitial pneumonia and pulmonary edema, as well as generalized infection of endothelial cells and macrophages in major organ tissues. Histologic lesions corresponded to the presence of viral antigen in affected tissues. To date, there have been no small animal models available to study LNV infection and pathogenesis. The Turkish hamster model of LNV infection may be important in the study of LNV-induced HPS pathogenesis and development of disease treatment and prevention strategies.

  13. Role of Selenium from Different Sources in Prevention of Pulmonary Arterial Hypertension Syndrome in Broiler Chickens.

    Science.gov (United States)

    Zamani Moghaddam, A K; Mehraei Hamzekolaei, M H; Khajali, F; Hassanpour, H

    2017-03-20

    Pulmonary arterial hypertension (PAH) syndrome in broilers is associated with hypoxia, which prevails at high altitude. Oxidative stress is the pathogenic mechanism underlying PAH. Because selenium is key element in the structure of antioxidant enzymes, we evaluated pulmonary hypertensive responses in broiler chickens fed with diets supplemented with organic or nano-selenium. One hundred forty-four broilers (starting at 5 days old) were fed with (i) control group: birds received a standard diet; (ii) nano-selenium group: birds were fed with basal diet supplemented with nano-selenium at 0.3 mg/kg; and (iii) organic selenium group: birds received basal diet supplemented with organic selenium at 0.3 mg/kg. We assessed growth performance, carcass characteristics, antioxidant variables, blood parameters, and small intestine morphology. Although Se supplementation did not affect growth performance, carcass traits, and organ weight (P > 0.05), the right to total ventricular weight ratio (RV:TV), malondialdehyde concentration in the liver, and heterophil to lymphocyte ratio were significantly lower in the nano-selenium group relative to the control (P right ventricular hypertrophy as reflected by reduced RV:TV, reduced levels of lipid peroxidation in the liver, and improved gut function.

  14. Central venous catheter-related blood stream infections in patients receiving intravenous iloprost for pulmonary hypertension.

    Science.gov (United States)

    Sammut, D; Elliot, C A; Kiely, D G; Armstrong, I J; Martin, L; Wilkinson, J; Sephton, P; Jones, J; Hamilton, N; Hurdman, J; McLellan, E; Sabroe, I; Condliffe, R

    2013-07-01

    Catheter-related blood stream infection (CR-BSI) in patients with pulmonary hypertension (PH) receiving intravenous iloprost via an indwelling central line has previously not been fully described. Recent studies have suggested a link between the pH of prostanoid infusions and the rate and nature of CR-BSI. We have investigated CR-BSI in patients receiving intravenous iloprost at our unit. Databases and hospital records were interrogated for all patients receiving intravenous iloprost between September 2007 and June 2012. Fifty-nine patients received intravenous iloprost via an indwelling central catheter with a total of 23,072 treatment days. There were 15 episodes of CR-BSI, identified using a systematic screening protocol, involving 11 patients giving an overall CR-BSI rate of 0.65/1,000 treatment days. CR-BSI rate for Gram-positive organisms was 0.26/1,000 treatment-days and for Gram-negative organisms was 0.39/1,000 treatment-days. The pH of iloprost in typical dosing regimens was comparable to the pH used in standard-diluent treprostinil and dissimilar to alkaline epoprostenol infusions. The proportion of Gram-negative CR-BSI was similar to that reported for standard-diluent treprostinil. CRP was normal on admission in 33 % of cases of confirmed CR-BSI and remained normal in 13 % of cases. CR-BSI rates with intravenous iloprost are comparable to those observed for other prostanoids. The high proportion of Gram-negative organisms observed and the neutral pH of iloprost infusions support the previously hypothesised link between pH and antimicrobial activity. Although usually elevated during a CR-BSI, CRP may be normal in early infection and a normal result cannot completely exclude infection.

  15. Rapidly progressive antineutrophil cytoplasm antibodies associated with pulmonary-renal syndrome in a 10-year-old girl

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    Fermin Blanco Filho

    2001-01-01

    Full Text Available CONTEXT: The term pulmonary-renal syndrome has been used frequently to describe the clinical manifestations of a great number of diseases in which pulmonary hemorrhage and glomerulonephritis coexist. The classic example of this type of vasculitis is Goodpasture´s syndrome, a term used to describe the association of pulmonary hemorrhage, glomerulonephritis and the presence of circulating antiglomerular basement membrane antibodies (anti-GBM. Among the several types of systemic vasculitides that can present clinical manifestations of the pulmonary-renal syndrome, we focus the discussion on two types more frequently associated with antineutrophil cytoplasm antibodies (ANCA, microscopic polyangiitis and Wegener´s granulomatosis, concerning a 10 year old girl with clinical signs and symptoms of pulmonary-renal syndrome, with positive ANCA and rapidly progressive evolution. CASE REPORT: We describe the case of a 10-year-old girl referred to our hospital for evaluation of profound anemia detected in a primary health center. Five days before entry she had experienced malaise, pallor and began to cough up blood-tinged sputum that was at first attributed to dental bleeding. She was admitted to the infirmary with hemoglobin = 4 mg/dL, hematocrit = 14%, platelets = 260,000, white blood cells = 8300, 74% segmented, 4% eosinophils, 19% lymphocytes and 3% monocytes. Radiographs of the chest revealed bilateral diffuse interstitial alveolar infiltrates. There was progressive worsening of cough and respiratory distress during the admission day, when she began to cough up large quantities of blood and hematuria was noted. There was rapid and progressive loss of renal function and massive lung hemorrhage. The antineutrophil cytoplasm antibody (ANCA test with antigen specificity for myeloperoxidase (anti-MPO was positive and the circulating anti-GBM showed an indeterminate result.

  16. Three-view bedside ultrasound for the differentiation of acute respiratory distress syndrome from cardiogenic pulmonary edema.

    Science.gov (United States)

    Mantuani, Daniel; Nagdev, Arun; Stone, Michael

    2012-09-01

    Bedside ultrasound is being increasingly used by emergency physicians (EPs) for the differentiation of acute dyspnea in critically ill patients. Lung ultrasound is emerging as a highly sensitive tool in diagnosing alveolar interstitial edema with the presence of diffuse “B-lines” arising from the pleural line. However, when used independently, lung ultrasound is unable to differentiate between cardiogenic and noncardiogenic causes of pulmonary edema. This case report describes a rapid 3-view or “triple scan” sonographic examination to differentiate acute respiratory distress syndrome (ARDS) from cardiogenic pulmonary edema.

  17. Prevalence and profile of congenital heart disease and pulmonary hypertension in Down syndrome in a pediatric cardiology service

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    Felipe Alves Mourato

    2014-06-01

    Full Text Available OBJECTIVE:To determine the frequence and profile of congenital heart defects in Down syndrome patients referred to a pediatric cardiologic center, considering the age of referral, gender, type of heart disease diagnosed by transthoracic echocardiography and its association with pulmonary hypertension at the initial diagnosis.METHODS:Cross-sectional study with retrospective data collection of 138 patients with Down syndrome from a total of 17,873 records. Descriptive analysis of the data was performed, using Epi-Info version 7.RESULTS: Among the 138 patients with Down syndrome, females prevailed (56.1% and 112 (81.2% were diagnosed with congenital heart disease. The most common lesion was ostium secundum atrial septal defect, present in 51.8%, followed by atrioventricular septal defect, in 46.4%. Ventricular septal defects were present in 27.7%, while tetralogy of Fallot represented 6.3% of the cases. Other cardiac malformations corresponded to 12.5%. Pulmonary hypertension was associated with 37.5% of the heart diseases. Only 35.5% of the patients were referred before six months of age.CONCLUSIONS: The low percentage of referral until six months of age highlights the need for a better tracking of patients with Down syndrome in the context of congenital heart disease, due to the high frequency and progression of pulmonary hypertension.

  18. Pulmonary-renal syndrome in systemic sclerosis: a report of three cases and review of the literature.

    Science.gov (United States)

    Naniwa, Taio; Banno, Shogo; Sugiura, Yoshiki; Yokota, Kaori; Oosawa, Tomoyo; Maeda, Shinji; Hayami, Yoshihito; Takahashi, Nobuyuki; Ueda, Ryuzo; Matsumoto, Yoshifuji

    2007-01-01

    We describe three cases of acute renal failure with diffuse alveolar hemorrhage, which is designated pulmonary-renal syndrome (PRS), in systemic sclerosis (SSc) and review the literature to better define this rare but severe complication of SSc. The clinical course of three SSc patients with acute renal failure and concomitant diffuse alveolar hemorrhage are reported, and the literature published between 1967 and 2005 is reviewed following a PubMed search. Including our cases, a total of 19 SSc patients with acute renal failure and concomitant diffuse alveolar hemorrhage have been reported. Pulmonary-renal syndrome developing in SSc patients can be categorized clinicopathologically into three entities: PRS with thrombotic microangiopathy, PRS with small vessel vasculitides accompanied with SSc, and d-penicillamine-induced Goodpasture-like syndrome. Patients with scleroderma PRS with thrombotic microangiopathy, to which group our all patients belong, often developed diffuse alveolar hemorrhage after receiving high-dose corticosteroid therapy. Pulmonary-renal syndrome is a fatal complication of SSc and results from different pathogenic processes. Prompt differential diagnosis between the subsets is critical, because therapeutic strategy may differ in the use of high-dose corticosteroid and plasma exchange between the subsets of PRS. Clinical courses of the patients with PRS with thrombotic microangiopathy suggest that high-dose corticosteroid therapy is a trigger of diffuse alveolar hemorrhage in patients with diffuse SSc with signs of thrombotic microangiopathy.

  19. Pulmonary thromboembolism in a child with sickle cell hemoglobin d disease in the setting of acute chest syndrome.

    Science.gov (United States)

    Villanueva, Hazel; Kuril, Sandeepkumar; Krajewski, Jennifer; Sedrak, Aziza

    2013-01-01

    Introduction. Sickle cell hemoglobin D disease (HbSD) is a rare variant of sickle cell disease (SCD). Incidence of pulmonary thromboembolism (PE) and deep venous thrombosis (DVT) in children with HbSD is unknown. PE and DVT are known complications of SCD in adults but have not been reported in the literature in children with HbSD. Case Report. We are reporting a case of a 12-year-old boy with HbSD with acute chest syndrome (ACS) complicated by complete thrombosis of the branch of the right pulmonary artery and multiple small pulmonary artery emboli seen on computed tomography (CT) pulmonary angiogram and thrombosis of the right brachial vein seen on Doppler ultrasound. Our patient responded to treatment with anticoagulant therapy. Conclusion. There are no cases reported in children with HbSD disease presenting as ACS with pulmonary thromboembolism. We suggest that PE should be suspected in patients presenting with ACS who do not show improvement with standard management. CT pulmonary angiogram should be utilized for early diagnosis and appropriate management as there is no current protocol for management of PE/DVT in pediatric patients with SCD.

  20. Therapeutic approaches to asthma-chronic obstructive pulmonary disease overlap syndromes.

    Science.gov (United States)

    Barnes, Peter J

    2015-09-01

    The recognition that there are some patients with features of asthma and chronic obstructive pulmonary disease (COPD) has highlighted the need to develop more specific treatments for these clinical phenotypes. Some patients with COPD have predominantly eosinophilic inflammation and might respond to high doses of inhaled corticosteroids and newly developed specific antieosinophil therapies, including blocking antibodies against IL-5, IL-13, IL-33, and thymic stromal lymphopoietin, as well as oral chemoattractant receptor-homologous molecule expressed on TH2 cells antagonists. Other patients have severe asthma or are asthmatic patients who smoke with features of COPD-induced inflammation and might benefit from treatments targeting neutrophils, including macrolides, CXCR2 antagonists, phosphodiesterase 4 inhibitors, p38 mitogen-activating protein kinase inhibitors, and antibodies against IL-1 and IL-17. Other patients appear to have largely fixed obstruction with little inflammation and might respond to long-acting bronchodilators, including long-acting muscarinic antagonists, to reduce hyperinflation. Highly selected patients with severe asthma might benefit from bronchial thermoplasty. Some patients with overlap syndromes can be conveniently treated with triple fixed-dose combination inhaler therapy with an inhaled corticosteroid, long-acting β2-agonist, and long-acting muscarinic antagonist, several of which are now in development. Corticosteroid resistance is a feature of asthma-COPD overlap syndrome, and understanding the various molecular mechanisms of this resistance has identified novel therapeutic targets and presented the prospect of therapies that can restore corticosteroid responsiveness.

  1. Retinal oximetry measures systemic hypoxia in central nervous system vessels in chronic obstructive pulmonary disease.

    Science.gov (United States)

    Eliasdottir, Thorunn Scheving; Bragason, David; Hardarson, Sveinn Hakon; Vacchiano, Charles; Gislason, Thorarinn; Kristjansdottir, Jona Valgerdur; Kristjansdottir, Gudrun; Stefánsson, Einar

    2017-01-01

    Determination of the blood oxyhemoglobin saturation in the retinal vessels of the eye can be achieved through spectrophotometric retinal oximetry which provides access to the state of oxyhemoglobin saturation in the central nervous system circulation. The purpose of this study was to test the capability of the Oxymap T1 oximeter to detect systemic hypoxemia and the effect of supplemental oxygen on retinal vessel oxyhemoglobin saturation. Oxygen saturation of hemoglobin in retinal arterioles and venules was measured in 11 subjects with severe chronic obstructive pulmonary disease (COPD) on long term oxygen therapy. Measurements were made with and without their daily supplemental oxygen. Eleven healthy age and gender matched subjects were measured during ambient air breathing for comparison of oxyhemoglobin saturation in retinal arterioles and venules. Retinal arteriolar oxyhemoglobin saturation in COPD subjects inspiring ambient air was compared with finger pulse oximetry and blood samples from radial artery. COPD subjects had significantly lower oxyhemoglobin saturation during ambient air breathing than healthy controls in both retinal arterioles (87.2%±4.9% vs. 93.4%±4.3%, p = 0.02; n = 11) and venules (45.0%±10.3% vs. 55.2%±5.5%, p = 0.01). Administration of their prescribed supplemental oxygen increased oxyhemoglobin saturation in retinal arterioles (87.2%±4.9% to 89.5%±6.0%, p = 0.02) but not in venules (45.0%±10.3% to 46.7%±12.8%, p = 0.3). Retinal oximetry values were slightly lower than radial artery blood values (mean percentage points difference = -5.0±5.4, 95% CI: -15.68 to 5.67) and finger pulse oximetry values (-3.1±5.5, 95% CI: -14.05 to 7.84). The noninvasive Oxymap T1 retinal oximetry detects hypoxemia in central nervous system vessels in patients with severe COPD compared with healthy controls. The instrument is sensitive to changes in oxygen breathing but displays slightly lower measures than finger pulse oximetry or radial artery

  2. Should waist circumference be replaced by index of central obesity (ICO) in definition of metabolic syndrome?

    Science.gov (United States)

    Parikh, R; Mohan, V; Joshi, S

    2012-01-01

    Waist circumference has been widely used as the parameter of central obesity in defining metabolic syndrome. Global consensus definition of metabolic syndrome has suggested various race- and gender-specific cutoffs of waist circumference for quantifying central obesity. We have earlier proposed that using index of central obesity (ICO), the need for race- and gender-specific cutoffs may be obviated. We propose that waist circumference be supplanted with index of central obesity in all definitions of metabolic syndrome. Using index of central obesity a common cutoff of 0.5 applicable across races and genders might be obtained. Moreover, it will enhance the sensitivity of definition by diagnosing subjects who are shorter than general population. Among 258 male and 242 female diabetic patients, subjects with metabolic syndrome (defined as per gender-specific waist circumference cutoffs), were found to have a common lower range of index of central obesity suggesting that a common cutoff of waist circumference may be obtained. In another study from National Health and Nutrition Examination Survey database, replacing index of central obesity with waist circumference was found to enhance the specificity and sensitivity of definition of metabolic syndrome.

  3. Simple pulmonary eosinophilia (loeffler's syndrome): chest radiographic and CT findings

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    Jung, Kyung Jae; Lee, Kyung Soo; Kim, Tae Sung; Chung, Man Pyo; Choi, Dong Chull; Kwon, O Jung [Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul (Korea, Republic of)

    2000-01-01

    The purpose of our study is to describe the chest radiographic and CT findings of simple pulmonary eosinophilia. Twenty-six patients with simple pulmonary eosinophilia underwent chest radiography and CT scanning; the results were analyzed retrospectively by two chest radiologists, focusing on the patterns and distribution of the parenchymal abnormalities. The chest radiographs were normal in eight patients (31%), while among the remaining 18 patients, they showed subtle opacity (n=3D9), nodules (n=3D8), consolidation (n=3D2), and mass (n=3D1). Follow-up chest radiographs (m=3D18) demonstrated complete (n=3D16) or partial (n=3D1) resolution of parenchymal lesions or migratory lesions (n=3D1). On CT, nodule(s) (n=3D19) were most commonly seen, followed by ground-glass opacity (n=3D16), consolidation (n=3D3), and mass (n=3D1). A peripheral halo surrounding a nodule or an area of consolidation was seen in 18 patients. The nodules(s) (n=3D19) were subpleural (n=3D13) or random (=3D6). Areas of ground-glass opacity (n=3D16) were subpleural (n=3D13), random (n=3D2), or central (n=3D1). All lesions were patchy rather than diffuse. Follow-up CT in nine patients showed complete (n=3D7) or partial (n=3D2) resolution of parenchymal lesions. Chest radiographs of patients with simple pulmonary eosinophilia often reveal no abnormality. The most common finding is subtle opacity or nodule(s), while CT reveals transient nodule(s) with a surrounding halo or transient areas of ground glass opacity. (author)

  4. Clinical experience of double sleeve lobectomy of the bronchus and the pulmonary artery in patients with central lung cancer

    Institute of Scientific and Technical Information of China (English)

    Yukang Kuang; Changying Guo; Laiduo Zeng; Dongsheng Wang; Binglin Yin; Jiufa Wu; Jian Huang; Zhisheng He; Jianfeng Zhu; Feng Jiang

    2013-01-01

    Objective: The aim of our study was to introduce the surgical method and evaluate the efficacy of double sleeve lobectomy of the bronchus and the pulmonary artery in treatment for the central lung cancer. Method: From March 1995 to October 2010, double sleeve lobectomy of the bronchus and the pulmonary artery was performed in 45 cases with central lung cancer that involved the bronchial opening of an upper lobe of the lungs or the main bronchus and pulmonary artery but didn't involve any lower lobes. Among them, left upper lobectomy was performed in 37 cases, right medium-upper lobectomy was performed in 6 cases and right upper lobectomy was performed in 2 cases. Results: Postoperative complications were found in 12 cases. Among them, 3 cases were arrhythmia, 1 case was acute heart failure, 6 cases were obstructive pneumonia and pulmonary atelectasis, 2 cases were bronchial anastomotic fistula. Two cases died of cerebral infarction and massive hemoptysis respectively. Thirty-one cases were squamous carcinoma, 7 cases were adenocarcinoma, 4 cases were small cell lung cancer, 1 case was adenosquamous carcinoma, 1 case was sarcomatoid carcinomas, 1 case was mucinous adenocarcinoma. Ten cases were T3N0M0, 11 cases were T3N1M0, 17 cases were T3N2M0, 2 cases were T4N1M0, 5 cases were T4N2M0. The 1-year, 3-year, 5-year survival rates were 84.4% (38/45), 51.7% (15/29), 53.8% (7/13) respectively. Conclusion: The double sleeve lobectomy of the bronchus and the pulmonary artery can maximumly reserve the normal lung tissues while removing tumors, and avoid pneumonectomy. The surgery was safe and effective, while it required a high technique.

  5. Pulmonary-renal syndromes: Experience from an Indian Intensive Care Unit

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    Srinivas Rajagopala

    2015-01-01

    Full Text Available Background: The etiology of patients presenting with pulmonary-renal syndrome (PRS to Intensive Care Units (ICUs in India is not previously reported. Aims: The aim was to describe the prevalence, etiology, clinical manifestations, and outcomes of PRS in an Indian ICU and identify variables that differentiate immunologic causes of PRS from tropical syndromes presenting with PRS. Materials and Methods: We conducted a prospective observational study of all patients presenting with PRS over 1-year. Clinical characteristics of patients with "definite PRS" were compared with those with "PRS mimics." Results: We saw 27 patients with "provisional PRS" over the said duration; this included 13 patients with "definite PRS" and 14 with "PRS mimics." The clinical symptoms were similar, but patients with PRS were younger and presented with longer symptom duration. Ninety-two percent of the PRS cohort required mechanical ventilation, 77% required vasopressors and 61.5% required dialysis within 48 h of ICU admission. The etiologic diagnosis of PRS was made after ICU admission in 61.5%. Systemic lupus erythrematosus (54% was the most common diagnosis. A combination of biopsy and serology was needed in the majority (69%, 9/13. Pulse methylprednisolone (92% and cyclophosphamide (61.5% was the most common protocol employed. Patients with PRS had more alveolar hemorrhage, hypoxemia and higher mortality (69% when compared to "PRS mimics." Conclusion: The spectrum of PRS is different in the tropics and tropical syndromes presenting with PRS are not uncommon. Multicentric studies are needed to further characterize the burden, etiology, treatment protocols, and outcomes of PRS in India.

  6. Central nervous system involvement in primary Sjogren`s syndrome manifesting as multiple sclerosis.

    Science.gov (United States)

    Liu, Jing-Yao; Zhao, Teng; Zhou, Chun-Kui

    2014-04-01

    Central nervous system symptoms in patients with primary Sjogren`s syndrome are rare. They can present as extraglandular manifestations and require a differential diagnosis from multiple sclerosis. Due to a variety of presentations, Sjogren`s syndrome with neurologic involvement may be difficult to diagnose. Here, we report a case of a 75-year-old woman who was first diagnosed with multiple sclerosis in 2010, but who was subsequently diagnosed with primary Sjogren`s syndrome 2 years later after showing signs of atypical neurologic manifestations. Therefore, primary Sjogren`s syndrome should be suspected in patients who present with atypical clinical and radiologic neurologic manifestations.

  7. Combined Pulmonary Fibrosis and Emphysema Syndrome: A New Phenotype within the Spectrum of Smoking-Related Interstitial Lung Disease

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    Karina Portillo

    2012-01-01

    Full Text Available Combined pulmonary fibrosis and emphysema (CPFE is a recently defined syndrome, in which centrilobular and/or paraseptal emphysemas in upper lung zones coexist with pulmonary fibrosis in lower lobes in individuals. These patients have a characteristic lung function profile, with unexpected subnormal dynamic and static lung volumes, contrasting with a significant reduction of carbon monoxide transfer (DLco and exercise hypoxemia. Pulmonary hypertension is highly prevalent in CPFE and is the leading determinant of death. Tobacco smoking has been proposed as the main factor in its etiology, though the pathophysiology and its natural history remain to be determined. High-resolution computed axial tomography is the mandatory tool to confirm the diagnosis. Currently, there is no consensus about its treatment since those published to date on this issue are limited to well-characterised series of cases; hence, a better understanding of this entity may help in the development of future therapeutic approaches.

  8. Effect of pulmonary surfactant combined with mucosolvan on immune function, liver and kidney function in neonatal respiratory distress syndrome

    Institute of Scientific and Technical Information of China (English)

    Juan Ma; Xiao-Lei Wang; Zheng-Ying Li; Tao-Ying Chen

    2016-01-01

    Objective:To explore the pulmonary surfactant combined with mucosolvan on immune function, liver and kidney function in neonatal respiratory distress syndrome, provide help for the treatment.Methods:A total of 160 cases of neonatal respiratory distress syndrome in our hospital were selected and randomly divided into observation group and control group according to the random number table method, 80 cases in each group, the control group was given conventional therapy, the observation group was given pulmonary surfactant combined with mucosolvan treatment on the basis of conventional therapy, before treatment and 3 days after treatment, the arterial blood gas correlation indexes, respiratory distress syndrome related factors, immune related factors, liver and kidney function indexes were detected in the 2 groups.Results:Compared with before treatment, in the observation group and the control group after treatment, arterial blood gas indexes PaO2, TCO2, SaO2 significantly increased, while PaCO2 significantly decreased, related cytokines KL-6, MIF-1 and HMGB-1 significantly decreased, immunologic factors IFN-γ and IL-4 significantly increased, while IL-10 and TNF-α significantly decreased, liver function indexes AST, ALT and renal function indexes BUN, CRE decreased significantly, the differences had statistically significant; compared with the control group after treatment, in the observation group after combined treatment, arterial blood gas indexes PaO2, TCO2, SaO2 significantly increased, PaCO2 significantly decreased, related cytokines KL-6, MIF-1 and HMGB-1 significantly decreased, immunologic factors IL-10 and IL-4 significantly increased, IFN-γ and TNF-α significantly decreased, liver function indexes AST, ALT and renal function indexes BUN, CRE decreased significantly; the differences had statistically significant.Conclusion:Pulmonary surfactant combined with mucosolvan can improve the respiratory distress syndrome related factors, immune function, liver

  9. Our patients followed up with a diagnosis of neurogenic pulmonary edema.

    Science.gov (United States)

    Sarı, Mehmet Yusuf; Yıldızdaş, Rıza Dinçer; Yükselmiş, Ufuk; Horoz, Özden Ögür

    2015-12-01

    Neurogenic pulmonary edema is a clinical situation which developes as a result of central nervous system injury. It is rare in the childhood. Neurogenic pulmonary edema is a clinical diagnosis. Although the pathogenesis is not elucidated well, there is increase in pulmonary interstitial and alveolar fluid. The main principle in treatment of neurogenic pulmonary edema is supportive treatment and decreasing intracranial pressure as in acute respiratory distress syndrome. In this article, clinical properties of our two patients diagnosed with neurogenic pulmonary edema developed as a result of central nervous system injury are presented.

  10. Our patients followed up with a diagnosis of neurogenic pulmonary edema

    Science.gov (United States)

    Sarı, Mehmet Yusuf; Yıldızdaş, Rıza Dinçer; Yükselmiş, Ufuk; Horoz, Özden Ögür

    2015-01-01

    Neurogenic pulmonary edema is a clinical situation which developes as a result of central nervous system injury. It is rare in the childhood. Neurogenic pulmonary edema is a clinical diagnosis. Although the pathogenesis is not elucidated well, there is increase in pulmonary interstitial and alveolar fluid. The main principle in treatment of neurogenic pulmonary edema is supportive treatment and decreasing intracranial pressure as in acute respiratory distress syndrome. In this article, clinical properties of our two patients diagnosed with neurogenic pulmonary edema developed as a result of central nervous system injury are presented. PMID:26884694

  11. Differences in the effects of Asian dust on pulmonary function between adult patients with asthma and those with asthma–chronic obstructive pulmonary disease overlap syndrome

    Science.gov (United States)

    Watanabe, Masanari; Noma, Hisashi; Kurai, Jun; Sano, Hiroyuki; Ueda, Yasuto; Mikami, Masaaki; Yamamoto, Hiroyuki; Tokuyasu, Hirokazu; Kato, Kazuhiro; Konishi, Tatsuya; Tatsukawa, Toshiyuki; Shimizu, Eiji; Kitano, Hiroya

    2016-01-01

    Background Asian dust (AD) exposure exacerbates pulmonary dysfunction in patients with asthma. Asthma–chronic obstructive pulmonary disease overlap syndrome (ACOS), characterized by coexisting symptoms of asthma and chronic obstructive pulmonary disease, is considered a separate disease entity. Previously, we investigated the effects of AD on pulmonary function in adult patients with asthma. Here, we present the findings of our further research on the differences in the effects of AD exposure on pulmonary function between patients with asthma alone and those with ACOS. Methods Between March and May 2012, we conducted a panel study wherein we monitored daily peak expiratory flow (PEF) values in 231 adult patients with asthma. These patients were divided into 190 patients with asthma alone and 41 patients with ACOS in this study. Daily AD particle levels were measured using light detection and ranging systems. Two heavy AD days (April 23 and 24) were determined according to the Japan Meteorological Agency definition. A linear mixed model was used to estimate the association between PEF and AD exposure. Results Increments in the interquartile range of AD particles (0.018 km−1) led to PEF changes of −0.50 L/min (95% confidence interval, −0.98 to −0.02) in patients with asthma alone and −0.11 L/min (−0.11 to 0.85) in patients with ACOS. The PEF changes after exposure to heavy AD were −2.21 L/min (−4.28 to −0.15) in patients with asthma alone and −2.76 L/min (−6.86 to 1.35) in patients with ACOS. In patients with asthma alone, the highest decrease in PEF values was observed on the heavy AD day, with a subsequent gradual increase over time. Conclusion Our results suggest that the effects of AD exposure on pulmonary function differ between patients with asthma alone and ACOS, with the former exhibiting a greater likelihood of decreased pulmonary function after AD exposure. PMID:26869784

  12. [Acute necrotizing capillaritis in an adolescent dying from a a Goodpasture-like pulmonary-renal syndrome].

    Science.gov (United States)

    Maresi, E; Becchina, G; Orlando, E; Ottoveggio, G

    1995-12-01

    In this report the authors describe a rare case of pulmonary/renal syndrome like-Goodpasture's disease characterized by a necrotizing acute capillaritis in the lung, kidney, liver, spleen and heart. In the heart the capillaritis was associated with a contiguous acute ischemic lesion. The clinical and pathologic findings of the case suggest that the presence of vasculitis should not exclude a diagnosis of Goodpasture's Syndrome and that the multisystemic capillaritis would demonstrate the organ-non-specificity of anti-glomerular/alveolar basement membrane antibody.

  13. Is non-thyroidal illness syndrome a predictor for prolonged weaning in intubated chronic obstructive pulmonary disease patients?

    OpenAIRE

    Yasar, Zehra; Kirakli, Cenk; Cimen, Pınar; Ucar, Zeynep Zeren; Talay, Fahrettin; Tibet, Gultekin

    2015-01-01

    Introduction: Non-thyroidal illness syndrome (NTIS) is considered to be associated with adverse outcomes in intensive care unit (ICU) patients. In this study, we evaluated the association between NTIS and prolonged weaning in chronic obstructive pulmonary disease (COPD) patients admitted to the ICU. Materials and methods: In total, 125 patients with COPD admitted to our ICU who underwent invasive mechanical ventilation (MV) were enrolled. We collected each patient’s baseline characteristics i...

  14. A Case of Antiphospholipid Syndrome Refractory to Secondary Anticoagulating Prophylaxis after Deep Vein Thrombosis-Pulmonary Embolism

    OpenAIRE

    Gu, Kang Mo; Shin,Jong Wook; Park, In Won

    2014-01-01

    Antiphospholipid syndrome (APS) is an acquired systemic autoimmune disorder characterized by a combination of clinical criteria, including vascular thrombosis or pregnancy morbidity and elevated antiphospholipid antibody titers. It is one of the causes of deep vein thrombosis and pulmonary embolism that can be critical due to the mortality risk. Overall recurrence of thromboembolism is very low with adequate anticoagulation prophylaxis. The most effective treatment to prevent recurrent thromb...

  15. Central serous chorioretinopathy as a presenting symptom of endogenous Cushing syndrome: a case report.

    Science.gov (United States)

    Iannetti, Ludovico; Spinucci, Giovanni; Pesci, Francesca Romana; Vicinanza, Roberto; Stigliano, Antonio; Pivetti-Pezzi, Paola

    2011-01-01

    To report a case of Cushing syndrome due to adrenocortical adenoma revealed by central serous chorioretinopathy. A 45-year-old man presented with blurred vision and metamorphopsia in the left eye. He reported few episodes of high blood pressure in the last 3 months. Visual acuity was 20/40 in the left eye. Fundus oculi examination revealed central serous chorioretinopathy in the left eye. Grade 1 hypertension was found. Increased serum and urinary levels of cortisol and reduced serum levels of ACTH were observed. Diagnosis of Cushing syndrome was made. Computed tomography scan revealed a right adrenal mass that was surgically removed; histologic examination showed an adrenocortical adenoma. Three months after surgical treatment, visual acuity improved to 20/20 and central serous chorioretinopathy completely resolved. Central serous chorioretinopathy may be the presenting symptom of Cushing syndrome in a patient with adrenocortical adenoma.

  16. Left Ventricular Mass index and Pulmonary Artery Pressure in Patients with the Obstructive Sleep Apnea Syndrome

    Directory of Open Access Journals (Sweden)

    Seyed Hashem Sezavar

    2016-07-01

    Full Text Available Background: Sleep apnea is accompanied by some cardiovascular complications. It has even been hypothesized that sleep apnea, itself, can induce some of these complications. Given such controversies, we assessed the left ventricular mass index (LVMI and systolic pulmonary artery pressure in patients with sleep apnea.Methods: Through convenience sampling, 56 patients with the obstructive sleep apnea syndrome (OSAS were included in the present descriptive cross-sectional study. Patients with any past history of hypertension and diabetes mellitus were excluded. The apnea severity was assessed via the polysomnography-derived apnea-hypopnea index (AHI. All the patients underwent transthoracic echocardiography. In this cross-sectional study - data regarding age, gender, smoking, systolic and diastolic blood pressures, polysomnographic parameters (AHI, severity of disease, mean heart rate, mean oxygen saturation [SaO2], lowest SaO2, and duration of SaO2 below 90% [d.SaO2 < 90%], and  echocardiographic parameters (systolic pulmonary artery pressure and LVMI were accumulated and processed.Results: Fifty-two men and 14 women at a mean age of 49.29 ± 11.79 years participated in this study. Systolic and was significantly high in the severe group compared with the mild group (128.21 ± 9.73 mmHg vs. 119.23 ± 12.5 mmHg; p value = 0.007. The LVMI was increased parallel to an increase in the severity of the OSAS, but that increase was not statistically significant (p value = 0.161. The d.SaO2 < 90% was positively correlated with the LVMI, and this relationship remained true after adjustment for the body mass index (r = 0.27; p value = 0.042.Conclusion: Severe OSAS was accompanied by a higher blood pressure. The LVMI did not differ significantly between the patients with the OSAS and those who did not suffer from other risk factors of cardiac diseases.

  17. Obstructive Sleep Apnea Hypopnea Syndrome as a Reason for Active Management of Pulmonary Embolism

    Institute of Scientific and Technical Information of China (English)

    Jiang Xie; Yong-Xiang Wei; Shuang Liu; Wei Zhang; Xiang-Feng Zhang; Jie Li

    2015-01-01

    Background:Obstructive sleep apnea hypopnea syndrome (OSAHS) constitutes an independent factor for high warfarin dose for patients with pulmonary embolism (PE).The aim of this study was to investigate whether the 6-month anticoagulation treatment by warfarin is enough for patients with PE complicated by OSAHS.Methods:We investigated 97 PE patients,32 of them had OSAHS and 65 non-OSAHS.Warfarin was administered for 6-month if no abnormal circumstances occurred.All patients were followed up for 18 months.Adverse events (AE) included death,major bleeding,hospitalization due to heart failure or pulmonary hypertension,and recurrence or aggravation of PE (including deep vein thrombosis).Recurrence rate of PE after warfarin cessation was compared between the two groups.Results:OSAHS patients required a significantly higher dose of warfarin than their non-OSAHS counterparts (4.73 mg vs.3.61 mg,P < 0.001).During warfarin treatment,no major bleeding and aggravation of PE occurred among OSAHS patients,and the rates of various AE were not significantly different between the OSAHS and non-OSAHS groups.PE recurrence was higher in OSAHS than non-OSAHS groups after withdrawal of warfarin (21.43% vs.6.78%,P =0.047).Compared with non-OSAHS patients,OSAHS group had lower international normalized ratio (INR) value but higher plasminogen on baseline and INR resumed to a relatively low level after warfarin discontinuation.Conclusions:OSAHS patients may present with hypercoagulation and relatively high-risk of recurrence of PE after cessation of 6-month warfarin treatment.

  18. Pulmonary Langerhans Cell Histiocytosis in an Adult Male Presenting with Central Diabetes Insipidus and Diabetes Mellitus: A Case Report

    Science.gov (United States)

    Choi, Yeun Seoung; Lim, Jung Soo; Kwon, Woocheol; Jung, Soon-Hee; Park, Il Hwan; Lee, Myoung Kyu; Lee, Won Yeon; Yong, Suk Joong; Lee, Seok Jeong; Jung, Ye-Ryung; Choi, Jiwon; Choi, Ji Sun; Jeong, Joon Taek; Yoo, Jin Sae

    2015-01-01

    Pulmonary Langerhans cell histiocytosis is an uncommon diffuse cystic lung disease in adults. In rare cases, it can involve extrapulmonary organs and lead to endocrine abnormalities such as central diabetes insipidus. A 42-year-old man presented with polyphagia and polydipsia, as well as a dry cough and dyspnea on exertion. Magnetic resonance imaging of the hypothalamic-pituitary system failed to show the posterior pituitary, which is a typical finding in patients with central diabetes insipidus. This condition was confirmed by a water deprivation test, and the patient was also found to have type 2 diabetes mellitus. Computed tomographic scanning of the lungs revealed multiple, irregularly shaped cystic lesions and small nodules bilaterally, with sparing of the costophrenic angles. Lung biopsy through video-assisted thoracoscopic surgery revealed pulmonary Langerhans cell histiocytosis. On a follow-up visit, only 1 year after the patient had quit smoking, clinical and radiological improvement was significant. Here, we report an uncommon case of pulmonary Langerhans cell histiocytosis that simultaneously presented with diabetes insipidus and diabetes mellitus. PMID:26508947

  19. Quantitative computed tomography measurements of emphysema for diagnosing asthma-chronic obstructive pulmonary disease overlap syndrome

    Directory of Open Access Journals (Sweden)

    Xie M

    2016-05-01

    Full Text Available Mengshuang Xie, Wei Wang, Shuang Dou, Liwei Cui, Wei Xiao Department of Pulmonary Medicine, Qilu Hospital, Shandong University, Jinan, People’s Republic of China Background: The diagnostic criteria of asthma–COPD overlap syndrome (ACOS are controversial. Emphysema is characteristic of COPD and usually does not exist in typical asthma patients. Emphysema in patients with asthma suggests the coexistence of COPD. Quantitative computed tomography (CT allows repeated evaluation of emphysema noninvasively. We investigated the value of quantitative CT measurements of emphysema in the diagnosis of ACOS.Methods: This study included 404 participants; 151 asthma patients, 125 COPD patients, and 128 normal control subjects. All the participants underwent pulmonary function tests and a high-resolution CT scan. Emphysema measurements were taken with an Airway Inspector software. The asthma patients were divided into high and low emphysema index (EI groups based on the percentage of low attenuation areas less than −950 Hounsfield units. The characteristics of asthma patients with high EI were compared with those having low EI or COPD.Results: The normal value of percentage of low attenuation areas less than −950 Hounsfield units in Chinese aged >40 years was 2.79%±2.37%. COPD patients indicated more severe emphysema and more upper-zone-predominant distribution of emphysema than asthma patients or controls. Thirty-two (21.2% of the 151 asthma patients had high EI. Compared with asthma patients with low EI, those with high EI were significantly older, more likely to be male, had more pack-years of smoking, had more upper-zone-predominant distribution of emphysema, and had greater airflow limitation. There were no significant differences in sex ratios, pack-years of smoking, airflow limitation, or emphysema distribution between asthma patients with high EI and COPD patients. A greater number of acute exacerbations were seen in asthma patients with high

  20. Correlation between chronic obstructive pulmonary disease and obstructive sleep apnea syndrome in a general population in Iran

    Directory of Open Access Journals (Sweden)

    Babak Amra

    2011-01-01

    Full Text Available Background: The aim of this study was to evaluate epidemiological relationship between chronic obstructive pulmonary disease and sleep apnea syndrome in a sample of Persian population. Methods: As a part of a population-based cross-sectional study, 3900 randomly selected individuals aged 15 years or older were invited to take part in the survey; 3770 individuals (96.6% agreed to fill out the respiratory and sleep questionnaire. Those subjects suspected to have either chronic obstructive pulmonary disease and/or obstructive sleep apnea underwent spirometry and polysomnography test if indicated. Spirometric measurements were performed on 420 invited responders. Polysomnography measurements were performed on 25 of the responders. Results: Prevalence rates for sleep apnea, chronic obstructive pulmonary disease and current asthma were 4.98%, 5.7% and 3.1%, respectively. Logistic regression showed independent associations between sleep apnea and chronic obstructive pulmonary disease. There was no significant independent association between sleep apnea symptoms and current asthma and wheeze ever. Conclusions: These observations indicated relationship between chronic obstructive pulmonary disease and obstructive sleep apnea. These observations indicated the necessity of further studies to explain the possible common pathogenic mechanisms involved in two disease entities.

  1. Centrally-located pulmonary hamartoma diagnosed in a 16-year-old ...

    African Journals Online (AJOL)

    Pulmonary hamartomas are the most common benign tumors of the lung. ... also known as mesenchymomas, are non-neoplastic tumor-like malformations of the lung ... symptoms including recurrent respiratory tract infections, obstructive ...

  2. [Partial anomalous pulmonary venous connection as incidental finding: Explanation for apparently paradoxical central venous blood gas analysis results].

    Science.gov (United States)

    Brandt, L; Artmeier-Brandt, U

    2015-10-01

    A partial anomalous pulmonary venous connection (PAPVC) is a congenital abnormality of the great thoracic vessels the incidence of which is underestimated and is associated with a left-right shunt. It rarely develops into a right-sided cardiac insufficiency. Because of the mostly low left-right shunt volume, a PAPVC is often asymptomatic and mostly incidentally detected in advanced age. Incorrect positioning of a central venous catheter or paradoxical blood gas parameters can serve to indicate the presence of a PAPVC. This article presents the case a 50-year-old patient with a PAPVC of the left upper lobe pulmonary vein draining into the left innominate vein without prior clinical symptoms. Blood gas analyses from the superior vena cava, where the catheter placement was confirmed by computed tomography angiography, showed unexplainable arterial values. The anatomical abnormality was confirmed by computed tomography.

  3. Síndrome pulmón-riñón Pulmonary-renal syndrome

    Directory of Open Access Journals (Sweden)

    Jorge A. Risso

    2009-12-01

    Full Text Available El síndrome pulmón-riñón se define como una combinación de hemorragia alveolar difusa y glomerulonefritis. La coexistencia de estas dos afecciones clínicas se produce por enfermedades con distintos mecanismos patogénicos. Las vasculitis sistémicas primarias y el síndrome de Goodpasture son las etiologías más frecuentes. El lupus eritematoso sistémico, otras colagenopatías, las vasculitis con anticuerpos anticitoplasma de los neutrófilos negativos y las secundarias a drogas son causas mucho menos comunes. El diagnóstico temprano basado en criterios clínicos, radiológicos, de laboratorio e histológicos, permite iniciar el tratamiento disminuyendo su elevada morbi-mortalidad. La terapéutica se basa en altas dosis de corticoides, inmunosupresores, inhibidores del factor de necrosis tumoral y plasmaféresis.The pulmonary-renal syndrome is defined as a combination of diffuse alveolar hemorrhage and glomerulonephritis. The coexistence of these two clinical conditions is due to diseases with different pathogenic mechanisms. Primary systemic vasculitis and Goodpasture syndrome are the most frequent etiologies. Systemic lupus erythematosus, connective tissue diseases, negative anti neutrophil cytoplasmic antibody vasculitis and those secondary to drugs are far less common causes. An early diagnosis based on clinical, radiologic, laboratory and histologic criteria enables early treatment, thus diminishing its high morbility-mortality rate. Therapy is based on high doses of corticosteroids, immunosuppressants, tumor necrosis factor inhibitors and plasmapheresis.

  4. Isolated Richter's syndrome in central nervous system: case report Sindrome de Richter isolada em sistema nervoso central: relato de caso

    Directory of Open Access Journals (Sweden)

    Lucilene S.R. Resende

    2005-06-01

    Full Text Available Diffuse large cell non Hodgkin's lymphoma associated with chronic lymphoid leukemia (CLL, or Richter's syndrome, is a rare and serious complication. Isolated Richter's syndrome in the central nervous system is very rare; only 12 cases have been reported. We describe a 74-year-old patient with diffuse large cell non Hodgkin's lymphoma in the right frontal region with the appearance of multiform glioblastoma.Linfoma não Hodgkin difuso de grandes células em paciente portador de leucemia linfóide crônica (LLC, ou síndrome de Richter, é complicação rara e grave nesta leucemia. Síndrome de Richter isolada no sistema nervoso central é muito rara, tendo sido encontrados apenas 12 casos descritos. Descrevemos paciente de 74 anos, que apresentou linfoma não Hodgkin difuso de grandes células em região frontal direita, simulando glioblastoma multiforme.

  5. Serological diagnosis of hantavirus pulmonary syndrome in a febrile patient in Colombia.

    Science.gov (United States)

    Mattar, Salim; Garzon, Denisse; Tadeu, Luis; Faccini-Martínez, Alvaro A; Mills, James N

    2014-08-01

    Hantavirus pulmonary syndrome (HPS) is an often fatal rodent-borne zoonosis caused by any of at least 20 hantavirus genotypes distributed throughout the Americas. Although HPS has been documented in several bordering countries, it has not been reported in Colombia. Here we report seroconversion to a hantavirus in paired samples from a hospitalized patient with symptoms compatible with HPS from Montería, Córdoba Department, north-western Colombia. Tests for regionally endemic agents including Plasmodium, Leptospira, Salmonella, dengue virus, Brucella, Rickettsia, human immunodeficiency virus and hepatitis viruses were negative. Because the patient was enrolled in a clinical trial for hemorrhagic fevers conducted by the University of Córdoba, serum samples were collected on admission and at discharge. Testing using Sin Nombre virus ELISA showed IgG and IgM seroconversion between samples. The eventual finding of this first clinical case of hantavirus infection in Colombia is consistent with the high prevalence of hantavirus antibodies in humans in the region and the likely exposure of the patient to rodents. The clinical presentation was similar to that found in neighbouring Panama.

  6. Hantavirus pulmonary syndrome in Brazil: clinical aspects of three new cases

    Directory of Open Access Journals (Sweden)

    FERREIRA Marcelo S.

    2000-01-01

    Full Text Available Hantavirus pulmonary syndrome (HPS has been recognized recently in Brazil, where 28 cases have been reported as of September 1999. We report here the clinical and laboratory findings of three cases whose diagnoses were confirmed serologically. All the patients were adults who presented a febrile illness with respiratory symptoms that progressed to respiratory failure that required artificial ventilation in two of them. Laboratory findings were most of the time consistent with those reported in the United States in patients infected with the Sin Nombre virus, and included elevated hematocrit and thrombocytopenia; presence of atypical lymphocytes was observed in one patient. The chest radiological findings observed in all the patients were bilateral, diffuse, reticulonodular infiltrates. Two patients died. Histopathological examination of the lungs of these patients revealed interstitial and alveolar edema, alveolar hemorrhage, and mild interstitial pneumonia characterized by infiltrate of immunoblasts and mononuclear cells. In the epidemiologic investigation of one of the cases, serologic (ELISA tests were positive in 3 (25% out of 12 individuals who shared the same environmental exposure. HPS should be included in the differential diagnosis of interstitial pneumonia progressing to acute respiratory failure.

  7. Ribavirin Protects Syrian Hamsters against Lethal Hantavirus Pulmonary Syndrome — After Intranasal Exposure to Andes Virus

    Science.gov (United States)

    Ogg, Monica; Jonsson, Colleen B.; Camp, Jeremy V.; Hooper, Jay W.

    2013-01-01

    Andes virus, ANDV, harbored by wild rodents, causes the highly lethal hantavirus pulmonary syndrome (HPS) upon transmission to humans resulting in death in 30% to 50% of the cases. As there is no treatment for this disease, we systematically tested the efficacy of ribavirin in vitro and in an animal model. In vitro assays confirmed antiviral activity and determined that the most effective doses were 40 µg/mL and above. We tested three different concentrations of ribavirin for their capability to prevent HPS in the ANDV hamster model following an intranasal challenge. While the highest level of ribavirin (200 mg/kg) was toxic to the hamster, both the middle (100 mg/kg) and the lowest concentration (50 mg/kg) prevented HPS in hamsters without toxicity. Specifically, 8 of 8 hamsters survived intranasal challenge for both of those groups whereas 7 of 8 PBS control-treated animals developed lethal HPS. Further, we report that administration of ribavirin at 50 mg/kg/day starting on days 6, 8, 10, or 12 post-infection resulted in significant protection against HPS in all groups. Administration of ribavirin at 14 days post-infection also provided a significant level of protection against lethal HPS. These data provide in vivo evidence supporting the potential use of ribavirin as a post-exposure treatment to prevent HPS after exposure by the respiratory route. PMID:24217424

  8. Ribavirin Protects Syrian Hamsters against Lethal Hantavirus Pulmonary Syndrome — After Intranasal Exposure to Andes Virus

    Directory of Open Access Journals (Sweden)

    Jay W. Hooper

    2013-11-01

    Full Text Available Andes virus, ANDV, harbored by wild rodents, causes the highly lethal hantavirus pulmonary syndrome (HPS upon transmission to humans resulting in death in 30% to 50% of the cases. As there is no treatment for this disease, we systematically tested the efficacy of ribavirin in vitro and in an animal model. In vitro assays confirmed antiviral activity and determined that the most effective doses were 40 µg/mL and above. We tested three different concentrations of ribavirin for their capability to prevent HPS in the ANDV hamster model following an intranasal challenge. While the highest level of ribavirin (200 mg/kg was toxic to the hamster, both the middle (100 mg/kg and the lowest concentration (50 mg/kg prevented HPS in hamsters without toxicity. Specifically, 8 of 8 hamsters survived intranasal challenge for both of those groups whereas 7 of 8 PBS control-treated animals developed lethal HPS. Further, we report that administration of ribavirin at 50 mg/kg/day starting on days 6, 8, 10, or 12 post-infection resulted in significant protection against HPS in all groups. Administration of ribavirin at 14 days post-infection also provided a significant level of protection against lethal HPS. These data provide in vivo evidence supporting the potential use of ribavirin as a post-exposure treatment to prevent HPS after exposure by the respiratory route.

  9. Hantavirus pulmonary syndrome in the Province of Rio Negro, Argentina, 1993-1996.

    Science.gov (United States)

    Cantoni, G; Lazaro, M; Resa, A; Arellano, O; Amestoy, A M; De Bunder, S; Herrero, E; Perez, A; Larrieu, E

    1997-01-01

    Early in 1995 the first case of Hantavirus Pulmonary Syndrome was serologically confirmed in El Bolsón (Province of Río Negro, Argentina), corresponding to the third outbreak reported in Argentina. A total of 26 cases of HPS related to the Andean region of Rio Negro Province, were reported from 1993 to 1996, 17 in El Bolsón, 4 in San Carlos de Bariloche, and 5 in Buenos Aires. The incidence rate was 5.03 x 100,000 with a mortality rate of 51.85 x 100. The occurrence of cases was mainly seasonal, with a significantly greater number in the spring, and the persons affected mainly lived in urban or periurban areas. In four cases, the affected individuals were members of a couple, spouses or live-in contacts. Seven cases were Health workers (physicians, nurses or administrative staff). Twelve cases were related among them, due to an outbreak of 80 days. Two of them did not visit the Andean region. A total of 139 rodents were captured and seven of them, Olygoryzomys longicaudatus, were found to be serologically positive. The possibility of infection by contact with rodents or fecal matter is being analyzed and also hypothesis related with interhuman transmission.

  10. Epithelioid pleural mesothelioma concurrently associated with miliary pulmonary metastases and minimal change nephrotic syndrome - A hitherto undescribed case.

    Science.gov (United States)

    Tsukamoto, Yoshitane; Otsuki, Taiichiro; Hao, Hiroyuki; Kuribayashi, Kozo; Nakano, Takashi; Kida, Aritoshi; Nakanishi, Takeshi; Funatsu, Eriko; Noguchi, Chihiro; Yoshihara, Shunya; Kaku, Koji; Hirota, Seiichi

    2015-12-01

    Malignant pleural mesothelioma (MPM) is the aggressive disease typically spreading along the pleural surface and encasing the lung, leading to respiratory failure or cachexia. Rare cases with atypical clinical manifestation or presentation have been reported in MPM. We experienced a unique case of MPM concurrently associated with miliary pulmonary metastases and nephrotic syndrome. A 73-year-old Japanese man with past history of asbestos exposure was referred to our hospital for the investigation of the left pleural effusion. Chest computed tomography showed thickening of the left parietal pleura. Biopsy specimen of the pleura showed proliferating epithelioid tumor cells, leading to the pathological diagnosis of epithelioid MPM with the aid of immunohistochemistry. After the diagnosis of MPM, chemotherapy was performed without effect. Soon after the clinical diagnosis of progressive disease with skull metastasis, edema and weight gain appeared. Laboratory data met the criteria of nephrotic syndrome, and renal biopsy with electron microscopic examination revealed the minimal change disease. Steroid therapy was started but showed no effect. Around the same time of onset of nephrotic syndrome, multiple miliary lung nodules appeared on chest CT. Transbronchial biopsy specimen of the nodules showed the metastatic MPM in the lung. The patient died because of the worsening of the general condition. To our knowledge, this is the first case of MPM concurrently associated with multiple miliary pulmonary metastases and nephrotic syndrome.

  11. Bilateral widespread mechanical pain sensitivity in carpal tunnel syndrome: evidence of central processing in unilateral neuropathy.

    Science.gov (United States)

    Fernández-de-las-Peñas, César; de la Llave-Rincón, Ana Isabel; Fernández-Carnero, Josué; Cuadrado, María Luz; Arendt-Nielsen, Lars; Pareja, Juan A

    2009-06-01

    The aim of this study was to investigate whether bilateral widespread pressure hypersensitivity exists in patients with unilateral carpal tunnel syndrome. A total of 20 females with carpal tunnel syndrome (aged 22-60 years), and 20 healthy matched females (aged 21-60 years old) were recruited. Pressure pain thresholds were assessed bilaterally over median, ulnar, and radial nerve trunks, the C5-C6 zygapophyseal joint, the carpal tunnel and the tibialis anterior muscle in a blinded design. The results showed that pressure pain threshold levels were significantly decreased bilaterally over the median, ulnar, and radial nerve trunks, the carpal tunnel, the C5-C6 zygapophyseal joint, and the tibialis anterior muscle in patients with unilateral carpal tunnel syndrome as compared to healthy controls (all, P < 0.001). Pressure pain threshold was negatively correlated to both hand pain intensity and duration of symptoms (all, P < 0.001). Our findings revealed bilateral widespread pressure hypersensitivity in subjects with carpal tunnel syndrome, which suggest that widespread central sensitization is involved in patients with unilateral carpal tunnel syndrome. The generalized decrease in pressure pain thresholds associated with pain intensity and duration of symptoms supports a role of the peripheral drive to initiate and maintain central sensitization. Nevertheless, both central and peripheral sensitization mechanisms are probably involved at the same time in carpal tunnel syndrome.

  12. Myelodysplastic syndrome complicated by central diabetes insipidus and cerebral salt wasting syndrome with peculiar change in magnetic resonance images.

    Science.gov (United States)

    Sano, Soichi; Yamagami, Keiko; Morikawa, Takashi; Yoshioka, Katsunobu

    2010-01-01

    Central diabetes insipidus (CDI) could occurs in patients with myelodysplastic syndrome (MDS) or acute myeloid leukemia (AML), because of infiltration of leukemic cells into the neurohypophysis or some other reason and it is closely associated with abnormalities of chromosome 7. We report a case of MDS with abnormalities of chromosome 7, presenting as CDI followed by deterioration of polyuria and hyponatremia with a decreased extracellular fluid volume. Magnetic resonance imaging (MRI) revealed symmetrically enhanced lesions in the hypothalamus. Fludrocortisone treatment normalized his serum sodium level and cerebral salt wasting syndrome (CSWS) was suspected.

  13. Proteoglycan and proteome profiling of central human pulmonary fibrotic tissue utilizing miniaturized sample preparation: a feasibility study

    DEFF Research Database (Denmark)

    Malmström, Johan; Larsen, Kristoffer; Hansson, Lennart;

    2002-01-01

    The objective of this study was to isolate fibrotic cells from human lung biopsies taken from different central pulmonary locations. A comparison was made of cell morphology, proteoglycan- and protein-expression in mesenchymal cell cultures obtained from human bronchial biopsies from patients...... tissue; one of contractile type with lamellipodia that facilitate migration and a second cell type with an increased cell size, which most likely is of a synthetic phenotype. This is the first evidence of alterations in the proteoglycan expression pattern of versican, perlecan, biglycan and decorin which...

  14. Upper extremity acute compartment syndrome during tissue plasminogen activator therapy for pulmonary embolism in a morbidly obese patient.

    Science.gov (United States)

    Tuna, Serkan; Duymus, Tahir Mutlu; Mutlu, Serhat; Ketenci, Ismail Emre; Ulusoy, Ayhan

    2015-01-01

    Deep vein thrombosis (DVT) and pulmonary embolism (PE) are more frequently observed in morbidly obese patients. Tissue plasminogen activator (tPA) is a thrombolytic agent which dissolves the thrombus more rapidly than conventional heparin therapy and reduces the mortality and morbidity rates associated with PE. Compartment syndrome is a well-known and documented complication of thrombolytic treatment. In awake, oriented and cooperative patients, the diagnosis of compartment syndrome is made based on clinical findings including swelling, tautness, irrational and continuous pain, altered sensation, and severe pain due to passive stretching. These clinical findings may not be able to be adequately assessed in unconscious patients. In this case report, we present compartment syndrome observed, for which fasciotomy was performed on the upper right extremity of a 46-year old morbidly obese, conscious female patient who was receiving tPA due to a massive pulmonary embolism. Compartment syndrome had occurred due to the damage caused by the repeated unsuccessful catheterisation attempts to the brachial artery and the accompanying tPA treatment. Thus, the bleeding that occurred in the volar compartment of the forearm and the anterior compartment of the arm led to acute compartment syndrome (ACS). After relaxation was brought about in the volar compartment of the forearm and the anterior compartment of the arm, the circulation in the limb was restored. As soon as the diagnosis of compartment syndrome is made, an emergency fasciotomy should be performed. Close follow-up is required to avoid wound healing problems after the fasciotomy. Copyright © 2015 The Authors. Published by Elsevier Ltd.. All rights reserved.

  15. Combined central diabetes insipidus and cerebral salt wasting syndrome in children.

    Science.gov (United States)

    Lin, Jainn-Jim; Lin, Kuang-Lin; Hsia, Shao-Hsuan; Wu, Chang-Teng; Wang, Huei-Shyong

    2009-02-01

    Central diabetes insipidus, a common consequence of acute central nervous system injury, causes hypernatremia; cerebral salt wasting syndrome can cause hyponatremia. The two conditions occurring simultaneous are rarely described in pediatric patients. Pediatric cases of combined diabetes insipidus and cerebral salt wasting after acute central nervous system injury between January 2000 and December 2007 were retrospectively reviewed, and clinical characteristics were systemically assessed. Sixteen patients, aged 3 months to 18 years, met study criteria: 11 girls and 5 boys. The most common etiologies were severe central nervous system infection (n = 7, 44%) and hypoxic-ischemic event (n = 4, 25%). In 15 patients, diabetes insipidus was diagnosed during the first 3 days after acute central nervous system injury. Onset of cerebral salt wasting syndrome occurred 2-8 days after the onset of diabetes insipidus. In terms of outcome, 13 patients died (81%) and 3 survived under vegetative status (19%). Central diabetes insipidus and cerebral salt wasting syndrome may occur after acute central nervous system injury. A combination of both may impede accurate diagnosis. Proper differential diagnoses are critical, because the treatment strategy for each entity is different.

  16. Non-stroke Central Neurologic Manifestations in Antiphospholipid Syndrome.

    Science.gov (United States)

    Yelnik, Cécile M; Kozora, Elizabeth; Appenzeller, Simone

    2016-02-01

    Thrombotic manifestations of antiphospholipid syndrome (APS) are well known, and various non-stroke neuro-psychiatric manifestations (NPMs) have also been consistently described, but their place in APS remains unclear. Some syndromes, such as migraine or cognitive dysfunction, are frequently described in APS, whereas others, like seizure, multiple sclerosis-like symptoms, transverse myelitis, movement disorders, or psychiatric symptoms, are rarely found. Overlap with other autoimmune diseases, in particular with systemic lupus erythematosus, the lack of large sample size prospective studies, and discrepancies in antiphospholipid antibody (aPL) determinations complicate the study of the relationship between those disorders and aPL/APS. This review article aimed to summarize updated data on pathophysiologic, epidemiologic, and radiologic findings about non-stroke NPM described in primary APS and aPL-positive patients without overlap of other autoimmune diseases.

  17. The Riley-Day syndrome. Familial dysautonomy, central autonomic dysfunction.

    Science.gov (United States)

    François, J

    1977-01-01

    The Riley-Day syndrome is characterized by a dysfunction of the autonomous nervous system, sensory disturbances, neurological disorders, psychical anomalies and important ophthalmological symptoms, such as absence of tears, corneal anaesthesia, keratinized conjunctiva and cornea; myosis after instillation of methacholine. The diagnosis is based on the absence of fungiform papillae of the tongue and the absence of reaction after intradermic injection of histamine. The inheritance is autosomal recessive. The disease results probably from an enzymatic insufficiency.

  18. Bland-White-Garland syndrome of anomalous left coronary artery arising from the pulmonary artery (ALCAPA): a historical review

    Energy Technology Data Exchange (ETDEWEB)

    Cowles, Robert A. [Morgan Stanley Children' s Hospital of New York-Presbyterian, Division of Pediatric Surgery,Columbia University College of Physicians and Surgeons, New York, NY (United States); Berdon, Walter E. [Morgan Stanley Children' s Hospital of New York-Presbyterian, Department of Pediatric Radiology, Columbia University College of Physicians and Surgeons, New York, NY (United States)

    2007-09-15

    The landmark 1933 case report from Massachusetts General Hospital by Bland, White and Garland (Am Heart J 8:787-801) described a 3-month-old child with progressive feeding problems, cardiomegaly on chest radiography, and EKG evidence of left ventricular damage. Of interest was the fact that the vigilant father of the infant was Aubrey Hampton, a radiologist and future chairman of radiology at Massachusetts General Hospital. At autopsy, the left coronary artery originated from the pulmonary artery rather than from the aorta. Effective treatment for this condition was not available until 1960 when Sabiston, Neill and Taussig showed that the blood flowed from the left coronary artery toward the pulmonary artery. The anomalous left coronary artery was ligated at its junction with the pulmonary artery and the child survived. This historical review of Bland-White-Garland syndrome, now known as anomalous left coronary artery arising from the pulmonary artery (ALCAPA), stresses the continued diagnostic significance of cardiomegaly on chest radiography and EKG changes suggesting left ventricular damage in 2- to 3-month-old infants with feeding intolerance or irritability. With a high index of suspicion, an echocardiogram can be obtained to confirm the diagnosis. Modern surgical methods involve left coronary artery translocation and afford excellent outcomes. (orig.)

  19. [Long-term effects of sildenafil in a patient with scleroderma-associated pulmonary hypertension and Raynaud's syndrome].

    Science.gov (United States)

    Rosenkranz, S; Caglayan, E; Diet, F; Karasch, T; Weihrauch, J; Wassermann, K; Erdmann, E

    2004-08-13

    A 65-year-old woman was admitted because of dyspnea at rest and peripheral edema due to scleroderma-associated pulmonary fibrosis and hypertension, as well as Raynaud's phenomenon. She had a marked restrictive ventilatory disorder with severe impairment of diffusion capacity. Right heart catheterization demonstrated a mean pulmonary artery pressure of 50 mmHg. She was able to walk only 220 m. All usual methods of treatment failed to give satisfactory results so that sildenafil (phospherodiesterase type-5 |PDE-5| inhibitor; Viagra ((R)) was given, even though it is not licensed for this indications ("off-label", as a therapeutic attempt. This achieved definite reduction in pulmonary arterial pressure and significantly improved the clinical symptoms. In particular, it drastically reduced the level of atrial natriuretic peptide, an important prognostic marker in right heart failure. Sildenafil also significantly raised peripheral perfusion and the signs of Raynaud's syndrome. PDE-5 inhibitors are efficacious in scleroderma-associated pulmonary hypertension and may also provide a new option in the treatment of Raynaud's disease.

  20. Chiari malformation and central sleep apnea syndrome: efficacy of treatment with adaptive servo-ventilation

    Directory of Open Access Journals (Sweden)

    Jorge Marques do Vale

    2014-10-01

    Full Text Available The Chiari malformation type I (CM-I has been associated with sleep-disordered breathing, especially central sleep apnea syndrome. We report the case of a 44-year-old female with CM-I who was referred to our sleep laboratory for suspected sleep apnea. The patient had undergone decompressive surgery 3 years prior. An arterial blood gas analysis showed hypercapnia. Polysomnography showed a respiratory disturbance index of 108 events/h, and all were central apnea events. Treatment with adaptive servo-ventilation was initiated, and central apnea was resolved. This report demonstrates the efficacy of servo-ventilation in the treatment of central sleep apnea syndrome associated with alveolar hypoventilation in a CM-I patient with a history of decompressive surgery.

  1. Involvement of central serotonergic systems in dextromethorphan-induced behavioural syndrome in rats.

    Science.gov (United States)

    Gaikwad, R V; Gaonkar, R K; Jadhav, S A; Thorat, V M; Jadhav, J H; Balsara, J J

    2005-07-01

    Dextromethorphan, a noncompetitive blocker of the N-methyl-D-aspartate (NMDA) type of glutamate receptor, at 45, 60 and 75 mg/kg, ip doses induced a behavioural syndrome characterised by reciprocal forepaw treading, lateral head-weaving, hind-limb abduction and flat body posture. Such type of behavioural syndrome is induced by 8-hydroxy-2- (di-n-propylamino) tetralin (8-OH-DPAT) by directly stimulating the central postsynaptic 5-hydroxytryptamine (5-HT, serotonin) receptors of the 5-HT1A type. Pretreatment with buspirone (5, 10 mg/kg, ip) and l-propranolol (10, 20 mg/kg, ip) antagonised the behavioural syndrome induced by 8-OH-DPAT and dextromethorphan. Pretreatment with p-chlorophenylalanine (100 mg/kg/day x 4 days) antagonised the behavioural syndrome induced by dextromethorphan and dexfenfluramine but had no significant effect on 8-OH-DPAT induced behavioural syndrome. This indicates that dextromethorphan induces the behavioural syndrome by releasing 5-HT from serotonergic neurons with resultant activation of the postsynaptic 5-HT1A receptors by the released 5-HT. Pretreatment with fluoxetine (10 mg/kg, ip) significantly potentiated the behavioural syndrome induced by dextromethorphan and 5-hydroxytryptophan but significantly antagonised dexfenfluramine induced behavioural syndrome. This indicates that dextromethorphan releases 5-HT by a mechanism which differs from that of dexfenfluramine. Dextromethorphan may be releasing 5-HT by blocking the NMDA receptors and thereby counteracting the inhibitory influence of l-glutamate on 5-HT release.

  2. The primary Maxillary Central Incisor in the Solitary Median Maxillary Central Incisor syndrome

    DEFF Research Database (Denmark)

    Kjaer, I; Balslev-Olesen, M

    2012-01-01

    Solitary Median Maxillary Central Incisor (SMMCI) is a developmental anomaly in the permanent dentition with one single central incisor in the maxilla, positioned exactly in the midline. This condition has been associated with extra- and intraoral malformations in the frontonasal segment of the c...

  3. Sudden Unexplained Nocturnal Death Syndrome in Central China (Hubei)

    Science.gov (United States)

    Chen, Zhenglian; Mu, Jiao; Chen, Xinshan; Dong, Hongmei

    2016-01-01

    Abstract A retrospective study was conducted at Tongji Forensic Medical Center in Hubei (TFMCH) from 1999 to 2014. Forty-nine cases of sudden unexplained nocturnal death syndrome (SUNDS) were collected. The SUNDS rate was 1.0% in the total number of cases, in which an incidence was fluctuating over the years. Interestingly, April and January, and 3:00 to 6:00 am were the peak months and times of death. Among the decedents, farmers and migrant workers accounted for 67.3%. The syndrome predominantly attacked males in their 30s. One victim had sinus tachycardia. Thirteen victims (26.5%) were witnessed and had abnormal symptoms near death. Macroscopically, compared to sudden noncardiac deaths, the weights of brain, heart, and lungs had no statistical difference in SUNDS. Microscopically, the incidence of lung edema (45 cases, 91.8%) was significantly higher in SUNDS group than in the control group (27 cases, 55.1%). 82.9% of 35 SUNDS cases examined displayed minor histological anomalies of the cardiac conduction system (CCS), including mild or moderate fatty, fibrous or fibrofatty tissue replacement, insignificant stenosis of node artery, and punctate hemorrhage in the node area. These findings suggested that minor CCS abnormalities might be the substrates for some SUNDS deaths. Therefore, SUNDS victims might suffer ventricular fibrillation and acute cardiopulmonary failure before death. Further in-depth studies are needed to unveil the underlying mechanisms of SUNDS. PMID:26945374

  4. Impact of Restless Legs Syndrome on the Sleep Quality in Chronic Obstructive Pulmonary Disease

    Directory of Open Access Journals (Sweden)

    Erdal İn

    2016-04-01

    Full Text Available Objective: It is well known that various sleep disorders are common in cases of chronic obstructive pulmonary disease (COPD. However, restless legs syndrome (RLS has not been extensively studied in these patients. The aim of the current study was to investigate the prevalence of RLS and its impact on sleep quality in patients with COPD. Methods: The study included a total of 50 patients with COPD with a mean age of 67.2±7.7 years; 39 (78% were male. The RLS diagnosis was made based on the questionnaire items standardized by the International RLS study group. Dyspnea severity (mMRC, quality of life (CAT, sleep quality [Pittsburgh sleep quality index (PSQI], and daytime sleepiness [Epworth sleepiness scale (ESS] were evaluated using certain specific questionnaires. The subjects were studied in two groups: RLS-positive and RLS-negative groups. Results: In the study population, RLS was detected in 17 (34% patients. It was found that the RLS-positive subjects had a longer disease duration (p=0.006, a higher hospital admission rate (p=0.008, and lower spirometric values (p=0.023 for FVC; p=0.001 for FEV1. The CAT score was significantly higher in the RLS-positive group (p=0.019. The RLS-positive group had higher PSQI and ESS scores (p<0.001 for both. There were negative correlations between PSQI, ESS scores, and spirometric measures (FVC and FEV1, whereas PSQI and ESS scores had positive correlations with disease duration, mMRC, and CAT scores. Conclusion: In our study, it was observed that RLS is a common condition in patients with COPD. As the duration and severity of COPD increases, RLS becomes more prevalent and sleep quality deteriorates.

  5. Pulmonary embolism excluded acute coronary syndrome by coronarography: a retrospective analysis

    Institute of Scientific and Technical Information of China (English)

    LIU Chun-ping; LI Jun-xia; NIU Li-li; CHEN Hang-wei; TAN Bo; WANG Ya-ping

    2012-01-01

    Background Pulmonary embolism (PE) is often mistaken as acute coronary syndromes (ACS) because of the considerable overlap in their clinical features.We evaluated the factors causing misdiagnosis of PE as ACS and factors that differentiate PE from ACS to improve the diagnosis efficacy of PE.Methods The medical records of 22 consecutive PE patients,between 2001 and 2010,who were initially suspected of ACS were retrieved.ACS was ruled out by coronary artery angiography before a definite diagnosis of PE was given.Twenty-two contemporary cases of ACS matched by age and sex were recruited as controls.Clinical manifestations,electrocardiograms (ECG),and biomarkers of these patients were reviewed retrospectively.The factors causing misdiagnosis of PE as ACS and factors differentiating PE from ACS were evaluated.Results We found two leading causes of misdiagnosis of PE as ACS.One is that PE can resemble ACS in several clinical aspects (symptoms and signs,ECG findings,plasma cardiac troponin I,and D-dimer).The other is the insufficient recognition of PE by clinicians.Risk factors for venous thromboembolism (VTE),especially deep venous thrombosis (DVT),together with signs of PE,such as unexplained dyspnea or hypoxemia,and right ventricular pressure overload on ECGs are valuable in differentiating the two diseases.Conclusions Differentiation between PE and ACS is sometimes challenging.Adequate awareness of the risk factors for VTE and the signs of PE are crucial in the diagnosis of PE.

  6. Nociceptive transmission and modulation via P2X receptors in central pain syndrome.

    Science.gov (United States)

    Kuan, Yung-Hui; Shyu, Bai-Chuang

    2016-05-26

    Painful sensations are some of the most frequent complaints of patients who are admitted to local medical clinics. Persistent pain varies according to its causes, often resulting from local tissue damage or inflammation. Central somatosensory pathway lesions that are not adequately relieved can consequently cause central pain syndrome or central neuropathic pain. Research on the molecular mechanisms that underlie this pathogenesis is important for treating such pain. To date, evidence suggests the involvement of ion channels, including adenosine triphosphate (ATP)-gated cation channel P2X receptors, in central nervous system pain transmission and persistent modulation upon and following the occurrence of neuropathic pain. Several P2X receptor subtypes, including P2X2, P2X3, P2X4, and P2X7, have been shown to play diverse roles in the pathogenesis of central pain including the mediation of fast transmission in the peripheral nervous system and modulation of neuronal activity in the central nervous system. This review article highlights the role of the P2X family of ATP receptors in the pathogenesis of central neuropathic pain and pain transmission. We discuss basic research that may be translated to clinical application, suggesting that P2X receptors may be treatment targets for central pain syndrome.

  7. Pulmonary contusion and acute respiratory distress syndrome (ARDS) as complications of blunt chest trauma

    National Research Council Canada - National Science Library

    Michalska, Agata; Jurczyk, Agnieszka P; Machała, Waldemar; Szram, Stefan; Berent, Jarosław

    2009-01-01

    .... The authors of the article would like to emphasize the pathophysiology and diagnostic difficulties in such blunt chest trauma complications as pulmonary contusions and acute respiratory distress...

  8. Central obesity measurements predict metabolic syndrome in a retrospective cohort study of postmenopausal women

    Directory of Open Access Journals (Sweden)

    Manuel Rosety-Rodríguez

    2013-12-01

    Full Text Available Introduction: The various diagnostic classifications in the literature concur as regards the important role of abdominal obesity in the onset and progression of metabolic syndrome. Accordingly, this study was aimed at clarifying whether central obesity measurements assessed by dual X-ray absorptiometry (DXA may predict metabolic syndrome in Spanish postmenopausal women. Material and methods: This historical cohort study included a total of 1326 postmenopausal women aged > 45 years old who had routinely undergone DXA to measure their bone mineral density between january 2006 and january 2011. The regions of interest (ROI envisaged in our study by using DXA were the lumbar regions L1-L4 and L4-L5. At the same time, they underwent a complete medical examination including personal medical history assessment, biochemical blood analysis, blood pressure measurement and anthropometrical evaluation. Metabolic syndrome was diagnosed attending to the criteria established by National Cholesterol Education Program Adult Treatment Panel III (NECP-ATP-III. Results: During the observation period, 537 women, representing 40.5% of the total studied, met the diagnostic criteria for metabolic syndrome. L1-L4 and L4-L5 abdominal fat mass determinations were associated with the development of metabolic syndrome in all regression models tested, showing an increasing gradient from the lowest to highest quintile. Conclusion: Central adiposity measurements assessed by DXA, especially L1-L4 region of interest, could be considered a powerful predictor of metabolic syndrome in postmenopausal women.

  9. Chronic central serous chorioretinopathy as a presenting symptom of Cushing syndrome

    NARCIS (Netherlands)

    Dijk, E.H. van; Dijkman, G.; Biermasz, N.R.; Haalen, F.M. van; Pereira, A.M.; Boon, C.J.F.

    2016-01-01

    PURPOSE: To describe 4 patients who were diagnosed with chronic central serous chorioretinopathy (cCSC), which appeared to be the presenting symptom of Cushing syndrome (CS). METHODS: In this retrospective review of charts, all patients received extensive ophthalmologic examination and

  10. Theory of Mind and Central Coherence in Adults with High-Functioning Autism or Asperger Syndrome

    Science.gov (United States)

    Beaumont, Renae; Newcombe, Peter

    2006-01-01

    The study investigated theory of mind and central coherence abilities in adults with high-functioning autism (HFA) or Asperger syndrome (AS) using naturalistic tasks. Twenty adults with HFA/AS correctly answered significantly fewer theory of mind questions than 20 controls on a forced-choice response task. On a narrative task, there were no…

  11. Catheter-directed Thrombolysis in Acute Superior Vena Cava Syndrome Caused by Central Venous Catheters.

    Science.gov (United States)

    Cui, Jie; Kawai, Tasuo; Irani, Zubin

    2015-01-01

    Indwelling central venous catheters have been reported to increase the risk of superior venous cava (SVC) syndrome. This case report describes the development of acute SVC syndrome in a 28-year-old woman with end-stage renal disease implanted with a left-side hemodialysis reliable outflow graft and a right-side double lumen hemodialysis catheter via internal jugular veins. Her symptoms were not alleviated after catheter removal and systemic anticoagulation therapy. She was eventually treated with catheter-directed thrombolysis and a predischarge computer tomographic venogram on postthrombolytic procedure day 7 showed patent central veins and patient remained asymptomatic. This case demonstrates that catheter-directed thrombolysis can be safely employed to treat refractory catheter-induced acute SVC syndrome in end-stage renal disease patients.

  12. Pulmonary Neoplasms in Patients with Birt-Hogg-Dubé Syndrome: Histopathological Features and Genetic and Somatic Events.

    Science.gov (United States)

    Furuya, Mitsuko; Tanaka, Reiko; Okudela, Koji; Nakamura, Satoko; Yoshioka, Hiromu; Tsuzuki, Toyonori; Shibuya, Ryo; Yatera, Kazuhiro; Shirasaki, Hiroki; Sudo, Yoshiko; Kimura, Naoko; Yamada, Kazuaki; Uematsu, Shugo; Kunimura, Toshiaki; Kato, Ikuma; Nakatani, Yukio

    2016-01-01

    Birt-Hogg-Dubé syndrome (BHD) is an inherited disorder caused by genetic mutations in the folliculin (FLCN) gene. Individuals with BHD have multiple pulmonary cysts and are at a high risk for developing renal cell carcinomas (RCCs). Currently, little information is available about whether pulmonary cysts are absolutely benign or if the lungs are at an increased risk for developing neoplasms. Herein, we describe 14 pulmonary neoplastic lesions in 7 patients with BHD. All patients were confirmed to have germline FLCN mutations. Neoplasm histologies included adenocarcinoma in situ (n = 2), minimally invasive adenocarcinoma (n = 1), papillary adenocarcinoma (n = 1), micropapillary adenocarcinoma (n = 1), atypical adenomatous hyperplasia (n = 8), and micronodular pneumocyte hyperplasia (MPH)-like lesion (n = 1). Five of the six adenocarcinoma/MPH-like lesions (83.3%) demonstrated a loss of heterozygosity (LOH) of FLCN. All of these lesions lacked mutant alleles and preserved wild-type alleles. Three invasive adenocarcinomas possessed additional somatic events: 2 had a somatic mutation in the epidermal growth factor receptor gene (EGFR) and another had a somatic mutation in KRAS. Immunohistochemical analysis revealed that most of the lesions were immunostained for phospho-mammalian target of rapamycin (p-mTOR) and phospho-S6. Collective data indicated that pulmonary neoplasms of peripheral adenocarcinomatous lineage in BHD patients frequently exhibit LOH of FLCN with mTOR pathway signaling. Additional driver gene mutations were detected only in invasive cases, suggesting that FLCN LOH may be an underlying abnormality that cooperates with major driver gene mutations in the progression of pulmonary adenocarcinomas in BHD patients.

  13. Pulmonary Neoplasms in Patients with Birt-Hogg-Dube Syndrome: Histopathological Features and Genetic and Somatic Events.

    Directory of Open Access Journals (Sweden)

    Mitsuko Furuya

    Full Text Available Birt-Hogg-Dubé syndrome (BHD is an inherited disorder caused by genetic mutations in the folliculin (FLCN gene. Individuals with BHD have multiple pulmonary cysts and are at a high risk for developing renal cell carcinomas (RCCs. Currently, little information is available about whether pulmonary cysts are absolutely benign or if the lungs are at an increased risk for developing neoplasms. Herein, we describe 14 pulmonary neoplastic lesions in 7 patients with BHD. All patients were confirmed to have germline FLCN mutations. Neoplasm histologies included adenocarcinoma in situ (n = 2, minimally invasive adenocarcinoma (n = 1, papillary adenocarcinoma (n = 1, micropapillary adenocarcinoma (n = 1, atypical adenomatous hyperplasia (n = 8, and micronodular pneumocyte hyperplasia (MPH-like lesion (n = 1. Five of the six adenocarcinoma/MPH-like lesions (83.3% demonstrated a loss of heterozygosity (LOH of FLCN. All of these lesions lacked mutant alleles and preserved wild-type alleles. Three invasive adenocarcinomas possessed additional somatic events: 2 had a somatic mutation in the epidermal growth factor receptor gene (EGFR and another had a somatic mutation in KRAS. Immunohistochemical analysis revealed that most of the lesions were immunostained for phospho-mammalian target of rapamycin (p-mTOR and phospho-S6. Collective data indicated that pulmonary neoplasms of peripheral adenocarcinomatous lineage in BHD patients frequently exhibit LOH of FLCN with mTOR pathway signaling. Additional driver gene mutations were detected only in invasive cases, suggesting that FLCN LOH may be an underlying abnormality that cooperates with major driver gene mutations in the progression of pulmonary adenocarcinomas in BHD patients.

  14. Effects of Xuanbai Chengqi decoction on lung compliance for patients with exogenous pulmonary acute respiratory distress syndrome

    Directory of Open Access Journals (Sweden)

    Mao ZR

    2016-02-01

    Full Text Available Zhengrong Mao,1 Haifeng Wang2,3 1Department of Critical Care Medicine, The First Affiliated Hospital of Henan, University of Traditional Chinese Medicine, 2Department of Respiratory Medicine, The First Affiliated Hospital of Henan University of Traditional Chinese Medicine, 3Collaborative Innovation Center for Respiratory Disease Diagnosis and Treatment & Chinese Medicine Development of Henan Province, Zhengzhou City, Henan, People’s Republic of China Objective: To observe the effects of Xuanbai Chengqi decoction on lung compliance for patients with exogenous pulmonary acute respiratory distress syndrome. Subjects and methods: A total of 53 patients with exogenous pulmonary acute respiratory distress syndrome, who were admitted to the intensive care unit of the First Affiliated Hospital of Henan University of Traditional Chinese Medicine from March 2009 to February 2013, were selected. They were randomly divided into the treatment group (25 cases and the control group (28 cases. Both the groups were treated with conventional treatment and lung-protective ventilation strategy; apart from these, enema therapy with Xuanbai Chengqi decoction was given to the treatment group. Meanwhile, static lung compliance, dynamic lung compliance, peak airway pressure, plateau pressure, and positive end-expiratory pressure (PEEP for patients in both the groups were observed and recorded at 24, 48, and 72 hours after the drug was used. Moreover, variations in the duration of parenteral nutrition, incidence rate of complications, and case fatality rate in patients after treatment were recorded. Results: For patients in the treatment group, at 48 and 72 hours after treatment, the static lung compliance and dynamic lung compliance were significantly higher than those in the control group, while plateau pressure, peak airway pressure, and PEEP were significantly lower than those before treatment. At the same time, PEEP for patients in the treatment group at 72

  15. Pulmonary Thromboembolism in a Child with Sickle Cell Hemoglobin D Disease in the Setting of Acute Chest Syndrome

    OpenAIRE

    Hazel Villanueva; Sandeepkumar Kuril; Jennifer Krajewski; Aziza Sedrak

    2013-01-01

    Introduction. Sickle cell hemoglobin D disease (HbSD) is a rare variant of sickle cell disease (SCD). Incidence of pulmonary thromboembolism (PE) and deep venous thrombosis (DVT) in children with HbSD is unknown. PE and DVT are known complications of SCD in adults but have not been reported in the literature in children with HbSD. Case Report. We are reporting a case of a 12-year-old boy with HbSD with acute chest syndrome (ACS) complicated by complete thrombosis of the branch of the right pu...

  16. Anesthetic management of right atrial mass removal and pulmonary artery thrombectomy in a patient with primary antiphospholipid antibody syndrome

    Directory of Open Access Journals (Sweden)

    Rawat SKS

    2010-01-01

    Full Text Available Antiphospholipid antibody syndrome (APLAS characterises a clinical condition of arterial and venous thrombosis associated with phospholipids directed antibodies. APLAS occurs in 2% of the general population. However, one study demonstrated that 7.1% of hospitalised patients were tested positive for at least one of the three anticardiolipin antibody idiotype. Antiphospholipid antibodies often inhibit phospholipids dependent coagulation in vitro and interfere with laboratory testing of hemostasis. Therefore, the management of anticoagulation during cardiopulmonary bypass can be quite challenging in these patients. Here, we present a case of right atrial mass removal and pulmonary thrombectomy in a patient of APLAS.

  17. Severe Tumor Lysis Syndrome and Acute Pulmonary Edema Requiring Extracorporeal Membrane Oxygenation Following Initiation of Chemotherapy for Metastatic Alveolar Rhabdomyosarcoma.

    Science.gov (United States)

    Sanford, Ethan; Wolbrink, Traci; Mack, Jennifer; Rowe, R Grant

    2016-05-01

    We present an 8-year-old male with metastatic alveolar rhabdomyosarcoma (ARMS) who developed precipitous cardiopulmonary collapse with severe tumor lysis syndrome (TLS) 48 hr after initiation of chemotherapy. Despite no detectable pulmonary metastases, acute hypoxemic respiratory failure developed, requiring extracorporeal membrane oxygenation (ECMO). Although TLS has been reported in disseminated ARMS, this singular case of life-threatening respiratory deterioration developing after initiation of chemotherapy presented unique therapeutic dilemmas. We review the clinical aspects of this case, including possible mechanisms of respiratory failure, and discuss the role of ECMO utilization in pediatric oncology.

  18. An Isolated Pulmonary Hematoma Mimicking a Lung Tumor as the Initial Finding of Vascular Ehlers-Danlos Syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Kang, Eun Ju; Lee, Ki Nam; Choi, Pil Jo [Dept. of Radiology, Dong-A University Medicine Center, Dong-A University College of Medicine, Busan (Korea, Republic of); Ki, Chang Seok [Dept. of Radiology, Sungkyunkwan University School of Medicine, Seoul (Korea, Republic of)

    2012-09-15

    The vascular type of Ehlers-Danlos syndrome (vEDS) is an uncommon inherited disorder characterized by abnormalities in type III collagen, presenting itself as arterial dissection or rupture. We report a case of an isolated pulmonary hematoma mimicking a lung tumor in an 18-year-old man which turned out to be the initial finding of vEDS. Pneumothorax and hemothorax occurred repeatedly for 15 months following the surgical removal of the mass, and were treated by repeated left upper and lower lobectomy and thoracotomy. The diagnosis of vEDS was confirmed by pathologic and genetic studies.

  19. Progress in study on central nervous system injuries caused by obstructive sleep apnea syndrome

    Directory of Open Access Journals (Sweden)

    ZHAO Xiang-xiang

    2013-05-01

    Full Text Available Chronic and repetitive intermittent hypoxia and dysfunction of sleep architecture mainly contribute to obstructive sleep apnea syndrome (OSAS. More and more evidences demonstrate it is a systemic disease, which is common encountered in clinic and strongly related to the systemic lesion of central nervous system. The central nervous system complications comprise cognitive impairment, brain atrophy and the growing risk of stroke and so on. Early treatment for OSAS has a positive significance on complications of central nervous system, and even the damage can be completely reversed.

  20. B cell activating factor is central to bleomycin- and IL-17-mediated experimental pulmonary fibrosis.

    Science.gov (United States)

    François, Antoine; Gombault, Aurélie; Villeret, Bérengère; Alsaleh, Ghada; Fanny, Manoussa; Gasse, Paméla; Adam, Sylvain Marchand; Crestani, Bruno; Sibilia, Jean; Schneider, Pascal; Bahram, Seiamak; Quesniaux, Valérie; Ryffel, Bernhard; Wachsmann, Dominique; Gottenberg, Jacques-Eric; Couillin, Isabelle

    2015-01-01

    Idiopathic pulmonary fibrosis (IPF) is a progressive devastating, yet untreatable fibrotic disease of unknown origin. We investigated the contribution of the B-cell activating factor (BAFF), a TNF family member recently implicated in the regulation of pathogenic IL-17-producing cells in autoimmune diseases. The contribution of BAFF was assessed in a murine model of lung fibrosis induced by airway administered bleomycin. We show that murine BAFF levels were strongly increased in the bronchoalveolar space and lungs after bleomycin exposure. We identified Gr1(+) neutrophils as an important source of BAFF upon BLM-induced lung inflammation and fibrosis. Genetic ablation of BAFF or BAFF neutralization by a soluble receptor significantly attenuated pulmonary fibrosis and IL-1β levels. We further demonstrate that bleomycin-induced BAFF expression and lung fibrosis were IL-1β and IL-17A dependent. BAFF was required for rIL-17A-induced lung fibrosis and augmented IL-17A production by CD3(+) T cells from murine fibrotic lungs ex vivo. Finally we report elevated levels of BAFF in bronchoalveolar lavages from IPF patients. Our data therefore support a role for BAFF in the establishment of pulmonary fibrosis and a crosstalk between IL-1β, BAFF and IL-17A.

  1. Cerebral salt wasting syndrome in children with acute central nervous system injury.

    Science.gov (United States)

    Jiménez, Raquel; Casado-Flores, Juan; Nieto, Monserrat; García-Teresa, María Angeles

    2006-10-01

    The purpose of this investigation was to describe the causes, clinical pattern, and treatment of cerebral salt wasting syndrome in children with acute central nervous system injury. This retrospective study focused on patientscerebral salt wasting syndrome, over a period of 7 years, in the pediatric intensive care unit of a tertiary care hospital. Selection criteria included evidence of hyponatremia (serum sodium120 mEq/L), and volume depletion. Fourteen patients were identified with cerebral salt wasting syndrome, 12 after a neurosurgical procedure (8 brain tumor, 4 hydrocephalus) and 2 after severe brain trauma. In 11 patients the cerebral salt wasting syndrome was diagnosed during the first 48 hours of admission. Prevalence of cerebral salt wasting syndrome in neurosurgical children was 11.3/1000 surgical procedures. The minimum sodium was 122+/-7 mEq/L, the maximum urine osmolarity 644+/-59 mOsm/kgH2O. The maximum sodium supply was 1 mEq/kg/h (range, 0.1-2.4). The mean duration of cerebral salt wasting syndrome was 6+/-5 days (range 1-9). In conclusion, cerebral salt wasting syndrome can complicate the postoperative course of children with brain injury; it is frequently present after surgery for brain tumors and hydrocephalus and in patients with severe head trauma. Close monitoring of salt and fluid balance is essential to prevent severe neurologic and hemodynamic complications.

  2. The Role of Pulmonary Veins in Cancer Progression from a Computed Tomography Viewpoint

    Science.gov (United States)

    Chang, Hung; Liao, Tzu-Yao; Wen, Ming-Sheng; Yu, Chih-Teng

    2016-01-01

    Background. We studied the role of pulmonary veins in cancer progression using computed tomography (CT) scans. Methods. We obtained data from 260 patients with pulmonary vein obstruction syndrome (PVOS). We used CT scans to investigate pulmonary lesions in relation to pulmonary veins. We divided the lesions into central and peripheral lesions by their anatomical location: in the lung parenchymal tissue or pulmonary vein; in the superior or inferior pulmonary vein; and by unilateral or bilateral presence in the lungs. Results. Of the 260 PVOS patients, 226 (87%) had central lesions, 231 (89%) had peripheral lesions, and 190 (75%) had mixed central and peripheral lesions. Among the 226 central lesions, 93% had lesions within the superior pulmonary vein, either bilaterally or unilaterally. Among the 231 peripheral lesions, 65% involved bilateral lungs, 70% involved lesions within the inferior pulmonary veins, and 23% had obvious metastatic extensions into the left atrium. All patients exhibited nodules within their pulmonary veins. The predeath status included respiratory failure (40%) and loss of consciousness (60%). Conclusion. CT scans play an important role in following tumor progression within pulmonary veins. Besides respiratory distress, PVOS cancer cells entering centrally can result in cardiac and cerebral events and loss of consciousness or can metastasize peripherally from the pulmonary veins to the lungs.

  3. EVALUATION OF CHRONIC OBSTRUCTIVE PULMONARY DISEASE PATIENTS WITH OBSTRUCTIVE SLEEP APNOEA - OVERLAP SYNDROME

    Directory of Open Access Journals (Sweden)

    Vaddadi Sailendra

    2016-09-01

    , nasal airflow, tracheal breath sounds, thoracic wall movements, abdominal movements, transcutaneous oxygen saturation and body position. The sleep data recorded by the computer were manually scored for sleep stages, apnoeas and hypopnoeas. The sleep scoring was done according to R and K classification. In the study of the 36 COPD patients, 6 patients had mild COPD, 22 had moderate COPD and 8 had severe COPD. STATISTICAL ANALYSIS A Significant Correlation (p value <0.05 between Neck Circumference and RDI is found to exist. There is a small, but insignificant positive correlation between BMI and RDI. No Correlation is found to exist between Sleep Efficiency and RDI. A significant negative correlation (p value <0.05 is found to exist between PO2 and RDI in patients with Severe COPD. None of the other parameters have any significant correlation with RDI. RESULTS AND CONCLUSIONS Our study which consisted of COPD in different stages of severity showed the occurrence of overlap syndrome is 13.88%. We found that neck circumference per se is responsible for the increased AHI in COPD patients (r=0.381, p<0.05. 3 patients from Mild COPD and 2 patients from Moderate COPD had OSA (overlap syndrome. None of the Severe COPD patients had OSA. Pulmonary functions did not predict the occurrence of obstructive sleep apnoea in COPD and only Neck circumference per se contributes to the raised AHI in COPD patients.

  4. SYNDROME OF INADEQUATE SECRETION OF ANTIDIURETIC HORMONE ASSOCIATED TO PULMONARY THROMBOEMBOLISM: A NON-DESCRIBED CAUSE OF HYPONATREMIA IN THE LITERATURE.

    Directory of Open Access Journals (Sweden)

    Carlos G. Musso

    2008-01-01

    Full Text Available Syndrome of inadequate secretion of antidiuretic hormone (SIADH is a condition that can be associated to various sort of lung diseases.In this report we present a clinical case where a SIADH associated to pulmonary thromboembolism was documented. As long as we know, this association has not been described yet in the literature, and we hypothesized that lung cytokines released due to pulmonary necrosis could be the main stimulus to vasopressin secretion in this entity.Conclusion: Pulmonary thromboembolism should be incorporated in the list of lung causes of SIADH.

  5. Characteristics of pulmonary cysts in Birt-Hogg-Dube syndrome: Thin-section CT findings of the chest in 12 patients

    Energy Technology Data Exchange (ETDEWEB)

    Tobino, Kazunori, E-mail: tobino@juntendo.ac.jp [Department of Respiratory Medicine, Juntendo University, School of Medicine, 2-1-1 Hongo, Bunkyo-Ku, Tokyo 113-8421 (Japan); Gunji, Yoko [Department of Respiratory Medicine, Juntendo University, School of Medicine, 2-1-1 Hongo, Bunkyo-Ku, Tokyo 113-8421 (Japan); Kurihara, Masatoshi [Pneumothorax Center, Nissan Tamagawa Hospital, 4-8-1 Seta, Setagaya-Ku, Tokyo 158-0095 (Japan); The Study Group of Pneumothorax and Cystic Lung Diseases, 4-8-1 Seta, Setagaya-Ku, Tokyo 158-0095 (Japan); Kunogi, Makiko; Koike, Kengo [Department of Respiratory Medicine, Juntendo University, School of Medicine, 2-1-1 Hongo, Bunkyo-Ku, Tokyo 113-8421 (Japan); Tomiyama, Noriyuki [Department of Radiology, Osaka University Graduate School of Medicine, 2-2 Yamadaoka, Suita, Osaka 565-0871 (Japan); Johkoh, Takeshi [Department of Radiology, Kinki Central Hospital of Mutual Aid Association of Public School Teachers, Kurumazuka 3-1, Itami, Hyogo 664-0872 (Japan); Kodama, Yuzo [Department of Respiratory Medicine, Juntendo University, School of Medicine, 2-1-1 Hongo, Bunkyo-Ku, Tokyo 113-8421 (Japan); Iwakami, Shin-ichiro [Department of Respiratory Medicine, Juntendo University, Shizuoka Hospital, 1129 Nagaoka, Izunokuni, Shizuoka 410-2295 (Japan); Kikkawa, Mika [Biochemical Research Center, Division of Proteomics and Biomolecular Sciences, Juntendo University, Graduate School of Medicine, Bunkyo-Ku, Tokyo (Japan); Takahashi, Kazuhisa [Department of Respiratory Medicine, Juntendo University, School of Medicine, 2-1-1 Hongo, Bunkyo-Ku, Tokyo 113-8421 (Japan); Seyama, Kuniaki [Department of Respiratory Medicine, Juntendo University, School of Medicine, 2-1-1 Hongo, Bunkyo-Ku, Tokyo 113-8421 (Japan); Study Group of Pneumothorax and Cystic Lung Diseases, 4-8-1 Seta, Setagaya-Ku, Tokyo 158-0095 (Japan)

    2011-03-15

    Purpose: To describe in detail the characteristic chest computed tomography (CT) findings of Birt-Hogg-Dube (BHD) syndrome. Materials and methods: Thin-section chest CT scans of consecutive 12 patients with genetically diagnosed BHD syndrome were retrospectively evaluated by two observers, especially about the characteristics (distribution, number, size, shape and relation to pleura) of pulmonary cysts. Interobserver agreement in the identification of abnormalities on the CT images was achieved using the {kappa} statistic, and the degree of interobserver correlation for the characterization of pulmonary cysts was assessed using the Spearman rank correlation coefficient. Results: Multiple pulmonary cysts were seen in all patients. The number of cysts in each patient was various (range, 29-407), and cysts of various sizes (from a few mm to 2 cm or more) were seen in all patient. 76.6% (mean) of cysts were irregular-shaped, and 40.5% (mean) of cysts were located along the pleura. The mean extent score of cysts was 13% of the whole lung, and the distribution of cysts was predominantly in the lower medial zone. Finally, cysts abutting or including the proximal portions of lower pulmonary arteries or veins were also seen in all patients. Conclusion: Multiple, irregular-shaped cysts of various sizes with lower medial lung zone predominance are characteristic CT findings of BHD syndrome. Cysts abutting or including the proximal portions of lower pulmonary arteries or veins may also exist in this syndrome in a high probability.

  6. Decrease in pulmonary artery pressure after administration of thoracic epidural anesthesia in a patient with Marfan syndrome awaiting aortic valve replacement procedure.

    Science.gov (United States)

    Chakravarthy, Murali; Jawali, Vivek; Patil, Timmannagowda; Krishnamoorthy, Jayaprakash

    2011-08-01

    Thoracic epidural anesthesia is an adjunct to general anesthesia in cardiac surgery. Decrease in heart rate and blood pressure are frequently seen beneficial effects. There are several other hemodynamic effects of thoracic epidural anesthesia such as decrease in systemic vascular resistance, cardiac index, left ventricular stroke work index among others. However, the effect of thoracic epidural anesthesia on pulmonary artery pressure (PAP) has not been studied extensively in humans. Thoracic epidural anes-thesia decreased pulmonary artery pressure in experimen-tally induced pulmonary hypertension in animals. The mechanisms involved in such reduction are ill understood. We describe in this report, a significant reduction in PAP in a patient with Marfan's syndrome scheduled to under-go aortic valve replacement. The possible mechanisms of decrease in pulmonary artery pressure in the described case are, decrease in the venous return to the heart, decrease in the systemic vascular resistance, decrease in the right ventric-ular function and finally, improvement in myocardial contraction secondary to all the above. The possibility of Marfan's syndrome contributing to the decrease in PAP appears remote. The authors present this case to generate discussion about the possible mechanisms involved in thoracic epidural anesthesia producing beneficial effects in patients with secondary pulmonary hypertension. Thoracic epidural anesthesia appears to decrease pulmonary artery pressure by a combination of several mechanisms, some unknown to us. This occurrence, if studied and understood well could be put to clinical use in pulmonary hypertensives.

  7. Hemorrhagic Fever with Renal Syndrome in the New, and Hantavirus Pulmonary Syndrome in the Old World: paradi(se)gm lost or regained?

    Science.gov (United States)

    Clement, Jan; Maes, Piet; Van Ranst, Marc

    2014-07-17

    Since the first clinical description in 1994 of the so-called "Hantavirus Pulmonary Syndrome" (HPS) as a "newly recognized disease", hantavirus infections have always been characterized as presenting in two distinct syndromes, the so-called "Hemorrhagic Fever with Renal Syndrome" (HFRS) in the Old World, with the kidney as main target organ, in contrast to HPS in the New World, with the lung as main target organ. However, European literature mentions already since 1934 a mostly milder local HFRS form, aptly named "nephropathia epidemica" (NE), and caused by the prototype European hantavirus species Puumala virus (PUUV). Several NE reports dating from the 1980s and early 1990s described already non-cardiogenic HPS-like lung involvement, prior to any kidney involvement, and increasing evidence is now mounting that a considerable clinical overlap exists between HPS and HFRS. Moreover, growing immunologic insights point to common pathologic mechanisms, leading to capillary hyperpermeability, the cardinal feature of all hantavirus infections, both of the New and Old World. It is now perhaps time to reconsider the paradigm of two "different" syndromes caused by viruses of the same Hantavirus genus in the same Bunyaviridae family, and to agree on a common, more logical disease denomination, such as simply and briefly "Hantavirus fever".

  8. Central nervous system syndromes in solid organ transplant recipients.

    Science.gov (United States)

    Wright, Alissa J; Fishman, Jay A

    2014-10-01

    Solid organ transplant recipients have a high incidence of central nervous system (CNS) complications, including both focal and diffuse neurologic deficits. In the immunocompromised host, the initial clinical evaluation must focus on both life-threatening CNS infections and vascular or anatomic lesions. The clinical signs and symptoms of CNS processes are modified by the immunosuppression required to prevent graft rejection. In this population, these etiologies often coexist with drug toxicities and metabolic abnormalities that complicate the development of a specific approach to clinical management. This review assesses the multiple risk factors for CNS processes in solid organ transplant recipients and establishes a timeline to assist in the evaluation and management of these complex patients.

  9. Effect of transjugular intrahepatic portosystemic shunt on pulmonary gas exchange in patients with portal hypertension and hepatopulmonary syndrome

    Institute of Scientific and Technical Information of China (English)

    Graciela Martínez-Pallí; Britt B Drake; Joan-Carles García-Pagán; Joan-Albert Barberà; Miguel R Arguedas; Robert Rodriguez-Roisin; Jaume Bosch; Michael B Fallon

    2005-01-01

    AIM: To assess the impact of transjugular intrahepatic portosystemic shunt (TIPS) on pulmonary gas exchange and to evaluate the use of TIPS for the treatment of hepatopulmonary syndrome (HPS).METHODS: Seven patients, three of them with advanced HPS, in whom detailed pulmonary function tests were performed before and after TIPS placementat the University of Alabama Hospital and at the Hospital Clinic, Barcelona, were considered.RESULTS: TIPS patency was confirmed by hemodynamic evaluation. No changes in arterial blood gases were observed in the overall subset of patients. Transient arterial oxygenation improvement was observed in only one HPS patient, early after TIPS, but this was not sustained 4 mo later.CONCLUSION: TIPS neither improved nor worsened pulmonary gas exchange in patients with portal hypertension. This data does not support the use of TIPS as a specific treatment for HPS. However, it does reinforce the view that TIPS can be safely performed for the treatment of other complications of portal hypertension in patients with HPS.

  10. Studies on monitoring hemodynamics and oxygen dynamics of adult respiratory distress syndrome secondary to high altitude pulmonary edema

    Institute of Scientific and Technical Information of China (English)

    Ma Siqing; Wu Tianyi; Cheng Qiang; Li Pei; Bian Huiping

    2013-01-01

    To study monitoring hemodynamics and oxygen dynamics of adult respiratory distress syndrome (ARDS)secondary to high altitude pulmonary edema (HAPE),we performed clinic and laboratory studies in 8 patients who preliminarily developed high altitude cerebral edema (HACE) and then ARDS occurred at an altitude of 4500 m.After an initial emergency treatment on high mountains,all the patients were rapidly transported to a hospital at a lower altitude of 2808 m.The right cardiac catheterizations were carried out within 5 h after hospitalized.The monitoring hemodynamics and oxygen dynamics were studied via a thermodilution Swan-Gaze catheter.The results showed that before treatments at the beginning of monitoring,there presented a significant pulmonary artery hypertension with a decreased cardiac function,and a lower oxygen metabolism in all the 8 patients.However,after some effective treatments,including mechanical ventilation and using dexamethasone,furosemide,etc,four days later the result of a repeated monitoring showed that their pulmonary artery pressure had been decreased with an improved cardiac function with all the oxygen metabolic indexes increased significantly.Our studies suggested that performing monitoring hemodynamics in patients with ARDS secondary to HAPE will define the clinical therapeutic measures which will benefit the outcome.

  11. The Role of Inspiratory Muscle Training in Sickle Cell Anemia Related Pulmonary Damage due to Recurrent Acute Chest Syndrome Attacks

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    Burcu Camcıoğlu

    2015-01-01

    Full Text Available Background. The sickling of red blood cells causes a constellation of musculoskeletal, cardiovascular, and pulmonary manifestations. A 32-year-old gentleman with sickle cell anemia (SCA had been suffering from recurrent acute chest syndrome (ACS. Aim. To examine the effects of inspiratory muscle training (IMT on pulmonary functions, respiratory and peripheral muscle strength, functional exercise capacity, and quality of life in this patient with SCA. Methods. Functional exercise capacity was evaluated using six-minute walk test, respiratory muscle strength using mouth pressure device, hand grip strength using hand-held dynamometer, pain using Visual Analogue Scale, fatigue using Fatigue Severity Scale, dyspnea using Modified Medical Research Council Scale, and health related quality of life using European Organization for Research and Treatment of Cancer QOL measurement. Results. A significant improvement has been demonstrated in respiratory muscle strength, functional exercise capacity, pain, fatigue, dyspnea, and quality of life. There was no admission to emergency department due to acute chest syndrome in the following 12 months after commencing regular erythrocytapheresis. Conclusion. This is the first report demonstrating the beneficial effects of inspiratory muscle training on functional exercise capacity, respiratory muscle strength, pain, fatigue, dyspnea, and quality of life in a patient with recurrent ACS.

  12. Envolvimento pulmonar na síndrome de Sweet paraneoplásica Pulmonary involvement in paraneoplastic Sweet's syndrome

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    Marcelo Pavesi

    2006-12-01

    Full Text Available Síndrome de Sweet (dermatose neutrofílica febril aguda é caracterizada por lesões cutâneas inflamatórias acompanhadas por febre, leucocitose e mal-estar, devendo ser reconhecida por hematologistas, já que freqüentemente é paraneoplásica. O envolvimento sistêmico pode estar presente e o comprometimento pulmonar, apesar de raro, tem sido reportado na forma de infiltração pulmonar bilateral, bronquiolite obliterante e derrame pleural. Há infiltração neutrofílica densa na histologia. Apresentamos o caso de uma paciente com leucemia mielóide crônica que desenvolveu a síndrome de Sweet com infiltrações pulmonares não infecciosas, as quais não foram responsivas à antibioticoterapia, porém mostraram melhora clínica com a corticoterapia.Sweet's syndrome (acute febrile neutrophilic dermatosis is characterized by inflammatory skin lesions accompanied by fever, leukocytosis and malaise, it should be recognized by hematologists, because it can be a paraneoplastic manifestation. Systemic involvement may be present and lung lesions, despite of the rarity, have been reported in the form of bilateral pulmonary infiltrations, bronchiolitis obliterans and pleural effusion. Histopathology shows dense neutrophilic infiltrates. We present a patient with chronic myeloid leukemia who developed Sweet's syndrome with bilateral pulmonary infiltrations, which were non-responsive to antibiotics but showed clinical improvement on steroid therapy.

  13. Pulmonary Valve Stenosis

    Science.gov (United States)

    ... growths called carcinoid tumors in the digestive system. Rheumatic fever. This complication of an infection caused by streptococcus ... valve stenosis later in life, including: Carcinoid syndrome Rheumatic fever Noonan's syndrome Mild to moderate pulmonary valve stenosis ...

  14. Mechanisms of pulmonary cyst pathogenesis in Birt-Hogg-Dube syndrome: The stretch hypothesis.

    Science.gov (United States)

    Kennedy, John C; Khabibullin, Damir; Henske, Elizabeth P

    2016-04-01

    Loss-of-function mutations in the folliculin gene (FLCN) on chromosome 17p cause Birt-Hogg-Dube syndrome (BHD), which is associated with cystic lung disease. The risk of lung collapse (pneumothorax) in BHD patients is 50-fold higher than in the general population. The cystic lung disease in BHD is distinctive because the cysts tend to be basilar, subpleural and lentiform, differentiating BHD from most other cystic lung diseases. Recently, major advances in elucidating the primary functions of the folliculin protein have been made, including roles in mTOR and AMPK signaling via the interaction of FLCN with FNIP1/2, and cell-cell adhesion via the physical interaction of FLCN with plakophilin 4 (PKP4), an armadillo-repeat containing protein that interacts with E-cadherin and is a component of the adherens junctions. In addition, in just the last three years, the pulmonary impact of FLCN deficiency has been examined for the first time. In mouse models, evidence has emerged that AMPK signaling and cell-cell adhesion are involved in alveolar enlargement. In addition, the pathologic features of human BHD cysts have been recently comprehensively characterized. The "stretch hypothesis" proposes that cysts in BHD arise because of fundamental defects in cell-cell adhesion, leading to repeated respiration-induced physical stretch-induced stress and, over time, expansion of alveolar spaces particularly in regions of the lung with larger changes in alveolar volume and at weaker "anchor points" to the pleura. This hypothesis ties together many of the new data from cellular and mouse models of BHD and from the human pathologic studies. Critical questions remain. These include whether the consequences of stretch-induced cyst formation arise through a destructive/inflammatory program or a proliferative program (or both), whether cyst initiation involves a "second hit" genetic event inactivating the remaining wild-type copy of FLCN (as is known to occur in BHD-associated renal cell

  15. Periodic fever syndromes in Eastern and Central European countries: results of a pediatric multinational survey

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    Stefan Mariana

    2010-12-01

    Full Text Available Abstract Objective To analyze the prevalence of diagnosed and suspected autoinflammatory diseases in Eastern and Central European (ECE countries, with a particular interest on the diagnostic facilities in these countries. Methods Two different strategies were used to collect data on patients with periodic fever syndromes from ECE countries- the Eurofever survey and collection of data with the structured questionnaire. Results Data from 35 centers in 14 ECE countries were collected. All together there were 11 patients reported with genetically confirmed familial Mediterranean fever (FMF, 14 with mevalonate-kinase deficiency (MKD, 11 with tumor necrosis factor receptor associated periodic syndrome (TRAPS and 4 with chronic infantile neurological cutaneous and articular syndrome (CINCA. Significantly higher numbers were reported for suspected cases which were not genetically tested. All together there were 49 suspected FMF patients reported, 24 MKD, 16 TRAPS, 7 CINCA and 2 suspected Muckle-Wells syndrome (MWS patients. Conclusions The number of genetically confirmed patients with periodic fever syndromes in ECE countries is very low. In order to identify more patients in the future, it is important to organize educational programs for increasing the knowledge on these diseases and to establish a network for genetic testing of periodic fever syndromes in ECE countries.

  16. Thin-section chest CT findings of primary Sjoegren's syndrome: correlation with pulmonary function

    Energy Technology Data Exchange (ETDEWEB)

    Taouli, Bachir; Mourey, Isabelle [Department of Radiology, Universite Pierre et Marie Curie, Paris (France); Brauner, Michel W.; Lemouchi, Djamel [Department of Radiology, Universite Paris XIII, Bobigny (France); Grenier, Philippe A. [Department of Radiology, Universite Pierre et Marie Curie, Paris (France); Institut National de la Sante et de la Recherche Medicale, Universite Pierre et Marie Curie, Paris (France)

    2002-06-01

    The purpose of this study was to describe thin-section CT findings of lung involvement in patients with primary Sjoegren's syndrome (PSS), and to correlate them with pulmonary function tests (PFT). The chest thin-section CT examinations of 35 patients with proven diagnosis of PSS and respiratory symptoms were retrospectively assessed by two observers, in a first step independently with interobserver evaluation, and in a second step in consensus. The extent of the most frequent CT findings was scored. Correlation was made with PFT in 31 of these patients. Three main CT patterns were identified with good interobserver agreement (kappa coefficient 0.71): 19 of 35 (54%) large and/or small airways disease; 7 of 35 (20%) interstitial lung fibrosis (ILF); and 5 of 35 (14%) suggestive of lymphocytic interstitial pneumonia (LIP). The CT scans were normal in 2 patients (6%) and showed only dilatation of pulmonary vessels due to pulmonary arterial hypertension in two others (6%). Airway disease patients had predominantly obstructive profiles (mean FEV{sub 1}/FVC ratio 69.7{+-}12.7%, mean MEF{sub 25} 50.1{+-}22.9%), whereas patients with ILF and LIP had predominantly restrictive profiles and/or a decreased diffusing lung capacity (mean TLC 87.0{+-}26.0 and 64.6{+-}18.6%, mean DL{sub CO} 57.4{+-}21.2 and 52.0{+-}8.0%). Significant correlation (p<0.01) was found between the scores of ground-glass attenuation and TLC (r=-0.84) and DL{sub CO} (r=-0.70) and between the score of air trapping and FEV1 (r=-1.0). In patients with PSS and respiratory symptoms, thin-section CT may provide characterization of lung involvement which correlates with pulmonary function. (orig.)

  17. Prenatal diagnosis and outcome of absent pulmonary valve syndrome: contemporary single-center experience and review of the literature.

    Science.gov (United States)

    Wertaschnigg, D; Jaeggi, M; Chitayat, D; Shannon, P; Ryan, G; Thompson, M; Yoo, S J; Jaeggi, E

    2013-02-01

    To review the anomaly spectrum of prenatally detected absent pulmonary valve syndrome (APVS) and the outcome after diagnosis. Previous fetal studies reported survival rates of ≤ 25% for patients with intended postnatal care. Clinical data and echocardiograms of 12 cases with a fetal diagnosis of APVS between 2000 and 2010 were analyzed in this retrospective single-center study. Collected parameters included: gestational age at referral, associated fetal abnormalities, cardiothoracic ratio, maximum diameters of pulmonary annulus and main and branch pulmonary arteries, ventricular dimensions and function as well as ventricular Doppler flows. Karyotyping included fluorescence in-situ hybridization (FISH) analysis for microdeletion 22q11.2. Median gestational age at diagnosis was 24 weeks. Three subtypes of APVS were observed: (1) with tetralogy of Fallot (TOF) and no arterial duct (n = 10; 83%); (2) isolated, with a large arterial duct (n = 1; 8%); and (3) with tricuspid atresia, right ventricular dysplasia and a restricted duct (n = 1; 8%). The cardiothoracic ratio and pulmonary artery dimensions were increased in all cases. The karyotype was abnormal in 70% of fetuses with TOF and their mortality rate was significantly higher due to pregnancy termination (n = 3) or perinatal demise (n = 2) (hazard ratio, 5; 95% CI, 0.87-28.9; P = 0.015). Of seven live births with active postnatal care, six children (86%) were alive without residual respiratory symptoms at a median follow-up of 4.7 (range, 2.1-10.6) years. Outcome after fetal diagnosis of APVS was significantly better in this study compared with those of previous fetal series, with a low mortality rate for actively managed patients. Copyright © 2012 ISUOG. Published by John Wiley & Sons, Ltd.

  18. Flash pulmonary edema in patients with renal artery stenosis--the Pickering Syndrome

    DEFF Research Database (Denmark)

    Pelta, Anna; Andersen, Ulrik B; Just, Sven

    2010-01-01

    We report the prevalence of flash pulmonary edema in patients consecutively referred for balloon angioplasty of uni- or bilateral renal artery stenosis (PTRA), and describe the characteristics of this special fraction of the patients. We further report two unusual cases.......We report the prevalence of flash pulmonary edema in patients consecutively referred for balloon angioplasty of uni- or bilateral renal artery stenosis (PTRA), and describe the characteristics of this special fraction of the patients. We further report two unusual cases....

  19. Multifocal central serous chorioretinopathy with photoreceptor-retinal pigment epithelium diastasis in heritable pulmonary arterial hypertension

    DEFF Research Database (Denmark)

    Li, Xiao Qiang; Pryds, Anders; Carlsen, Jørn;

    2015-01-01

    with clinical examination, enhanced depth optical coherence tomography, fluorescein and indocyanine green angiography, and fundus photography. RESULTS: At presentation, atypical central serous chorioretinopathy with multiple retinal pigment epithelial detachments, a thick subfoveal choroid, and dilated...

  20. [Molecular genetics of familial tumour syndromes of the central nervous system].

    Science.gov (United States)

    Murnyák, Balázs; Szepesi, Rita; Hortobágyi, Tibor

    2015-02-01

    Although most of the central nervous system tumours are sporadic, rarely they are associated with familial tumour syndromes. These disorders usually present with an autosomal dominant inheritance and neoplasia develops at younger age than in sporadic cases. Most of these tumours are bilateral, multiplex or multifocal. The causative mutations occur in genes involved in cell cycle regulation, cell growth, differentiation and DNA repair. Studying these hereditary cancer predisposition syndromes associated with nervous system tumours can facilitate the deeper understanding of the molecular background of sporadic tumours and the development of novel therapeutic agents. This review is an update on hereditary tumour syndromes with nervous system involvement with emphasis on molecular genetic characteristics and their clinical implications.

  1. Central Retinal Artery Occlusion in a Patient with Metabolic Syndrome X

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    Sonja Predrag Cekić

    2010-01-01

    Full Text Available Purpose: To report a case of central retinal artery occlusion (CRAO in a patient with metabolic syndrome X. Case Report: A 64 year-old-man presented with abrupt, painless, and severe loss of vision in his left eye. Indirect ophthalmoscopy disclosed signs compatible with CRAO and laboratory investigations revealed erythrocyte sedimentation rate of 74 mm/h, C-reactive protein (CRP level of 21 mg/l, hyperglycemia, hyperuricemia, hypertriglyceridemia and hypercholesterolemia. Fluorescein angiography and immunological studies excluded other systemic disorders. The patient met the full criteria of the National Cholesterol Education Program for metabolic syndrome X. Conclusion: In addition to different vascular complications such as stroke, and cardiovascular disease, metabolic syndrome X may be associated with retinal vascular occlusions.

  2. Ramsay Hunt Syndrome Associated with Central Nervous System Involvement in an Adult

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    Tommy L. H. Chan

    2016-01-01

    Full Text Available Ramsay Hunt syndrome associated with varicella zoster virus reactivation affecting the central nervous system is rare. We describe a 55-year-old diabetic female who presented with gait ataxia, right peripheral facial palsy, and painful vesicular lesions involving her right ear. Later, she developed dysmetria, fluctuating diplopia, and dysarthria. Varicella zoster virus was detected in the cerebrospinal fluid by polymerase chain reaction. She was diagnosed with Ramsay Hunt syndrome associated with spread to the central nervous system. Her facial palsy completely resolved within 48 hours of treatment with intravenous acyclovir 10 mg/kg every 8 hours. However, cerebellar symptoms did not improve until a tapering course of steroid therapy was initiated.

  3. Two uncommon manifestations of leptospirosis:Sweet’s syndrome and central nervous system vasculitis

    Institute of Scientific and Technical Information of China (English)

    Peter George

    2011-01-01

    To leptospirosis is the commonest spirocheatal infection in the tropical and temperate countries of Indian sub-continent and Africa and the most common zoonosis worldwide. The protean manifestation of this infectious disease is a challenge for practising clinicians across the world. In poor developing countries, at most clinical suspicion it is essential in the diagnosis of this disease. In this report, we are able to document two uncommon manifestations of leptospirosis, namely Sweet’s syndrome and central nervous system vasculitis.

  4. Preschool weight and body mass index in relation to central obesity and metabolic syndrome in adulthood.

    Science.gov (United States)

    Graversen, Lise; Sørensen, Thorkild I A; Petersen, Liselotte; Sovio, Ulla; Kaakinen, Marika; Sandbaek, Annelli; Laitinen, Jaana; Taanila, Anja; Pouta, Anneli; Järvelin, Marjo-Riitta; Obel, Carsten

    2014-01-01

    If preschool measures of body size routinely collected at preventive health examinations are associated with adult central obesity and metabolic syndrome, a focused use of these data for the identification of high risk children is possible. The aim of this study was to test the associations between preschool weight and body mass index (BMI) and adult BMI, central obesity and metabolic alterations. The Northern Finland Birth Cohort 1966 (NFBC1966) (N = 4111) is a population-based cohort. Preschool weight (age 5 months and 1 year) and BMI (age 2-5 years) were studied in relation to metabolic syndrome as well as BMI, waist circumference, lipoproteins, blood pressure, and fasting glucose at the age of 31 years. Linear regression models and generalized linear regression models with log link were used. Throughout preschool ages, weight and BMI were significantly linearly associated with adult BMI and waist circumference. Preschool BMI was inversely associated with high-density lipoprotein levels from the age of 3 years. Compared with children in the lower half of the BMI range, the group of children with the 5% highest BMI at the age of 5 years had a relative risk of adult obesity of 6.2(95% CI:4.2-9.3), of adult central obesity of 2.4(95% CI:2.0-2.9), and of early onset adult metabolic syndrome of 2.5(95% CI:1.7-3.8). High preschool BMI is consistently associated with adult obesity, central obesity and early onset metabolic syndrome. Routinely collected measures of body size in preschool ages can help to identify children in need of focused prevention due to their increased risk of adverse metabolic alterations in adulthood.

  5. Bacteria-associated haemophagocytic syndrome and septic pulmonary embolism caused by Burkholderia cepacia complex in a woman with chronic granulomatous disease.

    Science.gov (United States)

    Hisano, Michi; Sugawara, Kana; Tatsuzawa, Osamu; Kitagawa, Michihiro; Murashima, Atsuko; Yamaguchi, Koushi

    2007-05-01

    Chronic granulomatous disease (CGD) is a primary immunodeficiency characterized by recurrent infections with certain types of bacteria and fungi. Presented herein is the case of a 29 year old woman with CGD who suffered from bacteria-associated haemophagocytic syndrome and a septic pulmonary embolism following a uterine infection and sepsis, caused by Burkholderia cepacia complex.

  6. Peripheral arterial blood pressure versus central crterial blood pressure monitoring in critically ill patients after Cardio-pulmonary Bypass.

    Science.gov (United States)

    Ahmad, Rana Altaf; Ahmad, Suhail; Naveed, Anjum; Baig, Mirza Ahmad Raza

    2017-01-01

    To determine the accuracy of peripheral (radial) arterial access as compared to central (femoral) arterial access for measurement of invasive blood pressure (IBP) in critically ill patients after cardiopulmonary bypass. Sixty patients (60) who required high inotropic/vasopressor support on weaning from cardio-pulmonary bypass and weaned off in 2(nd) attempt were included in this study. The duration of this study was from June 2015 to August 2016. Radial and femoral arterial access was achieved in all patients for simultaneous measurement of blood pressure. Arterial pressures were noted after 5, 15 and 30 minutes of weaning from cardiopulmonary bypass for both radial and femoral artery simultaneously. Mean age of study patients was 56.48±11.17 years. 85% patients were male. There was significant difference in systolic blood pressure, diastolic blood pressure and mean arterial pressures between the radial artery and femoral artery cannulation. Mean arterial pressures were significantly high in femoral artery as compared to the radial artery. The mean arterial pressures after five minutes of weaning using central access were 76.28±10.21 mmHg versus 64.15±6.76 mmHg in peripheral arterial access (p-value arterial pressures after 15 minutes of weaning from cardiopulmonary bypass 78.70±10.12 mmHg in central access versus 72.03±6.76 mmHg using peripheral arterial access (p-value arterial pressures were less marked as compared to the previous differences after 30 minutes of weaning from cardiopulmonary bypass as compared to the earlier readings (p-value 0.001). Peripheral arterial pressures are unreliable in critically ill patients after cardiopulmonary bypass receiving high dose of inotropic drugs. Central arterial access should be used in these patients to get accurate estimates of patients' blood pressure in early periods after cardiopulmonary bypass.

  7. The Rare Painful Phenomena - Chronic Paroxysmal Hemicrania-tic Syndrome as a Clinically Isolated Syndrome of the Central Nervous System.

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    Ljubisavljevic, Srdjan; Prazic, Ana; Lazarevic, Miodrag; Stojanov, Dragan; Savic, Dejan; Vojinovic, Slobadan

    2017-02-01

    The association of paroxysmal hemicrania with trigeminal neuralgia (TN) has been described and called paroxysmal hemicrania-tic syndrome (PH-tic). We report the case of a patient diagnosed as having chronic PH-tic (CPH-tic) syndrome as a clinically isolated syndrome of the central nervous system (CNS) (CIS).A forty year old woman was admitted to our hospital suffering from right facial pain for the last 2 years. The attacks were paroxysmal, neuralgiform, consisting of throb-like sensations, which developed spontaneously or were triggered by different stimuli in right facial (maxilar and mandibular) areas. Parallel with those, she felt a throbbing orbital and frontal pain with homolateral autonomic symptoms such as conjunctival injection, lacrimation, and the feeling that the ear on the same side was full. This pain lasted most often between 15 and 20 minutes. Beyond hemifacial hypoesthesia in the region of right maxilar and mandibular nerve, the other neurological finding was normal. Magnetic resonance imaging (MRI) study showed a T2-weighted multiple hyperintense paraventricular lesion and hyperintense lesion in the right trigeminal main sensory nucleus and root inlet, all of them being hypointense on T1-weighted image. All of these lesions were hypointense in gadolinium-enhanced T1-weighted images. Neurophysiological studies of trigeminal nerve (somatosensory evoked potentials and blink reflex) correlated with MRI described lesions. The patient's pain bouts were improved immediately after treatment with indomethacin, and were completely relieved with lamotrigine for a longer period. According to the actual McDonald's criteria, clinical state was defined as CIS which was clinically presented by CPH-tic syndrome.Even though it is a clinical rarity and its etiology is usually idiopathic, CPH-tic syndrome can also be symptomatic. When dealing with symptomatic cases, like the one described here, when causal therapy is not possible due to the nature of the primary

  8. MAGNETIC RESONANCE IMAGING OF ACUTE CENTRAL CORD SYNDROME: CORRELATION WITH PROGNOSIS

    Institute of Scientific and Technical Information of China (English)

    2001-01-01

    Objective. To report the magnetic resonance imaging (MRI) findings of acute cervical central cord syndrome and to determine their correlation with the prognosis. Methods. MRI findings of 35 patients with acute central cord syndrome were studied and compared with the recovery rate of ASIA score at presentation and in follow-up. Results. MRI data demonstrated spinal cord compression for 32 patients, spinal cord swelling for 16 patients, and abnormal signal intensity within the spinal cord for 19 patients, including 14 with edema and 3 with hematoma. No significant difference of the recovery rate was noted between the patients treated nonoperatively and operatively (P >0.05). There was a significant inverse correlation between the recovery rate and the degree of spinal cord compression as shown in MRI scans (P<0.01). The presence of hematoma in MRI scans was associated with poor prognosis, as demonstrated by a significant difference of the recovery rate (P< 0.01) among the patients with normal intensity, edema and hematoma within the spinal cord. Conclusions. MRI scans provide an efficient assistance for decision-making and accurate prognostic information regarding neurological function, and therefore should routinely be performed within the early phase of acute central cord syndrome.

  9. T Cells Are Not Required for Pathogenesis in the Syrian Hamster Model of Hantavirus Pulmonary Syndrome

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    Hammerbeck, Christopher D.; Hooper, Jay W.

    2011-01-01

    Andes virus (ANDV) is associated with a lethal vascular leak syndrome in humans termed hantavirus pulmonary syndrome (HPS). In hamsters, ANDV causes a respiratory distress syndrome closely resembling human HPS. The mechanism for the massive vascular leakage associated with HPS is poorly understood; however, T cell immunopathology has been implicated on the basis of circumstantial and corollary evidence. Here, we show that following ANDV challenge, hamster T cell activation corresponds with the onset of disease. However, treatment with cyclophosphamide or specific T cell depletion does not impact the course of disease or alter the number of surviving animals, despite significant reductions in T cell number. These data demonstrate, for the first time, that T cells are not required for hantavirus pathogenesis in the hamster model of human HPS. Depletion of T cells from Syrian hamsters did not significantly influence early events in disease progression. Moreover, these data argue for a mechanism of hantavirus-induced vascular permeability that does not involve T cell immunopathology. PMID:21775442

  10. Association of metabolic syndrome with chronic obstructive pulmonary disease in an Indian population

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    Amitava Acharyya

    2016-01-01

    Full Text Available Background and Aims: Chronic obstructive pulmonary disease (COPD is thought to have increased association with metabolic syndrome (MS which represents a cluster of factors that increase the risk of cardiovascular diseases and diabetes mellitus. However, the extent of association of COPD with MS and its individual components are still an unsettled issue, and it is likely to vary from population to population. Under the above context, this study was undertaken to investigate the association of MS and its components with COPD. Materials and Methods: With a cross-sectional analytic design, 77 COPD and an equal number of non-COPD (apparently healthy participants were studied purposively. The two groups were found to be matched by age, sex, and monthly income groups. The data of COPD patients and non-COPD participants were collected from a tertiary level hospital in Kolkata and a locality of Greater Kolkata, respectively. They were interviewed, and the frequencies of MS were assessed using 3 criteria (National Cholesterol Education Program-Third Adult Treatment Panel [NCEP ATP III], modified NCEP ATP III, and International Diabetic Federation [IDF]. Anthropometric measurements were taken, and fasting blood sample was collected to test the fasting blood glucose (FBG, triglyceride (TG, and high-density lipoprotein (HDL of respondents. Logistic regression was applied to estimate the odds ratio (OR. Results: Among the COPD subjects, 44%, 46%, and 31% had coexisting MS as defined by NCEP ATP III, modified NCEP ATP III, and IDF criteria, respectively. The corresponding percentages in the non-COPD groups were 31%, 38%, and 32%. On multivariate analysis, a significant association of MS (P < 0.015 with COPD was found only when the NCEP ATP III criteria were used. The (mean ± standard deviation FBG concentration among COPD and non-COPD groups was 130 ± 65 mg/dl and 97 ± 26 mg/dl, which was significantly different (P < 0.001. The difference in systolic blood

  11. HANTAVIRUS PULMONARY SYNDROME IN THE PROVINCE OF RIO NEGRO, ARGENTINA, 1993-1996

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    Gustavo CANTONI

    1997-07-01

    Full Text Available Early in 1995 the first case of Hantavirus Pulmonary Syndrome was serologically confirmed in El Bolsón (Province of Río Negro, Argentina, corresponding to the third outbreak reported in Argentina. A total of 26 cases of HPS related to the Andean region of Rio Negro Province, were reported from 1993 to 1996, 17 in El Bolsón, 4 in San Carlos de Bariloche, and 5 in Buenos Aires. The incidence rate was 5.03 x 100000 with a mortality rate of 51.85 x 100. The occurrence of cases was mainly seasonal, with a significantly greater number in the spring, and the persons affected mainly lived in urban or periurban areas. In four cases, the affected individuals were members of a couple, spouses or live-in contacts. Seven cases were Health workers (physicians, nurses or administrative staff. Twelve cases were related among them, due to an outbreak of 80 days. Two of them did not visit the Andean region. A total of 139 rodents were captured and seven of them, Olygoryzomys longicaudatus, were found to be serologically positive. The possibility of infection by contact with rodents or fecal matter is being analyzed and also hypothesis related with interhuman transmissionEm 1995, o primeiro caso de Síndrome Pulmonar pelo Hantavírus (HPS foi sorologicamente confirmado em El Bolsón (Província de Rio Negro, Argentina, correspondendo ao terceiro surto relatado na Argentina. Um total de 26 casos de HPS relacionados com a região Andina, Província de Rio Negro, foram relatados de 1993 a 1996, 17 em El Bolsón, 4 em São Carlos de Bariloche e 5 em Buenos Aires. O índice de incidência foi de 5,03 x 100.000 com índice de mortalidade de 51,85 x 100. A ocorrência de casos foi principalmente sasonal, com números significativamente maiores na primavera, e as pessoas afetadas viviam principalmente nas áreas urbanas e periurbanas. Em 4 casos, os indivíduos afetados eram membros de um casal, esposas ou viviam em contato. Sete casos eram trabalhadores da Saúde (m

  12. Systemic-pulmonary artery shunts in infants: modified Blalock-Taussig and central shunt procedures.

    Science.gov (United States)

    Brooks, Andre

    2014-01-01

    Access is gained through a midline sternotomy, the thymus partially excised and the superior part of the pericardium is opened. The innominate vein is retracted and the innominate artery is mobilized up to the bifurcation. The aorta is retracted to the left, the superior vena cavae to the right and the right atrial appendage inferiorly. The adventitia around the right pulmonary artery (PA) is dissected, taking care to incise the bulky pericardial reflection between the superior vena cavae and the trachea. Heparin is administrated. An occlusive clamp is applied to the right PA to test for haemodynamic tolerance prior to proceeding with the interposition of a suitable size artificial vascular prosthesis, based on the weight of the patient, between the innominate artery, or proximal subclavian artery and the right PA. Alternatively, if a sufficient main PA is present and adequate flow from a patent ductus arteriosus an end-to-side interposition shunt may be constructed between the ascending aorta and the main PA, provided the patient is stable with the test occlusion of the main PA. The management of the patent arterial ductus depends on whether or not there is forward flow through the PA.

  13. Editorial review: an update on central sensitivity syndromes and the issues of nosology and psychobiology.

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    Yunus, Muhammad B

    2015-01-01

    Central sensitization (CS), simply defined as an amplified response of the central nervous system to peripheral input, is a concept of great importance in clinical medicine. It has helped to explain aspects of the pathophysiology of common diseases, e.g. fibromyalgia syndrome (FMS), irritable bowel syndrome, vulvodynia, headaches, chronic pelvic pain and other overlapping conditions (collectively called central sensitivity syndromes, or CSS). It also applies to pain of complex regional pain syndrome, osteoarthritis (OA), rheumatoid arthritis (RA) and post-operative pain. The pathology-pain gap in CSS is readily explained by CS. Many FMS and other CSS patients have peripheral pathology, e.g. nociceptive areas in the muscles, arthritis, small fiber neuropathy and inflammation. Pro-inflammatory cytokines are elevated in some patients. Identification of CS in patients with structural pathology, e.g. OA and RA, has helped to explain why not all patients benefit from nonsteroidal anti-inflammatory drugs or joint replacement surgery, and require therapy directed at CS. Glial cells are important in pain processing. Remarkable advances have been achieved in neuroimaging, including visualization of grey matter and white matter, not only during provoked pain but also pain at rest. Based on CS mechanisms, targeted individual therapy may now be possible. Appropriate nosology is important particularly for effective patient care. Dichotomy of neurochemical-structural ("functional") and structural ("organic") pathology should be abandoned; many patients have both. Psychobiology is also biology. Patient-blaming terms like somatization, somatizer and catastrophizing should be avoided. For therapy, both pharmacological and non- pharmacological approaches are important, including recognition of subgroups and person/patient-centered care.

  14. Plasma Inflammatory Cytokine IL-4, IL-8, IL-10, and TNF-α Levels Correlate with Pulmonary Function in Patients with Asthma-Chronic Obstructive Pulmonary Disease (COPD) Overlap Syndrome.

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    Huang, Ai-Xia; Lu, Li-Wen; Liu, Wen-Juan; Huang, Mao

    2016-08-09

    BACKGROUND The aim of this study was to investigate the plasma inflammatory cytokine levels and their correlations with pulmonary function in patients with asthma-chronic obstructive pulmonary disease overlap syndrome (ACOS). MATERIAL AND METHODS Between January 2013 and December 2014, a total of 96 patients with asthma, acute exacerbation of chronic obstructive pulmonary disease (AECOPD), or ACOS were enrolled, and 35 healthy people were included as a control group. Fasting plasma interleukin (IL)-4, IL-8, IL-10, and tumor necrosis factor alpha (TNF-α) levels were detected using enzyme-linked immunosorbent assay (ELISA). Correlations between the plasma inflammatory cytokine levels and forced expiratory volume in 1 second (FEV1), FEV1/predicted value ratio (FEV1%pred), and FEV1/forced vital capacity (FVC) were analyzed. RESULTS IL-4 and IL-8 levels showed statistically significant differences among the 3 groups of patients (both PCOPD, and ACOS.

  15. Therapeutic Dilemmas Regarding Anticoagulation: An Experience in a Patient with Nephrotic Syndrome, Pulmonary Embolism, and Traumatic Brain Injury

    Science.gov (United States)

    Akimoto, Tetsu; Yamazaki, Tomoyuki; Kusano, Eiji; Nagata, Daisuke

    2016-01-01

    Patients with active bleeding complications who concomitantly develop overt pulmonary embolism (PE) present distinct therapeutic dilemmas, since they are perceived to be at substantial risk for the progression of the embolism in the absence of treatment and for aggravation of the hemorrhagic lesions if treated with anticoagulants. A 76-year-old patient with nephrotic syndrome, which is associated with an increased risk of thromboembolism, concurrently developed acute PE and intracranial bleeding because of traumatic brain injury. In this case, we prioritized the treatment for PE with the intravenous unfractionated heparin followed by warfarinization. Despite the transient hemorrhagic progression of the brain contusion after the institution of anticoagulation, our patient recovered favorably from the disease without any signs of neurological compromise. Several conundrums regarding anticoagulation that emerged in this case are also discussed. PMID:27840582

  16. Surgical treatment of tracheoesophageal fistula in a patient with severe acute respiratory syndrome complicated with extensive pulmonary fibrosis.

    Science.gov (United States)

    Lee, Jang-Ming; Lee, Pei-Lin; Kuo, Shuenn-Wen; Hwang, Juey-Jen; Lee, Yung-Chie

    2004-12-01

    Tracheoesophageal fistula is an uncommon complication usually associated with chronic usage of ventilator and pressure necrosis of the tracheoesophageal wall. A 56-year-old female patient with severe acute respiratory syndrome (SARS) with tracheoesophageal fistula is reported. She was intubated for ventilatory support 3 days after admission because of progressive respiratory distress. Methylprednisolone pulse therapy followed by a maintenance dosage was given due to persistence of bilateral pulmonary fibrosis. Thirty three days after admission, she underwent tracheostomy because of difficulty in weaning from the ventilator. Ten days after tracheostomy, she developed tracheoesophageal fistula, which was confirmed by bronchoscopy and panendoscopy. Tracheal resection and primary repair for the esophageal defect was performed via a cervical incision combined with partial sternotomy. She was weaned from the ventilator soon after the surgery and discharged 34 days after the operation. In SARS patients with persistent pulmonary fibrosis and under prolonged corticosteroid treatment, special care should be given to avoid intubation-related tracheal injury during the period of ventilatory support. The tracheoesophageal fistula, once developed, can be repaired in a single stage after improvement of the nutritional status.

  17. Primary Nephrotic Syndrome in Adults as a Risk Factor for Pulmonary Embolism: An Up-to-Date Review of the Literature

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    Aibek E. Mirrakhimov

    2014-01-01

    Full Text Available Patients with nephrotic syndrome are at an increased risk for thrombotic events; deep venous thrombosis, renal vein thrombosis, and pulmonary embolism are quite common in patients with nephrotic syndrome. It is important to note that nephrotic syndrome secondary to membranous nephropathy may impose a greater thrombotic risk for unclear reasons. Increased platelet activation, enhanced red blood cell aggregation, and an imbalance between procoagulant and anticoagulant factors are thought to underlie the excessive thrombotic risk in patients with nephrotic syndrome. The current scientific literature suggests that patients with low serum albumin levels and membranous nephropathy may benefit from primary prophylactic anticoagulation. A thorough approach which includes accounting for all additional thrombotic risk factors is, therefore, essential. Patient counseling regarding the pros and cons of anticoagulation is of paramount importance. Future prospective randomized studies should address the question regarding the utility of primary thromboprophylaxis in patients with nephrotic syndrome.

  18. Central retinal artery occlusion following laser treatment for ocular ischemic aortic arch syndrome

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    Shah, Payal J.

    2015-12-01

    Full Text Available Objective: Ocular ischemic syndrome is a rare blinding condition generally caused by disease of the carotid artery. We describe a 69-year-old female with a 50 pack-year smoking history with aortic arch syndrome causing bilateral ocular ischemic syndrome. Methods: The patient presented with progressive visual loss and temple pain. Slit lamp biomicroscopy revealed bilateral iris neovascularization. This finding prompted a cardiovascular work up. Panretinal photocoagulation with retrobulbar block was performed in the right eye. Results: A temporal artery biopsy was negative. The carotid duplex sound showed only a 1–39% stenosis. MRA revealed a more proximal occlusion of the aortic branch for which she underwent subclavian carotid bypass surgery. At the one month follow up, the right eye suffered profound vision loss secondary to a central retinal artery occlusion. Conclusion: Ocular neovascularization may be one of the clinical manifestations of aortic arch syndrome. This case also illustrates the limitations of relying solely on carotid duplex ultrasound testing. We caution against overly aggressive panretinal photocoagulation utilizing retrobulbar anesthesia.

  19. Chronic central serous chorioretinopathy as a presenting symptom of Cushing syndrome.

    Science.gov (United States)

    van Dijk, Elon H C; Dijkman, Greet; Biermasz, Nienke R; van Haalen, Femke M; Pereira, Alberto M; Boon, Camiel J F

    2016-08-04

    To describe 4 patients who were diagnosed with chronic central serous chorioretinopathy (cCSC), which appeared to be the presenting symptom of Cushing syndrome (CS). In this retrospective review of charts, all patients received extensive ophthalmologic examination and endocrinologic analyses. A 56-year-old man and a 49-year-old woman were treated because of bilaterally active, therapy-resistant cCSC. The clinical sign indicative for CS leading to referral to the endocrinologist was muscle weakness in the man and plethora in the woman. In a 37-year-old woman with known diabetes mellitus and central obesity, bilateral cCSC was diagnosed before CS screening. Another 49-year-old woman was treated for unilateral cCSC for 4 years. Complaints of fatigue, muscle weakness, central adiposity, and skin atrophy led to referral and a CS diagnosis due to bilateral macronodular adrenal hyperplasia. In all patients, CS surgery resulted in complete resolution of subretinal fluid. During postsurgical follow-up, no reactivation of cCSC was observed. Chronic CSC can be the principal manifestation of relatively mildly symptomatic and unrecognized CS. In patients with cCSC, ophthalmologists should have a high index of suspicion for clinical signs of CS that warrant endocrinologic analysis. Cushing syndrome surgery can stop active subretinal fluid leakage in cCSC.

  20. [The trigemino-cervical complex. Integration of peripheral and central pain mechanisms in primary headache syndromes].

    Science.gov (United States)

    Busch, V; Frese, A; Bartsch, T

    2004-10-01

    The activation of the trigeminal nociceptive system is the neural substrate of pain in primary headache syndromes such as migraine and cluster headache. The nociceptive inflow from the meninges to the spinal cord is relayed in brainstem neurones of the trigemino-cervical complex (TCC). Two important mechanisms of pain transmission are reviewed: convergence of nociceptive trigeminal and cervical afferents and sensitization of trigemino-cervical neurones. These mechanisms have clinical correlates such as hyperalgesia, allodynia, spread and referral of pain to trigeminal or cervical dermatomes. Neurones in the TCC are subject to a modulation of pain-modulatory circuits in the brainstem such as the periaqueductal grey (PAG). Recent experimental and clinical findings of a modulation of these pain processes are discussed. The review focuses on TCC neurones as integrative relay neurones between peripheral and central pain mechanisms. The understanding of these mechanisms has implications for the understanding of the clinical phenomenology in primary headache syndromes and the development of therapeutical options.

  1. Application of transpulmonary thermodilution monitoring (PiCCO in patient with neurogenic pulmonary edema and acute obstructive hydrocephalus due to a central neurocytoma: A case report

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    Ryoichi Iwata

    2017-06-01

    Full Text Available Neurogenic pulmonary edema (NPE is an acute life-threatening complication associated with many forms of central nervous system injury. Its pathophysiology is still debated. We report a patient with acute obstructive hydrocephalus due to a central neurocytoma who also had NPE, for which serial transpulmonary thermodilution monitoring (PiCCO was performed. Insertion of the PiCCO, which provides information about the patient's cardiac output, preload status and amount of lung water, revealed a high pulmonary vascular permeability index (PVPI and low global end-diastolic volume (GEDV without cardiac dysfunction, indicating permeability edema, which led to our changing the therapeutic strategy. Using PiCCO monitoring to balance the preload and extent of pulmonary edema enabled achievement of an optimal cardiac preload for organ perfusion, resulting in normalization of pulmonary edema by day 2. PiCCO facilitates understanding of the mechanism of NPE, guiding the management of fluid balance and the choice of vasopressors in patients with life-threatening NPE.

  2. Pneumocystis colonization, airway inflammation, and pulmonary function decline in acquired immunodeficiency syndrome.

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    Norris, Karen A; Morris, Alison; Patil, Sangita; Fernandes, Eustace

    2006-01-01

    As a result of improved diagnosis, treatment, and supportive care for HIV-infected patients, AIDS in developed countries has now become a chronic infection with prolonged survival time, but longterm complications are increasing contributors to morbidity and mortality. HIV-infected patients are at increased risk for the development of pulmonary complications, including chronic obstructive pulmonary disease (COPD); however, the mechanisms associated with this increased susceptibility have not been defined. Infectious agents may contribute to the development of COPD by upregulating inflammatory mediators in the lung that act in concert with cigarette smoke to promote lung pathology. Studies in human subjects and non-human primate models of AIDS suggest that the inflammatory response to asymptomatic carriage or colonization by the opportunistic pathogen, Pneumocystis sp. (Pc), is similar to that of COPD, which is characterized by influx of CD8+ T cells, neutrophils, and macrophages into the lungs. We have shown a high frequency of Pc colonization among asymptomatic HIV-infected subjects and in non-HIV infected subjects with COPD. To investigate the role of Pc in the progression of obstructive lung disease in HIV infections, we developed a non-human primate model of Pc colonizatoin and infection in simian immunodeficiency virus (SIV)-infected macaques. These animals develop a prolonged colonization state characterized by a persistent influx of CD8+ T cells and neutrophils, and local increases in IL-8, IFN-gamma, and TNF-alpha. SIV-infected Pc-colonized monkeys show progressive decline in pulmonary function compared to SIV-infected monkeys. We hypothesize that in the context of AIDS-immune dysfunction, Pc colonization induces inflammatory responses leading to changes in pulmonary function and architecture similar to that seen in emphysema. Information gained from these studies will lead to the development of interventions to prevent lung injury associated with Pc

  3. Osteoporosis as One of the Systematic Syndromes of the Chronic Obstructive Pulmonary Disease

    OpenAIRE

    Shevchuk-Budz, U. I.; Korzh, G. Z.

    2014-01-01

    The article highlights the questions related to the features of osteoporosis systemic symptoms in patients with chronic obstructive pulmonary disease (COPD). Osteoporosis and COPD are closely connected by pathogenesis, that is why it is necessary to take into account the severity of chronic systemic inflammation and system complications, the duration of the disease, patient’s clinical group, the action of harmful factors that influence on an origin and course of disease (smoking index), doses...

  4. Central Diabetes Insipidus and Cisplatin-Induced Renal Salt Wasting Syndrome: A Challenging Combination.

    Science.gov (United States)

    Cortina, Gerard; Hansford, Jordan R; Duke, Trevor

    2016-05-01

    We describe a 2-year-old female with a suprasellar primitive neuroectodermal tumor and central diabetes insipidus (DI) who developed polyuria with natriuresis and subsequent hyponatremia 36 hr after cisplatin administration. The marked urinary losses of sodium in combination with a negative sodium balance led to the diagnosis of cisplatin-induced renal salt wasting syndrome (RSWS). The subsequent clinical management is very challenging. Four weeks later she was discharged from ICU without neurological sequela. The combination of cisplatin-induced RSWS with DI can be confusing and needs careful clinical assessment as inaccurate diagnosis and management can result in increased neurological injury.

  5. Potential Central Nervous System Involvement in Sudden Unexpected Infant Deaths and the Sudden Infant Death Syndrome.

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    Thach, Bradley T

    2015-07-01

    Sudden unexpected infant death (SUID) in infancy which includes Sudden Infant Death Syndrome (SIDS) is the commonest diagnosed cause of death in the United States for infants 1 month to 1 year of age. Central nervous system mechanisms likely contribute to many of these deaths. We discuss some of these including seizure disorders, prolonged breath holding, arousal from sleep and its habituation, laryngeal reflex apnea potentiated by upper airway infection, and failure of brainstem-mediated autoresuscitation. In the conclusions section, we speculate how lives saved through back sleeping might result in later developmental problems in certain infants who otherwise might have died while sleeping prone.

  6. Diferencias regionales y Síndrome Pulmonar por Hantavirus (enfermedad emergente y tropical en Argentina Regional differences and Hantavirus pulmonary syndrome (an emerging and tropical disease in Argentina

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    Sergio Sosa-Estani

    2001-01-01

    Full Text Available Se describen algunos factores que habrían favorecido a caracterizar la expresión del Síndrome Pulmonar por Hantavirus en Argentina. Estos factores muestran diversos orígenes que van desde los procesos de ocupación del espacio y de producción, la estructura laboral, el patrón de migración humana, la etnia, la dinámica de reservorios y su relación con los tipos de virus, y el comportamiento del hombre. Estos factores se expresan en tres marcos ecológicos asociados a diferentes regiones geográficas de Argentina: 1 Noroeste, 2 Central (Pampa húmeda y 3 Sur Andina. Este complejo escenario obliga a abordar con la misma complejidad las investigaciones, para identificar determinantes primarios, biológicos, sociales y ambientales, causales de salud o enfermedad en su estrecha interacción y no individualmente. Este abordaje permitirá diseñar estrategias apropiadas para mejorar las condiciones de salud. Las mismas deberían ser diseñadas y transferidas por equipos transdisciplinarios de investigación, donde la participación de la comunidad desde las primeras etapas de desarrollo es esencial para la sustentabilidad de la estrategia.Factors related to the characteristics of Hantavirus pulmonary syndrome in Argentina are described. Factors from different scientific fields converge to form the syndrome's analytical framework. Some of these factors are the history of spatial occupation, work and production structures, human migration patterns, ethnic composition, reservoir dynamics and its relationship to the different circulating viruses, and human behavior. Furthermore, the multiple factors are expressed in three ecological frameworks, associated with three different geographical regions of Argentina: 1 Northwest; 2 Central ("wet Pampa"; and 3 South Andean. In order to understand the actual causality of health or disease as an interaction of many factors, research on the primary biological, social, and environmental determinants of

  7. 40. Hoarseness of voice as a primary presentation of pulmonary hypertension: A rare case of Ortner’s (cardio-vocal syndrome

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    K. Naeem

    2016-07-01

    Full Text Available Ortner’s (cardio-vocal syndrome is a rare disease entity that involves compression of the left recurrent laryngeal nerve due to enlargement of cardiovascular structures. The syndrome was first described by Norbert Ortner (an Austrian physician in 1897 and is usually reported to be associated with mitral stenosis. However, we report a rare case when a 20-year-old girl presented with hoarseness of voice and was found to have an enlarged main pulmonary trunk due to pulmonary hypertension that was compressing the left recurrent laryngeal nerve. The case highlights a rare presentation of a relatively common disease and stresses the importance of detailed cardiac workup in a patient with hoarseness of voice. We present the case and review the literature. A 20-year-old lady was referred from primary health center due to complaints of hoarseness of voice for the last four months. She reported only mild exertional palpitations and shortness of breath. She did not report any previous cardiac problem in childhood. Examination revealed a loud pulmonary component of 2nd heart sound, a pan-systolic murmur at the tricuspid area, and a palpable thrill. There was no evidence of right heart failure or any stigmata of connective tissue disease. Electrocardiography showed sinus rhythm with dominant R-wave in lead V1 indicating right ventricular hypertrophy. Laboratory tests did not reveal any abnormality. 2-D transthoracic echocardiograhy revealed normal left ventricle in size and function, right ventricular hypertrophy (6 mm thickness of free wall, mild to moderate tricuspid regurgitation, pulmonary hypertension (pulmonary pressure 60–65 mmHg and an enlarged main pulmonary trunk. Computed tomography (CT of the chest showed dilated main pulmonary trunk (max diameter 42 mm. An angiogram excluded pulmonary emboli. The patient was also seen by the Otolaryngology team in view of the main complaint of hoarseness of voice. Indirect laryngoscopy showed left vocal

  8. [A case of Avellis' syndrome with ipsilateral central facial palsy due to a small medullary infarction].

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    Takahashi, K; Kitani, M; Fukuda, H

    2000-04-01

    We report a 51-year-old man with mild left central facial palsy and left Avellis' syndrome due to a small medullary infarction. On admission, neurological examination revealed hoarseness, dysphasia, absent left gag reflex, palsies of the left vocal cord and left soft palate, and hypalgesia and thermohypesthesia on the right side of the trunk and extremities. In addition, he had a mild left central facial palsy. He had no nausea, vomiting, vertigo, hiccups, nystagmus, Horner's sign, facial numbness, or paresis or ataxia of the limbs. A T2 weighted MRI showed a small, high signal intensity area in the left dorsal region of the medulla and this lesion was presumed to involve the nucleus ambiguus and a part of the spinothalamic tract. These findings suggest that an aberrant supranuclear pathway, looping around the nucleus ambiguus to the facial nucleus exists in our patient.

  9. Clinical course, management and in-hospital outcomes of acute coronary syndrome in Central Asian women

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    Ravshanbek Kurbanov

    2011-03-01

    Full Text Available The paper describes features history, risk factors and hospital management of acute coronary syndrome (ACS in women of the Central Asia (829 ACS patients are included, and also comparison of hospital outcomes of ACS in groups of men and women.ACS in women in the Central Asia comprises features as more non Q-wave myocardial infarction frequency, smaller adherence to treatment of dyslipidemia, more obesity degrees. Women arrive to hospital average in 4 hours after men; have more percent of the atypical form. In-hospital outcomes in men and women significantly did not differ because of general tendency to late arriving to the hospital and small quantity of revascularization procedures. The reasons of differences between men and women in the region are mostly connected to social sphere (late arriving, non-treated dyslipidemia than to physiological peculiarities.

  10. Reduction of central neuropathic pain with ketamine infusion in a patient with Ehlers–Danlos syndrome: a case report

    Science.gov (United States)

    Lo, Tony Chung Tung; Yeung, Stephen Tung; Lee, Sujin; Skavinski, Kira; Liao, Solomon

    2016-01-01

    Objective Ehlers–Danlos syndrome frequently causes acute and chronic pain because of joint subluxations and dislocations secondary to hypermobility. Current treatments for pain related to Ehlers–Danlos syndrome and central pain syndrome are inadequate. This case report discusses the therapeutic use of ketamine intravenous infusion as an alternative. Case report A 27-year-old Caucasian female with a history of Ehlers–Danlos syndrome and spinal cord ischemic myelopathy resulting in central pain syndrome, presented with severe generalized body pain refractory to multiple pharmacological interventions. After a 7-day course of ketamine intravenous infusion under controlled generalized sedation in the intensive care unit, the patient reported a dramatic reduction in pain levels from 7–8 out of 10 to 0–3 out of 10 on a numeric rating scale and had a significant functional improvement. The patient tolerated a reduction in her pain medication regimen, which originally included opioids, gabapentin, pregabalin, tricyclic antidepressants, and nonsteroidal anti-inflammatory drugs. Conclusion Ketamine infusion treatment has been used in various pain syndromes, including central neuropathic pain, ischemic pain, and regional pain syndrome. Reports have suggested that ketamine modulates pain by the regression of N-methyl-D-aspartate receptor to a resting state. As such, propagation of nociceptive signal to brain is interrupted allowing for the restoration of physiological balance between pain inhibition and facilitation. The present report shows that this treatment option can be used in patients with refractory central pain syndrome in the setting of spinal cord myelopathy secondary to Ehlers–Danlos syndrome. In addition, as seen in this case, this protocol can potentially decrease the chronic use of pain medication, such as opioids.

  11. Mixed partial anomalous pulmonary venous drainage coexistent with an aortic valve abnormality – analysis of ultrasound diagnostics in a 10-year-old girl with Turner syndrome

    Science.gov (United States)

    Karolczak, Maciej A.; Komarnicka, Justyna; Mirecka, Małgorzata

    2014-01-01

    The authors present a case of echocardiographic diagnosis of a rare congenital cardiovascular anomaly in the form of mixed partial anomalous pulmonary veins connection in a 10-year-old girl with Turner syndrome and congenital mild stenosis of insufficient bicuspid aortic valve, made while diagnosing the causes of intestinal tract bleeding. The article presents various diagnostic difficulties leading to the delayed determination of a correct diagnosis, resulting from the absence of symptoms of circulatory failure in the early stage of the disease and the occurrence of severe and dominant auscultatory phenomena typical for congenital aortic valve defect which effectively masked the syndromes of increased pulmonary flow. The authors discuss the role of the impact of phenotypic characteristics of the Turner syndrome, in particular a short webbed neck restricting the suprasternal echocardiographic access and the presence of psychological factors associated with a long-term illness. The importance of indirect echocardiographic symptoms suggesting partial anomalous pulmonary veins connection in the presence of bicuspid aortic valve, e.g. enlargement of the right atrium and right ventricle, and paradoxical interventricular septum motion were emphasized in patients lacking ASD, pulmonary hypertension or tricupid and pulmonary valve abnormalities. The methodology of echocardiographic examination enabling direct visualization of the abnormal vascular structures was presented. Special attention was paid to the significance of highly sensitive echocardiographic projections: high right and left parasternal views in sagittal and transverse planes with patient lying on the side, with the use of two-dimensional imaging and color Doppler. Finally, the limitations of echocardiography resulting from the visualization and tracking of abnormal vascular structures hidden behind ultrasound non-conductive tissues were indicated, as was the role of other diagnostic modalities, such as angio

  12. Mixed partial anomalous pulmonary venous drainage coexistent with an aortic valve abnormality - analysis of ultrasound diagnostics in a 10-year-old girl with Turner syndrome.

    Science.gov (United States)

    Mądry, Wojciech; Karolczak, Maciej A; Komarnicka, Justyna; Mirecka, Małgorzata

    2014-03-01

    The authors present a case of echocardiographic diagnosis of a rare congenital cardiovascular anomaly in the form of mixed partial anomalous pulmonary veins connection in a 10-year-old girl with Turner syndrome and congenital mild stenosis of insufficient bicuspid aortic valve, made while diagnosing the causes of intestinal tract bleeding. The article presents various diagnostic difficulties leading to the delayed determination of a correct diagnosis, resulting from the absence of symptoms of circulatory failure in the early stage of the disease and the occurrence of severe and dominant auscultatory phenomena typical for congenital aortic valve defect which effectively masked the syndromes of increased pulmonary flow. The authors discuss the role of the impact of phenotypic characteristics of the Turner syndrome, in particular a short webbed neck restricting the suprasternal echocardiographic access and the presence of psychological factors associated with a long-term illness. The importance of indirect echocardiographic symptoms suggesting partial anomalous pulmonary veins connection in the presence of bicuspid aortic valve, e.g. enlargement of the right atrium and right ventricle, and paradoxical interventricular septum motion were emphasized in patients lacking ASD, pulmonary hypertension or tricupid and pulmonary valve abnormalities. The methodology of echocardiographic examination enabling direct visualization of the abnormal vascular structures was presented. Special attention was paid to the significance of highly sensitive echocardiographic projections: high right and left parasternal views in sagittal and transverse planes with patient lying on the side, with the use of two-dimensional imaging and color Doppler. Finally, the limitations of echocardiography resulting from the visualization and tracking of abnormal vascular structures hidden behind ultrasound non-conductive tissues were indicated, as was the role of other diagnostic modalities, such as angio

  13. Isolated right pulmonary artery agenesis with aplasia of right upper lobe and with anomalous arterial supply from celiac axis, anomalous venous drainage

    Directory of Open Access Journals (Sweden)

    Tushar Madhav Kalekar

    2015-01-01

    Full Text Available Pulmonary artery agenesis is the complete absence of the right or left pulmonary artery. This lesion is commonly associated with other cardiac anomalies and is usually diagnosed incidentally. Unilateral absence of pulmonary artery with lung hypoplasia is very rare. Ipsilateral hypoplastic or absent central pulmonary artery in these patients will have a systemic arterial supply to peripheral pulmonary arteries of the affected lung from the descending thoracic or upper abdominal aorta. These findings are usually characterized as pulmonary veno lobar syndrome or scimitar syndrome when associated with ipsilateral total or partial anomalous pulmonary venous return from the lower lobe which drains into the inferior vena cava or less commonly to the hepatic, azygous, or portal vein, or into the right atrium and lung agenesis/hypoplasia.

  14. Rarity of isolated pulmonary embolism and acute aortic syndrome occurring outside of the field of view of dedicated coronary CT angiography

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    Lee, Hwa Yeon; Song, In Sup (Dept. of Diagnostic Radiology Chung-Ang Univ. College of Medicine, Seoul (Korea, Republic of)); Yoo, Seung Min; Rho, Ji Young (Dept. of Diagnostic Radiology CHA Medical Univ. Hospital, Bundang (Korea, Republic of)), email: smyoo68@hanmail.net; Moon, Jae Youn; Kim, In Jai; Lim, Sang Wook; Sung, Jung Hoon; Cha, Dong Hun (Dept. of Cardiology CHA Medical Univ. Hospital, Bundang (Korea, Republic of)); White, Charles S. (Dept. of Diagnostic Radiology Univ. of Maryland, Baltimore (United States))

    2011-05-15

    Background Although triple rule-out CT angiography (TRO) to simultaneously evaluate acute coronary syndrome (ACS), pulmonary embolism (PE), and acute aortic syndrome (AAS) is increasingly used in many institutions, TRO is inevitably associated with increased radiation exposure due to extended z-axis coverage compared with dedicated coronary CT angiography (DCTA). Purpose To determine the frequency of exclusion of findings of AAS, PE, and significant incidental non-cardiac pathology that may be the cause of acute chest pain when using a restricted DCTA field of view (FOV). Material and Methods We retrospectively reviewed CT images and charts of 103 patients with acute PE and 50 patients with AAS. Either non-ECG gated dedicated pulmonary or aortic CT angiography was performed using 16- or 64-slice multidetector CT (MDCT). We analyzed the incidence of isolated PE, AAS, or significant non-cardiac pathology outside of DCTA FOV (i.e. from tracheal carina to the base of heart). Results There were two cases of isolated PE (2/103, 1.9%) excluded from the FOV of DCTA. One case of PE was isolated to the subsegmental pulmonary artery in the posterior segment of the right upper lobe. In the second case, pulmonary embolism in the left main pulmonary artery was located out of the FOV of DCTA because the left main pulmonary artery was retracted upwardly by fibrotic scar in the left upper lobe due to prior tuberculosis. There was no case of AAS and significant non-cardiac pathology excluded from the FOV of DCTA. AAS (n = 50) consisted of penetrating atherosclerotic ulcer (n = 7), intramural hematoma (n = 5) and aortic dissection (n = 38). Conclusion As isolated PE, AAS, and significant non-cardiac pathology outside of the DCTA FOV rarely occur, DCTA may replace TRO in the evaluation of patients with non-specific acute chest pain and a low pre-test probability of PE or aortic dissection

  15. Central poststroke pain: somatosensory abnormalities and the presence of associated myofascial pain syndrome

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    de Oliveira Rogério Adas

    2012-09-01

    Full Text Available Abstract Background Central post-stroke pain (CPSP is a neuropathic pain syndrome associated with somatosensory abnormalities due to central nervous system lesion following a cerebrovascular insult. Post-stroke pain (PSP refers to a broader range of clinical conditions leading to pain after stroke, but not restricted to CPSP, including other types of pain such as myofascial pain syndrome (MPS, painful shoulder, lumbar and dorsal pain, complex regional pain syndrome, and spasticity-related pain. Despite its recognition as part of the general PSP diagnostic possibilities, the prevalence of MPS has never been characterized in patients with CPSP patients. We performed a cross-sectional standardized clinical and radiological evaluation of patients with definite CPSP in order to assess the presence of other non-neuropathic pain syndromes, and in particular, the role of myofascial pain syndrome in these patients. Methods CPSP patients underwent a standardized sensory and motor neurological evaluation, and were classified according to stroke mechanism, neurological deficits, presence and profile of MPS. The Visual Analogic Scale (VAS, McGill Pain Questionnaire (MPQ, and Beck Depression Scale (BDS were filled out by all participants. Results Forty CPSP patients were included. Thirty-six (90.0% had one single ischemic stroke. Pain presented during the first three months after stroke in 75.0%. Median pain intensity was 10 (5 to 10. There was no difference in pain intensity among the different lesion site groups. Neuropathic pain was continuous-ongoing in 34 (85.0% patients and intermittent in the remainder. Burning was the most common descriptor (70%. Main aggravating factors were contact to cold (62.5%. Thermo-sensory abnormalities were universal. MPS was diagnosed in 27 (67.5% patients and was more common in the supratentorial extra-thalamic group (P Conclusions The presence of MPS is not an exception after stroke and may present in association with CPSP

  16. Central poststroke pain: somatosensory abnormalities and the presence of associated myofascial pain syndrome

    Science.gov (United States)

    2012-01-01

    Background Central post-stroke pain (CPSP) is a neuropathic pain syndrome associated with somatosensory abnormalities due to central nervous system lesion following a cerebrovascular insult. Post-stroke pain (PSP) refers to a broader range of clinical conditions leading to pain after stroke, but not restricted to CPSP, including other types of pain such as myofascial pain syndrome (MPS), painful shoulder, lumbar and dorsal pain, complex regional pain syndrome, and spasticity-related pain. Despite its recognition as part of the general PSP diagnostic possibilities, the prevalence of MPS has never been characterized in patients with CPSP patients. We performed a cross-sectional standardized clinical and radiological evaluation of patients with definite CPSP in order to assess the presence of other non-neuropathic pain syndromes, and in particular, the role of myofascial pain syndrome in these patients. Methods CPSP patients underwent a standardized sensory and motor neurological evaluation, and were classified according to stroke mechanism, neurological deficits, presence and profile of MPS. The Visual Analogic Scale (VAS), McGill Pain Questionnaire (MPQ), and Beck Depression Scale (BDS) were filled out by all participants. Results Forty CPSP patients were included. Thirty-six (90.0%) had one single ischemic stroke. Pain presented during the first three months after stroke in 75.0%. Median pain intensity was 10 (5 to 10). There was no difference in pain intensity among the different lesion site groups. Neuropathic pain was continuous-ongoing in 34 (85.0%) patients and intermittent in the remainder. Burning was the most common descriptor (70%). Main aggravating factors were contact to cold (62.5%). Thermo-sensory abnormalities were universal. MPS was diagnosed in 27 (67.5%) patients and was more common in the supratentorial extra-thalamic group (P <0.001). No significant differences were observed among the different stroke location groups and pain questionnaires and

  17. Myoclonic status and central fever in Angelman syndrome due to paternal uniparental disomy.

    Science.gov (United States)

    Nicita, Francesco; Garone, Giacomo; Papetti, Laura; Consoli, Federica; Magliozzi, Monia; De Luca, Alessandro; Spalice, Alberto

    2015-01-01

    Myoclonic status in nonprogressive encephalopathy (MSNE) is an early-onset, drug-resistant epileptic syndrome characterized by occurrence of continuous diffuse epileptiform abnormalities, associated with positive and/or negative phenomena and accompanied by transient and recurring motor, cognitive, and behavioral impairment. MSNE has been reported in Angelman syndrome (AS) secondary to 15q11-13 deletions or UBE3A mutations but not to paternal uniparental disomy (UPD). We describe the case of a male patient with AS caused by UPD who developed a myoclonic status (MS) associated with long-lasting fever of central origin, both promptly regressed with introduction of levetiracetam. Only three descriptions of thermal dysregulation in AS exist, and none of the previously reported cases were associated with MS or with UPD. Association of MS and central fever expands the spectrum of epileptic and non-epileptic features in UPD-related AS and provides a further evidence of hypothalamus involvement in the pathogenesis of this neurodevelopmental disorder.

  18. Pulmonary toxicity of components of textile paint linked to the Ardystil syndrome: intratracheal administration in hamsters.

    Science.gov (United States)

    Clottens, F L; Verbeken, E K; Demedts, M; Nemery, B

    1997-01-01

    OBJECTIVES: It was hypothesised from an epidemiological investigation that a formula change from Acramin FWR (a polyurea) to Acramin FWN (a polyamide-amine) had led to severe pulmonary disease in textile printing sprayers in SPAIN AND ALGERIA. To verify this, the pulmonary toxicity of the components of the paint systems involved was assessed in experimental animals. METHODS: Individual components and relevant mixtures, diluted in phosphate buttered saline, were given by intratracheal instillation of 2 ml/kg to hamsters. Pulmonary toxicity was assessed on days 3, 7, 14, 28, and 92 after a single intratracheal instillation, by histology and by measuring wet and dry lung weight, protein concentration, the activities of lactate dehydrogenase, alkaline phosphatase, beta-N-acetyl-glucosaminidase, and gamma-glutamyltransferase, inflammatory cell number and distribution in bronchoalveolar lavage fluid (BALF), and hydroxyproline content in dried lung tissue. RESULTS: Based on the doses that killed 50% of the animals (LD50s), the various components were found to be 10 to 1250 times more toxic when given intratracheally than when given orally (according to reported oral LD50s in rats). Acramin FWN, Acramin FWR, Acrafix FHN, or their mixtures caused lung damage. Protein concentration, enzyme activities, total cell number, and percentage of polymorphonuclear neutrophils were increased in BALF during the first week after intratracheal instillation. Lung weights remained high for at least a month. Histology showed inflammatory cell infiltration and subsequent fibrosis with collagen deposition. This finding was confirmed by an increased hydroxyproline content in dried lung tissue. Acramoll W did not show toxic effects. CONCLUSIONS: The study suggests that there is no major difference, in hamsters, between the acute intratracheal toxicity of Acramin FWR and that of Acramin FWN. Consequently, there is no simple toxicological explanation for the epidemiological hypothesis. However

  19. An Unusual Type of Localized Hypertrophic Cardiomyopathy With Wolf Parkinson White Syndrome Presenting With Pulmonary Edema

    Science.gov (United States)

    Vatan, Mehmet Bulent; Gunduz, Huseyin; Gurel, Safiye; Kocayigit, Ibrahim; Vural, Ahmet; Demirtas, Saadet; Cakar, Mehmet Akif; Gunduz, Yasemin

    2012-01-01

    Hypertrophic cardiomyopathy (HCM) is an autosomal dominant heart disease that is the most common genetic cardiac disorder. The disease is characterized by excessive thickening of the left ventricular myocardium. The anterior portion of the interventricular ventricular septum is often involved. Asymmetric hypertrophy of apical site, left ventricular free wall, and right ventricle are less common in hypertrophic cardiomyopathy that occur in 1% cases. We report a case of a patient with an unusual type of hypertrophic cardiomyopathy and Wolf Parkinson White (WPW) presenting with pulmonary edema.

  20. Pulmonary ultrasound and pulse oximetry versus chest radiography and arterial blood gas analysis for the diagnosis of acute respiratory distress syndrome: a pilot study

    OpenAIRE

    Bass, Cameron M.; Sajed, Dana R.; Adedipe, Adeyinka A.; West, T Eoin

    2015-01-01

    Introduction In low-resource settings it is not always possible to acquire the information required to diagnose acute respiratory distress syndrome (ARDS). Ultrasound and pulse oximetry, however, may be available in these settings. This study was designed to test whether pulmonary ultrasound and pulse oximetry could be used in place of traditional radiographic and oxygenation evaluation for ARDS. Methods This study was a prospective, single-center study in the ICU of Harborview Medical Center...

  1. Severe Pulmonary Suppuration with Infection-Induced Systemic Inflammatory Response Syndrome following Tongue Cancer Surgery in a Patient Undergoing Tocilizumab Therapy for Rheumatoid Arthritis

    Directory of Open Access Journals (Sweden)

    Kenji Yamagata

    2014-01-01

    Full Text Available A 65-year-old woman with rheumatoid arthritis treated by tocilizumab (TCZ presented with tongue squamous cell carcinoma. While surgery was performed without any complications the aspiration pneumonia rapidly worsened by postoperative day 2 and severe pulmonary suppuration in the right lung field with infection-induced systemic inflammatory response syndrome (SIRS was diagnosed. Antibiotic and respirator treatment improved her condition. The anti-inflammatory effect of TCZ may mask the symptoms and signs of severe infection with SIRS.

  2. A review of pulmonary coagulopathy in acute lung injury, acute respiratory distress syndrome and pneumonia

    NARCIS (Netherlands)

    Nieuwenhuizen, Laurens; de Groot, Philip G.; Grutters, Jan C.; Biesma, Douwe H.

    2009-01-01

    Enhanced bronchoalveolar coagulation is a hallmark of many acute inflammatory lung diseases such as acute lung injury, acute respiratory distress syndrome and pneumonia. Intervention with natural anticoagulants in these diseases has therefore become a topic of interest. Recently, new data on the rol

  3. Anti-hLAMP2-antibodies and dual positivity for anti-GBM and MPO-ANCA in a patient with relapsing pulmonary-renal syndrome

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    Kistler Thomas

    2011-06-01

    Full Text Available Abstract Background Pulmonary-renal syndrome associated with anti-glomerular basement membrane (GBM antibodies, also known as Goodpasture's syndrome, is a rare but acute and life-threatening condition. One third of patients presenting as anti-GBM antibody positive pulmonary-renal syndrome or rapidly progressive glomerulonephritis are also tested positive for anti-neutrophil cytoplasmic antibodies (ANCA. Whilst anti-GBM disease is considered a non-relapsing condition, the long-term course of double-positive patients is less predictable. Case Presentation We report a patient with such dual positivity, who presented with pulmonary hemorrhage, crescentic glomerulonephritis and membranous nephropathy. Plasmapheresis in combination with immunosuppresive therapy led to a rapid remission but the disease relapsed after two years. The serum of the patient was tested positive for antibodies to human lysosomal membrane protein 2 (hLAMP2, a novel autoantigen in patients with active small-vessel vasculitis (SVV. The anti-hLAMP2 antibody levels correlated positively with clinical disease activity in this patient. Conclusion We hypothesize that this antibody may indicate a clinical course similar to ANCA-associated vasculitis in double-positive patients. However, this needs to be confirmed on comprehensive patient cohorts.

  4. Immunophenotypic and Ultrastructural Analysis of Mast Cells in Hermansky-Pudlak Syndrome Type-1: A Possible Connection to Pulmonary Fibrosis.

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    Arnold S Kirshenbaum

    Full Text Available Hermansky-Pudlak Syndrome type-1 (HPS-1 is an autosomal recessive disorder caused by mutations in HPS1 which result in reduced expression of the HPS-1 protein, defective lysosome-related organelle (LRO transport and absence of platelet delta granules. Patients with HPS-1 exhibit oculocutaneous albinism, colitis, bleeding and pulmonary fibrosis postulated to result from a dysregulated immune response. The effect of the HPS1 mutation on human mast cells (HuMCs is unknown. Since HuMC granules classify as LROs along with platelet granules and melanosomes, we set out to determine if HPS-1 cutaneous and CD34+ culture-derived HuMCs have distinct granular and cellular characteristics. Cutaneous and cultured CD34+-derived HuMCs from HPS-1 patients were compared with normal cutaneous and control HuMCs, respectively, for any morphological and functional differences. One cytokine-independent HPS-1 culture was expanded, cloned, designated the HP proMastocyte (HPM cell line and characterized. HPS-1 and idiopathic pulmonary fibrosis (IPF alveolar interstitium showed numerous HuMCs; HPS-1 dermal mast cells exhibited abnormal granules when compared to healthy controls. HPS-1 HuMCs showed increased CD63, CD203c and reduced mediator release following FcɛRI aggregation when compared with normal HuMCs. HPM cells also had the duplication defect, expressed FcɛRI and intracytoplasmic proteases and exhibited less mediator release following FcɛRI aggregation. HPM cells constitutively released IL-6, which was elevated in patients' serum, in addition to IL-8, fibronectin-1 (FN-1 and galectin-3 (LGALS3. Transduction with HPS1 rescued the abnormal HPM morphology, cytokine and matrix secretion. Microarray analysis of HPS-1 HuMCs and non-transduced HPM cells confirmed upregulation of differentially expressed genes involved in fibrogenesis and degranulation. Cultured HPS-1 HuMCs appear activated as evidenced by surface activation marker expression, a decrease in mediator

  5. Immunophenotypic and Ultrastructural Analysis of Mast Cells in Hermansky-Pudlak Syndrome Type-1: A Possible Connection to Pulmonary Fibrosis

    Science.gov (United States)

    Kirshenbaum, Arnold S.; Cruse, Glenn; Desai, Avanti; Bandara, Geethani; Leerkes, Maarten; Lee, Chyi-Chia R.; Fischer, Elizabeth R.; O’Brien, Kevin J.; Gochuico, Bernadette R.; Stone, Kelly; Gahl, William A.; Metcalfe, Dean D.

    2016-01-01

    Hermansky-Pudlak Syndrome type-1 (HPS-1) is an autosomal recessive disorder caused by mutations in HPS1 which result in reduced expression of the HPS-1 protein, defective lysosome-related organelle (LRO) transport and absence of platelet delta granules. Patients with HPS-1 exhibit oculocutaneous albinism, colitis, bleeding and pulmonary fibrosis postulated to result from a dysregulated immune response. The effect of the HPS1 mutation on human mast cells (HuMCs) is unknown. Since HuMC granules classify as LROs along with platelet granules and melanosomes, we set out to determine if HPS-1 cutaneous and CD34+ culture-derived HuMCs have distinct granular and cellular characteristics. Cutaneous and cultured CD34+-derived HuMCs from HPS-1 patients were compared with normal cutaneous and control HuMCs, respectively, for any morphological and functional differences. One cytokine-independent HPS-1 culture was expanded, cloned, designated the HP proMastocyte (HPM) cell line and characterized. HPS-1 and idiopathic pulmonary fibrosis (IPF) alveolar interstitium showed numerous HuMCs; HPS-1 dermal mast cells exhibited abnormal granules when compared to healthy controls. HPS-1 HuMCs showed increased CD63, CD203c and reduced mediator release following FcɛRI aggregation when compared with normal HuMCs. HPM cells also had the duplication defect, expressed FcɛRI and intracytoplasmic proteases and exhibited less mediator release following FcɛRI aggregation. HPM cells constitutively released IL-6, which was elevated in patients’ serum, in addition to IL-8, fibronectin-1 (FN-1) and galectin-3 (LGALS3). Transduction with HPS1 rescued the abnormal HPM morphology, cytokine and matrix secretion. Microarray analysis of HPS-1 HuMCs and non-transduced HPM cells confirmed upregulation of differentially expressed genes involved in fibrogenesis and degranulation. Cultured HPS-1 HuMCs appear activated as evidenced by surface activation marker expression, a decrease in mediator content and

  6. Central venous line complications with chronic ambulatory infusion of prostacyclin analogues in pediatric patients with pulmonary arterial hypertension.

    Science.gov (United States)

    Marr, Courtney R; McSweeney, Julia E; Mullen, Mary P; Kulik, Thomas J

    2015-06-01

    Chronic infusion of prostacyclin (PGI2) via a Broviac central venous line (CVL) is attended by risk of CVL-related complications, but we know of only one report regarding CVL-associated bloodstream infection (BSI) with PGI2 in children and none regarding other complications. We conducted a retrospective cohort study involving pediatric patients with pulmonary hypertension treated with chronic intravenous infusion of PGI2 at Boston Children's Hospital and determined the rate (per 1,000 line-days) of various CVL-related complications. We also determined how often complications necessitated line replacement and hospitalization, time to replacement of CVLs, and interpatient variability in the incidence of complications. From 1999 until 2014, 26 patients meeting follow-up criteria had PGI2 infusion, representing 43,855 line-days; mean follow-up was 56 months (range, 1.4-161 months). The CVL complication rates (per 1,000 line-days) were as follows: CVL-BSI, 0.25; superficial line infection, 0.48; impaired integrity, 0.59; occlusion, 0.09; and malposition, 0.32. The total complication rate was 1.73 cases per 1,000 line-days. All CVL-BSI and malposition cases were treated with CVL removal and replacement. Of CVLs with impaired integrity, 23 could be repaired and 3 required replacement. Six of 21 superficial CVL infections required replacement of the CVL. Three of 4 occluded CVLs were replaced. CVL complications occasioned 65 hospitalizations. There was marked interpatient variability in the rate of complications, much but not all of which appeared to be related to duration of CVL placement. We conclude that non-BSI complications are very significant and that efforts to teach and emphasize other aspects of line care are therefore very important.

  7. The prevalence and etiology of polycystic ovarian syndrome (PCOS) as a cause of female infertility in central Travancore

    OpenAIRE

    K. Roy George; N A Malini

    2014-01-01

    Recent alarming rise in the incidence of polycystic ovary syndrome (PCOS), the most common cause of female infertility is becoming a major concern among adolescent women worldwide. Altered hormonal and metabolic profiles are one of the common clinical manifestations in PCOS. The aim of the present study was to determine the prevalence and the etiology of polycystic ovary syndrome (PCOS) as a cause of female infertility in Central Travancore women, in view of their change in life s...

  8. Reduction of central neuropathic pain with ketamine infusion in a patient with Ehlers–Danlos syndrome: a case report

    Directory of Open Access Journals (Sweden)

    Lo TC

    2016-09-01

    Full Text Available Tony Chung Tung Lo,1,* Stephen Tung Yeung,2,* Sujin Lee,1 Kira Skavinski,3 Solomon Liao,4 1Department of Physical Medicine and Rehabilitation, University of California Irvine, Orange, CA, 2Department of Immunology, University of Connecticut School of Medicine, Farmington, CT, 3Department of Palliative Medicine, University of California San Diego, La Jolla, 4Department of Palliative Medicine, University of California Irvine, Orange, CA, USA *These authors contributed equally to this work Objective: Ehlers–Danlos syndrome frequently causes acute and chronic pain because of joint subluxations and dislocations secondary to hypermobility. Current treatments for pain related to Ehlers–Danlos syndrome and central pain syndrome are inadequate. This case report discusses the therapeutic use of ketamine intravenous infusion as an alternative. Case report: A 27-year-old Caucasian female with a history of Ehlers–Danlos syndrome and spinal cord ischemic myelopathy resulting in central pain syndrome, presented with severe generalized body pain refractory to multiple pharmacological interventions. After a 7-day course of ketamine intravenous infusion under controlled generalized sedation in the intensive care unit, the patient reported a dramatic reduction in pain levels from 7–8 out of 10 to 0–3 out of 10 on a numeric rating scale and had a significant functional improvement. The patient tolerated a reduction in her pain medication regimen, which originally included opioids, gabapentin, pregabalin, tricyclic antidepressants, and nonsteroidal anti-inflammatory drugs. Conclusion: Ketamine infusion treatment has been used in various pain syndromes, including central neuropathic pain, ischemic pain, and regional pain syndrome. Reports have suggested that ketamine modulates pain by the regression of N-methyl-D-aspartate receptor to a resting state. As such, propagation of nociceptive signal to brain is interrupted allowing for the restoration of

  9. Growth hormone deficiency and central precocious puberty in Klinefelter syndrome: report of a case and review of KIGS database.

    Science.gov (United States)

    Gonzales-Ellis, Bryn A; Pingul, Mia M; Reddy, Sujana; Boney, Charlotte M; Wajnrajch, Michael P; Quintos, Jose Bernarto

    2013-01-01

    Growth hormone deficiency (GHD) and central precocious puberty (CPP) have each, individually, been described in patients with Klinefelter syndrome. However, the combination of GHD, CPP, and Klinefelter syndrome has never been reported. We described a Klinefelter syndrome patient who developed GHD at age 2 10/12 years and CPP at 8 6/12 years. Despite CPP, GnRH agonist therapy was not initiated because of his excellent predicted adult height. At 11 8/12 years, his height was 164.6 cm, close to his mid-parental target height of 165 cm. We report an additional nine patients with Klinefelter syndrome and GHD from the Pfizer International Growth Study (KIGS) database, none of whom had CPP. We conclude that the combination of GHD and CPP is very rare in Klinefelter syndrome and that CPP is unlikely to compromise final adult height.

  10. A REPORT OF CENTRAL RETINAL ARTERY OCCLUSION (CRAO , IN YOUNG MALES IN ITS INITIAL MANIFESTATION, AS PRIMAR Y ANTIPHOSPHOLIPID SYNDROME

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    Rani

    2013-05-01

    Full Text Available ABSTRACT: AIM: To report a case of Central Retinal Artery Occlusi on (CRAO in young males in its initial manifestation as Primary Antiphospholipid Syndrome. METHODS: 32 year healthy male, with abrupt sudden painless loss of vision in r ight eye since 48 hours, with Grade 2 Relative afferent pupillary defect, visual acuity of hand movements in OD and 6/18 in OS. Fundoscopy disclosed signs compatible of central reti nal artery occlusion confirmed with FFA. Carotid Doppler imaging and echocardiography was done to determine the source. RESULTS: Antiphospholipid antibody cofactor, beta2-glycoprotein 1 antibodies, IgM, was positive with titre of more than 94 un its/ml on two occasions, 1 2 weeks apart, with normal range being less than 20 units/ml for each isotope (IgG, IgM, or IgA .According to the 2006 revised Sapporo criteria Antiphospholipid syndrome was diagnosed. Thor ough examination excluded other system involvement. Immunological studies excluded other systemic disorders. CONCLUSIONS: In literature, prevalence of CRAO is 0.85% for every 100000 and prevalence of Antiphospholipid Syndrome in patients showing a major retinal vascula r obstruction is 5% - 33%. Antiphospholipid syndrome should be ruled out in every young patient who presents with Central retinal artery occlusion. Association must be considered, as Central retinal artery occlusion could be the initial manifestation of ant iphospholipid syndrome with high risk of recurrence.

  11. Radiological pulmonary manifestations of acquired immunodeficiency syndrome; Manifestacoes radiologicas pulmonares da sindrome da imunodeficiencia adquirida

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    Marchiori, Edson; Melo, Alessandro Severo Alves de; Ossa, Alfonso Jaramillo [Universidade Federal Fluminense, Niteroi, RJ (Brazil). Dept. de Radiologia

    1999-06-01

    In this article are reviewed the principal radiologic manifestations of inflammatory and tumoral diseases the compromise the lungs of patients with acquired immunodeficiency syndrome. In the group of inflammatory diseases the radiologic aspects of pneumocystosis, cytomegalovirus disease, cryptococcosis, tuberculosis and bacterial pneumonias are emphasized. In the neoplasic diseases' group the aspects of lymphoma and Kaposi's sarcoma are specially presented. (author)

  12. Novel camelid antibody fragments targeting recombinant nucleoprotein of Araucaria hantavirus: a prototype for an early diagnosis of Hantavirus Pulmonary Syndrome.

    Science.gov (United States)

    Pereira, Soraya S; Moreira-Dill, Leandro S; Morais, Michelle S S; Prado, Nidiane D R; Barros, Marcos L; Koishi, Andrea C; Mazarrotto, Giovanny A C A; Gonçalves, Giselle M; Zuliani, Juliana P; Calderon, Leonardo A; Soares, Andreimar M; Pereira da Silva, Luiz H; Duarte dos Santos, Claudia N; Fernandes, Carla F C; Stabeli, Rodrigo G

    2014-01-01

    In addition to conventional antibodies, camelids produce immunoglobulins G composed exclusively of heavy chains in which the antigen binding site is formed only by single domains called VHH. Their particular characteristics make VHHs interesting tools for drug-delivery, passive immunotherapy and high-throughput diagnosis. Hantaviruses are rodent-borne viruses of the Bunyaviridae family. Two clinical forms of the infection are known. Hemorrhagic Fever with Renal Syndrome (HFRS) is present in the Old World, while Hantavirus Pulmonary Syndrome (HPS) is found on the American continent. There is no specific treatment for HPS and its diagnosis is carried out by molecular or serological techniques, using mainly monoclonal antibodies or hantavirus nucleoprotein (N) to detect IgM and IgG in patient serum. This study proposes the use of camelid VHHs to develop alternative methods for diagnosing and confirming HPS. Phage display technology was employed to obtain VHHs. After immunizing one Lama glama against the recombinant N protein (prNΔ₈₅) of a Brazilian hantavirus strain, VHH regions were isolated to construct an immune library. VHHs were displayed fused to the M13KO7 phage coat protein III and the selection steps were performed on immobilized prNΔ₈₅. After selection, eighty clones recognized specifically the N protein. These were sequenced, grouped based mainly on the CDRs, and five clones were analyzed by western blot (WB), surface plasmon resonance (SPR) device, and ELISA. Besides the ability to recognize prNΔ85 by WB, all selected clones showed affinity constants in the nanomolar range. Additionaly, the clone KC329705 is able to detect prNΔ₈₅ in solution, as well as the native viral antigen. Findings support the hypothesis that selected VHHs could be a powerful tool in the development of rapid and accurate HPS diagnostic assays, which are essential to provide supportive care to patients and reduce the high mortality rate associated with hantavirus

  13. Novel camelid antibody fragments targeting recombinant nucleoprotein of Araucaria hantavirus: a prototype for an early diagnosis of Hantavirus Pulmonary Syndrome.

    Directory of Open Access Journals (Sweden)

    Soraya S Pereira

    Full Text Available In addition to conventional antibodies, camelids produce immunoglobulins G composed exclusively of heavy chains in which the antigen binding site is formed only by single domains called VHH. Their particular characteristics make VHHs interesting tools for drug-delivery, passive immunotherapy and high-throughput diagnosis. Hantaviruses are rodent-borne viruses of the Bunyaviridae family. Two clinical forms of the infection are known. Hemorrhagic Fever with Renal Syndrome (HFRS is present in the Old World, while Hantavirus Pulmonary Syndrome (HPS is found on the American continent. There is no specific treatment for HPS and its diagnosis is carried out by molecular or serological techniques, using mainly monoclonal antibodies or hantavirus nucleoprotein (N to detect IgM and IgG in patient serum. This study proposes the use of camelid VHHs to develop alternative methods for diagnosing and confirming HPS. Phage display technology was employed to obtain VHHs. After immunizing one Lama glama against the recombinant N protein (prNΔ₈₅ of a Brazilian hantavirus strain, VHH regions were isolated to construct an immune library. VHHs were displayed fused to the M13KO7 phage coat protein III and the selection steps were performed on immobilized prNΔ₈₅. After selection, eighty clones recognized specifically the N protein. These were sequenced, grouped based mainly on the CDRs, and five clones were analyzed by western blot (WB, surface plasmon resonance (SPR device, and ELISA. Besides the ability to recognize prNΔ85 by WB, all selected clones showed affinity constants in the nanomolar range. Additionaly, the clone KC329705 is able to detect prNΔ₈₅ in solution, as well as the native viral antigen. Findings support the hypothesis that selected VHHs could be a powerful tool in the development of rapid and accurate HPS diagnostic assays, which are essential to provide supportive care to patients and reduce the high mortality rate associated with

  14. [Pseudoexfoliation syndrome: no central zone of pseudoexfoliation material in patients with pseudophakia - a clinical study].

    Science.gov (United States)

    Reiter, C; Gramer, E; Gramer, G

    2012-03-01

    1. An evaluation of medical findings and photodocumentation of 6 patients with pseudoexfoliation (PEX) material on the anterior surface of posterior chamber intraocular lenses was undertaken. Molecular genetic analysis of mutations in LOX-L1 was performed in order to confirm the association with pseudoexfoliation syndrome or glaucoma. Age of patients, the maximal intraocular pressure and perimetry with Octopus and Goldmann perimeters were documented as well as the time between implantation of the posterior chamber lens and diagnosis of pseudoexfoliation material on the anterior surface of posterior chamber lens. 2. Consecutive examinations of 35 patients with pseudoexfoliation syndrome or -glaucoma and pseudophakia were made to evaluate the frequency of patients with pseudoexfoliation material on the anterior surface of posterior chamber lenses. 1. The characteristic formation of stripe-shaped peripheral pseudoexfoliation material is seen in all examined patients on the anterior surface of posterior chamber lenses, but there is no central homogeneous round zone of pseudoexfoliation material in all patients. 2. the mean observation time of patients with pseudophakia and pseudoexfoliation syndrome or glaucoma is 4.4 ± 3.9 years. 5.7 % of the patients show pseudoexfoliation material in the periphery of posterior chamber lenses. The mean time between implantation of the intraocular lens and diagnosis of pseudoexfoliation material on the lenses is 3 years. The lack of a central homogeneous round zone of pseudoexfoliation material deposits on the anterior surface of posterior chamber lenses seems to be characteristic. The change in topography of PEX material on intraocular lenses is described here for the first time. A knowledge of this change in the topography of pseudoexfoliation material in pseudophakia is important for glaucoma screening, because pseudoexfoliation deposits can only be detected in mydriasis due to the peripheral location on the intraocular lens

  15. Meandering right pulmonary vein associated with severe and progressive "idiopathic-like" pulmonary hypertensive vascular disease.

    Science.gov (United States)

    Cuenca, Sofia; Bret, Montserrat; del Cerro, Maria Jesus

    2016-03-01

    Congenital anomalies of the pulmonary veins are rare. Meandering right pulmonary vein, considered a part of the Scimitar syndrome spectrum, is often an incidental finding during chest imaging. We present the case of a 4-year-old girl diagnosed with meandering pulmonary vein, who developed pulmonary hypertensive disease with an aggressive course, in spite of absence of hypoxia or elevated pulmonary wedge pressure.

  16. Two Japanese Cases of Birt-Hogg-Dubé Syndrome with Pulmonary Cysts, Fibrofolliculomas, and Renal Cell Carcinomas

    Directory of Open Access Journals (Sweden)

    Yukako Murakami

    2014-02-01

    Full Text Available Birt-Hogg-Dubé syndrome (BHD is a rare autosomal dominant inherited disease caused by a germline mutation in the folliculin gene mapped in the region of chromosome 17p11.2. BHD predisposes the patient to cutaneous fibrofolliculomas (FFs, pulmonary cysts (PCs, and renal cell carcinoma (RC. Here, we present two cases of BHD in Japanese patients. One patient was a 37-year-old female, and the other a 35-year-old male. Each of the patients was affected by all three symptoms of BHD. Both patients had unremarkable FFs, asymptomatic PCs, and asymptomatic RC. The presence of RC was revealed by abdominal ultrasonic examination. We also summarized the data from 62 Asian cases of BHD from the available literature and found that their FFs were unremarkable. In addition, the proportion of BHD patients with FF is smaller for Asian patients than it is for Caucasian patients. We also found that it is rare for BHD patients in Asia to show all three symptoms of BHD. Careful inspection of the skin as well as skin biopsies are important for the early detection of BHD cases in Asia.

  17. Electrocardiographic differentiation between acute coronary syndrome and acute pulmonary embolism associated with inverted T waves in precordial leads

    Institute of Scientific and Technical Information of China (English)

    ZHAN Zhong-qun; WANG Chong-quan; HE Chao-rong; WANG Zhi-xiao; MAO Shan

    2010-01-01

    Background Inverted T waves in precordial leads are often seen in patients with acute pulmonary embolism (APE) and acute coronary syndrome (ACS). The purpose of this study was to analyze the electrocardiogram (ECG) difference between APE and ACS related inverted T waves in precordial leads. Methods The ECG difference among 62 patients with APE and 125 patients with ACS related inverted T waves in precordial leads were compared. Results Compared with ACS, Patients with APE were more frequently associated with incomplete or complete RBBB or slurred S wave in lead V1, the sum of the depth of inverted T waves in leads V1 and V2 not less than in lead V3 and V4 (inverted TV1 + V2 ≥ inverted TV3 + V4), and inverted T waves in leads V1 and Ⅲ. Conclusions Complete or incomplete RBBB or slurred S wave in lead V1, inverted T waves in leads V1 and III, and inverted TV1 + V2 ≥ inverted TV3 + V4 are useful criteria for predicting APE.

  18. Application of MODIS GPP to Forecast Risk of Hantavirus Pulmonary Syndrome Based on Fluctuations in Reservoir Population Density

    Science.gov (United States)

    Loehman, R.; Heinsch, F. A.; Mills, J. N.; Wagoner, K.; Running, S.

    2003-12-01

    Recent predictive models for hantavirus pulmonary syndrome (HPS) have used remotely sensed spectral reflectance data to characterize risk areas with limited success. We present an alternative method using gross primary production (GPP) from the MODIS sensor to estimate the effects of biomass accumulation on population density of Peromyscus maniculatus (deer mouse), the principal reservoir species for Sin Nombre virus (SNV). The majority of diagnosed HPS cases in North America are attributed to SNV, which is transmitted to humans through inhalation of excretions and secretions from infected rodents. A logistic model framework is used to evaluate MODIS GPP, temperature, and precipitation as predictors of P. maniculatus density at established trapping sites across the western United States. Rodent populations are estimated using monthly minimum number alive (MNA) data for 2000 through 2002. Both local meteorological data from nearby weather stations and 1.25 degree x 1 degree gridded data from the NASA DAO were used in the regression model to determine the spatial sensitivity of the response. MODIS eight-day GPP data (1-km resolution) were acquired and binned to monthly average and monthly sum GPP for 3km x 3km grids surrounding each rodent trapping site. The use of MODIS GPP to forecast HPS risk may result in a marked improvement over past reflectance-based risk area characterizations. The MODIS GPP product provides a vegetation dynamics estimate that is unique to disease models, and targets the fundamental ecological processes responsible for increased rodent density and amplified disease risk.

  19. The use of intratracheal pulmonary ventilation and partial liquid ventilation in newborn piglets with meconium aspiration syndrome.

    Science.gov (United States)

    Onasanya, Babatunde I.; Rais-Bahrami, Khodayar; Rivera, Oswaldo; Seale, Winslow R.; Short, Billie L.

    2001-01-01

    OBJECTIVE: To determine whether intratracheal pulmonary ventilation (ITPV) combined with partial liquid ventilation (PLV) improves oxygenation and ventilation at lower mean airway and peak inspiratory pressures when compared with conventional mechanical ventilation in a piglet model of meconium aspiration syndrome. DESIGN: Prospective, randomized, interventional study. SETTING: Animal Research Laboratory at the Children's National Medical Center, Washington, DC. SUBJECTS: Twenty newborn piglets, 1 to 2 wks of age, 1.8-2.8 kg in weight. INTERVENTION: The animals were anesthetized, paralyzed, and intubated with a 4.0 mm (internal diameter) endotracheal tube via a tracheostomy and were ventilated. Catheters were placed in the femoral artery and vein. Seven milliliters per kilogram of 20% human meconium was insufflated into the lungs over 30 mins. Dynamic pulmonary compliance was measured before and after instillation of meconium. Animals were ventilated to maintain arterial blood gases in a normal range, that is, pH = 7.35-7.45, Paco(2) = 40-45 torr (5.3-6.0 kPa), and Pao(2) = 70-90 torr (9.3-12.0 kPa). Ventilator settings were increased as needed to a maximum setting of Fio(2) = 1.0, peak inspiratory pressure (PIP) = 40 cm H(2)O, positive end-expiratory pressure = 5 cm H(2)O, and intermittent mandatory ventilation = 60 bpm. After a period of stabilization, 30 mL/kg of perflubron (Liquivent; Alliance Pharmaceutical Corp., San Diego, CA) was given intratracheally over 30 mins and the animals were randomized to either ITPV or control group. Measurements and RESULTS: Arterial blood gases were taken every 30 mins, and ventilatory settings were adjusted to achieve the targeted blood gas parameters. The animals' temperature, arterial blood pressure, heart rate, and oxygen saturation were monitored continuously. There was a significant decrease in the dynamic pulmonary compliance measurements in both groups immediately after meconium instillation. Compliance measurements

  20. Lack of differential pattern in central adiposity and metabolic syndrome in Barrett's esophagus and gastroesophageal reflux disease.

    LENUS (Irish Health Repository)

    Healy, L A

    2012-02-01

    Obesity is an established risk factor for esophageal adenocarcinoma, although the mechanism is unclear. A pathway from reflux to inflammation through metaplasia is the dominant hypothesis, and an added role relating to visceral adiposity and the metabolic syndrome has been mooted in Barrett\\'s esophagus (BE) patients. Whether BE differs from gastroesophageal reflux disease (GERD) in obesity and metabolic syndrome profiles is unclear, and this was the focus of this study. Patients with proven BE or GERD were randomly selected from the unit data registry and invited to attend for metabolic syndrome screening, anthropometry studies including segmental body composition analysis, and laboratory tests including fasting lipids, insulin, and C-reactive protein. Metabolic syndrome was defined using the National Cholesterol Education Program (NCEP) and the International Diabetes Federation (IDF) criteria. One hundred and eighteen BE patients and 113 age- and sex-matched GERD controls were studied. The incidence of obesity (body mass index >30 kg\\/m(2)) was 36% and 38%, respectively, with the pattern of fat deposition predominantly central and an estimated trunk fat mass of 13 and 14 kg, respectively. Using the NCEP criteria, metabolic syndrome was significantly more common in the BE cohort (30% vs 20%, P < 0.05), but there was no significant difference using IDF criteria (42% vs 37%, P= 0.340). Central obesity and the metabolic syndrome are common in both Barrett\\'s and GERD cohorts, but not significantly different, suggesting that central obesity and the metabolic syndrome does not per se impact on the development of BE in a reflux population. In BE, the importance of obesity and the metabolic syndrome in disease progression merits further study.

  1. A Case of Central Pontine Myelinolysis Caused by Hypophosphatemia Secondary to Refeeding Syndrome.

    Science.gov (United States)

    Yamashita, Chikara; Shigeto, Hiroshi; Maeda, Norihisa; Torii, Takako; Ohyagi, Yasumasa; Kira, Jun-Ichi

    2015-01-01

    Central pontine myelinolysis (CPM), which was originally considered to be the result of rapid correction of chronic hyponatremia, is not necessarily accompanied by hyponatremia or drastic changes in serum sodium level. Here, we report a case of an anorexic 55-year-old male with a history of pharyngo-laryngo-esophagogastrectomy, initially hospitalized with status epilepticus. Although his consciousness gradually recovered as we were controlling his convulsion, it deteriorated again with new onset of anisocoria, and magnetic resonance imaging (MRI) at this point revealed CPM. Rapid change of serum sodium or osmolarity, which is often associated with CPM, had not been apparent throughout his hospitalization. Instead, a review of the serum biochemistry test results showed that serum phosphate had drastically declined the day before the MRI first detected CPM. In this case, we suspect that hypophosphatemia induced by refeeding syndrome greatly contributed to the occurrence of CPM.

  2. A Case of Central Pontine Myelinolysis Caused by Hypophosphatemia Secondary to Refeeding Syndrome

    Directory of Open Access Journals (Sweden)

    Chikara Yamashita

    2015-10-01

    Full Text Available Central pontine myelinolysis (CPM, which was originally considered to be the result of rapid correction of chronic hyponatremia, is not necessarily accompanied by hyponatremia or drastic changes in serum sodium level. Here, we report a case of an anorexic 55-year-old male with a history of pharyngo-laryngo-esophagogastrectomy, initially hospitalized with status epilepticus. Although his consciousness gradually recovered as we were controlling his convulsion, it deteriorated again with new onset of anisocoria, and magnetic resonance imaging (MRI at this point revealed CPM. Rapid change of serum sodium or osmolarity, which is often associated with CPM, had not been apparent throughout his hospitalization. Instead, a review of the serum biochemistry test results showed that serum phosphate had drastically declined the day before the MRI first detected CPM. In this case, we suspect that hypophosphatemia induced by refeeding syndrome greatly contributed to the occurrence of CPM.

  3. A Case of Turner Syndrome with Concomitant Transient Hypogammaglobulinaemia of Infancy and Central Diabetes Insipidus

    Science.gov (United States)

    Korkmaz, Hüseyin Anıl; Özkan, Behzat; Hazan, Filiz; Büyükinan, Muammer; Çelik, Tanju

    2013-01-01

    Turner syndrome (TS) is a genetic disorder that affects development in females and is characterized by the complete or partial absence of the second sex chromosome, or monosomy X. TS is associated with abnormalities in lymphatic and skeletal development, in growth, and in gonadal function. Cardiac and renal malformations and a number of specific cognitive findings may also be encountered in these patients. An increased risk for hypothyroidism, sensorineural hearing loss, hypertension, and other problems has also been reported. We present the case of a patient with TS accompanied by transient hypogammaglobulinaemia of infancy (THI) and central diabetes insipidus, which we believe is the first reported TS patient with these concomitant disorders. Conflict of interest:None declared. PMID:23419422

  4. Mutation of fibulin-1 causes a novel syndrome involving the central nervous system and connective tissues.

    Science.gov (United States)

    Bohlega, Saeed; Al-Ajlan, Huda; Al-Saif, Amr

    2014-05-01

    Fibulin-1 is an extracellular matrix protein that has an important role in the structure of elastic fibers and basement membranes of various tissues. Using homozygosity mapping and exome sequencing, we discovered a missense mutation, p.(Cys397Phe), in fibulin-1 in three patients from a consanguineous family presented with a novel syndrome of syndactyly, undescended testes, delayed motor milestones, mental retardation and signs of brain atrophy. The mutation discovered segregated with the phenotype and was not found in 374 population-matched alleles. The affected cysteine is highly conserved across vertebrates and its mutation is predicted to abolish a disulfide bond that defines the tertiary structure of fibulin-1. Our findings emphasize the crucial role fibulin-1 has in development of the central nervous system and various connective tissues.

  5. [A case of central alveolar hypoventilation syndrome associated with cerebral infarction].

    Science.gov (United States)

    Yamada, A; Kamoda, M; Ikezoe, K; Tsukaguchi, M; Katanaka, J; Deguchi, K; Miki, H; Takeuchi, H

    1993-03-01

    Central alveolar hypoventilation syndrome (CAH), or Ondine's curse, is a very rare disease characterized by dysfunction of respiratory center in the brain stem. Here, we report a case of CAH associated with cerebral infarction. A 59-year-old man developed right facial sensory deficit at age 56. Then, the facial sensory deficit spread to the left side and dysarthria and dysphagia also developed. Since age 58, he often developed respiratory failure and consciousness disturbance. Arterial blood gas analysis revealed alveolar hypoventilation and respiratory acidosis. Disorders of peripheral organs such as lung, airway, thorax and neuromuscular diseases were ruled out. Brain MRI showed cerebral infarction in the brain stem. We diagnosed him as CAH associated with brain stem infarction.

  6. Pulmonary Artery Embolotherapy in a Patient with Type I Hepatopulmonary Syndrome after Liver Transplantation

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Hae Won; Suh, Kyung Suk; Kim, Joo Hyun; Shin, Woo Young; Yi, Nam Joon; Jae, Hwan Jun; Chung, Jin Wook; Oh, So Won; Lee, Kuhn Uk [Konkuk University College of Medicine, Seoul (Korea, Republic of); Kang, Keon Wook [Seoul National University College of Medicine, Seoul (Korea, Republic of)

    2010-08-15

    Although liver transplantation (LT) is the only effective treatment option for hepatopulmonary syndrome (HPS), the post-LT morbidity and mortality have been high for patients with severe HPS. We performed post-LT embolotherapy in a 10-year-old boy who had severe type I HPS preoperatively, but he failed to recover early from his hypoxemic symptoms after an LT. Multiple embolizations were then successfully performed on the major branches that formed the abnormal vascular structures. After the embolotherapy, the patient had symptomatic improvement and he was discharged without complications

  7. Catastrophic antiphospholipid syndrome and pulmonary embolism in a 3-year-old child

    Energy Technology Data Exchange (ETDEWEB)

    Olivier, Carine; Blondiaux, Eleonore; Dacher, Jean-Nicolas [University Hospital of Rouen, Department of Radiology, Rouen (France); Blanc, Thierry [University Hospital of Rouen, Department of Neonatal Medicine, Rouen (France); Borg, Jeanne-Yvonne [University Hospital of Rouen, Haematology Laboratory, Rouen (France)

    2006-08-15

    We report a rare example of catastrophic antiphospholipid syndrome (CAPS) in a young child. A 3-year-old girl with no previous medical history presented with extensive and recurrent thromboses. The diagnosis of CAPS was based on the occurrence of cardiopulmonary embolism in the child with a high titre of autoantibodies directed against phospholipids and beta-2-glycoprotein 1. In spite of a relatively rapid diagnosis and multiple treatments, the outcome was unfavourable. Multimodality imaging, including both ultrasonography and spiral CT, allowed close follow-up of the thromboses. (orig.)

  8. Handbook of pulmonary emergencies

    Energy Technology Data Exchange (ETDEWEB)

    Spaquolo, S.V.; Medinger, A

    1986-01-01

    This book presents information on the following topics: clinical assessment of the patient with pulmonary disease; interpretation of arterial blood gases in the emergency patient; life-threatening pneumonia; extrapulmonic ventilatory failure; acute inhalation lung disease; pulmonary edema; near drowning; chest trauma; upper airway emergencies; chronic lung disease with acute respiratory decompensation; acute respiratory failure in the patient with chronic airflow obstruction; asthma; hemoptysis; embolic pulmonary disease; superior vena cava syndrome; catastrophic pleural disease; ventilatory assistance and its complications; and ventilator emergencies.

  9. An unusual case of pulmonary-renal syndrome associated with defects in type IV collagen composition and anti-glomerular basement membrane autoantibodies.

    Science.gov (United States)

    Charytan, David; MacDonald, Brian; Sugimoto, Hikaru; Pastan, Stephen; Staton, Gerald; Hennigar, Randy; Kalluri, Raghu

    2005-04-01

    Commercial serological assays for the presence of anti-glomerular basement membrane (GBM) antibodies are thought to be indicative of Goodpasture's syndrome. We report a case in which commercial tests inaccurately suggested that a patient with a pulmonary-renal syndrome had Goodpasture's disease. Additional laboratory testing using recombinant type IV collagen NC1 domain proteins showed that the autoantibodies in question were not directed against the Goodpasture antigen (the alpha3NC1 domain), but against the alpha2NC1 domain of type IV collagen. Our findings represent the first known case of human autoantibodies to the alpha2NC1 domain. Further investigation showed that this patient has decreased alpha3 and alpha5 chain expression in the GBM and defects in type IV collagen, resembling abnormalities in patients with Alport's syndrome.

  10. Central nervous system mechanisms contributing to the cachexia-anorexia syndrome.

    Science.gov (United States)

    Plata-Salamán, C R

    2000-10-01

    The cachexia-anorexia syndrome occurs in chronic pathophysiologic processes including cancer, infection with human immunodeficiency virus, bacterial and parasitic diseases, inflammatory bowel disease, liver disease, obstructive pulmonary disease, cardiovascular disease, and rheumatoid arthritis. Cachexia makes an organism susceptible to secondary pathologies and can result in death. Cachexia-anorexia may result from pain, depression or anxiety, hypogeusia and hyposmia, taste and food aversions, chronic nausea, vomiting, early satiety, malfunction of the gastrointestinal system (delayed digestion, malabsorption, gastric stasis and associated delayed emptying, and/or atrophic changes of the mucosa), metabolic shifts, cytokine action, production of substances by tumor cells, and/or iatrogenic causes such as chemotherapy and radiotherapy. The cachexia-anorexia syndrome also involves metabolic and immune changes (mediated by either the pathophysiologic process, i.e., tumor, or host-derived chemical factors, e.g., peptides, neurotransmitters, cytokines, and lipid-mobilizing factors) and is associated with hypertriacylglycerolemia, lipolysis, and acceleration of protein turnover. These changes result in the loss of fat mass and body protein. Increased resting energy expenditure in weight-losing cachectic patients can occur despite the reduced dietary intake, indicating a systemic dysregulation of host metabolism. During cachexia, the organism is maintained in a constant negative energy balance. This can rarely be explained by the actual energy and substrate demands by tumors in patients with cancer. Overall, the cachectic profile is significantly different than that observed during starvation. Cachexia may result not only from anorexia and a decreased caloric intake but also from malabsorption and losses from the body (ulcers, hemorrhage, effusions). In any case, the major deficit of a cachectic organism is a negative energy balance. Cytokines are proposed to participate

  11. Operative Treatment of Haglund Syndrome With Central Achilles Tendon-Splitting Approach.

    Science.gov (United States)

    Ahn, Jae Hoon; Ahn, Chi-Young; Byun, Chu-Hwan; Kim, Yoon-Chung

    2015-01-01

    Haglund syndrome is characterized by chronic posterior heel pain associated with a posterosuperior calcaneal prominence. We assessed the clinical and radiologic outcomes after operative treatment of Haglund syndrome using the central tendon-splitting approach. Fifteen feet in 15 patients were investigated retrospectively after surgery. Of the 15 patients, 14 were males (93.3%) and 1 was female (6.7%). Their mean age was 33.1 ± 8.2 (range 20 to 50) years. The mean follow-up duration was 3.5 ± 1.5 years (range 24 to 90 months). The American Orthopaedic Foot and Ankle Society ankle-hindfoot Scale and Victorian Institute of Sport Assessment-Achilles scores were investigated to assess the clinical outcomes. Patient satisfaction was assessed at the latest follow-up visit. The lateral talo-first metatarsal angle, calcaneal pitch angle, Fowler-Philip angle, and parallel pitch line were measured to assess the foot shape and radiographic outcomes. Clinically, the mean American Orthopaedic Foot and Ankle Society ankle-hindfoot scale score increased from 62.1 ± 7.5 preoperatively to 92.5 ± 3.5 at the latest follow-up visit. The mean Victorian Institute of Sport Assessment-Achilles score increased from 53.2 ± 7.4 to 89.6 ± 3.4. All patients were satisfied with the operative results. Radiographically, all patients had cavus feet with an increased lateral talo-first metatarsal angle (mean +5.9° ± 5.0°) and calcaneal pitch angle (mean 26.0° ± 3.8°). The mean Fowler-Philip angle decreased from 58.9° ± 15.0° to 32.5° ± 7.2° postoperatively, and the positive parallel pitch line had changed to a negative value in all cases. Operative treatment with the central tendon-splitting approach appears to be safe and satisfactory for intractable Haglund syndrome.

  12. Ordovas-Oxidized LDL is associated with metabolic syndrome traits independently of central obesity and insulin resistance

    Science.gov (United States)

    This study assesses whether oxidative stress, using oxidized LDL (ox-LDL) as a proxy, is associated with metabolic syndrome (MS), whether ox-LDL mediates the association between central obesity and MS, and whether insulin resistance mediates the association between ox-LDL and MS. We examined baselin...

  13. Pitfalls in the diagnosis of reversible cerebral vasoconstriction syndrome and primary angiitis of the central nervous system

    NARCIS (Netherlands)

    Koopman, K; Uyttenboogaart, M; Luijckx, G J; De Keyser, J; Vroomen, P C A J

    2007-01-01

    A case of a 51-year-old woman with reversible cerebral vasoconstriction syndrome (RCVS) without an associative cause is reported. Initially the diagnosis primary angiitis of the central nervous system (PACNS) was considered. Both diagnosis are rare and can mimic each other. Distinction between both

  14. Pulmonary mycoses among the clinically suspected cases of pulmonary tuberculosis

    OpenAIRE

    Tshering Ongmu Bhutia; Luna Adhikari

    2015-01-01

    Background: This study was carried with the main objectives: (1) to find out the occurrence of pulmonary mycoses in clinically suspected pulmonary tuberculosis cases at central referral hospital, Tadong, Sikkim. (2) To find out the various fungi causing pulmonary mycoses in clinically suspected pulmonary tuberculosis cases. Methods: 200 clinically suspected pulmonary tuberculosis cases who visited the department of microbiology for the diagnostic microscopic examination of sputum sample f...

  15. Neurological involvement in primary Sjogren syndrome: a focus on central nervous system.

    Directory of Open Access Journals (Sweden)

    Manuela Morreale

    Full Text Available OBJECTIVES: Sjögren syndrome is an autoimmune disease involving mainly salivary and lacrimal glands. Beyond widely described PNS involvement, high variable prevalence of CNS manifestations ranging from 2.5 and 60% of all pSS patients has been reported, without specific syndrome definition. The aim of this cohort study was to evaluate the prevalence of CNS signs and symptoms in pSS patients and to identify possible biomarkers of CNS damage. METHODS: 120 patients with pSS diagnosis according to the 2002 American-European Consensus Group criteria were enrolled after exclusion of secondary causes. All patients underwent to a wide neurological, neuropsychological, psychiatric, neuroradiological and ultrasonographic evaluation. RESULTS: Central and peripheral nervous system involvement was observed in 81 patients with a prevalence of 67.5%. The prevalence of CNS involvement was significantly higher than PNS disease (p 0.001. 68 patients (84% shown non-focal CNS symptoms and 64 (79% focal CNS deficits with headache as the most common feature (46.9%, followed by cognitive (44.4% and mood disorders (38.3%. Particularly, we observed a high prevalence of migraine without aura, subcortical frontal executive functions and verbal memory impairment and apathy/alexythimia. MR spectroscopy revealed a reduction of NAA levels or NAA/Cr ratio decrease in subcortical frontal and basal ganglia white matter, while ultrasonography showed an impairment of microvasculature response. At multivariate analysis, headache, cognitive disorders and psychiatric symptoms was significantly associated to serological markers (anti-SSA, MRS and ultrasonographic features. CONCLUSIONS: The higher prevalence of MWO-mimic headache, cognitive dys-executive syndrome and mood disorders observed in this series confirmed previous evidences of a higher diffused CNS compromission rather than focal involvement such as SM-like clinical course or NMO-like syndrome. The association with

  16. Oxidized LDL Is Associated With Metabolic Syndrome Traits Independently of Central Obesity and Insulin Resistance.

    Science.gov (United States)

    Hurtado-Roca, Yamilee; Bueno, Hector; Fernandez-Ortiz, Antonio; Ordovas, Jose Maria; Ibañez, Borja; Fuster, Valentin; Rodriguez-Artalejo, Fernando; Laclaustra, Martin

    2017-02-01

    This study assesses whether oxidative stress, using oxidized LDL (ox-LDL) as a proxy, is associated with metabolic syndrome (MS), whether ox-LDL mediates the association between central obesity and MS, and whether insulin resistance mediates the association between ox-LDL and MS. We examined baseline data from 3,987 subjects without diabetes in the Progression of Early Subclinical Atherosclerosis (PESA) Study. For the second, third, and fourth ox-LDL quartiles versus the first, the odds ratios (95% CI) for MS were 0.84 (0.52, 1.36), 1.47 (0.95, 2.32), and 2.57 (1.66, 4.04) (P insulin resistance (HOMA-IR). Results showing the same trend were found for all MS components except glucose concentration. Ox-LDL mediated 13.9% of the association of waist circumference with triglycerides and only 1-3% of the association with HDL-cholesterol, blood pressure, and insulin concentration. HOMA-IR did not mediate the association between ox-LDL and MS components. This study found higher ox-LDL concentrations were associated with MS and its components independently of central obesity and insulin resistance. Ox-LDL may reflect core mechanisms through which MS components develop and progress in parallel with insulin resistance and could be a clinically relevant predictor of MS development. © 2017 by the American Diabetes Association.

  17. Bilateral and Simultaneous Central Retinal Vein Occlusion in a Patient with Obstructive Sleep Apnea Syndrome

    Directory of Open Access Journals (Sweden)

    Andrea Govetto

    2014-05-01

    Full Text Available Purpose: To describe a case of bilateral and simultaneous central retinal vein occlusion (RVO in a young patient diagnosed with obstructive sleep apnea syndrome (OSAS. Case Report: A 38-year-old man with morbid obesity and daytime sleepiness presented with a history of bilateral vision loss. His visual acuity (VA was hand movements, and fundus examination (FE revealed bilateral central RVO. General medical examination revealed untreated hypertension and type II respiratory failure. Laboratory tests for thrombophilia showed increased hematocrit (59% and high levels of fibrinogen and C-reactive protein. Other causes of congenital and acquired hypercoagulability were ruled out. Pathologic polysomnography led to the diagnosis of OSAS. The patient was treated with antihypertensive drugs and continuous positive air pressure. In addition, he received intravitreal ranibizumab. At 10 months after presentation, his VA was no light perception in the right eye and hand movements in the left eye. FE revealed bilateral retinal and optic nerve atrophy, and the occurrence of a nonarteritic anterior ischemic neuropathy in the right eye was considered.

  18. Respiratory Failure Associated with the Lipodystrophy Syndrome in an HIV-Positive Patient with Compromised Lung Function

    Directory of Open Access Journals (Sweden)

    Natasha Press

    2001-01-01

    Full Text Available Protease inhibitors, used as treatment in human immunodeficiency virus (HIV infection, are associated with a syndrome of peripheral lipodystrophy, central adiposity, hyperlipidemia and insulin resistance. An HIV-positive patient with chronic obstructive pulmonary disease is presented who developed the lipodystrophy syndrome that is associated with the use of protease inhibitors. It is postulated that the lipodystrophy syndrome further compromised his lung function, leading to respiratory failure. Patients who have pulmonary disease and are taking protease inhibitors require monitoring of clinical status and pulmonary function tests.

  19. Bilateral Central Retinal Vein Occlusions Combined with Artery Occlusions in A Patient with Acquired Immune Deficiency Syndrome

    Institute of Scientific and Technical Information of China (English)

    Feng Wen; Xuemei Chen; Haitai Li; Ruiduan Liao; Dezheng Wu

    2002-01-01

    Purpose: This is the first report of a bilateral nonischemic central retinal vein occlusionscombined with artery occlusions in a patient with acquired immune deficiency syndrome(AIDS). Methods: Case report. Results: A 22-year-old Chinese(male) with a positive human immunodeficiency virus(HIV) infection developed bilateral nonischemic central retinal vein occlusions combinedwith artery occlusions and severe vision loss. The manifestations of the fundus andfluorescein angiography were similar in both eyes.Conclusion: This case report provides the evidences that central retinal vein and arteryocclusions are probably part of the spectrum of AIDS vascular diseases.

  20. Circulating vascular progenitor cells and central arterial stiffness in polycystic ovary syndrome.

    Directory of Open Access Journals (Sweden)

    Cecile Dessapt-Baradez

    Full Text Available OBJECTIVE: Subjects with Polycystic ovarian syndrome (PCOS are at increased risk of Type 2 diabetes mellitus (T2DM. The mechanism of this enhanced risk is unclear. Circulating vascular progenitor cells (VPC are immature bone marrow derived cells capable of differentiating into mature endothelial cells. VPC number/function and central arterial stiffness predict cardio-metabolic disease in at-risk populations. DESIGN: We studied VPC and arterial stiffness measures in non-obese PCOS subjects as compared to age and body mass index (BMI matched healthy controls in a cross-sectional study. METHODS: Fourteen subjects with PCOS and 12 controls of similar age, BMI (all <30 kg/m(2 and metabolic profile were studied. VPC number and in vitro function were studied by flow cytometry and tube formation assays respectively. Augmentation index (AIx, a measure of central arterial stiffness, and central (aortic blood pressures (BP were measured by applanation tonometry. RESULTS: Subjects with PCOS had a reduced number, mean±SEM, of circulating CD34(+133(+ VPCs (317.5±51.0 vs. 558.3±101.2, p = 0.03 and impaired in vitro tube formation (completed tube area 1.0±0.06 vs. 1.2±0.05×10(6 µm(2 p = 0.02. PCOS subjects had significantly higher AIx (18.4±1.9% vs. 4.9±2.0% and this difference remained significant even after adjustments for age, BMI and smoking (p = 0.003 in multivariate analyses. Central systolic and pulse pressure were higher in PCOS subjects but these differences were not statistically significant after adjustment for age. Brachial systolic and pulse pressures were similar. VPC number/function and arterial stiffness or BP measures were not correlated. CONCLUSIONS: Non-obese PCOS is characterized by a reduced VPC number, impaired VPC function and increased central arterial stiffness. These changes in novel vascular risk markers may explain the enhanced risk of T2DM and CVD in PCOS.

  1. Evaluation of the Endothelial Cell Density and the Central Corneal Thickness in Pseudoexfoliation Syndrome and Pseudoexfoliation Glaucoma

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    Bożydar T. Tomaszewski

    2014-01-01

    Full Text Available Purpose. Evaluation of central corneal thickness (CCT and endothelial cell density (ECD in patients with senile cataract and coexisting pseudoexfoliation (PEX syndrome with glaucoma (PEXG and without glaucoma using specular microscopy. Participants and Methods. The study included 122 patients (217 eyes. In this group of patients we identified 133 eyes with PEX syndrome (65 with glaucoma, 68 without glaucoma and 84 eyes without PEX syndrome. ECD and CCT were measured in each eye by specular microscopy. Results. ECD in eyes with PEX syndrome without glaucoma (2297 ± 359 cell/mm2 and in eyes with PEXG (2241 ± 363 cell/mm2 was lower than in the control group (2503 ± 262 cell/mm2 (P<0.001. CCT in eyes with PEXG (508.2 ± 32.6 μm was thinner than in eyes with PEX syndrome without glaucoma (529.7 ± 30.3 μm and control group (527.7 ± 29.4 μm (P<0.001. Conclusions. This research shows that in eyes with PEX syndrome, both with and without glaucoma, ECD was statistically significantly lower than in the control group. In patients with PEXG, CCT was statistically significantly thinner than in the PEX syndrome and control group.

  2. Comparison of pulmonary function in patients with COPD, asthma-COPD overlap syndrome, and asthma with airflow limitation

    Directory of Open Access Journals (Sweden)

    Kitaguchi Y

    2016-05-01

    Full Text Available Yoshiaki Kitaguchi, Masanori Yasuo, Masayuki Hanaoka First Department of Internal Medicine, Shinshu University School of Medicine, Matsumoto, Japan Background: This study was conducted in order to investigate the differences in the respiratory physiology of patients with chronic obstructive pulmonary disease (COPD, asthma-COPD overlap syndrome (ACOS, and asthma with airflow limitation (asthma FL+. Methods: The medical records for a series of all stable patients with persistent airflow limitation due to COPD, ACOS, or asthma were retrospectively reviewed and divided into the COPD group (n=118, the ACOS group (n=32, and the asthma FL+ group (n=27. All the patients underwent chest high-resolution computed tomography (HRCT and pulmonary function tests, including respiratory impedance. Results: The low attenuation area score on chest HRCT was significantly higher in the COPD group than in the ACOS group (9.52±0.76 vs 5.09±1.16, P<0.01. The prevalence of bronchial wall thickening on chest HRCT was significantly higher in the asthma FL+ group than in the COPD group (55.6% vs 25.0%, P<0.01. In pulmonary function, forced expiratory volume in 1 second (FEV1 and peak expiratory flow rate were significantly higher in the asthma FL+ group than in the ACOS group (76.28%±2.54% predicted vs 63.43%±3.22% predicted, P<0.05 and 74.40%±3.16% predicted vs 61.08%±3.54% predicted, P<0.05, respectively. Although residual volume was significantly lower in the asthma FL+ group than in the COPD group (112.05%±4.34% predicted vs 137.38%±3.43% predicted, P<0.01 and the ACOS group (112.05%±4.34% predicted vs148.46%±6.25% predicted, P<0.01, there were no significant differences in functional residual capacity or total lung capacity. The increase in FEV1 in response to short-acting ß2-agonists was significantly greater in the ACOS group than in the COPD group (229±29 mL vs 72±10 mL, P<0.01 and the asthma FL+ group (229±29 mL vs 153±21 mL, P<0.05. Regarding

  3. DNA vaccine-derived human IgG produced in transchromosomal bovines protect in lethal models of hantavirus pulmonary syndrome.

    Science.gov (United States)

    Hooper, Jay W; Brocato, Rebecca L; Kwilas, Steven A; Hammerbeck, Christopher D; Josleyn, Matthew D; Royals, Michael; Ballantyne, John; Wu, Hua; Jiao, Jin-an; Matsushita, Hiroaki; Sullivan, Eddie J

    2014-11-26

    Polyclonal immunoglobulin-based medical products have been used successfully to treat diseases caused by viruses for more than a century. We demonstrate the use of DNA vaccine technology and transchromosomal bovines (TcBs) to produce fully human polyclonal immunoglobulins (IgG) with potent antiviral neutralizing activity. Specifically, two hantavirus DNA vaccines [Andes virus (ANDV) DNA vaccine and Sin Nombre virus (SNV) DNA vaccine] were used to produce a candidate immunoglobulin product for the prevention and treatment of hantavirus pulmonary syndrome (HPS). A needle-free jet injection device was used to vaccinate TcB, and high-titer neutralizing antibodies (titers >1000) against both viruses were produced within 1 month. Plasma collected at day 10 after the fourth vaccination was used to produce purified α-HPS TcB human IgG. Treatment with 20,000 neutralizing antibody units (NAU)/kg starting 5 days after challenge with ANDV protected seven of eight animals, whereas zero of eight animals treated with the same dose of normal TcB human IgG survived. Likewise, treatment with 20,000 NAU/kg starting 5 days after challenge with SNV protected immunocompromised hamsters from lethal HPS, protecting five of eight animals. Our findings that the α-HPS TcB human IgG is capable of protecting in animal models of lethal HPS when administered after exposure provides proof of concept that this approach can be used to develop candidate next-generation polyclonal immunoglobulin-based medical products without the need for human donors, despeciation protocols, or inactivated/attenuated vaccine antigen. Copyright © 2014, American Association for the Advancement of Science.

  4. [The management of adult female patients with Eisenmenger syndrome and advanced pulmonary arterial hypertension treatment: single center experience and follow-up for 5 years].

    Science.gov (United States)

    Taçoy, Gülten; Başer, Hatice Duygu; Türkoğlu, Sedat; Cengel, Atiye

    2014-09-01

    Eisenmenger syndrome (ES) occurs as the most advanced form of pulmonary arterial hypertension (PAH) in patients with congenital heart disease. In this study, we aimed to evaluate the management of ES patients, follow-up and specific PAH treatment applying and clinical outcomes during 5 years. During the period of the month between May 2008 and 2013 ES female patients were included in the study and followed an average for 5 years. Clinical findings, brain natriuretic peptide levels, transthoracic and right heart catheterization findings, 6-min walking test distance were recorded. PAH specific treatment as bosentan, iloprost and sildenafil was given to patients according to guidelines. The patients were evaluated with 3 months intervals as requirement for hospitalization, combination treatment, and mortality. A total of 12 patients were included in the study. All of the patients were women, the mean age was 36.5. As prognostic echocardiographic data, the patients had high pulmonary artery pressure (109.81 ± 24.94 mmHg) related with increased right ventricular wall thickness, elevated right atrial pressure, severe pulmonary regurgitation in 40%, shortened pulmonary acceleration time, diminished myocardial tissue Doppler velocities of the left and right ventricles, increased right atrium area/left atrial area ratio (1.35 ± 0.40), lower right ventricular fractional area change. During the follow-up period of 5 years, a total of 16 events occurred. Combination treatment was required in 8 patients. Eisenmenger syndrome is a multi-system affecting disease and due to high morbidity and mortality risk patients with ES should be followed by specialized centers. PAH specific treatment improves the disease course and survival of patients.

  5. A Sleeve Gastrectomy Complicated by Mesenteric Vein Thrombosis, Abdominal Compartment Syndrome and Pulmonary Emboli: Case Report

    Directory of Open Access Journals (Sweden)

    Erika Leung

    2015-09-01

    Full Text Available Background: Obesity is a growing problem all over the world, including the United States. Single-incision laparoscopic sleeve gastrectomy is a surgery performed for patients who want to lose weight. The number of deaths resulting from thromboembolic complications from bariatric surgeries continues to be of major concern. Case Description: A 38-year-old female was admitted for single incision sleeve gastrectomy and was discharged home three days later. Subsequently she began to have abdominal pain, nausea and vomiting. A CT scan revealed superior mesenteric vein thrombosis with small bowel ischemia, splenic infarction and main and right portal vein branch thrombosis. An exploratory laparotomy demonstrated necrotic bowel due to abdominal compartment syndrome, and an area of small bowel was resected due to internal hernia. Surgical management of the patient during her second hospital stay included a decompressive laparotomy, internal hernia reduction, a small bowel resection. Discussion: Superior mesenteric vein thrombosis can be a life-threatening complication and present with non-specific presentations; thus, it is imperative that it is identified and managed promptly as these cases carry significant morbidity and mortality. Obese patients who undergo bariatric surgery frequently have other co-morbidities; many of which can complicate a case further. Mesenteric vein thromboses are normally treated with unfractionated or low-molecular-weight heparin.

  6. Pulmonary involvement in Churg-Strauss syndrome: an analysis of CT, clinical, and pathologic findings

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Yoon Kyung; Lee, Kyung Soo; Chong, Semin; Chung, Myung Jin; Yi, Chin A.; Kim, Ha Young [Samsung Medical Center, Sungkyunkwan University School of Medicine, Department of Radiology and Center for Imaging Science, Seoul (Korea); Chung, Man Pyo [Samsung Medical Center, Sungkyunkwan University School of Medicine, Division of Pulmonary and Critical Care Medicine, Department of Medicine, Seoul (Korea); Han, Joungho [Samsung Medical Center, Sungkyunkwan University School of Medicine, Department of Pathology, Seoul (Korea)

    2007-12-15

    We tried to assess retrospectively thin-section CT findings of Churg-Strauss syndrome (CSS) in 25 patients and to compare these findings with clinical and histopathologic findings. Of 25 patients, 19 (76%) had parenchymal abnormalities at CT; small nodules (n = 12; 63%), ground-glass opacity (n = 10; 53%), bronchial wall thickening (n = 10; 53%), and consolidation (n = 8; 42%). Parenchymal abnormalities (n = 19) were categorizable as an airway pattern in 11 and an airspace pattern in eight. Patients with an airway pattern (n = 5) had obstructive (n = 3) or combined (n = 2) PFT results, whereas those with an airspace pattern (n = 4) had restrictive (n = 3) or obstructive (n = 1) results. Parenchymal opacities at CT corresponded histologically to areas of eosinophilic pneumonia, necrotizing granulomas, and granulomatous vasculitis; small nodules to eosinophilic bronchiolitis and peribronchiolar vasculitis; and bronchial wall thickening to airway wall eosinophil and lymphocyte infiltration. Patients with airspace pattern responded more readily to treatment than those with airway pattern. CT shows lung parenchymal abnormalities in about three-quarters of CSS patients and these abnormalities can be categorized as airspace or airway patterns. This classification helps predict PFT data, underlying histopathology, and treatment response. (orig.)

  7. Hantavirus pulmonary syndrome and rodent reservoirs in the savanna-like biome of Brazil's southeastern region.

    Science.gov (United States)

    Limongi, J E; Oliveira, R C; Guterres, A; Costa Neto, S F; Fernandes, J; Vicente, L H B; Coelho, M G; Ramos, V N; Ferreira, M S; Bonvicino, C R; D'Andrea, P S; Lemos, E R S

    2016-04-01

    This paper describes the diversity of rodent fauna in an area endemic for hantavirus cardiopulmonary syndrome (HCPS) in Brazil, the population dynamics and the relationship of rodents with hantavirus in the Cerrado (savanna-like) biome. Additionally, an analysis is made of the partial S segment sequences of the hantaviruses obtained from serologically confirmed human HCPS cases and from rodent specimens. Rodents were collected during four campaigns. Human serum samples were collected from suspected cases of HCPS at hospitals in the state of Minas Gerais. The samples antibody-reactive by ELISA were processed by RT-PCR. The PCR product was amplified and sequenced. Hantavirus was detected only in Necromys lasiurus, the wild rodent species most prevalent in the Cerrado biome (min-max: 50-83·7%). All the six human serum samples were hantavirus seropositive and five showed amplified PCR products. The analysis of the nucleotide sequences showed the circulation of a single genotype, the Araraquara hantavirus. The environmental changes that have occurred in the Cerrado biome in recent decades have favoured N. lasiurus in interspecific competition of habitats, thus increasing the risk of contact between humans and rodent species infected with hantavirus. Our data corroborate the definition of N. lasiurus as the main hantavirus reservoir in the Cerrado biome.

  8. Mitochondrial dysfunction as a central actor in intellectual disability-related diseases: an overview of Down syndrome, autism, Fragile X and Rett syndrome.

    Science.gov (United States)

    Valenti, Daniela; de Bari, Lidia; De Filippis, Bianca; Henrion-Caude, Alexandra; Vacca, Rosa Anna

    2014-10-01

    Clinical manifestations typical of mitochondrial diseases are often present in various genetic syndromes associated with intellectual disability, a condition leading to deficit in cognitive functions and adaptive behaviors. Until now, the causative mechanism leading to intellectual disability is unknown and the progression of the condition is poorly understood. We first report latest advances on genetic and environmental regulation of mitochondrial function and its role in brain development. Starting from the structure, function and regulation of the oxidative phosphorylation apparatus, we review how mitochondrial biogenesis and dynamics play a central role in neurogenesis and neuroplasticity. We then discuss how dysfunctional mitochondria and alterations in reactive oxygen species homeostasis are potentially involved in the pathogenesis of various neurodevelopmental syndromes with a special focus on Down, Rett, Fragile X syndromes and autism spectrum disorders. Finally, we review and suggest novel therapeutic approaches aimed at improving intellectual disability by activating mitochondrial function and reducing oxidative stress to amiliorate the quality of life in the subjects affected.

  9. Association between sarcopenia and metabolic syndrome in chronic obstructive pulmonary disease: the Korea National Health and Nutrition Examination Survey (KNHANES) from 2008 to 2011.

    Science.gov (United States)

    Chung, Jae Ho; Hwang, Hee-Jin; Han, Chang Hoon; Son, Bong Soo; Kim, Do Hyung; Park, Moo Suk

    2015-02-01

    It is not clear whether the restrictive or obstructive pattern of spirometry is associated with metabolic syndrome. We investigated the associations between restrictive and obstructive spirometric patterns and metabolic risk factors using data from the Korea National Health and Nutrition Examination Survey (KNHANES). Additionally, we investigated whether sarcopenia is associated with metabolic syndrome in patients with chronic obstructive pulmonary disease (COPD). Using data from KNHANES between 2008 and 2011, we enrolled 8,145 subjects (normal lung function: 6,077, obstructive spirometric pattern: 1,039, restrictive pattern: 1,029) aged ≥40 years who underwent anthropometric measurement, laboratory tests, spirometry and estimation of appendicular muscle mass. Sarcopenia was defined as an appendicular skeletal muscle mass divided by body weight squared obstructive pattern in males was 0.99 (95% CI, 0.79-1.26) after adjustments for covariables (female restrictive pattern (ORs, 1,45; 95% CI, 1.09-1.91) and female obstructive pattern (ORs 0.73; 95% CI, 0.49-1.09). After adjustment for other confounding factors, the risk of metabolic syndrome was higher in sarcopenic male (OR, 1.88; 95% CI, 1.27-2.77) with COPD than in those without sarcopenia. The restrictive spirometric pattern is associated with metabolic syndrome, and sarcopenia may contribute to the risk of metabolic syndrome in male patients with COPD.

  10. Systemic 5-fluorouracil treatment causes a syndrome of delayed myelin destruction in the central nervous system

    Directory of Open Access Journals (Sweden)

    Han Ruolan

    2008-04-01

    Full Text Available Abstract Background Cancer treatment with a variety of chemotherapeutic agents often is associated with delayed adverse neurological consequences. Despite their clinical importance, almost nothing is known about the basis for such effects. It is not even known whether the occurrence of delayed adverse effects requires exposure to multiple chemotherapeutic agents, the presence of both chemotherapeutic agents and the body's own response to cancer, prolonged damage to the blood-brain barrier, inflammation or other such changes. Nor are there any animal models that could enable the study of this important problem. Results We found that clinically relevant concentrations of 5-fluorouracil (5-FU; a widely used chemotherapeutic agent were toxic for both central nervous system (CNS progenitor cells and non-dividing oligodendrocytes in vitro and in vivo. Short-term systemic administration of 5-FU caused both acute CNS damage and a syndrome of progressively worsening delayed damage to myelinated tracts of the CNS associated with altered transcriptional regulation in oligodendrocytes and extensive myelin pathology. Functional analysis also provided the first demonstration of delayed effects of chemotherapy on the latency of impulse conduction in the auditory system, offering the possibility of non-invasive analysis of myelin damage associated with cancer treatment. Conclusions Our studies demonstrate that systemic treatment with a single chemotherapeutic agent, 5-FU, is sufficient to cause a syndrome of delayed CNS damage and provide the first animal model of delayed damage to white-matter tracts of individuals treated with systemic chemotherapy. Unlike that caused by local irradiation, the degeneration caused by 5-FU treatment did not correlate with either chronic inflammation or extensive vascular damage and appears to represent a new class of delayed degenerative damage in the CNS.

  11. Congenital central hypoventilation syndrome associated with Hirschsprung's Disease: case report and literature review

    Science.gov (United States)

    Sandoval, Renata Lazari; Zaconeta, Carlos Moreno; Margotto, Paulo Roberto; Cardoso, Maria Teresinha de Oliveira; França, Evely Mirella Santos; Medina, Cristina Touguinha Neves; Canó, Talyta Matos; de Faria, Aline Saliba

    2016-01-01

    Abstract Objective: To report the case of a newborn with recurrent episodes of apnea, diagnosed with Congenital Central hypoventilation syndrome (CCHS) associated with Hirschsprung's disease (HD), configuring Haddad syndrome. Case description: Third child born at full-term to a non-consanguineous couple through normal delivery without complications, with appropriate weight and length for gestational age. Soon after birth he started to show bradypnea, bradycardia and cyanosis, being submitted to tracheal intubation and started empiric antibiotic therapy for suspected early neonatal sepsis. During hospitalization in the NICU, he showed difficulty to undergo extubation due to episodes of desaturation during sleep and wakefulness. He had recurrent episodes of hypoglycemia, hyperglycemia, metabolic acidosis, abdominal distension, leukocytosis, increase in C-reactive protein levels, with negative blood cultures and suspected inborn error of metabolism. At 2 months of age he was diagnosed with long-segment Hirschsprung's disease and was submitted to segment resection and colostomy through Hartmann's procedure. A genetic research was performed by polymerase chain reaction for CCHS screening, which showed the mutated allele of PHOX2B gene, confirming the diagnosis. Comments: This is a rare genetic, autosomal dominant disease, caused by mutation in PHOX2B gene, located in chromosome band 4p12, which results in autonomic nervous system dysfunction. CCHS can also occur with Hirschsprung's disease and tumors derived from the neural crest. There is a correlation between phenotype and genotype, as well as high intrafamilial phenotypic variability. In the neonatal period it can simulate cases of sepsis and inborn errors of metabolism. PMID:26838603

  12. F-18 FDG PET scan findings in patients with pulmonary involvement in the hypereosinophilic syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Jae Hoon; Kim, Tae Hoon; Yun, Mi Jin [College of Medicine, Yonsei University, Seoul (Korea, Republic of)] (and others)

    2005-08-15

    Hypereosinophilic syndrome (HES) is an infiltrative disease of eosinophils affecting multiple organs including the lung. F-18 2-fluoro-2-deoxyglucose (F-18 FDG) may accumulate at sites of inflammation or infection, making interpretation of whole body PET scan difficult in patients with cancer. This study was to evaluate the PET findings of HES with lung involvement and to find out differential PET features between lung malignancy and HES with lung involvement. F-18 FDG PET and low dose chest CT scan was performed for screening of lung cancer. Eight patients who showed ground-glass attenuation (GGA) and consolidation on chest CT scan with peripheral blood eosinophilia were included in this study. The patients with history of parasite infection, allergy and collagen vascular disease were excluded. CT features and FDG PET findings were meticulously evaluated for the distribution of GGA and consolidation and nodules on CT scan and mean and maximal SUV of abnormalities depicted on F-18 FDG PET scan. In eight patients, follow-up chest CT scan and FDG PET scan were done one or two weeks after initial study. F-18 FDG PET scan identified metabolically active lesions in seven out of eight patients. Maximal SUV was ranged from 2.8 to 10.6 and mean SUV was ranged from 2.2 to 7.2. Remaining one patient had maximal SUV of 1.3. On follow-up FDG PET scan taken on from one to four weeks later showed decreased degree of initially noted FDG uptakes or migration of previously noted abnormal FDG uptakes. Lung involvement in the HES might be identified as abnormal uptake foci on FDG PET scan mimicking lung cancer. Follow-up FDG PET and CT scan for the identification of migration or resolution of abnormalities and decrement of SUV would be of help for the differentiation between lung cancer and HES with lung involvement.

  13. Impaired Neural Structure and Function Contributing to Autonomic Symptoms in Congenital Central Hypoventilation Syndrome

    Directory of Open Access Journals (Sweden)

    Ronald M Harper

    2015-10-01

    Full Text Available Congenital central hypoventilation syndrome (CCHS patients show major autonomic alterations in addition to their better-known breathing deficiencies. The processes underlying CCHS, mutations in the PHOX2B gene, target autonomic neuronal development, with frame shift extent contributing to symptom severity. Many autonomic characteristics, such as impaired pupillary constriction and poor temperature regulation, reflect parasympathetic alterations, and can include disturbed alimentary processes, with malabsorption and intestinal motility dyscontrol. The sympathetic nervous system changes can exert life-threatening outcomes, with dysregulation of sympathetic outflow leading to high blood pressure, time-altered and dampened heart rate and breathing responses to challenges, cardiac arrhythmia, profuse sweating, and poor fluid regulation. The central mechanisms contributing to failed autonomic processes are readily apparent from structural and functional magnetic resonance imaging studies, which reveal substantial cortical thinning, tissue injury, and disrupted functional responses in hypothalamic, hippocampal, posterior thalamic, and basal ganglia sites and their descending projections, as well as insular, cingulate, and medial frontal cortices, which influence subcortical autonomic structures. Midbrain structures are also compromised, including the raphe system and its projections to cerebellar and medullary sites, the locus coeruleus, and medullary reflex integrating sites, including the dorsal and ventrolateral medullary nuclei. The damage to rostral autonomic sites overlaps metabolic, affective and cognitive regulatory regions, leading to hormonal disruption, anxiety, depression, behavioral control, and sudden death concerns. The injuries suggest that interventions for mitigating hypoxic exposure and nutrient loss may provide cellular protection, in the same fashion as interventions in other conditions with similar malabsorption, fluid turnover

  14. Pulmonary oedema associated with acidosis in patients with cholera.

    Science.gov (United States)

    Greenough, W B; Hirschhorn, N; Gordon, R S; Lindenbaum, J; Ally, K M

    1976-06-01

    Five patients with severe acidosis and pulmonary oedema complicating cholera were seen at the Cholera Research Laboratory, Dacca, in a two-year period. All had had inadequate treatment. Their disease resulted in acidosis prior to admission; only the two who subsequently survived received volumes of sodium bicarbonate solutions sufficiently large to repair completely their acidosis. Saline alone worsened pulmonary congestion, while alkali appeared to relieve it despite the accompanying volume expansion. These observations are consistent with the known redistribution of blood to the central circulation in acidosis. Timely and proper treatment of cholera will avert this syndrome, when use of isotonic sodium bicarbonate sufficient to correct acidosis may be very helpful.

  15. Guidelines for the prevention of central venous catheter-related blood stream infections with prostanoid therapy for pulmonary arterial hypertension

    OpenAIRE

    Doran, A. K.; Ivy, D. D.; Barst, R.J.; Hill, N.; Murali, S; Benza, R. L.

    2008-01-01

    Intravenous prostanoids are the backbone of therapy for advanced pulmonary arterial hypertension (PAH) and have improved long-term outcome and quality of life. Currently, two prostanoids are approved by the US Food and Drug administration for parenteral administration: epoprostenol (Flolan) and treprostinil (Remodulin). Chronic intravenous therapy presents considerable challenges for patients and caregivers who must learn sterile preparation of the medication, operation of the pump, and care ...

  16. Anterior cervical surgery methods for central cord syndrome without radiographic spinal fracture-dislocation

    Institute of Scientific and Technical Information of China (English)

    Chengwei JING; Qin FU; Xiaojun XU

    2009-01-01

    This study was aimed to explore the anterior cervical surgery methods to treat central cord syndrome without radiographic spinal fracture-dislocation (CCSWORFD), retrospectively analyze the cases of CCSWORFD, and evaluate the curative effect of anterior cervical surgery methods for CCSWORFD. Twenty four cases of CCSWORFD (19 males and 5 females), all suffering from cervical hyperextension injury, between 45-68 (average 59) years old, were operated on by anterior cervical surgery methods. Among these, 18 cases had been followed up for 6-24 (average 15) months; 18 cases, who had anterior decompression and plate fixation with titanium mesh bone grafting or iliac bone grafting achieved reliable effects based on the Japanese Orthopedics Association (JOA) evaluation (improved scores of cases with titanium mesh bone grafting, t = 2.800, P0.05). Most of these cases had degeneration of cervical vertebra. The decompression which relieves the oppression to the spinal cord can help lessen edema of the spinal cord, and early fixation for stability of cervical vertebra is better for the recovery of spinal cord injury. Anterior operation with titanium mesh bone grafting or iliac bone grafting are both reliable curative methods for CCSWORFD, and titanium mesh bone grafting can avoid the trauma of the supplying graft. Mesh bone grafting can also shorten hospital stay.

  17. Similar deficits of central histaminergic system in patients with Down syndrome and Alzheimer disease.

    Science.gov (United States)

    Schneider, C; Risser, D; Kirchner, L; Kitzmüller, E; Cairns, N; Prast, H; Singewald, N; Lubec, G

    1997-02-01

    In order to study whether Alzheimer-like neuropathological changes involve the central histaminergic system we measured the concentration of histamine, its precursor histidine as well as the activity of histidine decarboxylase (HDC) and histamine-N-methyl-transferase (HMT) in frontal cortex of aging Down syndrome (DS) patients, Alzheimer patients and control individuals. The study populations were also investigated for choline acetyltransferase (ChAT) activity, since reduced ChAT activity is an established biochemical hallmark in DS and Alzheimer disease (AD). HDC and ChAT activity were reduced in brains of both DS and Alzheimer patients versus control patients. Additionally, we observed a significant decrease of histamine levels in the DS group. Histamine levels in AD brains tended to be decreased. Histidine concentrations and HMT activities were comparable between the three groups. Thus, our results for the first time show histaminergic deficits in brains of patients with DS resembling the neurochemical pattern in AD. Neuropathological changes may be responsible for similar neurochemical alterations of the histaminergic system in both dementing disorders.

  18. Central nervous system involvement in primary Sjögren's syndrome

    Directory of Open Access Journals (Sweden)

    Xiao-yu DONG

    2015-04-01

    Full Text Available Clinical and imaging data of 4 cases of primary Sjögren's syndrome (pSS combined with central nervous system (CNS lesions were retrospectively analyzed. Clinical symptoms of 4 patients were as follows: 2 cases had fever; 2 cases had blurred vision; 2 cases had lower extremity weakness; one case had single lower limb weakness; one case had unilateral limb weakness; one case had hemisensory reduction; one case had bilateral abducens paralysis; 3 cases had peripheral neuropathy. Analysis of cerebrospinal fluid (CSF revealed increased CSF pressure (210-270 mm H2O in 3 cases, increased number of leukocyte [(50-380 × 10 6/L] in 3 cases, and increased protein (1.30-2.56 g/L in 3 cases. Brain MRI showed multiple lesions in white matters. After high-dose steroid therapy, 3 cases had good prognosis, and one had recurrent lower extremity weakness after 6 months. pSS combined with CNS lesions is rare. Lumbar puncture and brain MRI is important for diagnosis, and high-dose steroid therapy may have good therapeutic effect. DOI: 10.3969/j.issn.1672-6731.2015.04.014

  19. [Plasma metabonomic studies on the stable phase chronic obstructive pulmonary disease patients of Fei-qi deficiency syndrome and the Chinese materia medica intervention].

    Science.gov (United States)

    Liu, Zhi-gang; Li, Ze-geng; Peng, Bo

    2011-12-01

    By using the metabonomics method, to study the plasma metabonomics of the stable phase chronic obstructive pulmonary disease (COPD) patients of Fei-qi deficiency syndrome (FQDS), and of Chinese materia medica (CMM) intervention, thus exploring possibly existent biomarkers. Forty stable phase COPD patients of FQDS were recruited as Group A. Liuwei Buqi Capsule (LWBQC) was given to them as intervention. A healthy control group (Group B, 37 cases) was set up. The pulmonary function test was performed on patients in Group B and Group A before and after intervention. The plasma metabolites were detected using high performance liquid chromatography-mass spectrometry (HPLC-MS/MS). Statistical data were analyzed using principal component analysis (PCA) and partial least squares (PLS). The original spectrum and data of plasma metabonomics were compared between the two groups. The whole spectrum amino acid metabonomics tests were performed in the two groups. Compared with Group B, the pulmonary function significantly decreased before intervention in Group A (P plasma metabonomics, and potential markers could be detected. The intervention of the stable phase COPD patients of FQDS by Chinese medicine could brought positive changes in the metabolic profiling and amino acid metabolism.

  20. Extracorporeal membrane oxygenation as a platform for recovery: a case report of a child with pulmonary hemorrhage, refractory hypoxemic respiratory failure, and new onset goodpasture syndrome.

    Science.gov (United States)

    Dalabih, Abdallah; Pietsch, John; Jabs, Kathy; Hardison, Daphne; Bridges, Brian C

    2012-06-01

    We report a case of a 9-year-old female with acute pulmonary hemorrhage and refractory hypoxemic respiratory failure secondary to Goodpasture syndrome (GS). After failing treatment with high frequency oscillatory ventilation and inhaled nitric oxide, she was successfully managed with venovenous extracorporeal membrane oxygenation (VV ECMO). The patient's weight at the time of cannulation was 31 kg. A 19 French 18 cm (arterial) Biomedicus cannula was inserted in the right internal jugular vein and used as the drain. A 17 French 50 cm (venous) Biomedicus cannula was inserted in the right femoral vein and used as the return. Then the patient was anticoagulated with 100 units/kg of intravenous heparin and the circuit was primed with one unit of packed red blood cells. VV ECMO was performed with an SIII Sorin roller head pump with integrated servo regulator and a Quadrox D Bioline coated oxygenator. Despite systemic anticoagulation with heparin, the patient's pulmonary hemorrhage resolved. Extracorporeal membrane oxygenation served as a platform through which we were able to provide renal replacement therapy and plasmapheresis. The patient was successfully discharged home with normal pulmonary function.

  1. Pulmonary edema

    Science.gov (United States)

    ... congestion; Lung water; Pulmonary congestion; Heart failure - pulmonary edema ... Pulmonary edema is often caused by congestive heart failure . When the heart is not able to pump efficiently, blood ...

  2. Chronic obstructive pulmonary disease

    Directory of Open Access Journals (Sweden)

    V K Vijayan

    2013-01-01

    Full Text Available The global prevalence of physiologically defined chronic obstructive pulmonary disease (COPD in adults aged >40 yr is approximately 9-10 per cent. Recently, the Indian Study on Epidemiology of Asthma, Respiratory Symptoms and Chronic Bronchitis in Adults had shown that the overall prevalence of chronic bronchitis in adults >35 yr is 3.49 per cent. The development of COPD is multifactorial and the risk factors of COPD include genetic and environmental factors. Pathological changes in COPD are observed in central airways, small airways and alveolar space. The proposed pathogenesis of COPD includes proteinase-antiproteinase hypothesis, immunological mechanisms, oxidant-antioxidant balance, systemic inflammation, apoptosis and ineffective repair. Airflow limitation in COPD is defined as a postbronchodilator FEV1 (forced expiratory volume in 1 sec to FVC (forced vital capacity ratio <0.70. COPD is characterized by an accelerated decline in FEV1. Co morbidities associated with COPD are cardiovascular disorders (coronary artery disease and chronic heart failure, hypertension, metabolic diseases (diabetes mellitus, metabolic syndrome and obesity, bone disease (osteoporosis and osteopenia, stroke, lung cancer, cachexia, skeletal muscle weakness, anaemia, depression and cognitive decline. The assessment of COPD is required to determine the severity of the disease, its impact on the health status and the risk of future events (e.g., exacerbations, hospital admissions or death and this is essential to guide therapy. COPD is treated with inhaled bronchodilators, inhaled corticosteroids, oral theophylline and oral phosphodiesterase-4 inhibitor. Non pharmacological treatment of COPD includes smoking cessation, pulmonary rehabilitation and nutritional support. Lung volume reduction surgery and lung transplantation are advised in selected severe patients. Global strategy for the diagnosis, management and prevention of Chronic Obstructive Pulmonary Disease

  3. Chronic obstructive pulmonary disease.

    Science.gov (United States)

    Vijayan, V K

    2013-02-01

    The global prevalence of physiologically defined chronic obstructive pulmonary disease (COPD) in adults aged >40 yr is approximately 9-10 per cent. Recently, the Indian Study on Epidemiology of Asthma, Respiratory Symptoms and Chronic Bronchitis in Adults had shown that the overall prevalence of chronic bronchitis in adults >35 yr is 3.49 per cent. The development of COPD is multifactorial and the risk factors of COPD include genetic and environmental factors. Pathological changes in COPD are observed in central airways, small airways and alveolar space. The proposed pathogenesis of COPD includes proteinase-antiproteinase hypothesis, immunological mechanisms, oxidant-antioxidant balance, systemic inflammation, apoptosis and ineffective repair. Airflow limitation in COPD is defined as a postbronchodilator FEV1 (forced expiratory volume in 1 sec) to FVC (forced vital capacity) ratio disease and chronic heart failure), hypertension, metabolic diseases (diabetes mellitus, metabolic syndrome and obesity), bone disease (osteoporosis and osteopenia), stroke, lung cancer, cachexia, skeletal muscle weakness, anaemia, depression and cognitive decline. The assessment of COPD is required to determine the severity of the disease, its impact on the health status and the risk of future events (e.g., exacerbations, hospital admissions or death) and this is essential to guide therapy. COPD is treated with inhaled bronchodilators, inhaled corticosteroids, oral theophylline and oral phosphodiesterase-4 inhibitor. Non pharmacological treatment of COPD includes smoking cessation, pulmonary rehabilitation and nutritional support. Lung volume reduction surgery and lung transplantation are advised in selected severe patients. Global strategy for the diagnosis, management and prevention of Chronic Obstructive Pulmonary Disease guidelines recommend influenza and pneumococcal vaccinations.

  4. Central Hypoventilation Syndrome Complicated with Lateral Medullary Infarction after Endovascular Treatment of the Vertebral Artery Dissecting Aneurysm: A Case Report

    OpenAIRE

    TANAKA, Katsuhiro; Kanamaru, Hideki; Morikawa, Atsunori; Kawaguchi, Kenji

    2016-01-01

    Lateral medullary infarction rarely leads to central hypoventilation syndrome (CHS). CHS is a life-threatening disorder characterized by hypoventilation during sleep. We report the first case of CHS as a complication of lateral medullary infarction after endovascular treatment. A 65-year-old man presented twice with severe headache. Computed tomography revealed subarachnoid hemorrhage and cerebral angiography showed a right vertebral dissecting aneurysm involving the posterior inferior cerebe...

  5. Wilson-Mikity综合征的X线及CT诊断%Pulmonary CT and radiography in the diagnosis of Wilson-Mikity syndrome

    Institute of Scientific and Technical Information of China (English)

    赵如恩; 吴慧莹; 曹亚先; 艾斌; 肖伟强; 刘立炜

    2012-01-01

    目的:探讨X线及CT在Wilson-Mikity综合征诊断中的应用价值.方法:回顾性分析16例临床确诊的Wilson-Mikity综合征患儿的临床及影像学资料.16例患儿在疾病的早、中、晚期均进行了胸部X线检查,7例行CT检查.结果:7例出生后1周内胸片未见异常;16例出生后2~3周内胸片显示:13例双肺弥漫性片状和索条状间质浸润,14例其间有小的囊状透亮区出现;双肺基底部多个小囊状病灶,其中5例融合扩大并扩展到上肺野以下肺野明显,肺透亮度增加.7例胸部CT表现为间质性肺炎、小囊状透亮影及小叶性肺气肿.结论:X线及CT能反映Wilson-Mikity综合征的特征性影像表现,对诊断有重要价值,在明确病灶范围及形态上,CT较X线更有优势.%Objective: To study the value of pulmonary radiography and CT in-the diagnosis of Wilson-Mikity syndrome (WMS). Methods:The clinical and imaging materials of 16 cases with clinically diagnosed Wilson-Mikity syndrome were analyzed retrospectively. Pulmonary radiography was performed in early,mid and advanced periods of all 16 cases and 7 cases had CT plain scan. Results:The major findings of pulmonary radiography and CT of Wilson-Mikity syndrome were as follows:no abnormal changes were observed in 7 cases within 1 week after parturition. Of the 16 cases checked 2~3 weeks after parturition,there were bilateral diffuse patchy and streaky interstitial infiltrations throughout the lung (13 cases), small cystic translucency within the above-mentioned areas (14 cases) was observed. In 5 cases, there were multiple small sacciform translucencies within bilateral basal parts of lungs,part of which coalesced and expanded to the upper lung fields with over-distension of lower lung fields presented as pulmonary hyper-translucency. Conclusion: Characteristic imaging appearances of Wilison-Mikity syndrome could be displayed on CT and pulmonary radiography, which provide significant value for the diagnosis. CT

  6. Hereditary renal adysplasia, pulmonary hypoplasia and Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome: a case report

    Science.gov (United States)

    2010-01-01

    Background Hereditary renal adysplasia is an autosomal dominant trait with incomplete penetrance and variable expression that is usually associated with malformative combinations (including Müllerian anomalies) affecting different mesodermal organs such as the heart, lung, and urogenital system. Case report A case showing pulmonary hypoplasia, hip dysplasia, hereditary renal adysplasia, and Mayer-Rokitansky-Kuster-Hauser syndrome in adulthood is reported here. The i.v. pyelography showed right renal agenesis with a normal left kidney and ureter. Ultrasound and Magnetic Resonance Imaging also showed right renal agenesis with multicystic embryonary remnants in the right hemipelvis probably corresponding to a dysgenetic kidney. An uretrocystoscopy showed absence of ectopic ureter and of the right hemitrigone. She was scheduled for a diagnostic laparoscopy and creation of a neovagina according to the McIndoe technique with a prosthesis and skin graft. Laparoscopy confirmed the absence of the uterus. On both sides, an elongated, solid, rudimentary uterine horn could be observed. Both ovaries were also elongated, located high in both abdominal flanks and somewhat dysgenetics. A conventional cytogenetic study revealed a normal female karyotype 46, XX at a level of 550 GTG bands. A CGH analysis was performed using a 244K oligoarray CGH detecting 11 copy number variants described as normal variants in the databases. The 17q12 and 22q11.21 microdeletions described in other MRKH patients were not present in this case. Four years after operation her evolution is normal, without symptoms and the neovagina is adequately functional. The geneticists have studied her family history and the pedigree of the family is shown. Conclusions We suggest that primary damage to the mesoderm (paraaxil, intermediate, and lateral) caused by mutations in a yet unidentified gene is responsible for: 1) skeletal dysplasia, 2) inappropriate interactions between the bronchial mesoderm and endodermal

  7. Hereditary renal adysplasia, pulmonary hypoplasia and Mayer-Rokitansky-Küster-Hauser (MRKH syndrome: a case report

    Directory of Open Access Journals (Sweden)

    Acién Maribel

    2010-04-01

    Full Text Available Abstract Background Hereditary renal adysplasia is an autosomal dominant trait with incomplete penetrance and variable expression that is usually associated with malformative combinations (including Müllerian anomalies affecting different mesodermal organs such as the heart, lung, and urogenital system. Case report A case showing pulmonary hypoplasia, hip dysplasia, hereditary renal adysplasia, and Mayer-Rokitansky-Kuster-Hauser syndrome in adulthood is reported here. The i.v. pyelography showed right renal agenesis with a normal left kidney and ureter. Ultrasound and Magnetic Resonance Imaging also showed right renal agenesis with multicystic embryonary remnants in the right hemipelvis probably corresponding to a dysgenetic kidney. An uretrocystoscopy showed absence of ectopic ureter and of the right hemitrigone. She was scheduled for a diagnostic laparoscopy and creation of a neovagina according to the McIndoe technique with a prosthesis and skin graft. Laparoscopy confirmed the absence of the uterus. On both sides, an elongated, solid, rudimentary uterine horn could be observed. Both ovaries were also elongated, located high in both abdominal flanks and somewhat dysgenetics. A conventional cytogenetic study revealed a normal female karyotype 46, XX at a level of 550 GTG bands. A CGH analysis was performed using a 244K oligoarray CGH detecting 11 copy number variants described as normal variants in the databases. The 17q12 and 22q11.21 microdeletions described in other MRKH patients were not present in this case. Four years after operation her evolution is normal, without symptoms and the neovagina is adequately functional. The geneticists have studied her family history and the pedigree of the family is shown. Conclusions We suggest that primary damage to the mesoderm (paraaxil, intermediate, and lateral caused by mutations in a yet unidentified gene is responsible for: 1 skeletal dysplasia, 2 inappropriate interactions between the bronchial

  8. DNA vaccine-generated duck polyclonal antibodies as a postexposure prophylactic to prevent hantavirus pulmonary syndrome (HPS.

    Directory of Open Access Journals (Sweden)

    Rebecca Brocato

    Full Text Available Andes virus (ANDV is the predominant cause of hantavirus pulmonary syndrome (HPS in South America and the only hantavirus known to be transmitted person-to-person. There are no vaccines, prophylactics, or therapeutics to prevent or treat this highly pathogenic disease (case-fatality 35-40%. Infection of Syrian hamsters with ANDV results in a disease that closely mimics human HPS in incubation time, symptoms of respiratory distress, and disease pathology. Here, we evaluated the feasibility of two postexposure prophylaxis strategies in the ANDV/hamster lethal disease model. First, we evaluated a natural product, human polyclonal antibody, obtained as fresh frozen plasma (FFP from a HPS survivor. Second, we used DNA vaccine technology to manufacture a polyclonal immunoglobulin-based product that could be purified from the eggs of vaccinated ducks (Anas platyrhynchos. The natural "despeciation" of the duck IgY (i.e., Fc removed results in an immunoglobulin predicted to be minimally reactogenic in humans. Administration of ≥ 5,000 neutralizing antibody units (NAU/kg of FFP-protected hamsters from lethal disease when given up to 8 days after intranasal ANDV challenge. IgY/IgYΔFc antibodies purified from the eggs of DNA-vaccinated ducks effectively neutralized ANDV in vitro as measured by plaque reduction neutralization tests (PRNT. Administration of 12,000 NAU/kg of duck egg-derived IgY/IgYΔFc protected hamsters when administered up to 8 days after intranasal challenge and 5 days after intramuscular challenge. These experiments demonstrate that convalescent FFP shows promise as a postexposure HPS prophylactic. Moreover, these data demonstrate the feasibility of using DNA vaccine technology coupled with the duck/egg system to manufacture a product that could supplement or replace FFP. The DNA vaccine-duck/egg system can be scaled as needed and obviates the necessity of using limited blood products obtained from a small number of HPS survivors. This

  9. DNA Vaccine-Generated Duck Polyclonal Antibodies as a Postexposure Prophylactic to Prevent Hantavirus Pulmonary Syndrome (HPS)

    Science.gov (United States)

    Brocato, Rebecca; Josleyn, Matthew; Ballantyne, John; Vial, Pablo; Hooper, Jay W.

    2012-01-01

    Andes virus (ANDV) is the predominant cause of hantavirus pulmonary syndrome (HPS) in South America and the only hantavirus known to be transmitted person-to-person. There are no vaccines, prophylactics, or therapeutics to prevent or treat this highly pathogenic disease (case-fatality 35–40%). Infection of Syrian hamsters with ANDV results in a disease that closely mimics human HPS in incubation time, symptoms of respiratory distress, and disease pathology. Here, we evaluated the feasibility of two postexposure prophylaxis strategies in the ANDV/hamster lethal disease model. First, we evaluated a natural product, human polyclonal antibody, obtained as fresh frozen plasma (FFP) from a HPS survivor. Second, we used DNA vaccine technology to manufacture a polyclonal immunoglobulin-based product that could be purified from the eggs of vaccinated ducks (Anas platyrhynchos). The natural “despeciation" of the duck IgY (i.e., Fc removed) results in an immunoglobulin predicted to be minimally reactogenic in humans. Administration of ≥5,000 neutralizing antibody units (NAU)/kg of FFP-protected hamsters from lethal disease when given up to 8 days after intranasal ANDV challenge. IgY/IgYΔFc antibodies purified from the eggs of DNA-vaccinated ducks effectively neutralized ANDV in vitro as measured by plaque reduction neutralization tests (PRNT). Administration of 12,000 NAU/kg of duck egg-derived IgY/IgYΔFc protected hamsters when administered up to 8 days after intranasal challenge and 5 days after intramuscular challenge. These experiments demonstrate that convalescent FFP shows promise as a postexposure HPS prophylactic. Moreover, these data demonstrate the feasibility of using DNA vaccine technology coupled with the duck/egg system to manufacture a product that could supplement or replace FFP. The DNA vaccine-duck/egg system can be scaled as needed and obviates the necessity of using limited blood products obtained from a small number of HPS survivors. This is the

  10. Antiviral Biologic Produced in DNA Vaccine/Goose Platform Protects Hamsters Against Hantavirus Pulmonary Syndrome When Administered Post-exposure

    Science.gov (United States)

    Henderson, Thomas; Nilles, Matthew L.; Kwilas, Steve A.; Josleyn, Matthew D.; Hammerbeck, Christopher D.; Schiltz, James; Royals, Michael; Ballantyne, John; Hooper, Jay W.; Bradley, David S.

    2015-01-01

    Andes virus (ANDV) and ANDV-like viruses are responsible for most hantavirus pulmonary syndrome (HPS) cases in South America. Recent studies in Chile indicate that passive transfer of convalescent human plasma shows promise as a possible treatment for HPS. Unfortunately, availability of convalescent plasma from survivors of this lethal disease is very limited. We are interested in exploring the concept of using DNA vaccine technology to produce antiviral biologics, including polyclonal neutralizing antibodies for use in humans. Geese produce IgY and an alternatively spliced form, IgYΔFc, that can be purified at high concentrations from egg yolks. IgY lacks the properties of mammalian Fc that make antibodies produced in horses, sheep, and rabbits reactogenic in humans. Geese were vaccinated with an ANDV DNA vaccine encoding the virus envelope glycoproteins. All geese developed high-titer neutralizing antibodies after the second vaccination, and maintained high-levels of neutralizing antibodies as measured by a pseudovirion neutralization assay (PsVNA) for over 1 year. A booster vaccination resulted in extraordinarily high levels of neutralizing antibodies (i.e., PsVNA80 titers >100,000). Analysis of IgY and IgYΔFc by epitope mapping show these antibodies to be highly reactive to specific amino acid sequences of ANDV envelope glycoproteins. We examined the protective efficacy of the goose-derived antibody in the hamster model of lethal HPS. α-ANDV immune sera, or IgY/IgYΔFc purified from eggs, were passively transferred to hamsters subcutaneously starting 5 days after an IM challenge with ANDV (25 LD50). Both immune sera, and egg-derived purified IgY/IgYΔFc, protected 8 of 8 and 7 of 8 hamsters, respectively. In contrast, all hamsters receiving IgY/IgYΔFc purified from normal geese (n=8), or no-treatment (n=8), developed lethal HPS. These findings demonstrate that the DNA vaccine/goose platform can be used to produce a candidate antiviral biological product

  11. Antiviral Biologic Produced in DNA Vaccine/Goose Platform Protects Hamsters Against Hantavirus Pulmonary Syndrome When Administered Post-exposure.

    Directory of Open Access Journals (Sweden)

    Nicole Haese

    Full Text Available Andes virus (ANDV and ANDV-like viruses are responsible for most hantavirus pulmonary syndrome (HPS cases in South America. Recent studies in Chile indicate that passive transfer of convalescent human plasma shows promise as a possible treatment for HPS. Unfortunately, availability of convalescent plasma from survivors of this lethal disease is very limited. We are interested in exploring the concept of using DNA vaccine technology to produce antiviral biologics, including polyclonal neutralizing antibodies for use in humans. Geese produce IgY and an alternatively spliced form, IgYΔFc, that can be purified at high concentrations from egg yolks. IgY lacks the properties of mammalian Fc that make antibodies produced in horses, sheep, and rabbits reactogenic in humans. Geese were vaccinated with an ANDV DNA vaccine encoding the virus envelope glycoproteins. All geese developed high-titer neutralizing antibodies after the second vaccination, and maintained high-levels of neutralizing antibodies as measured by a pseudovirion neutralization assay (PsVNA for over 1 year. A booster vaccination resulted in extraordinarily high levels of neutralizing antibodies (i.e., PsVNA80 titers >100,000. Analysis of IgY and IgYΔFc by epitope mapping show these antibodies to be highly reactive to specific amino acid sequences of ANDV envelope glycoproteins. We examined the protective efficacy of the goose-derived antibody in the hamster model of lethal HPS. α-ANDV immune sera, or IgY/IgYΔFc purified from eggs, were passively transferred to hamsters subcutaneously starting 5 days after an IM challenge with ANDV (25 LD50. Both immune sera, and egg-derived purified IgY/IgYΔFc, protected 8 of 8 and 7 of 8 hamsters, respectively. In contrast, all hamsters receiving IgY/IgYΔFc purified from normal geese (n=8, or no-treatment (n=8, developed lethal HPS. These findings demonstrate that the DNA vaccine/goose platform can be used to produce a candidate antiviral

  12. INVESTIGATION OF CENTRAL HEMODYNAMICS VIA RIGHT HEART AND PULMONARY ARTERY CATHETERIZATION IN PATIENTS WITH SYSTEMIC CONNECTIVE TISSUE DISEASES

    Directory of Open Access Journals (Sweden)

    E. V. Nikolaeva

    2015-01-01

    Full Text Available Pulmonary arterial hypertension (PAH associated with systemic connective tissue diseases (SCTD is a poor prognostic manifestation of the latter that result in death if untreated. The invasive determination of hemodynamic parameters is prominent in diagnosing the disease and determining its treatment policy and prognosis.Objective: to analyze the results of catheterization in PAH-SCTD patients admitted to the V.A. Nasonova Research Institute of Rheumatology.Subjects and methods. The investigation included 59 patients admitted to the V.A. Nasonova Research Institute of Rheumatology from September 2009 to September 2014. PAH was diagnosed in accordance with the conventional guidelines. All the patients underwent right heart and pulmonary artery (PA catheterization at the diagnosis and over time during treatment.Results and discussion. All the patients included in the trial met the pre-capillary pulmonary hypertension (PH criteria: mean pulmonary artery pressure (MPAP ≥25 mm Hg; and PA wedge pressure (PAWP <15 mm Hg. The exclusion of other causes of PH (pulmonary fibrosis, left heart disease, and thromboembolism, as well as a high transpulmonary pressure gradient >15 mm Hg and pulmonary vascular resistance (PVR >3 Wood units could diagnose PAH in all our patients. There was a statistically highly significant association between pathological hemodynamic changes and functional class (FC. FC was found to be most closely correlated with right atrial pressure (RAP, cardiac output (CO, PVR, and cardiac index (CI. Among the most common manifestations of heart failure, only the presence of peripheral edemas was associated with worse hemodynamic parameters in PAH. It should be noted that out of two biomarkers (N-terminal pro-brain natriuretic peptide and uric acid, the former is largely related to the magnitude of changes in hemodynamic factors. The critical values of hemodynamic parameters were due to extreme edema – anasarca (RAP >17 mm Hg

  13. 肾综合征出血热和汉坦病毒肺综合征研究进展%Hantavirus pulmonary syndrome and hemorrhagic fever with renal syndrome

    Institute of Scientific and Technical Information of China (English)

    白雪帆; 王平忠

    2011-01-01

    肾综合征出血热(hemorrhagic fever with renal syndrome,HFRS)和汉坦病毒肺综合征(hantavirus pulmonary syndrome,HPS)均是由汉坦病毒属病毒(hantaviruses)引起的急性传染病.HFRS的病原体——汉滩病毒(Hantaan virus,HTNV)、汉城病毒(SEOV)、普马拉病毒(PUUV)和多布拉伐病毒(DOBV)等主要分布于有着几千年文明历史的欧亚大陆,国际上称为旧世界汉坦病毒(hantavirus in old world);

  14. Distribution of Wfs1 protein in the central nervous system of the mouse and its relation to clinical symptoms of the Wolfram syndrome

    DEFF Research Database (Denmark)

    Luuk, H.; Koks, S.; Plaas, M.;

    2008-01-01

    Mutations in the coding region of the WFS1 gene cause Wolfram syndrome, a rare multisystem neurodegenerative disorder of autosomal recessive inheritance. Patients with Wolfram syndrome display considerable clinical pleiomorphism, and symptoms such as neurological complications and psychiatric dis...... and psychiatric symptoms found in Wolfram syndrome. Enrichment of Wfs1 protein in the central extended amygdala suggests a role in the modulation of anxiety and fear Udgivelsesdato: 2008/8/20...

  15. Gait kinematic analysis in patients with a mild form of central cord syndrome

    Directory of Open Access Journals (Sweden)

    Crespo-Ruiz Beatriz

    2011-02-01

    Full Text Available Abstract Background Central cord syndrome (CCS is considered the most common incomplete spinal cord injury (SCI. Independent ambulation was achieved in 87-97% in young patients with CCS but no gait analysis studies have been reported before in such pathology. The aim of this study was to analyze the gait characteristics of subjects with CCS and to compare the findings with a healthy age, sex and anthropomorphically matched control group (CG, walking both at a self-selected speed and at the same speed. Methods Twelve CCS patients and a CG of twenty subjects were analyzed. Kinematic data were obtained using a three-dimensional motion analysis system with two scanner units. The CG were asked to walk at two different speeds, at a self-selected speed and at a slower one, similar to the mean gait speed previously registered in the CCS patient group. Temporal, spatial variables and kinematic variables (maximum and minimum lower limb joint angles throughout the gait cycle in each plane, along with the gait cycle instants of occurrence and the joint range of motion - ROM were compared between the two groups walking at similar speeds. Results The kinematic parameters were compared when both groups walked at a similar speed, given that there was a significant difference in the self-selected speeds (p Conclusions The gait pattern of CCS patients showed a decrease of knee and ankle sagittal ROM during level walking and an increase in hip abduction to increase base of support. The findings of this study help to improve the understanding how CCS affects gait changes in the lower limbs.

  16. The cerebral cost of breathing: an FMRI case-study in congenital central hypoventilation syndrome.

    Directory of Open Access Journals (Sweden)

    Mike Sharman

    Full Text Available Certain motor activities--like walking or breathing--present the interesting property of proceeding either automatically or under voluntary control. In the case of breathing, brainstem structures located in the medulla are in charge of the automatic mode, whereas cortico-subcortical brain networks--including various frontal lobe areas--subtend the voluntary mode. We speculated that the involvement of cortical activity during voluntary breathing could impact both on the "resting state" pattern of cortical-subcortical connectivity, and on the recruitment of executive functions mediated by the frontal lobe. In order to test this prediction we explored a patient suffering from central congenital hypoventilation syndrome (CCHS, a very rare developmental condition secondary to brainstem dysfunction. Typically, CCHS patients demonstrate efficient cortically-controlled breathing while awake, but require mechanically-assisted ventilation during sleep to overcome the inability of brainstem structures to mediate automatic breathing. We used simultaneous EEG-fMRI recordings to compare patterns of brain activity between these two types of ventilation during wakefulness. As compared with spontaneous breathing (SB, mechanical ventilation (MV restored the default mode network (DMN associated with self-consciousness, mind-wandering, creativity and introspection in healthy subjects. SB on the other hand resulted in a specific increase of functional connectivity between brainstem and frontal lobe. Behaviorally, the patient was more efficient in cognitive tasks requiring executive control during MV than during SB, in agreement with her subjective reports in everyday life. Taken together our results provide insight into the cognitive and neural costs of spontaneous breathing in one CCHS patient, and suggest that MV during waking periods may free up frontal lobe resources, and make them available for cognitive recruitment. More generally, this study reveals how the

  17. Diurnal variation in central corneal thickness and intraocular pressure in eyes with pseudoexfoliation syndrome without glaucoma

    Directory of Open Access Journals (Sweden)

    Stuart Keel

    2014-01-01

    Full Text Available Aim: The aim was to ascertain if any differences exist in diurnal central corneal thickness (CCT and intra-ocular pressure (IOP between eyes with pseudoexfoliation (PXF syndrome without glaucoma and eyes with no ocular pathology. A secondary aim was to determine whether there was a significant relationship between CCT and IOP. Settings and Design: This study was a prospective design conducted within a hospital setting. Materials and Methods: The experimental group consisted of seven participants with bilateral PXF (14 eyes and the control group comprised of 15 participants (30 eyes. Testing included CCT and IOP measured at four different times on one given day (8.00 a.m.; 11 a.m.; 2 p.m. and 5 p.m.. Statistical Analysis: The data were analyzed with the generalized linear latent mixed model. Results: PXF eyes displayed a significantly thinner overall mean CCT (520 μm compared to controls (530 μm. Furthermore, a significant reduction in CCT and IOP occurred in the PXF group from 8 a.m. to 5 p.m. The mean overall IOP in PXF eyes was significantly lower than the control group. A significant association between IOP and CCT was also found in PXF eyes. Conclusions: Displaying a significantly thinner mean CCT highlights the importance of measuring CCT in an ophthalmic clinical setting as to avoid falsely underestimated IOP measurements in such a high-risk glaucoma population. Furthermore, a statistically significant correlation between IOP and CCT in PXF eyes suggests that the reduction in CCT that occurred in PXF eyes between 8 a.m. and 5 p.m. may be partly responsible for the reduction in IOP measurements.

  18. The central role of the non alcoholic fatty liver disease in metabolic syndrome.

    Science.gov (United States)

    Hurjui, Daniela Maria; Niţă, Otilia; Graur, Lidia Iuliana; Mihalache, Laura; Popescu, Dana Stefana; Graur, Mariana

    2012-01-01

    Non-alcoholic fatty liver disease (NAFLD) covers a spectrum of liver disease from steatosis to non-alcoholic steatohepatitis (NASH) and cirrhosis. Most NAFLD patients are hyperinsulinaemic and more insulin resistant compared with nonsteatotic healthy subjects, and there is a near universal association between NAFLD and insulinresistance (IR) irrespective of obesity. The metabolic syndrome (MS) is highly prevalent in the general adult population (approximatively 22%) and it carries an increased cardiovascular morbidity and mortality. Pathophysiologic considerations, clinical associations, and laboratory investigations support that IR and hyperinsulinaemia have a central role in pathogenesis of both MS and NAFLD. The fatty liver is resistant to the action of insulin to suppress hepatic glucose production, which results in hyperglycaemia and, further, in hyperinsulinemia. The MS is associated with maldistribution of body fat, increased free fatty acids (FFAs) and IR, leading to type 2 diabetes, hypertension, dyslipidemia. Visceral fat is an important clinical marker of metabolic cardiovascular risk and a marker of IR in multiple tissues, independent of body mass index (BMI). NAFLD and atherosclerosis share common molecular mediators and NAFLD itself might play an early role in the development and progression of atherosclerosis. These data suggest that NAFLD should be considered part of a multi-organ system derangement in insulin sensitivity, and help explain why NAFLD is so closely linked with diabetes, MS and is an important risk factor for coronary heart disease. NAFLD may be the hepatic manifestation of the MS and raises the possibility that it may play an early role in the etiology of MS.

  19. Predictor of Mortality in Acquired Immunodeficiency Syndrome Patients with Central Nervous System Opportunistic Infections

    Directory of Open Access Journals (Sweden)

    Agustina Lestari

    2016-12-01

    Full Text Available Background: Indonesia’s increase of acquired immunodeficiency syndrome (AIDS cases is one of the fastest. Mortality cases of AIDS also increase per year. Central nervous system (CNS opportunistic infection is one of the most likely manifestations on advanced stage of Human immunodeficiency virus (HIV/AIDS patients. Common CNS opportunistic infections are toxoplasma infection and tuberculous meningitis (TBM. The study aimed to analyze the predictor of mortality in AIDS patients with CNS opportunistic infections. Methods: This study reviewed 151 medical records from AIDS patients with CNS opportunistic infection admitted to the Department of Neurology, Dr. Hasan Sadikin General Hospital Bandung from 2007-2012. This study was conducted from April to November 2013. Patients’ clinical manifestations (seizure, headache, altered consciousness, laboratory examination (CD4+ level count and treatment history (antiretroviral or ART and cotrimoxazole were collected. Chi-square and logistic regression test were used to determine the mortality predictor in patients. Results: Mortality rate was 37.7%. Clinical manifestations from patients were seizure 29.8%, altered consciousness 66.2%, and headache 88.7%. Patients had cotrimoxazole treatment 44.4% and 38.4% patients had ART. CD4+ level count data from 86 patients were obtained, 94% had CD4+ level count ≤200. Bivariate analysis showed altered consciousness had significant mortality predictor (Odd ratio (OR: 29.944; 95% Confidence interval (CI 6.9–129.945; p<0,0. Multivariate analysis showed ART had highest predictive mortality value (OR: 2.968; 95% CI 1.236–7.126; p=0.015. Conclusions: Altered consciousness and no antiretroviral treatment are mortality predictors in AIDS patients with CNS opportunistic infections.

  20. Ophthalmologic impairment during adulthood in central congenital hypoventilation syndrome: a longitudinal cohort analysis of nine patients.

    Science.gov (United States)

    Boulanger-Scemama, Elise; Fardeau, Christine; Straus, Christian; Simon, Claude; Touitou, Valérie; Touhami, Sara; Amini, Maryam; Similowski, Thomas; LeHoang, Phuc

    2014-12-01

    Congenital central hypoventilation syndrome (CCHS) is a rare dysgenetic form of neurocristopathy associated with dysfunction of the autonomic nervous system. Ophthalmologic abnormalities are reported in CCHS children, and range from pupillary and iris abnormalities to ptosis, strabismus and convergence deficiency. Since earlier CCHS diagnosis and multidisciplinary management, combined with improved ventilatory support techniques, the lifespan of children with CCHS has been considerably lengthened. The oldest of them have now reached adult age and we report in this study the results of their ophthalmologic examination. Nine CCHS adult patients were prospectively included during a 14-month period. Each patient underwent complete ophthalmologic examination, static pupillometry with scotopic and photopic pupillary diameter (PD) measures, Humphrey 24-2 visual field analysis, macular OCT and complete orthoptic assessment including a Hess-Lancaster test. Ophthalmologic abnormalities were found in six of the nine patients (66%). The main features were strabismus in six patients (66%). Four patients (44%) displayed abnormal pupillary function, with a decrease in average scotopic PD (3.8 ± 1.4 mm), average photopic PD (3.5 ± 1.2 mm), and average percentage of pupillary constriction (7.6 ± 8.5%). Three patients (33%) exhibited iris abnormalities such as iris atrophy, smooth iris surface and atrophic sphincter. This study allowed the description of ophthalmologic abnormalities occurring in CCHS in a series of adult patients, thus improving current knowledge of the disease. The prevalence of pupillary and iris lesions were lower than those observed in a series of children, suggesting that they could be considered as systemic disease severity markers.

  1. Treatment of neuroblastoma in congenital central hypoventilation syndrome with a PHOX2B polyalanine repeat expansion mutation: New twist on a neurocristopathy syndrome.

    Science.gov (United States)

    Armstrong, Amy E; Weese-Mayer, Debra E; Mian, Amir; Maris, John M; Batra, Vandana; Gosiengfiao, Yasmin; Reichek, Jennifer; Madonna, Mary Beth; Bush, Jonathan W; Shore, Richard M; Walterhouse, David O

    2015-11-01

    Neuroblastoma in patients with congenital central hypoventilation syndrome (CCHS) as part of a neurocristopathy syndrome is a rare finding and has only been associated with paired-like homeobox 2b (PHOX2B) non-polyalanine-repeat-expansion mutations. To the best of our knowledge, we report the first case of a child with CCHS and Hirschsprung disease who had a PHOX2B polyalanine-repeat-expansion mutation (PARM) (genotype 20/33) and developed high-risk neuroblastoma. We further describe his treatment including chemotherapy and therapeutic I(131) -metaiodobenzylguanidine. This case highlights the need to consider neuroblastoma in patients with CCHS and the longest PHOX2B PARMs and to individualize treatment based on co-morbidities.

  2. Nutritional status of adult patients with pulmonary tuberculosis in rural central India and its association with mortality.

    Science.gov (United States)

    Bhargava, Anurag; Chatterjee, Madhuri; Jain, Yogesh; Chatterjee, Biswaroop; Kataria, Anju; Bhargava, Madhavi; Kataria, Raman; D'Souza, Ravi; Jain, Rachna; Benedetti, Andrea; Pai, Madhukar; Menzies, Dick

    2013-01-01

    Under-nutrition is a known risk factor for TB and can adversely affect treatment outcomes. However, data from India are sparse, despite the high burden of TB as well as malnutrition in India. We assessed the nutritional status at the time of diagnosis and completion of therapy, and its association with deaths during TB treatment, in a consecutive cohort of 1695 adult patients with pulmonary tuberculosis in rural India during 2004 - 2009.Multivariable logistic regression was used to obtain adjusted estimates of the association of nutritional status with deaths during treatment. At the time of diagnosis, median BMI and body weights were 16.0 kg/m(2)and 42.1 kg in men, and 15.0 kg/m(2)and 34.1 kg in women, indicating that 80% of women and 67% of men had moderate to severe under-nutrition (BMInutrition. Half of women and one third of men remained moderately to severely underweight at the end of treatment. 60 deaths occurred in 1179 patients (5%) in whom treatment was initiated. Severe under-nutrition at diagnosis was associated with a 2 fold higher risk of death. Overall, a majority of patients had evidence of chronic severe under-nutrition at diagnosis, which persisted even after successful treatment in a significant proportion of them. These findings suggest the need for nutritional support during treatment of pulmonary TB in this rural population.

  3. Rare case of Primary Pulmonary Extranodal Non-Hodgkin’s Lymphoma in a Patient with Sjogrens Syndrome: Role of FDG-PET/CT in the Initial Staging and Evaluating Response to Treatment

    Directory of Open Access Journals (Sweden)

    Gonca G. Bural

    2012-12-01

    Full Text Available A 64-year old woman with a long standing Sjogren’s syndrome was undergoing evaluation for renal transplant surgery when two pulmonary opacities were detected on chest CT. Subsequent biopsy revealed extranodal marginal B-cell non-Hodgkin’s lymphoma (NHL. An FDG-PET/CT scan was then performed which demonstrated isolated FDG avid pulmonary involvement. After therapy, FDG-PET/CT scans showed good response to treatment with near complete resolution of FDG avidity. This rare case illustrates the rare pulmonary manifestation of extranodal lymphoma in a patient with Sjogren’s syndrome and emphasizes the value of FDG PET/CT in the initial staging and evaluation of response to treatment, which has not previously been published. (MIRT 2012;21:117-120

  4. Reversible cerebral vasoconstriction syndrome (RCVS) in antiphospholipid antibody syndrome (APLA): the role of centrally acting vasodilators. Case series and review of literature.

    Science.gov (United States)

    Gupta, Sarthak; Zivadinov, Robert; Ramasamy, Deepa; Ambrus, Julian L

    2014-12-01

    Reversible cerebral vasoconstriction syndrome (RCVS) is Raynaud's phenomenon of the brain. Changes in neurological function are dependent upon which areas of the brain are deprived of normal blood flow. Antiphospholipid antibody syndrome (APLA) is a common cause of Raynaud's phenomenon that can occur anywhere in the body, including the brain. Management of CNS vasospasm generally involves the use of centrally acting calcium channel blockers, which have been shown to relieve the associated headaches and transient neurological symptoms associated with it. Three patients with APLA and RCVS from our clinics are illustrated. It is demonstrated that the use of centrally acting calcium channel-blocking drugs, such as nimodipine, which prevent and reverse CNS vasospasm, led to clinical improvement in our patients over the course of 5-9 years. All of them had MRIs done at the initiation of therapy and 5-9 years after being on therapy. MRI measures of T2 lesion volumes (LVs) and number were obtained. All three patients had a good response in controlling clinical symptoms related to CNS vasospasm, Raynaud's phenomenon, visual disturbances, confusion, headaches, and hearing loss. There was also a resolution in the MRI findings of these patients. This case series of three patients shows a clinical improvement and decrease in T2 LV and number in patients with APLA and Raynaud's syndrome on centrally acting calcium channel blockers. RCVS is much more common than that currently appreciated. APLA is the common cause of RCVS. Further studies are needed to determine the optimal methods to diagnose RCVS and optimal therapies to treat it.

  5. The clinical usefulness of extravascular lung water and pulmonary vascular permeability index to diagnose and characterize pulmonary edema: a prospective multicenter study on the quantitative differential diagnostic definition for acute lung injury/acute respiratory distress syndrome

    Science.gov (United States)

    2012-01-01

    Introduction Acute lung injury (ALI)/acute respiratory distress syndrome (ARDS) is characterized by features other than increased pulmonary vascular permeability. Pulmonary vascular permeability combined with increased extravascular lung water content has been considered a quantitative diagnostic criterion of ALI/ARDS. This prospective, multi-institutional, observational study aimed to clarify the clinical pathophysiological features of ALI/ARDS and establish its quantitative diagnostic criteria. Methods The extravascular lung water index (EVLWI) and the pulmonary vascular permeability index (PVPI) were measured using the transpulmonary thermodilution method in 266 patients with PaO2/FiO2 ratio ≤ 300 mmHg and bilateral infiltration on chest radiography, in 23 ICUs of academic tertiary referral hospitals. Pulmonary edema was defined as EVLWI ≥ 10 ml/kg. Three experts retrospectively determined the pathophysiological features of respiratory insufficiency by considering the patients' history, clinical presentation, chest computed tomography and radiography, echocardiography, EVLWI and brain natriuretic peptide level, and the time course of all preceding findings under systemic and respiratory therapy. Results Patients were divided into the following three categories on the basis of the pathophysiological diagnostic differentiation of respiratory insufficiency: ALI/ARDS, cardiogenic edema, and pleural effusion with atelectasis, which were noted in 207 patients, 26 patients, and 33 patients, respectively. EVLWI was greater in ALI/ARDS and cardiogenic edema patients than in patients with pleural effusion with atelectasis (18.5 ± 6.8, 14.4 ± 4.0, and 8.3 ± 2.1, respectively; P edema or pleural effusion with atelectasis patients (3.2 ± 1.4, 2.0 ± 0.8, and 1.6 ± 0.5; P edema patients. A PVPI value of 2.6 to 2.85 provided a definitive diagnosis of ALI/ARDS (specificity, 0.90 to 0.95), and a value < 1.7 ruled out an ALI/ARDS diagnosis (specificity, 0.95). Conclusion

  6. Hantavirus Pulmonary Syndrome (HPS)

    Science.gov (United States)

    ... Clinical Information General Hantavirus Technical/Clinical Information Ecology Virology Airborne Transmission HPS Technical/Clinical Information Clinical Manifestation Treatment Histopathology Pathology/ Pathogenesis Diagnostics ...

  7. Bilateral upper-extremity deep vein thrombosis following central cord syndrome

    OpenAIRE

    Onmez, Hilal; Cingoz, Havva Turac; Kucuksen, Sami; Anliacık, Emel; Yaşar, Ozan; Yilmaz, Halim; Salli, Ali

    2013-01-01

    Deep vein thrombosis (DVT) is a common complication following spinal cord injury (SCI). Although DVT of the upper extremity is much less common than DVT of the lower extremities, the risk of pulmonary embolism following upper-extremity DVT should not be disregarded.

  8. Septic ankle with purulence tracking up the flexor hallucis longus tendon sheath leading to deep venous thrombosis/pulmonary embolism and compartment syndrome.

    Science.gov (United States)

    Waryasz, Gregory R; McClure, Philip; Vopat, Bryan G

    2015-06-01

    The differential diagnosis for lower extremity swelling and ankle pain is broad and can have overlapping and related diagnoses. If there is concern for more than one diagnosis, the practitioner should perform a thorough physical examination, order the appropriate studies, and perform the correct procedures to completely diagnose and treat the patient. This article presents the case of a 19-year-old male who presented with 5 days of left ankle pain, fevers, and swelling without any known trauma to the area. Physical examination was concerning for a septic ankle joint, cellulitis, deep venous thrombosis, and compartment syndrome. Duplex venous ultrasound confirmed a deep venous thrombosis in the popliteal vein. Joint aspiration of the ankle had gross purulence with the presence of methicillin-resistant Staphylococcus aureus. The patient was taken emergently to the operating room where he was found to have gross purulence in the deep posterior compartment, medial and lateral soft tissues of the ankle, and gross purulence in the ankle joint. The deep posterior compartment also had significant muscle necrosis and evidence of compartment syndrome. This case presents the possibility of a septic ankle leading to compartment syndrome and deep venous thrombosis/pulmonary embolism due to the intra-articular nature of the flexor hallucis longus tendon sheath. Case report, Level IV. © 2014 The Author(s).

  9. Unilateral central retinal artery occlusion as the sole presenting sign of Susac syndrome in a young man: case report

    Directory of Open Access Journals (Sweden)

    Samira Luiza dos Apóstolos-Pereira

    2013-06-01

    Full Text Available We report the case of a 24-year-old man presenting with sudden visual loss in the left eye from a central retinal artery occlusion. An extensive clinical investigation revealed no etiology. Three weeks later, however, the patient developed hearing loss followed by encephalopathy and multiple branch retinal artery occlusions in the right eye. Fluorescein angiography confirmed retinal vascular occlusions with no sign of vasculitis. The neurological examination revealed a diffuse encephalopathy while the MRI scan disclosed several small areas of infarcts in the brain. Bilateral sensorineural hearing loss was confirmed on audiometry. The patient was diagnosed with Susac syndrome and treated with methylprednisolone and cyclophosphamide, resulting in slight improvement and stabilization. This case shows that Susac syndrome may be diagnosed late due to the absence at onset of one or more of the symptoms of the classic triad (encephalopathy, multiple branch retinal artery occlusions and hearing loss. This case also serves to emphasize that Susac syndrome should be considered in the differential diagnosis of central retinal artery occlusion, even in apparently healthy young men.

  10. Pulmonary manifestations of leptospirosis

    Directory of Open Access Journals (Sweden)

    Sameer Gulati

    2012-01-01

    Full Text Available Leptospirosis has a spectrum of presentation which ranges from mild disease to a severe form comprising of jaundice and renal failure. Involvement of the lung can vary from subtle clinical features to deadly pulmonary hemorrhage and acute respiratory distress syndrome. Of late, it has been identified that leptospirosis can present atypically with predominant pulmonary manifestations. This can delay diagnosis making and hence optimum treatment. The purpose of this review is to bring together all the reported pulmonary manifestations of leptospirosis and the recent trends in the management.

  11. Superior vena cava syndrome associated with right-to left shunt through systemic-to-pulmonary venous collaterals

    Energy Technology Data Exchange (ETDEWEB)

    Juan, Yu Hsiang; Saboo, Sachin S.; Anand, Vishal; Chatzizisis, Yiannis S.; Steigner, Michael L. [Brigham and Women' s Hospital, Harvard Medical School, Boston (United States); Lin, Yu Ching [Chang Gung Memorial Hospital, Keelung and Chang Gung University, Keelung (China)

    2014-04-15

    Superior vena cava (SVC) obstruction is associated with the gradual development of venous collaterals. We present a rare form of systemic-to-pulmonary subpleural collateral pathway that developed in the bridging subpleural pulmonary veins in a 54-year-old woman with complete SVC obstruction. This uncommon collateral pathway represents a rare form of acquired right-to-left shunt due to previous pleural adhesions with an increased risk of stroke due to right-to-left venous shunting, which requires lifelong anticoagulation.

  12. Dispersal syndromes related to edge distance in cerrado sensu stricto fragments of central-western Brazil

    Directory of Open Access Journals (Sweden)

    André Vitor Fleuri Jardim

    2009-10-01

    Full Text Available The main selective forces affecting the fruiting strategies are related to the environment in which plants occur. As a savanna, microclimatic conditions should not vary in relation to distance from edge in cerrado sensu stricto fragments. Thus, we postulated that the importance of different dispersal syndromes would not vary towards the fragment core. Our aim was to test in four cerrado sensu stricto fragments in central Brazil whether the absolute density of anemo-, auto-, and zoochorous individuals varied in relation to edge distance. According to results, the absolute density of anemo-and autochorous individuals did not vary, whereas those of zoochorous individuals increased with edge distance, pointing out that there were other factors rather than abiotic conditions shifting zoochorous species to the interior of cerrado sensu stricto fragments.As principais forças seletivas que afetam as estratégias de frutificação estão relacionadas ao ambiente em que as plantas ocorrem. Como fragmentos de cerrado sensu stricto são savânicos, as condições microclimáticas não devem variar em relação à distância da borda. Assim, postulamos que a importância das diferentes síndromes de dispersão não varia da borda ao interior de um fragmento de vegetação. O objetivo deste trabalho foi testar, em quatro fragmentos de cerrado sensu stricto (centro-oeste do Brasil, se a densidade absoluta de indivíduos anemo, auto e zoocóricos variava em função da distância da borda. Segundo nossos resultados, a densidade absoluta de indivíduos anemo e autocóricos não variou significativamente borda em direção ao interior dos fragmentos, enquanto que a dos indivíduos zoocóricos aumentou, indicando que existem outros fatores, que não as condições abióticas, deslocando as espécies zoocóricas para o interior dos fragmentos de cerrado sensu stricto.

  13. Central Hypoventilation Syndrome Complicated with Lateral Medullary Infarction after Endovascular Treatment of the Vertebral Artery Dissecting Aneurysm: A Case Report.

    Science.gov (United States)

    Tanaka, Katsuhiro; Kanamaru, Hideki; Morikawa, Atsunori; Kawaguchi, Kenji

    2016-10-01

    Lateral medullary infarction rarely leads to central hypoventilation syndrome (CHS). CHS is a life-threatening disorder characterized by hypoventilation during sleep. We report the first case of CHS as a complication of lateral medullary infarction after endovascular treatment. A 65-year-old man presented twice with severe headache. Computed tomography revealed subarachnoid hemorrhage and cerebral angiography showed a right vertebral dissecting aneurysm involving the posterior inferior cerebellar artery. After emergent endovascular patent artery occlusion, he developed Wallenberg syndrome and experienced apnea and a conscious disturbance episode due to CHS on postoperative days 6 and 16. Intensive respiratory care including intubation, tracheostomy, mechanical ventilation, and rehabilitation prevented subsequent recurrence of apnea and the CHS resolved completely. CHS after unilateral medullary infarction involving respiratory centers tends to occur in the acute and subacute phase and may be lethal without careful respiratory management.

  14. Free testosterone level correlated with the metabolic abnormalities dependent on central obesity in women with polycystic ovary syndrome.

    Science.gov (United States)

    Dong, Z; Chen, X; Li, L; Huang, J; Yin, Q; Yang, D

    2012-06-01

    Women with polycystic ovary syndrome (PCOS) have increased risks of developing metabolic abnormalities compared with the women without PCOS. Hyperandrogenemia is one of the most important characteristics of PCOS. However, the correlations between hyperandrogenemia and metabolic disorders are uncertain. To elucidate the relationship between androgen indices and metabolic abnormalities in Chinese women with PCOS. A retrospective analysis of the anthropometric and biochemical records of 408 women with PCOS. The prevalence of metabolic syndrome (MetS) was 15.7% in women with PCOS. No association existed between total testosterone (TT) and metabolic profile. Free testosterone (FT) correlated with most of the metabolic variables by unadjusted correlation analyses. The women with elevated FT levels exhibited more unfavorable metabolic profiles compared with the women with normal FT levels. After adjusting for the confounding factors by multivariate logistic regression analysis, the women with elevated FT levels had higher prevalence of central obesity than the women with normal FT levels (odds ratio [OR] 2.346, p=0.042). Women with reduced sex hormone-binding globulin levels were more likely to have central obesity, overweight, raised fasting glucose, insulin resistance, and raised diastolic blood pressure. Raised dehydroepiandrosterone sulfate (DHEAS) level was associated with a lower probability of having central obesity (OR 0.293, p=0.001) and overweight (OR 0.47, p=0.023). FT has closer association with metabolic parameters than TT. FT can involve in the development of metabolic disorders dependent on central obesity. Raised DHEAS level can reduce the risks of central obesity and overweight. © J. A. Barth Verlag in Georg Thieme Verlag KG Stuttgart · New York.

  15. Klinefelter's syndrome in azoospermic infertile males of Vidarbha region, Central India

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    Gajanan L. Maske

    2016-04-01

    Results: Three subjects had a chromosomal count of 47 in all the metaphase studied. The additional chromosome was closely matching with the X chromosome. Hence the karyotype showed numerical aberration with an extra and lsquo;X' chromosome i. e. 47, XXY suggestive of Klinefelter's syndrome. This was confirmed by G-banding. All of the three subjects had bilateral testicular atrophy and one had typical features of Klinefelter's syndrome except gynecomastia. Conclusions: On cytogenetic analysis of 30 azoospermic infertile subjects, chromosomal abnormality of 47, XXY (Klinefelter's syndrome was found in 3 subjects. The total percentage of Klinefelter's syndrome in present study comes to 10%. [Int J Res Med Sci 2016; 4(4.000: 1045-1050

  16. Animal models of Central Diabetes Insipidus: Human relevance of acquired beyond hereditary syndromes and the role of oxytocin.

    Science.gov (United States)

    Bernal, Antonio; Mahía, Javier; Puerto, Amadeo

    2016-07-01

    The aim of this study was to review different animal models of Central Diabetes Insipidus, a neurobiological syndrome characterized by the excretion of copious amounts of diluted urine (polyuria), a consequent water intake (polydipsia), and a rise in the serum sodium concentration (hypernatremia). In rodents, Central Diabetes Insipidus can be caused by genetic disorders (Brattleboro rats) but also by various traumatic/surgical interventions, including neurohypophysectomy, pituitary stalk compression, hypophysectomy, and median eminence lesions. Regardless of its etiology, Central Diabetes Insipidus affects the neuroendocrine system that secretes arginine vasopressin, a neurohormone responsible for antidiuretic functions that acts trough the renal system. However, most Central Diabetes Insipidus models also show disorders in other neurobiological systems, specifically in the secretion of oxytocin, a neurohormone involved in body sodium excretion. Although the hydromineral behaviors shown by the different Central Diabetes Insipidus models have usually been considered as very similar, the present review highlights relevant differences with respect to these behaviors as a function of the individual neurobiological systems affected. Increased understanding of the relationship between the neuroendocrine systems involved and the associated hydromineral behaviors may allow appropriate action to be taken to correct these behavioral neuroendocrine deficits.

  17. Miliary tuberculosis with no pulmonary involvement in myelodysplastic syndromes: a curable, yet rarely diagnosed, disease: case report and review of the literature

    Directory of Open Access Journals (Sweden)

    Krambovitis Elias

    2008-03-01

    Full Text Available Abstract Background Although tuberculosis is not uncommon among patients with myelodysplastic syndrome (MDS, only a few reports of such patients suffering from miliary tuberculosis (MT exist. MT often presents as a fever of unknown origin and it is a curable disease, yet fatal if left untreated. Case presentation We report a case of MT with no clinical or laboratory indications of pulmonary involvement in a patient with MDS, and review the relevant literature. Mycobacterium tuberculosis was isolated from the liquid culture of a bone marrow aspirate. Conclusion Even if the initial diagnostic investigation for a fever of obscure etiology is negative, MT should not be excluded from the differential diagnosis list. Since it is a curable disease, persistent and vigorous diagnostic efforts are warranted. In suspected cases, mycobacterial blood cultures should be collected as soon as possible after hospital admission and early bone marrow aspirate with mycobacterial cultures is advocated.

  18. 汉坦病毒肺综合征研究的若干进展%Advancements in researches of hantavirus pulmonary syndrome

    Institute of Scientific and Technical Information of China (English)

    罗端德

    2001-01-01

    @@ 自从1993年5月美国西南部四角地区暴发以急性呼吸衰竭为主要表现的汉坦病毒肺综合征(Hantavirus pulmonary syndrome,HPS)以来,目前美国28个州均有病例发现.除美国外,美洲的加拿大、巴西、巴拉圭、阿根廷、智利、玻利维亚以及欧洲的德国、南斯拉夫、瑞典、比利时等国均报告了发生HPS的病例.我国是汉坦病毒感染的高发区,是否存在此病值得警惕.现将近年来国外研究情况简述如下.

  19. Caplan syndrome

    Science.gov (United States)

    ... CT scan of the chest Joint x-rays Pulmonary function tests Rheumatoid factor test and other blood tests Treatment There is no specific treatment for Caplan syndrome, other than treating any lung and joint disease. ... MD, MHS, Associate Professor of Medicine, Pulmonary, Allergy, and Critical Care, Perelman School of Medicine, ...

  20. Pulmonary ultrasound and pulse oximetry versus chest radiography and arterial blood gas analysis for the diagnosis of acute respiratory distress syndrome: a pilot study.

    Science.gov (United States)

    Bass, Cameron M; Sajed, Dana R; Adedipe, Adeyinka A; West, T Eoin

    2015-07-21

    In low-resource settings it is not always possible to acquire the information required to diagnose acute respiratory distress syndrome (ARDS). Ultrasound and pulse oximetry, however, may be available in these settings. This study was designed to test whether pulmonary ultrasound and pulse oximetry could be used in place of traditional radiographic and oxygenation evaluation for ARDS. This study was a prospective, single-center study in the ICU of Harborview Medical Center, a referral hospital in Seattle, Washington, USA. Bedside pulmonary ultrasound was performed on ICU patients receiving invasive mechanical ventilation. Pulse oximetric oxygen saturation (SpO2), partial pressure of oxygen (PaO2), fraction of inspired oxygen (FiO2), provider diagnoses, and chest radiograph closest to time of ultrasound were recorded or interpreted. One hundred and twenty three ultrasound assessments were performed on 77 consecutively enrolled patients with respiratory failure. Oxygenation and radiographic criteria for ARDS were met in 35 assessments. Where SpO2 ≤ 97%, the Spearman rank correlation coefficient between SpO2/FiO2 and PaO2/FiO2 was 0.83, p arterial blood gas testing and chest radiography are not readily available.

  1. Normal Uricemia in Lesch–Nyhan Syndrome and the Association with Pulmonary Embolism in a Young Child—A Case Report and Literature Review

    Directory of Open Access Journals (Sweden)

    Jeng-Dau Tsai

    2014-08-01

    Full Text Available Deficiency of hypoxanthine phosphoribosyltransferase activity is a rare inborn error of purine metabolism with subsequent uric acid overproduction and neurologic presentations. The diagnosis of Lesch–Nyhan syndrome (LNS is frequently delayed until self-mutilation becomes evident. We report the case of a boy aged 1 year and 10 months who was diagnosed with profound global developmental delay, persistent chorea, and compulsive self-mutilation since the age of 1 year. Serial serum uric acid levels showed normal uric acid level, and the spot urine uric acid/creatinine ratio was >2. The hypoxanthine phosphoribosyltransferase cDNA showed the deletion of exon 6, and the boy was subsequently diagnosed to have LNS. He also had respiratory distress due to pulmonary embolism documented by chest computed tomography scan. This report highlights the need to determine the uric acid/creatinine ratio caused by increased renal clearance in LNS in young children. The presence of pulmonary embolism is unusual and may be the consequence of prolonged immobilization.

  2. Normal uricemia in Lesch-Nyhan syndrome and the association with pulmonary embolism in a young child-a case report and literature review.

    Science.gov (United States)

    Tsai, Jeng-Dau; Chen, Shan-Ming; Lin, Chien-Heng; Ku, Min-Sho; Tsao, Teng-Fu; Sheu, Ji-Nan

    2014-08-01

    Deficiency of hypoxanthine phosphoribosyltransferase activity is a rare inborn error of purine metabolism with subsequent uric acid overproduction and neurologic presentations. The diagnosis of Lesch-Nyhan syndrome (LNS) is frequently delayed until self-mutilation becomes evident. We report the case of a boy aged 1 year and 10 months who was diagnosed with profound global developmental delay, persistent chorea, and compulsive self-mutilation since the age of 1 year. Serial serum uric acid levels showed normal uric acid level, and the spot urine uric acid/creatinine ratio was >2. The hypoxanthine phosphoribosyltransferase cDNA showed the deletion of exon 6, and the boy was subsequently diagnosed to have LNS. He also had respiratory distress due to pulmonary embolism documented by chest computed tomography scan. This report highlights the need to determine the uric acid/creatinine ratio caused by increased renal clearance in LNS in young children. The presence of pulmonary embolism is unusual and may be the consequence of prolonged immobilization. Copyright © 2013. Published by Elsevier B.V.

  3. Chronic obstructive pulmonary disease and metabolic syndrome%慢性阻塞性肺疾病和代谢综合征

    Institute of Scientific and Technical Information of China (English)

    刘瑢; 陈亚红

    2010-01-01

    慢性阻塞性肺疾病(chronic obstructive pulmonary disease,COPD)和代谢综合征(metabolic syndrome,MS)均是慢性疾病,患病人数多、社会负担重,二者在一定程度上相互影响,发病机制是亦有许多相似之处.吸烟、缺氧、氧化应激、不活动的生活方式、细胞因子、脂肪因子的改变以及糖皮质激素的应用可能是COPD患者中MS高发的原因,而高血糖对感染、免疫及肺功能的影响可能使COPD患者的预后恶化.本文对此方向的研究进展进行综述.%Chronic obstructive pulmonary disease (COPD)and metabolic syndrome(MS) both are chronic diseases with leading cause of morbidity and mortality worldwide and result social burden.They impact each other in some way and have some similarity in pathogenesis.The reasons including smoking,hypoxia,oxidative stress,a sedentary lifestyle,cytokines,adipocytokines,and corticosteroid therapy may explain that MS is frequent in patients with COPD.However,the influence of hyperglycemia to infection,immunity and lung function could deteriorate the prognosis from COPD.This review is to discuss the relationship between COPD and MS.

  4. Multiple pulmonary hamartomas: a case report

    Energy Technology Data Exchange (ETDEWEB)

    Ryu, Chang Woo; Kim, Jeung Sook; Lee, Hyeon Jae; Lee, Yong Hee [Pochon CHA Univ. College of Medicine, Sungnam (Korea, Republic of)

    1999-07-01

    Hamartomas are the most common benign neoplasms of the lung. However, multiple pulmonary hamartomas are very rare. We describe the radiographic findings of multiple pulmonary hamartomas without Carney's or pulmonary hamartoma syndrome with review the literature describing to this entity.

  5. Esquistossomose pulmonar. I. Forma crônica extensa sem hipertensão pulmonar e na ausência de hipertensão portal Pulmonary Schistosomiasis. I. Extensive chronic form without pulmonary hypertension and portal hypertension syndrome

    Directory of Open Access Journals (Sweden)

    Jayme Neves

    1980-12-01

    right; cardiac area was apparently normal. Microthoracotomy showed dominant pleural lesion enclosing the right superior lobe, affecting widely the parenchyma. Decortication of medium and right inferior lobes, partial reexpansion of their parenchyma, and pulmonary biopsy were performed. Histopathological study disclosed a predomiantly focal, but relatively extensive, and severe case of pulmonary arteritis without cor pulmonale. This pulmonary involvement occured isolated, not related to a hepato-splenic symptomatology, and consequently there was no syndrome of portal hypertension to indicate the preferentiai way of migration of the eggs, and eventually of the worms to the lungs.

  6. Morbidade respiratória nos pacientes com e sem síndrome pulmonar obstrutiva submetidos a cirurgia abdominal alta Respiratory morbidity in patients with and without pulmonary obstrutive syndrome after upper abdominal surgery

    Directory of Open Access Journals (Sweden)

    E.D.B. Pereira

    2000-03-01

    Full Text Available OBJETIVO: Estudar a morbidade respiratória nos pacientes com síndrome pulmonar obstrutiva submetidos a cirurgia abdominal alta. CASUÍSTICA E MÉTODO: Durante o pré-operatório, 196 pacientes candidatos à cirurgia abdominal alta eletiva responderam a um questionário padronizado e logo em seguida realizaram espirometria. Houve acompanhamento no pós-operatório até a alta hospitalar ou óbito. Os pacientes foram divididos em quatro grupos: 27 pacientes com DPOC (diagnóstico de bronquite crônica ou enfisema e VEF1/CVFBACKGROUND: We wanted to determine the postoperative pulmonary complicatiosn after upper abdominal surgery in patients with pulmonary obstrutive syndrome. METHODS: We have studied 196 patients prospectively analyzed in preoperative period with spirometry and followed for observation of PPC. The patients were divided in four groups: COPD - those with chronic bronchitis or emphysema and VEF1/CVF 70% (23 patients. NORMAL - patients without pulmonary disease and normal spirometry (102 patients. RESULTS: Postoperative pulmonary complication was recognized when the patient presented atelectasis with clinical or gasometric alterations; bronchospasm that needed bronchodilator therapy; respiratory failure; mechanical ventilation or orotracheal entubation more than 48 hours in postosurgery period; tracheobronchitis characterized by the presence of purulent sputum with normal x-ray; pneumonia. Patients with pulmonary obstruction had experienced higher rates of pulmonar complications (32% vs 6%,p<0,05. The presence of obstuctive lung disease was associated with an increased number of ventilator days, but was not associeted with longer intensive care unit or hospital stay. CONCLUSIONS: The incidence of postoperative pulmonary complications was strongly associated with the presence of pulmonary obstrutive syndrome.

  7. Central Sensitization Syndrome and the Initial Evaluation of a Patient with Fibromyalgia: A Review

    OpenAIRE

    Fleming, Kevin C.; Mary M. Volcheck

    2015-01-01

    In both primary care and consultative practices, patients presenting with fibromyalgia (FM) often have other medically unexplained somatic symptoms and are ultimately diagnosed as having central sensitization (CS). Central sensitization encompasses many disorders where the central nervous system amplifies sensory input across many organ systems and results in myriad symptoms. A pragmatic approach to evaluate FM and related symptoms, including a focused review of medical records, interviewing ...

  8. Antiphospholipid Syndrome - A Case Report of Pulmonary Thromboembolism, Followed with Acute Myocardial Infarction in Patient with Systemic Sclerosis

    Directory of Open Access Journals (Sweden)

    Marija Vavlukis

    2015-11-01

    CONCLUSION: The acquired antiphospholipid syndrome is common condition in patients with systemic autoimmune diseases, but relatively rare in patients with systemic sclerosis. Never the less, we have to be aware of it when treating the patients with systemic sclerosis.

  9. Frontal lobe dementia syndrome as a first manifestation of primary angiitis of the central nervous system (PACNS).

    Science.gov (United States)

    Bönstrup, Marlene; Ott, Katja; Glatzel, Markus; Magnus, Tim

    2016-02-01

    This case presents a clinical course of a frontal lobe dysexecutive syndrome with dementia caused by a primary angiitis of the central nervous system (PACNS) of exclusively very small vessels. An isolated frontal lobe dementia syndrome as a primary manifestation of PACNS highlights the diverse clinical manifestations of the disease. The patient presented with a progressive cognitive decline with loss of memory, disinhibited behavior, inappropriate affect and frontal release signs. The diagnostic workup essentially revealed a lymphocytic pleocytosis in the cerebrospinal fluid and a generalized cortical atrophy without any vascular abnormalities. To grasp a diagnosis for this enigmatic clinical picture of a frontal lobe syndrome with signs of inflammation we targeted a tissue-based diagnosis. A brain biopsy gave the decisive hint towards a microvasculitis. Although the histopathologic picture showed peculiarities, a destruction of the vascular bed of very small vessels by lymphocytic infiltration was evident. Our case illustrates an uncommon clinical picture of a PACNS and points to shortcomings of the current histopathologic criteria if only very small vessels are involved.

  10. Síndrome do incisivo central superior solitário: relato de caso Solitary median maxillary central incisor syndrome: case report

    Directory of Open Access Journals (Sweden)

    Eduardo Machado

    2010-08-01

    Full Text Available INTRODUÇÃO: a presença de um incisivo central superior solitário é um evento bastante incomum na população. A prevalência da chamada Síndrome do Incisivo Central Superior Solitário (SICSS é verificada em 1:50.000 nascimentos, sendo registrado um maior acometimento no sexo feminino. Essa alteração no desenvolvimento da oclusão dentária é caracterizada por más formações estruturais, sobretudo na região de linha média do paciente. O diagnóstico precoce e o tratamento adequado dessa síndrome são de grande importância, pois essa condição talvez seja um indicativo de que o paciente pode apresentar outras más formações congênitas severas, não devendo ser a SICSS considerada uma simples anomalia dentária. Os procedimentos ortodônticos, nesses casos, variam dependendo do grau de comprometimento das estruturas ósseas da maxila, da oclusão em si, e principalmente da sutura palatina mediana. OBJETIVO: discutir, baseado em evidências científicas, aspectos importantes relacionados à SICSS, bem como apresentar um caso clínico de paciente do sexo feminino com SICSS, que foi submetida a tratamento ortodôntico na Clínica Odontológica Integrada Infantil da Universidade Federal de Santa Maria / RS. CONCLUSÃO: pela análise crítica da literatura, verifica-se ser muito importante o diagnóstico correto e precoce acerca dessa síndrome, visto que há possibilidade da mesma estar associada a outros problemas de desenvolvimento. Além disso, o paciente acometido pela SICSS deve ser assistido por uma equipe multidisciplinar de saúde, de forma a otimizar os resultados clínicos e devolver-lhe qualidade de vida.INTRODUCTION: The presence of a single median maxillary central incisor is an uncommon event in the population. The prevalence of the Solitary Median Maxillary Central Incisor (SMMCI syndrome is about 1:50,000 live births, occurring more in women. This alteration in the development of the dental occlusion is

  11. Pulmonary embolus

    Science.gov (United States)

    ... Blood clot - lung; Embolus; Tumor embolus; Embolism - pulmonary; DVT-pulmonary embolism; Thrombosis - pulmonary embolism ... area). This type of clot is called a deep vein thrombosis (DVT) . The blood clot breaks off and travels ...

  12. Overweight, obesity and central obesity in women with polycystic ovary syndrome: a systematic review and meta-analysis.

    Science.gov (United States)

    Lim, S S; Davies, M J; Norman, R J; Moran, L J

    2012-01-01

    BACKGROUND Polycystic ovary syndrome (PCOS) is closely associated with obesity but the prevalence of obesity varies between published studies. The objective of this research was to describe the prevalence of overweight, obesity and central obesity in women with and without PCOS and to assess the confounding effect of ethnicity, geographic regions and the diagnostic criteria of PCOS on the prevalence. METHODS MEDLINE, EMBASE, CINAHL, Cochrane Central Register of Controlled Trials (CENTRAL) and PSYCINFO were searched for studies reporting the prevalence of overweight, obesity or central obesity in women with and without PCOS. Data were presented as prevalence (%) and risk ratio (RR) [95% confidence interval (CI)]. Random-effect models were used to calculate pooled RR. RESULTS This systematic review included 106 studies while the meta-analysis included 35 studies (15129 women). Women with PCOS had increased prevalence of overweight [RR (95% CI): 1.95 (1.52, 2.50)], obesity [2.77 (1.88, 4.10)] and central obesity [1.73 (1.31, 2.30)] compared with women without PCOS. The Caucasian women with PCOS had a greater increase in obesity prevalence than the Asian women with PCOS compared with women without PCOS [10.79 (5.36, 21.70) versus 2.31 (1.33, 4.00), P obesity and central obesity. Although our findings support a positive association between obesity and PCOS, our conclusions are limited by the significant heterogeneity between studies and further studies are now required to determine the source of this heterogeneity. Clinical management of PCOS should include the prevention and management of overweight and obesity.

  13. Eisenmengers syndrom

    DEFF Research Database (Denmark)

    Jensen, Annette Schophuus; Iversen, Kasper; Vejlstrup, Niels G;

    2009-01-01

    Congenital heart disease with left-to-right shunt can induce proliferation, vasoconstriction and thrombosis in the pulmonary vascular bed. Eventually, the patient may develop Eisenmenger syndrome defined as pulmonary arterial hypertension caused by high pulmonary vascular resistance with right......-to-left shunt and cyanosis. Patients with Eisenmenger syndrome suffer a high risk of complications in connection with acute medical conditions, extra-cardiac surgery and pregnancy. This article describes the precautions that should be taken to reduce morbidity and mortality in these patients. Udgivelsesdato...

  14. Sildenafil attenuates pulmonary arterial pressure but does not improve oxygenation during ARDS

    NARCIS (Netherlands)

    Cornet, A.D.; Hofstra, J.J.; Swart, E.L.; Girbes, A.R.J.; Juffermans, N.P.

    2010-01-01

    OBJECTIVE: Pulmonary hypertension is a characteristic feature of acute respiratory distress syndrome (ARDS) and contributes to mortality. Administration of sildenafil in ambulatory patients with pulmonary hypertension improves oxygenation and ameliorates pulmonary hypertension. Our aim was to determ

  15. Primary ciliary dyskinesia: Kartagener syndrome with central giant cell granuloma. A case report.

    Science.gov (United States)

    Türkoğlu, Kivanç; Orhan, Kaan; Demir, Pinar; Karabulut, Bariş; Can-Karabulut, Deniz C

    2010-10-01

    This paper describes a clinical case of both giant cell granuloma and Kartagener syndrome in a 15-year-old male patient, with emphasis on the radiographic aspects of this extremely unusual pathology. To our knowledge, the presence of these 2 rare clinical conditions in the same patient has not been previously reported.

  16. Screening with spirometry is a useful predictor of later development of noninfectious pulmonary syndromes in patients undergoing allogeneic stem cell transplantation.

    Science.gov (United States)

    Thompson, Philip A; Lim, Andrew; Panek-Hudson, Yvonne; Tacey, Mark; Hijazi, Ramzi; Ng, Ashley P; Szer, Jeff; Ritchie, David; Bajel, Ashish

    2014-06-01

    Noninfectious pulmonary syndromes (NIPS) frequently complicate allogeneic stem cell transplantation (allo-SCT). The most common and serious is the bronchiolitis obliterans syndrome, characterized by irreversible fixed airflow obstruction, impaired quality of life, and a high mortality. Treatment for established symptomatic disease is relatively ineffective. We therefore sought to identify potential predictive factors for development of NIPS, which may identify patients at risk in whom earlier intervention may be of benefit. Spirometry and diffusing capacity for carbon monoxide were performed before allo-SCT, day 100, and 1 year after allo-SCT. We retrospectively analyzed spirometry in consecutive patients having allo-SCT from 2004 to 2010, along with computed tomography and bronchoalveolar lavage results to identify cases of NIPS. Cases of bronchiolitis obliterans syndrome were defined as per current National Institutes of Health consensus guidelines. Spirometry results and baseline variables were compared between patients with and without NIPS to identify early predictors and risk factors for NIPS. Of 235 assessable patients, 23 (9.8%) developed NIPS. Median time of onset was day 367 (interquartile range [IQR], 144 to 544 days). Changes in forced expiratory volume in 1 second (ΔFEV1.0) was the best predictor of later NIPS development. Median ΔFEV1.0 from pretransplant to day 100 in patients later developing NIPS was -12% (IQR, -25% to -1%) versus -1% (IQR, -7% to +6%) in unaffected patients, P = .002. From pretransplant to 1 year, ΔFEV1.0 was -19% (IQR, -37% to -6%) versus -3% (IQR, -10% to +4%) in patients later developing NIPS and unaffected patients, respectively, P Spirometry is a potentially useful screening test for identification of presymptomatic NIPS. We recommend 3-monthly spirometry surveillance for up to 2 years post-transplant. Our findings require prospective validation to identify patients in whom earlier intervention may potentially modify the

  17. [Pulmonary Manifestations of Vasculitis].

    Science.gov (United States)

    von Vietinghoff, S

    2016-11-01

    The variable symptoms and signs of pulmonary vasculitis are a diagnostic and therapeutic challenge. Vasculitis should be considered in rapidly progressing, severe and unusual manifestations of pulmonary disease. Clinical examination of other organ systems typically affected by vasculitis such as skin and kidney and autoantibody measurements are complementary approaches to manage this situation. Pulmonary involvement is common in small vessel vasculitis including anti-GBM disease (Goodpasture syndrome) and the ANCA-associated vasculitides. Life threatening pulmonary hemorrhage and irreversible damage of other organs, frequently the kidney, are important complications necessitating rapid diagnosis of these conditions.Vasculitides are rare diseases of multiple organs and therapies including biologics are evolving rapidly, requiring cooperation of specialities and with specialized centres to achieve best patient care. All involved physicians should be aware of typical complications of immunosuppressive therapy. © Georg Thieme Verlag KG Stuttgart · New York.

  18. Abdominal Compartment Syndrome: pathophysiology and definitions

    OpenAIRE

    Cheatham Michael L

    2009-01-01

    Abstract "Intra-abdominal hypertension", the presence of elevated intra-abdominal pressure, and "abdominal compartment syndrome", the development of pressure-induced organ-dysfunction and failure, have been increasingly recognized over the past decade as causes of significant morbidity and mortality among critically ill surgical and medical patients. Elevated intra-abdominal pressure can cause significant impairment of cardiac, pulmonary, renal, gastrointestinal, hepatic, and central nervous ...

  19. Hypoxic pulmonary hypertension and novel ATP-sensitive potassium channel opener: the new hope on the horizon

    Institute of Scientific and Technical Information of China (English)

    Yu JIN; Wei-ping XIE; Hong WANG

    2012-01-01

    Hypoxic pulmonary hypertension (HPH) is a syndrome characterized by the increase of pulmonary vascular tone and the structural remodeling of peripheral pulmonary arteries.The aim of specific therapies for hypoxic pulmonary hypertension is to reduce pulmonary vascular resistance,reverse pulmonary vascular remodeling,and thereby improving right ventricular function.Iptakalim,a lipophilic para-amino compound with a low molecular weight,has been demonstrated to be a new selective ATP-sensitive potassium (KATP) channel opener via pharmacological,electrophysiological,biochemical studies,and receptor binding tests.In hypoxia-induced animal models,iptakalim decreases the elevated mean pressure in pulmonary arteries,and attenuates remodeling in the right ventricle,pulmonary arteries and airways.Furthermore,iptakalim has selective antihypertensive effects,selective vasorelaxation effects on smaller arteries,and protective effects on endothelial cells,but no effects on the central nervous,respiratory,digestive or endocrine systems at therapeutic dose.Our previous studies demonstrated that iptakalim inhibited the effects of endothelin-1,reduced the intracellular calcium concentration and inhibited the proliferation of pulmonary artery smooth muscle cells.Since iptakalim has been shown safe and effective in both experimental animal models and phase I clinical trials,it can be a potential candidate of HPH in the future.

  20. Síndrome do desconforto respiratório agudo pulmonar e extrapulmonar: existem diferenças? Pulmonary and extrapulmonary acute respiratory distress syndrome: are they different?

    Directory of Open Access Journals (Sweden)

    Cristiane S. N. Baez Garcia

    2008-06-01

    Full Text Available JUSTIFICATIVA E OBJETIVOS: A patogênese da síndrome do desconforto respiratório agudo (SDRA tem sido explicada pela presença de uma agressão direta (SDRA pulmonar e/ou indireta (SDRA extrapulmonar ao parênquima pulmonar. Evidências indicam que a fisiopatologia da doença pode diferir com o tipo de lesão. O objetivo deste estudo foi apresentar breve revisão das diferenças entre a SDRA pulmonar e a SDRA extrapulmonar e discutir as interações entre os aspectos morfofuncionais e a resposta aos diferentes tratamentos. CONTEÚDO: Esta revisão bibliográfica baseou-se em uma pesquisa sistemática de artigos experimentais e clínicos sobre SDRA incluídos nas bases de dados MedLine e SciElo nos últimos 20 anos. Muitos pesquisadores concordam, com base em estudos experimentais, que a SDRA pulmonar e a SDRA extrapulmonar não são idênticas no que diz respeito aos aspectos morfofuncionais, a resposta à pressão positiva ao final da expiração (PEEP, manobra de recrutamento alveolar, posição prona e outras terapias farmacológicas. Entretanto, os estudos clínicos têm descrito resultados contraditórios, os quais podem ser atribuídos à dificuldade de se classificar a SDRA em uma ou outra etiologia, e de se precisar o início, a fase e a gravidade da SDRA nos pacientes. CONCLUSÕES: Pacientes com SDRA de etiologias distintas perduram sendo considerados como pertencendo a uma mesma síndrome e, assim, são tratados da mesma forma. Logo, é fundamental entender as diferenças fisiopatológicas entre a SDRA pulmonar e extrapulmonar para que a terapia seja mais bem direcionada.BACKGROUND AND OBJECTIVES: The pathogenesis of acute respiratory distress syndrome (ARDS has been described by the presence of direct (pulmonary and/or indirect (extrapulmonary insult to the lung parenchyma. Evidence indicates that the pathophysiology of ARDS may differ according to the type of primary insult. This article presents a brief overview of differences

  1. Central obesity as a precursor to the metabolic syndrome in the AusDiab study and Mauritius.

    Science.gov (United States)

    Cameron, Adrian J; Boyko, Edward J; Sicree, Richard A; Zimmet, Paul Z; Söderberg, Stefan; Alberti, K George M M; Tuomilehto, Jaakko; Chitson, Pierrot; Shaw, Jonathan E

    2008-12-01

    Evidence from epidemiologic studies that central obesity precedes future metabolic change and does not occur concurrently with the appearance of the blood pressure, glucose, and lipid abnormalities that characterize the metabolic syndrome (MetS) has been lacking. Longitudinal surveys were conducted in Mauritius in 1987, 1992, and 1998, and in Australia in 2000 and 2005 (AusDiab). This analysis included men and women (aged > or = 25 years) in three cohorts: AusDiab 2000-2005 (n = 5,039), Mauritius 1987-1992 (n = 2,849), and Mauritius 1987-1998 (n = 1,999). MetS components included waist circumference, systolic blood pressure, fasting and 2-h postload plasma glucose, high-density lipoprotein (HDL) cholesterol, triglycerides, and homeostasis model assessment of insulin sensitivity (HOMA-S) (representing insulin sensitivity). Linear regression was used to determine which baseline components predicted deterioration in other MetS components over 5 years in AusDiab and 5 and 11 years in Mauritius, adjusted for age, sex, and ethnic group. Baseline waist circumference predicted deterioration (P Mauritius 1987-1992, and four of six in Mauritius 1987-1998. In contrast, an increase in waist circumference between baseline and follow-up was only predicted by insulin sensitivity (HOMA-S) at baseline, and only in one of the three cohorts. These results suggest that central obesity plays a central role in the development of the MetS and appears to precede the appearance of the other MetS components.

  2. Glucose-regulated protein 78 may play a crucial role in promoting the pulmonary microvascular remodeling in a rat model of hepatopulmonary syndrome

    Science.gov (United States)

    Zhang, Huiying; Lv, Minli; Zhao, Zhongfu; Jia, Jiantao; Zhang, Lili; Xiao, Peng; Wang, Limin; Li, Chen; Ji, Jingquan; Tian, Xiaoxia; Li, Xujiong; Fan, Yimin; Lai, Lina; Liu, Yan; Li, Baohong; Zhang, Cuiying; Liu, Mingshe; Guo, Jianhong; Han, Dewu; Ji, Cheng

    2015-01-01

    Objective This study is to investigate the role of glucose-regulated protein 78 (GRP78) in the pulmonary microvascular remodeling during hepatopulmonary syndrome (HPS) development. Methods The rat models with liver cirrhosis and HPS were induced by multiple pathogenic factors for 4 to 8 wk. The concentrations of alanine transferase (ALT) and endotoxin in plasma were detected in the models, followed by the detection of GRP78 expression. RT-PCR, quantitative real-time PCR and Western blotting were employed to assess the mRNA and protein expression levels of vascular endothelial growth factor (VEGF), respectively. Immunohistochemistry staining was used to examine the expression of a specific vascular marker, factor VIII-related antigen (FVIII-RAg), and several cell proliferation- and apoptosis-related proteins, including CHOP/GADD153, caspase-12, Bcl-2 and nuclear factor (NF)-κB. Results The levels of endotoxin and ALT in plasma were gradually increased as the disease progressed, so did GRP78, which were in a positive correlation. The expression levels of VEGF (both mRNA and protein) and FVIII-RAg were significantly elevated in the HPS models, indicating active angiogenesis, which was also positively correlated with GRP78 expression. Furthermore, the expression levels of the pro-apoptotic proteins of CHOP/GADD153 and caspase-12 were dramatically decreased, while the anti-apoptotic proteins of Bcl-2 and NF-κB were significantly elevated, in the HPS models. There were also close correlations between these proteins and GRP78. Conclusions Over-expression of GRP78 in lungs may be the critical pathogenic factor for HPS. Through promoting cell proliferation and survival and inhibiting apoptosis, GRP78 may promote the pulmonary microvascular remodeling in HPS pathogenesis. Our results provide a potential therapeutic target for clinical prevention and treatment for HPS and related complications. PMID:24768185

  3. Imbalance between pulmonary angiotensin-converting enzyme and angiotensin-converting enzyme 2 activity in acute respiratory distress syndrome

    NARCIS (Netherlands)

    Wosten-van Asperen, Roelie M.; Bos, Albert; Bem, Reinout A.; Dierdorp, Barbara S.; Dekker, Tamara; van Goor, Harry; Kamilic, Jelena; van der Loos, Chris M.; van den Berg, Elske; Bruijn, Martijn; van Woensel, Job B.; Lutter, Rene

    2013-01-01

    Objective: Angiotensin-converting enzyme and its effector peptide angiotensin II have been implicated in the pathogenesis of acute respiratory distress syndrome. Recently, angiotensin-converting enzyme 2 was identified as the counter-regulatory enzyme of angiotensin-converting enzyme that converts

  4. Imbalance between pulmonary angiotensin-converting enzyme and angiotensin-converting enzyme 2 activity in acute respiratory distress syndrome

    NARCIS (Netherlands)

    Wosten-van Asperen, Roelie M.; Bos, Albert; Bem, Reinout A.; Dierdorp, Barbara S.; Dekker, Tamara; van Goor, Harry; Kamilic, Jelena; van der Loos, Chris M.; van den Berg, Elske; Bruijn, Martijn; van Woensel, Job B.; Lutter, Rene

    2013-01-01

    Objective: Angiotensin-converting enzyme and its effector peptide angiotensin II have been implicated in the pathogenesis of acute respiratory distress syndrome. Recently, angiotensin-converting enzyme 2 was identified as the counter-regulatory enzyme of angiotensin-converting enzyme that converts a

  5. A PROSPECTIVE, OBSERVATIONAL STUDY OF ADVERSE REACTIONS TO DRUG REGIME FOR MULTI-DRUG RESISTANT PULMONARY TUBERCULOSIS IN CENTRAL INDIA.

    Directory of Open Access Journals (Sweden)

    Dr. Rohan C. Hire

    2014-09-01

    Full Text Available Abstract Objective: 1 To assess the adverse drug reactions of second line anti-tubercular drugs used to treat Multi-drug resistant Tuberculosis (MDR TB in central India on the basis of causality, severity and avoidability scales. 2 To study the relationship of type of MDR TB (primary or secondary and presence of diabetes mellitus (DM with mean smear conversion time. Material and Methods: A prospective, observational study was carried out on diagnosed multidrug resistant tuberculosis patients enrolled for DOTS‑Plus regimen at TB and Chest Disease Department from January to December 2012. They were followed for 9 months thereafter and encountered adverse drug reactions (ADRs were noted along with the time of sputum conversion. The data were analysed by Chi-square or Fisher’s exact test and unpaired student’s‘t’ test. Results: Total 64 ADRs were reported in 55 patients out of total 110 patients (n = 110. As per the Naranjo causality assessment of ADRs, 7 patients had “definite” causal relation, 45 had “probable” causal relation and 3 had “possible” causal relation with drugs of DOTS Plus regime. As per the Hartwig’s severity assessment scale, there were total 7 ADRs in Level 1, 6 in Level 2, 33 in Level 3 and 9 in Level 4. Hallas avoidability assessment scale divided the ADRs as 3 being “Definitely avoidable”, 26 “Possibly avoidable”, 23 “Not avoidable” and 3 “unevaluable”. . Mean sputum smear conversion time is significantly higher in patients with secondary type than that of primary type of MDR TB (p = 0.0001 and in patients with DM than those without DM (p <0.0001. Conclusion: ADRs were common in patients of MDR TB on DOTs-Plus drug regime. It was due to lack of availability of safer and equally potent drugs in DOTs-Plus drug regime compared to DOTS regime in non-resistant TB. The frequency and severity of ADRs can be reduced by strict vigilance about known and unknown ADRs, monitoring their laboratory and

  6. Central sensitization syndrome and the initial evaluation of a patient with fibromyalgia: a review.

    Science.gov (United States)

    Fleming, Kevin C; Volcheck, Mary M

    2015-04-01

    In both primary care and consultative practices, patients presenting with fibromyalgia (FM) often have other medically unexplained somatic symptoms and are ultimately diagnosed as having central sensitization (CS). Central sensitization encompasses many disorders where the central nervous system amplifies sensory input across many organ systems and results in myriad symptoms. A pragmatic approach to evaluate FM and related symptoms, including a focused review of medical records, interviewing techniques, and observations, is offered here, giving valuable tools for identifying and addressing the most relevant symptoms. At the time of the clinical evaluation, early consideration of CS may improve the efficiency of the visit, reduce excessive testing, and help in discerning between typical and atypical cases so as to avoid an inaccurate diagnosis. Discussion of pain and neurophysiology and sensitization often proves helpful.

  7. Central Sensitization Syndrome and the Initial Evaluation of a Patient with Fibromyalgia: A Review

    Directory of Open Access Journals (Sweden)

    Kevin C. Fleming

    2015-04-01

    Full Text Available In both primary care and consultative practices, patients presenting with fibromyalgia (FM often have other medically unexplained somatic symptoms and are ultimately diagnosed as having central sensitization (CS. Central sensitization encompasses many disorders where the central nervous system amplifies sensory input across many organ systems and results in myriad symptoms. A pragmatic approach to evaluate FM and related symptoms, including a focused review of medical records, interviewing techniques, and observations, is offered here, giving valuable tools for identifying and addressing the most relevant symptoms. At the time of the clinical evaluation, early consideration of CS may improve the efficiency of the visit, reduce excessive testing, and help in discerning between typical and atypical cases so as to avoid an inaccurate diagnosis. Discussion of pain and neurophysiology and sensitization often proves helpful.

  8. Combined central retinal artery and vein occlusion in Churg-Strauss syndrome

    DEFF Research Database (Denmark)

    Hamann, Steffen; Johansen, Sven; Hamann, Steffen Ellitsgaard

    2006-01-01

    describe the clinical features and evolution of the case after treatment. RESULTS: A combined occlusion of the central retinal artery and central retinal vein was diagnosed by the funduscopic appearance of retinal whitening, macular cherry-red spot, papilloedema, retinal haemorrhages in all four quadrants...... a very poor prognosis for vision, due to the resulting retinal ischaemia, and a poor general prognosis due to the late stage of the systemic disease. Corticosteroids should be instigated promptly in order to prevent further systemic or ocular vasculitis....

  9. Atypical presentation of CLIPPERS syndrome: a new entity in the differential diagnosis of central nervous system rheumatologic diseases.

    Science.gov (United States)

    Gul, Maryam; Chaudhry, Ammar A; Chaudhry, Abbas A; Sheikh, Mubashir A; Carsons, Steven

    2015-04-01

    Numerous autoimmune diseases can affect the central nervous system (CNS), and variable clinical presentations confound the differential diagnosis. The challenging task of properly characterizing various CNS autoimmune diseases enables patients to be rapidly triaged and appropriately treated. In this review article, we aim to explore different CNS manifestations of rheumatologic diseases with emphasis on the utility of imaging and cerebrospinal fluid findings. We review the classic physical examination findings, characteristic imaging features, cerebrospinal fluid results, and serum biomarkers. In addition, we also present a unique case of newly described autoimmune entity CLIPPERS syndrome. Our case is unique in that this is the first case which demonstrates involvement of the supratentorial perivascular spaces in addition to the classic infratentorial involvement as initially described by Pittock et al (Brain. 2010;133:2626-2634).

  10. Preschool weight and body mass index in relation to central obesity and metabolic syndrome in adulthood

    DEFF Research Database (Denmark)

    Graversen, Lise; Sørensen, Thorkild; Petersen, Liselotte

    2014-01-01

    Artiklen ser på vægt og BMI i alderen 0-5 år, og de to faktorers betydning for overvægt og metabolisk syndrom i voksenalderen. Studiet viser, at der er en lineær sammenhæng mellem vægt (0-1 år) og BMI (2-5 år) og senere BMI og taljeomfang. Studiet viser også, at børn, der ligger blandt de øverste 5...... % (sammenlignet med børn blandt de nederste 50 % i vægt og BMI) har en øget risiko, der stiger op gennem førskolealderen. Således har børn, der ligger blandt de øverste 5 % i BMI i 5-årsalderen en 6,2 gange større risiko for svær overvægt og en 2,5 gange større risiko for metabolisk syndrom som voksne. Det...... betyder, at en tredjedel af disse børn var svært overvægtige, og at en fjerdedel havde metabolisk syndrom som voksne. Undersøgelsen bygger på data for 2120 børn fra den populationsbaserede Northern Finland Birth Cohort (født i 1966), som er fulgt tæt med højde- og vægtmålinger i barnealderen og har...

  11. Association of CHRDL1 mutations and variants with X-linked megalocornea, Neuhauser syndrome and central corneal thickness.

    Directory of Open Access Journals (Sweden)

    Alice E Davidson

    Full Text Available We describe novel CHRDL1 mutations in ten families with X-linked megalocornea (MGC1. Our mutation-positive cohort enabled us to establish ultrasonography as a reliable clinical diagnostic tool to distinguish between MGC1 and primary congenital glaucoma (PCG. Megalocornea is also a feature of Neuhäuser or megalocornea-mental retardation (MMR syndrome, a rare condition of unknown etiology. In a male patient diagnosed with MMR, we performed targeted and whole exome sequencing (WES and identified a novel missense mutation in CHRDL1 that accounts for his MGC1 phenotype but not his non-ocular features. This finding suggests that MMR syndrome, in some cases, may be di- or multigenic. MGC1 patients have reduced central corneal thickness (CCT; however no X-linked loci have been associated with CCT, possibly because the majority of genome-wide association studies (GWAS overlook the X-chromosome. We therefore explored whether variants on the X-chromosome are associated with CCT. We found rs149956316, in intron 6 of CHRDL1, to be the most significantly associated single nucleotide polymorphism (SNP (p = 6.81×10(-6 on the X-chromosome. However, this association was not replicated in a smaller subset of whole genome sequenced samples. This study highlights the importance of including X-chromosome SNP data in GWAS to identify potential loci associated with quantitative traits or disease risk.

  12. Nevirapine-induced Stevens-Johnson syndrome from a tertiary care hospital in Central India

    Directory of Open Access Journals (Sweden)

    Vandana Badar

    2014-04-01

    Full Text Available A 42 year old man, a known case of AIDS receiving antiretroviral therapy – stavudine, lamivudine, and nevirapine since 4 weeks, was admitted with erythematous rash, with blisters, and abnormal liver function test. A diagnosis of Steven-Johnson syndrome was made excluding other opportunistic infection and differential diagnosis. Nevirapine was discontinued until the patient recovered. After recovery patient was started with stavudine, lamivudine, and efavirenz treatment without re-challenge with nevirapine. [Int J Basic Clin Pharmacol 2014; 3(2.000: 408-409

  13. Metabolic syndrome in the rural population of Wardha, Central India: An exploratory factor analysis

    Directory of Open Access Journals (Sweden)

    Pradeep R Deshmukh

    2013-01-01

    Full Text Available Background and Objectives: Metabolic syndrome - a plausible precondition for type II diabetes and cardiovascular diseases is also on rise. To understand the mechanistic complexity of metabolic syndrome it is imperative to study the specific contribution of the determinants of metabolic syndrome. Such study can help to identify the most significant factor which may be of use in early detection as well as prevention efforts. Such information is scarcely available from India and especially from rural India. Hence, the present study was undertaken to explore for such factor which might be considered crucial for development of such pathogenesis particularly in rural population of Wardha. Methods: A cross-sectional study comprising of 300 subjects was carried out in rural area of Primary Health Center, attached to medical college with approximate 31,000 populations. The anthropometric parameters such as height, weight, waist circumference were measured. Overnight fasting samples were collected for lipid profile (total cholesterol, triglyceride, high density lipoproteins, low density lipoproteins, very low density lipoproteins and fasting blood glucose levels. The National Cholesterol Education Programme Adult Treatment Panel, ATP-III guidelines were used to categorize the study subjects. As many of the variables are highly intercorrelated, exploratory factor analysis was carried out to reduce the data to a smaller number of independent factors that accounts for the most of the variances in the data. Principal component analysis was used as a method of extraction. Results: For both sexes, three factors were extracted accounting for about 71% variance in the measured variables. An adiposity factor which accounted for highest explained variance (28%, was the initial factor extracted. It was loaded positively by waist circumference, triglyceride, and very low density lipoprotein and negatively loaded by high density lipoprotein. Second factor extracted

  14. Hypocretin Deficiency Associated with Narcolepsy Type 1 and Central Hypoventilation Syndrome in Neurosarcoidosis of the Hypothalamus.

    Science.gov (United States)

    Mayo, Mary Catherine; Deng, Jane C; Albores, Jeffrey; Zeidler, Michelle; Harper, Ronald M; Avidan, Alon Y

    2015-09-15

    We report a case of a 53-year-old man presenting with depressed alertness and severe excessive sleepiness in the setting of neurosarcoidosis. Neuroimaging demonstrated hypothalamic destruction due to sarcoidosis with a CSF hypocretin level of 0 pg/mL. The patient also experienced respiratory depression that presumably resulted from hypocretin-mediated hypothalamic dysfunction as a result of extensive diencephalic injury. This is a novel case, demonstrating both hypocretin deficiency syndrome, as well as respiratory dysfunction from destruction of hypocretin neurons and extensive destruction of key diencephalic structures secondary to the underlying neurosarcoidosis.

  15. Refeeding syndrome.

    Science.gov (United States)

    Fernández López, M T; López Otero, M J; Alvarez Vázquez, P; Arias Delgado, J; Varela Correa, J J

    2009-01-01

    Refeeding syndrome is a complex syndrome that occurs as a result of reintroducing nutrition (oral, enteral or parenteral) to patients who are starved or malnourished. Patients can develop fluid-balance abnormalities, electrolyte disorders (hypophosphataemia, hypokalaemia and hypomagnesaemia), abnormal glucose metabolism and certain vitamin deficiencies. Refeeding syndrome encompasses abnormalities affecting multiple organ systems, including neurological, pulmonary, cardiac, neuromuscular and haematological functions. Pathogenic mechanisms involved in the refeeding syndrome and clinical manifestations have been reviewed. We provide suggestions for the prevention and treatment of refeeding syndrome. The most important steps are to identify patients at risk, reintroduce nutrition cautiously and correct electrolyte and vitamin deficiencies properly.

  16. 急性脑损伤继发神经源性肺水肿%Neurogenic pulmonary edema secondary to acute central nervous system injury

    Institute of Scientific and Technical Information of China (English)

    梁锦泉; 冯子泽

    2013-01-01

    Objective To explore the pathogenesis and summarize the clinical characteristic and treatment experience of neurogenic pulmonary edema (NPE) secondary to acute central nervous system injury. Methods The data of 18 cases of NPE were retrospectively analyzed by reviewing medical literature. Results The age ranged from 19 to 69. The patients became acutely dyspneic, tachypneic and hypoxic following neurologic injury. Pink, frothy sputum was seen and bilateral crackles and rales were appreciated on auscultation. Chest X-ray or CT revealed diffuse bilateral pulmonary infiltrates. Eventually, they were cured by mechanical ventilation and other combined treatment. According to glasgow prognosis score, 13 cases of them got good recovery, 3 cases got moderate disability, 2 cases got severe disability. Conclusion NPE is characterized by the development of respiratory failure soon after neurologic injury. The primary therapy is to control the development of the damage to central nervous system, break the vicious circle, and apply mechanical ventilation, vascular active drugs and sedatives. After weaning, sputum suction via fiberoptic bronchoscopy shall be administered until the symptoms are relieved.%目的探讨脑损伤继发神经源性肺水肿的发病机制,总结其临床特点和救治经验。方法整理近年来18例急性脑损伤继发神经源性肺水肿病例的临床资料,并结合文献分析。结果患者19~69岁,均在急性脑损伤后出现呼吸困难,咳粉红色泡沫痰,血氧饱和度下降,双肺满布湿啰音,胸部CT/DR提示双肺弥漫性渗出性病变,最终经机械通气等综合处理后治愈。根据格拉斯哥预后评分(glasgow prognosis score,GOS),恢复良好(5分)13例,轻残(4分)3例,重残(3分)2例。结论急性脑损伤继发神经源性肺水肿的临床特点是均有急性脑损害的基础,短期内出现呼吸衰竭,基本救治方法是控制脑损伤的进展,打断

  17. [Visceral syndrome differentiation in traditional Chinese medicine and the changes of nutrition status or blood gas analysis in patients with chronic obstructive pulmonary disease].

    Science.gov (United States)

    Mao, Bing; Li, Wen; Liang, Wen-qiong

    2007-09-01

    To investigate the relationship between visceral syndrome differentiation in traditional Chinese medicine (TCM) and the changes of nutrition status or blood gas analysis in patients with chronic obstructive pulmonary disease (COPD). Sixty patients with COPD in stable phase were divided into dysfunction of lung (DL, n=20) group, dysfunction of lung and spleen (DLS, n=16) group and dysfunction of lung, spleen and kidney (DLSK, n=24) group according to syndrome differentiation of TCM. Moreover, the basic electric rhythm, percentage of bradygastria in electrogastrogram (EGG), ratio of actual body weight (ABW) to ideal body weight (IBW) and serum albumin level were determined to evaluate the nutrition status of the patients with COPD. Blood gas analysis was made too. The basic electric rhythm values in the DL, DLS and DLSK groups were (56.20+/-13.24)%, (47.38+/-10.24)% and (41.35+/-10.01)%, respectively. Compared with the DL group, the basic electric rhythm values in the DLS and DLSK groups decreased obviously (P0.05). The incidence rates of ABW/IBW0.05). The incidence rates of serum albumin less than 35 g/L were 20%, 37.5% and 54.17%, respectively, and there were no differences among the three groups (P>0.05). PO2 in the DLS group [(63.56+/-15.06) mmHg] and DLSK group [(63.17+/-19.05) mmHg] were decreased as compared with the DL group [(78.15+/-16.63) mmHg] (P0.05). Several dysfunction organs described in TCM were involved in COPD followed by the increased severity, hypoxemia and PCO2. Dysfunction of gastrointestinal tract and malnourishment took place accordingly.

  18. A novel technique to predict pulmonary capillary wedge pressure utilizing central venous pressure and tissue Doppler tricuspid/mitral annular velocities.

    Science.gov (United States)

    Uemura, Kazunori; Inagaki, Masashi; Zheng, Can; Li, Meihua; Kawada, Toru; Sugimachi, Masaru

    2015-07-01

    Assessing left ventricular (LV) filling pressure (pulmonary capillary wedge pressure, PCWP) is an important aspect in the care of patients with heart failure (HF). Physicians rely on right ventricular (RV) filling pressures such as central venous pressure (CVP) to predict PCWP, assuming concordance between CVP and PCWP. However, the use of this method is limited because discordance between CVP and PCWP is observed. We hypothesized that PCWP can be reliably predicted by CVP corrected by the relationship between RV and LV function, provided by the ratio of tissue Doppler peak systolic velocity of tricuspid annulus (S(T)) to that of mitral annulus (S(M)) (corrected CVP:CVP·S(T)/S(M)). In 16 anesthetized closed-chest dogs, S T and S M were measured by transthoracic tissue Doppler echocardiography. PCWP was varied over a wide range (1.8-40.0 mmHg) under normal condition and various types of acute and chronic HF. A significantly stronger linear correlation was observed between CVP·S(T)/S(M) and PCWP (R2 = 0.78) than between CVP and PCWP (R2 = 0.22) (P 10.5 mmHg predicted PCWP >18 mmHg with 85% sensitivity and 88% specificity. Area under ROC curve for CVP·S T/S M to predict PCWP >18 mmHg was 0.93, which was significantly larger than that for CVP (0.66) (P < 0.01). Peripheral venous pressure (PVP) corrected by S T/S M (PVP·S(T)/S(M) also predicted PCWP reasonably well, suggesting that PVP·S(T)/S (M) may be a minimally invasive alternative to CVP·S(T)/S(M) In conclusion, our technique is potentially useful for the reliable prediction of PCWP in HF patients.

  19. Meckel syndrome

    National Research Council Canada - National Science Library

    Salonen, R; Paavola, P

    1998-01-01

    Meckel syndrome (MKS) is a lethal syndrome with a central nervous system malformation, usually occipital meningoencephalocele, bilaterally large multicystic kidneys with fibrotic changes of the liver, and polydactyly in most cases...

  20. Neurogenic pulmonary edema following Cryptococcal meningoencephalitis associated with HIV infection.

    Science.gov (United States)

    Kondo, Reiichiro; Sugita, Yasuo; Arakawa, Kenji; Nakashima, Shinji; Umeno, Yumi; Todoroki, Keita; Yoshida, Tomoko; Takase, Yorihiko; Kage, Masayoshi; Oshima, Koichi; Yano, Hirohisa

    2015-08-01

    Neurogenic pulmonary edema (NPE) is a clinical syndrome characterized by the acute onset of pulmonary edema following a significant central nervous system insult. Only a few cases of NPE after Cryptococcal meningitis have been reported. We report a case of NPE following Cryptococcal meningoencephalitis. A 40-year-old man with no medical history was hospitalized for disturbance of consciousness. Blood glucose level was 124 mg/dL. Non-contrast head computed tomography showed no abnormalities. Lumbar puncture revealed a pressure of over 300 mm H2 O and cerebrospinal fluid (CSF) confirmed a white blood cell count of 65/mm(3) . The CSF glucose level was 0 mg/dL. The patient was empirically started on treatment for presumptive bacterial and viral meningitis. Four days after, the patient died in a sudden severe pulmonary edema. Autopsy was performed. We found at autopsy a brain edema with small hemorrhage of the right basal ganglia, severe pulmonary edema and mild cardiomegaly. Histologically, dilated Virchow-Robin spaces, crowded with Cryptococci were observed. In the right basal ganglia, Virchow-Robin spaces were destroyed with hemorrhage and Cryptococci spread to parenchyma of the brain. No inflammatory reaction of the lung was seen. Finally, acute pulmonary edema in this case was diagnosed as NPE following Cryptococcal meningoencephalitis. After autopsy, we found that he was positive for serum antibodies to human immunodeficiency virus. © 2015 Japanese Society of Neuropathology.

  1. Combined measurement of fetal lung volume and pulmonary artery resistance index is more accurate for prediction of neonatal respiratory distress syndrome in preterm fetuses: A Pilot Study.

    Science.gov (United States)

    Laban, Mohamed; Mansour, Ghada; El-Kotb, Ahmed; Hassanin, Alaa; Laban, Zina; Saleh, Abdelrahman

    2017-10-02

    To estimate optimal cut-off values for mean fetal lung volume (FLV) and pulmonary artery resistance index (PA-RI) as noninvasive measures to predict neonatal respiratory distress syndrome (RDS) in preterm fetuses. A prospective study conducted at Ain Shams University Maternity Hospital, Egypt from May 2015 to July 2017: eighty eligible women diagnosed with preterm labor were recruited at 32-36 weeks' gestation. Before delivery, three-dimensional ultrasound was used to estimate FLV using virtual organ computer-aided analysis (VOCAL), while PA-RI was measured by Doppler ultrasonography. A total of 80 women were examined. 37 (46%) of the newborns developed neonatal RDS. FLV was significantly lower in neonates who developed RDS (p = 0.04), whereas PARI was significantly higher in those who didn't (p = 0.02). Cut-off values of FLV ≤ 27.2 cm(3) and PARI ≥ 0.77 predicted the subsequent development of RDS. Combining both cut-offs generated a more sensitive and specific methodical approach for the prediction of RDS (sensitivity 100%, specificity 88.5%). Measurement of FLV or PA-RI can predict RDS in preterm fetuses. Combined use of both measures bolstered their predictive significance.

  2. Correlation of pulmonary surfactant protein B, C and neonatal respiratory distress syndrome%肺表面蛋白B、C与新生儿呼吸窘迫综合征相关性研究进展

    Institute of Scientific and Technical Information of China (English)

    新春

    2015-01-01

    新生儿呼吸窘迫综合征(neonatal respiratory distress syndrome,NRDS)是新生儿较常见的危重症之一,其发病与肺表面蛋白(surfactant protein,SP)基因变异有密切相关.SP是肺表面活性物质(pul-monary surfactant,PS)的重要组成部分,在肺表面活性物质的结构、代谢、功能方面起重要作用.SP-B和SP-C在PS中极为重要,有密切关联.研究其等位基因变异与NRDS的关系有助于早期基因干预,促进临床诊断和治疗,对降低新生儿病死率具有长远的意义.%Neonatal respiratory distress syndrome is one of the common critically ill newborn's disease, the pathogenesis of which is closely related to gene mutation of the pulmonary suffactant protein(SP).Surfactant protein is an important component of the pulmonary surfactant, which plays an important role in the structure, metabolism, and function of pulmonary surfactant.SP-B and SP-C in pulmonary surfactant is extremely important and closely related to each other.Study on relationship between the SP gene allelic variation and neonatal respiratory distress syndrome could contribute to early genetic intervention, promote the clinical diagnosis and treatment, and bring far-reaching significance to reduce the neonatal mortality rate.

  3. Uric acid in metabolic syndrome: From an innocent bystander to a central player.

    Science.gov (United States)

    Kanbay, Mehmet; Jensen, Thomas; Solak, Yalcin; Le, Myphuong; Roncal-Jimenez, Carlos; Rivard, Chris; Lanaspa, Miguel A; Nakagawa, Takahiko; Johnson, Richard J

    2016-04-01

    Uric acid, once viewed as an inert metabolic end-product of purine metabolism, has been recently incriminated in a number of chronic disease states, including hypertension, metabolic syndrome, diabetes, non-alcoholic fatty liver disease, and chronic kidney disease. Several experimental and clinical studies support a role for uric acid as a contributory causal factor in these conditions. Here we discuss some of the major mechanisms linking uric acid to metabolic and cardiovascular diseases. At this time the key to understanding the importance of uric acid in these diseases will be the conduct of large clinical trials in which the effect of lowering uric acid on hard clinical outcomes is assessed. Elevated uric acid may turn out to be one of the more important remediable risk factors for metabolic and cardiovascular diseases. Copyright © 2015 European Federation of Internal Medicine. All rights reserved.

  4. Pathology of tissue loss (white syndrome) in Acropora sp. corals from the Central Pacific

    Science.gov (United States)

    Work, Thierry M.; Aeby, Greta S.

    2011-01-01

    We performed histological examination of 69 samples of Acropora sp. manifesting different types of tissue loss (Acropora White Syndrome-AWS) from Hawaii, Johnston Atoll and American Samoa between 2002 and 2006. Gross lesions of tissue loss were observed and classified as diffuse acute, diffuse subacute, and focal to multifocal acute to subacute. Corals with acute tissue loss manifested microscopic evidence of necrosis sometimes associated with ciliates, helminths, fungi, algae, sponges, or cyanobacteria whereas those with subacute tissue loss manifested mainly wound repair. Gross lesions of AWS have multiple different changes at the microscopic level some of which involve various microorganisms and metazoa. Elucidating this disease will require, among other things, monitoring lesions over time to determine the pathogenesis of AWS and the potential role of tissue-associated microorganisms in the genesis of tissue loss. Attempts to experimentally induce AWS should include microscopic examination of tissues to ensure that potentially causative microorganisms associated with gross lesion are not overlooked.

  5. Imaging findings of disseminated pulmonary tuberculosis in patients with acquired immunodeficiency syndrome%艾滋病并发肺结核播散的影像表现

    Institute of Scientific and Technical Information of China (English)

    宋文艳; 赵祖琦; 赵大伟; 刘晋新; 官宛华; 梁艺; 贾翠宇; 张瑞池

    2013-01-01

    Objective To study the imaging findings of disseminated pulmonary tuberculosis in patients with acquired immunodeficiency syndrome (AIDS).Methods X-ray and multi-slice CT (MSCT)data from 33 AIDS patients with disseminated pulnonary tuberculosis confirmed by clinical manifestations and laboratory tests were analyzed retrospectively.Results Thirty patients underwent initial chest radiography examination,29 patients showed abnormal appearances,including bilateral disseminations in 21 patients and unilateral multiple disseminations in 8 patients.All patients underwent MSCT examination,26 patients showed bilateral disseminations and 7 patients showed unilateral multiple disseminations.The abnormal pulmonary appearances included nodule (n =25),miliary nodule (n =22),air-space consolidation (n =22),cavity (n =11),fibrosis (n =7),ground-glass opacity (n =7),pneumatocele (n =4),calcification (n =2).There were 20 patients with more than 3 abnormal appearances and 13 patients with one or two abnormal appearances.The extra-pulmonary tuberculosis included pleural effusion (n =33),lymphadenopathy (n =30),intestinal tuberculosis (n =3),splenic tuberculosis (n =1) and cerebral tuberculosis (n =1).Conclusion Disseminated pulmonary tuberculosis should be highly suspected in AIDS patients with diffused nodules,miliary nodules,air-space consolidations or multiple cavities,accompanied with pleural effusion and lymphadenopathy.%目的 探讨艾滋病(AIDS)并发肺结核肺内播散的影像特点.方法 回顾性分析经临床及实验室检查证实的33例AIDS并发肺结核肺内播散的X线胸片及MSCT影像资料,总结其影像表现特征.结果 首诊X线胸片检查30例,29例表现异常,包括病灶呈双侧播散性分布21例,单侧多发病变8例.所有患者均行MSCT扫描,病变呈单侧和双侧肺内多发分布分别为7例和26例;病变形态包括结节(25例)、粟粒(22例)、气腔实变(22例)、空洞(11例)、纤维索条(7例)、磨玻璃影(7

  6. Mass Spectrometry-based Proteomics in Acute Respiratory Distress Syndrome: A Powerful Modality for Pulmonary Precision Medicine

    Science.gov (United States)

    Xu, Xue-Feng; Dai, Hua-Ping; Li, Yan-Ming; Xiao, Fei; Wang, Chen

    2016-01-01

    Objective: Acute respiratory distress syndrome (ARDS) is an acute and lethal clinical syndrome that is characterized by hypoxemic respiratory failure and diffuse alveolar inflammatory damage. This review aimed to search and discuss the mass spectrometry (MS)-based proteomic studies on different subsets of ARDS patients. Data Sources: Original research articles were collected from the PubMed database published in English up to December 2015. Study Selection: The literature search was done using the term “(acute lung injury OR acute respiratory distress syndrome) AND (proteomics OR proteome OR mass spectrum OR differential in-gel electrophoresis OR two-dimensional polyacrylamide gel electrophoresis)”. Related original research articles were included and were carefully analyzed. Results: Eight original proteomic researches on ARDS patients were found. The common proteomic modalities were two-dimensional (2D) high-performance liquid chromatography-based electronic spray ion-MS/MS and 2D-polyacrylamide gel electrophoresis/differential in-gel electrophoresis-based matrix-assisted laser desorption ionization-time of flight/MS. They compared the proteome between ARDS patients and normal controls and analyzed the dynamic changes of proteome at different ARDS stages or severity. The disturbed proteome in ARDS patients includes plasma acute-phase proteins, inflammatory/immune-associated proteins, and coagulation proteins. Conclusions: Although several previous studies have provided some useful information about the lung proteome in ARDS patients and gained several interesting disease-associated biomarkers, clinical proteomic studies in ARDS patients are still in the initial stage. An increased cooperation is still needed to establish a global and faithful database containing disease-specific proteome from the largest ARDS subsets. PMID:27647196

  7. Mass Spectrometry-based Proteomics in Acute Respiratory Distress Syndrome: A Powerful Modality for Pulmonary Precision Medicine

    Institute of Scientific and Technical Information of China (English)

    Xue-Feng Xu; Hua-Ping Dai; Yan-Ming Li; Fei Xiao; Chen Wang

    2016-01-01

    Objective:Acute respiratory distress syndrome (ARDS) is an acute and lethal clinical syndrome that is characterized by hypoxemic respiratory failure and diffuse alveolar inflammatory damage.This review aimed to search and discuss the mass spectrometry (MS)-based proteomic studies on different subsets of ARDS patients.Data Sources:Original research articles were collected from the PubMed database published in English up to December 2015.Study Selection:The literature search was done using the term "(acute lung injury OR acute respiratory distress syndrome)AND (proteomics OR proteome OR mass spectrum OR differential in-gel electrophoresis OR two-dimensional polyacrylamide gel electrophoresis)".Related original research articles were included and were carefully analyzed.Results:Eight original proteomic researches on ARDS patients were found.The common proteomic modalities were two-dimensional (2D)high-performance liquid chromatography-based electronic spray ion-MS/MS and 2D-polyacrylamide gel electrophoresis/differential in-gel electrophoresis-based matrix-assisted laser desorption ionization-time of flight/MS.They compared the proteome between ARDS patients and normal controls and analyzed the dynamic changes ofproteome at different ARDS stages or severity.The disturbed proteome in ARDS patients includes plasma acute-phase proteins,inflammatory/immune-associated proteins,and coagulation proteins.Conclusions:Although several previous studies have provided some useful information about the lung proteome in ARDS patients and gained several interesting disease-associated biomarkers,clinical proteomic studies in ARDS patients are still in the initial stage.An increased cooperation is still needed to establish a global and faithful database containing disease-specific proteome from the largest ARDS subsets.

  8. [Rheumatoid arthritis and combined pulmonary fibrosis and emphysema].

    Science.gov (United States)

    Fernández Casares, Marcelo; Fielli, Mariano; Cristaldo, Laura; Zárate, Lucía; Nieves Capozzi, María

    2015-01-01

    The combination of pulmonary fibrosis and emphysema is a syndrome described in the last years, which has its own characteristics and it is not only the casual association between the two entities. The idiopathic pulmonary fibrosis is the most common type of pulmonary fibrosis. However other interstitial lung diseases could be part of this syndrome. Among them is the connective tissue disease-associated interstitial lung disease. We report a case of this syndrome associated with rheumatoid arthritis. It has the peculiarity that the connective disease became overt several years after the presentation of combined pulmonary fibrosis and emphysema syndrome, which is infrequently reported in the literature.

  9. Clinical study on high-resolution CT and pulmonary function in severe acute respiratory syndrome patients during recovery phase

    Institute of Scientific and Technical Information of China (English)

    YIN Cheng-hong; HE Zheng-yi; MA Da-qing; ZHANG Shu-wen; WANG Bao-en; WANG Chao; WEN Yan; JIANG Li; LIU Ying; JIAO Yun-min; CHEN Jiang-hong; TANG Shu-zhen; YUE Mao-xing

    2005-01-01

    @@ Severe acute respiratory syndrome (SARS) is an acute respiratory infectious disease caused by a novel coronavirus, firstly broke out in November 2002 in Guangdong and prevailed quickly in Beijing, Hong Kong, Taiwan and other regions of China. It was one of the most potential pandemic diseases and had affected more than 20 other countries.1,2 There have been a lot of resear-ches2-7 in terms of its etiology, epidemiology, pathogenesis, clinical characteristics, diagnostics, treatment and prevention, vaccines and so on.

  10. Pulmonary Atresia

    Science.gov (United States)

    ... Kawasaki Disease Long Q-T Syndrome Marfan Syndrome Metabolic Syndrome Mitral Valve Prolapse Myocardial Bridge Myocarditis Obstructive Sleep Apnea Pericarditis Peripheral Vascular Disease Rheumatic Fever Sick Sinus Syndrome Silent Ischemia Stroke Sudden ...

  11. 纤维支气管镜在ICU肺源性ARDS患者治疗中应用的价值%Value of fiberoptic bronchoscopy in treatment of pulmonary acute respiratory distress syndrome in ICU

    Institute of Scientific and Technical Information of China (English)

    汲崇良

    2014-01-01

    Objective To explore the application value of fiberoptic bronchoscopy in the pulmonary acute respiratory distress syndrome in ICU.Methods 45 patients with acute respiratory distress syndrome in ICU from October 2010 to October 2013 were selected and divided into two groups.They were given conventional therapy and routine therapy combined with fiberoptic bronchoscopy treatment.Results The heart rate,airway pressure,and oxygenation index of patients with pulmonary acute respiratory distress syndrome after bronchoalveolar lavage and sputum aspiration treatment were significantly better than those in control group (all P <0.05).Conclusions Fiberoptic bronchoscopy has certain value in pulmonary acute respiratory distress syndrome in ICU.%目的 探讨纤维支气管镜在ICU肺源性ARDS患者治疗中应用的价值.方法 从本院ICU2010年10月至2013年10月收治的ARDS患者中随机选择45例进行研究.随机分为2组,分别予以常规治疗和常规治疗联合纤维支气管镜治疗.结果 观察组经纤维支气管镜支气管肺泡灌洗吸痰治疗,心率、气道压力、氧合指数均显著优于对照组(P值均<0.05).结论 纤维支气管镜在ICU肺源性ARDS患者治疗中具有一定的价值.

  12. Ictal central apnea and bradycardia in temporal lobe epilepsy complicated by obstructive sleep apnea syndrome

    Directory of Open Access Journals (Sweden)

    Yoko Nishimura

    2015-01-01

    Full Text Available We describe the case of a 12-year-old boy who developed temporal lobe epilepsy (TLE with daily complex partial seizures (CPS and monthly generalized seizures. Moreover, he frequently snored while asleep since early childhood. Polysomnography (PSG revealed severe obstructive sleep apnea with apnea–hypopnea index (AHI of 37.8/h. Video-PSG with simultaneous electroencephalography (EEG recording captured two ictal apneic episodes during sleep, without any motor manifestations. The onset of rhythmic theta activity in the midtemporal area on EEG was preceded by the onset of apnea by several seconds and disappeared soon after cessation of central apnea. One episode was accompanied by ictal bradycardia of <48 beats/min which persisted for 50 s beyond the end of epileptic activity. After treatment with carbamazepine and tonsillectomy/adenoidectomy, the seizures were well controlled and AHI decreased to 2.5/h. Paroxysmal discharges also disappeared during this time. Uncontrolled TLE complicated by sleep apnea should be evaluated for the presence of ictal central apnea/bradycardia.

  13. Pulmonary hypertension

    Science.gov (United States)

    ... clots in the lung ( pulmonary embolism ) Heart failure Heart valve disease HIV infection Low oxygen levels in the blood for a long time (chronic) Lung disease, such as COPD or pulmonary fibrosis Medicines (for example, certain diet drugs) Obstructive sleep ...

  14. Pulmonary Edema

    Science.gov (United States)

    ... Accessed March 13, 2014. Pinto DS, et al. Pathophysiology of cardiogenic pulmonary edema. http://www.uptodate.com/ ... hvd/. Accessed March 10, 2014. What is pulmonary hypertension? National Heart, Lung, and Blood Institute. http://www. ...

  15. Pulmonary Fibrosis

    Science.gov (United States)

    Pulmonary fibrosis is a condition in which the tissue deep in your lungs becomes scarred over time. This ... blood may not get enough oxygen. Causes of pulmonary fibrosis include environmental pollutants, some medicines, some connective tissue ...

  16. Pulmonary Rehabilitation

    Science.gov (United States)

    ... shortness of breath and increase your ability to exercise. You may have heard that pulmonary rehabilitation is only for people with COPD (chronic obstructive pulmonary disease). We now know that ...

  17. Role of central histaminergic system in lorazepam withdrawal syndrome in rats.

    Science.gov (United States)

    Nath, C; Gupta, M B

    2001-04-01

    Effects of histaminergic agonists and antagonists were investigated on withdrawal signs in lorazepam-dependent rats. Physical dependence was developed by giving lorazepam admixed with the food in the following dose schedule (in mg/kg given daily x days): 10 x 4, 20 x 4, 40 x 4, 80 x 4, and 120 x 7. The parameters observed during the periods of administration of lorazepam and after its withdrawal were spontaneous locomotor activity (SLA), reaction time to pain, foot shock aggression (FSA), and audiogenic seizures. During the withdrawal period, the rats were divided into groups of 10 each. Control-withdrawal group did not receive any drug. The drugs (in mg/kg administered intramuscularly)--L-histidine (50), histamine-N-methyl (2), promethazine (10), pheniramine (10), astemizole (10), and thioperamide (1)--were given separately in other groups daily during the withdrawal period. The withdrawal signs in control group were hyperkinesia, hyperaggression, and audiogenic seizures. L-Histidine, precursor of histamine, and thioperamide, antagonist of H3 receptor, potentiated hyperkinesia, hyperaggression, and audiogenic seizures. Histamine-N-methyl, agonist of H3 receptor, and H1 receptor antagonists, promethazine and pheniramine, blocked all the withdrawal signs. Astemizole, a peripheral antagonist of H1 receptor, could not affect any withdrawal sign. It may be concluded that histamine H1 receptors are facilitatory and H3 receptors are inhibitory for benzodiazepine (BZD) withdrawal syndrome.

  18. The influence of frailty syndrome on acceptance of illness in elderly patients with chronic obstructive pulmonary disease

    Directory of Open Access Journals (Sweden)

    Uchmanowicz I

    2016-09-01

    Full Text Available Izabella Uchmanowicz,1 Beata Jankowska-Polanska,1 Mariusz Chabowski,2 Bartosz Uchmanowicz,1 Andrzej M Fal3 1Department of Clinical Nursing, Faculty of Health Science, Wroclaw Medical University, 2Division of Nursing in Surgical Procedures, Department of Clinical Nursing, Faculty of Health Science, Wroclaw Medical University, 3Department of Healthcare Organisation and Economics, National Institute of Public Health, National Institute of Hygiene, Warsaw, Poland Abstract: COPD is one of the most debilitating diseases. Frailty syndrome and advanced age may decrease the acceptance of illness, quality of life, and worsen health conditions in these patients, as well as lead to an increase in health care expenses. The aim of the study was to assess how the level of frailty affects the acceptance of illness in elderly patients with COPD. We also aimed to evaluate the associations between sociodemographic and clinical factors and the level of acceptance of illness, anxiety, and frailty in this group of patients. The study included 102 COPD patients with a mean age of 63.2 (standard deviation =6.5 years and grades I (3%, II (37%, III (52%, and IV (8% by Global Initiative for Chronic Obstructive Lung Disease. The Polish versions of the Acceptance of Illness Scale and Tilburg frailty indicator were used. Frailty syndrome was found in 77 (75.5% patients, with an average score of 7.42 (standard deviation =2.24. Coexisting diseases such as hypertension (46.07%, coronary artery disease (32.35%, heart failure (28.43%, diabetes (18.63%, and heart arrhythmia (9.8% were found among the subjects. The overall level of acceptance of illness was 20.6 (standard deviation =7.62. A lower level of acceptance of illness was associated with a higher level of frailty, especially in the physical and social domain. Elderly patients with severe COPD are more prone to frailty and decreased acceptance of their disease in comparison to patients with other chronic diseases

  19. A novel Sin Nombre virus DNA vaccine and its inclusion in a candidate pan-hantavirus vaccine against hantavirus pulmonary syndrome (HPS) and hemorrhagic fever with renal syndrome (HFRS).

    Science.gov (United States)

    Hooper, Jay W; Josleyn, Matthew; Ballantyne, John; Brocato, Rebecca

    2013-09-13

    Sin Nombre virus (SNV; family Bunyaviridae, genus Hantavirus) causes a hemorrhagic fever known as hantavirus pulmonary syndrome (HPS) in North America. There have been approximately 200 fatal cases of HPS in the United States since 1993, predominantly in healthy working-age males (case fatality rate 35%). There are no FDA-approved vaccines or drugs to prevent or treat HPS. Previously, we reported that hantavirus vaccines based on the full-length M gene segment of Andes virus (ANDV) for HPS in South America, and Hantaan virus (HTNV) and Puumala virus (PUUV) for hemorrhagic fever with renal syndrome (HFRS) in Eurasia, all elicited high-titer neutralizing antibodies in animal models. HFRS is more prevalent than HPS (>20,000 cases per year) but less pathogenic (case fatality rate 1-15%). Here, we report the construction and testing of a SNV full-length M gene-based DNA vaccine to prevent HPS. Rabbits vaccinated with the SNV DNA vaccine by muscle electroporation (mEP) developed high titers of neutralizing antibodies. Furthermore, hamsters vaccinated three times with the SNV DNA vaccine using a gene gun were completely protected against SNV infection. This is the first vaccine of any kind that specifically elicits high-titer neutralizing antibodies against SNV. To test the possibility of producing a pan-hantavirus vaccine, rabbits were vaccinated by mEP with an HPS mix (ANDV and SNV plasmids), or HFRS mix (HTNV and PUUV plasmids), or HPS/HFRS mix (all four plasmids). The HPS mix and HFRS mix elicited neutralizing antibodies predominantly against ANDV/SNV and HTNV/PUUV, respectively. Furthermore, the HPS/HFRS mix elicited neutralizing antibodies against all four viruses. These findings demonstrate a pan-hantavirus vaccine using a mixed-plasmid DNA vaccine approach is feasible and warrants further development. Copyright © 2013 The Authors. Published by Elsevier Ltd.. All rights reserved.

  20. A novel Sin Nombre virus DNA vaccine and its inclusion in a candidate pan-hantavirus vaccine against hantavirus pulmonary syndrome (HPS) and hemorrhagic fever with renal syndrome (HFRS)☆

    Science.gov (United States)

    Hooper, Jay W.; Josleyn, Matthew; Ballantyne, John; Brocato, Rebecca

    2014-01-01

    Sin Nombre virus (SNV; family Bunyaviridae, genus Hantavirus) causes a hemorrhagic fever known as hantavirus pulmonary syndrome (HPS) in North America. There have been approximately 200 fatal cases of HPS in the United States since 1993, predominantly in healthy working-age males (case fatality rate 35%). There are no FDA-approved vaccines or drugs to prevent or treat HPS. Previously, we reported that hantavirus vaccines based on the full-length M gene segment of Andes virus (ANDV) for HPS in South America, and Hantaan virus (HTNV) and Puumala virus (PUUV) for hemorrhagic fever with renal syndrome (HFRS) in Eurasia, all elicited high-titer neutralizing antibodies in animal models. HFRS is more prevalent than HPS (>20,000 cases per year) but less pathogenic (case fatality rate 1–15%). Here, we report the construction and testing of a SNV full-length M gene-based DNA vaccine to prevent HPS. Rabbits vaccinated with the SNV DNA vaccine by muscle electroporation (mEP) developed high titers of neutralizing antibodies. Furthermore, hamsters vaccinated three times with the SNV DNA vaccine using a gene gun were completely protected against SNV infection. This is the first vaccine of any kind that specifically elicits high-titer neutralizing antibodies against SNV. To test the possibility of producing a pan-hantavirus vaccine, rabbits were vaccinated by mEP with an HPS mix (ANDV and SNV plasmids), or HFRS mix (HTNV and PUUV plasmids), or HPS/HFRS mix (all four plasmids). The HPS mix and HFRS mix elicited neutralizing antibodies predominantly against ANDV/SNV and HTNV/PUUV, respectively. Furthermore, the HPS/HFRS mix elicited neutralizing antibodies against all four viruses. These findings demonstrate a pan-hantavirus vaccine using a mixed-plasmid DNA vaccine approach is feasible and warrants further development. PMID:23892100