Churg-Strauss syndrome in childhood: a case report.

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Albahri, Ziad; Minxová, Lenka; Lukeš, Antonín; Mawiri, Abdul Al; Štefáčková, Šárka

2014-10-01

Churg-Strauss syndrome is a rare form of small-vessel vasculitis. In the current report, we describe the case of a 17-year-old Czech girl predominantly characterized by peripheral neuropathy, the presence of cardiac and pulmonary involvement, hypereosinophilia, asthma, and sinusitis that led to the diagnosis of Churg-Strauss syndrome. © The Author(s) 2013.

Asma e síndrome de Churg-Strauss Asthma and Churg-Strauss syndrome

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Soloni Afra Pires Levy

2006-08-01

Full Text Available Relata-se o caso de uma mulher de 25 anos com síndrome de Churg-Strauss, cujos sintomas surgiram aos dezesseis anos, logo após o início do uso de contraceptivo oral. O quadro clínico evoluiu rapidamente com asma persistente grave, polipose nasal, rinite perene obstrutiva, eosinofilia periférica e tecidual, e mononeurite. A síndrome de Churg-Strauss é uma doença que exige suspeita precoce, diagnóstico preciso, tratamento agressivo e monitoramento periódico, devendo ser considerada no diagnóstico diferencial de asma persistente moderada e grave. O caso relatado chama a atenção para possível participação hormonal e surgimento em idade precoce.We report the case of a 25-year-old woman with Churg-Strauss syndrome, the symptoms of which had first appeared soon after she began taking oral contraceptive at the age of sixteen. The clinical profile evolved rapidly to severe persistent asthma, nasal polyposis, perennial obstructive rhinitis, eosinophilia (peripheral/tissue and mononeuritis. Churg-Strauss syndrome is the type of disease that demands early detection, accurate diagnosis, aggressive treatment and periodic monitoring. It should be considered in the differential diagnosis of moderate and severe persistent asthma. The case reported calls attention to possibility that there is a hormonal component and that the disease can present early onset.

[Mastitis revealing Churg-Strauss syndrome].

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Dannepond, C; Le Fourn, E; de Muret, A; Ouldamer, L; Carmier, D; Machet, L

2014-01-01

Churg-Strauss syndrome often involves the skin, and this may sometimes reveal the disease. A 25-year-old woman was referred to a gynaecologist for inflammation of the right breast with breast discharge. Cytological analysis of the liquid showed numerous inflammatory cells, particularly polymorphonuclear eosinophils and neutrophils. Ultrasound examination of the breast was consistent with galactophoritis. CRP was normal, and hypereosinophilia was seen. The patient was subsequently referred to a dermatology unit. Skin examination revealed inflammation of the entire breast, which was painful, warm and erythematous; the border was oedematous with blisters. Necrotic lesions were also present on the thumbs and knees. Skin biopsy of the breast showed a dermal infiltrate with abundant infiltrate of polymorphonuclear eosinophils, including patchy necrosis and intraepidermal vesicles. Histological examination of a biopsy sample from a thumb revealed eosinophilic granuloma and leukocytoclastic vasculitis. The patient was also presenting asthma, pulmonary infiltrates and mononeuropathy at L3, consistent with Churg-Strauss syndrome. Breast involvement in Churg-Strauss syndrome is very rare (only one other case has been reported). This is the first case in which the breast condition revealed the disease. Cutaneous involvement of the breast is, however, also compatible with Wells' cellulitis. The lesions quickly disappeared with 1mg/kg/d oral prednisolone. Copyright © 2013 Elsevier Masson SAS. All rights reserved.

Churg-Strauss syndrome presenting with conjunctival and eyelid masses: a case report.

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Ameli, F; Phang, K S; Masir, N

2011-12-01

Churg-Strauss syndrome, a small and medium vessel vasculitis, was first described by Churg and Strauss in 1951. It is characterised by the presence of asthma, prominent tissue and blood eosinophilia, systemic vasculitis, and pulmonary and systemic necrotising allergic granulomas. Involvement of the skin, heart and gastrointestinal tract is well documented, but ocular presentation is unusual. We describe a 40-year-old lady who presented with recurrent upper eyelid swelling due to conjunctival lesions. Although she has chronic asthma, Churg-Strauss syndrome was never suspected. The diagnosis of Churg-Strauss syndrome was only made following histological examination of the conjunctival lesions.

Churg-Strauss syndrome: a case with unusual manifestations; Sindrome de Churg-Strauss: a proposito de un caso con manifestaciones poco usuales

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Restrepo, Mauricio; Gonzalez, Luis Alonso; Vasquez, Gloria

2008-07-01

Churg-Strauss syndrome, a necrotizing systemic vasculitis which involves the small and (more rarely) the medium-sized vessels, is a primary vasculitis strongly associated with anti neutrophil cytoplasm antibodies (ANCA). It is characterized by the presence of asthma, eosinophilia and extravascular eosinophilic granulomas. Herein, we report a 36-year-old woman with a history of late onset asthma and allergic rhinitis who developed central nervous system involvement, peripheral neuropathy, leucocytoclastic vasculitis and eosinophilia. Interestingly, unusual clinical manifestations of Churg-Strauss syndrome such as mesenteric micro aneurysms and jaw claudication were present in this particular patient. A brief review of the literature of Churg-Strauss syndrome is presented.

Eosinophilic leukocytoclastic vasculitis - a spectrum ranging from Wells' syndrome to Churg-Strauss syndrome?

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Ratzinger, Gudrun; Zankl, Julia; Eisendle, Klaus; Zelger, Bernhard

2014-01-01

Wells' syndrome is defined as an inflammatory disorder with the histopathological presence of eosinophilic infiltrates and flame figures in the absence of vasculitis. Eosinophilic leukocytoclastic vasculitis shows eosinophilic infiltrates in combination with vasculitic changes. And Churg Strauss Syndrome comprises all three characteristics - eosinophilic infiltrates, vasculitis and flame figures. To determine whether these three diseases are distinct entities or different manifestations of a similar clinicopathologic process. Histopathological samples and clinical courses of 17 patients with eosinophilic infiltrates, flame figures and clinical features of Wells' syndrome were re-evaluated. Histopathologically, we focused on the presence or absence of vasculitic features. Clinically, we included only patients who were diagnosed with Wells' syndrome at least once in the course of their disease. 4 patients were finally diagnosed with Wells' syndrome, 5 with eosinophilic leukocytoclastic vasculitis and 6 with Churg Strauss syndrome. Further, we had one case of an overlap between Wells' syndrome and eosinophilic vasculitis and one case of Wegener granulomatosis. Vasculitic features were found in the samples of all patients. Histologically, we find vasculitic features in typical presentations of Wells' syndrome. Clinically, we find typical features of Wells' syndrome in patients finally diagnosed with eosinophilic leukocytoclastic vasculitis or Churg Strauss syndrome. Furthermore, we have observed and formerly reported 3 patients with progression from Wells' syndrome to Churg Strauss syndrome. Thus, we assume that eosinophilic leukocytoclastic vasculitis might form a bridge between Wells' syndrome and Churg Strauss syndrome.

An Abdominal Presentation of Churg-Strauss Syndrome

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Rees, J. R. E.; Burgess, P.

2010-01-01

Churg-Strauss syndrome is a small and medium vessel vasculitis that is also known as allergic granulomatous angiitis. It most commonly presents with an asthma like symptoms. It was first described in Mount Siani Hospital, New York in 1951 by Jacob Churg and Lotte Stauss and was recognised after the study of a series of 13 patients who had asthma, eosinophilia, granulomatous inflammation necrotising systemic vasculitis and necrotising glomerulonephritis. We describe a case of Churg-Strauss syndrome presenting with abdominal pain and later during the hospital admission a mono-neuritis multiplex syndrome affecting the lower limbs. The patient presented in such an atypical fashion with abdominal signs and symptoms that they required laparotomy and the diagnosis was made after histological examination of tissue taken at the time of surgery. Treatment with immunosuppression and aggressive rehabilitation achieved a progressive recovery which continued on discharge from hospital. PMID:20814555

Churg-Strauss syndrome from an orthopaedic perspective.

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Kung, K L; Yee, P K

2015-12-01

Churg-Strauss syndrome, which has been frequently described by physicians in the literature, is a small and medium-sized vessel systemic vasculitis typically associated with asthma, lung infiltrates, and hypereosinophilia. We report a case of Churg-Strauss syndrome with presenting symptoms of bilateral lower limb weakness and numbness only. The patient was admitted to an orthopaedic ward for management and a final diagnosis was reached following sural nerve biopsy. The patient's symptoms responded promptly to steroid treatment and she was able to walk with a stick 3 weeks following admission. This report emphasises the need to be aware of this syndrome when managing patients with neurological deficit in order to achieve prompt diagnosis and treatment.

[Parotid involvement in Churg-Strauss syndrome].

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Bonnet, R; Bertin, H; Delemazure, A S; Clairand, R; Mercier, J; Corre, P

2014-06-01

Churg-Strauss syndrome is a rare systemic vascularitis. This disease causes eosinophilic tissue infiltration. The most frequent manifestations are cortico-dependent asthma, mono- or polyneuropathy, paranasal sinus polyposis, and digestive and renal dysfunction. Salivary glands are very rarely involved. We describe a case of CSS in a patient presenting with bilateral parotid swelling. The morphological study of salivary glands revealed an unusual thickening of the salivary duct walls. Salivary gland involvement in Churg and Strauss syndrome can be difficult to demonstrate histologically; it does not usually present in the clinical foreground of the disease, and can be a source of misdiagnosis. The biopsy should be performed in the symptomatic gland, away from any previous corticoid treatment. Copyright © 2014 Elsevier Masson SAS. All rights reserved.

Anosmia and hypogeusia in Churg-Strauss syndrome.

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Tallab, Hussam F; Doty, Richard L

2014-05-13

Churg-Strauss syndrome is a rare disorder that is often misdiagnosed. In this report we describe a 57-year-old man with Churg-Strauss syndrome who presented with symptoms of lessened smell and taste function that occurred approximately 3 months before the onset of his neurological symptoms. Psychophysical testing using a battery of well-validated smell and taste tests revealed that the patient had total anosmia and marked hypogeusia. While one anecdotal report exists in the Spanish literature that alludes to the presence of anosmia in a single case of this syndrome, no further confirmation of such dysfunction has appeared in the literature. These findings support the concept that smell and taste loss may be an early sign of this disorder. 2014 BMJ Publishing Group Ltd.

Central retinal artery occlusion in a patient with ANCA-negative Churg-Strauss syndrome

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Kumano, Yuji; Yoshida, Noriko; Fukuyama, Satoru; Miyazaki, Masanori; Enaida, Hiroshi; Matsui, Takaaki

2012-01-01

Ocular involvement in Churg-Strauss syndrome is infrequent. We describe the case of a 54-year-old woman with eosinophilia and involvement of the respiratory tract, skin, and peripheral nervous system, fulfilling the American College of Rheumatology criteria for Churg-Strauss syndrome. The patient presented with acute, painless vision loss in her right eye. Central retinal artery occlusion (CRAO) without accompanying retinal vasculitis was diagnosed by angiographic findings and funduscopic findings of retinal whitening with a cherry-red spot. Although her antineutrophil cytoplasmic antibody (ANCA) status was negative, CRAO was thought to be an ocular manifestation of Churg-Strauss syndrome, and appropriate treatment was planned. She was treated with high-dose corticosteroids and anticoagulant therapy. Her macular edema improved, but visual recovery was poor. Specific therapy to alter inflammation, blood coagulation, and rheology reportedly plays an important role in ANCA-positive patients with Churg-Strauss syndrome who develop CRAO. Regardless of ANCA status, high-dose corticosteroids should be considered for CRAO in patients with Churg-Strauss syndrome, as discussed in this case. PMID:22927731

Churg-strauss syndrome: a case report.

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Zhdan, Vyacheslav М; Kitura, Yevdokiia М; Kitura, Oksana Ye; Babanina, Maryna Yu; Tkachenko, Maksym V; Lebid, Volodymyr G

A clinical case of Churg-Strauss syndrome has been reported on the 53-year-old female patient Ts. with bronchial asthma and allergic rhinitis. The main clinical signs and syndromes depending on the stage of the disease are presented, as well as therapeutic treatment of patients with this disease.

Churg-Strauss syndrome concomitant with chronic symmetrical dacryoadenitis suggesting Mikulicz's disease.

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Hanioka, Yusuke; Yamagami, Keiko; Yoshioka, Katsunobu; Nakamura, Tomomi; Kishida, Masatsugu; Nakamura, Tomoyuki; Yamaguchi, Toshimasa; Koshimo, Naomi; Inoue, Takeshi; Imanishi, Masahito

2012-01-01

A case of Churg-Strauss syndrome complicated by chronic symmetrical dacryoadenitis suggestive of Mikulicz's disease is herein presented. A 72-year-old Japanese man, who had been previously diagnosed with asthma, presented with weakness of the left leg and purpura on the lower extremities. A neurological examination showed multiple mononeuropathies and a laboratory examination revealed elevated eosinophil counts, IgE levels and the presence of Myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCAs). Churg-Strauss syndrome was diagnosed, although the patient also exhibited bilateral swelling of the lachrymal glands. Furthermore, elevated serum IgG4 levels, an infiltration of a relatively large number of IgG4-positive plasmacytes in the nasal mucosa and hypocomplementemia were also observed. These findings were consistent with a diagnosis of Mikulicz's disease (MD). Oral prednisolone (30 mg) was administered and the swelling of the lachrymal glands resolved. Churg-Strauss syndrome may be accompanied by Mikulicz's disease (an IgG4-related disease), and common pathogeneses between Churg-Strauss syndrome and IgG4-related disease may exist.

A Case of Loeffler's Endocarditis Associated with Churg-Strauss Syndrome

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Seo, Jeong-Sook; Kim, Dae-Hee; Kang, Duk-Hyun; Song, Jae-Kwan

2010-01-01

Loeffler's endocarditis is generally caused by hypereosinophilic syndrome. It is a restrictive cardiomyopathy characterized with eosinophilia and eosionophilic penetration leading to the fibrous thickening of endocardium of both ventricles, apical obliteration and heart failure. We report a case of a 23-year-old male with Loeffler's endocarditis caused by Churg-Strauss syndrome. The echocardiogram showed that biventricular failure with large thrombus in left ventricle. His symptoms and typical echocardiographic findings markedly improved within 2 months after treatment for Churg-Strauss syndrome. PMID:20661332

Churg-Strauss Syndrome associated with montelukast: Three Case Reports

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Fatih Yildiz

2014-04-01

Full Text Available Churg-Strauss syndrome (new name Eosinophilic granulomatosis and polyangiitis; asthma, fever, peripheral blood eosinophilia, eosinophilic tissue infiltration, small and medium sized arteries characterized by necrotizing granulomatous inflammation is a multisystemic disorder. Classified in ANCA associated vasculitis. The drugs such as leukotriene receptor antagonists (montelukast, zafirlukast, pranlukast, inhaled glucocorticoids, omalizumab, cocaine and clarithromycin is thought to be associated with Churg-Strauss Syndrome cases have been reported. Herein we presented a rare three CSS cases associated with montelukast. [Cukurova Med J 2014; 39(2.000: 347-352

Combined central retinal artery and vein occlusion in Churg-Strauss syndrome

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Hamann, Steffen; Johansen, Sven; Hamann, Steffen Ellitsgaard

2006-01-01

PURPOSE: To describe a rare case of Churg-Strauss syndrome presenting with severe visual loss due to a combined central retinal vein and artery occlusion. METHODS: A 42-year old man with a medical history of asthma and blood hypereosinophilia developed a sudden loss of vision in his right eye. We...... and dilated and tortuous veins. The diagnosis was confirmed by a fluorescein angiogram showing absence of retinal filling and normal choroidal filling. Churg-Strauss syndrome was diagnosed based on the necessary presence of four of six criteria for the disease proposed by the American College of Rheumatology...... the vascular occlusion and experienced no visual improvement. CONCLUSION: Combined central retinal artery and vein occlusion can occur in Churg-Strauss syndrome. We suggest that regional vasculitis may be the pathological mechanism underlying the vascular occlusions observed in our case. The condition carries...

Acute coronary syndrome associated with Churg-Strauss syndrome

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Annette Doris Wagner

2007-11-01

Full Text Available Annette Doris Wagner1, Gerd Peter Meyer2, Markus Rihl3, Anke Rathmann2, Ulrike Wittkop1, Henning Zeidler4, Hermann Haller1, Joachim Lotz51Department Internal Medicine, Division of Nephrology; 2Division of Cardiology; 3Division of Rheumatology; 4Rheumatologikum Hannover; 5Department of Diagnostic Radiology; Hannover Medical School, Carl-Neuberg-Strasse 1, 30625 Hannover, GermanyAbstract: A 41-year old female patient was admitted with acute onset of dyspnea and chest pain. Previous history revealed asthma, chronic sinusitis and eosinophilic proctitis. Electrocardiogram showed anterior ST-segment elevations and inferior ST-segment depression. Immediate heart catheterization revealed a distally occluded left anterior descending coronary artery, the occlusion being reversible after nitroglycerine. Cardiac magnetic resonance imaging was consistent with perimyocarditis. Hypereosinophilia and IgE elevation were present and Churg-strauss syndrome was diagnosed.Keywords: Churg-Strauss syndrome (CSS, carditis, cardiac MRI

Giant coronary aneurysm in Churg-Strauss syndrome

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Hartmann, M.; Hartmann, M.; Wajon, E.M.; van Houwelingen, G.K.; Stoel, M.G.; von Birgelen, Clemens

2012-01-01

A 64-year-old male with Churg-Strauss syndrome (CSS) presented with worsening dyspnoea without chest pain besides having long-standing asthma. There were no cardiovascular risk factors. The electrocardiogram showed signs of prior anterior infarction. Echocardiography demonstrated severely depressed

Churg-Strauss syndrome involving the breast: a rare cause of eosinophilic mastitis

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Villalba-Nuno, Virtudes [Department of Radiology, C.A.P. II Sant Feliu, Marquesa de Castellbell, Sant Feliu de Llobregat (Spain); Sabate, Josep M; Gomez, Antonio; Torrubia, Sofia [Department of Radiology, Hospital de Sant Pau, Barcelona (Spain); Vidaller, Antonio [Department of Internal Medicine, Hospital de Bellvitge, L' Hospitalet de Llobregat (Spain); Catala, Isabel [Department of Pathology, Hospital de Bellvitge, L' Hospitalet de Llobregat (Spain); Escobedo, Agustin [Department of Oncology, Hospital Duran i Reynals, L' Hospitalet de Llobregat (Spain)

2002-03-01

Churg-Strauss syndrome is a rare immunoallergic disorder that usually affects lungs, skin and nervous system. The clinical and radiological findings of Churg-Strauss disease involving the breast are reported and attention is drawn to the fact that, although uncommonly, the breast can be involved by immunological diseases. (orig.)

Churg-Strauss syndrome involving the breast: a rare cause of eosinophilic mastitis

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Villalba-Nuno, Virtudes; Sabate, Josep M.; Gomez, Antonio; Torrubia, Sofia; Vidaller, Antonio; Catala, Isabel; Escobedo, Agustin

2002-01-01

Churg-Strauss syndrome is a rare immunoallergic disorder that usually affects lungs, skin and nervous system. The clinical and radiological findings of Churg-Strauss disease involving the breast are reported and attention is drawn to the fact that, although uncommonly, the breast can be involved by immunological diseases. (orig.)

Congestive cardiomyopathy and endobronchial granulomas as manifestations of Churg-Strauss syndrome.

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Alvarez-Sala, R.; Prados, C.; Armada, E.; Del Arco, A.; Villamor, J.

1995-01-01

Churg-Strauss syndrome is a systemic vasculitis. Its most frequent complications are heart diseases and asthma. Usually, cardiological manifestations are pericarditis, cardiac failure and myocardial infarction. Endobronchial granulomas identified by bronchoscopy are unusual. We present the case of a man with congestive cardiomyopathy and endobronchial granulomas macroscopically visible at bronchoscopy. After a review of medical literature, we found one case of congestive cardiomyopathy and no cases of endobronchial granulomas observed by bronchoscopy associated with Churg-Strauss syndrome. Images Figure PMID:7644400

Churg-Strauss syndrome: a case report*

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Fernandes, Gabriel Lacerda; Teixeira, Arivaldo Araújo; Antón, Ana Graziela Santana; Reis, Alan Timóteo Rodrigues; de Freitas, Ana Carolina Rezende; Basílio, Dunya Bachour

2014-01-01

Churg-Strauss syndrome is a rare systemic disease primarily characterized by hypereosinophilia, asthma and vasculitis. The lung is the organ most frequently involved. In the present report, the authors describe a relatively rare finding in this disease - the presence of a pulmonary nodule -, while recalling the main radiological findings and the most relevant differential diagnoses. PMID:25741095

Churg-Strauss syndrome: a case report

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Fernandes, Gabriel Lacerda; Reis, Alan Timoteo Rodrigues; Freitas, Ana Carolina Rezende de; Basilio, Dunya Bachour, E-mail: lacerdagabriel@hotmail.com [Hospital de Base do Distrito Federal (HBDF), Brasilia, DF (Brazil); Teixeira, Arivaldo Araujo [Diagnostico das Americas (DASA/Exame-Pasteur), Brasilia, DF (Brazil); Anton, Ana Graziela Santana [Hospital Brasilia, Brasilia, DF (Brazil)

2014-07-15

Churg-Strauss syndrome is a rare systemic disease primarily characterized by hypereosinophilia, asthma and vasculitis. The lung is the organ most frequently involved. In the present report, the authors describe a relatively rare finding in this disease - the presence of a pulmonary nodule -, while recalling the main radiological findings and the most relevant differential diagnoses. (author)

Chronic calcific constrictive pericarditis complicating Churg-Strauss syndrome: first reported case.

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Aboukhoudir, Falah; Pansieri, Michel; Rekik, Sofiene

2014-10-01

Churg-Strauss syndrome is a necrotizing systemic vasculitis characterized by extravascular granulomas and eosinophilic infiltrates of small vessels. Although cardiac complications are considered to be relatively common, no case of constrictive calcified pericarditis has ever been previously described in this setting. In this report, we present the case of a 46-year-old man with Churg-Strauss syndrome, in whom we were able to document the development of symptomatic calcific constrictive pericarditis during a 10-year period despite long-term corticosteroid therapy. Georg Thieme Verlag KG Stuttgart · New York.

Complete remission of coronary vasculitis in Churg-Strauss Syndrome by prednisone and cyclophosphamide

NARCIS (Netherlands)

Riksen, N.P.; Gehlmann, H.R.; Brouwer, A.E.; Deuren, M. van

2013-01-01

The heart is involved in up to 50% of all patients with Churg-Strauss syndrome, but vasculitis of the coronary arteries has only been rarely documented. We present a young patient with severe coronary aneurysms and stenotic lesions due to a Churg-Strauss vasculitis. Prompt therapy with prednisone

Churg-Strauss syndrome associated with AA amyloidosis: a case report.

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Maamar, Mouna; Tazi-Mezalek, Zoubida; Harmouche, Hicham; El Hamany, Zitouna; Adnaoui, Mohammed; Aouni, Mohammed

2012-01-01

Churg Strauss syndrome is a rare systemic and pulmonary vasculitis exceptionally associated with AA amyloidosis. We report the case of a 65-year old woman with past medical history of asthma. She developed polyarthralgia, headache and purpura. A laboratory workout found hypereosinophilia (1150/µL), positive p-ANCA, microscopic haematuria and proteinuria at 2g/day. A diagnosis of Churg-Strauss syndrome was established based on five criteria of the American College of Rheumatology (ACR). Renal biopsy showed an important type AA amyloid deposit. The patient was treated with steroids with a good response of the vasculitis and amyloidosis with disappearance of the proteinuria.

Bullous eosinophilic cellulitis (Wells' syndrome) associated with Churg-Strauss syndrome

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Schuttelaar, M L A; Jonkman, M F

We report a patient with Churg-Strauss syndrome (CSS) with asthma, eosinophilia, nasal polyposis and ANCA-associated multisystem vasculitis, who's skin eruption started with erythematous urticarial-plaques followed by haemorrhagic bullae. Histology of the plaques revealed 'flame figures' in the

Churg-strauss syndrome

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Subhasish Ghosh

2011-01-01

Full Text Available Churg-Strauss syndrome (CSS is a rare granulomatous necrotizing small vessel vasculitis characterized by the presence of asthma, sinusitis, and hypereosinophilia. The cause of this allergic angiitis and granulomatosis is unknown. Other common manifestations are pulmonary infiltrates, skin, gastrointestinal, and cardiovascular involvement. No data have been reported regarding the role of immune complexes or cell mediated mechanisms in this disease, although autoimmunity is evident with the presence hypergammaglobulinemia, increased levels of IgE and Antineutrophil cytoplasmic antibody (positive in 40%. We report the case of a 27-year-old lady presenting with painful swelling of predominantly lower limbs with extensive vesicles and ecchymotic patches and fever shortly after stopping systemic steroids taken for a prolonged duration (2002--2010. The aim of this case report is to point to the possibility of CSS in patients presenting with extensive skin lesions masquerading as Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis Syndrome (SJS/TENS.

Complete remission of coronary vasculitis in Churg-Strauss Syndrome by prednisone and cyclophosphamide.

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Riksen, Niels P; Gehlmann, Helmut; Brouwer, Annemarie E; van Deuren, Marcel

2013-03-01

The heart is involved in up to 50% of all patients with Churg-Strauss syndrome, but vasculitis of the coronary arteries has only been rarely documented. We present a young patient with severe coronary aneurysms and stenotic lesions due to a Churg-Strauss vasculitis. Prompt therapy with prednisone and cyclophosphamide resulted in the complete resolution of all lesions.

Churg-Strauss syndrome masquerading as an acute coronary syndrome.

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Triantafyllis, Andreas S; Sakadakis, Eleftherios A; Papafilippaki, Argyro; Katsimbri, Pelagia; Panou, Fotios; Anastasiou-Nana, Maria; Lekakis, Ioannis

2015-02-01

Churg-Strauss Syndrome (CSS) is a rare vasculitis with multiorgan involvement. Cardiac manifestations are common causing serious complications. We report a case of CSS masquerading as a non-ST elevation myocardial infarction with heart failure. CSS should be considered in the differential diagnosis of an acute coronary syndrome(ACS)with normal coronary arteries when history of asthma, peripheral eosinophilia and multisystemic involvement is present.

[Acute coronary syndrome as a first manifestation of Churg-Strauss syndrome].

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Asdonk, T; Pabst, S; Clauberg, R; Schaefer, C; Skowasch, D; Nickenig, G; Tiyerili, V

2012-03-01

A 53-year-old woman was admitted to our chest pain unit because of an acute coronary syndrome (non ST-elevation myocardial infarction). She complained of asthma, chronic sinusitis and involuntary weight loss, occasional fever and night sweats over the past six months. Coronary angiography did not show any signs of macroscopic coronary artery disease, while echocardiography demonstrated a hemodynamically not significant pericardial effusion. Magnetic resonance imaging of the heart revealed a subendocardial scar, extension and localization pointing to a vascular genesis. Thoracic computed tomography revealed pulmonary opacities and blood tests showed an eosinophilia, leading to the clinical diagnosis of Churg-Strauss syndome. The patient responded quickly to oral steroids, and blood parameters returned to normal. Acute coronary syndrome in youngish patients without classical cardiovascular risk factors is suggestive for myocarditis but also for vasculitis. Churg-Strauss syndrome usually responds quickly to immunosuppressive therapy, associated with a rather good prognosis without high mortality. © Georg Thieme Verlag KG Stuttgart · New York.

Churg-Strauss syndrome

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Ilić Tatjana

2002-01-01

Full Text Available Churg-Strauss syndrome (CSS is medium blood vessels vasculitis with predilection for lungs in patients with bronchial asthma, chronic eosinophilic pneumonia and positive ANCA in the sera in 55-67%. This is a case report of a 60 years old female patient with bronchial asthma, peripheral pulmonary infiltrations, blood eosinophilia, xerophtalmia, tachycardia, chronic rhino sinusitis, polyneuropathia and negative immunological tests: CIC (PEG, CRYO, ANA (IIF, RF (agglutination and ANCA (IIF: pANCA and cANCA; ELISA: proteinase 3, lactoferrin, myeloperoxidase, elastase, cathepsin G. Eosinophilic infiltrates in the tissues tested by skin and salivary gland biopsies were not found. The patient had fulfilled five clinical diagnostic criteria and responded well to immunosuppressive therapy, so this case could be classified as the ANCA negative angiitis and granulomatous of CSS type.

“Peripheral Neuropathy Crippling Bronchial Asthma”: Two Rare Case Reports of Churg-Strauss Syndrome

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Kamal Kishore Pandita

2014-01-01

Full Text Available Churg-Strauss syndrome (CSS is a rare cause of vasculitic neuropathy. Although rare and potentially fatal, Churg-Strauss syndrome (CSS is easily diagnosable and treatable. The presence of bronchial asthma with peripheral neuropathy in a patient alerts a physician to this diagnosis. This is vividly illustrated by the presented two cases who had neuropathy associated with bronchial asthma, eosinophilia, sinusitis, and positive perinuclear antineutrophil cytoplasmic antibodies (p-ANCA test, which improved with administration of steroids.

[A case of hemorrhagic cholecystitis associated with Churg-Strauss syndrome].

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Koizumi, Satomi; Kamisawa, Terumi; Kuruma, Sawako; Chiba, Kazuro; Tabata, Taku; Koizumi, Koichi; Kurata, Masanao; Horiguchi, Shinichiro; Hishima, Tsunekazu

2016-01-01

A woman in her 70s with Churg-Strauss syndrome presented with epigastric pain. She was being treated with steroids at the time of admission. Computed tomography showed swelling of the gallbladder, and percutaneous transhepatic cholangiography revealed bloody secretion. On duodenoscopy, bleeding was observed from the orifice of the major duodenal papilla. Emergency cholecystectomy was performed under a diagnosis of hemorrhagic cholecystitis;intraoperatively, extensive hematoma was detected in the thickened wall of the gallbladder. Subsequent histopathological examination revealed mucosal ulceration with infiltration of inflammatory cells, torn small vessels, and extensive transmural bleeding and abscess formation in the thickened wall of the gallbladder. We considered that the hemorrhagic cholecystitis was induced by either vasculitis or corticosteroid therapy. To the best of our knowledge, this is the first report of hemorrhagic cholecystitis associated with Churg-Strauss syndrome.

The Churg-Strauss syndrome: An unusual presentation

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G K Manu

2013-01-01

Full Text Available The Churg-strauss syndrome (CSS, also referred to as allergic angiitis and granulomatosis is characterized by asthma, peripheral and tissue eosinophilia, extravascular granuloma formation, and vasculitis of multiple organ systems. It is an uncommon disease with an estimated annual incidence of 1-3 per million. Here, we report a case of CSS with glomerulocentric granulomatous reaction with interstitial eosinophils and involvement of retinal vessels.

A Presentation of Massive Hemoptysis in a Patient with Churg-Strauss Syndrome

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Fadi Hikmat

2014-01-01

Full Text Available Given that Churg-Strauss syndrome is a systemic small-vessel vasculitis, it is not usually considered in patients who present with massive hemoptysis, which is typically caused by bronchiectasis, cancer or, in some cases, aberrant bronchial arteries. This article, however, describes a novel case involving a 50-year-old Churg-Strauss patient who presented with sudden-onset massive hemoptysis. Details of the physical examination, laboratory investigations and several imaging studies, including computed tomography, bronchoscopy and three-dimensional imaging, are presented.

An Abdominal Presentation of Churg-Strauss Syndrome

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J. R. E. Rees

2010-01-01

inflammation necrotising systemic vasculitis and necrotising glomerulonephritis. We describe a case of Churg-Strauss syndrome presenting with abdominal pain and later during the hospital admission a mono-neuritis multiplex syndrome affecting the lower limbs. The patient presented in such an atypical fashion with abdominal signs and symptoms that they required laparotomy and the diagnosis was made after histological examination of tissue taken at the time of surgery. Treatment with immunosuppression and aggressive rehabilitation achieved a progressive recovery which continued on discharge from hospital.

Churg–Strauss syndrome: Case series

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T.M. Alfaro

2012-03-01

Full Text Available Churg–Strauss syndrome (CSS is a systemic necrotizing vasculitis of the small and medium vessels, associated with extravascular eosinophilic granulomas, peripheral eosinophilia and asthma.This is a rare syndrome of unknown etiology, affecting both genders and all age groups.CSS patients usually respond well to steroid treatment, although relapses are common after it ends. Timely diagnosis and treatment generally lead to a good prognosis with a 90% survival rate at one year.A brief review of CSS is presented, with particular attention to diagnosis, therapy and recent developments in this area.The authors then report and discuss the clinical, laboratory and imaging characteristics of four patients admitted to an Internal Medicine Department with this diagnosis. The treatment, response and follow-up of the cases are also described. Resumo: A síndrome de Churg-Strauss (SCS é uma vasculite sistémica necrotizante, que afeta os vasos de pequeno e médio calibre e se associa a granulomas eosinofílicos extravasculares, eosinofilia periférica e asma.É uma síndrome rara, de etiologia desconhecida e que afeta ambos os géneros e todos os grupos etários.Os doentes com SCS geralmente apresentam boa resposta à terapêutica com glucocorticoides, embora as recidivas sejam frequentes após a sua suspensão. O diagnóstico e terapêutica atempada levam geralmente a um bom prognóstico, com uma sobrevivência de 90% um ano após o diagnóstico.Neste artigo é apresentada uma breve revisão da SCS, com particular atenção ao diagnóstico, terapêutica e progressos recentes nesta área.De seguida, os autores apresentam e discutem as características clínicas, laboratoriais e imagiológicas de quatro doentes internados num Serviço de Medicina Interna com este diagnóstico. O tratamento instituído, as respostas observadas e o seguimento dos casos são também descritos. Keywords

Churg-Strauss syndrome associated with AA amyloidosis: a case ...

African Journals Online (AJOL)

Churg Strauss syndrome is a rare systemic and pulmonary vasculitis exceptionally associated with AA amyloidosis. We report the case of a 65-year old woman with past medical history of asthma. She developed polyarthralgia, headache and purpura. A laboratory workout found hypereosinophilia (1150/μL), positive ...

Churg-Strauss Syndrome as an Unusual Cause of Dysphagia: Case Report.

Science.gov (United States)

Park, Jihye; Im, Sun; Moon, Su-Jin; Park, Geun-Young; Jang, Yongjun; Kim, Yeonjin

2015-06-01

Systemic vasculitis is a rare disease, and the diagnosis is very difficult when patient shows atypical symptoms. We experienced an unusual case of dysphagia caused by Churg-Strauss syndrome with lower cranial nerve involvement. A 74-year-old man, with a past history of sinusitis, asthma, and hearing deficiency, was admitted to our department for evaluation of dysphagia. He also complained of recurrent bleeding of nasal cavities and esophagus. Brain magnetic resonance imaging did not show definite abnormality, and electrophysiologic findings were suggestive of mononeuritis multiplex. Dysphagia had not improved after conventional therapy. Biopsy of the nasal cavity showed extravascular eosinophilic infiltration. All these findings suggested a rare form of Churg-Strauss syndrome involving multiple lower cranial nerves. Dysphagia improved after steroid therapy.

Churg Strauss Syndrome: a Review | Borke | Nigerian Journal of ...

African Journals Online (AJOL)

Churg Strauss syndrome is a medical condition of unknown aetiology characterized by asthma, eosinophilia and finally vasculitis involving small vessels in the limbs and nasal sinuses and the lungs. The purpose of this review is to highlight the natural history of this condition, the pathogenesis, clinical features and ...

A case of Churg-Strauss syndrome: tissue diagnosis established by sigmoidoscopic rectal biopsy.

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Leen, E J; Rees, P J; Sanderson, J D; Wilkinson, M L; Filipe, M I

1996-01-01

A case is presented of Churg-Strauss syndrome in a young man in whom the definitive diagnostic procedure was a full thickness sigmoidoscopic rectal biopsy, with submucosal sampling. Gastrointestinal changes in Churg-Strauss syndrome, a rare systemic illness characterised by asthma, blood and tissue eosinophilia, vasculitis, and granulomatous inflammation are common but poorly reported. The endoscopic and histopathological features of a case are described and emphasise the potential value of a limited sigmoidoscopy in establishing the diagnosis, when lower gastrointestinal symptoms are present. Images Figure 1 Figure 2 Figure 3 Figure 4 Figure 5 Figure 6 Figure 7 PMID:8801216

[Update Churg-Strauss syndrome].

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Moosig, F; Hellmich, B

2012-11-01

The Churg-Strauss syndrome (CSS) is the rarest subtype of the so-called anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) and has the lowest frequency of ANCA-positivity (around 30%). In addition to asthma and blood eosinophilia, CSS is characterized by end-organ damage, which can be caused by either vasculitis and/or tissue infiltration of eosinophilic granulocytes. The CSS shares many etiological and clinical features of other hypereosinophilic syndromes. Recently, a distinct genetic background could be demonstrated for both the ANCA-positive and ANCA-negative subtypes of CSS as compared to the other two forms of AAV. Among other cytokines, interleukin-5 (IL-5) could be identified as a key mediator of eosinophilia. Therefore, recent clinical trials in CSS aimed to target IL-5. Outside of clinical trials, treatment of CSS is adapted to disease stage and activity, as recommended for other types of AAV.

Churg-Strauss syndrome following cessation of allergic desensitization vaccination: a case report

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Mokri Bahareh

2010-06-01

Full Text Available Abstract Introduction Churg-Strauss syndrome is a vasculitis of medium to small sized vessels. Diagnosis is mainly clinical with findings of asthma, eosinophilia, rhinosinusitis and signs of vasculitis in major organs. Case presentation We present a case of a 19-year-old Persian male who developed signs and symptoms of this syndrome related to hyposensitization treatments for allergy control. Conclusions No unifying etiology for the disease can be presented as it is found associated with environmental factors, medications, infections and is even considered a variant of asthma with predisposition to vasculitic involvement. Therefore, it is important to recognize this disease and be aware of underdiagnosis because of emphasis on pathologic evidence. Here, we present a case of allergic desensitization causing Churg-Strauss syndrome in the absence of other known factors.

Oculomotor Nerve Palsy following Cardiac Tamponade with Churg-Strauss Syndrome: A Case Report

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Kazuki Suganuma

2011-10-01

Full Text Available A 57-year-old man with a history of more than 10 years of bronchial asthma and chronic sinusitis complained of double vision which developed 18 days after cardiac tamponade with eosinophil-rich fluid (eosinophils 30%. He had oculomotor nerve palsy, and a blood test revealed eosinophilia (12,700/mm3 and elevation of both C-reactive protein and rheumatoid factor. He was diagnosed as having Churg-Strauss syndrome. His symptoms were relieved by corticosteroid therapy. Our case and previous cases in the literature revealed that oculomotor nerve palsy in Churg-Strauss syndrome is associated with pupil involvement and may be relieved by corticosteroid treatment.

ANCA-negative Churg-Strauss Syndrome

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Syed Jamil Abdal

2016-08-01

Full Text Available A rare and a disease of unknown etiology, Churg-Strauss syndrome (CSS is a granulomatous necrotizing small vessel vasculitis characterized by the presence of asthma, sinusitis, and hypereosinophilia, which is initially described by Churg and Strauss in 1951. Because of its clinical and pathological features that overlap with those of the other anti-neutrophil antibody (ANCA-associated systemic vasculitides (AASVs and now the disease is classified as AASVs. The ANCA status may dictate the clinical phenotype. ANCA-positive patients are significantly more likely to have disease manifesta­tions associated with small-vessel vasculitis, including oecrotising glomemlonephritis, mononeuritis and purpura, whereas ANCA-negative cases predominantly likely to have cardiac and lung involvement. The objective of this case report is to point out the possibility of vasculitic rash in ANCA-negative CSS in a 35-year-old man and the disease rarely occurs in Bangladeshi population. We analyze the history, clinical examinations and relevant investigations related to the patient to establish the diagnosis in our department. The clinical scenario and biopsy help us to attain the diagnosis. But due to unavailability of patients' cohort we have limitations of comparison of ANCA status in Bangladeshi populations. Though ANCA-positive and ANCA-negative CSS differ phenotypically, primary therapy for both the conditions is systemic glucocorticoids. Additional immunosuppressive agents like cyclophosphamide, mycophenolate mofetil, azathioprine, rituxin1ab are occasionally added in patients with more advanced or refractory disease.

Anterior ischemic optic neuropathy in a patient with Churg-Strauss syndrome.

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Lee, Ji Eun; Lee, Seung Uk; Kim, Soo Young; Jang, Tae Won; Lee, Sang Joon

2012-12-01

We describe a patient with Churg-Strauss syndrome who developed unilateral anterior ischemic optic neuropathy. A 54-year-old man with a history of bronchial asthma, allergic rhinitis, and sinusitis presented with sudden decreased visual acuity in his right eye that had begun 2 weeks previously. The visual acuity of his right eye was 20 / 50. Ophthalmoscopic examination revealed a diffusely swollen right optic disc and splinter hemorrhages at its margin. Goldmann perimetry showed central scotomas in the right eye and fluorescein angiography showed remarkable hyperfluorescence of the right optic nerve head. Marked peripheral eosinphilia, extravascular eosinophils in a bronchial biopsy specimen, and an increased sedimentation rate supported the diagnosis of Churg-Strauss syndrome. Therapy with methylprednisolone corrected the laboratory abnormalities, improved clinical features, and preserved vision, except for the right central visual field defect. Early recognition of this systemic disease by ophthalmologists may help in preventing severe ocular complications.

Churg-Strauss syndrome and hemorragic vasculitis

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Rui Moreira Marques

2011-12-01

Full Text Available Churg-Strauss syndrome (CSS is a rare syndrome characterized by sinusitis, asthma and peripheral eosinophilia. This vasculitic syndrome affects medium and small-sized vessels, the lung being the most commonly affected organ, followed by the skin. The authors report a case of a 59-year-old male with a past history of asthma and allergic rhinitis. He presented necrohemorragic lesions in the distal phalanx of the 2nd, 3rd and 4th fingers of the lefthand and petechial lesions in the plant of both feet, accompanied by asthenia, anorexia and weight loss. The analytical study revealed leukocytosis with eosinophilia, elevated inflammatory parameters and p-ANCA positive antibodies. The diagnosis of CSS was established based on clinical and histopathological data. Cutaneous manifestations of hemorragic vasculitis are rare in CSS syndrome but can be the first manifestation of the disease. The recognition of this presentation is important for the early diagnosis and treatment of this syndrome.

Churg-Strauss syndrome and hemorrhagic vasculitis

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Marques, Rui Moreira; Cabral, Ana Rita; Monteiro, Antonio; Henriques, Pedro

2011-01-01

Churg-Strauss syndrome (CSS) is a rare syndrome characterized by sinusitis, asthma and peripheral eosinophilia. This vasculitic syndrome affects medium and small-sized vessels, the lung being the most commonly affected organ, followed by the skin. The authors report a case of a 59-year-old male with a past history of asthma and allergic rhinitis. He presented necrohemorragic lesions in the distal phalanx of the 2nd, 3rd and 4th fingers of the left-hand and petechial lesions in the plant of both feet, accompanied by asthenia, anorexia and weight loss. The analytical study revealed leukocytosis with eosinophilia, elevated inflammatory parameters and p-ANCA positive antibodies. The diagnosis of CSS was established based on clinical and histopathological data. Cutaneous manifestations of hemorragic vasculitis are rare in CSS syndrome but can be the first manifestation of the disease. The recognition of this presentation is important for the early diagnosis and treatment of this syndrome. PMID:25386301

Usefulness of cardiac resonance imaging in Churg-Strauss syndrome.

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Nadeau, Pierre L; Kumar, Andreas; O'Connor, Kim; Couture, Christian Y; Bourgault, Christine; Dubois, Michelle; Sénéchal, Mario

2016-12-01

: Churg-Strauss syndrome (CSS) is a rare entity that is characterized by widespread vasculitis, which affects both small and medium-sized blood vessels of nearly all organs. More than 50% of these cases have cardiac involvement, which is the major cause of morbidity and mortality. We describe a case of a patient with cardiac biopsy proven CSS, and we discuss the usefulness of cardiovascular MRI for its diagnosis.

Síndrome de Churg-Strauss: relato de caso = Churg-Strauss Syndrome: case report

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Haggsträm, Fábio Maraschin

2008-01-01

Conclusão: apesar de infreqüente, a síndrome de Churg-Strauss deve sempre fazer parte do diagnóstico diferencial da asma de difícil controle, em razão do seu prognóstico desfavorável e da boa resposta ao tratamento

Familial Churg-Strauss Syndrome in a Sister and Brother.

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Alyasin, Soheyla; Khoshkhui, Maryam; Amin, Reza

2015-06-01

Churg-Strauss syndrome (CSS) is a granulomatous small vessel vasculitis. It is characterized by asthma, allergic granulomatosis and vasculitis. This syndrome is rare in children. A 5 years old boy was admitted with cough, fever and dyspnea for 2 weeks. On the basis of laboratory data (peripheral eosinophilia), associated with skin biopsy, and history of CSS in his sister, this disease was eventually diagnosed. The patient had good response to corticosteroid. In every asthmatic patient with prolonged fever, eosinophilia and multisystemic involvment, CSS should be considered.

Churg-Strauss Syndrome following PTU Treatment

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R. A. M. Quax

2009-01-01

Full Text Available Propylthiouracil (PTU is a frequently prescribed drug in the treatment of hyperthyroidism. The use of PTU is, however, accompanied by numerous potentially serious side effects including vasculitis. PTU-related vasculitides can present as haematuria, pulmonary haemorrhage, or cutaneous lesion together with aspecific symptoms such as fever, myalgia, arthralgia, and fatigue. Cerebral involvement is seldom observed. We present a 49-year-old female with Graves' disease and asthma, who developed paresis of the proximal extremities, eosinophilia, pulmonary, and cutaneous lesions following treatment with PTU. A cerebral vasculitis consistent with Churg-Strauss syndrome (CSS was suspected. Although cerebral involvement is seldom observed with PTU treatment, cerebral vasculitis should be considered in patients developing CNS symptoms.

Cutaneous manifestations of Churg-Strauss syndrome: key to diagnosis.

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Marques, Camila Carneiro; Fernandes, Elizabeth Leocadia; Miquelin, Gabriela Momente; Colferai, Mariana Morais Tavares

2017-01-01

Churg-Strauss syndrome is a rare systemic vasculitis characterized by asthma and other allergy symptoms as well as eosinophilia and necrotizing vasculitis involving small and medium-sized vessels. Its prevalence in the general population ranges from 1-3 cases per million a year, varying according to the population studied. The authors describe a case of a female patient affected by the disease with important systemic manifestations and not very florid skin lesions.

Cutaneous manifestations of Churg-Strauss syndrome: key to diagnosis*

Science.gov (United States)

Marques, Camila Carneiro; Fernandes, Elizabeth Leocadia; Miquelin, Gabriela Momente; Colferai, Mariana Morais Tavares

2017-01-01

Churg-Strauss syndrome is a rare systemic vasculitis characterized by asthma and other allergy symptoms as well as eosinophilia and necrotizing vasculitis involving small and medium-sized vessels. Its prevalence in the general population ranges from 1-3 cases per million a year, varying according to the population studied. The authors describe a case of a female patient affected by the disease with important systemic manifestations and not very florid skin lesions. PMID:29267447

Effective intravenous immunoglobulin therapy for Churg-Strauss syndrome (allergic granulomatous angiitis complicated by neuropathy of the eighth cranial nerve: a case report

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Ozaki Yoshio

2012-09-01

Full Text Available Abstract Introduction We report the case of a patient with Churg-Strauss syndrome with eighth cranial nerve palsy. Vestibulocochlear nerve palsy is extremely rare in Churg-Strauss syndrome. To the best of our knowledge, only one case of complicated neuropathy of the eighth cranial nerve has been described in a previous report presenting an aggregate calculation, but no differentiation between polyarteritis nodosa and Churg-Strauss syndrome was made. High-dose immunoglobulin was administered to our patient, and her neuropathy of the eighth cranial nerve showed improvement. Case presentation At the age of 46, a Japanese woman developed Churg-Strauss syndrome that later became stable with low-dose prednisolone treatment. At the age of 52, she developed sudden difficulty of hearing in her left ear, persistent severe rotary vertigo, and mononeuritis multiplex. At admission, bilateral perceptive deafness of about 80dB and eosinophilia of 4123/μL in peripheral blood were found. A diagnosis of cranial neuropathy of the eighth cranial nerve associated with exacerbated Churg-Strauss syndrome was made. Although high doses of steroid therapy alleviated the inflammatory symptoms and markers, the vertigo and bilateral hearing loss remained. Addition of a high-dose immunoglobulin finally resulted in marked alleviation of the symptoms associated with neuropathy of the eighth cranial nerve. Conclusions A high dose of immunoglobulin therapy shows favorable effects in neuropathy of the eighth cranial nerve, but no reports regarding its efficacy in cranial neuropathy have been published.

Churg-Strauss syndrome and leukotriene antagonist use: a respiratory perspective.

LENUS (Irish Health Repository)

Nathani, N

2008-10-01

Churg-Strauss syndrome (CSS) is a rare granulomatous small vessel vasculitis that occurs against a background of longstanding asthma. Leukotriene antagonists (LTAs) are used in the management of asthma and may facilitate a reduction in steroid dosage. Reports of the development of CSS in patients with asthma following the initiation of LTA therapy suggest either a causal association or an unmasking of latent CSS as steroid doses fall. We have undertaken a systematic review to establish whether evidence of a drug induced syndrome exists.

Eosinophilic myocarditis due to Churg-Strauss syndrome with markedly elevated eosinophil cationic protein.

Science.gov (United States)

Hara, Tomoya; Yamaguchi, Koji; Iwase, Takashi; Kadota, Muneyuki; Bando, Mika; Ogasawara, Kozue; Bando, Sachiko; Ise, Takayuki; Niki, Toshiyuki; Ueda, Yuka; Tomita, Noriko; Taketani, Yoshio; Yamada, Hirotsugu; Soeki, Takeshi; Wakatsuki, Tetsuzo; Sata, Masataka

2013-01-01

A 67-year-old woman with asthma visited our hospital with increasing dyspnea and new-onset paresthesia and purpura in her legs. Physical examination showed a wheeze, pretibial edema, and surrounding purpura. Chest X-rays showed cardiac decompensation and an electrocardiogram revealed a new ST-T change. Laboratory data showed leukocytosis, hypereosinophilia (10,450/μL), troponin T(+), elevated BNP, and markedly elevated eosinophil cationic protein (ECP) (> 150 ng/mL). Echocardiography revealed diffuse left ventricular hypokinesis (ejection fraction 30%) with increased wall thickness. Coronary angiography was normal. Cardiac magnetic resonance imaging implied diffuse myocardial edema and subendocardial late gadolinium enhancement. Skin biopsy of purpura showed superfi cial perivascular dermatitis with remarkable eosinophilic infiltrations. No evidence of drug allergies, parasitic infection, or myeloproliferative disorder was detected. Based on these findings, a diagnosis of eosinophilic myocarditis due to Churg-Strauss syndrome was considered. She was administered prednisolone at a dose of 1 mg/kg, cyclophosphamide, and diuretics. Several markers of eosinophilic myocarditis and heart failure gradually improved, including ECP. She was discharged 30 days later with no cardiac event. Eosinophilic myocarditis is characterized by predominantly eosinophilic infi ltration. Eosinophilic granule proteins, such as ECP and major basic protein, play important roles in the pathogenesis of eosinophilic myocarditis. We experienced a rare case of eosinophilic myocarditis due to Churg-Strauss syndrome. Markedly elevated ECP played an important role in the early diagnosis and subsequent reduction in ECP served as a marker of monitoring. In an asthmatic patient with dyspnea, hypereosinophilia, and vasculitis, Churg-Strauss syndrome with eosinophilic myocarditis should be considered.

Eosinophilic granulomatosis with polyangitis (Churg-Strauss syndrome: a diagnostic rarity with an atypical presentation

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Sujeet Raina

2014-01-01

Full Text Available We report a case of a 33-year-old woman who presented to us with symptoms of bronchial asthma and peripheral neuropathy. After investigations, the diagnosis of eosinophilic granulomatosis and polyangitis (Churg-Strauss syndrome was made.

Churg-Strauss syndrome: A rare cause of pleural effusion

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Govind Singh Rajawat

2016-01-01

Full Text Available Churg-Strauss syndrome (CSS is a rare, small-vessel vasculitis associated with a prominent allergic component, asthma, and blood or tissue eosinophilia. Granulomas, eosinophils, and palisading histiocytes in extravascular tissues are hallmarks of this disorder. The presence of asthma or allergy as well as more than 10% of eosinophils in blood is 95% sensitive and 99% specific, respectively, in distinguishing CSS among a subgroup of patients with well-documented systemic vasculitis. We present a case of pleural effusion which was finally diagnosed as CSS. Considering its rarity, this case is reported.

Churg-Strauss Syndrome and pregnancy Successful treatment with intravenous immunoglobulin - Reply

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M. Galeazzi

2011-09-01

Full Text Available La sindrome di Churg-Strauss è una malattia estremamente rara e ancora più raro è riscontrarla in una paziente in stato di gravidanza. Il trattamento iniziale della malattia consiste nella somministrazione di alte dosi di corticosteroidi. I pazienti più gravi o che rispondono poco o insoddisfacientemente ai corticosteroidi vengono solitamente trattati con farmaci citotossici. Le immunoglobuline somministrate per via endovenosa (IgEV stanno dimostrando di essere efficaci nel trattamento di questa patologia, tuttavia non esiste un consenso universale sulla loro effettiva utilità nelle vasculiti sistemiche. Noi presentiamo il caso di una donna con sindrome di Churg-Strauss resistente al trattamento con corticosteroidi e ciclofosfamide. Allorché si riscontrò che la paziente era al 3° mese di gravidanza fu iniziata una terapia con alte dosi di IgEV con ottimi risultati. Questo caso conferma l’utilità del trattamento con IgEV della sindrome di Churg-Strauss e ne dimostra l’efficacia anche in stato di gravidanza.

A laryngeal presentation of Churg-Strauss syndrome in childhood

International Nuclear Information System (INIS)

AlAmmar, Ahmed Y; Yasin, Subhan S; AlMuhsen, Saleh Zaid; AlSaadi Muslim M; AlSohaibanic, Mohammad O

2009-01-01

A 10- year-old female, known to have bronchial asthma, presented with an unusual laryngeal lesion, eventually diagnosed as Churg-Strauss syndrome (CSS). She was referred to our hospital with history of recurrent stridor. On endoscopyhe, the larynx showed signs similar to recurrent respiratory papillomatosis (RRP). CSS is a systemic disorder and is now defined as one of the ANCA (antineutrophil cytoplasmic antibodies) - associated vasculitides. CSS is a systemic disease that may involve unusual sites like the laryynx. Such an unusual presenatation of CSS should be kept in mind, especially in patients with history of asthma. (author)

A laryngeal presentation of Churg-Strauss syndrome in childhood

Energy Technology Data Exchange (ETDEWEB)

AlAmmar, Ahmed Y; Yasin, Subhan S; AlMuhsen, Saleh Zaid [Dept. of Otolaryngology, Head and Neck Surgery, King Abdulaziz Univ. Hospital, Riyadh (Saudi Arabia); M, AlSaadi Muslim [Dept. of Pediatrics, King Abdulaziz Univ. Hospital, Riyadh (Saudi Arabia); AlSohaibanic, Mohammad O [Dept. of Pathology, King Abdulaziz Univ. Hospital, Riyadh (Saudi Arabia)

2009-07-01

A 10- year-old female, known to have bronchial asthma, presented with an unusual laryngeal lesion, eventually diagnosed as Churg-Strauss syndrome (CSS). She was referred to our hospital with history of recurrent stridor. On endoscopyhe, the larynx showed signs similar to recurrent respiratory papillomatosis (RRP). CSS is a systemic disorder and is now defined as one of the ANCA (antineutrophil cytoplasmic antibodies) - associated vasculitides. CSS is a systemic disease that may involve unusual sites like the laryynx. Such an unusual presenatation of CSS should be kept in mind, especially in patients with history of asthma. (author)

Churg-Strauss syndrome presenting with acute kidney injury in a case of primary focal segmental glomerulosclerosis.

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Patil, Sachin B; Vanikar, Aruna V; Gumber, Manoj R; Kute, Vivek B; Shah, Pankaj R; Patel, Himanshu V; Trivedi, Hargovind L

2014-01-01

Churg-Strauss syndrome (CSS) also called allergic granulomatosis and angiitis is a multisystem disorder. Churg-Strauss syndrome is defined as an eosinophil-rich, granulomatous inflammation involving the respiratory tract, along with necrotizing vasculitis affecting small- to medium-sized vessels, and is associated with asthma and eosinophilia. Renal involvement in CSS varies from 26 to 88 % but is usually of mild to moderate stage, and advanced renal failure is uncommon. We encountered an unusual case of 27-year-old man with asthma and primary focal segmental glomerulosclerosis diagnosed as CSS showing myeloperoxidase anti-neutrophil cytoplasmic antibody-associated acute kidney injury with crescentic glomerulonephritis. Patient responded to steroid and cyclophosphamide. Over a follow-up of 2 months, he has no hematuria/eosinophilia and serum creatinine of 2.3 mg/dL has decreased to 1.7 mg/dL.

Crescentic glomerulonephritis in non-asthmatic Churg-Strauss syndrome.

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Kaul, Anupma; Sharma, Raj Kumar; Jaisuresh, Krishna Swamy; Agrawal, Vinita

2014-03-01

A 58-year-old male presented with sensory motor polyneuropathy and rapidly progressive renal failure. Investigations revealed marked peripheral eosinophilia and elevated perinuclear antineutrophil cytoplasmic antibody titers. Renal biopsy showed pauci-immune cre-scentic glomerulonephritis with interstitial eosinophil infiltrates. He had no history of asthma. Computed tomography of the chest and X-ray of the paranasal sinuses were normal. On Day 1, the patient developed ileal perforation. Resected ileal segments showed small vessel vasculitis with extravascular eosinophils. A diagnosis of non-asthmatic variant of Churg-Strauss syndrome was made. Renal recovery was achieved in 12 weeks with a combination therapy of corticosteroid and cyclophosphamide. The patient has been relapse-free for 12 months on oral prednisolone therapy.

Síndrome de Churg-Strauss: casuística

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T.M. Alfaro

2012-03-01

Full Text Available Resumo: A síndrome de Churg-Strauss (SCS é uma vasculite sistémica necrotizante, que afeta os vasos de pequeno e médio calibre e se associa a granulomas eosinofílicos extravasculares, eosinofilia periférica e asma.É uma síndrome rara, de etiologia desconhecida e que afeta ambos os géneros e todos os grupos etários.Os doentes com SCS geralmente apresentam boa resposta à terapêutica com glucocorticoides, embora as recidivas sejam frequentes após a sua suspensão. O diagnóstico e terapêutica atempada levam geralmente a um bom prognóstico, com uma sobrevivência de 90% um ano após o diagnóstico.Neste artigo é apresentada uma breve revisão da SCS, com particular atenção ao diagnóstico, terapêutica e progressos recentes nesta área.De seguida, os autores apresentam e discutem as características clínicas, laboratoriais e imagiológicas de quatro doentes internados num Serviço de Medicina Interna com este diagnóstico. O tratamento instituído, as respostas observadas e o seguimento dos casos são também descritos. Abstract: Churg-Strauss syndrome (CSS is a systemic necrotizing vasculitis of the small and medium vessels, associated with extravascular eosinophilic granulomas, peripheral eosinophilia and asthma.This is a rare syndrome of unknown etiology, affecting both genders and all age groups.CSS patients usually respond well to steroid treatment, although relapses are common after it ends. Timely diagnosis and treatment generally lead to a good prognosis with a 90% survival rate at one year.A brief review of CSS is presented, with particular attention to diagnosis, therapy and recent developments in this area.The authors then report and discuss the clinical, laboratory and imaging characteristics of four patients admitted to an Internal Medicine Department with this diagnosis. The treatment, response and follow-up of the cases are also described. Palavras-chave: Síndrome de Churg-Strauss, Vasculite, Revisão, Casu

Crescentic glomerulonephritis in non-asthmatic Churg-Strauss syndrome

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Anupma Kaul

2014-01-01

Full Text Available A 58-year-old male presented with sensory motor polyneuropathy and rapidly progressive renal failure. Investigations revealed marked peripheral eosinophilia and elevated perinuclear antineutrophil cytoplasmic antibody titers. Renal biopsy showed pauci-immune cre-scentic glomerulonephritis with interstitial eosinophil infiltrates. He had no history of asthma. Computed tomography of the chest and X-ray of the paranasal sinuses were normal. On Day 1, the patient developed ileal perforation. Resected ileal segments showed small vessel vasculitis with extravascular eosinophils. A diagnosis of non-asthmatic variant of Churg-Strauss syndrome was made. Renal recovery was achieved in 12 weeks with a combination therapy of corticosteroid and cyclophosphamide. The patient has been relapse-free for 12 months on oral prednisolone therapy.

Síndrome de Churg Strauss

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Édgar Alberto Sánchez Morales

2002-07-01

Full Text Available El Síndrome de Churg-Strauss denominado Granulomatosis alérgica y angeitis está caracterizado por una vasculitis sistémica de pequeños vasos, granulo mas extravasculares e hipereosinofilia. Inicialmente descrito por Jacob Churg y Lotte Strauss, dos patólogos quienes en 1951 publicaron la descripción de 13 pacientes postmortem con infiltración tisular por eosinófilos, vasculitis necrotizante y granulomas extravasculares (3; los casos descritos tenían clínicamente asma severa, fiebre, hipereosinofilia y evidencia de anormalidades vasculares en varios órganos y sistemas.

Churg-Strauss syndrome presenting as polymiositis: report of a case

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E. Presotto

2011-06-01

Full Text Available We reported the case of a male patient with Churg-Strauss syndrome (CSS heralding as symptoms typical of polymiositis. During high-dose cortisone therapy (1.5 mg/kg/day, he developed a severe multiplex mononeuritis, poorly responsive to immunoglobulins and methotrexate administration. After 6 months he developed a partial deficiency of the right sciatic popliteus and the radial nerves. Sural nerve biopsy showed a characteristic necrotizing vasculitis of the epineural vessels with granulocyte and eosinophil infiltrates. In the course of CSS, peripheral nervous system involvement is frequent and can lead to disability. For this reason, it must be promptly recognized and properly treated.

Eosinophilic granulomatosis with polyangiitis (formerly known as Churg-Strauss syndrome as a differential diagnosis of hypereosinophilic syndromes

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Yuri Albuquerque Pessoa Santos

2017-01-01

Full Text Available Eosinophilic granulomatosis with polyangiitis (EGPA, formerly known as Churg-Strauss syndrome, is a rare systemic disease situated between primary small vessel vasculitides associated with antineutrophil cytoplasmic antibodies (ANCAs and hypereosinophilic syndromes (HES. Here, we present a case of EGPA in a 38-year-old male, with a previous diagnosis of asthma, who presented with fever, migratory lung infiltrates and systemic eosinophilia that was refractory to previous courses of antibiotics. This case highlights the importance of the primary care physician understanding the differential diagnosis of pulmonary eosinophilic syndromes.

A pseudoleukemic blood differentiation in a 13-year-old child: an extraordinary presentation of Churg-Strauss syndrome

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Mutsaers, E. R.; Witteveen, R.; van den Bosch-Ruis, W.; Kuijpers, T. W.; van Houten, M. A.; van den Berg, J. M.

2013-01-01

Churg-Strauss syndrome (CSS) is a rare systemic vasculitis of the small-and medium-size vessels. It is mostly seen in elderly patients presenting as de novo asthma, eosinophilia, and vasculitic organ involvement. In childhood, CSS is extremely rare. The course of pediatric CSS is usually severe and

Cutting edge issues in the Churg-Strauss syndrome.

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Szczeklik, Wojciech; Jakieła, Bogdan; Adamek, Dariusz; Musiał, Jacek

2013-02-01

Churg-Strauss syndrome (CSS) is a rare systemic small-vessel vasculitis that develops in the background of bronchial asthma, which is characterized by eosinophilia and eosinophilic infiltration of various tissues. It belongs to the group of antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitides. The triggering factors and pathogenesis of CSS are still unknown. The possible role of eotaxin-3 and CCR4-related chemokines in selective recruitment of eosinophils to the target tissues in CSS has been recently suggested, but the role of eosinophilic inflammation in the development of vasculitic lesions is not completely understood. From the clinical view, two distinct phenotypes of the disease are slowly emerging depending on the ANCA-positivity status. Glucocorticoids are still the mainstay of treatment; however, data are accumulating regarding the beneficial role of novel immunosuppressants and biologic compounds, especially in patients with poorer prognosis.

Unusual finding in pediatric Churg-Strauss: renal lesions on CT

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Oldan, Jorge; McCauley, Roy; Pilichowska, Monica; Milner, Lawrence; Lopez-Benitez, Jorge M.

2011-01-01

After a 19-year-old female experienced several weeks of unrelieved fevers, an abdominal CT revealed multiple low-attenuation renal lesions. As the differential included lymphoma, infections and infarcts, a core biopsy of the kidney was performed, which revealed changes consistent with Churg-Strauss syndrome. (orig.)

Unusual finding in pediatric Churg-Strauss: renal lesions on CT

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Oldan, Jorge; McCauley, Roy [Tufts Medical Center, Department of Radiology, Boston, MA (United States); Pilichowska, Monica [Tufts Medical Center, Department of Pathology, Boston, MA (United States); Milner, Lawrence [Tufts Medical Center, Department of Nephrology, Boston, MA (United States); Lopez-Benitez, Jorge M [Tufts Medical Center, Department of Rheumatology, Boston, MA (United States)

2011-08-15

After a 19-year-old female experienced several weeks of unrelieved fevers, an abdominal CT revealed multiple low-attenuation renal lesions. As the differential included lymphoma, infections and infarcts, a core biopsy of the kidney was performed, which revealed changes consistent with Churg-Strauss syndrome. (orig.)

Heart transplantation in patients with eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome).

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Groh, Matthieu; Masciocco, Gabriella; Kirchner, Elizabeth; Kristen, Arnt; Pellegrini, Carlo; Varnous, Shaïda; Bortman, Guillermo; Rosenberg, Mark; Brucato, Antonio; Waterworth, Paul; Bonacina, Edgardo; Facchetti, Fabio; Calabrese, Leonard; Gregorini, Gina; Scali, Juan Jose; Starling, Randall; Frigerio, Maria; D'Armini, Andrea Maria; Guillevin, Loïc

2014-08-01

Heart involvement is the leading cause of death of patients with eosinophilic granulomatosis with polyangiitis (EGPA; formerly Churg-Strauss syndrome) and is more frequent in anti-neutrophil cytoplasm antibody (ANCA)-negative patients. Post-transplant outcome has only been reported once. We conducted a retrospective international multicenter study. Patients satisfying the criteria of the American College of Rheumatology and/or revised Chapel Hill Consensus Conference Nomenclature were identified by collaborating vasculitis and transplant specialists, and the help of the Churg-Strauss Syndrome Association. Nine ANCA(-) patients who received transplants between October 1987 and December 2009 were identified. The vasculitis and cardiomyopathy diagnoses were concomitant for 5 patients and separated by 12 to 288 months for the remaining 4 patients. Despite ongoing immunosuppression, histologic examination of 7 (78%) patients' explanted hearts showed histologic patterns suggestive of active vasculitis. The overall 5-year survival rate was low (57%), but rose to 80% when considering only the 6 patients transplanted during the last decade. After survival lasting 3 to 60 months, 4 (44%) patients died sudden deaths. The search for EGPA-related cardiomyopathy is mandatory early in the course of this type of vasculitis. Indeed, prompt treatment with corticosteroids and cyclophosphamide may achieve restore cardiac function. Most patients in this series were undertreated. For patients with refractory EGPA, heart transplantation should be performed, which carries a fair prognosis. No optimal immunosuppressive strategy has yet been identified. Copyright © 2014 International Society for Heart and Lung Transplantation. All rights reserved.

Subarachnoid and Intracerebral Hemorrhage in Patients with Churg-Strauss Syndrome: Two Case Reports

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Go, Myeong Hoon; Park, Jeong Un; Kang, Jae Gyu

2012-01-01

Churg-Strauss syndrome (CSS) is a systemic necrotizing vasculitis of the small and medium vessels, associated with extravascular eosinophilic granulomas, peripheral eosinophilia, and asthma. The exact etiology of CSS is unknown. This syndrome commonly affects the lungs, peripheral nerves, skin, heart, and gastrointestinal tract, but rarely the central nervous system. Subarachnoid and intracerebral hemorrhage in CSS patients is extremely rare; however, clinicians should consider that CSS may be a cause of intracranial hemorrhage and its high rate of mortality and morbidity. The authors report on two cases of subarachnoid and intracerebral hemorrhage with CSS and discuss a brief review of CSS. PMID:23210058

Sindroma de Churg-Strauss: um diagnóstico de asma e eosinofília

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Tiago Tribolet de Abreu

2001-11-01

Full Text Available RESUMO: Introdução: O S. de Churg-Strauss (SCS é uma entidade clínica em que um quadro de asma e eosinofilia coexiste com um atingimento multissistémico.Caso-clínico: Um homem de 63 anos, com diagnóstico de asma e rinite alérgica desde os 55 anos, inicia um quadro de febre, artrite, polineuropatia e hematúria. Verificou-se a existência de eosinofilia marcada e sinusopatia maxilar e etmoidal bilateral. Iniciou corticoterapia, com regressão completa do quadro clínico e laboratorial. Está actualmente assintomático, com Prednisolona 5 mg/dia.Discussão: Revendo os diagnósticos diferenciais de doença pulmonar com eosinofilia,verificámos que a presença de atingimento extrapulmonar permite considerarmos o SCS e o S. hipereosinofílico idiopático como únicas hipóteses. A existência de asma selecciona o SCS como o diagnóstico correcto.Conclusões: a perante um doente com asma e eosinofilia (superior a 10%, o S. de Churg-Strauss é um diagnóstico a ter sempre presente; b o diagnóstico é principalmente clínico, de acordo com os critérios de classificação; c a corticoterapia é muito eficaz e o seu desmame lento permite doses de manutenção baixas.REV PORT PNEUMOL 2001; VII (6: ABSTRACT: Background: The Churg-Strauss syndrome (CSS is a disease in which asthma and eosinophilia occur with multisystemic manifestations.Case-report: A 63 year old male, with a diagnosis of asthma and allergic rhinitis for 8 years, has fever, arthritis, peripheral neuropathy and hematuria. Marked eosinophilia and sinusitis were found. Corticosteroids were given, with complete clinical and laboratorial remission. The patient is asymptomatic with a daily dose of 5 mg of Prednisolone.Discussion: Reviewing the differential diagnosis of pulmonary disease and eosinophilia, the extrapulmonary involvement only exists in the CSS and the hypereosinophilic syndrome. Asthma is present only in the former, and so the Churg-Strauss syndrome was the correct

Echocardiographic Changes in Eosinophilic Endocarditis Induced by Churg-Strauss Syndrome.

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Masaki, Nobuyuki; Issiki, Ami; Kirimura, Masato; Kamiyama, Tetsuo; Sasaki, Osamu; Ito, Hiroyuki; Maruyama, Yoshiaki; Nishioka, Toshihiko

Eosinophilic myocarditis may be accompanied by Churg-Strauss syndrome (CSS). We report a case of CSS that was accompanied by myocardial changes in the early stage. A 71-year-old woman complained of mild chest pain at rest, but routine echocardiography did not reveal any endocardial abnormalities. Four months later, the patient was hospitalized due to congestive heart failure with neuropathy of both upper extremities. A diagnosis of eosinophilic myocarditis was made based on the patient's laboratory results and the presence of mural thrombus. This case illustrates that, although early eosinophilic myocarditis is an important differential diagnosis in patients with chest pain, it may be difficult to identify in without an apparent mural thrombus.

Churg-Strauss syndrome presenting with eosinophilic myocarditis: a diagnostic challenge.

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Correia, Ana Sofia; Gonçalves, Alexandra; Araújo, Vítor; Almeida e Silva, João; Pereira, José Manuel; Rodrigues Pereira, Pedro; Pizarro, Manuel; Silva, João Carlos; Maciel, Maria Júlia

2013-09-01

Churg-Strauss syndrome (CSS) is an unusual disease that presents as systemic vasculitis and peripheral eosinophilia in patients with an atopic constitution. Cardiac involvement is unusual and often not prominent on initial presentation, but is an important cause of morbidity and mortality in patients with CSS. We report the case of a young woman with severe acute myocarditis. Coronary arteriography demonstrated extensive focal vasculopathy, consistent with coronary vasculitis, and myocardial biopsy showed eosinophilic myocarditis. This presentation led to an initial diagnosis of CSS in this patient and appropriate therapy resulted in a spectacular remission of disease activity. Copyright © 2012 Sociedade Portuguesa de Cardiologia. Published by Elsevier España. All rights reserved.

Churg-Strauss syndrome: A case report

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Dinić Miroslav Ž.

2013-01-01

Full Text Available Introduction. Churg-Strauss syndrome (CSS is an allergic granulomatous angiitis, a rare disease of small and medium arteries and veins, associated with the presence of perinuclear antineutrophil cytoplasmic antibodies (p-ANCA. According to the American College of Rheumatology (ACR, there are four or more criteria out of six for the diagnosis: asthma, eosinophilia (> 10% in peripheral blood, paranasal sinusitis, pulmonary infiltrates, histological evidence of vasculitis with extravascular eosinophils, and mononeuritis multiplex or polyneuropathy. Case report. We reported a female patient, aged 80 years, with asthma for many decades and repeatedly verified eosinophilia in peripheral blood, in which CSS was suspected only after the occurrence of skin changes in the form of vesicles, vesiculopustule, purpuric macula, papule and petechiae. Further tests verified pulmonary infiltrates, paranasal sinusitis, extravascular eosinophils on histopathologic sample of skin tissue, and polyneuropathy. The treatment started with methylprednisolone (60 mg/d, with decreasing doses, and continued with pulse doses of cyclophosphamide (800 mg once monthly, also corticosteroid ointment for skin lesions. Conclusion. Despite long-standing pulmonary symptoms and laboratory findings of eosinophilia, the appearance of skin changes raised suspicion of possible CSS. Skin changes resolved and the patient was reffered to rheumatologist.

Coronary involvement in Churg-Strauss syndrome.

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Dendramis, Gregory; Paleologo, Claudia; Piraino, Davide; Arrotti, Salvatore; Assennato, Pasquale

2015-01-01

Systemic autoimmune diseases are themselves a relevant and independent risk factor for atherosclerosis and coronary ectasia. We describe a case of a 58-year-old Caucasian man who was admitted to our department for unstable angina. History of asthma, paranasal sinus abnormality, and peripheral eosinophilia given a high suspicion of Churg-Strauss syndrome (CSS). Diagnosis was performed with 5 of the 6 American College of Rheumatology criteria. The knowledge that CSS is often associated with significant coronary artery involvement and the persistence of chest pain led us to performing immediately a coronary angiography. Coronary angiography showed diffuse ectasic lesions, chronic occlusion of left anterior descending artery with homocoronary collateral circulation from left circumflex artery and subocclusive stenosis in the proximal tract of posterior descending artery. The early recognition of CSS, an aggressive invasive diagnostic approach, and an early appropriate therapy are important to prevent the progressive and permanent cardiac damage in these patients. In the setting of a multidisciplinary approach, careful cardiac assessment is an essential step in CSS, even in mildly symptomatic patients. Copyright © 2015 Cardiological Society of India. Published by Elsevier B.V. All rights reserved.

Churg-strauss syndrome without respiratory symptoms in a child

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R B Basak

2011-01-01

Full Text Available Churg-Strauss Syndrome (CSS is rare in children. It consists of a small- and medium-sized vessel vasculitis, with skin and peripheral nerve involvement. It is characterized by eosinophilia, extravascular necrotizing granuloma, and eosinophilic infiltration of multiple organs particularly the lungs, but may also involve the gastrointestinal tract, the heart, and the kidneys. The condition is usually associated with a preceding history of asthma or allergic sinusitis. It has rarely been reported in children, where most of the cases had pre-existing asthma, allergic rhinitis, or atopic disease. We report a 10-year-old Arab girl proven to have CSS, with no history of asthma or allergic rhinitis, who presented with tender cutaneous nodules of lower extremities, foot drop, and peripheral eosinophilia, without any clinical respiratory symptoms or signs.

Cotrimoxazole-Induced Hypoglycaemia in a Patient with Churg-Strauss Syndrome

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Russell Senanayake

2013-01-01

Full Text Available Cotrimoxazole is a commonly used antimicrobial agent which is traditionally indicated in the management of pneumocystis infection of which HIV and immunosuppressed individuals are at high risk. Furthermore, it can be used on the long term for prophylactic indications. Hypoglycaemia following commencement of cotrimaoxazole is a rare adverse effect which was first described in 1988. We describe a case of hypoglycaemia shortly following initiation of cotrimoxazole indicated as long-term prophylaxis on a background of Churg-Strauss syndrome. The patient was symptomatic for hypoglycaemia despite simultaneous use of high-dose prednisolone; however, the hypoglycaemia did not require a hospital admission. We will explore the risk factors, monitoring requirements, and the mechanism by which co-trimoxazole induces hypoglycaemia.

Catheter-Directed Thrombolysis of Deep Vein Thrombosis in a Patient with Churg-Strauss Syndrome: A Case Report

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Park, Jun Beom; Kim, See Hyung; Choi, Jin Soo; Kim, Young Hwan [Dongsan Hospital, Keimyung University, Daegu (Korea, Republic of)

2010-10-15

Vasculitis by Churg-Strauss Syndrome (CSS) is an uncommon disease characterized by the inflammation of blood vessel walls and can involve many organs. The clinical manifestations and courses of vasculitis are highly variable. Deep vein thrombosis has rarely been reported in vasculitis by CSS. We report a case of deep vein thrombosis associated with CSS that was successfully treated by catheter-directed thrombolysis.

Catheter-Directed Thrombolysis of Deep Vein Thrombosis in a Patient with Churg-Strauss Syndrome: A Case Report

International Nuclear Information System (INIS)

Park, Jun Beom; Kim, See Hyung; Choi, Jin Soo; Kim, Young Hwan

2010-01-01

Vasculitis by Churg-Strauss Syndrome (CSS) is an uncommon disease characterized by the inflammation of blood vessel walls and can involve many organs. The clinical manifestations and courses of vasculitis are highly variable. Deep vein thrombosis has rarely been reported in vasculitis by CSS. We report a case of deep vein thrombosis associated with CSS that was successfully treated by catheter-directed thrombolysis

Multiple cerebral and cerebellar infarcts as the first clinical manifestation in a patient with Churg-Strauss syndrome: case report and literature review.

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Cheng, Meng-Ju; Huang, Pai-Hao; Liao, Pin-Wen; Chen, Jen-Tse; Chiang, Tsuey-Ru

2012-12-01

Churg-Strauss syndrome (CSS) is a rare autoimmune disease with small-vessel vasculitis. Neurological manifestation of CSS is common. Central nervous system is less frequently involved than that of peripheral nervous system. We report a case of 60-year-old man who presented with acute onset of right hemiparesis and impaired cognition. The presence of hypereosinophilia, asthma, sinusitis and extravascular eosinophil accumulation led to the diagnosis of Churg-Strauss syndrome. Brain magnetic resonance imaging (MRI) revealed multiple infarcts in bilateral cerebral and cerebellar hemispheres. The neurophysiology study did not reveal peripheral neuropathy. The patient was effectively treated with methylprednisolone, cyclophosphamide and warfarin. Symptoms and signs of central nervous system can be the initial neurological manifestation of CSS patients. CSS should be considered while patients have stroke and hypereosinophilia. In our patient, there is a good response to timely steroid, immunosuppressant and anticoagulant therapies.

Churg-Strauss syndrome associated with antiphospholipid antibodies in a patient with retinal vasculitis.

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Sánchez-Vicente, J L; Gálvez-Carvajal, S; Medina-Tapia, A; Rueda, T; González-García, L; Szewc, M; Muñoz-Morales, A

2016-11-01

We present the case of a 69-year-old woman with unilateral retinal vasculitis. Investigations showed asthma, rhinosinusitis, nasal polyposis, peripheral blood eosinophilia, increased sedimentation rate, proteinuria, and antiphospholipid antibodies. Anti-neutrophil cytoplasmic antibodies (ANCA) were negative. Although her anti-neutrophil cytoplasmatic antibody (ANCA) status was negative, taking into account the other clinical and laboratory features, retinal vasculitis was thought to be an ocular manifestation of Churg-Strauss syndrome. Treatment was started with high-dose corticosteroids and anticoagulant therapy. Copyright © 2016 Sociedad Española de Oftalmología. Publicado por Elsevier España, S.L.U. All rights reserved.

Polyangiitis overlap syndrome of granulomatosis with polyangiitis (Wegener's granulomatosis) and eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome).

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Uematsu, Hironori; Takata, Shohei; Sueishi, Katsuo; Inoue, Hiromasa

2014-02-27

Polyangiitis overlap syndrome is defined as systemic vasculitis that cannot be classified into one of the well-defined vasculitic syndromes. In this report, a female patient who presented with vasculitis-like and asthmatic symptoms was diagnosed as having polyangiitis overlap syndrome of granulomatosis with polyangiitis (GPA; formerly known as Wegener's granulomatosis) and eosinophilic granulomatosis with polyangiitis (EGPA; formerly known as Churg-Strauss syndrome). The patient fulfilled the American College of Rheumatology diagnostic criteria for GPA and EGPA. She was successfully treated with immunosuppressants and steroids and has been in remission for 20 months. It is important to establish a proper diagnosis and introduce an appropriate treatment modality in patients with this rare and serious pathology to prevent irreversible organ damage.

Coronary involvement in Churg-Strauss syndrome: a case report with CT findings.

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Doo, Kyung Won; Yong, Hwan Seok; Kang, Eun-Young

2013-12-01

We report a case of Churg-Strauss syndrome (CSS) associated with coronary artery involvement, as demonstrated on coronary CT angiography (CCTA), without specific cardiac symptoms. A 69-year-old male had an 8-year history of bronchial asthma and chronic sinusitis with hypereosinophilia (35 %), polyneuropathy, and a positive antineutrophil cytoplasmic antibody titer, so he was diagnosed with CSS. The patient had no specific cardiac symptoms, but CCTA showed vasculitis and a saccular aneurysm involving the proximal coronary arteries. The 3-year follow-up CCTA demonstrated an increase in the extent of soft-tissue wall thickening and infiltration involving the coronary arteries. Although vasculitis of the major coronary arteries is not a prominent feature of CSS, our case suggests that the coronary arteries may also be targeted in this syndrome.

[Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)].

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Guillevin, Loïc

2012-10-01

Eosinophilic granulomatosis with polyangiitis (EGPA) (Churg-Strauss), is a rare necrotizing vasculitis of small-sized vessels, associated to antimyeloperoxydase ANCA in 40% of patients. EGPA occurs in patients with asthma. Asthma is sever, associated with eosinophilia and extrapulmonary symptoms. Among them, mononeuritis multiplex is the most frequent symptom. When cardiac involvement is present, prognosis is poor. Despite a good overall prognosis, deaths are caused by vasculitis activity, gastrointestinal and cardiac involvement. Treatment is well codified based on steroids, which are quickly effective. Immunosuppressants combined with corticosteroids are compulsory to treat the most sever forms, mainly when cardiac and gastrointestinal or renal symptoms are present. Copyright © 2012. Published by Elsevier Masson SAS.

Eosinophilic myocarditis due to Churg-Strauss syndrome mimicking reversible dilated cardiomyopathy.

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Chen, Ming-xian; Yu, Bi-lian; Peng, Dao-quan; Zhou, Sheng-hua

2014-01-01

A 41-year-old woman with a history of asthma arrived at the emergency room of our hospital with dyspnea. The electrocardiogram showed no specific results. Echocardiography defects revealed an obvious decrease in the left ventricular systolic function and enlargement of the left chamber. We initially considered her condition to be dilated cardiomyopathy. However, she had eosinophilia in the peripheral blood and elevated cardiac enzymes. The coronary angiography showed normal coronary arteries. Single photon emission computed tomography (SPECT) showed infiltrative myocardial disease. She was then diagnosed with eosinophil infiltrations. Combined with peripheral nerve injury and lung involvement, she was diagnosed as having Churg-Strauss syndrome. After initiating prednisone treatment, her eosinophilia and rising cardiac enzymes recovered to normal, and both her echocardiographic abnormalities and symptoms noticeably improved. Copyright © 2014 Elsevier Inc. All rights reserved.

Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA) Consensus Task Force recommendations for evaluation and management

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Groh, Matthieu; Pagnoux, Christian; Baldini, Chiara; Bel, Elisabeth; Bottero, Paolo; Cottin, Vincent; Dalhoff, Klaus; Dunogué, Bertrand; Gross, Wolfgang; Holle, Julia; Humbert, Marc; Jayne, David; Jennette, J. Charles; Lazor, Romain; Mahr, Alfred; Merkel, Peter A.; Mouthon, Luc; Sinico, Renato Alberto; Specks, Ulrich; Vaglio, Augusto; Wechsler, Michael E.; Cordier, Jean-François; Guillevin, Loïc

2015-01-01

To develop disease-specific recommendations for the diagnosis and management of eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome) (EGPA). The EGPA Consensus Task Force experts comprised 8 pulmonologists, 6 internists, 4 rheumatologists, 3 nephrologists, 1 pathologist and 1

Low Median Nerve Palsy as Initial Manifestation of Churg-Strauss Syndrome.

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Roh, Young Hak; Koh, Young Do; Noh, Jung Ho; Gong, Hyun Sik; Baek, Goo Hyun

2017-06-01

Anterior interosseous nerve (AIN) syndrome is typically characterized by forearm pain and partial or complete dysfunction of the AIN-innervated muscles. Although the exact etiology and pathophysiology of the disorder remain unclear, AIN syndrome is increasingly thought to be an inflammatory condition of the nerve rather than a compressive neuropathy because the symptoms often resolve spontaneously following prolonged observation. However, peripheral neuropathy can be 1 of the first symptoms of systemic vasculitis that needs early systemic immunotherapy to prevent extensive nerve damage. Churg-Strauss syndrome (CSS; eosinophilic granulomatosis with polyangiitis) is 1 type of primary systemic vasculitis that frequently damages the peripheral nervous system. CSS-associated neuropathy usually involves nerves of the lower limb, and few studies have reported on the involvement of the upper limb alone. We report on a rare case of low median nerve palsy as the initial manifestation of CSS. The patient recovered well with early steroid treatment for primary systemic vasculitis. Copyright © 2017 American Society for Surgery of the Hand. Published by Elsevier Inc. All rights reserved.

Acute coronary syndrome caused by coronary vasospasms associated with Churg-Strauss syndrome: effects of betamethasone therapy.

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Suzuki, Yuji; Nishiyama, Osamu; Sakai, Toshiaki; Niiyama, Masanobu; Itoh, Tomonori; Nakamura, Motoyuki

2014-01-01

A 42-year-old woman with a history of aspirin-induced asthma was admitted with severe chest pain. Emergency coronary angiography revealed coronary artery spasms. The administration of vasodilators did not suppress the anginal symptoms, and the differential white blood cell count continued to show eosinophilia. The patient's symptoms of aspirin-induced asthma, eosinophilia and other allergic states led to the diagnosis of Churg-Strauss syndrome (CSS). After starting betamethasone therapy, the eosinophilia and cardiac symptoms rapidly disappeared. Although coronary vasospasms related to CSS are rare, the present case suggests that a differential white blood cell count should be obtained in patients with refractory coronary vasospasms.

A Case of Montelukast-Induced Churg-Strauss Syndrome Associated with Liver Dysfunction

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Keiji Matsui

2011-01-01

Full Text Available A 64-year-old woman was admitted to hospital due to protracted diarrhea and liver dysfunction. The patient was diagnosed as Churg-Strauss syndrome (CSS due to asthma, paranasal sinusitis, hypereosinophilia, and polyneuropathy. There was a history of taking montelukast, a leukotriene receptor antagonist (LTRA, which is thought to have some relationship with CSS. The liver biopsy specimen showed eosinophilic infiltration and centrolobular fatty change. In this paper, we review the relationship between LTRA and CSS. Several lines of evidence suggest that leukotriene plays an important role in maintaining neural tissues. We also review the potential relationship between centrolobular fatty change and pivoxil-containing antibiotics, which was prescribed for sinusitis before admission. Carnitine deficiency induced by pivoxil-containing agents may cause impaired fatty acid oxidation in mitochondria.

Churg-Strauss syndrome presenting as scar reactivation: histopathologic features and an illustration of 'locus minoris resistentiae'.

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Gee, Sarah N; Harris, Anna C; Zimarowski, Mary Jane

2013-05-01

We report a 33-year-old female with cutaneous involvement by Churg-Strauss syndrome confined to surgical scars that were obtained 13 years before. She presented to the emergency department with 2-day history of fever, night sweats, right-sided weakness, hoarseness and worsening asthma symptoms. She was found to have an eosinophilia and two sub-5-mm pulmonary nodules. The patient also reported that the scars on her right thumb, inner wrist and back had been swollen, red and painful for 2 days. Examination revealed tender, erythematous, well-healed edematous scars studded with small skin colored papules. She had no clinical findings that were classic for cutaneous vasculitis. A skin biopsy of a scar revealed perivascular and palisading granulomatous inflammation consisting of histiocytes and neutrophils with leukocytoclasia. Focal vascular injury was identified. Scattered tissue eosinophils were seen. Special stains were negative for infection. Thereafter, she was started on intravenous steroids, at which point the fever, pulmonary and cutaneous symptoms subsided. Although scar sarcoidosis is a well-described phenomenon, granulomatous inflammation and vasculitis seen in Churg-Strauss syndrome exclusively manifesting in well-healed surgical scars highlights the unique features seen in this case and draws attention to the concept of locus minoris resistentiae. This case also highlights how a skin biopsy in the setting of suspected systemic vasculitis can confirm the presence of vasculitis and/or granulomatous inflammation and obviate the need for more invasive, higher risk procedures such as lung biopsy. Copyright © 2013 John Wiley & Sons A/S. Published by Blackwell Publishing Ltd.

ANCA-Negative Churg-Strauss Syndrome Presenting as Acute Multiple Cerebral Infarcts: A Case Report.

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Psychogios, Klearchos; Evmorfiadis, Ilias; Dragomanovits, Spyros; Stavridis, Athanasios; Takis, Konstantinos; Kaklamanis, Loukas; Stathis, Pantelis

2017-03-01

Eosinophilic granulomatosis with polyangiitis (EGPA, previously named Churg-Strauss syndrome) is a form of necrotizing vasculitis occurring in patients with asthma and eosinophilia. Ischemic stroke is a relatively rare complication of the disease. We report a case of a 63-year-old woman with multiple embolic infarcts, hypereosinophilia (for >7 years), and skin rash. Elevated cardiac enzymes and cardiac magnetic resonance imaging were consistent with endomyocarditis. The simultaneous presence of history of asthma, sinusitis, hypereosinophilia, and vasculitis led to the diagnosis of EGPA. This case contributes to the recent debate of the 2 possible presentations of the disease according to the ANCA (antineutrophil cytoplasmic antibodies) status. We furthermore underscore the need for careful differential diagnosis of the "ANCA negative" cases with persistent hypereosinophilia from the idiopathic hypereosinophilic syndrome. Copyright © 2017 National Stroke Association. Published by Elsevier Inc. All rights reserved.

A case of Churg-Strauss syndrome and central retinal artery occlusion with good visual recovery

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Yuki Kamata

2013-01-01

Full Text Available Here we report a case of Churg-Strauss syndrome (CSS and central retinal artery occlusion (CRAO, with good visual recovery. A 58-year-old Japanese man with CSS experienced acute painless loss of vision in his right eye. CRAO was diagnosed by fundoscopic findings (retinal whitening with a cherry-red spot. Steroid pulse therapy (methylprednisolone at 1 g daily for 3 days followed by combined treatment with prednisolone (30 mg/day and cyclophosphamide (150 mg/day was administered; his visual acuity recovered to 20/30 in 1 month, and no recurrence has occurred for 1 year. Steroid pulse therapy may be effective for CRAO in CSS patients.

Cardiogenic Shock: An Unusual Initial Presentation of Churg-Strauss Syndrome

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M. Apirami

2018-01-01

Full Text Available Churg-Strauss syndrome (CSS is a rare autoimmune condition, characterized by necrotizing extravascular eosinophil rich granulomatous inflammation of the tissues and disseminated small-medium sized vessel vasculitis in a patient with bronchial asthma and tissue eosinophilia. Though pulmonary involvement is the predominant feature of CSS, extra pulmonary involvement, in particular, cardiac involvement, denotes an adverse outcome. Here we report a 50-year-old female who presented with cardiogenic shock due to an acute coronary event as the initial manifestation of CSS. A subsequent coronary angiogram revealed normal epicardial coronaries. She was a patient with bronchial asthma and developed vasculitic rash, bilateral sensory motor polyneuropathy, and migratory peripheral lung field shadows in the background of peripheral eosinophilia during the course of the illness. She was diagnosed as having CSS based on ACR criteria and aggressively treated with immunosuppressants according to her Five-Factor Score and has shown prompt response to therapy. This case report adds to the literature another rare initial presentation of CSS to the existing array of its clinical manifestations.

[Cardiac involvement in Churg-Strauss syndrome].

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Brucato, Antonio; Maestroni, Silvia; Masciocco, Gabriella; Ammirati, Enrico; Bonacina, Edgardo; Pedrotti, Patrizia

2015-09-01

Churg-Strauss syndrome, recently renamed eosinophilic granulomatosis with polyangiitis (EGPA), is a rare form of systemic vasculitis, characterized by disseminated necrotizing vasculitis with extravascular granulomas occurring among patients with asthma and tissue eosinophilia. EGPA is classified as a small and medium-sized vessel vasculitis associated with antineutrophil cytoplasmic antibodies (ANCA) and the hypereosinophilic syndrome. Typical clinical features include asthma, sinusitis, transient pulmonary infiltrates and neuropathy. Blood eosinophils are often >1500/µl or more than 10% on the differential leukocyte count. Blood eosinophils should always be tested in unexplained cardiac disorders, and may normalize even after low doses of corticosteroids. ANCA are positive in 40-60% of cases, mainly anti-myeloperoxidase. Heart involvement occurs in approximately 15-60% of EGPA patients, especially those who are ANCA negative. Any cardiac structure can be involved, and patients present with myocarditis, heart failure, pericarditis, arrhythmia, coronary arteritis, valvulopathy, intracavitary cardiac thrombosis. Although cardiovascular involvement is usually an early manifestation, it can also occur later in the course of the disease. A significant proportion of patients with cardiac involvement is asymptomatic. In the absence of symptoms and major ECG abnormalities, cardiac involvement may be detected in nearly 40% of the patients. All patients with EGPA should be studied not only with a detailed history of cardiac symptoms and ECG, but also with echocardiography; if abnormalities are detected, a cardiac magnetic resonance study should be performed. Coronary angiography and endomyocardial biopsy should be reserved to selected cases. Heart involvement carries a poor prognosis and causes 50% of the deaths of these patients. It is often insidious and underestimated. Optimal therapy is therefore important and based on high-dose corticosteroids plus immunosuppressive

Síndroma de Churg-Strauss – a propósito de um caso clínico

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Susana Oliveira

2005-01-01

Full Text Available Resumo: A síndroma de Churg-Strauss (SCS é uma vasculite sistémica pouco comum, que atinge preferencialmente vasos de pequeno calibre, envolve múltiplos órgãos e surge quase sempre associada a asma e eosinofilia.Os autores fazem uma revisão sobre esta doença e discutem o seu diagnóstico diferencial a propósito do caso clínico de uma mulher com diagnóstico de asma brônquica que aos 65 anos inicia quadro compatível com SCS.Rev Port Pneumol 2005; XI (1: 73-83 Abstract: Churg-Strauss syndrome (CSS is a rare systemic vasculitis, that essentially affects medium-sized vessels, involves multiple organs and in the majority of cases is related to asthma and eosinophilia.The authors make a review of this disease and discuss its differential diagnosis presenting the case of a woman with asthma that at the age of 65 is diagnosed with CSS.Rev Port Pneumol 2005; XI (1: 73-83 Palavras-Chave: Churg-Strauss, asma, vasculite, ANCA, Key words: Churg-Strauss, asthma, vasculitis, ANCA

Churg-Strauss Syndrome Leading to Small Bowel Infarction: An Unusual Case of Abdominal Pain in a Young Patient

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Sunil Sookram

1998-01-01

Full Text Available A 33-year-old man with a history of severe asthma presented to the emergency department with a week-long history of severe unrelenting abdominal pain, nausea and decreased appetite. He was admitted to hospital, and routine gastrointestinal investigations were performed, which did not elucidate the cause of his abdominal pain. Exploratory laparotomy demonstrated patchy infarction of the entire small bowel, characteristic of Churg-Strauss syndrome. The patient subsequently underwent 12 separate laparotomies to salvage surviving small bowel. The patient is maintained on total parenteral nutrition.

The linkage between Churg-Strauss syndrome and leukotriene receptor antagonists: fact or fiction?

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McDanel, Deanna L; Muller, Barbara A

2005-01-01

Epidemiologic evidence has shown that the worldwide prevalence of asthma is increasing. The leukotriene receptor antagonists (LTRAs) represent a new class of therapy for asthma. They have been developed in the last decade and play a pivotal steroid-sparing role in treating the inflammatory component of asthma. Consequently, reports of Churg-Strauss syndrome (CSS), a rare form of systemic vasculitis, have been recognized as a potential side effect in individuals with moderate to severe asthma on LTRA therapy. The serious nature of this disorder is worthy of prompt recognition by clinicians and aggressive therapy to avoid the subsequent longstanding effects of vasculitis. To validate the postulated linkage between the LTRAs and CSS, this review comprehensively evaluates reported cases in the literature and supports a pathophysiological relationship between the LTRAs and the development of CSS. PMID:18360552

Leukocytoclastic vasculitis: A window to systemic Churg Strauss syndrome

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Sudhir V Medhekar

2012-01-01

Full Text Available A twenty year old male presented with purpuric lesions with chronic painful ulcers over the lower extremities and a recurrent pruritic rash on the trunk for 10 years. He was diagnosed as idiopathic leukocytoclastic vasculitis (LCV after investigations failed to reveal a systemic association. He was treated with immunosuppressants at each visit with partial remission. In 2004, he was diagnosed with bronchial asthma and allergic rhinitis. In his recent admission, he showed necrotic ulcers on legs and extensive shiny, truncal micropapules. Examination revealed maxillary sinus tenderness and loss of sensation on the medial aspect of the left lower limb. Biopsy of ulcer and the micropapules showed the presence of extravascular eosinophils, while hematological investigations showed peripheral eosinophilia of 18%, raised serum Immunoglobulin E (IgE, Anti nuclear antibody (ANA positivity and negative antineutrophil cytoplasmic antibody (ANCA. Radiography confirmed maxillary sinusitis, nerve conduction studies revealed mononeuritis of the anterior tibial nerve and pulmonary function tests (PFT were normal. Clinical examination and investigations pointed towards the diagnosis of Churg-Strauss syndrome (CSS. This report highlights the development of full-blown CSS over a period of 12 years in a patient initially diagnosed as idiopathic LCV, emphasizing the need for regular follow-up of resistant and recurrent cases of LCV.

Churg-Strauss syndrome with concomitant occurrence of ischemic stroke and relapsing purpura.

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Tanaka, Koji; Koga, Masatoshi; Ishibashi-Ueda, Hatsue; Matsumoto, Chiho; Toyoda, Kazunori

2012-11-01

A 77-year-old woman suffering from chronic bronchial asthma and chronic atrial fibrillation who had had a previous ischemic stroke presented to our emergency unit with gait disturbance. She had new-onset truncal ataxia, right hemiparesis, and right sensory disturbance related to the previous stroke. Her lower legs were slightly swollen and had a reddened appearance. Her medical history included mitral valve replacement because of severe mitral valve regurgitation. Her white blood cell count was 8600/μL, mainly consisting of eosinophils (4480/μL; 52.1%). Serum nonspecific immunoglobulin E was elevated to 1600 IU/mL (normal range Churg-Strauss syndrome (CSS). Skin lesions and eosinophilia disappeared after oral corticosteroid therapy. In this case, cerebellar infarction occurred with purpuric rash despite well-controlled anticoagulation. Patients with CSS may suffer from ischemic stroke when the condition of CSS deteriorates. Copyright © 2012 National Stroke Association. Published by Elsevier Inc. All rights reserved.

Diagnosis of Churg-Strauss Syndrome Presented With Neuroendocrine Carcinoma: A Case Report.

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Park, Dayun; Lee, Ho Jun; Lee, Kwang Hoon; Kwon, Bum Sun; Park, Jin-Woo; Nam, Ki Yeun; Lee, Kyoung Hwan

2017-06-01

Churg-Strauss syndrome (CSS) is a rare systemic vasculitis that affect small and medium-sized blood vessels and is accompanied by asthma, eosinophilia, and peripheral neuropathy. This report describes a case of a 52-year-old man who had a history of sinusitis, asthma, and thymus cancer and who had complained of bilateral lower extremity paresthesia and weakness for a month. Peripheral neuropathy was detected by electrodiagnostic studies. Resection of a mediastinal mass, which was diagnosed as thymic neuroendocrine carcinoma, was performed five months before his visit. After thymectomy, peripheral blood tests revealed a gradual increase in eosinophils. Two months after surgery, he was admitted to the hospital for dyspnea, and nodules of focal consolidation were found in his chest X-ray. One month later, pyoderma occurred in the right shin, and the skin biopsy showed extravascular eosinophilic infiltration. He was diagnosed with CSS after thymectomy, and we report a very rare case of CSS presented with thymic neuroendocrine carcinoma.

Hospital Morbidity Database for Epidemiological Studies on Churg-Strauss Syndrome.

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Kanecki, Krzysztof; Nitsch-Osuch, Aneta; Gorynski, Paweł; Tarka, Patryk; Tyszko, Piotr

2017-01-01

Churg-Strauss syndrome or more accurately eosinophilic granulomatosis with polyangiitis (EGPA) is a small-vessel necrotizing vasculitis with a characteristic late-onset allergic rhinitis and asthma. The use of hospital morbidity database is an important element of the epidemiological analysis of this rare disease. The present study was undertaken to assess the incidence of EGPA and factors related to its epidemiology in Poland; the first analysis of the kind in Poland, enabling a comparison in the European context. This is a retrospective, population-based study using hospital discharge records with EGPA diagnosis, collected for a National Institute of Public Health survey covering the period from 2008 to 2013. The group consisted of 344 patients (206 females and 138 males) with the first-time hospitalization for EGPA. The major findings are that the annual incidence of EGPA in Poland was 1.5 per million (95% confidence intervals: 1.2-1.8), with the point prevalence of 8.8 per million at the end of 2013. A greater incidence of EGPA was observed in the regions with urban predominance. We conclude that discharge records may be a useful element of epidemiological studies on EGPA.

Churg-Strauss syndrome associated with rapid deterioration of left ventricular diastolic dysfunction and conduction disturbance.

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Chin, Jung Yeon; Yi, Jeong Eun; Youn, Ho-Joong

2013-10-01

Cardiac involvement in Churg-Strauss syndrome (CSS) is a major cause of mortality. Here we report a case of a 75-year-old woman with eosinophilic endomyocarditis due to CSS. An electrocardiogram showed intraventricular conduction delay, and echocardiography showed an impaired relaxation pattern and biventricular apical thickening. Magnetic resonance imaging revealed subendocardial delayed enhancement with biventricular apical thrombi. Endomyocardial biopsy showed perivascular eosinophilic infiltration. Despite resolution of the hypereosinophilia after steroid therapy, her left ventricular (LV) diastolic function worsened into a restrictive pattern and she died with a ventricular escape rhythm on her 14th day in the hospital. This case is unusual in that there was rapid progression of the LV diastolic dysfunction and conduction disturbance due to CSS. © 2013, Wiley Periodicals, Inc.

Experience in managing patients with Churg-Strauss syndrome.

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A. A. Khanyukov

2018-04-01

Full Text Available Our goal was to analyze the possibilities of impro­ving the diagnostics of CSS and to improve the effectiveness of treatment according to the existing literature and our own experience of long-term care for patients with eosinophilic granulomatosis with polyangiitis or Churg-Strauss syndrome (CSS. The medical histories of three female patients aged 26 to 46-years and a 20-year-old male patient were considered. The duration of the disease before the established diagnosis was 5-17 years. Anamnesis and medical documents analysis showed a typical CSS debut in the form of allergic rhinitis, nasal polyps, which were recurrent after polypectomy, and respiratory disorders, which were regarded as bronchitis or bronchial asthma – corresponding to the first phase, also called the prodromal or allergic stage of CSS. The prodromal period lasts up to 10 years or more and is characterized by various allergic manifestations, more often –  pollinosis or bronchial asthma, that is difficult to control. But CSS can be suspected because of low effectiveness of the therapy with inhaled steroids, lack of effect of antibiotics and eosinophilia more than 10% that occurs periodically. Even in the third stage of CSS in systemic manifestations of vasculitis and severe secondary lesions of organs and tissues with functional impairment, constant intake of maintenance doses of corticosteroids and cytostatics allows to achieve stabilization of the process in patients with CSS.

[A case of Churg-Strauss syndrome with subarachnoid hemorrhage].

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Ito, Miiko; Kato, Naoki; Su, Ching-Chan; Kayama, Takamasa

2014-03-01

Churg-Strauss syndrome (CSS) is a vasculitis syndromes and is only rarely complicated by subarachnoid hemorrhage. In the current report, we describe a case of CSS with subarachnoid hemorrhage, which showed a favorable outcome following conservative treatment. A 68-year-old man with CSS on maintenance steroid therapy underwent MRI/A during tinnitus aggravation, and showed dilation of the left middle cerebral artery and stenosis of the peripheral area of the right vertebral artery. After 2 months, he presented sudden pain in the occipitocervical area, and CT revealed subarachnoid hemorrhage. Intracranial 3D CT-A and MRI/A showed the development of a protrusion at the base of the left anterior cerebral artery. Although both findings suggested cerebral artery dissection, the source of hemorrhage could not be identified. The 2009 Japanese Guidelines for the Management of Stroke recommends early diagnosis and treatment of hemorrhagic cerebral artery dissection because of the high risk of re-bleeding. However, considering the risks of vasculitis aggravation, development of systemic complications, and recurrence, conservative treatment was selected. In addition, owing to the risk of complications associated with the frequent use of iodinated contrast agents and angiography procedures, patient was followed up using MRI. His course was favorable, and he was discharged despite mild right abducens paralysis. When patients with hemorrhagic cerebral artery dissection have a history of allergic diseases, CCS should be considered; conservative treatment consisting of rest, strict blood pressure control, and steroid therapy may be the most appropriate option for certain patients.

Serum biomarkers are similar in Churg-Strauss syndrome and hypereosinophilic syndrome.

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Khoury, P; Zagallo, P; Talar-Williams, C; Santos, C S; Dinerman, E; Holland, N C; Klion, A D

2012-09-01

Churg-Strauss syndrome (CSS) and hypereosinophilic syndrome (HES) overlap considerably in clinical presentation. A reliable means of distinguishing between these groups of patients is needed, especially in the setting of glucocorticoid therapy. A retrospective chart review of 276 adult subjects referred for evaluation of eosinophilia > 1500/μl was performed, and subjects with a documented secondary cause of eosinophilia or a PDGFR -positive myeloproliferative neoplasm were excluded. The remaining subjects were assessed for the presence of American College of Rheumatology (ACR) criteria. Laboratory and clinical parameters were compared between subjects with biopsy-proven vasculitis (CSS; n = 8), ≥4 ACR criteria (probable CSS; n = 21), HES with asthma and/or sinusitis without other CSS-defining criteria (HESwAS; n = 20), HES without asthma or sinusitis (HES; n = 18), and normal controls (n = 8). Serum biomarkers reported to be associated with CSS were measured using standard techniques. There were no differences between the subjects with definite or probable CSS or HES with respect to age, gender, or maintenance steroid dose. Serum CCL17, IL-8, and eotaxin levels were significantly increased in eosinophilic subjects as compared to normal controls, but were similar between the eosinophilic groups. Serum CCL17 correlated with eosinophil count (P < 0.0001, r = 0.73), but not with prednisone dose. In patients with a history of asthma and sinusitis, distinguishing between ANCA-negative CSS and PDGFR-negative HES is difficult because of significant overlap in clinical presentation and biomarker profiles. Published 2012. This article is a U.S. Government work and is in the public domain in the USA.

Combined Churg-Strauss syndrome and allergic bronchopulmonary aspergillosis - case report and review of the literature.

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Ren, Shaohua

2013-01-01

A rare case of combined Churg-Strauss syndrome (CSS) and allergic bronchopulmonary aspergillosis (ABPA) was presented. A 41-year-old woman was diagnosed with CSS based upon asthma, eosinophilia (23%), chest radiographic findings, paranasal sinusitis, peripheral neuropathy and positive p- anti-neutrophil cytoplasmic antibodies (pANCA). The diagnosis of ABPA was established on the pathological findings of allegic mucin impaction and fungal hyphae on lung biopsy. It was further proved by positive serum IgE and IgG antibodies specific to afumigatus. The clinical investigation features were reviewed in the patients with combined CSS and ABPA. All patients had the time sequence of the development of CSS after ABPA uniformly, suggesting immunopathogenesis involving the emergence of CSS. The role of lung biopsy in the diagnosis of the condition was emphasized. © 2012 Blackwell Publishing Ltd.

Clinical features and prognostic factors of Churg-Strauss syndrome.

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Kim, Mi-Yeong; Sohn, Kyoung-Hee; Song, Woo-Jung; Park, Heung-Woo; Cho, Sang-Heon; Min, Kyung-Up; Kang, Hye-Ryun

2014-01-01

Churg-Strauss syndrome (CSS) is a rare systemic necrotizing small-vessel vasculitis, with accompanying bronchial asthma, eosinophilia, and eosinophilic infiltration of various tissues. The purposes of our study were to characterize the clinical features of CSS and to identify factors associated with CSS prognosis in Koreans. Medical records were reviewed retrospectively for all physician-diagnosed CSS patients in the Seoul National University Hospital between January 1990 and March 2011. Data from 52 CSS patients were analyzed. The respiratory tract was the most commonly involved organ (90.4%). Renal involvement was less frequent in antineutrophilic cytoplasmic antibody (ANCA)(-) patients than in ANCA(+) patients (p = 0.048). Clinical remission occurred in 95.3% of patients, but 16.3% of them relapsed. Patients who maintained remission for more than 6 months were relatively older (median, 51 years) at diagnosis (p = 0.004), had been diagnosed in earlier stages (p = 0.027), showed more frequent respiratory involvement (p = 0.024) and generalized symptoms (p = 0.039), and showed less frequent cutaneous involvement (p = 0.030) than those who did not achieve persistent (> 6 months) remission. Patients who achieved persistent remission also showed higher C-reactive protein (CRP) levels (p = 0.031) than those who did not. ANCA(-) CSS patients showed less frequent renal involvement. Characteristics of good responders were older age, diagnosis at earlier stages, less cutaneous involvement, more respiratory involvement, high CRP values, and more generalized symptoms.

Churg-Strauss Syndrome as an Unusual Aetiology of Stroke with Haemorrhagic Transformation in a Patient with No Cardiovascular Risk Factors

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Tiina Sairanen

2011-01-01

Full Text Available Background: We present here a case of haemorrhagic brain infarction in a middle-aged and physically active male, who had never smoked. This case report aims to remind the internist and neurologist to bear in mind unusual aetiologies of brain infarcts in patients without classical cardiovascular risk factors. Case Description: A 49-year-old male with pulmonary asthma and a prior history of nasal polyps had a wake-up stroke with left-sided symptoms and speech disturbance. A head MRI and MR angiography revealed a recent haemorrhagic infarct in the right putamen and corona radiata. The left hemiparesis progressed to sensory-motor hemiplegia on the 4th day. In the head CT, it was shown that the haemorrhagic infarct had progressed to a large haematoma. A pansinusitis was also diagnosed. The aetiological investigations revealed a minor atrial septal defect (ASD with shunting and a heterozygotic clotting factor V R506Q mutation. A remarkable blood eosinophilia of 9.80 E9/l (42% together with fever, sinusitis, wide-spread bilateral nodular pulmonary infiltrates that did not respond to wide-spectrum antimicrobial treatment, positive anti-neutrophilic cytoplasmic antibodies, a high myeloperoxidase antibody level and slightly positive anti-proteinase 3 antibodies suggested the diagnosis of Churg-Strauss syndrome. These inflammatory symptoms and findings promptly responded to treatment with corticosteroids and cyclophosphamide. Conclusions: Even after the concomitant findings of the low risk factors, i.e. small ASD and heterozygotic clotting factor mutation, continued search for the final aetiology of stroke revealed Churg-Strauss syndrome, which was the key to the treatment.

Churg-Strauss Syndrome as an Unusual Aetiology of Stroke with Haemorrhagic Transformation in a Patient with No Cardiovascular Risk Factors

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Sairanen, Tiina; Kanerva, Mari; Valanne, Leena; Lyytinen, Jukka; Pekkonen, Eero

2011-01-01

Background We present here a case of haemorrhagic brain infarction in a middle-aged and physically active male, who had never smoked. This case report aims to remind the internist and neurologist to bear in mind unusual aetiologies of brain infarcts in patients without classical cardiovascular risk factors. Case Description A 49-year-old male with pulmonary asthma and a prior history of nasal polyps had a wake-up stroke with left-sided symptoms and speech disturbance. A head MRI and MR angiography revealed a recent haemorrhagic infarct in the right putamen and corona radiata. The left hemiparesis progressed to sensory-motor hemiplegia on the 4th day. In the head CT, it was shown that the haemorrhagic infarct had progressed to a large haematoma. A pansinusitis was also diagnosed. The aetiological investigations revealed a minor atrial septal defect (ASD) with shunting and a heterozygotic clotting factor V R506Q mutation. A remarkable blood eosinophilia of 9.80 E9/l (42%) together with fever, sinusitis, wide-spread bilateral nodular pulmonary infiltrates that did not respond to wide-spectrum antimicrobial treatment, positive anti-neutrophilic cytoplasmic antibodies, a high myeloperoxidase antibody level and slightly positive anti-proteinase 3 antibodies suggested the diagnosis of Churg-Strauss syndrome. These inflammatory symptoms and findings promptly responded to treatment with corticosteroids and cyclophosphamide. Conclusions Even after the concomitant findings of the low risk factors, i.e. small ASD and heterozygotic clotting factor mutation, continued search for the final aetiology of stroke revealed Churg-Strauss syndrome, which was the key to the treatment. PMID:21468361

A case report for fatal Churg-Strauss syndrome complications: first reported death due to rapid progression of prominently huge hepatic capsular hematoma and multi-system organ hemorrhage

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Qian, Jiejing; Tong, Hongyan; Chen, Feifei; Mai, Wenyuan; Lou, Yinjun; Jin, Jie

2014-01-01

Churg-Strauss syndrome (CSS) is a rare disease that has an extremely low incidence rate. CSS prognosis is good, in general; and there are no reports of multiple-organ hemorrhage in CSS. We report a unique case of CSS, wherein, an elderly man experienced multiple organ hemorrhage -- a particularly huge hematoma under the capsule of the liver and poor prognosis. PMID:25419420

Churg-Strauss Syndrome with Cardiac Involvement: A Case Report with CT and MRI Findings

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Lim, Seong Joo; Cho, Young Jun; Kim, Keum; Hwang, Cheol Mok; Kim, Dae Ho [Dept. of Radiology, Konyang University College of Medicine, Daegu (Korea, Republic of); Choi, Eu Gene [Dept. of Internal Medicine, Konyang University College of Medicine, Daegu (Korea, Republic of)

2012-02-15

This is a case report of Churg-Strauss Syndrome (CSS) associated with cardiac involvement which is demonstrated in chest CT and cardiac MRI (CMR) without specific cardiac symptoms. A 32-year-old woman had a 3-year history of bronchial asthma, chronic sinusitis, and otitis media. The patient had various typical findings of CSS. The patient had no specific cardiac symptoms or signs such as chest pain, palpitations, syncope, or murmur, but she had diffuse low attenuation lesions in the inner wall of the left ventricle (LV) in contrast-enhanced CT. This corresponded to the area of subendocardial hyperenhancement in delayed contrast-enhanced CMR images. She was treated with steroids for 2 months. Follow-up delayed contrast-enhanced CMR of the LV showed a decrease in the size of the subendocardial enhancement area, and she had no symptoms. Therefore, the radiologist and clinician both should pay careful attention to observe possible cardiac involvement in case of CSS.

Churg-Strauss Syndrome with Cardiac Involvement: A Case Report with CT and MRI Findings

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Lim, Seong Joo; Cho, Young Jun; Kim, Keum; Hwang, Cheol Mok; Kim, Dae Ho; Choi, Eu Gene

2012-01-01

This is a case report of Churg-Strauss Syndrome (CSS) associated with cardiac involvement which is demonstrated in chest CT and cardiac MRI (CMR) without specific cardiac symptoms. A 32-year-old woman had a 3-year history of bronchial asthma, chronic sinusitis, and otitis media. The patient had various typical findings of CSS. The patient had no specific cardiac symptoms or signs such as chest pain, palpitations, syncope, or murmur, but she had diffuse low attenuation lesions in the inner wall of the left ventricle (LV) in contrast-enhanced CT. This corresponded to the area of subendocardial hyperenhancement in delayed contrast-enhanced CMR images. She was treated with steroids for 2 months. Follow-up delayed contrast-enhanced CMR of the LV showed a decrease in the size of the subendocardial enhancement area, and she had no symptoms. Therefore, the radiologist and clinician both should pay careful attention to observe possible cardiac involvement in case of CSS.

A Case Report of Angina Bullosa Haemorrhagica (ABH in Patient with Churg Strauss Syndrome

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M. Koopaie

2015-11-01

Full Text Available Bullosa Haemorrhagica (ABH is a rare oral blood blisters disorder. This recurrent disorder is restricted to oral mucosa and its difference with other oral vesiculobullos disorders is that blood vesicles are formed after minor trauma and in the absence of any blood dyscrasia, vesiculobullos disease and systemic disorder. This blood blisters are without pain, with color of dark red and full of bloods that rapidly enlarge and rapidly burst. The incidence of Angina Bullosa Haemorrhagica is unknown. Diagnosis of this disorder and differentiation from vesiculobullos disease like pemphigus and pemphiguid is important for patient's health. In this report we introduce a 55 years old woman with a 4­years history of oral blood blisters. These vesicles were in lateral border and ventral surface of tongue and on the ferenum. In medical history the patient had churg strauss syndrome and using inhalation corticosteroid for control of asthmatic attacks (An etiologic factor for appearing or aggravation of the disease.

Churg-Strauss syndrome and persistent heart failure: active disease or damage?

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Lin, Yih Chang; Oliveira, Guilherme H M; Villa-Forte, Alexandra

2013-10-01

Churg-Strauss syndrome (CSS) is a rare small-vessel vasculitis typically associated with adult-onset asthma, peripheral and tissue hypereosinophilia, migratory pulmonary infiltrates, upper respiratory tract symptoms, and clinical evidence of systemic vasculitis. Cardiac involvement is a well-recognized complication with an estimated prevalence of 60%. Heart disease is associated with poor prognosis, accounting for almost 50% mortality in CSS. We present a case of a 48-year-old woman with CSS complicated by congestive heart failure with left ventricular ejection fraction of 25%, who was initially treated with long course of high-dose steroids without any clinical or echocardiographic improvement. She was referred to our hospital 1 year later and was initiated with cyclophosphamide 2 mg/kg per day and prednisone 60 mg/d followed by slow taper. Subsequently, the patient had remarkable improvement. Patient was then transitioned to azathioprine for 1.5 years with sustained disease remission. It may be difficult to determine myocardial disease activity status versus tissue damage in CSS with prolonged duration of heart failure symptoms. This is the first case report demonstrating that CSS cardiac disease may remain active despite 1 year of corticosteroid therapy, and significant improvement or remission can still be achieved by administering more aggressive cytotoxic immunosuppressive therapy.

Churg-Strauss Syndrome in a Seven-year-old Boy: A Case Report

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Abdolreza Malek

2018-06-01

Full Text Available Churg-Strauss syndrome (CSS is a type of vasculitis of small-to-medium sized vessels. This syndrome is known by a history of bronchial asthma with systemic necrotizing vasculitis and peripheral blood hypereosinophilia. It is currently called eosinophilic granulomatosis with polyangiitis (EGPA. This disease affects both genders and all age groups, but it is very rare among children. CSS diagnosis is based on clinical findings such as asthma, eosinophilia, rhinosinusitis, and signs of vasculitis in major organs. In cases where steroids alone or in combination with other immunosuppressive agents are used as treatment, the outcome and long-term survival are usually satisfying. In comparison with other types of systemic vasculitis, the mortality rate of this syndrome is low. In this study, we present the case of a 7-year-old boy with poorly controlled bronchial asthma since three years of age. This case had developed purpuric skin lesions, sinusitis, arthritis, and weakness of the limbs with symptoms of mononeuritis multiplex at the age of seven. After being admitted to our hospital, a series of studies, including complete blood count-diff, chest X-ray, paranasal sinus radiography, brain magnetic resonance imaging, nerve conduction study, spirometry, and serological tests for autoantibodies, were performed and he was diagnosed with CSS. Thereafter, he received regular corticosteroid therapy in combination with methotrexate, and his symptoms were generally well-controlled with the beginning of the treatment. The clinical characteristics, diagnosis, and management of CSS in children are also reviewed in this study.

Sequential rituximab and omalizumab for the treatment of eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome).

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Aguirre-Valencia, David; Posso-Osorio, Iván; Bravo, Juan-Carlos; Bonilla-Abadía, Fabio; Tobón, Gabriel J; Cañas, Carlos A

2017-09-01

Eosinophilic granulomatosis with polyangiitis (EGPA), formerly known as Churg-Strauss syndrome (CSS), is a small vessel vasculitis associated with eosinophilia and asthma. Clinical manifestations commonly seen in patients presenting with EGPA range from upper airway and lung involvement to neurological, cardiac, cutaneous, and renal manifestations. Treatment for severe presentations includes steroids, cyclophosphamide, plasmapheresis, and recently, rituximab. Rituximab is associated with a good response in the treatment of vasculitis, but a variable response for the control of allergic symptoms. Here, we report a 16-year-old female patient with severe EGPA (gastrointestinal and cutaneous vasculitis, rhinitis and asthma) refractory to conventional treatment. She was treated with rituximab, which enabled rapid control of the vasculitis component of the disease, but there was no response to rhinitis and asthma. Additionally, she developed severe bronchospasm during rituximab infusion. Sequential rituximab and omalizumab were initiated, leading to remission of all manifestations of vasculitis, rhinitis, and asthma, in addition to bronchospasm related to rituximab infusion.

A Case Report of Churg-Strauss Syndrome Presenting With Cardiogenic Shock Treated With Extracorporeal Membrane Oxygenation.

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Cui, Na; Su, Longxiang; Wang, Hao; Long, Yun; Pang, Cheng; Yang, Fei; Liu, Dawei

2015-10-01

Churg-Strauss Syndrome (CSS) complicated with cardiogenic shock is rare. Few case reports have described successful treatment of this rare disease. However, no one has reported on the application of mechanical life support with extracorporeal membrane oxygenation (ECMO) to treat this life-threatening disease.A 36-year-old female with limb numbness for >10 days, chest tightness for 2 days, and worsening dyspnea for 5 h presented in the emergency room. Vital signs showed a low blood pressure (104/60 mm Hg), increased heart rate (158 bpm), and respiration rate (28 bpm). Laboratory tests revealed that eosinophil was significantly increased (WBC: 34.46 × 10/L, neutrophil: 7.56 × 10/L[21.9%], eosinophil: 23.84 × 10/L[69.2%]), and serum myocardial enzymes was abnormal (CK 1049U/L, CKMB-mass 145.1 μg/L, cTnI 16.24 μg/L). Myocardial injury (tachycardia with ST elevation) and poor heart function (LVEF 31%) were found by electrocardiogram and transthoracic echocardiography. On the next day, cardiogenic shock had been developed as demonstrated by deteriorating the perfusion index.Churg-Strauss Syndrome with cardiogenic shock.A series of conservative therapy with drugs such as corticosteroids, anticoagulant, antiplatelet, nitrates, calcium antagonists, inotrope, and vasopressors were initiated on the day of admission. The treatment was ineffective and a cardiogenic shock developed on the next day. Thus, ECMO was initiated immediately to stabilize circulation and perfusion. At the same time, high-dose corticosteroids combined with immunosuppressive therapy were continuously used.Symptoms of cardiogenic shock were gradually improved after ECMO treatment. Elevated values of cardiac enzymes were decreased and the dose of vasoactive drugs was reduced. Extracorporeal membrane oxygenation was discontinued after 8 days, and the patient was eventually weaned off the ventilator. The patient was discharged after 40 days treatment.Once a CSS develops into a

Cardiac tamponade leading to the diagnosis of eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome): a case report and review of the literature.

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Yano, Toshiyuki; Ishimura, Shutaro; Furukawa, Tetsuaki; Koyama, Masayuki; Tanaka, Marenao; Shimoshige, Shinya; Hashimoto, Akiyoshi; Miura, Tetsuji

2015-11-01

Eosinophilic granulomatosis with polyangiitis (EGPA), which was previously called Churg-Strauss syndrome, is a necrotizing systemic vasculitis of unknown cause accompanied by prominent eosinophilia. Cardiovascular complications, including eosinophilic myocarditis, are a major cause of mortality in this disorder. Acute pericarditis with slight pericardial effusion is a typical manifestation in EGPA, though hemodynamically significant pericardial effusion has been reported in a few cases. We report a case that initially presented with isolated cardiac tamponade, which was followed by systemic manifestations of EGPA over 3 weeks. Including the present case, previous EGPA cases with cardiac tamponade are reviewed to delineate its clinical characteristics.

Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss Syndrome) Complicated by Perforation of the Small Intestine and Cholecystitis.

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Ohnuki, Yoichi; Moriya, Yusuke; Yutani, Sachiko; Mizuma, Atsushi; Nakayama, Taira; Ohnuki, Yuko; Uda, Shuji; Inomoto, Chie; Yamamoto, Soichiro; Nakamura, Naoya; Takizawa, Shunya

2018-03-01

We report a case of eosinophilic granulomatosis with polyangiitis (EGPA; formerly known as Churg-Strauss syndrome) complicated by perforation of the small intestine and necrotizing cholecystitis. A 69-year-old man with a history of bronchial asthma was admitted with mononeuritis multiplex. The laboratory findings included remarkable eosinophilia. He was treated with corticosteroids and his laboratory indices showed improvement; however, his functional deficits remained. His neuropathy gradually improved after the addition of intravenous immunoglobulin (IVIG). He was subsequently treated with oral prednisolone (40 mg/day) as maintenance therapy. Within a month after finishing IVIG, he developed perforation of the small intestine and necrotizing cholecystitis. Intestinal perforation has often been reported as a gastrointestinal complication of EGPA. In contrast, cholecystitis is a rare complication. We report this case because the manifestation of more than one complication is extremely rare. Gastrointestinal symptoms may be a complication of EGPA itself and/or immunosuppressive treatment.

A case report and literature review of Churg-Strauss syndrome presenting with myocarditis.

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Qiao, Lu; Gao, Dengfeng

2016-12-01

Churg-Strauss syndrome (CSS) is a multisystem disorder characterized by asthma, prominent peripheral blood eosinophilia, and vasculitis signs. Here we report a case of CSS presenting with acute myocarditis and heart failure and review the literature on CSS with cardiac involvement. A 59-year-old man with general fatigue, numbness of limbs, and a 2-year history of asthma was admitted to the department of orthopedics. Eosinophilia, history of asthma, lung infiltrates, peripheral neurological damage, and myocarditis suggested the diagnosis of CSS. Transthoracic echocardiography revealed a dilated hypokinetic left ventricle (left ventricular ejection fraction ∼40%) with mild segmental abnormalities in the septal and apical segments. By reviewing the present case reports, we concluded that (1) the younger age of CSS, the greater occurrence rate of complicating myocarditis and the poorer prognosis; (2) female CSS patients are older than male patients; (3) patients with cardiac involvement usually have a history of severe asthma; (4) markedly increased eosinophil count suggests a potential diagnosis of CSS (when the count increases to 20% of white blood cell counts or 8.1 × 109/L, eosinophils start to infiltrate into myocardium); and (5) negative ANCA status is associated with heart disease in CSS.

Juvenile Churg-Strauss Syndrome as an Etiology of Myocarditis and Ischemic Stroke in Adolescents; a Case Report

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Moradinejad, Mohammad-Hassan; Rezaei, Amir; Ziaee, Vahid

2011-01-01

Background Churg-Strauss syndrome (CSS), a systemic vasculitis accompanied by asthma and eosinophilia, almost invariably affects the lung and is frequently associated with cutaneous involvement. It rarely has cardiac involvement. We report an unusual case of CSS with myocardial involvement and stroke. Case Presentation A 16-year old female suffered of allergic asthma for 4 years. She was under treatment with oral prednisolone and seretide inhalation. After CSS diagnosis, she developed paroxysmal atrial tachycardia. Serum levels of Troponin I and Troponin T were increased indicating massive myocardial damage probably due to myocarditis. After 5 months she developed acute hemiparesis without any evidence of ischemic or hemorrhagic event. She was treated with IVIg, intravenous pulses of methylprednisone and cyclophosphamide for each complication. Conclusion Myocarditis and stroke may also complicate CSS which should be taken in consideration for better management. PMID:23056844

A pseudoleukemic blood differentiation in a 13-year-old child: an extraordinary presentation of Churg-Strauss syndrome.

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Mutsaers, E R; Witteveen, R; van den Bosch-Ruis, W; Kuijpers, T W; van Houten, M A; van den Berg, J M

2013-03-01

Churg-Strauss syndrome (CSS) is a rare systemic vasculitis of the small- and medium-size vessels. It is mostly seen in elderly patients presenting as de novo asthma, eosinophilia, and vasculitic organ involvement. In childhood, CSS is extremely rare. The course of pediatric CSS is usually severe and often lethal. We present a case of a 13-year-old girl with a short history of asthma, marked eosinophilia, and multiorgan involvement. The extremely high level of blood eosinophilic granulocytes (51.6 × 10(9)/L) prompted a workup for eosinophilic leukemia before the diagnosis CSS could be made. Subsequently, the disease was successfully treated. This case report shows a classical case of childhood CSS, remarkable because of the presence of extreme hypereosinophilia. It underlines the importance of CSS as a life-threatening cause of hypereosinophilia in children.

[A case of Churg-Strauss syndrome with short duration from the onset of asthma to diagnosis of vasculitis].

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Fuse, Yoshikazu

2013-01-01

A 68-year-old woman was hospitalized because of bronchial asthma and a high myeloperoxidase antineutrophil cytoplasmic antibody (MPO-ANCA) level. She had suffered from rhinitis from one year before hospitalization, body weight loss from three months before, and asthma from one month before. On admission, she complained of dyspnea and body weight loss of over 6 kg. On laboratory tests, high MPO-ANCA and urinary abnormalities were found. On the next day, a renal biopsy was performed and histology showed necrotizing vasculitis with cellular crescents. Churg-Strauss syndrome (CSS) was diagnosed on the basis of the clinical course and histological findings. Prednisolone therapy induced rapid symptom remission, which was achieved within one month from the onset of asthma to the diagnosis of CSS. Early diagnosis and early care led to a good prognosis.

Churg-Strauss vasculitis and idiopathic hypereosinophyl syndrome: role of molecular biology in the differential diagnosis of hypereosinophyl syndrome

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A. d'Ascanio

2011-09-01

Full Text Available Objective: Hypereosinophilic syndromes are a heterogeneous group of uncommon disorders characterized by the presence of marked peripheral blood eosinophilia, tissue eosinophilia, or both, resulting in a wide variety of clinical manifestations, often without an identifiable cause. Churg-Strauss syndrome is a systemic vasculitis characterized by prominent peripheral eosinophilia, asthma and systemic involvement. The presence of mild to severe eosinophilia and systemic involvement raise the search of many trigger factor that need to be ruled out. Distinguishing CSS from idiopathic hypereosinophilic syndrome may be particularly challenging, especially in ANCA negative patients. Methods: The aim of the present study was to present a small case series of patients referred to a Rheumatology Unit for mild to severe eosinophilia and signs and symptoms of systemic involvement and to outline the clinical significance of molecular biology in the work-up of hypereosinophilia. Results: Eleven patients with moderate to severe peripheral eosinophylia, were referred to our Unit from 1996 to 2007. Female to male ratio was 7/4, mean age 40.54 (range 22-75. Three out of eleven patients resulted positive for molecular biology. The diagnosis of idiopathic hypereosinophylia was confirmed in one out of three on the basis of the clinical picture and bone marrow biopsy. Conclusions: Molecular biology may be useful in the screening and in the follow-up of a new hypereosinophylic patient.

Pulmonary involvement in Churg-Strauss syndrome: an analysis of CT, clinical, and pathologic findings

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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.)

Pulmonary involvement in Churg-Strauss syndrome: an analysis of CT, clinical, and pathologic findings

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Kim, Yoon Kyung; Lee, Kyung Soo; Chong, Semin; Chung, Myung Jin; Yi, Chin A.; Kim, Ha Young; Chung, Man Pyo; Han, Joungho

2007-01-01

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.)

Multiple Thromboembolic Cerebral Infarctions from the Aorta in a Patient with Churg-Strauss Syndrome.

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Okada, Hideo

2017-02-01

Ischemic stroke is a rare complication of Churg-Strauss syndrome (CSS) and its pathogenesis has not been well clarified yet. We report a case of cerebral infarction in a patient with CSS due to embolism from a thrombus on the wall of the aorta. A 39-year-old man had multiple cerebral infarctions with symptoms of mild left hemiparesis and reduced vision. He was clinically diagnosed to have CSS based on remarkable eosinophilia, history of asthma, sinusitis, pulmonary infiltrates, and histologically proven extravascular eosinophilic infiltrates in the specimen of gastric mucosa. Cerebral angiography did not show any stenotic lesions in cerebral arteries. A thrombus was detected on the wall of the aorta by transesophageal echocardiography, which was considered as the source of embolism. The thrombus resolved on follow-up examination 3 months after the onset of the stroke. This is the first case report on cerebral infarction caused by aortogenic thromboembolism in a CSS patient. Other than cerebral vasculitis, embolism from cardiovascular system, including the wall of the aorta, is a possible cause of cerebral infarctions in a CSS patient. Copyright © 2017 National Stroke Association. Published by Elsevier Inc. All rights reserved.

[Anesthetic management for surgery of giant coronary aneurysms complicated with Churg-Strauss syndrome].

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Kido, Koji; Tokuda, Rui; Suzuki, Tomofumi; Hanashiro, Ako; Kobashigawa, Teruyo; Mayama, Takashi; Kamikawa, Michie

2014-04-01

Few cases of Churg-Strauss syndrome (CSS) complicated by giant coronary aneurysms (CAs)have been reported thus far. We report a case of CSS in a 60-year-old man who underwent surgery for giant CAs, and was managed with anesthetics. The patient developed acute myocardial infarction, and was diagnosed with giant CAs in the right coronary artery (RCA, 11 cm) and circumflex artery (3 cm). The CA in RCA was communicating with the right ventricle. He had a history of pericardiectomy for pericarditis caused by the CSS and developed thrombocytopenia due to consumptive coagulopathy within the CAs. An operation, including ligation and excision of the CAs, and coronary artery bypass grafting was performed under general anesthesia and cardiopulmonary bypass. There was massive hemorrhage followed by hemodynamic instability while detaching the tight pericardial adhesion and fragile surface of the CAs. Massive transfusion was required along with inotropes administration and intraaortic balloon support. In this case, determination of the appropriate surgical timing was difficult because symptoms of the CSS became worse followed by rapid enlargement of the CAs, myocardial infarction, and thrombocytopenia. Steroids were administered for treating CSS, and the blood transfusion was sufficient. However, it was difficult to control the hemorrhage and maintain hemodynamic stability.

Optimal therapy and prospects for new medicines in eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome).

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Pagnoux, Christian; Groh, Matthieu

2016-10-01

The prevalence of eosinophilic granulomatosis with polyangiitis (EGPA; previously known as Churg-Strauss syndrome) is lower than that of other antineutrophil cytoplasm antibody (ANCA)-associated vasculitides (AAV's), and only a few randomized controlled trials have been conducted for this rare disease. However, recent international efforts have helped delineate the best treatment approach. At present, EGPA conventional therapy is by default similar to that of other AAVs. Limited, non-severe EGPA can initially be treated with glucocorticoids (GCs) alone. Patients with life-threatening manifestations and/or major organ involvement must receive a combination of GCs and an immunosuppressant, mainly cyclophosphamide. Remission can be achieved in >85% of patients with these first-line treatments, but vasculitis relapses occur in more than one-third of patients, and about 85% cannot stop GC treatment because of GC-dependent asthma and/or ENT manifestations. A few biologic agents, including rituximab or mepolizumab, are now under investigation after interesting preliminary results. Expert commentary: Treatment for EGPA still has several unmet needs. Several biologic agents are now under investigation in randomized controlled trials, but a few others should be considered soon. Their benefit should be demonstrated for devising more EGPA-tailored therapeutic strategies (ideally GC-free).

Not All Coughs Are Asthma or Allergies: Churg-Strauss Syndrome.

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Bodoutchian, Ani A; Jain, Hitender; Velez, Tania

2016-09-01

Cardiomyopathy in patients with Churg-Strauss syndrome (CSS) carries a poor prognosis, with a high 5-year mortality rate, and requires treatment with immunosuppressive therapy. There is no single pathognomonic test or clinical finding for diagnosing CSS; instead, it is based upon meeting four of six criteria. A 47-year-old woman with a 6-month medical history of "asthma" presented to our hospital with a 1-month history or dyspnea on exertion. She denied any chest pain, cough, fever, orthopnea, or leg swelling. She was afebrile and normotensive, and her physical examination was unremarkable. Her white blood cell count was 23,000/mm(3) with 23% eosinophils, and her troponin T level was 1.08 ​Ng/ml. Extensive work-up revealed CSS. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: Emergency physicians should be aware of unusual cases because not every patient that walks into an emergency department has a simple case of "asthma" or "allergies." Our patient had been diagnosed with asthma for 6 months before her symptoms had progressed to a point that prompted her to seek medical attention in a hospital. Emergency physicians are in a unique position to identify patients who present with recurrent complaints of asthma-especially late-onset asthma, which gradually worsens and is refractory to usual treatment. A complete blood cell count with a differential will prove valuable. Assessments of a patient's C-reactive protein level and erythrocyte sedimentation rate are inexpensive and check for signs of inflammation, although they are nonspecific. A chest radiograph or computed tomography scan of the chest or sinuses in some patients can also prove to be of value. Prompt recognition and treatment with steroids is imperative to ablate vasculitis tissue damage because this can improve the outcome. Copyright © 2016 Elsevier Inc. All rights reserved.

Churg-Strauss Syndrome: The Clinical Features and Long-term Follow-up of 17 Patients

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Oh, Mi-Jung; Lee, Jin-Young; Kwon, Nam-Hee

2006-01-01

Churg-Strauss syndrome (CSS) is a rare multi-system vasculitis; some cases have been reported in Korea. The aim of this study is to describe the clinical features, treatment outcome, and long-term follow-up of CSS from a single Korean medical center. Between 1995 and 2004, seventeen patients were diagnosed with CSS at the Department of Medicine of the Samsung Medical Center, Sungkyunkwan University School of Medicine. The diagnosis of CSS is based on the classification criteria of the American Collage of Rheumatology. All patients had asthma. As in other case series, the lung, peripheral nervous system, and skin were the most commonly involved organs. During the active stage of the disease, most of the patients exhibited peripheral blood eosinophilia and an elevated serum eosinophil cationic protein level. Ten patients were treated with pulses of methylprednisolone followed by tapering and cyclophosphamide, and the others were treated with corticosteroids alone. The outcomes after long-term follow-up were generally good. One patient who was refractory to initial treatment died of heart failure during the follow-up period. CSS was highly variable in its presentation and course. The manifestations may range from mild symptoms to life-threatening conditions. The outcome after long-term follow-up was as good as that of previous studies. PMID:16614512

Multimodality assessment of cardiac involvement in Churg-Strauss syndrome patients in clinical remission

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Szczeklik, W.; Miszalski-Jamka, T.; Mastalerz, L.; Sokolowska, B.; Dropinski, J.; Musial, J.; Banys, R.; Hor, K.N.; Mazur, W.

2011-01-01

Cardiac involvement in Churg-Strauss syndrome (CSS) is not uncommon, but its frequency varies widely and may depend on the activity of the disease. Therefore, the cardiac involvement in CSS patients in clinical remission was assessed in the present study. In 20 CSS patients in remission and 20 sex- and age-matched healthy controls, an electrocardiogram (ECG) stress test, echocardiography, and 24-h ECG Holter monitoring were performed, together with cardiac magnetic resonance imaging (cMRI). Cardiac involvement was present in 90% (18/20) of CSS patients. Left ventricular ejection fraction (LVEF) was on average lower in the CSS group than in controls (P<0.05), with 7 patients showing systolic heart failure (LVEF <50%). cMRI changes included late gadolinium enhancement lesions in the LV in 89% of patients (17/19), present in all layers of the myocardium. Signs of ongoing inflammation (early gadolinium enhancement) and edema (T2-weighted imaging) were present in 6/19 patients. Holter monitoring revealed both supraventricular and ventricular arrhythmias more frequently in CSS patients when compared with controls (P<0.05). Absolute eosinophil count before the initiation of treatment was higher in rhythm disturbances (P<0.05), and inversely correlated with LV systolic function (rho -0.65). Heart involvement in CSS patients who are in clinical remission is very common. It is characterized not only by fibrosis, but also by an active inflammatory process. The latter finding might influence therapeutic decisions in CSS patients in full clinical remission. (author)

Multimodality assessment of cardiac involvement in Churg-Strauss syndrome patients in clinical remission

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Szczeklik, W; Miszalski-Jamka, T; Mastalerz, L; Sokolowska, B; Dropinski, J; Musial, J [Medical Coll., Jagiellonian Univ., Krakow (Poland); Banys, R [John Paul II Hospital, Krakow (Poland); Hor, K N [Cincinnati Children' s Medical Center, OH (United States); Mazur, W [Heart and Vascular Center at The Christ Hospitals, OH (United States)

2011-02-15

Cardiac involvement in Churg-Strauss syndrome (CSS) is not uncommon, but its frequency varies widely and may depend on the activity of the disease. Therefore, the cardiac involvement in CSS patients in clinical remission was assessed in the present study. In 20 CSS patients in remission and 20 sex- and age-matched healthy controls, an electrocardiogram (ECG) stress test, echocardiography, and 24-h ECG Holter monitoring were performed, together with cardiac magnetic resonance imaging (cMRI). Cardiac involvement was present in 90% (18/20) of CSS patients. Left ventricular ejection fraction (LVEF) was on average lower in the CSS group than in controls (P<0.05), with 7 patients showing systolic heart failure (LVEF <50%). cMRI changes included late gadolinium enhancement lesions in the LV in 89% of patients (17/19), present in all layers of the myocardium. Signs of ongoing inflammation (early gadolinium enhancement) and edema (T2-weighted imaging) were present in 6/19 patients. Holter monitoring revealed both supraventricular and ventricular arrhythmias more frequently in CSS patients when compared with controls (P<0.05). Absolute eosinophil count before the initiation of treatment was higher in rhythm disturbances (P<0.05), and inversely correlated with LV systolic function (rho -0.65). Heart involvement in CSS patients who are in clinical remission is very common. It is characterized not only by fibrosis, but also by an active inflammatory process. The latter finding might influence therapeutic decisions in CSS patients in full clinical remission. (author)

Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome and pulmonary thromboembolism: an overlooked concomitance

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Vilma Takayasu

2013-06-01

Full Text Available The Eosinophilic Granulomatosis with Polyangiitis (formerly Churg- Strauss Syndrome (EGPA is a systemic inflammatory disease characterized by the presence of rhinitis, asthma, peripheral eosinophilia, and vasculitis—the latter being characteristic of the late stage of the disease. After several years from the onset of the disease, small- and medium-sized vessel vasculitis ensues, undertaking various organs and systems. Upper and lower airways, skin, nervous system, gastrointestinal tract, heart, and kidneys are the most commonly involved organs. It is believed that tissue injury is the result of processes mediated by antineutrophil cytoplasmic antibody (ANCA, or toxic mediators released by eosinophils. Although it is classified as ANCA-associated vasculitis, these autoantibodies are present in only 40% of cases. The authors report the case of a patient with EGPA, who had a history of asthma, peripheral and central neuropathy, palpable purpura, gastrointestinal micro perforation, peripheral eosinophilia, and the presence of myeloperoxidase-antineutrophil cytoplasmic antibody. Inflammatory parameters improved after the initiation of treatment, but 1 month after hospital discharge the patient developed symptoms compatible with pulmonary embolism and died. Thrombophilia that occurs in EGPA is due to the interaction between the inflammatory response and eosinophilia with the clotting system resulting in a pro-thrombotic state. Although not yet well-determined, the authors call attention to the possibility of the impact of thromboembolic events on the prognosis of patients with EGPA. In addition to the adequate immunosuppressive treatment, prophylaxis and treatment for thrombosis should never be overlooked.

Diagnosis and classification of eosinophilic granulomatosis with polyangiitis (formerly named Churg-Strauss syndrome).

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Mouthon, Luc; Dunogue, Bertrand; Guillevin, Loïc

2014-01-01

Recently, a group of experts in the field suggested to rename Churg-Strauss syndrome as eosinophilic granulomatosis with polyangiitis (EGPA). This condition, first described in 1951, is a rare small- and medium-sized-vessel vasculitis characterized by an almost constant association with asthma and eosinophilia, and, by the presence of anti-myeloperoxidase (MPO) antineutrophil cytoplasm antibodies (ANCA) in 30-38% of the patients. Vasculitis typically develops in a previously asthmatic and eosinophilic middle-aged patient. Asthma is severe, associated with eosinophilia and extrapulmonary symptoms. Most frequently EGPA involves the peripheral nerves and skin. Other organs, however, may be affected and must be screened for vasculitis, especially those associated with a poorer prognosis, such as the heart, kidney and gastrointestinal tract, as assessed by the recently revised Five-Factor Score (FFS). Recent insights, particularly concerning clinical differences associated with ANCA status, showed that EGPA patients might constitute a heterogeneous group. Thus, EGPA patients with anti-MPO ANCA suffered more, albeit not exclusively, from vasculitis symptoms, such as glomerulonephritis, mononeuritis multiplex and alveolar hemorrhage, whereas ANCA-negative patients more frequently develop heart involvement. This observation led to the hypothesis that EGPA might be divided into different clinical and pathophysiological subtypes, which could be managed better with more specifically adapted therapies. For now, EGPA treatment still relies mainly on corticosteroids and, when necessary for patients with poorer prognoses, combined immunosuppressant drugs, especially cyclophosphamide. Overall survival of EGPA patients is good, despite not uncommon relapses. Copyright © 2014 Elsevier Ltd. All rights reserved.

Successful management of Churg-Strauss syndrome using omalizumab as adjuvant immunomodulatory therapy: first documented pediatric case.

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Iglesias, E; Camacho Lovillo, M; Delgado Pecellín, I; Lirola Cruz, M J; Falcón Neyra, M D; Salazar Quero, J C; Bernabeu-Wittel, J; González Valencia, J P; Neth, O

2014-03-01

Churg-Strauss syndrome (CSS) is an anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis; it is extremely rare in childhood and defined according to the Chapel-Hill Consensus as an eosinophil-rich and granulomatous inflammation involving the respiratory tract and necrotizing vasculitis affecting small to medium-sized vessels. Children commonly have a history of asthma and sinusitis whilst clinical presentation typically involves pulmonary tract and less frequently skin, heart, gastrointestinal tract, and peripheral nerves. Cardiopulmonary disease is higher in children and prognosis is worse. It is associated with significant eosinophilia and raised serum IgE-levels. ANCA are only found in 25% of childhood cases. Here we report the case of a 10-year-old girl who presented to us with vomiting, abdominal pain, and weight loss, paresthesias of lower extremities and breathlessness as well as a history of asthma, sinusitis and allergic rhinitis. She was treated with corticosteroids, cyclophosphamide, intravenous immunoglobulin, mycophenolate mofetil (MMF), and rituximab. However, remission was only achieved after initiation of omalizumab therapy, a recombinant humanized anti-IgE antibody. To the best of our knowledge this is the first pediatric patient suffering from CSS successfully managed with adjuvant anti-IgE therapy resulting in the control of respiratory as well as gastrointestinal symptoms. © 2013 Wiley Periodicals, Inc.

Churg-Strauss Syndrome Following Vaccination Against 2010 Influenza A (H1N1): A Case Report.

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Fu, Mu-Hui; Tsai, Wan-Chen; Lan, Jui; Lu, Cheng-Hsien; Lee, Lian-Hui; Huang, Chih-Cheng

2014-09-01

Churg-Strauss syndrome (CSS) is a systemic inflammatory disorder characterized by asthma, transient pulmonary infiltration, hyper-eosinophilia, and systemic vasculitis. Reported triggering factors include infections, drugs, allergic desensitization, and vaccinations, although cases involving the latter two are extremely rare. Herein, we describe a patient who developed CSS after receiving an H1N1 vaccination. A 55-year-old woman presented with fever, skin eruptions, and sensory impairment of her feet within one week after an H1N1 vaccine injection. A chest X-ray showed pulmonary infiltrations in both lower lung fields. Eosinophilia was noted in a hematological test, and an electrophysiological study revealed a pattern of mononeuritis multiplex. A skin biopsy was performed which revealed palisading necrotizing granuloma around a degenerated dermis and eosinophilic infiltration of the blood vessel walls. These findings combined with the hematological and electrophysiological findings met the criteria of CSS according to the American College of Rheumatology. The patient recovered well after steroid treatment. This case highlights the possibility that the H1N1 vaccination can trigger CSS. Due to the rarity of reported autoimmune events after vaccine administration and the obscure causal association between autoimmunity and a vaccine, further post-marketing surveillance and research are necessary to clarify the relationship and identify risk factors.

Churg-Strauss syndrome with coexistence of eosinophilic vasculitis, granulomatous phlebitis and granulomatous dermatitis in bullous pemphigoid-like blisters.

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Ishibashi, Masafumi; Kudo, Saori; Yamamoto, Kyoko; Shimai, Nobuko; Chen, Ko-Ron

2011-03-01

The main histopathological features in the cutaneous lesions of Churg-Strauss syndrome (CSS) are dermal leukocytoclastic vasculitis with a variable eosinophilic infiltrate and non-vasculitic tissue eosinophilia with granuloma formation. This wide histopathological spectrum may account for the various skin manifestations of CSS. However, the unique histopathological combination of dermal eosinophilic vasculitis and subcutaneous granulomatous phlebitis accompanied by bulla formation has not been previously described. We report an unusual CSS case showing dermal necrotizing eosinophilic vasculitis and granulomatous phlebitis in purpuric lesions coupled with subepidermal blistering. The blisters showed dermal granulomatous dermatitis and eosinophilia without evidence of vasculitis. Dermal necrotizing eosinophilic vasculitis was characterized by fibrinoid alteration of the vessel wall, a prominent perivascular eosinophilic infiltrate, a few infiltrating histiocytes along the affected vessel wall, and the absence of neutrophilic infiltration. The underlying subcutaneous granulomatous phlebitis was characterized by an angiocentric histiocytic infiltrate surrounded by marked eosinophilic infiltrate. Deposition of cytotoxic proteins and radicals derived from eosinophils in the vessel walls and papillary dermis followed by a secondary granulomatous response may account for the unique clinical and histopathological features in this case. Copyright © 2010 John Wiley & Sons A/S.

Development of eosinophilic granulomatosis with poliangiitis (Churg-Strauss syndrome) and brain tumor in a patient after more than 7 years of omalizumab use: A case report.

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Borekci, S; Aydin, O; Hatemi, G; Gemicioglu, B

2015-03-01

Omalizumab is a monoclonal anti-immunoglobulin E antibody used for the treatment of severe perennial allergic asthma. Previous reports have suggested that omalizumab treatment can be associated with the development of eosinophilic granulomatosis with poliangiitis (EGPA) (formerly known as Churg-Strauss syndrome) and an increased risk of malignancy. Long-term risks of omalizumab treatment are not very well defined. Here, we report the case of a 75-year-old woman with concurrent occurrence of EGPA and brain tumor after more than 7 years of omalizumab treatment. The possibility of EGPA should be borne in mind during long-term treatment with omalizumab. Despite the absence of definitive data, an association may also exist between the development of malignancy and omalizumab use. © The Author(s) 2015.

Antineutrophil cytoplasmic autoantibodies and myeloperoxidase autoantibodies in clinical expression of Churg-Strauss syndrome.

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Healy, Bridget; Bibby, Susan; Steele, Richard; Weatherall, Mark; Nelson, Harold; Beasley, Richard

2013-02-01

The clinical significance of antineutrophil cytoplasmic antibodies (ANCAs) in the phenotypic expression of Churg-Strauss syndrome (CSS) is uncertain. We sought to investigate the relationship between ANCA status and the clinical expression of CSS in a case series derived from the US Food and Drug Administration's adverse events database. All cases of CSS reported to the US Food and Drug Administration from 1997 to April 2003 were reviewed. Information about basic demographics, suspect medication use, clinical manifestations, histologic findings, ANCA staining patterns, and the presence of antibodies to myeloperoxidase (anti-MPO) or proteinase 3 (anti-PR3) was recorded when available. There were 93 case reports of CSS with sufficient documentation, including ANCA status. There were 38 (40.9%) of 93 cases with positive ANCA results, of which 15 cases reported a positive ELISA, all of which were positive for anti-MPO. ANCA negativity was associated with an increased proportion of cardiac involvement (risk difference [RD], 38.2%; 95% CI, 25.3% to 51.0%), gastrointestinal involvement (RD, 25.5%; 95% CI, 13.9% to 37.0%), pulmonary infiltrates (odds ratio, 4.9; 95% CI, 1.5-16.2), and the outcome of a life-threatening event or death (RD, 30.9%; 95% CI, 18.7% to 43.1%) when compared with anti-MPO-positive cases. ANCA negativity was associated with a decreased proportion of peripheral neuropathy (odds ratio, 0.3; 95% CI, 0.07-0.9). These findings support the hypothesis that the presence or absence of autoantibodies influences the clinical expression and severity of CSS. Copyright © 2012 American Academy of Allergy, Asthma & Immunology. Published by Mosby, Inc. All rights reserved.

Churg-Strauss syndrome: a new endotype of severe asthma? Results of 14 Turkish patients.

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Yılmaz, İnsu; Çelik, Gülfem; Aydın, Ömür; Özdemir, Seçil Kepil; Soyyiğit, Şadan; Sözener, Zeynep; Özgüçlü, Selcan; Atasoy, Çetin; Düzgün, Nurşen; Mungan, Dilşad; Sin, Betül; Demirel, Yavuz Selim; Mısırlıgil, Zeynep

2015-07-01

Churg-Strauss syndrome (CSS) is a rare multisystem vasculitis. Considering the variation of autoimmune diseases in different races, it is of interest to determine whether any outstanding features exist for Turkish patients with CSS. The aim of this study was to evaluate the clinical and serological features of the disease, the treatment, and long-term follow-up details, and to investigate possible etiological factors of Turkish CSS patients. The study included 14 patients who were diagnosed with CSS, and followed by our department between 2004 and 2012. Possible etiological factors, initial symptoms, clinical presentations, treatment, as well as outcomes were documented. The study was approved by the local ethics. All patients fulfilled the American College of Rheumatology criteria. Initial symptoms were worsening asthma (n = 14; 100%) and skin lesions (n = 6; 43%). All patients had a diagnosis of asthma and nasal polyps, whereas 57.1% had aspirin hypersensitivity at the time of diagnosis. The lungs (100%) and skin (43%) were most commonly involved. Peripheral eosinophilia dominated on initial presentations of all patients. Initial treatments included oral methyl prednisolone in all cases, whereas cyclophosphamide and azathioprine were used in three cases. Relapses were detected in five cases. None of the cases were able to stop the oral corticosteroid treatment. No fatalities were observed. We herein describe a new severe asthma endotype in connection with CSS. We suggest that physicians who deal with uncontrolled severe asthma cases should consider CSS in the presence of nasal polyps, aspirin hypersensitivity, and especially peripheral blood eosinophilia over 10%. © 2014 John Wiley & Sons Ltd.

123I-MIBG imaging detects cardiac involvement and predicts cardiac events in Churg-Strauss syndrome

International Nuclear Information System (INIS)

Horiguchi, Yoriko; Morita, Yukiko; Tsurikisawa, Naomi; Akiyama, Kazuo

2011-01-01

In Churg-Strauss syndrome (CSS) it is important to detect cardiac involvement, which predicts poor prognosis. This study evaluated whether 123 I-metaiodobenzylguanidine (MIBG) scintigraphy could detect cardiac damage and predict cardiac events in CSS. 123 I-MIBG scintigraphy was performed in 28 patients with CSS, 12 of whom had cardiac involvement. The early and delayed heart to mediastinum ratio (early H/M and delayed H/M) and washout rate were calculated by using 123 I-MIBG scintigraphy and compared with those in control subjects. Early H/M and delayed H/M were significantly lower and the washout rate was significantly higher in patients with cardiac involvement than in those without and in controls (early H/M, p = 0.0024, p = 0.0001; delayed H/M, p = 0.0002, p = 0.0001; washout rate, p = 0.0012, p = 0.0052 vs those without and vs controls, respectively). Accuracy for detecting cardiac involvement was 86% for delayed H/M and washout rate and 79% for early H/M and B-type natriuretic peptide (BNP). Kaplan-Meier analysis showed significantly lower cardiac event-free rates in patients with early H/M ≤ 2.18 and BNP > 21.8 pg/ml than those with early H/M > 2.18 and BNP ≤ 21.8 pg/ml (log-rank test p = 0.006). Cardiac sympathetic nerve function was damaged in CSS patients with cardiac involvement. 123 I-MIBG scintigraphy was useful in detecting cardiac involvement and in predicting cardiac events. (orig.)

Childhood-onset eosinophilic granulomatosis with polyangiitis (formerly Churg-Strauss syndrome): a contemporary single-center cohort.

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Gendelman, Samantha; Zeft, Andrew; Spalding, Steven J

2013-06-01

To date only 38 cases of childhood-onset eosinophilic granulomatosis with polyangiitis (cEGPA; formerly Churg-Strauss syndrome) have been reported. Additional patients with cEGPA could enhance the understanding of this rare and life-threatening condition. Our objectives were (1) to determine the frequency of specific organ system involvement; (2) to examine initial therapeutic regimen; and (3) to document disease and therapy-related morbidity in a contemporary cohort of patients with cEGPA. Retrospective review of patients evaluated at the Cleveland Clinic between 2003 and 2011 who met either American College of Rheumatology or Lanham criteria for EGPA and whose age was < 18 years at symptom onset. Nine patients (8 female; 7 white) were identified. Median age at onset of rhinitis/asthma symptom was 13 years and median age at diagnosis of cEGPA was 15 years. All patients demonstrated eosinophilia, upper airway disease (allergic rhinitis, chronic sinusitis, and/or nasal polyps), and pulmonary involvement. Other frequently involved organ systems included musculoskeletal (67%), gastrointestinal (67%), cutaneous (67%), neurologic (56%), and cardiac (44%). Antineutrophil cytoplasmic antibody (ANCA) serologies were negative in all patients. The medications used most frequently for initial therapy included oral (44%) or intravenous corticosteroids (56%) and azathioprine (67%). Disease or therapeutic complications occurred in half of the cohort and included heart failure, stroke, and sequela from longterm, high-dose steroids. Eosinophilia, in combination with upper airway, pulmonary, musculoskeletal, neurologic, and cardiac manifestations, is frequently observed in cEGPA. ANCA titers are often negative. Steroids are the mainstay of initial therapy but steroid-related side effects occur regularly.

Cardiac involvement in ANCA (+) and ANCA (-) Churg-Strauss syndrome evaluated by cardiovascular magnetic resonance.

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Mavrogeni, Sophie; Karabela, Georgia; Gialafos, Elias; Stavropoulos, Efthymios; Spiliotis, George; Katsifis, Gikas; Kolovou, Genovefa

2013-10-01

The cardiovascular magnetic resonance (CMR) pattern of Churg-Strauss syndrome (CSS) includes myopericarditis, diffuse subendocardial vasculitis or myocardial infarction with or without cardiac symptoms and is usually associated with lack of antineutrophil cytoplasmic antibodies (ANCA). To correlate the CMR pattern with ANCA in CSS, compare it with healthy controls and systemic lupus erythematosus (SLE) patients and re-evaluate 2 yrs after the first CMR. 28 consecutive CSS, aged 42±7 yrs, were referred for CMR and 2 yrs re-evaluation. The CMR included left ventricular ejection fraction (LVEF), T2-weighted (T2-W), early (EGE) and late gadolinium enhanced (LGE) imaging. Their results were compared with 28 systemic lupus erythematosus (SLE) under remission and 28 controls with normal myocardial perfusion, assessed by scintigraphy. CMR revealed acute cardiac lesions in all ANCA (-) CSS with active disease and acute cardiac symptoms and only in one asymptomatic ANCA (+) CSS, with active disease. Diffuse subendocardial fibrosis (DSF) or past myocarditis was identified in both ANCA(+) and ANCA (-) CSS, but with higher incidence and fibrosis amount in ANCA (-) CSS (p<0.05). In comparison to SLE, both ANCA (+) and ANCA (-) CSS had higher incidence of DSF, lower incidence of myocarditis and no evidence of myocardial infarction, due to coronary artery disease (p<0.05). In 2 yrs CMR follow up, 1/3 of CSS with DSF presented LV function deterioration and one died, although immunosuppressive treatment was given early after CSS diagnosis. Cardiac involvement either as DSF or myocarditis, can be detected in both ANCA (+) and ANCA (-) CSS, although more clinically overt in ANCA (-). DSF carries an ominous prognosis for LV function. CMR, due to its capability to detect disease severity, before cardiac dysfunction takes place, is an excellent tool for CSS risk stratification and treatment individualization.

{sup 123}I-MIBG imaging detects cardiac involvement and predicts cardiac events in Churg-Strauss syndrome

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Horiguchi, Yoriko; Morita, Yukiko [National Hospital Organization Sagamihara National Hospital, Department of Cardiology, Sagamihara City, Kanagawa (Japan); Tsurikisawa, Naomi; Akiyama, Kazuo [National Hospital Organization Sagamihara National Hospital, Clinical Research Centre for Allergy and Rheumatology, Sagamihara City, Kanagawa (Japan)

2011-02-15

In Churg-Strauss syndrome (CSS) it is important to detect cardiac involvement, which predicts poor prognosis. This study evaluated whether {sup 123}I-metaiodobenzylguanidine (MIBG) scintigraphy could detect cardiac damage and predict cardiac events in CSS. {sup 123}I-MIBG scintigraphy was performed in 28 patients with CSS, 12 of whom had cardiac involvement. The early and delayed heart to mediastinum ratio (early H/M and delayed H/M) and washout rate were calculated by using {sup 123}I-MIBG scintigraphy and compared with those in control subjects. Early H/M and delayed H/M were significantly lower and the washout rate was significantly higher in patients with cardiac involvement than in those without and in controls (early H/M, p = 0.0024, p = 0.0001; delayed H/M, p = 0.0002, p = 0.0001; washout rate, p = 0.0012, p = 0.0052 vs those without and vs controls, respectively). Accuracy for detecting cardiac involvement was 86% for delayed H/M and washout rate and 79% for early H/M and B-type natriuretic peptide (BNP). Kaplan-Meier analysis showed significantly lower cardiac event-free rates in patients with early H/M {<=} 2.18 and BNP > 21.8 pg/ml than those with early H/M > 2.18 and BNP {<=} 21.8 pg/ml (log-rank test p = 0.006). Cardiac sympathetic nerve function was damaged in CSS patients with cardiac involvement. {sup 123}I-MIBG scintigraphy was useful in detecting cardiac involvement and in predicting cardiac events. (orig.)

Intravenous immunoglobulin for chronic residual peripheral neuropathy in eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome): a multicenter, double-blind trial.

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Koike, Haruki; Akiyama, Kazuo; Saito, Toyokazu; Sobue, Gen

2015-03-01

Eosinophilic granulomatosis with polyangiitis (EGPA), previously called Churg-Strauss syndrome, frequently affects the peripheral nervous system. We conducted a multicenter, double-blind, three-arm treatment period, randomized, pre-post trial to assess the efficacy of intravenous immunoglobulin (IVIg) administration for residual peripheral neuropathy in patients with EGPA that is in remission, indicated by laboratory indices. Twenty-three patients were randomly assigned into three groups, in which the timing of IVIg and placebo administration was different. Each group received one course of intervention and two courses of placebo at 2-week intervals. Treatment effects were assessed every 2 weeks for 8 weeks. The primary outcome measure, the amount of change in the manual muscle testing sum score 2 weeks after IVIg administration, significantly increased (p = 0.002). The results over time suggested that this effect continued until the last assessment was done 8 weeks later. The number of muscles with manual muscle testing scores of three or less (p = 0.004) and the neuropathic pain scores represented by the visual analogue scale (p = 0.005) also improved significantly 2 weeks after IVIg administration. This study indicates that IVIg treatment for EGPA patients with residual peripheral neuropathy should be considered even when laboratory indices suggest remission of the disease.

Síndrome de Churg Strauss: uma vasculite rara Churg strauss Syndrome: a rare vasculitis

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Luciana Calvo Mardegan

2004-04-01

Full Text Available O objetivo deste trabalho é avaliar as características demográficas, clínicas, laboratoriais e histopatológica de pacientes com diagnóstico de síndrome de Churg Strauss (SCS acompanhados no ambulatório de vasculites do Hospital das Clínicas da Universidade Estadual de Campinas (HC/Unicamp. Foi realizado estudo retrospectivo dos prontuários dos pacientes com vasculite primária, classificados como SCS pelos critérios do American College of Rheumathology (ACR. Foram observados cinco pacientes com diagnóstico de SCS (M/F:3/2, caucasóides. A média de idade do início da doença foi 46 anos (40-55. A média de tempo de seguimento foi de 2,37 anos (0,25-6. Sintomas sistêmicos, asma, neuropatia periférica e lesão cutânea foram observados em todos os pacientes. Envolvimento cardiovascular, renal e de trato gastrintestinal estiveram presentes cada qual em um paciente. Todos os pacientes apresentaram eosinofilia (>10%,e o p-ANCA foi positivo nos três casos investigados. A radiografia de tórax revelou infiltrado pulmonar intersticial em dois casos; em um, associado à derrame pleural. Na histopatologia, evidências de eosinófilos extravascular foram encontradas nas biópsias cutâneas de 3/4 pacientes. Todos os pacientes foram tratados com prednisona. Em 3, houve a necessidade de associar-se imunossupressor. Nenhum paciente evoluiu a óbito. A SCS é uma patologia rara, com acometimento sistêmico, sendo a asma uma das principais e mais precoce manifestação. Todos os pacientes apresentaram sintomas sistêmicos, acometimento de pele e sistema nervoso periférico. Envolvimento renal, cardíaco e gastrointestinal foi observado, cada qual em um único paciente, diferindo de outras séries nas quais esses acometimentos são mais freqüentes. Embora seja uma patologia grave e de prognóstico reservado, observamos evolução favorável com tratamento.The aim of this report is to verify the demographic, clinical, laboratorial and

Scintigraphy in a case of Churg-Strauss vasculitis with myocardial involvement using 111In-labelled antimyosin

International Nuclear Information System (INIS)

Krause, T.; Schuemichen, C.; Beck, A.; Moser, E.; Lang, B.; Hohnloser, S.

1990-01-01

A case of Churg-Strauss vasculitis in a young woman is reported. Diagnosis was confirmed by muscle biopsy. Affection of lungs, kidneys and skin was evident. In addition, myocarditis was suspected on clinical evidence. A highly positive scintigraphy with 111 In-antimyosin enabled diagnosis and assessment of damage to the myocytes. With a heart-to-lung ratio of 3.0 the accumulated activity in the myocardium was higher than usually found in myocarditis. This finding supports the hypothesis of an additional ischemic necrosis. (orig.) [de

Effect of delayed diagnosis on disease course and management of Churg-Strauss syndrome: a retrospective study.

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Sokołowska, Barbara; Szczeklik, Wojciech; Mastalerz, Lucyna; Kuczia, Paweł; Wodkowski, Michał; Stodółkiewicz, Edyta; Macioł, Karolina; Musiał, Jacek

2013-03-01

Delayed diagnosis in patients with Churg-Strauss syndrome (CSS) is largely attributed to the variable and nonspecific presentation of the disease's initial symptoms. The aim of the study was to evaluate the effect of delayed diagnosis on the course of CSS. We conducted a retrospective study of 30 CSS patients followed up in our department. In each patient, we assessed the delay in CSS diagnosis (the time when patients already fulfilled four out of six of the American College of Rheumatology criteria and the diagnosis was not yet established), the disease activity at the time of diagnosis, and organ involvement during CSS course. A median value of 2 weeks was chosen as the cutoff point after which the diagnosis was considered as delayed. Sixteen patients were diagnosed before (group 1) and 14 patients after this cutoff point (group 2). In group 2, we found a higher Birmingham Vasculitis Activity Score at the moment of diagnosis (20.4 vs 25.1, p < 0.05) and a more severe disease course, resulting in more frequent hospitalization rates (0.64 vs 2.26/year, p < 0.00001), higher corticosteroids dose requirements (5.87 vs 11.57 mg/day converted to methylprednisolone, p < 0.0001), and additional immunosuppressive therapy administration (56.2 vs 92.8 %, p < 0.05) to maintain disease remission. All six perinuclear pattern of antineutrophil cytoplasmic antibobodies (pANCA)-positive patients (20 %) were found in group 1. Concluding, the delay in diagnosis of CSS of more than 2 weeks was found to be associated with a disease course that was more severe. The presence of the pANCA antibodies may occasionally facilitate establishment of the diagnosis.

Clinical and laboratory characteristics of 19 patients with Churg-Strauss syndrome from a single South Australian centre.

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Whyte, A F; Smith, W B; Sinkar, S N; Kette, F E; Hissaria, P

2013-07-01

Churg-Strauss syndrome (CSS) is a rare, idiopathic systemic vasculitis. There is emerging evidence of an association between the presence or absence of antineutrophil cytoplasmic antibodies (ANCA) and clinical phenotype. Thromboembolism is an increasingly recognised complication of the disease. Given the paucity of Australian data, the aim of this study was to examine the clinical and laboratory features of CSS in a single Australian centre. We performed a retrospective review of all patients who fulfilled the American College of Rheumatology classification criteria for CSS managed at the Department of Immunology, Royal Adelaide Hospital between 2002 and 2008. Nineteen patients were included. All patients had asthma and most had upper airway involvement. Peripheral nerve, musculoskeletal, gastrointestinal and cutaneous involvement was common. Renal and cardiac involvement was uncommon in this series. Histological confirmation was obtained in 15 patients (78.9%). Ten patients (52.6%) were ANCA+, and these were more likely to have musculoskeletal involvement, such as arthralgia or myalgia (odds ratio 57, P = 0.005). Thrombosis was a feature at diagnosis in six patients (31.6%); two of these recurred with relapse. Sixteen patients (84.2%) were followed up; five died, and mean survival was 8.9 years. This is the first Australian study to focus on CSS. Our results demonstrate similar presentation and prognosis of CSS to previous descriptions; however, we noted that musculoskeletal involvement was more common in ANCA+ patients. In our series, thrombosis was a significant complication and we suggest that thromboprophylaxis may be warranted. © 2013 The Authors; Internal Medicine Journal © 2013 Royal Australasian College of Physicians.

Association of asthma therapy and Churg-Strauss syndrome: an analysis of postmarketing surveillance data.

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DuMouchel, William; Smith, Eric T; Beasley, Richard; Nelson, Harold; Yang, Xionghu; Fram, David; Almenoff, June S

2004-07-01

Churg-Strauss syndrome (CSS), also known as allergic granulomatous angiitis (AGA), is a rare vasculitis that occurs in patients with bronchial asthma. The nature of the association of CSS with various asthma therapies is unclear. This study investigated the associations of different multidrug asthma therapy regimens and the reporting of AGA (the preferred code for CSS in the coding dictionary for the Adverse Event Reporting System [AERS]) by applying an iterative method of disproportionally analysis to th AERS database maintained by the US Food and Drug Administration. The public-release version of the AERS database was used to identify reports of AGA in patients receiving asthma therapy. Reporting of AGA was examined using iterative disproportionality methods in patients receiving > or =1 of the following drug classes: inhaled corticosteroid (ICS), leukotriene receptor antagonist (LTRA), short-acting beta(2)-agonist (SABA), or long-acting beta(2)-agonist (LABA). The Bayesian data-mining algorithm known as the multi-item gamma poisson shrinker was used to determine the relative reporting rates by calculation of the empirical Bayes geometric mean (EBGM) and its 90% CI (EB05 = lower limit and EB95 = upper limit) for each drug. Subset analyses were performed for each drug with different medication combinations to differentiate the relative reporting of AGA for each. A strong association was found between LTRA use and AGA (EBGM = 104.0, EB05 = 95.0, EB95 = 113.8) that persisted with all combinations of therapy studied. AGA was also associated with the ICS, SABA and LABA classes (EBGM values of 27.8, 14.6 and 40.4, respectively). However, the latter associations were mostly dependent on the presence of concurrent LTRA and, to a lesser extemt, oral corticosteroid therapy and became negligible (ie, EB05 < 2) for patients who were not receiving these concurrent treatments. Differences based on relative reporting were observed in the patterns of association of AGA with LTRA

Anterior ischaemic optic neuropathy in eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome): a case report and review of the literature.

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Padovano, Ilaria; Pazzola, Giulia; Pipitone, Nicolò; Cimino, Luca; Salvarani, Carlo

2014-01-01

We report a 62-year-old man with mild fever, headache and acute visual loss in his right eye due to anterior ischaemic optic neuropathy (AION), followed a few days later by pain in the legs and left arm associated with numbness and weakness. Giant cell arteritis complicated by AION was suspected at the beginning and high-dose oral glucocorticoids were started. However, on the basis of the past medical history of nasal polyposis, asthma, and hypereosynophilia as well as of further investigations (biopsy of the nasal mucosa showing granulomatous inflammation with a rich eosinophilic infiltrate, electromyography demonstrating, mononeuritis multiplex and positive p-ANCA), eosinophilic granulomatosis with polyangiitis (EGPA), previously known as Churg-Strauss syndrome, was diagnosed. Because visual acuity in the right eye deteriorated despite glucocorticoid therapy, pulse intravenous cyclophosphamide was started, subsequently replaced by oral azathioprine, while prednisone was slowly tapered. This treatment led to gradual improvement of the neurological symptoms, whereas the right visual impairment remained unchanged. EGPA-related AION is an uncommon lesion that is probably due to vasculitic involvement of posterior ciliary and/or chorioretinal arteries. The prognosis of established AION is poor for the affected eye, even when glucocorticoid treatment is started immediately. However, early recognition of AION and prompt aggressive treatment with high-dose glucocorticoids plus cyclophosphamide can prevent visual loss in the unaffected eye.

Modulation of MMP-2 and MMP-9 in Churg-Strauss syndrome respiratory mucosa: potential monitoring parameters.

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Leone, A; Uzzo, M L; Gerbino, A; Tortorici, S; Tralongo, P; Cappello, F; Incandela, S; Spatola, G F; Jurjus, A R

2014-01-01

Churg-Strauss (CSS) syndrome is rare and of unknown etiology. It is associated with vasculitis, blood eosinophilia and granulomatosis, and affects multiple organs and systems at various stages of the disease. Specific diagnostic and monitoring tests are not yet available. This study aims to assess the changes in MMP-2 and MMP-9 along with the histopathological alterations in two cases of CSS, as possible potential diagnostic and monitoring criteria. Two adult male patients were diagnosed with CSS in the otorhinolaryngology clinic in the University of Palermo, based on multiple clinical and histopathologic criteria. Biopsies of respiratory mucosa were taken after the consent of the patients, processed for routine histopathology and immunohistochemistry as well as quantitative polymerase chain reaction (qPCR). Similar biopsies were also taken from a non- CSS patient. The Assessment of MMP-2 and MMP-9 was performed using both immunohistochemistry and qPCR techniques. Histopathological alterations in the respiratory mucosa were consistent with vasculitis and granulomatous tissue formation, in addition to inflammatory cell infiltration with abundance of eosinophils. Immunohistochemistry assay performed on the samples derived from the two CSS patients showed a relative and remarkable increase of both MMP-2 and MMP-9 compared to controls. Such an increase was consistent with the qPCR results which depicted a significant increase between 20 and 30% for both MMP-2 and MMP-9, respectively. Since the secretion of MMPs is an essential step in angiogenesis, could these enzymatic factors be used as parameters to diagnose or monitor the evolution of CSS? The small number of samples analyzed in this study does not allow us to suggest a general statement correlating the increase in expression of MMP-2 and MMP-9 to the appearance or evolution of vasculitis; it is only speculative.

Contrasting association of a non-synonymous leptin receptor gene polymorphism with Wegener's granulomatosis and Churg-Strauss syndrome.

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Wieczorek, Stefan; Holle, Julia U; Bremer, Jan P; Wibisono, David; Moosig, Frank; Fricke, Harald; Assmann, Gunter; Harper, Lorraine; Arning, Larissa; Gross, Wolfgang L; Epplen, Joerg T

2010-05-01

There is evidence that the leptin/ghrelin system is involved in T-cell regulation and plays a role in (auto)immune disorders such as SLE, RA and ANCA-associated vasculitides (AAVs). Here, we evaluate the genetic background of this system in WG. We screened variations in the genes encoding leptin, ghrelin and their receptors, the leptin receptor (LEPR) and the growth hormone secretagogue receptor (GHSR). Three single nucleotide polymorphisms (SNPs) in each gene region were analysed in 460 German WG cases and 878 ethnically matched healthy controls. A three-SNP haplotype of GHSR was significantly associated with WG [P = 0.0067; corrected P-value (P(c)) = 0.026; odds ratio (OR) = 1.30; 95% CI 1.08, 1.57], as was one non-synonymous SNP in LEPR (Lys656Asn, P = 0.0034; P(c) = 0.013; OR = 0.72; 95% CI 0.58, 0.90). These four SNPs were re-analysed in independent cohorts of 226 German WG cases and 519 controls. While the GHSR association was not confirmed, allele frequencies of the LEPR SNP were virtually identical to those from the initial cohorts. Analysis of this SNP in the combined WG and control panels revealed a significant association of the LEPR 656Lys allele with WG (P = 0.00032; P(c) = 0.0013; OR = 0.72; 95% CI 0.60, 0.86). Remarkably, the Lys656Asn SNP showed contrasting allele distribution in two cohorts of 108 and 88 German cases diagnosed with Churg-Strauss syndrome (CSS, combined P = 0.0067; OR = 1.41; 95% CI 1.10, 1.81), whereas identical allele frequencies were revealed when comparing British WG and microscopic polyangiitis cases. While GHSR has to be further evaluated, these data provide profound evidence for an association of the LEPR Lys656Asn SNP with AAV, resulting in opposing effects in WG and CSS.

A 92-Year-Old Male with Eosinophilic Asthma Presenting with Recurrent Palpable Purpuric Plaques

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Negbenebor, Nicole A.; Khalifian, Saami; Foreman, Ruth K.; Kroshinsky, Daniela

2018-01-01

Churg-Strauss syndrome or eosinophilic granulomatosis with polyangiitis is a systemic vasculitis affecting the small and medium-sized vasculature. It is commonly associated with asthma and eosinophilia. Most patients are diagnosed at around the age of 40. We report a case of biopsy-confirmed Churg-Strauss syndrome in a 92-year-old male with a history of eosinophilic asthma and peripheral eosinophilia who was later diagnosed with Churg-Strauss syndrome. PMID:29719830

A 92-Year-Old Male with Eosinophilic Asthma Presenting with Recurrent Palpable Purpuric Plaques

Directory of Open Access Journals (Sweden)

Nicole A. Negbenebor

2018-03-01

Full Text Available Churg-Strauss syndrome or eosinophilic granulomatosis with polyangiitis is a systemic vasculitis affecting the small and medium-sized vasculature. It is commonly associated with asthma and eosinophilia. Most patients are diagnosed at around the age of 40. We report a case of biopsy-confirmed Churg-Strauss syndrome in a 92-year-old male with a history of eosinophilic asthma and peripheral eosinophilia who was later diagnosed with Churg-Strauss syndrome.

Relationship among antineutrophil cytoplasmic antibody, blood urea nitrogen and complement in patients with eosinophilic granulomatosis polyangiitis (Churg-Strauss syndrome).

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Kawakami, Tamihiro; Kimura, Satoko; Takeuchi, Sora; Soma, Yoshinao

2013-07-01

Eosinophilic granulomatosis with polyangiitis (EGPA), also known as Churg-Strauss syndrome, is an antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis characterized by a history of asthma, hypereosinophilia. The prevalence of ANCA in EGPA is less common than in other ANCA-associated vasculitis. Increasing evidence of complement activation in the pathogenesis of ANCA-associated vasculitis has been provided by studies in animal models. We examined EGPA patients with cutaneous manifestations as an initial sign and investigated the correlations among clinical, serological and histopathological findings. We focused on differences among ANCA, blood urea nitrogen and complement levels such as complement 3 (C3), C4 and total complement hemolytic activity (CH50). We retrospectively investigated the records of 22 patients (11 male and 11 female) with EGPA admitted to our hospital from 1997-2012. Ten of the 22 patients (46%) were positive for serum myeloperoxidase (MPO)-ANCA. In contrast, all the patients were negative for serum proteinase 3 ANCA. There was a significantly positive correlation between serum CH50 and C4 levels in patients with EGPA. Serum blood urea nitrogen (BUN) levels differed significantly between MPO-ANCA-positive and -negative patients. Serum CH50 levels were higher in MPO-ANCA-positive patients compared to negative patients. Serum BUN levels were higher in elevated CH50 patients compared to normal and low CH50-negative patients. We propose that positive findings for MPO-ANCA with CH50 high activity may be a risk factor for developing renal insufficiency. Assuming there are correlations between the presence of ANCA and complements, earlier diagnosis based on initial efficacious treatment for EGPA. © 2013 Japanese Dermatological Association.

Churg-Strauss syndrome cardiac involvement evaluated by cardiac magnetic resonance imaging and positron-emission tomography: a prospective study on 20 patients

International Nuclear Information System (INIS)

Marmursztejn, Julien; Guillevin, Loic; Cohen, Pascal; Guilpain, Philippe; Pagnoux, Christian; Mouthon, Luc; Trebossen, Regine; Legmann, Paul; Vignaux, Olivier; Duboc, Denis

2013-01-01

Churg-Strauss syndrome (CSS) cardiac involvement is associated with a poor prognosis. Recently cardiac MRI (CMRI) has emerged as a promising technique to detect early CSS cardiac involvement. However, CMRI-detected myocardial delayed enhancement (MDE) could correspond to fibrosis or inflammation. Fluoro-2-deoxyglucose PET (FDG-PET) was previously used in other systemic diseases to distinguish between them. To determine whether the CMRI-MDE detected in CSS patients reflected fibrosis or myocardial inflammation, patients in CSS remission underwent FDG-PET. Twenty consecutive CSS patients in remission (BVAS = 0) were recruited. Fourteen patients [eight men, six women; mean (S.D.) age 49 (9) years; mean disease duration 3.5 (2.9) years] with CMRI-detected MDE, and six patients [four men, two women; mean (S.D.) age 44 (15) years; mean disease duration 3.5 (5.3) years] with normal CMRI underwent FDG-PET. Segments with MDE on CMRI were analysed on FDG-PET images, with myocardial FDG hypo-fixation defining fibrosis and hyper-fixation corresponding inflammation. Among the 14 patients with MDE on CMRI, FDG-PET showed 10 had hypo-fixation, 2 had hyper-fixation and 2 had normal scans. CSS duration at the time of CMRI was shorter for patients with myocardial inflammation than in those with fibrosis. The six patients with normal CMRI had normal FDG-PET images. For CSS patients in remission, CMRI detected subclinical active myocardial lesions and could be recommended to assess cardiac involvement. However, because CMRI-detected MDE can reflect fibrosis or inflammation, FDG-PET might help to distinguish between the two. (authors)

Churg-Strauss syndrome cardiac involvement evaluated by cardiac magnetic resonance imaging and positron-emission tomography: a prospective study on 20 patients

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Marmursztejn, Julien [Department of Cardiology, Service Frederic Joliot CEA Orsay, Hopital Cochin, AP-HP, Universite Paris Descartes, Sorbonne Paris Cite, Paris, (France); Department of Radiology, Service Frederic Joliot CEA Orsay, Hopital Cochin, AP-HP, Universite Paris Descartes, Sorbonne Paris Cite, Paris, (France); Guillevin, Loic; Cohen, Pascal; Guilpain, Philippe; Pagnoux, Christian; Mouthon, Luc [Department of Internal Medicine, Service Frederic Joliot CEA Orsay, Hopital Cochin, AP-HP, Universite Paris Descartes, Sorbonne Paris Cite, Paris, (France); Trebossen, Regine [Department of Nuclear Medicine, Service Frederic Joliot CEA Orsay, Hopital Cochin, AP-HP, Universite Paris Descartes, Sorbonne Paris Cite, Paris, (France); Legmann, Paul; Vignaux, Olivier [Department of Radiology, Service Frederic Joliot CEA Orsay, Hopital Cochin, AP-HP, Universite Paris Descartes, Sorbonne Paris Cite, Paris, (France); Duboc, Denis [Department of Cardiology, Service Frederic Joliot CEA Orsay, Hopital Cochin, AP-HP, Universite Paris Descartes, Sorbonne Paris Cite, Paris, (France)

2013-07-01

Churg-Strauss syndrome (CSS) cardiac involvement is associated with a poor prognosis. Recently cardiac MRI (CMRI) has emerged as a promising technique to detect early CSS cardiac involvement. However, CMRI-detected myocardial delayed enhancement (MDE) could correspond to fibrosis or inflammation. Fluoro-2-deoxyglucose PET (FDG-PET) was previously used in other systemic diseases to distinguish between them. To determine whether the CMRI-MDE detected in CSS patients reflected fibrosis or myocardial inflammation, patients in CSS remission underwent FDG-PET. Twenty consecutive CSS patients in remission (BVAS = 0) were recruited. Fourteen patients [eight men, six women; mean (S.D.) age 49 (9) years; mean disease duration 3.5 (2.9) years] with CMRI-detected MDE, and six patients [four men, two women; mean (S.D.) age 44 (15) years; mean disease duration 3.5 (5.3) years] with normal CMRI underwent FDG-PET. Segments with MDE on CMRI were analysed on FDG-PET images, with myocardial FDG hypo-fixation defining fibrosis and hyper-fixation corresponding inflammation. Among the 14 patients with MDE on CMRI, FDG-PET showed 10 had hypo-fixation, 2 had hyper-fixation and 2 had normal scans. CSS duration at the time of CMRI was shorter for patients with myocardial inflammation than in those with fibrosis. The six patients with normal CMRI had normal FDG-PET images. For CSS patients in remission, CMRI detected subclinical active myocardial lesions and could be recommended to assess cardiac involvement. However, because CMRI-detected MDE can reflect fibrosis or inflammation, FDG-PET might help to distinguish between the two. (authors)

Churg-Strauss Syndrome: a syndrome described on clinical observation and autopsy findings

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Fernando Peixoto Ferraz de Campos

2013-06-01

Full Text Available Dr. Jacob Churg (1910-2005 was born in Dolhinow, a Belarusian city that later became a part of Poland and in 1939 was ceded to Russia during the partition of Poland by Russia and Germany. Churg’s father, Wolf, was a physician, and his mother, Gita, a dentist. Jacob recalled reading his father’s medical books and pestering him with many questions. He graduated with a medical degree from the University of Wilno (Poland in 1933 and started working at the Department of Internal Medicine. He realized that he was less confident dealing with patients than with “tissues.” In 1936, at the same university, he began his studies in experimental pathology.

The ANCA Vasculitis Questionnaire (AAV-PRO©)

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2017-05-01

Eosinophilic Granulomatosis With Polyangiitis (Churg-Strauss) (EGPA); Churg-Strauss Syndrome (CSS); Granulomatosis With Polyangiitis (Wegener's) (GPA); Wegener Granulomatosis (WG); Microscopic Polyangiitis (MPA); ANCA-Associated Vasculitis (AAV); Vasculitis

Churg–Strauss Syndrome Presenting with Endobronchial Masses

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Veli Çetinsu

2015-12-01

Full Text Available Churg–Strauss syndrome is a condition with unknown etiology and asthma, allergic rhinitis, eosinophilic infiltration of blood and tissues, and transient infiltration of the lungs. It occurs mostly in the 3rd–4th decades of life with an incidence of 2.4/1000000. Presentation frequently involves nodular lung infiltrations, infiltrations with cavity, ground-glass appearance, and alveolar opacity. However, endobronchial mass is an unexpected presentation. In the current case report, we present a 45-year-old male patient who was receiving asthma therapy for 5 years. In the last follow-up visit, we identified a mass in the right hilum on X-ray radiography and performed fiberoptic bronchoscopy. Pathologic examination of biopsy material verified the diagnosis of Churg–Strauss syndrome. Bronchial mass is an unexpected presentation of Churg–Strauss syndrome and pathologic examination is essential to distinguish it from pulmonary malignancies

Unfavorably Altered Fibrin Clot Properties in Patients with Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss Syndrome): Association with Thrombin Generation and Eosinophilia.

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Mastalerz, Lucyna; Celińska-Lӧwenhoff, Magdalena; Krawiec, Piotr; Batko, Bogdan; Tłustochowicz, Witold; Undas, Anetta

2015-01-01

Given reports on the increased prevalence of thromboembolic incidents in patients with eosinophilic granulomatosis with polyangiitis (EGPA; Churg-Strauss syndrome), we investigated whether fibrin clot properties are unfavorably altered in EGPA. Ex vivo plasma fibrin clot characteristics, including clot permeability, turbidimetry and efficiency of fibrinolysis using two assays, were investigated in 34 consecutive patients with remission in EGPA according to the Birmingham Vasculitis Activity Score version 3 (23 female, 11 male), aged 48 (range, 21-80) years. The control group comprised 34 age- and sex- matched volunteers. Compared with controls, patients with EGPA were characterized by denser fiber clots (estimated pore size, Ks, 7.30±0.93 vs 10.14±1.07 10-9 cm2), faster fibrin polymerization (lag phase in a turbidimetric curve, 41.8±3.6 vs 47.4±2.9 s), thicker fibrin fibers (maximum absorbance, ΔAbs, 0.87±0.09 vs 0.72±0.07), higher maximum levels of D-dimer released from clots (DDmax 4.10±0.46 vs 3.54±0.35 mg/L), and prolonged clot lysis time (t50%; 9.50±1.45 vs 7.56±0.87 min); all p<0.0001. Scanning electron microscopy images confirmed denser plasma fibrin networks composed of thinner fibers formed in EGPA. Antineutrophil cytoplasmic antibody status and C-reactive protein did not affect clot variables. Multivariate analysis adjusted for fibrinogen showed that Ks was predicted by eosinophil count, peak thrombin generation, factor VIII, and soluble CD40 ligand, whereas eosinophil count, peak thrombin generation and antiplasmin predicted t50%. This study is the first to show that EGPA is associated with prothrombotic plasma fibrin clot phenotype, which may contribute to thromboembolic manifestations reported in this disease.

Unfavorably Altered Fibrin Clot Properties in Patients with Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss Syndrome: Association with Thrombin Generation and Eosinophilia.

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Lucyna Mastalerz

Full Text Available Given reports on the increased prevalence of thromboembolic incidents in patients with eosinophilic granulomatosis with polyangiitis (EGPA; Churg-Strauss syndrome, we investigated whether fibrin clot properties are unfavorably altered in EGPA.Ex vivo plasma fibrin clot characteristics, including clot permeability, turbidimetry and efficiency of fibrinolysis using two assays, were investigated in 34 consecutive patients with remission in EGPA according to the Birmingham Vasculitis Activity Score version 3 (23 female, 11 male, aged 48 (range, 21-80 years. The control group comprised 34 age- and sex- matched volunteers.Compared with controls, patients with EGPA were characterized by denser fiber clots (estimated pore size, Ks, 7.30±0.93 vs 10.14±1.07 10-9 cm2, faster fibrin polymerization (lag phase in a turbidimetric curve, 41.8±3.6 vs 47.4±2.9 s, thicker fibrin fibers (maximum absorbance, ΔAbs, 0.87±0.09 vs 0.72±0.07, higher maximum levels of D-dimer released from clots (DDmax 4.10±0.46 vs 3.54±0.35 mg/L, and prolonged clot lysis time (t50%; 9.50±1.45 vs 7.56±0.87 min; all p<0.0001. Scanning electron microscopy images confirmed denser plasma fibrin networks composed of thinner fibers formed in EGPA. Antineutrophil cytoplasmic antibody status and C-reactive protein did not affect clot variables. Multivariate analysis adjusted for fibrinogen showed that Ks was predicted by eosinophil count, peak thrombin generation, factor VIII, and soluble CD40 ligand, whereas eosinophil count, peak thrombin generation and antiplasmin predicted t50%.This study is the first to show that EGPA is associated with prothrombotic plasma fibrin clot phenotype, which may contribute to thromboembolic manifestations reported in this disease.

Serum biomarkers in patients with relapsing eosinophilic granulomatosis with polyangiitis (Churg-Strauss.

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Christian Dejaco

Full Text Available Previous studies suggest a role for eotaxin-3, TARC/CCL17 and IgG4 in newly-diagnosed patients with eosinophilic granulomatosis with polyangiitis (EGPA, Churg-Strauss with highly active disease. The role of these biomarkers in relapsing disease is unclear.Serum levels of TARC/CCL17, eotaxin-3, IgG4, and IgG4/IgG ratio were determined in serum samples from a longitudinal cohort of patients with EGPA (105 visits of 25 patients. Epidemiological, clinical and laboratory data were available for all visits.At the first visit, 80% of patients were using glucocorticoids and 68% additional immunosuppressive drugs. Disease flares were seen at 18 visits. The median BVAS and BVAS/WG scores at time of relapse were 4 and 2, respectively. None of the biomarkers tested were useful to discriminate between active disease and remission. Patients treated with prednisone had lower eotaxin-3 and eosinophil levels compared to patients not taking glucocorticoids irrespective of disease activity. Use of immunosuppressive agents was not associated with biomarker levels.Serum levels of TARC/CCL17, eotaxin-3, IgG4, and IgG4/IgG ratio do not clearly differentiate active and inactive disease in established EGPA. Defining biomarkers in EGPA remains a challenge especially during times of glucocorticoid use.

Joint Damage in ANCA-Associated Systemic Vasculitis. Report II: Wegener’s and Churg-Strauss Granulomatous Polyangiitis

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D.V. Pomazan

2016-08-01

Full Text Available Wegener’s (granulomatosis with polyangiitis, GPA and Churg-Strauss (eosinophilic polyangiitis, EPA vasculitis are treated as a single variant of systemic necrotizing granulomatosis vasculitis, associated with anti-neutrophil cytoplasmic antibodies (ANCA. There is an urgent need for further study of articular syndrome in patients with ANCA-SV. Objective: to evaluate the incidence and nature of the lesion of the joints at GPA and EPA, connection with extra-articular signs of the disease. Material and methods. The study involved 58 patients with ANCA-SV, among which there were 28 patients with GPA (16 men and 12 women aged from 17 to 70 years old and 30 with EPA (14 men and 16 women aged 19–70 years old.The average duration of the disease in the first and second groups was 4 years and 11 years, respectively. I, II and III degree activity of the GPA were in the ratio 1 : 6 : 7 and in the cases of EPA — 1 : 3 : 4. The lung pathology was diagnosed in all cases with EPA, in patients with GPA — in 68 % of cases. In addition, 2.3 times less frequently cutaneous syndrome was detected. Results. The lesion of the joints in the form of arthritis or arthralgia occurs in 1/2 of the number of patients with granulomatous ANCA-SV in the ratio of HPA to EPA as 1 : 2, which is associated with the severity of extra-articular manifestations of disease, and in cases of EPA — with the level of antibodies to proteinase-3. Patients with EPA significantly more frequently had lesions of the maxillary joints, digital joints of foot, ankle, metatarsophalangeal, hip, sacroiliac and vertebral, and the last 4 were not diagnosed in patients with HPA. Epiphyseal osteoporosis, subchondral sclerosis, osteocytes, artrocalcinosis and changes of the menisci horns of the knee-joints were observed only at EPA. Conclusions. The severity of arthropathy prevails in EPA, compared to GPA, that is associated with great inflammatory degenerative changes of the articular, but intra

VASCULITIS DE CHURG-STRAUSS: PRESENTACIÓN CLÍNICA COMO GLOMERULONEFRITIS EXTRACAPILAR NECROTIZANTE PAUCI-INMUNE CON NEFRITIS TUBULO-INTERSTICIAL EOSINOFÍLICA.

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Fernanda da Cunha

2004-01-01

Full Text Available RESUMEN: Se presenta el caso clínico de una mujer de 81 años con antecedentes patología pulmonar obstructiva crónica "idiopática" que desarrolló un cuadro de insuficiencia renal aguda aparentemente prerrenal, con posterior oliguria y eosinofilia simulando una nefropatía túbulo-intersticial alérgica. La evolución atípica y la presencia de p-ANCA, sugerían una vasculitis, patología que se confirmó con la biopsia renal. Los resultados anatomopatológicos revelaron la existencia de una glomerulonefritis necrotizante con semilunas e infiltrado eosinófilo. Estos datos y el historial de la paciente llevaron al diagnóstico de vasculitis de Churg-Strauss. El tratamiento inicial con corticoides y ciclofosfamida y posteriormente con Azatioprina mostró excelentes resultados con mejoría de la función renal, de los parámetros inflamatorios y de la clínica sistémica, que la paciente mantuvo posteriormente. ABSTRACT: We report a case of a 81 year-old woman with idiopathic chronic obstructive pulmonary disease, who developed a functional acute renal failure with delayed oliguria and eosinophily, simulating an acute interstitial nephropathy. The unusual clinical course and the presence of antimyeloperoxidase antibodies (p-ANCA suggested a vasculitis; the renal biopsy confirmed this diagnosis. The histology revealed a crescentic glomerulonephritis with eosinophilic infiltration. With these data and the pulmonary history of the patient, a diagnosis of Churg-Struss Vasculitis was made. The initial treatment with steroids and ciclophosphamide switched later to azatioprine, was succeeded with a sustained improvement in renal function, inflammatory markers and clinical course.

Low Dose Naltrexone to Improve Physical Health in Patients With Vasculitis

Science.gov (United States)

2018-06-08

Eosinophilic Granulomatosis With Polyangiitis (EGPA); Churg-Strauss Syndrome (CSS); Giant Cell Arteritis; Granulomatosis With Polyangiitis; Microscopic Polyangiitis; Polyarteritis Nodosa; Takayasu Arteritis

Educational Needs of Patients With Systemic Vasculitis

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2014-07-11

Behcet's Disease; Churg-Strauss Syndrome; Vasculitis, Central Nervous System; Giant Cell Arteritis; Wegener Granulomatosis; Henoch-Schoenlein Purpura; Microscopic Polyangiitis; Polyarteritis Nodosa; Takayasu's Arteritis

Pediatric Vasculitis Initiative

Science.gov (United States)

2017-05-01

Wegeners Granulomatosis (Granulomatosis With Polyangiitis); Microscopic Polyangiitis; Churg Strauss Syndrome (Eosinophilic Granulomatosis With Polyangiitis); Polyarteritis Nodosa; Takayasu Arteritis; Primary CNS Vasculitis; Unclassified Vasculitis

A nationwide survey on the epidemiology and clinical features of eosinophilic granulomatosis with polyangiitis (Churg-Strauss) in Japan.

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Sada, Ken-Ei; Amano, Koichi; Uehara, Ritei; Yamamura, Masahiro; Arimura, Yoshihiro; Nakamura, Yoshikazu; Makino, Hirofumi

2014-07-01

We conducted a cross-sectional nationwide survey to determine eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA) prevalence and clinical features in Japan. Data for EGPA patients in 2008 were collected from 1,564 hospitals. In total, 965 patients were reported from 365 departments. In a second survey, clinical data for 473 patients were obtained. We estimated that 1,866 (95% CI: 1,640-2,092) patients have EGPA in Japan (prevalence, 17.8/1,000,000). Of the 473 patients in the second survey, 315 fulfilled American College of Rheumatology (ACR) criteria or Lanham's criteria for EGPA. The mean age (± SD) of the 315 at onset was 55 ± 14 years, male to female ratio 1:2. 93% of patients had neurological manifestations, which were the organ system most frequently involved. Among 277 patients tested for myeloperoxidase (MPO)-/p anti-neutrophil cytoplasmic antibody (ANCA), 139 (50%) were positive, while only 6 of 238 were positive for proteinase3 (PR3)-/cANCA. MPO-ANCA-positive patients had renal involvement, mucous membrane or ophthalmological symptoms, and ENT symptoms more frequently, whereas cutaneous lesions and cardiovascular involvement were less common. The prevalence of EGPA and the frequency of MPO-/p-ANCA-positivity in Japanese EGPA patients were mostly similar to those of Western countries. However, female predominance and a high frequency of neurological manifestations characterized Japanese patients.

Reproductive Health in Men and Women With Vasculitis

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2014-06-25

Giant Cell Arteritis; Takayasu's Arteritis; Polyarteritis Nodosa; Wegener's Granulomatosis; Microscopic Polyangiitis; Churg-Strauss Syndrome; Behcet's Disease; Kawasaki Disease; Henoch-schoenlein Purpura; Vasculitis, Central Nervous System; Drug-induced Necrotizing Vasculitis

VCRC Tissue Repository

Science.gov (United States)

2018-04-23

Aortitis; Cutaneous Vasculitis; Eosinophilic Granulomatosis With Polyangiitis; Giant Cell Arteritis; Granulomatosis With Polyangiitis (Wegener's); Henoch-Schonlein Purpura; IgA Vasculitis; Microscopic Polyangiitis; Polyarteritis Nodosa; Takayasu Arteritis; Churg-Strauss Syndrome

СИНДРОМ ЧАРДЖ-СТРОССА В СОЧЕТАНИИ С АУТОИММУННОЙ ТРОМБОЦИТОПЕНИЕЙ. 20 ЛЕТ НАБЛЮДЕНИЯ

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Д. Л. Виноградов

2015-01-01

Full Text Available The description of a rare combination syndrome Churg-Strauss syndrome (CSS with autoimmune thrombocytopenia. The disease is characterized by slow progression and refractory to standard types of immunosuppressive therapy. The first positive results are used prolonged courses of immunosuppressive of dipeptide thymodepressin

Takayasu's Arteritis

Science.gov (United States)

... of an active inflammatory illness. These may include “constitutional symptoms” (fever, fatigue, weight loss), arthritis, and non- ... Disease Eosinophilic Granulomatosis with Polyangiitis, formerly Churg-Strauss Syndrome (EGPA) Cryoglobulinemia Giant Cell Arteritis Henoch-Schönlein Purpura ...

New advances in the pathogenesis of ANCA-associated vasculitides

NARCIS (Netherlands)

Chen, M.; Kallenberg, C. G. M.

2009-01-01

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are a group of autoimmune disorders including Wegener granulomatosis (WG), microscopic polyangiitis (MPA), Churg-Strauss syndrome (CSS) and renal-limited vasculitis (RLV). This paper reviews updated information on the

Pathogenesis of PR3-ANCA associated vasculitis

NARCIS (Netherlands)

Kallenberg, C. G. M.

2008-01-01

Wegener's Granulomatosis (WG) is closely associated with antineutrophil cytoplasmic autoantibodies (ANCA), particularly those directed to proteinase 3 (PR3). ANCA directed to myeloperoxidase (MPO) are associated with microscopic polyangiitis (MPA) and the Churg Strauss syndrome. PR3-ANCA associated

Think twice – Diagnostic delay in a patient with acute chest pain

DEFF Research Database (Denmark)

Bang, Caecilie Larsen; Porsbjerg, Celeste

2016-01-01

to consider alternative diagnoses in patients with atypical symptoms and a low risk profile. Heart involvement is the most critical and potentially lethal systemic manifestation in eosinophilic granulomatosis with polyangiitis (EGPA, formerly known as Churg-Strauss syndrome), which makes a quick diagnosis...

Vasculitis Pregnancy Registry

Science.gov (United States)

2018-04-30

Vasculitis; Behcet's Disease; CNS Vasculitis; Cryoglobulinemic Vasculitis; Eosinophilic Granulomatosis With Polyangiitis (EGPA); Churg-Strauss Syndrome (CSS); Granulomatosis With Polyangiitis (GPA); Wegener's Granulomatosis; IgA Vasculitis; Henoch-Schoenlein Purpura (HSP); Microscopic Polyangiitis (MPA); Polyarteritis Nodosa (PAN); Takayasu Arteritis (TAK); Urticarial Vasculitis; Systemic Vasculitis

Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome) presenting as diffuse myositis.

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Parent, Marc-Etienne; Larue, Sandrine; Ellezam, Benjamin

2014-11-21

Eosinophilic granulomatosis with polyangiitis is a complex multisystemic syndrome with heterogeneous presentation. Most often, there is a clinical history of asthma or other atopic conditions, and current presentation generally includes signs of cutaneous or pulmonary involvement. Very few reports described myalgia or weakness as the chief complaint. Of these, only a few included muscle biopsy evaluation and none showed convincing evidence of primary myositis. We believe this report is the first to demonstrate true myositis in the setting of early eosinophilic granulomatosis with polyangiitis. This report describes a 74 year old Caucasian man, with no known allergies, presenting severe myalgia, muscle weakness, jaw claudication, and fever. Blood work showed marked eosinophilia and high creatine kinase levels. Biceps brachialis muscle biopsy revealed eosinophilic necrotizing vasculitis and true myositis with myophagocytosis of non-necrotic fibers and strong sarcolemmal MHC-1 overexpression by immunohistochemistry. This patient was successfully treated with prednisone and azathioprine. Our finding of true myositis in a case of eosinophilic granulomatosis with polyangiitis suggests that primary auto-immunity against muscle fibers, distinct from the secondary effects of vasculitis, can occur in this entity and may represent an overlap syndrome. Early recognition of eosinophilic granulomatosis with polyangiitis in patients presenting with myositis may provide an opportunity to treat the vasculitis before onset of severe multisystemic disease. We recommend the use of muscle biopsy with immunohistochemistry for MHC-1 to confirm the diagnosis of myositis in the setting of eosinophilic granulomatosis with polyangiitis.

Case report

African Journals Online (AJOL)

abp

2012-06-11

Jun 11, 2012 ... Abstract. Churg Strauss syndrome is a rare systemic and pulmonary vasculitis exceptionally associated with AA amyloidosis. We report the case of a 65-year old woman with past medical history of asthma. She developed polyarthralgia, headache and purpura. A laboratory workout found hypereosinophilia ...

Churg–Strauss syndrome associated with antiphospholipid antibodies in a patient with recurrent myocardial and cerebral ischemia

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Paroli M

2012-11-01

Full Text Available Marino Paroli,1 Alessandro Polidoro,1 Simone Romano,1 Daniele Accapezzato21Department of Biotechnology and Medical-Surgical Sciences, 2Department of Internal Medicine and Medical Specialties, Sapienza University of Rome, Rome, ItalyAbstract: We report on a case of Churg–Strauss syndrome (CSS associated with the presence of antiphospholipid antibodies. The patient had a history of recurrent myocardial infarction and presented with acute ischemic cerebral disease. Eosinophilia with typical lung and skin lesions led us to diagnose the patient with CCS. We hypothesize that the presence of antiphospholipid antibodies significantly contributed to the ischemic events. We suggest that the search for antiphospholipid antibodies should be included in the laboratory work-up in CSS patients and patients affected by primary systemic vasculitides in general. Moreover, anticoagulant treatment appears to be warranted in all CSS patients and antiphospholipid antibodies to counteract this thrombosis-favoring association.Keywords: Churg–Strauss syndrome, antiphospholipid antibodies, ischemic disease

Anti-IgE Monoclonal Antibody (Omalizumab) in Refractory and Relapsing Eosinophilic Granulomatosis With Polyangiitis (Churg-Strauss): Data on Seventeen Patients.

Science.gov (United States)

Jachiet, Marie; Samson, Maxime; Cottin, Vincent; Kahn, Jean-Emmanuel; Le Guenno, Guillaume; Bonniaud, Philippe; Devilliers, Hervé; Bouillet, Laurence; Gondouin, Anne; Makhlouf, Fatma; Meaux-Ruault, Nadine; Gil, Helder; Bienvenu, Boris; Coste, André; Groh, Matthieu; Giraud, Violaine; Dominique, Stéphane; Godeau, Bertrand; Puéchal, Xavier; Khouatra, Chahera; Ruivard, Marc; Le Jeunne, Claire; Mouthon, Luc; Guillevin, Loïc; Terrier, Benjamin

2016-09-01

To describe the efficacy and safety of omalizumab, an anti-IgE monoclonal antibody, in patients with refractory and/or relapsing eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA). We conducted a nationwide retrospective study including EGPA patients who received omalizumab. Response was defined as the absence of asthma and/or sinonasal exacerbations with a prednisone dosage of ≤7.5 mg/day (complete response) or >7.5 mg/day (partial response). Seventeen patients (median age 45 years) received omalizumab for severe steroid-dependent asthma (88%) and/or sinonasal involvement (18%). After a median follow-up of 22 months, 6 patients (35%) achieved a complete response, 5 patients (30%) achieved a partial response, and 6 patients (35%) had no improvement. The median Birmingham Vasculitis Activity Score decreased from 2.5 at baseline to 0.5 at 12 months. The median number of exacerbations per month decreased from 1 at baseline to 0 at 12 months, and the median forced expiratory volume in 1 second increased from 63% of the percent predicted at baseline to 85% of the percent predicted at 12 months. The median prednisone dosage decreased from 16 mg/day at baseline to 11 mg/day at 6 months and 9 mg/day at 12 months. Omalizumab was discontinued in 8 patients (47%) during follow-up, because of remission (12.5%), adverse event despite disease remission (12.5%), refractory disease (25%), or relapse (50%). Relapses included retrobulbar optic neuritis attributable to EGPA in 2 patients and severe asthma flare in 2 others. The results of this study suggest that omalizumab may have a corticosteroid-sparing effect in EGPA patients with asthmatic and/or sinonasal manifestations, but reducing the corticosteroid dose may also increase the risk of severe EGPA flares, which raises the question of the safety of omalizumab in patients with EGPA. © 2016, American College of Rheumatology.

A cross-sectional study of the Birmingham Vasculitis Activity Score version 3 in systemic vasculitis

DEFF Research Database (Denmark)

Suppiah, Ravi; Mukhtyar, Chetan; Flossmann, Oliver

2011-01-01

and the vasculitis damage index (VDI) to demonstrate that the BVAS v. 3 measures disease activity. Results. WG (63%), Churg-Strauss syndrome (9%) and microscopic polyangiitis (9%) were the most common diagnoses. The BVAS v. 3 showed convergent validity with the VAI [¿¿=¿0.82 (95% CI 0.77, 0.85)], PGA [¿¿=¿0.85 (95...

Veno-occlusive disease of the colon - CT findings

International Nuclear Information System (INIS)

Rademaker, J.

1998-01-01

Venous occlusion is a rare cause of ischemic bowel disease and is usually brought about by thrombosis that may occur as a complication of systemic disorders like systemic lupus erythematosus, Behcet disease or Churg-Strauss syndrome. This report describes a patient with veno-occlusive disease of the colon caused by lymphocytic phlebitis. Typical CT findings included homogeneous bowel wall thickening and vascular engorgement. (orig.) (orig.)

Kampo medicines improved blood test and QOL in two vasculitis cases of Churg–Strauss syndrome and Henoch–Shönlein purpura after inadequate treatment with conventional therapies

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Yasuyo Hijikata

2010-02-01

Full Text Available Yasuyo Hijikata1, Yumiko Ikemoto2, Urara Kohdera31Toyodo Hijikata Clinic, Osaka, Japan; 2St Mary’s Hospital, Himeji, Japan; 3Nakano Children’s Hospital, Osaka, JapanObjective: Based on the tenets of traditional Chinese medicine (TCM theory, Kampo medicines were selected and applied to two cases of Churg–Strauss syndrome and Henoch–Shönlein purpura. Two vasculitis syndrome patients exhibited persistent symptoms and abnormal blood tests after treatment with conventional therapies.Methods: As the two cases had “blood stagnation” and “damps and heat” and one had a “yang deficiency” in terms of TCM theory, we applied certain selected Kampo medicines.Results: In case 1, the patient presented with hypereosinophilia, venous thrombosis, pulmonary infarction, decreased platelet count, ulner nerve palsy and Raynaud’s phenomena, which led to a diminished quality of life. After starting the Kampo medicines, the patient improved quickly and recovered within 11 months. In case 2, persistent purpura, abdominal pain, and bloody feces quickly improved and disappeared after Kampo treatment. After starting the Kampo medicines, prednisolone was stopped at 21 days without any sign of relapse to date.Conclusion: Kampo medicines helped clear the persistent abnormal symptoms and laboratory findings of vasculitis syndromes, Churg–Strauss syndrome and Henoch–Shönlein purpura, which had responded inadequately to the conventional therapies administered.Keywords: Kampo medicine, vasculitis, Churg–Strauss syndrome, Henoch–Shönlein purpura

Veno-occlusive disease of the colon - CT findings

Energy Technology Data Exchange (ETDEWEB)

Rademaker, J [Department of Radiology I, Medical School Hannover (Germany)

1998-10-01

Venous occlusion is a rare cause of ischemic bowel disease and is usually brought about by thrombosis that may occur as a complication of systemic disorders like systemic lupus erythematosus, Behcet disease or Churg-Strauss syndrome. This report describes a patient with veno-occlusive disease of the colon caused by lymphocytic phlebitis. Typical CT findings included homogeneous bowel wall thickening and vascular engorgement. (orig.) (orig.) With 1 fig., 4 refs.

Manifestações otorrinolaringológicas nas doenças reumáticas auto-imunes Otorhinolaryngologic manifestations of autoimmune rheumatic diseases

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Daniela Bergamim Pereira

2006-04-01

systemic lupus erythematosus, Wegener's granulomatosis, relapsing polychondritis, polyarteritis nodosa, Cogan's syndrome, Sjögren's syndrome, and less frequently in Churg-Strauss syndrome and Behçet's disease. Nose and paranasal sinuses are variably affected during the course of Wegener's granulomatosis, Churg-Strauss syndrome, relapsing polychondritis and sarcoidosis. Recurrent mucosal ulcerations are common in systemic lupus erythematosus and Behçet's disease. Xerostomia is a common feature of primary and secondary Sjögren's syndrome; salivary gland enlargement may be also seen in these patients, as well as in patients with sarcoidosis. The cricoarytenoid joint can be involved during the course of rheumatoid arthritis, ankylosing spondylitis and gout; osteoarthritic changes have also been described. Trigeminal nerve dysfunction may occur in patients with Sjögren's syndrome, systemic sclerosis, systemic lupus erythematosus and mixed connective tissue disease. Peripheral facial nerve palsy has been described to complicate the course of Sjögren's syndrome and sarcoidosis.

Churg-Strauss's Syndrome; Review of literature | Adefuye | Nigerian ...

African Journals Online (AJOL)

Diffuse organ involvement, especially cardiovascular (CVS), GIT, rare involvement of CNS and renal system suggest poorer prognosis and possible fatal outcome. Patients with these poor prognostic outlooks, especially cardiac, receive aggressive treatment using corticosteroids combined with immunosuppresants (chiefly ...

Risk factors of atherosclerosis and clinical and morphological comparisons in systemic vasculitides

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Leonid Aleksandrovich Strizhakov

2012-01-01

Full Text Available Objective: to study the incidence rates of angina, myocardial infarction (MI, stroke, and the frequency of endovascular interventions in patients with systemic vasculitides, and the incidence rate of atherosclerosis according to autopsy data. Subjects and methods. Three hundred and twenty-one patients with systemic vasculitides: Wegener's granulomatosis (n = 138, Takayasu's arteritis (n = 79, polyarteritis nodosum (n = 55, and Churg-Strauss syndrome (n = 49 were examined; 55 autopsies were analyzed in patients with the above diseases. Results. Fifty-one (15.6% of the 321 patients were diagnosed as having cardiovascular diseases (CVD: angina pectoris (7.1% and MI (3.1% and endovascular interventions (0.9%. The risk of cardiovascular events was found to be associated with traditional risk factors, such as male gender and age. Arterial hypertension, hypercholesterolaemia, and increased serum creatinine were more frequently detected in the CVD group that showed no significant differences from the non-CVD group. According to autopsy results, atherosclerosis was identified in the patients with Wegener's granulomatosis (52%, Takayasu's arteritis (50%, polyarteritis nodosum (52.6%, and Churg-Strauss syndrome (57.1%. Conclusion. CVD and atherosclerosis are common in systemic vasculitides, which requires the traditional risk factors of atherosclerosis to be actively corrected.

Response of Complex Undefined Hypereosinophilic Syndrome to Treatment with Imatinib.

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Yılmaz, İnsu; Kaynar, Leylagül; Tutar, Nuri; Pala, Çiğdem; Canöz, Özlem; Yıldırım, Afra; Büyükoğlan, Hakan; Gülmez, İnci

2016-07-01

Hypereosinophilic syndomes (HESs) include potentially lethal multisystem disorders characterized by eosinophilic infiltration of a variable spectrum of target organs, predominantly the skin, heart, lungs, gastrointestinal tract, and nervous system. Based on recent advances in molecular and genetic diagnostic techniques and increasing experience with differences in clinical features and prognosis, subtypes have been defined, including "myeloproliferative-HES ", "lymphocytic-HES", "familial eosinophilia", "overlap HES", "undefined HES" ("complex undefined HES", "simple undefined HES", "episodic undefined HES") and "eosinophil associated diseases" (such as Churg-Strauss syndrome). HES should be kept in mind in the differential diagnosis of eosinophilic lung diseases especially in patients with peripheral eosinophilia and pulmonary infiltrates. Corticosteroids represent an effective firstline approach to decreasing eosinophil counts in the majority of cases. Imatinib might be used for corticosteroid nonresponders. We herein report a patient with "complex undefined HES" who had disease resistant to corticosteroids, but who had a significant response after treatment with imatinib.

Necrobiotic granuloma: An update

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Achyut Pokharel

2018-01-01

Full Text Available Granulomatous disorders comprise a large family sharing the common histological denominator of granuloma formation. Collagenolytic or necrobiotic granuloma is one in which a granulomatous infiltrate develops around a central area of altered collagen and elastic fibers. The altered fibers exhibit new staining patterns, becoming either more basophilic which gives blue appearance (“blue granuloma” or eosinophilic giving red appearance (“red granuloma”. Conditions which exhibit blue granuloma include granuloma annulare, Wegener's granulomatosis, and rheumatoid vasculitis, whereas red granulomas include necrobiosis lipoidica, necrobiosis xanthogranuloma, rheumatoid nodules, Churg-Strauss syndrome, and eosinophilic cellulitis (Well's syndrome.

Eosinofilia pulmonar Pulmonary eosinophilia

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Luiz Eduardo Mendes Campos

2009-06-01

and treatment. Clinical and radiological presentations can include simple pulmonary eosinophilia, chronic eosinophilic pneumonia, acute eosinophilic pneumonia, allergic bronchopulmonary aspergillosis and pulmonary eosinophilia associated with a systemic disease, such as in Churg-Strauss syndrome and hypereosinophilic syndrome. Asthma is frequently concomitant and can be a prerequisite, as in allergic bronchopulmonary aspergillosis and Churg-Strauss syndrome. In diseases with systemic involvement, the skin, the heart and the nervous system are the most affected organs. The radiological presentation can be typical, or at least suggestive, of one of three types of pulmonary eosinophilia: chronic eosinophilic pneumonia, acute eosinophilic pneumonia and allergic bronchopulmonary aspergillosis. The etiology of pulmonary eosinophilia can be either primary (idiopathic or secondary, due to known causes, such as drugs, parasites, fungal infection, mycobacterial infection, irradiation and toxins. Pulmonary eosinophilia can be also associated with diffuse lung diseases, connective tissue diseases and neoplasia.

CUTANEOUS VASCULITIDES AND CUTANEOUS MANIFESTATIONS OF SYSTEMIC VASCULITIDES

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Vesna Jurčić

2009-06-01

plexes with complement activation. Typical representatives are Henoch-Schönlein purpura, cryoglobulinemic vasculitis and vasculitis in systemic lupus erythematosus. Necrotizing arteriolitis and arteritis of small arteries are usually found in ANCA positive vasculitides. Medium-sized arteries can be involved in both ANCA positive vasculitides and polyarteritis nodosa. Conclusions This article presents the clinical picture, histopathologic characteristics, pathogenetic mechanisms and treatment of some clinicopathologic entities of vasculitides, such as Henoch-Schönlein purpura, urticarial vasculitis, cryoglobulinemic vasculitis, microscopic polyangiitis, Wegener\\'s granulomatosis, Churg-Strauss syndrome and polyarteritis nodosa.

Levi-Strauss, Claude (1908-2009)

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Bouchet, Dominique

2015-01-01

Before Lévi-Strauss, anthropology was merely empirical and mostly interested in identifying different cultures’ contextual framework. Lévi-Strauss is not just interested in understanding and explaining different cultures from within. He wants to figure out how humans think fundamentally and from...... there to understand cultural diversity. Lévi-Strauss is the first anthropologist to connect cultural diversity with the unity of humans, that is to say, with human nature. All humans do not think the same, neither do they unite in the same way, but they compose in the same way. They have the same propensity to create...

[Non allergic simple eosinophilic pneumonia--Löffler syndrome--a case report study].

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Meta-Jevtović, Ivana; Tomović, Miroslav S; Mojsilović, Slavica; Petrović, Marina

2008-01-01

Löffler syndrome is an acute, pneumonia of unknown ethiology. This disease is not often associated with bronchial asthma. In its asymptomatic form, this disease is reversible, transient, self-limited with no requests for specific therapy regimen. In the symptomatic form, as well as during its progression, treatment with steroids is very effective. Furthermore, in both acute eosinophilic and idiopathic chronic eosinophilic form, this kind of therapy ensures survival. The case of a 53-year-old Caucasian woman was presented with 2-month history of low grade fever, shortness of breath, cough and reduced exercise tolerance. Although she had an allergic accident on insects in history, non allergy reactions as well as an obstructive disease with that kind of origin were not detected on admission. The diagnosis of simple eosinophilic pneumonia (SEP) (Löffler's syndrome) was confirmed by transbronchial biopsy and by sternal testing. The peripheral blood eosinophilia with pulmonary eosinophilic infiltrates on X ray chest radiography were observed during clinical examination. Biopsy specimen of the lung parenchym showed changes associated with Löffler's syndrome. The diagnosis was, also, confirmed according to the radiographic findings of unilateral migratory infiltrates consistent pneumonia. Churg Strauss syndrome (CSS) has to be considered in this differential diagnosis. Frequently, this disease has extrinsic bronchial asthma with eosinophilic pneumonia in history: asthma is often associated with allergic bronchopulmonary aspergillosis. In the reported case, treatment with steroids resulted in a marked clinical improvement compared to nonsteroid therapy.

Vasculites dos médios e grandes vasos Medium and large vessel vasculitis

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Hebert Roberto Clivati Brandt

2009-02-01

Full Text Available As vasculites são constituídas por um grande grupo de síndromes caracterizadas por inflamação e necrose da parede dos vasos sangüíneos, resultando em estreitamento ou oclusão do lúmen. A distribuição dos vasos envolvidos varia consideravelmente e serve de base para a classificação das síndromes vasculíticas: grandes vasos (arterite de Takayasu, arterite temporal; pequenos e médios vasos (poliarterite nodosa, síndrome de Churg-Strauss, granulomatose de Wegener, vasculites nas doenças reumáticas; e pequenos vasos (vasculite de hipersensibilidade, púrpura de Henoch-Schonlein, poliangeíte microscópica, crioglobulinemia. Este artigo revisa as vasculites dos médios e grandes vasos e opções terapêuticas atuais; além de oferecer orientação sobre abordagem diagnóstica e tratamento dos pacientes com suspeita de vasculite cutânea.Vasculitis comprises a broad group of syndromes characterized by inflammation and necrosis on the walls of blood vessels, resulting in narrowing or occlusion of the lumen. The distribution of involved blood vessel varies considerably and serves as the basis for one classification of vasculitic syndromes: large vessels (Takayasu arteritis, giant cell arteritis; medium and small muscular arteries (polyarteritis nodosa, Churg-Strauss syndrome, Wegener's granulomatosis, vasculitis in rheumatic diseases; and small vessels ("hypersensitivity" vasculitis, Henoch-Schonlein purpura, microscopic polyangiitis, cryoglobulinemia. This article reviews medium and large vessel vasculitis and current treatment options; it also presents a comprehensive approach to diagnosing and treating patients with suspected cutaneous vasculitis.

Eosinophilic fasciitis

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Karolina Niklas

2015-01-01

Full Text Available Eosinophilic fasciitis is a rare connective tissue disease with unclear etiology and pathogenesis. It is classified as a scleroderma-like syndrome. The disease is characterized by fibrosis of the skin and subcutaneous tissues with significant thickening of fascia. Visceral involvement is rare. Characteristic feature in laboratory tests is peripheral blood eosinophilia. Differential diagnosis should be performed, including ruling out systemic sclerosis, nephrogenic systemic fibrosis, eosinophilia-myalgia syndrome, scleromyxedema, hypereosinophilic syndrome or Churg-Strauss syndrome. Final diagnosis is confirmed by histopathological examination. In treatment of the disease corticosteroids and/or immunosuppressive drugs are used. Some other drugs showed activity in this disease e.g. dapsone, infiximab or rituximab. Prognosis is rather good but sometimes a long-term treatment is necessary. In this paper we summarized the current knowledge on eosinophilic fasciitis.

Atualização do tratamento das vasculites associadas a anticorpo anticitoplasma de neutrófilos Treatment of antineutrophil cytoplasmic antibody-associated vasculitis: update

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Alfredo Nicodemos Cruz Santana

2011-12-01

Full Text Available As vasculites antineutrophil cytoplasmic antibody (ANCA, anticorpo anticitoplasma de neutrófilos associadas (VAAs são caracterizadas por uma inflamação sistêmica das artérias de pequeno e médio calibre (especialmente no trato respiratório superior e inferior, e nos rins. As VAAs compreendem a granulomatose de Wegener (agora chamada de granulomatose com poliangeíte, poliangeíte microscópica, VAA limitada ao rim e a síndrome de Churg-Strauss. Neste artigo, discutiremos as fases de tratamento dessas vasculites, como fase de indução (com ciclofosfamida ou rituximab e fase de manutenção (com azatioprina, metotrexato ou rituximab. Além disso, discutiremos como manusear os casos refratários à ciclofosfamida.In its various forms, antineutrophil cytoplasmic antibody (ANCA-associated vasculitis (AAV is characterized by a systemic inflammation of the small and medium-sized arteries (especially in the upper and lower respiratory tracts, as well as in the kidneys. The forms of AAV comprise Wegener's granulomatosis (now called granulomatosis with polyangiitis, microscopic polyangiitis, renal AAV, and Churg-Strauss syndrome. In this paper, we discuss the phases of AAV treatment, including the induction phase (with cyclophosphamide or rituximab and the maintenance phase (with azathioprine, methotrexate, or rituximab. We also discuss how to handle patients who are refractory to cyclophosphamide.

Early Outcomes in Children With Antineutrophil Cytoplasmic Antibody-Associated Vasculitis

DEFF Research Database (Denmark)

Morishita, Kimberly A; Moorthy, Lakshmi N; Lubieniecka, Joanna M

2017-01-01

diagnosed before their eighteenth birthday as having granulomatosis with polyangiitis (Wegener's), microscopic polyangiitis, eosinophilic granulomatosis with polyangiitis (Churg-Strauss), or ANCA-positive pauci-immune glomerulonephritis. The primary outcome measure was achievement of disease remission...

Peripheral Ulcerative Keratitis Associated with Autoimmune Disease: Pathogenesis and Treatment

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Yan Cao

2017-01-01

Full Text Available Peripheral ulcerative keratitis (PUK is type of crescent-shaped inflammatory damage that occurs in the limbal region of the cornea. PUK is always combined with an epithelial defect and the destruction of the peripheral corneal stroma. PUK may have a connection to systemic conditions, such as long-standing rheumatoid arthritis (RA, systemic lupus erythematosus (SLE, Wegener granulomatosis (WG, relapsing polychondritis, classic polyarteritis nodosa and its variants, microscopic polyangiitis, and Churg-Strauss syndrome. However, the most common connection is with RA, which is also the focus of this review. The pathogenesis of PUK is still unclear. It is thought that circulating immune complexes and cytokines exert an important influence on the progression of this syndrome. Treatment is applied to inhibit certain aspects of PUK pathogenesis.

Eosinophilic Granulomatosis with Polyangiitis and Diffuse Gastrointestinal Involvement

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Diana L. Franco

2014-10-01

Full Text Available Eosinophilic granulomatosis with polyangiitis (EGPA, formerly named Churg-Strauss syndrome, is a rare systemic small- and medium-sized-vessel vasculitis, characterized by the presence of severe asthma as well as blood and tissue eosinophilia. Gastrointestinal (GI symptoms, like diarrhea and abdominal pain, are common; however, there are few reports of histologic evidence of GI involvement. We report the case of a patient on treatment for EGPA who presented with recurrent small bowel obstruction and choledocholithiasis. Biopsies of the esophagus, small bowel and common bile duct showed diffuse eosinophilia, with clear EGPA in the GI tract. Improved awareness of GI EGPA may allow for timely management of this disorder.

THE 2015 INTERNATIONAL GUIDELINES FOR THE DIAGNOSIS AND TREATMENT OF EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS

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T. V. Beketova

2016-01-01

Full Text Available In 2015, guidelines for the diagnosis and treatment of eosinophilic granulomatosis with polyangiitis, formerly known as Churg-Strauss syndrome, were prepared with the participation of experts from 5 countries of Europe, the USA, and Canada, who accumulated scientific achievements and currently gained clinical experience. The proposed guidelines should not be regarded as final standards, but must become a guide for the selection of a personification strategy for managing patients with eosinophilic granulomatosis with polyangiitis and serve as a starting point for further investigations. The purposes of the publication are general characterization of the key points of the guidelines and discussion of some debate problems. 

As duas naturezas de Lévi-Strauss

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Philippe Descola

2011-11-01

Full Text Available Aborda-se aqui a complexidade do status do par conceitual natureza e cultura no pensamento de Lévi-Strauss. Ao mesmo tempo ferramenta de análise, cena filosófica dos primórdios e antinomia a superar, revisita-se os diferentes usos e significados na obra de Lévi-Strauss do conceito de natureza e sua relação com o de cultura. Mostra-se como é possível reconhecer na obra de Lévi-Strauss dois conceitos de natureza: por um lado, uma natureza que se opõe à cultura num programa científico formulado em termos classicamente dualistas e, por outro, uma teoria do conhecimento decididamente monista que considera o espírito como parte e produto desse mesmo mundo. Argumenta-se que se o dualismo entre cultura e natureza fundou o pensamento estruturalista de Lévi-Strauss, é na própria obra deste que encontramos os argumentos e meios de superá-lo. A vocação do estruturalismo na antropologia de hoje, no entanto, é de ir mais longe neste caminho do que foi o próprio fundador.

Levi-Strauss : UNESCO = anthropology : politics

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Ljiljana Gavrilović

2016-02-01

Full Text Available This paper discusses Levi-Strauss engagement in UNESCO activities. On the one hand, Levi-Strauss engagement in Pakistan had an important impact on his later work, as well as on his overall view about the world and culture( s; while on the other, Levi-Strauss’s definitions of the relationship between race/nation and culture had an important influence on the ways in which UNESCO dealt with the ideas about culture and on the ways these ideas were implemented in its global strategies of action in/towards culture. On the one hand, this engagement included a delivery of the European world view (as a superior one to everyone who does not have it; while on the other, it also meant a protection of material remains of different cultures world-wide and protection of non-material heritage and/as entire "Othern-ness", whenever it is located, and (at least in theory however it looks like. In that sense, Levi- Strauss public engagement had an important impact not only on anthropology, as a discipline that he dedicated his life to, but also to the formation of contemporary understanding of politically correct relationship towards the "Other".

Theory, Practice, and Modernity: Leo Strauss on Rousseau's Epicureanism.

Science.gov (United States)

Holley, Jared

2017-01-01

This article reconstructs Leo Strauss's reading of Rousseau's Epicureanism to argue that his work is unified by an abiding concern with the problem of theory and practice. Strauss sought to clarify the distinction between theory and practice he considered a fundamental precondition of any properly philosophical reflection on political life, and he explained the pernicious obscuring of that distinction through a narrative tracing the modern modifications of classical Epicureanism. Strauss's critical history of modern political thought is thus part of his attempt to restore the classical distinction between theory and practice to the status of a philosophical problem in modernity.

Pulmonary manifestations from systemic vasculitides

International Nuclear Information System (INIS)

Reuter, M.; Both, M.; Schnabel, A.

2007-01-01

Pulmonary vasculitides predominantly involve the small arterioles, capillaries and venules and include Wegener's granulomatosis, microscopic polyangiitis and the Churg-Strauss syndrome. Takayasu's arteriitis is a large vessel disease and may affect the main pulmonary arteries causing stenoses and occlusions. Knowledge of the natural course of disease and of clinical manifestations of pulmonary disease is helpful for an understanding of imaging findings. For this reason this article gives an overview not only of radiologic findings in chest X-ray and high resolution CT of the lungs but as well of clinical aspects of pulmonary vasculitides. Next to determination of disease extension the determination of disease activity is in the foreground of diagnostic imaging in vasculitides. Within this context principals of immunosuppressive therapy will be recognized. (orig.)

Pulmonary manifestations from systemic vasculitides; Pulmonale Manifestationen bei systemischen Vaskulitiden

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Reuter, M [Vivantes Klinikum Neukoelln, Berlin (Germany). Inst. fuer Radiologie und Interventionelle Therapie; Both, M [UKSH, Kiel (Germany). Klinik fuer Diagnostische Radiologie; Schnabel, A [Sana-Rheumazentrum Baden-Wuerttemberg, Bad Wildbad (Germany). Klinik fuer Internistische Rheumatologie und Klinische Immunologie

2007-06-15

Pulmonary vasculitides predominantly involve the small arterioles, capillaries and venules and include Wegener's granulomatosis, microscopic polyangiitis and the Churg-Strauss syndrome. Takayasu's arteriitis is a large vessel disease and may affect the main pulmonary arteries causing stenoses and occlusions. Knowledge of the natural course of disease and of clinical manifestations of pulmonary disease is helpful for an understanding of imaging findings. For this reason this article gives an overview not only of radiologic findings in chest X-ray and high resolution CT of the lungs but as well of clinical aspects of pulmonary vasculitides. Next to determination of disease extension the determination of disease activity is in the foreground of diagnostic imaging in vasculitides. Within this context principals of immunosuppressive therapy will be recognized. (orig.)

Major disruptions, inverse cascades, and the Strauss equations

International Nuclear Information System (INIS)

Montgomery, D.

1982-01-01

Current-carrying plasmas in a strong dc magnetic field are subject to violent disruptions above certain thresholds. At present difficult to verify, explanations are typically sought in terms of tearing modes. An alternative explanation is in terms of inverse magnetic helicity cascades, generated from a variety of possible sources of small-scale MHD turbulence. Strongly anisotropic MHD plasmas may be described by the Strauss equations. Indications of turbulent inverse cascade behavior for the Strauss equations are sought, in parallel with earlier examples from MHD and fluid mechanics

Angeitis necrotizante sistémica no supurativa

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Gabriel Toro G.

1972-04-01

Full Text Available 1. Se estudian 3 casos de angeítis necrotizante sistémica no supurativa: un hombre de 36 y una mujer de 58 años con granulomatosis de Wegener y un hombre de 30 años con granulomatosis alérgica del tipo descrito por Churg y Strauss. 2. Se prefiere el hombre de angeítis necrotizante sistémica no supurativa como lo ha hecho Paronetto cuando trata este capítulo desde el punto de vista inmunopatológico. 3. Consideran los autores que la lesión renal es la periglomerulitis granulomatosa parahiliar y que su presencia ya en la biopsia tiene gran valor diagnóstico. 4. Se subraya que el cuadro clínico, la marcada eosinofilia tisular y la presencia de imágenes micromultinodulares en el ganglio linfático, apoyan sólidamente un diagnóstico de granulomatosis alérgica de la forma descrita por Churg y Strauss. 5. Se hace una amplia revisión bibliográfica.

Strauss: Der Rosenkavalier - Suite / Michael Kennedy

Index Scriptorium Estoniae

Kennedy, Michael

1990-01-01

Uuest heliplaadist "Strauss: Der Rosenkavalier - Suite, Salome-Dance of the seven veils, Capriccio-Prelude, Intermezzo, Morgen Mittag um elf! Felicity Lott, Scottish National Orchestra, Neeme Järvi" Chandos ABRD 1397. ABTD 1397. CHAN 8758

Levi-Strauss and the Opera

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Vlado Kotnik

2016-02-01

Full Text Available This paper is dedicated to Claude Levi-Strauss and his structural reading of opera as metaphorical ‘composing’ of an anthropological grand opera, materialized in the four-volume study of Mythologiques, which refers to Wagner’s tetralogy of The Ring. He created a type of comparative view of the function and structure of myth schemes in Amerindian culture and the orchestral scores of Wagner’s operas, and implicitly signalled that European music, with its eminent representation – opera – has had the same value or similar symbolic position in the mind and life of a contemporary European that myth has had in ‘the savage mind’. Through this, he can lead us to the understanding of opera as myth and metaphor. However, the paper extends the discussion on Levi-Strauss to a broader historical picture of the relationship between opera and mythology as two symbolic systems of European culture.

Think twice

DEFF Research Database (Denmark)

Bang, Cæcilie Larsen; Porsbjerg, Celeste Michala

2016-01-01

Heart involvement is the most critical and potentially lethal systemic manifestation in eosinophilic granulomatosis with polyangiitis (EGPA). We present a case of acute chest pain in a 58-year-old male with severe asthma, which regressed after sublingual administration of nitroglycerine. At the t......Heart involvement is the most critical and potentially lethal systemic manifestation in eosinophilic granulomatosis with polyangiitis (EGPA). We present a case of acute chest pain in a 58-year-old male with severe asthma, which regressed after sublingual administration of nitroglycerine...... that it is important to consider alternative diagnoses in patients with atypical symptoms and a low risk profile. Heart involvement is the most critical and potentially lethal systemic manifestation in eosinophilic granulomatosis with polyangiitis (EGPA, formerly known as Churg-Strauss syndrome), which makes a quick...

Batik Kreatif Amri Yahya dalam Perspektif Strukturalisme Levi-Strauss

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Irfa'ina Rohana Salma

2016-04-01

Full Text Available ABSTRAKPada saat ini sebagaian besar pengembangan motif batik mengacu pada ragam hias tradisional, sehingga hasilnya cenderung monoton. Perlu penyegaran visual dan diversifikasi gagasan untuk menghasilkan motif batik modern yang baru, unik, kreatif, dan inovatif. Tujuan kajian ini adalah menginspirasi para seniman, perajin, desainer untuk menciptakan motif kreatif sebagai diversifikasi produk yang semakin memperkaya khasanah batik Indonesia, dengan mengkaji batik kreatif karya Amri Yahya. Batik kreatif Amri Yahya telah mendapat pengakuan internasional sebagai batik modern. Metode yang digunakan yaitu deskriptif analitis untuk mendeskripsikan dan menganalisis objek seni yaitu batik karya Amri Yahya dengan perspektif Strukturalisme Levi-Strauss. Dari kajian ini dapat disimpulkan bahwa batik kreatif karya Amri Yahya dihasilkan dari keberanian dan kebebasan berekspresi serta konsistensi dalam berkarya seni.Kata kunci: batik, kreatif, Amri Yahya, strukturalisme Levi-StraussABSTRACTAt this time almost all of the batik motive bulk development refers to the traditional decoration, so the results tend to be monotonous. It needs a visual refreshment and diversity idea to produce a modern motive that is new, unique, creative, and innovative. The purpose of this study was to inspire the artists, craft men, designers to create the creative motifs as the diversified products to enrichIndonesian batik, reviewing creative batik of Amri Yahya. These Amri Yahya creative batik hasreceived international recognition as a modern batik. The method used is descriptive analysis todescribe artwork object that is Amri Yahya batik with Levi -Strauss's Structuralism perspective. From this study it can be concluded that the creative work of Amri Yahya batik produced from the encouragement and independecy of expression, as well as consistency in the artwork.Keywords: batik, creative, Amri Yahya, levi - strauss structuralis

Lévi-Strauss, Caduveo Body Painting and the Readymade

DEFF Research Database (Denmark)

Wiseman, Boris Nicholas Daniel

2008-01-01

The aim of this article is to explore some convergences between aesthetics and the anthropology of art, two disciplines often thought of as incompatible or mutually exclusive. Its impetus is the conviction that we have much to gain by a more systematic and concerted attempt at constituting an eth...... that is implicit in Lévi-Strauss's works but not articulated as such, is a boundary marking processes, one that is central to the way in which we create an order of the world around us.......-aesthetics, i.e. a decentred aesthetics enriched by the dynamic of cross-cultural comparison. I will take as my starting point Lévi-Strauss's classic studies of Caduveo body painting and try to show how, beyond the clichés often repeated about structuralism, they provide valuable insights for an understanding...... will argue here that Lévi-Strauss's own theorisation of the relations between nature, culture and art enables us to see them, in at least one of their dimensions, as prime examples of the fulfilling of the mytho-poetic function. What I will place, here, at the core of mytho-poetic function, following a view...

An overlap of granulomatosis with polyangiitis and eosinophilic granulomatosis with polyangiitis

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Sujit Surendran

2017-01-01

Full Text Available We present a case report of overlap of granulomatosis with polyangiitis (GPA; formerly known as Wegener’s granulomatosis and eosinophilic granulomatosis with polyangiitis (EGPA; formerly known as Churg-Strauss syndrome. We report a 45-year-old female who presented with rapidly progressive renal failure associated with fever, polyarthralgia, and respiratory symptoms with cytoplasmic antineutrophilic cytoplasmic antibody (ANCA and proteinase (PR-3 antigen positivity. Computerized tomography scan of the chest showed diffuse alveolar hemorrhage with renal biopsy revealing pauci-immune necrotizing crescentic glomerulonephritis with intense eosinophilic infiltration suggestive of eosinophilic GPA (EGPA. Our patient had ANCA-associated vasculitis (AAV with features suggestive of both GPA and EGPA. She was treated with methylprednisolone and cyclophosphamide and attained remission after 2 weeks of therapy. This is a rare report of a patient with AAV having features of both EGPA and GPA.

Allergic granulomatous angiitis

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Trifunović Gordana

2004-01-01

Full Text Available Allergic granulomatous angiitis (AGA - Churg-Strauss syndrome, is a rare autoimmune disease characterized by three distinct clinical phases prodromal, eosinophilic, and vasculitic, and most of respiratory symptoms and signs begin in the first two phases of the disease. Two female patients of different age, who fulfilled the diagnostic criteria for AGA, and were in different phases and with the different duration of the disease are presented. The first patient (24 years of age was admitted to the hospital due to aggravation of asthma, heart failure, and polyneuropathy. The second one (45 years of age was also hospitalized due to the worsening of asthma polyneuropathy, and fever. Both were treated continuously with glucocorticoids. The older patient also received a total of six pulse doses of cyclophosphamide. Satisfactory response to such a treatment was achieved in both cases.

R. Strauss: Symphonia domestica, Op. 53 / David Nice

Index Scriptorium Estoniae

Nice, David

1994-01-01

Uuest heliplaadist "R. Strauss: Symphonia domestica, Op. 53. National Youth Orchestra of Great Britain / Christopher Seaman. Pickwick IMP Classics CD PCD 1080; Selected comparisons: SNO, Järvi (3/88) Chandos CHAN 8572"

Strauss: Metamorphosen for 23 solo strings / Michael Kennedy

Index Scriptorium Estoniae

Kennedy, Michael

1990-01-01

Uuest heliplaadist "Strauss: Metamorphosen for 23 solo strings, Tod und Verklärung, Op. 24, Drei Hymnen, Op.71. Felicity Lott (sop.), Scottish National Orchestra, Neeme Järvi" Chandos ABRD 1374. ABTD 1374. CHAN 8734

Reassessing Jacob Strauss and the Mosaic Code

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Joel McDurmon

2012-01-01

Full Text Available This article reviewed claims made by modern scholars Ford Lewis Battles, G.H. Williams, and Theodore Tappert concerning the views of Jacob Strauss (1480–1530, court preacher at Eisenach, particularly in regard to the imposition of Mosaic Law upon the civil realm. Most pointedly, Battles claims Strauss proposed to replace European civil law completely with the ‘entire Mosaic code’. This study examined Strauss’s relevant writings to determine his position on Mosaic Law and civil law and demonstrated that the claims of Battles, Williams, and Tappert were not supported by the primary source evidence. Selections from Strauss’ 51 theses on usury are translated into English for the first time. To a much lesser degree, this study addressed the issue in regard to the Weimar court preacher Wolfgang Stein, against whom the same claims were made. A paucity of evidence rendered those claims dubious in his case. In the end we were left only with unsubstantiated second-hand claims against these men.

Levi-Strauss's "Bricolage" and Theorizing Teachers' Work.

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Hatton, Elizabeth

1989-01-01

A teacher's work is compared to Claude Levi-Strauss's concept of "bricolage." A "bricoleur" is a professional do-it-yourself person, falling somewhere between an odd-job person and a craftsperson. The concept helps to explain pedagogical inadequacy by linking inherent limiting features of teachers' work and some causal…

[Diseases to differentiate from COPD, with emphasis on bronchial asthma].

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Kanazawa, Minoru

2007-04-01

Differential diagnosis of chronic obstructive pulmonary disease (COPD) from asthma is not a difficult task for many clinicians. Patients with COPD have a history of heavy smoking and show a slowly progressive dyspnea on exertion and there is little variability in symptoms, and they show a poor response to bronchodilators and corticosteroids. Asthma usually begins in early childhood with atopy, shows episodic dyspnea with wheezing, especially during night and early morning. Some patients, however, show adult onset, irreversible airflow limitation, and neutrophilic airway inflammation. The airway remodeling in asthma may be the cause of confusing pathophysiology. Other diseases showing airway hyperresponsiveness, such as allergic bronchopulmonary aspergillosis, Churg-Strauss syndrome, and left heart failure presenting cardiac asthma, may sometimes show similar clinical pictures to COPD. Chronic airway diseases are also possible candidates for differential diagnosis of COPD. Bronchiectasis, sinobronchial syndrome, diffuse panbronchiolitis, obliterative bronchiolitis, and other chronic airway diseases should be considered. Some interstitial lung diseases, such as smoking-related interstitial lung diseases and lymphangioleiomyomatosis, often show obstructive ventilatory impairment, and therefore should be considered in differential diagnosis of COPD.

Hemorragia alveolar maciça como manifestação inicial de poliangeíte microscópica Diffuse alveolar hemorrhage as initial manifestation of microscopic polyangiitis

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Cristiano Barbosa Campanholo

2007-02-01

Full Text Available Hemorragia alveolar (HA é uma manifestação clínica com alta taxa de mortalidade que deve ser investigada, reconhecida e estabilizada. Causas possíveis para a HA incluem infecções respiratórias ou sistêmicas, malformações arteriovenosas, estenose mitral, discrasias sangüíneas e doenças auto-imunes, como o lúpus eritematoso sistêmico (LES, a síndrome de Goodpasture e as vasculites sistêmicas primárias, principalmente aquelas associadas aos anticorpos anticitoplasma de neutrófilos (Anca, como a granulomatose de Wegener, síndrome de Churg-Strauss e a poliangeíte microscópica. Relatamos o caso de uma paciente jovem que apresentou quadro grave de HA necessitando ventilação mecânica assistida com pressão expiratória final positiva (Peep. Na ausência de evidências de infecção, discrasias sangüíneas ou malformações arteriovenosas, a paciente foi submetida à corticoterapia e à imunossupressão, com controle satisfatório da atividade da doença, que persiste após 24 meses de seguimento. Os dados clínicos, laboratoriais e histopatológicos permitiram estabelecer o diagnóstico de poliangeíte microscópica.Alveolar hemorrhage (AH is a clinical manifestation with high mortality rate that must be promptly investigated, recognized and stabilized. Causes of AH include systemic and respiratory tract infections, arterio-venous malformations, blood dyscrasias and autoimmune diseases such as systemic lupus erithematosus, Goodpasture syndrome and primary systemic vasculitis, specially the antineutrophil cytoplasmic antibodies-associated vasculitis such as Wegener’s granulomatosis, Churg-Strauss syndrome and microscopic polyangiitis. We report the case of a young female patient who developed severe AH necessitating orotracheal intubation and mechanic assisted ventilation with positive end expiratory pressure. Since no evidence of infection, malformation or blood dyscrasia was found, immediate therapy with glucocorticoids and

[Cavitating lung lesions in the course of ANCA-associated vasculitis: differential diagnostic aspects].

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Kirchner, J; Raab, H P; Länger, F; Wigand, R; Mitrou, P; Jacobi, V

1998-05-01

Antineutrophil cytoplasmatic antibodies (ANCA)-associated vasculitides (Wegener's granulomatosis, microscopic polyangiitis, Churg-Strauss syndrome) show quite variable courses. Clinical features of the full blown generalized systemic vasculitis are usually found in the respiratory tract and the kidney. Pulmonary involvement of Wegener's granulomatosis shows commonly nodules and cavitations but also diffuse alveolar hemorrhage. We report the case of a 57 year-old man suffering from dyspnea, thoracal pain, arthralgia, purpura, scleritis and tinitus. Specimen of the kidney showed segmental glomerulosclerosis and tubulointerstitial nephritis. Because of the presence of cANCA Wegener's disease was assumed. Pulmonary infiltrates developed under immunosuppressive treatment with cyclophosphamid. As differential diagnosis of the pulmonary infiltrates, we considered invasive pulmonary aspergillosis as well as infiltrates due to Wegener's granulomatosis. In spite of maximal therapeutic management of patient died of respiratory and cardiovascular failure. The findings at autopsy showed distinct invasive pulmonary aspergillosis and perifocal hemorrhage.

Grounded Theory and Pragmatism: The Curious Case of Anselm Strauss

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Antony Bryant

2009-07-01

Full Text Available Sir Arthur CONAN DOYLE's stories featuring Sherlock Holmes are justly famous the world over. In The Memoirs of Sherlock Holmes (1993 one story entitled Silver Blaze contains an exchange between Holmes and a Scotland Yard detective as follows: Gregory (Scotland Yard detective: "Is there any other point to which you would wish to draw my attention?" Holmes: "To the curious incident of the dog in the night-time." Gregory: "The dog did nothing in the night-time." Holmes: "That was the curious incident." In similar fashion I wish to draw attention to the curious case of Anselm STRAUSS: There is already a good deal of work pointing to the continuities between the Grounded Theory Method (GTM and the Pragmatism of John DEWEY and Charles PEIRCE. This has usually focused on Anselm STRAUSS with his Chicago-influenced Pragmatist background, although STRAUSS himself never articulated the way in which Pragmatism informed or could be brought to bear on the method as it evolved from the 1960s onwards. This paper argues that many of the contentious issues surrounding GTM can be resolved if they are understood against the context of some of the core tenets of Pragmatism, particularly the ways in which some of the more recent Pragmatists such as Richard RORTY have brought them back as a focus of attention. In so doing is raises the question of why, given his intellectual background and formation, Anselm STRAUSS did so little to bring Pragmatist ideas into GTM in its later embodiments and extended statements. That is the "curious incident" to which specific attention is drawn at several points in what follows; it remains a perplexing one, with perhaps no convincing solution, unlike the Sherlock Holmes mystery alluded to above. URN: urn:nbn:de:0114-fqs090325

Kas tõesti võib Strauss-Kahn puhta nahaga pääseda? / Heiki Suurkask

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Suurkask, Heiki, 1972-

2011-01-01

IMF-i endine juht ja võimalik Prantsuse presidendikandidaat Dominique Strauss-Kahn, keda süüdistatakse vägistamiskatses, on nüüdseks koduarestist vabastatud. DNA-proov on selgelt kinnitanud seksuaalset läbikäimist Strauss-Kahni ja hotelli toateenija vahel, kuid ei kinnita veel, kas tegemist oli vabatahtliku või pealesurutuga

A case of undeclared debt? Claude Lévi-Strauss' ambiguities and paradoxes toward analytical psychology.

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Serina, Florent

2018-06-09

If his relationship to Sigmund Freud's psychoanalysis has been widely acknowledged, Claude Lévi-Strauss' rapport with C.G. Jung's analytical psychology remains quite obscure. While secondary commentary has been abundant, its approach has above all been intertextual, to the detriment of a rigorously historical reading. Even if certain arguments put forward by supporters of so-called "influence" deserve to be taken into account, especially because they highlight Lévi-Strauss ambiguities and paradoxes toward Jung, this paper provides proof that a precise reading of the texts, with the help of recent studies on the intellectual genesis of Lévi-Strauss, lead to reject the thesis of an unstated debt owed by the French anthropologist to the Zurich psychologist. © 2018 Wiley Periodicals, Inc.

Rituximab in the treatment of refractory or relapsing eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome).

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Thiel, Jens; Hässler, Fabian; Salzer, Ulrich; Voll, Reinhard E; Venhoff, Nils

2013-09-24

Eosinophilic granulomatosis with polyangiitis (EGPA) is part of antineutrophil cytoplasmic antibodies (ANCAs)-associated vasculitides. In EGPA small-vessel vasculitis is associated with eosinophilia and asthma. About 40% of EGPA patients are ANCA-positive, suggesting a role for B cells in the pathogenesis of EGPA. B cell-depleting therapy with rituximab (RTX) can be effective in ANCA-positive EGPA, but very few patients have been published to date. The role of RTX in the treatment of ANCA-negative EGPA is unclear. We report a single-center cohort of patients with eosinophilic granulomatosis with polyangiitis. Of these patients, nine (six ANCA-positive, three ANCA-negative) had been treated with RTX for relapsing or refractory disease on standard immunosuppressive treatment. In a retrospective analysis, data on treatment response, frequency of relapses, adverse events, and peripheral B-cell reconstitution were evaluated. Furthermore, serum immunoglobulin concentrations, ANCA status, and peripheral B cell subpopulations were assessed after RTX treatment. All patients had high disease activity before RTX treatment. At presentation 3 months after RTX therapy, all ANCA-positive and ANCA-negative patients had responded to RTX, with one patient being in complete remission, and eight patients being in partial remission. After a mean follow-up of 9 months, C-reactive protein concentrations had normalized, eosinophils had significantly decreased, and prednisone had been tapered in all patients. In all patients, RTX therapy was combined with a standard immunosuppressive therapy. Within the 9-month observation period, no relapse was recorded. Three patients were preemptively retreated with RTX, and during the median follow-up time of 3 years, no relapse occurred in these patients. During the follow-up of 13 patient-years, five minor but no major infections were recorded. In our analysis on nine patients with EGPA resistant to standard therapy, rituximab proved to be an efficient and safe treatment for ANCA-positive and ANCA-negative patients. Preemptive retreatment with RTX, combined with standard maintenance immunosuppressants, resulted in a sustained treatment response. Prospective, randomized trials evaluating the use of RTX in EGPA are warranted.

The paradigm of description in ethnographic translation: the translator Levi-Strauss in Tristes Tropiques

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Alice Maria de Araújo Ferreira

2014-11-01

Full Text Available Published in 1955, Tristes Tropiques by Claude Levi-Strauss is an account of the journey that the author and ethnographer made on the American continent, especially in Brazil, in 1930. With a free poetic style not restricted by the austerity of scientific work, Levi-Strauss introduced a reflection that established a crucial rupture in ethnographic studies and in the humanities in general, or rather, the rupture of the gaze. His aim is not precisely the culture of the indigenous people in Brazil, but Levi-Strauss himself as the subject of the gaze. How may one grasp an object that changes as one gazes at it? How does the gaze affect the object while gazing, observing, analyzing, describing, and translating it? Current essay discusses what the translator does to the speech of the other when translating it. Different translation strategies from Portuguese into French proposed by Levi-Strauss in Tristes Tropiques are discussed. Conceiving ethnographic translation from the description paradigm and as an encounter of cultures (but not as replacement, the author analyzes the process performed within the gap between the gaze experience and the production of speech of such gaze to understand the value produced at the end of the axis corresponding to the ethnographic translation-description.

Mononeuropatia Múltipla como forma de apresentação da Granulomatose Eosinofílica com Poliangeíte

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Tiago Manuel Fernandes

2018-03-01

Full Text Available Resumo: A Granulomatose Eosinofílica com Poliangeíte (EGPA, previamente denominada Síndrome de Churg-Strauss, é uma vasculite associada ao anticorpo anti-citoplasma dos neutrófilos (ANCA que atinge vasos de pequeno calibre. Os autores descrevem o caso de uma mulher com instalação subaguda de parestesias, disestesias, paraparésia crural a impossibilitar a marcha e lesões purpúricas no tornozelo. A electromiografia mostrou mononeuropatia múltipla com envolvimento dos nervos Mediano esquerdo, Cubital e Femoral direitos. Estudo analítico com eosinofilia e ANCA positivo. A biópsia cutânea mostrou vasculite necrotizante e infiltrado eosinofílico. Cumpre a definição de Chapel Hill Conference Consensus e reúne os critérios do American College of Rheumatology pelo que foi assumido o diagnóstico de EGPA. Iniciou corticoterapia e ciclofosfamida, com melhoria clínica e laboratorial. Este caso clínico ilustra a importância do reconhecimento do padrão da neuropatia periférica na apresentação desta entidade rara que pode ser incapacitante se houver atraso no diagnóstico e tratamento. Abstract: Eosinophilic Granulomatosis with Polyangiitis (EGPA, formerly known as Churg-Strauss Syndrome, is a anti-neutrophil cytoplasmic antibody (ANCA associated vasculites affecting small vessels. We describe a women with subacute complaints of paresthesias, disesthesias, crural paraparesis with lost of gait and purpuric lesions on her ankle. Electromyogram revealed multiplex mononeuropathy with left median nerve, right ulnar and femoral nerves involvement. Laboratory analysis with eosinophilia and positive ANCA. She fulfilled the Chapel Hill Conference Consensus definition and the criteria of American College of Rheumatology for EGPA. Corticosteroids and cyclophosphamide were begun with good clinical and laboratorial response. This report ilustrates the importance of identifying the neuropatic pattern as initial manifestation of this rare condition

A ideia, a série e a forma: Desafios da imagem no pensamento de Claude Lévi-Strauss

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Carlo Severi

2011-11-01

Full Text Available Neste artigo, parte-se da abordagem teórica da arte por Lévi-Strauss para aplicá-la a um objeto não explorado pelo próprio autor: a arte abstrata no Ocidente. O texto inicia com uma polêmica entre Lévi-Strauss e o teórico do surrealismo, André Breton, em torno da magia da arte. A polêmica põe a nu a originalidade de Lévi-Strauss enquanto teórico da arte, em diálogo permanente com os Modernistas de sua época, assim como a centralidade da arte na sua teoria estruturalista. Se para Breton a magia da arte é universal porque apela para processos de apreensão intuitivas, opostos à razão, para Lévi-Strauss o desafio da universalidade da arte consiste no fato de este ser um dos lugares privilegiados de exploração do pensamento formal. Lévi-Strauss propõe interpretar paralelamente, no interior de cada universo cultural, as coordenadas constitutivas da imagem e as operações mentais que essas coordenadas implicam. Se Lévi-Strauss seguiu este caminho para dar sentido aos arabescos kadiwéu, propõe-se aqui seguir esta trilha na exploração da arte abstrata.

Off-Label Uses of Omalizumab.

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El-Qutob, David

2016-02-01

The off-label use of medicines is a common and extensive clinical practice. Omalizumab has been licensed for use in severe allergic asthma and chronic urticaria. Omalizumab dosing was based on body weight and baseline serum IgE concentration. All patients are required to have a baseline IgE between 30 and 700 IU/ml and body weight not more than 150 kg. The use of off-label drugs may lead to several problems including adverse effects and an increased risk/benefit balance. In this article, there are summarized off-label uses of omalizumab in the last recent years in diseases in which IgE maybe or certainly has a corner role such as allergic rhinitis, allergic bronchopulmonary aspergillosis, anaphylaxis, keratoconjunctivitis, food allergy, drug allergy, urticaria, angioedema, non-atopic asthma, atopic dermatitis, nasal polyps, Churg-Strauss syndrome, eosinophilic otitis media, chronic rhinosinusitis, bullous pemphigoid, contact dermatitis, and others. Use in pregnancy asthmatic women and pre-co-administration with specific immunotherapy will also be revised.

Testing, testing, testing: an insidious hypereosinophilia

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Livia Robbiolo

2013-04-01

Full Text Available Aim of the study: This case focuses on the difficulty in recognizing this rare entity characterized by systemic vasculitis in patients with history of asthma. Clinical case: We report a case of a 46-year-old man with history of allergic rhinitis and referred episodes of shortness of breath recognizing as acute bronchitis who presented with fever, skin eruption, peripheral eosonophilia, muscle weakness, abdominal pain and progressively dyspnea. Methods: Chest radiograph and computed tomography on admission showed consolidation in both lung fields and pericardial effusion. P-serum ANCA were positive. Neurological examination revealed mononeuritis multiplex. A skin biopsy was performed with presence of eosinophil granulocytes. The condition did not respond to antibiotics. He was supposed to have vasculitis and steroid treatment was started at a dose of 1 mg/kg; eosinophilia decreased but there was no clinical improvement. Abdominal pains were progressively more severe; emergency laparotomy revealed ileum infiammation, histopathological examination was consistent with Churg-Strauss syndrome.

From Leo Strauss to Collapse Theory: Considering the Neoconservative Attack on Modernity and the Work of Education

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Smith, David Geoffrey

2008-01-01

This paper locates the work of Leo Strauss within the broader conservative assault on modernity and especially its roots in liberalism. Four themes from Strauss's work are identified, then hermeneutically engaged for their relevance to educational practice in global times. The four themes are: (1) the liberal/modern concept of an open society is…

Normativity II – Towards an Integral Perspective | Strauss | South ...

African Journals Online (AJOL)

This is a follow-up article of Strauss 2011. In order to transcend the shortcomings present in the dialectical legacy regarding normativity, this article further explores key elements within the dialectical tradition focused on the basic motive of nature and freedom and the effect it had on modern social contract theories which ...

In memoriam. La obra de Claude Lévi–Strauss (1908–2009. Unidad humana y diferencia cultural

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Álvaro Pazos

2010-11-01

Full Text Available Uno de los temas fundamentales de la obra del antropólogo francés Claude Lévi–Strauss, recientemente fallecido, es el de la relación entre unidad humana y diferencia cultural. De diversas maneras, tanto en sus trabajos teóricos como en sus intervenciones públicas, Lévi– Strauss adoptó una particular forma de relativismo cultural. En este artículo se repasa lo que esta posición debe a los presupuestos básicos del estructuralismo; se refl exiona asimismo sobre las diversas nociones de Lévi–Strauss respecto de la unidad humana; y se introduce una crítica del relativismo estructuralista y una defensa de un universalismo de lo social.

Entre Carl Schmitt y Thomas Hobbes . Un es tudio del liberalismo moderno a partir del pensamiento de Leo Strauss

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José Daniel Parra Quintero

2010-01-01

Full Text Available El presente ensayo es una lectura sobre el liberalismo moderno desde el pensamiento de Leo Strauss. A partir de su análisis del Concepto de lo político de Carl Schmitt y su crítica de la "despolitización y neutralización" liberal, Strauss responde con una afirmación del problema del derecho natural, para lo cual es fundamental como primer paso estudiar la filosofía política de Thomas Hobbes. Desde allí Strauss se plantea un punto de contraste hacia una consideración clásica del derecho natural que pueda hacer frente al nihilismo político o cosmopolitanismo apolítico en la modernidad tardía, resultado ambos tanto del histori-cismo radical como del positivismo metodológico. Strauss antepone a la afirmación de la voluntad del poder soberano la búsqueda del derecho natural como representación de la idea de la justicia.

Louis Althusser, Leo Strauss, and Democratic Leadership

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Tamulis, Bron Cohen

2014-01-01

In taking up the topic of political leadership, I seek to analyze the legacy of social science in light of the sobering political events of the twentieth century. This dissertation is a composite study of the work of two philosophers, and, specifically, what they endeavored to accomplish by thinking and writing on the subject of politics. I concentrated on the post-War landscape, and the philosophers Louis Althusser and Leo Strauss, in order to analyze the way in which the figure of Machiav...

Respiratory manifestations of eosinophilic granulomatosis with polyangiitis (Churg-Strauss)

NARCIS (Netherlands)

Cottin, Vincent; Bel, Elisabeth; Bottero, Paolo; Dalhoff, Klaus; Humbert, Marc; Lazor, Romain; Sinico, Renato A.; Sivasothy, Pasupathy; Wechsler, Michael E.; Groh, Matthieu; Marchand-Adam, Sylvain; Khouatra, Chahéra; Wallaert, Benoit; Taillé, Camille; Delaval, Philippe; Cadranel, Jacques; Bonniaud, Philippe; Prévot, Grégoire; Hirschi, Sandrine; Gondouin, Anne; Dunogué, Bertrand; Chatté, Gérard; Briault, Amandine; Jayne, David; Guillevin, Loïc; Cordier, Jean-François

2016-01-01

The respiratory manifestations of eosinophilic granulomatosis with polyangiitis (EGPA) have not been studied in detail.In this retrospective multicentre study, EGPA was defined by asthma, eosinophilia and at least one new onset extra-bronchopulmonary organ manifestation of disease.The study

M. CEBOTARI AND R. STRAUSS: CHRONICLE OF PROFESSIONAL AND PERSONAL CONTACTS (1942–1949

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PILIPEȚCHII SERGHEI

2017-06-01

Full Text Available The article presents information organized in chronological order on the professional and personal contacts of M. Cebotari with R. Strauss in the last seven years of their lives (1942–1949. The development of military events during the war, as well as the advanced age of the maestro partially stopped relations between the two musicians. This period shows the fact that M. Cebotari not only maintained her interest in Straus`s vocal music, but also began to perform in new roles, adding to her repertoire the dramatic soprano parts. The article is well supported with quotes of the contemporaries, and with reviews from the newspapers of that time.

Comparación entre los diagramas TTS obtenidos mediante los ensayos normalizados Huey y Strauss modificado para el acero inoxidable AISI 304

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Otero, E.

1996-04-01

Full Text Available This work presents a comparison of Temperature-Time-Sensitization TTS diagrams obtained by Huey and modified Strauss standard, for detecting susceptibility to intergranular attack. The difference between these tests is the use of a strong or a slightly smooth oxidizing electrolyte. The diagrams obtained are different and if a sample tested by modified Strauss is sensitized, then it will be sensitized in the Huey test, but the contrary is not always true. This difference is because a sensitized sample has to have a continuous band lacking in chromium along the grain boundary in order to be sensitized in the modified Strauss test. This condition is not necessary in the Huey test.

Se comparan los diagramas Temperatura-Tiempo-Sensibilización TTS, obtenidos mediante los ensayos normalizados de corrosión intergranular Huey y Strauss modificado, que, respectivamente, utilizan electrólitos fuerte y suavemente oxidantes, mostrándose que los diagramas obtenidos son diferentes. Si una muestra evaluada mediante el ensayo Strauss modificado se encuentra sensibilizada, entonces lo estará también en el ensayo Huey, pero no necesariamente ocurrirá lo contrario; esta diferencia se asocia a la necesidad de la existencia de una zona continua empobrecida en cromo a lo largo del límite de grano para que el material se sensibilice con respecto al ensayo Strauss modificado, condición innecesaria para el caso del ensayo Huey.

Strauss, Richard. Ariane a Naxos: opera seulement, integrale. Miliza Korjus / Michel Parouty

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Parouty, Michel

1995-01-01

Uuest heliplaadist "Strauss, Richard. Ariane a Naxos: opera seulement, integrale. Miliza Korjus (Najade). Orchestre de l'Opea l'Efat de Vienne" Arlecchino ARL 14-16, distribution Dante (3 CD: 390 F). 1935, 1944. TT: 3h 46'17"

A Diagnostic Algorithm for Eosinophilic Granulomatosis with Polyangiitis Initially Diagnosed as Lumbar Disc Hernia or Lumbar Spinal Stenosis: Personal Experience and Review of the Literature.

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Nagata, Kosei; Yamamoto, Shinichi; Miyoshi, Kota; Sato, Masaki; Arino, Yusuke; Mikami, Yoji

2016-08-01

Eosinophilic granulomatosis with polyangiitis (EGPA, Churg-Strauss syndrome) is a rare systemic vasculitis and is difficult to diagnose. EGPA has a number of symptoms including peripheral dysesthesia caused by mononeuropathy multiplex, which is similar to radiculopathy due to lumbar disc hernia or lumbar spinal stenosis. Therefore, EGPA patients with mononeuropathy multiplex often visit orthopedic clinics, but orthopedic doctors and spine neurosurgeons have limited experience in diagnosing EGPA because of its rarity. We report a consecutive series of patients who were initially diagnosed as having lumbar disc hernia or lumbar spinal stenosis by at least 2 medical institutions from March 2006 to April 2013 but whose final diagnosis was EGPA. All patients had past histories of asthma or eosinophilic pneumonia, and four out of five had peripheral edema. Laboratory data showed abnormally increased eosinophil counts, and nerve conduction studies of all patients revealed axonal damage patterns. All patients recovered from paralysis to a functional level after high-dose steroid treatment. We shortened the duration of diagnosis from 49 days to one day by adopting a diagnostic algorithm after experiencing the first case.

Strauss, R.: Symphony in F minor, Op. 12... Tokyo Metropolitan Symphony Orchestra / David Nice

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Nice, David

1994-01-01

Uuest heliplaadist "Strauss, R.: Symphony in F minor, Op. 12... Tokyo Metropolitan Symphony Orchestra / Hiroshi Wakasugi. Denon CD CO-75 860 (54 minutes); Symphony - comparative version: SNO, Järvi" (8/93)(CHAN) CHAN 9166

Lévi-Strauss no Brasil: a formação do etnólogo

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Peixoto Fernanda

1998-01-01

Full Text Available O artigo procura acompanhar o período brasileiro de Claude Lévi-Strauss (1935-1938, rastreando suas publicações, aulas e pesquisas no momento em que era professor de sociologia na Universidade de São Paulo, com o objetivo de compreender o lugar ocupado pelo Brasil na sua trajetória. O interesse é mostrar que apesar de breve, e de modo geral silenciada, a etapa brasileira do autor foi fundamental para os desdobramentos de sua futura carreira como etnólogo e americanista. O texto procura também avaliar o significado da vinda de Lévi-Strauss para o Brasil no contexto francês da época, quando o americanismo era ainda terreno pouco explorado, ao contrário do africanismo, "vocação" em voga no momento.

Lévi-Strauss en el pensamiento filosófico latinoamericano. : Claude Lévi-Strauss: El pensamiento de un siglo. "Psicoanalisis y Antropologia hoy"

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Gonzalez , Patricia; de Pablo , Elisabeth; Winkler , Lisette; Borie , Cesar

2008-01-01

Corpus "ASA-SHS"; Nacido en 1908, Claude LEVI-STRAUSS, es considerado el fundador de la antropología estructural e introductor a las ciencias sociales del enfoque estructuralista basado en la lingüistica estructural de Saussure. Por la influencia de su obra, dentro y fuera de la antropología, es considerado uno de los intelectuales más influyentes del siglo XX. Con motivo del aniversario del antropólogo, el Coloquio internacional "Claude Lévi-Srauss: el pensamiento de un siglo “psicoanalisis ...

The Strauss and Carpenter Prognostic Scale in subjects clinically at high risk of psychosis

NARCIS (Netherlands)

Nieman, D. H.; Velthorst, E.; Becker, H. E.; de Haan, L.; Dingemans, P. M.; Linszen, D. H.; Birchwood, M.; Patterson, P.; Salokangas, R. K. R.; Heinimaa, M.; Heinz, A.; Juckel, G.; von Reventlow, H. G.; Morrison, A.; Schultze-Lutter, F.; Klosterkötter, J.; Ruhrmann, S.; McGorry, Patrick D.; McGlashan, Thomas H.; Knapp, Martin; van de Fliert, Reinaud; Klaassen, Rianne; Picker, Heinz; Neumann, Meike; Brockhaus-Dumke, Anke; Pukrop, Ralf; Svirskis, Tanja; Huttunen, Jukka; Laine, Tiina; Ilonen, Tuula; Ristkari, Terja; Hietala, Jarmo; Skeate, Amanda; Gudlowski, Yehonala; Ozgürdal, Seza; French, Paul; Stevens, Helen

2013-01-01

To investigate the predictive value of the Strauss and Carpenter Prognostic Scale (SCPS) for transition to a first psychotic episode in subjects clinically at high risk (CHR) of psychosis. Two hundred and forty-four CHR subjects participating in the European Prediction of Psychosis Study were

Etiology of cutaneous vasculitis: utility of a systemic approach

Science.gov (United States)

Chanussot-Deprez, Caroline; Vega-Memije, María Elisa; Flores-Suárez, Luis; Ríos-Romero, Celia; Cabiedes-Contreras, Javier; Reyes, Edgardo; Rangel-Gamboa, Lucia

2018-01-01

Cutaneous vasculities (CV) represents a diagnostic challenge, occurs as primary cutaneous disorder or as a manifestation of other entities. To search the cause of CV. Methods: Patients with CV were prospectively evaluated. In all patients, skin biopsies were drawn, and direct immunofluorescence was done in most of the patients. American College of Rheumatology (ACR) and Chapel Hill Consensus Conference Criteria (CHCC) were used for classification. 32 patients were studied. There was female predominance (71.8%). Children presented drug-associated CV or Schönlein-Henoch púrpura (SHP). Adults presented more frequently SHP, systemic lupus erythematosus or paraneoplastic vasculitis, other diagnosis as polyarteritis nodosa, microscopic polyangiitis, thrombotic vasculitis (post-puerperal), antiphospholipid syndrome, Churg-Strauss syndrome, and drug-associated CV were presented. Using the ACR and CHCC criteria, 50% of cases were classified. In our institution, during this work the etiologic diagnostic of CV increased more than twice. However, in the case of HSV or LA and SHP none of the proposed criteria had high specificity; other parameters were used to discern between both. Six patients remained as not classified. In our view, cryoglobulins and hepatitis serology do not seem useful unless patient’s history supports they need to be done. Unclassified patients were followed-up closely for 2 years. Copyright: © 2018 SecretarÍa de Salud

Novel targets of omalizumab in asthma.

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Sattler, Caroline; Garcia, Gilles; Humbert, Marc

2017-01-01

Omalizumab is a recombinant humanized anti-IgE monoclonal antibody approved in the US for moderate to severe persistent allergic asthma (severe persistent asthma in the European Union), uncontrolled despite treatment with inhaled corticosteroids and long-acting beta2 agonists. It reduces asthma exacerbations, symptoms, oral corticosteroid doses, and improves quality of life. Omalizumab may have an antiviral effect when used as a preventive therapy for fall exacerbations in children and teenagers. Two proof-of-concept studies have evaluated omalizumab in nonatopic asthma and showed that it is safe and possibly efficacious in some patients. Omalizumab has been successfully studied as add-on to specific immunotherapy in moderate allergic asthma. Its safety in pregnancy has been assessed in the EXPECT registry. Case series also report positive effects in cases of allergic bronchopulmonary aspergillosis, and in nasal disorders frequently associated with asthma. Last, omalizumab may have corticosteroid-sparing effect in a subset of patients with eosinophilic granulomatosis with polyangiitis (formerly Churg-Strauss syndrome). Recent studies argue in favor of positive effects of omalizumab beyond its current indications in asthma. Well-designed studies are needed in order to demonstrate the safety and efficacy of omalizumab in these possible novel indications.

The vasculitic neuropathies: an update.

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Collins, Michael P

2012-10-01

Vasculitic neuropathy is a heterogeneous disorder that usually occurs in systemic diseases, but less commonly appears as nonsystemic vasculitic neuropathy (NSVN). This review is intended to highlight recent developments in the field of vasculitic neuropathies. A Peripheral Nerve Society guideline provides data-driven consensus recommendation on classification of vasculitic neuropathies and diagnosis/treatment of NSVN. NSVN is sometimes accompanied by subclinical inflammation of adjacent skin. Amyotrophic lateral sclerosis with sensory involvement can mimic NSVN. Systemic vasculitides with neuropathy include polyarteritis nodosa, microscopic polyangiitis (MPA), rheumatoid vasculitis, Churg-Strauss syndrome (CSS), and hepatitis C-related mixed cryoglobulinemic vasculitis (MCV). At autopsy, MPA affects limb nerves diffusely, with maximal damage in proximal/middle segments. CSS can be accompanied by antineutrophil cytoplasmic antibodies (ANCAs), but most patients with neuropathy lack ANCAs. Cryoglobulinemic neuropathies are usually caused by vasculitis, irrespective of phenotype. Two randomized trials revealed rituximab to be noninferior to cyclophosphamide for inducing remission in ANCA-associated vasculitis. Many reports also document efficacy of rituximab in MCV. Consensus guidelines on NSVN should be evaluated prospectively. MPA-associated vasculitic neuropathy results from vasculitic lesions distributed diffusely throughout peripheral extremity nerves. Rituximab is effective for ANCA-associated and cryoglobulinemic vasculitis with neuropathy.

Geertz versus Levi-Strauss: latent structural dispositions in Geertz "theory of culture"?

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Gordana Gorunović

2016-02-01

Full Text Available These are two authors, in Foucauldian terms that certainly belong to the most influential individuals in socio-cultural anthropology, as well as in the social sciences and interdisciplinary research more broadly. Claude Levi- Strauss became some kind of an "intellectual hero" during the domination of structuralism in the mid-twentieth century and during the 1960s, while Clifford Geertz was an ‘icon and ambassador’ of anthropology in the second half of the twentieth century. They are both one of the founders of the discourse theory. They not only established a distinct theoretical approaches and methods – structural (Levi-Strauss and interpretative anthropology (Clifford Geertz, but through their intellectual authority they also inspired paradigms and intellectual movements making structuralism and "interpretation of culture" more than some passing episodes in the history of social though (in terms of "trendy ideas". My aim is to make some parallels between these two authors, who despite all the differences that are evident in their epistemological discourses, theoretical approaches and methods (as well as in their ethnographic and anthropological writings itself still have some similarities in their theorisation and interpretation of culture, which I would like to stress in this paper.

Co-brand strategy of evaluation of visual images in furniture design: Jimmy S.P.A. and STRAUSS as examples

Science.gov (United States)

Chen, Tien-Li; Pan, Fang-Ming; Tsai, Jen-Hui

2013-03-01

This study aimed to investigate the correlation of the image associated by the design Co-Brand (Jimmy S.P.A. and STRAUSS) and the impression perceived by subject of viewers. Visual images were used to examine the merit of the evaluation. The best result is provided using an object as an appropriate evaluation method. There are a lot of factors which influence to evaluation of a design. This study is limited to distinguish the appearance from Jimmy's picture books transform furniture and so on. Co-Brand of Jimmy S.P.A. and STRAUSS is not easy because there are not from the same cultural, and industry background and applying different marketing strategy, it is a way to combine the two brands by designing, used questionnaire of SD (Semantic differential evaluation) evaluation method to test out the perception of viewers, the objective of this study is to investigate and appraised the Co-Brands use by of the image in furniture from patrons. SD evaluation result showed, if design cannot understand the perception image of Jimmy S.P.A and STRAUSS with viewers mind, furniture design also can't transmit feeling with design.

Ética e profética nas Mitológicas de Lévi-Strauss

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Renato Sztutman

2009-06-01

Full Text Available Este artigo intenta extrair das Mitológicas de Claude Lévi-Strauss uma ética e uma filosofia política (em princípio, ameríndias. Busca-se aqui a um só tempo responder e estender a provocação de Pierre Clastres, para quem a análise estrutural não teria se ocupado de tamanhas questões. Para realizar esta discussão, o foco será dado em duas obras de Lévi-Strauss: As origens dos modos à mesa, o terceiro volume das Mitológicas, e História de lince, a última das "pequenas mitológicas". Se a primeira finda com um excurso sobre a "moral dos mitos", a segunda principia com uma reflexão sobre a capacidade da mitologia de fornecer leituras da história, por exemplo, o "encontro" com o mundo europeu. Essas leituras, de sua parte, devem estar na base do que a literatura etnológica recente reconheceu como movimentos proféticos.This article focus Claude Lévi-Strauss' reflection on amerindian ethic and political thought. We suggest that we could read many passages of Mythologiques as if they contained sorts of replies as well as developments of some of Pierre Clastres' provocations and critics directed to structuralism. We will analyse particularly two books: Les origines des manières à table, Mythologiques' third volume, and Histoire de lynx, the last of the petites mythologiques. The first one ends with an interesting reflection on the "mythical moralism", while the second one starts with the problem of how amerindian mythologies can offer interpretations of history, interpretations of the "encounter" with europeans, for instance. These, we suppose, must be on the basis of what ethnological recent literature called "prophetic movements".

The Influence of Johannes Fabian on Claude Lévi -Strauss

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Miloš Milenković

2016-02-01

Full Text Available In his explicitly theoretical works, Johannes Fabian, one of the key intradisciplinary "affinities" of the author-founder of postmodern anthropology, constructed a specific view of Claude Lévi-Strauss's structuralism, which, as reinterpreted by critical anthropologists, was to fundamentally shape the history of anthropological post-structuralism. In order to explain that the constitution of the subject of research through research itself – i.e. "the invention of the subject of anthropology" – should be accorded the status of the crucial problem of anthropological methodology, Fabian imputed to Lévi-Strauss a rigid and amateurish preconception of research in the natural sciences as inherently positivist. The opposition structuralism/poststructuralism = positivism/postpositivism thus implied was to permanently reshape discussions on realism in critical anthropology, with absurd consequences. The most important of these was the supposedly "antirealist" character of postmodern anthropology, as a direct derivative of critical anthroplogy, which, being aware that reality is created by research, was now seen to play a somehow more ethical and less repressive part in knowledge/power games. The delight at the discovery of this methodological commonplace – that the subject of research is constituted by research itself – might have been but a matter of passing interest, being itself a common enough phenomenon, had not this particular intradisciplinary exchange led to the discrediting of the entire methodological package associated with structuralism. Paradoxically, the retaining of a realistic, radically antipositivist structural method would have made possible the achievement of the cultural-critical ambitions of postmodern anthropology's research program, had not the method been ineptly contaminated by this chain of activist reinterpretation which eventually frustrated the aims it had set out to accomplish.

Defense of Truth (criticism and reconstructing the model of truth in the modern age and its influence on political philosophy from the viewpoints of Husserl and Strauss

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MH Jamshidi

2014-02-01

Full Text Available Husserl and Strauss are two philosophers that intended to reform philosophic thought and thereupon political philosophy. But despite of common view about end and foundations, how and why Husserl is included in the class of pioneers of battle against metaphysical philosophy and Strauss is reputed against him as the leader of classic and Conservative Philosophy? According to Thomas Spreagens’ epistemological method, even though this Germanic teacher and his pupil have common preoccupation about the sunset of philosophical truth-seeking thought, their different understanding of themes of this crisis and ideal aspect have been ended in different and maybe opposite philosophies. Unlike Husserl who set a kind of Humanism and inter-subjective understanding as intellectual and cultural base for his plan, i.e. Phenomenology, Strauss talks about the natural essence of political issues and dependence of political philosophy on regulations which are out of human’s control. Thus, the type of post-metaphysical political philosophy that finds political objects inside ordinary life and social and historical relations and understandings is derived from Husserl’s Phenomenology, but Strauss’ insistence on classic political philosophy cautiously seeks to answer fundamental and pervasive political questions, while continuously and modestly exploring the nature of political issues.

INDIAN CUISINE AS A PASSING LINK FROM "CULTURE" TO "NATURE": INVERTING LÉVI-STRAUSS

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Mártin César Tempass

2011-06-01

Full Text Available According to Claude Lévi-Strauss, the cuisine constitutes a passing link from stage of nature to stage of culture. Based on ethnographic research among the Mbyá-Guarani, this article analyzes this assertion under the light of the Amerindian cosmology. However, the dichotomy between nature and culture does not apply to indigenous groups, since for these nature and culture constitute a hybrid. To this hybridism might also be added the domain of supernatural. In terms of analysis this hybrid configuration does not allow any kind of transition from one domain to another. Yet the transition is possible among the three possible conditions of life in cosmos – animality, humanity and divinity. Human beings may perform the transition to animality as well as the transition to divinity. But no matter the course of the transition, although in different conditions in cosmos, from humanity one will always achieve animality. As conceptualized by Lévi-Strauss, from culture always occurs the trasition to nature. However, in one direction ex-humans will control nature beings, in another one, they will be controlled by nature. This isn’t just about a peculiarity of indigenous groups, it may also be found in societies so-called “modern”, however under others shapes.

Schmidt. Sinfonie Nr. 1 E-Dur; Strauss. Vier sinfonische Zwischenspiele aus Intermezzo. Detroit Symphony Orchestra, Neeme Järvi / Helge Grünewald

Index Scriptorium Estoniae

Grünewald, Helge

1996-01-01

Uuest heliplaadist "Schmidt. Sinfonie Nr. 1 E-Dur; Strauss. Vier sinfonische Zwischenspiele aus Intermezzo. Detroit Symphony Orchestra, Neeme Järvi. Chandos/Koch CD 9357 (WD: 68'20") DDD (WD:114'36")

Claude Lévi-Strauss: El pensamiento de un siglo. "Psicoanalisis y Antropologia hoy"

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2009-12-01

Full Text Available Presentación (en español Nacido en 1908, Claude lévi-Strauss es considerado como el fundador de la antropología estructural e introductor a las ciencias sociales del enfoque estructuralista basado en la linguística estructural de Saussure. Por la influencia de su obra, dentro y fuera de la antropología, es uno de los intelectuales más influyentes del siglo XX. Con motivo del aniversario del antropólogo, el Coloquio internacional "Claude Lévi-Srauss: el pensamiento de un siglo “psicoanalisis ...

Biomarkers in Vasculitis

Science.gov (United States)

Monach, Paul A.

2014-01-01

Purpose of review Better biomarkers are needed for guiding management of patients with vasculitis. Large cohorts and technological advances had led to an increase in pre-clinical studies of potential biomarkers. Recent findings The most interesting markers described recently include a gene expression signature in CD8+ T cells that predicts tendency to relapse or remain relapse-free in ANCA-associated vasculitis, and a pair of urinary proteins that are elevated in Kawasaki disease but not other febrile illnesses. Both of these studies used “omics” technologies to generate and then test hypotheses. More conventional hypothesis-based studies have indicated that the following circulating proteins have potential to improve upon clinically available tests: pentraxin-3 in giant cell arteritis and Takayasu’s arteritis; von Willebrand factor antigen in childhood central nervous system vasculitis; eotaxin-3 and other markers related to eosinophils or Th2 immune responses in eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome); and MMP-3, TIMP-1, and CXCL13 in ANCA-associated vasculitis. Summary New markers testable in blood and urine have the potential to assist with diagnosis, staging, assessment of current disease activity, and prognosis. However, the standards for clinical usefulness, in particular the demonstration of either very high sensitivity or very high specificity, have yet to be met for clinically relevant outcomes. PMID:24257367

Polymorphisms in genes encoding leptin, ghrelin and their receptors in German multiple sclerosis patients.

Science.gov (United States)

Rey, Linda K; Wieczorek, Stefan; Akkad, Denis A; Linker, Ralf A; Chan, Andrew; Hoffjan, Sabine

2011-01-01

Multiple sclerosis (MS) is a neuro-inflammatory, autoimmune disease influenced by environmental and polygenic components. There is growing evidence that the peptide hormone leptin, known to regulate energy homeostasis, as well as its antagonist ghrelin play an important role in inflammatory processes in autoimmune diseases, including MS. Recently, single nucleotide polymorphisms (SNPs) in the genes encoding leptin, ghrelin and their receptors were evaluated, amongst others, in Wegener's granulomatosis and Churg-Strauss syndrome. The Lys656Asn SNP in the LEPR gene showed a significant but contrasting association with these vasculitides. We therefore aimed at investigating these polymorphisms in a German MS case-control cohort. Twelve SNPs in the LEP, LEPR, GHRL and GHSR genes were genotyped in 776 MS patients and 878 control subjects. We found an association of a haplotype in the GHSR gene with MS that could not be replicated in a second cohort. Otherwise, no significant differences in allele or genotype frequencies were observed between patients and controls in this particular cohort. Thus, the present results do not support the hypothesis that genetic variation in the leptin/ghrelin system contributes substantially to the pathogenesis of MS. However, a modest effect of GHSR variation cannot be ruled out and needs to be further evaluated in future studies. Copyright © 2011 Elsevier Ltd. All rights reserved.

Efficiency of using rituximab in a patient with generalized granulomatosis with polyangiitis: A case report

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Gulazyk Malikovna Koilubaeva

2014-01-01

Full Text Available Systemic vasculitides (SVs are characterized by inflammation of the blood vessels wall; the spectrum of their clinical manifestations depends on the type, extent, and location of affected vessels and the activity of systemic inflammation. The etiology of most primary SVs is unknown. Antineutrophil cytoplasmic antibodies (ANCAs are implicated in its pathogenesis. The presence of ANCAa in patients' serum and the correlation of their level with the severity of clinical manifestations served as a basis for identifying a subgroup of systemic necrotizing vasculitides associated with ANCA synthesis: granulomatosis with polyangiitis (GPA, microscopic polyangiitis, and Churg – Strauss syndrome. GPA is characterized by systemic granulomatous necrotizing vasculitis involving the small vessels of the upper respiratory tract, lung, and kidney.The paper describes a case of difficult diagnosis and successful rituximab (RTM treatment of generalized GPA in a 45-year-old female patients. The disease occurred with local damage to the upper respiratory tract, granulomatous inflammation of the pulmonary vessels to form multiple infiltrates with lung tissue destruction elements and necrotizing glomerulonephritis. Despite intensive immunosuppressive treatment, there was a rapid GPA progression with the further development of respiratory failure, which had been induced by stenotic laryngitis subglottica leading to tracheostoma. Damage to the organ of vision could lead to severe complications, including amaurosis. RMT was shown to be effective in treating generalized GPA with a poor prognosis.

Assessment of subpleural opacities on high-resolution CT

International Nuclear Information System (INIS)

Choi, Hee Seok; Kim, Jeung Sook; Kang, Eun Young; Kim, Hak Hee

2007-01-01

The purpose of this study was to assess the value of HRCT for determining the cause of subpleural opacities. We evaluated 49 cases of subpleural opacities on HRCT scan, among with the patients with subpleural opacities seen on the conventional chest radiographs. Two 'blinded' reviewers retrospectively analyzed the CT scans by working in consensus. The patients consisted of COP (n = 14), NSIP (n = 13), UIP (n = 10), fibrosis associated with connective tissue disease or drug toxicity (n = 4), CEP (n = 4), Churg-Strauss syndrome (n = 2), DIP (n = 1) and AIP (n = 1). The predominant findings were consolidation (57%) with a peribronchovascular distribution (57%) in the COP patients, GGO (69%) and the associated focal reticular densities (61%) in the NSIP patients, and reticular or reticulonodular densities with a paucity of GGO in the UIP patients (100%). For the diagnosis of COP, NSIP and UIP, the use of HRCT demonstrated a high sensitivity (86%, 85% and 90%, respectively), specificity (97%, 86% and 95%) and accuracy (94%, 86% and 94%). Although an overlap of CT findings is seen for diseases showing subpleural opacities, consolidation with a subpleural and peribronchovascular distribution is highly suggestive for COP, subpleural GGO is highly suggestive of NSIP, subpleural reticular or reticulonodular densities with a paucity of GGO is highly suggestive of UIP and subpleural consolidation accompanied by reticular densities is suggestive of fibrosis

Assessment of subpleural opacities on high-resolution CT

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Choi, Hee Seok; Kim, Jeung Sook [Dngguk University International Hospital, Goyang (Korea, Republic of); Kang, Eun Young [Korea University Guro Hospital, Seoul (Korea, Republic of); Kim, Hak Hee [Asan Medical Center, University of Ulsan College of Medicine, Seoul (Korea, Republic of)

2007-11-15

The purpose of this study was to assess the value of HRCT for determining the cause of subpleural opacities. We evaluated 49 cases of subpleural opacities on HRCT scan, among with the patients with subpleural opacities seen on the conventional chest radiographs. Two 'blinded' reviewers retrospectively analyzed the CT scans by working in consensus. The patients consisted of COP (n = 14), NSIP (n = 13), UIP (n = 10), fibrosis associated with connective tissue disease or drug toxicity (n = 4), CEP (n = 4), Churg-Strauss syndrome (n = 2), DIP (n = 1) and AIP (n = 1). The predominant findings were consolidation (57%) with a peribronchovascular distribution (57%) in the COP patients, GGO (69%) and the associated focal reticular densities (61%) in the NSIP patients, and reticular or reticulonodular densities with a paucity of GGO in the UIP patients (100%). For the diagnosis of COP, NSIP and UIP, the use of HRCT demonstrated a high sensitivity (86%, 85% and 90%, respectively), specificity (97%, 86% and 95%) and accuracy (94%, 86% and 94%). Although an overlap of CT findings is seen for diseases showing subpleural opacities, consolidation with a subpleural and peribronchovascular distribution is highly suggestive for COP, subpleural GGO is highly suggestive of NSIP, subpleural reticular or reticulonodular densities with a paucity of GGO is highly suggestive of UIP and subpleural consolidation accompanied by reticular densities is suggestive of fibrosis.

Vasculites pulmonares: novas visões de uma velha conhecida Pulmonary forms of vasculitis: new perspectives on an old acquaintance

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Thais Thomaz Queluz

2005-07-01

after the discovery of antineutrophil cytoplasmic antibodies in 1982, and current classifications include Goodpasture's syndrome, forms of vasculitis related to antineutrophil cytoplasmic antibodies, forms caused by immune complexes and other forms. The evidence that antineutrophil cytoplasmic antibodies are involved in the pathogenesis of such conditions led to considerable advances in the diagnosis and treatment of vasculitis. Wegener's granulomatosis, Churg-Strauss syndrome and microscopic polyangiitis, all forms of vasculitis that have been associated with antineutrophil cytoplasmic antibodies, are the systemic forms that most affect the lungs. The most common presentation of such forms is characterized by cough, diffuse alveolar hemorrhage or difficult-to-control asthma. In Takayasu's arteritis, Behçet's disease and Henoch-Schönlein purpura, as well as in forms of vasculitis related to collagen diseases, pulmonary involvement is less common. In all forms of vasculitis, there is evidence that immune system processes are involved. It is based on this knowledge that new therapeutic approaches are proposed.

Defense of Truth (criticism and reconstructing the model of truth in the modern age and its influence on political philosophy from the viewpoints of Husserl and Strauss

Directory of Open Access Journals (Sweden)

MH Jamshidi

2014-03-01

Full Text Available Husserl and Strauss are two philosophers that intended to reform philosophic thought and thereupon political philosophy. But despite of common view about end and foundations, how and why Husserl is included in the class of pioneers of battle against metaphysical philosophy and Strauss is reputed against him as the leader of classic and Conservative Philosophy? According to Thomas Spreagens’ epistemological method, even though this Germanic teacher and his pupil have common preoccupation about the sunset of philosophical truth-seeking thought, their different understanding of themes of this crisis and ideal aspect have been ended in different and maybe opposite philosophies. Unlike Husserl who set a kind of Humanism and inter-subjective understanding as intellectual and cultural base for his plan, i.e. Phenomenology, Strauss talks about the natural essence of political issues and dependence of political philosophy on regulations which are out of human’s control. Thus, the type of post-metaphysical political philosophy that finds political objects inside ordinary life and social and historical relations and understandings is derived from Husserl’s Phenomenology, but Strauss’ insistence on classic political philosophy cautiously seeks to answer fundamental and pervasive political questions, while continuously and modestly exploring the nature of political issues.

The wild thought and child psycholoy: new approaches from the dialogue of Claude Levi-Strauss and Maurice Merleau-Ponty

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Paulo Victor Albertoni Lisboa

2012-05-01

Full Text Available The ideas about “logical”, “non-logical” and “pre-logical” consider an understanding about rationality or the discretion to state what rationality  intelligible is. Ethnology and phenomenology, when considering the dialog of Claude Lévi-Strauss and Maurice Merleau-Ponty, have in common the enlargement of concept about “rational” or “reason” as both authors make it intelligible as certain logical procedures that were previously not apprehensible in themselves. While Lévi-Strauss says there is no thought of the primitive, but only the savage mind, in the other words, a mode of knowledge closer to perception and imagination and which operates according to the logic of the concrete, in another sense, Maurice Merleau-Ponty draws on their work, about the perception and the development of an ontology of the sensible, to contest the claim that children have pre-logical thought, when judged against the criterion of “representation”. The two authors have, therefore, the perception is the foundation for the assertion of the savage mind or child psychology, and converge on the location of the arts space for the possibility of learning to see the world differently in Western culture.

A note on asymptotic expansions for sums over a weakly dependent random field with application to the Poisson and Strauss processes

DEFF Research Database (Denmark)

Jensen, J.L.

1993-01-01

Previous results on Edgeworth expansions for sums over a random field are extended to the case where the strong mixing coefficient depends not only on the distance between two sets of random variables, but also on the size of the two sets. The results are applied to the Poisson and the Strauss...

Clinical features and outcomes of ANCA-associated renal vasculitis

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Sidy Mohamed Seck

2012-01-01

Full Text Available To determine the patterns and outcomes of the pauci-immune vasculitis in the nephrology department at hospital La Conception in Marseille, we conducted a retrospective study including all patients with diagnosis of pauci-immune renal vasculitis between January 1, 2000 and December 31, 2007. Among 33 cases, 25 were diagnosed as Wegener granulomatosis (WG, seven as microscopic polyangitis (MPA and one as Churg-Strauss syndrome (SCS. The median age of the patients was 57.7 years and the sex-ratio (M/F was 1.6. The visceral mani-festations included kidneys (100% of patients, lungs (75%, ENT (52% of WG, and nervous system (57% of MPA. The mean serum creatinine at admission was 3.3 mg/dL. Renal biopsies revealed a pauci-immune crescentic gromerulonephritis in 96% of the cases. Two patients with WG received plasmapheresis and seven patients required emergency hemodialysis. Induction therapy comprised cyclophosphamide IV and corticosteroids, while maintenance therapy included azathioprine for the majority of patients. Eighty four percent of the patients experienced complete remission after induction therapy. During maintenance therapy relapses were more frequent among patients with MPA (28% compared to WG cases (12%. After 35 months of follow-up, eight patients ended on chronic hemodialysis, and five patients died. ANCA associated vasculitis are frequent in our patients. Long-term outcomes are relatively good despite a mortality rate of 15% and 25% of the patients entering dialysis after three years of follow-up.

A CULINÁRIA INDÍGENA COMO ELO DE PASSAGEM DA “CULTURA” PARA A “NATUREZA”: INVERTENDO LÉVI-STRAUSS

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MÁRTIN CÉSAR TEMPASS

2011-06-01

Full Text Available According to Claude Lévi-Strauss, the cuisine constitutes a passing link fromstage of nature to stage of culture. Based on ethnographic research among the Mbyá-Guarani,this article analyzes this assertion under the light of the Amerindian cosmology. However, thedichotomy between nature and culture does not apply to indigenous groups, since for thesenature and culture constitute a hybrid. To this hybridism might also be added the domain ofsupernatural. In terms of analysis this hybrid configuration does not allow any kind oftransition from one domain to another. Yet the transition is possible among the three possibleconditions of life in cosmos – animality, humanity and divinity. Human beings may perform thetransition to animality as well as the transition to divinity. But no matter the course of thetransition, although in different conditions in cosmos, from humanity one will always achieveanimality. As conceptualized by Lévi-Strauss, from culture always occurs the trasition to nature.However, in one direction ex-humans will control nature beings, in another one, they will becontrolled by nature. This isn’t just about a peculiarity of indigenous groups, it may also befound in societies so-called “modern”, however under others shapes.Segundo Claude Lévi-Strauss, a culinária constitui um elo de passagem da etapada natureza para o estágio da cultura. A partir da pesquisa etnográfica realizada entre osMbyá-Guarani, o presente artigo analisa esta afirmação sob a luz da cosmologia ameríndia.No entanto, a dicotomia entre natureza e cultura não se aplica aos grupos indígenas, pois, paraestes, as categorias natureza e cultura constituem um híbrido. E a este hibridismo podemosacrescentar também o domínio da sobrenatureza. E, em termos de análise, essa configuraçãohibrida não possibilita haver qualquer tipo de passagem entre um domínio e outro. Mas apassagem é possível entre as três possíveis condições de vida no cosmos

Cross-cultural structures of concentric and diametric dualism in Lévi-Strauss' structural anthropology: structures of relation underlying the self and ego relation?

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Downes, Paul

2003-02-01

The parallels between Jung's and Lévi-Strauss' concentric cross-cultural structures of the unconscious are highlighted. Lévi-Strauss' basic contrasts between concentric and diametric dualisms are developed into psychologically relevant differences regarding symmetry, connection and separation, and interaction between foreground and background structures respectively. These contrasts between concentric and diametric structures are applied as a common structural framework for understanding Jungian conceptions of the self, the ego and their relations, Freudian views of compensation and repetition in obsessional neurosis, and Winnicott's conception of a child's transitional object. Developing the contrasts between concentric and diametric structures reveals a compensatory relation between both structures. This supports an argument that concentric structures express the self and diametric structures express the ego in their mutual compensatory interrelation. Contrasting concentric with diametric structures challenges traditional Western logic, including Fordham's view that it is contradictory to treat the self as both centre and totality. It also develops an understanding of Jung's transcendent function which seeks to go beyond the ego-shadow opposition.

Femineidad y ficción en la obra operística de Richard Strauss

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María José Sánchez Usón

2014-01-01

Full Text Available Tras la muerte de Wagner y Brahms, Richard Straussemergió como uno de los compositores más importantesy populares de la música del siglo XX. Su trayectoria vitaly profesional, azarosa y turbulenta, al igual que las décadasque vivió, estuvo marcada por la realidad femenina.Es innegable que las mujeres de la vida de Strauss, todasde fuerte y distintiva personalidad, dejaron en él huellastan hondas que fue necesario llevarlas a escena, no sólocomo una aportación valiosa a la tipología caracterológicafemenina, sino también como un ejercicio terapéuticopersonal. Así, mujeres reales y mujeres ficticias se confundenen sus obras en un proceso creativo único: el delhombre-artista.

Living between reality and virtuality – on the work of Monika Fleischmann and Wolfgang Strauss

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Ryszard W. Kluszczyński

2012-12-01

Full Text Available It becomes clear that new media begin to function as multiplicators of new worlds, creating numerous virtual environments. This diversified, compound reality in many ways takes a shape of a network. New media art in this field of events has various functions. From one hand, it is one of the most refined instruments in the process of networkisation. From the other hand, it is also the tool of verification for these processes, critically but without prejudice evaluating its development, attentively observing what shapes do they took, as well as their real or possible results. In each case, new media art is an active participant of these processes, making full use of possibilities provided by the network system. Frequently while performing its activities it is intersected or linked with the processes which are a part of the world of science, creating together with them new cultural paradigm. Monika Fleischmann and Wolfgang Strauss undertook their joint, creative actions in combined fields of art and science in late 1980s. It was a special moment of a breakthrough for the formation of the character of modern civilization. Events taking place back then, as being a part of a sphere of phenomena generated by communication and information sciences, had enormous influence on the process of shaping a new cultural formation – cyber culture, and because of this, at the same time, had enormous influence on our ways of reality perception. From the beginning of their cooperation, Fleischmann and Strauss have found the tools appropriate for the realization of their works in the environemt of the internet. Their’s art has developed in parallel to the development of computer and internet environment, depicting dynamics of this network and inscribing, with the undertaken issues, in the circle of problems generated through out a development and transformations of accompanied cyber-cultural concepts.

[The relationship between the structuralism of Levi-Strauss and linguistic methods. An attempt at applying structuralist phonologic models to the study of pathologic language].

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Lhote, E

1975-01-01

The structuralism which is generally accepted as a 'good French' school of thinking, presents in some of its applications the short-time character of fashion in such a way that one often forgets what this philosophy implicates. In this essay, the author intends to show that even though a structural method really exists, one cannot say who exactly is 'the father' of structuralism. But during the long maturity period of thought that resulted in structuralism, one important step has been made in France by Lévi-Strauss who has been using a great number of linguistic concepts, and at the same time has contributed to the consequent progress realized in that discipline. In the light of linguistics and of the works made by Lévi-Strauss some notions are redefined: those of structure, those of oppositions in the systems, those of relations inside one system, and the interactions between 'significant' and 'signifié'. Born from recent research in mathematics and cybernetics, the notion of Model, very fertile in linguistics, is an important element in the Lévi-Strauss structural methodology. With the help of examples taken from phonetics and phonology, the author intends to show how to elaborate a model, of what nature are the links between the observed object and the model, and what can be the applications of that method in the study of pathological languages; the author also intends to show that the main interest in a model of structural type is its explicative and previsional quality. A critical study of the structural analysis makes it possible to show the real contribution of structuralism: the author presents new ways that have been revealed by structuralism and some developments later on favorized by this discipline, mainly in linguistics. Remark. In this study of structuralism, the author places herself on the level with 'method' and not with 'philosophy'.

A pragmatic approach to vasculitis in the gastrointestinal tract.

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Chetty, Runjan; Serra, Stefano

2017-06-01

Although vasculitis involving the gastrointestinal tract (GIT) is an uncommon occurrence, occasionally vasculitis can present as haemorrhagic infarction or ischaemia for which a length of bowel is removed. Invariably, the appropriate clinical history is not forthcoming, or vasculitis is not clinically suspected. The purpose of this overview is to provide the practising gastrointestinal (GI) pathologist with a framework to recognise and diagnose vasculitides within the GIT. The classification may be approached by aetiological agent or size of vessel involved; an international consensus group now favours the latter approach. The symptoms that systemic and/or localised vasculitis may cause in the GIT are protean and non-specific. As a result, pathologists examining resection specimens for unexplained haemorrhagic infarction or ischaemia should be aware that vasculitis may be a potential cause. Several well-known systemic vasculitides such as polyarteritis nodosa, microscopic polyangiitis, eosinophilic granulomatosis with polyangiitis or Churg-Strauss syndrome and granulomatosis with polyangiitis or Wegener's granulomatosis can occur in the GIT. The latter three constitute the antineutrophil cytoplasmic antibody-positive vasculitides. In addition, the so-called solitary organ vasculitis (SOV) can occur in the GIT as the harbinger of later onset systemic vasculitis, and be the cause of the GIT symptoms. In addition, SOV can occur incidentally and coexist with GIT disease such as gallstones or polyps, and there may be no manifestations of systemic vasculitis for years, or not at all. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/.

Corporate strategies and clothing supply chains reshaping towards the United States: Levi Strauss & Co firm’s situation

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Guadalupe García de León P.

2009-07-01

Full Text Available This article presents a summary of the corporate strategies of the Levi Strauss & Co. firm, a company that has had an important role in each one of the phases of Mexico’s process of integration into the global apparel production: the integration under the shared–production programs, the first stage of NAFTA, and the current stage of decline of Mexican exports and re–positioning of China. The central argument is that, by analyzing the competitive strategies of the leading firms of global apparel networks—as in the case of Levi’s—, we can get closer to the restructuring logic of these networks and understand the changing terms of the international insertion of the countries and firms involved, as in the case of Mexico.

Myocardial late gadolinium enhancement in specific cardiomyopathies by cardiovascular magnetic resonance: a preliminary experience.

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Silva, Caterina; Moon, James C; Elkington, Andrew G; John, Anna S; Mohiaddin, Raad H; Pennell, Dudley J

2007-12-01

Late gadolinium enhancement cardiovascular magnetic resonance (CMR) can visualize myocardial interstitial abnormalities. The aim of this study was to assess whether regions of abnormal myocardium can also be visualized by late enhancement gadolinium CMR in the specific cardiomyopathies. A retrospective review of all referrals for gadolinium CMR with specific cardiomyopathy over 20 months. Nine patients with different specific cardiomyopathies were identified. Late enhancement was demonstrated in all patients, with a mean signal intensity of 390 +/- 220% compared with normal regions. The distribution pattern of late enhancement was unlike the subendocardial late enhancement related to coronary territories found in myocardial infarction. The affected areas included papillary muscles (sarcoid), the mid-myocardium (Anderson-Fabry disease, glycogen storage disease, myocarditis, Becker muscular dystrophy) and the global sub-endocardium (systemic sclerosis, Loeffler's endocarditis, amyloid, Churg-Strauss). Focal myocardial late gadolinium enhancement is found in the specific cardiomyopathies, and the pattern is distinct from that seen in infarction. Further systematic studies are warranted to assess whether the pattern and extent of late enhancement may aid diagnosis and prognostic assessment.

Progranulin antibodies in autoimmune diseases.

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Thurner, Lorenz; Preuss, Klaus-Dieter; Fadle, Natalie; Regitz, Evi; Klemm, Philipp; Zaks, Marina; Kemele, Maria; Hasenfus, Andrea; Csernok, Elena; Gross, Wolfgang L; Pasquali, Jean-Louis; Martin, Thierry; Bohle, Rainer Maria; Pfreundschuh, Michael

2013-05-01

Systemic vasculitides constitute a heterogeneous group of diseases. Autoimmunity mediated by B lymphocytes and their humoral effector mechanisms play a major role in ANCA-associated vasculitis (AAV) as well as in non-ANCA associated primary systemic vasculitides and in the different types of autoimmune connective tissue disorders and rheumatoid arthritis. In order to detect autoantibodies in systemic vasculitides, we screened protein macroarrays of human cDNA expression libraries with sera from patients with ANCA-associated and ANCA-negative primary systemic vasculitides. This approach led to the identification of antibodies against progranulin, a 88 kDA secreted glycoprotein with strong anti-inflammatory activity in the course of disease of giant-cell arteritis/polymyalgia rheumatica (14/65), Takayasu's arteritis (4/13), classical panarteritis nodosa (4/10), Behcet's disease (2/6) and in the course of disease in granulomatosis with polyangiitis (31/75), Churg-Strauss syndrome (7/23) and in microscopic polyangiitis (7/19). In extended screenings the progranulin antibodies were also detected in other autoimmune diseases such as systemic lupus erythematosus (39/91) and rheumatoid arthritis (16/44). Progranulin antibodies were detected only in 1 of 97 healthy controls. Anti-progranulin positive patients with systemic vasculitides, systemic lupus erythematosus or rheumatoid arthritis had significant lower progranulin plasma levels, indicating a neutralizing effect. In light of the anti-inflammatory effects of progranulin, progranulin antibodies might exert pro-inflammatory effects thus contributing to the pathogenesis of the respective autoimmune diseases and might serve as a marker for disease activity. This hypothesis is supported by the fact that a positive progranulin antibody status was associated with active disease in granulomatosis with polyangiitis. Copyright © 2012 Elsevier Ltd. All rights reserved.

Sinusitis with eosinophilic otitis media

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Kawano, Toshiro; Ishitoya, Junichi; Tsukuda, Mamoru

2007-01-01

Eosinophilic otitis media is an intractable inflammation of the middle ear combined with bronchial asthma. According to a national epidemiological investigation on eosinophilic otitis media, it is assumed that eosinophilic otitis media are combined with sinusitis in about 74% of their cases. On the other hand, organizational images of eosinophilic otitis media and eosinophilic sinusitis are similar, and steroid therapy is effective together, and it is thought that they are involved in the idea of one airway one disease, but the details of sinusitis combined with the eosinophilic otitis media are unidentified. Therefore, we examined the kinds of the sinusitis combined with eosinophilic otitis media. We diagnosed 18 cases (male: 2 cases, female: 16 cases) (average age: 54.6 years old) as eosinophilic otitis media according to the diagnostic criteria. And, by the CT views of a paranasal sinus, blood tests, existence of the nasal polyp, etc, we investigated the kinds of sinusitis combined with eosinophilic otitis media. It turned out that bronchial asthma was combined with eosinophilic otitis media in 17 of 18 cases (airway hypersensitivity did sthenia of one case, but the asthma did not yet developed), and 6 cases were combined with aspirin induced asthma (AIA), and 3 cases were combined with Churg-Strauss syndromes (CSS). 10 case (55.6%) of 17 eosinophilic otitis media were combined with eosinophilic sinusitis. And 4 cases (22.2%) of 17 eosinophilic otitis media were combined with chronic sinusitis, 4 cases (22.2%) of 17 eosinophilic otitis media were not combined with sinusitis. We concluded that eosinophilic otitis media was not always combined with eosinophilic sinusitis. The idea of one airway one disease was not applied to this examination. (author)

Sinusitis with eosinophilic otitis media

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Kawano, Toshiro; Ishitoya, Junichi [Yokohama City Univ., Medical Center, Yokohama, Kanagawa (Japan); Tsukuda, Mamoru [Yokohama City Univ., Graduate School of Medicine, Yokohama, Kanagawa (Japan)

2007-09-15

Eosinophilic otitis media is an intractable inflammation of the middle ear combined with bronchial asthma. According to a national epidemiological investigation on eosinophilic otitis media, it is assumed that eosinophilic otitis media are combined with sinusitis in about 74% of their cases. On the other hand, organizational images of eosinophilic otitis media and eosinophilic sinusitis are similar, and steroid therapy is effective together, and it is thought that they are involved in the idea of one airway one disease, but the details of sinusitis combined with the eosinophilic otitis media are unidentified. Therefore, we examined the kinds of the sinusitis combined with eosinophilic otitis media. We diagnosed 18 cases (male: 2 cases, female: 16 cases) (average age: 54.6 years old) as eosinophilic otitis media according to the diagnostic criteria. And, by the CT views of a paranasal sinus, blood tests, existence of the nasal polyp, etc, we investigated the kinds of sinusitis combined with eosinophilic otitis media. It turned out that bronchial asthma was combined with eosinophilic otitis media in 17 of 18 cases (airway hypersensitivity did sthenia of one case, but the asthma did not yet developed), and 6 cases were combined with aspirin induced asthma (AIA), and 3 cases were combined with Churg-Strauss syndromes (CSS). 10 case (55.6%) of 17 eosinophilic otitis media were combined with eosinophilic sinusitis. And 4 cases (22.2%) of 17 eosinophilic otitis media were combined with chronic sinusitis, 4 cases (22.2%) of 17 eosinophilic otitis media were not combined with sinusitis. We concluded that eosinophilic otitis media was not always combined with eosinophilic sinusitis. The idea of one airway one disease was not applied to this examination. (author)

Características generales de 29 pacientes con vasculitis de pequeños vasos General characteristics of 29 patients with small vessel vasculitis

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Nicolás Di Benedetto

2010-04-01

period of 16 years were reviewed in a retrospective way. All patients selected fulfilled diagnostic criteria of small vessel vasculitis. The data were extracted and the analysis of survival was completed by phone. Later a bibliographical search was carried out and the results were compared. Thirteen patients with Wegener's granulomatosis, 6 with Churg-Strauss syndrome and 10 with microscopic polyangiitis were included. Fifty five percent (16 were under 55 years old when diagnosis was made and male/female ratio was 2.6 to 1. The diagnostic delay was over a year in 46% of the cases. Respiratory and ear-nose-throat were the most frequently affected systems. Anti-neutrophil cytoplasmic antibodies were present in 79% of patients. Overall mortality was 24% (7/29. There were several differences between the results of our series and the literature: the presentation form, affected systems and percentage of patients with anti-neutrophil cytoplasmic antibodies. Greater diagnostic delay and worse prognosis were observed in anti-neutrophil cytoplasmic antibody negative patients. Special attention should be given to these antibodies since they constitute a significant tool at the time of diagnosis.

Investigação de fatores associados à asma de difícil controle Investigation of factors associated with difficult-to-control asthma

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Ana Carla Sousa de Araujo

2007-10-01

ínicas. The patients were divided into two groups: controlled severe asthma and difficult-to-control severe asthma. After new attempts to optimize the severe asthma treatment, a questionnaire was applied, and additional tests for factors associated with difficult-to-control asthma, such as environmental and occupational exposure, smoking history, social factors, rhinitis/sinusitis, gastroesophageal reflux disease (GERD, obstructive sleep apnea, congestive heart failure (CHF, pulmonary embolism, cystic fibrosis, vocal cord dysfunction, alpha-1 antitrypsin deficiency, and Churg-Strauss syndrome, were performed. RESULTS: 77 patients with severe asthma were selected, of which 47 suffered from hard-to-control asthma, being 68.1% female, with mean age of 44.4 years (±14.4, and forced expiratory volume in one second of 54.7% (±18.3. The most factors most often associated with difficult-to-control asthma were noncompliance with treatment (68%, rhinitis/sinusitis (57%, GERD (49%, environmental exposure (34%, occupational exposure (17%, smoking history (10%, obstructive sleep apnea (2%, and CHF (2%. At least one of these factors was identified in every case. CONCLUSIONS: Noncompliance with treatment was the factor most often associated with difficult-to-control asthma, underscoring the need to investigate comorbidities in the evaluation of patients with this form of the disease.

The environmental pollutant hexachlorobenzene causes eosinophilic and granulomatous inflammation and in vitro airways hyperreactivity in the Brown Norway rat

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Michielsen, C.; Zeamari, S.; Vos, J.; Leusink-Muis, A.; Bloksma, N.

2002-01-01

Based on observations that the persistent environmental pollutant hexachlorobenzene (HCB) induces inflammatory skin lesions and eosinophilic and granulomatous lung pathology as well as in vivo airways hyperresponsiveness to methacholine in the BN/SsNOlaHsd rat (Michielsen et al., Toxicol Appl Pharmacol 172:11-20, 2001), which are features of human Churg-Strauss syndrome (CSS), we have investigated whether HCB induced other features of CSS such as asthma and systemic vasculitis involving the heart and kidneys in this strain of rat. To this end, BN/SsNOlaHsd rats received control feed or feed supplemented with 450 mg/kg HCB. On days 6, 14 or 21, tracheas were isolated to assess non-specific in vitro airways hyperresponsiveness (AHR) to cumulative concentrations of arecoline and serotonin. In addition, lungs were lavaged to count and differentiate lavage cells, and skin, lungs, heart, kidneys, and lymph nodes were processed for histopathological investigation. HCB induced eosinophilic and granulomatous lung pathology in the BN/SsNOlaHsd rat, which became more severe with time and was associated with significant in vitro AHR to arecoline. Moreover, as in CSS-patients, systemic effects on spleen and lymph nodes were observed in HCB-fed BN/SsNOlaHsd rats, as well as development of skin lesions with vascular changes and eosinophilic infiltrates. In contrast, cardiac or renal involvement, frequently seen in CSS-patients, was not seen in HCB-fed rats. More importantly, there were no indications of necrotizing vasculitis, a hallmark feature of CSS, in the lungs and skin of BN/SsNOlaHsd rats. Thus, it is concluded that the persistent environmental pollutant HCB possibly induces a mild or early stage of CSS in the BN/SsNOlaHsd rat that may evolve into fully developed CSS after prolonged exposure to HCB. (orig.)

The environmental pollutant hexachlorobenzene causes eosinophilic and granulomatous inflammation and in vitro airways hyperreactivity in the Brown Norway rat

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Michielsen, C; Zeamari, S; Vos, J [Department of Pathology, Faculty of Veterinary Medicine, Utrecht University (Netherlands); Leusink-Muis, A; Bloksma, N [Department of Pharmacology and Pathophysiology, Utrecht Institute for Pharmaceutical Sciences and Faculty of Biology, Utrecht University, Utrecht (Netherlands)

2002-05-01

Based on observations that the persistent environmental pollutant hexachlorobenzene (HCB) induces inflammatory skin lesions and eosinophilic and granulomatous lung pathology as well as in vivo airways hyperresponsiveness to methacholine in the BN/SsNOlaHsd rat (Michielsen et al., Toxicol Appl Pharmacol 172:11-20, 2001), which are features of human Churg-Strauss syndrome (CSS), we have investigated whether HCB induced other features of CSS such as asthma and systemic vasculitis involving the heart and kidneys in this strain of rat. To this end, BN/SsNOlaHsd rats received control feed or feed supplemented with 450 mg/kg HCB. On days 6, 14 or 21, tracheas were isolated to assess non-specific in vitro airways hyperresponsiveness (AHR) to cumulative concentrations of arecoline and serotonin. In addition, lungs were lavaged to count and differentiate lavage cells, and skin, lungs, heart, kidneys, and lymph nodes were processed for histopathological investigation. HCB induced eosinophilic and granulomatous lung pathology in the BN/SsNOlaHsd rat, which became more severe with time and was associated with significant in vitro AHR to arecoline. Moreover, as in CSS-patients, systemic effects on spleen and lymph nodes were observed in HCB-fed BN/SsNOlaHsd rats, as well as development of skin lesions with vascular changes and eosinophilic infiltrates. In contrast, cardiac or renal involvement, frequently seen in CSS-patients, was not seen in HCB-fed rats. More importantly, there were no indications of necrotizing vasculitis, a hallmark feature of CSS, in the lungs and skin of BN/SsNOlaHsd rats. Thus, it is concluded that the persistent environmental pollutant HCB possibly induces a mild or early stage of CSS in the BN/SsNOlaHsd rat that may evolve into fully developed CSS after prolonged exposure to HCB. (orig.)

Focus on audiologic impairment in eosinophilic granulomatosis with polyangiitis.

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Seccia, Veronica; Fortunato, Susanna; Cristofani-Mencacci, Lodovica; Dallan, Iacopo; Casani, Augusto P; Latorre, Manuela; Paggiaro, Pierluigi; Bartoli, Maria Laura; Sellari-Franceschini, Stefano; Baldini, Chiara

2016-12-01

To evaluate the clinical features of audiologic impairment and its relationship with the nasal, vestibular, and rheumatologic profile in a cohort of patients with eosinophilic granulomatosis with polyangiitis (EGPA), formerly named Churg-Strauss syndrome. Prospective cross-sectional study. Thirty-nine patients with EGPA, considered controlled according to the Birmingham Vasculitis Activity Score and the Vasculitis Damage Index, underwent a complete audiologic evalutaion with otomicroscopy, impedance audiometry, speech audiometry, and auditory brainstem responses; rhinologic evaluation was made by means of fiberoptic endoscopy and nasal cytology; the clinical evaluation was completed with analysis of the facial function and, in patients with referred vertigo, with videonystagmography (VNG). Data were compared to the rheumatologic profile (eosinophil count, antineutrophil cytoplasmic antibodies status). Thirty-four of 39 patients fulfilled the inclusion criteria. Of those, 18 (52, 8%) were affected by variable degrees of hearing loss (sensorineural hearing loss [SNHL]) in four cases (11, 8%), mixed sensorineural and conductive hearing loss in two (5, 9%), presbycusis in six (17, 6%), and otitis media with effusion (OME) in six (17, 6%). Vestibular impairment was represented by benign paroxysmal positional vertigo and unspecific dizziness in three (8, 8%) and four cases (11, 8%), respectively, all with normal VNG. Ear involvement was statistically related to the EGPA vasculitic pattern and independent from the nasal impairment, cytology, and duration of nasal symptoms. No facial palsy was registered. In our experience, the largest in the existing literature, the otological involvement in EGPA is common and may occur variably as SNHL or OME. Otological involvement occurs early in the course of the disease process, but is nonspecific in making the diagnosis of EGPA. Its recognition is therefore fundamental. 4. Laryngoscope, 126:2792-2797, 2016. © 2016 The American

Revisiting the systemic vasculitis in eosinophilic granulomatosis with polyangiitis (Churg-Strauss): A study of 157 patients by the Groupe d'Etudes et de Recherche sur les Maladies Orphelines Pulmonaires and the European Respiratory Society Taskforce on eosinophilic granulomatosis with polyangiitis (Churg-Strauss).

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Cottin, Vincent; Bel, Elisabeth; Bottero, Paolo; Dalhoff, Klaus; Humbert, Marc; Lazor, Romain; Sinico, Renato A; Sivasothy, Pasupathy; Wechsler, Michael E; Groh, Matthieu; Marchand-Adam, Sylvain; Khouatra, Chahéra; Wallaert, Benoit; Taillé, Camille; Delaval, Philippe; Cadranel, Jacques; Bonniaud, Philippe; Prévot, Grégoire; Hirschi, Sandrine; Gondouin, Anne; Dunogué, Bertrand; Chatté, Gérard; Briault, Christophe; Pagnoux, Christian; Jayne, David; Guillevin, Loïc; Cordier, Jean-François

2017-01-01

To guide nosology and classification of patients with eosinophilic granulomatosis with polyangiitis (EGPA) based on phenotype and presence or absence of ANCA. Organ manifestations and ANCA status were retrospectively analyzed based on the presence or not of predefined definite vasculitis features or surrogates of vasculitis in patients asthma, eosinophilia, and at least one systemic organ manifestation attributable to systemic disease. The study population included 157 patients (mean age 49.4±14.1), with a follow-up of 7.4±6.4years. Patients with ANCA (31%) more frequently had weight loss, myalgias, arthralgias, biopsy-proven vasculitis, glomerulonephritis on biopsy, hematuria, leukocytoclastic capillaritis and/or eosinophilic infiltration of arterial wall on biopsy, and other renal disease. A total of 41% of patients had definite vasculitis manifestations (37%) or strong surrogates of vasculitis (4%), of whom only 53% had ANCA. Mononeuritis multiplex was associated with systemic vasculitis (p=0.005) and with the presence of ANCA (p<0.001). Overall, 59% of patients had polyangiitis as defined by definite vasculitis, strong surrogate of vasculitis, mononeuritis multiplex, and/or ANCA with at least one systemic manifestation other than ENT or respiratory. Patients with polyangiitis had more systemic manifestations including arthralgias (p=0.02) and renal disease (p=0.024), had higher peripheral eosinophilia (p=0.027), and a trend towards less myocarditis (p=0.057). Using predefined criteria of vasculitis and surrogates of vasculitis, ANCA alone were found to be insufficient to categorise patients with vasculitis features. We suggest a revised nomenclature and definition for EGPA and a new proposed entity referred to as hypereosinophilic asthma with systemic (non vasculitic) manifestations. Copyright © 2016 Elsevier B.V. All rights reserved.

Focus on the Involvement of the Nose and Paranasal Sinuses in Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss Syndrome): Nasal Cytology Reveals Infiltration of Eosinophils as a Very Common Feature.

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Seccia, Veronica; Baldini, Chiara; Latorre, Manuela; Gelardi, Matteo; Dallan, Iacopo; Cristofani-Mencacci, Lodovica; Sellari-Franceschini, Stefano; Bartoli, Maria Laura; Bacci, Elena; Paggiaro, Pierluigi

2018-01-01

Eosinophilic granulomatosis with polyangiitis (EGPA) is a necrotizing vasculitis that predominantly affects small- to medium-sized vessels. It is characterized by a wide spectrum of extrapulmonary symptoms, including sinonasal and paranasal sinus abnormalities. These are the most common features of this disease, constituting diagnostic criteria for EGPA. However, the actual clinical features, cellular mechanisms and impact on patients' quality of life (QoL) are still a matter of study. Thirty-nine EGPA patients underwent multidimensional rhinological evaluations, including rhinofibroscopy, nasal cytology, and QoL questionnaires. This was coupled with respiratory and rheumatological assessments. Twenty-eight patients were diagnosed with chronic rhinosinusitis (CRS). Of these, 18 had nasal polyposis (NP). Chronic rhinitis was diagnosed in 10 patients. Of these, 3 had allergic rhinitis (AR) and seven had non-AR (NAR). Overall, only 1 patient (2.6%) was normal. Nasal cytology showed that hypereosinophilia was present in 17/28 patients with CRS, 4/7 patients with NAR and all patients with AR. SNOT-22 and SF-36 showed a severe impact of nasal symptoms on QoL. No differences in asthma control or rheumatological patterns for EGPA were observed among patients with or without NP. Even when the rheumatological assessment scored EGPA "under control" according to the Birmingham Vasculitis Activity Score and Vasculitis Damage Index, sinonasal diseases and related nasal inflammatory processes were not controlled. Therefore, there is a need for clinical monitoring and targeted treatment to control the inflammatory processes and improve the QoL of EGPA patients. © 2018 S. Karger AG, Basel.

Safety and efficacy of montelukast as adjunctive therapy for treatment of asthma in elderly patients

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Scichilone N

2013-10-01

Full Text Available Nicola Scichilone, Salvatore Battaglia, Alida Benfante, Vincenzo BelliaDipartimento Biomedico di Medicina Interna e Specialistica, University of Palermo, Palermo, ItalyAbstract: Asthma is a disease of all ages. This assumption has been challenged in the past, because of several cultural and scientific biases. A large body of evidence has accumulated in recent years to confirm that the prevalence of asthma in the most advanced ages is similar to that in younger ages. Asthma in the elderly may show similar functional and clinical characteristics to that occurring in young adults, although the frequent coexistence of comorbid conditions in older patients, together with age-associated changes in the human lung, may lead to more severe forms of the disease. Management of asthma in the elderly follows specific guidelines that apply to all ages, although most behaviors are pure extrapolation of what has been tested in young ages. In fact, age has always represented an exclusion criterion for eligibility to clinical trials. This review focuses specifically on the safety and efficacy of leukotriene modifiers, which represent a valid option in the treatment of allergic asthma, both as an alternative to first-line drugs and as add-on treatment to inhaled corticosteroids. Available studies specifically addressing the role of montelukast in the elderly are scarce; however, leukotriene modifiers have been demonstrated to be safe in this age group, even though cases of acute hepatitis and occurrence of Churg-Strauss syndrome have been described in elderly patients; whether this is associated with age is to be confirmed. Furthermore, leukotriene modifiers provide additional benefit when added to regular maintenance therapy, not differently from young asthmatics. In elderly patients, the simpler route of administration of leukotriene modifiers, compared with the inhaled agents, could represent a more effective strategy in improving the outcomes of asthma therapy

Influence of integrated quality management system on processing management in management and production staff’s opinion in Strauss Cafe Poland Ltd Company

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Justyna Górna

2009-01-01

Full Text Available In the article are presented the results of the research determining the influence of the integrated quality management system on management process in the assessment of management and regular employees of Strauss Cafe Poland Ltd. According to the ISO 9001 requirements in order to effectively manage the organisation, should identify operations. The activities requiring resources and managed in order to enable the transformation of inflow into outflow should be evaluated as a process. The analysis of the gathered data shows the essential influence of the systems (QMS and HACCP on the management process in the company. The management staff emphasized the significant influence of the quality management system whereas production staff of HACCP system. Both of these groups of employees in the survey shows the beurocracy as a negative result of these systems, while positive results are improvement of image of the company and increase of both products and services quality.

Minding experience: An exploration of the concept of "experience" in the early French anthropology of Durkheim, Lévy-Bruhl, and Lévi-Strauss.

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Throop, C Jason

2003-01-01

In line with the growing concern with the unexamined reliance upon the concept of "experience" in anthropology, this article explores in some detail the various usages and definitions of the concept in the work of three of early French anthropology's most influential theorists: Emile Durkheim (1858-1918), Lucien Lévy-Bruhl (1857-1939), and Claude Lévi-Strauss (1908-). With its important influence on both British and American anthropology, the early French anthropological tradition, as epitomized in the writings of these three thinkers, has indeed played a pivotal role in shaping many current taken-for-granted understandings of the concept of experience in the discipline of anthropology as a whole. In the process of exploring how experience is viewed by these three scholars, this paper will thus take some initial steps toward the historical contextualization of many of the unquestioned assumptions underpinning current understandings of experience in the discipline of anthropology and the social sciences more generally. Copyright 2003 Wiley Periodicals, Inc.

De Sahlins a Claude Lévi-strauss: no setor transpacífico do sistema mundial

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Marcos Lanna

2001-12-01

Full Text Available Privilegiando o fato de a obra de Marshall Sahlins dar bom uso à teoria lévi-straussiana da troca, este artigo reinterpreta a análise do primeiro sobre o "setor transpacífico do 'sistema mundial'". Mais do que uma crítica a Sahlins, o artigo pretende aprofundar a demonstração deste último do fato de que entendermos sistemas de trocas "nativos" (ou "autóctones" ou "indígenas" é condição necessária para entendermos relações mais amplas, como aquelas entre estes sistemas e os "capitalistas" (ou "ocidentais", seja lá como os definamos. Isto nos leva a minimizar a distinção entre trocas inter e intra culturais, conclusão esta que se reforça com uma rápida especificação da noção de troca das Estruturas elementares do parentesco apresentada no final do artigo.Focussing on the fact that Marshall Sahlins' work revolves around the Lévy-Strauss's theory of exchange, this article reinterprets the analysis of the first on the "trans-pacific sector of the 'world system'". Rather than a critique of Sahlins, this article proposes to further elaborate the latter's demonstration that the comprehension of "native" (or autochthonous, or indigenous systems of exchange is a necessary condition for the comprehension of wider relations, such as those between these systems and their "capitalist" (or "Occidental", etc. counterparts. This leads us to minimize the distinction between inter- and intra-cultural exchanges, a conclusion reinforced by a rapid look at the notion of exchange in the Elementary Structures of Kinship presented at the end of the article.

Idiopathic Pulmonary Hemosiderosis in a Young Adult Patient: A Rare Case

Directory of Open Access Journals (Sweden)

Abhishek Agarwal

2018-01-01

Full Text Available Idiopathic pulmonary hemosiderosis (IPH is often an ignored and rare cause of diffuse alveolar hemorrhage (DAH. It is characterized by triad of hemoptysis, anemia, and alveolar opacity on radiology. It is a diagnosis of exclusion, established after ruling out other causes of DAH such as Goodpasture’s syndrome, large vessel vasculitis, small vessel vasculitis associated with anti-neutrophil cytoplasmic antibody (ANCA (Wegener’s granulomatosis, Churg–Strauss syndrome, microscopic polyangiitis, immune complex-related vasculitis (collagen vascular diseases, Henoch–Schönlein purpura, mixed cryoglobulinemia drug reactions, anticoagulation and thrombocytopenia. Though it is a disease primarily affecting children, we hereby report a case of IPH in an adult patient who responded dramatically to oral corticosteroid.

Value of serum IgG4 in the diagnosis of IgG4-related disease and in differentiation from rheumatic diseases and other diseases.

Science.gov (United States)

Yamamoto, Motohisa; Tabeya, Tetsuya; Naishiro, Yasuyoshi; Yajima, Hidetaka; Ishigami, Keisuke; Shimizu, Yui; Obara, Mikiko; Suzuki, Chisako; Yamashita, Kentaro; Yamamoto, Hiroyuki; Hayashi, Toshiaki; Sasaki, Shigeru; Sugaya, Toshiaki; Ishida, Tadao; Takano, Ken-Ichi; Himi, Tetsuo; Suzuki, Yasuo; Nishimoto, Norihiro; Honda, Saho; Takahashi, Hiroki; Imai, Kohzoh; Shinomura, Yasuhisa

2012-06-01

IgG4-related disease (IgG4-RD) is a novel disease entity that includes Mikulicz's disease, autoimmune pancreatitis (AIP), and many other conditions. It is characterized by elevated serum IgG4 levels and abundant IgG4-bearing plasmacyte infiltration of involved organs. We postulated that high levels of serum IgG4 would comprise a useful diagnostic tool, but little information is available about IgG4 in conditions other than IgG4-RD, including rheumatic diseases. Several reports have described cutoff values for serum IgG4 when diagnosing IgG4-RD, but these studies mostly used 135 mg/dL in AIP to differentiate from pancreatic cancer instead of rheumatic and other common diseases. There is no evidence for a cutoff serum IgG4 level of 135 mg/dL for rheumatic diseases and common diseases that are often complicated with rheumatic diseases. The aim of this work was to re-evaluate the usual cutoff serum IgG4 value in AIP (135 mg/dL) that is used to diagnose whole IgG4-RD in the setting of a rheumatic clinic by measuring serum IgG4 levels in IgG4-RD and various disorders. We therefore constructed ROC curves of serum IgG4 levels in 418 patients who attended Sapporo Medical University Hospital due to IgG4-RD and various rheumatic and common disorders. The optimal cut-off value of serum IgG4 for a diagnosis of IgG4-RD was 144 mg/dL, and the sensitivity and specificity were 95.10 and 90.76%, respectively. Levels of serum IgG4 were elevated in IgG4-RD, Churg-Strauss syndrome, multicentric Castleman's disease, eosinophilic disorders, and in some patients with rheumatoid arthritis, systemic sclerosis, chronic hepatitis, and liver cirrhosis. The usual cut-off value of 135 mg/dL in AIP is useful for diagnosing whole IgG4-RD, but high levels of serum IgG4 are sometimes observed in not only IgG4-RD but also other rheumatic and common diseases.

Levi-Strauss Ve Roland Barthes’ın Yaklaşımıyla “God Of War III” Oyununun Mitsel Çözümlemesi

OpenAIRE

SEPETÇİ, Tülin

2016-01-01

Bu çalışmanın amacı, Roland Barthes ve Levi-Strauss’un mit kuramlarını bir popüler kültür ürünü olarak ele alınan “God of War III” adlı video oyunu bağlamında karşılaştırmaktır. Çalışmada öncelikle tarihsel süreç içerisinde mitlerin işlevi ve “oyun” kavramının mitlerle ilişkisinden, ardından Levi-Strauss ve Roland Barthes’ın mit yaklaşımları ve analizinden bahsedilmiş, ardından “God of War III” adlı video oyununa mitsel çözümleme yapılmıştır. Her iki düşünürün mit yaklaşımları çerçevesinde ön...

Understanding Bartter syndrome and Gitelman syndrome.

Science.gov (United States)

Fremont, Oliver T; Chan, James C M

2012-02-01

We aim to review the clinical features of two renal tubular disorders characterized by sodium and potassium wasting: Bartter syndrome and Gitelman syndrome. Selected key references concerning these syndromes were analyzed, together with a PubMed search of the literature from 2000 to 2011. The clinical features common to both conditions and those which are distinct to each syndrome were presented. The new findings on the genetics of the five types of Bartter syndrome and the discrete mutations in Gitelman syndrome were reviewed, together with the diagnostic workup and treatment for each condition. Patients with Bartter syndrome types 1, 2 and 4 present at a younger age than classic Bartter syndrome type 3. They present with symptoms, often quite severe in the neonatal period. Patients with classic Bartter syndrome type 3 present later in life and may be sporadically asymptomatic or mildly symptomatic. The severe, steady-state hypokalemia in Bartter syndrome and Gitelman syndrome may abruptly become life-threatening under certain aggravating conditions. Clinicians need to be cognizant of such renal tubular disorders, and promptly treat at-risk patients.

Epidemiology of ANCA associated vasculitis

Directory of Open Access Journals (Sweden)

Wenche Koldingsnes

2009-10-01

Full Text Available ANCA associated vasculitis (AAV comprises three syndromes with systemic vasculitis (Wegener’s granulomatosis (WG, Churg Strauss syndrome (CSS and icroscopic polyangiitis (MPA, which all involve small and medium sized vessels and are associated with antibodies against cytoplasmatic antibodies in neutrophils (ANCA. Polyarteritis nodosa (PAN is included in this review as it also affects medium sized vessels, and has many clinical findings in common with the AAV.Since the recognition of ANCA, increasing data have become available on the epidemiology of these vasculitidis. WG constitutes half of the AAV and its prevalence has increased from 30/million in the late 1980’s in the USA to 160/million in this century in northern Europe. The prevalence for the whole group of primary systemic vasculitides is now 300/million in Sweden. The annual incidence of WG increased from 6.0/million to 14/million during the 1990’s in Tromsø, but it is unknown if this is a true increase or the result of an increased awareness of the diagnosis. For the whole group of AAV, the annual incidence in most more recent studies is relatively constant over time and by geographical location, ranging from 13 to 21/million. Nonetheless there are interesting differences in the prevalence of specific vasculitis between different geographical areas, as well as for sub specificities of ANCA.There seems to be a South-North gradient for WG and PR3-ANCA with high figures reported from northern Europe and southern New Zealand. In European studies WG is 90% PR3-ANCA positive. MPA which is predominantly MPO-ANCA associated are more frequent in the Mediterranean countries and also has an increasing gradient towards east-Asia, as almost all AAV in China and Japan are diagnosed as MPA, predominantly MPO-ANCA positive.There are also some ethnic and gender differences. WG is most prevalent among Caucasians in the USA and in people with European ancestors in Paris and in New Zealand, less

Systemic Polyarteritis Nodosa as the Cause of Sudden Onset Bilateral Sensorineural Hearing Loss Following Lassa Virus Infection

Science.gov (United States)

2016-07-05

weakness; 5) mono- or polyneuropathy; 6) hypertension ; 7) elevated BUN or creatinine; 8) antibodies to hepatitis B surface antigen in serum; 9...day 6, with those of the two survivors peaking 14 days after infection followed by virus clearance by day 28 (Fig. 1C). Blood chemistry and...Surg 109, 559 (1993); published online EpubSep ( 14. J. Churg, A. Churg, Idiopathic and secondary vasculitis: a review. Mod Pathol 2, 144-160 (1989

[Maxillofacial and dental abnormalities in some multiple abnormality syndromes. "Cri du chat" syndrome, Wilms' tumor-aniridia syndrome; Sotos syndrome; Goldenhar syndrome].

Science.gov (United States)

Berio, A; Trucchi, R; Meliota, M

1992-05-01

The paper describes the maxillo-facial and dental anomalies observed in some chromosome and non-chromosome poly-malformative syndromes ("Cri du chat" syndrome; Wilms' tumour; Sotos' syndrome; Goldenhar's syndrome). The Authors emphasise the possibility of diagnosing these multiple deformity syndromes from maxillo-facial alterations in early infancy; anomalous tooth position and structure cal also be successfully treated immediately after the first appearance of teeth. This is a particularly promising field of pediatrics and preventive pediatric medicine.

Marfan syndrome masked by Down syndrome?

NARCIS (Netherlands)

Vis, J.C.; Engelen, K. van; Timmermans, J.; Hamel, B.C.J.; Mulder, B.J.

2009-01-01

Down syndrome is the most common chromosomal abnormality. A simultaneous occurrence with Marfan syndrome is extremely rare. We present a case of a 28-year-old female with Down syndrome and a mutation in the fibrillin-1 gene. The patient showed strikingly few manifestations of Marfan syndrome.

The developmental trajectory of disruptive behavior in Down syndrome, fragile X syndrome, Prader-Willi syndrome and Williams syndrome.

Science.gov (United States)

Rice, Lauren J; Gray, Kylie M; Howlin, Patricia; Taffe, John; Tonge, Bruce J; Einfeld, Stewart L

2015-06-01

The aim of this study was to investigate the developmental trajectories of verbal aggression, physical aggression, and temper tantrums in four genetic syndrome groups. Participants were part of the Australian Child to Adult Development Study (ACAD), which collected information from a cohort of individuals with an intellectual disability at five time points over 18 years. Data were examined from a total of 248 people with one of the four following syndromes: Down syndrome, Fragile X syndrome, Prader-Willi syndrome, or Williams syndrome. Changes in behaviors were measured using validated items from the Developmental Behavior Checklist (DBC). The results indicate that, while verbal aggression shows no evidence of diminishing with age, physical aggression, and temper tantrums decline with age before 19 years for people with Down syndrome, Fragile X syndrome, and William syndrome; and after 19 years for people with Prader-Willi syndrome. These findings offer a somewhat more optimistic outlook for people with an intellectual disability than has previously been suggested. Research is needed to investigate the mechanisms predisposing people with PWS to persistence of temper tantrums and physical aggression into adulthood. © 2015 Wiley Periodicals, Inc.

Hamartomatous polyposis syndromes

DEFF Research Database (Denmark)

Jelsig, Anne Marie; Qvist, Niels; Brusgaard, Klaus

2014-01-01

Hamartomatous Polyposis Syndromes (HPS) are genetic syndromes, which include Peutz-Jeghers syndrome, Juvenile polyposis syndrome, PTEN hamartoma tumour syndrome (Cowden Syndrom, Bannayan-Riley-Ruvalcaba and Proteus Syndrome) as well as hereditary mixed polyposis syndrome. Other syndromes such as ......Hamartomatous Polyposis Syndromes (HPS) are genetic syndromes, which include Peutz-Jeghers syndrome, Juvenile polyposis syndrome, PTEN hamartoma tumour syndrome (Cowden Syndrom, Bannayan-Riley-Ruvalcaba and Proteus Syndrome) as well as hereditary mixed polyposis syndrome. Other syndromes...

Seckel syndrome: an overdiagnosed syndrome.

OpenAIRE

Thompson, E; Pembrey, M

1985-01-01

Five children in whom a diagnosis of Seckel syndrome had previously been made were re-examined in the genetic unit. One child had classical Seckel syndrome, a sib pair had the features of the syndrome with less severe short stature, and in two children the diagnosis was not confirmed. Seckel syndrome is only one of a group of low birth weight microcephalic dwarfism and careful attention should be paid to fulfillment of the major criteria defined by Seckel before the diagnosis is made. There r...

New Features of Disease after Diagnosis in Six Forms of Systemic Vasculitis

Science.gov (United States)

Grayson, Peter C.; Cuthbertson, David; Carette, Simon; Hoffman, Gary S.; Khalidi, Nader A.; Koening, Curry L.; Langford, Carol A.; Maksimowicz-McKinnon, Kathleen; Monach, Paul A.; Seo, Philip; Specks, Ulrich; Ytterberg, Steven R.; Merkel, Peter A.

2015-01-01

Objective To quantify the occurrence of features of vasculitis that initially present after diagnosis in 6 types of primary vasculitis. Methods Standardized collection of data on 95 disease manifestations in 6 vasculitides, including granulomatosis with polyangiitis (Wegener's, GPA), microscopic polyangiitis (MPA), eosinophilic granulomatosis with polyangiitis (Churg-Strauss, EGPA), polyarteritis nodosa (PAN), giant cell arteritis (GCA), and Takayasu's arteritis (TAK), was performed within a set of multicenter longitudinal, observational cohorts. For each form of vasculitis, the frequency of disease-specific manifestations at diagnosis was compared to the cumulative frequency of each manifestation. The percentage of patients who initially developed “severe” manifestations after diagnosis, defined as organ- or life-threatening in the small and medium vessel vasculitides (GPA, MPA, EGPA, PAN) and as ischemic/vascular in the large vessel vasculitides (GCA, TAK), was reported. Results Out of 838 patients with vasculitis, 490 (59%) experienced ≥ 1 new disease manifestation after diagnosis. On average, patients with vasculitis experienced 1.3 new manifestations after diagnosis (GPA - 1.9, MPA - 1.2, EGPA - 1.5, PAN - 1.2, GCA - 0.7, TAK - 1.0). New severe manifestations occurred after diagnosis in 224 (27%) out of 838 patients (GPA - 26%, MPA - 19%, EGPA - 21%, PAN - 23%, GCA - 24%, and TAK - 44%). Timing of onset of new manifestations was not significantly associated with disease duration. Conclusion A majority of patients with vasculitis develop new disease features after diagnosis, including a substantial number of new, severe manifestations. Ongoing assessment of patients with established vasculitis should remain broad in scope. PMID:23908447

Superior Mesenteric Artery Syndrome or Wilkie Syndrome

International Nuclear Information System (INIS)

Castano Llano, Rodrigo; Chams Anturi, Abraham; Arango Vargas, Paula

2009-01-01

We described three cases of superior mesenteric artery (SMA) syndrome, also known as Wilkie's syndrome, chronic duodenal ileus, or cast syndrome. This syndrome occurs when the third portion of the duodenum is compressed between the SMA and the aorta. The major risk factors for development of SMA syndrome are rapid weight loss and surgical correction of spinal deformities. The clinical presentation of SMA syndrome is variable and nonspecific, including nausea, vomiting, abdominal pain, and weight loss. The diagnosis is based on endoscopic, radiographic and tomographic findings of duodenal compression by the SMA. The treatment of SMA syndrome is aimed at the precipitating factor, which usually is related to weight loss. Therefore, conservative therapy with nutritional supplementation is the initial approach, and surgery is reserved for those who do not respond to nutritional therapy.

Beals Syndrome

Science.gov (United States)

... the syndrome. How does Beals syndrome compare with Marfan syndrome? People with Beals syndrome have many of the ... bone) and aortic enlargement problems as people with Marfan syndrome, and treatments for these problems are the same. ...

Serotonin syndrome

Science.gov (United States)

Hyperserotonemia; Serotonergic syndrome; Serotonin toxicity; SSRI - serotonin syndrome; MAO - serotonin syndrome ... brain area. For example, you can develop this syndrome if you take migraine medicines called triptans together ...

[Association Budd Chiari syndrome, antiphospholipid syndrome and Grave's disease].

Science.gov (United States)

Mouelhi, Leila; Chaieb, Mouna; Debbeche, Radhouane; Salem, Mohamed; Sfar, Imene; Trabelsi, Sinda; Gorgi, Yosr; Najjar, Taoufik

2009-02-01

Antiphospholipid syndrome is revealed by Budd Chiari syndrome in 5% of the cases. Antiphospholipid syndrome is characterized by venous or arterial thrombosis, foetal loss and positivity of antiphospholipid antibodies, namely lupus anticoagulant, anticardiolipin antibodies and anti-beta2-glycoprotein I. Anticardiolipin antibodies was reported in auto-immune thyroid disorders, particularly in Grave's disease. Antiphospholipid syndrome associated to Grave's disease was reported in only three cases. To describe a case report of association of Grave's disease and antiphospholipid syndrome. We report the first case of Grave's disease associated with antiphospholipid syndrome, revealed by Budd Chiari syndrome. Our observation is particular by the fact that it is about a patient presenting a Grave's disease associated with antiphospholipid syndrome revealed by Budd Chiari syndrome. This triple association has never been reported in literature. Although association between antiphospholipid syndrome and Grave's disease was previously described, further studies evaluating the coexistence of these two affections in the same patient would be useful.

Duane Syndrome

Science.gov (United States)

... Frequently Asked Questions Español Condiciones Chinese Conditions Duane Syndrome En Español Read in Chinese What is Duane Syndrome? Duane syndrome, also called Duane retraction syndrome (DRS), ...

Goldenhar Syndrome in Association with Duane Syndrome

Directory of Open Access Journals (Sweden)

U D Shrestha

2012-03-01

Full Text Available Goldenhar syndrome (GHS is also known as Oculo-Auriculo-Vertebral (OAV syndrome or Branchial arch syndrome. Duane retraction syndrome (DRS is a congenital disorder of ocular motility characterized by limited abduction, adduction or both. It is unilateral in 80% of cases. The important and interesting part of this eight months old child is presence of GHS with DRS. She has bilateral invol-vement, which is seen in only 5-8% of GHS, as compared to high incidence of unilateral involve-ment. This child also had refractive error of + 6.00/ - 1.5 * 180. At four year of age her vision with glass was 6/9. Children with GHS and DRS should have early eye examination done to treat the problem of refractive error. Keywords: Duane retraction syndrome; goldenhar syndrome, refractive error.

Fanconi syndrome

Science.gov (United States)

De Toni-Fanconi syndrome ... Fanconi syndrome can be caused by faulty genes, or it may result later in life due to kidney damage. Sometimes the cause of Fanconi syndrome is unknown. Common causes of Fanconi syndrome in ...

OCULO-CEREBRO-RENAL SYNDROME (LOWE'S SYNDROME)

Institute of Scientific and Technical Information of China (English)

无

1991-01-01

Oculo-cerebro-renal syndrome (Lowe's syndrome) is characterized by mental and motor retardation, cataract, glaucoma and renal abnormalities. It is an X-linked recessive metabolic disease. Two brothers suffering from Lowe's syndrome are reported. Their mother with lenticular opacities and peculiar facial appearance is in concordance with the obligate carrier. The ocular changes and heridity are discussed.

Neurofibromatosis-Noonan syndrome or LEOPARD Syndrome? A clinical dilemma.

Directory of Open Access Journals (Sweden)

Tullu M

2000-04-01

Full Text Available Neurofibromatosis (NF, Noonan syndrome (NS, and LEOPARD syndrome are all autosomal dominant conditions, each being a distinct clinical entity by itself. Rarely, one encounters cases with features of NF and NS and is termed as the ′Neurofibromatosis-Noonan syndrome′ (NF-NS. The authors report a clinical dilemma with major clinical features of the NF-NS syndrome and LEOPARD syndrome co-existing in the same patient. Also, features of Noonan syndrome and LEOPARD syndrome are compared with the case reported.

LEOPARD syndrome is not linked to the Marfan syndrome and the Watson syndrome loci

Energy Technology Data Exchange (ETDEWEB)

Rass-Rothchild, A.: Abeliovitch, D.; Kornstein, A. [Tel Aviv Univ. (Israel)]|[Hebrew Univ., Jerusalem (Israel)

1994-09-01

The acronym LEOPARD stands for a syndromic association of Lentigines, Eletrocardiographic changes, Ocular hypertelorism, Pulmonic stenosis, Abnormal genitalia, Retardation of growth and sensorineural Deafness. Inheritance is autosomal dominant with high penetrance and variable expressivity. In 1990 Torok et al. reported on the association of LEOPARD and Marfan syndrome. In addition a clinical similarity (cardiac and cutaneous involvement) exists with the Watson syndrome (neurofibromatosis and pulmonic stenosis) which is linked to the marker D17S33 on chromosome 17. We studied possible linkage of LEOPARD syndrome to the Marfan syndrome locus on chromosome 15 (D15S1, MF13, and (TAAAA)n repeats) and to the NF-1 locus on chromosome 17 in a family with 9 cases of LEOPARD syndrome. Close linkage between LEOPARD syndrome and both the Marfan locus on chromosome 15 and the NF-1 locus on chromosome 17 was excluded (lod score <-2.0 through {theta} = 0.1).

Metabolic syndrome and polycystic ovary syndrome: an intriguing overlapping.

Science.gov (United States)

Caserta, Donatella; Adducchio, Gloria; Picchia, Simona; Ralli, Eleonora; Matteucci, Eleonora; Moscarini, Massimo

2014-06-01

Metabolic syndrome is an increasing pathology in adults and in children, due to a parallel rise of obesity. Sedentary lifestyle, food habits, cultural influences and also a genetic predisposition can cause dyslipidemia, hypertension, abdominal obesity and insulin resistance which are the two main features of metabolic syndrome. Polycystic ovary syndrome (PCOS) is a condition directly associated with obesity, insulin resistance (HOMA index) and metabolic syndrome, and it is very interesting for its relationship and overlap with the metabolic syndrome. The relationship between the two syndromes is mutual: PCOS women have a higher prevalence of metabolic syndrome and also women with metabolic syndrome commonly present the reproductive/endocrine trait of PCOS. Prevention and treatment of metabolic syndrome and PCOS are similar for various aspects. It is necessary to treat excess adiposity and insulin resistance, with the overall goals of preventing cardiovascular disease and type 2 diabetes and improving reproductive failure in young women with PCOS. First of all, lifestyle changes, then pharmacological therapy, bariatric surgery and laparoscopic ovarian surgery represent the pillars for PCOS treatment.

Cyber threats and responses in the banking sector

CSIR Research Space (South Africa)

Strauss

2017-10-01

Full Text Available stream_source_info Strauss_CSIR2017.pdf.txt stream_content_type text/plain stream_size 5207 Content-Encoding UTF-8 stream_name Strauss_CSIR2017.pdf.txt Content-Type text/plain; charset=UTF-8 CSIR Conference Cyber... Threats and Responses in the banking sector Paul Strauss Chief Information Risk Officer October 2017 CSIR / Standard Bank / page 2 Cyber Risk Drivers 4% of www content Academic Information Medical Records Legal Documents Scientific...

Barber-Say syndrome and Ablepharon-Macrostomia syndrome: An overview

NARCIS (Netherlands)

de Maria, Beatrice; Mazzanti, Laura; Roche, Nathalie; Hennekam, Raoul C.

2016-01-01

Barber-Say syndrome (BSS) and Ablepharon-Macrostomia syndrome (AMS) are congenital malformation syndromes caused by heterozygous mutations in TWIST2. Here we provide a critical review of all patients published with these syndromes. We excluded several earlier reports due to misdiagnosis or

Autosomal dominant syndrome resembling Coffin-Siris syndrome.

Science.gov (United States)

Flynn, Maureen A; Milunsky, Jeff M

2006-06-15

Coffin-Siris syndrome is a multiple congenital anomaly/mental retardation syndrome with phenotypic variability [OMIM 135900]. The diagnosis is based solely on clinical findings, as there is currently no molecular, biochemical, or cytogenetic analysis available to confirm a diagnosis. Although typically described as an autosomal recessive disorder, autosomal dominant inheritance has also been infrequently reported. We describe a mother and her two daughters who all have features that resemble Coffin-Siris syndrome. However, this is not a completely convincing diagnosis given that hypertelorism is not a feature of Coffin-Siris syndrome and the family is relatively mildly affected. Yet, this family provides further evidence of an autosomal dominant mode of inheritance for a likely variant of Coffin-Siris syndrome (at least in some families). In addition, Sibling 1 had premature thelarche. She is the second reported individual within the spectrum of Coffin-Siris syndrome to have premature thelarche, indicating that it may be a rare clinical feature. Copyright 2006 Wiley-Liss, Inc.

West syndrome in a patient with Schinzel-Giedion syndrome.

Science.gov (United States)

Miyake, Fuyu; Kuroda, Yukiko; Naruto, Takuya; Ohashi, Ikuko; Takano, Kyoko; Kurosawa, Kenji

2015-06-01

Schinzel-Giedion syndrome is a rare recognizable malformation syndrome defined by characteristic facial features, profound developmental delay, severe growth failure, and multiple congenital anomalies. The causative gene of Schinzel-Giedion syndrome, SETBP1, has been identified, but limited cases have been confirmed by molecular analysis. We present a 9-month-old girl affected by West syndrome with Schinzel-Giedion syndrome. Congenital severe hydronephrosis, typical facial features, and multiple anomalies suggested a clinical diagnosis of Schinzel-Giedion syndrome. Hypsarrhythmia occurred at 7 months of age and was temporarily controlled by adrenocorticotropic hormone (ACTH) therapy during 5 weeks. SETBP1 mutational analysis showed the presence of a recurrent mutation, p.Ile871Thr. The implications in management of Schinzel-Giedion syndrome are discussed. © The Author(s) 2014.

Cushing syndrome

Science.gov (United States)

Hypercortisolism; Cortisol excess; Glucocorticoid excess - Cushing syndrome ... The most common cause of Cushing syndrome is taking too much ... Cushing syndrome . Prednisone, dexamethasone, and prednisolone ...

Russell-Silver syndrome

Science.gov (United States)

Silver-Russell syndrome; Silver syndrome; RSS; Russell-Silver syndrome ... One in 10 children with this syndrome has a problem involving chromosome 7. In other people with the syndrome, it may affect chromosome 11. Most of the time, it ...

[Cockett's syndrome, May-Thurner syndrome, or iliac vein compression syndrome].

Science.gov (United States)

Gil Martín, A R; Carreras Aja, M; Arrieta Ardieta, I; Labayen Azparren, I

2014-01-01

Iliac vein compression syndrome (also known as May-Thurner syndrome or Cockett's syndrome) is a rare clinical entity in which the left common iliac vein is compressed when it passes between the right common iliac artery and the spine. The sustained compression and trauma caused by the pulsatile force of the artery on the vein damage the intima and lead to the formation of membranes or bands in the vascular lumen that hinder or obstruct the flow of blood in the vein, favoring thrombus formation. The current treatment strategy of choice is endovascular vein patch angioplasty and stenting with the aim of improving the caliber of the lumen and enabling normal venous drainage. We present two cases of May-Thurner syndrome and review the clinical and CT findings. Copyright © 2011 SERAM. Published by Elsevier Espana. All rights reserved.

LEOPARD syndrome

Science.gov (United States)

Multiple lentigines syndrome; Noonan syndrome with multiple lentigines ... Genetics Home Reference -- ghr.nlm.nih.gov/condition/noonan-syndrome-with-multiple-lentigines National Organization for Rare Disorders -- ...

Dravets syndrom

DEFF Research Database (Denmark)

Hansen, Lars Kjaersgård; Rasmussen, Niels Henrik; Ousager, Lilian Bomme

2010-01-01

Dravet syndrome is an epileptic syndrome of infancy and early childhood. Most cases of Dravet syndrome seem to be due to a genetic defect causing the sodium channel to malfunction. We describe the main features of the syndrome. This epilepsy is medically intractable, but we call attention...... to the fact that some medications are of benefit and some could exacerbate the condition. Early recognition of the syndrome including by genetic testing could possibly improve outcome and reduce the need for other specialized investigations. Udgivelsesdato: 2010-Feb-22...

A Chinese patient with pusher syndrome and unilateral spatial neglect syndrome.

Science.gov (United States)

Chen, Xiao-Wei; Lin, Cheng-He; Zheng, Hua; Lin, Zhen-Lan

2014-07-01

To observe clinical manifestations, behavioral characteristics, and effects of rehabilitation on a patient with pusher syndrome and unilateral spatial neglect caused by right thalamic hemorrhage. Assessment of pusher syndrome was made by the Scale for Contraversive pushing (SCP), and unilateral spatial neglect syndrome was diagnosed using line cancellation, letter and star cancellation, line bisection tests and copy and continuation of graphic sequence test. Behavioral therapy, occupational therapy, reading training and traditional Chinese medicine methods were adopted for treatment of pusher syndrome and unilateral spatial neglect. The patient showed typical pusher syndrome and unilateral spatial neglect symptoms. The pusher syndrome and unilateral spatial neglect symptoms were significantly improved following rehabilitation treatments. Pusher syndrome and unilateral spatial neglect syndrome occurred simultaneously after right thalamic hemorrhage. Early rehabilitation therapy can reduce the symptoms of pusher syndrome and unilateral spatial neglect syndrome and improve motor function.

Aarskog syndrome

Science.gov (United States)

Aarskog disease; Aarskog-Scott syndrome; AAS; Faciodigitogenital syndrome; Gaciogenital dysplasia ... Aarskog syndrome is a genetic disorder that is linked to the X chromosome. It affects mainly males, but females ...

Ectrodactyly-ectodermal dysplasia clefting syndrome (EEC syndrome).

Science.gov (United States)

Koul, Monika; Dwivedi, Rahul; Upadhyay, Vinod

2014-01-01

Ectrodactyly-ectodermal dysplasia- clefting syndrome (also k/a. split hand- split foot malformation /split hand-split foot ectodermal dysplasia- cleft syndrome/ectodermal dysplasia cleft lip/cleft palate syndrome) a rare form of ectodermal dysplasia, is an autosomal dominant disorder inherited as a genetic trait and characterized by a triad of (i) ectrodactyly, (ii) ectodermal dysplasia and, (iii) & facial clefts.

Ectrodactyly-ectodermal dysplasia clefting syndrome (EEC syndrome)

OpenAIRE

Koul, Monika; Dwivedi, Rahul; Upadhyay, Vinod

2014-01-01

Ectrodactyly-ectodermal dysplasia- clefting syndrome (also k/a. split hand- split foot malformation /split hand-split foot ectodermal dysplasia- cleft syndrome/ectodermal dysplasia cleft lip/cleft palate syndrome) a rare form of ectodermal dysplasia, is an autosomal dominant disorder inherited as a genetic trait and characterized by a triad of (i) ectrodactyly, (ii) ectodermal dysplasia and, (iii) & facial clefts.

Cushing's Syndrome

OpenAIRE

宗, 友厚; 伊藤, 勇; 諏訪, 哲也; 武田, 純; MUNE, Tomoatsu

2003-01-01

Sixteen cases of verified Cushing's syndrome, and twelve cases of probable Cushing's syndrome were reviewed and data on them were compared with various reports on Cushing's syndrome in the literature.

Polycystic ovary syndrome and metabolic syndrome.

Science.gov (United States)

Ali, Aus Tariq

2015-08-01

Polycystic ovary syndrome (PCOS) is a heterogeneous disorder, where the main clinical features include menstrual irregularities, sub-fertility, hyperandrogenism, and hirsutism. The prevalence of PCOS depends on ethnicity, environmental and genetic factors, as well as the criteria used to define it. On the other hand, metabolic syndrome is a constellation of metabolic disorders which include mainly abdominal obesity, insulin resistance, impaired glucose metabolism, hypertension and dyslipidaemia. These associated disorders directly increase the risk of Type 2 diabetes mellitus (DMT2), coronary heart disease (CHD), cardiovascular diseases (CVD) and endometrial cancer. Many patients with PCOS have features of metabolic syndrome such as visceral obesity, hyperinsulinaemia and insulin resistance. These place patients with PCOS under high risk of developing cardiovascular disease (CVD), Type 2 diabetes (DMT2) and gynecological cancer, in particular, endometrial cancer. Metabolic syndrome is also increased in infertile women with PCOS. The aim of this review is to provide clear and up to date information about PCOS and its relationship with metabolic syndrome, and the possible interaction between different metabolic disorders.

Tourette syndrome

Science.gov (United States)

Gilles de la Tourette syndrome; Tic disorders - Tourette syndrome ... Tourette syndrome is named for Georges Gilles de la Tourette, who first described this disorder in 1885. The disorder is likely passed down through families. ...

Metabolic syndrome in acute coronary syndrome

International Nuclear Information System (INIS)

Bhalli, M.A.; Aamir, M.; Mustafa, G.

2011-01-01

Objective: To determine the frequency of metabolic syndrome in male patients presenting with acute coronary syndrome Study design: A Descriptive study Place and duration of study: Armed Forces Institute of Cardiology and National Institute of Heart Diseases, Rawalpindi, from October 2007 to September 2008 Patients and Methods: Male patients with acute coronary syndrome (ACS) were included. Patients having angioplasty (PCI), coronary artery bypass surgery in the past and other co-morbid diseases were excluded. All patients were assessed for the presence of five components of metabolic syndrome including hypertension, HDL-Cholesterol and triglycerides, glucose intolerance and abdominal obesity. Systolic, diastolic blood pressures, waist circumference (WC) and body mass index (BMI) were measured. ECG, cardiac enzymes, fasting glucose and lipid profile were also done. Results: A total of 135 male patients of ACS were studied with a mean age of 54.26 +- 11 years. Metabolic syndrome (MS) was present in 55 (40.7%) patients. MS with all five components was documented in 4 (7.27%) while MS with four and three components was seen in 23 (41.81%) and 28 (50.90%) patients respectively. Only 24 (43.63%) patients with MS had diabetes mellitus, remaining 31(56.36%) were non diabetic. Frequencies of diabetes, hypertension and family history of CAD were significantly higher (p<0.05) in patients with metabolic syndrome as compared to patients with normal metabolic status. Conclusion: Metabolic syndrome is fairly common and important risk factor in patients of IHD. Other risk factors like smoking, dyslipidemia, hypertension and diabetes were also frequently found. Public awareness to control the risk factors can reduce the prevalence of CAD in our country. (author)

Metabolic syndrome in acute coronary syndrome

Energy Technology Data Exchange (ETDEWEB)

Bhalli, M A; Aamir, M; Mustafa, G [Combined Military Hospital, Abbottabad (Pakistan)

2011-06-15

Objective: To determine the frequency of metabolic syndrome in male patients presenting with acute coronary syndrome Study design: A Descriptive study Place and duration of study: Armed Forces Institute of Cardiology and National Institute of Heart Diseases, Rawalpindi, from October 2007 to September 2008 Patients and Methods: Male patients with acute coronary syndrome (ACS) were included. Patients having angioplasty (PCI), coronary artery bypass surgery in the past and other co-morbid diseases were excluded. All patients were assessed for the presence of five components of metabolic syndrome including hypertension, HDL-Cholesterol and triglycerides, glucose intolerance and abdominal obesity. Systolic, diastolic blood pressures, waist circumference (WC) and body mass index (BMI) were measured. ECG, cardiac enzymes, fasting glucose and lipid profile were also done. Results: A total of 135 male patients of ACS were studied with a mean age of 54.26 +- 11 years. Metabolic syndrome (MS) was present in 55 (40.7%) patients. MS with all five components was documented in 4 (7.27%) while MS with four and three components was seen in 23 (41.81%) and 28 (50.90%) patients respectively. Only 24 (43.63%) patients with MS had diabetes mellitus, remaining 31(56.36%) were non diabetic. Frequencies of diabetes, hypertension and family history of CAD were significantly higher (p<0.05) in patients with metabolic syndrome as compared to patients with normal metabolic status. Conclusion: Metabolic syndrome is fairly common and important risk factor in patients of IHD. Other risk factors like smoking, dyslipidemia, hypertension and diabetes were also frequently found. Public awareness to control the risk factors can reduce the prevalence of CAD in our country. (author)

Exogenous Cushing syndrome

Science.gov (United States)

Cushing syndrome - corticosteroid induced; Corticosteroid-induced Cushing syndrome; Iatrogenic Cushing syndrome ... Cushing syndrome is a disorder that occurs when your body has a higher than normal level of the hormone ...

Development and Validation of Case-Finding Algorithms for the Identification of Patients with ANCA-Associated Vasculitis in Large Healthcare Administrative Databases

Science.gov (United States)

Sreih, Antoine G.; Annapureddy, Narender; Springer, Jason; Casey, George; Byram, Kevin; Cruz, Andy; Estephan, Maya; Frangiosa, Vince; George, Michael D.; Liu, Mei; Parker, Adam; Sangani, Sapna; Sharim, Rebecca; Merkel, Peter A.

2016-01-01

Purpose To develop and validate case-finding algorithms for granulomatosis with polyangiitis (Wegener’s, GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (Churg-Strauss, EGPA). Methods 250 patients per disease were randomly selected from 2 large healthcare systems using the International Classification of Diseases version 9 (ICD9) codes for GPA/EGPA (446.4) and MPA (446.0). 16 case-finding algorithms were constructed using a combination of ICD9 code, encounter type (inpatient or outpatient), physician specialty, use of immunosuppressive medications, and the anti-neutrophil cytoplasmic antibody (ANCA) type. Algorithms with the highest average positive predictive value (PPV) were validated in a third healthcare system. Results An algorithm excluding patients with eosinophilia or asthma and including the encounter type and physician specialty had the highest PPV for GPA (92.4%). An algorithm including patients with eosinophilia and asthma and the physician specialty had the highest PPV for EGPA (100%). An algorithm including patients with one of the following diagnoses: alveolar hemorrhage, interstitial lung disease, glomerulonephritis, acute or chronic kidney disease, the encounter type, physician specialty, and immunosuppressive medications had the highest PPV for MPA (76.2%). When validated in a third healthcare system, these algorithms had high PPV (85.9% for GPA, 85.7% for EGPA, and 61.5% for MPA). Adding the ANCA type increased the PPV to 94.4%, 100%, and 81.2% for GPA, EGPA, and MPA respectively. Conclusion Case-finding algorithms accurately identify patients with GPA, EGPA, and MPA in administrative databases. These algorithms can be used to assemble population-based cohorts and facilitate future research in epidemiology, drug safety, and comparative effectiveness. PMID:27804171

Targets to treat metabolic syndrome in polycystic ovary syndrome.

Science.gov (United States)

Mahalingaiah, Shruthi; Diamanti-Kandarakis, Evanthia

2015-01-01

Metabolic syndrome is comprised of a combination of the following states: increased insulin resistance, dyslipidemia, cardiovascular disease, and increased abdominal obesity. Women with polycystic ovary syndrome (PCOS) have an increased risk of developing metabolic syndrome over the course of their lives. Metabolic syndrome increases risk of major cardiovascular events, morbidity, quality of life, and overall health care costs. Though metabolic syndrome in women with PCOS is an area of great concern, there is no effective individual medical therapeutic to adequately treat this issue. This article will review key aspects of metabolic syndrome in PCOS. We will discuss classic and novel therapeutics to address metabolic syndrome in women with PCOS. We will conclude with the importance of developing strategic interventions to increase the compliance to lifestyle and dietary modification, in addition to appreciation of the emerging pharmaceutical therapeutics available. Innovation in lifestyle modification, including diet, exercise, with and without dedicated stress reduction techniques is the future in treatment of metabolic syndrome in PCOS. Application of novel interventions, such as group medical care, may improve future adherence to lifestyle modification recommendations, in addition to or in combination with pharmaceutical therapeutics.

Targets to treat metabolic syndrome in polycystic ovary syndrome

Science.gov (United States)

Mahalingaiah, Shruthi; Diamanti-Kandarakis, Evanthia

2016-01-01

Introduction Metabolic syndrome is comprised of a combination of the following states: increased insulin resistance, dyslipidemia, cardiovascular disease, and increased abdominal obesity. Women with polycystic ovary syndrome (PCOS) have an increased risk of developing metabolic syndrome over the course of their lives. Metabolic syndrome increases risk of major cardiovascular events, morbidity, quality of life, and overall health care costs. Though metabolic syndrome in women with PCOS is an area of great concern, there is no effective individual medical therapeutic to adequately treat this issue. Areas Covered This article will review key aspects of metabolic syndrome in PCOS. We will discuss classic and novel therapeutics to address metabolic syndrome in women with PCOS. We will conclude with the importance of developing strategic interventions to increase the compliance to lifestyle and dietary modification, in addition to appreciation of the emerging pharmaceutical therapeutics available. Expert Opinion Innovation in lifestyle modification, including diet, exercise, with and without dedicated stress reduction techniques is the future in treatment of metabolic syndrome in PCOS. Application of novel interventions, such as group medical care, may improve future adherence to lifestyle modification recommendations, in addition to or in combination with pharmaceutical therapeutics. PMID:26488852

Concurrent Van der Woude syndrome and Turner syndrome: A case report.

Science.gov (United States)

Los, Evan; Baines, Hayley; Guttmann-Bauman, Ines

2017-01-01

Most cases of Van der Woude syndrome are caused by a mutation to interferon regulatory factor 6 on chromosome 1. Turner syndrome is caused by complete or partial absence of the second sex chromosome in girls. We describe a unique case of the two syndromes occurring concurrently though apparently independently in a girl with Van der Woude syndrome diagnosed at birth and Turner syndrome at 14 years 9 months. Short stature was initially misattributed to Van der Woude syndrome and pituitary insufficiency associated with clefts before correctly diagnosing Turner syndrome. We discuss the prevalence of delayed diagnosis of Turner syndrome, the rarity of reports of concurrent autosomal chromosome mutation and sex chromosome deletion, as well as the need to consider the diagnosis of Turner syndrome in all girls with short stature regardless of prior medical history.

Milk-alkali syndrome

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Calcium-alkali syndrome; Cope syndrome; Burnett syndrome; Hypercalcemia; Calcium metabolism disorder ... Milk-alkali syndrome is almost always caused by taking too many calcium supplements, usually in the form of calcium carbonate. Calcium ...

Marfan Syndrome

Science.gov (United States)

Marfan syndrome is a disorder that affects connective tissue. Connective tissues are proteins that support skin, bones, blood vessels, ... A problem with the fibrillin gene causes Marfan syndrome. Marfan syndrome can be mild to severe, and ...

Brief Report: Repetitive Behaviour Profiles in Williams Syndrome: Cross Syndrome Comparisons with Prader-Willi and Down Syndromes

Science.gov (United States)

Royston, R.; Oliver, C.; Moss, J.; Adams, D.; Berg, K.; Burbidge, C.; Howlin, P.; Nelson, L.; Stinton, C.; Waite, J.

2018-01-01

This study describes the profile of repetitive behaviour in individuals with Williams syndrome, utilising cross-syndrome comparisons with people with Prader-Willi and Down syndromes. The Repetitive Behaviour Questionnaire was administered to caregivers of adults with Williams (n = 96), Prader-Willi (n = 103) and Down (n = 78) syndromes. There were…

Loeys-Dietz Syndrome

Science.gov (United States)

... to the signs and symptoms of Loeys-Dietz syndrome. Marfan syndrome is different from Loeys-Dietz syndrome in that the gene mutation which causes Marfan syndrome is in fibrillin-1 (FBN-1), a protein ...

Pre-Menstrual Syndrome in Women with Down Syndrome

Science.gov (United States)

Mason, Linda; Cunningham, Cliff

2009-01-01

Background: Prevalence of pre-menstrual syndrome (PMS) may be higher in women with Down syndrome due to syndrome specific characteristics in biochemistry, psychopathology and lifestyle. Recognition of PMS may be difficult for women with intellectual disabilities and their carers. Method: A daily diary, used to diagnose PMS with typical women, was…

Williams syndrome

Science.gov (United States)

Williams-Beuren syndrome ... Williams syndrome is caused by not having a copy of several genes. It may be passed down in families. ... history of the condition. However, people with Williams syndrome have a 50% chance of passing the disorder ...

Coexistence of Reverse Capgras Syndrome, Subjective Double and Cotard Syndrome

Directory of Open Access Journals (Sweden)

Azadeh Mashayekhi

2016-01-01

Full Text Available Misidentification syndrome is a condition in which the person thinks that familiar persons have been replaced with other one. Coexistence of some types of this syndrome has been reported with other psychiatric syndromes. In this report, we present a 47-year-old married man with coexistence of reverse Capgras and subjective double syndromes with Cotard syndrome. There is no previous report of coexistence of these three forms of delusions in a single case.

Nevoid Basal Cell Carcinoma Syndrome (Gorlin Syndrome).

Science.gov (United States)

Bresler, Scott C; Padwa, Bonnie L; Granter, Scott R

2016-06-01

Nevoid basal cell carcinoma syndrome, or basal cell nevus syndrome (Gorlin syndrome), is a rare autosomal dominantly inherited disorder that is characterized by development of basal cell carcinomas from a young age. Other distinguishing clinical features are seen in a majority of patients, and include keratocystic odontogenic tumors (formerly odontogenic keratocysts) as well as dyskeratotic palmar and plantar pitting. A range of skeletal and other developmental abnormalities are also often seen. The disorder is caused by defects in hedgehog signaling which result in constitutive pathway activity and tumor cell proliferation. As sporadic basal cell carcinomas also commonly harbor hedgehog pathway aberrations, therapeutic agents targeting key signaling constituents have been developed and tested against advanced sporadically occurring tumors or syndromic disease, leading in 2013 to FDA approval of the first hedgehog pathway-targeted small molecule, vismodegib. The elucidation of the molecular pathogenesis of nevoid basal cell carcinoma syndrome has resulted in further understanding of the most common human malignancy.

Aicardi Syndrome

Science.gov (United States)

... from Aicardi-Goutieres syndrome, which is an inherited encephalopathy that affects newborn infants.) × Definition Aicardi syndrome is a rare genetic ... from Aicardi-Goutieres syndrome, which is an inherited encephalopathy that affects newborn infants.) View Full Definition Treatment There is no ...

Fournier gangrene associated with hyper IgE syndrome (Job syndrome).

Science.gov (United States)

Hori, Junichi; Yamaguchi, Satoshi; Watanabe, Masaki; Osanai, Hiroaki; Hori, Masako

2008-04-01

We report a case of a 32-year-old man with hyper IgE syndrome (Job syndrome) who developed Fournier gangrene due to infectious multiple atheromas of the scrotal skin that progressed to the right groin and thigh. The patient required surgical debridement and subsequent skin grafting. This is a rare case of Fournier gangrene associated with hyper IgE syndrome (Job syndrome). When a patient without diabetes mellitus has repeated infections and atopic-like dermatitis, Job syndrome should be considered.

Down Syndrome

Science.gov (United States)

... Down syndrome increases as a woman gets older. Down syndrome cannot be cured. Early treatment programs can help improve skills. They may include ... occupational, and/or educational therapy. With support and treatment, many ... Down syndrome live happy, productive lives. NIH: National Institute of ...

High Power Vanadate lasers

CSIR Research Space (South Africa)

Strauss

2006-07-01

Full Text Available stream_source_info Strauss1_2006.pdf.txt stream_content_type text/plain stream_size 3151 Content-Encoding UTF-8 stream_name Strauss1_2006.pdf.txt Content-Type text/plain; charset=UTF-8 Laser Research Institute... University of Stellenbosch www.laser-research.co.za High Power Vanadate lasers H.J.Strauss, Dr. C. Bollig, R.C. Botha, Prof. H.M. von Bergmann, Dr. J.P. Burger Aims 1) To develop new techniques to mount laser crystals, 2) compare the lasing properties...

TAFRO Syndrome.

Science.gov (United States)

Igawa, Takuro; Sato, Yasuharu

2018-02-01

TAFRO syndrome is a newly recognized variant of idiopathic multicentric Castleman disease (iMCD) that involves a constellation of syndromes: thrombocytopenia (T), anasarca (A), fever (F), reticulin fibrosis (R), and organomegaly (O). Thrombocytopenia and severe anasarca accompanied by relatively low serum immunoglobulin levels are characteristic clinical findings of TAFRO syndrome that are not present in iMCD-not otherwise specified (iMCD-NOS). Lymph node biopsy is recommended to exclude other diseases and to diagnose TAFRO syndrome, which reveals characteristic histopathological findings similar to hyaline vascular-type CD. TAFRO syndrome follows a more aggressive course, compared with iMCD-NOS, and there is no standard treatment. Copyright © 2017 Elsevier Inc. All rights reserved.

Development and characteristics of children with Usher syndrome and CHARGE syndrome.

Science.gov (United States)

Dammeyer, Jesper

2012-09-01

Individuals with Usher syndrome or CHARGE syndrome are faced with a number of difficulties concerning hearing, vision, balance, and language development. The aim of the study is to describe the developmental characteristics of children with Usher syndrome and CHARGE syndrome, respectively. Data about the developmental characteristics of 26 children with Usher syndrome and 17 children with CHARGE syndrome was obtained. Associations between deafblindness (dual sensory loss), motor development (age of walking), language abilities, and intellectual outcome of these children were explored for each group independently. Both groups of children face a number of difficulties associated with vision, hearing, language, balance and intellectual outcome. Intellectual disability and/or language delay was found among 42% of the children with Usher syndrome and among 82% of the children with CHARGE syndrome. Intellectual disability was associated with language delay and age of walking for both groups. Even though Usher and CHARGE are two different genetic syndromes, both groups are challenged with a number of similar developmental delays. Clinicians need to be aware of several developmental issues in order to offer adequate support to children with Usher or CHARGE syndrome. Copyright © 2012 Elsevier Ireland Ltd. All rights reserved.

Pregnancy outcome in joint hypermobility syndrome and Ehlers-Danlos syndrome.

Science.gov (United States)

Sundelin, Heléne E K; Stephansson, Olof; Johansson, Kari; Ludvigsson, Jonas F

2017-01-01

An increased risk of preterm birth in women with joint hypermobility syndrome or Ehlers-Danlos syndrome is suspected. In this nationwide cohort study from 1997 through 2011, women with either joint hypermobility syndrome or Ehlers-Danlos syndrome or both disorders were identified through the Swedish Patient Register, and linked to the Medical Birth Register. Thereby, 314 singleton births to women with joint hypermobility syndrome/Ehlers-Danlos syndrome before delivery were identified. These births were compared with 1 247 864 singleton births to women without a diagnosis of joint hypermobility syndrome/Ehlers-Danlos syndrome. We used logistic regression, adjusted for maternal age, smoking, parity, and year of birth, to calculate adjusted odds ratios for adverse pregnancy outcomes. Maternal joint hypermobility syndrome/Ehlers-Danlos syndrome was not associated with any of our outcomes: preterm birth (adjusted odds ratio = 0.6, 95% confidence interval 0.3-1.2), preterm premature rupture of membranes (adjusted odds ratio = 0.8; 95% confidence interval 0.3-2.2), cesarean section (adjusted odds ratio = 0.9, 95% confidence interval 0.7-1.2), stillbirth (adjusted odds ratio = 1.1, 95% confidence interval 0.2-7.9), low Apgar score (adjusted odds ratio = 1.6, 95% confidence interval 0.7-3.6), small for gestational age (adjusted odds ratio = 0.9, 95% confidence interval 0.4-1.8) or large for gestational age (adjusted odds ratio = 1.2, 95% confidence interval 0.6-2.1). Examining only women with Ehlers-Danlos syndrome (n = 62), we found a higher risk of induction of labor (adjusted odds ratio = 2.6; 95% confidence interval 1.4-4.6) and amniotomy (adjusted odds ratio = 3.8; 95% confidence interval 2.0-7.1). No excess risks for adverse pregnancy outcome were seen in joint hypermobility syndrome. Women with joint hypermobility syndrome/Ehlers-Danlos syndrome do not seem to be at increased risk of adverse pregnancy outcome. © 2016 Nordic Federation of

Wolf-Hirschhorn (4p-) syndrome with West syndrome.

Science.gov (United States)

Motoi, Hirotaka; Okanishi, Tohru; Kanai, Sotaro; Yokota, Takuya; Yamazoe, Tomohiro; Nishimura, Mitsuyo; Fujimoto, Ayataka; Yamamoto, Takamichi; Enoki, Hideo

2016-01-01

Wolf-Hirschhorn syndrome (WHS) is a chromosome disorder (4p-syndrome) which is characterized by craniofacial features and epileptic seizures. Here, we report a case of WHS with West syndrome, in whom the seizures were refractory to several antiepileptic drugs but were responsive to the addition of lamotrigine. The patient had epileptic spasms at age seven months. The interictal electroencephalogram was hypsarrhythmic. After adding lamotrigine, seizures decreased remarkably, and spasms disappeared. We have identified and described the very rare case of a girl with WHS who also developed West syndrome. In this case, adding lamotrigine to her medications effectively treated the spasms.

Rowell syndrome

Directory of Open Access Journals (Sweden)

Ramesh Y Bhat

2014-01-01

Full Text Available Rowell syndrome is a rare disease consisting of erythema multiforme-like lesions associated with lupus erythematosus. The syndrome occurs mostly in middle-aged women. The authors describe the syndrome in a 15-year-old boy who responded well to systemic steroids and hydroxychloroquine.

[Poland's syndrome].

Science.gov (United States)

Slezak, R; Sasiadek, M

2000-08-01

Poland's syndrome consists of the variable clinical features, but always includes unilateral aplasia of the chest wall muscles and ipsilateral anomalies of upper extremity. The incidence of Poland's syndrome, reported by different authors ranges from 1:10,000 to 1:100,000 and is observed more frequently in males than in females with the right side of the body affected more often than the left. The etiology of this syndrome is still discussed. However most of described cases were sporadic, rare familial incidence of Poland's syndrome were also presented. Therefore different etiologic factors of the Poland's syndrome are taken into account: genetic, vascular compromise during early stages of embriogenesis but also teratogenic effect of environmental xenobiotics (e.g. cigarette smoking by pregnant women). The authors present also the case of 20-years old man with inherited bilateral syndactyly with the right side aplasia of major pectoralis muscle and face asymmetry. The familial history was negative in respect to the features, associated with Poland's syndrome.

[Gardner syndrome--parent alienation syndrome (PAS). Diagnosis or family reality?].

Science.gov (United States)

Namysłowska, Irena; Heitzman, Janusz; Siewierska, Anna

2009-01-01

The authors present characteristics of Parental Alienation Syndrome (PAS) proposed by Gardner as well as data, which may help to differentiate that syndrome with real psychological, physical and sexual abuse. The consequences of Gardner Syndrome for legal decisions in the court cases of child custody and the critique of this syndrome in forensic and psychiatric literature are also discussed, and several questions posed. Authors propose to treat Gardner Syndrome not as as a child disorder but as a specific, dynamic family situation, which occurs sometimes, during divorce and fight about child custody.

Burning Mouth Syndrome and "Burning Mouth Syndrome".

Science.gov (United States)

Rifkind, Jacob Bernard

2016-03-01

Burning mouth syndrome is distressing to both the patient and practitioner unable to determine the cause of the patient's symptoms. Burning mouth syndrome is a diagnosis of exclusion, which is used only after nutritional deficiencies, mucosal disease, fungal infections, hormonal disturbances and contact stomatitis have been ruled out. This article will explore the many causes and treatment of patients who present with a chief complaint of "my mouth burns," including symptomatic treatment for those with burning mouth syndrome.

Marfan Syndrome (For Teens)

Science.gov (United States)

... genetic disorder called Marfan syndrome. What Is Marfan Syndrome? Marfan syndrome is named after Antoine Marfan, the French ... immediately. What's Life Like for Teens With Marfan Syndrome? Marfan syndrome affects people differently, so life is not ...

Learning about Marfan Syndrome

Science.gov (United States)

... Additional Resources for Marfan Syndrome What is Marfan syndrome? Marfan syndrome is one of the most common inherited ... FAQ Top of page Additional Resources For Marfan Syndrome Marfan syndrome [nlm.nih.gov] From Medline Plus Marfan ...

The design, construction and first-phase heavy vehicle simulator testing results on full scale ultra-thin reinforced concrete test sections at Rayton, South Africa

CSIR Research Space (South Africa)

Du Plessis, L

2016-09-01

Full Text Available .J. Jordaan2, P.J. Strauss3, A. Kilian4 1. L. du Plessis, CSIR Built Environment, Meiring Naudé Street, Brummeria, Pretoria, South Africa, Phone: 012 841 2922, E-mail: lplessis@csir.co.za Corresponding author 2. G.J. Jordaan, Tshepega Engineering (Pty...) Ltd, PO Box 33783 Glenstantia 0010 Phone: 012 665 2722, and University of Pretoria, Pretoria, 0001, E-mail: jordaangj@tshepega.co.za 3. P.J. Strauss, Pieter Strauss CC, PO Box 588 La Montagne 0184, Phone: 012 807 0367, E-mail: Pieter...

Comparative study of thermal lensing in low-doped Nd:YVO4 and Nd:GdVO4 of equal doping concentration

CSIR Research Space (South Africa)

Strauss

2009-06-01

Full Text Available stream_source_info Strauss_2009.pdf.txt stream_content_type text/plain stream_size 4705 Content-Encoding UTF-8 stream_name Strauss_2009.pdf.txt Content-Type text/plain; charset=UTF-8 Comparative Study of Thermal Lensing... in Low-doped Nd:YVO4 and Nd:GdVO4 of Equal Doping Concentration H. J. Strauss1,2, C. Bollig1, H. M. von Bergmann2 and M. J. D. Esser1 1. National Laser Centre, Council for Scientific and Industrial Research, PO Box 395, Pretoria 0001, South Africa 2...

Divorce in families of children with Down Syndrome or Rett Syndrome.

Science.gov (United States)

Lederman, Vivian Renne Gerber; Alves, Bianca dos Santos; Negrão, Juliana; Maria, Juliana Negrão; Schwartzman, José Salomão; D'Antino, Maria Eloisa Famá; Brunoni, Decio

2015-05-01

This study evaluates the impact in the stability and management of the marriage of parents of a child with Down or Rett Syndrome. Morbidity of the syndromes and the marital status of the couples before and after the birth of the affected children were considered variables. The divorce rate in families with Down syndrome was 10%, similar to the Brazilian rate population. In Rett Syndrome, the divorce rate was significantly higher, 23.5%. The higher morbidity of Rett Syndrome, and the moment of diagnosis could be relevant factors for the increased divorce rate related to this syndrome.

The sick-building syndrome; Das Sick-Building-Syndrom

Energy Technology Data Exchange (ETDEWEB)

Henne, A.; Neumann, H.F.; Winneke, G.

1992-12-31

The sick-building syndrome is characterized by the presence of general, non-specific symptoms (e.g., headache, tiredness, respiratory problems, eye trouble, vertigo, nausea, unspecific hypersensitivity) in association with a particular indoor ambience. It is clearly distinguishable from `building-related illness`, referring to a well-defined clinical syndrome due to staying in a building and for which a cause can, in general, be established. Disorders in the case of the sick-building syndrome are manifold and confirmed objectifiable results are hardly available so far. Yet there are some organ-related methods for the confirmation of findings concerning, for instance, the eyes, the skin and the area of the nose. The causes of the incidence of sick-building syndrome are more or less unclear. It is a multifactorial phenomenon involving physical, biological, chemical, individual-specific and psychological factors. Buildings where sick-building syndrome occurs typically exhibit certain properties. The European Community has already made proposals for the investigation of incriminated buildings. A systematic survey by questionnaire together with individual interviews plays an import part towards clarifying the syndrome. (orig./UWA) [Deutsch] Das Sick-Building-Syndrom beschreibt das Vorhandensein von allgemeinen, nicht spezifischen Symptomen (z.B. Kopfschmerzen, Muedigkeit, Atembeschwerden, Augenreizungen, Schwindelgefuehl, Uebelkeit, unspezifische Ueberempfindlichkeit), assoziiert mit einer besonderen Innenraumumgebung. Deutlich hiervon abzugrenzen ist die ``Building related illness``, bei der ein klinisch definiertes Krankheitsbild vorliegt, das durch den Aufenthalt im Gebaeude verursacht wird und fuer das im allgemeinen eine Ursache ermittelt werden kann. Das Beschwerdebild beim Sick-Building-Syndrom ist vielfaeltig, und gesicherte, objektivierbare Befunde liegen hierzu bisher kaum vor. Dennoch gibt es einige organbezogenen Methoden zur Befundabsicherung, z.B. fuer das

Hypokalaemia: Bartter's syndrome or pseudo-Bartter's syndrome?

OpenAIRE

Robb, J D; Delargy, M A; Nolan, M; Tomkin, G H

1984-01-01

The difficulties in the diagnosis of hypokalaemia are often considerable. This paper reports three patients who presented with hypokalaemia. Investigations are described which may help to distinguish Bartter's syndrome from pseudo-Bartter's syndrome.

Syndromes with supernumerary teeth.

Science.gov (United States)

Lubinsky, Mark; Kantaputra, Piranit Nik

2016-10-01

While most supernumerary teeth are idiopathic, they can be associated with a number of Mendelian syndromes. However, this can also be a coincidental finding, since supernumerary teeth occur in 6% or more of the normal population. To better define this relationship, we analyzed the evidence for specific associations. We excluded conditions with a single affected patient reported, supernumerary teeth adjacent to clefts or other forms of alveolar disruption (as secondary rather than primary findings), and natal teeth, which can involve premature eruption of a normal tooth. Since, the cause of supernumerary teeth shows considerable heterogeneity, certain findings are less likely to be coincidental, such as five or more supernumerary teeth in a single patient, or locations outside of the premaxilla. We found only eight genetic syndromes with strong evidence for an association: cleidocranial dysplasia; familial adenomatous polyposis; trichorhinophalangeal syndrome, type I; Rubinstein-Taybi syndrome; Nance-Horan syndrome; Opitz BBB/G syndrome; oculofaciocardiodental syndrome; and autosomal dominant Robinow syndrome. There is also suggestive evidence of an association with two uncommon disorders, Kreiborg-Pakistani syndrome (craniosynostosis and dental anomalies), and insulin-resistant diabetes mellitus with acanthosisnigricans. An association of a Mendelian disorder with a low frequency manifestation of supernumerary teeth is difficult to exclude without large numbers, but several commonly cited syndromes lacked evidence for clear association, including Hallermann-Streiff syndrome, Fabry disease, Ehlers-Danlos syndrome, Apert and Crouzon syndromes, Zimmermann-Laband syndrome, and Ellis-van Creveld syndrome. © 2016 Wiley Periodicals, Inc. © 2016 Wiley Periodicals, Inc.

What Is Usher Syndrome?

Science.gov (United States)

... Action You are here Home › Retinal Diseases Listen Usher Syndrome What is Usher syndrome? How is Usher syndrome ... available? Are there any related diseases? What is Usher Syndrome? Usher syndrome is an inherited condition characterized by ...

Autoimmune/inflammatory syndrome induced by adjuvants (Shoenfeld's syndrome) - An update.

Science.gov (United States)

Watad, A; Quaresma, M; Brown, S; Cohen Tervaert, J W; Rodríguez-Pint, I; Cervera, R; Perricone, C; Shoenfeld, Y

2017-06-01

Autoimmune/inflammatory syndrome induced by adjuvants (ASIA) has been widely described in many studies conducted thus far. The syndrome incorporates five immune-mediated conditions, all associated with previous exposure to various agents such as vaccines, silicone implants and several others. The emergence of ASIA syndrome is associated with individual genetic predisposition, for instance those carrying HLA-DRB1*01 or HLA-DRB4 and results from exposure to external or endogenous factors triggering autoimmunity. Such factors have been demonstrated as able to induce autoimmunity in both animal models and humans via a variety of proposed mechanisms. In recent years, physicians have become more aware of the existence of ASIA syndrome and the relationship between adjuvants exposure and autoimmunity and more cases are being reported. Accordingly, we have created a registry that includes at present more than 300 ASIA syndrome cases that have been reported by different physicians worldwide, describing various autoimmune conditions induced by diverse adjuvants. In this review, we have summarized the updated literature on ASIA syndrome and the knowledge accumulated since 2013 in order to elucidate the association between the exposure to various adjuvant agents and its possible clinical manifestations. Furthermore, we especially referred to the relationship between ASIA syndrome and systemic lupus erythematosus (SLE) and antiphospholipid syndrome (APS).

Toxic shock syndrome

Science.gov (United States)

Staphylococcal toxic shock syndrome; Toxic shock-like syndrome; TSLS ... Toxic shock syndrome is caused by a toxin produced by some types of staphylococcus bacteria. A similar problem, called toxic shock- ...

Management of moyamoya syndrome in patients with Noonan syndrome.

Science.gov (United States)

Gupta, Mihir; Choudhri, Omar A; Feroze, Abdullah H; Do, Huy M; Grant, Gerald A; Steinberg, Gary K

2016-06-01

A few isolated reports have described an association between Noonan syndrome and cerebrovascular abnormalities, including moyamoya syndrome. These reports have been limited to pediatric patients presenting with recurrent transient ischemic attacks (TIA) or headaches. Management has primarily been pharmacologic, with only one prior report of surgical revascularization to our knowledge. We report four cases of Noonan syndrome patients presenting with headaches and/or sensorimotor strokes in childhood that caused unilateral sensorimotor impairment. Cerebral angiography and MRI revealed bilateral moyamoya syndrome. All patients underwent successful bilateral extracranial-to-intracranial revascularization. The first patient was a 10-year-old girl who presented following a hemorrhagic stroke and recovered well after indirect bypass. The second patient was an adult with a history of childhood stroke whose symptoms progressed in adulthood. She underwent a direct bypass and improved, but continued to experience TIA at her 4 year follow-up. The third patient was a 7-year-old girl with headaches and a new onset TIA who failed pharmacological therapy and subsequently underwent bilateral indirect bypass. The fourth patient was a 24-year-old woman with worsening headaches and an occluded left middle cerebral artery from unilateral moyamoya syndrome. A left sided direct bypass was completed given delayed MRI perfusion with poor augmentation. To our knowledge these are the first reported surgical cases of combined Noonan and moyamoya syndrome. These cases highlight the need to recognize moyamoya syndrome in patients with Noonan syndrome. Early surgical revascularization should be pursued in order to prevent symptom progression. Copyright © 2015 Elsevier Ltd. All rights reserved.

Obstructive sleep apnoea/hypopnoea syndrome in adults with Down syndrome

OpenAIRE

Hill, Elizabeth A.

2016-01-01

Key points Adults with Down syndrome are predisposed to obstructive sleep apnoea/hypopnoea syndrome (OSAHS) due to overlap between the Down syndrome phenotype and OSAHS risk factors. The prevalence of OSAHS in adults with Down syndrome is estimated at 35?42%. This is up to ten-times higher than in the general adult population. Symptoms of OSAHS, including behavioural and emotional disturbances as well as standard symptoms such as sleepiness, should be monitored as part of regular health surve...

What is Metabolic Syndrome?

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... Intramural Research Home / Metabolic Syndrome Metabolic Syndrome Also known as What Is Metabolic syndrome ... metabolic risk factors to be diagnosed with metabolic syndrome. Metabolic Risk Factors A Large Waistline Having a large ...

Prune belly syndrome

Science.gov (United States)

Eagle-Barrett syndrome; Triad syndrome ... The exact causes of prune belly syndrome are unknown. The condition affects mostly boys. While in the womb, the developing baby's abdomen swells with fluid. Often, the cause is ...

Gorlin-goltz syndrome

Directory of Open Access Journals (Sweden)

B V Shobha

2011-01-01

Full Text Available Gorlin-Goltz syndrome also known as nevoid basal cell carcinoma syndrome (NBCCS is an infrequent multisystemic disease inherited in a dominant autosomal way, which shows a high level of penetrance and variable expressiveness. It is characterized by keratocystic odontogenic tumors (KCOT in the jaw, multiple basal cell carcinomas and skeletal abnormalities. This syndrome may be diagnosed early by a dentist by routine radiographic examination in the first decade of life, as KCOTs are usually one of the first manifestations of the NBCCS syndrome. This article reports the case of a 12-year-old girl with Gorlin-Goltz syndrome, emphasizing its clinical and radiographic manifestation. This study highlights the importance of health professionals in the early diagnosis of this syndrome and a multidisciplinary approach to provide a better diagnosis and prognosis.

Refeeding syndrome

OpenAIRE

Tripathy, Swagata; Mishra, Padmini; Dash, S. C.

2008-01-01

Refeeding syndrome is a potentially fatal medical condition that may affect malnourished patients in response to an inappropriately rapid overfeeding. This commonly occurs following the institution of nutritional support, especially parenteral or enteral nutrition. The most characteristic pathophysiology of refeeding syndrome relates to the rapid consumption of phosphate after glucose intake and subsequent hypophosphatemia. Refeeding syndrome can manifest as either metabolic changes （hypokala...

Multi-pass 1.9 um Tm:YLF slab laser pump source

CSIR Research Space (South Africa)

Strauss

2010-09-01

Full Text Available stream_source_info Strauss1_2010.pdf.txt stream_content_type text/plain stream_size 4116 Content-Encoding ISO-8859-1 stream_name Strauss1_2010.pdf.txt Content-Type text/plain; charset=ISO-8859-1 Multi-Pass 1.9 ?m Tm...:YLF Slab Laser Pump Source H.J. Strauss1, S.C. Burd2, W. Koen1, M.J.D. Esser1, C. Jacobs1, O.J.P. Collett1, K. Nyangaza1, D. Preussler1 and C. Bollig1 1 Laser Centre, Council for Scientific and Industrial Research, PO Box 395, Pretoria, 0001, South...

Nevoid basal cell carcinoma syndrome

Science.gov (United States)

NBCC syndrome; Gorlin-Goltz syndrome; Basal cell nevus syndrome; BCNS; Basal cell cancer - nevoid basal cell carcinoma syndrome ... Nevoid basal cell carcinoma nevus syndrome is a rare genetic ... syndrome is known as PTCH ("patched"). The gene is passed down ...

The wellness syndrome

DEFF Research Database (Denmark)

Mik-Meyer, Nanna

2015-01-01

Klumme. Wellness er blevet et syndrom, og dets symptomer er angst, selvbebrejdelser og skyldfølelse. Kommentar med udgangspunkt i: Carl Cederström & Andre Spicer, "The Wellness Syndrome" (Polity Books, 2015. 200 p.).......Klumme. Wellness er blevet et syndrom, og dets symptomer er angst, selvbebrejdelser og skyldfølelse. Kommentar med udgangspunkt i: Carl Cederström & Andre Spicer, "The Wellness Syndrome" (Polity Books, 2015. 200 p.)....

Turner Syndrome: Other FAQs

Science.gov (United States)

... Other FAQs Share Facebook Twitter Pinterest Email Print Turner Syndrome: Other FAQs Basic information for topics, such as " ... been diagnosed with Turner syndrome. Now what? Is Turner syndrome inherited? Turner syndrome is usually not inherited, but ...

Bardet-Biedl syndrome and Usher syndrome.

Science.gov (United States)

Koenig, Rainer

2003-01-01

Bardet-Biedl syndrome (BBS) and Usher syndrome (USH) are the most prevalent syndromic forms of retinitis pigmentosa (RP), together they make up almost a quarter of the patients with RP. BBS is defined by the association of retinopathy, obesity, hypogonadism, renal dysfunction, postaxial polydactyly and mental retardation. This clinically complex syndrome is genetically heterogeneous with linkage to more than 6 loci, and 4 genes have been cloned so far. Recent molecular data present evidence that, in some instances, the clinical manifestation of BBS requires recessive mutations in 1 of the 6 BBS loci plus one or two additional mutations in a second BBS locus (tri- or tetra-allelic inheritance). USH is characterized by the combination of congenital or early-onset sensorineural deafness, RP, and variable degrees of vestibular dysfunction. Each of the three clinical types is genetically heterogeneous: 7 loci have been mapped for type 1, three loci for type 2, and two loci for type 3. Currently, 6 USH genes (MYO7A, USH1C, CDH23, PCDH15, USH2A, USH3) have been identified. Pathogenetically, mutations of the USH1 genes seem to result in defects of auditory and retinal sensory cells, the USH 2 phenotype is caused by defects of extracellular matrix or cell surface receptor proteins, and USH3 may be due to synaptic disturbances. The considerable contribution of syndromic forms of RP requires interdisciplinary approaches to the clinical and diagnostic management of RP patients.

Abdominal compartment syndrome with acute reperfusion syndrome

International Nuclear Information System (INIS)

Maleeva, A.

2017-01-01

Abdominal compartment syndrome was recognized clinically in the 19th century when Marey and Burt observed its association with declines in respiratory function. Abdominal compartment syndrome is first used as a medical terminology from Fietsman in a case of ruptured abdominal aortic aneurysm. A condition caused by abnormally increased pressure within the abdomen. Causes of abdominal compartment syndrome include trauma, surgery, or infection. Common symptoms: abdominal distension, fast heart rate, insufficient urine production, or low blood pressure Medical procedure: nasogastric intubation Surgery: laparotomy Specialists: radiologist, primary care provider (PCP), surgeon, and emergency medicine doctor [6, 10]. Keywords: Stomach. Gastroparesis . Diabetes Mellitus [bg

Munchausen syndrome and Munchausen syndrome by proxy in dermatology.

Science.gov (United States)

Boyd, Alan S; Ritchie, Coleman; Likhari, Sunaina

2014-08-01

Patients with Munchausen syndrome purposefully injure themselves, often with the injection of foreign materials, to gain hospital admission and the attention associated with having a difficult-to-identify condition. Munchausen syndrome by proxy occurs when a child's caregiver, typically the mother, injures the child for the same reasons. Cases of Munchausen syndrome and Munchausen syndrome by proxy with primary cutaneous involvement appear to be rarely described in the literature suggesting either that diagnosis is not made readily or that it is, in fact, an uncommon disorder. At the center of both conditions is significant psychological pathology and treatment is difficult as many patients with Munchausen syndrome when confronted with these diagnostic possibilities simply leave the hospital. Little is known about the long-term outcome or prognosis of these patients. Copyright © 2014 American Academy of Dermatology, Inc. Published by Mosby, Inc. All rights reserved.

Roberts-SC syndrome, a rare syndrome and cleft palate repair

Directory of Open Access Journals (Sweden)

Murthy Jyotsna

2008-01-01

Full Text Available Roberts SC syndrome is a rare syndrome with only 17 previously recognized patients reported in medical literature. The syndrome is characterized by multiple malformations, particularly, symmetrical limb reduction, craniofacial anomalies such as bilateral cleft lip and palate, micrognathia, and severe growth and mental retardation. Our patient, a young child of five years having Roberts-SC, was successfully operated for cleft palate under general anesthesia. The main features of the syndrome and the technical problems of anesthesia and surgery are discussed in this report.

Sjogren-Larsson Syndrome

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... Or In Memory Of Obituaries Contact Us Donate Sjogren-Larsson Syndrome What causes SLS? SLS is caused by mutations ... methods of diagnosing SLS. Other Clinical Names for Sjogren-Larsson Syndrome Other clinical names of Sjogren-Larsson Syndrome include: ...

Redefining syndromic surveillance

Directory of Open Access Journals (Sweden)

Rebecca Katz

2011-12-01

Full Text Available With growing concerns about international spread of disease and expanding use of early disease detection surveillance methods, the field of syndromic surveillance has received increased attention over the last decade. The purpose of this article is to clarify the various meanings that have been assigned to the term syndromic surveillance and to propose a refined categorization of the characteristics of these systems. Existing literature and conference proceedings were examined on syndromic surveillance from 1998 to 2010, focusing on low- and middle-income settings. Based on the 36 unique definitions of syndromic surveillance found in the literature, five commonly accepted principles of syndromic surveillance systems were identified, as well as two fundamental categories: specific and non-specific disease detection. Ultimately, the proposed categorization of syndromic surveillance distinguishes between systems that focus on detecting defined syndromes or outcomes of interest and those that aim to uncover non-specific trends that suggest an outbreak may be occurring. By providing an accurate and comprehensive picture of this field’s capabilities, and differentiating among system types, a unified understanding of the syndromic surveillance field can be developed, encouraging the adoption, investment in, and implementation of these systems in settings that need bolstered surveillance capacity, particularly low- and middle-income countries.

Gorlin-Goltz Syndrome

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Padma Pandeshwar

2012-01-01

Full Text Available The Gorlin-Goltz syndrome (GGS (the nevoid basal cell carcinoma syndrome—NBCCS is a rare autosomal dominant syndrome caused due to mutations in the PTCH (patched gene found on chromosome arm 9q. The syndrome, characterized by increased predisposition to develop basal cell carcinoma and associated multiorgan anomalies, has a high level of penetrance and variable expressiveness. GGS is a multidisciplinary problem, early diagnosis of which allows introduction of secondary prophylaxis and following an appropriate treatment to delay the progress of the syndrome. The following report emphasizes the need for awareness of the diagnostic criteria of this syndrome in cases with no typical skin lesions.

Sjögren syndrome

Science.gov (United States)

Xerostomia - Sjögren syndrome; Keratoconjunctivitis sicca - Sjögren; Sicca syndrome ... The cause of Sjögren syndrome is unknown. It is an autoimmune disorder. This means the body attacks healthy tissue by mistake. The syndrome occurs most ...

Marfan Syndrome (For Parents)

Science.gov (United States)

... en español Síndrome de Marfan What Is Marfan Syndrome? Marfan syndrome is a genetic disorder of the body's ... bones , blood vessels, and organs. What Causes Marfan Syndrome? Marfan syndrome happens because of an abnormality in one ...

Nevoid basal cell carcinoma syndrome (Gorlin-Goltz syndrome).

Science.gov (United States)

Kiran, N K; Tilak Raj, T N; Mukunda, K S; Rajashekar Reddy, V

2012-10-01

The Gorlin-Goltz syndrome, also known as nevoid basal cell carcinoma syndrome (NBCCS), is an infrequent multisystemic disease inherited in a dominant autosomal way, which shows a high level of penetrance and variable expressiveness. It is characterized by odontogenic keratocysts in the jaw, multiple basal cell nevi carcinomas and skeletal abnormalities. This syndrome may be diagnosed early by a dentist by routine radiographic exams in the first decade of life, since the odontogenic keratocysts are usually one of the first manifestations of the syndrome. This case report presents a patient diagnosed as NBCCS by clinical, radiographic and histological findings in a 13-year-old boy. This paper highlights the importance of early diagnosis of NBCCS which can help in preventive multidisciplinary approach to provide a better prognosis for the patient.

Nevoid basal cell carcinoma syndrome (Gorlin-Goltz syndrome

Directory of Open Access Journals (Sweden)

N K Kiran

2012-01-01

Full Text Available The Gorlin-Goltz syndrome, also known as nevoid basal cell carcinoma syndrome (NBCCS, is an infrequent multisystemic disease inherited in a dominant autosomal way, which shows a high level of penetrance and variable expressiveness. It is characterized by odontogenic keratocysts in the jaw, multiple basal cell nevi carcinomas and skeletal abnormalities. This syndrome may be diagnosed early by a dentist by routine radiographic exams in the first decade of life, since the odontogenic keratocysts are usually one of the first manifestations of the syndrome. This case report presents a patient diagnosed as NBCCS by clinical, radiographic and histological findings in a 13-year-old boy. This paper highlights the importance of early diagnosis of NBCCS which can help in preventive multidisciplinary approach to provide a better prognosis for the patient.

``Battered child`` syndrome; Das ``Battered-Child``-Syndrom

Energy Technology Data Exchange (ETDEWEB)

Elsner, K.; Merk, J.; Sokiranski, R. [Ulm Univ. (Germany). Abt. Diagnostische Radiologie

1997-10-01

Synonyms for the `battered child` syndrome (BCS) are terms describing the physical and body aspects of the process, such as `child abuse`, or `non-accidental injury`. These are to be distinguished from the psychic aspects and abuse, emotional and bodily neglect, and sexual abuse. Most cases are one or another combination of these aspects. Radiology is the essential method for giving proof of such abuses, identifying the signs of maltreatment in a medical record, or for disproving suspected abuse. (orig./AJ) [Deutsch] Als Synonym fuer das `Battered-Child`-Syndrom (BCS) stehen die Begriffe der koerperlichen-/physikalischen-Kindesmisshandlung, im angelsaechsischen Sprachraum die Begriffe `Child Abuse` und `Nonaccidental Injury`. Vom Syndrom abzugrenzen sind die seelische Misshandlung, die seelische und koerperliche Vernachlaessigung, und der sexuelle Missbrauch. Kombinationsformen sind nicht selten. Bei der Diagnostik des Syndroms spielt die Radiologie eine entscheidende Rolle. So hilft der Einsatz adaequater Untersuchungsmethoden, den Tatbestand der Misshandlung zu identifizieren und zu dokumentieren, aber auch einen Verdacht zu widerlegen. (orig./AJ)

Hepatorenal syndrome

Science.gov (United States)

... 2016:chap 153. Nevah MI, Fallon MB. Hepatic encephalopathy, hepatorenal syndrome, hepatopulmonary syndrome, and other systemic complications of liver disease. In: Feldman M, Friedman LS, Brandt LJ, ...

Prevalence of Burnout Syndrome in patients admitted with acute coronary syndrome.

Science.gov (United States)

Prosdócimo, Ana Cláudia Giaxa; Lucina, Luciane Boreki; Marcia, Olandoski; Jobs, Priscila Megda João; Schio, Nicolle Amboni; Baldanzi, Fernanda Fachin; Costantini, Costantino Ortiz; Benevides-Pereira, Ana Maria Teresa; Guarita-Souza, Luiz Cesar; Faria-Neto, José Rocha

2015-03-01

Burnout Syndrome is the extreme emotional response to chronic occupational stress, manifesting as physical and mental exhaustion. Although associated with higher prevalence of cardiovascular risk factors, no study so far has evaluated whether the Burnout Syndrome could be a prevalent factor in non-elderly individuals active in the labor market, admitted for acute coronary syndrome (ACS). To evaluate the prevalence of the Burnout Syndrome in non-elderly, economically active patients, hospitalized with ACS. Cross-sectional study conducted in a tertiary and private cardiology center, with economically active patients aged Burnout Syndrome was evaluated with the Burnout Syndrome Inventory (BSI), which assesses workplace conditions and four dimensions that characterize the syndrome: emotional exhaustion (EE), emotional distancing (EmD), dehumanization (De) and professional fulfillment (PF). The Lipp's Stress Symptoms Inventory for Adults (LSSI) was applied to evaluate global stress. Of 830 patients evaluated with suspected ACS, 170 met the study criteria, 90% of which were men, overall average age was 52 years, and 40.5% had an average income above 11 minimum wages. The prevalence of the Burnout Syndrome was 4.1%. When we evaluated each dimension individually, we found high EE in 34.7%, high De in 52.4%, high EDi in 30.6%, and low PF in 5.9%. The overall prevalence of stress was 87.5%. We found a low prevalence of Burnout Syndrome in an economically active, non-elderly population among patients admitted for ACS in a tertiary and private hospital.

Turner Syndrome (For Teens)

Science.gov (United States)

... Staying Safe Videos for Educators Search English Español Turner Syndrome KidsHealth / For Teens / Turner Syndrome What's in this ... en español El síndrome de Turner What Is Turner Syndrome? Turner syndrome (TS) is a genetic condition found ...

DIDMOAD (Wolfram Syndrome

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Masoud Nashibi

2016-07-01

Full Text Available Wolfram syndrome was first described by physician D J Wolfram and Wagener in 1938. This autosomal recessive syndrome is also referred to as DIDMOAD syndrome which stands for Diabetes Insipidus, Insulin Dependent Diabetes Mellitus, Optic Atrophy and Deafness

[The Capgras syndrome].

Science.gov (United States)

Anikina, M A; Levin, O S

2013-01-01

The Capgras syndrome is one of delusional-like misidentification syndrome in which a person holds a delusion that one or several his/her friends or relatives have been replaced by an identical-looking impostor. As any other delusional disorder, the Capgras syndrome is characterized by stability despite the indisputable arguments against fault views. Initially, this syndrome was considered as a presentation of schizophrenia but later it has been described in brain organic disorders, primarily in elderly patients with dementia.

Revesz syndrome

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Dayane Cristine Issaho

2015-04-01

Full Text Available Revesz syndrome is a rare variant of dyskeratosis congenita and is characterized by bilateral exudative retinopathy, alterations in the anterior ocular segment, intrauterine growth retardation, fine sparse hair, reticulate skin pigmentation, bone marrow failure, cerebral calcification, cerebellar hypoplasia and psychomotor retardation. Few patients with this syndrome have been reported, and significant clinical variations exist among patients. This report describes the first Brazilian case of Revesz syndrome and its ocular and clinical features.

Cardiorenal Syndrome in Acute Heart Failure Syndromes

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Mohammad Sarraf

2011-01-01

Full Text Available Impaired cardiac function leads to activation of the neurohumoral axis, sodium and water retention, congestion and ultimately impaired kidney function. This sequence of events has been termed the Cardiorenal Syndrome. This is different from the increase in cardiovascular complications which occur with primary kidney disease, that is, the so-called Renocardiac Syndrome. The present review discusses the pathogenesis of the Cardiorenal Syndrome followed by the benefits and potential deleterious effects of pharmacological agents that have been used in this setting. The agents discussed are diuretics, aquaretics, natriuretic peptides, vasodilators, inotropes and adenosine α1 receptor antagonists. The potential role of ultrafiltration is also briefly discussed.

Interstitial Cystitis/Painful Bladder Syndrome and Associated Medical Conditions With an Emphasis on Irritable Bowel Syndrome, Fibromyalgia and Chronic Fatigue Syndrome

DEFF Research Database (Denmark)

Nickel, J.C.; Tripp, D.A.; Pontari, M.

2010-01-01

of associated conditions increased (ie localized, regional, systemic), pain, stress, depression and sleep disturbance increased while social support, sexual functioning and quality of life deteriorated. Anxiety and catastrophizing remained increased in all groups. Symptom duration was associated......Purpose: We characterized and compared the impact of clinical phenotypic associations between interstitial cystitis/painful bladder syndrome and controls in relation to potentially related conditions, particularly irritable bowel syndrome, fibromyalgia and chronic fatigue syndrome. Materials...... cystitis/painful bladder syndrome vs controls was irritable bowel syndrome 38.6% vs 5.2%, fibromyalgia 17.7% vs 2.6% and chronic fatigue syndrome 9.5% vs 1.7% (all p

Plummer-Vinson syndrome

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Novacek Gottfried

2006-09-01

Full Text Available Abstract Plummer-Vinson or Paterson-Kelly syndrome presents as a classical triad of dysphagia, iron-deficiency anemia and esophageal webs. Exact data about epidemiology of the syndrome are not available; the syndrome is extremely rare. Most of the patients are white middle-aged women, in the fourth to seventh decade of life but the syndrome has also been described in children and adolescents. The dysphagia is usually painless and intermittent or progressive over years, limited to solids and sometimes associated with weight loss. Symptoms resulting from anemia (weakness, pallor, fatigue, tachycardia may dominate the clinical picture. Additional features are glossitis, angular cheilitis and koilonychia. Enlargement of the spleen and thyroid may also be observed. One of the most important clinical aspects of Plummer-Vinson syndrome is the association with upper alimentary tract cancers. Etiopathogenesis of Plummer-Vinson syndrome is unknown. The most important possible etiological factor is iron deficiency. Other possible factors include malnutrition, genetic predisposition or autoimmune processes. Plummer-Vinson syndrome can be treated effectively with iron supplementation and mechanical dilation. In case of significant obstruction of the esophageal lumen by esophageal web and persistent dysphagia despite iron supplementation, rupture and dilation of the web are necessary. Since Plummer-Vinson syndrome is associated with an increased risk of squamous cell carcinoma of the pharynx and the esophagus, the patients should be followed closely.

Genetics Home Reference: antiphospholipid syndrome

Science.gov (United States)

... Share: Email Facebook Twitter Home Health Conditions Antiphospholipid syndrome Antiphospholipid syndrome Printable PDF Open All Close All Enable ... area? Other Names for This Condition anti-phospholipid syndrome antiphospholipid antibody syndrome Hughes syndrome Related Information How are ...

Genetics Home Reference: Costello syndrome

Science.gov (United States)

... other genetic conditions, cardiofaciocutaneous syndrome (CFC syndrome) and Noonan syndrome . In affected infants, it can be difficult to ... These individuals may actually have CFC syndrome or Noonan syndrome , which are caused by mutations in related genes. ...

Metabolic syndrome and menopause

Directory of Open Access Journals (Sweden)

Jouyandeh Zahra

2013-01-01

Full Text Available Abstract Background The metabolic syndrome is defined as an assemblage of risk factors for cardiovascular diseases, and menopause is associated with an increase in metabolic syndrome prevalence. The aim of this study was to assess the prevalence of metabolic syndrome and its components among postmenopausal women in Tehran, Iran. Methods In this cross-sectional study in menopause clinic in Tehran, 118 postmenopausal women were investigated. We used the adult treatment panel 3 (ATP3 criteria to classify subjects as having metabolic syndrome. Results Total prevalence of metabolic syndrome among our subjects was 30.1%. Waist circumference, HDL-cholesterol, fasting blood glucose, diastolic blood pressure ,Systolic blood pressure, and triglyceride were significantly higher among women with metabolic syndrome (P-value Conclusions Our study shows that postmenopausal status is associated with an increased risk of metabolic syndrome. Therefore, to prevent cardiovascular disease there is a need to evaluate metabolic syndrome and its components from the time of the menopause.

Wolfram syndrome 1 and Wolfram syndrome 2.

Science.gov (United States)

Rigoli, Luciana; Di Bella, Chiara

2012-08-01

Wolfram syndrome 1 (WS1) is an autosomal recessive disorder characterized by diabetes insipidus, diabetes mellitus, optic atrophy, and deafness (DI DM OA D syndrome) associated with other variable clinical manifestations. The causative gene for WS1 (WFS1) encoding wolframin maps to chromosome 4p16.1. Wolframin has an important function in maintaining the homeostasis of the endoplasmic reticulum (ER) in pancreatic β cells. Recently, another causative gene, CISD2, has been identified in patients with a type of Wolfram syndrome (WS2) resulting in early optic atrophy, diabetes mellitus, deafness, decreased lifespan, but not diabetes insipidus. The CISD2-encoded protein ERIS (endoplasmic reticulum intermembrane small protein) also localizes to ER, but does not interact directly with wolframin. ERIS maps to chromosome 4q22. Numerous studies have shown an interesting similarity between WFS1 and CISD2 genes. Experimental studies demonstrated that the Cisd2 knockout (Cisd2) mouse shows premature aging and typical symptoms of Wolfram syndrome. These researches provide interesting insight into the relation of neurodegenerative diseases, mitochondrial disorders, and autophagy and are useful for the pathophysiological understanding of both Wolfram syndrome and mitochondrial-mediated premature aging. The knowledge of WS1 and WS2 pathogenesis, and of the interactions between WFS1 and CISD2 genes, is useful for accurate diagnostic classification and for diagnosis of presymptomatic individuals.

How Exemplar Counselor Advocates Develop Social Justice Interest: A Qualitative Investigation

Science.gov (United States)

Swartz, Melissa Robinson; Limberg, Dodie; Gold, Joshua

2018-01-01

The authors examined the experiences of 10 peer-nominated exemplar counselor advocates using grounded theory methodology (Strauss & Corbin, [Strauss, A., 1998]). Analysis by the authors yielded a model of how exemplar counselor advocates develop a social justice interest and provided key insights on how counselor educators can enhance social…

Interaction Between Syndromic and Non-Syndromic Factors Affecting Speech and Language Development in Treacher-Collins Syndrome

Directory of Open Access Journals (Sweden)

Marziyeh Poorjavad

2011-09-01

Full Text Available Background: Treacher-Collins syndrome is a congenital craniofacial disorder with multiple anomalies. This syndrome affects the maxilla, mandible, eyes, middle and outer ears, and soft palate. Conductive hearing loss due to the deformities of the middle and external ears is prevalent. The characteristics of this syndrome include multiple and serious threats to normal communication development in children. In this study, speech and language features of a Persian speaking child with this syndrome are presented.Case: The case was an 8-year old girl with Treacher-Collins syndrome and bilateral moderate conductive hearing loss due to atretic canal. In language and speech assessments, moderate hypernasality, numerous compensatory errors and morphosyntactic deficits were observed. There were 13 phonemes that were incorrectly produced at least in one position. Besides, she used 22 types of phonological processes that were abnormal and disappear before the age of three in normal Persian speaking children.Conclusion: Moderate hearing loss, velopharyngeal incompetency, malocclusion and dental anomalies, attention deficit/hyperactivity disorder (ADHD and environmental factors resulted in severe speech and language disorders in this case. These disorders affected her academic performance as well. Moderate hypernasality, numerous compensatory errors, and excessive and abnormal use of phonological processes were not presented as prevalent characteristics of Treacher-Collins syndrome in other resources.

Urofacial syndrome

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Kamal F Akl

2012-01-01

Full Text Available The urofacial syndrome is characterized by functional obstructive uropathy asso-ciated with an inverted smile. The importance of the subject is that it sheds light, not only on the muscles of facial expression, but also on the inheritance of voiding disorders and lower urinary tract malformations. We report a 10-year-old-male patient who had the urofacial syndrome. Early diagnosis of the urofacial syndrome is important to avoid upper urinary tract damage and renal failure.

Felty syndrome

Science.gov (United States)

Seropositive rheumatoid arthritis (RA); Felty's syndrome ... The cause of Felty syndrome is unknown. It is more common in people who have had rheumatoid arthritis (RA) for a long time. People with ...

Pendred Syndrome

Science.gov (United States)

... other possible long-term consequences of the syndrome. Children with Pendred syndrome should start early treatment to gain communication skills, such as learning sign language or cued speech or learning to ...

Unusual headache syndromes.

Science.gov (United States)

Queiroz, Luiz P

2013-01-01

Some headache syndromes have few cases reported in the literature. Their clinical characteristics, pathogenesis, and treatment may have not been completely defined. They may not actually be uncommon but rather under-recognized and/or underreported. A literature review of unusual headache syndromes, searching PubMed and ISI Web of Knowledge, was performed. After deciding which disorders to study, relevant publications in scientific journals, including original articles, reviews, meeting abstracts, and letters or correspondences to the editors were searched. This paper reviewed the clinical characteristics, the pathogenesis, the diagnosis, and the treatment of five interesting and unusual headache syndromes: exploding head syndrome, red ear syndrome, neck-tongue syndrome, nummular headache, and cardiac cephalgia. Recognizing some unusual headaches, either primary or secondary, may be a challenge for many non-headache specialist physicians. It is important to study them because the correct diagnosis may result in specific treatments that may improve the quality of life of these patients, and this can even be life saving. © 2013 American Headache Society.

Burnout Syndrome of Teachers

OpenAIRE

Semrádová, Michaela

2013-01-01

The bachelor's thesis covers burnout syndrome of teachers. Defines burnout syndrome, describes its causes and symptoms. Describes teaching as helping profession and focousing on stressful situations at school. In the last chapter described different prevention strategies burnout syndrome. Key words: burnout syndrome, teaching, teacher, helping professions, beginning teacher, stress

Prevalence of Burnout Syndrome in Patients Admitted with Acute Coronary Syndrome

Directory of Open Access Journals (Sweden)

Ana Cláudia Giaxa Prosdócimo

2015-03-01

Full Text Available Background: Burnout Syndrome is the extreme emotional response to chronic occupational stress, manifesting as physical and mental exhaustion. Although associated with higher prevalence of cardiovascular risk factors, no study so far has evaluated whether the Burnout Syndrome could be a prevalent factor in non-elderly individuals active in the labor market, admitted for acute coronary syndrome (ACS. Objective: To evaluate the prevalence of the Burnout Syndrome in non-elderly, economically active patients, hospitalized with ACS. Methods: Cross-sectional study conducted in a tertiary and private cardiology center, with economically active patients aged <65 years, hospitalized with diagnosis of ACS. The Burnout Syndrome was evaluated with the Burnout Syndrome Inventory (BSI, which assesses workplace conditions and four dimensions that characterize the syndrome: emotional exhaustion (EE, emotional distancing (EmD, dehumanization (De and professional fulfillment (PF. The Lipp’s Stress Symptoms Inventory for Adults (LSSI was applied to evaluate global stress. Results: Of 830 patients evaluated with suspected ACS, 170 met the study criteria, 90% of which were men, overall average age was 52 years, and 40.5% had an average income above 11 minimum wages. The prevalence of the Burnout Syndrome was 4.1%. When we evaluated each dimension individually, we found high EE in 34.7%, high De in 52.4%, high EDi in 30.6%, and low PF in 5.9%. The overall prevalence of stress was 87.5%. Conclusion: We found a low prevalence of Burnout Syndrome in an economically active, non-elderly population among patients admitted for ACS in a tertiary and private hospital.

"Nine" syndrome: A new neuro-ophthalmologic syndrome: Report of two cases

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Rohan R Mahale

2015-01-01

Full Text Available "Eight-and-a-half" syndrome is a rare condition involving the ipsilateral abducens nucleus or paramedian pontine reticular formation (PPRF, the ipsilateral medial longitudinal fasciculus (MLF, and the adjacent facial colliculus/facial nerve fascicle. The condition is often caused by a lesion (vascular or demyelinating in the dorsal tegmentum of the caudal pons. There are new variants of this syndrome caused by extension of lesion to involve new adjacent structures in pontine tegmentum. We report two patients with different etiology presenting with clinical features suggestive of eight-and-a-half syndrome associated with hemiataxia representing "nine" syndrome (8– + – = 9 adding new dimension to "eight-and-a-half" syndrome.

Mobius syndrome redefined: a syndrome of rhombencephalic maldevelopment.

NARCIS (Netherlands)

Verzijl, H.T.F.M.; Zwaag, A. van der; Cruysberg, J.R.M.; Padberg, G.W.A.M.

2003-01-01

OBJECTIVE: To investigate the variable clinical picture of Mobius syndrome (MIM no. 157900) and to further understand the pathogenesis of the disorder. METHODS: A standardized questionnaire was submitted to 37 Dutch patients with Mobius syndrome. All underwent standardized neurologic examination

Bartter syndrome

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... this page: //medlineplus.gov/ency/article/000308.htm Bartter syndrome To use the sharing features on this page, please enable JavaScript. Bartter syndrome is a group of rare conditions that affect ...

Dravet Syndrome

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... and supports a broad program of basic and clinical research on all types of epilepsy, including Dravet syndrome. Study of the genetic defects responsible for Dravet syndrome and related ... Publications Definition Dravet ...

Genetics Home Reference: Marfan syndrome

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... Share: Email Facebook Twitter Home Health Conditions Marfan syndrome Marfan syndrome Printable PDF Open All Close All Enable Javascript ... Marfan syndrome KidsHealth from Nemours Foundation MalaCards: marfan syndrome Orphanet: Marfan syndrome Your Genes Your Health from Cold Spring ...

Does wastewater discharge have relations with increase of Turner syndrome and Down syndrome?

Science.gov (United States)

Choi, Intae

2017-01-01

The purpose of this study is to examine whether water and air pollutants have a relationship with an increase in the genetic disorders Turner syndrome and Down syndrome, which are caused by congenital chromosomal abnormalities, and to generate a hypothesis about the genetic health effects of environmental pollutants. A panel regression based on random effect was conducted on Korea's metropolitan councils from 2012 to 2014. The dependent variable was the number of Turner syndrome and Down syndrome cases, and the main independent variables were those regarding the water and air pollution. Air pollutants did not have a significant impact on the number of Turner syndrome and Down syndrome cases; however, the increase in number of wastewater discharge companies did have a significant relationship with the number of cases. The more the number of wastewater discharge companies, the more the number Turner syndrome and Down syndrome cases were observed. Therefore, scientific investigation on water and air pollutants in relation with genetic health effects needs to be performed.

Coffin-Siris syndrome with Mayer-Rokitansky-Küster-Hauser syndrome: a case report

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Shukla Umesh

2010-11-01

Full Text Available Abstract Introduction We report the case of an unusual association of Coffin-Siris syndrome with Mayer-Rokitansky-Küster-Hauser syndrome. This association has never previously been reported in the medical literature. Case presentation A nine-year-old Indian girl was referred to our hospital for growth retardation, mental retardation, lax joints, generalized hypertrichosis, and hypoplastic fifth fingernails and toenails. A thorough medical examination and evaluation revealed she had phenotypic features of Coffin-Siris syndrome, with Mayer-Rokitansky-Küster-Hauser syndrome on radiological evaluation. The karyotype of our patient was normal. Conclusion In an unexplained case of mental retardation with facies suggestive of Coffin-Siris syndrome, association with Mayer-Rokitansky-Küster-Hauser syndrome should be considered and the patient should be evaluated for the same. Both of these syndromes may have a common pathogenesis, as yet unknown. This case report has broad implications, as similar cases in future may give insights into the pathogenesis of both these syndromes.

Coffin-Siris syndrome with Mayer-Rokitansky-Küster-Hauser syndrome: a case report.

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Goyal, Deepak; Yadav, Dinesh K; Shukla, Umesh; Sethi, Sidharth K

2010-11-08

We report the case of an unusual association of Coffin-Siris syndrome with Mayer-Rokitansky-Küster-Hauser syndrome. This association has never previously been reported in the medical literature. A nine-year-old Indian girl was referred to our hospital for growth retardation, mental retardation, lax joints, generalized hypertrichosis, and hypoplastic fifth fingernails and toenails. A thorough medical examination and evaluation revealed she had phenotypic features of Coffin-Siris syndrome, with Mayer-Rokitansky-Küster-Hauser syndrome on radiological evaluation. The karyotype of our patient was normal. In an unexplained case of mental retardation with facies suggestive of Coffin-Siris syndrome, association with Mayer-Rokitansky-Küster-Hauser syndrome should be considered and the patient should be evaluated for the same. Both of these syndromes may have a common pathogenesis, as yet unknown. This case report has broad implications, as similar cases in future may give insights into the pathogenesis of both these syndromes.

Rett Syndrome

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Rett syndrome is a rare genetic disease that causes developmental and nervous system problems, mostly in girls. It's related to autism spectrum disorder. Babies with Rett syndrome seem to grow and develop normally at first. ...

Caplan syndrome

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... enable JavaScript. Rheumatoid pneumoconiosis (RP; also known as Caplan syndrome) is swelling (inflammation) and scarring of the ... avoid exposure to inorganic dust. Alternative Names RP; Caplan syndrome; Pneumoconiosis - rheumatoid; Silicosis - rheumatoid pneumoconiosis; Coal worker's ...

Sotos Syndrome

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... Clinical Trials Organizations Publications Definition Sotos syndrome (cerebral gigantism) is a rare genetic disorder caused by mutation ... have also been reported. × Definition Sotos syndrome (cerebral gigantism) is a rare genetic disorder caused by mutation ...

Chronic exertional compartment syndrome with medial tibial stress syndrome in twins.

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Banerjee, Purnajyoti; McLean, Christopher

2011-06-14

Chronic exertional compartment syndrome and medial tibial stress syndrome are uncommon conditions that affect long-distance runners or players involved in team sports that require extensive running. We report 2 cases of bilateral chronic exertional compartment syndrome, with medial tibial stress syndrome in identical twins diagnosed with the use of a Kodiag monitor (B. Braun Medical, Sheffield, United Kingdom) fulfilling the modified diagnostic criteria for chronic exertional compartment syndrome as described by Pedowitz et al, which includes: (1) pre-exercise compartment pressure level >15 mm Hg; (2) 1 minute post-exercise pressure >30 mm Hg; and (3) 5 minutes post-exercise pressure >20 mm Hg in the presence of clinical features. Both patients were treated with bilateral anterior fasciotomies through minimal incision and deep posterior fasciotomies with tibial periosteal stripping performed through longer anteromedial incisions under direct vision followed by intensive physiotherapy resulting in complete symptomatic recovery. The etiology of chronic exertional compartment syndrome is not fully understood, but it is postulated abnormal increases in intramuscular pressure during exercise impair local perfusion, causing ischemic muscle pain. No familial predisposition has been reported to date. However, some authors have found that no significant difference exists in the relative perfusion, in patients, diagnosed with chronic exertional compartment syndrome. Magnetic resonance images of affected compartments have indicated that the pain is not due to ischemia, but rather from a disproportionate oxygen supply versus demand. We believe this is the first report of chronic exertional compartment syndrome with medial tibial stress syndrome in twins, raising the question of whether there is a genetic predisposition to the causation of these conditions. Copyright 2011, SLACK Incorporated.

Moyamoya syndrome in a patient with Noonan-like syndrome with loose anagen hair.

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Choi, Jin-Ho; Oh, Moon-Yeon; Yum, Mi-Sun; Lee, Beom Hee; Kim, Gu-Hwan; Yoo, Han-Wook

2015-03-01

Noonan-like syndrome with loose anagen hair is one of the RASopathies characterized by Noonan syndrome-like features with unique ectodermal abnormalities. This syndrome is caused by mutations in the SHOC2 gene. We encountered a patient with moyamoya syndrome associated with Noonan-like syndrome with loose anagen hair presenting with transient ischemic attacks. A 6-year-old girl was diagnosed with Noonan-like syndrome with loose anagen hair because of profound short stature and ectodermal anomalies such as sparse and easily pluckable hair. A heterozygous mutation of c.4A>G (p.S2G) in the SHOC2 gene was identified, and recombinant human growth hormone therapy was initiated at 8 years of age. At age 10, she manifested recurrent left hemiplegia. Moreover, cerebrovascular imaging revealed occlusion or narrowing of both internal carotid arteries and both middle cerebral arteries with distal moyamoya-like vessels. She is treated with aspirin and calcium channel blocker. We describe the first case of Noonan-like syndrome with loose anagen hair associated with moyamoya syndrome, although it has been reported to be associated with a few cases of other RASopathies, including Noonan, cardiofaciocutaneous, and Costello syndromes. This report emphasizes the associations between cerebrovascular anomalies and Noonan-like syndrome with loose anagen hair. Copyright © 2015 Elsevier Inc. All rights reserved.

Abdominal vascular syndromes: characteristic imaging findings

International Nuclear Information System (INIS)

Cardarelli-Leite, Leandro; Velloni, Fernanda Garozzo; Salvadori, Priscila Silveira; Lemos, Marcelo Delboni; D'Ippolito, Giuseppe

2016-01-01

Abdominal vascular syndromes are rare diseases. Although such syndromes vary widely in terms of symptoms and etiologies, certain imaging findings are characteristic. Depending on their etiology, they can be categorized as congenital - including blue rubber bleb nevus syndrome, Klippel-Trenaunay syndrome, and hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber syndrome) - compressive - including 'nutcracker' syndrome, median arcuate ligament syndrome, Cockett syndrome (also known as May-Thurner syndrome), and superior mesenteric artery syndrome. In this article, we aimed to illustrate imaging findings that are characteristic of these syndromes, through studies conducted at our institution, as well as to perform a brief review of the literature on this topic. (author)

Abdominal vascular syndromes: characteristic imaging findings

Energy Technology Data Exchange (ETDEWEB)

Cardarelli-Leite, Leandro; Velloni, Fernanda Garozzo; Salvadori, Priscila Silveira; Lemos, Marcelo Delboni; D' Ippolito, Giuseppe, E-mail: leandrocleite@gmail.com [Universidade Federal de Sao Paulo (EPM/UNIFESP), Sao Paulo, SP (Brazil). Escola Paulista de Mediciana. Departmento de Diagnostico por Imagem

2016-07-15

Abdominal vascular syndromes are rare diseases. Although such syndromes vary widely in terms of symptoms and etiologies, certain imaging findings are characteristic. Depending on their etiology, they can be categorized as congenital - including blue rubber bleb nevus syndrome, Klippel-Trenaunay syndrome, and hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber syndrome) - compressive - including 'nutcracker' syndrome, median arcuate ligament syndrome, Cockett syndrome (also known as May-Thurner syndrome), and superior mesenteric artery syndrome. In this article, we aimed to illustrate imaging findings that are characteristic of these syndromes, through studies conducted at our institution, as well as to perform a brief review of the literature on this topic. (author)

Cushing's Syndrome

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Cushing's syndrome is a hormonal disorder. The cause is long-term exposure to too much cortisol, a hormone that ... your body to make too much cortisol. Cushing's syndrome is rare. Some symptoms are Upper body obesity ...

Usher Syndrome

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Usher syndrome is an inherited disease that causes serious hearing loss and retinitis pigmentosa, an eye disorder that causes ... and vision. There are three types of Usher syndrome: People with type I are deaf from birth ...

Metabolic Syndrome

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Metabolic syndrome is a group of conditions that put you at risk for heart disease and diabetes. These conditions ... agree on the definition or cause of metabolic syndrome. The cause might be insulin resistance. Insulin is ...

Reye Syndrome

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Reye syndrome is a rare illness that can affect the blood, liver, and brain of someone who has recently ... a viral illness, seek medical attention immediately. Reye syndrome can lead to a coma and brain death, ...

Turner Syndrome

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Turner syndrome is a genetic disorder that affects a girl's development. The cause is a missing or incomplete ... t work properly. Other physical features typical of Turner syndrome are Short, "webbed" neck with folds of skin ...

Apert syndrome (acrocephalosyndactyly

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Milovanović J.

2014-01-01

Full Text Available Apert syndrome is named for the French physician, Eugen Apert who was, in 1906. described anomalous shape of the skull with coronary suture synostosis and hypoplasia sphenoethmoidmaxillary part of the face and fingers syndactyly of hands and feet. Apert syndrome accounts for about 4,5% of all craniosynostosis. With the prevalence of 1:160 000-200 000, inherited in an autosomal domi­nant, and in 25% of cases are fresh mutations in the gene. This syndrome has no predilection by gender and race, varies in severity form in witch it is manifested. Anomality of internal organs are very rare, but half of the patients with this syndrome have mental retardation. Apert syndrome has no cure, but surgery can help to correct some of the problems.

Coexistent Brugada Syndrome and Wolff-Parkinson-White Syndrome: What is the Optimal Management?

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Abhishek Jaiswal, MBBS

2013-09-01

Full Text Available Coexistent Brugada syndrome and Wolff-Parkinson-White (WPW syndrome is rare, and as such poses management challenges. The overlap of symptoms attributable to each condition, the timing of ventricular stimulation after accessory pathway ablation and the predictive value of programmed stimulation in Brugada syndrome are controversial. We describe a case of coexistent Brugada syndrome and WPW syndrome in a symptomatic young adult. We discuss our treatment approach and the existing literature along with the challenges in management of such cases.

Cowden syndrome

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Ravi Prakash S

2010-01-01

Full Text Available Cowden syndrome or multiple hamartoma syndrome is an autosomal dominant condition with variable expressions that result mainly from mutation in the PTEN gene on arm 10q. It is characterized by multiple hamartomatous neoplasms of the skin, oral mucosa, gastrointestinal tract, bones, CNS, eyes, and genitourinary tract. Mucocutaneous features include trichilemmomas, oral mucosal papillomatosis, acral keratosis, and palmoplantar keratosis. Here we present a case of Cowden syndrome in a 14-year-old female patient with the chief complaint of multiple oral papillomatous lesions.

Metabolic syndrome and the risk of adverse cardiovascular events after an acute coronary syndrome.

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Cavallari, Ilaria; Cannon, Christopher P; Braunwald, Eugene; Goodrich, Erica L; Im, KyungAh; Lukas, Mary Ann; O'Donoghue, Michelle L

2018-05-01

Background The incremental prognostic value of assessing the metabolic syndrome has been disputed. Little is known regarding its prognostic value in patients after an acute coronary syndrome. Design and methods The presence of metabolic syndrome (2005 International Diabetes Federation) was assessed at baseline in SOLID-TIMI 52, a trial of patients within 30 days of acute coronary syndrome (median follow-up 2.5 years). The primary endpoint was major coronary events (coronary heart disease death, myocardial infarction or urgent coronary revascularization). Results At baseline, 61.6% ( n = 7537) of patients met the definition of metabolic syndrome, 34.7% (n = 4247) had diabetes and 29.3% had both ( n = 3584). The presence of metabolic syndrome was associated with increased risk of major coronary events (adjusted hazard ratio (adjHR) 1.29, p metabolic syndrome was numerically but not significantly associated with the risk of major coronary events (adjHR 1.13, p = 0.06). Conversely, diabetes was a strong independent predictor of major coronary events in the absence of metabolic syndrome (adjHR 1.57, p metabolic syndrome identified patients at highest risk of adverse outcomes but the incremental value of metabolic syndrome was not significant relative to diabetes alone (adjHR 1.07, p = 0.54). Conclusions After acute coronary syndrome, diabetes is a strong and independent predictor of adverse outcomes. Assessment of the metabolic syndrome provides only marginal incremental value once the presence or absence of diabetes is established.

Cases of Trichohepatoenteric Syndrome (Syndromic Diarrhea with Underlying Crohn’s Disease

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Е. А. Roslavtseva

2015-01-01

Full Text Available Tricho-hepato-enteric syndrome (syndromic, phenotypic diarrhea, SD/THES is a rare inborn disease, which affects bowels. It is caused by the mutation of genes SKIV2L or TTC37. Manifestations include intrauterine hypotrophy, severe chronic diarrhea, which starts in infancy, characteristic facial features and hair growth abnormalities, immune disorders. There are data on two patients dealing with tricho-hepato-enteric syndrome with underlying Crohn’s disease. This is the first description of cases of aggravated tricho-hepatoenteric syndrome ever found in Russian medical literature. 

A new familial intrauterine growth retardation syndrome the "3-M syndrome".

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Spranger, J; Opitz, J M; Nourmand, A

1976-09-01

Two pairs of siblings are described with proportionate dwarfism due to skeletal hypoplasia of prenatal onset. The head size was normal for age and disproportionately large for height. The patients had a characteristic face different from that seen in the Silver-Russell syndrome. The family data are in accordance with autosomal recessive inheritance. In spite of some similarities, the bulk of clinical and genetic evidence suggests that the described intrauterine growth retardation syndrome is different from the Silver-Russell syndrome and presents an apparently "new" entity which has been designated 3-M syndrome.

Are ECG abnormalities in Noonan syndrome characteristic for the syndrome?

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Raaijmakers, R; Noordam, C; Noonan, J A; Croonen, E A; van der Burgt, C J A M; Draaisma, J M T

2008-12-01

Of all patients with Noonan syndrome, 50-90% have one or more congenital heart defects. The most frequent occurring are pulmonary stenosis (PS) and hypertrophic cardiomyopathy. The electrocardiogram (ECG) of a patient with Noonan syndrome often shows a characteristic pattern, with a left axis deviation, abnormal R/S ratio over the left precordium, and an abnormal Q wave. The objective of this study was to determine if these ECG characteristics are an independent feature of the Noonan syndrome or if they are related to the congenital heart defect. A cohort study was performed with 118 patients from two university hospitals in the United States and in The Netherlands. All patients were diagnosed with definite Noonan syndrome and had had an ECG and echocardiography. Sixty-nine patients (58%) had characteristic abnormalities of the ECG. In the patient group without a cardiac defect (n = 21), ten patients had a characteristic ECG abnormality. There was no statistical relationship between the presence of a characteristic ECG abnormality and the presence of a cardiac defect (p = 0.33). Patients with hypertrophic cardiomyopathy had more ECG abnormalities in total (p = 0.05), without correlation with a specific ECG abnormality. We conclude that the ECG features in patients with Noonan syndrome are characteristic for the syndrome and are not related to a specific cardiac defect. An ECG is very useful in the diagnosis of Noonan syndrome; every child with a Noonan phenotype should have an ECG and echocardiogram for evaluation.

Brain-hepato-renal syndrome (Zellweger syndrome). Report of two cases and a review of the syndrome

International Nuclear Information System (INIS)

Ruiz, T.; Caparros, C.; Blanco, A.; Lopez, A.M.

1997-01-01

Cerebro-hepato-renal syndrome is a rare disorder that is transmitted by autosomal recessive inheritance. Children with this syndrome present mongoloid facies and severe muscle hypotonic at birth. Scimitar-like knee calcifications are considered a pathognomonic feature of this disorder. We present two patients with Zellweger syndrome, according to the diagnosis suggested by our Radiodiagnostic Service. Our objective is to stress the importance of the radiological findings, which in many cases are decisive in establishing the definitive diagnosis. (Author) 10 refs

Does wastewater discharge have relations with increase of Turner syndrome and Down syndrome?

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Intae Choi

2017-08-01

Full Text Available The purpose of this study is to examine whether water and air pollutants have a relationship with an increase in the genetic disorders Turner syndrome and Down syndrome, which are caused by congenital chromosomal abnormalities, and to generate a hypothesis about the genetic health effects of environmental pollutants. A panel regression based on random effect was conducted on Korea’s metropolitan councils from 2012 to 2014. The dependent variable was the number of Turner syndrome and Down syndrome cases, and the main independent variables were those regarding the water and air pollution. Air pollutants did not have a significant impact on the number of Turner syndrome and Down syndrome cases; however, the increase in number of wastewater discharge companies did have a significant relationship with the number of cases. The more the number of wastewater discharge companies, the more the number Turner syndrome and Down syndrome cases were observed. Therefore, scientific investigation on water and air pollutants in relation with genetic health effects needs to be performed.

Skin symptoms in four ectodermal dysplasia syndromes including two case reports of Rapp-Hodgkin-Syndrome.

Science.gov (United States)

Knaudt, Björn; Volz, Thomas; Krug, Markus; Burgdorf, Walter; Röcken, Martin; Berneburg, Mark

2012-01-01

The skin, hair and nail changes in four distinct ectodermal dysplasia syndromes are compared and reviewed. These syndromes comprise Christ-Siemens-Touraine syndrome; ectrodactyly, ectodermal dysplasia and cleft lip/palate syndrome; ankyloblepharon-ectodermal defects-cleft lip/palate syndrome and Rapp-Hodgkin syndrome. A comprehensive overview of the dermatological signs and symptoms in these syndromes was generated from the database of the Ectodermal Dysplasia Network Germany, the clinical findings in the patients seen in our department and an extensive review of the literature. The findings included abnormalities of skin, sweating, hair and nails. These clinical findings are discussed in relation to the underlying molecular defects known to play a role in these four ectodermal dysplasia syndromes.

Postural tachycardia syndrome and other forms of orthostatic intolerance in Ehlers-Danlos syndrome.

Science.gov (United States)

Roma, Maria; Marden, Colleen L; De Wandele, Inge; Francomano, Clair A; Rowe, Peter C

2018-03-05

To review the association between orthostatic intolerance syndromes and both joint hypermobility and Ehlers-Danlos syndrome, and to propose reasons for identifying hereditary connective tissue disorders in those with orthostatic intolerance in the context of both clinical care and research. We searched the published peer-reviewed medical literature for papers reporting an association between joint hypermobility or Ehlers-Danlos syndrome and orthostatic intolerance. We identified 10 relevant papers. Although methodological variability between studies introduces some limitations, the published literature consistently identifies a significantly higher prevalence of orthostatic intolerance symptoms in patients with joint hypermobility or Ehlers-Danlos syndrome than in healthy controls, and a significantly higher prevalence of cardiovascular and autonomic abnormalities both at rest and during orthostatic challenge. Postural tachycardia syndrome is the most commonly recognized circulatory disorder. The severity of orthostatic symptoms in those with EDS correlates with impairments in quality of life. There is a strong association between several forms of cardiovascular dysfunction, most notably postural tachycardia syndrome, and joint hypermobility or Ehlers-Danlos syndrome. We propose that recognition of joint hypermobility and Ehlers-Danlos syndrome among those with orthostatic intolerance syndromes has the potential to improve clinical care and the validity of research findings. Copyright © 2018 Elsevier B.V. All rights reserved.

Infarto agudo do miocárdio como apresentação inicial de granulomatose eosinofílica com poliangiite (anteriormente, síndrome de Churg Strauss

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Wahinuddin Sulaiman

2014-10-01

Full Text Available A granulomatose eosinofílica com poliangiite é uma vasculite primária rara, caracterizada por hipereosinofilia, asma de surgimento tardio e granulomas eosinofílicos extravasculares. Relatamos um caso apresentado inicialmente com infarto do miocárdio e que, ulteriormente, teve prosseguimento apenas com asma, manifestações cutâneas e neuropatia periférica. Os parâmetros laboratoriais revelaram hipereosinofilia com um padrão perinuclear negativo de autoanticorpos citoplásmicos antineutrófilos (p-ANCA. A biópsia de pele demonstrou vasculite leucocitoclástica com infiltração eosinofílica, diante de uma angiografia coronária normal. Os sintomas do paciente melhoraram com metilprednisolona IV, pulsoterapia com ciclofosfamida e azatioprina.

Gardner's syndrome

International Nuclear Information System (INIS)

Sobrado Junior, C.W.; Bresser, A.; Cerri, G.G.; Habr-Gama, A.; Pinotti, H.W.; Magalhaes, A.

1988-01-01

A case of familiar poliposis of colon related to a right mandibular osteoma is reported (this association is usually called Gardner's syndrome). Radiologic pictures ae shown and some commentaries about this syndrome concerning the treatment are made. (author) [pt

Confirmation that RIPK4 mutations cause not only Bartsocas-Papas syndrome but also CHAND syndrome.

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Busa, Tiffany; Jeraiby, Mohammed; Clémenson, Alix; Manouvrier, Sylvie; Granados, Viviana; Philip, Nicole; Touraine, Renaud

2017-11-01

CHAND syndrome is an autosomal recessive disorder characterized by curly hair, ankyloblepharon, and nail dysplasia. Only few patients were reported to date. A homozygous RIPK4 mutation was recently identified by homozygosity mapping and whole exome sequencing in three patients from an expanded consanguineous kindred with a clinical diagnosis of CHAND syndrome. RIPK4 was previously known to be implicated in Bartsocas-Papas syndrome, the autosomal recessive form of popliteal pterygium syndrome. We report here two cases of RIPK4 homozygous mutations in a fetus with severe Bartsocas-Papas syndrome and a patient with CHAND syndrome. The patient with CHAND syndrome harbored the same mutation as the one identified in the family previously reported. We thus confirm the implication of RIPK4 gene in CHAND syndrome in addition to Bartsocas-Papas syndrome and discuss genotype/phenotype correlations. © 2017 Wiley Periodicals, Inc.

Drug treatment of metabolic syndrome.

Science.gov (United States)

Altabas, Velimir

2013-08-01

The metabolic syndrome is a constellation of risk factors for cardiovascular diseases including: abdominal obesity, a decreased ability to metabolize glucose (increased blood glucose levels and/or presence of insulin resistance), dyslipidemia, and hypertension. Patients who have developed this syndrome have been shown to be at an increased risk of developing cardiovascular disease and/or type 2 diabetes. Genetic factors and the environment both are important in the development of the metabolic syndrome, influencing all single components of this syndrome. The goals of therapy are to treat the underlying cause of the syndrome, to reduce morbidity, and to prevent complications, including premature death. Lifestyle modification is the preferred first-step treatment of the metabolic syndrome. There is no single effective drug treatment affecting all components of the syndrome equally known yet. However, each component of metabolic syndrome has independent goals to be achieved, so miscellaneous types of drugs are used in the treatment of this syndrome, including weight losing drugs, antidiabetics, antihypertensives, antilipemic and anticlothing drugs etc. This article provides a brief insight into contemporary drug treatment of components the metabolic syndrome.

Numerical Magnitude Processing Impairments in Genetic Syndromes: A Cross-Syndrome Comparison of Turner and 22Q11.2 Deletion Syndromes

Science.gov (United States)

Brankaer, Carmen; Ghesquière, Pol; De Wel, Anke; Swillen, Ann; De Smedt, Bert

2017-01-01

Cross-syndrome comparisons offer an important window onto understanding heterogeneity in mathematical learning disabilities or dyscalculia. The present study therefore investigated symbolic numerical magnitude processing in two genetic syndromes that are both characterized by mathematical learning disabilities: Turner syndrome and 22q11.2 deletion…

Neonatal bartter syndrome

International Nuclear Information System (INIS)

Parkash, J.; Salat, S. M.; Khan, I.A.

2006-01-01

A pre-term baby girl was born following a pregnancy complicated by severe polyhydramnios at a gestational age of 36 weeks. She was initially suffering from respiratory distress consistent with idiopathic respiratory distress syndrome, and altered electrolyte imbalance with hyponatremia, hypokalemia and hypochloremic metabolic alkalosis. However, during the third week of life when she had dehydration along with significant electrolyte imbalance, Bartter's syndrome was considered which was supported by findings of high renin and aldosterone levels. Treatment was done by correction of electrolytes and dehydration along with indomethacin. The drug was well tolerated. The infant showed correction of electrolyte imbalance. The features of this case suggest an extreme form of Bartter's syndrome presenting from the early days of life. The syndrome is reported because of it's rarity and alerts pediatricians to the antenatal and neonatal variant of Bartter's syndrome. (author)

Kallmann syndrome and ichthyosis: a case of contiguous gene deletion syndrome

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Irene Berges-Raso

2017-09-01

Full Text Available Kallmann syndrome is a genetically heterogeneous form of hypogonadotropic hypogonadism caused by gonadotropin-releasing hormone deficiency and characterized by anosmia or hyposmia due to hypoplasia of the olfactory bulbs; osteoporosis and metabolic syndrome can develop due to longstanding untreated hypogonadism. Kallmann syndrome affects 1 in 10 000 men and 1 in 50 000 women. Defects in 17 genes, including KAL1, have been implicated. Kallmann syndrome can be associated with X-linked ichthyosis, a skin disorder characterized by early onset dark, dry, irregular scales affecting the limb and trunk, caused by a defect of the steroid sulfatase gene (STS. Both KAL1 and STS are located in the Xp22.3 region; therefore, deletions in this region cause a contiguous gene syndrome. We report the case of a 32-year-old man with ichthyosis referred for evaluation of excessive height (2.07 m and weight (BMI: 29.6 kg/m2, microgenitalia and absence of secondary sex characteristics. We diagnosed Kallmann syndrome with ichthyosis due to a deletion in Xp22.3, a rare phenomenon.

Neonatal respiratory distress syndrome

Science.gov (United States)

Hyaline membrane disease (HMD); Infant respiratory distress syndrome; Respiratory distress syndrome in infants; RDS - infants ... improves slowly after that. Some infants with severe respiratory distress syndrome will die. This most often occurs ...

Dress syndrome with sepsis, acute respiratory distress syndrome and pneumomediastinum

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Prabhas Prasun Giri

2011-01-01

Full Text Available Drug rash with eosinophilia and systemic symptoms (DRESS syndrome reflects a serious hypersensitivity reaction to drugs, and is characterized by skin rash, fever, lymph node enlargement, and internal organ involvement. So far, numerous drugs such as sulfonamides, phenobarbital, sulfasalazine, carbamazepine, and phenytoin have been reported to cause DRESS syndrome. We report a case of a 10-year-old girl who developed clinical manifestations of fever, rash, lymphadenopathy, hypereosinophilia, and visceral involvement (hepatitis and pneumonitis after taking phenobarbital for seizures, with subsequent development of sepsis, acute respiratory distress syndrome (ARDS and spontaneous air leak syndrome (pnemothorax and pneumomediastinum. She was put on steroids and various antibiotics and was ventilated, but ultimately succumbed to sepsis and pulmonary complications.

MOMO Syndrome with Holoprosencephaly and Cryptorchidism: Expanding the Spectrum of the New Obesity Syndrome

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Sheetal Sharda

2011-01-01

Full Text Available There are multiple genetic disorders with known or unknown etiology grouped under obesity syndromes. Inspite of having multisystem involvement and often having a characteristic presentation, the understanding of the genetic causes in the majority of these syndromes is still lacking. The common obesity syndromes are Bardet-Biedl, Prader-Willi, Alstrom, Albright's hereditary osteodystrophy, Carpenter, Rubinstein-Taybi, Fragile X, and Börjeson-Forssman-Lehman syndrome. The list is ever increasing as new syndromes are being added to it. One of the recent additions is MOMO syndrome, with about five such cases being reported in literature. Expanding the spectrum of clinical features, we report the first case of MOMO syndrome from India with lobar variant of holoprosencephaly and cryptorchidism, which have not been reported previously.

Duane retraction syndrome type 1 with Usher syndrome type 2: an unreported association.

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Khurana, Bhawna Piplani; Khurana, Aruj Kumar; Grover, Sumit

2015-05-07

Duane retraction syndrome is characterized by globe retraction and palpebral fissure narrowing on adduction, with restriction of abduction, adduction, or both. Usher syndrome type 2 consists of congenital bilateral sensorineural hearing loss and retinitis pigmentosa. The authors present a case with a yet unreported association between Duane retraction syndrome type 1 and Usher syndrome type 2. Copyright 2015, SLACK Incorporated.

Rare case of nephrotic syndrome: Schimke syndrome.

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Pedrosa, Anna Kelly Krislane de Vasconcelos; Torres, Luiz Fernando Oliveira; Silva, Ana Corina Brainer Amorim da; Dantas, Adrianna Barros Leal; Zuntini, Káthia Liliane da Cunha Ribeiro; Aguiar, Lia Cordeiro Bastos

2016-01-01

Schimke syndrome corresponds to dysplasia of bone and immunity, associated with progressive renal disease secondary to nephrotic syndrome cortico-resistant, with possible other abnormalities such as hypothyroidism and blond marrow aplasia. It is a rare genetic disorder, with few reports in the literature. The most frequent renal involvement is nephrotic syndrome with focal segmental glomerulosclerosis and progressive renal failure. The objective of this study was to report a case of Schimke syndrome, diagnostic investigation and management of the case. Resumo A síndrome Schimke corresponde à displasia imuno-óssea, associada à doença renal progressiva secundária à síndrome nefrótica córtico-resistente, podendo haver outras anormalidades como hipotireoidismo e aplasia de medula óssea. Trata-se de uma patologia genética rara, com poucos relatos na literatura. O acometimento renal mais frequente é uma síndrome nefrótica por glomeruloesclerose segmentar e focal e falência renal progressiva. O objetivo deste estudo foi relatar um caso de síndrome de Schimke, investigação diagnóstica e condução do caso.

Gorlin-goltz syndrome

International Nuclear Information System (INIS)

Ahmed, N.; Salman, M.; Mansoor, M.A.

2007-01-01

Multiple jaw cysts are a characteristic manifestation of basal cell nevus (Gorlin) syndrome. Gorlin-Goltz syndrome is characterized by symptoms primarily involving the skin, central nervous system, and skeletal system. In 90% of the patients, nevoid basal cell carcinoma syndrome is associated with recurring odontogenic keratocysts. This patient showed recurrent jaw and maxillary cysts, for which he was followed for 2 years. (author)

Cardio-renal syndrome

OpenAIRE

Gnanaraj, Joseph; Radhakrishnan, Jai

2016-01-01

Cardio-renal syndrome is a commonly encountered problem in clinical practice. Its pathogenesis is not fully understood. The purpose of this article is to highlight the interaction between the cardiovascular system and the renal system and how their interaction results in the complex syndrome of cardio-renal dysfunction. Additionally, we outline the available therapeutic strategies to manage this complex syndrome.

Tourette Syndrome

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If you have Tourette syndrome, you make unusual movements or sounds, called tics. You have little or no control over them. Common tics are throat- ... spin, or, rarely, blurt out swear words. Tourette syndrome is a disorder of the nervous system. It ...

Lemierre's syndrome

DEFF Research Database (Denmark)

Johannesen, Katrine; Bødtger, Uffe; Heltberg, Ole

2014-01-01

Lemierre's syndrome is an often un-diagnosed disease seen in previously healthy young subjects, presenting with symptoms of pharyngitis, fever and elevated markers of inflammation. The syndrome is characterised by infectious thrombosis of the jugular vein due to infection with Fusobacteria, causing...

DIE NAAM “NEDERDUITSE GEREFORMEERDE KERK”

African Journals Online (AJOL)

P.J. Strauss. DIE NAAM “NEDERDUITSE. GEREFORMEERDE KERK”. ABSTRACT. An investigation into the name of the Dutch Reformed Church (DRC) reveals that it is a reformed church in the ... P.J. Strauss, Fakulteit Teologie, Universiteit van die Vrystaat, Suid‑Afrika. ..... Dit is so omdat die staat en sy howe die reg in die ...

Sleep overlap syndrome

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Fariba Rezaeetalab

2016-12-01

Full Text Available Overlap syndrome, which is known as the coexistence of chronic obstructive pulmonary disease (COPD and obstructive sleep apnea (OSA, was first defined by Flenley. Although it can refer to concomitant occurrence of any of the pulmonary diseases and OSA, overlap syndrome is commonly considered as the coexistence of OSA and COPD. This disease has unique adverse health consequences distinct from either condition alone. Given the high prevalence of each solitary disease, overlap syndrome is also likely to be common and clinically relevant. Despite the fact that overlap syndrome has been described in the literature for nearly 30 years, paucity of evaluations and studies limited the discussion on diagnosis, prevalence, pathophysiology, treatment, and outcomes of this disease. This review article addresses these issues by reviewing several recent studies conducted in Iran or other countries. This review suggests that overlap syndrome has worse outcomes than either disease alone. Our findings accentuated the urgent need for further studies on overlap syndrome and all overlaps between OSA and chronic pulmonary disease to provide a deeper insight into diagnosis and non-invasive treatments of this disease.

What Causes Cushing's Syndrome?

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... Share Facebook Twitter Pinterest Email Print What causes Cushing syndrome? Cushing syndrome can develop for two reasons: Medication ... uhs ), thyroid, or thymus How Tumors Can Cause Cushing Syndrome Normally, the pituitary gland in the brain controls ...

Clinicopathological study of vasculitic peripheral neuropathy

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Rong-fang DONG

2014-06-01

Full Text Available Objective To summarize the clinical features and neuropathological characteristics in patients with vasculitic peripheral neuropathy (VPN. Methods Clinical manifestations, laboratory examination and neuromuscular biopsy characteristics of 11 patients with VPN were retrospectively analyzed. The lesion of nerve, muscle and skin was observed under optical and electron microscope. Immunohistochemical analyses were carried out to detect neurofilament (NF, myelin basic protein (MBP, peripheral myelin protein 22 (PMP22 and S-100 protein (S-100 and further observing the neuropathy of neuraxon, myelin sheath and Schwann cells, and to detect human leukocyte antigen DR (HLA-DR, CD68, CD3 and CD20 to observe inflammatory cell infiltration. Immunofluorescent staining was used to detect the deposition of IgA, IgM, IgG and addiment C3 on vascular wall. The staining of periodic acid-Schiff (PAS, NADH-tetrazolium reductase (NADH-TR and modified Gomori trichrome (MGT were used to judge the myopathy. Results 1 Angiopathies were mainly manifested by small vessels of epineurium and perineurium, and infiltrated inflammatory cells were mainly CD3 + T cells. Three patients had active vasculitis, and 8 patients had non-active vasculitis. Among these 8 patients, 4 patients mainly presented fibrous obliteration of blood vessel, with slight inflammatroy cell infiltration, and the other 4 patients mainly showed perivascular inflammation. 2 Neuropathy: 6 patients had axon degeneration, and 5 patients had axon degeneration associated with demyelination. All of them demonstrated a reduction in myelinated fibers, mainly large diameter myelinated fibers, even on end-stage. 3 Muscle biopsy showed neurogenic atrophy. 4 Clinicopathologic diagnosis: among these 11 patients, 8 patients were diagnosed as systemic vasculitic peripheral neuropathy (SVPN, among whom 5 patients were diagnosed as primary systemic vasculitis [including 1 patient as Churg-Strauss syndrome (CSS, 2 patients as

[The refeeding syndrome].

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Lambers, Wietske M; Kraaijenbrink, Bastiaan; Siegert, Carl E H

2015-01-01

The refeeding syndrome may occur during reintroduction of carbohydrates in malnourished patients. This syndrome is characterized by reduced plasma electrolyte levels, hypophosphataemia being most prevalent. The symptoms can vary from minor symptoms to severe neurological or cardiac symptoms. The pathophysiological mechanism comprises an increase in insulin levels, resulting in shifts of phosphate, potassium and magnesium into the intracellular environment, as well as fluid retention and relative deficiency of vitamin B1. There is growing interest in the screening and treatment of patients with malnutrition, due to which the incidence of refeeding syndrome is probably increasing. Currently, there is no single definition of this syndrome and therefore there is no solid scientific basis for screening and treatment. In this article we describe the rationale for screening and additional laboratory investigations. A prospective, controlled trial is important to define the clinical relevance of the refeeding syndrome and optimize its treatment.

Subclinical nephritic syndrome in children cohabiting with pediatric patients, Presenting acute nephritic syndrome

OpenAIRE

Guerrero-Tinoco Gustavo Adolfo; Julio-Barrios Emil

2012-01-01

Introduction: subclinical nephritic syndrome is the presence of hematuria, hypocomplementemiaand/or proteinuria without the presence of signs and/or symptoms.Objective: to determine the incidence of subclinical nephritic syndrome in childrenliving with pediatric patients diagnosed with acute nephritic syndrome.Methods: family visit to identify children living together in the two previous months, with pediatric patients hospitalized with acute nephritic syndrome, at Hospital InfantilNapoleon F...

Relationships among personality traits, metabolic syndrome, and metabolic syndrome scores: The Kakegawa cohort study.

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Ohseto, Hisashi; Ishikuro, Mami; Kikuya, Masahiro; Obara, Taku; Igarashi, Yuko; Takahashi, Satomi; Kikuchi, Daisuke; Shigihara, Michiko; Yamanaka, Chizuru; Miyashita, Masako; Mizuno, Satoshi; Nagai, Masato; Matsubara, Hiroko; Sato, Yuki; Metoki, Hirohito; Tachibana, Hirofumi; Maeda-Yamamoto, Mari; Kuriyama, Shinichi

2018-04-01

Metabolic syndrome and the presence of metabolic syndrome components are risk factors for cardiovascular disease (CVD). However, the association between personality traits and metabolic syndrome remains controversial, and few studies have been conducted in East Asian populations. We measured personality traits using the Japanese version of the Eysenck Personality Questionnaire (Revised Short Form) and five metabolic syndrome components-elevated waist circumference, elevated triglycerides, reduced high-density lipoprotein cholesterol, elevated blood pressure, and elevated fasting glucose-in 1322 participants aged 51.1±12.7years old from Kakegawa city, Japan. Metabolic syndrome score (MS score) was defined as the number of metabolic syndrome components present, and metabolic syndrome as having the MS score of 3 or higher. We performed multiple logistic regression analyses to examine the relationship between personality traits and metabolic syndrome components and multiple regression analyses to examine the relationship between personality traits and MS scores adjusted for age, sex, education, income, smoking status, alcohol use, and family history of CVD and diabetes mellitus. We also examine the relationship between personality traits and metabolic syndrome presence by multiple logistic regression analyses. "Extraversion" scores were higher in those with metabolic syndrome components (elevated waist circumference: P=0.001; elevated triglycerides: P=0.01; elevated blood pressure: P=0.004; elevated fasting glucose: P=0.002). "Extraversion" was associated with the MS score (coefficient=0.12, P=0.0003). No personality trait was significantly associated with the presence of metabolic syndrome. Higher "extraversion" scores were related to higher MS scores, but no personality trait was significantly associated with the presence of metabolic syndrome. Copyright © 2018 Elsevier Inc. All rights reserved.

SNEDDON’S SYNDROME

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Valentin Valtchev

2008-10-01

Full Text Available Sneddon’s syndrome is usually characterized by the association of an ischemic cerebrovascular disease and a widespread livedo reticularis. The incidence of Sneddon syndrome is 4/1000 000. We present 42-year-old woman with livedo reticularis, recurrence ischaemic cerebrovascular accidents, two repetitive miscarriages and positive anti-2GPi antibodies. Skin biopsy specimens reveal inflammatory changes of small- to medium-sized arteries and subendothelial proliferation and fibrosis. The diagnosis Sneddon syndrome is confirmed by skin biopsy, and MR evidence. We suggest that anti-2GPi antibodies may be pathophysiologically related to the clinical manifestation observed in some patients with Sneddon syndrome.

Sjogren′s Syndrome: A Review

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Rani Somani

2011-01-01

Full Text Available Sjogren′s syndrome, also known as "Mikulicz disease" or "Sicca syndrome" is a systemic autoimmune disease in which immune cells attack and destroy the exocrine glands that produce tears and saliva. It can exist by itself (primary Sjogren syndrome or develop in association with another disorder such as rheumatoid arthritis, systemic sclerosis, primary biliary cirrhosis or Hashimoto thyroiditis (associated Sjogren syndrome. Hallmarks are the dry mouth and dry eyes known as the Sicca syndrome. Sjogren syndrome affects t million to 4 million people in the United States- Most are over 40 years old at the time of diagnosis. As there is no known cure for Sjogren syndrome, treatment focuses on relieving symptoms and preventing complications. The most serious complication associated with primary Sjogren syndrome is the development of a lymphoproliferative disease. primarily non-Hodgkin lymphoma.

The ``eco-syndrome`` and what causes it; Das Oeko-Syndrom`` und seine Ausloeser

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Voack, C. [Technische Univ. Muenchen (Germany). Klinik und Poliklinik fuer Dermatolologie und Allergologie; Borelli, S. [Technische Univ. Muenchen (Germany). Klinik und Poliklinik fuer Dermatolologie und Allergologie; Ring, J. [Technische Univ. Muenchen (Germany). Klinik und Poliklinik fuer Dermatolologie und Allergologie

1997-01-24

Headache, tiredness, skin irritation etc. may be the results of adverse effects on health due to toxic substances in residential buildings and at workplaces. Possible causes of syndromes that are hard to objectify but associated with serious afflictions such as the ``eco-syndrome`` or Multiple Chemical Sensitivity (MCS) and the Sick Building Syndrome (SBS) are listed. (VHE) [Deutsch] Bei Kopfschmerzen, Muedigkeit, Hautirritationen u.ae. besteht die Moeglichkeit einer gesundheitlichen Beeintraechtigung durch Schadstoff im Wohn- oder Arbeitsbereich. Moegliche Ausloeser fuer schwer objektivierbare, aber mit hohem Leidensdruck verbundene Krankheitsbilder wie Oeko-Syndrom(Multiple Chemical Sensivity, MCS) und SBS (Sick-Building-Syndrom) werden aufgelistet. (VHE)

[Williams-Beuren syndrome (Williams syndrome). Case report].

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Miklós, Györgyi; Fekete, György; Haltrich, Irén; Tóth, Miklós; Reismann, Péter

2017-11-01

Williams syndrome is a rare genetic disorder, that occurs equally in all ethnic groups and both sexes. The diagnosis might be missed during childhood in mild cases. However, establishing the diagnosis is important, not only to find the cause of intellectual disability but to look for cardiovascular, endocrine, psychiatry, urology and other conditions, which can occur at any age in the patients' lifetime. This case report presents the story of 47-year-old woman, who was admitted with haematemesis. During her stay on the ward, in the light of the distinctive facial features, mental retardation, and social behaviour patterns, the possibility of Williams syndrome emerged. Later, the diagnosis was confirmed by genetic analysis. This female is the oldest living patient with Williams syndrome in Hungary. Orv Hetil. 2017; 158(47): 1883-1888.

Rorschach Inkblot Method data at baseline and after 2 years treatment of consecutively admitted patients with first-episode schizophrenia

DEFF Research Database (Denmark)

Rosenbaum, Bent; Andersen, Palle Bent; Knudsen, Per Bjerregaard

2012-01-01

Background: The Rorschach Inkblot Method is regarded as an important clinical instrument for detailed diagnostic description of the integrative capacities of individuals in psychotic states and as an instrument for measuring progression in the course of treatment. Aims: To describe relevant...... Rorschach variables at baseline in a group of consecutively admitted patients with first-episode schizophrenia. Furthermore, to describe the changes in these variables from baseline to year 2 for the group of patients given psychiatric standard treatment, and to compare these changes with changes in other...... outcome measures [Positive and Negative Syndrome Scale (PANSS), Global Assessment of Functioning (GAF), Strauss-Carpenter and socio-demographic variables]. Methods: In a prospective study, 34 patients consecutively admitted to treatment for a first episode of schizophrenia were tested using Exner...

The association between the metabolic syndrome and metabolic syndrome score and pulmonary function in non-smoking adults.

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Yoon, Hyun; Gi, Mi Young; Cha, Ju Ae; Yoo, Chan Uk; Park, Sang Muk

2018-03-01

This study assessed the association of metabolic syndrome and metabolic syndrome score with the predicted forced vital capacity and predicted forced expiratory volume in 1 s (predicted forced expiratory volume in 1 s) values in Korean non-smoking adults. We analysed data obtained from 6684 adults during the 2013-2015 Korean National Health and Nutrition Examination Survey. After adjustment for related variables, metabolic syndrome ( p metabolic syndrome score ( p metabolic syndrome score with metabolic syndrome score 0 as a reference group showed no significance for metabolic syndrome score 1 [1.061 (95% confidence interval, 0.755-1.490)] and metabolic syndrome score 2 [1.247 (95% confidence interval, 0.890-1.747)], but showed significant for metabolic syndrome score 3 [1.433 (95% confidence interval, 1.010-2.033)] and metabolic syndrome score ⩾ 4 [1.760 (95% confidence interval, 1.216-2.550)]. In addition, the odds ratio of restrictive pulmonary disease of the metabolic syndrome [1.360 (95% confidence interval, 1.118-1.655)] was significantly higher than those of non-metabolic syndrome. Metabolic syndrome and metabolic syndrome score were inversely associated with the predicted forced vital capacity and forced expiratory volume in 1 s values in Korean non-smoking adults. In addition, metabolic syndrome and metabolic syndrome score were positively associated with the restrictive pulmonary disease.