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

Index Scriptorium Estoniae

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

Clinical presentation of Churg-Strauss syndrome in children. : A 12-year-old-boy with ANCA-negative Churg-Strauss syndrome.

NARCIS (Netherlands)

F.G.E.M. Razenberg (Femke); J.W.C.M. Heynens (Jan); G. Jan de Vries (Geeuwke); L. Duijts (Liesbeth); J.C. de Jongste (Johan); J. de Blic (Jacques); P.P.R. Rosias (Philippe)

2012-01-01

textabstractChurg-Strauss syndrome is an uncommon multisystem disorder characterized by asthma, eosinophilia and vasculitis. We report on a 12-year-old boy with asthma and deterioration of his general condition, who was eventually diagnosed with an ANCA-negative Churg-Strauss syndrome. The

Churg-Strauss syndrome associated with montelukast therapy

Science.gov (United States)

Tuggey, J; Hosker, H

2000-01-01

Churg-Strauss syndrome is a rare form of eosinophilic vasculitis associated with asthma. There have been several recent case reports of the condition in association with leukotriene antagonists and it has been speculated that the Churg-Strauss syndrome was unmasked when oral corticosteroids were withdrawn. We report a case of Churg-Strauss syndrome associated with montelukast therapy in an asthmatic patient in whom there had been no recent oral corticosteroid use. We believe that this is the first such reported case and would suggest that clinicians need to be vigilant in all patients who develop systemic symptoms when starting treatment with leukotriene antagonists.

 PMID:10950903

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

Directory of Open Access Journals (Sweden)

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.

Wells syndrome and its relationship to Churg-Strauss syndrome.

Science.gov (United States)

Ratzinger, Gudrun; Zankl, Julia; Zelger, Bernhard

2013-08-01

  Wells syndrome has been described as an inflammatory disorder based on typical clinical appearance combined with the histopathological presence of eosinophilic infiltrates and flame figures in the absence of vasculitis. Churg-Strauss syndrome, on the other hand, is primarily a diffuse, necrotizing vasculitis but is also typically displaying eosinophils and flame figures. Despite several parallels, the present understanding of these two diseases excludes any pathogenetic relationship.   We describe the clinical course and histopathological appearance of three patients who had initially been diagnosed with Wells syndrome that developed into Churg-Strauss syndrome during the course of their disease.   The clinical presentation of all three patients led to the diagnosis of Wells syndrome by independent specialists. Histopathology showed an eosinophilic infiltrate and flame figures next to features of leukocytoclastic vasculitis. Detailed examination revealed asthma bronchiale and additional symptoms indicating Churg-Strauss syndrome. The initial diagnosis of Wells syndrome had to be revised to Churg-Strauss syndrome.   We conclude that Wells syndrome could be the starting point of a pathogenetic process that might reach its maximum in Churg-Strauss syndrome. As a clinical consequence, patients with Wells syndrome should be evaluated and followed for Churg-Strauss syndrome. © 2013 The International Society of Dermatology.

[Sir William Richard Gowers: author of the "bible of neurology"].

Science.gov (United States)

Hirose, Genjiro

2014-11-01

William Richard Gowers is one of the great pioneers in neurology and the author of the well-known neurology textbook, "A Manual of Diseases of the Nervous System." His concepts of neurology are based on meticulously and carefully accumulated knowledge of history, observations, and neurological examinations of patients with various neurological diseases. He is not only a great neurologist but also a great teacher who loves teaching students and physicians through well-prepared lectures. We can glean the essence of the field of neurology through his life story and numerous writings concerning neurological diseases.

Síndrome de Churg Strauss

Directory of Open Access Journals (Sweden)

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.

[Cardiac tamponade and myocarditis in Churg-Strauss syndrome].

Science.gov (United States)

Baili, L; Aydi, Z; Soussi, G; Ben Dhaou B, B; Zidi, A; Berraies, A; Boussema, F; Kammoun, S; Hamzaoui, A; Kraiem, S; Ben Miled M'rad, K; Rokbani, L

2014-09-01

The successive occurrence of pericardial tamponade and myocarditis during a Churg-Strauss syndrome is exceptionally described. We report a patient in whom pericardial tamponade and myocarditis were the presenting manifestation of a Churg-Strauss syndrome. A 58-year-old woman was admitted because of alteration of the clinical status with eosinophilia. One month ago, she was hospitalized for a pericardial tamponade treated by pericardial drainage. Acute myocarditis was diagnosed on chest pain during the second hospitalization. The etiologic inquiry ended in the diagnosis of Churg-Strauss complicated with a double cardiac involvement. A good response of clinical and biological anomalies was obtained after corticosteroid and immunosuppressive treatment. Isolated or multiple involvements of cardiac tunics should lead to make diagnosis of systemic vasculitis. A complete initial assessment and a close observation of the patients followed for Churg-Strauss syndrome is imperative to detect a cardiac achievement and set up an early treatment. Copyright © 2013 Elsevier Masson SAS. All rights reserved.

As duas naturezas de Lévi-Strauss

Directory of Open Access Journals (Sweden)

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.

Churg-strauss syndrome

Directory of Open Access Journals (Sweden)

Brar B

2009-01-01

Full Text Available We report a case of 29-year-old man who presented with cutaneous vasculitis and was subsequently diagnosed as a case of Churg-Strauss syndrome. The patient fulfilled five out of the six criteria of the syndrome developed by American College of Rheumatology.

Churg-Strauss syndrome: a case with unusual manifestations

International Nuclear Information System (INIS)

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

2008-01-01

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.

Levi-Strauss : UNESCO = anthropology : politics

Directory of Open Access Journals (Sweden)

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".

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

Energy Technology Data Exchange (ETDEWEB)

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.

Cases of Churg-Strauss syndrome in patients receiving montelukast

Directory of Open Access Journals (Sweden)

Petrović Jelena

2012-01-01

Full Text Available Churg-Strauss syndrome is a rare disorder, but in patients with asthma it may develop as an adverse effect of the administered drugs. The aim of this study was to investigate possible causal relationship between montelukast and the occurrence of Churg-Strauss syndrome. Medical literature was reviewed by searching the databases 'Medline' and 'Googlescholar', in order to detect published cases of Churg-Strauss syndrome associated with use of montelukast. In this article is included 13 publications which contain the following keywords: montelukast, Churg-Strauss syndrome and side effects. Relationship between use of montelukast and development of Churg-Strauss syndrome was not clearly causal, although montelukast was associated with development and relapse of the syndrome. This fact supports the hypothesis that leukotriene antagonists are involved in the pathogenesis of this serious disease. Special attention should be paid to appearance of new symptoms in an asthmatic patient, already treated with corticosteroids, who start receiving leukotriene antagonists, especially if the dose of corticosteroids is reduced. Definitive confirmation or rejection of the hypothesis that leukotriene antagonists are directly involved in the development of this syndrome require further investigations.

A Case of Churg-Strauss Syndrome Associated with Antiphospholipid Antibodies

Science.gov (United States)

Ferenczi, Katalin; Chang, Timothy; Camouse, Melissa; Han, Rujing; Stern, Robert; Willis, Joseph; Cooper, Kevin D.; Gilliam, Anita C.

2008-01-01

BACKGROUND Churg-Strauss syndrome (CSS) is a systemic vasculitis affecting small and medium-sized blood vessels, almost invariably affecting the lung and frequently associated with cutaneous involvement. Microvascular vaso-occlusion leading to digital gangrene is not a feature of CSS. OBSERVATIONS We report an unusual case of a patient with Churg Strauss Syndrome with antiphospholipid antibodies who developed severe digital gangrene in addition to cutaneous vasculitis. CONCLUSION The presence of antiphospholipid antibodies is not a feature usually seen in association with Churg-Strauss syndrome. While the full clinical spectrum of Churg Strauss Syndrome is still being defined, identification of additional features associated with this syndrome might help to better understand the pathogenesis of the disease and to have an impact on management and prognosis. PMID:17175066

Small bowel involvement documented by capsule endoscopy in Churg-Strauss syndrome.

Science.gov (United States)

Beye, Birane; Lesur, Gilles; Claude, Pierre; Martzolf, Lionel; Kieffer, Pierre; Sondag, Daniel

2015-01-01

Churg-Strauss syndrome is a small and medium vessel vasculitis and is also known as allergic granulomatous angiitis. Gastrointestinal involvement is common in patients with Churg-Strauss syndrome (20-50%). The most common symptoms are abdominal pain, diarrhoea and occasionally gastrointestinal bleeding and perforation. We present a case of Churg-Strauss syndrome with small bowel lesions documented by video capsule endoscopy.

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.

Churg–Strauss Syndrome Presenting with Endobronchial Masses

Directory of Open Access Journals (Sweden)

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

Síndrome de Churg-Strauss: casuística

Directory of Open Access Journals (Sweden)

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

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.

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

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

Science.gov (United States)

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.

[Mastitis revealing Churg-Strauss syndrome].

Science.gov (United States)

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.

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

Directory of Open Access Journals (Sweden)

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.

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

Directory of Open Access Journals (Sweden)

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

Churg–Strauss syndrome: Case series

Directory of Open Access Journals (Sweden)

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

[Parotid involvement in Churg-Strauss syndrome].

Science.gov (United States)

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.

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

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

Directory of Open Access Journals (Sweden)

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.

Churg-Strauss syndrome from an orthopaedic perspective.

Science.gov (United States)

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.

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

Science.gov (United States)

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 concomitant with chronic symmetrical dacryoadenitis suggesting Mikulicz's disease.

Science.gov (United States)

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.

75 FR 39910 - Prince William Sound Resource Advisory Committee; Meeting

Science.gov (United States)

2010-07-13

... DEPARTMENT OF AGRICULTURE Forest Service Prince William Sound Resource Advisory Committee; Meeting AGENCY: Forest Service, USDA. ACTION: Notice of meeting. SUMMARY: The Prince William Sound Resource..., Anchorage, Alaska 99503. Send written comments to Prince William Sound Resource Advisory Committee, c/o USDA...

Anosmia and hypogeusia in Churg-Strauss syndrome.

Science.gov (United States)

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.

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

Energy Technology Data Exchange (ETDEWEB)

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

International Nuclear Information System (INIS)

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.)

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 associated with montelukast: Three Case Reports

Directory of Open Access Journals (Sweden)

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

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

Science.gov (United States)

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: a case report.

Science.gov (United States)

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.

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

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

DEFF Research Database (Denmark)

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

2006-01-01

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...

Giant coronary aneurysm in Churg-Strauss syndrome

NARCIS (Netherlands)

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

Batik Kreatif Amri Yahya dalam Perspektif Strukturalisme Levi-Strauss

Directory of Open Access Journals (Sweden)

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...

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

Directory of Open Access Journals (Sweden)

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

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"

An Abdominal Presentation of Churg-Strauss Syndrome

Science.gov (United States)

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

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

Science.gov (United States)

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.

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

Science.gov (United States)

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

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

Science.gov (United States)

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.

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

Directory of Open Access Journals (Sweden)

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.

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

Directory of Open Access Journals (Sweden)

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.

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

Churg-Strauss syndrome and hemorragic vasculitis

Directory of Open Access Journals (Sweden)

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

Science.gov (United States)

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

Welcoming a New Generation to College: The Millennial Students

Science.gov (United States)

Elam, Carol; Stratton, Terry; Gibson, Denise D.

2007-01-01

High on any required reading list for college-level student affairs officers and high school counselors is "Millennials Rising: The Next Great Generation" by Neil Howe and William Strauss (2000). Experts on generational change in the United States, the authors contend that the current generation of college-age and pre-college-age…

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

Science.gov (United States)

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…

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

Science.gov (United States)

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.

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

Science.gov (United States)

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.

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

Directory of Open Access Journals (Sweden)

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.

Acute coronary syndrome associated with Churg-Strauss syndrome

Directory of Open Access Journals (Sweden)

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

Grounded Theory and Pragmatism: The Curious Case of Anselm Strauss

Directory of Open Access Journals (Sweden)

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

Index Scriptorium Estoniae

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

ANCA-negative Churg-Strauss Syndrome

Directory of Open Access Journals (Sweden)

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.

Churg-Strauss syndrome

Directory of Open Access Journals (Sweden)

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.

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

Science.gov (United States)

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.

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 ...

The Churg-Strauss syndrome: An unusual presentation

Directory of Open Access Journals (Sweden)

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

Science.gov (United States)

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.

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

Directory of Open Access Journals (Sweden)

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.

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

Science.gov (United States)

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

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

Directory of Open Access Journals (Sweden)

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.

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

Science.gov (United States)

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'.

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

Directory of Open Access Journals (Sweden)

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.

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 ...

A new case of keratoconus associated with Williams-Beuren syndrome.

Science.gov (United States)

Viana, Melissa Machado; Frasson, Maria; Leão, Letícia Lima; Stofanko, Martin; Gonçalves-Dornelas, Higgor; Cunha, Pricila da Silva; de Aguiar, Marcos José Burle

2013-09-01

Williams-Beuren syndrome is a multisystemic genetic disorder caused by a contiguous gene deletion at 7q11.23. Keratoconus is a complex disease and it is suspected to have a genetic origin, although the specific gene responsible for keratoconus has not been identified. Although there are several ocular features in Williams-Beuren syndrome, keratoconus is not regularly described as part of this syndrome. To report a new patient with keratoconus and Williams-Beuren syndrome. This is the third case of an association between Williams-Beuren syndrome and keratoconus. The authors believe that the Williams-Beuren syndrome chromosome region can be a possible target for further investigation as the genetic basis of keratoconus.

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.

Attention to Faces in Williams Syndrome

Science.gov (United States)

Riby, Deborah M.; Jones, Nicola; Brown, Philippa H.; Robinson, Lucy J.; Langton, Stephen R. H.; Bruce, Vicki; Riby, Leigh M.

2011-01-01

Williams syndrome (WS) is associated with distinct social behaviours. One component of the WS social phenotype is atypically prolonged face fixation. This behaviour co-exists with attention difficulties. Attention is multi-faceted and may impact on gaze behaviour in several ways. Four experiments assessed (i) attention capture by faces, (ii)…

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 ...

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

Science.gov (United States)

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…

Northern gas: Williams petrochemical feasibility study

Energy Technology Data Exchange (ETDEWEB)

Chappell, D. [Williams Energy Canada, Calgary, AB (Canada)

2002-07-01

Williams Energy is a company that is involved in the following fields: gas pipelines, exploration and production, midstream, refining, petrochemical, power, and marketing and trading. The author provides an overview of the global and Canadian infrastructure before proceeding to discuss Arctic gas, which is viewed by Williams Energy as necessary. It favors the Alaska Highway route with a consortium for project development. Williams performed a petrochemical study to determine the feasibility of a petrochemical complex utilizing natural gas liquid from an Arctic gas pipeline. The scope of the study encompassed facilities (extraction plant, cracker, polyolefins plant), size (world scale, approximately 2 billion pounds per year), and location (Fairbanks or Alberta). The study led to the following findings: (1) review of several scenarios for both locations, (2) complex to produce two grades of polyethylene, (3) feedstock cost favors Alaska, (4) construction costs lower in Alberta, (5) and the primary market for the Alaska complex would be northeast Asia, while the primary market for the Alberta location would be the United States. It was determined that both options would be viable, however Alberta was favored due to the polyethylene forecast. The challenges still being faced by Williams include low frac spread, market inefficiencies, empress volumes, carbon dioxide, and fuel and electricity cost. Each of these challenges is discussed separately. The author concludes by indicating that incremental ethane is available, carbon dioxide issues need resolution, and Alberta and Alaska are attractive for Arctic ethane petrochemical production. figs.

An Authorization Logic with Explicit Time

Science.gov (United States)

2008-02-02

that η-logic can be used in specifying the behavior of systems with time-dependent authorization policies. In such cases, the logic can be used to...10(4):265– 310, November 1992. [26] Christopher Lesniewski-Laas, Bryan Ford, Jacob Strauss, M. Frans Kaashoek, and Robert Morris. Alpaca : extensible

N-acetylcysteine for neuropsychiatric symptoms in a woman with Williams syndrome.

Science.gov (United States)

Pineiro, Mildred Lopez; Roberts, Antoinette M; Waxler, Jessica L; Mullett, Jennifer E; Pober, Barbara R; McDougle, Christopher J

2014-11-01

Williams syndrome is a relatively rare genetic disorder caused by the hemizygous microdeletion of a region in chromosome 7q11.23. Individuals with Williams syndrome typically present with a highly social, overfriendly, and empathic personality. Comorbid medical and neuropsychiatric disorders are common. Reports of effective pharmacological treatment of associated neuropsychiatric disorders are limited. The authors describe the successful treatment of interfering anger, aggression, and hair-pulling with N-acetylcysteine in a 19-year-old woman with Williams syndrome. The neuropsychiatric symptoms emerged 1 week following an upper gastrointestinal endoscopy, for which fentanyl, midazolam, and propofol were used as anesthetics. The patient's treatment course and hypothesized mechanisms underlying the clinical presentation and symptom resolution are described. © The Author(s) 2014.

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

NARCIS (Netherlands)

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

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

Science.gov (United States)

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

The Influence of Johannes Fabian on Claude Lévi -Strauss

Directory of Open Access Journals (Sweden)

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.

Alternative solution model for the ternary carbonate system CaCO3 - MgCO3 - FeCO3 - I. A ternary Bragg-Williams ordering model

Science.gov (United States)

McSwiggen, P.L.

1993-01-01

The minerals of the ternary carbonate system CaCO3 - MgCO3 - FeCO3 represent a complex series of solid solutions and ordering states. An understanding of those complexities requires a solution model that can both duplicate the subsolidus phase relationships and generate correct values for the activities. Such a solution model must account for the changes in the total energy of the system resulting from a change in the ordering state of the individual constituents. Various ordering models have been applied to binary carbonate systems, but no attempts have previously been made to model the ordering in the ternary system. This study derives a new set of equations that allow for the equilibrium degree of order to be calculated for a system involving three cations mixing on two sites, as in the case of the ternary carbonates. The method is based on the Bragg-Williams approach. From the degree of order, the mole fractions of the three cations in each of the two sites can be determined. Once the site occupancies have been established, a Margules-type mixing model can be used to determine the free energy of mixing in the solid solution and therefore the activities of the various components. ?? 1993 Springer-Verlag.

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 ...

Investigating the ''social brain'' through Williams syndrome

International Nuclear Information System (INIS)

Nagamine, Masanori; Mimura, Masaru; Reiss, A.L.; Hoeft, F.

2010-01-01

Recent advances in social cognitive neuroscience have led to the concept of the ''social brain''. The social brain includes neural processes specialized for processing social information necessary for the recognition of self and others, and interpersonal relationships. Because of its unique behavioral phenotypic features which includes 'hypersociability', Williams syndrome has gained popularity among social cognitive neuroscientists. Individuals with Williams syndrome share the same genetic risk factor for cognitive-behavioral dysfunction utilizing brain imaging to elucidate endophenotype provides us with an unprecendented opportunity to study gene, brain and behavior relationships especially those related to social cognition. In this review, we provide an overview of neuroimaging studies on social cognition in Williams syndrome and discuss the neural basis of the social brain. (author)

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...

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

Directory of Open Access Journals (Sweden)

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.

Churg-strauss syndrome

Directory of Open Access Journals (Sweden)

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.

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

Directory of Open Access Journals (Sweden)

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.

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

Directory of Open Access Journals (Sweden)

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.

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

International Nuclear Information System (INIS)

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

Energy Technology Data Exchange (ETDEWEB)

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.)

An Abdominal Presentation of Churg-Strauss Syndrome

Directory of Open Access Journals (Sweden)

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.

Louis Althusser, Leo Strauss, and Democratic Leadership

OpenAIRE

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...

Narrative self-constitution and vulnerability to co-authoring.

Science.gov (United States)

McConnell, Doug

2016-02-01

All people are vulnerable to having their self-concepts shaped by others. This article investigates that vulnerability using a theory of narrative self-constitution. According to narrative self-constitution, people depend on others to develop and maintain skills of self-narration and they are vulnerable to having the content of their self-narratives co-authored by others. This theoretical framework highlights how vulnerability to co-authoring is essential to developing a self-narrative and, thus, the possibility of autonomy. However, this vulnerability equally entails that co-authors can undermine autonomy by contributing disvalued content to the agent's self-narrative and undermining her authorial skills. I illustrate these processes with the first-hand reports of several women who survived sexual abuse as children. Their narratives of survival and healing reveal the challenges involved in (re)developing the skills required to manage vulnerability to co-authoring and how others can help in this process. Finally, I discuss some of the implications of co-authoring for the healthcare professional and the therapeutic relationship.

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

Directory of Open Access Journals (Sweden)

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

33 CFR 167.1702 - In Prince William Sound: Prince William Sound Traffic Separation Scheme.

Science.gov (United States)

2010-07-01

... 33 Navigation and Navigable Waters 2 2010-07-01 2010-07-01 false In Prince William Sound: Prince William Sound Traffic Separation Scheme. 167.1702 Section 167.1702 Navigation and Navigable Waters COAST....1702 In Prince William Sound: Prince William Sound Traffic Separation Scheme. The Prince William Sound...

Books authored/co-authored and edited/co-edited by members of staff of the Department of Medieval/Medieval and Renaissance Archaeology, Aarhus University, 1971-2014

DEFF Research Database (Denmark)

Roesdahl, Else

2015-01-01

Chronologically organized list of books authored/co-authored and edited/co-edited by members of staff of the Department of Medieval and Renaissance Archaeology, Aarhus University, 1971-2014......Chronologically organized list of books authored/co-authored and edited/co-edited by members of staff of the Department of Medieval and Renaissance Archaeology, Aarhus University, 1971-2014...

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

Directory of Open Access Journals (Sweden)

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.

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

Science.gov (United States)

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.

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

Directory of Open Access Journals (Sweden)

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

Directory of Open Access Journals (Sweden)

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.

[Williams-Beuren syndrome (Williams syndrome). Case report].

Science.gov (United States)

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.

[Update Churg-Strauss syndrome].

Science.gov (United States)

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 masquerading as an acute coronary syndrome.

Science.gov (United States)

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.

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

Index Scriptorium Estoniae

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"

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

Science.gov (United States)

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.

Usefulness of cardiac resonance imaging in Churg-Strauss syndrome.

Science.gov (United States)

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.

Dental management of patient with Williams Syndrome - A case report.

Science.gov (United States)

Wong, Daniel; Ramachandra, Srinivas Sulugodu; Singh, Ashish Kumar

2015-01-01

Williams syndrome is a multisystemic rare genetic disorder caused by deletion of 26-28 genes in the long arm of chromosome 7. It is characterized by developmental and physical abnormalities including congenital cardiovascular abnormalities, mental retardation, neurological features, growth deficiency, genitourinary manifestations, gastrointestinal problems, musculoskeletal problems, unique behavioral characteristics, and dental problems. Dental abnormalities include malocclusion, hypodontia, malformed teeth, taurodontism, pulp stones, increased space between teeth, enamel hypoplasia, and high prevalence of dental caries. Authors report a 17-year-old female patient with underlying Williams syndrome. Oral features and problems seen in the patient are listed. Malocclusion and screwdriver shaped teeth were noticed. Generalized widening of the periodontal ligament space with vital teeth was seen. This finding has not been reported in cases of Williams syndrome earlier. Precautions taken during dental treatment in patients with Williams syndrome are also discussed.

Churg-Strauss Syndrome following PTU Treatment

Directory of Open Access Journals (Sweden)

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.

A new method to construct co-author networks

Science.gov (United States)

Liu, Jie; Li, Yunpeng; Ruan, Zichan; Fu, Guangyuan; Chen, Xiaowu; Sadiq, Rehan; Deng, Yong

2015-02-01

In this paper, we propose a new method to evaluate the importance of nodes in a given network. The proposed method is based on the PageRank algorithm. However, we have made necessary improvements to combine the importance of the node itself and that of its community status. First, we propose an improved method to better evaluate the real impact of a paper. The proposed method calibrates the real influence of a paper over time. Then we propose a scheme of evaluating the contribution of each author in a paper. We later develop a new method to combine the information of the author itself and the structure of the co-author network. We use the number of co-authorship to calculate the effective distance between two authors, and evaluate the strength of their influence to each other with the law of gravity. The strength of influence is used to build a new network of authors, which is a comprehensive topological representation of both the quality of the node and its role in network. Finally, we apply our method to the Erdos co-author community and AMiner Citation Network to identify the most influential authors.

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

International Nuclear Information System (INIS)

Jung, Kyung Hwa; Hwang, Mi Soo; Kim, Sun Yong; Chang, Jae Chun; Park, Bok Hwan

1988-01-01

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

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

Directory of Open Access Journals (Sweden)

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.

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.

Lévi-Strauss no Brasil: a formação do etnólogo

Directory of Open Access Journals (Sweden)

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.

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

Directory of Open Access Journals (Sweden)

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.

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

Science.gov (United States)

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.

Familial Churg-Strauss Syndrome in a Sister and Brother.

Science.gov (United States)

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.

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

OpenAIRE

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 ...

Readings on the Tragedies of William Shakespeare. The Greenhaven Press Literary Companion to British Authors.

Science.gov (United States)

Swisher, Clarice, Ed.

Designed for young adults, this book on William Shakespeare's tragedies is one of an anthology series providing accessible resources for students researching great literary lives and works. Contributing writers' essays in the book are taken from a wide variety of sources and are edited to accommodate the reading and comprehension levels of young…

Churg-Strauss syndrome: A rare cause of pleural effusion

Directory of Open Access Journals (Sweden)

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.

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

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

NARCIS (Netherlands)

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

Afinidades seletivas: uma comparação entre as sociobiologias da literatura de Pierre Bourdieu e Raymond Williams Selective kinships: comparing the literarture sociologies od Pierre Bourdieu and Raymond Williams

Directory of Open Access Journals (Sweden)

Enio Passiani

2009-10-01

Full Text Available As sociologias da literatura de Bourdieu e Williams estão apoiadas em dois conceitos fundamentais: “habitus” e “estruturas de sentimento”, respectivamente. E é clara a semelhança entre eles: ambos tentam traduzir uma espécie de consciência prática adquirida pelos agentes sociais a partir de um processo particular de socialização. Para o primeiro, o “habitus” é internalizado na experiência vivida num campo específi co; ao passo que, para o segundo, tal consciência é formada no interior dos grupos culturais. Nesse sentido, ambas as noções dizem respeito a um conjunto de valores compartilhados que aproximam e afastam certos agentes uns dos outros, criando solidariedade e rivalidades. Esta comunicação pretende demonstrar que, apesar das diferenças que existem, os conceitos dos autores não supervalorizam nem a reprodução nem a mudança social, mas, ao contrário, tentam explicar esses fenômenos como jogo dialético. Palavras-chave: Pierre Bourdieu. Raymond Williams. Sociologia da literatura. “Habitus”. Estruturas de sentimento. The literature sociologies of Bourdieu and Williams are structured in two fundamental concepts: “habitus” and “sentiment structures”, respectively. It’s very clear the resemblance between them: both try to translate a kind of practical conscience, acquired by the social agents from a particular socializing process. To the fi rst, the “habitus” is internalized in the living experience of a specifi c fi eld; while that to the second that conscience is formed in the interior of cultural groups. In this sense, both ideas talk about shared values which approximate and withdraw some agents one from the others, creating solidarity and competition. This text intends to show that, despite of the existing differences, the authors concepts don’t overestimate neither the reproduction, nor the social change, but, otherwise, try to explain these phenomena as a dialectic game. Keywords

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.

Raymond Williams and local cultures

OpenAIRE

B Longhurst

1991-01-01

In this paper it is maintained that Raymond Williams's writings on culture are of great importance to current developments in cultural geography. His work is periodised into three stages and its different subject matters identified. An interpretation of Williams's theory of culture is offered which places particular emphasis on his concepts of 'structure of feeling' and 'knowable community'. The creative tension between Williams's holistic treatment of culture and his stress on cultural strug...

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

Energy Technology Data Exchange (ETDEWEB)

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

1988-06-15

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

Third Semiannual Report to the Congress by the United States Atomic Energy Commission, February 2, 1948

Energy Technology Data Exchange (ETDEWEB)

Lilienthal, David E.; Bacher, Robert F.; Pike, Sumner T.; Strauss, Lewis L.; Waymack, William W.

1948-02-02

The document includes the letter of submittal and the third semiannual report. These reports are called for pursuant to Section 17 of the Atomic Energy Act of 1946. The letter of submittal was titled ''Letter from the Chairman and Members of the United States Atomic Energy Commission transmitting pursuant to law the third semiannual report of the Atomic Energy Commission''. It was authored by David E. Lilienthal, Chairman, and Robert F. Bacher, Sumner T. Pike, Lewis L. Strauss, and William W. Waymack of the AEC.

Crescentic glomerulonephritis in non-asthmatic Churg-Strauss syndrome.

Science.gov (United States)

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.

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

Science.gov (United States)

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.

Genetics Home Reference: Williams syndrome

Science.gov (United States)

... do well on tasks that involve spoken language, music, and learning by repetition (rote memorization). Affected individuals ... Resources (5 links) Disease InfoSearch: Williams syndrome Genetic Science Learning Center, University of Utah MalaCards: williams-beuren ...

Ética e profética nas Mitológicas de Lévi-Strauss

Directory of Open Access Journals (Sweden)

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".

William Butler Yeats’s ‘The Symbolic System’ of William Blake

Directory of Open Access Journals (Sweden)

Arianna Antonielli

2008-03-01

Full Text Available The theosophical systems formulated by great poets, such as William Blake and William Butler Yeats, represent a personal idiosyncratic actualization of an ancient repertoire of magical symbols and occult visions. This study wants to focus the attention on the philosophical, mythical, and esoteric syncretism that W. B. Yeats drew from William Blake’s symbolical system. A fundamental step of Yeats’s deep investigation into the Blakean ‘vision’ was given by his monumental work, written together with Edwin John Ellis, on Blake’s poetic and pictorial production, completed in 1893 with a three-volume edition entitled The Works of William Blake, Poetic, Symbolic, and Critical. This work, published in London by Bernard Quaritch, deeply influenced Yeats’s symbolical and imaginary system, determining its subsequent development up to its codification in the volume of A Vision. With WWB, Yeats was able to systematize for the first time his own thought, giving unity to his Weltanschauung and his poetry. Following this hypothesis, I concentrated on Yeats’s and Ellis’s numerous analyses dedicated to Blake’s mythological and symbolical corpus and, in particular, I examined the last chapter of the first volume of the Quaritch edition. This chapter, entitled “The Symbolic System”, constitutes an unquestionable link between Yeats the reader and scholar of Blake, and Yeats the poet and follower of Blake.

Crescentic glomerulonephritis in non-asthmatic Churg-Strauss syndrome

Directory of Open Access Journals (Sweden)

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.

CO-AUTHORING SCIENTIFIC PAPERS IN MANAGEMENT: INTERNATIONAL ACADEMICS’ POINTS OF VIEW

Directory of Open Access Journals (Sweden)

Manuel Aníbal Silva Portugal Vasconcelos Ferreira

2015-12-01

Full Text Available Responding to institutional pressure to publish more papers, and in respected journals, management academics are seeking to increase their productivity by establishing co-authoring ties that speed up the process, from the initial stages of defining the research question to publication in a journal. Understanding the motives, benefits and hazards of co-authorship, especially using the experiences of highly reputed scholars, may help improve the efficiency and quality of research efforts. In this paper, we examine the responses to six questions sent to thirteen prolific international researchers, by email. In essence, we sought to understand such aspects as: the origins of co-authoring ties, the importance of having co-authors, the pitfalls of and methods for coordinating co-authorships, and what constitutes a contribution that warrants co-authorship. Results show that (1 international researchers evaluate their co-authorships positively, (2 many co-authorships emerge from prior supervision or personal proximity, more so than ties to the same institution, (3 the ordering of names is defined a priori and managed actively, (4 the benefits sought after are mainly specialization and complementary competences, (5 co-authorship is given based on actual contribution, albeit there are many forms of contribution, and (6 the pressure to publish, and an increasing difficulty of publishing in the top journals are the drivers of co-authorship. The analyses point to important insights for young Brazilian scholars.

A human neurodevelopmental model for Williams syndrome.

Science.gov (United States)

Chailangkarn, Thanathom; Trujillo, Cleber A; Freitas, Beatriz C; Hrvoj-Mihic, Branka; Herai, Roberto H; Yu, Diana X; Brown, Timothy T; Marchetto, Maria C; Bardy, Cedric; McHenry, Lauren; Stefanacci, Lisa; Järvinen, Anna; Searcy, Yvonne M; DeWitt, Michelle; Wong, Wenny; Lai, Philip; Ard, M Colin; Hanson, Kari L; Romero, Sarah; Jacobs, Bob; Dale, Anders M; Dai, Li; Korenberg, Julie R; Gage, Fred H; Bellugi, Ursula; Halgren, Eric; Semendeferi, Katerina; Muotri, Alysson R

2016-08-18

Williams syndrome is a genetic neurodevelopmental disorder characterized by an uncommon hypersociability and a mosaic of retained and compromised linguistic and cognitive abilities. Nearly all clinically diagnosed individuals with Williams syndrome lack precisely the same set of genes, with breakpoints in chromosome band 7q11.23 (refs 1-5). The contribution of specific genes to the neuroanatomical and functional alterations, leading to behavioural pathologies in humans, remains largely unexplored. Here we investigate neural progenitor cells and cortical neurons derived from Williams syndrome and typically developing induced pluripotent stem cells. Neural progenitor cells in Williams syndrome have an increased doubling time and apoptosis compared with typically developing neural progenitor cells. Using an individual with atypical Williams syndrome, we narrowed this cellular phenotype to a single gene candidate, frizzled 9 (FZD9). At the neuronal stage, layer V/VI cortical neurons derived from Williams syndrome were characterized by longer total dendrites, increased numbers of spines and synapses, aberrant calcium oscillation and altered network connectivity. Morphometric alterations observed in neurons from Williams syndrome were validated after Golgi staining of post-mortem layer V/VI cortical neurons. This model of human induced pluripotent stem cells fills the current knowledge gap in the cellular biology of Williams syndrome and could lead to further insights into the molecular mechanism underlying the disorder and the human social brain.

Author Details

African Journals Online (AJOL)

Visemith, William Muffee. Vol 10, No 1 (2001) - Articles Determining the Optimum Level of Working Capital in the Cameroon Business Environment Abstract. ISSN: 0856-6372. AJOL African Journals Online. HOW TO USE AJOL... for Researchers · for Librarians · for Authors · FAQ's · More about AJOL · AJOL's Partners ...

Generalized Bragg-Williams method for 'antiferromagnetic' lattice gases

International Nuclear Information System (INIS)

Osorio, R.

1983-01-01

The many-sublattice Bragg-Williams approximation of statistical mechanics is applied to the two-dimensional square and triangular lattice-gas models with nearest-neighbor repulsive interactions. Each problem is solved through both the canonical and grand-canonical methods. The present treatment emphasizes the duality between concentration and chemical potential and illustrates the appearance of first- and second -order transitions in each method. (Author) [pt

Trouble in Paradise: Problems in Academic Research Co-authoring.

Science.gov (United States)

Bozeman, Barry; Youtie, Jan

2016-12-01

Scholars and policy-makers have expressed concerns about the crediting of coauthors in research publications. Most such problems fall into one of two categories, excluding deserving contributors or including undeserving ones. But our research shows that there is no consensus on "deserving" or on what type of contribution suffices for co-authorship award. Our study uses qualitative data, including interviews with 60 US academic science or engineering researchers in 14 disciplines in a set of geographically distributed research-intensive universities. We also employ data from 161 website posts provided by 93 study participants, again US academic scientists. We examine a variety of factors related to perceived unwarranted exclusion from co-author credit and unwarranted inclusion, providing an empirically-informed conceptual model to explain co-author crediting outcomes. Determinants of outcomes include characteristics of disciplines and fields, institutional work culture, power dynamics and team-specific norms and decision processes.

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

Directory of Open Access Journals (Sweden)

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

Iterative Authoring Using Story Generation Feedback: Debugging or Co-creation?

Science.gov (United States)

Swartjes, Ivo; Theune, Mariët

We explore the role that story generation feedback may play within the creative process of interactive story authoring. While such feedback is often used as 'debugging' information, we explore here a 'co-creation' view, in which the outcome of the story generator influences authorial intent. We illustrate an iterative authoring approach in which each iteration consists of idea generation, implementation and simulation. We find that the tension between authorial intent and the partially uncontrollable story generation outcome may be relieved by taking such a co-creation approach.

Handedness and corpus callosal morphology in Williams syndrome.

Science.gov (United States)

Martens, Marilee A; Wilson, Sarah J; Chen, Jian; Wood, Amanda G; Reutens, David C

2013-02-01

Williams syndrome is a neurodevelopmental genetic disorder caused by a hemizygous deletion on chromosome 7q11.23, resulting in atypical brain structure and function, including abnormal morphology of the corpus callosum. An influence of handedness on the size of the corpus callosum has been observed in studies of typical individuals, but handedness has not been taken into account in studies of callosal morphology in Williams syndrome. We hypothesized that callosal area is smaller and the size of the splenium and isthmus is reduced in individuals with Williams syndrome compared to healthy controls, and examined age, sex, and handedness effects on corpus callosal area. Structural magnetic resonance imaging scans were obtained on 25 individuals with Williams syndrome (18 right-handed, 7 left-handed) and 25 matched controls. We found that callosal thickness was significantly reduced in the splenium of Williams syndrome individuals compared to controls. We also found novel evidence that the callosal area was smaller in left-handed participants with Williams syndrome than their right-handed counterparts, with opposite findings observed in the control group. This novel finding may be associated with LIM-kinase hemizygosity, a characteristic of Williams syndrome. The findings may have significant clinical implications in future explorations of the Williams syndrome cognitive phenotype.

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

Directory of Open Access Journals (Sweden)

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.

Brief Report: Major Depressive Disorder with Psychotic Features in Williams Syndrome--A Case Series

Science.gov (United States)

Valdes, Francisca; Keary, Christopher J.; Mullett, Jennifer E.; Palumbo, Michelle L.; Waxler, Jessica L.; Pober, Barbara R.; McDougle, Christopher J.

2018-01-01

Descriptions of individuals with Williams syndrome (WS) and co-morbid major depressive disorder (MDD) with psychotic features have not appeared in the literature. In addition to reviewing previous reports of psychotic symptoms in persons with WS, this paper introduces clinical histories and therapeutic management strategies for three previously…

33 CFR 167.1700 - In Prince William Sound: General.

Science.gov (United States)

2010-07-01

... 33 Navigation and Navigable Waters 2 2010-07-01 2010-07-01 false In Prince William Sound: General... Schemes and Precautionary Areas Pacific West Coast § 167.1700 In Prince William Sound: General. The Prince William Sound Traffic Separation Scheme consists of four parts: Prince William Sound Traffic Separation...

Author Details

African Journals Online (AJOL)

Williams, C. Vol 17, No 2 (2013) - Articles The influence of service brand equity on the strength of brand relationships in the fast food industry. Abstract PDF. ISSN: 1998-8125. AJOL African Journals Online. HOW TO USE AJOL... for Researchers · for Librarians · for Authors · FAQ's · More about AJOL · AJOL's Partners ...

Co-Authoring: A Natural Form of Cooperative Learning.

Science.gov (United States)

Chapman, Ann D.; And Others

1992-01-01

Presents a method of improving student term papers that employs an approach requiring students to co-author a paper with other students in a group. Describes the four key elements of successful cooperative learning. Provides details about how this approach can be carried out. (HB)

Cutting edge issues in the Churg-Strauss syndrome.

Science.gov (United States)

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.

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

2014-02-01

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.

I Know! It's Backwards Day! Gender Roles and William's Doll

Science.gov (United States)

Van de Kleut, Geraldine

2007-01-01

This article presents a case study of an exploration of gender roles in a second-grade classroom. The author discusses some of the discursive identities in which she and her students are positioned, and then uses the picture book William's Doll to introduce a discussion of discursive gender identities with her students. She then asks students to…

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

Science.gov (United States)

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.

Infrared astronomy seeing the heat : from William Herschel to the Herschel space observatory

CERN Document Server

Clements, David L

2014-01-01

Uncover the Secrets of the Universe Hidden at Wavelengths beyond Our Optical GazeWilliam Herschel's discovery of infrared light in 1800 led to the development of astronomy at wavelengths other than the optical. Infrared Astronomy - Seeing the Heat: from William Herschel to the Herschel Space Observatory explores the work in astronomy that relies on observations in the infrared. Author David L. Clements, a distinguished academic and science fiction writer, delves into how the universe works, from the planets in our own Solar System to the universe as a whole. The book first presents the major t

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

NARCIS (Netherlands)

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

William Rowan Hamilton: Mathematical genius

International Nuclear Information System (INIS)

Wilkins, D.R.

2006-01-01

This year Ireland celebrates the bicentenary of the mathematician William Rowan Hamilton, best remembered for quaternions and for his pioneering work on optics and dynamics. Two centuries after his birth, the extent to which terms such as Hamiltonian and Hamiltonian system have entered the everyday language of mathematicians and physicists testifies to the continuing impact of the scientific work of William Rowan Hamilton. (U.K.)

Churg-Strauss syndrome: A case report

Directory of Open Access Journals (Sweden)

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.

Science.gov (United States)

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.

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

Directory of Open Access Journals (Sweden)

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.

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

Science.gov (United States)

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.

Science.gov (United States)

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 without respiratory symptoms in a child

Directory of Open Access Journals (Sweden)

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.

William Rowan Hamilton: Mathematical genius

Energy Technology Data Exchange (ETDEWEB)

Wilkins, D.R. [School of Mathematics, Trinity College, Dublin (Ireland)]. E-mail: dwilkins@maths.tcd.ie

2005-08-01

This year Ireland celebrates the bicentenary of the mathematician William Rowan Hamilton, best remembered for 'quaternions' and for his pioneering work on optics and dynamics. Two centuries after his birth, the extent to which terms such as 'Hamiltonian' and 'Hamiltonian system' have entered the everyday language of mathematicians and physicists testifies to the continuing impact of the scientific work of William Rowan Hamilton. (U.K.)

33 CFR 110.233 - Prince William Sound, Alaska.

Science.gov (United States)

2010-07-01

... 33 Navigation and Navigable Waters 1 2010-07-01 2010-07-01 false Prince William Sound, Alaska. 110... ANCHORAGES ANCHORAGE REGULATIONS Anchorage Grounds § 110.233 Prince William Sound, Alaska. (a) The anchorage grounds. In Prince William Sound, Alaska, beginning at a point at latitude 60°40′00″ N., longitude 146°40...

Williams Syndrome with a “Twist”

Directory of Open Access Journals (Sweden)

Despoina Maritsi

2010-01-01

Full Text Available Williams syndrome is a rare genetic condition with multisystemic involvement, caused by a microscopic deletion in the chromosome band 7q11.23. We describe the first case of a toddler with Williams syndrome who developed Benign Paroxysmal Torticollis (BPT, a benign dystonic disorder of unknown aetiology.

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.

Science.gov (United States)

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.

76 FR 1130 - Prince William Sound Resource Advisory Committee

Science.gov (United States)

2011-01-07

... DEPARTMENT OF AGRICULTURE Forest Service Prince William Sound Resource Advisory Committee AGENCY: Forest Service, USDA. ACTION: Notice of meeting. SUMMARY: The Prince William Sound Resource Advisory... conducted: The Prince William Sound Resource Advisory Committee (RAC) will be discussing and voting on...

77 FR 45331 - Prince William Sound Resource Advisory Committee

Science.gov (United States)

2012-07-31

... DEPARTMENT OF AGRICULTURE Forest Service Prince William Sound Resource Advisory Committee AGENCY: Forest Service, USDA. ACTION: Notice of meeting. SUMMARY: The Prince William Sound Resource Advisory... Prince William Sound Resource Advisory Committee (RAC) will be discussing and voting on proposals that...

Ending Caleb Williams: on storytelling influencing William Godwin’s politics

Directory of Open Access Journals (Sweden)

Peterson Roberto da Silva

2017-12-01

Full Text Available William Godwin escreveu o romance gótico Things as They Are; or, the Adventures of Caleb Williams (“As Coisas como Elas São; ou, as Aventuras de Caleb Williams”, em tradução livre em 1794 para disseminar suas ideias filosóficas e políticas. Inicio o artigo revisando a literatura para demonstrar como sua teoria influenciou tanto sua decisão de escrever ficções quanto o tipo de ficção que ele escreveu, mostrando como o autor usou e inovou convenções do gênero gótico para transmitir suas ideias para um público mais amplo. Então comparo duas perspectivas divergentes na literatura quanto à maneira como suas narrativas influenciaram suas ideias políticas e filosóficas, em particular a partir de comentários acerca do significado e do impacto de mudanças de última hora ao desfecho de Caleb Williams. Argumento que essa relação ambígua entre narrativa e política na obra de Godwin é relevante para reflexões políticas contemporâneas sobre a relação entre representação e ação política, ou como narrativas sobre a condição humana e realidades políticas podem afetas as ideias, atitudes e relações sociais de seus narradores.

The Impact of Personality on History: An Interview with William L. Shirer.

Science.gov (United States)

Social Education, 1983

1983-01-01

William L. Shirer, author of Rise and Fall of the Third Reich, discusses a wide variety of subjects, including the personalities of Roosevelt and Hitler, why the Germans succumbed to Nazism, McCarthyism in the United States, and the heroic resistance of the Russians to German invasion. (CS)

76 FR 18715 - Prince William Sound Resource Advisory Committee

Science.gov (United States)

2011-04-05

... DEPARTMENT OF AGRICULTURE Forest Service Prince William Sound Resource Advisory Committee AGENCY: Forest Service, USDA. ACTION: Notice of meeting. SUMMARY: The Prince William Sound Resource Advisory... meeting is open to the public. The following business will be conducted: The Prince William Sound Resource...

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

Directory of Open Access Journals (Sweden)

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.

Williams propylene upgrading

Energy Technology Data Exchange (ETDEWEB)

Chappell, D. [Williams Energy Canada Inc., Edmonton, AB (Canada)

2004-07-01

Edmonton-based Williams Energy Canada Inc. extracts petrochemicals from oil sands and operates a straddle plant business and an olefins business. This presentation provided an update of both businesses and reviewed the advantage of polypropylene production in Alberta, with reference to premium markets and to comparative rail costs to Chicago via Texas, and rail costs to Chicago from Alberta. Williams' straddle plant business includes the Cochrane Straddle Plant, the Empress 2 Straddle Plant, and the Empress 5 Straddle Plant. The Fort McMurray Extraction Plant was also described along with the Redwater Olefins Fractionator and its potential for salt cavern storage and distribution. It was noted that Alberta is well positioned for polypropylene production because it already has a secure supply and an excellent distribution network. tabs., figs.

Williams propylene upgrading

International Nuclear Information System (INIS)

Chappell, D.

2004-01-01

Edmonton-based Williams Energy Canada Inc. extracts petrochemicals from oil sands and operates a straddle plant business and an olefins business. This presentation provided an update of both businesses and reviewed the advantage of polypropylene production in Alberta, with reference to premium markets and to comparative rail costs to Chicago via Texas, and rail costs to Chicago from Alberta. Williams' straddle plant business includes the Cochrane Straddle Plant, the Empress 2 Straddle Plant, and the Empress 5 Straddle Plant. The Fort McMurray Extraction Plant was also described along with the Redwater Olefins Fractionator and its potential for salt cavern storage and distribution. It was noted that Alberta is well positioned for polypropylene production because it already has a secure supply and an excellent distribution network. tabs., figs

Williams-Beuren's Syndrome: A Case Report.

Science.gov (United States)

Zamani, Hassan; Babazadeh, Kazem; Fattahi, Saeid; Mokhtari-Esbuie, Farzad

2012-01-01

Williams-Beuren syndrome is a rare familial multisystem disorder occurring in 1 per 20,000 live births. It is characterized by congenital heart defects (CHD), skeletal and renal anomalies, cognitive disorder, social personality disorder and dysmorphic facies. We present a case of Williams syndrome that presented to us with heart murmur and cognitive problem. A 5-year-old girl referred to pediatric cardiologist because of heart murmurs. She had a systolic murmur (2-3/6) in right upper sternal border with radiation to right cervical region. She also had a bulge forehead. Angiography showed mild supra valvular aortic stenosis and mild multiple peripheral pulmonary stenosis. Fluorescent in situ hybridization (FISH) was performed and the result was: 46.XX, ish del (7q11.2) (ELN X1) (7q22 X2) ELN deletion compatible with Williams syndrome. Peripheral pulmonary artery stenosis is associated with Noonan syndrome, Alagille syndrome, Cutis laxa, Ehler-Danlos syndrome, and Silver-Russel syndrome. The patient had peripheral pulmonary artery stenosis, but no other signs of these syndromes were present, and also she had a supravalvular aortic stenosis which was not seen in other syndromes except Williams syndrome. Conclusion. According to primary symptoms, paraclinical and clinical finding such as dysmorphic facies, cognitive disorder and congenital heart defect, Williams syndrome was the first diagnosis. We suggest a more attention for evaluating heart murmur in childhood period, especially when the patient has abnormal facial features or mental problem.

Williams-Beuren's Syndrome: A Case Report

Directory of Open Access Journals (Sweden)

Hassan Zamani

2012-01-01

Full Text Available Williams-Beuren syndrome is a rare familial multisystem disorder occurring in 1 per 20,000 live births. It is characterized by congenital heart defects (CHD, skeletal and renal anomalies, cognitive disorder, social personality disorder and dysmorphic facies. We present a case of Williams syndrome that presented to us with heart murmur and cognitive problem. A 5-year-old girl referred to pediatric cardiologist because of heart murmurs. She had a systolic murmur (2-3/6 in right upper sternal border with radiation to right cervical region. She also had a bulge forehead. Angiography showed mild supra valvular aortic stenosis and mild multiple peripheral pulmonary stenosis. Fluorescent in situ hybridization (FISH was performed and the result was: 46.XX, ish del (7q11.2 (ELN X1 (7q22 X2 ELN deletion compatible with Williams syndrome. Peripheral pulmonary artery stenosis is associated with Noonan syndrome, Alagille syndrome, Cutis laxa, Ehler-Danlos syndrome, and Silver-Russel syndrome. The patient had peripheral pulmonary artery stenosis, but no other signs of these syndromes were present, and also she had a supravalvular aortic stenosis which was not seen in other syndromes except Williams syndrome. Conclusion. According to primary symptoms, paraclinical and clinical finding such as dysmorphic facies, cognitive disorder and congenital heart defect, Williams syndrome was the first diagnosis. We suggest a more attention for evaluating heart murmur in childhood period, especially when the patient has abnormal facial features or mental problem.

Revolution and Exploration: the English Translations of Rousseau and Humboldt by Helen Maria Williams

Directory of Open Access Journals (Sweden)

Paula Yurss Lasanta

2017-12-01

Full Text Available British author Helen Maria Williams (1759-1827 was a well-known figure in the eighteenth century literary circles, whose work was praised by Elizabeth Montagu, Samuel Johnson, Dorothy and William Wordsworth, Mary Wollstonecraft, Hester Piozzi or Alexander von Humboldt.  In her early poems  Edwin and Eltruda (1782, An Ode to the Peace (1783 and Peru (1784, Williams starts to reveal her political tendencies by appealing to strong empathic feelings as a key to social and political transformation. As a result of her interest in politics, she travelled to France in 1790 and published her most acclaimed work Letters from France (1790. However, the rest of her production has received little critical attention by modern scholars, who have overlooked her involvement in translation. Williams’ only extant novel, Julia (1790 is in fact a creative translation of Rousseau’s Julie ou La Nouvelle Héloïse (1761, in which Williams includes poems that evidence her interest in revolutionary politics. Four years later, she translated Bernardin de Saint Pierre’s Paul et Virginie, while she was imprisoned in Paris. While translating novels was regarded as a respectable exercise for women writers, Williams challenges gender assumptions by translating Researches (1814 and the seven volumes of Personal Narrative (1814-1829, which had been produced by one of the most influential eighteenth century scientists, Alexander von Humboldt. This article interrogates how Williams makes use of translation to access areas of knowledge traditionally restricted to men, such as philosophy, politics and science. For this purpose, I will focus on her translations of the work of two leading intellectual figures of the eighteenth century,  Rousseau and Von Humboldt.

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"

Directory of Open Access Journals (Sweden)

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 ...

William Bagley versus Arthur Bestor: Why the Standard Story Is Not True

Science.gov (United States)

Null, J. Wesley

2008-01-01

This essay challenges the conventional understanding of William Bagley and Arthur Bestor, which suggests that they held similar views in curriculum and teacher education. The author thinks this view is completely wrong and provides a radical new interpretation of Bagley and Bestor that uncovers a lost tradition within the field of education.…

A comparative study of first and all-author co-citation counting, and two different matrix generation approaches applied for author co-citation analyses

DEFF Research Database (Denmark)

Schneider, Jesper Wiborg; Larsen, Birger; Ingwersen, Peter

2009-01-01

XML documents extracted from the IEEE collection. These data allow the construction of ad-hoc citation indexes, which enables us to carry out the hitherto largest all-author co-citation study. Four ACA are made, combining the different units of analyses with the different matrix generation approaches...

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

Energy Technology Data Exchange (ETDEWEB)

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

The Next Great Generation?

Science.gov (United States)

Brownstein, Andrew

2000-01-01

Discusses ideas from a new book, "Millennials Rising: The Next Great Generation," (by Neil Howe and William Strauss) suggesting that youth culture is on the cusp of a radical shift with the generation beginning with this year's college freshmen who are typically team oriented, optimistic, and poised for greatness on a global scale. Includes a…

William Wilde: Historian.

Science.gov (United States)

Geary, L

2016-05-01

This essay attempts to assess William Wilde as a social historian. It examines some of his contributions to the discipline of history and looks particularly at 'The food of the Irish', which was published in the Dublin University Magazine in February 1854.

Language and Communicative Development in Williams Syndrome

Science.gov (United States)

Mervis, Carolyn B.; Becerra, Angela M.

2007-01-01

Williams syndrome, a genetic disorder caused by a microdeletion of approximately 25 genes on chromosome 7q11.23, is associated with mild to moderate intellectual disability or learning difficulties. Most individuals with Williams syndrome evidence a cognitive profile including relative strengths in verbal short-term memory and language, and…

WE-G-213-02: The AAPM Award Eponyms: William D. Coolidge, Edith H. Quimby, and Marvin M.D. Williams - Who Were They and What Did They Do?

Energy Technology Data Exchange (ETDEWEB)

Rothenberg, L. [Memorial Sloan-Kettering Cancer Center (United States)

2015-06-15

was hired by Giacchino Failla as a radiation physicist at Memorial Hospital for Cancer in New York City. Failla had studied with Madame Curie and obtained his doctoral degree in her laboratory. After many groundbreaking medical physics studies from 1919 until 1942, they both moved to Columbia University. Dr. Quimby developed a widely employed dosimetry system for single plane implants with radium and radon seeds, and a dosimetry methodology for internal radionuclides. She was author of more than 75 scientific publications, and of significant textbooks including the first comprehensive physics textbook for radiologists “Physical Foundations of Radiology”, which was co-authored with Otto Glasser, Lauriston Taylor and James Weatherwax in the first edition, with Russell Morgan added for the second edition and Paul Goodwin for the fourth edition. With Sergei Feitelberg, M.D. she published two editions of “Radioactive Isotopes in Medicine and Biology: Basic Physics and Instrumentation”. Quimby became a renowned examiner for the American Board of Radiology when the third ABR examination, given in 1936, added physics. She served as President of the American Radium Society, received the RSNA Gold Medal, and also numerous prestigious awards given to women in science. Edith Quimby was a Charter Member of AAPM. The AAPM Lifetime Achievement Award was renamed the Edith H. Quimby Lifetime Achievement Award in her honor in 2011. Marvin Martin Dixon Williams (1902–1981) Marvin Williams was born in Walla Walla, WA in 1902, and attended the same college as Edith Quimby, graduating from Whitman College in 1926. He was greatly influenced to go into medical physics by her accomplishments. During his early career, Williams worked with James Weatherwax in Philadelphia while he was working toward an M.S. from the University of Pennsylvania. In 1931 Williams was awarded a Ph.D. in Biophysics from the University of Minnesota, with the work actually performed at the Mayo Clinic

WE-G-213-02: The AAPM Award Eponyms: William D. Coolidge, Edith H. Quimby, and Marvin M.D. Williams - Who Were They and What Did They Do?

International Nuclear Information System (INIS)

Rothenberg, L.

2015-01-01

was hired by Giacchino Failla as a radiation physicist at Memorial Hospital for Cancer in New York City. Failla had studied with Madame Curie and obtained his doctoral degree in her laboratory. After many groundbreaking medical physics studies from 1919 until 1942, they both moved to Columbia University. Dr. Quimby developed a widely employed dosimetry system for single plane implants with radium and radon seeds, and a dosimetry methodology for internal radionuclides. She was author of more than 75 scientific publications, and of significant textbooks including the first comprehensive physics textbook for radiologists “Physical Foundations of Radiology”, which was co-authored with Otto Glasser, Lauriston Taylor and James Weatherwax in the first edition, with Russell Morgan added for the second edition and Paul Goodwin for the fourth edition. With Sergei Feitelberg, M.D. she published two editions of “Radioactive Isotopes in Medicine and Biology: Basic Physics and Instrumentation”. Quimby became a renowned examiner for the American Board of Radiology when the third ABR examination, given in 1936, added physics. She served as President of the American Radium Society, received the RSNA Gold Medal, and also numerous prestigious awards given to women in science. Edith Quimby was a Charter Member of AAPM. The AAPM Lifetime Achievement Award was renamed the Edith H. Quimby Lifetime Achievement Award in her honor in 2011. Marvin Martin Dixon Williams (1902–1981) Marvin Williams was born in Walla Walla, WA in 1902, and attended the same college as Edith Quimby, graduating from Whitman College in 1926. He was greatly influenced to go into medical physics by her accomplishments. During his early career, Williams worked with James Weatherwax in Philadelphia while he was working toward an M.S. from the University of Pennsylvania. In 1931 Williams was awarded a Ph.D. in Biophysics from the University of Minnesota, with the work actually performed at the Mayo Clinic

Social Cognition in Williams Syndrome: Face Tuning.

Science.gov (United States)

Pavlova, Marina A; Heiz, Julie; Sokolov, Alexander N; Barisnikov, Koviljka

2016-01-01

Many neurological, neurodevelopmental, neuropsychiatric, and psychosomatic disorders are characterized by impairments in visual social cognition, body language reading, and facial assessment of a social counterpart. Yet a wealth of research indicates that individuals with Williams syndrome exhibit remarkable concern for social stimuli and face fascination. Here individuals with Williams syndrome were presented with a set of Face-n-Food images composed of food ingredients and in different degree resembling a face (slightly bordering on the Giuseppe Arcimboldo style). The primary advantage of these images is that single components do not explicitly trigger face-specific processing, whereas in face images commonly used for investigating face perception (such as photographs or depictions), the mere occurrence of typical cues already implicates face presence. In a spontaneous recognition task, participants were shown a set of images in a predetermined order from the least to most resembling a face. Strikingly, individuals with Williams syndrome exhibited profound deficits in recognition of the Face-n-Food images as a face: they did not report seeing a face on the images, which typically developing controls effortlessly recognized as a face, and gave overall fewer face responses. This suggests atypical face tuning in Williams syndrome. The outcome is discussed in the light of a general pattern of social cognition in Williams syndrome and brain mechanisms underpinning face processing.

Facilitating Co-Authoring: Reflections of Content and Language Lecturers

Science.gov (United States)

Wright, J.

2010-01-01

During a content and language project at a University of Technology (UoT) in Cape Town, South Africa, pairs of language and content lecturers, whose broad definition of integration was "the provision of linguistic access to content knowledge", co-authored ten integrated textbooks. Their intention was to assist first year learners with…

Epistemologia pragmatyczna Michaela Williamsa (PRAGMATIST EPISTEMOLOGY BY MICHAEL WILLIAMS

Directory of Open Access Journals (Sweden)

Renata Ziemińska

2007-06-01

Full Text Available The article presents three main elements of Williams' epistemology: the concept of knowledge, the problem of skepticism and the concept of truth. Williams takes knowledge not as pure descriptive but partly normative concept (to know is to be engaged and entitled. He rejects the demonstrative conception of knowledge (knowledge is infallible and prefers the fallibilist conception of knowledge (knowledge is uncertain and fallible. Williams is good at bringing skeptical presuppositions to light: the demonstrative conception of knowledge and the conception of justification with Prior Grounding Requirement, epistemological realism and priority for internal knowledge. He rightly observes that when we change that presuppositions (skeptic's context, knowledge does exist. However, Williams-fallibilist is close to a skeptic: they both agree that our beliefs are uncertain. The difference is only whether some of our beliefs deserve to be called knowledge. The most important worries concern Williams' concept of truth (deflationary pragmatism. According to Williams truth has no nature and it is not a goal of inquiry. However, if truth is not a goal, we can hardly understand the previous discussion with skepticism and the defense of rationality.

33 CFR 161.60 - Vessel Traffic Service Prince William Sound.

Science.gov (United States)

2010-07-01

... William Sound. 161.60 Section 161.60 Navigation and Navigable Waters COAST GUARD, DEPARTMENT OF HOMELAND... Movement Reporting System Areas and Reporting Points § 161.60 Vessel Traffic Service Prince William Sound... Cape Hinchinbrook Light to Schooner Rock Light, comprising that portion of Prince William Sound between...

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

Science.gov (United States)

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.

Skin findings in Williams syndrome.

Science.gov (United States)

Kozel, Beth A; Bayliss, Susan J; Berk, David R; Waxler, Jessica L; Knutsen, Russell H; Danback, Joshua R; Pober, Barbara R

2014-09-01

Previous examination in a small number of individuals with Williams syndrome (also referred to as Williams-Beuren syndrome) has shown subtly softer skin and reduced deposition of elastin, an elastic matrix protein important in tissue recoil. No quantitative information about skin elasticity in individuals with Williams syndrome is available; nor has there been a complete report of dermatologic findings in this population. To fill this knowledge gap, 94 patients with Williams syndrome aged 7-50 years were recruited as part of the skin and vascular elasticity (WS-SAVE) study. They underwent either a clinical dermatologic assessment by trained dermatologists (2010 WSA family meeting) or measurement of biomechanical properties of the skin with the DermaLab™ suction cup (2012 WSA family meeting). Clinical assessment confirmed that soft skin is common in this population (83%), as is premature graying of the hair (80% of those 20 years or older), while wrinkles (92%), and abnormal scarring (33%) were detected in larger than expected proportions. Biomechanical studies detected statistically significant differences in dP (the pressure required to lift the skin), dT (the time required to raise the skin through a prescribed gradient), VE (viscoelasticity), and E (Young's modulus) relative to matched controls. The RT (retraction time) also trended longer but was not significant. The biomechanical differences noted in these patients did not correlate with the presence of vascular defects also attributable to elastin insufficiency (vascular stiffness, hypertension, and arterial stenosis) suggesting the presence of tissue specific modifiers that modulate the impact of elastin insufficiency in each tissue. © 2014 Wiley Periodicals, Inc.

Discoverers of the universe William and Caroline Herschel

CERN Document Server

Hoskin, Michael

2011-01-01

Discoverers of the Universe tells the gripping story of William Herschel, the brilliant, fiercely ambitious, emotionally complex musician and composer who became court astronomer to Britain's King George III, and of William's sister, Caroline, who assisted him in his observations of the night sky and became an accomplished astronomer in her own right. Together, they transformed our view of the universe from the unchanging, mechanical creation of Newton's clockmaker god to the ever-evolving, incredibly dynamic cosmos that it truly is. William was in his forties when his amateur observations usi

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

Science.gov (United States)

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.

The William and Flora Hewlett Foundation | IDRC - International ...

International Development Research Centre (IDRC) Digital Library (Canada)

The William and Flora Hewlett Foundation. The William and Flora Hewlett Foundation. http://www.hewlett.org/. Think Tank Initiative. This initiative is creating high-quality independent research and policy institutions throughout the developing world. View more. Think Tank Initiative. Growth and Economic Opportunities for ...

Mental Health Problems in Adults with Williams Syndrome

Science.gov (United States)

Stinton, Chris; Elison, Sarah; Howlin, Patricia

2010-01-01

Although many researchers have investigated emotional and behavioral difficulties in individuals with Williams syndrome, few have used standardized diagnostic assessments. We examined mental health problems in 92 adults with Williams syndrome using the Psychiatric Assessment Schedule for Adults with Developmental Disabilities--PAS-ADD (Moss,…

34 CFR 685.100 - The William D. Ford Federal Direct Loan Program.

Science.gov (United States)

2010-07-01

... 34 Education 3 2010-07-01 2010-07-01 false The William D. Ford Federal Direct Loan Program. 685...) OFFICE OF POSTSECONDARY EDUCATION, DEPARTMENT OF EDUCATION WILLIAM D. FORD FEDERAL DIRECT LOAN PROGRAM Purpose and Scope § 685.100 The William D. Ford Federal Direct Loan Program. (a) Under the William D. Ford...

Congenital heart defects in Williams syndrome.

Science.gov (United States)

Yuan, Shi-Min

2017-01-01

Yuan SM. Congenital heart defects in Williams syndrome. Turk J Pediatr 2017; 59: 225-232. Williams syndrome (WS), also known as Williams-Beuren syndrome, is a rare genetic disorder involving multiple systems including the circulatory system. However, the etiologies of the associated congenital heart defects in WS patients have not been sufficiently elucidated and represent therapeutic challenges. The typical congenital heart defects in WS were supravalvar aortic stenosis, pulmonary stenosis (both valvular and peripheral), aortic coarctation and mitral valvar prolapse. The atypical cardiovascular anomalies include tetralogy of Fallot, atrial septal defects, aortic and mitral valvular insufficiencies, bicuspid aortic valves, ventricular septal defects, total anomalous pulmonary venous return, double chambered right ventricle, Ebstein anomaly and arterial anomalies. Deletion of the elastin gene on chromosome 7q11.23 leads to deficiency or abnormal deposition of elastin during cardiovascular development, thereby leading to widespread cardiovascular abnormalities in WS. In this article, the distribution, treatment and surgical outcomes of typical and atypical cardiac defects in WS are discussed.

Recent paleoseismicity record in Prince William Sound, Alaska, USA

Science.gov (United States)

Kuehl, Steven A.; Miller, Eric J.; Marshall, Nicole R.; Dellapenna, Timothy M.

2017-12-01

Sedimentological and geochemical investigation of sediment cores collected in the deep (>400 m) central basin of Prince William Sound, along with geochemical fingerprinting of sediment source areas, are used to identify earthquake-generated sediment gravity flows. Prince William Sound receives sediment from two distinct sources: from offshore (primarily Copper River) through Hinchinbrook Inlet, and from sources within the Sound (primarily Columbia Glacier). These sources are found to have diagnostic elemental ratios indicative of provenance; Copper River Basin sediments were significantly higher in Sr/Pb and Cu/Pb, whereas Prince William Sound sediments were significantly higher in K/Ca and Rb/Sr. Within the past century, sediment gravity flows deposited within the deep central channel of Prince William Sound have robust geochemical (provenance) signatures that can be correlated with known moderate to large earthquakes in the region. Given the thick Holocene sequence in the Sound ( 200 m) and correspondingly high sedimentation rates (>1 cm year-1), this relationship suggests that sediments within the central basin of Prince William Sound may contain an extraordinary high-resolution record of paleoseismicity in the region.

The plays of Lillian Hellman, Clifford Odets and William Inge on Slovene stages

Directory of Open Access Journals (Sweden)

Mateja Slunjski

2002-12-01

Full Text Available After the Second World War, Slovene theatres started to include in their repertoires more and more American authors and their plays. Their choice were varied, from serious dramas by Eugene O'Neill, Tennessee Williams and Arthur Miller, to comedies by Norman Krasna and John Van Druten dependant mostly on the availability of the texts. In the immediate postwar years the theatres liked to present playwrights with progressive ideas in their plays, such as Lillian Hellman and her "The Little Foxes", which was successfully produced at three Slovene theatres, while her "The Children's Hour" received negative reviews. In the fifties, three theatres chose two plays by Clifford Odets, "Golden Boy" and "Country Girl", mainly because of his socially critical ideas and his admiration of humanity. His plays were rather well received; however, the critics doubted his literary genius. William Inge and his plays, "Come Back, Little Sheba", "Picnic" and "Bus Stop", chosen mainly because of the warmth with which the author depicted the tragedy and the beauty of life, were produced at three Slovene theatres. The author received rather wide-ranging opinions from the critics. Some thought him to be an unoriginal depicter of dull American life, while others praised his lyricism and new dramatic techniques.

Emotional Responsivity in Young Children with Williams Syndrome

Science.gov (United States)

Fidler, Debbie J.; Hepburn, Susan L.; Most, David E.; Philofsky, Amy; Rogers, Sally J.

2007-01-01

The hypothesis that young children with Williams syndrome show higher rates of emotional responsivity relative to other children with developmental disabilities was explored. Performance of 23 young children with Williams syndrome and 30 MA-matched children with developmental disabilities of nonspecific etiologies was compared on an adaptation of…

Malformations vasculaires au cours du syndrome de Williams ...

African Journals Online (AJOL)

Le syndrome de Williams-Beuren est une maladie génétique rare, il associe classiquement une dysmorphie faciale assez spécifique, des malformations cardiovasculaires et un profil neuropsychologique particulier. Nous rapportons les observations de trois enfants atteints du syndrome de Williams-Beuren en insistant ...

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

Science.gov (United States)

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.

William Carlos Williams’ cubism: The sensory dimension

Directory of Open Access Journals (Sweden)

J-L Kruger

1995-05-01

Full Text Available In this article the cubism of the American poet William Carlos Williams is discussed as a product of sensory elements combined with techniques derived from the work of the visual artists associated with this style. Through the study o f a number of poems written in the period between 1917 and 1923 it is shown that Williams employs the cubist intersection of sensory planes in particular to create a sensory dimension that not only renews the traditions and mode of poetry, but also reveals the cubist concern with the defamiliarization and foregrounding of fragments of everyday experiences. Ultimately the article is an attempt to indicate Williams’ incorporation o f a sensual dimension in creating a style that achieves modernist presentation revealing an independence from both traditional literary and visual styles.

Infantile ictal apneas in a child with williams-beuren syndrome.

Science.gov (United States)

Myers, Kenneth A; McLeod, D Ross; Bello-Espinosa, Luis

2013-02-01

Williams-Beuren syndrome is a genetic disorder rarely associated with seizures. The few described cases of Williams-Beuren syndrome and epilepsy have primarily involved infantile spasms and deletions extending beyond the common deletion region for this disorder. We present the case of a 5-week-old child with ictal apneas and typical Williams-Beuren syndrome deletion. Diagnosis was challenging, because the child had cardiac, respiratory, and gastrointestinal abnormalities typically associated with Williams-Beuren syndrome, which are also associated with cyanotic episodes. The results of interictal electroencephalography were normal, illustrating that prolonged electroencephalography is often essential in evaluation of suspected ictal apneas. Seizure freedom was achieved with carbamazepine. Sudden death is seen in Williams-Beuren syndrome, and this case raises the question whether some of these cases may be related to ictal apneas and could potentially be preventable with appropriate pharmaceutical intervention. Copyright © 2013 Elsevier Inc. All rights reserved.

How Flexible is the Use of Egocentric Versus Allocentric Frame of Reference in the Williams Syndrome Population?

Science.gov (United States)

Heiz, J; Majerus, S; Barisnikov, K

2017-09-28

This study examined the spontaneous use of allocentric and egocentric frames of reference and their flexible use as a function of instructions. The computerized spatial reference task created by Heiz and Barisnikov (2015) was used. Participants had to choose a frame of reference according to three types of instructions: spontaneous, allocentric and egocentric. The performances of 16 Williams Syndrome participants between 10 and 41 years were compared to those of two control groups (chronological age and non-verbal intellectual ability). The majority of Williams Syndrome participants did not show a preference for a particular frame of reference. When explicitly inviting participants to use an allocentric frame of reference, all three groups showed an increased use of the allocentric frame of reference. At the same time, an important heterogeneity of type of frame of reference used by Williams Syndrome participants was observed. Results demonstrate that despite difficulties in the spontaneous use of allocentric and egocentric frames of reference, some Williams Syndrome participants show flexibility in the use of an allocentric frame of reference when an explicit instruction is provided. © The Author 2017. Published by Oxford University Press. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.

Vernacular Authorship in Late Medieval Religious Discourse. The Case of William Flete’s Remedies against Temptations

Directory of Open Access Journals (Sweden)

Gabriella Del Lungo Camiciotti

2012-03-01

Full Text Available Despite the fact that the main topic of William Flete’s Remedies against Temptations was a pivotal concern of late medieval spiritual literature and the treatise in letter form was widely circulated in both Latin and English, it has remained rather marginal to critical discourse. Neither epistolary space as the site of interaction author/audience nor the role of spiritual authorities in establishing themselves as real authors of religious texts as distinguished from compilers and scribes have been specifically investigated. The paper focuses on the dialogic construction of the authorial voice in William Flete’s Remedies against Temptations through the analysis of the linguistic and discursive strategies used in the vernacular version of this work of spiritual advice. The most relevant strategy is the choice of the letter format to address a female audience as it allows to transfer authoritative religious discourse into English and to assert the writer’s status of author of a text addressed to both religious women and the lay public. In addition, the paper aims at highlighting the relevance of stylistic analysis to delineate the construction of the textual vernacular author in the context of audience recognition. In addressing a non-academic public, the author of one of the English versions of Remedies against Temptations engages with Latin learning and asserts himself as the author of a vernacular theology text.

Celiac disease in patients with Williams-Beuren syndrome.

Science.gov (United States)

Mıhçı, Ercan; Nur, Banu Güzel; Berker-Karaüzüm, Sibel; Yılmaz, Aygen; Artan, Reha

2015-01-01

Celiac disease is an autoimmune, gastrointestinal disorder characterized by intolerance to the dietary grain protein gluten. An increased prevalence of celiac disease has been reported in Down syndrome and Turner syndrome, but there has been only few previous reports with respect to the association of celiac disease in Williams-Beuren syndrome. The aim of this study was to evaluate the frequency of celiac disease in our 24 Williams-Beuren syndrome patients. Gastrointestinal problems and celiac disease symptoms of patients were noted. All patients were analyzed by the titer of tissue transglutaminases IgA and IgG. HLA genotyping and intestinal biopsy was performed to the patients with positive serology. We also performed gluten free diet in the presence of compatible symptoms, serology, HLA genotyping and intestinal biopsy. In our study, two patients had positive tTG antibodies, but only one had positive biopsy finding for celiac disease. The frequency of celiac disease in patients with Williams-Beuren syndrome was estimated as 1/24 (4.1%). Though the number of participants in this study was limited, the results show that the frequency of celiac disease is higher in Williams-Beuren syndrome compared to the general population. We suggest that a high suspicion and testing for celiac disease should be recommended at certain intervals in all cases with Williams-Beuren syndrome to detect the cause of growth retardation and gastrointestinal problems.

WE-G-213-00: History Symposium: Radiological Physics Pioneers: Roentgen and the AAPM Award Eponyms - William Coolidge, Edith Quimby, and Marvin Williams - Who Were They and What Did They Do?

Energy Technology Data Exchange (ETDEWEB)

NONE

2015-06-15

was hired by Giacchino Failla as a radiation physicist at Memorial Hospital for Cancer in New York City. Failla had studied with Madame Curie and obtained his doctoral degree in her laboratory. After many groundbreaking medical physics studies from 1919 until 1942, they both moved to Columbia University. Dr. Quimby developed a widely employed dosimetry system for single plane implants with radium and radon seeds, and a dosimetry methodology for internal radionuclides. She was author of more than 75 scientific publications, and of significant textbooks including the first comprehensive physics textbook for radiologists “Physical Foundations of Radiology”, which was co-authored with Otto Glasser, Lauriston Taylor and James Weatherwax in the first edition, with Russell Morgan added for the second edition and Paul Goodwin for the fourth edition. With Sergei Feitelberg, M.D. she published two editions of “Radioactive Isotopes in Medicine and Biology: Basic Physics and Instrumentation”. Quimby became a renowned examiner for the American Board of Radiology when the third ABR examination, given in 1936, added physics. She served as President of the American Radium Society, received the RSNA Gold Medal, and also numerous prestigious awards given to women in science. Edith Quimby was a Charter Member of AAPM. The AAPM Lifetime Achievement Award was renamed the Edith H. Quimby Lifetime Achievement Award in her honor in 2011. Marvin Martin Dixon Williams (1902–1981) Marvin Williams was born in Walla Walla, WA in 1902, and attended the same college as Edith Quimby, graduating from Whitman College in 1926. He was greatly influenced to go into medical physics by her accomplishments. During his early career, Williams worked with James Weatherwax in Philadelphia while he was working toward an M.S. from the University of Pennsylvania. In 1931 Williams was awarded a Ph.D. in Biophysics from the University of Minnesota, with the work actually performed at the Mayo Clinic

WE-G-213-00: History Symposium: Radiological Physics Pioneers: Roentgen and the AAPM Award Eponyms - William Coolidge, Edith Quimby, and Marvin Williams - Who Were They and What Did They Do?

International Nuclear Information System (INIS)

2015-01-01

was hired by Giacchino Failla as a radiation physicist at Memorial Hospital for Cancer in New York City. Failla had studied with Madame Curie and obtained his doctoral degree in her laboratory. After many groundbreaking medical physics studies from 1919 until 1942, they both moved to Columbia University. Dr. Quimby developed a widely employed dosimetry system for single plane implants with radium and radon seeds, and a dosimetry methodology for internal radionuclides. She was author of more than 75 scientific publications, and of significant textbooks including the first comprehensive physics textbook for radiologists “Physical Foundations of Radiology”, which was co-authored with Otto Glasser, Lauriston Taylor and James Weatherwax in the first edition, with Russell Morgan added for the second edition and Paul Goodwin for the fourth edition. With Sergei Feitelberg, M.D. she published two editions of “Radioactive Isotopes in Medicine and Biology: Basic Physics and Instrumentation”. Quimby became a renowned examiner for the American Board of Radiology when the third ABR examination, given in 1936, added physics. She served as President of the American Radium Society, received the RSNA Gold Medal, and also numerous prestigious awards given to women in science. Edith Quimby was a Charter Member of AAPM. The AAPM Lifetime Achievement Award was renamed the Edith H. Quimby Lifetime Achievement Award in her honor in 2011. Marvin Martin Dixon Williams (1902–1981) Marvin Williams was born in Walla Walla, WA in 1902, and attended the same college as Edith Quimby, graduating from Whitman College in 1926. He was greatly influenced to go into medical physics by her accomplishments. During his early career, Williams worked with James Weatherwax in Philadelphia while he was working toward an M.S. from the University of Pennsylvania. In 1931 Williams was awarded a Ph.D. in Biophysics from the University of Minnesota, with the work actually performed at the Mayo Clinic

Williams Syndrome and 15q Duplication: Coincidence versus Association.

Science.gov (United States)

Khokhar, Aditi; Agarwal, Swashti; Perez-Colon, Sheila

2017-01-01

Williams syndrome is a multisystem disorder caused by contiguous gene deletion in 7q11.23, commonly associated with distinctive facial features, supravalvular aortic stenosis, short stature, idiopathic hypercalcemia, developmental delay, joint laxity, and a friendly personality. The clinical features of 15q11q13 duplication syndrome include autism, mental retardation, ataxia, seizures, developmental delay, and behavioral problems. We report a rare case of a girl with genetically confirmed Williams syndrome and coexisting 15q duplication syndrome. The patient underwent treatment for central precocious puberty and later presented with primary amenorrhea. The karyotype revealed 47,XX,+mar. FISH analysis for the marker chromosome showed partial trisomy/tetrasomy for proximal chromosome 15q (15p13q13). FISH using an ELN -specific probe demonstrated a deletion in the Williams syndrome critical region in 7q11.23. To our knowledge, a coexistence of Williams syndrome and 15q duplication syndrome has not been reported in the literature. Our patient had early pubertal development, which has been described in some patients with Williams syndrome. However, years later after discontinuing gonadotropin-releasing hormone analogue treatment, she developed primary amenorrhea.

Colour discrimination and categorisation in Williams syndrome

OpenAIRE

Farran, E. K.; Cranwell, M. B.; Alvarez, J.; Franklin, A.

2013-01-01

Individuals with Williams syndrome (WS) present with impaired functioning of the dorsal visual stream relative to the ventral visual stream. As such, little attention has been given to ventral stream functions in WS. We investigated colour processing, a predominantly ventral stream function, for the first time in nineteen individuals with Williams syndrome. Colour discrimination was assessed using the Farnsworth-Munsell 100 hue test. Colour categorisation was assessed using a match-to-sample ...

76 FR 44893 - Prince William Sound Resource Advisory Committee

Science.gov (United States)

2011-07-27

... DEPARTMENT OF AGRICULTURE Forest Service Prince William Sound Resource Advisory Committee AGENCY: Forest Service, USDA. ACTION: Notice of meeting. SUMMARY: The Prince William Sound Resource Advisory... District, 145 Forest Station Road, Girdwood, AK; Prince Willam Sound Community College, 303 Lowe Street...

33 CFR 165.1704 - Prince William Sound, Alaska-regulated navigation area.

Science.gov (United States)

2010-07-01

... 33 Navigation and Navigable Waters 2 2010-07-01 2010-07-01 false Prince William Sound, Alaska... District § 165.1704 Prince William Sound, Alaska-regulated navigation area. (a) The following is a... Hinchinbrook Light to Schooner Rock Light, comprising that portion of Prince William Sound between 146°30′ W...

Florence Jessie Mac Williams

Indian Academy of Sciences (India)

CPMGIKAlBGE-340/2003-05. Resonance - January 2005. Licenced to post WPP No.6 RT Nagar Postoffice. Florence Jessie Mac Williams. (1917 - 1990). Registered with Registrar of Newspapers in India vide Regn. No. 66273/96. ISSN 0971-8044. Price per copy: Rs 40.

Browse Author Index

African Journals Online (AJOL)

A B C D E F G H I J K L M N O P Q R S T U V W X Y Z All. W. Walker, Melanie · Walters, Shirley · Williams, Kevin · Winberg, Chris · Winberg, Christine · Wisker, Gina · Wolff, Karin 1 - 7 of 7 Items. ISSN: 2310-7103. AJOL African Journals Online. HOW TO USE AJOL... for Researchers · for Librarians · for Authors · FAQ's · More ...

Mailability v. the Crusader: Williams v. O'Brien.

Science.gov (United States)

Simmons, Charles E.

The issues of prior restraint and press censorship are examined in this paper, which focuses on the 1970 Williams v. O'Brien court case. The paper discusses the litigation, in which Robert F. Williams, as an American citizen living in Peking, China, sued the United States Postmaster General over the banning of the May 1967 issue of "The…

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.

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...

David Owen WILLIAMS

CERN Multimedia

2006-01-01

Lidy Williams-Oonk and her children Mark & Marietta, being unable to thank everybody individually, would like to express their sincere thanks to friends and colleagues at CERN and abroad for their great help and support, their messages and flowers, as well as their donations to the Ligue Genevoise contre le Cancer, on the death of their beloved husband and father.

77 FR 19301 - Prince William Sound Regional Citizens' Advisory Council Charter Renewal

Science.gov (United States)

2012-03-30

... DEPARTMENT OF HOMELAND SECURITY Coast Guard [USCG-2012-0099] Prince William Sound Regional... Prince William Sound Regional Citizens' Advisory Council (PWSRCAC) as an alternative voluntary advisory group for Prince William Sound, Alaska. This certification allows the PWSRCAC to monitor the activities...

Interaction with William Carnall

International Nuclear Information System (INIS)

Judd, Brian R.

2005-01-01

A personal account is given of interaction with William T. Carnall during the period 1977-1988, when I made regular visits to the Argonne National Laboratory to discuss the theoretical background to the spectroscopic work he was carrying out on the lanthanides and actinides

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

Global and local music perception in children with Williams syndrome.

Science.gov (United States)

Deruelle, Christine; Schön, Daniele; Rondan, Cécilie; Mancini, Josette

2005-04-25

Musical processing can be decomposed into the appreciation of global and local elements. This global/local dissociation was investigated with the processing of contour-violated and interval-violated melodies. Performance of a group of 16 children with Williams syndrome and a group of 16 control children were compared in a same-different task. Control participants were more accurate in detecting differences in the contour-violated than in the interval-violated condition while Williams syndrome individuals performed equally well in both conditions. This finding suggests that global precedence may occur at an early perceptual stage in normally developing children. In contrast, no such global precedence is observed in the Williams syndrome population. These data are discussed in the context of atypical cognitive profiles of individuals with Williams syndrome.

Intracranial arteries in individuals with the elastin gene hemideletion of Williams syndrome.

Science.gov (United States)

Wint, D P; Butman, J A; Masdeu, J C; Meyer-Lindenberg, A; Mervis, C B; Sarpal, D; Morris, C A; Berman, K F

2014-01-01

Williams syndrome, a rare genetic disorder with a striking neurobehavioral profile characterized by extreme sociability and impaired visuospatial construction abilities, is caused by a hemideletion that includes the elastin gene, resulting in frequent supravavular aortic stenosis and other stenotic arterial lesions. Strokes have been reported in Williams syndrome. Although the extracranial carotid artery has been studied in a sample of patients with Williams syndrome, proximal intracranial arteries have not. Using MRA, we studied the intracranial vessels in 27 participants: 14 patients with Williams syndrome (age range, 18-44 years; mean age, 27.3 ± 9.1; 43% women) and 13 healthy control participants with similar age and sex distribution (age range, 22-52 years; mean age, 33.4 ± 7.6; 46% women). All participants with Williams syndrome had hemideletions of the elastin gene. Blinded to group allocation or to any other clinical data, a neuroradiologist determined the presence of intracranial vascular changes in the 2 groups. The Williams syndrome group and the healthy control group had similar patency of the proximal intracranial arteries, including the internal carotid and vertebral arteries; basilar artery; and stem and proximal branches of the anterior cerebral artery, MCA, and posterior cerebral arteries. The postcommunicating segment of the anterior cerebral artery was longer in the Williams syndrome group. Despite the elastin haploinsufficiency, the proximal intracranial arteries in Williams syndrome preserve normal patency.

[Association between Williams syndrome and adrenal insufficiency].

Science.gov (United States)

Rchachi, Meryem; Larwanou, Maazou Mahamane; El Ouahabi, Hanan; Ajdi, Farida

2017-01-01

Williams syndrome is a developmental disorder including dysmorphia, cardiovascular malformations and a specific neuropsychological profile together with other associated disorders. We report the case of a 17-year old girl, born of a non-inbred marriage, with Williams syndrome discovered during an assessment of degree of failure to thrive. Its association with primary adrenal insufficiency makes it unique. Diagnosis is confirmed by cytogenetic and molecular analysis. Its management consists of the implementation of treatment for adrenal insufficiency associated with a clinico-biological monitoring.

White matter integrity deficits in prefrontal-amygdala pathways in Williams syndrome.

Science.gov (United States)

Avery, Suzanne N; Thornton-Wells, Tricia A; Anderson, Adam W; Blackford, Jennifer Urbano

2012-01-16

Williams syndrome is a neurodevelopmental disorder associated with significant non-social fears. Consistent with this elevated non-social fear, individuals with Williams syndrome have an abnormally elevated amygdala response when viewing threatening non-social stimuli. In typically-developing individuals, amygdala activity is inhibited through dense, reciprocal white matter connections with the prefrontal cortex. Neuroimaging studies suggest a functional uncoupling of normal prefrontal-amygdala inhibition in individuals with Williams syndrome, which might underlie both the extreme amygdala activity and non-social fears. This functional uncoupling might be caused by structural deficits in underlying white matter pathways; however, prefrontal-amygdala white matter deficits have yet to be explored in Williams syndrome. We used diffusion tensor imaging to investigate prefrontal-amygdala white matter integrity differences in individuals with Williams syndrome and typically-developing controls with high levels of non-social fear. White matter pathways between the amygdala and several prefrontal regions were isolated using probabilistic tractography. Within each pathway, we tested for between-group differences in three measures of white matter integrity: fractional anisotropy (FA), radial diffusivity (RD), and parallel diffusivity (λ(1)). Individuals with Williams syndrome had lower FA, compared to controls, in several of the prefrontal-amygdala pathways investigated, indicating a reduction in white matter integrity. Lower FA in Williams syndrome was explained by significantly higher RD, with no differences in λ(1), suggestive of lower fiber density or axon myelination in prefrontal-amygdala pathways. These results suggest that deficits in the structural integrity of prefrontal-amygdala white matter pathways might underlie the increased amygdala activity and extreme non-social fears observed in Williams syndrome. Copyright © 2011 Elsevier Inc. All rights reserved.

THE INTELLECTUAL STRUCTURE OF ONLINE SHOPPING RESEARCH: AUTHOR CO-CITATION ANALYSIS

Directory of Open Access Journals (Sweden)

Wann-Yih Wu

2016-06-01

Full Text Available This study was conducted to investigate the intellectual structure of the online shopping field in the last decade using co-citation analysis. The citations were obtained from the database of WOS (World of Science, and articles (authors were used as the units of analysis. Based on the results, this research revealed the main categories in the field of online shopping and the relationships between the subfields of research subjects and among authors and identified the authors who play a central role in the conceptual domains of online shopping. This research outlines the intellectual structure of the field of online shopping and its development trends. It also provides details about the influence of individual authors and the evolving structure of this research field over time.

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

Directory of Open Access Journals (Sweden)

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.

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

Science.gov (United States)

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.

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

International Nuclear Information System (INIS)

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

Energy Technology Data Exchange (ETDEWEB)

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)

Discovering Structure in Auditory Input: Evidence from Williams Syndrome

Science.gov (United States)

Elsabbagh, Mayada; Cohen, Henri; Karmiloff-Smith, Annette

2010-01-01

We examined auditory perception in Williams syndrome by investigating strategies used in organizing sound patterns into coherent units. In Experiment 1, we investigated the streaming of sound sequences into perceptual units, on the basis of pitch cues, in a group of children and adults with Williams syndrome compared to typical controls. We showed…

Sir William Turner (1832-1916) - Lancastrian, anatomist and champion of the Victorian era.

Science.gov (United States)

Wessels, Quenton; Correia, Janine Carla; Taylor, Adam M

2016-11-01

Sir William Turner, a Lancastrian, was renowned as a scientist, anatomist and a great reformer of medical education. His students became anatomists at various international institutions, which consequently shaped the future of anatomy as a subject matter both in the United Kingdom and in South Africa. Although Turner's accomplishments have been documented, little is known about the details that determined his career path and the individuals that shaped his future. Here the authors aim to highlight some aspects of Turner's academic achievements and his personal life as well as how he crossed paths with other great minds of the Victorian era including Richard Owen, Charles Darwin, James Paget and Joseph Lister. © The Author(s) 2015.

75 FR 16159 - Prince William Sound Regional Citizens' Advisory Council (PWSRCAC) Charter Renewal

Science.gov (United States)

2010-03-31

... DEPARTMENT OF HOMELAND SECURITY Coast Guard [USCG-2010-0121] Prince William Sound Regional... the Prince William Sound Regional Citizens' Advisory Council (PWSRCAC) as an alternative voluntary advisory group for Prince William Sound, Alaska. This certification allows the PWSRCAC to monitor the...

Shoreline oiling conditions in Prince William Sound following the Exxon Valdez oil spill

International Nuclear Information System (INIS)

Neff, J.M.; Owens, E.H.; Stoker, S.W.; McCormick, D.M.

1995-01-01

Following the Exxon Valdez oil spill of March 24, 1989, in Prince William Sound, Alaska, Exxon conducted comprehensive, systematic shoreline surveys in cooperation with federal and state authorities to obtain information on the distribution and magnitude of shoreline oiling and to identify natural and cultural resources requiring special protection. Similar joint surveys were performed during the springs of 1990, 1991, and 1992 on all Prince william Sound and Gulf of Alaska shorelines that were suspected of having remnants of weathered oil and that would benefit from further cleanup. In the springs of 1990, 1991, and 1992, isolated pockets of subsurface oil were found, chiefly in small scattered zones in coarse cobble/boulder sediments in the upper intertidal or supratidal zones. In 1991, about one-third of the subdivisions in Prince William Sound with surface oil also contained some subsurface oil. The areal extent of this subsurface oil declined by nearly 70% between 1991 and 1992, from about 37,000 m 2 to about 12,000 m 2 . Moreover, where subsurface oil remained in 1992, it was present in lesser amounts. Rates of oil removal were greatest on coastal sections treated early in the spring and summer of 1989. Where shoreline treatment was delayed, the subsequent rate of removal of oil from the shore by natural processes was slower. 27 refs., 10 figs., 3 tabs

On the normalization and visualization of author co-citation data: Salton's cosine versus the Jaccard index

NARCIS (Netherlands)

Leydesdorff, L.

2008-01-01

The debate about which similarity measure one should use for the normalization in the case of Author Co-citation Analysis (ACA) is further complicated when one distinguishes between the symmetrical co-citation - or, more generally, co-occurrence - matrix and the underlying asymmetrical citation -

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

Directory of Open Access Journals (Sweden)

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

Anderson, Prof. Basil Williams

Indian Academy of Sciences (India)

Home; Fellowship. Fellow Profile. Elected: 1964 Honorary. Anderson, Prof. Basil Williams. Date of birth: 3 July 1901. Date of death: 24 February 1984. YouTube; Twitter; Facebook; Blog. Academy News. IAS Logo. 29th Mid-year meeting. Posted on 19 January 2018. The 29th Mid-year meeting of the Academy will be held ...

Series, Prof. George William

Indian Academy of Sciences (India)

Home; Fellowship. Fellow Profile. Elected: 1984 Honorary. Series, Prof. George William. Date of birth: 22 February 1920. Date of death: 2 January 1995. YouTube; Twitter; Facebook; Blog. Academy News. IAS Logo. 29th Mid-year meeting. Posted on 19 January 2018. The 29th Mid-year meeting of the Academy will be held ...

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

Science.gov (United States)

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.

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

Directory of Open Access Journals (Sweden)

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.

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

Directory of Open Access Journals (Sweden)

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.

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

Directory of Open Access Journals (Sweden)

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.

The use of emotions in narratives in Williams syndrome.

Science.gov (United States)

Van Herwegen, Jo; Aznar, Ana; Tenenbaum, Harriet

2014-01-01

Although individuals with Williams syndrome are very sociable, they tend to have limited contact and friendships with peers. In typically developing children the use of positive emotions (e.g., happy) has been argued to be related to peer relationships and popularity. The current study investigated the use and development of emotion words in Williams syndrome using cross-sectional developmental trajectories and examined children's use of different types of emotion words. Nineteen children with Williams syndrome (WS) and 20 typically developing (TD) children matched for chronological age told a story from a wordless picture book. Participants with WS produced a similar number of emotion words compared to the control group and the use of emotion words did not change when plotted against chronological age or vocabulary abilities in either group. However, participants with WS produced more emotion words about sadness. Links between emotion production and friendships as well as future studies are discussed. After reading this article, readers will be able to: explain the development of positive and negative emotions in Williams syndrome and recognize that emotion production is atypical in this population. Copyright © 2014 Elsevier Inc. All rights reserved.

33 CFR 167.1701 - In Prince William Sound: Precautionary areas.

Science.gov (United States)

2010-07-01

... 33 Navigation and Navigable Waters 2 2010-07-01 2010-07-01 false In Prince William Sound: Precautionary areas. 167.1701 Section 167.1701 Navigation and Navigable Waters COAST GUARD, DEPARTMENT OF... Traffic Separation Schemes and Precautionary Areas Pacific West Coast § 167.1701 In Prince William Sound...

A MacWilliams Identity for Convolutional Codes : The General Case

NARCIS (Netherlands)

Gluesing-Luerssen, Heide; Schneider, Gert

A MacWilliams Identity for convolutional codes will be established. It makes use of the weight adjacency matrices of the code and its dual, based on state space realizations (the controller canonical form) of the codes in question. The MacWilliams Identity applies to various notions of duality

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

Directory of Open Access Journals (Sweden)

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

Probed Serial Recall in Williams Syndrome: Lexical Influences on Phonological Short-Term Memory

Science.gov (United States)

Brock, Jan; McCormack, Teresa; Boucher, Jill

2005-01-01

Williams syndrome is a genetic disorder that, it has been claimed, results in an unusual pattern of linguistic strengths and weaknesses. The current study investigated the hypothesis that there is a reduced influence of lexical knowledge on phonological short-term memory in Williams syndrome. Fourteen children with Williams syndrome and 2…

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

Science.gov (United States)

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.

78 FR 28953 - William D. Ford Federal Direct Loan Program

Science.gov (United States)

2013-05-16

.... Ford Federal Direct Loan Program; Interim Final Rule #0;#0;Federal Register / Vol. 78, No. 95... [Docket ID ED-2013-OPE-0066] RIN 1840-AD13 William D. Ford Federal Direct Loan Program AGENCY: Office of... Secretary amends the William D. Ford Federal Direct Loan Program (Direct Loan Program) regulations to...

Pursuing the Panderer: An Analysis of "United States v. Williams"

Science.gov (United States)

McGrain, Patrick N.; Moore, Jennifer L.

2010-01-01

In May 2008, the Supreme Court addressed whether the government can regulate the ownership and distribution of virtual child pornography. "U.S. v. Williams" marked the first time the Court directly addressed the concept of pandering virtual child pornography. This article examines the Court's decision in "U.S. v. Williams" and…

Prince William Sound, Alaska ESI: INDEX

Data.gov (United States)

National Oceanic and Atmospheric Administration, Department of Commerce — This data set comprises the Environmental Sensitivity Index (ESI) data for Prince William Sound, Alaska. ESI data characterize estuarine environments and wildlife by...

Using novel control groups to dissect the amygdala's role in Williams syndrome.

Science.gov (United States)

Thornton-Wells, Tricia A; Avery, Suzanne N; Blackford, Jennifer Urbano

2011-07-01

Williams syndrome is a neurodevelopmental disorder with an intriguing behavioral phenotype-hypersociability combined with significant non-social fears. Previous studies have demonstrated abnormalities in amygdala function in individuals with Williams syndrome compared to typically-developing controls. However, it remains unclear whether the findings are related to the atypical neurodevelopment of Williams syndrome, or are also associated with behavioral traits at the extreme end of a normal continuum. We used functional magnetic resonance imaging (fMRI) to compare amygdala blood-oxygenation-level-dependent (BOLD) responses to non-social and social images in individuals with Williams syndrome compared to either individuals with inhibited temperament (high non-social fear) or individuals with uninhibited temperament (high sociability). Individuals with Williams syndrome had larger amygdala BOLD responses when viewing the non-social fear images than the inhibited temperament control group. In contrast, when viewing both fear and neutral social images, individuals with Williams syndrome did not show smaller amygdala BOLD responses relative to the uninhibited temperament control group, but instead had amygdala responses proportionate to their sociability. These results suggest heightened amygdala response to non-social fear images is characteristic of WS, whereas, variability in amygdala response to social fear images is proportionate to, and might be explained by, levels of trait sociability.

Femineidad y ficción en la obra operística de Richard Strauss

Directory of Open Access Journals (Sweden)

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.

76 FR 1187 - Application for Recertification of Prince William Sound Regional Citizens' Advisory Council

Science.gov (United States)

2011-01-07

... Prince William Sound Regional Citizens' Advisory Council AGENCY: Coast Guard, DHS. ACTION: Notice of... on, the application for recertification submitted by the Prince William Sound Regional Citizen's... advisory group in lieu of a Regional Citizens' Advisory Council for Prince William Sound, Alaska. This...

Dreamers in dialogue: evolution, sex and gender in the utopian visions of William Morris and William Henry Hudson

Directory of Open Access Journals (Sweden)

Caterina Novák

2013-12-01

Full Text Available The aim of this article is to explore the parallels between two late-nineteenth-century utopias,William Henry Hudsons A Crystal Age (1882 and William Morriss News from Nowhere (1891. Itaims to explore how these two works respond to the transition from a kinetic to a static conception ofutopia that under pressure from evolutionary and feminist discourses took place during the period.Particular focus lies on the way in which this is negotiated through the depiction of evolution, sexuality,and gender roles in the respective novels, and how the depiction of these disruptive elements may workas a means of ensuring the readers active engagement in political, intellectual and emotional terms.

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

Directory of Open Access Journals (Sweden)

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.

Becoming Co-Authors: Toward Sharing Authority in Religious Education

Science.gov (United States)

Kim, Hyun-Sook

2009-01-01

This article offers an alternative model, the model of shared authority, to the traditional, authoritarian model for authority and obedience for Religious Education. This model moves away from the authoritarian model of a teacher as the authority and the students as obedient listeners in the direction of a shared authority model in which teachers…

A MacWilliams Identity for Convolutional Codes: The General Case

OpenAIRE

Gluesing-Luerssen, Heide; Schneider, Gert

2008-01-01

A MacWilliams Identity for convolutional codes will be established. It makes use of the weight adjacency matrices of the code and its dual, based on state space realizations (the controller canonical form) of the codes in question. The MacWilliams Identity applies to various notions of duality appearing in the literature on convolutional coding theory.

Leukocytoclastic vasculitis: A window to systemic Churg Strauss syndrome

Directory of Open Access Journals (Sweden)

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.

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

Science.gov (United States)

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.

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

Directory of Open Access Journals (Sweden)

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?

Science.gov (United States)

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

Learning by observation: insights from Williams syndrome.

Science.gov (United States)

Foti, Francesca; Menghini, Deny; Mandolesi, Laura; Federico, Francesca; Vicari, Stefano; Petrosini, Laura

2013-01-01

Observing another person performing a complex action accelerates the observer's acquisition of the same action and limits the time-consuming process of learning by trial and error. Observational learning makes an interesting and potentially important topic in the developmental domain, especially when disorders are considered. The implications of studies aimed at clarifying whether and how this form of learning is spared by pathology are manifold. We focused on a specific population with learning and intellectual disabilities, the individuals with Williams syndrome. The performance of twenty-eight individuals with Williams syndrome was compared with that of mental age- and gender-matched thirty-two typically developing children on tasks of learning of a visuo-motor sequence by observation or by trial and error. Regardless of the learning modality, acquiring the correct sequence involved three main phases: a detection phase, in which participants discovered the correct sequence and learned how to perform the task; an exercise phase, in which they reproduced the sequence until performance was error-free; an automatization phase, in which by repeating the error-free sequence they became accurate and speedy. Participants with Williams syndrome beneficiated of observational training (in which they observed an actor detecting the visuo-motor sequence) in the detection phase, while they performed worse than typically developing children in the exercise and automatization phases. Thus, by exploiting competencies learned by observation, individuals with Williams syndrome detected the visuo-motor sequence, putting into action the appropriate procedural strategies. Conversely, their impaired performances in the exercise phases appeared linked to impaired spatial working memory, while their deficits in automatization phases to deficits in processes increasing efficiency and speed of the response. Overall, observational experience was advantageous for acquiring competencies

Learning by observation: insights from Williams syndrome.

Directory of Open Access Journals (Sweden)

Francesca Foti

Full Text Available Observing another person performing a complex action accelerates the observer's acquisition of the same action and limits the time-consuming process of learning by trial and error. Observational learning makes an interesting and potentially important topic in the developmental domain, especially when disorders are considered. The implications of studies aimed at clarifying whether and how this form of learning is spared by pathology are manifold. We focused on a specific population with learning and intellectual disabilities, the individuals with Williams syndrome. The performance of twenty-eight individuals with Williams syndrome was compared with that of mental age- and gender-matched thirty-two typically developing children on tasks of learning of a visuo-motor sequence by observation or by trial and error. Regardless of the learning modality, acquiring the correct sequence involved three main phases: a detection phase, in which participants discovered the correct sequence and learned how to perform the task; an exercise phase, in which they reproduced the sequence until performance was error-free; an automatization phase, in which by repeating the error-free sequence they became accurate and speedy. Participants with Williams syndrome beneficiated of observational training (in which they observed an actor detecting the visuo-motor sequence in the detection phase, while they performed worse than typically developing children in the exercise and automatization phases. Thus, by exploiting competencies learned by observation, individuals with Williams syndrome detected the visuo-motor sequence, putting into action the appropriate procedural strategies. Conversely, their impaired performances in the exercise phases appeared linked to impaired spatial working memory, while their deficits in automatization phases to deficits in processes increasing efficiency and speed of the response. Overall, observational experience was advantageous for

On Reading Poems: Visual & Verbal Icons in William Carlos Williams' «Landscape With The Fall Of Icarus»

Directory of Open Access Journals (Sweden)

Irene R. Fairley

1981-09-01

Full Text Available Williams' admiration for Brueghel's landscape is coded in structural and stylistic correspondences between the poem and the painting. Structures in the poem have effects analogous to the use of devices of color, line, foregrounding in the painting. The poem, like the painting, presents a «neutral» scene but subtly insures the reader's involvement. Further, Williams draws a visual statement so that graphic features suggest a global image isomorphic with the motif of descent. Features of the poem, such as line and clause length, syntactic construction, semantic coherence, are discussed as factors that contribute to rapid glancing and increase readability. A study of readers shows their sensitivity to graphic features including the poem's shape, and corroborates the importance of stylistic features.

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

Science.gov (United States)

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.

Interview: William and Barbara Christopher.

Science.gov (United States)

Teaching Exceptional Children, 1986

1986-01-01

Asserting that the biggest mistake educators can make is to set themselves up as experts vis-a-vis parents as amateurs, Barbara and William Christopher emphasize the importance of continued education for the autistic individual and relate their experiences with the educational community as they sought help for their autistic son. (JC)

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

Science.gov (United States)

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.

Science.gov (United States)

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.

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

Science.gov (United States)

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.

Evaluation of sea otter capture after the Exxon Valdez oil spill, Prince William Sound, Alaska

Science.gov (United States)

Bodkin, James L.; Weltz, F.; Bayha, Keith; Kormendy, Jennifer

1990-01-01

After the T/V Exxon Valdez oil spill into Prince William Sound, the U.S. Fish and Wildlife Service and Exxon Company, U.S.A., began rescuing sea otters (Enhydra lutris). The primary objective of this operation was to capture live, oiled sea otters for cleaning and rehabilitation. Between 30 March and 29 May 1989, 139 live sea otters were captured in the sound and transported to rehabilitation centers in Valdez, Alaska. Within the first 15 days of capture operations, 122 (88%) otters were captured. Most sea otters were captured near Knight, Green, and Evans islands in the western sound. The primary capture method consisted of dipnetting otters out of water and off beaches. While capture rates declined over time, survival of captured otters increased as the interval from spill date to capture date increased. The relative degree of oiling observed for each otter captured declined over time. Declining capture rates led to the use of tangle nets. The evidence suggests the greatest threat to sea otters in Prince William Sound occurred within the first 3 weeks after the spill. Thus, in the future, the authors believe rescue efforts should begin as soon as possible after an oil spill in sea otter habitat. Further, preemptive capture and relocation of sea otters in Prince William Sound may have increased the number of otters that could have survived this event.

Experience in managing patients with Churg-Strauss syndrome.

Directory of Open Access Journals (Sweden)

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.

The Professor and the Student, Sir Ronald Aylmer Fisher (1890-1962) and William Sealy Gosset (1876-1937): Careers of two giants in mathematical statistics.

Science.gov (United States)

Vyas, Shreena A; Desai, Sukumar P

2015-05-01

Sir Ronald Aylmer Fisher and William Sealy Gosset were responsible for laying the foundations of statistical inference. Tests that bear their names are used by students and researchers in a wide variety of scientific disciplines. Similar and different in many respects, their lives and careers are the subject of this essay. They were not teacher and pupil; in fact the student was 14 years older than the professor. Their careers did not require them to interact with one another much but they were aware of one another's work. Although Sir Ronald is assigned the role of the professor, his success as a teacher was impaired by his inability to understand the limitations of his students. Meanwhile Gosset was forced to publish his work under the pseudonym 'Student' in order to make contributions to the field of mathematical statistics. Both men are undisputed giants in the field of statistics and we celebrate their achievements as much as we try to understand their struggles. © The Author(s) 2015 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav.

78 FR 11857 - Agency Information Collection Activities; Comment Request; William D. Ford Federal Direct Loan...

Science.gov (United States)

2013-02-20

...; Comment Request; William D. Ford Federal Direct Loan Program (DL) Regulations AGENCY: Department of... in response to this notice will be considered public records. Title of Collection: William D. Ford.... Abstract: The William D. Ford Federal Direct Loan Program regulations cover areas of program administration...

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

Science.gov (United States)

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.

Rorty, Williams, and Davidson: Skepticism and Metaepistemology

Directory of Open Access Journals (Sweden)

Christopher Ranalli

2013-07-01

Full Text Available We revisit an important exchange on the problem of radical skepticism between Richard Rorty and Michael Williams. In his contribution to this exchange, Rorty defended the kind of transcendental approach to radical skepticism that is offered by Donald Davidson, in contrast to Williams’s Wittgenstein-inspired view. It is argued that the key to evaluating this debate is to understand the particular conception of the radical skeptical problem that is offered in influential work by Barry Stroud, a conception of the skeptical problem which generates metaepistemological ramifications for anti-skeptical theories. In particular, we argue that, contra Williams, Rorty’s view that Davidson was offering a theoretical diagnosis of radical skepticism can be consistently maintained with his transcendental approach.

William D. Ford Federal Direct Loan Program. Final Regulations. Federal Register, Department of Education, 34 CFR Part 685

Science.gov (United States)

National Archives and Records Administration, 2014

2014-01-01

The purpose of this regulatory action is to amend the regulations governing the William D. Ford Federal Direct Loan (Direct Loan) Program (§ 685.200 of title 34 of the Code of Federal Regulations (CFR)). These regulations strengthen and improve administration of the Federal Direct PLUS Loan Program authorized under title IV of the Higher Education…

Music and Anxiety in Williams Syndrome: A Harmonious or Discordant Relationship?

Science.gov (United States)

Dykens, Elisabeth M.; Rosner, Beth A.; Ly, Tran; Sagun, Jaclyn

2005-01-01

In this two-part study, we assessed musical involvements in two samples of persons with Williams syndrome compared to others with mental retardation and also related musicality to anxiety and fears in Study 2. Relative to others with mental retardation, those with Williams syndrome were more likely to take music lessons, play an instrument, and…

Mathematics Education Problems and Attempts to Solve Them in Nowadays Lithuanian School

OpenAIRE

Malaukytė Ieva

2017-01-01

The decreasing number of the Lithuanian residents has strong impact on the educational system: the number of pupils is decreasing, the schools are getting closed. School is considered to be the provider of educational services, so it is necessary to search, how to preserve and attract clients – pupils. The growing competition induces search for distinctiveness among the schools. According to the theory of generations of William Strauss and Neil Howe, now we have to educate representatives of ...

Prince William Sound, Alaska ESI: HYDRO (Hydrology)

Data.gov (United States)

National Oceanic and Atmospheric Administration, Department of Commerce — This data set comprises the Environmental Sensitivity Index (ESI) data for Prince William Sound, Alaska. ESI data characterize estuarine environments and wildlife by...

Prince William Sound, Alaska ESI: INVERT (Invertebrates)

Data.gov (United States)

National Oceanic and Atmospheric Administration, Department of Commerce — This data set comprises the Environmental Sensitivity Index (ESI) data for Prince William Sound, Alaska. ESI data characterize estuarine environments and wildlife by...

Imaging of cardiovascular malformations in Williams syndrome

International Nuclear Information System (INIS)

Li Shiguo; Zhao Shihua; Jiang Shiliang; Huang Lianjun; Xu Zhongying; Ling Jian; Zheng Hong; Yan Chaowu; Lu Jinguo

2008-01-01

Objective: To evaluate the imaging methods for cardiovascular malformations in Williams syndrome(WS). Methods: Thirteen cases of WS (7 males and 6 females) aged 10 months to 13 years were involved in this study. All patients underwent chest X-ray radiography, electrocardiography, echocardiography and physical examination. 3 cases underwent electronic beam computed tomography (EBCT), cardiac catheterization and angiography were performed in 8 cases. Results: Twelve patients were referred to our hospital for cardiac murmur and 1 case for cyanosis after birth. 7 patients were found with 'elfin-like' facial features, 6 patients with pulmonary arterial stenosis, 2 cases with patent ductus arteriosus, 2 cases with severe pulmonary hypertension and 1 case with total endocardial cushion defect. Sudden death occurred in 2 patients during and after catheterization, respectively. Conclusions: Conventional angiography is the golden standard for the diagnosis of cardiovascular malformations in WS. Noninvasive methods such as MSCT and MRI should be suggested because of the risk of sudden death in conventional angiography. (authors)

Who Was the Real William Shakespeare?

Science.gov (United States)

Edwards, Michael Todd

2009-01-01

This article highlights a project that encourages students to connect reading and mathematics instruction by using a data analysis approach. Students analyze sonnets from statistical, literary, and historical points of view in an effort to uncover the true identity of William Shakespeare. (Contains 10 figures.)

Mr. William Shakespeare and the Internet.

Science.gov (United States)

Reeves, Barbara

2000-01-01

Describes resources and links on a Web site entitled "Mr. William Shakespeare and the Internet," with suggestions for using them with students. Highlights include: historical context; impact of events/situations on works; motivational/preparatory lessons; reading and understanding Shakespeare; analysis of works; language; assessing other…

MacWilliams Identity for M-Spotty Weight Enumerator

Science.gov (United States)

Suzuki, Kazuyoshi; Fujiwara, Eiji

M-spotty byte error control codes are very effective for correcting/detecting errors in semiconductor memory systems that employ recent high-density RAM chips with wide I/O data (e.g., 8, 16, or 32bits). In this case, the width of the I/O data is one byte. A spotty byte error is defined as random t-bit errors within a byte of length b bits, where 1 le t ≤ b. Then, an error is called an m-spotty byte error if at least one spotty byte error is present in a byte. M-spotty byte error control codes are characterized by the m-spotty distance, which includes the Hamming distance as a special case for t =1 or t = b. The MacWilliams identity provides the relationship between the weight distribution of a code and that of its dual code. The present paper presents the MacWilliams identity for the m-spotty weight enumerator of m-spotty byte error control codes. In addition, the present paper clarifies that the indicated identity includes the MacWilliams identity for the Hamming weight enumerator as a special case.

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…

[Vascular malformations in the Williams-Beuren syndrome: report of three new cases].

Science.gov (United States)

Sator, Hicham; Rhouni, Fatima Ezzahra; Benjouad, Ibitihale; Rhouni, Fatima Ezzahra; Benjouad, Ibitihale; Dafiri, Rachida; Chat, Latifa

2016-01-01

The Williams-Beuren syndrome is a rare genetic disease. It combines classically specific facial dysmorphism, cardiovascular malformations and specific neuropsychological profile. We report three cases of Williams-Beuren syndrome in children with particular emphasis on vascular abnormalities observed on CT angiography and MR angiography.

[Cardiac involvement in Churg-Strauss syndrome].

Science.gov (United States)

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

Stranger Danger Awareness in Williams Syndrome

Science.gov (United States)

Riby, D. M.; Kirk, H.; Hanley, M.; Riby, L. M.

2014-01-01

Background: The developmental disorder Williams syndrome (WS) is characterised by a distinctive cognitive profile and an intriguing social phenotype. Individuals with the disorder are often highly social engaging with familiar and unfamiliar people and once in an interaction they often show subtle abnormalities of social behaviour. Atypically…

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

Science.gov (United States)

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.

33 CFR 164.43 - Automatic Identification System Shipborne Equipment-Prince William Sound.

Science.gov (United States)

2010-07-01

... 33 Navigation and Navigable Waters 2 2010-07-01 2010-07-01 false Automatic Identification System Shipborne Equipment-Prince William Sound. 164.43 Section 164.43 Navigation and Navigable Waters COAST GUARD... Automatic Identification System Shipborne Equipment—Prince William Sound. (a) Until December 31, 2004, each...

The Values of Masculinity in William Shakespeare’s Macbeth

Directory of Open Access Journals (Sweden)

Mafruha Ferdous

2017-04-01

Full Text Available The theme of gender plays a vital role in William Shakespeare’s famous political play Macbeth. From the very beginning of the play the dramatist focuses on the importance of masculinity in gaining power and authority. Lady Macbeth along with the three witches are as important characters as Macbeth. Because they influence Macbeth profoundly. And Shakespeare very carefully draws the character of Lady Macbeth who being a female sometimes exhibits more masculinity than Macbeth. Similarly is the case of the three witches. Though they look like women they are also bearded which prove the presence of masculinity in their nature. Throughout the play several times the exposition of masculinity is demanded from the character of Macbeth. So the value of masculinity plays an important part in the drama.

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...

Fading-Figure Tracing in Williams Syndrome

Science.gov (United States)

Nagai, Chiyoko; Inui, Toshio; Iwata, Makoto

2011-01-01

Williams syndrome (WS) is a neurodevelopmental disorder characterized by severe impairment of visuospatial abilities. Figure-drawing abilities, which are thought to reflect visuospatial abilities, have yet to be fully investigated in WS. The purpose of the present study was to clarify whether drawing abilities differ between WS individuals and…

77 FR 43276 - Notice of Proposed Information Collection Requests; Federal Student Aid; William D. Ford Federal...

Science.gov (United States)

2012-07-24

...; William D. Ford Federal Direct Loan Program General Forbearance Request SUMMARY: Borrowers who receive loans through the William D. Ford Federal Direct Loan Program will use this form to request forbearance... considered public records. Title of Collection: William D. Ford Federal Direct Loan Program General...

Seasonal distribution of Dall's porpoise in Prince William Sound, Alaska

Science.gov (United States)

Moran, John R.; O'Dell, Matthew B.; Arimitsu, Mayumi L.; Straley, Janice M.; Dickson, Danielle M. S.

2018-01-01

Dall's porpoise, Phocoenoides dalli, are a conspicuous predator in the Prince William Sound ecosystem, yet there has been little effort directed towards monitoring this species since the 1980s, prior to the Exxon Valdez oil spill. We used vessel-based surveys to examine the seasonal distribution of Dall's porpoise in the waters of Prince William Sound during eight years from 2007 to 2015. Over the course of 168 days and 15,653 km of survey effort, 921 Dall's porpoise were encountered in 210 groups. We estimate an encounter rate of 0.061 porpoise/km traveled or 1 porpoise encountered for every 16.5 km traveled. Dall's porpoise were found throughout the year in Prince William Sound, and used a wide range of habitats, including those not considered typical of the species, such as bays, shallow water, and nearshore waters. Dall's porpoise seasonally shifted their center of distribution from the western passages in fall to the bays of the eastern Sound in winter and spring. Dall's porpoises were widely dispersed throughout the Sound in summer. We identified potential Dall's porpoise habitat (depth, slope, and distance from shore) within Prince William Sound using generalized additive models (GAM). Dall's porpoise were found in deeper water during summer and in shallowest water during spring. We propose that their use of novel habitats is a function of reduced predation risk associated with the decline of their main predator, killer whales (Orcinus orca), following the Exxon Valdez oil spill, and the presence of overwintering and spawning Pacific herring (Clupea pallasii). While the size of the Dall's porpoise population within Prince William Sound remains unknown, our encounter rates were lower than those reported in the 1970s. Their high metabolic rate and ubiquitous presence makes them one of the more important, yet understudied, forage fish predators in the region.

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

Science.gov (United States)

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.

Williams-Beuren syndrome associated with single kidney and nephrocalcinosis: a case report.

Science.gov (United States)

Abidi, Kamel; Jellouli, Manel; Ben Rabeh, Rania; Hammi, Yousra; Gargah, Tahar

2015-01-01

Williams-Beuren syndrome is a rare neurodevelopmental disorder, characterized by congenital heart defects, abnormal facial features, mental retardation with specific cognitive and behavioral profile, growth hormone deficiency, renal and skeletal anomalies, inguinal hernia, infantile hypercalcaemia. We report a case with Williams-Beuren syndrome associated with a single kidney and nephrocalcinosis complicated by hypercalcaemia. A male infant, aged 20 months presented growth retardation associated with a psychomotor impairment, dysmorphic features and nephrocalcinosis. He had also hypercalciuria and hypercalcemia. Echocardiography was normal. DMSA renal scintigraphy showed a single functioning kidney. The FISH generated one ELN signal in 20 metaphases read and found the presence of ELN deletion, with compatible Williams-Beuren syndrome.

77 FR 47374 - Notice of Proposed Information Collection Requests; Federal Student Aid; William D. Ford Federal...

Science.gov (United States)

2012-08-08

...; William D. Ford Federal Direct Loan (Direct Loan) Program/ Federal Family Loan (FFEL) Program: Deferment... which borrowers in the William D. Ford Federal Direct Loan (Direct Loan) and Federal Family Education... considered public records. Title of Collection: William D. Ford Federal Direct Loan (Direct Loan) Program...

MRI Amygdala Volume in Williams Syndrome

Science.gov (United States)

Capitao, Liliana; Sampaio, Adriana; Sampaio, Cassandra; Vasconcelos, Cristiana; Fernandez, Montse; Garayzabal, Elena; Shenton, Martha E.; Goncalves, Oscar F.

2011-01-01

One of the most intriguing characteristics of Williams Syndrome individuals is their hypersociability. The amygdala has been consistently implicated in the etiology of this social profile, particularly given its role in emotional and social behavior. This study examined amygdala volume and symmetry in WS individuals and in age and sex matched…

Enough room for Williams and IMF? / Paul Beckman

Index Scriptorium Estoniae

Beckman, Paul

1999-01-01

Lõppesid Leedu ja USA energeetikakompanii Williams International läbirääkimised Leedu naftakompleksis osaluse omandamise asjus. IMF uurib Leedu majanduslikku arengut, mida tehing Williamsiga komplitseerib

Whither Sir William?

Directory of Open Access Journals (Sweden)

Stephen J. Greenberg, MSLS, PhD

2017-04-01

Full Text Available There are times when something is simply so familiar that we can no longer see it at all. It can be a story, or a concept, or even a flesh-and-blood person. Familiarity breeds not only contempt, but a kind of invisibility as well. For too many of us, such is the case with Sir William Osler. In his time (1849–1919, many considered him to be one of the greatest practitioners, teachers, and writers ever in the field of medicine. He was instrumental in the founding of the Medical Library Association (MLA and was elected its second president.

The Williams syndrome prosociality gene GTF2I mediates oxytocin reactivity and social anxiety in a healthy population.

Science.gov (United States)

Procyshyn, Tanya L; Spence, Jason; Read, Silven; Watson, Neil V; Crespi, Bernard J

2017-04-01

The neurohormone oxytocin plays a central role in human social behaviour and cognition, and oxytocin dysregulation may contribute to psychiatric disorders. However, genetic factors influencing individual variation in the oxytocinergic system remain poorly understood. We genotyped 169 healthy adults for a functional polymorphism in GTF2I ( general transcription factor II-I ), a gene associated with high prosociality and reduced social anxiety in Williams syndrome, a condition reported to involve high oxytocin levels and reactivity. Participants' salivary oxytocin levels were measured before and after watching a validated empathy-inducing video. Oxytocin reactivity, defined as pre- to post-video percentage change in salivary oxytocin, varied substantially and significantly between individuals with different GTF2I genotypes, with, additionally, a trend towards an interaction between genotype and sex. Individuals with more oxytocin-reactive genotypes also reported significantly lower social anxiety. These findings suggest a model whereby GTF2I has a continuum of effects on human sociality, from the extreme social phenotypes and oxytocin dysregulation associated with gene deletion in Williams syndrome, to individual differences in oxytocin reactivity and sociality associated with common polymorphisms in healthy populations. © 2017 The Author(s).

ABORDAGEM DIAGNÓSTICA DA SÍNDROME DE WILLIAMS - BEUREN.

OpenAIRE

Santos, Marina Machado

2016-01-01

As anomalias cromossômicas causam fenótipos específicos e complexos resultantes de desequilíbrios na dose normal de genes localizados em um segmento cromossômico específico, sendo responsáveis por 60 % ou mais das síndromes genéticas identificáveis. A Síndrome de Williams-Beuren (SWB) caracteriza-se por anomalias múltiplas e deficiência intelectual, descrita independentemente por Williams et al. (1961) e por Beuren et al. (1962). A SWB consiste em uma doença genética causada pela microdeleção...

Florence Jessie Mac Williams (1917-1990)

Indian Academy of Sciences (India)

Home; Journals; Resonance – Journal of Science Education; Volume 10; Issue 1. Florence Jessie Mac Williams (1917-1990). Featured Scientist Volume 10 Issue 1 January 2005 pp 98-98. Fulltext. Click here to view fulltext PDF. Permanent link: https://www.ias.ac.in/article/fulltext/reso/010/01/0098-0098. Resonance ...

Outcome in Adult Life for People with Williams Syndrome Results from a Survey of 239 Families

Science.gov (United States)

Howlin, P.; Udwin, O.

2006-01-01

BACKGROUND: Although there has been considerable research into the genotype and phenotype of Williams syndrome, there have been relatively few studies of long-term prognosis. As a preliminary to a more detailed investigation of adults with Williams syndrome, a parental questionnaire was distributed to members of the UK Williams Syndrome…

Coronary ostium occlusion by coronary cusp displacement in Williams syndrome.

Science.gov (United States)

Shiohama, Tadashi; Fujii, Katsunori; Ebata, Ryota; Funabashi, Nobusada; Matsumiya, Goro; Saito, Yuko Kazato; Takechi, Fumie; Yonemori, Yoko; Nakatani, Yukio; Shimojo, Naoki

2016-06-01

Williams syndrome is a contiguous gene deletion syndrome resulting from a heterozygous deletion on chromosome 7q11.23, and is characterized by distinctive facial features and supravalvular aortic stenosis (SVAS). This syndrome rarely presents unpredictable cardiac death, and yet, as illustrated in the present case, it is still not possible to predict it, even on close monitoring. We herein describe the case of a 6-year-old Japanese girl with Williams syndrome, who had sudden cardiac collapse due to cardiac infarction after pharyngitis. Cardiac failure followed a critical course that did not respond to catecholamine support or heart rest with extracardiac mechanical support. Although marked coronary stenosis was not present, the left coronary cusp abnormally adhered to the aortic wall, which may synergistically cause coronary ostium occlusion with SVAS. Altered hemodynamic state, even that caused by the common cold, may lead to critical myocardial events in Williams syndrome with SVAS. © 2015 Japan Pediatric Society.

Who Invented the Word Asteroid: William Herschel or Stephen Weston?

Science.gov (United States)

Cunningham, Clifford J.; Orchiston, Wayne

2011-11-01

William Herschel made the first serious study of 1 Ceres and 2 Pallas in the year 1802. He was moved by their dissimilarities to the other planets to coin a new term to distinguish them. For this purpose he enlisted the aid of his good friends William Watson and Sir Joseph Banks. Watson gave him a long list of possible names, which Herschel rejected. With a lifetime of experience classifying and naming newly found objects in nature, Banks became the man both Erasmus Darwin (in 1781) and William Herschel (in 1802) turned to for sage advice in developing a new descriptive language. In the case of Ceres and Pallas, Banks turned the task over to his friend, the noted philologist Stephen Weston, FRS. It has recently been stated by a noted British historian that it was Weston - not Herschel - who coined the term 'asteroid' to collectively describe Ceres and Pallas. This claim is investigated, and parallels are drawn in the use of neologism in astronomy and botany.

Clinical features and prognostic factors of Churg-Strauss syndrome.

Science.gov (United States)

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.

The scientific legacy of William Herschel

CERN Document Server

2018-01-01

This book presents a modern scholarly analysis of issues associated with England’s most famous astronomer, William Herschel. The world’s leading experts on Herschel, discoverer of the planet Uranus, here offer their combined wisdom on many aspects of his life and astronomical research. Solar system topics include comets, Earth’s Moon, and the spurious moons of Uranus, all objects whose observation was pioneered by Herschel.  The contributors examine his study of the structure of the Milky Way and an in-depth look at the development of the front view telescopes he built. The popular subject of extraterrestrial life is looked at from the point of view of both William Herschel and his son John, both of whom had an interest in the topic. William’s personal development through the educational system of the late eighteenth-century is also explored, and the wide range of verse and satire in various languages associated with his discoveries is collected here for the first time. Hershel worked at a time of i...

Holocene deposition and megathrust splay fault geometries within Prince William Sound, Alaska

Science.gov (United States)

Finn, S.; Liberty, L. M.; Haeussler, P. J.; Pratt, T. L.

2011-12-01

New high resolution sparker seismic reflection data, in conjunction with reprocessed legacy seismic data, provide the basis for a new fault, fold, and Holocene sediment thickness database for Prince William Sound, Alaska. Additionally, legacy airgun seismic data in Prince William Sound and the Gulf of Alaska tie features on these new sparker data to deeper portions of megathrust splay faults. We correlate regionally extensive bathymetric lineaments within Prince William Sound to megathrust splay faults, such as the ones that ruptured in the 1964 M9.2 earthquake. Lastly, we estimate Holocene sediment thickness within Prince William Sound to better constrain the Holocene fault history throughout the region. We identify three seismic facies related to Holocene, Quaternary, and Tertiary strata that are crosscut by numerous high angle normal faults in the hanging wall of the megathrust splay faults. The crustal-scale seismic reflection profiles show splay faults emerging from 20 km depth between the Yakutat block and North American crust and surfacing as the Hanning Bay and Patton Bay faults. A change in exhumation rates, slip rates, and fault orientation appears near Hinchinbrook that we attribute to differences in subducted slab geometry. Based on our slip rate analysis, we calculate average Holocene displacements of 20 m and 100 m in eastern and western Prince William Sound, respectively. Landward of two splay faults exposed on Montague Island, we observe subsidence, faulting, and landslides that record deformation associated with the 1964 and older megathrust earthquakes.

March 1964 Prince William Sound, USA Images

Data.gov (United States)

National Oceanic and Atmospheric Administration, Department of Commerce — The Prince William Sound magnitude 9.2 Mw earthquake on March 28, 1964 at 03:36 GMT (March 27 at 5:36 pm local time), was the largest U.S. earthquake ever recorded...

Attentional Disengagement in Adults with Williams Syndrome

Science.gov (United States)

Lense, Miriam D.; Key, Alexandra P.; Dykens, Elisabeth M.

2011-01-01

Williams syndrome (WS) is a neurodevelopmental disorder characterized by a distinctive behavioral and cognitive profile, including widespread problems with attention. However, the specific nature of their attentional difficulties, such as inappropriate attentional allocation and/or poor attentional disengagement abilities, has yet to be…

Sleep-EEG in dizygotic twins discordant for Williams syndrome.

Science.gov (United States)

Bódizs, Róbert; Gombos, Ferenc; Szocs, Katalin; Réthelyi, János M; Gerván, Patrícia; Kovács, Ilona

2014-01-30

Reports on twin pairs concordant and discordant for Williams syndrome were published before, but no study unravelled sleep physiology in these cases yet. We aim to fill this gap by analyzing sleep records of a twin pair discordant for Williams syndrome extending our focus on presleep wakefulness and sleep spindling. We performed multiplex ligation-dependent probe amplification of the 7q11.23 region of a 17 years old dizygotic opposite-sex twin pair discordant for Williams syndrome. Polysomnography of laboratory sleep at this age was analyzed and followed-up after 1.5 years by ambulatory polysomnography. Sleep stages scoring, EEG power spectra and sleep spindle analyses were carried out. The twin brother showed reduced levels of amplification for all of the probes in the 7q11.23 region indicating a typical deletion spanning at least 1.038 Mb between FKBP6 and CLIP2. The results of the twin sister showed normal copy numbers in the investigated region. Lower sleep times and efficiencies, as well as higher slow wave sleep percents of the twin brother were evident during both recordings. Roughly equal NREM, Stage 2 and REM sleep percents were found. EEG analyses revealed state and derivation-independent decreases in alpha power, lack of an alpha spectral peak in presleep wakefulness, as well as higher NREM sleep sigma peak frequency in the twin brother. Faster sleep spindles with lower amplitude and shorter duration characterized the records of the twin brother. Spectra show a striking reliability and correspondence between the two situations (laboratory vs. home records). Alterations in sleep and specific neural oscillations including the alpha/sigma waves are inherent aspects of Williams syndrome.

Auditory Attraction: Activation of Visual Cortex by Music and Sound in Williams Syndrome

Science.gov (United States)

Thornton-Wells, Tricia A.; Cannistraci, Christopher J.; Anderson, Adam W.; Kim, Chai-Youn; Eapen, Mariam; Gore, John C.; Blake, Randolph; Dykens, Elisabeth M.

2010-01-01

Williams syndrome is a genetic neurodevelopmental disorder with a distinctive phenotype, including cognitive-linguistic features, nonsocial anxiety, and a strong attraction to music. We performed functional MRI studies examining brain responses to musical and other types of auditory stimuli in young adults with Williams syndrome and typically…

Florence Jessie MacWilliams (1917-1990)

Indian Academy of Sciences (India)

famous mathematician Oscar Zariski, well known for his work in algebraic geometry, at. Johns Hopkins University, following him to Harvard University to study with him for a year. There was a break in her studies for many years following her marriage in 1941 to. W al ter Mac Williams, an engineer, and the birth and raising of ...

Prince William Sound, Alaska ESI: FISH (Fish Polygons)

Data.gov (United States)

National Oceanic and Atmospheric Administration, Department of Commerce — This data set comprises the Environmental Sensitivity Index (ESI) data for Prince William Sound, Alaska. ESI data characterize estuarine environments and wildlife by...

Prince William Sound, Alaska ESI: NESTS (Bird Nests)

Data.gov (United States)

National Oceanic and Atmospheric Administration, Department of Commerce — This data set comprises the Environmental Sensitivity Index (ESI) data for Prince William Sound, Alaska. ESI data characterize estuarine environments and wildlife by...

Lord Byron's physician: John William Polidori on somnambulism.

Science.gov (United States)

Finger, Stanley; Stiles, Anne

2013-01-01

John William Polidori (1795-1821) was the Edinburgh-trained physician hired by Lord Byron to accompany him to Switzerland, where he participated in the story-telling event proposed by Byron that led, with Polidori's help, to Mary Shelley's Frankenstein. Although those interested in English literature might also remember Polidori as the author of The Vampyre, one of the first extended works of fiction about vampires, his earlier interest in somnambulism and trance states is only beginning to be appreciated. Even more than students of Romantic literature, historians of science and medicine seem little aware of what Polidori had written about oneirodynia, a synonym for somnambulism, and how his thoughts from 1815 about such activities reflected the changing medical zeitgeist at this time. This chapter examines Polidori's medical thesis in a neuroscience context and compares what he wrote to the writings of several other physicians who were fascinated by nocturnal wanderings, their causes, their manifestations, and their possible treatments. © 2013 Elsevier B.V. All rights reserved.

William Shakespeare’s“Hamlet”and Oedipus Complex

Institute of Scientific and Technical Information of China (English)

迪丽努尔

2014-01-01

“Hamlet”is one of the great dramas of William Shakespeare. This paper by describing the relationship between Hamlet and his mother, the Ghost and his uncle, tries to approve that Oedipus complex is the main reason of Hamlet’s kil ing his uncle.

De wereld in 18 lessen: Christopher Williams

NARCIS (Netherlands)

Berrebi, S.

2011-01-01

Hij is een echte artist’s artist, de conceptuele fotograaf Christopher Williams. Sinds hij in Duitsland woont en werkt, vindt zijn afgewogen werk, dat de fotografie tegelijk eert en ondervraagt, steeds makkelijker onderdak in Europese kunstinstituten, zoals dit voorjaar bij Museum Dhondt-Dhaenens in

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

Directory of Open Access Journals (Sweden)

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

Science.gov (United States)

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

Word-Preserving Arts: Material Inscription, Ekphrasis, and Spatial Form in the Later Work of William Wordsworth

DEFF Research Database (Denmark)

Simonsen, Peter

William Wordsworth, posterity, reading, material inscription, ekphrasis, description, sonnet, portraiture......William Wordsworth, posterity, reading, material inscription, ekphrasis, description, sonnet, portraiture...

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

Energy Technology Data Exchange (ETDEWEB)

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

International Nuclear Information System (INIS)

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

Directory of Open Access Journals (Sweden)

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.

Colour discrimination and categorisation in Williams syndrome.

Science.gov (United States)

Farran, Emily K; Cranwell, Matthew B; Alvarez, James; Franklin, Anna

2013-10-01

Individuals with Williams syndrome (WS) present with impaired functioning of the dorsal visual stream relative to the ventral visual stream. As such, little attention has been given to ventral stream functions in WS. We investigated colour processing, a predominantly ventral stream function, for the first time in nineteen individuals with Williams syndrome. Colour discrimination was assessed using the Farnsworth-Munsell 100 hue test. Colour categorisation was assessed using a match-to-sample test and a colour naming task. A visual search task was also included as a measure of sensitivity to the size of perceptual colour difference. Results showed that individuals with WS have reduced colour discrimination relative to typically developing participants matched for chronological age; performance was commensurate with a typically developing group matched for non-verbal ability. In contrast, categorisation was typical in WS, although there was some evidence that sensitivity to the size of perceptual colour differences was reduced in this group. Copyright © 2013 Elsevier Ltd. All rights reserved.

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

Science.gov (United States)

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.

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

Science.gov (United States)

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.

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

Science.gov (United States)

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

The world in eighteen lessons: Christopher Williams

NARCIS (Netherlands)

Berrebi, S.

2011-01-01

Conceptual photographer Christopher Williams is a real artist's artist. Ever since he moved to Germany, his measured work, which both reveres and examines the art of photography, has more and more easily found its way into European art institutes, such as this spring at Museum Dhondt-Dhaenens in

Analysis of Speech Fluency in Williams Syndrome

Science.gov (United States)

Rossi, Natalia Freitas; Sampaio, Adriana; Goncalves, Oscar F.; Giacheti, Celia Maria

2011-01-01

Williams syndrome (WS) is a neurodevelopmental genetic disorder, often referred as being characterized by dissociation between verbal and non-verbal abilities, although the number of studies disputing this proposal is emerging. Indeed, although they have been traditionally reported as displaying increased speech fluency, this topic has not been…

Attribution of Negative Intention in Williams Syndrome

Science.gov (United States)

Godbee, Kali; Porter, Melanie A.

2013-01-01

People with Williams syndrome (WS) are said to have sociable and extremely trusting personalities, approaching strangers without hesitation. This study investigated whether people with WS are less likely than controls to attribute negative intent to others when interpreting a series of ambiguous pictures. This may, at least partially, explain…

Autistic disorder in patients with Williams-Beuren syndrome: a reconsideration of the Williams-Beuren syndrome phenotype.

Science.gov (United States)

Tordjman, Sylvie; Anderson, George M; Botbol, Michel; Toutain, Annick; Sarda, Pierre; Carlier, Michèle; Saugier-Veber, Pascale; Baumann, Clarisse; Cohen, David; Lagneaux, Céline; Tabet, Anne-Claude; Verloes, Alain

2012-01-01

Williams-Beuren syndrome (WBS), a rare developmental disorder caused by deletion of contiguous genes at 7q11.23, has been characterized by strengths in socialization (overfriendliness) and communication (excessive talkativeness). WBS has been often considered as the polar opposite behavioral phenotype to autism. Our objective was to better understand the range of phenotypic expression in WBS and the relationship between WBS and autistic disorder. The study was conducted on 9 French individuals aged from 4 to 37 years old with autistic disorder associated with WBS. Behavioral assessments were performed using Autism Diagnostic Interview-Revised (ADI-R) and Autism Diagnostic Observation Schedule (ADOS) scales. Molecular characterization of the WBS critical region was performed by FISH. FISH analysis indicated that all 9 patients displayed the common WBS deletion. All 9 patients met ADI-R and ADOS diagnostic criteria for autism, displaying stereotypies and severe impairments in social interaction and communication (including the absence of expressive language). Additionally, patients showed improvement in social communication over time. The results indicate that comorbid autism and WBS is more frequent than expected and suggest that the common WBS deletion can result in a continuum of social communication impairment, ranging from excessive talkativeness and overfriendliness to absence of verbal language and poor social relationships. Appreciation of the possible co-occurrence of WBS and autism challenges the common view that WBS represents the opposite behavioral phenotype of autism, and might lead to improved recognition of WBS in individuals diagnosed with autism.

William Barlow and the Determination of Atomic Arrangement in Crystals.

Science.gov (United States)

Mauskopf, Seymour H

2015-04-01

William Barlow (1845-1934) was an important if unconventional scientist, known for having developed the 'closest-packing' atomic models of crystal structure. He resumed an early nineteenth-century tradition of utilizing crystallographical and chemical data to determine atomic arrangements in crystals. This essay recounts Barlow's career and scientific activity in three parts: (a) His place in the tradition of determining atomic arrangement in context of this earlier tradition and of contemporaneous developments of crystallography and chemistry, (b) his unconventional career, and (c) the 'success' of his program to determine atomic arrangements in crystals and its influence on the work of William Lawrence Bragg.

77 FR 63308 - J. William Foley Incorporated v. United Illuminating Company; Notice of Complaint

Science.gov (United States)

2012-10-16

... DEPARTMENT OF ENERGY Federal Energy Regulatory Commission [Docket No. EL12-106-000] J. William Foley Incorporated v. United Illuminating Company; Notice of Complaint Take notice that on October 5...) and 825(e), J. William Foley Incorporated (Complainant) filed a formal complaint against United...

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

Science.gov (United States)

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.

Application of Choi—Williams Reduced Interference Time Frequency Distribution to Machinery Diagnostics

Directory of Open Access Journals (Sweden)

Howard A. Gaberson

1995-01-01

Full Text Available This article discusses time frequency analysis of machinery diagnostic vibration signals. The short time Fourier transform, the Wigner, and the Choi–Williams distributions are explained and illustrated with test cases. Examples of Choi—Williams analyses of machinery vibration signals are presented. The analyses detect discontinuities in the signals and their timing, amplitude and frequency modulation, and the presence of different components in a vibration signal.

Increased overall cortical connectivity with syndrome specific local decreases suggested by atypical sleep-EEG synchronization in Williams syndrome.

Science.gov (United States)

Gombos, Ferenc; Bódizs, Róbert; Kovács, Ilona

2017-07-21

Williams syndrome (7q11.23 microdeletion) is characterized by specific alterations in neurocognitive architecture and functioning, as well as disordered sleep. Here we analyze the region, sleep state and frequency-specific EEG synchronization of whole night sleep recordings of 21 Williams syndrome and 21 typically developing age- and gender-matched subjects by calculating weighted phase lag indexes. We found broadband increases in inter- and intrahemispheric neural connectivity for both NREM and REM sleep EEG of Williams syndrome subjects. These effects consisted of increased theta, high sigma, and beta/low gamma synchronization, whereas alpha synchronization was characterized by a peculiar Williams syndrome-specific decrease during NREM states (intra- and interhemispheric centro-temporal) and REM phases of sleep (occipital intra-area synchronization). We also found a decrease in short range, occipital connectivity of NREM sleep EEG theta activity. The striking increased overall synchronization of sleep EEG in Williams syndrome subjects is consistent with the recently reported increase in synaptic and dendritic density in stem-cell based Williams syndrome models, whereas decreased alpha and occipital connectivity might reflect and underpin the altered microarchitecture of primary visual cortex and disordered visuospatial functioning of Williams syndrome subjects.

Notas no percurso: linguagem musical e Síndrome de Williams/Notes on the way: musical language and Williams Syndrome

Directory of Open Access Journals (Sweden)

Paulo Sérgio Souza Junior

2009-12-01

Full Text Available O presente artigo versa sobre uma síndrome em particular chamada Síndrome de Williams-Beuren. Esta síndrome possui uma relação específica com a música, relação esta que estará no fulcro de nossas preocupações durante as páginas que se seguem. Num primeiro momento faremos uma apresentação da síndrome e de suas singularidades; em seguida nos debruçaremos sobre a especificidade que aqui está em questão, ou seja, a relação da síndrome com o universo sonoro; por fim, traremos um caso com o qual tivemos contato envolvendo a educação musical em contexto da síndrome, e discutiremos a respeito dos elementos que esse contato nos trouxe para pensar essa curiosa e relevante relação entre arte-educação e o desenvolvimento neuropsicológico atípico. The present article is about a particular syndrome, called Williams-Beuren. This syndrome has a very specific relationship with music, which will be at the heart of our concerns over the following pages. Initially we will make a presentation of the syndrome and its singularities. Then we will focus on the uniqueness of the correlation between Williams-Beuren and the musical world. Finally, we will discuss a case which involves musical education in the context of this syndrome, and discuss about the elements, brought by that contact, that are useful to think about this curious and relevant relationship between art education and atypical neuropsychological development.

Co-authoring History: Montpellier, the Vendée, and the Co-authorship of the Sources

Directory of Open Access Journals (Sweden)

István M. Szijártó

2013-10-01

Full Text Available The discourses of literature and history are generally regarded as two distinct genres. This essay sets out to investigate the use of fictitious, that is, the invented, as well as real elements, in addition to narrative tools in some literary and historical texts to examine whether there is evidence for a fundamental difference between them in this respect. In the first half of the article, from the juxtaposition of Merle’s historical novel, En nos vertes années, to Le Roy Ladurie’s The Beggar and the Professor, we shall see that real and fictitious elements are also interwoven in Merle’s text, just as history uses fictitious elements, necessarily and tacitly, or, in some works, in a rather provocative way. In the second half of this essay, in examining literary and historical narratives of the counter-revolution in the Vendée, it will become evident that historians also use the same narrative techniques as writers to orientate readers. While these findings would confound the normative distinction between history and literature, we cannot, however, finally conclude that there is no fundamental difference between literary fiction and history. Arguing against Alun Munslow, who claims in Authoring the Past that “’doing history’ is an authorial activity,” this present article tries to argue that, while in many aspects writing history is indistinguishable from writing fiction, the historian has co-authors: the sources themselves may enter the process of writing history. This is a conclusion that emerges from the analysis of Simon Schama’s Citizens. His text about the revolt in the Vendée points to a potential advantage of history when compared to literary fiction: historians may feel obliged to change their original point of view under the burden of the fact they themselves have enumerated—something we can call the latent but inherent co-authorship of the sources in historical narratives.

77 FR 58819 - Notice of Submission for OMB Review; Federal Student Aid; William D. Ford Federal Direct Loan...

Science.gov (United States)

2012-09-24

... DEPARTMENT OF EDUCATION Notice of Submission for OMB Review; Federal Student Aid; William D. Ford... William D. Ford Federal Direct Loan Program will use this form to request forbearance on their loans when... of Collection: William D. Ford Federal Direct Loan Program General Forbearance Request. OMB Control...

77 FR 29988 - Notice of Submission for OMB Review; Federal Student Aid; William D. Ford Federal Direct Loan...

Science.gov (United States)

2012-05-21

... DEPARTMENT OF EDUCATION Notice of Submission for OMB Review; Federal Student Aid; William D. Ford... borrowers in the William D. Ford Federal Direct Loan (Direct Loan) and Federal Family Education Loan (FFEL... of Collection: William D. Ford Federal Direct Loan Program Deferment Request Forms. OMB Control...

Native Sons: A Critical Study of Twentieth-Century Negro American Authors.

Science.gov (United States)

Margolies, Edward

This analysis of 20th-century Negro literature contains chapters discussing 16 authors: (1) "The First Forty Years: 1900-1940," including W. E. B. DuBois, Charles W. Chesnutt, James W. Johnson, Paul L. Dunbar, Langston Hughes, Jean Toomer, Claude McKay, and Countee Cullen; (2) "Migration: William Attaway and 'Blood on the Forge'"; (3) "Richard…

Clinical utility of an array comparative genomic hybridization analysis for Williams syndrome.

Science.gov (United States)

Yagihashi, Tatsuhiko; Torii, Chiharu; Takahashi, Reiko; Omori, Mikimasa; Kosaki, Rika; Yoshihashi, Hiroshi; Ihara, Masahiro; Minagawa-Kawai, Yasuyo; Yamamoto, Junichi; Takahashi, Takao; Kosaki, Kenjiro

2014-11-01

To reveal the relation between intellectual disability and the deleted intervals in Williams syndrome, we performed an array comparative genomic hybridization analysis and standardized developmental testing for 11 patients diagnosed as having Williams syndrome based on fluorescent in situ hybridization testing. One patient had a large 4.2-Mb deletion spanning distally beyond the common 1.5-Mb intervals observed in 10/11 patients. We formulated a linear equation describing the developmental age of the 10 patients with the common deletion; the developmental age of the patient with the 4.2-Mb deletion was significantly below the expectation (developmental age = 0.51 × chronological age). The large deletion may account for the severe intellectual disability; therefore, the use of array comparative genomic hybridization may provide practical information regarding individuals with Williams syndrome. © 2014 Japanese Teratology Society.

Shepard Award Winners, Part 2: Dr. Tracie Williams

Centers for Disease Control (CDC) Podcasts

2009-07-29

This podcast highlights the accomplishments of Dr. Tracie Williams, recipient of the prestigious 2009 CDC Charles C. Shepard Award.  Created: 7/29/2009 by Centers for Disease Control and Prevention (CDC).   Date Released: 7/29/2009.

Sir William Jenner (1815-1898) and Queen Victoria.

Science.gov (United States)

Seddon, David; Queen Victoria

2004-08-01

This biographical note outlines the work of Sir William Jenner (1815-1898), court physician 1861-93 and President of the Royal College of Physicians 1881-8. He was the first to distinguish between typhoid and typhus both clinically and pathologically.

The contribution of CLIP2 haploinsufficiency to the clinical manifestations of the Williams-Beuren syndrome.

Science.gov (United States)

Vandeweyer, Geert; Van der Aa, Nathalie; Reyniers, Edwin; Kooy, R Frank

2012-06-08

Williams-Beuren syndrome is a rare contiguous gene syndrome, characterized by intellectual disability, facial dysmorphisms, connective-tissue abnormalities, cardiac defects, structural brain abnormalities, and transient infantile hypercalcemia. Genes lying telomeric to RFC2, including CLIP2, GTF2I and GTF2IRD1, are currently thought to be the most likely major contributors to the typical Williams syndrome cognitive profile, characterized by a better-than-expected auditory rote-memory ability, a relative sparing of language capabilities, and a severe visual-spatial constructive impairment. Atypical deletions in the region have helped to establish genotype-phenotype correlations. So far, however, hardly any deletions affecting only a single gene in the disease region have been described. We present here two healthy siblings with a pure, hemizygous deletion of CLIP2. A putative role in the cognitive and behavioral abnormalities seen in Williams-Beuren patients has been suggested for this gene on the basis of observations in a knock-out mouse model. The presented siblings did not show any of the clinical features associated with the syndrome. Cognitive testing showed an average IQ for both and no indication of the Williams syndrome cognitive profile. This shows that CLIP2 haploinsufficiency by itself does not lead to the physical or cognitive characteristics of the Williams-Beuren syndrome, nor does it lead to the Williams syndrome cognitive profile. Although contribution of CLIP2 to the phenotype cannot be excluded when it is deleted in combination with other genes, our results support the hypothesis that GTF2IRD1 and GTF2I are the main genes causing the cognitive defects associated with Williams-Beuren syndrome. Copyright © 2012 The American Society of Human Genetics. Published by Elsevier Inc. All rights reserved.

Brief Report: Developing Spatial Frequency Biases for Face Recognition in Autism and Williams Syndrome

Science.gov (United States)

Leonard, Hayley C.; Annaz, Dagmara; Karmiloff-Smith, Annette; Johnson, Mark H.

2011-01-01

The current study investigated whether contrasting face recognition abilities in autism and Williams syndrome could be explained by different spatial frequency biases over developmental time. Typically-developing children and groups with Williams syndrome and autism were asked to recognise faces in which low, middle and high spatial frequency…

Denigrating Carl Rogers: William Coulson's Last Crusade.

Science.gov (United States)

Kirschenbaum, Howard

1991-01-01

Reviews William Coulson's assertions that Carl Rogers, Abraham Maslow, and he initiated the humanistic education field, that Rogers repudiated his philosophy late in life, and that they owe the nation's parents an apology. Argues that these charges are groundless and provides examples and quotations from Rogers' later writings to show how Rogers…

Fears, Hyperacusis and Musicality in Williams Syndrome

Science.gov (United States)

Blomberg, Stefan; Rosander, Michael; Andersson, Gerhard

2006-01-01

The study investigated the prevalence of fear and hyperacusis and the possible connections between fear, hyperacusis and musicality in a Swedish sample of individuals with Williams syndrome (WS). The study included 38 individuals and a cross-sectional design, with no matched control group. Two persons, who knew the participant well, completed a…

Executive Functions in Individuals with Williams Syndrome

Science.gov (United States)

Menghini, D.; Addona, F.; Costanzo, F.; Vicari, S.

2010-01-01

Background: The present study was aimed at investigating working memory (WM) and executive functions capacities in individuals with Williams syndrome (WS) as compared with mental-age matched typically developing (TD) children. Method: In order to serve the study goal, a sizeable battery of tasks tapping WM as well as attention, memory, planning,…

Läbi legendide William Shakespeare'i poole / Maris Peters

Index Scriptorium Estoniae

Peters, Maris

2010-01-01

Tutvustus: Wells, Stanley. Kas on tõsi, et Shakespeare ...? / tõlkinud Maris Peters. Tallinn : Argo, 2010. Raamat William Shakespeare kohta käivatest legendidest, kuuldustest ja teooriatest ning tema teoste autorsusest

Charles William Lacaillade. Biologist, Parasitologist, Educator, and Mentor.

Science.gov (United States)

Imperato, Pascal James

2017-02-01

Charles William Lacaillade (1904-1978) was an eminent biologist in the middle decades of the twentieth century. He was born in Lawrence, Massachusetts of parents whose ancestors were French Canadians. His father, also named Charles William Lacaillade, was a dentist who graduated from Tufts University School of Dentistry in 1898. His mother, Elodia Eno, came from a family of very successful businessmen. Lacaillade was the third of six children. His two older brothers, Harold Carleton and Hector Eno, both graduated from the University of Louisville, School of Dentistry, while his younger brother, Lawrence, became a businessman. His sister, Luemma, married Dr. Henry Steadman, a veterinarian, while his youngest sister, Gloria, married a U.S. Army officer, Lieutenant Colonel Victor Anido. Lacaillade received his MS and PhD degrees in biology and zoology from Harvard University. He then became a fellow at The Rockefeller Institute for Medical Research. At both institutions, he studied under some of the most eminent biological scientists of the time. These included Rudolf W. Glaser, George Howard Parker, Theobald Smith, Carl TenBroeck, and William Morton Wheeler. At the Rockefeller Institute, he co-discovered the vector and mode of transmission of Eastern Equine Encephalomyelitis. This discovery, and the research he conducted with Rudolf W. Glaser, quickly established him as an outstanding biological researcher. However, a change in leadership at the Rockefeller Institute resulted in research priorities being given to the disciplines of general physiology, physical chemistry, and nutrition. This shift in the research agenda away from the biological sciences precluded career advancement at the Rockefeller Institute for post-doctoral fellows like Lacaillade. It was the height of the Great Depression, and even biologists with terminal doctoral degrees found it difficult to find positions. In 1935, Lacaillade accepted a position as an assistant in biology at St. John

William and Caroline Herschel pioneers in late 18th-century astronomy

CERN Document Server

Hoskin, Michael

2013-01-01

This beautifully structured book presents the essentials of William and Caroline Herschel's pioneering achievements in late 18th-century astronomy. Michael Hoskin shows that William Herschel was the first observational cosmologist and one of the first observers to attack the sidereal universe beyond the solar system:Herschel built instruments far better than any being used at the royal observatory. Aided by his sister Caroline, he commenced a great systematic survey that led to his discovery of Uranus in 1781.Unlike observers before him, whose telescopes did not reveal them as astronomical obj

Herbert Hoover and William Shakespeare: Two Writers on Ethics.

Science.gov (United States)

Somers, Margaret L.

1979-01-01

Outlines the ways in which Herbert Hoover and William Shakespeare wrote about professional ethics (for engineers and kings, respectively) using the writing techniques of concreteness, audience awareness, and development by induction. (TJ)

‘Excess of It’: Reviewing 'William Shakespeare’s Long Lost First Play (abridged'

Directory of Open Access Journals (Sweden)

Ronan James Hatfull

2016-10-01

Full Text Available It is timely in 2016, the 400th anniversary of Shakespeare’s death, to consider his legacy as a figure ingrained within popular culture. This critical review will investigate one of the chief exponents and parodists of the dichotomy which Shakespeare symbolises between supposed ‘highbrow’ and ‘lowbrow’ culture: the Reduced Shakespeare Company, a comedic theatre troupe who, to use their own slogan of droll self-deprecation, have been ‘reducing expectations since 1981’. The review will investigate the company’s most recent and tenth production, William Shakespeare’s Long Lost First Play (abridged, as a template for considering Shakespearean parody, focusing on the contemporary process of adapting and condensing Shakespeare’s texts within a populist context. Debuted at the Folger Shakespeare Library in April 2016, the play was first performed in the United Kingdom in August 2016 as part of the Edinburgh Festival Fringe. It is those performances upon which this review focuses. It will also use primary material drawn from live interviews and rehearsal observations conducted with Reed Martin and Austin Tichenor, the company’s managing partners, co-directors, co-writers and performers.

78 FR 45515 - Agency Information Collection Activities; Comment Request; William D. Ford Federal Direct Loan...

Science.gov (United States)

2013-07-29

... DEPARTMENT OF EDUCATION [Docket No.: ED-2013-ICCD-0099] Agency Information Collection Activities; Comment Request; William D. Ford Federal Direct Loan Program Repayment Plan Selection Form AGENCY... notice will be considered public records. Title of Collection: William D. Ford Federal Direct Loan...

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

Science.gov (United States)

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.

Psycholinguistic Abilities of Children with Williams Syndrome

Science.gov (United States)

Rossi, Natalia F.; Heinze, Elena Garayzabal; Giacheti, Celia M.; Goncalves, Oscar F.; Sampaio, Adriana

2012-01-01

The objective of this study was to investigate the psycholinguistic abilities of children with Williams syndrome (WS) and typically developing children using the Illinois Test of Psycholinguistic Abilities (ITPA). Performance on the ITPA was analysed in a group with WS (N=20, mean age=8.5 years, SD=1.62) and two typically developing groups,…

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

Science.gov (United States)

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.

78 FR 52169 - Agency Information Collection Activities; Comment Request; William D. Ford Federal Direct Loan...

Science.gov (United States)

2013-08-22

... DEPARTMENT OF EDUCATION [Docket No. ED-2013-ICCD-0109] Agency Information Collection Activities; Comment Request; William D. Ford Federal Direct Loan (Direct Loan) Program Federal Direct PLUS Loan Master... to this notice will be considered public records. Title of Collection: William D. Ford Federal Direct...

Williams syndrome deletions and duplications: Genetic windows to understanding anxiety, sociality, autism, and schizophrenia.

Science.gov (United States)

Crespi, Bernard J; Procyshyn, Tanya L

2017-08-01

We describe and evaluate an integrative hypothesis for helping to explain the major neurocognitive features of individuals with Williams syndrome region deletions and duplications. First, we demonstrate how the cognitive differences between Williams syndrome individuals, individuals with duplications of this region, and healthy individuals parallel the differences between individuals subject to effects of increased or decreased oxytocin. Second, we synthesize evidence showing that variation in expression of the gene GTF2I (General Transcription Factor II-I) underlies the primary social phenotypes of Williams syndrome and that common genetic variation in GTF2I mediates oxytocin reactivity, and its correlates, in healthy populations. Third, we describe findings relevant to the hypothesis that the GTF2I gene is subject to parent of origin effects whose behavioral expression fits with predictions from the kinship theory of genomic imprinting. Fourth, we describe how Williams syndrome can be considered, in part, as an autistic syndrome of Lorna Wing's 'active-but-odd' autism subtype, in contrast to associations of duplications with both schizophrenia and autism. Copyright © 2017 Elsevier Ltd. All rights reserved.

Williams syndrome and mature B-Leukemia: A random association?

Science.gov (United States)

Decimi, Valentina; Fazio, Grazia; Dell'Acqua, Fabiola; Maitz, Silvia; Galbiati, Marta; Rizzari, Carmelo; Biondi, Andrea; Cazzaniga, Giovanni; Selicorni, Angelo

2016-12-01

Williams syndrome (WBS) is a rare neurodevelopmental disorder with specific phenotypic characteristics and cardiac abnormalities, but is not considered as a cancer predisposing condition. However, in rare cases, malignancies have been described in patients with WBS, with hematologic cancer (mainly Burkitt Lymphoma and Acute Lymphoblastic Leukemia) as the most represented. We report here the case of a boy with WS and B-NHL. This is the unique case within the large cohort of patients (n = 117) followed in our institution for long time (mean clinical follow-up, 13 years). We herewith propose that the BCL7B gene, located in the chromosomal region commonly deleted in Williams syndrome, could potentially have a role in this particular association. Copyright © 2016 Elsevier Masson SAS. All rights reserved.

Ischemic Stroke in Williams-Beuren Syndrome: A Case Report

Directory of Open Access Journals (Sweden)

Wei-Der Lee

2009-04-01

Full Text Available A 15-year-old girl was admitted because of an acute onset of facial palsy and right hemiparesis. The patient had a history of moderate mental retardation and developmental delay. On admission, her vital signs were stable, except for high blood pressure. Magnetic resonance imaging demonstrated an infarct involving the left internal capsule and putamen. Because of the patient's young age, an extensive stroke survey was performed. Williams-Beuren syndrome was finally confirmed by fluorescent in situ hybridization. Compared with the previously reported cases, no evidence of cerebral arterial stenosis or cardiac abnormalities was found by noninvasive imaging techniques. Because Williams-Beuren syndrome is a complex, multiple congenital anomaly syndrome with prominent cardiovascular features, regular assessment and antihypertensive treatment are necessary to minimize the lifelong cardiovascular risk in patients with this syndrome.

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

Science.gov (United States)

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

Experiences of Bullying for Individuals with Williams Syndrome

Science.gov (United States)

Fisher, Marisa H.; Lough, Emma; Griffin, Megan M.; Lane, Laurel A.

2017-01-01

Background: Individuals with intellectual disability experience high rates of bullying, but it is not known how people with specific syndromes, such as Williams syndrome (WS), experience and respond to bullying. Given their behavioral profile, including hypersociability and heightened anxiety, and their risk for experiencing other forms of…

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

Science.gov (United States)

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.

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

Directory of Open Access Journals (Sweden)

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.

Conceptualizing neurodevelopmental disorders through a mechanistic understanding of fragile X syndrome and Williams syndrome.

Science.gov (United States)

Fung, Lawrence K; Quintin, Eve-Marie; Haas, Brian W; Reiss, Allan L

2012-04-01

The overarching goal of this review is to compare and contrast the cognitive-behavioral features of fragile X syndrome (FraX) and Williams syndrome and to review the putative neural and molecular underpinnings of these features. Information is presented in a framework that provides guiding principles for conceptualizing gene-brain-behavior associations in neurodevelopmental disorders. Abnormalities, in particular cognitive-behavioral domains with similarities in underlying neurodevelopmental correlates, occur in both FraX and Williams syndrome including aberrant frontostriatal pathways leading to executive function deficits, and magnocellular/dorsal visual stream, superior parietal lobe, inferior parietal lobe, and postcentral gyrus abnormalities contributing to deficits in visuospatial function. Compelling cognitive-behavioral and neurodevelopmental contrasts also exist in these two disorders, for example, aberrant amygdala and fusiform cortex structure and function occurring in the context of contrasting social behavioral phenotypes, and temporal cortical and cerebellar abnormalities potentially underlying differences in language function. Abnormal dendritic development is a shared neurodevelopmental morphologic feature between FraX and Williams syndrome. Commonalities in molecular machinery and processes across FraX and Williams syndrome occur as well - microRNAs involved in translational regulation of major synaptic proteins; scaffolding proteins in excitatory synapses; and proteins involved in axonal development. Although the genetic variations leading to FraX and Williams syndrome are different, important similarities and contrasts in the phenotype, neurocircuitry, molecular machinery, and cellular processes in these two disorders allow for a unique approach to conceptualizing gene-brain-behavior links occurring in neurodevelopmental disorders.

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

Science.gov (United States)

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.

Anxiety and Repetitive Behaviours in Autism Spectrum Disorders and Williams Syndrome: A Cross-Syndrome Comparison

Science.gov (United States)

Rodgers, Jacqui; Riby, Deborah M.; Janes, Emily; Connolly, Brenda; McConachie, Helen

2012-01-01

Children with Autism Spectrum Disorder or Williams syndrome are vulnerable to anxiety. The factors that contribute to this risk remain unclear. This study compared anxiety in autism spectrum disorder and Williams Syndrome and examined the relationship between repetitive behaviours and anxiety. Thirty-four children with autism and twenty children…

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

Science.gov (United States)

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.

Electrophysiological Correlates of Semantic Processing in Williams Syndrome

Science.gov (United States)

Pinheiro, Ana P.; Galdo-Alvarez, Santaigo; Sampaio, Adriana; Niznikiewicz, Margaret; Goncalves, Oscar F.

2010-01-01

Williams syndrome (WS), a genetic neurodevelopmental disorder due to microdeletion in chromosome 7, has been described as a syndrome with an intriguing socio-cognitive phenotype. Cognitively, the relative preservation of language and face processing abilities coexists with severe deficits in visual-spatial tasks, as well as in tasks involving…

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

Science.gov (United States)

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.

Science.gov (United States)

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.

Repetition Priming in Adults with Williams Syndrome: Age-Related Dissociation between Implicit and Explicit Memory

Science.gov (United States)

Krinsky-McHale, Sharon J.; Kittler, Phyllis; Brown, W. Ted; Jenkins, Edmund C.; Devenny, Darlynne A.

2005-01-01

We examined implicit and explicit memory in adults with Williams syndrome. An age-related dissociation was found; repetition priming (reflecting implicit memory) did not show change with age, but free recall (reflecting explicit memory) was markedly reduced. We also compared the performance of adults with Williams syndrome to adults with Down…

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

Science.gov (United States)

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.

Williams syndrome predisposes to vascular stiffness modified by antihypertensive use and copy number changes in NCF1.

Science.gov (United States)

Kozel, Beth A; Danback, Joshua R; Waxler, Jessica L; Knutsen, Russell H; de Las Fuentes, Lisa; Reusz, Gyorgy S; Kis, Eva; Bhatt, Ami B; Pober, Barbara R

2014-01-01

Williams syndrome is caused by the deletion of 26 to 28 genes, including elastin, on human chromosome 7. Elastin insufficiency leads to the cardiovascular hallmarks of this condition, namely focal stenosis and hypertension. Extrapolation from the Eln(+/-) mouse suggests that affected people may also have stiff vasculature, a risk factor for stroke, myocardial infarction, and cardiac death. NCF1, one of the variably deleted Williams genes, is a component of the nicotinamide adenine dinucleotide phosphate (NADPH) oxidase complex and is involved in the generation of oxidative stress, making it an interesting candidate modifier for vascular stiffness. Using a case-control design, vascular stiffness was evaluated by pulse wave velocity in 77 Williams cases and matched controls. Cases had stiffer conducting vessels than controls (PWilliams syndrome. Pulse wave velocity increased with age at comparable rates in cases and controls, and although the degree of vascular stiffness varied, it was seen in both hypertensive and normotensive Williams participants. Use of antihypertensive medication and extension of the Williams deletion to include NCF1 were associated with protection from vascular stiffness. These findings demonstrate that vascular stiffness is a primary vascular phenotype in Williams syndrome and that treatment with antihypertensives or agents inhibiting oxidative stress may be important in managing patients with this condition, potentially even those who are not overtly hypertensive.

Adaptive Functioning in Williams Syndrome: A Systematic Review

Science.gov (United States)

Brawn, Gabrielle; Porter, Melanie

2018-01-01

Literature on the level of adaptive functioning and relative strengths and weaknesses in functioning of individuals with Williams syndrome (WS) was reviewed. The electronic databases PsycINFO, PubMed, Expanded Academic, Web of Science, Scopus and ProQuest were searched electronically for relevant articles and dissertations using the search terms…

Potential Beneficial Effects of Tulbaghia violacea William Henry ...

African Journals Online (AJOL)

Tulbaghia violacea William Henry Harvey (Harv. Alliaceae) is a small bulbous herb belonging to the family Alliaceae. It is used in South Africa to treat fever, colds, asthma, paralysis, and hypertension. Meanwhile, cardiovascular disease accounts for about 30 % of total global death, with most of these deaths occurring in low ...

77 FR 58818 - Notice of Proposed Information Collection Requests; Federal Student Aid; William D. Ford Federal...

Science.gov (United States)

2012-09-24

... DEPARTMENT OF EDUCATION Notice of Proposed Information Collection Requests; Federal Student Aid; William D. Ford Federal Direct Loan Program Regulations-- Servicemembers Civil Relief Act SUMMARY: Upon a... in response to this notice will be considered public records. Title of Collection: William D. Ford...

Estrutura e significado em "Uma rosa para Emily", de William Faulkner

Directory of Open Access Journals (Sweden)

Carlos Daghlian

2004-01-01

Full Text Available Trata-se de uma análise do consagrado conto "Uma Rosa para Emily", de William Faulkner, voltada para alguns dos principais aspectos de sua estrutura. Após considerarmos o enredo, discutimos a construção das personagens, com destaque para a protagonista, fazendo um levantamento e comentários sobre possíveis fontes de inspiração, destacando, entre outras, aspectos da biografia da poeta Emily Dickinson, a ficção e a poesia de E. A. Poe, romances de Charles Dickens e Henry James, o conto de Sherwood Anderson e a poesia de William Blake, Emily Dickinson, Robert Browning e John Crowe Ransom, acrescentando paralelos com o conto "Bartleby, o escrivão", de Herman Melville. Analisamos, então, o foco narrativo, os símbolos e o significado, ressaltando aqui o desenvolvimento temático da narrativa.This is an analysis of the well-known short story "A Rose for Emily," by William Faulkner, concentrating on some of the main aspects of its structure. A consideration of the plot is followed by a discussion of characterization, with emphasis on the protagonist, by means of a survey and comments on possible sources of inspiration including, among others, aspects of Emily Dickinson's biography, E. A. Poe's fiction and poetry, novels by Charles Dickens and Henry James, Sherwood Anderson's short stories, and the poetry of William Blake, Emily Dickinson, Robert Browning, and John Crowe Ransom, in addition to Herman Melville's short story "Bartleby, the Scrivener." The narrative focus, symbolism and meaning, stressing the thematic development of the narrative, are then analyzed.

Bayesian stock assessment of Pacific herring in Prince William Sound, Alaska.

Science.gov (United States)

Muradian, Melissa L; Branch, Trevor A; Moffitt, Steven D; Hulson, Peter-John F

2017-01-01

The Pacific herring (Clupea pallasii) population in Prince William Sound, Alaska crashed in 1993 and has yet to recover, affecting food web dynamics in the Sound and impacting Alaskan communities. To help researchers design and implement the most effective monitoring, management, and recovery programs, a Bayesian assessment of Prince William Sound herring was developed by reformulating the current model used by the Alaska Department of Fish and Game. The Bayesian model estimated pre-fishery spawning biomass of herring age-3 and older in 2013 to be a median of 19,410 mt (95% credibility interval 12,150-31,740 mt), with a 54% probability that biomass in 2013 was below the management limit used to regulate fisheries in Prince William Sound. The main advantages of the Bayesian model are that it can more objectively weight different datasets and provide estimates of uncertainty for model parameters and outputs, unlike the weighted sum-of-squares used in the original model. In addition, the revised model could be used to manage herring stocks with a decision rule that considers both stock status and the uncertainty in stock status.

Bayesian stock assessment of Pacific herring in Prince William Sound, Alaska.

Directory of Open Access Journals (Sweden)

Melissa L Muradian

Full Text Available The Pacific herring (Clupea pallasii population in Prince William Sound, Alaska crashed in 1993 and has yet to recover, affecting food web dynamics in the Sound and impacting Alaskan communities. To help researchers design and implement the most effective monitoring, management, and recovery programs, a Bayesian assessment of Prince William Sound herring was developed by reformulating the current model used by the Alaska Department of Fish and Game. The Bayesian model estimated pre-fishery spawning biomass of herring age-3 and older in 2013 to be a median of 19,410 mt (95% credibility interval 12,150-31,740 mt, with a 54% probability that biomass in 2013 was below the management limit used to regulate fisheries in Prince William Sound. The main advantages of the Bayesian model are that it can more objectively weight different datasets and provide estimates of uncertainty for model parameters and outputs, unlike the weighted sum-of-squares used in the original model. In addition, the revised model could be used to manage herring stocks with a decision rule that considers both stock status and the uncertainty in stock status.

Neural Correlates of Amusia in Williams Syndrome

OpenAIRE

Lense, Miriam D.; Dankner, Nathan; Pryweller, Jennifer R.; Thornton-Wells, Tricia A.; Dykens, Elisabeth M.

2014-01-01

Congenital amusia is defined by marked deficits in pitch perception and production. Though historically examined only in otherwise typically developing (TD) populations, amusia has recently been documented in Williams syndrome (WS), a genetic, neurodevelopmental disorder with a unique auditory phenotype including auditory sensitivities and increased emotional responsiveness to music but variable musical skill. The current study used structural T1-weighted magnetic resonance imaging and diffus...

76 FR 13667 - Commercial Furniture Group, Inc., Formerly Known as Falcon Products, Inc., Shelby Williams, Howe...

Science.gov (United States)

2011-03-14

... Furniture Group, Inc., Formerly Known as Falcon Products, Inc., Shelby Williams, Howe and Thonet, Including...., Formerly Known as Falcon Products, Inc., Shelby Williams, Howe and Thonet, Chicago, IL; Amended Certification Regarding Eligibility To Apply for Worker Adjustment Assistance In accordance with Section 223 of...

IMPLIED AUTHOR IN PHILOSOPHICAL NOVELS

Directory of Open Access Journals (Sweden)

Olga Senkāne

2014-10-01

Full Text Available The present article falls within a number of papers about research on specification of philosophical novels. The aim of this article is to analyze author’s function as a narrative category in classical philosophical novels (Franz Kafka "The Trial" (1925, "The Castle" (1926, Jean-Paul Sartre "Nausea" (1938, Hermann Hesse "The Glass Bead Game" (1943, Albert Camus "The Plague" (1947 and a novel of Latvian prose writer Ilze Šķipsna "Neapsolītās zemes" ["Un-Promised Lands"] (1970. The analysis is based on theoretical ideas of structural narratologists Gerard Genette, William Labov, Seymuor Chatman, Wolf Schmid, as well as philosophers Edmund Husserl, Jean-Paul Sartre, Paul Ricouer and semioticians Yuri Lotman (Юрий Лотман and Umberto Eco. The real author can ”enter” the text only indirectly—as an image, with the help of the storyteller, and the way how this ”entry” happens is determined by the narration of the real author or narrative (communication skills of the author. Thus, the author and implied author are functionally different concepts: author as a real person develops the concept idea, his intention is to define the concept under his original vision; narrator, in its turn, communicates with the reader, representing the concept, and his aim is to select appropriate means of communication with regard to reader’s perceptual abilities.

Report of Some Comets: The Discovery of Uranus and Comets by William, Caroline, and John Herschel

Science.gov (United States)

Pasachoff, Jay M.; Olson, R. J. M.

2011-01-01

We report on the discovery and drawings of comets by William, Caroline, and John Herschel. The first discovery, by William Herschel, in 1781 from Bath, published in the Philosophical Transactions of the Royal Society with the title "Report of a Comet," turned out to be Uranus, the first planet ever discovered, Mercury through Saturn having been known since antiquity. William's sister Caroline was given duties of sweeping the skies and turned out to be a discoverer of 8 comets in her own right, in addition to keeping William's notes. Caroline's comets were discovered from Slough between 1786 and 1797. In the process, we also discuss original documents from the archives of the Royal Society and of the Royal Astronomical Society. We conclude by showing comet drawings that we have recently attributed to John Herschel, including Halley's Comet from 1836, recently located in the Ransom Center of the University of Texas at Austin. Acknowledgments: Planetary astronomy at Williams College is supported in part by grant NNX08AO50G from NASA Planetary Astronomy. We thank Peter Hingley of the Royal Astronomical Society and Richard Oram of the Harry Ransom Center of The University of Texas at Austin for their assistance.

Ethical Life in Kierkegaard and Williams

OpenAIRE

Stocker, Barry

2018-01-01

A discussion of how the criticisms of ethical theory in Søren Kierkegaard and Bernard Williams both reinforce each other and also provide some challenges to each other. Despite Williams’ brief and dismissive encounter with Kierkegaard around the reading of a ancient tragedy, both oppose any tendency to see the characters in those tragedies as lacking in agency. Both are consistently concerned with how the individual struggles for some ethical agency and how no individual can be free of the in...

Prince William Sound, Alaska ESI: M_MAMMAL (Marine Mammal Polygons)

Data.gov (United States)

National Oceanic and Atmospheric Administration, Department of Commerce — This data set comprises the Environmental Sensitivity Index (ESI) data for Prince William Sound, Alaska. ESI data characterize estuarine environments and wildlife by...

Librarian co-authors correlated with higher quality reported search strategies in general internal medicine systematic reviews.

Science.gov (United States)

Rethlefsen, Melissa L; Farrell, Ann M; Osterhaus Trzasko, Leah C; Brigham, Tara J

2015-06-01

To determine whether librarian and information specialist authorship was associated with better reported systematic review (SR) search quality. SRs from high-impact general internal medicine journals were reviewed for search quality characteristics and reporting quality by independent reviewers using three instruments, including a checklist of Institute of Medicine Recommended Standards for the Search Process and a scored modification of the Peer Review of Electronic Search Strategies instrument. The level of librarian and information specialist participation was significantly associated with search reproducibility from reported search strategies (Χ(2) = 23.5; P Librarian co-authored SRs had significantly higher odds of meeting 8 of 13 analyzed search standards than those with no librarian participation and six more than those with mentioned librarian participation. One-way ANOVA showed that differences in total search quality scores between all three groups were statistically significant (F2,267 = 10.1233; P librarian or information specialist co-authors are correlated with significantly higher quality reported search strategies. To minimize bias in SRs, authors and editors could encourage librarian engagement in SRs including authorship as a potential way to help improve documentation of the search strategy. Copyright © 2015 Elsevier Inc. All rights reserved.

Edward Christopher Williams and His Impact on Librarianship.

Science.gov (United States)

Latimer, Carlos

Edward Christopher Williams had a major impact on librarianship, not only as the first documented African American to graduate from a library school, but also as a developer of education for librarians and as an active member of the American Library Association (ALA) and the Ohio Library Association. This study used the historical methodology…

Orientation Perception in Williams Syndrome: Discrimination and Integration

Science.gov (United States)

Palomares, Melanie; Landau, Barbara; Egeth, Howard

2009-01-01

Williams Syndrome (WS) is a rare neurodevelopmental disorder, which stems from a genetic deletion on chromosome 7 and causes a profound weakness in visuospatial cognition. Our current study explores how orientation perception may contribute to the visuospatial deficits in WS. In Experiment 1, we found that WS individuals and normal 3-4 year olds…

Health and Social Outcomes in Adults with Williams Syndrome: Findings from Cross-Sectional and Longitudinal Cohorts

Science.gov (United States)

Elison, Sarah; Stinton, Chris; Howlin, Patricia

2010-01-01

Previous studies have investigated trajectories of cognitive, language and adaptive functioning in Williams syndrome (WS) but little is known about how other aspects of the Williams syndrome behavioural phenotype change across the life-span. Therefore, the present study examined age associated changes in a number of different domains of…

Comparison of the acute effects of Tulbaghia violacea William Henry ...

African Journals Online (AJOL)

William Henry Harvey (Alliaceae) on blood pressure and heart rate of ... (WKY), and compare the results obtained with those for adult spontaneously hypertensive rats (SHR). Methods: T. ... have similar secondary metabolites and biological ...

ProRisk : risk analysis instrument : developed for William properties

NARCIS (Netherlands)

van Doorn, W.H.W.; Egeberg, Ingrid; Hendrickx, Kristoff; Kahramaner, Y.; Masseur, B.; Waijers, Koen; Weglicka, K.A.

2005-01-01

This report presents a Risk Analysis Instrument developed for William Properties. Based on the analysis, it appears that the practice of Risk Analysis exists within the organization, yet rather implicit. The Risk Analysis Instrument comes with a package of four components: an activity diagram, a

Doctor William Gunn (1804-1890): From the South Pacific Islands to Chatham Royal Dockyard.

Science.gov (United States)

Biddle, Richard

2016-11-24

Doctor William Gunn had a long and varied career in the Royal Navy. After spending time on anti-slavery patrols along the west coast of Africa, he was posted to the south Pacific. At Pitcairn Island, he treated the inhabitants during an influenza epidemic, proving himself to be a determined and dedicated practitioner. Subsequently, he was appointed head of the medical department at Chatham Royal Dockyard (1859-1865), an appointment that coincided with the final stages of the Royal Navy's transition from sail and wood to steam and iron. The impact of these changes on the health of dockworkers was quickly felt at Chatham, and Gunn found himself in charge during the building of the first iron warship in a royal dockyard. His story thus offers a window through which to observe a practitioner confronting the health issues and medical uncertainties thrown up by technological change in the Victorian era. © The Author(s) 2016.

William Ospina y la búsqueda de la franja amarilla

Directory of Open Access Journals (Sweden)

Víctor Valero Bernal

2016-12-01

Full Text Available El objetivo de este artículo es el de analizar el ensayo «Lo que le falta a Colombia» del escritor William Ospina. En este ensayo Ospina profundiza en los principales problemas sociales y políticos que Colombia atravesaba a finales del s. XX así como en las posibles soluciones que podrían adoptarse. Los planteamientos que emplea el escritor colombiano pueden relacionarse con una gran variedad de conceptos propios del ámbito de la política y la sociología. A lo largo de este trabajo mostraremos las analogías existentes entre el análisis de William Ospina y las ideas de pensadores tan alejados en el tiempo como Thomas Hobbes, Rousseau, John Rawls o Charles Tilly.

The work experience of a patient affected by Williams Syndrome: a pilot project at the Bambino Gesù Children's Hospital.

Science.gov (United States)

De Lorenzo, Francesca; Macchiaiolo, Marina; Carlevaris, Carla Maria; Bartuli, Andrea

2017-05-31

A new approach has been designed at the Bambino Gesù Children's Hospital in Rome aimed at increasing empowerment in Williams Syndrome individuals through tutor-assisted work activities. Williams Syndrome is characterized by a combination of distinguishing physical traits, congenital anomalies, intellectual disabilities, and a specific developmental profile.This manuscript describes the case of a Williams Syndrome patient.There are only few papers in the scientific literature describing interventions targeting improvement in the quality of life of adult Williams Syndrome individuals. Therefore, this experience may prove useful to several patients, their families, and the experts helping them.We described an example of intervention aimed at guiding and facilitating a Williams Syndrome patient within a work environment, taking into consideration the peaks and valleys of these individuals' specific abilities.Based on our results, we also stressed the need to promote a set of projects and initiatives aimed at enhancing as much as possible self-sufficiency and psycho-affective balance in Williams Syndrome individuals, in order to protect their dignity and self-esteem.

La Fondation William et Flora Hewlett | IDRC - International ...

International Development Research Centre (IDRC) Digital Library (Canada)

La Fondation William et Flora Hewlett. http://www.hewlett.org/ · What we do · Funding · Resources · About IDRC. Knowledge. Innovation. Solutions. Careers · Contact Us · Site map. Sign up now for IDRC news and views sent directly to your inbox each month. Subscribe · Copyright · Open access policy · Privacy policy ...

Advocating for Inclusion of Children with Williams Syndrome

Science.gov (United States)

Self, Michelle A.

2010-01-01

The purpose of this study was to describe and explore the experience of inclusion of students with Williams syndrome, a rare genetic condition of a microdeletion on chromosome 7 which has medical, behavior, and cognitive issues. The study was conducted by gaining an understanding from the parents' point of view. The study was twofold. First, the…

A Conversation with William A. Fowler Part II

Science.gov (United States)

Greenberg, John

2005-06-01

Physicist William A.Fowler initiated an experimental program in nuclear astrophysics after World War II. He recalls here the Steady State versus Big Bang controversy and his celebrated collaboration with Fred Hoyle and Geoffrey and Margaret Burbidge on nucleosynthesis in stars. He also comments on the shift away from nuclear physics in universities to large accelerators and national laboratories.

[William Harvey revisited ].

Science.gov (United States)

Steinke, Hubert

2015-07-01

William Harvey's discovery of the circulation of the blood is often described as a product of the Scientific Revolution of the Seventeenth Century. Modern research has, however, shown thatHarvey followed the Aristotelian research tradition and thus tried to reveal the purpose of the organs through examination of various animals. His publication of 1628 has to be read as an argument of natural philosophy, or, more precisely, as a series of linked observations, experiments and philosophical reasonings from which the existence of circulation has to be deduced as a logical consequence. Harvey did not consider experiments as superior to philosophical reasoning nor intended he to create a new system of medicine. He believed in the vitality of the heart and the blood and rejected Francis Bacon's empirism and the mechanistic rationalism of Descartes. Harvey's contribution and originality lied less in his single observations and experiments but in the manner how he linked them with critical reasoning and how he accepted, presented and defended the ensuing radical findings.

Approach to downstream planning for nearshore response and sensitive areas protection outside Prince William Sound, Alaska

International Nuclear Information System (INIS)

DeCola, E.G.; Robertson, T.L.; Robertson, R.; Banta, J.

2004-01-01

This study assessed the need for an oil spill response plan for downstream coastal communities that could be affected by oil spilled from tankers travelling in Prince William Sound, Alaska. For the purpose of oil spill contingency planning, the State of Alaska has been divided into the Kodiak and Cook Inlet sub-areas that are at risk for downstream impacts from a Prince William Sound oil spill. The 1989 Exxon Valdez oil spill provided an example of a worst-case scenario oil spill from a tanker in Prince William Sound, but the oil spill planning system that has evolved in Alaska does not adequately plan for on oil spill that originates in one sub-area of the state, but impacts other sub-areas in the downstream spill path. This study analyzed the gaps that exist in the current response planning system in the Prince William Sound, Cook Inlet and Kodiak sub-areas. A method was proposed to improve the existing response plans so that emergency response teams are better prepared to manage cross-boundary oil spills originating in Prince William Sound. The proposed method focuses on nearshore response and sensitive areas protection for coastlines and communities that are at risk for oil spills from a tanker travelling the Trans-Alaska Pipeline System (TAPS). 11 refs., 3 figs

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

Energy Technology Data Exchange (ETDEWEB)

Kim, Yoon Kyung; Lee, Kyung Soo; Chong, Semin; Chung, Myung Jin; Yi, Chin A; Kim, Ha Young [Samsung Medical Center, Sungkyunkwan University School of Medicine, Department of Radiology and Center for Imaging Science, Seoul (Korea); Chung, Man Pyo [Samsung Medical Center, Sungkyunkwan University School of Medicine, Division of Pulmonary and Critical Care Medicine, Department of Medicine, Seoul (Korea); Han, Joungho [Samsung Medical Center, Sungkyunkwan University School of Medicine, Department of Pathology, Seoul (Korea)

2007-12-15

We tried to assess retrospectively thin-section CT findings of Churg-Strauss syndrome (CSS) in 25 patients and to compare these findings with clinical and histopathologic findings. Of 25 patients, 19 (76%) had parenchymal abnormalities at CT; small nodules (n = 12; 63%), ground-glass opacity (n = 10; 53%), bronchial wall thickening (n = 10; 53%), and consolidation (n = 8; 42%). Parenchymal abnormalities (n = 19) were categorizable as an airway pattern in 11 and an airspace pattern in eight. Patients with an airway pattern (n = 5) had obstructive (n = 3) or combined (n = 2) PFT results, whereas those with an airspace pattern (n = 4) had restrictive (n = 3) or obstructive (n = 1) results. Parenchymal opacities at CT corresponded histologically to areas of eosinophilic pneumonia, necrotizing granulomas, and granulomatous vasculitis; small nodules to eosinophilic bronchiolitis and peribronchiolar vasculitis; and bronchial wall thickening to airway wall eosinophil and lymphocyte infiltration. Patients with airspace pattern responded more readily to treatment than those with airway pattern. CT shows lung parenchymal abnormalities in about three-quarters of CSS patients and these abnormalities can be categorized as airspace or airway patterns. This classification helps predict PFT data, underlying histopathology, and treatment response. (orig.)

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

International Nuclear Information System (INIS)

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.

Science.gov (United States)

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.

Characterisation of Sleep Problems in Children with Williams Syndrome

Science.gov (United States)

Annaz, Dagmara; Hill, Catherine M.; Ashworth, Anna; Holley, Simone; Karmiloff-Smith, Annette

2011-01-01

Sleep is critical to optimal daytime functioning, learning and general health. In children with established developmental disorders sleep difficulties may compound existing learning difficulties. The purpose of the present study was to evaluate the prevalence and syndrome specificity of sleep problems in Williams syndrome (WS), a…

78 FR 63464 - William D. Ford Federal Direct Loan Program Repayment Plan Selection Form; Extension of Public...

Science.gov (United States)

2013-10-24

... DEPARTMENT OF EDUCATION William D. Ford Federal Direct Loan Program Repayment Plan Selection Form; Extension of Public Comment Period; Correction AGENCY: Department of Education. ACTION: Correction notice... entitled, ``William D. Ford Federal Direct Loan Program Repayment Plan Selection Form''. ED is extending...

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

Science.gov (United States)

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.

Spinal pain in Danish school children – how often and how long?

DEFF Research Database (Denmark)

Dissing, Kristina Boe; Hartvigsen, Jan; Wedderkopp, Niels

Title Spinal pain in Danish school children – how often and how long? The CHAMPS Study-DK Authors First author: Kristina Boe Dissing1 Last author: Lise Hestbæk1,2 Co-authors: Jan Hartvigsen1,2, Christopher Williams3,4, Steven Kamper5, Eleanor Boyle1, Niels Wedderkopp6,7 Affiliations 1 Department...

Gender Attribution and Gender Agreement in French Williams Syndrome

Science.gov (United States)

Boloh, Yves; Ibernon, Laure; Royer, Stephanie; Escudier, Frederique; Danillon, Aurelia

2009-01-01

Previous studies on grammatical gender in French individuals with Williams syndrome (WS) have led to conflicting findings and interpretations regarding keys abilities--gender attribution and gender agreement. New production data from a larger SW sample (N = 24) showed that gender attribution scores in SW participants exactly mirrored those of…

Visually Guided Step Descent in Children with Williams Syndrome

Science.gov (United States)

Cowie, Dorothy; Braddick, Oliver; Atkinson, Janette

2012-01-01

Individuals with Williams syndrome (WS) have impairments in visuospatial tasks and in manual visuomotor control, consistent with parietal and cerebellar abnormalities. Here we examined whether individuals with WS also have difficulties in visually controlling whole-body movements. We investigated visual control of stepping down at a change of…

A Diagnosis to Consider in an Adult Patient with Facial Features and Intellectual Disability: Williams Syndrome.

Science.gov (United States)

Doğan, Özlem Akgün; Şimşek Kiper, Pelin Özlem; Utine, Gülen Eda; Alikaşifoğlu, Mehmet; Boduroğlu, Koray

2017-03-01

Williams syndrome (OMIM #194050) is a rare, well-recognized, multisystemic genetic condition affecting approximately 1/7,500 individuals. There are no marked regional differences in the incidence of Williams syndrome. The syndrome is caused by a hemizygous deletion of approximately 28 genes, including ELN on chromosome 7q11.2. Prenatal-onset growth retardation, distinct facial appearance, cardiovascular abnormalities, and unique hypersocial behavior are among the most common clinical features. Here, we report the case of a patient referred to us with distinct facial features and intellectual disability, who was diagnosed with Williams syndrome at the age of 37 years. Our aim is to increase awareness regarding the diagnostic features and complications of this recognizable syndrome among adult health care providers. Williams syndrome is usually diagnosed during infancy or childhood, but in the absence of classical findings, such as cardiovascular anomalies, hypercalcemia, and cognitive impairment, the diagnosis could be delayed. Due to the multisystemic and progressive nature of the syndrome, accurate diagnosis is critical for appropriate care and screening for the associated morbidities that may affect the patient's health and well-being.

Anaesthesia-related haemodynamic complications in Williams syndrome patients: a review of one institution's experience.

Science.gov (United States)

Olsen, M; Fahy, C J; Costi, D A; Kelly, A J; Burgoyne, L L

2014-09-01

Williams syndrome is a genetic disorder associated with cardiac pathology, including supravalvular aortic stenosis and coronary artery stenosis. Sudden cardiac death has been reported in the perioperative period and attributed to cardiovascular pathology. In this retrospective audit, case note and anaesthetic records were reviewed for all confirmed Williams syndrome patients who had received an anaesthetic in our institution between July 1974 and November 2009. There were a total of 108 anaesthetics administered in 29 patients. Twelve of the anaesthetics (11.1%) were associated with cardiac complications including cardiac arrest in two cases (1.85%). Of the two cardiac arrests, one patient died within the first 24 hours postanaesthetic and the other patient survived, giving an overall mortality of 0.9% (3.4%). We conclude that Williams syndrome confers a significant anaesthetic risk, which should be recognised and considered by clinicians planning procedures requiring general anaesthesia.

Author Details

African Journals Online (AJOL)

Mondal, KC. Vol 62 (2009) - Articles The Effect of Synthesis Parameters on the Catalytic Synthesis of Multiwalled Carbon Nanotubes using Fe-Co/CaCO3 Catalysts Abstract PDF PDF. ISSN: 0379-4350. AJOL African Journals Online. HOW TO USE AJOL... for Researchers · for Librarians · for Authors · FAQ's · More about ...

ANALYSIS OF RAIN BY WILLIAM SOMERSET MAUGHAM IN TERMS OF POST-COLONIAL TERMS

Directory of Open Access Journals (Sweden)

Şaban KÖKTÜRK

2015-04-01

Full Text Available This study dealt with the analysis of a short story, Rain by William Somerset Maugham in terms of post-colonial terms or elements. Before the analysis, the writer of the book was introduced and then the plot was explained so as to make readers familiar with the short story. In the analysis section, post-colonial elements or related terms such as missionary, colonial authority, the state of being subaltern, Manichean allegory, mimicry, ambivalence, surveillance, imperial hegemony, hybridity, essentialism, monolithic culture, alienation, dislocation, misuse of power, gender difference were analyzed from some of the excerpts whether to see how strong these concepts affect the course of the fiction. The study targeted at showing students of language & literature departments the application of the related terms or elements in such stories.

1 report, 6 authors? Easy!

CERN Multimedia

Anaïs Schaeffer

2015-01-01

In 2016, the CERN E-Publishing Service will be testing three co-authoring platforms to decide which is the most suitable for CERN. Whatever type of documents you co-write – scientific papers, internal reports or proceedings – a co-authoring tool will simplify the process. To make such a service available at CERN, the E-Publishing team needs your help.   At CERN, the E-Publishing Service is responsible for copy-editing scientific texts such as CERN reports, scientific papers, school or conference proceedings, etc., and provides support to CERN people regarding the style and layout of their publications. As part of its efforts to simplify the lives of CERN authors, the E-Publishing Service has decided to evaluate the benefits of a new service: a co-authoring platform. If you write on LaTeX or Word-like software, use a messaging application for comments and a calendar for deadlines, and then distribute the file by e-mail (as many times as necessary) – then a co-au...

50 CFR Figure 4 to Subpart E of... - Prince William Sound Rural and Non-Rural Areas

Science.gov (United States)

2010-10-01

... 50 Wildlife and Fisheries 7 2010-10-01 2010-10-01 false Prince William Sound Rural and Non-Rural Areas 4 Figure 4 to Subpart E of Part 300 Wildlife and Fisheries INTERNATIONAL FISHING AND RELATED... to Subpart E of Part 300—Prince William Sound Rural and Non-Rural Areas ER04NO09.010 [74 FR 57110...

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

Directory of Open Access Journals (Sweden)

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

Beyond Behaviour: Is Social Anxiety Low in Williams Syndrome?

Science.gov (United States)

Dodd, Helen F.; Schniering, Carolyn A.; Porter, Melanie A.

2009-01-01

Individuals with Williams syndrome (WS) exhibit striking social behaviour that may be indicative of abnormally low social anxiety. The present research aimed to determine whether social anxiety is unusually low in WS and to replicate previous findings of increased generalised anxiety in WS using both parent and self report. Fifteen individuals…

Biblionef SA: Bringing books to the bookless | Williams | Innovation

African Journals Online (AJOL)

Biblionef SA: Bringing books to the bookless. Jean Williams. Abstract. No abstract available. Innovation (2003) No. 26, pp 43-47. Full Text: EMAIL FULL TEXT EMAIL FULL TEXT · DOWNLOAD FULL TEXT DOWNLOAD FULL TEXT · AJOL African Journals Online. HOW TO USE AJOL... for Researchers · for Librarians ...

William Knocke receives 2008 Virginia Outstanding Civil Engineer Award

OpenAIRE

Daniilidi, Christina

2008-01-01

William R. Knocke, W.C. English Professor and head of the Charles E. Via, Jr. Department of Civil and Environmental Engineering at Virginia Tech, was awarded the 2008 Virginia Outstanding Civil Engineer Award at the Virginia Section of the American Society of Civil Engineers' (ASCE) banquet, held recently in Williamsburg, Va.

Language and Literacy Development of Children with Williams Syndrome

Science.gov (United States)

Mervis, Carolyn B.

2009-01-01

Williams syndrome is a rare neurodevelopmental disorder caused by deletion of approximately 25 genes on chromosome 7q11.23. Children with the syndrome evidence large individual differences in both broad language and reading abilities. Nevertheless, as a group, children with this syndrome show a consistent pattern characterized by relative…

Musicality Correlates with Sociability and Emotionality in Williams Syndrome

Science.gov (United States)

Ng, Rowena; Lai, Philip; Levitin, Daniel J.; Bellugi, Ursula

2013-01-01

Williams syndrome (WS) is a neurogenetic developmental disorder characterized by peaks and valleys of cognitive abilities. One peak that has been understudied is the affinity that many individuals with WS have with music. It remains unknown whether their high levels of musical interest, skill, and expressivity are related to their sociable…

Pulmonary arterial stent implantation in an adult with Williams syndrome

NARCIS (Netherlands)

Reesink, Herre J.; Henneman, Onno D. F.; van Delden, Otto M.; Biervliet, Jules D.; Kloek, Jaap J.; Reekers, Jim A.; Bresser, Paul

2007-01-01

We report a 38-year-old patient who presented with pulmonary hypertension and right ventricular dysfunction due to pulmonary artery stenoses as a manifestation of Williams syndrome, mimicking chronic thromboembolic pulmonary hypertension. The patient was treated with balloon angioplasty and stent

Author Details

African Journals Online (AJOL)

Tetana, ZN. Vol 65 (2012) - Articles The Synthesis of Nitrogen-Doped Multiwalled Carbon Nanotubes Using an Fe-Co/CaCO3 Catalyst Abstract PDF PDF. ISSN: 0379-4350. AJOL African Journals Online. HOW TO USE AJOL... for Researchers · for Librarians · for Authors · FAQ's · More about AJOL · AJOL's Partners · Terms ...

77 FR 66087 - Federal Perkins Loan Program, Federal Family Education Loan Program, and William D. Ford Federal...

Science.gov (United States)

2012-11-01

..., and 685 Federal Perkins Loan Program, Federal Family Education Loan Program, and William D. Ford... 685 RIN 1840-AD05 [Docket ID ED-2012-OPE-0010] Federal Perkins Loan Program, Federal Family Education... (Perkins Loan) program, Federal Family Education Loan (FFEL) program, and William D. Ford Federal Direct...

Binding of Visual and Spatial Short-Term Memory in Williams Syndrome and Moderate Learning Disability

Science.gov (United States)

Jarrold, Christopher; Phillips, Caroline; Baddeley, Alan D

2007-01-01

A main aim of this study was to test the claim that individuals with Williams syndrome have selectively impaired memory for spatial as opposed to visual information. The performance of 16 individuals with Williams syndrome (six males, 10 females; mean age 18y 7mo [SD 7y 6mo], range 9y 1mo-30y 7mo) on tests of short-term memory for item and…

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

Science.gov (United States)

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.

Increased number of papers co-authored by professor and his students in humanities and social sciences journals published in Korea

Directory of Open Access Journals (Sweden)

Rae Seong Hong

2017-02-01

Full Text Available Humanities and social sciences studies in Korea have remarkably low rates of co-authorship between professors and students. We chose a bibliometrics-based approach to characterize changes in the ratio of joint authorship between professors and students. Articles classified in the humanities and social sciences sectors that were published in journals registered in the Korean Citation Index during 2 phases over a 10-year period—2004 to 2006 (phase 1 and 2011 to 2013 (phase 2—were used as the main source for the analysis. The study results can be summarized as follows: first, the overall number of co-authored articles drastically increased from phase 1 to phase 2; the percentage of co-authorship articles increased from 34.8% to 47.7%, and the percentage of co-authorship between students and professors rose from 9.9% to 20.7%. This trend was particularly noticeable in the social sciences, such as accounting, social welfare, and economics/business administration. Second, papers written by scholars from Seoul National University, Yonsei University, and Korea University were often published in high-impact factor journals. Among those articles, the rate of professor-student co-authorship increased by 21.6% for 7 years. Third, the increase in professor-student co-authored articles published in high- impact factor journals was even sharper. These findings indicate that perceptions of professor-student co-authorship have changed in the humanities and social sciences. In the near future, positive perceptions toward joint research and joint authorship between professors and students are expected to become more widespread.

Prince William Sound, Alaska ESI: ESI (Environmental Sensitivity Index Shoreline Types)

Data.gov (United States)

National Oceanic and Atmospheric Administration, Department of Commerce — This data set comprises the Environmental Sensitivity Index (ESI) data for Prince William Sound, Alaska. ESI data characterize estuarine environments and wildlife by...

'Report of the Committee on Mediumistic Phenomena', by William James (1886): With an introduction by.

Science.gov (United States)

Alvarado, Carlos S

2016-03-01

Mediumship was a topic of great interest to some nineteenth-century students of mental phenomena. Together with the phenomena of hypnosis and other manifestations, mediumship was seen by many as a dissociative phenomenon. The purpose of this Classic Text is to present an excerpt of an article about the topic that William James (1842-1910) published in 1886 in the Proceedings of the American Society for Psychical Research about American medium Leonora E. Piper (1857-1950). The article, an indication of late nineteenth-century interactions between dissociation studies and psychical research, was the first report of research with Mrs Piper, a widely investigated medium of great importance for the development of mediumship studies. In addition to studying the case as a dissociative experience, James explored the possibility that Piper's mentation contained verifiable information suggestive of 'supernormal' knowledge. Consequently, James provides an example of a topic neglected in historical studies, the ideas of those who combined conventional dissociation studies with psychical research. © The Author(s) 2016.

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…

Astronaut William Fisher preparing to train in the WETF

Science.gov (United States)

1985-01-01

Astronaut William Fisher is shown in his extravehicular mobility unit (EMU) preparing to train in the Weightless Environment Training Facility (WETF). He is wearing the communications carrier assembly but not the full helmet (32102); Reflections of the WETF can be seen on the closed visor of the EMU helmet Fiser is wearing (32103).

Climatic data for Williams Lake, Hubbard County, Minnesota, 1983

Science.gov (United States)

Sturrock, A.M.; Rosenberry, D.O.; Engelbrecht, L.G.; Gothard, W.A.; Winter, T.C.

1984-01-01

Research on the hydrology of Williams Lake, north-central Minnesota includes study of evaporation. Presented here are those climatic data needed for energy-budget and mass-transfer studies,including: water-surface temperature, dry-bulb and wet-bulb air temperatures, wind speed, precipitation, and solar radiation. Data are collected at raft and land stations.

William E. Vidaver (1921-2017): an innovator, enthusiastic scientist, inspiring teacher and a wonderful friend.

Science.gov (United States)

Burr, A H Jay; Vidaver, Aaron; Schreiber, Ulrich; Bruce, Doug; Donnelly, Danielle J

2018-06-01

William (Bill) E. Vidaver (February 2, 1921-August 31, 2017), who did his Ph.D. with Laurence (Larry) R. Blinks at Stanford (1964) and a postdoc with C. Stacy French (1965), taught and did research at Simon Fraser University (SFU) for almost 30 years. Here he published over 80 papers in photosynthesis-related areas co-authored by his graduate students, postdocs, visiting professors and SFU colleagues. He developed a unique high-pressure cuvette for the study of oxygen exchange and studied high-pressure effects in photosynthesis. Ulrich (Uli) Schreiber, as a postdoctoral fellow from Germany, introduced measurements on chlorophyll (Chl) a fluorescence to Bill's lab, leading to the discovery of reversible inhibition of excitation energy transfer between photosynthetic pigments and of a pivotal role of O 2 in the oxidation of the electron transport chain between Photosystem II (PS II) and PS I. Bill's and Uli's work led to a patent of a portable chlorophyll fluorometer, the first available commercially, which was later modified to measure whole plantlets. The latter was used in pioneering measurement of the health of forest and crop plants undergoing in vitro clonal micropropagation. With several other researchers (including Doug Bruce, the late Radovan Popovic, and Sarah Swenson), he localized the quenching site of O 2 and showed a dampening effect on measurements of the four-step process of O 2 production by endogenous oxygen uptake. Bill is remembered as a hard-working but fun-loving person with a keen mind and strong sense of social justice.

Binding of visual and spatial short-term memory in Williams syndrome and moderate learning disability.

Science.gov (United States)

Jarrold, Christopher; Phillips, Caroline; Baddeley, Alan D

2007-04-01

A main aim of this study was to test the claim that individuals with Williams syndrome have selectively impaired memory for spatial as opposed to visual information. The performance of 16 individuals with Williams syndrome (six males, 10 females; mean age 18y 7mo [SD 7y 6mo], range 9y 1mo-30y 7mo) on tests of short-term memory for item and location information was compared with that shown by individuals with moderate learning difficulties (12 males, four females; mean age 10y 3mo [SD 1y], range 8y 6mo-11y 7mo) and typically developing children (six males, 10 females; mean age 6y 8mo [SD 7mo], range 5y 10mo-7y 9mo) of an equivalent level of visuospatial ability. A second aim was to determine whether individuals had impaired ability to 'bind' visual spatial information when required to recall 'item in location' information. In contrast to previous findings, there was no evidence that individuals with Williams syndrome were more impaired in the spatial than the visual memory condition. However, individuals with both Williams syndrome and moderate learning difficulties showed impaired memory for item in location information, suggesting that problems of binding may be generally associated with learning disability.

Williams Syndrome: Daily Challenges and Positive Impact on the Family

Science.gov (United States)

Scallan, Susan; Senior, Joyce; Reilly, Colin

2011-01-01

Background: Despite the distinctive physical, cognitive, personality and behavioural characteristics associated with Williams syndrome, few studies to date have examined parental experiences of raising a child with this genetic syndrome. Methods: This explorative pilot study employed predominantly qualitative methodologies via face-to-face…

77 FR 76414 - William D. Ford Federal Direct Loan Program

Science.gov (United States)

2012-12-28

... Federal Direct Loan Program AGENCY: Department of Education. ACTION: Final regulations; correction... Loan (Perkins Loan) Program; the Federal Family Education Loan (FFEL) Program; and the William D. Ford Federal Direct Loan (Direct Loan) Program, including the Public Service Loan Forgiveness (PSLF) Program...

Williams' paradox and the role of phenotypic plasticity in sexual systems.

Science.gov (United States)

Leonard, Janet L

2013-10-01

As George Williams pointed out in 1975, although evolutionary explanations, based on selection acting on individuals, have been developed for the advantages of simultaneous hermaphroditism, sequential hermaphroditism and gonochorism, none of these evolutionary explanations adequately explains the current distribution of these sexual systems within the Metazoa (Williams' Paradox). As Williams further pointed out, the current distribution of sexual systems is explained largely by phylogeny. Since 1975, we have made a great deal of empirical and theoretical progress in understanding sexual systems. However, we still lack a theory that explains the current distribution of sexual systems in animals and we do not understand the evolutionary transitions between hermaphroditism and gonochorism. Empirical data, collected over the past 40 years, demonstrate that gender may have more phenotypic plasticity than was previously realized. We know that not only sequential hermaphrodites, but also simultaneous hermaphrodites have phenotypic plasticity that alters sex allocation in response to social and environmental conditions. A focus on phenotypic plasticity suggests that one sees a continuum in animals between genetically determined gonochorism on the one hand and simultaneous hermaphroditism on the other, with various types of sequential hermaphroditism and environmental sex determination as points along the spectrum. Here I suggest that perhaps the reason we have been unable to resolve Williams' Paradox is because the problem was not correctly framed. First, because, for example, simultaneous hermaphroditism provides reproductive assurance or dioecy ensures outcrossing does not mean that there are no other evolutionary paths that can provide adaptive responses to those selective pressures. Second, perhaps the question we need to ask is: What selective forces favor increased versus reduced phenotypic plasticity in gender expression? It is time to begin to look at the question

The Role of Spiral Multidetector Dynamic CT in the Study of Williams-Campbell Syndrome

International Nuclear Information System (INIS)

Scioscio, V. di; Zompatori, M.; Mistura, I.; Montanari, P.; Santilli, L.; Luccaroni, R.; Sverzellati, N.

2006-01-01

Williams-Campbell syndrome is a cystic bronchiectatic disease secondary to deficiency or defect of cartilaginous plates in the wall of the airways. In the literature, two main forms are suggested: congenital and acquired (post-infectious). The most frequent symptoms are represented by recurrent pulmonary infections from childhood. Multislice spiral dynamic CT has a major role in the study of cystic pulmonary disease and in differentiating Williams-Campbell syndrome from the other causes of cystic bronchiectasis, in which even lung function tests can give deceptive results

The Role of Spiral Multidetector Dynamic CT in the Study of Williams-Campbell Syndrome

Energy Technology Data Exchange (ETDEWEB)

Scioscio, V. di; Zompatori, M.; Mistura, I.; Montanari, P.; Santilli, L.; Luccaroni, R.; Sverzellati, N. [Medical Univ. of Bologna, S. Orsola-Malpighi Policlinic (Italy). Dept. of Radiology

2006-10-15

Williams-Campbell syndrome is a cystic bronchiectatic disease secondary to deficiency or defect of cartilaginous plates in the wall of the airways. In the literature, two main forms are suggested: congenital and acquired (post-infectious). The most frequent symptoms are represented by recurrent pulmonary infections from childhood. Multislice spiral dynamic CT has a major role in the study of cystic pulmonary disease and in differentiating Williams-Campbell syndrome from the other causes of cystic bronchiectasis, in which even lung function tests can give deceptive results.

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

Science.gov (United States)

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.

Climatic data for Williams Lake, Hubbard County, Minnesota, 1985

Science.gov (United States)

Sturrock, A.M.; Rosenberry, D.O.; Winter, T.C.

1987-01-01

Research on the hydrology of Williams Lake, north-central Minnesota includes study of evaporation. Presented here are those climatic data needed for energy-budget and mass-transfer studies, including: water-surface temperature, dry-bulb and wet-bulb air temperatures, wind speed, precipitation, and solar and atmospheric radiation. Data are collected at raft and land stations.

Climatic data for Williams Lake, Hubbard County, Minnesota, 1984

Science.gov (United States)

Sturrock, A.M.; Rosenberry, D.O.; Scarborough, J.L.; Winter, T.C.

1986-01-01

Research on the hydrology of Williams Lake, north-central Minnesota includes study of evaporation. Presented here are those climatic data needed for energy-budget and mass-transfer studies, including: water-surface temperature, dry-bulb and wet-bulb air temperatures, wind speed, precipitation, and solar and atmospheric radiation. Data are collected at raft and land stations.

Random Thoughts on William Shakespeare and Medicine

OpenAIRE

KV Sahasranam

2017-01-01

Medicine and literature have always been connected over the ages. William Shakespeare (1564-1616) is no exception. There are plenty of references to medicine and diseases in the works of Shakespeare. The knowledge which Shakespeare has of medical conditions is much more than is expected of a common man. This is attributed to his association with practitioners of his time and reading of contemporary texts in medicine. Also his son in law Dr. John Hall who married Susanna, Shakespeare's eldest ...

Mapping cultural resource sites for the Prince William Sound Graphical Resource Database

International Nuclear Information System (INIS)

Wooley, C. B.; O'Brien, D. K.; Hillman, S. O.

1997-01-01

A software package for mapping digital data 'layers' of environmentally and/or culturally sensitive areas such as seabird colonies, seal haulouts, and sea otter concentrations in Prince William Sound and adjoining areas of southern Alaska has been developed by the Alyeska Pipeline Service Company. The data is to be added to an environmental computer mapping database. More than 1,800 known and reported coastal cultural resource sites have been identified. The database is part of the Prince William Sound Tanker Oil Discharge Prevention and Contingency Plan. The mappable data layers can be used to plan and execute whatever site protection program may be necessary, thus enhancing effective cultural resource protection during an oil spill response. 22 refs., 4 figs

77 FR 32625 - William J. Stevenson, Estate of Lynn E. Stevenson, Black Canyon Bliss, LLC; Notice of Application...

Science.gov (United States)

2012-06-01

... DEPARTMENT OF ENERGY Federal Energy Regulatory Commission [Project No. 8866-010] William J. Stevenson, Estate of Lynn E. Stevenson, Black Canyon Bliss, LLC; Notice of Application for Transfer of License, and Soliciting Comments and Motions To Intervene On April 23, 2012, William J. Stevenson, Estate...

78 FR 33395 - Annual Updates to the Income Contingent Repayment (ICR) Plan Formula for 2013-William D. Ford...

Science.gov (United States)

2013-06-04

... DEPARTMENT OF EDUCATION Annual Updates to the Income Contingent Repayment (ICR) Plan Formula for 2013--William D. Ford Federal Direct Loan Program Catalog of Federal Domestic Assistance (CFDA) Number... free, at 1- 800-877-8339. SUPPLEMENTARY INFORMATION: Under the William D. Ford Federal Direct Loan...

77 FR 72960 - William D. Ford Federal Direct Loan Program

Science.gov (United States)

2012-12-07

... Federal Direct Loan Program AGENCY: Office of Postsecondary Education, Department of Education. ACTION... document to establish the date for the early implementation of William D. Ford Federal Direct Loan (Direct Loan) program regulations that establish a new income-contingent repayment plan based on the President...

Capacity to improve fine motor skills in Williams syndrome.

Science.gov (United States)

Berencsi, A; Gombos, F; Kovács, I

2016-10-01

Individuals with Williams syndrome (WS) are known to have difficulties in carrying out fine motor movements; however, a detailed behavioural profile of WS in this domain is still missing. It is also unknown how great the capacity to improve these skills with focused and extensive practice is. We studied initial performance and learning capacity in a sequential finger tapping (FT) task in WS and in typical development. Improvement in the FT task has been shown to be sleep dependent. WS subjects participating in the current study have also participated in earlier polysomnography studies, although not directly related to learning. WS participants presented with great individual variability. In addition to generally poor initial performance, learning capacity was also greatly limited in WS. We found indications that reduced sleep efficiency might contribute to this limitation. Estimating motor learning capacity and the depth of sleep disorder in a larger sample of WS individuals might reveal important relationships between sleep and learning, and contribute to efficient intervention methods improving skill acquisition in WS. © 2016 The Authors. Journal of Intellectual Disability Research published by MENCAP and International Association of the Scientific Study of Intellectual and Developmental Disabilities and John Wiley & Sons Ltd.

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

Science.gov (United States)

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).

Case report of sudden death in a child with Williams syndrome ...

African Journals Online (AJOL)

A two year old child, confirmed with Williams syndrome (WS) ... and no relevant cardiac history such as chest pain or episodes ... brain, abdominal and pelvic organ blocks. .... a fully functional operating theatre complex presents a number.

WEAVE: the next generation wide-field spectroscopy facility for the William Herschel Telescope : The next generation wide-field spectroscopy facility for the William Herschel Telescope

NARCIS (Netherlands)

Dalton, Gavin; Trager, Scott C.; Abrams, Don Carlos; Carter, David; Bonifacio, Piercarlo; Aguerri, J. Alfonso L.; MacIntosh, Mike; Evans, Chris; Lewis, Ian; Navarro, Ramon; Agocs, Tibor; Dee, Kevin; Rousset, Sophie; Tosh, Ian; Middleton, Kevin; Pragt, Johannes; Terrett, David; Brock, Matthew; Benn, Chris; Verheijen, Marc; Cano Infantes, Diego; Bevil, Craige; Steele, Iain; Mottram, Chris; Bates, Stuart; Gribbin, Francis J.; Rey, Jürg; Rodriguez, Luis Fernando; Delgado, Jose Miguel; Guinouard, Isabelle; Walton, Nic; Irwin, Michael J.; Jagourel, Pascal; Stuik, Remko; Gerlofsma, Gerrit; Roelfsma, Ronald; Skillen, Ian; Ridings, Andy; Balcells, Marc; Daban, Jean-Baptiste; Gouvret, Carole; Venema, Lars; Girard, Paul

We present the preliminary design of the WEAVE next generation spectroscopy facility for the William Herschel Telescope (WHT), principally targeting optical ground-based follow up of upcoming ground-based (LOFAR) and spacebased (Gaia) surveys. WEAVE is a multi-object and multi-IFU facility utilizing

Arterial Hypertension in a Child with Williams-Beuren Syndrome (7q11.23 Chromosomal Deletion

Directory of Open Access Journals (Sweden)

Cristina de Sylos

2002-08-01

Full Text Available We report the case of a 7-year-old male child diagnosed with Williams-Beuren syndrome and arterial hypertension refractory to clinical treatment. The diagnosis was confirmed by genetic study. Narrowing of the descending aorta and stenosis of the renal arteries were also diagnosed. Systemic vascular alterations caused by deletion of the elastin gene may occur early in individuals with Williams-Beuren syndrome, leading to the clinical manifestation of systemic arterial hypertension refractory to drug treatment.

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

Science.gov (United States)

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

Relationship of the Williams-Poulios and Manning-Rosen Potential Energy Models for Diatomic Molecules

Science.gov (United States)

Jia, Chun-Sheng; Liang, Guang-Chuan; Peng, Xiao-Long; Tang, Hong-Ming; Zhang, Lie-Hui

2014-06-01

By employing the dissociation energy and the equilibrium bond length for a diatomic molecule as explicit parameters, we generate an improved form of the Williams-Poulios potential energy model. It is found that the negative Williams-Poulios potential model is equivalent to the Manning-Rosen potential model for diatomic molecules. We observe that the Manning-Rosen potential is superior to the Morse potential in reproducing the interaction potential energy curves for the {{a}3 Σu+} state of the 6Li2 molecule and the {{X}1 sum+} state of the SiF+ molecule.

Atypical Sleep Architecture and Altered EEG Spectra in Williams Syndrome

Science.gov (United States)

Gombos, F.; Bodizs, R.; Kovacs, I.

2011-01-01

Background: Williams syndrome (WS) is a neurodevelopmental genetic disorder characterised by physical abnormalities and a distinctive cognitive profile with intellectual disabilities (IDs) and learning difficulties. Methods: In our study, nine adolescents and young adults with WS and 9 age- and sex-matched typically developing (TD) participants…

Comparison of the acute effects of Tulbaghia violacea William Henry ...

African Journals Online (AJOL)

Purpose: To assess the effect of the crude methanol leaf extracts of Tulbaghia violacea William Henry Harvey (Alliaceae) on blood pressure (BP) and heart rate in ageing normotensive Wistar Kyoto rats (WKY), and compare the results obtained with those for adult spontaneously hypertensive rats (SHR). Methods: T.

Dual-Task Processing as a Measure of Executive Function: A Comparison between Adults with Williams and Down Syndromes

Science.gov (United States)

Kittler, Phyllis M.; Krinsky-McHale, Sharon J.; Devenny, Darlynne A.

2008-01-01

Behavioral phenotypes of individuals with Williams syndrome and individuals with Down syndrome have been contrasted in relation to short-term memory. People with Down syndrome are stronger visuospatially and those with Williams syndrome are stronger verbally. We examined short-term memory, then explored whether dual-task processing further…

77 FR 30266 - Annual Updates to the Income Contingent Repayment (ICR) Plan Formula for 2012; William D. Ford...

Science.gov (United States)

2012-05-22

... DEPARTMENT OF EDUCATION Annual Updates to the Income Contingent Repayment (ICR) Plan Formula for 2012; William D. Ford Federal Direct Loan Program AGENCY: Federal Student Aid, Department of Education... announces the annual updates to the ICR plan formula for 2012. Under the William D. Ford Federal Direct Loan...

77 FR 20796 - Annual Updates to the Income Contingent Repayment (ICR) Plan Formula for 2011; William D. Ford...

Science.gov (United States)

2012-04-06

... DEPARTMENT OF EDUCATION Annual Updates to the Income Contingent Repayment (ICR) Plan Formula for 2011; William D. Ford Federal Direct Loan Program AGENCY: Federal Student Aid, Department of Education... announces the annual updates to the ICR plan formula for 2011. Under the William D. Ford Federal Direct Loan...

A child of Williams-Beuren syndrome for inguinal hernia repair: Perioperative management concerns

Directory of Open Access Journals (Sweden)

Sangeeta Deka

2016-01-01

Full Text Available Williams-Beuren syndrome, commonly known as Williams syndrome (WS, is a multi-organ disorder. The principal anomalies of the syndrome are developmental delay, unusual craniofacial dysmorphic features, and cardiovascular anomalies such as valvular or supravalvular aortic stenosis, pulmonary artery stenosis, and coronary insufficiency. Sudden cardiac death during minor procedures even in the absence of gross cardiovascular pathology is the most dreaded complication in these patients. A 7-year-old child with WS was posted for left-sided inguinal hernia repair under general anesthesia. Our article describes the uneventful perioperative course of the patient and highlights the concerns and complications that may be an integral part with the syndrome.

William Henry Bragg, man and scientist, Nobel Laureate and First Professor of Physics, University of Adelaide 1886-1909.

Science.gov (United States)

Patterson, John; George, Robert

2018-03-01

In London, November 1915, a telegram was received at the home of William Henry Bragg from the secretary of the Academy of Science in Stockholm announcing the award of the Nobel Prize in Physics for "the analysis of crystal structures by means of X-rays". A second similar telegram was addressed to his 25 year old son William Lawrence Bragg (Jenkin, 2008). This article commemorates the centenary of that event and the unveiling of a bust of Sir William Bragg alongside that of his son, Sir Lawrence Bragg, on North Terrace in Adelaide where he spent 23 years of his early career. Copyright © 2018. Published by Elsevier Ltd.

The Interplay between Anxiety and Social Functioning in Williams Syndrome

Science.gov (United States)

Riby, Deborah M.; Hanley, Mary; Kirk, Hannah; Clark, Fiona; Little, Katie; Fleck, Ruth; Janes, Emily; Kelso, Linzi; O'Kane, Fionnuala; Cole-Fletcher, Rachel; Allday, Marianne Hvistendahl; Hocking, Darren; Cornish, Kim; Rodgers, Jacqui

2014-01-01

The developmental disorder Williams syndrome (WS) has been associated with an atypical social profile of hyper-sociability and heightened social sensitivity across the developmental spectrum. In addition, previous research suggests that both children and adults with WS have a predisposition towards anxiety. The current research aimed to explore…

Ronald William Hodges, 1934-2017: Eminent Lepidopterist and Great Mentor

Science.gov (United States)

Dr. Ronald William Hodges (1934-2017) was an eminent lepidopterist and former Research Leader of the Systematic Entomology Laboratory from 1976 to 1979. He published on 117 genera (21 new), 806 species (351 new), 36 subfamilies (7 new), and 16 families (7 new) about gelechioid micromoths. He was awa...

Author contributions to ecological publications: What does it mean to be an author in modern ecological research?

Directory of Open Access Journals (Sweden)

John M Logan

Full Text Available Authorship is a central element of scientific research carrying a variety of rewards and responsibilities, and while various guidelines exist, actual author contributions are often ambiguous. Inconsistent or limited contributions threaten to devalue authorship as intellectual currency and diminish authors' responsibility for published content. Researchers have assessed author contributions in the medical literature and other research fields, but similar data for the field of ecological research are lacking. Authorship practices in ecological research are broadly representative of a variety of fields due to the cross-disciplinary nature of collaborations in ecological studies. To better understand author contributions to current research, we distributed a survey regarding co-author contributions to a random selection of 996 lead authors of manuscripts published in ecological journals in 2010. We obtained useable responses from 45% of surveyed authors. Reported lead author contributions in ecological research studies consistently included conception of the project idea, data collection, analysis, and writing. Middle and last author contributions instead showed a high level of individual variability. Lead authorship in ecology is well defined while secondary authorship is more ambiguous. Nearly half (48% of all studies included in our survey had some level of non-compliance with Ecological Society of America (ESA authorship guidelines and the majority of studies (78% contained at least one co-author that did not meet International Committee of Medical Journal Editors (ICMJE requirements. Incidence of non-compliance varied with lead author occupation and author position. The probability of a study including an author that was non-compliant with ESA guidelines was lowest for professor-led studies and highest for graduate student and post doctoral researcher-led studies. Among studies with > two co-authors, all lead authors met ESA guidelines and only

Inversion of the Williams syndrome region is a common polymorphism found more frequently in parents of children with Williams syndrome.

Science.gov (United States)

Hobart, Holly H; Morris, Colleen A; Mervis, Carolyn B; Pani, Ariel M; Kistler, Doris J; Rios, Cecilia M; Kimberley, Kendra W; Gregg, Ronald G; Bray-Ward, Patricia

2010-05-15

Williams syndrome (WS) is a multisystem disorder caused by deletion of about 1.55 Mb of DNA (including 26 genes) on chromosome 7q11.23, a region predisposed to recombination due to its genomic structure. Deletion of the Williams syndrome chromosome region (WSCR) occurs sporadically. To better define chance for familial recurrence and to investigate the prevalence of genomic rearrangements of the region, 257 children with WS and their parents were studied. We determined deletion size in probands by metaphase FISH, parent-of-origin of the deleted chromosome by molecular genetic methods, and inversion status of the WSCR in both parents by interphase FISH. The frequency of WSCR inversion in the transmitting parent group was 24.9%. In contrast, the rate of inversion in the non-transmitting parent group (a reasonable estimate of the rate in the general population) was 5.8%. There were no significant gender differences with respect to parent-of-origin for the deleted chromosome or the incidence of the inversion polymorphism. There was no difference in the rate of spontaneous abortion for mothers heterozygous for the WSCR inversion relative to mothers without the inversion. We calculate that for a parent heterozygous for a WSCR inversion, the chance to have a child with WS is about 1 in 1,750, in contrast to the 1 in 9,500 chance for a parent without an inversion.