Wegener's Granulomatosis

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... male predominance. Granulomatosis with Polyangiitis typically occurs in middle age, but is found in people of all ages. ... with Polyangiitis in the upper respiratory tract. What’s New in Granulomatosis with Polyangiitis? In the past few ...

Wegener's granulomatosis in childhood

International Nuclear Information System (INIS)

McHugh, K.; Manson, D.; Eberhard, B.A.; Shore, A.; Laxer, R.M.

1991-01-01

The authors reviewed the medical histories and radiological examinations of five pediatric patients with a histologic diagnosis of Wegener's granulomatosis (WG) seen over a six year period in whom a total of 22 thoracic CT scans were performed. Involvement of both the upper and lower respiratory tracts was seen in all patients at presentation. One patient had subglottic stenosis necessitating tracheotomy. Pulmonary hemorrhage occurred in three patients at initial diagnosis. Classic cavitary lung nodules were seen in two patients - one at initial presentation, the other at relapse. The plain radiographic lower respiratory tract manifestations of pediatric WG were protean both at initial presentation and during follow up. Similarly, disease expression was highly variable on thoracic CT examinations but, overall multifocal parenchymal infiltrates with or without small peripheral nodules were the commonest thoracic CT manifestations. As a consequence of cytotoxic and corticosteroid therapy the long-term prognosis of WG has improved considerably in recent years. Knowledge of the varied patterns of the primary disease and potential for iatrogenic complications are necessary for successful radiologic assessment of pediatric patients with WG. (orig.)

Expression of recombinant proteinase 3, the autoantigen in Wegener's granulomatosis, in insect cells

NARCIS (Netherlands)

Van der Geld, YM; Smook, MLF; Huitema, MG; Harmsen, MC; Limburg, PC; Kallenberg, CGM

2002-01-01

Proteinase 3 (PR3) is the major autoantigen for anti-neutrophil cytoplasmic antibodies (ANCA) in patients with Wegener's granulomatosis. Little is known about the major antigenic sites on PR3. To facilitate epitope mapping, PR3 was cloned in insect cells using a baculovirus expression system. Four

Asymptomatic appearance of splenic infarction in Wegeners granulomatosis

International Nuclear Information System (INIS)

Martusewicz-Boros, M.; Roszkowski-Sliz, K.; Wiatr, E.; Baranska, I.; Bestry, I.

2011-01-01

Splenic involvements in Wegeners granulomatosis (WG) are rarely diagnosed ante-mortem, while an autopsy is able to reveal a high rate of spleen lesions (78 - 100%). To date, there have been a few reported cases of splenic abnormalities in WG, including: splenomegaly, capsular adhesion, dysfunction and infarction. We reported a case of biopsy-verified WG with radiological evidence of diffuse spleen infarction despite the lack of any clinical symptoms. We concluded that due to a potential risk of severe hemorrhagic complications when anticoagulant therapy is necessary, radiological assessment of spleen should be performed regularly in this group of patients, particularly because spleen involvement can be asymptomatic. (authors)

Wegener´s granulomatosis in a young patient preceded by localized cutaneous manifestations

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Jesper Smit

2011-10-01

Full Text Available Wegener´s granulomatosis (WG is a rare, systemic vasculitis involving multiple organs. The clinical presentation is highly diverse, and there is considerable risk of mortality if diagnosis and treatment are delayed. We present a case illustrating that patients with WG may initially present with localized cutaneous symptoms and signs.

Diversity of PR3-ANCA epitope specificity in Wegener's granulomatosis. Analysis using the biosensor technology

NARCIS (Netherlands)

Rarok, Agnieszka; van der Geld, Y.M.; Stegeman, Coen; Limburg, Piet; Kallenberg, Cees

Wegener's granulomatosis is a systemic disease characterized by the presence of antineutrophil cytoplasm autoantibodies specific for proteinase 3 (PR3-ANCA). The functional characteristics of PR3-ANCA differ between quiescent and active disease, suggesting changes in the properties of the

Cranial nerve palsy in Wegener's granulomatosis--lessons from clinical cases

DEFF Research Database (Denmark)

Nowack, Rainer; Wachtler, Paul; Kunz, Jürgen

2009-01-01

The problem of diagnosing vasculitic neuropathy is discussed based on case reports of two patients with Wegener's granulomatosis. One patient developed de novo 6(th) nerve palsy as an isolated relapse manifestation and the second patient a sequence of multiple cranial nerve palsies. Brain imaging...... by the overall clinical presentations. Cranial neuropathy may be the first obvious vasculitic manifestation preceding other organ disease, and since single reliable tests for its diagnosis are lacking, a multidisciplinary approach is advocated here to detect vasculitic manifestations in other organs....

Fulminant Wegener's granulomatosis: A case report

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Dinić Miroslav Ž.

2013-01-01

Full Text Available Introduction. Granulomatosis Wegener is anti-neutrophil cytoplasmic antibodies (ANCAs-associated systemic vasculitis of unknown etiology. It is manifested as granulomatous necrotizing inflammation of the upper and lower parts of the respiratory tract, glomerulonephritis and systemic vasculitis involving most frequently the skin and oral mucous membrane. Sera markers of this disease are c-ANCA and p-ANCA. Case report. We presented a female patient aged 52 years with purpuric spots that had appeared on the lower legs ten months before admission to our hospital. The disease ran an aggressive course, and a month before admission hemorrhagic bullae, skin ulcers, hoarseness, dyspnea, generalized arthralgia, fatigue and fever had rapidly developed. Histopathological examination of a skin sample revealed necrotizing vasculitis, so that sera markers concentrations were elevated (c-ANCA, p-ANCA. There was a perforation of the nasal septum found on rhinoscopy. During hospitalization acute abdominal pain occurred, a possible tumor in the small intestine and possible granulomas in the liver were seen by multislice computed tomography (MSCT examination, with normal findings on the lungs and kidneys. The treatment started with methylprednisolone: 500 mg/d i.v. infusion for consecutive 3 days, then 60 mg/d. On exploratory laparotomy small bowel perforation and diffuse peritonitis were found. Unstable in the postoperative period, the patient died on the day 12 of hospitalization. Conclusion. The reported patient was with fulminant Wegener’s granulomatosis, dominantly with skin changes and with gastrointestinal manifestation. This case accents the need for rapid systemic clinical evaluation in a severely ill patient with unclear diagnosis.

Pulmonary CT findings of Wegener's granulomatosis (WG). Follow-up images and pathology

International Nuclear Information System (INIS)

Uezono, Haruka; Noma, Satoshi; Sakamoto, Ryo

2009-01-01

We reviewed 16 cases of Wegener's granulomatosis (WG) with regard to CT findings, PR3-ANCA, and clinical courses. Because of PR3-ANCA, atypical manifestations of WG could have been diagnosed, including tiny nodules without symptoms and self-remissions. Radiologists should be aware of these atypical manifestations of WG. Seeing multiple nodules in lung, WG should be included in the differential diagnosis and extrapulmonary manifestation of WG should be evaluated. (author)

Wegener's granulomatosis occurring de novo during pregnancy.

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Alfhaily, F; Watts, R; Leather, A

2009-01-01

Wegener's granulomatosis (WG) is rarely diagnosed during the reproductive years and uncommonly manifests for the first time during pregnancy. We report a case of de novo WG presenting at 30 weeks gestation with classical symptoms of WG (ENT, pulmonary). The diagnosis was confirmed by radiological, laboratory, and histological investigations. With a multidisciplinary approach, she had a successful vaginal delivery of a healthy baby. She was treated successfully by a combination of steroids, azathioprine and intravenous immunoglobulin in the active phase of disease for induction of remission and by azathioprine and steroids for maintenance of remission. The significant improvement in her symptoms allowed us to continue her pregnancy to 37 weeks when delivery was electively induced. Transplacental transmission of PR3-ANCA occurred but the neonate remained well. This case of de novo WG during pregnancy highlights the seriousness of this disease and the challenge in management of such patients.

Functional defect of circulating regulatory CD4+T cells in patients with Wegener's granulomatosis in remission

NARCIS (Netherlands)

Abdulahad, Wayel Habib; Stegeman, Coen; van der Geld, Y.M.; Doornbos-van der Meer, B.; Limburg, Piet; Kallenberg, Cees

Objective. Accumulating data support the role of regulatory T cells, a subset of CD4+ T cells that expresses CD25(high) and the transcription factor fork-head box P3 (FoxP3), in controlling and preventing autoimmunity. In Wegener's granulomatosis (WG), an autoimmune vasculitis, up-regulation of CD25

Granulomatose de Wegener: Envolvimento otológico, nasal, laringotraqueal e pulmonar Wegener granulomatosis: Otologic, nasal, tracheobronchial and pulmonary involvement

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Sandra Figueiredo

2009-10-01

Full Text Available A granulomatose de Wegener é uma vasculite sistémica rara e idiopática caracterizada pelo atingimento dos pequenos vasos. A doença atinge, preferencialmente, as vias aérea superior e inferior e os rins, levando à formação de granulomas e necrose destes órgãos. As manifestações clínicas e o envolvimento orgânico variam largamente. O diagnóstico e tratamento precoce podem levar à recuperação total. No entanto, o atraso no diagnóstico pode ser fatal. Os autores apresentam o caso de uma doente de 33 anos com uma forma de apresentação grave e rara da doença, mas com uma evolução favorável após diagnóstico e tratamento adequado.Wegener granulomatosis is a rare systemic idiopathic disease characterized by involvement of small vessels - medium and small arteries, venules, arterioles and ocasionally large arteries. This disease has predilection for the upper and lower respiratory tract and the kidney, with granulomatous inflamation and necrosis. Clinical manifestations and organ involvement of the disease vary widely. Early diagnosis and treatment may lead to a full recovery. Without treatment, Wegener’s granulomatosis can be fatal. The authors present a case of a 33 year-old female, with severe disease, but with good outcome, after adequate diagnosis and treatment.

Plasma exchange for induction and cyclosporine A for maintenance of remission in Wegener's granulomatosis--a clinical randomized controlled trial

DEFF Research Database (Denmark)

Szpirt, Wladimir M; Heaf, James G; Petersen, Jørgen

2011-01-01

The use of plasma exchange (PE) for induction treatment of anti-neutrophil cytoplasm autoantibody (ANCA)-associated vasculitis (AAV), including Wegener's granulomatosis (WG), is still controversial. The use of PE in AAV is not commonly accepted in patients with a plasma creatinine...

EULAR/PRINTO/PRES criteria for Henoch-Schönlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part I: Overall methodology and clinical characterisation

DEFF Research Database (Denmark)

Ruperto, Nicolino; Ozen, Seza; Pistorio, Angela

2010-01-01

To report methodology and overall clinical, laboratory and radiographic characteristics for Henoch-Schönlein purpura (HSP), childhood polyarteritis nodosa (c-PAN), c-Wegener granulomatosis (c-WG) and c-Takayasu arteritis (c-TA) classification criteria.......To report methodology and overall clinical, laboratory and radiographic characteristics for Henoch-Schönlein purpura (HSP), childhood polyarteritis nodosa (c-PAN), c-Wegener granulomatosis (c-WG) and c-Takayasu arteritis (c-TA) classification criteria....

[A historic case of Wegener's granulomatosis: the physicist who discovered the electromagnetic waves: Heinrich Hertz].

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Feldmann, H

2005-06-01

A diary and an extensive correspondence of Heinrich Hertz and his family provide a meticulous documentation of his life, made available by Albrecht Folsing in 1997 through a carefully investigated biography, which permit the conclusion that H. Hertz died of Wegener's granulomatosis, 45 years before this disease had been explored. WEGENER'S GRANULOMATOSIS: The symptoms of the granulomatosis, that was first described by Friedrich Wegener in 1936 and 1939 are presented in short with literal quotations of the author: It begins with a refractory cold that will last until the end, then follow involvement of the paranasal sinuses, ears, mucous lining of mouth and pharynx, slight fever, weakness, in the final phase dissemination with nephritis, septic fever, arthritis, myalgia, paralyses leading to total immobility, exitus. The etiology is not fully understood but probably based on immunoreaction. H. HERTZ: CURRICULUM VITAE: Born in Hamburg, Germany, in 1857: after secondary school academic training in physics at Dresden, Munic and Berlin. 1885 professor of physics in Karlsruhe, then starting in 1889 at Bonn. 1887/88 exploration of the electromagnetic waves, now the basis for all radio communication, and exploration of the photoelectric effect, the basis of solar techniques. Numerous highranking distinctions. H. HERTZ: The case history is presented in detail based on numerous literal quotations from personal letters and the diary. After a few banal affections involving the teeth and toes the systemic disease became manifest in summer 1892 with a refractory cold which remained the focus of treatment until death. Granulation tissue in the nose that was excised several times: no tuberculosis, no carcinoma. Otitis with purulent mastoiditis, in October 1892 mastoidectomy, persistent purulence. Early in 1893 diagnosis of nephritis. Extraction of a superior tooth, punturing of the maxillary sinus, then twice radical opening of the maxillary sinus. Painful ankylosis and paralyses

Patients' perceptions of the effects of systemic lupus erythematosus on health, function, income, and interpersonal relationships : A comparison with Wegener's granulomatosis

NARCIS (Netherlands)

Boomsma, MM; Bijl, M; Stegeman, CA; Kallenberg, CGM; Hoffman, GS; Tervaert, JWC

2002-01-01

Objective. To describe the patients' perceptions of the effects of systemic lupus erythematosus (SLE) and Wegener's granulomatosis (WG) on health, function, income, and interpersonal relationships. Methods. 114 patients with SLE, and 79 patients with WG completed a self-administered questionnaire.

Lingual infarction in Wegener's Granulomatosis: A case report and review of the literature

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Brizman Eitan

2008-08-01

Full Text Available Abstract Wegener's granulomatosis (WG is a multi-system disease, characterised by the triad of necrotising granulomata affecting the upper and lower respiratory tracts, disseminated vasculitis and glomerulonephritis. Oral lesions are associated with up to 50% of cases, although are rare as a presenting feature. The most common oral lesions associated with WG are ulceration and strawberry gingivitis. We review the literature regarding oral manifestations of WG and present a case of lingual infarction, an extremely rare oral lesion associated with WG, in a severe, rapidly progressive and ultimately fatal form of the disease.

Wegener granulomatosis as an uncommon cause of panhypopituitarism in childhood.

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Kara, Ozlem; Demirel, Fatma; Acar, Banu Celikel; Cakar, Nilgün

2013-01-01

Wegener granulomatosis (WG) is a cytoplasmic antineutrophil cytoplasmic antibody (c-ANCA)-associated, multi-system, necrotizing granulomatous vasculitis. Inflammation of the nasal or oral mucosa, and lung and kidney involvements are typical in the course of the disease. In rare cases, pituitary involvement may occur and cause panhypopituitarism. Pituitary involvement is very rare, and only two pediatric case reports have been published to date out of a total of 24 cases. This is a case report of an adolescent patient who presented with panhypopituitarism symptoms and was later diagnosed with WG. A 16-year-old female patient complained of fever, headache, purulent nasal discharge and severe muscle and joint pain. Additionally, she had polyuria and polydipsia. Investigations revealed a pituitary mass and panhypopituitarism. Positivity of c-ANCA and renal biopsy result compatible with WG confirmed the diagnosis.

Chest CT findings in pediatric Wegener's granulomatosis

International Nuclear Information System (INIS)

Levine, Daniel; Akikusa, Jonathan; Manson, David; Silverman, Earl; Schneider, Rayfel

2007-01-01

Although pulmonary involvement occurs in the majority of children and adolescents with Wegener's granulomatosis (WG), relatively little has been published regarding the CT imaging manifestations in this group of patients. To determine the frequency and types of chest CT abnormalities in active pediatric WG (pWG). The study was a retrospective examination of 29 chest CT examinations performed at diagnosis (n=14) and during disease flares (n=15) in 18 children. The most common abnormalities were nodules (seen in 90% of examinations), ground-glass opacification (52%), and air-space opacification (45%). Of examinations with nodules, 73% demonstrated nodules >5 mm in diameter and 69% demonstrated more than five nodules; 17% had cavitary lesions. The only abnormality with a significant difference in prevalence between diagnosis and disease flares was air-space opacification, present in 71% and 20%, respectively (P < 0.01). In accordance with the findings of published adult studies and at variance with those of prior pediatric studies, our findings indicate that chest CT abnormalities in active pWG are frequent, most commonly comprising nodules and ground-glass opacification, which may be difficult to detect on plain radiography. We therefore advocate the routine use of chest CT for all affected patients, both at the time of presentation and during disease flares. (orig.)

Evaluation of capture ELISA for detection of antineutrophil cytoplasmic antibodies directed against proteinase 3 in Wegener's granulomatosis : first results from a multicentre study

NARCIS (Netherlands)

Csernok, E; Holle, J; Hellmich, B; Willem, J; Tervaert, C; Kallenberg, CGM; Limburg, PC; Niles, J; Pan, GL; Specks, U; Westman, K; Wieslander, J; Gross, WL

Objective: To evaluate the performance characteristics of direct and capture ELISA for the detection of PR3-ANCA in Wegener's granulomatosis (WG) in international ANCA reference laboratories. Methods: Serum samples were derived from patients with histological and clinical diagnosis of WG (n = 60),

Granulomatosis de Wegener Semejando Cáncer Epidermoide de Nasofaringe y Metástasis Pulmonares.

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Gustavo Adolfo Martín Small

2011-01-01

Full Text Available La granulomatosis de Wegener (GW es una enfermedad idiopática, con posible componente autoinmune, que aparece generalmente en la quinta década de vida, caracterizándose por lesiones granulomatosas necrotizantes y vasculitis en vías aéreas y riñón. Paciente femenino de 54 años, quien desde Noviembre del 2008, presenta rinorrea, prurito y eritema en borde nasal inferior izquierdo, recibe antibióticos sin mejoría de los síntomas. En las radiografías torácicas, se observan dos radiopacidades redondeadas, de 4 cm de diámetro, sugestivas de lesiones tumorales en ambos campos pulmonares. La biopsia reporta cáncer epidermoide de alto grado, sospechándose primario en nasofaringe. Es remitida, por deterioro de condiciones, al Servicio de Neumonología del Hospital Universitario de Caracas el 18/03/2009, presentando disnea, tos productiva, placas purpúricas dolorosas en manos y pies, hipoacusia, hiperpigmentación del paladar duro, leucoplaquias y lesiones costrosas en lengua, insuficiencia renal (creatinina en 6,11 mg/dL y trombocitopenia. En TAC de tórax, se evidencian tumores mayores de 5 cm de diámetro, algunos con bordes bien definidos y otros mal delimitados con broncograma aéreo. Diagnostico definitivo de GW por serología. La GW con su afectación sistémica puede simular otras entidades como neoplasias de cabeza y cuello con metástasis pulmonares. Cuando las manifestaciones sistémicas son floridas debe sospecharse GW como diagnóstico diferencial. Palabras claves: Wegener, Granulomatosis, Vasculitis, Cáncer epidermoide.

EULAR/PRINTO/PRES criteria for Henoch-Schönlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part II: Final classification criteria

DEFF Research Database (Denmark)

Ozen, Seza; Pistorio, Angela; Iusan, Silvia M

2010-01-01

To validate the previously proposed classification criteria for Henoch-Schönlein purpura (HSP), childhood polyarteritis nodosa (c-PAN), c-Wegener granulomatosis (c-WG) and c-Takayasu arteritis (c-TA)....

Massive Alveolar Hemorrhage During Wegener Granulomatosis: a Case Report

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Gökhan Perincek

2011-11-01

Full Text Available This is a presentation of Wegener Granulomatosis (WG disease. Even though the lungs are rarely affected. massive alveolar hemorrhage is seen which leads to mortality. The patient was a 28 year old man. His illness was diagnosed as WG and glomerulonephritis a year previously and he was treated by administration of methylprednisolone orally. He had been treated irregularly. He applied to the emergency service with hemoptysis and asthma complaints two days earlier. After the results of his examination Hb: 3.6 gr/dl, Htc:10.3%, Üre:131 mg /dl, kreatini: 7.7 mg/dl, pH: 7.41, pO2: 55 mmHg, pCO2:33 mmHg, and being diagnosed as alveolar consolidation on lung X-ray, he was taken to the intensive care unit with a diagnosis of a massive alveolar hemorrhagei. He was intubated and attached to mechanical ventilation. He was treated with parenteral 1 mg/kg/day methylprednisolone and, siklofosfamid 2 mg/kg/day. He was extubated on the 21st day. He was taken to the chest service department on 24th day. He is still being treated.

Estenose subglótica como manifestação clínica da granulomatose de Wegener em adolescentes: relato de caso e revisão de literatura Subglottic stenosis as a clinical manifestation of Wegener's granulomatosis in adolescents: report of a case and review of literature

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Márcia C. Machado

2003-10-01

Full Text Available A Granulomatose de Wegener é uma patologia caracterizada por vasculite sistêmica e inflamação granulomatosa necrotizante que compromete o trato respiratório alto, pulmões e rins, cujas manifestações iniciais, na maioria das vezes, ocorre em maiores de 20 anos de idade. As queixas clínicas otorrinolaringológicas estão, com freqüência, presentes nas fases iniciais da doença, sendo rinite, sinusopatia de repetição e epistaxe as mais comuns. A estenose subglógica parece estar correlacionada com a Granulomatose de Wegener em adolescentes. Relatamos o caso de uma paciente portadora de Granulomatose de Wegener cujo início dos sintomas foi na infância, tendo evoluído com estenose laríngea durante o curso da doença.Wegener's Granulomatosis is a well-characterized systemic vasculitis and necrotising granulomatous inflammation of the upper, lower respiratory tracts and the kidneys. The initial manifestations of the disease usually occur in patients over than twenty years old. The otolaryngologic symptoms like rhinorrhea, recurrent sinusitis and epistaxis are commonly present in early course of the disease. It seems that subglottic stenosis is correlated to Wegener's granumatosis in adolescents. We describe a case of a patient that developed subglottic stenosis associated with Wegener's granulomatosis in childhood during the course of the disease.

Assessment of the Item Selection and Weighting in the Birmingham Vasculitis Activity Score for Wegener's Granulomatosis

Science.gov (United States)

MAHR, ALFRED D.; NEOGI, TUHINA; LAVALLEY, MICHAEL P.; DAVIS, JOHN C.; HOFFMAN, GARY S.; MCCUNE, W. JOSEPH; SPECKS, ULRICH; SPIERA, ROBERT F.; ST.CLAIR, E. WILLIAM; STONE, JOHN H.; MERKEL, PETER A.

2013-01-01

Objective To assess the Birmingham Vasculitis Activity Score for Wegener's Granulomatosis (BVAS/WG) with respect to its selection and weighting of items. Methods This study used the BVAS/WG data from the Wegener's Granulomatosis Etanercept Trial. The scoring frequencies of the 34 predefined items and any “other” items added by clinicians were calculated. Using linear regression with generalized estimating equations in which the physician global assessment (PGA) of disease activity was the dependent variable, we computed weights for all predefined items. We also created variables for clinical manifestations frequently added as other items, and computed weights for these as well. We searched for the model that included the items and their generated weights yielding an activity score with the highest R2 to predict the PGA. Results We analyzed 2,044 BVAS/WG assessments from 180 patients; 734 assessments were scored during active disease. The highest R2 with the PGA was obtained by scoring WG activity based on the following items: the 25 predefined items rated on ≥5 visits, the 2 newly created fatigue and weight loss variables, the remaining minor other and major other items, and a variable that signified whether new or worse items were present at a specific visit. The weights assigned to the items ranged from 1 to 21. Compared with the original BVAS/WG, this modified score correlated significantly more strongly with the PGA. Conclusion This study suggests possibilities to enhance the item selection and weighting of the BVAS/WG. These changes may increase this instrument's ability to capture the continuum of disease activity in WG. PMID:18512722

Chest CT findings in pediatric Wegener's granulomatosis

Energy Technology Data Exchange (ETDEWEB)

Levine, Daniel [British Columbia Children' s Hospital, Department of Radiology and Nuclear Medicine, Vancouver, British Columbia (Canada); Akikusa, Jonathan [Royal Children' s Hospital Melbourne, Department of Rheumatology, Melbourne (Australia); Manson, David [Hospital for Sick Children, Department of Radiology, Toronto (Canada); Silverman, Earl; Schneider, Rayfel [Hospital for Sick Children, Department of Rheumatology, Toronto (Canada)

2007-01-15

Although pulmonary involvement occurs in the majority of children and adolescents with Wegener's granulomatosis (WG), relatively little has been published regarding the CT imaging manifestations in this group of patients. To determine the frequency and types of chest CT abnormalities in active pediatric WG (pWG). The study was a retrospective examination of 29 chest CT examinations performed at diagnosis (n=14) and during disease flares (n=15) in 18 children. The most common abnormalities were nodules (seen in 90% of examinations), ground-glass opacification (52%), and air-space opacification (45%). Of examinations with nodules, 73% demonstrated nodules >5 mm in diameter and 69% demonstrated more than five nodules; 17% had cavitary lesions. The only abnormality with a significant difference in prevalence between diagnosis and disease flares was air-space opacification, present in 71% and 20%, respectively (P < 0.01). In accordance with the findings of published adult studies and at variance with those of prior pediatric studies, our findings indicate that chest CT abnormalities in active pWG are frequent, most commonly comprising nodules and ground-glass opacification, which may be difficult to detect on plain radiography. We therefore advocate the routine use of chest CT for all affected patients, both at the time of presentation and during disease flares. (orig.)

A Case of Simultaneous, Biopsy-Proven, Classic, ANCA-Positive Wegener's Granulomatosis and Anti-GBM Disease, but without Detectible Circulating Anti-GBM Antibodies

OpenAIRE

Gmurczyk, Aleksandra; Ahya, Shubhada N.; Goldschmidt, Robert; Kim, George; Ho, L. Tammy; Nash, Kevin

2010-01-01

Wegener's granulomatosis (WG) is a systemic, necrotizing, granulomatous vasculitis of unknown etiology. Approximately 75% of cases present as classic WG with both pulmonary and renal involvement, while the remaining 25% of patients present with a limited form with either predominantly upper or lower respiratory tract symptoms. Ninety percent of WG patients have circulating anti–neutrophil cytoplasmic antibodies (ANCA), and approximately 10% have both circulating ANCA antibodies and concomitan...

Serial High-Resolution Computed Tomography Imaging in Patients with Wegener Granulomatosis: Differentiation Between Active Inflammatory and Chronic Fibrotic Lesions

International Nuclear Information System (INIS)

Lohrmann, C.; Uhl, M.; Schaefer, O.; Ghanem, N.; Kotter, E.; Langer, M.

2005-01-01

PURPOSE: To evaluate pulmonary pathologies in Wegener granulomatosis with sequential computed tomography (CT) in order to differentiate active inflammatory lesions from chronic fibrotic lesions. MATERIAL AND METHODS: Serial CT findings in 38 patients with Wegener granulomatosis were retrospectively analyzed (mean follow-up period, 21 months). The presence, extension, and distribution of the following findings were evaluated with CT: parenchymal nodules, masses, ground-glass attenuation, airspace consolidation, bronchial wall-thickening, bronchiectasis, linear areas of attenuation, pleural irregularities, pleural effusions, hilar and mediastinal lymphadenopathy. RESULTS: Observed in 92% of patients, nodules were the most common CT pathology. Areas of ground-glass attenuation, consolidation, masses of linear attenuation, and tracheal/bronchial wall-thickening were detected in 24%, 26%, 32%, 39%, and 68% of patients. At follow-up, the clearance of lesions was most consistent for areas of ground-glass attenuation (89%), masses (87%), and cavitated nodules (85%). In the follow-up scan, 58% of all nodules, 47% of pulmonary consolidations, and 66% of bronchial wall-thickening were completely resolved. Areas of bronchiectasis and septal/non-septal lines remained stable in 70% and 71% of patients. CONCLUSION: The majority of the lesions decreased or resolved completely with or without areas of linear attenuation. Ground-glass attenuation, cavitated nodules and masses appear to represent active inflammatory lesions. In most probability, areas of bronchiectasis and septal/non-septal lines more often represent chronic fibrotic changes rather than active inflammatory changes. In combination with clinical evaluation and bronchoscopy, CT assists in the assessment of disease activity

Serial High-Resolution Computed Tomography Imaging in Patients with Wegener Granulomatosis: Differentiation Between Active Inflammatory and Chronic Fibrotic Lesions

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Lohrmann, C.; Uhl, M.; Schaefer, O.; Ghanem, N.; Kotter, E.; Langer, M. [Univ. Hospital of Freiburg (Germany). Dept. of Radiology

2005-08-01

PURPOSE: To evaluate pulmonary pathologies in Wegener granulomatosis with sequential computed tomography (CT) in order to differentiate active inflammatory lesions from chronic fibrotic lesions. MATERIAL AND METHODS: Serial CT findings in 38 patients with Wegener granulomatosis were retrospectively analyzed (mean follow-up period, 21 months). The presence, extension, and distribution of the following findings were evaluated with CT: parenchymal nodules, masses, ground-glass attenuation, airspace consolidation, bronchial wall-thickening, bronchiectasis, linear areas of attenuation, pleural irregularities, pleural effusions, hilar and mediastinal lymphadenopathy. RESULTS: Observed in 92% of patients, nodules were the most common CT pathology. Areas of ground-glass attenuation, consolidation, masses of linear attenuation, and tracheal/bronchial wall-thickening were detected in 24%, 26%, 32%, 39%, and 68% of patients. At follow-up, the clearance of lesions was most consistent for areas of ground-glass attenuation (89%), masses (87%), and cavitated nodules (85%). In the follow-up scan, 58% of all nodules, 47% of pulmonary consolidations, and 66% of bronchial wall-thickening were completely resolved. Areas of bronchiectasis and septal/non-septal lines remained stable in 70% and 71% of patients. CONCLUSION: The majority of the lesions decreased or resolved completely with or without areas of linear attenuation. Ground-glass attenuation, cavitated nodules and masses appear to represent active inflammatory lesions. In most probability, areas of bronchiectasis and septal/non-septal lines more often represent chronic fibrotic changes rather than active inflammatory changes. In combination with clinical evaluation and bronchoscopy, CT assists in the assessment of disease activity.

High risk of pulmonary embolism and deep venous thrombosis but not of stroke in granulomatosis with polyangiitis (Wegener's)

DEFF Research Database (Denmark)

Faurschou, Mikkel; Obel, Niels; Baslund, Bo

2014-01-01

OBJECTIVE: To assess the incidence of stroke, pulmonary embolism (PE), and deep venous thrombosis (DVT) in granulomatosis with polyangiitis (Wegener's) (GPA). METHODS: Patients diagnosed with GPA at a Danish tertiary care center during 1993-2011 were identified (n = 180). Each patient was matched...... 20.2 [95% CI 5.1-81] for DVT). The incidence of stroke was not increased during this time interval (IRR 1.4 [95% CI 0.3-5.7]). From 2 years after GPA diagnosis, an increased incidence was found for DVT (IRR 4.5 [95% CI 1.7-11.8]) but not for PE (IRR 1.3 [95% CI 0.2-9.6]) or stroke (IRR 1.4 [95% CI 0...

Manifestação otológica localizada em paciente com granulomatose de Wegener Localized otologic manifestation in a patient with Wegener's granulomatosis

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Márcia Regina Rosa Scalcon

2008-08-01

Full Text Available A granulomatose de Wegener (GW é uma vasculite granulomatosa multissistêmica rara, idiopática, que acomete vasos de pequeno e médio calibres, e que classicamente envolvem os tratos respiratórios superior, inferior e rins. Na forma limitada da doença, diversos sítios anatômicos podem ser afetados isoladamente. Foi descrito o caso de uma paciente jovem, que iniciou o acometimento da GW por dispnéia, estridor laríngeo e hipacusia. A investigação evidenciou o acometimento localizado na glote, e envolvimento da membrana timpânica comprovado por biópsia. A paciente obteve resposta com o tratamento instituído, mas permaneceu com seqüelas otológicas. Revisa-se neste artigo algumas das manifestações focais da GW, salientando especialmente as manifestações otológicas.Wegener's granulomatosis (WG is a rare, idiopathic granulomatous vasculitis, affecting small and medium-sized vessels, classically involving upper and lower respiratory tracts and kidneys. In the limited form of the disease, several other sites can be affected. We describe the case of a young woman who presented her disease with dyspnea, laryngeal stridor and hearing loss. Investigation revealed localized glottis involvement and a tympanum biopsy confirmed granulomatous vasculitis. The patient had a partial response with the treatment, with residual deafness. We performed a review concerning the focal manifestations of WG, focusing in the ear involvement.

[Morphological pathology of vessels in granulomatosis with polyangiitis (Wegener's disease)].

Science.gov (United States)

Zerbino, D D; Zimba, E A

2015-01-01

to investigate the incidence of injuries in different vascular beds and the morphopathological changes in vessels in granulomatosis with polyangiitis. The morphopathological features of vascular injuries were investigated in 11 dead patients aged 16--74 years with granulomatosis with polyangiitis. Proliferative and destructive angiitis with predominant involvement of microcirculatory vessels and with development of necrosis-prone granulomas in their walls and perivascularly was established to underlie the clinical manifestations of granulomatosis with polyangiitis. The most typical localization of the pathologic process is the vessels of the upper respiratory tract, lungs, and kidneys. Cardiopulmonary and renal failures are causes of death in the majority of cases. It should be noted that the vessels of the heart, liver, and gastrointestinal tract are frequently involved in the pathological process. Vascular changes in these organs determine the clinical features of granulomatosis with polyangiitis and lead to a number of fatal complications. Granulomatosis with polyangiitis is a systemic disease with polymorphism of clinical manifestations, which requires in-depth analysis based on current precision patient examination methods, including a histopathological study.

T-cell mediated immunity in Wegener's granulomatosis

NARCIS (Netherlands)

Abdulahad, Wayel Habib

2008-01-01

Although the pathogenetic mechanisms involved in Wegener’s granulomatosis (WG) are not completely understood, considerable evidence support the concepts that activated T-cells play an important role in disease expression. It is, however, not clear which subsets of T-cells are involved in the

MACULA RETINAL VASCULITIS AND CHOROIDITIS ASSOCIATED WITH GRANULOMATOSIS WITH POLYANGIITIS.

Science.gov (United States)

Lim, Lik Thai; Vasudevan, Vinaya; Shelton, Julie; Vitale, Albert T; Moorthy, Ramana

2017-01-01

To report a case series of two cases of granulomatosis with polyangiitis, previously known as Wegener granulomatosis, which developed macular necrosis, not previously associated with granulomatosis with polyangiitis, healed with fibrosis, despite aggressive immune-modulating therapy and good control of systemic disease. Case series of two cases with observation of treatment progress. The results reported the progress of response to treatment in the two cases, which resulted in the final outcome of fibrosis in the macula region, despite being on aggressive immune-modulating therapy and good systemic control. Granulomatosis with polyangiitis can be associated with macular necrosis leading to fibrosis, unresponsive to immune-modulating therapy.

[Surgical treatment with dacryocystitis and retinal detachment in a patient with Wegener granulomatosis].

Science.gov (United States)

Metoki, Tomomi; Kubo, Masabumi; Takano, Yoshiko; Nakamura, Hideo; Nakazawa, Mitsuru

2003-06-01

We report surgical treatment of a patient with dacryocystitis and retinal detachment (RD), which are rare ophthalmic involvements of Wegener granulomatosis (WG). The patient was a 26-year-old man with WG. He was diagnosed as having WG 4 years ago and he has been treated by maintenance doses of predonisolone and cyclophosphamide. Rheumatoid factor and serum antinuclear antibody were negative. Cytoplasmic pattern-antineutrophil cytoplasmic antibody (C-ANCA) and renal function were normal. He was found to have nasolacrimal duct obstruction and lattice degeneration bilaterally, retinal tear with RD in the left eye and tear without RD in the right eye. No sign of vasculitis was found in fluorescein angiography. Bilateral dacryocystorhinostomy was performed without any sign of postoperative necrosis of the wound. After the surgery, epiphora and eye discharge disappeared and lacrimal passage has been maintained without obstruction. The pathological findings of his nasal mucosa and lacrimal sac showed chronic inflammation and no typical changes of WG. There was no abnormal change in the conjunctiva and sclera after an uncomplicated scleral buckling surgery. We conclude that operations such as dacryocystorhinostomy and scleral buckling surgery may be performed successfully when WG is controlled within the normal limits of C-ANCA.

Paquimeningite hipertrófica e parotidite: manifestações raras da granulomatose de Wegener Hypertrophic pachymeningitis and parotiditis: unusual ocurrences of the Wegener's granulomatosis

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Ana Paula Monteiro Gomides

2004-08-01

Full Text Available A granulomatose de Wegener é uma vasculite sistêmica dos vasos de médio e pequeno calibre. Classicamente, há formação de granulomas com necrose no trato respiratório e glomerulonefrite necrosante. Embora seu acometimento mais comum envolva o trato respiratório superior, pulmões e rins, uma vasta gama de manifestações em vários órgãos e tecidos é descrita. Relatamos o caso de um paciente que, paralelamente às manifestações típicas da doença, desenvolveu algumas alterações raras como a parotidite e a paquimeningite hipertrófica. Diante de quadros graves, atípicos e/ou refratários ao tratamento convencional, torna-se necessário o aprofundamento no estudo e uso de novas armas terapêuticas.The wegener's granulomatosis is a systemic angiitis of small and medium caliber vessels. Normally, it is characterized by the formation of granulomas with necrosis in the respiratory system and necrosing glomerulonephritis. even though it is more common in the upper respiratory system, in the lung and in the kidney, a great range of manifestation in other different organs and tissues can be described. we report the case of a patient who, in parallel with the typical symptoms of this disease, developed some rare alteration such as the parotiditis and the hypertrophic pachymeningitis. in face of serious situations, severe disease, atypical or non responsive to conventional treatments, it is necessary a more detailed study of the case and the use of new therapeutic drugs.

Granulomatose de Wegener – Envolvimento otológico, nasal, laringotraqueal e pulmonar

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Sandra Figueiredo

2009-09-01

Full Text Available Resumo: A granulomatose de Wegener é uma vasculite sistémica rara e idiopática caracterizada pelo atingimento dos pequenos vasos. A doença atinge, preferencial-mente, as vias aérea superior e inferior e os rins, levando à formação de granulomas e necrose destes órgãos. As manifestações clínicas e o envolvimento orgânico variam largamente. O diagnóstico e tratamento precoce podem levar à recuperação total. No entanto, o atraso no diagnóstico pode ser fatal. Os autores apresentam o caso de uma doente de 33 anos com uma forma de apresentação grave e rara da doença, mas com uma evolução favorável após diagnóstico e tratamento adequado.Rev Port Pneumol 2009; XV (5: 929-935 Abstract: Wegener granulomatosis is a rare systemic idiopathic disease characterized by involvement of small vessels – medium and small arteries, venules, arterioles and ocasionally large arteries. This disease has predilection for the upper and lower respiratory tract and the kidney, with granulomatous inflamation and necrosis. Clinical manifestations and organ involvement of the disease vary widely. Early diagnosis and treatment may lead to a full recovery. Without treatment, Wegener’s granulomatosis can be fatal. The authors present a case of a 33 year-old female, with severe disease, but with good outcome, after adequate diagnosis and treatment.Rev Port Pneumol 2009; XV (5: 929-935 Palavras-chave: Granulomatose de Wegener, forma limitada grave, Key-words: Wegener´s granulomatosis, severe limited form

Diagnostic Consideration for Sinonasal Wegener’s Granulomatosis Clinically Mistaken for Carcinoma

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Cristina La Rosa

2013-01-01

Full Text Available We report a case of Wegener’s granulomatosis clinically mistaken for carcinoma in a 21-year-old girl presenting with an ulcerated mass of the nasopharynx associated with enlarged laterocervical nodes. The lesion was clinically suspected as malignant on the basis of clinical and radiological findings (namely, computed tomography scan and positron emission tomography. However, multiple biopsies were not conclusive for malignancy showing histological change suggestive of Wegener’s granulomatosis. A serum determination of cANCA supported the diagnosis of Wegener’s granulomatosis. Clinical findings and image studies suggested an erroneous diagnosis of malignancy whereas a definitive diagnosis of Wegener’s granulomatosis was achieved only after repeated biopsies thus leading to a correct therapeutic approach. The Wegener granulomatosis must be added to the list of the differential diagnoses of the masses of the nasopharynx associated with or without enlarged laterocervical nodes.

Granulomatosis with polyangiitis presenting as facial nerve palsy in a teenager.

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Wang, James C; Leader, Brittany A; Crane, Ryan A; Koch, Bernadette L; Smith, Matthew M; Ishman, Stacey L

2018-04-01

Granulomatosis with polyangiitis (GPA, previously known as Wegener's granulomatosis) is an autoimmune systemic small-vessel vasculitis, associated with the presence of anti-neurophil cytoplasmic antibodies with a cytoplasmic staining pattern (c-ANCA). It is characterized by necrotizing granulomas, usually affecting the airways and kidneys. GPA should be considered when patients do not improve despite adequate treatment of otologic symptoms, when patients have unspecific symptoms suggesting systemic disease (e.g. fever, malaise), or when other organs are involved (kidney, lungs, etc.). We present an interesting case of a 14-year-old female with eight-weeks of bilateral otalgia, unilateral facial nerve palsy, decreased appetite, and fatigue refractory to steroid, anti-viral, and antibiotic treatment ultimately diagnosed with GPA. Copyright © 2018. Published by Elsevier B.V.

Subglottic stenosis in granulomatosis with polyangiitis (Wegener's granulomatosis): Report of 4 cases.

Science.gov (United States)

Horta-Baas, Gabriel; Hernández-Cabrera, María Fernanda; Catana, Rocío; Pérez-Cristóbal, Mario; Barile-Fabris, Leonor Adriana

2016-01-01

Subglottic stenosis (SGS) in granulomatosis with polyangiitis (GPA) may result from active disease or from chronic recurrent inflammation. The objective of the study was to describe the clinical features and treatment of patients with subglottic stenosis. We retrospectively reviewed the medical records of all patients with SGS due to GPA diagnosed at Rheumatology deparment between January 2000 and June 2015. We present 4 cases of SGS at our department during a period of 15 years. The interval between the presentation of the GPA and SGS varied between 2 and 144 months. The leading symptoms of SGS were dyspnoea on exertion and stridor. Three patients presented SGS without evidence of systemic activity. Two patients presented SGS grade i and received tracheal dilatation; two recurred and three needed a tracheostomy due to severe airway-limiting stenosis. SGS presents high morbidity. Even though subglottic dilatation provides symptomatic relief, recurrences may present. Severe airway-limiting stenosis often requires tracheostomy. Copyright © 2015 Elsevier España, S.L.U. and Sociedad Española de Reumatología y Colegio Mexicano de Reumatología. All rights reserved.

Results of endoscopic surgery and intralesional steroid therapy for airway compromise due to tracheobronchial Wegener's granulomatosis

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S. A. R. Nouraei

2008-04-01

Full Text Available Background: Upper airway compromise due to tracheobronchial stenosis commonly occurs in patients with Wegener's Granulomatosis (WG. There is at present no consensus on the optimal management of this life-threatening condition. Objective: To assess the results of laryngo-tracheo-bronchoscopy, intralesional steroid therapy, laser surgery, and dilatation in managing obstructive tracheobronchial WG. Methods: Records of eighteen previously-untreated stridulous patients with obstructive tracheobronchial WG, treated between 2004 and 2006 were prospectively recorded on an airway database and retrospectively reviewed. Information about patient and lesion characteristics and treatment details were recorded. Treatment progress was illustrated using a timeline plot, and intervention-free intervals were calculated with actuarial analysis. Results: There were nine males and the average age at presentation was 40 (16 years [range 13–74]. There were thirteen patients with tracheal, and five patients with tracheal and bronchial lesions. The average tracheal lesion height was 8 (3 mm, located 23 (9 mm below the glottis. There were 1, 10 and 7 Myer-Cotton grade I, II and III lesions respectively. Mean intervention-free interval following minimally-invasive treatment was 26 (2.8 months. Following endobronchial therapy the median intervention-free interval was 22 months (p>0.8 vs. tracheal lesions. No patient required a tracheostomy or endoluminal stenting. Conclusions: Intralesional steroid therapy and conservative endoluminal surgery is an effective strategy for treating airway compromise due to active tracheal and bronchial WG, obviating the need for airway bypass or stenting. We recommend the combination of endotracheal dilatation, conservative laser surgery and steroid therapy as the standard of care for treating airway compromise due to obstructive tracheobronchial WG.

Mastoidite e paralisia facial como manifestações iniciais de Granulomatose de Wegener Mastoiditis and facial paralysis as initial manifestations of Wegener's Granulomatosis

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André Souza de Albuquerque Maranhão

2012-04-01

Full Text Available A Granulomatose de Wegener (GW é caracterizada por granulomas necrotizantes e vasculite. Sem tratamento a doença tem prognóstico pobre com índice de mortalidade de 90% em 2 anos. Diversos autores citam as manifestações otológicas no curso da GW, entretanto não é especificado em que momento da doença elas apareceram, isto é, se como manifestação inicial ou subsequente a outros achados. Objetivo: Descrever três casos confirmados de GW que apresentaram inicialmente mastoidite e evoluíram com paralisia facial periférica (PFP. Material e Método: Estudo de série de casos. Pacientes diagnosticados com GW que apresentaram inicialmente manifestações otológicas são descritos. Resultados: Os três casos descritos abriram o quadro com otalgia, otorreia e hipoacusia unilateral, associada a paralisia facial periférica ipsilateral. Tiveram resposta inadequada aos tratamentos instituídos o que motivou uma investigação de outras doenças associadas. Nessas circunstâncias, detectaram-se títulos positivos de ANCA-C em todos pacientes, confirmando-se o diagnóstico de GW, após período variável de investigação. Institui-se o tratamento para GW observando-se melhora do quadro clínico, regressão da PFP e melhora parcial dos limiares auditivos. Conclusão: Complicações de otites médias agudas (mastoidite e PFP refratárias as terapêuticas habituais impõem a investigação de doenças associadas e a GW deverá ser pesquisada para que se possa fazer o diagnóstico o mais precocemente possível, alterando desta forma o prognóstico destes pacientes.Wegener's Granulomatosis (WG is characterized by necrotizing granulomas and vasculitis. If left untreated, the prognosis is poor - a 90% mortality rate within 2 years. Several authors have described the otologic manifestations of WG; these authors, however, have not mentioned the stage of the disease in which these findings present - whether as initial manifestations or subsequent to

The ANCA Vasculitis Questionnaire (AAV-PRO©)

Science.gov (United States)

2017-05-01

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

Active disease and residual damage in treated Wegener's granulomatosis: an observational study using pulmonary high-resolution computed tomography

International Nuclear Information System (INIS)

Komocsi, Andras; Reuter, Michael; Heller, Martin; Murakoezi, Henriette; Gross, Wolfgang L.; Schnabel, Armin

2003-01-01

The purpose of this study was to determine to what extent high-resolution computed tomography (HRCT) of the lungs can distinguish active inflammatory disease from inactive cicatricial disease in patients treated for Wegener's granulomatosis (WG). Twenty-eight WG patients with active pulmonary disease underwent a first HRCT examination immediately before standard immunosuppressive treatment and a second examination after clinical remission had been achieved. Lesions remaining after treatment were categorized as residual damage and were compared with findings during active disease to see by what features active and cicatricial disease can be distinguished. During active disease 17 patients had nodules/masses, 12 had ground-glass opacities, 6 had septal lines and 6 had non-septal lines. After treatment, ground-glass opacities had resolved completely. Nodules/masses had resolved in 8 patients and had diminished in 7 patients. Residual nodules were distinguished from nodules/masses in active disease by lack of cavitation and a diameter of mostly <15 mm. In one-third of patients lines resolved, but in 8 instances new lines evolved during immunosuppression. During a follow-up period of a median 26.5 months (range 20.0-33.8), patients with residual nodules or lines had no more relapses than patients with completely cleared lungs. Treated pulmonary WG leaves substantial residual damage. High-resolution CT does assist in the distinction between active and inactive lesions. Ground-glass opacities, cavitating nodules/masses and masses measuring more than 3 cm represent active disease ordinarily. Non-cavitary small nodules and septal or non-septal lines can be either active or cicatricial lesions. The nature of these lesions needs to be clarified by longitudinal observation. (orig.)

Wegener’s granulomatosis and pyoderma gangrenosum – rare causes of facial ulcerations

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Karolina Kędzierska

2016-03-01

Full Text Available Background: Pyoderma gangrenosum (PG is caused by immune system dysfunction, and particularly improper functioning of neutrophils. At least half of all PG patients also suffer from autoimmunological diseases, one of which is Wegener granulomatosis (WG. The purpose of this article was to compare cases of patients with WG and PG in terms of their clinical course, histopathology, and applied treatment. In both, histopathological features are not fully distinct. Data from microbiological and immunological evaluation and clinical presentation are required to establish the diagnosis. We also present the case of a patient with WG and deep facial skin lesions not responding to standard treatment. Methods: Systematic review of the literature in PubMed using the search terms “Wegener granulomatosis AND Pyoderma gangrenosum” and case report. Results: The finding of 22 reports in the literature (PubMed suggests that it is a rare phenomenon. This study revealed a similar rate of comorbidity of WG and PG in both genders and an increased incidence of both diseases after the age of 50. Among skin lesions there was a dominance of ulceration, most often deep and painful, covering a large area with the presence of advanced necrosis and destruction of the surrounding tissue. The most common location proved to be the cervical-cephalic area. The most popular treatment included steroids with cyclophosphamide. Discussion: The rarity of the coexistence of these two diseases results in a lack of effective therapy. In such cases sulfone derivatives are still effective and provide an alternative to standard immunosuppression methods. Hyperbaric therapy and plasmapheresis can also play an important complementary role.

Pediatric Vasculitis Initiative

Science.gov (United States)

2017-05-01

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

Genetically distinct subsets within ANCA-associated vasculitis.

LENUS (Irish Health Repository)

Lyons, Paul A

2012-07-19

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis is a severe condition encompassing two major syndromes: granulomatosis with polyangiitis (formerly known as Wegener\\'s granulomatosis) and microscopic polyangiitis. Its cause is unknown, and there is debate about whether it is a single disease entity and what role ANCA plays in its pathogenesis. We investigated its genetic basis.

Epidemiology of granulomatosis with polyangiitis (Wegener's granulomatosis) in Northern Italy: a 15-year population-based study.

Science.gov (United States)

Catanoso, Mariagrazia; Macchioni, Pierluigi; Boiardi, Luigi; Manenti, Lucio; Tumiati, Bruno; Cavazza, Alberto; Luberto, Ferdinando; Pipitone, Nicolò; Salvarani, Carlo

2014-10-01

To investigate the epidemiology of granulomatosis with polyangiitis (GPA) over a 15-year period in a defined area of northern Italy. All patients with incident GPA diagnosed from January 1, 1995 to December 31, 2009 living in the Reggio Emilia area were identified by looking at computerized hospital discharge diagnoses, by contacting Reggio Emilia Hospital physicians and community-based specialists, and by checking the databases of the pathology and the laboratory departments and the Reggio Emilia district database for rare diseases. Patients were classified according to the European Medicines Agency (EMA) algorithm. Patients were followed up from the time of diagnosis until either their death or December 31, 2011. For each case, we identified 20 control subjects from the same geographic area matched for age and gender. A total of 18 patients (7 men and 11 women) with GPA were identified. The overall age- and sex-adjusted incidence rate (IR) was 2.4 per million (95% CI: 1.2-3.5). The mean annual IR increased from 1.7/million/year during 1995-1999 to 3.4 during 2005-2009. The highest IR occurred in females aged 70-79 years (13.5 per million; 95% CI: 5.0-30.0) and in males aged ≥ 80 years (14.9 per million; 95% CI: 2.5-49.4). The prevalence of GPA on December 31, 2009 was 34.3 per million (95% CI: 20.3-54.2). The point prevalence per million increased from 17.8 (95% CI: 7.7-35.1) in 1999 to 34.3 (95% CI: 20.3-54.2) in 2009. Survival among individuals with GPA was significantly reduced compared to that observed in the matched control population (p < 0.001). In the Italian population, GPA is very uncommon and GPA patients have reduced survival. Copyright © 2014 Elsevier Inc. All rights reserved.

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

Science.gov (United States)

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

1998-05-01

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

Long-term patient survival in ANCA-associated vasculitis

DEFF Research Database (Denmark)

Flossmann, Oliver; Berden, Annelies; de Groot, Kirsten

2011-01-01

Wegener's granulomatosis and microscopic polyangiitis are antineutrophil cytoplasm antibodies (ANCA)-associated vasculitides with significant morbidity and mortality. The long-term survival of patients with ANCA associated vasculitis treated with current regimens is uncertain.......Wegener's granulomatosis and microscopic polyangiitis are antineutrophil cytoplasm antibodies (ANCA)-associated vasculitides with significant morbidity and mortality. The long-term survival of patients with ANCA associated vasculitis treated with current regimens is uncertain....

Impact of Vasculitis on Employment and Income

Science.gov (United States)

2016-01-26

Vasculitis; Systemic Vasculitis; Behcet's Disease; CNS Vasculitis; Cryoglobulinemic Vasculitis; Eosinophilic Granulomatosis; Temporal Arteritis; Wegener Granulomatosis; Henoch-Schoenlein Purpura; Microscopic Polyangiitis; Polyarteritis Nodosa (PAN); Takayasu's Arteritis; Urticarial Vasculitis

Vasculitis Pregnancy Registry

Science.gov (United States)

2018-04-30

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

Biologisk behandling af sjaeldne inflammatoriske reumatiske sygdomme

DEFF Research Database (Denmark)

Baslund, Bo

2008-01-01

The current status of the use of biological medicine in the treatment of adult onset morbus still, Wegeners granulomatosis and systemic lupus erythematosus (SLE) is reviewed. The need for controlled trials is emphasized. Anti-CD20 treatment for SLE patients with kidney involvement and patients...... with Wegeners granulomatosis seems promising. Anti-TNF and IL1 receptor antagonist can control disease activity in most patients with adult morbus still....

VCRC Tissue Repository

Science.gov (United States)

2018-04-23

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

Characteristics and Outcomes of Granulomatosis With Polyangiitis (Wegener) and Microscopic Polyangiitis Requiring Renal Replacement Therapy

DEFF Research Database (Denmark)

Hruskova, Zdenka; Stel, Vianda S; Jayne, David

2015-01-01

and matched nondiabetes patients. Patient and transplant survival after kidney transplantation, adjusted for time period and country, was better in AAV than in matched nondiabetes patients (HRs of 0.81 [95% CI, 0.67-0.99] and 0.82 [95% CI, 0.69-0.96], respectively). LIMITATIONS: No data for extrarenal...... for microscopic polyangiitis (prevailing in Southern Europe). Kidney transplantation was performed in 558 (22.2%) patients with vasculitis. The 10-year probability for survival on RRT after day 91 was 32.5% (95% CI, 29.9%-35.1%) in patients with vasculitis. Survival on RRT after day 91 did not differ between AAV...... manifestations, treatment, and relapses. CONCLUSIONS: Geographical differences in the incidence of RRT for kidney failure due to granulomatosis with polyangiitis and microscopic polyangiitis copied their distribution in the general population. Overall survival on RRT after day 91 for patients with AAV...

An overlap of granulomatosis with polyangiitis and eosinophilic granulomatosis with polyangiitis

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Sujit Surendran

2017-01-01

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

[Biological treatment of rare inflammatory rheumatic diseases

DEFF Research Database (Denmark)

Baslund, B.

2008-01-01

The current status of the use of biological medicine in the treatment of adult onset morbus still, Wegeners granulomatosis and systemic lupus erythematosus (SLE) is reviewed. The need for controlled trials is emphasized. Anti-CD20 treatment for SLE patients with kidney involvement and patients wi...... with Wegeners granulomatosis seems promising. Anti-TNF and IL1 receptor antagonist can control disease activity in most patients with adult morbus still Udgivelsesdato: 2008/6/9...

Early Outcomes in Children With Antineutrophil Cytoplasmic Antibody-Associated Vasculitis

DEFF Research Database (Denmark)

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

2017-01-01

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

Journey of Patients With Vasculitis From First Symptom to Diagnosis

Science.gov (United States)

2018-01-18

Vasculitis; Systemic Vasculitis; Behcet's Disease; CNS Vasculitis; Cryoglobulinemic Vasculitis; Eosinophilic Granulomatous Vasculitis; Temporal Arteritis; Giant Cell Arteritis; Granulomatosis With Polyangiitis; Wegener Granulomatosis; Henoch Schonlein Purpura; IgA Vasculitis; Microscopic Polyangiitis; Polyarteritis Nodosa; Takayasu Arteritis; Urticarial Vasculitis

Pulmonary lymphomatoid granulomatosis in a 4-year-old girl

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Lee, Subin; Kang, Mi-Jin; Lee, Jihae [Inje University Sanggye Paik Hospital, Department of Radiology, 1342, Dongilro, Nowon-gu, Seoul (Korea, Republic of); Kim, Hyun-Jung [Inje University Sanggye Paik Hospital, Department of Pathology, 1342, Dongilro, Nowon-gu, Seoul (Korea, Republic of); Kim, Hyo-Bin [Inje University Sanggye Paik Hospital, Department of Pediatrics, 1342, Dongilro, Nowon-gu, Seoul (Korea, Republic of)

2015-07-15

Lymphomatoid granulomatosis is a rare lymphoproliferative disease associated with the Epstein-Barr virus that commonly affects the lung. There is limited literature on cases of pediatric lymphomatoid granulomatosis. Half of all cases of lymphomatoid granulomatosis develop during the treatment of leukemia. Herein, we describe a case of lymphomatoid granulomatosis in a previously healthy child without leukemia. (orig.)

Genetically distinct subsets within ANCA-associated vasculitis

DEFF Research Database (Denmark)

Lyons, Paul A; Rayner, Tim F; Trivedi, Sapna

2012-01-01

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis is a severe condition encompassing two major syndromes: granulomatosis with polyangiitis (formerly known as Wegener's granulomatosis) and microscopic polyangiitis. Its cause is unknown, and there is debate about whether it is a single...

Granulomatosis with Polyangiitis (Wegener's)

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... American College of Rheumatology Committee on Communications and Marketing. This information is provided for general education only. Individuals should consult a qualified health care provider for professional medical advice, diagnosis and treatment of a medical ...

Meningitis granulomatosa, glomerulonefritis rápidamente progresiva y vasculitis Granulomatous meningitis, crescentic glomerulonephritis and vasculitis

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Ana Ludueña

2011-08-01

Full Text Available El compromiso meníngeo es una manifestación infrecuente de la granulomatosis de Wegener. Puede manifestarse como cefalea con hiperproteinorraquia y engrosamiento de la duramadre con aspecto granulomatoso, que se observa en la resonancia magnética. Presentamos un varón de 57 años con granulomatosis de Wegener que debutó con compromiso de vías aéreas superiores, oídos, órbitas y meningitis granulomatosa asintomática y que posteriormente evolucionó con mononeuritis múltiple y glomerulonefritis crescéntica ANCA positiva. La presencia de ANCA y el compromiso sistémico (vías aéreas superiores, oído, órbitas, nervios periféricos, duramadre y glomerulonefritis rápidamente progresiva permitieron en este caso llegar a un diagnóstico de certeza e iniciar el tratamiento inmunosupresor combinado (corticoides y ciclofosfamida. Evolucionó con remisión clínica y serológica (negativización de ANCA, pero persistiendo leve deterioro secuelar auditivo y de la función renal, sin recidiva de la enfermedad de base.Meningeal involvement is an infrequent manifestation of Wegener's granulomatosis. Clinical manifestations can be headache with high protein level in the cerebrospinal fluid and an enhanced MRI signal of granulomatous thickening of the duramater in the brain. We report a 57 year-old male with Wegener granulomatosis with onset manifestations of asymptomatic granulomatous meningitis, upper respiratory tract, ears and orbits involvement. He progressively developed ANCA positive multiple mononeuritis and crescentic glomerulonephritis. The diagnostic confirmation of Wegener's granulomatosis based on a positive ANCA test and on the evidence of systemic disease (crescentic glomerulonephritis and involvement of the upper respiratory tract, ears, orbits, peripheral nerves and duramater allowed a prompt initiation of aggressive immunosuppressive treatment with systemic cyclophosphamide and high - dosis corticosteroids. The patient entered

Granulomatous Lymphoproliferative Disorders: Granulomatous Slack Skin and Lymphomatoid Granulomatosis.

Science.gov (United States)

Gangar, Pamela; Venkatarajan, Sangeetha

2015-07-01

Granulomatous cutaneous T-cell lymphomas (CTCL) and lymphomatoid granulomatosis are considered granulomatous lymphoproliferative disorders. The most common types of granulomatous CTCL are granulomatous mycosis fungoides and granulomatous slack skin. Lymphomatoid granulomatosis is a rare Epstein-Barr virus driven lymphoproliferative disorder. This article reviews the etiopathogenesis, clinical presentation, systemic associations, and management of both granulomatous slack skin syndrome and lymphomatoid granulomatosis. Copyright © 2015 Elsevier Inc. All rights reserved.

Educational Needs of Patients With Systemic Vasculitis

Science.gov (United States)

2014-07-11

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

Pulmonary talc granulomatosis in a cocaine sniffer.

Science.gov (United States)

Oubeid, M; Bickel, J T; Ingram, E A; Scott, G C

1990-07-01

The development of pulmonary granulomatosis following intravenous injection of medications intended for oral use has been well described previously. Talc is the most commonly implicated agent. We present a case of talc granulomatosis which developed in a patient following cocaine sniffing and suggest that this may be the cause of development of granulomata in drug addicts who deny any history of intravenous drug abuse.

Eosinophilic Granulomatosis with Polyangiitis, formerly Churg-Strauss Syndrome (EGPA)

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... Strauss Syndrome (EGPA) Eosinophilic Granulomatosis with Polyangiitis, formerly Churg-Strauss Syndrome (EGPA) First Description Who gets EGPA (the “ ... granulomatosis with polyangiitis (EGP), formerly known as the Churg-Strauss Syndrome , is a systemic vasculitis. This disease was ...

[Granulomatosis with polyangiitis manifested as diabetes insipidus].

Science.gov (United States)

Pátek, Ondřej; Horáčková, Miroslava; Vítová, Lenka; Horváth, Rudolf; Háček, Jaromír; Schück, Otto

The case report shows a surprising presentation of pulmonary granulomatosis with polyangiitis (GPA) through symptoms of diabetes insipidus (DI) with granulomatous infiltration of the pituitary gland. The pituitary hormonal dysfunction as a result of granulomatosis of the pituitary gland is rare. Several studies have demonstrated that the incidence of the pituitary dysfunction reaches approx. 1 % of the patients with GPA. However it is mostly presented in patients with the disease already diagnosed. The patient described by us had no clinical expressions of GPA in the respiratory tract. He presented with polyuria and polydipsia. It was not until a more detailed examination of these symptoms was performed that a focal lung disease was detected and diagnosed as GPA. diabetes insipidus - granulomatosis with polyangiitis - granulomatous infiltration of the pituitary gland - pituitary hormonal dysfunction.

Case report

African Journals Online (AJOL)

iamong

2016-08-11

pulmonary capillaritis, bland pulmonary hemorrhage and diffuse alveolar damage) [3]. Generally, the pulmonary alveolar hemorrhage is caused by Wegener's granulomatosis, microscopic polyangitis, antiphospholipid antibody ...

Prevalência das manifestações clínicas iniciais da granulomatose de Wegener no Brasil: relato de seis casos e revisão da literatura Wegener's granulomatosis: prevalence of the initial clinical manifestations - report of six cases and review of the literature

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Carlos Ewerton Maia Rodrigues

2010-04-01

Full Text Available OBJETIVOS: Descrever as manifestações clínicas iniciais da Granulomatose de Wegener (GW diagnosticada no Brasil. PACIENTES E MÉTODOS: Análise retrospectiva de seis prontuários do Serviço de Reumatologia do Hospital Geral de Fortaleza (HGF, assim como a realização de um levantamento bibliográfico dos casos de GW descritos no Brasil obtidos dos bancos de dados LILACS, SciELO e MEDLINE. RESULTADOS: O estudo identificou 49 pacientes; 15 (31% do sexo masculino e 34 (69% do sexo feminino. A forma sistêmica ocorreu em 35 pacientes (73%: 28 adultos, cinco crianças e dois adolescentes. A doença limitada ocorreu em 13 adultos e uma criança. A média da idade adulta no início da doença foi de 42,2 anos (18 a 65 anos. O quadro clínico agudo, com sintomas há menos de três meses do diagnóstico, ocorreu em 41% (20/49 da casuística e a forma insidiosa, em 59% (29/49 dos pacientes. A prevalência das manifestações clínicas iniciais nos adultos com doença sistêmica (n = 28 foi 64% (18/28 das vias aéreas superiores (VAS, 36% (10/28 pulmonares, 18% (5/28 renais, 25% (7/28 oculares, 11% (3/28 cutâneas, 25% (7/28 musculoesqueléticas e 7% (2/28 neurológicas. Na forma limitada do adulto (n = 13, os sintomas prevalentes foram 84% (11/13 VAS, 23% (3/13 oculares e 15% (2/13 pulmonares. CONCLUSÃO: No Brasil, a prevalência das manifestações clínicas iniciais da GW foi semelhante aos resultados da literatura. A falta de especificidade dos sintomas pode retardar o diagnóstico na forma insidiosa da doença e aumentar a morbimortalidade das formas agudas.OBJECTIVES: To describe the initial clinical manifestations of Wegener's Granulomatosis (WG in Brazil. PATIENTS AND METHODS: Retrospective analysis of six medical records of WG patients followed-up at the Rheumatology Department of Hospital Geral of Fortaleza (HGF, as well as a bibliographic survey of cases of WG in Brazil on LILACS, SciELO, and MEDLINE databases. RESULTS: The study

"Wegener’s granulomatosis in a patient with Rheumatoid arthritis "

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"Ahmadi Nejad Z

2001-06-01

Full Text Available Wegener’s Granulomatosis is a necrotizing granulomatous vasculitis involving small and medium sized vessels. The syndrome is classically defined as having involvement of kidney, lungs and upper respiratory tract (i.e. sinuses. Wegener’s Granulomatosis may be present in other autoimmune or inflammatory diseases, particulary systemic lupus erythematosis (SLE, but most frequently has been associated with polyarteritis and glumerulonephritis.We present a case of wegener’s Granulomatosis (WG in a middle age lady with Rheumatiod Arthritis (RA; and discuss the implications of these two conditions co-existing in one patient. As for as we are aware, through medline and interent research, this is probably the fourth case with such as association of WG and RA and the fist one in Iran.Presentation of new uncontrollable sing and symptoms, in a previously well controlled RA patient, might suggest a new overlapping syndrome like Wegener’s Granulomatosis besides to flare up of previous disease as differential diagnosis

Reproductive Health in Men and Women With Vasculitis

Science.gov (United States)

2014-06-25

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

Clinical, imaging and histopathological features of isolated CNS lymphomatoid granulomatosis

International Nuclear Information System (INIS)

Patil, Anil Kumar; Alexander, Mathew; Nair, Bijesh; Chacko, Geeta; Mani, Sunithi; Sudhakar, Sniya

2015-01-01

Lymphomatoid granulomatosis is a rare systemic angiocentric/angiodestructive, B cell lymphoproliferative disorder. Central nervous system involvement occurs as part of systemic disease. Isolated central nervous system disease is rare with only few case reports. A 53-year-old male presented with progressive cognitive decline, extrapyramidal features, and altered sensorium with seizures over the last 4 years. His magnetic resonance imaging (MRI) of brain showed multiple small enhancing nodules in subependymal/ependymal regions and along the vessels. Brain biopsy showed atypical lymphohistiocytic infiltrate suggestive of lymphomatoid granulomatosis. There was no evidence of systemic disease; thus, isolated central nervous system lymphomatoid granulomatosis was diagnosed

Culturele Veranderingen: het wegen waard?

NARCIS (Netherlands)

Mérove Gijsberts

1993-01-01

Verslag van een verkennend onderzoek naar de effecten van weging op de bestanden 'Culturele Verkenningen'. In kaart wordt gebracht in hoeverre er sprake is van selectieve non-respons in de databestanden. Het verschijnsel non-respons wordt nader belicht, evenals het begrip 'wegen',

A Case of Wegener’s Granulomatosis Presenting with Unilateral Facial Nerve Palsy

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Roy Ujjawal

2016-01-01

Full Text Available Wegener’s granulomatosis or granulomatosis with polyangiitis is a necrotizing vasculitis affecting both arterioles and venules. The disease is characterized by the classical triad involving acute inflammation of the upper and lower respiratory tracts with renal involvement. However, the disease pathology can affect any organ system. This case presents Wegener’s granulomatosis presenting with facial nerve palsy as the first manifestation of the disease, which is rarely reported in medical literature.

Increased frequency of CCR4+ and CCR6+ memory T-cells including CCR7+CD45RAmed very early memory cells in granulomatosis with polyangiitis (Wegener's)

OpenAIRE

Fagin, Ursula; Pitann, Silke; Gross, Wolfgang L; Lamprecht, Peter

2012-01-01

Introduction Chemokine receptors play an important role in mediating the recruitment of T cells to inflammatory sites. Previously, small proportions of circulating Th1-type CCR5+ and Th2-type CCR3+ cells have been shown in granulomatosis with polyangiitis (GPA). Wondering to what extent CCR4 and CCR6 expression could also be implicated in T cell recruitment to inflamed sites in GPA, we investigated the expression of CCR4 and CCR6 on T cells and its association with T cell diversity and polari...

Antineutrophil cytoplasmic autoantibody-associated small-vessel vasculitis

NARCIS (Netherlands)

Kallenberg, Cees G. M.

Purpose of reviews This review focuses on recent advance in the diagnosis pathogenesis and treatment of antineutrophil cytoplasmic autoantibody-associated small-vessel vasculitis. Recent findings Antineutrophil cytoplasmic autoantibodies are closely associated with Wegener's granulomatosis and

Brief Report

DEFF Research Database (Denmark)

Faurschou, Mikkel; Westman, Kerstin; Rasmussen, Niels

2012-01-01

The NORAM (Nonrenal Wegener's Granulomatosis Treated Alternatively with Methotrexate [MTX]) trial demonstrated that MTX can replace cyclophosphamide (CYC) as remission-inducing treatment for patients with newly diagnosed early systemic antineutrophil cytoplasmic antibody-associated vasculitis. Du...

A Candidate Gene Approach to ANCA-Associated Vasculitis Reveals Links to the C3 and CTLA-4 Genes but not to the IL1-Ra And Fcγ-RIIa Genes.

OpenAIRE

Persson, Ulf; Gullstrand, Birgitta; Pettersson, Åsa; Sturfelt, Gunnar; Truedsson, Lennart; Segelmark, Mårten

2013-01-01

Background/Aims: The aim of the study is to search for associations between Antineutrophil cytoplasm antibody (ANCA)-associated vasculitis (AAV) and polymorphisms in the genes of four key molecules possibly involved in different pathogenic pathways; complement C3, CTLA-4, Fcγ-RIIa and IL1-Ra. Patients and Methods: Patients with AAV (n=105) subgrouped as microscopic polyangiitis or granulomatosis with polyangiitis (Wegener's granulomatosis) and myeloperoxidase (MPO) or proteinase 3 (PR3) A...

Pauci-immune necrotizing glomerulonephritis

NARCIS (Netherlands)

Rutgers, Abraham; Sanders, Jan S F; Stegeman, Coen A; Kallenberg, Cees G M

Pauci-immune necrotizing glomerulonephritis is the most frequent cause of rapidly progressive glomerulonephritis and, in most cases, is associated with antineutrophil cytoplasmic antibodies (ANCA). It is either the renal manifestation of Wegener's granulomatosis, microscopic polyangiitis of

Validation and correction of rainfall data from the WegenerNet high density network in southeast Austria

Science.gov (United States)

O, Sungmin; Foelsche, U.; Kirchengast, G.; Fuchsberger, J.

2018-01-01

Eight years of daily rainfall data from WegenerNet were analyzed by comparison with data from Austrian national weather stations. WegenerNet includes 153 ground level weather stations in an area of about 15 km × 20 km in the Feldbach region in southeast Austria. Rainfall has been measured by tipping bucket gauges at 150 stations of the network since the beginning of 2007. Since rain gauge measurements are considered close to true rainfall, there are increasing needs for WegenerNet data for the validation of rainfall data products such as remote sensing based estimates or model outputs. Serving these needs, this paper aims at providing a clearer interpretation on WegenerNet rainfall data for users in hydro-meteorological communities. Five clusters - a cluster consists of one national weather station and its four closest WegenerNet stations - allowed us close comparison of datasets between the stations. Linear regression analysis and error estimation with statistical indices were conducted to quantitatively evaluate the WegenerNet daily rainfall data. It was found that rainfall data between the stations show good linear relationships with an average correlation coefficient (r) of 0.97 , while WegenerNet sensors tend to underestimate rainfall according to the regression slope (0.87). For the five clusters investigated, the bias and relative bias were - 0.97 mm d-1 and - 11.5 % on average (except data from new sensors). The average of bias and relative bias, however, could be reduced by about 80 % through a simple linear regression-slope correction, with the assumption that the underestimation in WegenerNet data was caused by systematic errors. The results from the study have been employed to improve WegenerNet data for user applications so that a new version of the data (v5) is now available at the WegenerNet data portal (www.wegenernet.org).

New advances in the pathogenesis of ANCA-associated vasculitides

NARCIS (Netherlands)

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

2009-01-01

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

Plasma exchange and glucocorticoid dosing in the treatment of anti-neutrophil cytoplasm antibody associated vasculitis (PEXIVAS)

DEFF Research Database (Denmark)

Walsh, Michael; Merkel, Peter A; Peh, Chen Au

2013-01-01

Granulomatosis with polyangiitis (GPA, Wegener's) and microscopic polyangiitis (MPA) are small vessel vasculitides collectively referred to as anti-neutrophil cytoplasm antibody-associated vasculitis (AAV). AAV is associated with high rates of morbidity and mortality due to uncontrolled disease...

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

Science.gov (United States)

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

2014-11-21

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

Pathogenesis of PR3-ANCA associated vasculitis

NARCIS (Netherlands)

Kallenberg, C. G. M.

2008-01-01

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

Accelerated atherosclerosis in patients with systemic autoimmune diseases

NARCIS (Netherlands)

De Leeuw, K.; Kallenberg, Cees; Bijl, Marc; Shoenfeld, Y.; Gershwin, M.E.; Shoenfeld, Y; Gershwin, ME

2005-01-01

Systemic autoimmune diseases such as systemic lupus erythematosus and Wegener's granulomatosis are associated with a significantly increased prevalence of cardiovascular disease (CVD) compared with age- and sex-matched controls. Many risk factors are involved in the pathogenesis of atherosclerosis,

Wegener’s granulomatosis mimicking inflammatory bowel disease and presenting with chronic enteritis

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Shahedi K

2013-10-01

Full Text Available Kamyar Shahedi,1,2 Ramy Magdy Hanna,1,2 Oleg Melamed,1,2 James Wilson2,31Department of Medicine Olive-View UCLA Medical Center, Sylmar, CA, 2David Geffen School of Medicine at UCLA, Los Angeles, CA, 3UCLA Medical Center-UCLA Stone Center, Los Angeles, CA, USAAbstract: Wegener’s granulomatosis, also known as anti-neutrophil cytoplasmic antibody (ANCA-associated vasculitis, is a small vessel vasculitis with primarily pulmonary, renal, and sinus disease manifestations. The prevalence of Wegener’s granulomatosis is three cases per 100,000 patients. Cardiovascular, neurologic, cutaneous, and joint manifestations have been reported in many case reports and case series. Gastrointestinal manifestations are less noted in Wegener’s granulomatosis, although they have been previously reported in the form of intestinal perforation and intestinal ischemia. Additionally, there are characteristic findings of vasculitis that are noted with active Wegener’s granulomatosis of the small bowel. We report a case of an elderly patient who presented with weight loss, diarrhea, and hematochezia. His symptoms were chronic and had lasted for more than 1 year before diagnosis. Inflammatory bowel disease or chronic enteritis due to Salmonella arizonae because of reptile exposure originally were suspected as etiologies of his presentation. The findings of proteinuria, renal failure, and pauci-immune glomerulonephritis on renal biopsy, in conjunction with an elevated c-ANCA titer, confirmed the diagnosis of Wegener’s granulomatosis with associated intestinal vasculitis. This case demonstrates an atypical presentation of chronic duodenitis and jejunitis secondary to Wegener’s granulomatosis, which mimicked inflammatory bowel disease.Keywords: ANCA-associated vasculitis, Wegener’s syndrome, pauci-immune glomerulonephritis, Salmonella arizonae, inflammatory bowel disease

Characteristics of patients with orofacial granulomatosis.

LENUS (Irish Health Repository)

McCartan, B E

2011-10-01

Orofacial granulomatosis has mostly been described in reports of very small numbers of cases. Few large case groups have been described. The aim of this study was to describe the demographics, symptoms, clinical features and laboratory findings in a large cohort of cases.

Pituitary granulomatosis with polyangiitis

OpenAIRE

Slabu, Hannah; Arnason, Terra

2013-01-01

Granulomatosis with polyangiitis (GPA) is a small vessel vasculitis that can affect several organs, most commonly the respiratory tract and kidneys. Pituitary involvement is exceptionally rare. Most case reports of GPA of the pituitary gland have been described in middle-aged women who have concomitant ears, nose and throat involvement. The most frequent manifestation is diabetes insipidus due to a preponderance of posterior pituitary infiltration. The majority of cases sustain permanent dama...

An Unusual Radiologic Manifestation of Pulmonary Tuberculosis with Bilateral Multiple Lung Nodules and Diffuse Alveolar Hemorrhage: A Case Report

Energy Technology Data Exchange (ETDEWEB)

Jeong, Seo In; Seon, Hyun Ju; Kim, Yun Hyeon [Dept. of Radiology, Chunnam National University Hospital, Gwangju (Korea, Republic of); Choi, Sung [Dept. of Radiology, Chunnam National University Hwasun Hospital, Hwasun(Korea, Republic of)

2011-12-15

Pulmonary tuberculosis presenting as bilateral multiple lung nodules or diffuse alveolar hemorrhage is very rare. Here, we report a case of pulmonary tuberculosis presenting as bilateral multiple lung nodules and diffuse alveolar hemorrhage mimicking granulomatous vasculitis, such as Wegener's granulomatosis.

Pathogenesis of pulmonary vasculitis

NARCIS (Netherlands)

Heeringa, P; Schreiber, A; Falk, RJ; Jennette, JC

2004-01-01

Vasculitis is inflammation of blood vessels and can affect any type of vessel in any organ. Pulmonary vasculitis usually is a component of a systemic small vessel vasculitis. Three major forms of small vessel vasculitis that often affect the lungs are Wegener's granulomatosis, microscopic

[Biological treatment of rare inflammatory rheumatic diseases

DEFF Research Database (Denmark)

Baslund, B.

2008-01-01

The current status of the use of biological medicine in the treatment of adult onset morbus still, Wegeners granulomatosis and systemic lupus erythematosus (SLE) is reviewed. The need for controlled trials is emphasized. Anti-CD20 treatment for SLE patients with kidney involvement and patients wi...

Adolescente femenino con granulomatosis de Wegener fulminante

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S C Scheffler-Mendoza

2017-01-01

Full Text Available Adolescente femenino de 13 años de edad, originaria de Guerrero, grupo étnico mixteco, padres analfabetos y con poco entendimiento del español. Sin otros antecedentes de importancia para el padecimiento. Inició un mes previo con la aparición de una pápula hipercrómica en párpado superior derecho que en los 10 días previos al ingreso aumentó de volumen generando proptosis. Al interrogatorio se negó dolor, lagrimeo, fiebre, o pérdida de peso. Ingresó con fiebre (39.0°C, palidez generalizada, proptosis derecha con exposición de córnea y conjuntiva, eritema periorbitario, movimientos oculares y agudeza visual disminuidos (i.e. contaba dedos a 1 m, pupila con tendencia a midriasis, hiperreactividad a la luz, y fondo de ojo con papila hiperémica edematosa y tortuosidad peripapilar. El resto de exploración no mostró datos relevantes. Se hospitalizó para iniciar tratamiento antimicrobiano parenteral de amplio espectro (i.e. ceftriaxona y vancomicina y abordar proptosis unilateral.

CUTANEOUS VASCULITIDES AND CUTANEOUS MANIFESTATIONS OF SYSTEMIC VASCULITIDES

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Vesna Jurčić

2009-06-01

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

Pulmonary lymphomatoid granulomatosis in seven dogs (1976-1987)

International Nuclear Information System (INIS)

Berry, C.R.; Moore, P.F.; Thomas, W.P.; Sisson, D.; Koblik, P.D.

1990-01-01

Seven dogs with pulmonary lymphomatoid granulomatosis were reviewed. The disease occurred in six large-breed and one small-breed dogs. The dogs were five to 14 years old (mean, 8.4; median, 7), and four of seven dogs were males. Three dogs had been previously treated with adulticide therapy for canine dirofilariasis. Clinical histories included a progressive respiratory disease characterized by varying degrees of cough, dyspnea, exercise intolerance, and weight loss. Thoracic radiographic features included hilar lymphadenopathy, pulmonary masses of varying sizes, and mixed pulmonary patterns of lobar consolidation with ill-defined interstitial and alveolar pulmonary infiltrates. Cardiovascular changes compatible with chronic dirofilariasis were present in three dogs. The clinical course was usually progressive and fatal. The survival time ranged from six days to four years (mean, 12.5 mos; median, 3 mos). Gross and histologic features included mass lesions with areas of necrosis that replaced normal pulmonary architecture. Cytologically, these lesions were characterized by infiltration with pleomorphic, angioinvasive mononuclear cells that often resulted in vascular obliteration. The infiltrating cells resembled large lymphoid cells that possessed large hyperchromatic nuclei and small amounts of cytoplasm. Systemic lymphoid neoplasia with peripheral lymphadenopathy was diagnosed in two dogs. In both cases, lymph-node cytology was similar to the cellular infiltrates found in the lungs and consistent with a diagnosis of lymphomatoid granulomatosis. These features are compared with previously reported cases of canine lymphomatoid granulomatosis and those features identified in a similar disease described in man

Synchronous advanced pulmonary tuberculosis and acute virus myocarditis mimicked wegener granulomatosis in a 26-year-old man: A case report

Directory of Open Access Journals (Sweden)

Pešut Dragica P.

2016-01-01

Full Text Available Introduction. Tuberculosis patients are rarely asymptomatic. Acute virus myocarditis presents with a wide range of symptoms, from mild dyspnea or chest pain to cardiogenic shock and death. Case Outline. A 26-year-old Caucasian man non-smoker presented with one-week history of lower extremities’ swelling. The patient’s medical history also revealed a two-day episode of subfebrile temperature with scanty hemoptysis three weeks prior to admission. The episode had not provoked him to seek medical care. Physical examination revealed generalized oedema, and laboratory analysis showed signs of acute renal insufficiency. Enlarged heart and hilar shadows, bilateral massive cavitary pulmonary opacities and pleural effusion were found at chest radiography. Sputum smears were Mycobacteria negative on direct microscopy. Electrocardiogram changes and echocardiography were suggestive of acute myocarditis with dilated cardiomyopathy. IgM titer to adenovirus was positive. Under diuretics, angiotensin-converting-enzyme inhibitor, beta-blocker, antibiotics and bed rest, fast heart compensation and renal function repair were achieved. Radiographic pulmonary changes promptly regressed except for a cavity in the right upper lobe. Bronchial aspirate from the affected lobe was Mycobacteria positive on direct microscopy and culture positive for Mycobacterium tuberculosis. Standard anti-tuberculosis drug regimen led to recovery. Conclusion. In the unusual common existence of two diseases whose presentation initially mimicked Wegener’s granulomatosis, acute dilated cardiomyopathy contributed to pulmonary tuberculosis detection. To prevent diagnostic delay in tuberculosis, further efforts in population education are necessary together with continual medical education. [Projekat Ministarstva nauke Republike Srbije, br. 175095

A Rare Presentation of a Rare Disease: Pulmonary Lymphomatoid Granulomatosis

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Ghulam Rehman Mohyuddin

2012-01-01

Full Text Available A 70-year-old female presented with a 4-week history of dry cough and wheezing. Chest radiograph showed a 10.5 cm mass-like density in the anterior mediastinum which had not been previously visualized. Computed tomography scan (CT of the chest showed a right hilar mass encasing and narrowing right upper lobe bronchus and right mainstem bronchus and secondary atelectatic changes. Biopsy was consistent with a diagnosis of lymphomatoid granulomatosis Grade 3. She responded well clinically and radiologically to therapy. Lymphomatoid granulomatosis is a rare EBV-associated disorder which is considered a lymphoproliferative disease. The most common radiographic feature is multiple lung nodules. An isolated hilar mass is an exceptionally rare presentation of this rare disease.

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

NARCIS (Netherlands)

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

2016-01-01

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

Patch testing for food-associated allergies in orofacial granulomatosis.

LENUS (Irish Health Repository)

Fitzpatrick, Laura

2011-01-01

Food-associated allergies, especially to benzoates and cinnamon-related compounds, have been associated with orofacial granulomatosis and both standard and urticarial patch testing have been used to detect such allergies. Elimination diets have also been shown to be effective in some patients.

Review article: cinnamon- and benzoate-free diet as a primary treatment for orofacial granulomatosis.

Science.gov (United States)

Campbell, H E; Escudier, M P; Patel, P; Challacombe, S J; Sanderson, J D; Lomer, M C E

2011-10-01

Orofacial granulomatosis is a rare chronic granulomatous inflammatory disease of the lips, face and mouth. The aetiology remains unclear but may involve an allergic component. Improvements have been reported with cinnamon- and benzoate-free diets. To explore the prevalence of compound and food sensitivity and examine the dietary treatments used in orofacial granulomatosis. A comprehensive literature search was carried out and relevant studies from January 1933 to January 2010 were identified using the electronic database search engines; AGRIS 1991-2008, AMED 1985-2008, British Nursing and Index archive 1985-2008, EMBASE 1980-2008, evidence based medicine review databases (e.g. Cochrane DSR), International Pharmaceutical and Medline 1950-2008. Common sensitivities identified, predominantly through patch testing, were to benzoic acid (36%) food additives (33%), perfumes and flavourings (28%), cinnamaldehyde (27%), cinnamon (17%), benzoates (17%) and chocolate (11%). The cinnamon- and benzoate-free diet has been shown to provide benefit in 54-78% of patients with 23% requiring no adjunctive therapies. A negative or positive patch test result to cinnamaldehyde, and benzoates did not predict dietary outcome. The most concentrated source of benzoate exposure is from food preservatives. Use of liquid enteral formulas can offer a further dietary therapy, particularly in children with orofacial granulomatosis. Management of orofacial granulomatosis is challenging but cinnamon- and benzoate-free diets appear to have a definite role to play. © 2011 Blackwell Publishing Ltd.

Cancer preceding Wegener's granulomatosis: a case-control study

DEFF Research Database (Denmark)

Faurschou, Mikkel; Mellemkjaer, Lene; Sorensen, Inge J

2009-01-01

OBJECTIVE: To investigate whether patients with WG have an increased risk of malignancies prior to and/or around the time of the vasculitis diagnosis, as suggested by previous studies. METHODS: A total of 293 WG patients were included in the study. Ten gender- and age-matched controls were selected.......4; 95% CI 1.1, 38) based on two patients, who developed testis cancer >10 years before WG. The overall prevalence of malignancies diagnosed time...... interval (OR 4.0; 95% CI 1.4, 12). CONCLUSIONS: We did not find clear evidence of an increased prevalence of preceding cancer in our WG cohort, indicating that shared risk factors are of minor importance for the excess of malignancies that occur in WG patients after the vasculitis diagnosis. Furthermore...

Orbital lymphomatoid granulomatosis - a rare cause of proptosis

Energy Technology Data Exchange (ETDEWEB)

Du Toit, Jacqueline; Kilborn, Tracy [Department of Radiology, Red Cross Children' s Hospital, Rondebosch (South Africa); Eyssen, Ann van [Department of Oncology, Red Cross Children' s Hospital, Rondebosch (South Africa); Pillay, Komala [Department of Pathology, Red Cross Children' s Hospital, Rondebosch (South Africa)

2015-07-15

A 1-year-old girl with unilateral proptosis was found to have primary orbital lymphomatoid granulomatosis - a condition rarely occurring in children. This multisystem angiocentric, angiodestructive, lymphoproliferative disease typically involves the lungs, with ocular involvement being extremely uncommon. Our case serves to illustrate the imaging findings of this unusual condition and highlight a rare cause of proptosis. (orig.)

An atypical presentation of cardiac tamponade and periorbital swelling in a patient with eosinophilic granulomatosis with polyangiitis: a case report.

Science.gov (United States)

Keefe, Alexandra C; Hymas, Joseph C; Emerson, Lyska L; Ryan, John J

2017-09-24

Eosinophilic granulomatosis with polyangiitis is a rare, necrotizing systemic vasculitis associated with asthma and hypereosinophilia. Its cause and pathophysiology are still being elucidated. We report a case of eosinophilic granulomatosis with polyangiitis in a 50-year-old Caucasian woman who presented with chest pain, dyspnea at rest, fever, and periorbital swelling. She was found to have significant hypereosinophilia and cardiac tamponade physiology. A biopsy confirmed extensive infiltration of both lungs and pericardium by eosinophils. She did not have any anti-neutrophil cytoplasmic antibodies. Eosinophilic granulomatosis with polyangiitis diagnosis does not require the presence of anti-neutrophil cytoplasmic antibodies. Anti-neutrophil cytoplasmic antibody-positive and anti-neutrophil cytoplasmic antibody-negative eosinophilic granulomatosis with polyangiitis may present with different clinical phenotypes, perhaps suggesting two distinct disease etiologies and distinct pathophysiology.

Serum 25-hydroxyvitamin D levels in patients with Granulomatosis with Polyangiitis: association with respiratory infection

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Mariana O. Perez

Full Text Available OBJECTIVES: To determine the possible association of serum 25-hydroxyvitamin D (25OHD levels with disease activity and respiratory infection in granulomatosis with polyangiitis patients during two different periods: winter/spring and summer/autumn. METHODS: Thirty-two granulomatosis with polyangiitis patients were evaluated in the winter/spring, and the same patients (except 5 were evaluated in summer/autumn (n=27. The 25OHD levels were measured by radioimmunoassay. Disease activity was assessed by the Birmingham Vasculitis Activity Score Modified for Wegener’s Granulomatosis (BVAS/WG and antineutrophil cytoplasmic antibody (ANCA positivity. Respiratory infection was defined according the Centers for Disease Control and Prevention criteria. RESULTS: 25OHD levels were lower among patients in winter/spring than in summer/autumn (32.31±13.10 vs. 38.98±10.97 ng/mL, p=0.04. Seven patients met the criteria for respiratory infection: 5 in winter/spring and 2 in summer/autumn. Patients with respiratory infection presented lower 25OHD levels than those without infection (25.15±11.70 vs. 36.73±12.08 ng/mL, p=0.02. A higher frequency of low vitamin D levels (25OHD<20 ng/mL was observed in patients with respiratory infection (37.5% vs. 7.8, p=0.04. Serum 25OHD levels were comparable between patients with (BVAS/WG≥1 plus positive ANCA and without disease activity (BVAS/WG=0 plus negative ANCA (35.40±11.48 vs. 35.34±13.13 ng/mL, p=0.98. CONCLUSIONS: Lower 25OHD levels were associated with respiratory infection but not disease activity in granulomatosis with polyangiitis patients. Our data suggest that hypovitaminosis D could be an important risk factor for respiratory infection in granulomatosis with polyangiitis patients.

Meningitis granulomatosa, glomerulonefritis rápidamente progresiva y vasculitis

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Ana Ludueña

2011-08-01

Full Text Available El compromiso meníngeo es una manifestación infrecuente de la granulomatosis de Wegener. Puede manifestarse como cefalea con hiperproteinorraquia y engrosamiento de la duramadre con aspecto granulomatoso, que se observa en la resonancia magnética. Presentamos un varón de 57 años con granulomatosis de Wegener que debutó con compromiso de vías aéreas superiores, oídos, órbitas y meningitis granulomatosa asintomática y que posteriormente evolucionó con mononeuritis múltiple y glomerulonefritis crescéntica ANCA positiva. La presencia de ANCA y el compromiso sistémico (vías aéreas superiores, oído, órbitas, nervios periféricos, duramadre y glomerulonefritis rápidamente progresiva permitieron en este caso llegar a un diagnóstico de certeza e iniciar el tratamiento inmunosupresor combinado (corticoides y ciclofosfamida. Evolucionó con remisión clínica y serológica (negativización de ANCA, pero persistiendo leve deterioro secuelar auditivo y de la función renal, sin recidiva de la enfermedad de base.

Archive of Geosample Information from the Alfred-Wegener-Institut (AWI) Core Repository

Data.gov (United States)

National Oceanic and Atmospheric Administration, Department of Commerce — The Alfred-Wegener-Institut (AWI) made a one-time contribution to the Index to Marine and Lacustrine Geological Samples (IMLGS) database of metadata describing 1820...

Lymphomatoid and Mediastinal Granulomatosis in 2-Year-Old Child

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S.V. Vesely

2014-02-01

24.07.13 the operation was performed: right thoracotomy, removal of the tumor of the upper lobe of right lung. Immunohistochemical examination results were received: lymphomatoid granulomatosis, grade II. The postoperative period was uneventful. Sutures are removed, the wound healed by primary intention. 10.08.13 the child was discharged home. Further treatment in Minsk (Belarus is planned.

Anticorpos contra o citoplasma de neutrófilos Antineutrophil cytoplasmic antibodies

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Ari Stiel Radu

2005-07-01

Full Text Available A descoberta do marcador sorológico denominado anticorpo anticitoplasma de neutrófilos revolucionou o diagnóstico e o seguimento das vasculites pulmonares, especialmente da granulomatose de Wegener. Seu padrão pode ser citoplasmático e perinuclear. Sua titulação auxilia no diagnóstico e no seguimento das vasculites pulmonares.The discovery of the serological markers known as antineutrophil cytoplasmic antibodies revolutionized the diagnosis and follow-up treatment of the various forms of pulmonary vasculitis, especially that of Wegener's granulomatosis. The antineutrophil cytoplasmic antibodies pattern can be cytoplasmic or perinuclear. Determination of antineutrophil cytoplasmic antibodies titers aids the diagnosis and follow-up treatment of pulmonary vasculitis.

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

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T. V. Beketova

2016-01-01

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

Pulmonary gallium uptake in rats with granulomatosis induced by complete Freund adjuvant

International Nuclear Information System (INIS)

Stanislas-Leguern, G.; Masse, R.; Jaubert, F.; Chretien, J.; Huchon, G.

1988-01-01

To investigate the mechanism of gallium-67 uptake in lung granulomatosis, we studied 13 rats in which lung granulomatosis was induced by injection of complete Freund adjuvant (CFA) and 14 controls. Gallium uptake was assessed in bronchoalveolar lavage fluid and lavaged lung. The cells responsible for gallium uptake were identified by latent image activation autoradiography. Gallium activity in both lavaged lungs and bronchoalveolar cells (BAC) was higher in CFA-treated animals than in controls [172,205 +/- 134,783 DPM versus 44,456 +/- 14,486 DPM +/- SD (p less than 0.05) and 40,083 +/- 16,350 DPM versus 9100 +/- 4114 DPM (p less than 0.05), respectively]. In control rats, about two-thirds of total lung gallium was located in the interstitium, whereas in CFA-treated rats it was found in the mononuclear cells of lung granulomas. Gallium tracks were more numerous in the alveolar macrophages (AM) of CFA-treated rats than in control AM (28.4 +/- 10.0/field versus 8.4 +/- 3.8/field, p less than 0.001) but the number of tracks was proportional to the number of AM (52.4 +/- 18.7 versus 12.2 +/- 4.3, respectively; p less than 0.001). It is concluded that in rats with CFA-induced lung granulomatosis 1) pulmonary gallium uptake increases, 2) mononuclear cells are responsible for this uptake in both granulomas and AM, and 3) the increased uptake is due to the increased number of mononuclear cells

Treatment of orbital inflammation with rituximab in Wegener's granulomatosis

DEFF Research Database (Denmark)

Baslund, Bo; Wiencke, Anne Katrine; Rasmussen, Niels

2012-01-01

by CT-scan before treatment (3 had bilateral and 7 unilateral orbital involvement). Orbital symptoms at study baseline included pain, pressure sensation behind the eyes, epiphora, diplopia, and affection of the visual acuity. Nine out of ten patients experienced subjective improvement. Four patients...

Orofacial granulomatosis in children can be the initial manifestation of systemic disease: a presentation of two cases

DEFF Research Database (Denmark)

Simonsen, Anne Birgitte; Deleuran, Mette

2014-01-01

Orofacial granulomatosis is a chronic granulomatous condition characterized by relapsing and remitting lip swelling and oral involvement that may include deep ulcers, tags and cobblestone formation. It occurs as an independent entity but also in conjunction with systemic diseases...... such as tuberculosis, sarcoidosis and Crohn's disease. The clinical presentation is not indicative of concomitant systemic disease. To highlight the importance of thorough examination to rule out systemic disease, we present two childhood cases of orofacial granulomatosis, one of which was associated to Crohn......'s disease....

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

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Leonid Aleksandrovich Strizhakov

2012-01-01

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

First results from comparison of rainfall estimations by GPM IMERG with rainfall measurements from the WegenerNet high density network

Science.gov (United States)

Oo, Sungmin; Foelsche, Ulrich; Kirchengast, Gottfried; Fuchsberger, Jürgen

2016-04-01

The research level products of the Integrated Multi-Satellite Retrievals for Global Precipitation Measurement (IMERG "Final" run datasets) were compared with rainfall measurements from the WegenerNet high density network as part of ground validation (GV) projects of GPM missions. The WegenerNet network comprises 151 ground level weather stations in an area of 15 km × 20 km in south-eastern Austria (Feldbach region, ˜46.93° N, ˜15.90° E) designed to serve as a long-term monitoring and validation facility for weather and climate research and applications. While the IMERG provides rainfall estimations every half hour at 0.1° resolution, the WegenerNet network measures rainfall every 5 minutes at around 2 km2 resolution and produces 200 m × 200 m gridded datasets. The study was conducted on the domain of the WegenerNet network; eight IMERG grids are overlapped with the network, two of which are entirely covered by the WegenerNet (40 and 39 stations in each grid). We investigated data from April to September of the years 2014 to 2015; the date of first two years after the launch of the GPM Core Observatory. Since the network has a flexibility to work with various spatial and temporal scales, the comparison could be conducted on average-points to pixel basis at both sub-daily and daily timescales. This presentation will summarize the first results of the comparison and future plans to explore the characteristics of errors in the IMERG datasets.

The Wegener-Bergeron-Findeisen process – Its discovery and vital importance for weather and climate

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Trude Storelvmo

2015-04-01

Full Text Available The Wegener-Bergeron-Findeisen process refers to the rapid growth of ice crystals at the expense of surrounding cloud droplets, which frequently occurs in atmospheric mixed-phase clouds. The process is a result of the difference in saturation vapor pressures with respect to liquid and ice, and may in some circumstances lead to abrupt and complete cloud glaciation at temperatures between −40 °C and 0 °C in the Earth's atmosphere. The process is named after three eminent scientists who were active in the first half of the 20th century, among them being German meteorologist Walter Findeisen (1909–1945. In his classical paper published in 1938, Findeisen described the contemporary understanding of the Wegener-Bergeron-Findeisen process and other key cloud microphysical processes. Here, we compare the understanding of the aforementioned processes at the time with that of the present, and find that they are remarkably similar. We also discuss how the Wegener-Bergeron-Findeisen process is implemented in state-of-the-art numerical models of the atmosphere, and highlight its importance for both weather and climate.

Wegner′s granulomatosis developing for the first time in a patient eight years after starting maintenance hemodialysis

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Ahmad Ramadan Ali

2015-01-01

Full Text Available Wegener′s granulomatosis is a serious autoimmune disorder characterized by necrotizing small-vessel vasculitis. It is a multisystem disease that primarily affects the lung and kidneys. Previous studies indicated few relapses of vasculitis after hemodialysis due to uremic immunosuppression. Our case report describes an end-stage renal failure patient who had developed non-caseating lung granulomata with giant cell formation and fibrinoid necrosis of arterial media that is consistent with Wegner′s granulomatosis for the first time and eight years after initiation of maintenance hemodialysis. We believe that such a phenomenon has rarely been reported.

Angeitis necrotizante sistémica no supurativa

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Gabriel Toro G.

1972-04-01

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

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

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Sujeet Raina

2014-01-01

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

[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.

Comparison of disease activity measures for anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis

Science.gov (United States)

Merkel, PA; Cuthbertson, DD; Hellmich, B; Hoffman, GS; Jayne, DRW; Kallenberg, CGM; Krischer, JP; Luqmani, R; Mahr, AD; Matteson, EL; Specks, U; Stone, JH

2011-01-01

Aim Currently, several different instruments are used to measure disease activity and extent in clinical trials of anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis, leading to division among investigative groups and difficulty comparing study results. An exercise comparing six different vasculitis instruments was performed. Methods A total of 10 experienced vasculitis investigators from 5 countries scored 20 cases in the literature of Wegener granulomatosis or microscopic polyangiitis using 6 disease assessment tools: the Birmingham Vasculitis Activity Score (BVAS), The BVAS for Wegener granulomatosis (BVAS/WG), BVAS 2003, a Physician Global Assessment (PGA), the Disease Extent Index (DEI) and the Five Factor Score (FFS). Five cases were rescored by all raters. Results Reliability of the measures was extremely high (intraclass correlations for the six measures all=0.98). Within each instrument, there were no significant differences or outliers among the scores from the 10 investigators. Test/retest reliability was high for each measure: range=0.77 to 0.95. The scores of the five acute activity measures correlated extremely well with one another. Conclusions Currently available tools for measuring disease extent and activity in ANCA-associated vasculitis are highly correlated and reliable. These results provide investigators with confidence to compare different clinical trial data and helps form common ground as international research groups develop new, improved and universally accepted vasculitis disease assessment instruments. PMID:18664546

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

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Alfredo Nicodemos Cruz Santana

2011-12-01

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

Wegener’s granulomatosis of the breast: A case report

International Nuclear Information System (INIS)

Szabo-Moskal, Jadwiga

2014-01-01

Differential diagnosis and detection of malignant lesions in mammography poses a challenge for many diagnosticians. Although mammography continues to be the best and cost-effective way to detect breast cancer, it has its limitations due in part to the radiological appearance of changes of a very rare condition. We are presented with changes that have met well-known radiologic criteria for the diagnosis of malignancy, yet they turn out to be benign, because they belong to a group of extraordinary lesions. In June 2010, a 56-year-old woman found a lump in her right breast. Mammography performed at another medical center, revealed a change 12 mm in diameter, rated as BI-RADS 4C. Physical examination revealed a palpable lesion, 15 mm in diameter. Ultrasonography revealed hypoechogenic change with ill-defined outlines, size 14×10 mm – BI-RADS 5. Fine needle aspiration biopsy was done and revealed no atypical cells. The patient underwent an open surgical biopsy and a histopathological diagnosis was suggested to be a type of Wegener’s granulomatosis. Exclusion of cancer enabled the continuation of medical treatment of the underlying disease. There were no changes in the breast in follow-up studies. The differential diagnosis of breast tumors should include rare conditions that can sometimes mimic breast cancer. One of them is Wegener’s granulomatosis. Both, the diagnosis and treatment of the disease, require the cooperation of specialists from various fields, including clinicians, pathologists and radiologists

Experiences in therapy for lethal midline granuloma

International Nuclear Information System (INIS)

Tosaka, Kaoru; Ishikawa, Takeru

1982-01-01

Four cases of the lethal midline granuloma or malignant granuloma of the nose were treated by irradiation and chemotherapy, which are generally prescribed for malignant lymphomas. Clinical, histological and laboratory examination indicated that they were the lethal midline granuloma and clearly differentiated from Wegener's granulomatosis or malignant lymphoma. All of the cases exhibited primary remission. The four cases were observed up to 38, 22, 14, and 10 months since the beginning of the therapy, showing no local or general recurrence. (author)

Prospective study of radioimmunoassay for antibodies against neutrophil cytoplasm in diagnosis of systemic vasculitis

International Nuclear Information System (INIS)

Savage, C.O.S.; Winearls, C.G.; Jones, S.; Marshall, P.D.; Lockwood, C.M.

1987-01-01

The diagnosis and management of Wegener's granulomatosis and microscopic polyarteritis are complicated by the lack of specific diagnostic tests. The diagnostic performance of a solid-phase radioimmunoassay, which detects the autoantibodies against neutrophil cytoplasm present in these disorders, was assessed in a prospective study of patients with suspected vasculitis and/or rapidly progressive nephritis. The assay had a sensitivity and specificity of 96% when carried out in combination with a specific inhibition stage and indirect immunofluorescence staining of alcohol-fixed normal neutrophils. (author)

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

Successful endonasal dacryocystorhinostomy in a patient with Wegener’s granulomatosis

Directory of Open Access Journals (Sweden)

P Eloy

2009-11-01

Full Text Available P Eloy, E Leruth, B Bertrand, Ph RombauxENT and HNS department, Cliniques Universitaires de Mont-Godinne, Université Catholique de Louvain, 5530, Yvoir, BelgiumAbstract: Wegener’s granulomatosis (WG is one form of idiopathic autoimmune vasculitis. The disease has a predilection for the upper and lower respiratory tracts (lungs, nose, sinus, and kidneys. WG may be systemic, severe, and potentially lethal, but it may also be limited to the otolaryngological area or to the eyes and the orbits. Obstruction of the lacrimal pathway is a possible complication of the disease that affects approximately 7% of patients with WG. It usually occurs as a direct extension of sinonasal disease and typically is a late manifestation. Management of such a condition is generally viewed as difficult. We report the case of a patient with a quiescent WG limited to the otolaryngological area. This patient presented a bilateral obstruction of the nasolacrimal ducts caused by bilateral extensive adhesions in the nasal cavity. Because she had several episodes of left-side acute dacryocystitis which necessitated several courses of broad-spectrum antibiotics, she successfully underwent an endonasal endoscopic dacryocystorhinostomy using a diode laser and powered instrumentation. The authors describe the clinical case, the surgical technique, and review the literature.Keywords: Wegener’s granulomatosis, recurrent dacryocystitis, endonasal DCR, diode, laser, powered instrumentation

Eosinophilic Granulomatosis with Polyangiitis and Diffuse Gastrointestinal Involvement

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Diana L. Franco

2014-10-01

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

Recurrence of Intravenous Talc Granulomatosis following Single Lung Transplantation

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Richard C Cook

1998-01-01

Full Text Available Advanced pulmonary disease is an unusual consequence of the intravenous injection of oral medications, usually developing over a period of several years. A number of patients with this condition have undergone lung transplantation for respiratory failure. However, a history of drug abuse is often considered to be a contraindication to transplantation in the context of limited donor resources. A patient with pulmonary talc granulomatosis secondary to intravenous methylphenidate injection who underwent successful lung transplantation and subsequently presented with recurrence of the underlying disease in the transplanted lung 18 months after transplantation is reported.

Altered Liver Proteoglycan/Glycosaminoglycan Structure as a Manifestation of Extracellular Matrix Remodeling upon BCG-induced Granulomatosis in Mice.

Science.gov (United States)

Kim, L B; Shkurupy, V A; Putyatina, A N

2017-01-01

Experimental BCG-induced granulomatosis in mice was used to study changes in the dynamics of individual liver proteoglycan components reflecting phasic extracellular matrix remodeling, determined by the host-parasite interaction and associated with granuloma development. In the early BCG-granulomatosis period, the increase in individual proteoglycan components promotes granuloma formation, providing conditions for mycobacteria adhesion to host cells, migration of phagocytic cells from circulation, and cell-cell interaction leading to granuloma development and fibrosis. Later, reduced reserve capacity of the extracellular matrix, development of interstitial fibrosis and granuloma fibrosis can lead to trophic shortage for cells within the granulomas, migration of macrophages out of them, and development of spontaneous necrosis and apoptosis typical of tuberculosis.

Anti-proteinase 3 anti-neutrophil cytoplasm autoantibodies recapitulate systemic vasculitis in mice with a humanized immune system.

LENUS (Irish Health Repository)

Little, Mark A

2012-01-01

Evidence is lacking for direct pathogenicity of human anti-proteinase-3 (PR3) antibodies in development of systemic vasculitis and granulomatosis with polyangiitis (GPA, Wegener\\'s granulomatosis). Progress in study of these antibodies in rodents has been hampered by lack of PR3 expression on murine neutrophils, and by different Fc-receptor affinities for IgG across species. Therefore, we tested whether human anti-PR3 antibodies can induce acute vasculitis in mice with a human immune system. Chimeric mice were generated by injecting human haematopoietic stem cells into irradiated NOD-scid-IL2Rγ⁻\\/⁻ mice. Matched chimera mice were treated with human IgG from patients with: anti-PR3 positive renal and lung vasculitis; patients with non-vasculitic renal disease; or healthy controls. Six-days later, 39% of anti-PR3 treated mice had haematuria, compared with none of controls. There was punctate bleeding on the surface of lungs of anti-PR3 treated animals, with histological evidence of vasculitis and haemorrhage. Anti-PR3 treated mice had mild pauci-immune proliferative glomerulonephritis, with infiltration of human and mouse leukocytes. In 3 mice (17%) more severe glomerular injury was present. There were no glomerular changes in controls. Human IgG from patients with anti-PR3 autoantibodies is therefore pathogenic. This model of anti-PR3 antibody-mediated vasculitis may be useful in dissecting mechanisms of microvascular injury.

Successful application of extracorporeal membrane oxygenation due to pulmonary hemorrhage secondary to granulomatosis with polyangiitis

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Hohenforst-Schmidt W

2013-07-01

Full Text Available Wolfgang Hohenforst-Schmidt,1 Arndt Petermann,2 Aikaterini Visouli,3 Paul Zarogoulidis,4 Kaid Darwiche,5 Ioanna Kougioumtzi,6 Kosmas Tsakiridis,3 Nikolaos Machairiotis,6 Markus Ketteler,2 Konstantinos Zarogoulidis,4 Johannes Brachmann11II Medical Clinic, Coburg Clinic, University of Wuerzburg, Coburg, Germany; 2Division of Nephrology, Coburg Clinic, University of Wuerzburg, Coburg, Germany; 3Cardiothoracic Surgery Department, “Saint Luke” Private Hospital, Thessaloniki, Greece; 4Pulmonary Department, “G Papanikolaou” General Hospital, Aristotle University of Thessaloniki, Thessaloniki, Greece; 5Department of Interventional Pneumology, Ruhrlandklinik, West German Lung Center, University Hospital, University of Duisburg-Essen, Essen, Germany; 6Surgery Department (National Health System, University General Hospital of Alexandroupolis, Alexandroupolis, GreeceAbstract: Extracorporeal membrane oxygenation (ECMO is increasingly applied in adults with acute refractory respiratory failure that is deemed reversible. Bleeding is the most frequent complication during ECMO support. Severe pre-existing bleeding has been considered a contraindication to ECMO application. Nevertheless, there are cases of successful ECMO application in patients with multiple trauma and hemorrhagic shock or head trauma and intracranial hemorrhage. ECMO has proved to be life-saving in several cases of life-threatening respiratory failure associated with pulmonary hemorrhage of various causes, including granulomatosis with polyangiitis (Wegener’s disease. We successfully applied ECMO in a 65-year-old woman with acute life-threatening respiratory failure due to diffuse massive pulmonary hemorrhage secondary to granulomatosis with polyangiitis, manifested as severe pulmonary-renal syndrome. ECMO sustained life and allowed disease control, together with plasmapheresis, cyclophosphamide, corticoids, and renal replacement therapy. The patient was successfully weaned from ECMO

[ANCA-negative subglottic laryngeal stenosis in childhood].

Science.gov (United States)

Wittekindt, C; Lüers, J-C; Drebber, U; Guntinas-Lichius, O; Hüttenbrink, K-B

2007-10-01

A 15-year-old female, having developed recurrent infections of the upper airway, hoarseness, dyspnea, and nasal congestion, was referred to our department. There was no history of trauma or intubation. The subglottic space was circularly narrowed. The test for c-ANCA was negative. Chest X-ray and renal function were normal. A tracheotomy was performed; the histology showed infiltrating plasma cells, but no signs of vasculitis or granulomatous inflammation. One year later the patient developed acute renal failure. Biopsy of the kidney confirmed Wegener's disease. The laryngeal stenosis completely resolved after therapy with cyclophosphamide. Juvenile Wegener's granulomatosis is extremely rare; the larynx and trachea seem to be involved more frequently in children than in adults. The positive testing of c-ANCA can support the diagnosis; however, even when c-ANCA do not test positive, the disease can never be excluded. Surgical interventions within the larynx or trachea might only be considered after ineffective therapy with immunosuppressive drugs.

Low anti-staphylococcal IgG responses in granulomatosis with polyangiitis patients despite long-term Staphylococcus aureus exposure

NARCIS (Netherlands)

Glasner, Corinna; van Timmeren, Mirjan M; Stobernack, Tim; Omansen, Till F; Raangs, Gerwin; Rossen, John W; de Goffau, Marcus C; Arends, Jan P; Kampinga, Greetje A; Koedijk, Danny; Neef, Jolanda; Buist, Girbe; Tavakol, Mehri; van Wamel, Willem J B; Rutgers, Abraham; Stegeman, Coen A; Kallenberg, Cornelis; Heeringa, Peter; van Dijl, Jan Maarten

2015-01-01

Chronic nasal carriage of the bacterium Staphylococcus aureus in patients with the autoimmune disease granulomatosis with polyangiitis (GPA) is a risk factor for disease relapse. To date, it was neither known whether GPA patients show similar humoral immune responses to S. aureus as healthy

Low anti-staphylococcal IgG responses in granulomatosis with polyangiitis patients despite long-term Staphylococcus aureus exposure

NARCIS (Netherlands)

C. Glasner (Corinna); M.M. Van Timmeren (Mirjan M.); T. Stobernack (Tim); T.F. Omansen (Till F.); E.C. Raangs (Erwin C.); J.W. Rossen (John); M.C. De Goffau (Marcus C.); J.P.A. Arends (Jan); G.A. Kampinga (Greetje); D.G.A.M. Koedijk (Denny); J. Neef (Jolanda); G. Buist (Girbe); M. Tavakol (Mehri); W.J.B. van Wamel (Willem); A. Rutgers (Abraham); C.A. Stegeman; C.G.M. Kallenberg (Cees G. M.); P. Heeringa (Peter); J.M. Dijl (Jan Maarten)

2015-01-01

textabstractChronic nasal carriage of the bacterium Staphylococcus aureus in patients with the autoimmune disease granulomatosis with polyangiitis (GPA) is a risk factor for disease relapse. To date, it was neither known whether GPA patients show similar humoral immune responses to S. aureus as

Granulomatosis with polyangiitis confined to lacrimal gland, a case report.

Science.gov (United States)

Zuazo, F; González, M; Abdala, A; Olvera-Morales, O; Monroy, M H; Rodríguez-Reyes, A; Tovilla-Canales, J L; Nava-Castañeda, Á

2017-11-01

A 43 year-old woman consulted due to 2 months of swelling on the superolateral side of the left orbit, with pain and erythema. An excisional biopsy was performed that revealed vasculitis with polyangiitis of the lacrimal gland. A systemic study showed that no other system was compromised. Orbital involvement occurs in up to 60% of patients with granulomatosis with polyangiitis. The involvement of the lacrimal gland is rare and often unilateral. Serological tests are generally negative, both in initial stages, as in localized forms of the disease. Copyright © 2016 Sociedad Española de Oftalmología. Publicado por Elsevier España, S.L.U. All rights reserved.

Esclerite nodular e poliangiite granulomatosa (Wegener mimetizando tuberculose

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Cybelle Moreno Luize Franco

2015-04-01

Full Text Available Poliangiite granulomatosa é uma doença sistêmica que afeta qualquer órgão, com predileção pelo trato respiratório superior, pulmões e rins. Este artigo tem como objetivo relatar um caso atípico de uma paciente com esclerite nodular como manifestação inicial da poliangiite granulomatosa (Wegener, mimetizando um quadro de tuberculose. A paciente apresentou hiperemia ocular e baixa acuidade visual progressiva por 1,5 anos, seguido por dor ocular por dois meses. A paciente possuía nódulos subpleurais com densidade de partes moles, linfonodomegalia em janela aorto-pulmonar e espessamento pleural bilateral discreto, negativo para bacilos álcool-ácido resistentes (BAAR. O diagnóstico histológico revelou uma vasculite granulomatosa sugestiva de vasculite não infecciosa (poliangiite granulomatosa. Foi iniciada pulsoterapia com ciclofosfamida.

Necrobiotic granuloma: An update

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Achyut Pokharel

2018-01-01

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

Role of radiotherapy in the treatment of granuloma gangraenescens (lethal midline granuloma)

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Sack, H; Horch, H H; Schaefer, H E; Wustrow, F

1984-02-01

The granuloma gangraenescens in the oral, maxillary and facial region is a rare disease. It is a destroying process in the region of palate, nose, paranasal sinuses, cheeks and orbit with characteristic signs of granulomatosis, infection and malignancy. The disease shows often a lethal development with cachexia or sepsis. Besides local inflammations and tumors, the granulomatosis of Wegener, and the so-called necrotizing sialometaplasia are above all to be excluded by differential diagnosis. Five cases are presented in order to describe the diagnostic and therapeutic problems. In literature, the greatest efficacy is attributed to radiotherapy, however, a detailed definition of the most efficient irradiation conditions cannot be given yet because of the small number of cases. Good long-term results or recoveries can be achieved in 75 to 80% of cases by a relatively high radiation dose of 40 to 50 Gy administered within four to five weeks. During the observation time of two to six years, no one of the five patients treated here only by megavoltage therapy showed a recurrence.

The role of radiotherapy in the treatment of granuloma gangraenescens (lethal midline granuloma)

International Nuclear Information System (INIS)

Sack, H.; Horch, H.H.; Schaefer, H.E.; Wustrow, F.

1984-01-01

The granuloma gangraenescens in the oral, maxillary and facial region is a rare disease. It is a destroying process in the region of palate, nose, paranasal sinuses, cheeks and orbit with characteristic signs of granulomatosis, infection and malignancy. The disease shows often a lethal development with cachexia or sepsis. Besides local inflammations and tumors, the granulomatosis of Wegener, and the socalled necrotizing sialometaplasia are above all to be excluded by differential diagnosis. Five cases are presented in order to describe the diagnostic and therapeutic problems. In literature, the greatest efficacy is attributed to radiotherapy, however, a detailed definition of the most efficient irradiation conditions cannot be given yet because of the small number of cases. Good long-term results or recoveries can be achieved in 75 to 80% of cases by a relatively high radiation dose of 40 to 50 Gy administered within four to five weeks. During the observation time of two to six years, no one of the five patients treated here only by megavoltage therapy showed a recurrence. (orig.) [de

Diffuse abnormalities of the trachea: computed tomography findings

International Nuclear Information System (INIS)

Marchiori, Edson; Araujo Neto, Cesar de

2008-01-01

The aim of this pictorial essay was to present the main computed tomography findings seen in diffuse diseases of the trachea. The diseases studied included amyloidosis, tracheobronchopathia osteochondroplastica, tracheobronchomegaly, laryngotracheobronchial papillomatosis, lymphoma, neurofibromatosis, relapsing polychondritis, Wegener's granulomatosis, tuberculosis, paracoccidioidomycosis, and tracheobronchomalacia. The most common computed tomography finding was thickening of the walls of the trachea, with or without nodules, parietal calcifications, or involvement of the posterior wall. Although computed tomography allows the detection and characterization of diseases of the central airways, and the correlation with clinical data reduces the diagnostic possibilities, bronchoscopy with biopsy remains the most useful procedure for the diagnosis of diffuse lesions of the trachea. (author)

Presenting A Case with Tubulointerstitial Nephritis and Uveitis (TINU- Syndrome

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E Fotouhi Ardakani

2008-10-01

Full Text Available Concurrence of interstitial nephritis and uveitis named tubulointestitioal nephritis and uveitis syndrome (TINU are unusual and uncommon presentations of interstitial nephritis. This syndrome is considered after ruling out other differential diagnoses. A-38-year old man presented with acute renal failure and uveitis. The histologic findings of renal biopsy showed acute tubulointestitioal nephritis. The patient had no clinical and paraclinical manifestations of other etiologies of interstitial nephritis and uveitis such as Wegener's granulomatosis , Sjogren's syndrome or sarcoidosis. The diagnosis of TINU-Syndrome was therefore considered. The patient was treated by oral and ophthalmic prednisolone and had a good response to treatment.

Pulmonary talc granulomatosis mimicking malignant disease 30 years after last exposure: a case report

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Krimsky William S

2008-07-01

Full Text Available Abstract Introduction Pulmonary talc granulomatosis is a rare disorder characterized by the development of foreign body granuloma secondary to talc exposure. Previous case reports have documented the illness in current intravenous drug users who inject medications intended for oral use. We present a rare case of the disease in a patient with a distant history of heroin abuse who presented initially with history and imaging findings highly suggestive of malignancy. Case presentation A 53-year-old man reported a 4-month history of increasing dyspnea and weight loss. He had a long history of smoking and admission chest X-ray revealed a density in the right hemithorax. Computed tomography confirmed a probable mass with further speculated opacities in both lung fields suspicious for malignant spread. Biopsies obtained using endobronchial ultrasound-guided aspiration returned negative for malignancy and showed bronchial epithelial cells with foreign body giant cell reaction and polarizable birefringent talc crystals. Conclusion This case demonstrates a rare presentation of talc granulomatosis three decades after the last likely exposure. The history and imaging findings in a chronic smoker were initially strongly suggestive of malignant disease, and we recommend that talc-induced lung disease is considered in any patient with multiple scattered pulmonary lesions and a history of intravenous drug use. Confirmation of the disease by biopsy is essential, but unfortunately there are few successful proven management options for patients with worsening disease.

Lymphomatoid Granulomatosis: CT and FDG-PET Findings

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Chung, Jonathan H. [National Jewish Health, Denver (United States); Wu, Carol C.; Gilman, Matthew D.; Palmer, Edwin L.; Hasserjian, Robert P.; Sphepar, Jo-Anne O. [Massachusetts General Hospital, Boston (United States)

2011-11-15

Lymphomatoid granulomatosis (LG) is a rare, aggressive extranodal Epstein-Barr virus (EBV)-positive B-cell lymphoproliferative disease. The purpose of our study was to analyze the CT and fluorodeoxyglucose positron emission tomography (FDG-PET) findings of pulmonary LG. Between 2000 and 2009, four patients with pathologically proven pulmonary LG and chest CT were identified. Two of these patients also had FDG-PET. Imaging features of LG on CT and PET were reviewed. Pulmonary nodules or masses with peribronchovascular, subpleural, and lower lung zonal preponderance were present in all patients. Central low attenuation (4 of 4 patients), ground-glass halo (3 of 4 patients), and peripheral enhancement (4 of 4 patients) were observed in these nodules and masses. An air-bronchogram and cavitation were seen in three of four patients. FDG-PET scans demonstrated avid FDG uptake in the pulmonary nodules and masses. Pulmonary LG presents with nodules and masses with a lymphatic distribution, as would be expected for a lymphoproliferative disease. However, central low attenuation, ground-glass halo and peripheral enhancement of the nodules/masses are likely related to the angioinvasive nature of this disease. Peripheral enhancement and ground-glass halo, in particular, are valuable characteristic not previously reported that can help radiologists suggest the diagnosis of pulmonary LG.

Lymphomatoid Granulomatosis: CT and FDG-PET Findings

International Nuclear Information System (INIS)

Chung, Jonathan H.; Wu, Carol C.; Gilman, Matthew D.; Palmer, Edwin L.; Hasserjian, Robert P.; Sphepar, Jo-Anne O.

2011-01-01

Lymphomatoid granulomatosis (LG) is a rare, aggressive extranodal Epstein-Barr virus (EBV)-positive B-cell lymphoproliferative disease. The purpose of our study was to analyze the CT and fluorodeoxyglucose positron emission tomography (FDG-PET) findings of pulmonary LG. Between 2000 and 2009, four patients with pathologically proven pulmonary LG and chest CT were identified. Two of these patients also had FDG-PET. Imaging features of LG on CT and PET were reviewed. Pulmonary nodules or masses with peribronchovascular, subpleural, and lower lung zonal preponderance were present in all patients. Central low attenuation (4 of 4 patients), ground-glass halo (3 of 4 patients), and peripheral enhancement (4 of 4 patients) were observed in these nodules and masses. An air-bronchogram and cavitation were seen in three of four patients. FDG-PET scans demonstrated avid FDG uptake in the pulmonary nodules and masses. Pulmonary LG presents with nodules and masses with a lymphatic distribution, as would be expected for a lymphoproliferative disease. However, central low attenuation, ground-glass halo and peripheral enhancement of the nodules/masses are likely related to the angioinvasive nature of this disease. Peripheral enhancement and ground-glass halo, in particular, are valuable characteristic not previously reported that can help radiologists suggest the diagnosis of pulmonary LG.

MR imaging of central diabetes insipidus: a pictorial essay

International Nuclear Information System (INIS)

Shin, Ji Hoon; Lee, Ho Kyu; Choi, Choong Gon; Suh, Dae Chul; Kim, Chang Jin; Hong, Sung Kwan; Na, Dong Gyu

2001-01-01

Central diabetes insipidus (DI) can be the outcome of a number of diseases that affect the hypothalamic-neurohypophyseal axis. The causes of the condition can be classified as traumatic, inflammatory, or neoplastic. Traumatic causes include postoperative sella or transection of the pituitary stalk, while infectious or inflammatory causes include meningitis, lymphocytic hypophysitis, and granulomatous inflammations such as sarcoidosis and Wegener's granulomatosis. Various neoplastic conditions such as germinoma, Langerhans cell histiocytosis, metastasis, leukemic infiltration, lymphoma, teratoma, pituitary adenoma, craniopharyngioma, Rathke cleft cyst, hypothalamic glioma, and meningioma are also causes of central DI. In affected patients, careful analysis of these MR imaging features and correlation with the clinical manifestations can allow a more specific diagnosis, which is essential for treatment

The reversed halo sign: update and differential diagnosis

Science.gov (United States)

Godoy, M C B; Viswanathan, C; Marchiori, E; Truong, M T; Benveniste, M F; Rossi, S; Marom, E M

2012-01-01

The reversed halo sign is characterised by a central ground-glass opacity surrounded by denser air–space consolidation in the shape of a crescent or a ring. It was first described on high-resolution CT as being specific for cryptogenic organising pneumonia. Since then, the reversed halo sign has been reported in association with a wide range of pulmonary diseases, including invasive pulmonary fungal infections, paracoccidioidomycosis, pneumocystis pneumonia, tuberculosis, community-acquired pneumonia, lymphomatoid granulomatosis, Wegener granulomatosis, lipoid pneumonia and sarcoidosis. It is also seen in pulmonary neoplasms and infarction, and following radiation therapy and radiofrequency ablation of pulmonary malignancies. In this article, we present the spectrum of neoplastic and non-neoplastic diseases that may show the reversed halo sign and offer helpful clues for assisting in the differential diagnosis. By integrating the patient's clinical history with the presence of the reversed halo sign and other accompanying radiological findings, the radiologist should be able to narrow the differential diagnosis substantially, and may be able to provide a presumptive final diagnosis, which may obviate the need for biopsy in selected cases, especially in the immunosuppressed population. PMID:22553298

Granulomatosis with polyangiitis mimicking infective endocarditis in an adolescent male.

Science.gov (United States)

Varnier, Giulia Camilla; Sebire, Neil; Christov, Georgi; Eleftheriou, Despina; Brogan, Paul A

2016-09-01

Granulomatosis with polyangiitis (GPA) is a rare but serious small vessel vasculitis with heterogeneous clinical presentation ranging from mainly localised disease with a chronic course, to a florid, acute small vessel vasculitic form characterised by severe pulmonary haemorrhage and/or rapidly progressive vasculitis or other severe systemic vasculitic manifestations. Cardiac involvement is, however, uncommon in the paediatric population. We report a case of a 16-year-old male who presented with peripheral gangrene and vegetation with unusual location on the supporting apparatus of the tricuspid valve, initially considered to have infective endocarditis but ultimately diagnosed with GPA. We provide an overview of the limited literature relating to cardiac involvement in GPA, and the diagnostic challenge relating to infective endocarditis in this context, especially focusing on the interpretation of the antineutrophil cytoplasmic antibody (ANCA) and the characteristic clinical features to identify in order to promptly recognise GPA, since timely diagnosis and treatment are essential for this potentially life-threatening condition.

Contrast-enhanced ultrasound and computed tomography findings of granulomatosis with polyangiitis presenting with multiple intrarenal microaneurysms: A case report.

Science.gov (United States)

Kim, Youe Ree; Lee, Young Hwan; Lee, Jong-Ho; Yoon, Kwon-Ha

Granulomatosis with polyangiitis (GPA) is a systemic disorder that affects small- and medium- sized vessels in many organs. Although the kidneys are the second most commonly involved organ in patients with GPA, its manifestation as multiple intrarenal aneurysms is rare. We report an unusual manifestation of GPA with multiple intrarenal microaneurysms, as demonstrated by contrast-enhanced ultrasound and computed tomography. Copyright © 2017 Elsevier Inc. All rights reserved.

Cutaneous granulomatosis and combined immunodeficiency revealing Ataxia-Telangiectasia: a case report

Directory of Open Access Journals (Sweden)

Antoccia Antonio

2010-04-01

Full Text Available Abstract Ataxia-telangiectasia (A-T is a complex multisystem disorder characterized by progressive neurological impairment, variable immunodeficiency and oculo-cutaneous telangiectasia. A-T is a member of chromosomal breakage syndromes and it is caused by a mutation in the ataxia-telangiectasia mutated (ATM gene. Because of a wide clinical heterogeneity, A-T is often difficult to diagnose in children. We report an unusual case of a 3-year-old boy affected by A-T who presented exclusively with extensive cutaneous granulomatosis and severe combined immunodeficiency, without neurological abnormalities, at the time of diagnosis. This case clearly emphasizes the variable presentation of A-T syndrome and highlights the difficulties in the early diagnosis of A-T. A-T should be considered in children with evidence of combined humoral and cellular immunodeficiency associated with unexplained skin granulomatous lesions, even in the absence of the classic features of this syndrome.

A pragmatic approach to vasculitis in the gastrointestinal tract.

Science.gov (United States)

Chetty, Runjan; Serra, Stefano

2017-06-01

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

ANCA-associated vasculitis and malignancy

DEFF Research Database (Denmark)

Mahr, Alfred; Heijl, Caroline; Le Guenno, Guillaume

2013-01-01

of individual therapeutic agents is difficult to dissect, but cyclophosphamide has emerged as a major contributor to cancer development because of its direct carcinogenic properties. Awareness of cancer risk in AAV calls for increased implementation of measures to prevent or screen for cancer and development......In this review, we summarise the current understanding of the potential link between cancer and anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV), including granulomatosis with polyangiitis (Wegener's; GPA) and microscopic polyangiitis (MPA). As is true for many autoimmune...... or inflammatory rheumatic diseases, AAV diagnosis and therapy are associated with an increased risk of de novo cancer development, likely as a result of impaired immunosurveillance, direct oncogenicity of immunosuppressive agents and perhaps malignant degeneration of tissues undergoing chronic immune stimulation...

MR imaging of central diabetes insipidus: a pictorial essay

Energy Technology Data Exchange (ETDEWEB)

Shin, Ji Hoon; Lee, Ho Kyu; Choi, Choong Gon; Suh, Dae Chul; Kim, Chang Jin; Hong, Sung Kwan [Ulsan University College of Medicine, Seoul (Korea, Republic of); Na, Dong Gyu [Sungkyunkwan University College of Medicine, Seoul (Korea, Republic of)

2001-12-01

Central diabetes insipidus (DI) can be the outcome of a number of diseases that affect the hypothalamic-neurohypophyseal axis. The causes of the condition can be classified as traumatic, inflammatory, or neoplastic. Traumatic causes include postoperative sella or transection of the pituitary stalk, while infectious or inflammatory causes include meningitis, lymphocytic hypophysitis, and granulomatous inflammations such as sarcoidosis and Wegener's granulomatosis. Various neoplastic conditions such as germinoma, Langerhans cell histiocytosis, metastasis, leukemic infiltration, lymphoma, teratoma, pituitary adenoma, craniopharyngioma, Rathke cleft cyst, hypothalamic glioma, and meningioma are also causes of central DI. In affected patients, careful analysis of these MR imaging features and correlation with the clinical manifestations can allow a more specific diagnosis, which is essential for treatment.

Pulmonary manifestations from systemic vasculitides

International Nuclear Information System (INIS)

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

2007-01-01

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

Pulmonary manifestations from systemic vasculitides; Pulmonale Manifestationen bei systemischen Vaskulitiden

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

2007-06-15

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

Peripheral Ulcerative Keratitis Associated with Autoimmune Disease: Pathogenesis and Treatment

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Yan Cao

2017-01-01

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

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

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Yuri Albuquerque Pessoa Santos

2017-01-01

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

Eosinophilic granulomatosis with polyangiitis: an overview

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Andrea eGioffredi

2014-11-01

Full Text Available Eosinophilic granulomatosis with polyangiitis (EGPA is a multisystemic disorder, belonging to the small vessel ANCA-associated vasculitis, defined as a eosinophil-rich and necrotizing granulomatous inflammation often involving the respiratory tract, and necrotizing vasculitis predominantly affecting small to medium-sized vessels, associated with asthma and eosinophilia. EGPA pathogenesis is not well known: HLA-DRB1*04 and *07, HLA-DRB4 and IL10.2 haplotype of the IL-10 promoter gene are the most studied genetic determinants. Among the acquired pathogenetic factors, the exposure to different allergens, infections, vaccinations, drugs and silica exposure have been involved.Eosinophils are the most characteristic cells in EGPA and different studies have demonstrated their role as effector and immunoregulatory cells.EGPA is considered a disease with a prevalent activation of the Th2 cellular-mediated inflammatory response but also humoral immunity plays an important role. A link between B and T inflammatory responses may explain different disease features. EGPA typically develops into three sequential phases: the allergic phase, distinguished by the occurrence of asthma, allergic rhinitis and sinusitis, the eosinophilic phase, in which the main pathological finding is the eosinophilic organ infiltrations (e.g. lungs, heart and gastrointestinal system and the vasculitic phase, characterized by purpura, peripheral neuropathy and constitutional symptoms.ANCA (especially pANCA anti-MPO are present in 40-60% of the patients. An elevation of IgG4 is frequently found. Corticosteroids and cyclophosphamide are classically used for remission induction, while azathioprine and methotrexate are the therapeutic options for remission maintenance. B-cell depletion with rituximab has shown promising results for remission induction.

Suppurative dacroadenitis causing ocular sicca syndrome in classic Wegener′s granulomatosis

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Khanna Dhanita

2011-01-01

Full Text Available Wegener′s granulomatosis (WG is a multisystem vasculitic disorder which can commonly afflict various components of the eye. Here we describe some unusual ocular manifestations of the disease in one patient. A young male with history of upper respiratory tract symptoms including epistaxis, nasal stuffiness and maxillary sinus pain presented with bilateral lacrimal gland abscess and ptosis. Lacrimal gland biopsy revealed granulomatous vasculitis. Lung cavities, positive cytoplasmic-antineutrophil cytoplasmic antibodies and high titers of serine proteinase-3 antibodies confirmed the diagnosis of WG. The patient developed dry eyes after a month of first presentation. There was no dryness of mouth, suggesting the absence of salivary gland involvement, and antinuclear antibodies as well as antibodies against Ro and La antigens classical of primary Sjogren′s syndrome were absent. Granulomatous vasculitis of lacrimal gland leading to abscess formation and dryness of eyes has not been described in WG and reflects the aggressive nature of inflammatory process in this disease.

Acute laryngeal dyspnea as first presentation of granulomatosis with polyangiitis.

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Pajor, Anna Maria; Kwiatkowska, Sylwia; Kroczyńska-Bednarek, Jadwiga; Piotrowski, Wojciech J

2015-01-01

Granulomatosis with polyangiitis (GPA) is a multi-organ disease which mostly affects lungs, kidney, and head and neck region. We report a rare case of acute laryngeal dyspnea and rapidly progressive pulmonary changes as first manifestations of disease. A 53 year-old woman presented with symptoms of two-week dyspnea, which aggravated rapidly in the preceding hours. Laryngological examination revealed subglottic infiltrations and vocal fold oedema which required urgent tracheotomy. During few days she developed gingival ulcerations and pulmonary infiltration with negative serum c-ANCA titers. The histopathological examination of subglottic and gingival biopsies and the clinical picture established the diagnosis of GPA. She was treated with prednisone and cyclophosphamide with recovery; however, during over 3 years of follow-up, pulmonary symptoms relapsed and subglottic stenosis persisted. The difficulties in diagnosis and treatment in this unusual presentation of GPA are outlined with conclusion that in patients with subglottic infiltration, which develops rapidly, even when this is a sole presentation of the disease, and when c-ANCA are negative, GPA should always be considered.

Granulomatosis with Polyangiitis Presenting as Pauci-Immune Crescentic Glomerulonephritis in Pregnancy

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Ryan Kunjal

2016-01-01

Full Text Available Antineutrophil cytoplasmic antibody (ANCA associated vasculitis rarely affects females of reproductive age. A 28-year-old African American woman presented at 8 weeks of gestation with intractable vomiting attributed to hyperemesis gravidarum. She was found to have acute kidney injury that was unresponsive to vigorous fluid resuscitation and urine sediment examination was suggestive of an underlying glomerulonephritis. Serum c-ANCA and PR3 were elevated and there was no peripheral eosinophilia. During her course she also developed one episode of small volume hemoptysis with right upper lobe infiltrates on CT Chest. There were no cutaneous manifestations of vasculitis or upper respiratory symptoms. Renal biopsy revealed a pauci-immune crescentic glomerulonephritis (PICGN. The diagnosis was consistent with granulomatosis with polyangiitis (GPA. Management initially comprised teratogen sparing agents; steroids, intravenous immunoglobulin; and plasma exchange. The response was suboptimal and she became dependent on daily renal replacement therapy. Ultimately the pregnancy was terminated allowing for traditional treatment approaches with dramatic effect. This is the first case of GPA presenting as PICGN in pregnancy and highlights the challenges of its management.

Granulomatosis with Polyangiitis Presenting as Pauci-Immune Crescentic Glomerulonephritis in Pregnancy.

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Kunjal, Ryan; Makary, Raafat; Poenariu, Andreea

2016-01-01

Antineutrophil cytoplasmic antibody (ANCA) associated vasculitis rarely affects females of reproductive age. A 28-year-old African American woman presented at 8 weeks of gestation with intractable vomiting attributed to hyperemesis gravidarum. She was found to have acute kidney injury that was unresponsive to vigorous fluid resuscitation and urine sediment examination was suggestive of an underlying glomerulonephritis. Serum c-ANCA and PR3 were elevated and there was no peripheral eosinophilia. During her course she also developed one episode of small volume hemoptysis with right upper lobe infiltrates on CT Chest. There were no cutaneous manifestations of vasculitis or upper respiratory symptoms. Renal biopsy revealed a pauci-immune crescentic glomerulonephritis (PICGN). The diagnosis was consistent with granulomatosis with polyangiitis (GPA). Management initially comprised teratogen sparing agents; steroids, intravenous immunoglobulin; and plasma exchange. The response was suboptimal and she became dependent on daily renal replacement therapy. Ultimately the pregnancy was terminated allowing for traditional treatment approaches with dramatic effect. This is the first case of GPA presenting as PICGN in pregnancy and highlights the challenges of its management.

Computed tomography in the evaluation of the sphenoidal sinus lesions

International Nuclear Information System (INIS)

Shirahata, Yuichi; Yamazaki, Yoshio; Tachinbana, Toshiro; Onishi, Tosio.

1988-01-01

The sphenoidal sinus lesions, which were documented by sinus CT, were found in 32 sides of sphenoidal sinus of 19 patients seen in 14 months from January 1984 to February 1986. Sphenoiditis was frequently seen as in 17 % in the all poly-sinusitis, and sphenoidals cysts were seen in 4 side in 3 patients. The indications of the sphenoiditis by CT were thickening of the mucosa and opacificasion with soft tissue density. By sphenoid cysts, no contrast enhancement was seen and margins of the cyst wall were smooth and regular with bone erosion. Since erosion of the bone is also seen in Wegener's granulomatosis and carcinoma, the CT image of the bone erosion is not specific to either of them and does not contribute to the definitive diagnosis of malignancy. (author)

Modeling of the Wegener-Bergeron-Findeisen process-implications for aerosol indirect effects

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Storelvmo, T; Lohmann, U; Kristjansson, J E; Iversen, T; Kirkevaag, A; Seland, O

2008-01-01

A new parameterization of the Wegener-Bergeron-Findeisen (WBF) process has been developed, and implemented in the general circulation model CAM-Oslo. The new parameterization scheme has important implications for the process of phase transition in mixed-phase clouds. The new treatment of the WBF process replaces a previous formulation, in which the onset of the WBF effect depended on a threshold value of the mixing ratio of cloud ice. As no observational guidance for such a threshold value exists, the previous treatment added uncertainty to estimates of aerosol effects on mixed-phase clouds. The new scheme takes subgrid variability into account when simulating the WBF process, allowing for smoother phase transitions in mixed-phase clouds compared to the previous approach. The new parameterization yields a model state which gives reasonable agreement with observed quantities, allowing for calculations of aerosol effects on mixed-phase clouds involving a reduced number of tunable parameters. Furthermore, we find a significant sensitivity to perturbations in ice nuclei concentrations with the new parameterization, which leads to a reversal of the traditional cloud lifetime effect.

Modeling of the Wegener Bergeron Findeisen process—implications for aerosol indirect effects

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Storelvmo, T.; Kristjánsson, J. E.; Lohmann, U.; Iversen, T.; Kirkevåg, A.; Seland, Ø.

2008-10-01

A new parameterization of the Wegener-Bergeron-Findeisen (WBF) process has been developed, and implemented in the general circulation model CAM-Oslo. The new parameterization scheme has important implications for the process of phase transition in mixed-phase clouds. The new treatment of the WBF process replaces a previous formulation, in which the onset of the WBF effect depended on a threshold value of the mixing ratio of cloud ice. As no observational guidance for such a threshold value exists, the previous treatment added uncertainty to estimates of aerosol effects on mixed-phase clouds. The new scheme takes subgrid variability into account when simulating the WBF process, allowing for smoother phase transitions in mixed-phase clouds compared to the previous approach. The new parameterization yields a model state which gives reasonable agreement with observed quantities, allowing for calculations of aerosol effects on mixed-phase clouds involving a reduced number of tunable parameters. Furthermore, we find a significant sensitivity to perturbations in ice nuclei concentrations with the new parameterization, which leads to a reversal of the traditional cloud lifetime effect.

Granulomatosis de células de Langerhans: Presentación de un caso

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. Caridad Nazco Ríos

1998-12-01

Full Text Available Se reporta un inusual caso de granulomatosis de células de Langerhans localizado en maxilares en un paciente de 30 meses de edad, quien presentaba además de las lesiones líticas óseas, lesiones de aspecto tumoral en encía palatina y lingual de ambas arcadas dentarias. El examen óseo no arrojaba compromiso de otros huesos. El diagnóstico histológico fue corroborado por el Centro Nacional de Referencia en Anatomía Patológica.A unusual case of Langerhans´ cells is reported, located in maxillae of a 30 months old patients, who, apart from bone lithic injuries, showed injuries of tumoral type in lingual and palatine gingiva of both dental archs. According to bone examination, there weren´t involvement of other bones. Histologic diagnosis was corroborated by National Reference Center in Pathologic Anatomy.

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

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

2013-09-24

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

Mastoiditis and facial paralysis as initial manifestations of temporal bone systemic diseases - the significance of the histopathological examination.

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Maniu, Alma Aurelia; Harabagiu, Oana; Damian, Laura Otilia; Ştefănescu, Eugen HoraŢiu; FănuŢă, Bogdan Marius; Cătană, Andreea; Mogoantă, Carmen Aurelia

2016-01-01

Several systemic diseases, including granulomatous and infectious processes, tumors, bone disorders, collagen-vascular and other autoimmune diseases may involve the middle ear and temporal bone. These diseases are difficult to diagnose when symptoms mimic acute otomastoiditis. The present report describes our experience with three such cases initially misdiagnosed. Their predominating symptoms were otological with mastoiditis, hearing loss, and subsequently facial nerve palsy. The cases were considered an emergency and the patients underwent tympanomastoidectomy, under the suspicion of otitis media with cholesteatoma, in order to remove a possible abscess and to decompress the facial nerve. The common features were the presence of severe granulation tissue filling the mastoid cavity and middle ear during surgery, without cholesteatoma. The definitive diagnoses was made by means of biopsy of the granulation tissue from the middle ear, revealing granulomatosis with polyangiitis (formerly known as Wegener's granulomatosis) in one case, middle ear tuberculosis and diffuse large B-cell lymphoma respectively. After specific associated therapy facial nerve functions improved, and atypical inflammatory states of the ear resolved. As a group, systemic diseases of the middle ear and temporal bone are uncommon, but aggressive lesions. After analyzing these cases and reviewing the literature, we would like to stress upon the importance of microscopic examination of the affected tissue, required for an accurate diagnosis and effective treatment.

Rare occupational cause of nasal septum perforation: Nickel exposure

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Ertugrul Cagri Bolek

2017-10-01

Full Text Available Many etiologies are held accountable for nasal septum perforations. Topical nasal drug usage, previous surgeries, trauma, nose picking, squamous cell carcinoma, some rheumatological disorders such as granulomatosis with polyangiitis (Wegener granulomatosis, some infectious diseases such as syphilis and leprosy are among the causes of the perforations. Occupational heavy metal exposures by inhalation rarely may also cause nasal septum perforation. Here, we present a 29-year-old patient without any known diseases, who is a worker at a metallic coating and nickel-plating factory, referred for investigation of his nasal cartilage septum perforation from an otorhinolaryngology clinic. The patient questioning, physical examination and laboratory assessment about rheumatic and infectious diseases were negative. There was a metallic smell in the breath during the physical examination. The analysis showed serum nickel level at 31 μg/l and urine nickel at 18 μg/l (84.11 μg/g creatinine. Other possible serum and urine heavy metal levels were within normal ranges. Nickel exposure is usually together with other heavy metals (chromium or cadmium, it is rarely alone. Nickel ingested by inhalation usually leads to respiratory problems such as reduced olfactory acuity, ulcers, septum perforation or tumors of the nasal sinuses. This case demonstrates the importance of occupational anamnesis and awareness of diagnosis. Int J Occup Med Environ Health 2017;30(6:963–967

Neumayer III and Kohnen Station in Antarctica operated by the Alfred Wegener Institute

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Christine Wesche

2016-08-01

Full Text Available The Alfred Wegener Institute operates two stations in Dronning Maud Land, Antarctica. The German overwintering station Neumayer III is located on the Ekström Ice Shelf at 70°40’S and 08°16’W and is the logistics base for three long-term observatories (meteorology, air chemistry and geophysics and nearby research activities. Due to the vicinity to the coast (ca. 20 km from the ice shelf edge, the Neumayer III Station is the junction for many German Antarctic expeditions, especially as the starting point for the supply traverse for the second German station Kohnen. The summer station Kohnen is located about 600 km from the coast and 750 km from Neumayer III Station on the Antarctic plateau at 75°S and 00°04’E. It was erected as the base for the deep-drilling ice core project, which took place between 2001 and 2006. Since then Kohnen Station is used as a logistics base for different research projects.

Cogan's syndrome is a new nosological entity in the current classification of systemic vasculitides: A clinical case and a review of literature

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T. V. Beketova

2016-01-01

Full Text Available The paper describes a clinical case of the typical variant of Cogan's syndrome (CS, a new nosological entity in the current classification of systemic vasculitides (SV, which belongs to a group of variable vasculitides. The literature review highlights in detail the problems of the diagnosis and treatment of this rare disease.CS is characterized by inflammatory eye involvement (interstitial keratitis, uveitis, and episcleritis and hearing problems (sensorineural hearing loss, vestibular disorders with the possible development of vasculitis at other sites, aortitis, and aortic or mitral lesions. A systemic lesion involving the ears and eyes necessitates to rule out granulomatosis with polyangiitis (Wegener's and to make a differential diagnosis with a wide range of diseases. The given data underline the need for the interdisciplinary collaboration of rheumatologists, otorhinolaryngologists, audiologists, oculists, and cardiologists in order to improve the diagnosis and treatment of this form of SV. 

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

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

2017-09-01

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

Rare occupational cause of nasal septum perforation: Nickel exposure.

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Bolek, Ertugrul Cagri; Erden, Abdulsamet; Kulekci, Cagri; Kalyoncu, Umut; Karadag, Omer

2017-10-06

Many etiologies are held accountable for nasal septum perforations. Topical nasal drug usage, previous surgeries, trauma, nose picking, squamous cell carcinoma, some rheumatological disorders such as granulomatosis with polyangiitis (Wegener granulomatosis), some infectious diseases such as syphilis and leprosy are among the causes of the perforations. Occupational heavy metal exposures by inhalation rarely may also cause nasal septum perforation. Here, we present a 29-year-old patient without any known diseases, who is a worker at a metallic coating and nickel-plating factory, referred for investigation of his nasal cartilage septum perforation from an otorhinolaryngology clinic. The patient questioning, physical examination and laboratory assessment about rheumatic and infectious diseases were negative. There was a metallic smell in the breath during the physical examination. The analysis showed serum nickel level at 31 μg/l and urine nickel at 18 μg/l (84.11 μg/g creatinine). Other possible serum and urine heavy metal levels were within normal ranges. Nickel exposure is usually together with other heavy metals (chromium or cadmium), it is rarely alone. Nickel ingested by inhalation usually leads to respiratory problems such as reduced olfactory acuity, ulcers, septum perforation or tumors of the nasal sinuses. This case demonstrates the importance of occupational anamnesis and awareness of diagnosis. Int J Occup Med Environ Health 2017;30(6):963-967. This work is available in Open Access model and licensed under a CC BY-NC 3.0 PL license.

Anti-proteinase 3 anti-neutrophil cytoplasm autoantibodies recapitulate systemic vasculitis in mice with a humanized immune system.

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Mark A Little

Full Text Available Evidence is lacking for direct pathogenicity of human anti-proteinase-3 (PR3 antibodies in development of systemic vasculitis and granulomatosis with polyangiitis (GPA, Wegener's granulomatosis. Progress in study of these antibodies in rodents has been hampered by lack of PR3 expression on murine neutrophils, and by different Fc-receptor affinities for IgG across species. Therefore, we tested whether human anti-PR3 antibodies can induce acute vasculitis in mice with a human immune system. Chimeric mice were generated by injecting human haematopoietic stem cells into irradiated NOD-scid-IL2Rγ⁻/⁻ mice. Matched chimera mice were treated with human IgG from patients with: anti-PR3 positive renal and lung vasculitis; patients with non-vasculitic renal disease; or healthy controls. Six-days later, 39% of anti-PR3 treated mice had haematuria, compared with none of controls. There was punctate bleeding on the surface of lungs of anti-PR3 treated animals, with histological evidence of vasculitis and haemorrhage. Anti-PR3 treated mice had mild pauci-immune proliferative glomerulonephritis, with infiltration of human and mouse leukocytes. In 3 mice (17% more severe glomerular injury was present. There were no glomerular changes in controls. Human IgG from patients with anti-PR3 autoantibodies is therefore pathogenic. This model of anti-PR3 antibody-mediated vasculitis may be useful in dissecting mechanisms of microvascular injury.

The Origin of Continents and Oceans : A. Wegener (translated by J. Biram from the 4th revised German edition, 1929) Dover, New York N.Y., 1966, 246 pp., 63 illus., paperbound U.S.$2.00

NARCIS (Netherlands)

Bemmelen, R.W. van

In the old controversy between fixism and mobilism a new scientific tool, palaeomagnetism, has recently brought forward independent diagnostic facts. In the discussions about drift many authors quote Wegener's classic work. It is fortunate that it has now appeared in an excellent English translation

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

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

2018-03-01

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

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

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

2015-11-01

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

Thoracic vasculitis presenting as surgical problems.

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Jansen, Michael

2010-01-01

We present four patients with vasculitis manifesting with unusual clinical or pathological features, generating surgical problems. Two cases presented with pulmonary hypertension, with investigations and radiological evidence prompting clinical suspicion of pulmonary thrombo-embolic disease. First case, with an antecedant history of Wegener\\'s granulomatosis (WG), demonstrated following "embolectomy", WG involving the large pulmonary elastic arteries. The second case of inoperable "pulmonary thrombo-embolic disease" was subsequently found at limited post mortem to have giant cell arteritis, which affected widespread small peripheral pulmonary arterial vessels. The other two cases were of aortitis occurring in the background of immune-mediated disease, which had been treated with aggressive immunosuppression regimens. The first of these was a case of Cogan\\'s syndrome complicated by descending aortitis, a rarely reported phenomenon, with co-existent acute endocarditis of the aortic valve leaflets. Most cases of endocarditis in this context occur secondary to and in continuity with ascending aortitis. That this case, and a case of ascending aortitis occurring in the context of relapsing polychondritis occurred in the face of aggressive immunosuppression with an apparent clinical response, underscores the need to not accept a clinical picture at face value. This has implications for clinical management, particularly in the follow-up of surgical prosthetic devices such as grafts which may be used in these cases. All four cases emphasise the continued importance of histology and the post-mortem examination in elucidating previously undetected or unsuspected disease.

Purpura, petechiae, and bullae as first signs of juvenile granulomatosis with polyangiitis.

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Rawn, Saara; Miettunen, Paivi; Brown, Holly A; Schmeling, Heinrike

2014-12-01

We present a case of a 14-year-old girl who had a severe form of granulomatosis with polyangiitis (GPA) with extensive dermatological involvement, whose initial presentation was nonspecific leading to diagnostic confusion and initial consideration of infectious and other vasculitis causes. The patient presented with fever, congestion, malaise, and sinus pain. She was diagnosed with bacterial sinusitis and treated with antibiotics. Within weeks, she developed abdominal pain, hematuria, migratory arthritis, and palpable purpura and was diagnosed with Henoch-Schonlein purpura. She went on to develop hemoptysis and progression of the rash into erosive bullae. Investigations revealed that she was ANCA positive and had pauci-immune glomerulonephritis. Given her upper airway, pulmonary and renal involvement, and antineutrophil cytoplasmic antibodies positivity, a definitive diagnosis of a severe form of GPA was made. GPA is a chronic relapsing, life threatening vasculitis that predominantly affects small vessels. Our case demonstrates that GPA can present initially with nonspecific symptoms, including extensive dermatological involvement, leading to diagnostic confusion, and delays in treatment. In the case of a severe peripheral rash in the juvenile population and/or resistant upper airway symptoms, it is vital to consider a diagnosis of GPA to avoid serious organ or life threatening consequences.

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

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Christian Dejaco

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

A case of exorbitism in association with Wegener′s granulomatosis with renal involvement

Directory of Open Access Journals (Sweden)

S Beji

2012-01-01

Full Text Available Wegener′s granulomatosis (WG is a necrotizing granulomatous vasculitis invol-ving the nose, paranasal sinuses, lungs, and kidneys. Ocular involvement can occur in about 50% of cases. There are very few reports of WG with orbital inflammation and exorbitism. We report a case of a female patient who presented with exorbitism related to orbital inflammation secondary to WG, with renal involvement. A 29-year-old woman with a previous history of recurrent pan-sinusitis presented with bilateral exophthalmos and renal failure with rapidly progressive glo-merulonephritis. Computed tomography showed extensive bilateral soft tissue in the retro-orbital area. Immunologic tests showed the presence of type-C anti-neutrophil cytoplasmic antibodies and renal biopsy revealed pauci immune crescentic glomerulonephritis. The patient was treated with corticosteroids and pulses of cyclophosphamide followed by azathioprine and trimethoprim-sulfamethoxazole. After a follow-up of 10 months, the renal outcome was favorable with improvement of renal function but there was persistence of exorbitism and loss of visual function. Our case suggests that WG should be considered in the differential diagnosis of persistent bila-teral exophthalmos. Prompt recognition of this early manifestation is important for the institution of early treatment.

The use of abatacept in debilitating cavitating lung disease associated with rheumatoid arthritis, bronchocentric granulomatosis and aspergillosis.

LENUS (Irish Health Repository)

Neff, K

2010-06-01

A case of debilitating cavitating lung disease associated with rheumatoid arthritis and bronchocentric granulomatosis, which failed to respond to conventional medical or surgical treatment, is described. The patient was treated over 10 years with steroids, antimicrobial agents, disease-modifying antirheumatoid drugs and surgery. Lung function continued to decline and the patient presented for admission with recurrent pneumonia. Abatacept was initiated to modify the underlying immunopathology. Following 12 months of treatment with abatacept the patient has demonstrable improvement in lung function and lung anatomy, and has not presented to hospital with pneumonia. She has tolerated the treatment without complication. The use of abatacept has stabilised the lung disease in this case in the medium term and prevented readmission to hospital. These results suggest a larger role for abatacept in those with such disease in the future and may warrant further investigation.

Focus on audiologic impairment in eosinophilic granulomatosis with polyangiitis.

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

2016-12-01

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

Combined Allergic Bronchopulmonary Aspergillosis and Eosinophilic Granulomatosis with Polyangiitis: Three Cases and a Review of the Literature.

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Ishiguro, Takashi; Takayanagi, Noboru; Takaku, Yotaro; Kagiyama, Naho; Kurashima, Kazuyoshi; Sugita, Yutaka

2016-01-01

Previous reports of combined allergic bronchopulmonary mycosis (ABPM) and eosinophilic granulomatosis with polyangiitis (EGPA) are limited; however, among 41 cases of ABPM and 18 cases of EGPA treated at our hospital, we experienced three cases of combined ABPM and EGPA. In two of these cases, the diagnosis of EGPA preceded that of ABPM, however, one of the two cases had already shown findings suggestive of ABPM, such as mucous plugs and central bronchiectasis, at the time of the diagnosis of EGPA. In six previously reported cases of combined ABPM and EGPA, ABPM preceded EGPA in four cases. In the other two cases in which EGPA was diagnosed before or simultaneously with ABPM, findings suggestive of ABPM had been detected when EGPA was diagnosed, which suggests that sensitization to fungi resulting in ABPM may play an important role in the development of EGPA. Careful attention should therefore be paid to the possibility that these diseases may coexist during the course of either disease.

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

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

2010-05-01

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

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

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

2015-01-01

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

Lymphomatoid granulomatosis of central nervous system and lung driven by epstein barr virus proliferation: successful treatment with rituximab-containing chemotherapy.

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Fernandez-Alvarez, Ruben; Gonzalez, Me; Fernandez, Almudena; Gonzalez-Rodriguez, Ap; Sancho, Jm; Dominguez, Francisco; Fernandez, Carmen

2014-01-01

Lymphomatoid granulomatosis (LYG) is a very rare Epstein-Barr virus (EBV) associated B-cell lymphoproliferative disorder. We report the case of a 41-year-old man who presented with fever and respiratory symptoms. Computed tomography showed multiple nodules in both lung fields. Polymerase chain reaction (PCR) analysis for EBV was positive in bronchoalveolar lavage and biopsy of lung node yielded a diagnosis of LYG, grade III. Shortly after initiation of treatment with agressive chemotherapy, neurological deterioration appeared. Neuroimaging findings revealed hydrocephalus and PCR analysis of the cerebrospinal fluid (CSF) was positive for EBV. Treatment with intravenous rituximab led to rapid reduction of EBV load in CSF, along with clinical and radiological improvement. After completion of treatment with immunochemotherapy, an autologous stem cell transplantation was performed. Patient stays in remission 18 months after diagnosis.

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

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

2014-08-01

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

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

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Pagnoux, Christian; Groh, Matthieu

2016-10-01

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

Effect of Wegener-Bergeron-Findeisen Process to Black Carbon Simulation

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Qi, Ling; Li, Qinbin; He, Cenlin; Wang, Xin; Huang, Jianping

2016-04-01

We systematically investigated the effect of Wegener-Bergeron-Findeisen (WBF) process to black carbon (BC) simulation by a global 3D chemical transport model GEOS-Chem constrained by measurements of BC scavenging efficiencies, concentration in air, deposition fluxes, concentration in snow and washout ratios. Including effect of WBF process reduces the annual mean BC scavenging efficiencies (the ratio of BC in cloud droplets to total BC) at all altitudes by 43-76% in the Arctic. For mid latitude BC scavenging efficiencies decrease by 8-22%, 23-39%, and 41-50% in lower (0-2 km), middle (2-5 km) and upper troposphere (5-10 km), respectively. Simulated BC in air in the Arctic and at mid altitude (˜4 km) in mid latitude increases by ˜40%, and the discrepancy reduces from -65% to -30%. Simulated median BC in snow decreases from 25.7 to 22.4 ng g-1, by 15% in mid latitude and increases from 8.7 to 11.0 ng g-1, by 26% in the Arctic and the comparison with observations improves. The model overestimates washout ratios (ratio of BC in fresh snow/rain to BC in surface air) at most of the sites by up to a factor of 165. With effect of WBF process included, the discrepancy decreases to a factor of 72. The simulated BC burden increases from 0.22 to 0.35 mg m-2 yr-1 when effect of WBF process is included, partly explains the scaled up of BC burden in Bond et al., 2013. Moreover, burden above 5 km increases from 22% to 27% when WBF process is included, indicating a higher forcing efficiency. We also found that BC simulation is insensitive to the temperature criteria between mixed phase clouds and ice clouds. The simulated BC burden is the same when the temperature is set as -15° C and -25° C. This study also suggests that more observations are needed to better distinguish riming dominated and WBF dominated conditions and better parameterize BC scavenging efficiency under the two conditions.

Serum, liver, and lung levels of the major extracellular matrix components at the early stage of BCG-induced granulomatosis depending on the infection route.

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Kim, L B; Shkurupy, V A; Putyatina, A N

2015-01-01

Experiments on the model of mouse BCG-induced granulomatous showed that the content of glycosaminoglycans and proteoglycans in the extracellular matrix of the liver and lungs are changed at the early stages of inflammation (days 3 and 30 postinfection) before cell destruction in the organs begins. This is related to degradation of extracellular matrix structures. Their high content in the blood and interstitium probably contributes to the formation of granulomas, fibroblast proliferation and organ fibrosis. These processes depend on the infection route that determines different conditions for generalization of the inflammation process. Intravenous method of vaccine injection is preferable to use when designing the experiments simulating tuberculosis granulomatosis, especially for the analysis of its early stages.

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

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Vilma Takayasu

2013-06-01

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

Omalizumab in patients with eosinophilic granulomatosis with polyangiitis: a 36-month follow-up study.

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Detoraki, Aikaterini; Di Capua, Lorena; Varricchi, Gilda; Genovese, Arturo; Marone, Gianni; Spadaro, Giuseppe

2016-01-01

Eosinophilic granulomatosis with polyangiitis (EGPA) is a systemic vasculitis characterized by asthma and blood eosinophilia, with the lung being the organ most frequently affected. Oral glucocorticoids and/or immunosuppressive drugs are the mainstay therapy of EGPA. Occasional reports suggest that EGPA patients can be treated with omalizumab in addition to conventional therapy to achieve asthma control. To investigate the long-term effects of omalizumab in patients with EGPA and asthma (2 females, 3 males, age 41-64 years), we carried out a 36-month follow-up observational study. At the time of enrollment, the patients were on maintenance therapy and had moderate to severe allergic asthma, eosinophilia and rhinosinusitis. Mononeuropathy/polyneuropathy and/or histological evidence of tissue eosinophilic infiltration were also present. Patients were treated with omalizumab (300-600 mg s.c. every 2-4 weeks) as add-on therapy to prednisone, inhaled steroids and bronchodilators. During omalizumab treatment, spirometry, the asthma control test (ACT) score and eosinophilia were evaluated, and prednisone dosage was recorded. During the 36 months of omalizumab treatment asthma progressively improved as indicated by spirometry and the ACT score. Eosinophilia progressively decreased. The oral prednisone dose was reduced or withdrawn during treatment. No adverse events were recorded. In patients with EGPA and moderate to severe allergic asthma, omalizumab can be beneficial and safe. It enables corticosteroid tapering while decreasing eosinophilia and improving asthma symptoms over 36 months.

Fertility counseling and preservation practices in youth with lupus and vasculitis undergoing gonadotoxic therapy.

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Nahata, Leena; Sivaraman, Vidya; Quinn, Gwendolyn P

2016-11-01

To assess fertility counseling and preservation practices among children, adolescents, and young adults with rheumatic diseases undergoing cyclophosphamide (CTX) treatment. Retrospective chart review (2006-2016). Academic pediatric center. Male and female patients with systemic lupus erythematosus, Wegener's granulomatosis/granulomatosis with polyangiitis, or other vaculitides, receiving CTX treatment. None. Documentation of fertility counseling and fertility preservation. A total of 58 subjects met the inclusion criteria; 5 were excluded due to incomplete records, thus N = 53. Of these 75% were female (N = 40). Median age was 14 years at diagnosis and 15 years at first CTX treatment. A total of 51% of subjects (69% of males and 45% of females) had no documentation about potential fertility loss before CTX treatment. Among females where fertility counseling was documented, the only fertility preservation option discussed was leuprolide acetate (LA), which was pursued in all of these cases. Of 13 males (77% postpubertal), 3 were offered sperm banking, of whom 2 declined and the other attempted after treatment began and was azoospermic. Of 53 patients, 1 was referred to a fertility specialist. Mean cumulative CTX dose was 9.2 g in males and 8 g in females. Based on these findings, increasing awareness about infertility risk, fertility preservation options, and referral to fertility specialists is needed among pediatric rheumatologists. Prospective studies are needed to assess fertility outcomes in this patient population (including effectiveness of LA with regard to pregnancy rates [PRs]), as well as barriers/facilitators to fertility counseling and fertility preservation. Copyright © 2016 American Society for Reproductive Medicine. Published by Elsevier Inc. All rights reserved.

Vasculites dos médios e grandes vasos Medium and large vessel vasculitis

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Hebert Roberto Clivati Brandt

2009-02-01

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

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

Directory of Open Access Journals (Sweden)

Daniela Bergamim Pereira

2006-04-01

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

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

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Mouthon, Luc; Dunogue, Bertrand; Guillevin, Loïc

2014-01-01

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

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

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Gulazyk Malikovna Koilubaeva

2014-01-01

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

Comparing Presenting Clinical Features in 48 Children With Microscopic Polyangiitis to 183 Children Who Have Granulomatosis With Polyangiitis (Wegener's)

DEFF Research Database (Denmark)

Cabral, David A; Canter, Debra L; Muscal, Eyal

2016-01-01

with GPA, whose diagnoses had been classified according to both adult- and pediatric-specific criteria. Descriptive statistics were used for comparisons. RESULTS: In total, 231 of 440 patients (64% female) fulfilled the classification criteria for either MPA (n = 48) or GPA (n = 183). The median time...

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

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

2015-03-01

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

Typical tumors of the petrous bone; Typische Tumoren des Felsenbeins

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Ahlhelm, F.; Mueller, U. [Kantonsspital Baden AG, Abteilung fuer Neuroradiologie, Institut fuer Radiologie, Baden (Switzerland); Ulmer, S. [Medizinisch-Radiologisches Institut, Zuerich (Switzerland)

2014-04-15

In the region of the petrous bone, inner acoustic canal and cerebellopontine angle, a variety of different tissues can be found, such as bony, epithelial, neural and vascular structures. Tumorous or tumor-like lesions, vascular or bony malformations or other pathologies can therefore be found in all of these areas. We discuss various frequently occurring tumorous or tumor-like pathologies including congential lesions, such as mucoceles, inflammatory disorders including osteomyelitis, pseudotumors and Wegener's granulomatosis. Benign non-neoplastic lesions, such as cholesteatoma, cholesterol granuloma, epidermoid and benign neoplastic tumors, such as the most commonly found vestibular schwannoma, meningeoma, paraganglioma, vascular pathologies and finally malignant lesions, such as metastasis, chordoma or chondrosarcoma and endolymphatic sac tumor (ELST) are also discussed. The emphasis of this article is on the appearance of these entities in computed tomography (CT) and more so magnetic resonance imaging (MRI), it provides key facts and typical images and discusses possibilities how to distinguish these pathologies. (orig.) [German] In der Region des Felsenbein, inneren Gehoerkanals und Kleinhirnbrueckenwinkels findet sich eine Vielzahl an unterschiedlichen Gewebearten inklusive knoechernes, epitheliales, nervales und vaskulaeres Gewebe. Tumoren oder tumoraehnliche Laesionen, ossaere oder vaskulaere Pathologien koennen entsprechend dort gefunden werden. Wir diskutieren verschiedene Tumoren oder tumoraehnliche Pathologien inklusive angeborene Laesionen wie Muko- und Meningozelen, entzuendliche Veraenderungen wie die Osteomyelitis, Pseudotumoren, die Wegener-Granulomatose, nichtneoplastische Tumoren wie das Epidermoid, Cholesteatom oder Cholesterolgranulom und gutartige neoplastische Tumoren wie das am haeufigsten zu findende Vestibularisschwannom, das Paragangliom und das Meningeom, Gefaessprozesse/-pathologien und schliesslich maligne Laesionen wie Metastasen

Chronic respiratory disfunction due to diffuse alveolar hemorrhage in patients with systemic lupus erythematosus and primary vasculitis.

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Pérez Aceves, Eva; Pérez Cristóbal, Mario; Espinola Reyna, Gerardo A; Ariza Andraca, Raul; Xibille Fridmann, Daniel; Barile Fabris, Leonor A

2013-01-01

Pulmonary hemorrhage (PH) occurs in 2-5% of SLE patients, and is associated with a high mortality rate (79-90%). Diagnostic criteria for this complication include: 1) Pulmonary infiltrates, with at least ¾ of lung tissue involved in a chest x ray, 2) Acute respiratory failure, 3) A decrease of 3g/dL or more in hemoglobin levels. PH might lead to organized pneumonia, collagen deposition, and pulmonary fibrosis which in time might cause changes in pulmonary function tests with either restrictive or obstructive patterns. To evaluate the existence of abnormalities in pulmonary function tests after a PH episode. We included patients with SLE and primary vasculitis that developed PH. During the acute episode, we measured SLEDAI in SLE patients, five factor score in microscopic polyangiitis (MPA) and Birmingham Vasculitis Activity Store (BVAS) in granulomatosis with polyangiitis (GPA) (Wegener). We determined the number of PH events, treatment, and ventilator assistance requirements and correlated its association with abnormal pulmonary function tests. We included 10 patients, 7 with SLE, 2 with MPA and 1 with GPA (Wegener). The mean activity measures were: SLEDAI 20.4 ± 7.5, FFS 2, and BVAS 36. Treatment consisted in methylprednisolone (MPD) in 3 patients, MPD plus cyclophosphamide (CY) in 6 patients, and MPD, CY, IV immunoglobulin, and plasmapheresis in one patient. Five patients required ventilatory support. We found abnormalities in pulmonary function tests in 8 patients, three had an obstructive pattern and five a restrictive pattern; 2 patients did not show any change. We did not find a significant association with any of the studied variables. PH might cause abnormalities in pulmonary function tests and prolonged immunosuppressive treatment could be required. Copyright © 2012 Elsevier España, S.L. All rights reserved.

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

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

2013-06-01

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

Effects of Wegener-Bergeron-Findeisen Process on Global Black Carbon Distribution

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Qi, L.

2016-12-01

In mixed-phase clouds, the Wegener-Bergeron-Findeisen (WBF) process (ice crystals may grow while water drops evaporate, thereby releasing black carbon (BC) particles into the interstitial air) slows down wet scavenging of BC. Rimming (snowflakes fall and collect cloud water drops and the BC in them along their pathways), in contrast, results in more efficient wet scavenging. We systematically investigate the effects of WBF on BC scavenging efficiency, surface BCair, deposition flux, concentration in snow, and washout ratio using a global 3D chemical transport model. We differentiate riming- vs WBF-dominated in-cloud scavenging based on liquid water content and temperature. Specifically, we relate WBF to either temperature or ice mass fraction in mixed-phase clouds. We find that at Jungfraujoch, Switzerland and Abisko, Sweden, where WBF dominates, the discrepancies of simulated BC scavenging efficiency and washout ratio are significantly reduced (from a factor of 3 to 10% and from a factor of 4-5 to a factor of two). However, at Zeppelin, Norway, where riming dominates, simulation of BC scavenging efficiency, BCair, and washout ratio become worse (relative to observations) when WBF is included. There is thus an urgent need for extensive observations to distinguish and characterize riming- versus WBF-dominated aerosol scavenging in mixed-phase clouds and the associated BC scavenging efficiency. We find the reduction resulting from WBF to global BC scavenging efficiency varies substantially, from 8% in the tropics to 76% in the Arctic. The resulting annual mean BCair increases by up to 156% at high altitudes and at northern high latitudes. Overall, WBF halves the model-observation discrepancy (from -65% to -30%) of BCair across North America, Europe, China and the Arctic. Globally WBF increases BC burden from 0.22 to 0.29-0.35 mg m-2 yr-1, which partially explains the gap between observed and previous model simulated BC burdens over land (Bond et al., 2013). In

ANCA-Negative Granulomatosis with Polyangiitis Presenting with Hypertrophic Cranial Pachymeningitis, Abducens Nerve Palsy, and Stenosis of the Internal Carotid Artery

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Shohei Harabuchi

2017-01-01

Full Text Available We report a rare case of granulomatosis with polyangiitis (GPA presenting with hypertrophic cranial pachymeningitis (HCP, abducens nerve palsy, and stenosis of the internal carotid artery (ICA. A 59-year-old Japanese man presented with a year history of nasal obstruction and a 2-month history of slight headache. Histopathological examination of the granulomatous mucosa in the ethmoid sinuses resected by endoscopic sinus surgery revealed necrotizing vasculitis with multinucleated giant cells. The patient was diagnosed with the limited form of GPA as a result of the systemic examination. He declined immunosuppressive treatment. Eighteen months after the diagnosis of GPA, he presented with diplopia and severe headache. Though nasal findings indicating GPA were not observed in the nasal cavity, CT scan revealed a lesion of the right sphenoid sinus eroding the bone of the clivus. Gadolinium-enhanced MRI of the brain showed thickening of the dura mater around the right cavernous sinus and clivus. Magnetic resonance angiography and cerebral angiography revealed narrowing at the C5 portion of the ICA. Intravenous methylprednisolone pulse therapy followed by oral prednisolone and cyclophosphamide resolved headache and dramatically improved HCP and stenosis of the ICA.

Increased IgG4-Positive Plasma Cells in Granulomatosis with Polyangiitis: A Diagnostic Pitfall of IgG4-Related Disease

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Sing Yun Chang

2012-01-01

Full Text Available Granulomatosis with polyangiitis (Wegener’s (GPA may mimic IgG4-related disease (IgG4-RD on histologic examination of some biopsies, especially those from head and neck sites. IgG4 immunostain is often performed in this context for differential diagnosis with IgG4-RD. However, the prevalence of IgG4+ cells in GPA has not been explored. We examined the IgG4+ cells in 26 cases confirmed as GPA by a thorough clinical and pathologic assessment. Twenty-six biopsies consisted of 14 sinonasal/oral cavity/nasopharynx, 7 orbit/periorbital, 3 lung/pleura, 1 iliac fossa/kidney, and 1 dura specimens. Eight of 26 (31% biopsies revealed increased IgG4+ cells (>30/HPF and >40% in IgG4+/IgG+ ratio. The IgG4+ cells and IgG4+/IgG+ ratio ranged 37–137/hpf and 44–83%, respectively. Eight biopsies with increased IgG4+ cells were from sinonasal (n=4 or orbital/periorbital (n=4 sites. In conclusion, increased IgG4+ cells are not uncommonly seen in sinonasal or orbital/periorbital biopsies of GPA, which could pose as a diagnostic pitfall.

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

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

2014-07-01

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

Aortic {sup 18}F-FDG uptake in patients suffering from granulomatosis with polyangiitis

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Kemna, Michael J. [Maastricht University Medical Center, Department of Nephrology and Clinical Immunology, Maastricht (Netherlands); Maastricht University, Cardiovascular Research Institute Maastricht (CARIM), Maastricht (Netherlands); Bucerius, Jan [Maastricht University, Cardiovascular Research Institute Maastricht (CARIM), Maastricht (Netherlands); Maastricht University Medical Center, Department of Nuclear Medicine, Maastricht (Netherlands); University Hospital RWTH Aachen, Department of Nuclear Medicine, Aachen (Germany); Drent, Marjolein [Maastricht University, Department of Pharmacology and Toxicology, Maastricht (Netherlands); Voeoe, Stefan [Maastricht University, Cardiovascular Research Institute Maastricht (CARIM), Maastricht (Netherlands); Maastricht University Medical Center, Department of Nuclear Medicine, Maastricht (Netherlands); Veenman, Martine [Maastricht University, Cardiovascular Research Institute Maastricht (CARIM), Maastricht (Netherlands); Paassen, Pieter van [Maastricht University Medical Center, Department of Nephrology and Clinical Immunology, Maastricht (Netherlands); Tervaert, Jan Willem Cohen [Maastricht University, Cardiovascular Research Institute Maastricht (CARIM), Maastricht (Netherlands); Sint Franciscus Gasthuis, Noordoever Academy, Rotterdam (Netherlands); Kroonenburgh, Marinus J.P.G. van [Maastricht University Medical Center, Department of Nuclear Medicine, Maastricht (Netherlands)

2015-08-15

The objective of the study was to systematically assess aortic inflammation in patients with granulomatosis with polyangiitis (GPA) using {sup 18}F-2-deoxy-2-[{sup 18}F]fluoro-D-glucose (FDG) positron emission tomography (PET)/CT. Aortic inflammation was studied in PET/CT scans obtained from 21 patients with GPA; 14 patients with sarcoidosis were included as disease controls, 7 patients with stage I or II head and neck carcinoma ascertained during routine clinical practice were used as healthy controls (HC) and 5 patients with large vessel vasculitis (LVV) were used as positive controls. Aortic {sup 18}F-FDG uptake was expressed as the blood-normalized maximum standardized uptake value (SUV{sub max}), known as the target to background ratio (mean TBR{sub max}). The mean TBR{sub max} (interquartile range) of the aorta in patients with GPA, sarcoidosis, HC and LVV were 1.75 (1.32-2.05), 1.62 (1.54-1.74), 1.29 (1.22-1.52) and 2.03 (1.67-2.45), respectively. The mean TBR{sub max} was significantly higher in patients suffering from GPA or LVV compared to HC (p < 0.05 and p < 0.005, respectively) and tended to be higher in patients suffering from sarcoidosis, but this did not reach statistical significance (p = 0.098). The mean TBR{sub max} of the most diseased segment was significantly higher compared to HC [1.57 (1.39-1.81)] in LVV patients [2.55 (2.22-2.82), p < 0.005], GPA patients [2.17 (1.89-2.83), p < 0.005] and patients suffering from sarcoidosis [2.04 (1.88-2.20), p < 0.05]. In GPA patients, the mean TBR{sub max} of the aorta was significantly higher in patients with previous renal involvement [2.01 (1.69-2.53)] compared to patients without renal involvement in the past [1.60 (1.51-1.80), p < 0.05]. Interrater reproducibility with a second reader was high (all intraclass correlation coefficients >0.9). Patients suffering from GPA show marked aortic FDG uptake. (orig.)

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

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

2015-03-01

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

Bone marrow accumulation in gallium scintigraphy in patients with adult still's disease

International Nuclear Information System (INIS)

Kanegae, Futoshi; Tada, Yoshifumi; Ohta, Akihide; Ushiyama, Osamu; Suzuki; Noriaki; Koarada, Syuichi; Haruta, Yoshio; Yoshikai, Tomonori; Nagasawa, Kohei

2002-01-01

We investigated the features and the usefulness of gallium scintigraphy in the diagnosis and the assessment of Adult Still's disease (ASD) by retrospective case review. Gallium scintigraphy have been done for 11 cases of ASD (3 males and 8 females) and 4 females were positive. Among these, 67 Ga-citrate was accumulated to the bone marrow in all 4 cases and to the major joints in 2 cases. Positive cases were rather serious and administered more immunosuppressants than negative cases. In order to characterize gallium scintigraphy findings of ASD, i.e. bone marrow accumulation, we analyzed 130 cases of collagen vascular disease. Although 101 cases (77.7%) were positive, only 7 cases (5.4%) showed the accumulation of 67 Ga-citrate to the bone marrow. These include 3 cases with ASD, and 1 case with systemic lupus erythematosus, polyarteritis nodosa, Wegener's granulomatosis and Sjogren's syndrome. We also accumulated 18 patients who exhibited bone marrow accumulation of 69 Ga-citrate, and found that 7 patients had collagen vascular and their related diseases. In conclusion, bone marrow accumulation in gallium scintigraphy is a specific feature of collagen vascular diseases, especially ASD, and it is suggested that cases with positive gallium scintigraphy in ASD can be serious and resistant to treatment. (author)

Risk factors and treatment of pneumothorax secondary to granulomatosis with polyangiitis: a clinical analysis of 25 cases.

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Shi, Xuhua; Zhang, Yongfeng; Lu, Yuewu

2018-01-15

To investigate the risk factors and treatment strategies for pneumothorax secondary to granulomatosis with polyangiitis (GPA). Retrospective analysis of cases with pneumothorax secondary to GPA from our own practice and published on literature. A total of 25 patients, 18 males and 7 females, mean age 44 ± 15.7 years, were analyzed. Diagnosis included pneumothorax (11 cases), hydropneumothorax (n = 5), empyema (n = 8) and hemopneumothorax (n = 1). 88% (22/25) patients showed single/multiple pulmonary/ subpleural nodules with/without cavitation on chest imaging. Erythrocyte sedimentation rate and C-reactive protein were both elevated. Corticosteroids and immunosuppressive agents were used in 16 cases. Five cases received steroid pulse therapy, of which 4 patients survived. Pleural drainage was effective in some patients. Seven patients underwent surgical operations. In the 10 fatal cases, infection and respiratory failure were the most common cause. Lung biopsy/ autopsy showed lung/pleural necrotizing granulomatous vasculitis, breaking into the chest cavity, pleural fibrosis, bronchial pleural fistula, etc. The mean age in the death group was greater than the survival group (53 ± 12.9 years vs 40.1 ± 14.7 years, p = 0.05), the ineffective pleural drainage was also higher in the death group (5/5 vs 0/7, p = 0.01). Pneumothorax was seen in the active GPA, due to a variety of reasons, and gave rise to high fatality rate. Aggressive treatment of GPA can improve the prognosis. Older and lack of response for pleural drainage indicates poor prognosis.

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

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

2014-01-01

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

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

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

2016-08-01

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

Recepción temprana de la teoría de la deriva continental y su competencia con las teorías rivales

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Pérez-Malváez, Carlos

2003-06-01

Full Text Available In 1912 Alfred Lothar Wegener proposed the theory of the continental drift. Wegener thought that the displacement explained more paleontological and geophysical data than any other rival program. The great virtue of the drift theory resided in their great capacity of synthesis. Wegener appealed to the information provided by the Geology, Paleontology and Paleoclimatology.

Alfred Lothar Wegener propuso la teoría de la deriva continental en 1912. Wegener ciertamente pensó que el desplazamiento explicaba mas datos paleontológicos y geofísicos que cualquiera de los programas de investigación establecidos. Desde luego, la gran virtud de la teoría de la deriva fue su gran capacidad de síntesis. Wegener recurrió a la información que provenía de la Geología, Geofísica, Paleontología y Paleoclimatología.

In vitro T lymphocyte responses to proteinase 3 (PR3) and linear peptides of PR3 in patients with Wegener's granulomatosis (WG)

NARCIS (Netherlands)

Van der Geld, YM; Huitema, MG; Franssen, CFM; Limburg, PC; Kallenberg, CGM; van der Zee, R.

2000-01-01

T cell-mediated immunity is thought to play an important role in the pathogenesis of WG. In previous studies a minority of WG patients as well as some healthy controls showed in vitro proliferation of their peripheral blood mononuclear cells (PBMC) to PR3, the main autoantigen in WG. The relevant

Verkeersveiligheidsaudit en -inspectie.

NARCIS (Netherlands)

2007-01-01

In een verkeersveiligheidsaudit (VVA) en een verkeersveiligheidsinspectie (VVI) wordt weginfrastructuur getoetst op verkeersveiligheid. Een VVA toetst het ontwerp van nieuwe wegen of van reconstructie van bestaande wegen, terwijl een VVI bestaande wegen toetst. Een VVA beoogt dus al vóór de aanleg

Differential diagnosis of granulomatous lung disease: clues and pitfalls

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Shinichiro Ohshimo

2017-09-01

Full Text Available Granulomatous lung diseases are a heterogeneous group of disorders that have a wide spectrum of pathologies with variable clinical manifestations and outcomes. Precise clinical evaluation, laboratory testing, pulmonary function testing, radiological imaging including high-resolution computed tomography and often histopathological assessment contribute to make a confident diagnosis of granulomatous lung diseases. Differential diagnosis is challenging, and includes both infectious (mycobacteria and fungi and noninfectious lung diseases (sarcoidosis, necrotising sarcoid granulomatosis, hypersensitivity pneumonitis, hot tub lung, berylliosis, granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, rheumatoid nodules, talc granulomatosis, Langerhans cell histiocytosis and bronchocentric granulomatosis. Bronchoalveolar lavage, endobronchial ultrasound-guided transbronchial needle aspiration, transbronchial cryobiopsy, positron emission tomography and genetic evaluation are potential candidates to improve the diagnostic accuracy for granulomatous lung diseases. As granuloma alone is a nonspecific histopathological finding, the multidisciplinary approach is important for a confident diagnosis.

Differential diagnosis of granulomatous lung disease: clues and pitfalls: Number 4 in the Series "Pathology for the clinician" Edited by Peter Dorfmüller and Alberto Cavazza.

Science.gov (United States)

Ohshimo, Shinichiro; Guzman, Josune; Costabel, Ulrich; Bonella, Francesco

2017-09-30

Granulomatous lung diseases are a heterogeneous group of disorders that have a wide spectrum of pathologies with variable clinical manifestations and outcomes. Precise clinical evaluation, laboratory testing, pulmonary function testing, radiological imaging including high-resolution computed tomography and often histopathological assessment contribute to make a confident diagnosis of granulomatous lung diseases. Differential diagnosis is challenging, and includes both infectious (mycobacteria and fungi) and noninfectious lung diseases (sarcoidosis, necrotising sarcoid granulomatosis, hypersensitivity pneumonitis, hot tub lung, berylliosis, granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, rheumatoid nodules, talc granulomatosis, Langerhans cell histiocytosis and bronchocentric granulomatosis). Bronchoalveolar lavage, endobronchial ultrasound-guided transbronchial needle aspiration, transbronchial cryobiopsy, positron emission tomography and genetic evaluation are potential candidates to improve the diagnostic accuracy for granulomatous lung diseases. As granuloma alone is a nonspecific histopathological finding, the multidisciplinary approach is important for a confident diagnosis. Copyright ©ERS 2017.

Effects of the Wegener-Bergeron-Findeisen process on global black carbon distribution

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Qi, Ling; Li, Qinbin; He, Cenlin; Wang, Xin; Huang, Jianping

2017-06-01

We systematically investigate the effects of Wegener-Bergeron-Findeisen process (hereafter WBF) on black carbon (BC) scavenging efficiency, surface BCair, deposition flux, concentration in snow (BCsnow, ng g-1), and washout ratio using a global 3-D chemical transport model (GEOS-Chem). We differentiate riming- versus WBF-dominated in-cloud scavenging based on liquid water content (LWC) and temperature. Specifically, we implement an implied WBF parameterization using either temperature or ice mass fraction (IMF) in mixed-phase clouds based on field measurements. We find that at Jungfraujoch, Switzerland, and Abisko, Sweden, where WBF dominates in-cloud scavenging, including the WBF effect strongly reduces the discrepancies of simulated BC scavenging efficiency and washout ratio against observations (from a factor of 3 to 10 % and from a factor of 4-5 to a factor of 2). However, at Zeppelin, Norway, where riming dominates, simulation of BC scavenging efficiency, BCair, and washout ratio become worse (relative to observations) when WBF is included. There is thus an urgent need for extensive observations to distinguish and characterize riming- versus WBF-dominated aerosol scavenging in mixed-phase clouds and the associated BC scavenging efficiency. Our model results show that including the WBF effect lowers global BC scavenging efficiency, with a higher reduction at higher latitudes (8 % in the tropics and up to 76 % in the Arctic). The resulting annual mean BCair increases by up to 156 % at high altitudes and at northern high latitudes because of lower temperature and higher IMF. Overall, WBF halves the model-observation discrepancy (from -65 to -30 %) of BCair across North America, Europe, China and the Arctic. Globally WBF increases BC burden from 0.22 to 0.29-0.35 mg m-2 yr-1, which partially explains the gap between observed and previous model-simulated BC burdens over land. In addition, WBF significantly increases BC lifetime from 5.7 to ˜ 8 days. Additionally

Low Dose Naltrexone to Improve Physical Health in Patients With Vasculitis

Science.gov (United States)

2018-06-08

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

Evaluation of GPM IMERG Early, Late, and Final rainfall estimates using WegenerNet gauge data in southeastern Austria

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S. O

2017-12-01

Full Text Available The Global Precipitation Measurement (GPM Integrated Multi-satellite Retrievals for GPM (IMERG products provide quasi-global (60° N–60° S precipitation estimates, beginning March 2014, from the combined use of passive microwave (PMW and infrared (IR satellites comprising the GPM constellation. The IMERG products are available in the form of near-real-time data, i.e., IMERG Early and Late, and in the form of post-real-time research data, i.e., IMERG Final, after monthly rain gauge analysis is received and taken into account. In this study, IMERG version 3 Early, Late, and Final (IMERG-E,IMERG-L, and IMERG-F half-hourly rainfall estimates are compared with gauge-based gridded rainfall data from the WegenerNet Feldbach region (WEGN high-density climate station network in southeastern Austria. The comparison is conducted over two IMERG 0.1°  ×  0.1° grid cells, entirely covered by 40 and 39 WEGN stations each, using data from the extended summer season (April–October for the first two years of the GPM mission. The entire data are divided into two rainfall intensity ranges (low and high and two seasons (warm and hot, and we evaluate the performance of IMERG, using both statistical and graphical methods. Results show that IMERG-F rainfall estimates are in the best overall agreement with the WEGN data, followed by IMERG-L and IMERG-E estimates, particularly for the hot season. We also illustrate, through rainfall event cases, how insufficient PMW sources and errors in motion vectors can lead to wide discrepancies in the IMERG estimates. Finally, by applying the method of Villarini and Krajewski (2007, we find that IMERG-F half-hourly rainfall estimates can be regarded as a 25 min gauge accumulation, with an offset of +40 min relative to its nominal time.

Evaluation of GPM IMERG Early, Late, and Final rainfall estimates using WegenerNet gauge data in southeastern Austria

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O, Sungmin; Foelsche, Ulrich; Kirchengast, Gottfried; Fuchsberger, Juergen; Tan, Jackson; Petersen, Walter A.

2017-12-01

The Global Precipitation Measurement (GPM) Integrated Multi-satellite Retrievals for GPM (IMERG) products provide quasi-global (60° N-60° S) precipitation estimates, beginning March 2014, from the combined use of passive microwave (PMW) and infrared (IR) satellites comprising the GPM constellation. The IMERG products are available in the form of near-real-time data, i.e., IMERG Early and Late, and in the form of post-real-time research data, i.e., IMERG Final, after monthly rain gauge analysis is received and taken into account. In this study, IMERG version 3 Early, Late, and Final (IMERG-E,IMERG-L, and IMERG-F) half-hourly rainfall estimates are compared with gauge-based gridded rainfall data from the WegenerNet Feldbach region (WEGN) high-density climate station network in southeastern Austria. The comparison is conducted over two IMERG 0.1° × 0.1° grid cells, entirely covered by 40 and 39 WEGN stations each, using data from the extended summer season (April-October) for the first two years of the GPM mission. The entire data are divided into two rainfall intensity ranges (low and high) and two seasons (warm and hot), and we evaluate the performance of IMERG, using both statistical and graphical methods. Results show that IMERG-F rainfall estimates are in the best overall agreement with the WEGN data, followed by IMERG-L and IMERG-E estimates, particularly for the hot season. We also illustrate, through rainfall event cases, how insufficient PMW sources and errors in motion vectors can lead to wide discrepancies in the IMERG estimates. Finally, by applying the method of Villarini and Krajewski (2007), we find that IMERG-F half-hourly rainfall estimates can be regarded as a 25 min gauge accumulation, with an offset of +40 min relative to its nominal time.

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

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

2011-01-01

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

Bone marrow accumulation in gallium scintigraphy in patients with adult still's disease

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Kanegae, Futoshi; Tada, Yoshifumi; Ohta, Akihide; Ushiyama, Osamu; Suzuki; Noriaki; Koarada, Syuichi; Haruta, Yoshio; Yoshikai, Tomonori; Nagasawa, Kohei [Saga Medical School (Japan)

2002-12-01

We investigated the features and the usefulness of gallium scintigraphy in the diagnosis and the assessment of Adult Still's disease (ASD) by retrospective case review. Gallium scintigraphy have been done for 11 cases of ASD (3 males and 8 females) and 4 females were positive. Among these, 67 Ga-citrate was accumulated to the bone marrow in all 4 cases and to the major joints in 2 cases. Positive cases were rather serious and administered more immunosuppressants than negative cases. In order to characterize gallium scintigraphy findings of ASD, i.e. bone marrow accumulation, we analyzed 130 cases of collagen vascular disease. Although 101 cases (77.7%) were positive, only 7 cases (5.4%) showed the accumulation of {sup 67}Ga-citrate to the bone marrow. These include 3 cases with ASD, and 1 case with systemic lupus erythematosus, polyarteritis nodosa, Wegener's granulomatosis and Sjogren's syndrome. We also accumulated 18 patients who exhibited bone marrow accumulation of {sup 69}Ga-citrate, and found that 7 patients had collagen vascular and their related diseases. In conclusion, bone marrow accumulation in gallium scintigraphy is a specific feature of collagen vascular diseases, especially ASD, and it is suggested that cases with positive gallium scintigraphy in ASD can be serious and resistant to treatment. (author)

Proinflammatory genotype of interleukin-1 and interleukin-1 receptor antagonist is associated with ESRD in proteinase 3-ANCA vasculitis patients.

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Borgmann, Stefan; Endisch, Georg; Hacker, Ulrich T; Song, Bong-Seok; Fricke, Harald

2003-05-01

Small-vessel vasculitides are associated with antineutrophil cytoplasmic antibodies (ANCAs). Cytoplasmic ANCAs are targeted mainly against proteinase 3 (PR3), whereas myeloperoxidase (MPO) is the major antigen of perinuclear ANCAs. These relapsing vasculitides show heterogeneous clinical pictures, and disease severity may vary broadly from mild local organ manifestation to acute organ failure (eg, renal failure). We tested whether two cytokine polymorphisms in the interleukin-1beta (IL-1beta) and IL-1 receptor antagonist (IL-1ra) genes, known to determine cytokine secretion, are associated with clinical manifestations and outcome of ANCA-associated vasculitides. Polymerase chain reaction and restriction fragment length polymorphism analyses were performed to determine polymorphisms in the IL-1beta and IL-1ra genes in 79 patients with PR3-ANCA, 30 patients with MPO-ANCA vasculitis, and 196 healthy controls. The frequency of the so-called proinflammatory genotype, characterized by high secretion of IL-1beta and low secretion of its antagonist IL-1ra, was increased significantly in patients with PR3-ANCA with end-stage renal disease. Patients with a renal manifestation of PR3-ANCA vasculitis have an increased risk for developing end-stage renal disease when carrying the proinflammatory IL-1beta/IL-1ra genotype. Anti-inflammatory therapy specifically antagonizing the proinflammatory effect of IL-1beta may be a promising treatment for patients with Wegener's granulomatosis with renal manifestations.

Hydroxyurea for Treatment of Nephrotic Syndrome Associated With Polycythemia Vera.

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Hundemer, Gregory L; Rosales, Ivy A; Chen, Yi-Bin; Colvin, Robert B; Tolkoff-Rubin, Nina E

2016-09-01

Myeloproliferative disorders are a rare cause of focal segmental glomerulosclerosis (FSGS), although the mechanism is unclear. Hydroxyurea is commonly used in these disorders for its cytoreductive properties; however, the effect of this treatment on proteinuria or kidney function remains unclear in cases of myeloproliferative disorder-associated FSGS. We describe the clinical course of a patient with polycythemia vera and nephrotic-range proteinuria, demonstrated to have FSGS on biopsy. The patient had a distant history of granulomatosis with polyangiitis (Wegener's), for which he routinely had his kidney function and proteinuria measured, allowing for early detection of nephrotic syndrome soon after being diagnosed with polycythemia vera. Treatment with hydroxyurea resulted in rapid improvement in proteinuria that correlated with a decrease in hematocrit. This response was replicated 2 additional times when the patient was taken off and then restarted on hydroxyurea therapy. He now maintains a steady dose of hydroxyurea with favorable kidney measures (proteinuria with <1g/d of protein excretion and serum creatinine of 1.27mg/dL [corresponding to estimated glomerular filtration rate of 56mL/min/1.73 m(2)]). This case suggests that early screening and treatment for myeloproliferative disorder-associated FSGS may lead to improved long-standing kidney function. Copyright © 2016 National Kidney Foundation, Inc. Published by Elsevier Inc. All rights reserved.

Cocaine-induced encephalocele: case report and literature review.

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Albert, Ladislau; DeMattia, Joseph A

2011-01-01

The abuse of cocaine can lead to significant destruction of midline craniofacial structures. This process occurs secondary to myriad mechanisms, including ischemic necrosis, irritation by chemical adulterants, and direct trauma during its administration. Coupled with a prolonged chronic infection of intranasal and anterior skull base regions, an encephalocele can be formed. We report a case of an encephalocele secondary to cocaine use and its associated complications. A 56-year-old man presented with altered mental status and cerebritis secondary to the presence of an intranasal encephalocele. On computed tomography, extensive destruction of the anterior cranial fossa was observed. The patient had a 30-year history of intranasal cocaine abuse, and his urine tested positive for the presence of cocaine on admission. The patient was treated with intravenous antibiotics and underwent a repair of his cranial defect and resection of the encephalocele. The patient made a good recovery after treatment. Alternative causes of an encephalocele, including trauma, surgery, and congenital malformation, were ruled out in this patient. Histopathological analysis of the necrotic tissue and the absence of renal or pulmonary disease also indicated that the patient did not suffer from Wegener granulomatosis, a known cause of spontaneous intranasal lesions. To the best of our knowledge, this is the first report of an encephalocele likely induced solely by cocaine abuse.

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

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

2017-01-01

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

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

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

2013-07-01

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

Representation of Arctic mixed-phase clouds and the Wegener-Bergeron-Findeisen process in climate models: Perspectives from a cloud-resolving study

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Fan, Jiwen; Ghan, Steven; Ovchinnikov, Mikhail; Liu, Xiaohong; Rasch, Philip J.; Korolev, Alexei

2011-01-01

Two types of Arctic mixed-phase clouds observed during the ISDAC and M-PACE field campaigns are simulated using a 3-dimensional cloud-resolving model (CRM) with size-resolved cloud microphysics. The modeled cloud properties agree reasonably well with aircraft measurements and surface-based retrievals. Cloud properties such as the probability density function (PDF) of vertical velocity (w), cloud liquid and ice, regimes of cloud particle growth, including the Wegener-Bergeron-Findeisen (WBF) process, and the relationships among properties/processes in mixed-phase clouds are examined to gain insights for improving their representation in General Circulation Models (GCMs). The PDF of the simulated w is well represented by a Gaussian function, validating, at least for arctic clouds, the subgrid treatment used in GCMs. The PDFs of liquid and ice water contents can be approximated by Gamma functions, and a Gaussian function can describe the total water distribution, but a fixed variance assumption should be avoided in both cases. The CRM results support the assumption frequently used in GCMs that mixed phase clouds maintain water vapor near liquid saturation. Thus, ice continues to grow throughout the stratiform cloud but the WBF process occurs in about 50% of cloud volume where liquid and ice co-exist, predominantly in downdrafts. In updrafts, liquid and ice particles grow simultaneously. The relationship between the ice depositional growth rate and cloud ice strongly depends on the capacitance of ice particles. The simplified size-independent capacitance of ice particles used in GCMs could lead to large deviations in ice depositional growth.

Clinical Transcriptomics in Systemic Vasculitis (CUTIS)

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2018-04-10

Cryoglobulinemic Vasculitis (CV); Drug-induced Vasculitis; Eosinophilic Granulomatosis With Polyangiitis (EGPA); IgA Vasculitis; Isolated Cutaneous Vasculitis; Granulomatosis With Polyangiitis (GPA); Microscopic Polyangiitis (MPA); Polyarteritis Nodosa (PAN); Urticarial Vasculitis; Vasculitis

Anti-neutrophil cytoplasmic antibodies in rheumatoid arthritis: two case reports and review of literature

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Spoerl David

2012-12-01

Full Text Available Abstract Background Anti-neutrophil cytoplasmic antibodies are typically detected in anti-neutrophil cytoplasmic antibody associated vasculitis, but are also present in a number of chronic inflammatory non-vasculitic conditions like rheumatoid arthritis. Rare cases of granulomatosis with polyangiitis (formerly known as Wegener’s granulomatosis, a vasculitic disorder frequently associated with the presence of anti-neutrophil cytoplasmic antibodies in patients with rheumatoid arthritis have been described in literature. Case presentation We report two middle-aged female patients with rheumatoid arthritis who developed anti-neutrophil cytoplasmic antibodies and symptoms reminiscent of granulomatosis with polyangiitis. Despite the lack of antibodies specific for proteinase 3 and the absence of a classical histology, we report a probable case of granulomatosis with polyangiitis in the first patient, and consider rheumatoid vasculitis in the second patient. Conclusion Taken together with previous reports, these cases highlight that anti-neutrophil cytoplasmic antibodies have to be evaluated very carefully in patients with rheumatoid arthritis. In this context, anti-neutrophil cytoplasmic antibodies detected by indirect immunofluorescence appear to have a low diagnostic value for granulomatosis with polyangiitis. Instead they may have prognostic value for assessing the course of rheumatoid arthritis.

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

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

2014-01-01

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

Clinical features and outcomes of ANCA-associated renal vasculitis

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Sidy Mohamed Seck

2012-01-01

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

Association of STAT4 rs7574865 polymorphism with autoimmune diseases: a meta-analysis.

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Liang, Ya-Ling; Wu, Hua; Shen, Xi; Li, Pei-Qiang; Yang, Xiao-Qing; Liang, Li; Tian, Wei-Hua; Zhang, Li-Feng; Xie, Xiao-Dong

2012-09-01

The association between the signal transducer and activator of transcription 4 (STAT4) gene rs7574865 single nucleotide polymorphism and different autoimmune diseases remains controversial and ambiguous. We conducted this study to investigate whether combined evidence shows the association between STAT4 rs7574865 polymorphism and autoimmune diseases. Comprehensive Medline search and review of the references were used to get the relevant reports published before September 2011. Meta-analysis was conducted for genotype T/T (recessive effect), T/T + G/T (dominant effect) and T allele in random effects models. 40 studies with 90 comparisons including 32 systemic lupus erythematosus (SLE), 19 rheumatoid arthritis (RA), 3 type 1 diabetes (T1D), 11 Systemeric Sclerosis (SSc), 4 inflammatory bowed diseases (IBD), 3 Primary Sjogren's syndrome (pSS), 4 juvenile idiopathic arthritis (JIA), 2 Primary antiphospholipid syndrome (APS), 1 Autoimmune thyroid diseases, 1 multiple sclerosis, 1 Psoriasis, 1 Wegener's granulomatosis, 1 Type 2 diabetes, and 1 giant cell arteritis disease were available for this meta-analysis. The overall odds ratios for rs7574865 T-allele significantly increased in SLE, RA, T1D, SSc, JIA, and APS (OR = 1.56, 1.25, 1.13, 1.34, 1.25, and 2.15, respectively, P rs7574865 T allele confers susceptibility to SLE, RA, T1D, SSc, JIA, APS, IBD-UC, and pSS patients, supporting the hypothesis of association between STAT4 gene polymorphism and subgroup of autoimmune diseases.

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

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

2016-09-01

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

Improved survival with renal transplantation for end-stage renal disease due to granulomatosis with polyangiitis: data from the United States Renal Data System.

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Wallace, Zachary S; Wallwork, Rachel; Zhang, Yuqing; Lu, Na; Cortazar, Frank; Niles, John L; Heher, Eliot; Stone, John H; Choi, Hyon K

2018-05-14

Renal transplantation is the optimal treatment for selected patients with end-stage renal disease (ESRD). However, the survival benefit of renal transplantation among patients with ESRD attributed to granulomatosis with polyangiitis (GPA) is unknown. We identified patients from the United States Renal Data System with ESRD due to GPA (ESRD-GPA) between 1995 and 2014. We restricted our analysis to waitlisted subjects to evaluate the impact of transplantation on mortality. We followed patients until death or the end of follow-up. We compared the relative risk (RR) of all-cause and cause-specific mortality in patients who received a transplant versus non-transplanted patients using a pooled logistic regression model with transplantation as a time-varying exposure. During the study period, 1525 patients were waitlisted and 946 received a renal transplant. Receiving a renal transplant was associated with a 70% reduction in the risk of all-cause mortality in multivariable-adjusted analyses (RR=0.30, 95% CI 0.25 to 0.37), largely attributed to a 90% reduction in the risk of death due to cardiovascular disease (CVD) (RR=0.10, 95% 0.06-0.16). Renal transplantation is associated with a significant decrease in all-cause mortality among patients with ESRD attributed to GPA, largely due to a decrease in the risk of death to CVD. Prompt referral for transplantation is critical to optimise outcomes for this patient population. © Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2018. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

Peritoneal Keratin Granulomatosis Associated with Endometrioid Adenocarcinoma of the Uterine Corpus in a Woman with Polycystic Ovaries: A Potential Pitfall—A Case Report and Review of the Literature

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Helen J. Trihia

2017-01-01

Full Text Available Peritoneal keratin granulomatosis is a rare condition included under granulomatous lesions of the peritoneum. It can be secondary to neoplasms of the female genital tract and can mimic carcinomatosis intraoperatively. A case of a 40-year-old woman with a history of polycystic ovaries and a chief complaint of vaginal bleeding is presented. She was diagnosed with endometrioid adenocarcinoma with squamous differentiation in endometrial curettings. Intraoperatively, many peritoneal nodules were found, interpreted as peritoneal carcinomatosis. The woman underwent a total abdominal hysterectomy with bilateral salpingo-oophorectomy, omentectomy, bilateral pelvic lymphadenectomy, and appendicectomy. Multiple biopsies were taken, as well as peritoneal washings. Microscopic examination revealed multiple keratin granulomas on the serosal surface of the ovaries, fallopian tubes, appendix, and omentum. Lymph node metastasis was not found. Peritoneal keratin granulomas (PKGs have been reported in cases of endometrioid adenocarcinoma with squamous differentiation of the uterine corpus, ovary, and atypical adenomyoma. It should be noted that the prognosis of cases of peritoneal keratin granulomas without viable tumor cells is favourable and that the histologic examination is essential for its diagnosis. We report a case of PKG in a patient with endometrial carcinoma with squamous differentiation, being the first in a woman with polycystic ovaries.

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

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Nicolás Di Benedetto

2010-04-01

Full Text Available El objetivo de esta serie fue describir las características generales y manifestaciones clínicas de pacientes con vasculitis de pequeños vasos que concurrieron al servicio de autoinmunidad de un hospital de la comunidad y compararlas con la literatura. Se revisaron de manera retrospectiva las historias clínicas archivadas bajo el rótulo de vasculitis en un período de 16 años. Se seleccionaron aquellos pacientes que cumplieran criterios diagnósticos de vasculitis de pequeños vasos. Se extrajeron los datos de interés y se constató la supervivencia mediante contacto telefónico. Posteriormente se realizó una búsqueda bibliográfica y se compararon los resultados. Se incluyeron 13 pacientes con granulomatosis de Wegener, 6 con síndrome de Churg Strauss y 10 con poliangeítis microscópica. El 55% (16 fueron menores de 55 años al diagnóstico y hubo predominio del sexo femenino (2.6:1. La demora diagnóstica fue mayor a un año en el 46% de los casos. Los sistemas más frecuentemente afectados fueron respiratorio y otorrinolaringológico. El 79% presentó anticuerpos anti-citoplasma de neutrófilos (ANCA positivos. La mortalidad fue del 24% (7/29. Al comparar las características de los pacientes incluidos en esta serie con lo descripto en la literatura, se hallaron diferencias en cuanto a la forma de presentación, sistemas comprometidos y porcentaje de pacientes ANCA positivos; además se observó que el resultado negativo de este marcador parece asociarse con mayor demora diagnóstica y en consecuencia peor pronóstico, lo que remarca su importancia como herramienta adicional no invasiva al momento del diagnóstico.The objective of this series was to describe the general characteristics and clinical manifestations of patients with small vessel vasculitis who were assisted in the autoimmunity department of a community public hospital and to compare the results with the literature. Clinical records under the label of vasculitis in a

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

Science.gov (United States)

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

2015-01-01

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

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

Directory of Open Access Journals (Sweden)

Lucyna Mastalerz

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

Necrotizing RPGN with linear anti IgG deposits in a patient with history of granulomatosis with polyangiitis: a case report

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Parekh N

2014-11-01

Full Text Available Ninad Parekh, Edward Epstein, Suzanne El-Sayegh Department of Medicine, Division of Nephrology, Staten Island University Hospital, Staten Island, NY, USA Introduction: Diagnosing the etiology of a rapidly progressive glomerulonephritis is of vital importance to guide appropriate therapeutic management. This case highlights the complexity involved in establishing diagnosis when presentation is atypical. In certain cases diagnosis cannot be established based on clinical presentation or biopsy findings alone, and critical analysis of biopsy findings in context of clinical presentation is crucial to guide the clinical decision-making process.Case presentation: A 47-year-old Hispanic male with history of granulomatosis with polyangiitis (GPA in remission on azathioprine, presented with fatigue and lethargy. Physical examination was unremarkable. Laboratory data revealed elevated creatinine and otherwise normal electrolytes. Urinalysis showed numerous dysmorphic red blood cells with few red cell casts. His serologic results were all negative except anti-proteinase-3 antibody at very low titers. Kidney biopsy showed necrotizing crescentic glomerulonephritis with linear immunoglobulin G staining along the basement membrane.Conclusion: This case presented conflicting serologic and histopathologic findings. The presence of anti-proteinase-3 antibody supported diagnosis of recurrence of GPA. However, linear staining of immunoglobulin G (IgG on immunofluorescence (IF staining of renal biopsy supported anti-glomerular basement membrane (GBM disease. The treatment of anti-GBM disease and GPA both involve immunosuppression with prednisone and cyclophosphamide. However, patients with anti-GBM disease are also treated with plasmapheresis early in the disease presentation to prevent further damage. The patient with GPA, on the other hand, was shown to benefit from plasmapheresis only in the case of severe renal disease (serum creatinine level more than 5 mg/dL or

Leishmaniasis mucosa y otras lesiones destructivas centrofaciales

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G. Rodríguez

1994-12-01

Full Text Available Varias enfermedades producen lesiones del área centrofacial. Una de las más frecuentes en nuestro medio es la leishmaniasis destructivas mucosa. Como el INS es un centro de estudio de leishmaniasis, se atendieron pacientes o sus biopsias con afecciones del macizo centrofacial, primariamente remitidas con el diagnóstico clínico de leihsmaniasis mucosa. En un período de 7 años (1987-1993, se estudiaron 134 biopsias de estos pacientes. El diagnóstico de leishmaniasis mucosa fue definitivo en 26 casos, por demostración del amastigote con la coloración de hematoxilina eosina y el mismo diagnóstico se estableció por patrón histopatológico solamente, en 27 casos. Una técnica inmunoenzimática para demostrar los amastigotes no fue satisfactoria. La perforación banal del tabique nasal (52 biopsias es la entidad que el clínico y el patólogo confunden con mayor frecuencia con la leishmaniasis mucosa. Otras entidades demostradas fueron la paracoccidioidomicosis (3, histoplasmosis (2, rinosporidiosis (2. esporotricosis (l, tuberculosis bucal (3, lepra leprornatosa (l, escleroma nasal (2, granulomatosis de Wegener (2, linfomas angiocéntricos (4, aspiración crónica de cocaína (l, y carcinoma escamocelular palatino (1. La biopsia, luego de la historia clínica, es el pilar esencial para identificar y manejar adecuadamente estas lesiones, que pueden originar destrucción facial grave, a veces letal, si no se dispone del diagnóstico preciso y del tratamiento oportuno.

Meta-Analysis of Associations Between Interleukin-10 Polymorphisms and Susceptibility to Vasculitis.

Science.gov (United States)

Jung, Jae Hyun; Song, Gwan Gyu; Lee, Young Ho

2015-01-01

This study determined whether interleukin-10 (IL-10) polymorphisms are associated with susceptibility to vasculitis. A meta-analysis was conducted of the associations between the IL-10 -1082 G/A, -819 C/T, and -592 C/A polymorphisms and the haplotype of the IL-10-1082 G/A, -819 C/T, -592 C/A polymorphisms and vasculitis. A total of 21 comparative studies involving 4121 patients and 5504 controls were considered in the meta-analysis. Meta-analysis revealed no association between the IL-10-1082 G allele and vasculitis in all study subjects (OR = 0.927, 95% CI = 0.780-1.102, p = 0.389). However, disease-specific meta-analysis showed an association between Wegener's granulomatosis (WG) and the IL-10-1082 G allele (OR = 0.729, 95% CI = 0.547-0.971, p = 0.031). Meta-analysis revealed an association between vasculitis and the IL-10-819 C allele (OR = 0.804, 95% CI = 0.706-0.916, p = 0.001) in all study subjects and Behcet's disease (BD) (OR = 0.724, 95% CI = 0.679-0.781, p vasculitis in all study subjects (OR = 0.805, 95% CI = 0.619-0.938, p = 0.005) and BD (OR = 0.718, 95% CI = 0.661-0.781, p vasculitis in Europeans (OR = 1.239, 95% CI = 1.105-1.513, p = 0.035). This meta-analysis showed that IL-10 polymorphisms are associated with vasculitis susceptibility, especially in WG and BD.

Flexible bronchoscopic management of benign tracheal stenosis: long term follow-up of 115 patients

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Fox Benjamin D

2010-01-01

Full Text Available Abstract Background Management of benign tracheal stenosis (BTS varies with the type and extent of the disease and influenced by the patient's age and general health status, hence we sought to investigate the long-term outcome of patients with BTS that underwent minimally invasive bronchoscopic treatment. Methods Patients with symptomatic BTS were treated with flexible bronchoscopy therapeutic modalities that included the following: balloon dilatation, laser photo-resection, self-expanding metal stent placement, and High-dose rate endobronchial brachytherapy used in cases of refractory stent-related granulation tissue formation. Results A total of 115 patients with BTS and various cardiac and respiratory co-morbidities with a mean age of 61 (range 40-88 were treated between January 2001 and January 2009. The underlining etiologies for BTS were post - endotracheal intubation (N = 76 post-tracheostomy (N = 30, Wegener's granulomatosis (N = 2, sarcoidosis (N = 2, amyloidosis (N = 2 and idiopathic BTS (N = 3. The modalities used were: balloon dilatation and laser treatment (N = 98. Stent was placed in 33 patients of whom 28 also underwent brachytherapy. Complications were minor and mostly included granulation tissue formation. The overall success rate was 87%. Over a median follow-up of 51 months (range 10-100 months, 30 patients (26% died, mostly due to exacerbation of their underlying conditions. Conclusions BTS in elderly patients with co-morbidities can be safely and effectively treated by flexible bronchoscopic treatment modalities. The use of HDR brachytherapy to treat granulation tissue formation following successful airway restoration is promising.

Pulmonary lymphoproliferative disorders with affinity to lymphoma: a clinicopathoradiologic study of 16 cases

International Nuclear Information System (INIS)

Fernandez Cruz, J.; Gonzalez Garcia, A.; Escobar Casas, P.; Gomez Benitez, S.; Gonzalez Guirao, M.A.; Borderas, F.

1993-01-01

Pulmonary lymphoproliferative disorders include plasma cell granuloma, Castleman's disease, pseudolymphoma, lymphocytic interstitial pneumonia, angioimmunoblastic lymphadenopathy and lymphomatoid granulomatosis. We carried out a retrospective study for the purpose of analysing the clinical and radiological findings of 16 cases of pulmonary lymphoproliferative disorders seen during the decade 1980-1990. The cases comprised 8 lymphocytic interstitial pneumonia, 5 lymphomatoid granulomatosis, 2 plasma cell granuloma and 1 angioimmunoblastic lymphadenopathy. Owing to the overlap and low specificity of the radiological patterns in these processes, histopathological examination is required. In view of the frequent evolution of pulmonary lymphoproliferative disorders to malignant lymphoma (4 cases, 1 of lymphocytic interstitial pneumonia and 3 of lymphomatoid granulomatosis, in our series) we provide a description of the radiological changes that occur during this process. (orig.)

Think twice

DEFF Research Database (Denmark)

Bang, Cæcilie Larsen; Porsbjerg, Celeste Michala

2016-01-01

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

The Clinical Course of Late Diagnosed Fatal Cases of A (H1N1 Influenza in Poland 

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Marta Rorat

2013-06-01

Full Text Available Introduction: The most frequent complication of A (H1N1 influenza and the leading cause of death was pneumonia with a primary viral or mixed viral and bacterial etiology. 182 patients had died because of a pandemic influenza in Poland by 31st July 2010.Material and Methods: A retrospective study of 6 fatal cases of pandemic influenza, aged 23-41, including 3 women, hospitalised between November 2009 and February 2011 in different Polish medical centres.Results: We present the clinical course of 6 late diagnosed cases of A (H1N1 influenza. All patients presented typical flu-like symptoms in the beginning. 4/6 patients had severe disease risk factors: pregnancy, arthritis, Wegener granulomatosis and obesity. All patients were seen by doctors, no one had received antiviral therapy, 4/5 were treated with antibiotics before they were hospitalized. One patient had nosocomial infection. Patients were admitted to the hospital on the 3rd to 8th day of the disease. They received oseltamivir treatment on the 4th to 9th day. All patients developed pneumonia complicated by acute respiratory distress syndrome. Death appeared between the 4th and 27th day after the onset of symptoms. Autopsies were performed in 5 cases and revealed haemorrhagic pneumonia in 2 patients.Conclusion: Delayed diagnosis and antiviral treatment initiation has a significant impact on mortality in A (H1N1 influenza. During the influenza epidemic, patients presenting typical symptoms should always be suspected of having influenza. Antiviral treatment has to be initiated immediately, especially ifthere are risk factors of severe disease.

Different responses to treatment across classified diseases and severities in Japanese patients with microscopic polyangiitis and granulomatosis with polyangiitis: a nationwide prospective inception cohort study.

Science.gov (United States)

Sada, Ken-ei; Yamamura, Masahiro; Harigai, Masayoshi; Fujii, Takao; Takasaki, Yoshinari; Amano, Koichi; Fujimoto, Shouichi; Muso, Eri; Murakawa, Yohko; Arimura, Yoshihiro; Makino, Hirofumi

2015-11-02

This study aims to elucidate the prognosis and the effectiveness of current treatments for Japanese patients with microscopic polyangiitis (MPA) and granulomatosis with polyangiitis (GPA). Patients with newly diagnosed MPA and GPA were enrolled in a nationwide, prospective, inception cohort study from 22 tertiary Japanese institutions, and treatment patterns and responses were evaluated for 24 months. Primary outcome measures were rates of remission (Birmingham Vasculitis Activity Score, 0) and remission with low-dose glucocorticoids (GC) (prednisolone ≤ 10 mg) (GC remission). Of 156 enrolled patients, 78 MPA patients and 33 GPA patients were included. Concomitant cyclophosphamide (CY) was used in 24 MPA (31 %) and 20 GPA (60 %) patients during the initial 3 weeks of treatment. After 6 months, remission was achieved in 66 MPA (85 %) and 29 GPA (87 %) patients, while GC remission was obtained in only 31 MPA (40 %) and 13 GPA (39 %) patients. During the 24-month period, 14 MPA patients and 2 GPA patients died; end stage renal disease (ESRD) was noted in 13 MPA patients but no GPA patients. Patients with severe disease, according to the European Vasculitis Study Group (EUVAS) classification, showed poorer ESRD-free and overall survival rates than those with generalized disease (p < 0.0001). There were no differences in relapse-free survival rates between GPA and MPA, among EUVAS-defined disease severity categories, and between anti-neutrophil cytoplasmic antibody subspecialties. The majority of Japanese patients with MPA and GPA received treatment with high-dose GC and limited CY use, and showed high remission and relapse-free survival rates but low GC remission rates in clinical practice. University Hospital Medical Information Network Clinical Trials Registry UMIN000001648 . Registered 28 February 2009.

La modélisation de la face terrestre. Sur l'histoire des stratégies de modélisation de la dérive des continents

OpenAIRE

Grevsmühl, Sebastian

2008-01-01

International audience; Korrelierte Kontinente sind nicht die Erfindung Alfred Wegeners, wenngleich er dem Modell in »Die Entstehung der Kontinente« den Status einer Ikone der Moderne bereitet hat. Der Diskurs um die Korrelation beginnt schon im 16. Jahrhundert mit den ersten kartografischen Einschreibungen der kontinentalen Küstenlinien. Doch mit Wegeners umfassender »Verschiebungstheorie« erlangt die Figur der visuellen Korrelation eine erste, wenngleich umstrittene wissenschaftliche Fundie...

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

Science.gov (United States)

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

2018-01-01

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

Geschwisterbeziehungen in der Verarbeitung sexueller Traumatisierung: Der Film "Das Fest"

OpenAIRE

Sohni, Hans

2001-01-01

Filme reizen als Gegenstand psychoanalytischer Kunstinterpretation durch ihre strukturelle Nähe zum "szenischen Verstehen" und bereichern unsere Einsicht in menschliche Beziehungen. Der Film "Das Fest" lohnt wegen seiner besonderen ästhetischen Form und wegen seiner Aussage zur Geschwisterbeziehung. Nach filmjournalistischer Einschätzung zeigt er die Demontage der bürgerlichen Gesellschaft und die Reinszenierung destruktiver Gewalt. In der Interpretation dieses Beitrags führt der Film dagegen...

Granulomatosis with polyangiitis

Science.gov (United States)

... ncbi.nlm.nih.gov/pubmed/21372195 . Specks U, Merkel PA, Seo P, et al. Efficacy of remission- ... PA: Elsevier Saunders; 2016:chap 270. Stone JH, Merkel PA, Spiera R, et al. Rituximab versus cyclophosphamide ...

Neue Komponenten für das generative Fertigungsverfahren des 3D-Drucks

OpenAIRE

Presser, Jonas

2012-01-01

Kunststoffprodukte haben sich seit den fünfziger Jahren wegen ihrer Zähigkeit und ihres geringen Gewichts, aber auch wegen ihrer Verarbeitbarkeit durch das Schmel-ze¬verfahren des Spritzgusses durchgesetzt. Die Formenvielfalt und Akkuratesse, die der Spritzguss in schneller und preiswerter Produktion erlaubt, ist unerreicht. Aller¬dings muss dem Spritzguss immer der Bau eines Formwerkzeuges vorgeschaltet werden. Dessen Kosten amortisieren sich in der Großserienproduktion, nicht aber bei Klein...

The risk for cancer and genetic abnormalities after radioiodine treatment of hyperthyroidism; Zum Krebs- und genetischen Risiko nach Radioiodtherapie der Hyperthyreose

Energy Technology Data Exchange (ETDEWEB)

Reiners, C. [Wuerzburg Univ. (Germany). Klinik und Poliklinik fuer Nuklearmedizin

1997-12-01

According to recent studies, the risk for thyroid cancer is not increased after radioiodine treatment in patients with hyperthyroidism. Only the risk of cancer of the stomach seems to be increased slightly in patents treated with I-131 because of functional autonomy. However, the risk for gastric cancer is not increased after higher activities of I-131 because of thyroid cancer. There is no increased risk for genetic abnormalities after radioiodine treatment of hyperthyroidism. (orig.) [Deutsch] Aktuelle Studien zum Karzinomrisiko nach Radioiodtherapie wegen Hyperthyreose ergeben keinen Anhalt fuer eine erhoehte Inzidenz des Schilddruesenkarzinoms nach therapeutischer Gabe von I-131. Allenfalls scheint die Inzidenz von Magenkarzinomen bei Patienten nach Radioiodtherapie wegen funktioneller Autonomie geringfuegig erhoeht zu sein, obwohl diese Beobachtung bei Patienten nach hochdosierter Radioiodtherapie wegen eines Schilddruesenkarzinoms nicht gemacht wurde. Hinweise fuer ein erhoehtes genetisches Risiko nach Radioiodtherapie der Hyperthyreose ergeben sich nicht. (orig.)

Leukocyte scintigraphy with 99mTc-exametazime-labeled leukocytes is not useful for follow-up of systemic vasculitis

International Nuclear Information System (INIS)

Staudenherz, A.; Kletter, K.; Deicher, R.; Haas, M.; Hoerl, W.H.; Jilma, B.; Becherer, A.; Dudczak, R.

2002-01-01

Background: The prognosis of systemic vasculitis, for instance Wegener's granulomatosis (WG), was greatly improved by the introduction of immunosuppressive treatment. However, relapses are frequent and predictors are scarce. 111 In-leukocytes have been found to indicate unknown manifestations of WG and to predict later relapse. We prospectively investigated the value of 99m Tc-Exametazime ( 99m Tc-HMPAO)-labeled leukocytes with regard to specific patterns and for their usefulness in the follow-up of patients with WG. Methods: The vasculitis group consisted of 8 patients with WG and 2 with idiopathic necrotizing glomerulonephritis (ING). Seven patients with different inflammatory diseases served as controls. Leukocyte labeling with 99m Tc-HMPAO was done using a slightly modified Hammersmith protocol. Cell viability after labeling was verified in vivo by the exclusion of early lung and splenic uptake and in vitro by means of propidium iodide and FACS analysis. Static gamma camera images from the head, chest, abdomen, and pelvis were obtained up to 18 hours after injection of approximately 300 MBq 99m Tc-HMPAO-labeled leukocytes. Scintigrams were analyzed visually; for semiquantitative analysis ROIs were drawn over the nasal region, the right lung, kidneys, and liver. Results: Increased nasal leukocyte accumulation was found in 7/8 patients with WG and in 2/2 patients with ING. Of 2 patients who had a relapse 6 months later, one presented with, and one without nasal uptake. The kidney/liver ratio was higher in controls (0.24 ± 0.07 vs. 0.37 ± 0.11, p 99m Tc-HMPAO leukocyte scintigraphy failed to indicate or exclude a later relapse and is therefore not suitable as a diagnostic tool in the management of patients with systemic vasculitis. (author)

Leukocyte scintigraphy with 99mTc-exametazime-labeled leukocytes is not useful for follow-up of systemic vasculitis

International Nuclear Information System (INIS)

Becherer, A.; Dudczak, R.; Deicher, R.; Haas, M.; Hoerl, W.H.; Jilma, B.; Staudenherz, A.; Kletter, K.

2002-01-01

The prognosis of systemic vasculitis, for instance Wegener's granulomatosis (WG), was greatly improved by the introduction of immunosuppressive treatment. However, relapses are frequent and predictors are scarce. 111 In-leukocytes have been found to indicate unknown manifestations of WG and to predict later relapse. We prospectively investigated the value of 99m Tc-Exametazime ( 99m Tc-HMPAO)-labeled leukocytes with regard to specific patterns and for their usefulness in the follow-up of patients with WG. The vasculitis group consisted of 8 patients with WG and 2 with idiopathic necrotizing glomerulonephritis (ING). Seven patients with different inflammatory diseases served as controls. Leukocyte labeling with 99m Tc-HMPAO was done using a slightly modified Hammersmith protocol. Cell viability after labeling was verified in vivo by the exclusion of early lung and splenic uptake and in vitro by means of propidium iodide and FACS analysis. Static gamma camera images from the head, chest, abdomen, and pelvis were obtained up to 18 hours after injection of approximately 300 MBq 99m Tc-HMPAO-labeled leukocytes. Scintigrams were analyzed visually; for semiquantitative analysis ROls were drawn over the nasal region, the right lung, kidneys, and liver. Increased nasal leukocyte accumulation was found in 7/8 patients with WG and in 2/2 patients with ING. Of 2 patients who had a relapse 6 months later, one presented with, and one without nasal uptake. The kidney/liver ratio was higher in controls (0.24 ± 0.07 vs. 0.37 ± 0.11, p 99m Tc-HMPAO leukocyte scintigraphy failed to indicate or exclude a later relapse and is therefore not suitable as a diagnostic tool in the management of patients with systemic vasculitis. (author)

Effect of intensive plasma exchange (PE) in rapidly progressive crescentic glomerulonephritis (RPCGN).

Science.gov (United States)

D'Amico, G; Sinico, R; Fornasieri, A; Ferrario, F; Colasanti, G; Porri, M T; Paracchini, M L; Gibelli, A

1983-07-01

Ten adult patients with RPCGN (crescents in greater than 70% of glomeruli), primary in 6 and associated with systemic diseases in 4, were treated with PE, associated with oral steroids (P) and cyclophosphamide (C) in all cases and with intravenous methylprednisolone pulses (MP) in 7 cases. Four out of ten patients were anuric and needed dialysis treatment at the start of treatment. Therapeutic benefit, i.e. reversal of the trend to further deterioration and substantial improvement of GFR, was achieved in 8 out of 10 patients (80%), including 2 of 4 anuric patients, and in 7 of those (8) who had still active cellular crescents (87.5%). Similar therapeutic benefit had been achieved only in 10% of a comparable population of 10 patients with RPCGN treated before 1980 with P and C, without PE or MP pulses. It is difficult to establish whether the better therapeutic results in the more recently treated group were due to PE or to MP pulses of to both the new approaches, even though the clinical improvement obtained in all the 3 patients treated with PE without concomitant MP suggest a specific beneficial role for PE. RPCGN is a catastrophic illness characterized by progressive deterioration of kidney function, resulting in oliguria and uremia, usually within weeks or months. The most consistent histopathologic finding is the presence of extensive glomerular crescents resulting from proliferation of the extracapillary epithelial cell lining of Bowman's capsule. It is apparent that RPCGN is not a homogeneous entity, clinically, histologically or immunohistologically, but rather a clinicopathologic syndrome, the features of which may be seen in a variety of systemic disorders, including SLE, polyarteritis nodosa, Wegener's granulomatosis, Henoch-Schönlein purpura, cryoglobulinemia, and subacute bacterial endocarditis.(ABSTRACT TRUNCATED AT 250 WORDS)

Vasculitis asociadas a anticuerpos anti-citoplasma de neutrófilos: Clínica y tratamiento

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María Virginia Paolini

2013-04-01

Full Text Available Las vasculitis asociadas a anticuerpos anti-citoplasma de neutrófilos (ANCA comprenden a un grupo de enfermedades caracterizadas por la inflamación de la pared de pequeños vasos. Analizamos las características epidemiológicas y clínicas en una serie de 47 pacientes: 23 (49% granulomatosis de Wegener (GW, 15 (32% poliangeítis microscópica (PAM y nueve (19% vasculitis limitada al riñón (VLR. La edad media al inicio de los síntomas fue de 50.7 ± 14.9 años. La manifestación clínica más frecuente fue el compromiso renal en 41 (87% pacientes, seguido por el pulmonar en 26 (55% y el otorrinolaringológico en 17 (36%. En 26 (55% se asoció compromiso renal y pulmonar. La forma clínica más frecuente fue la generalizada en 23 (49%, seguida por la grave en 18 (38%. El 89% presentaron determinaciones de ANCA positivas. Cuatro (8% no recibieron tratamiento inmunosupresor de inicio. De los 43 que recibieron tratamiento de inicio, 29 (67% tuvieron remisión completa, con un tiempo de remisión promedio de 35.3 meses. Once (26% presentaron recaídas, diez (91% recaídas mayores y uno (9% menor. Doce (28% fallecieron, siete en forma temprana y cinco durante la evolución de la enfermedad. Quince (31% evolucionaron a insuficiencia renal crónica. Los 26 pacientes en seguimiento tuvieron respuesta al tratamiento y 20 (77% de ellos estaban en remisión al finalizar el estudio. Las vasculitis asociadas a ANCA continúan siendo enfermedades de alta morbilidad y mortalidad, a pesar de las mejorías logradas con los tratamientos inmunosupresores.

Pattern of mri brain abnormalities in rheumatic patients with neurological involvement: a tertiary care teaching hospital experience

International Nuclear Information System (INIS)

Parvez, K.; Arfaj, A.; Naseeb, F.; Daif, A.K.

2015-01-01

Objective: To explore the pattern of abnormalities seen on MRI in rheumatic patients with neurological manifestations and to interpret the findings in relation to clinical picture. Study Design: Descriptive study. Place and Duration of Study: Rheumatology unit, King Khalid University Hospital, Riyadh, Saudi Arabia from January 2013 to February 2014. Patients and Methods: We prospectively included rheumatic patients with neurological symptoms and signs. The clinical data were correlated with MRI findings by a team comprising of a rheumatologist, neurologist and neuro-radiologist. Data was analyzed using simple statistical analysis. Results: Fifty patients were recruited with a mean age of 36.4 ± 10.76 years (range 17-62). Among SLE patients with seizures, focal deficit and headache white matter hyperintensities were found in 9 (64.28%), 4 (50%), 4 (80%) patients respectively. Out of seven SLE patients with global dysfunction, 3 (42.85%) had brain atrophy and 2 (28.57%) normal MRI. In Behcet disease with focal deficit, 3 (75%) patients had white matter hyperintensities and 1 (25%) had brainstem involvement. In Behcet disease with headache, 2 (50%) had normal MRI, 1 (25%) brainstem hyper-intensities and 1 (25%) had subacute infarct. Two (66%) of three Primary APS patients had white matter hyperintensities while third (33%) had old infarct. Both patients of polyarteritisnodosa, had white matter hyperintensities. Out of two Wegener granulomatosis one had white matter hyperintensities and other had ischemic changes in optic nerves. The only one scleroderma patient had white matter hyperintensities. Conclusion: We found that white matter hyperintensities was the most common MRI abnormality in our study group which in most of the cases had poor clinical correlation. No distinct pattern of CNS involvement on MRI was observed in various rheumatic disorders. (author)

Pembrolizumab reactivates pulmonary granulomatosis

Directory of Open Access Journals (Sweden)

Majdi Al-dliw

2017-01-01

Full Text Available Sarcoid like reaction is a well-known entity that occurs as a consequence to several malignancies or their therapies. Immunotherapy has gained a lot of interest in the past few years and has recently gained approval as first line therapy in multiple advanced stage malignancies. Pneumonitis has been described as complication of such therapy. Granulomatous inflammation has been only rarely reported subsequent to immunotherapy. We describe a case of granulomatous inflammation reactivation affecting the lungs in a patient previously exposed to Pembrolizumab and have evidence of a distant granulomatous infection. We discuss potential mechanisms of the inflammation and assert the importance of immunosuppression in controlling the dis-inhibited immune system.

Abnormal reticuloendothelial function in patients with active vasculitis and idiopathic membranous glomerulopathy

Energy Technology Data Exchange (ETDEWEB)

van der Woude, F J; Piers, D A; van der Giessen, M; Hoedemaeker, P J; Hauw The, T; van der Hem, G K

1983-02-01

Reticuloendothelial function was assessed in 11 patients with systemic lupus erythematosus, 8 patients with Wegener's granulomatosus, and 20 patients with idiopathic membranous sup(99m)Tc-labeled heat-damaged red blood cells. With this method organ uptake could be measured by quantitative scintigraphy. There was no relation between the Tsub(1/2) of the blood disappearance curve and the Tsub(1/2) of the splenic uptake curve. The Tsub(1/2) of the blood disappearance curve was normal in all the patient groups. However, there was a significant shift from spleen to liver uptake in patients with active systemic lupus erythematosus, active Wegener's granulomatosus, and membranous glomerulopathy in comparison with a control group. There was no relation with age, level of circulating immune complexes, complement level, kidney function, or immunosuppressive treatment. We conclude that an increase of the liver component of reticuloendothelial function may compensate abnormalities in splenic function. This stresses the importance of quantitative scanning to detect such abnormalities. The study provides evidence for disease related hyposplenism in patients with active systemical lupus erythematosus, active Wegener's granulamatosus, and membranous glomerulopathy.

Abnormal reticuloendothelial function in patients with active vasculitis and idiopathic membranous glomerulopathy

International Nuclear Information System (INIS)

Woude, F.J. van der; Piers, D.A.; Giessen, M. van der; Hoedemaeker, P.J.; Hauw The, T.; Hem, G.K. van der; Rijksuniversiteit Groningen; Rijksuniversiteit Groningen

1983-01-01

Reticuloendothelial function was assessed in 11 patients with systemic lupus erythematosus, 8 patients with Wegener's granulomatosus, and 20 patients with idiopathic membranous sup(99m)Tc-labeled heat-damaged red blood cells. With this method organ uptake could be measured by quantitative scintigraphy. There was no relation between the Tsub(1/2) of the blood disappearance curve and the Tsub(1/2) of the splenic uptake curve. The Tsub(1/2) of the blood disappearance curve was normal in all the patient groups. However, there was a significant shift from spleen to liver uptake in patients with active systemic lupus erythematosus, active Wegener's granulomatosus, and membranous glomerulopathy in comparison with a control group. There was no relation with age, level of circulating immune complexes, complement level, kidney function, or immunosuppressive treatment. We conclude that an increase of the liver component of reticuloen-dothelial function may compensate abnormalities in splenic function. This stresses the importance of quantitative scanning to detect such abnormalities. The study provides evidence for disaase related hyposplenism in patients with active systemical lupus erythematosus, active Wegener's granulamatosus, and membranous glomerulopathy. (orig.)

Yellow Fever Vaccine in Patients With Rheumatic Diseases

Science.gov (United States)

2018-04-05

Systemic Lupus; Rheumatoid Arthritis; Spondyloarthritis; Inflammatory Myopathy; Systemic Sclerosis; Mixed Connective Tissue Disease; Takayasu Arteritis; Granulomatosis With Polyangiitis; Sjogren's Syndrome; Juvenile Idiopathic Arthritis; Juvenile Dermatomyositis

Genetically Distinct Subsets within ANCA-Associated Vasculitis

Science.gov (United States)

Lyons, Paul A.; Rayner, Tim F.; Trivedi, Sapna; Holle, Julia U.; Watts, Richard A.; Jayne, David R.W.; Baslund, Bo; Brenchley, Paul; Bruchfeld, Annette; Chaudhry, Afzal N.; Tervaert, Jan Willem Cohen; Deloukas, Panos; Feighery, Conleth; Gross, Wolfgang L.; Guillevin, Loic; Gunnarsson, Iva; P, Lorraine Harper M.R.C; Hrušková, Zdenka; Little, Mark A.; Martorana, Davide; Neumann, Thomas; Ohlsson, Sophie; Padmanabhan, Sandosh; Pusey, Charles D.; Salama, Alan D.; Sanders, Jan-Stephan F.; Savage, Caroline O.; Segelmark, Mårten; Stegeman, Coen A.; Tesař, Vladimir; Vaglio, Augusto; Wieczorek, Stefan; Wilde, Benjamin; Zwerina, Jochen; Rees, Andrew J.; Clayton, David G.; Smith, Kenneth G.C.

2013-01-01

BACKGROUND Antineutrophil cytoplasmic antibody (ANCA)–associated vasculitis is a severe condition encompassing two major syndromes: granulomatosis with polyangiitis (formerly known as Wegener’s granulomatosis) and microscopic polyangiitis. Its cause is unknown, and there is debate about whether it is a single disease entity and what role ANCA plays in its pathogenesis. We investigated its genetic basis. METHODS A genomewide association study was performed in a discovery cohort of 1233 U.K. patients with ANCA-associated vasculitis and 5884 controls and was replicated in 1454 Northern European case patients and 1666 controls. Quality control, population stratification, and statistical analyses were performed according to standard criteria. RESULTS We found both major-histocompatibility-complex (MHC) and non-MHC associations with ANCA-associated vasculitis and also that granulomatosis with polyangiitis and microscopic polyangiitis were genetically distinct. The strongest genetic associations were with the antigenic specificity of ANCA, not with the clinical syndrome. Anti–proteinase 3 ANCA was associated with HLA-DP and the genes encoding α1-antitrypsin (SERPINA1) and proteinase 3 (PRTN3) (P = 6.2×10−89, P = 5.6×10−12, and P = 2.6×10−7, respectively). Anti–myeloperoxidase ANCA was associated with HLA-DQ (P = 2.1×10−8). CONCLUSIONS This study confirms that the pathogenesis of ANCA-associated vasculitis has a genetic component, shows genetic distinctions between granulomatosis with polyangiitis and microscopic polyangiitis that are associated with ANCA specificity, and suggests that the response against the autoantigen proteinase 3 is a central pathogenic feature of proteinase 3 ANCA–associated vasculitis. These data provide preliminary support for the concept that proteinase 3 ANCA–associated vasculitis and myeloperoxidase ANCA–associated vasculitis are distinct autoimmune syndromes. (Funded by the British Heart Foundation and others.) PMID

MDX-010 in Treating Patients With Recurrent or Refractory Lymphoma

Science.gov (United States)

2014-05-22

Adult Grade III Lymphomatoid Granulomatosis; B-cell Chronic Lymphocytic Leukemia; Cutaneous B-cell Non-Hodgkin Lymphoma; Extranodal Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue; Intraocular Lymphoma; Nodal Marginal Zone B-cell Lymphoma; Recurrent Adult Burkitt Lymphoma; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Adult Diffuse Mixed Cell Lymphoma; Recurrent Adult Diffuse Small Cleaved Cell Lymphoma; Recurrent Adult Grade III Lymphomatoid Granulomatosis; Recurrent Adult Hodgkin Lymphoma; Recurrent Adult Immunoblastic Large Cell Lymphoma; Recurrent Adult Lymphoblastic Lymphoma; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Mantle Cell Lymphoma; Recurrent Marginal Zone Lymphoma; Refractory Hairy Cell Leukemia; Small Intestine Lymphoma; Splenic Marginal Zone Lymphoma; Testicular Lymphoma; Waldenström Macroglobulinemia

Genetically Modified T-cell Infusion Following Peripheral Blood Stem Cell Transplant in Treating Patients With Recurrent or High-Risk Non-Hodgkin Lymphoma

Science.gov (United States)

2018-01-26

Adult Grade III Lymphomatoid Granulomatosis; Cutaneous B-cell Non-Hodgkin Lymphoma; Extranodal Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue; Intraocular Lymphoma; Nodal Marginal Zone B-cell Lymphoma; Post-transplant Lymphoproliferative Disorder; Recurrent Adult Burkitt Lymphoma; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Adult Diffuse Mixed Cell Lymphoma; Recurrent Adult Diffuse Small Cleaved Cell Lymphoma; Recurrent Adult Grade III Lymphomatoid Granulomatosis; Recurrent Adult Immunoblastic Large Cell Lymphoma; Recurrent Adult Lymphoblastic Lymphoma; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Grade 3 Follicular Lymphoma; Recurrent Mantle Cell Lymphoma; Recurrent Marginal Zone Lymphoma; Recurrent Small Lymphocytic Lymphoma; Refractory Hairy Cell Leukemia; Small Intestine Lymphoma; Splenic Marginal Zone Lymphoma; Testicular Lymphoma; Waldenström Macroglobulinemia

Disease: H01688 [KEGG MEDICUS

Lifescience Database Archive (English)

Full Text Available systemic small vessel vasculitis such as granulomatosis with polyangiitis (GPA)....lectron microscopy. Approximately 90% of patients with PICG have circulating ANCA antibodies, leading to the

[Antineutrophil cytoplasmic antibodies associated with infective endocarditis: Literature review].

Science.gov (United States)

Langlois, V; Marie, I

2017-07-01

Antineutrophil cytoplasmic antibodies (ANCA) associated with infective endocarditis are a rare disorder. The condition can mimic primary systemic vasculitis (i.e. granulomatosis with polyangiitis, microscopic polyangiitis, and eosinophilic granulomatosis with polyangiitis). Thus, a wrong diagnosis of valvular involvement related to primary systemic vasculitis can be made in patients exhibiting ANCA associated with infective endocarditis. Because treatment of both conditions is different, this wrong diagnosis will lead to dramatic consequences in these latter patients. This review reports the state of knowledge and proposes an algorithm to follow when confronted to a possible case of ANCA associated with infective endocarditis. Copyright © 2016 Société Nationale Française de Médecine Interne (SNFMI). Published by Elsevier SAS. All rights reserved.

Human alpha-enolase from endothelial cells as a target antigen of anti-endothelial cell antibody in Behçet's disease.

Science.gov (United States)

Lee, Kwang Hoon; Chung, Hae-Shin; Kim, Hyoung Sup; Oh, Sang-Ho; Ha, Moon-Kyung; Baik, Ja-Hyun; Lee, Sungnack; Bang, Dongsik

2003-07-01

To identify and recombine a protein of the human dermal microvascular endothelial cell (HDMEC) that specifically reacts with anti-endothelial cell antibody (AECA) in the serum of patients with Behçet's disease (BD), and to evaluate the usefulness of this protein in BD. The proteomics technique, with 2-dimensional gel electrophoresis and matrix-assisted laser desorption ionization-time-of-flight (MALDI-TOF) mass spectrometry, was used to identify and recombine HDMEC antigen. Western blotting and enzyme-linked immunosorbent assay (ELISA) of recombinant protein isolated by gene cloning were performed on serum from healthy controls, patients with BD, and patients with other rheumatic diseases (rheumatoid arthritis, systemic lupus erythematosus, and Wegener's granulomatosis). Eighteen of 40 BD patients had serum IgM antibody to HDMEC antigen. The purified protein that reacted with AECA in BD patient sera was found to be alpha-enolase by 2-dimensional gel electrophoresis followed by immunoblotting and MALDI-TOF mass spectrometry. Recombinant alpha-enolase protein was isolated and refined by gene cloning. On Western blots, AECA-positive IgM from the sera of patients with active BD reacted strongly with recombinant human alpha-enolase. BD patient sera positive for anti-alpha-enolase did not react with human gamma-enolase. On dot-blotting, reactivity to human alpha-enolase was detected only in the IgM-positive group. Fifteen of the 18 AECA-positive sera that were positive for the HDMEC antigen showed reactivity to recombinant alpha-enolase IgM antibody by ELISA. The alpha-enolase protein is the target protein of serum AECA in BD patients. This is the first report of the presence of IgM antibodies to alpha-enolase in endothelial cells from the serum of BD patients. Although further studies relating this protein to the pathogenesis of BD will be necessary, alpha-enolase and its antibody may prove useful in the development of new diagnostic and treatment modalities in BD.

The origin of continents and oceans

National Research Council Canada - National Science Library

Wegener, Alfred 1880-1930; Biram, John

1966-01-01

.... Wegener proposed that in the remote past the earth's continents were not separate (as now), but formed one supercontinent which later split apart, the fragments gradually drifting away from one another...

Takayasu's Arteritis

Science.gov (United States)

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

Comparability of patients with ANCA-associated vasculitis enrolled in clinical trials or in observational cohorts

NARCIS (Netherlands)

Pagnoux, C.; Carette, S.; Khalidi, N. A.; Walsh, M.; Hiemstra, T. F.; Cuthbertson, D.; Langford, C.; Hoffman, G.; Koening, C. L.; Monach, P. A.; Moreland, L.; Mouthon, L.; Seo, P.; Specks, U.; Ytterbere, S.; Westman, K.; Hoglund, P.; Harper, L.; Flossmann, O.; Luqmani, R.; Savage, C.; Rasmussen, N.; de Groot, K.; Tesar, V.; Jayne, D.; Merkel, P. A.; Guillevin, L.; Stegeman, C. A.

2015-01-01

Objective. To analyse the differences between patients with granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA) entered into randomised clinical trials (RCTs) and those followed in large observational cohorts. Methods. The main characteristics and outcomes of patients with

RENAL VASCULITIS IN 2012 Reclassification and the introduction of biologicals

NARCIS (Netherlands)

Kallenberg, Cees G. M.

2012 saw the classification of the systemic vasculitides revised. Genetic studies showed that granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA) are different diseases with aberrant immune responses to different autoantigens. B-cell depletion with rituximab also acquired a

Roberto Mantovani (1854-1933 and his ideas on the expanding Earth, as revealed by his correspondence and manuscripts

Directory of Open Access Journals (Sweden)

Giancarlo Scalera

2009-06-01

Full Text Available

Roberto Mantovani (Parma, 25 March 1854-Paris, 10 January 1933 – a musician and geologist trained in the Duchy of Parma – was a Consul in the French island of La Réunion at the end of the nineteenth century. In 1878 drawing general conclusions from the similarity in shape between the facing banks of a river that flowed in a volcanic fracture, and the way in which the layers corresponded, Mantovani formulated a mobilistic theory, attributing the moving apart of the continents to the expansion of the entire planet. This theory is more general than that of Wegener from the first decades of the following century. Mantovani’s hypothesis was officially recognised by the French Geological Society in 1924, which incorporated it in its body of legitimate ideas. Encouraged by Bourcart in 1924, Wegener quoted the Italian in his famous book as one who offered ideas extraordinarily close to his own. A letter of Mantovani to Wegener, and the sceptical answer of the German scientist, have been recently found. In his letter Mantovani shows a greater awareness of the predecessors of the continental drift than Wegener

himself. Mantovani continued to disseminate his idea up to the last years of his life. A final pamphlet, of 1930, was printed with this dedication: «to the mathematicians, physicists, astronomers, geologists, and anyone interested in the great enigmas of the Universe». is biography has been reconstructed thanks to the correspondences carefully conserved in the archives of the Italian Ministry of Foreign Affairs, the Ethnographic Museum Luigi Pigorini, the Società Geografica Italiana, and in the private files of his direct descendants.

David Kolb: manieren van leren in verschillende discipline

NARCIS (Netherlands)

drs. J. (Jan) Kaldeway

2012-01-01

Hoewel er wegens psychometrische bezwaren academische kritiek is op de Kolbtest zijn de ideeën die eraan ten grondslag liggen nog alleszins actueel. In deze bijdrage worden die achterliggende ideeën besproken.

Detection of antineutrophil cytoplasmic antibodies (ANCAs)

DEFF Research Database (Denmark)

Damoiseaux, Jan; Csernok, Elena; Rasmussen, Niels

2017-01-01

of diagnosis) from 251 patients with ANCA-associated vasculitis (AAV), including granulomatosis with polyangiitis and microscopic polyangiitis, and from 924 disease controls were tested for the presence of cytoplasmic pattern/perinuclear pattern and atypical ANCA (A-ANCA) by indirect immunofluorescence (IIF...

Premature atherosclerosis in systemic autoimmune diseases

NARCIS (Netherlands)

Leeuw, Karina de

2008-01-01

Systemic autoimmune diseases such as systemic lupus erythematosus (SLE) and Wegener’s granulomatosis (WG) are associated with a significantly increased prevalence of cardiovascular disease (CVD) compared to age- and sex-matched controls. Many risk factors are involved in the pathogenesis of

Histiocytosis X | Mohammed | Annals of African Medicine

African Journals Online (AJOL)

Histiocytosis X (Langerhan's cell granulomatosis) is a disease of unknown aetiology, but viral origin has been implicated.1 It is a clinicopathologic entity characterized by proliferation of Langerhan's cell (which are not primarily phagocytic) in an appropriate milieu.2 ...

Marketing research among small and medium-sized business enterprises - a VDEW field investigation with baker`s shops; Marktforschung bei Gewerbebetrieben - VDEW-Baeckereibefragung

Energy Technology Data Exchange (ETDEWEB)

Fischer, A. [Isar-Amperwerke AG, Muenchen (Germany); Nickel, M. [VDEW-Hauptgeschaeftsstelle, Frankfurt am Main (Germany). Gruppe ``Energiewirtschaft und Statistik``

1998-12-14

The baker`s trade is a business of interest to electric utilities, as bakers have a high demand for process heat and their supply requirements are characterized by simple load histories. Hence competing electric utilities in the competitive market target this customer group with increasing efforts. The authors summarize the information obtained from the field investigation and the conclusions drawn. (orig./CB) [Deutsch] Die Baeckereien sind wegen ihres hohen Prozesswaermebedarfs und des guenstigen Lastverlaufs eine interessante Kundengruppe fuer die Stromversorger. Der Beratung und Betreuung dieser Kunden muss deshalb hohe Aufmerksamkeit gewidmet werden. Nicht zuletzt wegen des Wettbewerbs auf dem Energiemarkt muessen die Stromversorger die Kundenorientierung noch intensivieren. Auch dadurch entstehen neue Anforderungen an die Kundenberatung und -betreuung. Die Verfasser erlaeutern die wichtigsten Ergebnisse und Schlussfolgerungen aus einer Befragung von Baeckereibetrieben. (orig.)

Ocean iron fertilization - Moving forward in a sea of uncertainty

Digital Repository Service at National Institute of Oceanography (India)

Buesseler, K.O.; Doney, S.C.; Karl, D.M.; Boyd, P.W.; Caldeira, K.; Chai, F.; Coale, K.H.; de Baar, H.J.W.; Falkowski, P.G.; Johnson, K.S.; Lampitt, R.S.; Michaels, A.F.; Naqvi, S.W.A.; Smetacek, V.; Takeda, S.; Watson, A.J.

California, Los Angeles, CA, USA. 12National Institute of Oceanography, Goa, India. 13Alfred Wegener Institute for Polar and Marine Research, Bremerhaven, Germany. 14Department of Aquatic Bioscience, University of Tokyo, Tokyo, Japan. 15School...

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

Directory of Open Access Journals (Sweden)

Thais Thomaz Queluz

2005-07-01

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

Genetic loci of Staphylococcus aureus associated with anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitides

NARCIS (Netherlands)

Glasner, Corinna; de Goffau, Marcus C; van Timmeren, Mirjan M; Schulze, Mirja L; Jansen, Benita; Tavakol, Mehri; van Wamel, Willem J B; Stegeman, Coen A; Kallenberg, Cees G M; Arends, Jan P; Rossen, John W; Heeringa, Peter; van Dijl, Jan Maarten

2017-01-01

The proteinase 3 (PR3)-positive anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV) granulomatosis with polyangiitis (GPA) has been associated with chronic nasal S. aureus carriage, which is a risk factor for disease relapse. The present study was aimed at comparing the

Genetic loci of Staphylococcus aureus associated with anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitides

NARCIS (Netherlands)

C. Glasner (Corinna); M.C. De Goffau (Marcus C.); M.M. Van Timmeren (Mirjan M.); Schulze, M.L. (Mirja L.); Jansen, B. (Benita); M. Tavakol (Mehri); W.J.B. van Wamel (Willem); C.A. Stegeman; C.G.M. Kallenberg (Cees G. M.); J.P.A. Arends (Jan); J.W. Rossen (John); P. Heeringa (Peter); J.M. Dijl (Jan Maarten)

2017-01-01

textabstractThe proteinase 3 (PR3)-positive anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV) granulomatosis with polyangiitis (GPA) has been associated with chronic nasal S. aureus carriage, which is a risk factor for disease relapse. The present study was aimed at

Nigerian Quarterly Journal of Hospital Medicine - Vol 13, No 3-4 ...

African Journals Online (AJOL)

Mast Cell Quantification in Orofacial Granulomatosis · EMAIL FULL TEXT EMAIL FULL TEXT · DOWNLOAD FULL TEXT DOWNLOAD FULL TEXT. N N Nwizu, D G Macdonald, 34-39. http://dx.doi.org/10.4314/nqjhm.v13i3-4.12653 ...

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

DEFF Research Database (Denmark)

Bang, Caecilie Larsen; Porsbjerg, Celeste

2016-01-01

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

Follicular bronchiolitis in an HIV-infected individual on combination antiretroviral therapy with low CD4+ cell count but sustained viral suppression

DEFF Research Database (Denmark)

Rasmussen, Line D; Pedersen, Court; Madsen, Helle D

2017-01-01

A 36-year-old Danish man, living in Asia, was diagnosed with Pneumocystis pneumonia (PCP) and HIV in 2013 (CD4+ count: 6 cells/µL; viral load: 518 000 copies/mL). He initiated combination antiretroviral therapy. Later that year, he was also diagnosed with granulomatosis with polyangiitis and was ......A 36-year-old Danish man, living in Asia, was diagnosed with Pneumocystis pneumonia (PCP) and HIV in 2013 (CD4+ count: 6 cells/µL; viral load: 518 000 copies/mL). He initiated combination antiretroviral therapy. Later that year, he was also diagnosed with granulomatosis with polyangiitis...... tests demonstrated severely reduced lung capacity with an obstructive pattern and a moderately reduced diffusion capacity. High resolution computer tomography revealed minor areas with tree-in-bud pattern and no signs of air trapping on expiratory views. Lung biopsy showed lymphocytic infiltration...

The origin of continents and oceans

National Research Council Canada - National Science Library

Wegener, Alfred 1880-1930; Biram, John

1966-01-01

... and Antarctica up through the Indian Ocean, and closing the remaining gaps. Wegener then explained various phenomena in historical geology, geomorphy, paleontology, paleoclimatology, and similar areas of science in terms of this continental drift."--From back cover.

De veranderende taak van de dierenarts in de primaire produktie.

NARCIS (Netherlands)

Noordhuizen, J.P.T.M.; Stark, K.D.C.; Verheijden, J.H.M.; Agger, J.F.

1994-01-01

Enkele probleemgebieden uit de dierlijke produktiesector worden nader belicht. Het doel van dit artikel is aan te geven via welke wegen de dierenarts deze probleemgebieden kan benaderen en tevens wat daarvoor nodig is in onderzoek en opleiding

Beknopte literatuurstudie inzake categorie-indeling van wegen.

NARCIS (Netherlands)

Dijkstra, A. & Twisk, D.A.M.

1992-01-01

This literature study describes the road categorization from the road user point of view. The study describes the following subjects: (1) the mental load of road users; (2) road categories in relation to road safety; (3) a model for the traffic and transport system; (4) recognition of road types;

Sociale media: nieuwe wegen naar sociale innovatie

NARCIS (Netherlands)

Salverda, I.E.; Jagt, van der P.D.; Willemse, R.; Onwezen, M.C.; Top, J.L.

2013-01-01

Hoewel de rol en impact van internet en de sociale media in de samenleving algemeen worden aangenomen, is het nog niet duidelijk of en hoe het communiceren en delen van informatie via internet en de sociale media bijdragen aan het ontstaan van sociale innovatie. Hoofdvragen van deze verkenning zijn

Wegener and his Theory of Continental Drift

Indian Academy of Sciences (India)

principles of physics to investigate the earth, gleefully add that .... climates prevailing at the times of their formation were different from the present ..... An indication ofthis emerges from Gauss's law of electrostatics but it is proved rigorously in.

Kamferintoxicatie na inname van mottenballen 'wegens hoofdpijn'

NARCIS (Netherlands)

Peters, Anna-Linda; Dekker, Eline; Michels, Wieneke M.

2011-01-01

Camphor is a toxic hydrocarbon, found in numerous over-the-counter medicinal products and chemist-shop items. The consequences of camphor poisoning depend on the dose, and severe poisoning can result in death. Ingestion of camphor can cause seizures, apnoea, renal insufficiency, raised hepatic

Doelmatigheid van TENS-therapie bij subacute lumbosacrale radiculaire pijn (≤ 6 maanden) in vergelijking met een transforaminale epidurale injectie

NARCIS (Netherlands)

Dr. J.H. Groenewoud; E.E. Vegt; K.D. Meijers-Verhoeven

2015-01-01

Introductie Transcutane Elektrische Neuro Stimulatie (TENS) kan een bijdrage leveren aan de behandeling van lumbosacrale radiculaire pijn (LRP) maar wordt wegens onvoldoende evidence niet als standaard geadviseerd. Doel Vergelijken van het effect van TENS-behandeling met een transforaminale

Quantifying biometric life insurance risks with non-parametric smoothing methods

NARCIS (Netherlands)

Tomas, J.

2013-01-01

Julien Tomas beschrijft verdelingsvrije afrondingsmethoden van de sterfte-ervaring bij levensverzekeringen. Net als parametrische methoden neigen ook deze methoden naar onzuivere schattingen, maar zodanig dat het mogelijk is een grotere onzuiverheid op te laten wegen tegen een lagere

ANCA-negative limited Wegener′s granulomatosis

Directory of Open Access Journals (Sweden)

Ghosh A

2004-03-01

Full Text Available A 26-year-old man presented with epistaxis, nasal obstruction and a subcutaneous swelling over the left malar region with radiological evidence of a mass in the right nasal cavity. Histology of the lesions showed necrotizing granuloma with evidence of vasculitis. There was no other systemic involvement and the patient was ANCA-negative. Excellent response to systemic steroid and cyclophosphamide therapy was noted.

Plate Tectonics and Europa's Icy Shell

Indian Academy of Sciences (India)

defence of his theory with the 1915 publication of The Origin of Continents and Oceans. Wegener .... is one of the most promising places in our solar system to search .... Universe, Paperback Edition, Copernicus Books, pp.191–216, 2003.

Case note: EHRM (Grand Chamber) (zaaknr. 16354/06, LJN BX9103: Mouvement Raëlien Suisse/Zwitserland: verbod verspreiding posters is niet in strijd met de vrijheid van meningsuiting)

NARCIS (Netherlands)

Dommering, E.J.

2014-01-01

Mensenrechten. Vrijheid van meningsuiting. Weigering van vergunning voor het ophangen van posters van organisatie die contact met buitenaardse wezens nastreeft. Uiting is eerder commercieel dan politiek van aard wegens ‘bekerende’ werking. Vanwege vermelding website moet ook inhoud daarvan worden

Doorlatendheid van TRISOPLAST voor verschillende vloeistoffen

NARCIS (Netherlands)

Boels, D.; Veerman, G.J.

1996-01-01

TRISOPLAST is een mengsel van zand, bentoniet en een polymeer en wordt wegens zijn zeer lage doorlatendheid toegepast voor afdichtingen in bodembeschermende constructies. TRISOPLAST is ongevoelig voor aceton, ruwe olie, fenol en enigszins gevoelig voor diesel en zeewater na een blootstellingsduur

Bijzondere belastingen in tunnels : Eindrapport

NARCIS (Netherlands)

Molenaar, D.J.; Weerheijm, J.; Vervuurt, A.; Burggraaf, H.; Roekaerts, D.; Meijers, P.

2009-01-01

Verkeerstunnels en overkapte wegen (landtunnels) komen de milieukundige en stedenbouwkundige inpassing ten goede en maken meervoudig ruimtegebruik in de stad mogelijk. Het aantal tunnels en overkappingen groeit dan ook. Dit maakt het vervoer van explosiegevaarlijke stoffen en onder hoge druk

Somatostatin analogue scintigraphy and tuberculosis: case report

International Nuclear Information System (INIS)

Biancheri, I.; Rudenko, B.; Vautrin, P.; Raddoul, J.; Lamfichek, N.; Kantelip, B.; Mantion, G.

2005-01-01

Scintigraphy using a radiolabelled somatostatin analogue (111 In-pentetreotide) is useful in the detection of neuroendocrine tumors. But this radiopharmaceutical accumulates also in solid tumours or in inflammatory diseases such as granulomatosis. We present a case of 111 In-pentetreotide uptake in a tuberculous adenopathy. (author)

Systemic sarcoidosis complicated of acute renal failure: about 12 ...

African Journals Online (AJOL)

The sarcoidosis is a systemic granulomatosis affecting most frequently the lungs and the mediastinum. An acute renal failure reveals exceptionally this disease. It's a retrospective study implicating 12 cases of sarcoidosis complicated of acute renal failure. The aim of this study is to determine epidemiological, clinical, ...

Oral Crohn′s disease without intestinal manifestations

Directory of Open Access Journals (Sweden)

Gingisetty Harikishan

2012-01-01

Full Text Available Crohn′s disease is a granulomatous inflammatory bowel disease and was described in 1932 as a chronic granulomatous disorder of the terminal ileum and is now considered a distinct member of the inflammatory bowel disease family. It may affect any part of the gastrointestinal tract. Oral Crohn′s disease has been reported frequently in the last three decades with or without intestinal manifestations. In the latter case, it is considered as one of the orofacial granulomatosis. There has been much doubt whether intestinal manifestations of Crohn′s disease will eventually develop in the orofacial granulomatosis. We present a female patient aged 22 years with prominent clinical findings such as persistent swelling of lower and upper lip with fissuring and angular cheilitis, granulomatous gingival enlargement, and cobblestone or corrugated appearance of labial mucosa, which are suggestive of Crohn′s disease, but with no evidence of other gastrointestinal involvement. The patient underwent surgical treatment with external gingivectomy procedure. A 6-month follow-up showed minimal recurrence.

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

Directory of Open Access Journals (Sweden)

Cristiano Barbosa Campanholo

2007-02-01

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

Radiologic findings of diffuse Pulmonary hemorrhage

Energy Technology Data Exchange (ETDEWEB)

Seo, Mi Ra; Song, Koun Sik; Lee, Jin Seong; Lim, Tae Hwan [Ulsan Univ. College of Medicine, Seoul (Korea, Republic of)

1998-12-01

To describe the chest radiographic and CT findings of diffuse pulmonary hemorrhage. Two radiologists retrospectively analysed the chest radiographic and CT findings of six patients with diffuse pulmonary hemorrhage. Using open lung biopsy(n=3D2) and transbronchial lung biopsy or bronchoalveolar lavage(n=3D4), diagnosis was based on the presence of hemosiderin-laden macrophage or intra-alveolar hemorrhage. Underlying diseases were Wegener's granulomatosis(n=3D2), antiphospholipid antibody syndrome(n=3D2), Henoch-Schonlein purpura(n=3D1), and idopathic pulmonary hemosiderosis(n=3D1). In all patients, sequential chest radiographs, obtained during a one to six-month period, were available. HRCT scans were obtained in five patinets, and conventional CT scans in one. Follow-up HRCT scans were obtained in two. We also analyzed the patterns of involvement, distribution and sequential changes in the pulmonary abnormalities seen on chest radiographs and CT scans. Chest radiographs showed multifocal patchy consolidation(n=3D6), ground-glass opacity(n=3D3), and multiple granular or nodular opacity(n=3D3). These lesions were intermingled in five patients, while in one there was consolidation only. Sequential chest radiographs demonstrated the improvement of initial pulmonary abnormalities and appearance of new lesions elsewhere within 5-6 days, though within 7-25 (average, 13) days, these had almost normalized. HRCT scans showed patchy consolidation(n=3D5), multiple patchy ground-glass opacity(n=3D5), or ill-defined air space nodules(n=3D4). These lesions were intermingled in five patients, and in one, ground-glass opacity only was noted. In two patients there were interlobular septal thickening and intalobular reticular opacity. The distribution of these abnormalities was almost always bilateral, diffuse with no zonal predominancy, and spared the apex of the lung and subpleural region were less affected. Although chest radiographic and CT findings of diffuse pulmonary

Radiologic findings of diffuse Pulmonary hemorrhage

International Nuclear Information System (INIS)

Seo, Mi Ra; Song, Koun Sik; Lee, Jin Seong; Lim, Tae Hwan

1998-01-01

To describe the chest radiographic and CT findings of diffuse pulmonary hemorrhage. Two radiologists retrospectively analysed the chest radiographic and CT findings of six patients with diffuse pulmonary hemorrhage. Using open lung biopsy(n=3D2) and transbronchial lung biopsy or bronchoalveolar lavage(n=3D4), diagnosis was based on the presence of hemosiderin-laden macrophage or intra-alveolar hemorrhage. Underlying diseases were Wegener's granulomatosis(n=3D2), antiphospholipid antibody syndrome(n=3D2), Henoch-Schonlein purpura(n=3D1), and idopathic pulmonary hemosiderosis(n=3D1). In all patients, sequential chest radiographs, obtained during a one to six-month period, were available. HRCT scans were obtained in five patinets, and conventional CT scans in one. Follow-up HRCT scans were obtained in two. We also analyzed the patterns of involvement, distribution and sequential changes in the pulmonary abnormalities seen on chest radiographs and CT scans. Chest radiographs showed multifocal patchy consolidation(n=3D6), ground-glass opacity(n=3D3), and multiple granular or nodular opacity(n=3D3). These lesions were intermingled in five patients, while in one there was consolidation only. Sequential chest radiographs demonstrated the improvement of initial pulmonary abnormalities and appearance of new lesions elsewhere within 5-6 days, though within 7-25 (average, 13) days, these had almost normalized. HRCT scans showed patchy consolidation(n=3D5), multiple patchy ground-glass opacity(n=3D5), or ill-defined air space nodules(n=3D4). These lesions were intermingled in five patients, and in one, ground-glass opacity only was noted. In two patients there were interlobular septal thickening and intalobular reticular opacity. The distribution of these abnormalities was almost always bilateral, diffuse with no zonal predominancy, and spared the apex of the lung and subpleural region were less affected. Although chest radiographic and CT findings of diffuse pulmonary

De fietsstraat : Onderzoek naar fietsverbindingen door verblijfsgebieden

NARCIS (Netherlands)

Andriesse, H.C.; Hansen, J.A.

1996-01-01

De bestaande fietsverbindingen in de stad lopen meestal langs doorgaande wegen met druk autoverkeer. Door de toename van het autoverkeer zijn deze verbindingen niet altijd meer de meest geschikte fietsvoorzieningen. Ook de maatregelen die in het kader van duurzaam veilig wegverkeer worden

GM : Kun je dat eten? Gezondheidsrisico’s van gentech voedsel.

NARCIS (Netherlands)

Tiggelen, Bas van

2004-01-01

Genetische modificatie kan onbedoeld resulteren in producten met allergene eigenschappen,verhoogde toxiciteit, of verlies aan voedingswaarde. Dit zijn theoretische risico’s, over de hoogte waarvan weinig valt te zeggen wegens gebrek aan gegevens. Een deel van de mogelijke risico’s van GM-voedsel

Význam teorie kontinentálního driftu Alfreda Wegenera

Czech Academy of Sciences Publication Activity Database

Špičák, Aleš

2012-01-01

Roč. 55, č. 1 (2012), s. 3-6 ISSN 0300-4414 Institutional research plan: CEZ:AV0Z30120515 Institutional support: RVO:67985530 Keywords : Alfred Wegener * continental drift * plate tectonics * history of the Earth Sciences Subject RIV: DC - Siesmology, Volcanology, Earth Structure

Buiten Spelen : woudlopershandboek voor de erfgoedmedewerker 2.0

NARCIS (Netherlands)

E.R. (Else) Kuiper; drs. Dick Rijken

2008-01-01

Wat gebeurt er als medewerkers van erfgoedinstellingen de wereld in trekken en hun publiek opzoeken, soms letterlijk op straat? Raken ze het spoor bijster of vinden ze juist nieuwe wegen in onze chaotische netwerksamenleving? Dit woudlopershandboek is vooral het verslag van een dergelijk avontuur in

Meer of minder licht

NARCIS (Netherlands)

Graaf, A. van der; Steentjes, A.

2000-01-01

Interview Wout van Bommel, informatie over DYNO onderzoek van Alferdinck en Hogema bij TNO Technische Menskunde De natuur heeft 'recht op duisternis', zegt de milieubeweging, en daarom moet de verlichting op wegen worden teruggedrongen. De overheid wil dat ook, want de kosten moeten omlaag.

Endocarditis : Improving the chain of care

NARCIS (Netherlands)

Gomes, Anna

2018-01-01

Infectieuze endocarditis is een levensbedreigende ziekte dat een agressief diagnostisch en therapeutisch beleid vereist. Aangezien dit in de huidige praktijk nog niet optimaal geïmplementeerd is, is er behoefte aan verbetering van de zorg voor patiënten met (een verdenking op) endocarditis. Wegens

ESA CryoVEx 2011

DEFF Research Database (Denmark)

Skourup, Henriette; Barletta, Valentina Roberta; Einarsson, Indriði

data obtained with an airborne electromagnetic (AEM) induction sounder conducted by Alfred Wegener Institute (AWI) with fixed‐wing airplane (Polar‐5, Basler BT‐67). DTU Space airborne team visited five main validation sites: Devon ice cap (Canada), Austfonna ice cap (Svalbard), the EGIG line crossing...

Inzetbaarheid van RF en ICP-MS voor de vaststelling van metaalgehalten in verpakkingsmateriaal : Regeling Verpakking en Verpakkingsafval

NARCIS (Netherlands)

van Dijk J; van de Beek A; Ritsema R; LAC

2003-01-01

In het kader van de "Regeling Verpakking en Verpakkingsafval" worden eisen gesteld aan de gehalten van cadmium, lood, kwik en zeswaardige chroom. De som van de gehalten mag de grens van 100 ug/g niet overschrijden. Wegens analytische beperkingen wordt chroom als totaal chroom (Cr)

Imaging pulmonary fibrosis

International Nuclear Information System (INIS)

Brauner, M.W.; Rety, F.; Naccache, J.M.; Girard, F.; Valeyre, D.F.

2001-01-01

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

Imaging pulmonary fibrosis; Imagerie des fibroses pulmonaires

Energy Technology Data Exchange (ETDEWEB)

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

2001-02-01

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

Biogas uit bermmaaisel : duurzaam en haalbaar?

NARCIS (Netherlands)

Ehlert, P.A.I.; Zwart, K.B.; Spijker, J.H.

2010-01-01

In het kader van het Groningse project 'Een schone berm geeft energie!' heeft Alterra de duurzaamheid en haalbaarheid van de vergisting van bermmaaisel tot biogas onderzocht. Van drie provinciale wegen in de provincie Groningen is de vegetatie en de kwaliteit gemonitord en werd in het voor- en

Il comico come strategia in Gianni Celati & Co

DEFF Research Database (Denmark)

2012-01-01

in danese. Lene Waage Petersen ha organizzato l’incontro di Copenaghen insieme a Pia Schwarz Lausten, con la collaborazione del comitato scientifico composto da Marco Belpoliti, María J. Calvo Montoro e Anna Wegener, che ringraziamo per il loro contributo sostanziale alla realizzazione del convegno...

Ketenrendementen in de Nederlandse agribusiness: varkensvlees, zuivel, groente en fruit

NARCIS (Netherlands)

Backus, G.B.C.; Boone, J.A.; Pierick, ten E.; Bunte, F.H.J.; Calker, van K.J.

2007-01-01

De afgelopen jaren is de vraag naar de positie van de agrarische producent in de voedselketen aan de orde gesteld wegens de concentratie in de detailhandel en in de voedingsmiddelenindustrie. Doel van dit onderzoek is het berekenen en analyseren van rendementen in de verschillende schakels van vier

[The development of Rein van Bemmelens (1904-1983) undation theory: forty years of Dutch geology].

Science.gov (United States)

Barzilay, Willemjan

2009-01-01

The Dutch geologist Rein van Bemmelen was the greatest opponent of plate tectonics in The Netherlands. He lived and worked during an important period in the history of earth sciences. He had studied geology when Wegeners theory was introduced and enthusiastically received in the Netherlands and he worked as a geologists during the period in which, after Wegeners theory was rejected in The Netherlands, several Dutch geologists came with their own theories to explain the origin of continents and oceans and in which plate tectonics was introduced in The Netherlands. He had proposed his own theory, the undation theory, at the beginning of the 1930s and kept on developing it during the following years. He continued to do so until his death in 1983. The history of the undation theory thus sheds light on the history of geology in The Netherlands. I will trace the history of geology in The Netherlands using Rein van Bemmelen and his undation theory as a lens.

Radiation therapy of benign diseases: patterns of care study in Germany; Strahlentherapie von gutartigen Erkrankungen: eine Bestandsaufnahme fuer Deutschland

Energy Technology Data Exchange (ETDEWEB)

Seegenschmiedt, M.H. [Alfried Krupp Krankenhaus Essen (Germany). Klinik fuer Radioonkologie, Strahlentherapie und Nuklearmedizin; Katalinic, A. [Erlangen-Nuernberg Univ., Erlangen (Germany). Inst. fuer Medizinische Statistik und Dokumentation; Makoski, H.B. [Staedtische Kliniken Duisburg (Germany). Strahlenklinik; Haase, W. [St. Vincentius Krankenhaus Karlsruhe (Germany). Klinik fuer Radioonkologie und Strahlentherapie; Gademann, G. [Magdeburg Univ. (Germany). Klinik fuer Strahlentherapie; Hassenstein, E. [Krankenhaus Nordwest, Frankfurt am Main (Germany). Radioonkologische Klinik

1999-11-01

Therapiekonzepte bei gutartigen Erkrankungen erhoben wurden. 134 (88%) Institutionen (22 in Ost-, 112 in Westdeutschland; 30 in Universitaets-, 104 in oeffentlichen/privaten Krankenhaeusern) beantworteten alle Fragen. Die Haeufigkeiten und Durchschnittswerte pro Institution und ueber alle Institutionen hinweg wurden hinsichtlich geographischer Region und Krankenhaustyp analysiert. Im Durchschnitt standen zwei (Spanne 1 bis 7) Linearbeschleuniger/Kobaltgeraete und 1,4 (Spanne 0 bis 4) Orthovoltgeraete pro Institution zur Verfuegung; 32 (24%) besassen kein Orthovoltgeraet. Pro Jahr wurden im Mittel 20 082 Patienten behandelt: 456 (2%) wegen Entzuendungen (221 Hidradenitis, 78 Panaritium, 23 Parotitis, 134 nicht differenziert), 12 600 (63%) wegen degenerativer Erkrankungen (2 711 Peritendinitis humeroscapularis, 1 555 Epicondylopathia humeri, 1 382 Fersensporn, 2 434 arthrotische Erkrankungen, 4 518 nicht differenziert), 927 (5%) wegen hypertrophischer Erkrankungen (146 Morbus Dupuytren, 382 Keloide, 155 Induratio penis plastica, 244 nicht differenziert), 1 210 (6%) wegen funktioneller Erkrankungen (853 endokrine Orbitopathie, 357 nicht differenziert), 4 889 (24%) wegen anderer Erkrankungen und institutionelle Unterschiede (Universitaets- vs. Versorgungskrankenhaeuser) bei hypertrophischen und funktionellen Erkrankungen (p<0,05). Die meisten Therapiekonzepte waren im Niedrigdosisbereich (<10 Gy) angelegt, schwankten aber im Detail unsystematisch und in weiten Grenzen. (orig./MG)

Groenbemesters en aaltjesmanagement; De belangrijke rol van deze groene motor in de grond

NARCIS (Netherlands)

Korthals, G.W.; Sukkel, W.

2008-01-01

Groenbemesters spelen een essentiële rol in de (biologische) rotatie, ook al heeft het gebruik ervan zowel voor- als nadelen. Bij een doordachte keus uit het aanbod aan groenbemesters wegen de voordelen op tegen de nadelen. Een groenbemester wordt steeds vaker gezien als de 'groene' motor voor

Over het belang van Temminck's „Discours préliminaire" voor de zoologische Nomenclatuur

NARCIS (Netherlands)

Mees, G.F.

1957-01-01

Het gewoonlijk als „Discours préliminaire" aangeduide artikel van Temminck heeft nimmer die aandacht van dierkundigen getrokken, die het, wegens zijn systematisch belang, verdient. Ten dele vindt dit stellig zijn oorzaak in de betrekkelijke zeldzaamheid van het werk, ten dele ook in het feit dat het

Churg Strauss syndrome; Sindrome de Churg Strauss

Energy Technology Data Exchange (ETDEWEB)

Lopez Rengifo, Diana Milena; Contreras Zuniga, Eduardo; Osio, Luis Fernando

2007-07-01

The Churg-Strauss syndrome, also called allergic granulomatosis and angiitis, is a multisystem disorder characterized by allergic rhinitis, asthma, and prominent peripheral blood eosinophilia. The most common organ involved is the lung, followed by the skin. The Churg-Strauss syndrome, however, can affect any organ system, including the cardiovascular, gastrointestinal, renal, and central nervous systems.

Churg Strauss syndrome

International Nuclear Information System (INIS)

Lopez Rengifo, Diana Milena; Contreras Zuniga, Eduardo; Osio, Luis Fernando

2007-01-01

The Churg-Strauss syndrome, also called allergic granulomatosis and angiitis, is a multisystem disorder characterized by allergic rhinitis, asthma, and prominent peripheral blood eosinophilia. The most common organ involved is the lung, followed by the skin. The Churg-Strauss syndrome, however, can affect any organ system, including the cardiovascular, gastrointestinal, renal, and central nervous systems

Alfred Wegener - From Continental Drift to Plate Tectonics

Indian Academy of Sciences (India)

rise to such incredible heights? What makes ... pattern of land and sea throughout the geological history of the. Earth. ..... eventually reaches a level that exceeds the slipping-point of rocks on either .... Clement of Florida International. University ...

Pulmonary Foreign Body Granulomatosis in Dental Technician.

Science.gov (United States)

Chung, Sung Jun; Koo, Gun Woo; Park, Dong Won; Kwak, Hyun Jung; Yhi, Ji Young; Moon, Ji-Yong; Kim, Sang-Heon; Sohn, Jang Won; Yoon, Ho Joo; Shin, Dong Ho; Park, Sung Soo; Pyo, Ju Yeon; Oh, Young-Ha; Kim, Tae-Hyung

2015-10-01

Occupational lung diseases are caused by several toxic substances including heavy metals; however, the exact pathologic mechanisms remain unknown. In the workplace, dental technicians are often exposed to heavy metals such as cobalt, nickel, or beryllium and occasionally develop occupational lung diseases. We described a case of occupational lung disease in a patient who was employed as a dental technician for over a decade. A 31-year-old, non-smoking woman presented with productive cough and shortness of breath of several weeks duration. Chest computed tomography revealed a large number of scattered, bilateral small pulmonary nodules throughout the lung field, and multiple mediastinal lymph nodes enlargement. Percutaneous needle biopsy showed multifocal small granulomas with foreign body type giant cells suggestive of heavy metals inhalation. The patient's condition improved on simple avoidance strategy for several months. This case highlighted the importance of proper workplace safety.

Mast Cell Quantification in Orofacial Granulomatosis | Nwizu ...

African Journals Online (AJOL)

. Abstract. This study was to quantify mast cells (MC), their corresponding densities, surface areas and surface areas of their distribution in relation to oedema formation. Formalin fixed, wax embedded, oral tissue sections from 29 cases of OFG ...

Non bis in idem in Europa : de zaken Spasic en M.

NARCIS (Netherlands)

van Hattum, Wiene

2015-01-01

Binnen Schengen en de EU geldt de regel dat iemand die onherroepelijk is berecht niet nog eens mag worden vervolgd of gestraft wegens hetzelfde feit (non bis in idem). Geldt deze bescherming ook wanneer de straf wel definitief is geworden maar nog niet ten uitvoer is gelegd? Artikel 54 van de

The "Blue Banana" Revisited

NARCIS (Netherlands)

Faludi, A.K.F.

2015-01-01

This essay is about the “Blue Banana”. Banana is the name given subsequently by others to a Dorsale européenne (European backbone) identified empirically by Roger Brunet. In a background study to the Communication of the European Commission ‘Europe 2000’, Klaus Kunzmann and Michael Wegener put

Disease: H01468 [KEGG MEDICUS

Lifescience Database Archive (English)

Full Text Available cases, but the use of biological agents such as rituximab or mepolizumab seems to be a promising therapeutic... (>10% of total WBC) PR3-ANCA (+) Prednisolone [DG:DG00093] Cyclophosphamide [DG:DG00675] Rituximab [DR:D029...hy P, Guillevin L, Merkel PA, Jayne DR ... TITLE ... Rituximab for the treatment of eosinophilic granulomatosi

Turbulence, Turbulence Control, and Drag Reduction.

Science.gov (United States)

1987-08-01

Rick Jensen, John Miles, Mark Mork,,vn. ,r i:, i .t , Rue.I-, Paul Strykowski, Harry C Swinney and Peter Wegener. I am indebted t, mike Francis who...parameter. Some ex- itervals in this case obviousl peaks sharpl - aiples are the speed of propagation of the turbu- around some walue. This last fact

De ontwikkelingsgeschiedenis van Rein van Bemmelens (1904-1983 undatietheorie: veertig jaar Nederlandse geologie

Directory of Open Access Journals (Sweden)

Willemjan Barzilay

2009-03-01

Full Text Available The development of Rein van Bemmelens (1904-1983 undation theory: Forty years of Dutch geology The Dutch geologist Rein van Bemmelen was the greatest opponent of plate tectonics in the Netherlands. He lived and worked during an important period in the history of earth sciences. He had studied geology when Wegeners theory was introduced and enthusiastically received in the Netherlands and he worked as a geologists during the period in which, after Wegeners theory was rejected in the Netherlands, several Dutch geologists came with their own theories to explain the origin of continents and oceans and in which plate tectonics was introduced in the Netherlands. He had proposed his own theory, the undation theory, at the beginning of the 1930s and kept on developing it during the following years. He continued to do so until his death in 1983. The history of the undation theory thus sheds light on the history of geology in the Netherlands. I will trace the history of geology in the Netherlands using Rein van Bemmelen and his undation theory as a lense.

Impact and safety of open lung biopsy in patients with acute respiratory distress syndrome (ARDS).

Science.gov (United States)

Ortiz, G; Garay, M; Mendoza, D; Cardinal-Fernández, P

2018-02-28

Acute respiratory distress syndrome (ARDS) is an inflammatory lung disorder, and its pathological hallmark is diffuse alveolar damage (DAD). Given that open lung biopsy (OLB) can sometimes result in severe side effects, it is rarely performed in patients with ARDS. The aims of this study were to describe: (a) the rate of treatment change associated with the histological result; and (b) the incidence of side effects induced by OLB. A retrospective, single-center, descriptive observational study was carried out in Hospital Santa Clara (Bogotá, Colombia) from February 2007 to January 2014. Critically ill patients over 18 years of age, undergoing invasive mechanical ventilation, diagnosed with ARDS of unknown etiology, and with OLB performed at the bedside. ARDS was diagnosed according to the Berlin definition. DAD was defined by the presence of a hyaline membrane plus at least one of the following: intra-alveolar edema, alveolar type I cell necrosis, alveolar type II cell (cuboidal cells) proliferation progressively covering the denuded alveolar-capillary membrane, interstitial proliferation of fibroblasts and myofibroblasts, or organizing interstitial fibrosis. The rate of treatment change (RTC) was established according to whether the OLB pathology report resulted in: a) the prescription or discontinuation of an antimicrobial; b) the indication of new procedures; c) medical interconsultation; or d) limitation of therapeutic effort. Patients were followed-up until death or hospital discharge. This study was approved by the Ethics Committee. A total of 32 OLBs were performed during the study period; 17 were ruled out as they did not involve ARDS, and 15 were considered for further analysis. A histological diagnosis was reached in 14 of the 15 patients (12 DAD, one case of bronchiolitis obliterans organizing pneumonia and one case of Wegener's granulomatosis with alveolar hemorrhage). The RTC rate was 0.73. The most frequent intervention was discontinuation of

Inherent veilige 80 km/uur-wegen : ontwikkeling van een strategie voor een duurzaam-veilige (her)inrichting van doorgaande 80 km/uur-wegen. Deel I: keuze van de relevante wegen en het opstellen van criteria en eisen.

NARCIS (Netherlands)

Minnen, J. van

1993-01-01

The former Dienst Verkeerskunde of Rijkswaterstaat commissioned SWOV Institute for Road Safety Research to develop a strategy for an "inherent safe" reconstruction of 80 km/h through roads in the Netherlands. The first part of this study is the conceptualization phase. It classifies 80 km/h through

Fusion Protein Cytokine Therapy After Rituximab in Treating Patients With B-Cell Non-Hodgkin Lymphoma

Science.gov (United States)

2015-06-03

Anaplastic Large Cell Lymphoma; Cutaneous B-cell Non-Hodgkin Lymphoma; Extranodal Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue; Intraocular Lymphoma; Nodal Marginal Zone B-cell Lymphoma; Recurrent Adult Diffuse Small Cleaved Cell Lymphoma; Recurrent Adult Grade III Lymphomatoid Granulomatosis; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Marginal Zone Lymphoma; Small Intestine Lymphoma; Splenic Marginal Zone Lymphoma; Testicular Lymphoma; Waldenstrom Macroglobulinemia

Syndrome of Churg Strauss

International Nuclear Information System (INIS)

Sanchez Morales, Edgar Alberto; Saavedra Rodriguez, Alfredo; Henao Riveros, Sandra

2002-01-01

The Churg-Strauss syndrome denominated allergic granulomatosis and angeitis is characterized by a systemic vasculitis of small glasses, extravascular granulomas and hypereosinophilia. Initially described by Jacob Churg and Lotte Strauss, two pathologists who in 1951 they published the description of 13 patient postmortem with tisular infiltration for eosinophils, necrotizant vasculitis and extravascular granulomas. The paper includes nomenclature, classification approaches, pathogenesis, pathology, and clinical aspects and diagnostic

Syndrome of Churg Strauss; Sindrome de Churg Strauss

Energy Technology Data Exchange (ETDEWEB)

Sanchez Morales, Edgar Alberto; Saavedra Rodriguez, Alfredo; Henao Riveros, Sandra

2002-09-01

The Churg-Strauss syndrome denominated allergic granulomatosis and angeitis is characterized by a systemic vasculitis of small glasses, extravascular granulomas and hypereosinophilia. Initially described by Jacob Churg and Lotte Strauss, two pathologists who in 1951 they published the description of 13 patient postmortem with tisular infiltration for eosinophils, necrotizant vasculitis and extravascular granulomas. The paper includes nomenclature, classification approaches, pathogenesis, pathology, and clinical aspects and diagnostic.

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.

Weten is wegen . . . . : Ureumgehalte melk bevestigt noodzaak goede voerweging

NARCIS (Netherlands)

Teenstra, E.D.

2000-01-01

Afgelopen jaar is veel tijd en energie gestoken in het uniformeren van de gegevensstroom. Vrijwel alle deelnemers beschikken nu over weegapparatuur waarmee ze nauwkeurig de voeropname van het vee kunnen registreren. Voor de berekening van de stikstofbenutting van het vee levert dit belangrijke

Analyse von Kunststoffadditiven mittels Laserablation gekoppelt mit induktiv gekoppelter Plasma Massenspektrometrie

OpenAIRE

Börno, Fabian

2016-01-01

Die Laserablation gekoppelt mit der Massenspektrometrie mit induktiv gekoppeltem Plasma ist eine vielversprechende direkte Feststofftechnik, die sich jedoch bei der Analyse von Kunststoffen wegen des Mangels an matrixangepassten zertifizierten Referenzmaterialien nicht durchsetzen konnte. Vorherige Arbeiten belegen polymerabhängige Abtragsraten. Das oft als interner Standard verwendete Intensitätssignal des Kohlenstoffisotopes 13C zur Korrektur dieser Unterschiede wird in der Literatur kritis...

Quantum Metaphotonics

Science.gov (United States)

2016-03-24

National Laboratories), Ricky Gibson, Sander Zandbergen, Jasmine Sears , and Patrick Keiffer. Professor: Galina Khitrova References 1. M...Gehl, S. Zandbergen, R. Gibson, M. Béchu, N. Nader, J. Hendrickson, J. Sears , P. Keiffer, M. Wegener, and G. Khitrova, “Spectroscopic studies of...2012). 12. M. Gehl, R. Gibson, S. Zandbergen, P. Keiffer, J. Sears , and G. Khitrova, “Superconductivity in epitaxially grown self-assembled indium

Über das Sicherstellen von Güte/Validität im Rahmen einer multi-lokalen und multidisziplinären Studie

OpenAIRE

Crawford, H. Ken; Leybourne, Marnie L.; Arnott, Allan

2000-01-01

Qualitative Forschung wird häufig wegen ihres Mangels an Güte kritisiert. Um diese Situation zu verbessern, werden in der Literatur Kriterien wie "trustworthiness", "dependability" und "reliability" diskutiert. Im vorliegenden Beitrag werden Vorschläge zu dieser Debatte ausgehend von einer empirischen Untersuchung zu den Lernprozessen von australischen Farmern gemacht. An dieser Interviewstudie, die in drei Bundesstaaten durchgeführt wurde, waren Forschende unterschiedlicher Disziplinen betei...

Azathioprine induced Epstein-Barr virus positive mucocutaneous ulcer: A case report

Directory of Open Access Journals (Sweden)

Arneja S

2018-02-01

Full Text Available Introduction: Epstein-Barr virus positive mucocutaneous ulcer (EBVMUC is a rare, newly described provisional entity in the 2016 Update of World Health Organization classification of lymphoid neoplasms. The histomorphological and immunophenotypical and molecular features overlap with classical Hodgkin’s lymphoma (cHL and can be mistaken for the same. Case report: A 70-year-old male, a known case of diabetes mellitus and hypertension, was diagnosed with Wegener’s granulomatosis (granulomatosis with polyangiitis in 2007. He was treated with prednisolone and cyclophosphamide, the latter drug was replaced with azathioprine in 2010. He was apparently well since then, until he presented in 2016 with an anal ulcer with a fistula tract formation, the ulcer on histomorphology and immunohistochemistry was diagnosed as EBVMUC. Discussion: EBVMUC was first described in patients with iatrogenic induced immunosuppression.They have later been found to be associated with various other causes of immunosuppression, like solid organ transplant recipients and human immunodeficiency virus (HIV, common factor in all these being immunosuppression. Conclusion: The importance of recognizing this entity lies in its morphological and immunophenotypic overlap with classical Hodgkin’s lymphoma (cHL and unlike latter, most often complete resolution of disease occurs with reduction of immunosuppressive dose. Therefore, correct recognition of the entity is essential to avoid overtreatment as lymphoma.

Asymptomatic endoalveolar hemorrhage in a young male

Directory of Open Access Journals (Sweden)

Alain Kafyeke

2016-06-01

Full Text Available We describe the case of a young male affected by granulomatosis with polyangiitis presenting with non-specific complaints and complicated by the occurrence of a diffuse endoalveolar hemorrhage characterized by atypical clinical and radiological features. The importance of a rapid and aggressive diagnostic and therapeutic approach has to be strongly underlined. Available data regarding prevalence, clinical and radiological characteristics and treatment of this uncommon manifestation have also been hereby reviewed.

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

Science.gov (United States)

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

2018-01-01

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

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

Directory of Open Access Journals (Sweden)

Nicole A. Negbenebor

2018-03-01

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

Manifestações destrutivas da córnea e esclera associadas a doenças do tecido conectivo: relato de 9 casos Corneal and scleral destructive involvement associated with connective tissue disease: report of 9 cases

Directory of Open Access Journals (Sweden)

Namir Clementino Santos

2004-08-01

apropriado da doença de base.PURPOSE: To study the clinical features, diagnosis, treatment, and outcome of nine patients with corneal and scleral involvement associated to connective tissue disease. METHODS: Description of anterior segment abnormalities observed in nine patients with connective tissue disease, five of them previously diagnosed and four with the ocular involvement being the first manifestation of the disease. All patients were evaluated at the Corneal and External Diseases outpatient clinic of the Federal University of São Paulo - Escola Paulista de Medicina (UNIFESP-EPM between July/1999 to December/2000 and were submitted to a complete ophthalmologic examination, clinical evaluation and laboratory investigation. Clinical or surgical treatment was indicated according to the ocular disease evolution. RESULTS: The clinical diagnoses of the patients with anterior segment inflammatory disease were rheumatoid arthritis in seven patients (77.8%, systemic sclerosis and Wegener´s granulomatosis in each of the remaining patients (22.2%. The most frequent ocular manifestation was scleritis (66.6%, followed by peripheral ulcerative keratitis (55.5% and dry eye syndrome (44.4%. Eighty-nine percent (89% of the patients required immunosuppressive therapy to control the ocular inflammatory disease. Remission of the ocular symptoms were observed after 3 months of the beginning of imunossupressive therapy on average. In 55.5% of the patients a surgical approach (conjunctival resection, application of cyanoacrylate tissue adhesive, penetrating keratoplasty and scleral patch graft was necessary. CONCLUSION: The finding of corneal and scleral involvement associated with connective tissue disease is a sign of the disease activity and usually requires imunossupressive therapy. The ophthalmologists should be aware of these conditions in order to early diagnose and apply the appropriate treatment.

A review of studies on persuasion from the viewpoint of the Elaboration Likelihood Model (1)

OpenAIRE

Fukada, Hiromi; Kimura, Kenichi; Makino, Koshi; Higuchi, Masataka

2000-01-01

The purpose of this paper was to review studies on persuasion from the viewpoint of the Elaboration Likelihood Model based on Petty & Wegener (1998). The paper consists of the following four parts. 1. Introduction. 2. Multiple roles for persuasion variables. 3. Source variables: (1) credibility (expertise, trustworthiness), (2) attractiveness/likableness, (3) power, (4) additional source factors related to credibility, liking and power (speed of speech, demographic variables, majority/minorit...

Normzahlen, Toleranzen, Passungen

Science.gov (United States)

Böge, Gert; Böge, Wolfgang

Vor allem wegen der Kosten ist es sinnvoll, sich beim Festlegen von Maßen aller Art auf Vorzugszahlen zu beschränken (Baugrößen, Drehzahlen, Drehmomente, Leistungen, Drücke usw.). Man verwendet dazu eine geometrisch gestufte Zahlenfolge (siehe Teil Mathematik). Abb. 40.1 zeigt, dass bei der geometrischen Stufung die Werte im unteren Bereich fein, im oberen grob gestuft sind. Das ist nicht nur technisch sinnvoll.

Ongelijk leven, ongelijk sterven

OpenAIRE

Lecq, Fieke

2001-01-01

textabstractOverlappende generaties-modellen zijn een prachtig instrument voor economen, omdat ze via inzichtelijke mechanismen interessante uitkomsten opleveren. In deze modellen worden meerdere generaties gemodelleerd, die elk een voorraad geld of goederen bij geboorte meekrijgen, deze hoeveelheid tijdens hun leven veranderen door inkomen uit werk en uitgaven wegens consumptie en aan het eind van hun leven het restant nalaten aan de volgende generatie. Zolang mensen niet weten hoe oud ze pr...

Multi-Phase Combustion and Transport Processes Under the Influence of Acoustic Excitation

Science.gov (United States)

2014-01-01

waveguide. Alcohol fuels (ethanol and methonal) as well as aviation fuel replacements ( Fischer -Tropsch (FT) synfuel and an FT blend with JP-8) were studied...replacements ( Fischer -Tropsch (FT) synfuel and an FT blend with JP-8) were studied here. During acoustic excitation, the flame surrounding the droplet was...Wegener is approved. Chris R. Anderson Jeff D. Eldredge Ivett A. Leyva Owen I. Smith Ann R. Karagozian, Committee Chair University of California, Los

DASHBOARD.awi.de: Streaming and monitoring solutions for near real-time data

OpenAIRE

Koppe, Roland; Gerchow, Peter; Macario, Ana; Haas, Antonie; Schäfer-Neth, Christian; Rehmcke, Steven; Walter, Andreas; Düde, Tobias; Weidinger, Philipp; Schäfer, Angela; Pfeiffenberger, Hans

2018-01-01

DASHBOARD.awi.de is a component of our data flow framework designed to enable a semi-automated flow of sensor observations to archives (acronym O2A). The dramatic increase in the number and type of platforms and respective sensors operated by Alfred Wegener Institute along with complex project-driven requirements in terms of satellite communication, sensor monitoring, quality control and validation, processing pipelines, visualization, and archival under FAIR principles, led us to build a...

O2A - Data Flow Framework from Sensor Observations to Archives

OpenAIRE

Gerchow, Peter; Koppe, Roland; Macario, Ana; Haas, Antonie; Schäfer-Neth, Christian; Pfeiffenberger, Hans; Schäfer, Angela

2017-01-01

The Alfred Wegener Institute coordinates German polar research and is one of the most productive polar research institutions worldwide with scientists working in both Polar Regions – a task that can only be successful with the help of excellent infrastructure and logistics. Conducting research in the Arctic and Antarctic requires research stations staffed throughout the year as the basis for expeditions and data collection. It needs research vessels, aircrafts and long-term observ...

Vom Lamarckismus, vom Hakenkreuz und von meinen Katzen

OpenAIRE

Koch, Richard

2005-01-01

Richard Koch (1882-1949) zählt zu den bedeutendsten Medizintheoretikern und -historikern des 20. Jahrhunderts. Neben Arbeiten zu ihrem geschichtlichen Selbstverständnis ist Koch insbesondere wegen seiner Überlegungen zu den wissenschaftstheoretisch-philosophischen Grundlagen der Medizin in die Geschichtsbücher eingegangen. Als Jude mußte Koch Deutschland 1936 verlassen und emigrierte in die Sowjetunion. Er arbeitete bis zu seinem Tod 1949 als Arzt im kaukasischen Badeort Essentuki. Neben wiss...

Risk of subsequent ischemic and hemorrhagic stroke in patients hospitalized for immune-mediated diseases: a nationwide follow-up study from Sweden

Directory of Open Access Journals (Sweden)

Zöller Bengt

2012-06-01

Full Text Available Abstract Background Certain immune-mediated diseases (IMDs have been associated with increased risk for cardiovascular disorders. The aim of the present study was to examine whether there is an association between 32 different IMDs and first hospitalization for ischemic or hemorrhagic stroke. Methods All individuals in Sweden hospitalized with a main diagnosis of IMD (without previous or coexisting stroke, between January 1, 1987 and December 31, 2008 (n = 216,291, were followed for first hospitalization for ischemic or hemorrhagic stroke. The reference population was the total population of Sweden. Adjusted standardized incidence ratios (SIRs for ischemic and hemorrhagic stroke were calculated. Results Totally 20 and 15 of the 32 IMDs studied, respectively, were associated with an increased risk of ischemic and hemorrhagic stroke during the follow-up. The overall risks of ischemic and hemorrhagic stroke during the first year after hospitalization for IMD were 2.02 (95% CI 1.90–2.14 and 2.65 (95% CI 2.27–3.08, respectively. The overall risk of ischemic or hemorrhagic stroke decreased over time, to 1.50 (95% CI 1.46–1.55 and 1.83 (95% CI 1.69–1.98, respectively, after 1–5 years, and 1.29 (95% CI 1.23–1.35 and 1.47 (95% CI 1.31–1.65, respectively, after 10+ years. The risk of hemorrhagic stroke was ≥2 during the first year after hospitalization for seven IMDs: ankylosing spondylitis (SIR = 8.11, immune thrombocytopenic purpura (SIR = 8.60, polymyalgia rheumatica (SIR = 2.06, psoriasis (SIR = 2.88, rheumatoid arthritis (SIR = 3.27, systemic lupus erythematosus (SIR = 8.65, and Wegener´s granulomatosis (SIR = 5.83. The risk of ischemic stroke was ≥2 during the first year after hospitalization for twelve IMDs: Addison’s disease (SIR = 2.71, Crohn´s disease (SIR = 2.15, Grave´s disease (SIR = 2.15, Hashimoto´s thyroiditis (SIR = 2.99, immune thrombocytopenic purpura (SIR = 2

Demonstration of the proliferation marker Ki-67 in renal biopsies: correlation to clinical findings.

Science.gov (United States)

Nabokov, A; Waldherr, R; Ritz, E

1997-07-01

Assessment of cell proliferation in renal biopsy samples is a potentially promising analytical tool to evaluate disease activity. So far no information is available on the correlation between proliferative activity in different anatomic compartments of the kidney and clinical symptoms. To elucidate this issue, we examined renal biopsy specimens from 20 patients with systemic vasculitis (15 Wegener's granulomatosis, five microscopic polyangiitis), 20 patients with immunoglobulin (Ig) A nephropathy (IgAN), 13 patients with minimal-change disease (MCD), 11 patients with tubulointerstitial nephritis, and five patients with diabetes mellitus. The streptavidin-biotin-peroxidase complex technique was applied to autoclave-pretreated, formalin-fixed, paraffin-embedded tissue sections to label different cell types with the antibody MIB1 directed against the Ki-67 antigen. Proliferation index (PI) was estimated as the number of positively stained nuclei per glomerular cross-section or per square millimeter section area. The interstitial cells were discriminated by additional staining of Ki-67-processed samples with specific immune markers. In patients with vasculitis, PI was considerably elevated in the extracapillary glomerular compartment (0.86), in proximal tubules (6.24), and in the interstitium (8.62). High proliferative activity was also noted in interstitium (3.98) and proximal tubules (1.35) of patients with IgAN. Of particular interest was the increased interstitial proliferative activity (15.0) in diabetic patients. Resident renal cells, but not infiltrating cells, seemed to constitute the majority of the proliferating cell population in the interstitium. In systemic vasculitis, clinical disease activity was significantly correlated to endocapillary (r(s) = 0.58), extracapillary (r(s) = 0.67), proximal tubular (r(s) = 0.67), and interstitial PI (r(s) = 0.61). By multiple linear regression analysis, proximal tubular PI was correlated to the presence of hematuria

Two rare cases of Epstein-Barr virus-associated lymphoproliferative disorders in inflammatory bowel disease patients on thiopurines and other immunosuppressive medications.

Science.gov (United States)

Subramaniam, K; Cherian, M; Jain, S; Latimer, M; Corbett, M; D'Rozario, J; Pavli, P

2013-12-01

The setting of chronic immunosuppression in inflammatory bowel disease (IBD) may promote the proliferation of Epstein-Barr virus-positive neoplastic clones. We report two rare cases of Epstein-Barr virus-associated lymphoproliferative disorder in IBD patients: one resembled lymphomatoid granulomatosis, and the other was a lymphoma resembling Hodgkin lymphoma. There are currently no guidelines for the prevention of lymphoproliferative disorder in IBD patients on immunosuppressive therapy. © 2013 The Authors; Internal Medicine Journal © 2013 Royal Australasian College of Physicians.

Technetium-99m HMPAO labelled-leukocytes and gallium-67 scintigraphies in a Munchhausen's syndrome

International Nuclear Information System (INIS)

Alberini, J.L.; Devaux, J.Y.; Tenenbaum, F.; Richard, B.; Dell'Isola, D.

2000-01-01

We report an observation of investigations performed in a patient with several cervical abscesses in his previous history. Aim of scintigraphic examinations was to detect an evolutive infection and/or to identify a general disease like granulomatosis. Although there was no abnormality on the labelled-leucocytes scintigraphy, an infra-mandibular uptake was observed 10 days later on 67 Ga, without bone involvement. This 'discrepancy' between scintigraphic results and the clinical evolution allowed to confirm its management in nuclear medicine were discussed. (author)

Radiological and radioisotope diagnosis of pericarditides in lymphogranulomatosis

Energy Technology Data Exchange (ETDEWEB)

Astanov, B M; Kuz' min, V P; Shapiro, I B; Romagin, V K [Nauchno-Issledovatel' skij Inst. Meditsinskoj Radiologii, Obninsk (USSR)

1980-04-01

The radiological and scintigraphic results of 52 patients suffering from granulomatosis and pericarditis, the latter as a consequence of radiotherapy in 30 cases, were compared. The radiological examination after application of an artificial pneumopericardium allows the interpretation of both the quantity of the exudate and the condition of epicardium and pericardium. Applying additional scintigraphic methods central hemodynamic parameter can be examined; thus in exsudative pericarditis s decrease of the cardiac output and of the cardiac index could be stated intensifying with increasing exsudation.

Norrie-Syndrom

OpenAIRE

Flügel, T; Pflug, C; Niessen, A

2016-01-01

Hintergrund: Vorgestellt wurde in unserer Klinik ein 17-jähriger Junge mit angeborener Blindheit. Er leidet zusätzlich seit dem 14. Lebensjahr unter einer schubweise progredienten Schwerhörigkeit. Aktuell zeigte sich eine mittelgradige Schwerhörigkeit beidseits. Eine humangenetische Abklärung der Augenveränderungen hatte in der frühen Kindheit keine Auffälligkeiten ergeben. Die Familienanamnese ist bezogen auf Hörstörungen oder Blindheit leer. Wegen der neu aufgetretenen progredienten Schwer...

Zum Studium der Anthroposophie

OpenAIRE

Schlüter, Martin

2014-01-01

Johann Wolfgang v. Goethe und Rudolf Steiner haben beide auf verschiedenen Wegen eine Denkungsart entwickelt, deren Quelle diese Anschauungskraft ist. Eine Denkungsart, die nicht dabei stehenbleibt über die Dinge und Wesen nachzudenken, sondern die sie vielmehr in Gedankenform erstehen und so ihr Wesen unmittelbar erlebbar werden lässt. Goethe wandte sich dabei den Naturreichen zu und gestaltete seine ‚gegenständliche' Denkungsart zu einer naturwissenschaftlichen Methode aus, die er für weit ...

Dynamische Programm-Code-Verwaltung und -Optimierung für eingebettete Systeme

OpenAIRE

Schell, Dominic

2009-01-01

Bei Desktop-PCs ist ein großer Hauptspeicherausbau im Bereich jenseits von 1 GB dank gesunkener Speicherpreise und auf Grund gestiegener Anforderungen keine Seltenheit mehr. Es existiert jedoch eine Unmenge Kleinstrechner, wie z.B. im Automobilbereich eingesetzte Steuergeräte, die sich einen großen Speicherausbau wegen des Platzbedarfs der Speicherchips, deren Energiekonsum und den zusätzlichen Kosten nicht leisten können. Ein wichtiges Ziel beim Erzeugen der Software für eingebettete Systeme...

DATA.awi.de: A one-stop-shop framework for discovery

OpenAIRE

Koppe, Roland; Gerchow, Peter; Macario, Ana; Haas, Antonie; Schäfer-Neth, Christian; Schumacher, Stefanie; Driemel, Amelie; Cornils, Astrid; Hehemann, Laura; Eilers, Janik; Rehmcke, Steven; Walter, Andreas; Düde, Tobias; Weidinger, Philipp; Schäfer, Angela

2018-01-01

DATA.awi.de is a component of our data flow framework designed to enable a semi-automated flow of sensor observations to archives (acronym O2A). The dramatic increase in the number and type of platforms and respective sensors operated by Alfred Wegener Institute along with complex project-driven requirements in terms of satellite communication, sensor monitoring, quality control and validation, processing pipelines, visualization, and archival under FAIR principles, led us to build a gene...

SENSOR.awi.de: Management of heterogeneous platforms and sensors

OpenAIRE

Koppe, Roland; Gerchow, Peter; Macario, Ana; Haas, Antonie; Schäfer-Neth, Christian; Rehmcke, Steven; Walter, Andreas; Düde, Tobias; Weidinger, Philipp; Schäfer, Angela; Pfeiffenberger, Hans

2018-01-01

SENSOR.awi.de is a component of our data flow framework designed to enable a semi-automated flow of sensor observations to archives (acronym O2A). The dramatic increase in the number and type of platforms and respective sensors operated by Alfred Wegener Institute along with complex project-driven requirements in terms of satellite communication, sensor monitoring, quality control and validation, processing pipelines, visualization, and archival under FAIR principles, led us to build a g...

Cognitive continental drift: how attitudes can change the overall pattern of cognitive distances

OpenAIRE

Claus-Christian Carbon

2010-01-01

By the late Carboniferous period, the continents that today make up North America and Europe collided with the southern parts of Gondwana to form the western half of the last supercontinent Pangea. From this moment on, North America and Europe have steadily been drifting apart, as was initially described by Alfred Wegener in 1915. In this paper a cognitive counterpart of this continental drift is described—which progresses much faster than the phenomenon of plate tectonics. Distance estimatio...

History and Nature of Science enriched Problem-Based Learning on the origins of biodiversity and of continents and oceans

OpenAIRE

Sousa, Cristina

2014-01-01

[EN] The episode of the History of Science (HOS) on the theory of continental drift proposed by Alfred Wegener has been considered an excellent example for teaching students aspects of Nature of Science (NOS) and the relation of Science with social and tecnological contexts. We implemented a NOS and HOS-enriched Problem-Based Learning environment at the middle (year 7 of the Portuguese National Curriculum) and secondary level (year 10) for teaching the origins of biodiversity and of continent...

Validation of the CAR II model for Flanders, Belgium; Validatie van het model CAR II voor Vlaanderen

Energy Technology Data Exchange (ETDEWEB)

Marien, S.; Celis, D.; Roekens, E.

2013-04-15

In Flanders, Belgium, the CAR model (Calculation of Air pollution from Road traffic) for air quality along urban roads was recently extensively validated for NO2. More clarity has been gained about the quality and accuracy of this model [Dutch] In Vlaanderen is het CAR-model (Calculation of Air pollution from Road traffic) voor de luchtkwaliteit langs binnenstedelijke wegen onlangs uitvoerig gevalideerd voor NO2. Er is nu meer duidelijkheid over de kwaliteit en nauwkeurigheid van dit model.

La astrophysiques cause de la tectonique

OpenAIRE

Omerbashich , M

2016-01-01

Paper accepted for publishing on 31 Aug 2016 by Earthquake Science journal (http://link.springer.com/journal/11589), Editor-in-Chief: Chen Yuntai, member of Chinese Academy of Sciences. (ACCEPTANCE DECISION: https://sites.google.com/site/omerbashich/blog/AcceptanceNotice.png) Author rejected the offer as part of his protest against the way he and his discoveries were treated by the scientific community that resembled the treatment of Alfred Wegener and his historic discovery.; Tectonic earthq...

Technetium-99m HMPAO labelled-leukocytes and gallium-67 scintigraphies in a Munchhausen's syndrome; Scintigraphies aux polynucleaires marques (technitium-99m-HMPAO) et au gallium-67 dans le cadre d'un syndrome de Munchhausen

Energy Technology Data Exchange (ETDEWEB)

Alberini, J.L.; Devaux, J.Y.; Tenenbaum, F.; Richard, B. [Groupe Hospitalier Cochin - Saint-Vincent-de-Paul, 75 - Paris (France); Universite Rene Descartes, 75 - Paris (France); Dell' Isola, D. [Hopital Suisse de Paris, 92 - Issy-les-Moulineaux (France)

2000-06-01

We report an observation of investigations performed in a patient with several cervical abscesses in his previous history. Aim of scintigraphic examinations was to detect an evolutive infection and/or to identify a general disease like granulomatosis. Although there was no abnormality on the labelled-leucocytes scintigraphy, an infra-mandibular uptake was observed 10 days later on {sup 67}Ga, without bone involvement. This 'discrepancy' between scintigraphic results and the clinical evolution allowed to confirm its management in nuclear medicine were discussed. (author)

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.

The Role of the Immune Response in the Pathogenesis of Thyroid Eye Disease: A Reassessment.

Directory of Open Access Journals (Sweden)

James T Rosenbaum

Full Text Available Although thyroid eye disease is a common complication of Graves' disease, the pathogenesis of the orbital disease is poorly understood. Most authorities implicate the immune response as an important causal factor. We sought to clarify pathogenesis by using gene expression microarray.An international consortium of ocular pathologists and orbital surgeons contributed formalin fixed orbital biopsies. RNA was extracted from orbital tissue from 20 healthy controls, 25 patients with thyroid eye disease (TED, 25 patients with nonspecific orbital inflammation (NSOI, 7 patients with sarcoidosis and 6 patients with granulomatosis with polyangiitis (GPA. Tissue was divided into a discovery set and a validation set. Gene expression was quantified using Affymetrix U133 Plus 2.0 microarrays which include 54,000 probe sets.Principal component analysis showed that gene expression from tissue from patients with TED more closely resembled gene expression from healthy control tissue in comparison to gene expression characteristic of sarcoidosis, NSOI, or granulomatosis with polyangiitis. Unsupervised cluster dendrograms further indicated the similarity between TED and healthy controls. Heat maps based on gene expression for cytokines, chemokines, or their receptors showed that these inflammatory markers were associated with NSOI, sarcoidosis, or GPA much more frequently than with TED.This is the first study to compare gene expression in TED to gene expression associated with other causes of exophthalmos. The juxtaposition shows that inflammatory markers are far less characteristic of TED relative to other orbital inflammatory diseases.

Supra-molecular structure and chemical reactivity of cellulose I studied using CP/MAS (sup)13 C-NMR

CSIR Research Space (South Africa)

Chunilall, Viren

2013-08-01

Full Text Available There are a few traditional methods of analysing the chemical properties of cellulose I. Some of these methods include the Permanganate number determination, which is used to obtain the lignin content of the pulp [12]. The acid insoluble lignin content... – Fundamental Aspects 88 [10] Fengel D, Wegener G. Wood Chemistry, Ultrastructure, Reactions, Walter de Gruyter; 1984. [11] Uhlmann T. Ullmann's encyclopedia of industrial chemistry. Paper and Pulp. 1991; 18 (A). [12] Permanganate number of pulp, Tappi T...

Evaluatie snelheidscampagne op 80- en 100-km/uur wegen in Friesland.

NARCIS (Netherlands)

Oei, H.-l.

1996-01-01

The longer version of this study is published by the SWOV Institute for Road Safety Research, Serial Number R-95-24, see C 3855 (IRRD 875254). In 1994, a speed enforcement campaign was conducted on 39 roads of the provincial network in the Dutch province of Friesland. The whole campaign was

Central Diabetes Insipidus in Refractory Antineutrophil Cytoplasmic Antibody-associated Vasculitis.

Science.gov (United States)

Ohashi, Keiji; Morishita, Michiko; Watanabe, Haruki; Sada, Ken-Ei; Katsuyama, Takayuki; Miyawaki, Yoshia; Katsuyama, Eri; Narazaki, Mariko; Tatebe, Noriko; Watanabe, Katsue; Kawabata, Tomoko; Wada, Jun

2017-11-01

We herein describe two cases of refractory antineutrophil cytoplasmic antibody-associated vasculitis (AAV) complicated with diabetes insipidus (DI) possibly related to hypertrophic pachymeningitis (HP). One patient had microscopic polyangiitis and HP, which were refractory to cyclophosphamide, azathioprine, rituximab, mycophenolate mofetil (MMF), and mizoribine. Remission was finally achieved with the use of etanercept, but DI occurred 5 years later. The other patient had granulomatosis with polyangiitis, which that was refractory to cyclophosphamide, methotrexate, MMF, and rituximab. DI subsequently developed, but was successfully treated with etanercept. Dura mater hypertrophy was macroscopically observed in the latter case.

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

Anti-neutrophil cytoplasmic antibody-associated vasculitis with renal involvement: Analysis of 89 cases.

Science.gov (United States)

Caravaca-Fontán, Fernando; Yerovi, Estefanía; Delgado-Yagu E, María; Galeano, Cristina; Pampa-Saico, Saúl; Tenorio, Maria Teresa; Liaño, Fernando

2017-01-06

The anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis with renal involvement are associated with high morbi-mortality. In this study we analyse if the prognosis of these diseases have improved in recent years, and which factors influence the outcomes. Retrospective single-centre observational study, which included all patients diagnosed with microscopic polyangiitis and granulomatosis with polyangiitis with renal involvement in the last 25 years. Demographic, clinical and biochemical parameters of prognostic interest were recorded. The differences between four chronological periods were analysed, along with the determinants of a poor outcome (death or end-stage renal disease). Eighty-nine patients were included (mean age 64±15 years). Sixty-four patients (72%) had microscopic polyangiitis and 25 (28%) granulomatosis with polyangiitis. During the study period, 37 (42%) patients died. Through Cox regression analysis, the best determinants of mortality were the initial glomerular filtration rate (HR 0.911; P=.003), Charlson comorbidity index (HR 1.513; P<.0001) and tobacco smoking (HR 1.816; P=.003). 35% developed end-stage renal disease, and the best determinants (by competing-risk regression) were: initial glomerular filtration rate (sub-hazard ratio [SHR]: 0.791; P<.0001), proteinuria (SHR: 1.313; P<.0001), and smoking status (SHR: 1.848; P=.023). No differences were found in patients' mortality or renal survival between the different study periods. Prognosis of anti-neutrophil cytoplasm antibodies vasculitis with renal involvement treated with conventional immunosuppressive therapy remains unsatisfactory, and continues to have increased long-term complications and mortality. Copyright © 2016 Elsevier España, S.L.U. All rights reserved.

External therapy with radioactive urine: a pitfall during sceletal scintigraphy; Heisse Eigenurintherapie: ein ueberraschender Befund bei der Skelettszintigraphie

Energy Technology Data Exchange (ETDEWEB)

Scheidhauer, K.; Urbannek, V. [Klinik und Poliklinik fuer Nuklearmedizin, Univ. Koeln (Germany)

1997-12-01

A 41-year old male patient suffering from psoriasis arthropathica underwent a two-phase bone scan for activity of joint affections. Extensive diffuse skin contamination of the extremities in the delayed images was due to the use of the patient`s own radioactive urine as an embrocation for psoriasis exanthema. (orig.) [Deutsch] Bei einem 41jaehrigen Patienten mit Psoriasisarthropathie fiel in der Skelettszintigraphie in den Aufnahmen der Mineralisationsphase eine flaechige Aktivitaetsbelegung der Beine auf. Als Ursache stellte sich das zwischenzeitliche Einreiben der Extremitaeten durch den Patienten mit dem radioaktiven Eigenurin wegen eines Exantherms heraus. (orig.)

Energy as a technology: Thermodynamics, energy engineering, environment, renewable energy, rational energy use. 2. rev. ed.; Technologie Energie: Thermodynamik, Energietechnik, Umwelt, regenerative Energien, rationeller Energieeinsatz

Energy Technology Data Exchange (ETDEWEB)

Schuberth, R.

2000-07-01

The book discusses the technical and scientific fundamentals and the ecological consequences of anthropogenic energy conversion. The information is to make the public energy discussion more rational and less emotional. [German] Trotz oder gerade wegen der duesteren Zukunftsaussichten sind sachliche und fundierte Informationen ueber die Zusammenhaenge zu diesem Thema erforderlich. In diesem Buch werden schwerpunktmaessig die technisch-naturwissenschaftlichen Sachverhalte und die oekologischen Folgeerscheinungen, die im Zusammenhang mit der anthropogenen Energieumwandlung auftreten, dagestellt. Diese Kenntnisse sollten auch zu einer Versachlichung der Energiediskussion und einer rationaleren Beurteilung der Situation fuehren koennen. (orig.)

The cloud-phase feedback in the Super-parameterized Community Earth System Model

Science.gov (United States)

Burt, M. A.; Randall, D. A.

2016-12-01

Recent comparisons of observations and climate model simulations by I. Tan and colleagues have suggested that the Wegener-Bergeron-Findeisen (WBF) process tends to be too active in climate models, making too much cloud ice, and resulting in an exaggerated negative cloud-phase feedback on climate change. We explore the WBF process and its effect on shortwave cloud forcing in present-day and future climate simulations with the Community Earth System Model, and its super-parameterized counterpart. Results show that SP-CESM has much less cloud ice and a weaker cloud-phase feedback than CESM.

Insights into the pathogenesis and clinicopathological spectrum of oral vegetable granuloma. Case series with literature review

Directory of Open Access Journals (Sweden)

Shankargouda Patil

2017-10-01

Full Text Available Oral vegetable granuloma represents an inflammatory lesion of foreign body origin resulting from the implantation of vegetable matter. Controversy regarding its pathogenesis is reflected by the various terminologies used to describe the lesion. Its diverse clinical presentations are due to variations in the antigenic potential of the vegetable material and the host response. As the diagnosis is solely histopathological, it is critical to differentiate vegetable granuloma from other oral granulomatous lesions like tuberculosis, sarcoidosis and Wegner’s granulomatosis. Here, we report six cases with the varied clinicopathological presentation of hyaline ring granulomas in association with different pathological lesions.

[Successful treatment of surgically induced necrotizing sclerokeratitis (SINS) with systemic immunosuppresive agents and amniotic membrane grafting].

Science.gov (United States)

Cordero-Coma, M; Franco-Benito, M; García-Ruiz-de-Morales, J M; Alonso-Orcajo, N; Del Barrio-Manso, I

2009-11-01

We report the case of a 74-year-old female who developed a necrotizing sclerokeratitis affecting her left eye after uncomplicated cataract surgery. She had no previous history of systemic autoimmune disease. Histopathology of the lesion revealed necrotic granulomatosis with an increased number of plasma cells. Surgically induced necrotizing sclerokeratitis (SINS) is a serious entity which requires prompt and aggressive therapy to prevent its potential devastating ocular consequences. Conjunctival resection and amniotic membrane grafting may be necessary to temporarily interrupt local immunologic events in severe cases. However, associated systemic immunomodulatory therapy seems to be mandatory (Arch Soc Esp Oftalmol 2009; 84: 577-580).

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.

ESSReS-PEP, an international and interdisciplinary postgraduate education concept on Earth and Environmental Sciences

Science.gov (United States)

Grosfeld, Klaus; Lohmann, Gerrit; Ladstätter-Weißenmayer, Annette; Burrows, John

2013-04-01

Promoting young researchers is a major priority of the German Helmholtz Association. Since more than five years graduate and postgraduate education in the field of Earth System and Environmental Science has been established in Bremen and Bremerhaven, north-western Germany. Using the network and collaboration of experts and specialists on observational and paleoclimate data as well as on statistical data analysis and climate modelling from two Universities and the Helmholtz research institute on Polar and Marine Research, master and PhD students are trained to understand, decipher and cope with the challenges of recent climate change on an highly interdisciplinary and inter-institutional level. The existing research infrastructure at the Alfred Wegener Institute in Bremerhaven (AWI), University of Bremen, and Jacobs University Bremen offers a unique research environment to study past, present and future changes of the climate system, with special focus on high latitudinal processes. It covers all kind of disciplines, climate science, geosciences and biosciences, and provides a consistent framework for education and qualification of a new generation of expertly trained, internationally competitive master and PhD students. On postgraduate level, the Postgraduate Programme Environmental Physics (PEP) at the University of Bremen (www.pep.uni-bremen.de) educates the participants on the complex relationship between atmosphere, hydrosphere (ocean), cryosphere (ice region) and solid earth (land). Here, the learning of experimental methods in environmental physics at the most advanced level, numerical data analysis using supercomputers, and data interpretation via sophisticated methods prepare students for a scientific career. Within cooperation with the Ocean University of China (OUC) students are participating one year in the PEP programme during their master studies since 2006, to get finally a double degree of both universities. At the Alfred Wegener Institute for Polar

Towards new roads in environmental policy; Naar nieuwe wegen in het milieubeleid

Energy Technology Data Exchange (ETDEWEB)

NONE

2003-07-01

From this report it appears that the environmental policy in the Netherlands is not sufficient to deal with the environmental problems that will occur in the (near) future. In a separate report six cases are presented and discussed which illustrate the results of this report. [Dutch] Het beleid van de Nederlandse overheid is niet afdoende voor de milieuproblemen die zich in de (nabije) toekomst zullen voordoen. Voor nog openstaande en nieuwe milieuproblemen, zoals de problematiek van de klimaatverandering en de afnemende biodiversiteit, kan niet worden uitgegaan van de bestaande, vertrouwde beleidsconcepten. Deze pasten wel bij de zintuiglijk waarneembare milieuproblemen, zoals stank, geluidshinder of verontreinigd zwemwater. Maar bij de nieuwe problemen doen de effecten zich niet hier en nu voor, en is er daardoor nog geen gevoel van maatschappelijke urgentie. Immers, niemand merkt er nu nog wat van. Kennis over wat er mis kan gaan moet worden gemobiliseerd, en die kennis moet breed maatschappelijk worden gedeeld en gedragen. Het beleid moet dit zien te bereiken, omdat anders de milieubelangen op langere termijn het steeds zullen afleggen tegen andere belangen. Dit is de kern van het rapport. Het nationale milieubeleid staat meer dan voorheen voor de opgave om 'voor de fout uit te leren'. Dit vraagt om doordacht overheidshandelen voordat de nadelige effecten zich daadwerkelijk, voor het grote publiek zichtbaar, manifesteren. Deze opgave stelt hoge eisen aan de maatschappelijke legitimatie van milieubeleid, terwijl het gevoel van maatschappelijke urgentie van milieuproblemen steeds verder afneemt. Deze kwetsbaarheid in het huidige en komende milieubeleid wordt nog onvoldoende onderkend. Voor de vorming van nieuw milieubeleid zijn volgens de raad kennisontwikkeling, maatschappelijke inbedding van die kennis, en een goede schakeling tussen het nationale en internationale - vooral Europese - niveau van belang. Ten aanzien van het eerste punt dient de inbreng van wetenschappelijk hoogwaardige, onafhankelijke kennis over de nieuwe generatie milieuproblemen te worden vergroot. In de tweede plaats is een vertaling van die abstracte milieukennis naar de belevingswereld van de burgers noodzakelijk. In de derde plaats is van groot belang dat de Nederlandse inbreng in het internationale besluitvormingsproces adequaat geschiedt. Daar schort het nog wel aan. Zo worden in Europa richtlijnen aanvaard zonder dat in Nederland een goed beeld bestaat van de consequenties die dat heeft. De nitraatrichtlijn is daarvan een pijnlijk voorbeeld: de mestproblematiek van de boeren is volstrekt niet overzien toen de richtlijn tot stand kwam. De 'scharnierfunctie' tussen nationaal en internationaal beleid moet daarom worden versterkt. In een aparte publikatie (werkdocument) worden zes cases behandeld die in relatie staan tot de uitkomsten van dit rapport.

The three lost millennia of the last deglaciation (Alfred Wegener Medal Lecture)

Science.gov (United States)

Bard, Edouard

2013-04-01

Looking back more than thirty years, climate history over the last period of deglaciation was seen to portray a smooth transition between the last glacial maximum (LGM) centered around 18,000 years ago (based on radiocarbon), and the beginning of the Holocene at about 10,000 years before present. At that time, the renowned CLIMAP group used the stratigraphy available to reconstruct the glacial world by averaging paleothermometric data over a wide time window, ranging between at least 14,000 and 24,000 yr BP, over which period climate was assumed to be rather stable. Even if northern European pollen records showed several phases of vegetation shift, the exact duration and spatial coverage of these shifts was unknown and their climatic significance was not well-enough understood to be separated from other biological effects, such as plant migration following ice-sheet demise. Significant progress came from mass spectrometry developments applied to isotope geochronology in the mid- and late- 1980s. This allowed the precise analysis of radiocarbon on small samples such as foraminifera in marine sediments and enabled the measurement of U-Th ages for accurate dating of corals and speleothems. These technological improvements permitted meaningful comparisons between proxy records from the various archives originating from all latitudes and longitudes. Today, it is clear that the old LGM time window corresponds to a period of more than ten millennia during which there was significant climate variability, including a prominent cooling event at the beginning of the deglaciation. This cooling event is known as the 'Oldest Dryas' by palynologists, as 'Heinrich Event #1' (H1) by paleoceanographers, and has even been dubbed the 'Mystery Interval' by prominent authors as they puzzled while attempting to synthesize and interpret its records. The H1 drastic cooling, attributed to a pulse-like injection of ice and meltwater into the North Atlantic, was first evidenced in 1987 in sediments from the Iberian Margin. Three years later, significant improvements of the radiocarbon calibration demonstrated that about three millennia were missing from the deglaciation record. Accordingly, the LGM mean age was pushed from 18,000 to 21,000 yr BP, the midpoint of H1 was shifted from 13,500 to 16,000 yr BP, and the beginning of the Holocene was repositioned at about 11,500 yr BP. This new climate chronology was subsequently confirmed by counting 'cryovarves' within the GRIP and GISP2 Greenland ice cores. These studies have since been complemented by many other records from polar ice, marine and lacustrine sediments and cave speleothems. In addition to extending the chronology by three additional millennia, improvement also arose from the quality of the new geological archives. These archives have allowed studies at much higher resolution than was previously achieved in the framework of CLIMAP, which included many records based on deep-sea cores characterized by low sedimentation rate, and thus very susceptible to smoothing processes such as bioturbation. In addition, analytical geochemistry has only recently provided techniques adapted to the production of high-resolution time series of various proxies based on elemental ratios, on organic compounds or on stable and radiogenic isotopes. More than a dozen years after the H1 discovery, the same Iberian Margin sediments were used to show that H1 comprised at least two phases, H1a and H1b, based on ice rafted debris (IRD) and other proxies. It is now recognized that the entire H1 event (H1 sensu lato) is a three millennia-long period (ca. 17,500 to 14,500 yr BP). To illustrate the progress in this research field, I will review the key records that can be used to document the complex nature of this episode. The H1 (s.l.) included several phases of intense cooling, of precipitation changes - notably at low latitudes and in the Asian monsoon area, of retreat and decay of glacial ice-sheets - as evidenced in sediments collected in river mouths, and of sea-level rise as recorded in corals from Tahiti and Barbados. Various isotopic proxies of deep-sea ventilation have been used to identify variations during the H1 sub-phases of the Meridional Overturning Circulation (MOC), indicating that ocean heat transport was involved in the observed climate fluctuations. The various records documenting different climate parameters at many locations over the Earth can also be used in meaningful comparison with numerical model simulations performed in a transient mode. Collectively, these works allow to estimate the phase relationships between the causes (insolation and the greenhouse effect) and the often abrupt responses of the various components of the climate system, such as the atmosphere, oceans and ice sheets. Although these studies concern a naturally-occurring global warming that took place over a long time period, useful parallels will be drawn with the evolution of modern climate. In fact, the phase relationships between forcings (such as greenhouse gases and solar input) and changes in regional and global temperatures are also at the heart of modern global climate change. As for early deglaciation, the ocean can modulate warming regionally, thereby delaying, or even temporarily masking, long-term changes. Climate changes over the last century have been smaller in magnitude than those of the last deglaciation. Fortunately for us, there has been no recent collapse of gigantic ice masses such as the Laurentian and Fennoscandian ice sheets. However, most climate models show a 20 to 40% reduction of the MOC during the 21st century. Even if this change exerts only a minor influence on the projected magnitude of global warming, such a slowdown in ocean circulation change is generally sufficient to reduce the simulated warming over the North Atlantic with a resulting impact on adjacent continents, including Europe.

Cutaneous granulomatosis and combined immunodeficiency revealing Ataxia-Telangiectasia: a case report

OpenAIRE

Folgori, Laura; Scarselli, Alessia; Angelino, Giulia; Ferrari, Francesca; Antoccia, Antonio; Chessa, Luciana; Finocchi, Andrea

2010-01-01

Abstract Ataxia-telangiectasia (A-T) is a complex multisystem disorder characterized by progressive neurological impairment, variable immunodeficiency and oculo-cutaneous telangiectasia. A-T is a member of chromosomal breakage syndromes and it is caused by a mutation in the ataxia-telangiectasia mutated (ATM) gene. Because of a wide clinical heterogeneity, A-T is often difficult to diagnose in children. We report an unusual case of a 3-year-old boy affected by A-T who presented exclusively wi...

Granulomatosis with Polyangiitis Presenting as Pauci-Immune Crescentic Glomerulonephritis in Pregnancy

OpenAIRE

Kunjal, Ryan; Makary, Raafat; Poenariu, Andreea

2016-01-01

Antineutrophil cytoplasmic antibody (ANCA) associated vasculitis rarely affects females of reproductive age. A 28-year-old African American woman presented at 8 weeks of gestation with intractable vomiting attributed to hyperemesis gravidarum. She was found to have acute kidney injury that was unresponsive to vigorous fluid resuscitation and urine sediment examination was suggestive of an underlying glomerulonephritis. Serum c-ANCA and PR3 were elevated and there was no peripheral eosinophili...

Clean air litigation; Klagen fuer Saubere Luft

Energy Technology Data Exchange (ETDEWEB)

NONE

2016-11-11

The pollution of air by nitrogen dioxide (NO{sub 2}) in cities is one of the central challenges of air pollution in Germany, in addition to the pollution of particulate matter (PM10). In cooperation with the British non-governmental organization ClientEarth, the German Environmental Aid (DUH) filed a lawsuit in November 2015 for exceeding air quality limits for NO{sub 2}. The cities of Stuttgart, Frankfurt, Duesseldorf, Essen, Gelsenkirchen, Aachen, Cologne and Bonn are affected. Here, citizens are constantly exposed to high air pollution. Because of the excess of the NO{sub 2} limits at all traffic-related measuring stations in Berlin, the DUH initiated legal steps and filed a lawsuit too in June 2016. With the lawsuits, DUH wants to promote the implementation of measures to reduce NO{sub 2} in Germany. [German] Die Belastung der Luft durch Stickstoffdioxid (NO{sub 2}) in Staedten ist neben der Belastung durch Feinstaub (PM10) eine der zentralen Herausforderungen der Luftreinhaltung in Deutschland. In Zusammenarbeit mit der britischen Nichtregierungsorganisation ClientEarth legte die Deutsche Umwelthilfe (DUH) im November 2015 Klage wegen Ueberschreitung der Luftqualitaetsgrenzwerte fuer NO{sub 2}ein. Betroffen sind die Staedte Stuttgart, Frankfurt, Duesseldorf, Essen, Gelsenkirchen, Aachen, Koeln und Bonn. Hier sind Buergerinnen und Buerger anhaltend zu hoher Luftverschmutzung ausgesetzt. Wegen Ueberschreitung der NO{sub 2}-Grenzwerte an allen verkehrsnahen Messstationen in Berlin hat die DUH im Juni 2016 auch hier rechtliche Schritte eingeleitet und Klage eingereicht. Mit den Klagen will die DUH die Umsetzung von Massnahmen zur NO{sub 2}-Reduktion in Deutschland voranbringen.

Outcome of Renal Transplant in Recipients With Vasculitis.

Science.gov (United States)

Barbouch, Samia; Hajji, Meriam; Aoudia, Raja; Ounissi, Monther; Zammouri, Asma; Goucha, Rym; Ben Hamida, Fathi; Bacha, Mohammed Mongi; Abderrahim, Ezzedine; Ben Abdallah, Taieb

2017-02-01

End-stage renal disease develops in a high percentage of patients with vasculitis, in whom kidney transplant has become a therapeutic option. However, limited data are available on the prognosis and outcomes after kidney transplant in these patients. We aimed to compare the long-term graft survival and graft function in 8 renal transplant recipients with vasculitis (granulomatosis with polyangiitis, microscopic polyangiitis, Goodpasture syndrome, and Henoch-Schonlein purpura) with the other kidney recipients at a single center. We conducted a retrospective study of patients followed for chronic renal failure associated with vasculitis before renal transplant. We excluded patients with no biopsy-proven nephropathy. There was no difference in the occurrence of metabolic and cardiovascular complications in our case group compared with the other graft recipients. Infections were frequent and included cytomegalovirus and urinary tract infection. The rates of bacterial and viral infection were equivalent in our population. The incidence of allograft loss was estimated at 1.8%, less than that seen in our entire transplant population. The presence of vasculitis was not significantly related to renal failure (P = .07). Extrarenal relapse occurred in 1 patient with microscopic polyangiitis. Antineutrophil cytoplasmic antibody levels in patients with granulomatosis with polyangiitis and microscopic polyangiitis did not seem to influence the renal outcome (P = .08). Circulating antineutrophil cytoplasmic antibodies were associated with the development of vascular lesions in the graft but were not significantly correlated with graft survival (P = .07). This study supports the theory that renal transplant is an effective treatment option for patients with end-stage renal disease secondary to vasculitis. These patients fare similarly to, if not better than, other patients.

Progranulin antibodies in autoimmune diseases.

Science.gov (United States)

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

2013-05-01

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

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

Science.gov (United States)

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

2016-01-01

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

Erinnerung und Geschichte – Ein früher Bericht aus dem Frauen-KZ Moringen 1936/37

Directory of Open Access Journals (Sweden)

Christa Schikorra

2007-03-01

Full Text Available Der Erinnerungsbericht von Gabriele Herz ist einerseits bedeutsam wegen der Beschreibung individuelle Erfahrungen und der Einnahme einer persönlichen Perspektive. Andererseits stellt er ein bedeutendes Dokument für die Geschichtsschreibung zu frühen Konzentrationslagern dar, und hier insbesondere zu den Verfolgungserfahrungen jüdischer Frauen im Deutschland der 30er Jahre. In der äußerst aufschlussreichen Einleitung der Historikerin Jane Caplan, die den Anstoß für die Herausgabe dieses einzigartigen Dokuments gab, wird die Komplexität des Erinnerungszeugnisses von Gabriele Herz aufgezeigt und gewürdigt.

Planet earth a beginner's guide

CERN Document Server

Gribbin, John

2012-01-01

In this incredible expedition into the origins, workings, and evolution of our home planet, John Gribbin, bestselling author of In Search of Schrödinger's Cat, The Scientists, and In Search of the Multiverse, does what he does best: taking four and a half billion years of mind-boggling science and digging out the best bits. From the physics of Newton and the geology of Wegener, to the environmentalism of Lovelock, this is a must read for Earth's scientists and residents alike. Trained as an astrophysicist at Cambridge University, John Gribbin is currently Visiting Fellow in Astronomy at the University of Sussex, England.

CryoVex 2008

DEFF Research Database (Denmark)

Hvidegaard, Sine Munk; Forsberg, René; Skourup, Henriette

2009-01-01

Air Greenland. The main purpose was to collect coincident ASIRAS and laser elevation data form validation sites on land and sea ice and in addition offer logistical support to ground teams. Overflights of corner reflectors were done at main validation sites in order to calibrate the ASIRAS data....... The datasets from this campaign will be important for understanding the CryoSat-2 radar signals. The airborne part of CryoVEx 2008 was successfully carried out between April 15 and May 8 and the datasets have been stored and secured at DTU Space and Alfred Wegener Institute (AWI). Afterwards extensive data...

Subglotic Stenosis as manifestation in Wegener’s Disease. A case report

Directory of Open Access Journals (Sweden)

Sofía VALLE-OLSEN

2017-03-01

Full Text Available Introduction and objective: Subglottic stenosis in Wegener’s disease (WD represents a diagnostic challenge because it’s ability to be the unique manifestation. Description: A case of subglottic GW described in a woman 14 years. Discussion: Subglottic stenosis secondary to Wegener’s disease use to be associated with ENT symptoms, so these can give us the etiological suspicion. In the absence of these, we should include it in the differential diagnosis and try a histopathologic confirmation. Conclusions: In an isolated subglottic stenosis, rule Wegener's disease performing a biopsy of nasal mucosa and two serology tests.

Internationalisierung des Lehramts – eine Quadratur des Kreises? Zu Chancen und Grenzen eines grenzüberschreitenden Paradigmas der Lehrerbildung am Beispiel des Masterstudiengangs MEEF / LINT (Lehramt International Nizza-Regensburg

Directory of Open Access Journals (Sweden)

Jochen Mecke

2016-03-01

Full Text Available Die Internationalisierung ist nicht das Problem, sie ist die Lösung: In einer europäischen Gesellschaft, in der die Dynamik einer neuen Vielfalt derzeit eine der wenigen Konstanten darstellt, die nationenübergreifend Bestand hat und keine Grenzen kennt, brauchen wir eine Lehrerbildung, die strukturell und konzeptionell so gesamteuropäisch angelegt ist wie ihre Herausforderungen. Wir brauchen trotz und wegen verschiedener bildungspolitischer Unterschiede und Hürden neue Ansätze der Kooperation und Vernetzung, deren Chancen und Herausforderungen in der Folge exemplarisch am binationalen Master of Education MEEF / LINT (Lehramt International Deutsch-Französisch skizziert werden sollen.

Addison's disease presenting with perimyocarditis.

Science.gov (United States)

Baranski Lamback, Elisa; Morandi, Grazia; Rapti, Eleni; Christov, Georgi; Brogan, Paul A; Hindmarsh, Peter

2018-01-26

Polyglandular autoimmune syndrome (PGA) and eosinophilic granulomatosis with polyangiitis (EGPA) do not seem to represent a coincidental association. A case of a 15-year-old boy is reported who presented with severe systemic inflammation, perimyocarditis and cardiogenic shock, in whom EGPA was initially suspected and later diagnosed with autoimmune adrenalitis with PGA. The severity of the systemic inflammation and perimyocarditis suggests a more widespread autoimmune-mediated process. Autoimmune adrenal insufficiency should be considered in all cases of pericarditis and perimyocarditis, especially when the severity of clinical manifestations exceeds the expected for the severity of the cardiac findings, as timely identification and prompt treatment may be life-saving.

Myocarditis in auto-immune or auto-inflammatory diseases.

Science.gov (United States)

Comarmond, Cloé; Cacoub, Patrice

2017-08-01

Myocarditis is a major cause of heart disease in young patients and a common precursor of heart failure due to dilated cardiomyopathy. Some auto-immune and/or auto-inflammatory diseases may be accompanied by myocarditis, such as sarcoidosis, Behçet's disease, eosinophilic granulomatosis with polyangiitis, myositis, and systemic lupus erythematosus. However, data concerning myocarditis in such auto-immune and/or auto-inflammatory diseases are sparse. New therapeutic strategies should better target the modulation of the immune system, depending on the phase of the disease and the type of underlying auto-immune and/or auto-inflammatory disease. Copyright © 2017 Elsevier B.V. All rights reserved.

A foreign body granuloma of the stomach: a case report

International Nuclear Information System (INIS)

Rhee, Gwang Woo; Kim, Inn; Sohn, Hyung Kook; Koh, Byung Hee; Joo, Kyung Bin; Kim, Soon Yong; Park, Kyung NAm

1987-01-01

Granulomatous inflammation of the stomach similar to gastric neoplasm may occur in disseminated sarcoidosis, allergic granulomatosis. Crohn's disease, a variety of infectious disease, as a reaction to foreign material, and in the absence of any of the above as isolated granulomatous gastritis. In the foreign body induced granulomatous inflammation of the stomach, beryllium, kaolin, talcum, suture material and food can be causative materials. It is the intent of this case report to add to the list of more commonly occurring intramural gastric masses, the remote possibility of a foreign body granuloma. So radiologist should be aware of this entity and include it in their differential diagnosis of intramural gastric masses.

On the wrong track due to fine dust?; Wegen Feinstaub auf dem Holzweg?

Energy Technology Data Exchange (ETDEWEB)

Anon.

2008-07-01

Emission: The debate about the fine dust caused by wood furnaces causes a lot of excitement and emotions. Possibilities already exist today to solve this problem effectively. Solutions for plant technology and fuels with high quality offer sufficient approaches in order to allay the uncertainty of many end customers. (orig.)

Die DDC auf neuen Wegen - verbale Sucheinstiege für klassifikatorisch erschlossene Titel

Directory of Open Access Journals (Sweden)

Christiane Maibach

2014-12-01

Full Text Available Die Dewey-Dezimalklassifikation (DDC ist ein mächtiges Instrument der klassifikatorischen Inhaltserschließung. Immer mehr Bibliotheken im In- und Ausland erkennen den Nutzen der international am weitesten verbreiteten Universalklassifikation. Die Deutsche Nationalbibliothek (DNB setzt die DDC seit 2006 für die inhaltliche Erschließung der Bibliografiereihen A, B und H ein. Seitdem wurden rund 800.000 Publikationen mit DDC-Notationen versehen. Der Nutzen von Klassifikationen ist unter Bibliothekar/innen unumstritten. Die Bibliotheksnutzer/innen hingegen wissen oft nicht, dass der Katalog ihrer Bibliothek auch eine systematische Recherche ermöglicht. Ihre Recherchestrategie ist meist verbal. Durch Internetsuchmaschinen wird diese Strategie noch bestärkt. Daher liegt es nahe, auch für die klassifikatorisch erschlossenen Titel einen verbalen Sucheinstieg zu schaffen. Die DDC enthält nicht nur die in vielen Klassifikationssystemen üblichen Registereinträge und Klassenbenennungen, sondern auch die im Rahmen des Projekts CrissCross in großem Umfang erstellten Verknüpfungen zu Schlagwörtern der Gemeinsamen Normdatei (GND, die für diesen Zweck ausgewertet werden können. The Dewey Decimal Classification (DDC is a powerful indexing tool. A rising number of libraries worldwide recognize the benefit of this universal classification. Since 2006 the German National Library (DNB has used the DDC to index titles belonging to series A, B and H of the German National Bibliography. In 2012 100,000 publications were indexed with DDC notations. The benefits of classifications for indexing is widely accepted among librarians. However, library users take a different point of view. Mostly, they are not even aware that their library's catalogue offers a systematic search. Most library users prefer verbal search strategies. This is enforced by internet search engines, which have changed the search habits of library users. Therefore, the obvious solution is to create a verbal access to classified titles. The DDC does not only contain a relative index with verbal elements and class names, but also links to subject headings taken from the German Integrated Authority File (GND. These were established as a part of the CrissCross project. All of these verbal elements can be used to make the DDC accessible by words.

Die DDC auf neuen Wegen - verbale Sucheinstiege für klassifikatorisch erschlossene Titel

OpenAIRE

Christiane Maibach

2014-01-01

Die Dewey-Dezimalklassifikation (DDC) ist ein mächtiges Instrument der klassifikatorischen Inhaltserschließung. Immer mehr Bibliotheken im In- und Ausland erkennen den Nutzen der international am weitesten verbreiteten Universalklassifikation. Die Deutsche Nationalbibliothek (DNB) setzt die DDC seit 2006 für die inhaltliche Erschließung der Bibliografiereihen A, B und H ein. Seitdem wurden rund 800.000 Publikationen mit DDC-Notationen versehen. Der Nutzen von Klassifikationen ist unter Biblio...

Blijvend vlakke wegen - CROW-werkgroep integraal wegontwerp - evaluatie van schadecases uit de praktijk

NARCIS (Netherlands)

Egmond-Weijburg, C.; Galjaard, P.J.; Van Gurp, C.A.P.M.

2008-01-01

This report presents an inquiry into cases of pavement design, construction and maintenance in which distress has been observed instigated by deeper subgrade or by inadequate attention for the interaction between subgrade and pavement structure. Almost all cases are situated in areas with subgrade

Classification of Arctic, midlatitude and tropical clouds in the mixed-phase temperature regime

Science.gov (United States)

Costa, Anja; Meyer, Jessica; Afchine, Armin; Luebke, Anna; Günther, Gebhard; Dorsey, James R.; Gallagher, Martin W.; Ehrlich, Andre; Wendisch, Manfred; Baumgardner, Darrel; Wex, Heike; Krämer, Martina

2017-10-01

The degree of glaciation of mixed-phase clouds constitutes one of the largest uncertainties in climate prediction. In order to better understand cloud glaciation, cloud spectrometer observations are presented in this paper, which were made in the mixed-phase temperature regime between 0 and -38 °C (273 to 235 K), where cloud particles can either be frozen or liquid. The extensive data set covers four airborne field campaigns providing a total of 139 000 1 Hz data points (38.6 h within clouds) over Arctic, midlatitude and tropical regions. We develop algorithms, combining the information on number concentration, size and asphericity of the observed cloud particles to classify four cloud types: liquid clouds, clouds in which liquid droplets and ice crystals coexist, fully glaciated clouds after the Wegener-Bergeron-Findeisen process and clouds where secondary ice formation occurred. We quantify the occurrence of these cloud groups depending on the geographical region and temperature and find that liquid clouds dominate our measurements during the Arctic spring, while clouds dominated by the Wegener-Bergeron-Findeisen process are most common in midlatitude spring. The coexistence of liquid water and ice crystals is found over the whole mixed-phase temperature range in tropical convective towers in the dry season. Secondary ice is found at midlatitudes at -5 to -10 °C (268 to 263 K) and at higher altitudes, i.e. lower temperatures in the tropics. The distribution of the cloud types with decreasing temperature is shown to be consistent with the theory of evolution of mixed-phase clouds. With this study, we aim to contribute to a large statistical database on cloud types in the mixed-phase temperature regime.

NETs: The missing link between cell death and systemic autoimmune diseases?

Directory of Open Access Journals (Sweden)

Felipe eAndrade

2013-01-01

Full Text Available For almost 20 years, apoptosis and secondary necrosis have been considered the major source of autoantigens and endogenous adjuvants in the pathogenic model of systemic autoimmune diseases. This focus is justified in part because initial evidence in systemic lupus erythematosus (SLE guided investigators toward the study of apoptosis, but also because other forms of cell death were unknown. To date, it is known that many other forms of cell death occur, and that they vary in their capacity to stimulate as well as inhibit the immune system. Among these, NETosis (an antimicrobial form of death in neutrophils in which nuclear material is extruded from the cell forming extracellular traps, is gaining major interest as a process that may trigger some of the immune features found in SLE, granulomatosis with polyangiitis (formerly Wegener’s granulomatosis and Felty’s syndrome. Although there have been volumes of very compelling studies published on the role of cell death in autoimmunity, no unifying theory has been adopted nor have any successful therapeutics been developed based on this important pathway. The recent inclusion of NETosis into the pathogenic model of autoimmune diseases certainly adds novel insights into this paradigm, but also reveals a previously unappreciated level of complexity and raises many new questions. This review discusses the role of cell death in systemic autoimmune diseases with a focus on apoptosis and NETosis, highlights the current short comings in our understanding of the vast complexity of cell death, and considers the potential shift in the cell death paradigm in autoimmunity. Understanding this complexity is critical in order to develop tools to clearly define the death pathways that are active in systemic autoimmune diseases, identify drivers of disease propagation, and develop novel therapeutics.

Antineutrophil cytoplasmic antibody-associated vasculitides and IgG4-related disease: A new overlap syndrome.

Science.gov (United States)

Danlos, François-Xavier; Rossi, Giovanni Maria; Blockmans, Daniel; Emmi, Giacomo; Kronbichler, Andreas; Durupt, Stéphane; Maynard, Claire; Luca, Luminita; Garrouste, Cyril; Lioger, Bertrand; Mourot-Cottet, Rachel; Dhote, Robin; Arlet, Jean-Benoit; Hanslik, Thomas; Rouvier, Philippe; Ebbo, Mikael; Puéchal, Xavier; Nochy, Dominique; Carlotti, Agnès; Mouthon, Luc; Guillevin, Loïc; Vaglio, Augusto; Terrier, Benjamin

2017-10-01

Atypical manifestations have been described in patients with ANCA-associated vasculitides (AAV), such as pachymeningitis, orbital mass or chronic periaortitis. Because these manifestations have been associated to the spectrum of IgG4-related disease (IgG4-RD), we hypothesized that both diseases could overlap. We conducted a European retrospective multicenter observational study including patients fulfilling ACR and Chapel Hill criteria for AAV and IgG4-RD Comprehensive Diagnostic Criteria. Eighteen patients were included (median age 55.5years, 13 men). AAV and IgG4-RD were diagnosed concomitantly in 13/18 (72%) patients; AAV preceded IgG4-RD in 3/18 (17%) while IgG4-RD preceded AAV in 2/18 (11%). AAV diagnoses included granulomatosis with polyangiitis in 14 (78%), microscopic polyangiitis in 3 (17%), and eosinophilic granulomatosis with polyangiitis in one case. IgG4-RD diagnosis included definite IgG4-RD in 5 (28%) cases, probable IgG4-RD in 5 (28%) and possible IgG4-RD in 8 (44%). IgG4-RD manifestations were chronic periaortitis in 9/18 (50%) patients, orbital mass and tubulointerstitial nephritis in 4 (22%) cases, prevertebral fibrosis in 3 (17%), pachymeningitis and autoimmune pancreatitis in 2 (11%) cases. Patients required median number of 2 (range 0-4) lines of immunosuppressants in combination with glucocorticoids. During the follow-up (median 49,8months, range 17,25-108months), AAV manifestations relapsed in 10/18 (56%) cases and IgG4-RD lesions in 5/18 (28%). When used, mainly for relapses, rituximab showed response in all cases. AAV and IgG4-RD may overlap. Clinicians should consider that atypical manifestations during AAV could be related to IgG4-RD rather than to refractory granulomatous or vasculitic lesions. Copyright © 2017 Elsevier B.V. All rights reserved.

GIS and geodatabases application to global scale plate tectonics modelling

OpenAIRE

Hochard, Cyril; Stampfli, Gérard

2008-01-01

Les reconstructions palinspastiques fournissent le cadre idéal à de nombreuses études géologiques, géographiques, océanographique ou climatiques. En tant qu’historiens de la terre, les “reconstructeurs” essayent d’en déchiffrer le passé. Depuis qu’ils savent que les continents bougent, les géologues essayent de retracer leur évolution à travers les âges. Si l’idée originale de Wegener était révolutionnaire au début du siècle passé, nous savons depuis le début des années « soixante » que les c...

GIS and geodatabases application to global scale plate tectonics modelling

OpenAIRE

Hochard, C.

2008-01-01

Les reconstructions palinspastiques fournissent le cadre idéal à de nombreuses études géologiques, géographiques, océanographique ou climatiques. En tant qu?historiens de la terre, les "reconstructeurs" essayent d?en déchiffrer le passé. Depuis qu?ils savent que les continents bougent, les géologues essayent de retracer leur évolution à travers les âges. Si l?idée originale de Wegener était révolutionnaire au début du siècle passé, nous savons depuis le début des années « soixante » que les c...

Interspinöse Implantate in der Wirbelsäulenchirurgie - eine Frage der Indikation: Ein Fallbericht

Directory of Open Access Journals (Sweden)

Decristoforo I

2009-01-01

Full Text Available Ein 77-jähriger Patient war wegen manifester neurogener Claudicatio spinalis bei bekannter lumbaler Spinalkanalstenose in 3 Segmenten an einer auswärtigen Institution mit perkutanen interspinösen Implantaten versorgt worden, wonach sich die klinische Symptomatik nicht änderte. Erst die mikrochirurgische Dekompression und die Entfernung der Platzhalter führten zu einer deutlichen Besserung der Beschwerden und Verlängerung der schmerzfreien Gehstrecke. Anhand dieses Falles wird die Verwendung von interspinösen Platzhaltern beleuchtet. In der bisherigen klinischen Anwendung fehlen noch die Langzeitergebnisse. Eine korrekte Indikationsstellung zur Intervention bei Spinalkanalstenose ist Voraussetzung für ein zufriedenstellendes postoperatives Ergebnis.

Airborne observations of changes of ice sheet and sea ice in the Arctic using CryoVEx campaign data

DEFF Research Database (Denmark)

Hvidegaard, Sine Munk; Skourup, Henriette; Forsberg, René

measurements of ice sheet changes. The majority of the campaigns have been sponsored by the European Space Agency, ESA, as part of the CryoSat Validation Experiments – CryoVEx. These have been internationally coordinated efforts to collect coincident space‐borne, airborne, and in‐situ data for pre‐ and post...... cap (Svalbard), the EGIG line crossing the Greenland Ice Sheet, as well as the sea ice north of Alert and sea ice around Svalbard in the Fram Strait. Selected tracks were planned to match CryoSat‐2 passes and a few of them were flown in formation flight with the Alfred Wegener Institute (AWI) Polar‐5...

[Amateurism in Geology: the French Reception of the Continental Drift Theory (1920-1950)].

Science.gov (United States)

Le Vigouroux, Philippe; Gohau, Gabriel

At the beginning of the XXth century, Wegener proposed a theory – that of the roaming drift of the continents – unifying the rival theories of the Europeans and the Americans. As the work of a non-specialist who didn’t trouble himself with specific details, it raised numerous criticisms from specialists in various disciplines though others welcomed and supported it. Some even understood that despite its flaws, it started a new research program. Paradoxically, as regards its simplicity, nonspecialists – engineers, popularizers, secondary school teachers and even believers in para-sciences – gave it a favorable reception. Being amateurs, they continued to endorse it when specialists abandoned it.

Impact of pre-existing co-morbidities on mortality in granulomatosis with polyangiitis

DEFF Research Database (Denmark)

Faurschou, Mikkel; Ahlström, Magnus Glindvad; Lindhardsen, Jesper

2016-01-01

of pre-existing co-morbidities among the patients was quantified according to the Charlson Comorbidity Index (CCI). Each patient was matched with five age- and gender-matched population controls with no pre-existing co-morbidities captured by the CCI (CCI score = 0). The study subjects were followed...... throughout 2010. Cox regression analyses were used to calculate mortality rate ratios (MRRs). RESULTS: The median duration of follow-up in the GPA cohort was 5.8 years (interquartile range 2.3-10.0). Compared with their matched population controls, the MRR for patients presenting with a CCI score of 0 (n...

Pulmonary vasculitis: imaging features

International Nuclear Information System (INIS)

Seo, Joon Beom; Im, Jung Gi; Chung, Jin Wook; Goo, Jin Mo; Park, Jae Hyung; Yeon, Kyung Mo; Song, Jae Woo

1999-01-01

Vasculitis is defined as an inflammatory process involving blood vessels, and can lead to destruction of the vascular wall and ischemic damage to the organs supplied by these vessels. The lung is commonly affected. A number of attempts have been made to classify and organize pulmonary vasculitis, but because the clinical manifestations and pathologic features of the condition overlap considerably, these afforts have failed to achieve a consensus. We classified pulmonary vasculitis as belonging to either the angitiis-granulomatosis group, the diffuse pulmonary hemorrhage with capillaritis group, or 'other'. Characteristic radiographic and CT findings of the different types of pulmonary vasculitis are illustrated, with a brief discussion of the respective disease entities

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

Directory of Open Access Journals (Sweden)

Abhishek Agarwal

2018-01-01

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

[Pathomorphology of the intestine and regional lymphatic system in pseudotuberculosis].

Science.gov (United States)

Isachkoa, L M; Zhavoronkov, A A; Antonenko, F F; Timchenko, N F

1988-01-01

Available are data obtained at light and electron microscopy of operative specimens from patients with abdominal pseudotuberculosis and animals challenged orally with Yersinia pseudotuberculosis. The authors are the first to outline detailed characteristics of the intestinal and regional lymph node lesion arising in response to the infection and reflecting growing resistance to it. These features of pathological process involve marked tissue eosinophilia, necrosis due to phagocytes rexis, and granulomatosis suggesting a pronounced role in the pathogenesis of the body allergization in the course of infection. It is proposed to consider pseudotuberculosis-related changes in lymph nodes as lymphoblastic (early affection) and granulomatous-necrotic (advanced infection) lymphadenitis. The evidence obtained can promote differential diagnosis of pseudotuberculosis.

Sarcoid-like lung granulomas in a hemodialysis patient treated with a dipeptidyl peptidase-4 inhibitor.

Science.gov (United States)

Sada, Ken-Ei; Wada, Jun; Morinaga, Hiroshi; Tuchimochi, Shigeyuki; Uka, Mayu; Makino, Hirofumi

2014-04-01

It has been reported that the inhibition of dipeptidyl peptidase-4 (DPP-4)/CD26 on T-cells by DPP-4 enzymatic inhibitors suppresses lymphocyte proliferation and reduces the production of various cytokines, including tumor necrosis factor (TNF)-α. A 72-year-old female with diabetic nephropathy on hemodialysis developed multiple lung nodules following the administration of vildagliptin. A biopsy demonstrated the histology of granulomas without caseous necrosis. The discontinuation of vildagliptin resulted in the disappearance of the granulomas within 4 months. As granulomatosis often develops in patients under anti-TNF-α therapy, the accumulation of DPP-4 inhibitors or its metabolites is possibly linked to unrecognized complications, such as sarcoid-like lung granulomas.

A foreign body granuloma of the stomach: a case report

Energy Technology Data Exchange (ETDEWEB)

Rhee, Gwang Woo; Kim, Inn; Sohn, Hyung Kook; Koh, Byung Hee; Joo, Kyung Bin; Kim, Soon Yong; Park, Kyung NAm [Hanyang University School of Medicine, Seoul (Korea, Republic of)

1987-02-15

Granulomatous inflammation of the stomach similar to gastric neoplasm may occur in disseminated sarcoidosis, allergic granulomatosis. Crohn's disease, a variety of infectious disease, as a reaction to foreign material, and in the absence of any of the above as isolated granulomatous gastritis. In the foreign body induced granulomatous inflammation of the stomach, beryllium, kaolin, talcum, suture material and food can be causative materials. It is the intent of this case report to add to the list of more commonly occurring intramural gastric masses, the remote possibility of a foreign body granuloma. So radiologist should be aware of this entity and include it in their differential diagnosis of intramural gastric masses.

Chronicle pulmonary histoplasmosis

International Nuclear Information System (INIS)

Llanos, Elkin; Ojeda, Paulina

2004-01-01

Histoplasmosis is an acquired mycotic disease produced by the histoplasma capsulatum very frequent in Colombia, primarily affecting lungs. The pathogenesis of the histoplasmosis is similar to the one of tuberculosis. From the clinical point of view, this disease has several manifestations including the primary acute and chronic pulmonary forms. Histoplasmoma pulmonary disseminated histoplasmosis, mediastinal compromise due to granulomatosis and fibrosis, as well as ocular histoplasmosis. A clinical case of a 33-year old man is presented who consults for dry coughing of one year of evolution, without any other symptomatology, with a normal chest x-ray and after several studies including chest cat and fiber-bronchoscopy. A pulmonary histoplasmosis was determined by histopathology

Laterale 10-fach-Biopsie der Prostata liefert bei Karzinomverdacht überlegene Detektionsraten

Directory of Open Access Journals (Sweden)

von Knobloch R

2012-01-01

Full Text Available Einleitung:Die Sextantenbiopsie der Prostata ist für eine verlässliche Karzinomdiagnostik nicht ausreichend. Vielerorts werden bereits bei der Erstbiopsie 12 Stanzen entnommen. In einer großen Serie von 10-fach-Biopsien unter bilateraler lokaler Leitungsanästhesie, wie bereits 2002 publiziert [1], überprüften wir die Qualität unserer Technik als Standard für die Erstbiopsie. Methode:Zwischen April 2005 und Dezember 2007 führten wir bei 736 Männern (Durchschnittsalter 67,5 ± 8,39 Jahre die Erstbiopsie der Prostata durch. Indikation für die Biopsie war entweder ein erhöhter PSA-Wert oder ein auffälliger Tastbefund. Bei allen Männern erfolgte die Biopsie der Prostata unter bilateraler lokaler Leitungsanästhesie. Es wurden nur Proben aus der peripheren Zone der Prostata unter transrektaler Ultraschallkontrolle entnommen. Ergebnisse:Durch die Biopsie wurde in 372 von 736 Fällen (50,5 % ein Karzinom identifiziert. Die Karzinomdetektionsrate war proportional zum PSA-Wert und indirekt proportional zum Organvolumen. Es zeigte sich eine eindeutige Korrelation zwischen der Anzahl positiver Stanzen bei der Biopsie mit dem pathologischen Stadium des Prostatektomiepräparates. 12 Männer (1,6 % mussten wegen akuter Prostatitis und 5 (0,7 % wegen rektaler Blutung wieder ins Krankenhaus eingewiesen werden. Von den 182 Patienten des Untersuchers R. v. K. wurde der durchschnittliche Schmerz der Biopsie mit 1,69 von maximal 10 angegeben. Die Detektions- und Komplikationsraten dieser Biopsietechnik unterschieden sich nicht nach Durchführung von Assistenz- oder Fachärzten. Schlussfolgerung: Die präsentierte Biopsietechnik liefert mit nur 10 Stanzen eine überlegene Detektionsrate und eine geringe Rate an Komplikationen. Sie sollte als Basis für die Definition eines Standards für die Erstbiopsie der Prostata bei Karzinomverdacht dienen.

Zeitspiel ist keine Alternative - Warum der Wandel zur Pflicht wird

Science.gov (United States)

Dieper, Stephan

"Wege entstehen dadurch, dass man sie geht." (Franz Kafka) Die Welt der Digitalisierung ist voll von Wegen, die jemand gegangen ist, bevor dort ein Weg war. Manche dieser Wege stellten sich als Sackgasse heraus, manche als Abkürzung und aus anderen wurden ganze Wegenetze und Städte. Die Energiewelt wird durch den digitalen Wandel nicht verschont bleiben. Durch die intelligenten Messsysteme und die zugehörigen, neuen Strukturen werden energiefremden Wettbewerbern Chancen zum Markteintritt eröffnet. EVUs müssen sich darauf einstellen, dass der permanente Wandel nicht mehr enden wird. Doch auch den EVUs eröffnen sich Optionen. Um erfolgreich zu sein, müssen sie lernen loszugehen, ohne das genaue Ziel zu kennen.

DDR-Justiz und NS-Verbrechen Band 8 : Sammlung ostdeutscher Strafurteile wegen nationalsozialistischer Tötungsverbrechen

NARCIS (Netherlands)

Rüter, Christiaan F.

2006-01-01

Volume 8 contains the trial cases finalized between mid-May and the end of August 1949. Most of the cases in this volume involve denunciations, NS-crimes in detainment centers and final phase crimes. Also, there are some dealing with judicial crimes, mass extermination and war crimes, as well as

Autoimmune lymphoproliferative syndrome and non-Hodgkin lymphoma: what 18F-fluorodeoxyglucose positron emission tomography/computed tomography can do in the management of these patients? Suggestions from a case report.

Science.gov (United States)

Cistaro, A; Pazè, F; Durando, S; Cogoni, M; Faletti, R; Vesco, S; Vallero, S; Quartuccio, N; Treglia, G; Ramenghi, U

2014-01-01

A young patient with undefined autoimmune lymphoproliferative syndrome (ALPS-U) and low back pain underwent a CT and MRI study that showed enhancing vertebral lesions, some pulmonary nodules and diffuse latero-cervical lymphadenopathy. A (18)F-FDG-PET/CT scan showed many areas of intense (18)F-FDG uptake in multiple vertebrae, in some ribs, in the sacrum, in the liver, in both lungs, in multiple lymph nodes spread in the cervical, thoracic and abdominal chains. A bone marrow biopsy showed a "lymphomatoid granulomatosis", a rare variant of B-cell non-Hodgkin lymphoma (NHL). After the treatment, the (18)F-FDG-PET/CT scan showed a complete metabolic response. Copyright © 2013 Elsevier España, S.L. and SEMNIM. All rights reserved.

Ibrutinib in Treating Relapsed or Refractory B-Cell Non-Hodgkin Lymphoma in Patients With HIV Infection

Science.gov (United States)

2015-08-18

Adult B Acute Lymphoblastic Leukemia; Chronic Lymphocytic Leukemia; Cutaneous B-Cell Non-Hodgkin Lymphoma; Extranodal Marginal Zone Lymphoma of Mucosa-Associated Lymphoid Tissue; HIV Infection; Intraocular Lymphoma; Multicentric Angiofollicular Lymphoid Hyperplasia; Nodal Marginal Zone Lymphoma; Recurrent Adult Acute Lymphoblastic Leukemia; Recurrent Adult Burkitt Lymphoma; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Adult Diffuse Mixed Cell Lymphoma; Recurrent Adult Diffuse Small Cleaved Cell Lymphoma; Recurrent Adult Grade III Lymphomatoid Granulomatosis; Recurrent Adult Immunoblastic Lymphoma; Recurrent Adult Lymphoblastic Lymphoma; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Grade 3 Follicular Lymphoma; Recurrent Mantle Cell Lymphoma; Recurrent Marginal Zone Lymphoma; Recurrent Small Lymphocytic Lymphoma; Refractory Chronic Lymphocytic Leukemia; Refractory Hairy Cell Leukemia; Refractory Plasma Cell Myeloma; Small Intestinal Lymphoma; Splenic Marginal Zone Lymphoma; Testicular Lymphoma; Waldenstrom Macroglobulinemia

Looking in the mouth for Crohn's disease.

LENUS (Irish Health Repository)

Rowland, Marion

2012-02-01

It is widely acknowledged among gastroenterologists that the oral cavity may be involved in Crohn\\'s disease (CD). However, the specific manifestations are poorly appreciated. Although oral aphthous ulceration is probably not diagnostically useful in patients with suspected CD, disease-specific manifestations do occur and are particularly common in children presenting with CD. These manifestations can be subtle, often are subclinical, yet commonly harbor diagnostically useful material (granulomas). Orofacial granulomatosis (OFG) is conventionally used to describe patients with overt oral disease without obvious involvement of the gastrointestinal tract. However, many patients with OFG have subclinical intestinal CD or will progress to develop overt intestinal CD with time. The management of severe oral disease is challenging and lacks a clear evidence base.

Looking in the mouth for Crohn's disease.

LENUS (Irish Health Repository)

Rowland, Marion

2010-02-01

It is widely acknowledged among gastroenterologists that the oral cavity may be involved in Crohn\\'s disease (CD). However, the specific manifestations are poorly appreciated. Although oral aphthous ulceration is probably not diagnostically useful in patients with suspected CD, disease-specific manifestations do occur and are particularly common in children presenting with CD. These manifestations can be subtle, often are subclinical, yet commonly harbor diagnostically useful material (granulomas). Orofacial granulomatosis (OFG) is conventionally used to describe patients with overt oral disease without obvious involvement of the gastrointestinal tract. However, many patients with OFG have subclinical intestinal CD or will progress to develop overt intestinal CD with time. The management of severe oral disease is challenging and lacks a clear evidence base.

The radiographic findings of lymphoproliferative disorders of the lung

International Nuclear Information System (INIS)

Song Wei; Li Liping; Yan Hongzhen

2002-01-01

Objective: To study the radiographic findings of lymphoproliferative disorders of the lung. Methods: Twenty-five patients with lymphoproliferative disorders of the lung were examined by X-ray film, tomography, and CT. Results: Multiple and mediastinal lymphadenopathy were observed in 2 patients with pulmonary pseudolymphoma. Multiple nodules or masses were observed in 4 patients with pulmonary lymphomatoid granulomatosis. Hilar and mediastinal lymphadenopathy was observed in each patient with angioimmunoblastic lymphadenopathy, 2 patients had multiple nodules or masses, 8 patients had single or multiple patchy infiltrations, 10 had diffuse interstitial infiltrations. 3 patients with Castlemen' disease had a mass in the mediastinum, and another patient had mediastinal lymphadenopathy. Conclusion: Radiographic findings of lymphoproliferative disorders of the lung are varied, and the final diagnosis relies on pathology

Saddle-nose and bilateral cauliflower ear deformities with pyoderma gangrenosum-like ulcers, cavitary pulmonary lesions, digital gangrene and pulselessness in a young female.

Science.gov (United States)

Subhadarshani, Sweta; Gupta, Vishal; Chahal, Anurag; Verma, Kaushal K

2017-06-15

We report a young female who presented with saddle-nose and bilateral cauliflower ear deformities along with pyoderma gangrenosum-like ulcers, digital gangrene and pulselessness. Subsequently, she was found to have bilateral conductive hearing loss, a corneal opacity, mild aortic regurgitation and radiological evidence of cavitary changes in lungs and aortoarteritis. Our patient had a constellation of symptoms which posed a diagnostic challenge. Finally, a diagnosis of relapsing polychondritis with several unusual features was made. Overlap with Takayasu's arteritis and granulomatosis with polyangitis, which has been reported rarely in the literature, cannot be excluded. © BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

ORAL MANIFESTATIONS OF CROHN'S DISEASE: A CASE REPORT

Directory of Open Access Journals (Sweden)

Muhvić-Urek Miranda

2015-12-01

Full Text Available Crohn´s disease is a chronic inflammatory bowel disease still with unknown etiology. In 0.5-20 % of patients, extraintestinal lesions in the oral cavity can be presented in forms of orofacial granulomatosis, cobblestone and corrugated oral mucosa, mucosal tags, deep linear ulcerations with hyperplastic folds, pyostomatits vegetans, aphthous ulcers, angular cheilitis, labial/facial edema and gingival erythema/edema. We describe a case of a 28-year-old male who was presented with oral lesions of Crohn´s disease and treatment procedure. The patient was candidate for biologic treatment so dental procedures and preparation of the patient for treatment are described. Good communication and cooperation between the patient's doctor and dentist are important for successful treatment.

Vasculitis in patients with inflammatory bowel diseases: A study of 32 patients and systematic review of the literature.

Science.gov (United States)

Sy, Alice; Khalidi, Nader; Dehghan, Natasha; Barra, Lillian; Carette, Simon; Cuthbertson, David; Hoffman, Gary S; Koening, Curry L; Langford, Carol A; McAlear, Carol; Moreland, Larry; Monach, Paul A; Seo, Philip; Specks, Ulrich; Sreih, Antoine; Ytterberg, Steven R; Van Assche, Gert; Merkel, Peter A; Pagnoux, Christian

2016-02-01

Published small case series suggest that inflammatory bowel disease [IBD; Crohn's disease (CD) or ulcerative colitis (UC)] and vasculitis co-occur more frequently than would be expected by chance. To describe this association by an analysis of a large cohort of carefully studied patients and through a systematic literature review. Patients with both IBD and vasculitis enrolled in the Vasculitis Clinical Research Consortium (VCRC) Longitudinal Studies, followed in Canadian Vasculitis research network (CanVasc) centers and/or in the University of Toronto's IBD clinic were included in this case series. A systematic literature review of patients with IBD and vasculitis involved a PubMed search through February 2014. The main characteristics of patients with Takayasu arteritis (TAK) and IBD were compared to those in patients with TAK without IBD followed in the VCRC. The study identified 32 patients with IBD and vasculitis: 13 with large-vessel vasculitis [LVV; 12 with TAK, 1 with giant cell arteritis (GCA); 8 with CD, 5 with UC]; 8 with ANCA-associated vasculitis [AAV; 6 granulomatosis with polyangiitis (GPA), 2 with eosinophilic granulomatosis with polyangiitis (EGPA)]; 5 with isolated cutaneous vasculitis; and 6 with other vasculitides. Patients with LVV and AAV were mostly female (18/21). The diagnosis of IBD preceded that of vasculitis in 12/13 patients with LVV and 8/8 patients with AAV. The review of the literature identified 306 patients with IBD and vasculitis: 144 with LVV (133 TAK; 87 with IBD preceding LVV), 19 with AAV [14 GPA, 1 EGPA, 4 microscopic polyangiitis (MPA)], 66 with isolated cutaneous vasculitis, and 77 with other vasculitides. Patients with IBD and TAK were younger and had more frequent headaches, constitutional symptoms, or gastrointestinal symptoms compared to those patients in the VCRC who had TAK without IBD. These findings highlight the risk of vasculitis, especially TAK, in patients with IBD (both CD and UC). Copyright © 2015 Elsevier

How to adjust traffic management to air quality; Hoe kun je verkeersmanagement afstemmen op de kwaliteit van lucht?

Energy Technology Data Exchange (ETDEWEB)

Cornelissen, T. [Rijkswaterstaat, Den Haag (Netherlands)

2011-04-15

Matrix boards are used to manage traffic speed on roads. The Dutch InnovatieProgramma Luchtkwaliteit IPL (Air Quality Innovation Programme ) has looked at the option of using matrix boards to improve the air quality along the highways. The IPL has also examined other temporary measures that can be deployed at times of peak concentrations of PM10 and/or NO2. [Dutch] Met matrixborden kan op wegen de snelheid worden geregeld. In het InnovatieProgramma Luchtkwaliteit (IPL) is gekeken of men de matrixborden ook kan inzetten om de luchtkwaliteit langs de snelweg te verbeteren. Door het IPL is ook gezocht naar andere tijdelijke maatregelen die je in kunt zetten op die momenten dat er een piek in de concentraties aan PM10 en/of NO2 optreedt.

Process chain analysis for methanol production from biomass with the aid of mathematical models; Prozesskettenanalyse fuer die Methanolerzeugung aus Biomasse mit Hilfe von mathematischen Modellen

Energy Technology Data Exchange (ETDEWEB)

Saller, G; Krumm, W [Siegen Univ. (Gesamthochschule) (Germany)

1997-12-31

Using the example of primary energy consumption for methanol production from cellulose-rich biomass, it is shown that process chain analysis with mathematical modelling of the technical systems enables calculations of eco-inventories as a function of the relevant process parameters. Optimisation of process chains with regard to ecological indicators or cost necessitates mathematical modelling because of the many different parameters and parameter combinations involved. (orig) [Deutsch] Am Beispiel des Primaerenergieaufwands bei der Methanolgewinnung aus zellulosealtiger Biomasse wurde gezeigt, dass die Prozesskettenanalyse mit Abbildung der technischen Systeme in mathematischen Modellen die Berechnung von Oekoinventaren in Abhaengigkeit der relevanten Prozessparameter erlaubt. Die Optimierung von Prozessketten hinsichtlich oekologischer Indikatoren oder Kosten ist wegen der Vielzahl an unterschiedlichen Parametern und deren Kombinationsmoeglichkeiten nur mit Hilfe mathematischer Modelle moeglich. (orig)

Catálogo sistemático dos pólens das plantas arbóreas do Brasil Meridional: IV - Thymelaeaceae, Lythraceae, Lecythidaceae, Rhizophoraceae e Combretaceae

Directory of Open Access Journals (Sweden)

Ortrud Monika Barth

1965-01-01

Full Text Available Es wurden pollenmorphologisch diejenigen Arten der vorliegenden Familien untersucht, welche als Baeume in den suedbrasilianischen Waeldern auftreten, mit Ausnahme der krautigen Heimia myrtifolia. Gekennzeichnet durch drei einfache Colpori laesst sich die Gruppe von Buchenavia kleinii, Laguncularia racemosa (Combretaceae, Heimia myrtifolia (Lythraceae und Rhizophora mangle (Rhizophoraceae bilden. Wegen ihrer Pseudocolpi oder aehnlichen Laengsstreifen einer duenneren Sexine stellen die Pollenkoerner von Combretum futicosum, Terminalia autralis (Combretaceae und Lafoensia pacari (Lythraceae eine andere Gruppe dar. Daphnopsis (Thymelaeaceae besitzt 10 - 14 gleichmaessing verteilte Poren und eine Oerflaechenstruktur die der des Croton-Types der Euphorbiaceen sehr nahe kommt. Cariniana estrellensis (Lecythidaceae, mit nur drei Colpi, nimmt auch eine gesonderte Position ein. Es bestehen Beziehungen zwischen der Morphologie der Pollenkoerner der oben bearbeiteten Familien und jenen der Guttiferales und Rosales.

Process chain analysis for methanol production from biomass with the aid of mathematical models; Prozesskettenanalyse fuer die Methanolerzeugung aus Biomasse mit Hilfe von mathematischen Modellen

Energy Technology Data Exchange (ETDEWEB)

Saller, G.; Krumm, W. [Siegen Univ. (Gesamthochschule) (Germany)

1996-12-31

Using the example of primary energy consumption for methanol production from cellulose-rich biomass, it is shown that process chain analysis with mathematical modelling of the technical systems enables calculations of eco-inventories as a function of the relevant process parameters. Optimisation of process chains with regard to ecological indicators or cost necessitates mathematical modelling because of the many different parameters and parameter combinations involved. (orig) [Deutsch] Am Beispiel des Primaerenergieaufwands bei der Methanolgewinnung aus zellulosealtiger Biomasse wurde gezeigt, dass die Prozesskettenanalyse mit Abbildung der technischen Systeme in mathematischen Modellen die Berechnung von Oekoinventaren in Abhaengigkeit der relevanten Prozessparameter erlaubt. Die Optimierung von Prozessketten hinsichtlich oekologischer Indikatoren oder Kosten ist wegen der Vielzahl an unterschiedlichen Parametern und deren Kombinationsmoeglichkeiten nur mit Hilfe mathematischer Modelle moeglich. (orig)

Raw materials and energy - effects of globalization on the safety of German's supply sufficiency; Rohstoffe und Energie. Auswirkungen der Globalisierung auf die Versorgungssicherheit Deutschlands

Energy Technology Data Exchange (ETDEWEB)

Wellmer, F.W. [Technische Univ. Berlin (Germany)

2001-07-01

Raw material deposits are not mobile, so globalisation has always been an important element of raw materials production and trade. Raw materials production and transport should be regarded as a large-scale system. Lower freight rates have made overseas transport possible even for bulk raw materials. If we look at Germany's raw materials supply, we find that we are customers buying from the whole world. [German] Da Lagerstaetten ortsgebunden sind, ist Globalisierung seit jeher ein wesentliches Element von Rohstoffgewinnung und handel. Rohstoffgewinnung und Transportmoeglichkeiten muessen als Grosssystem betrachtet werden. Nicht zuletzt wegen der gesunkenen Frachtraten ist der ueberseeische Transport heute selbst fuer Massenrohstoffe moeglich geworden. Betrachtet man die Rohstoffversorgung Deutschlands, wird deutlich, dass wir Kunde der ganzen Welt sind. (orig.)

Osteoporose und Laktoseintoleranz

Directory of Open Access Journals (Sweden)

Obermayer-Pietsch B

2008-01-01

Full Text Available Laktosemalabsorption oder Laktoseintoleranz ist eine symptomatische Unverträglichkeit von Milchzucker (Laktose, zumeist aufgrund einer autosomal rezessiv erblichen, irreversiblen Abnahme des Enzyms Laktase im Jugendalter, die bei etwa der Hälfte der Weltbevölkerung auftritt. Die Betroffenen meiden oft instinktiv Milch und Milchprodukte wegen der damit verbundenen Malabsorptions-Symptome. Damit scheiden diese Nahrungsmittel aber auch als Kalziumlieferanten aus und es kann zu einer Mangelversorgung des Knochens mit Kalzium und damit zu Osteoporose kommen. Da in Österreich etwa 20–25 % der Bevölkerung betroffen sind, ist diese Stoffwechselveränderung nicht zu vernachlässigen. Diagnostische und begleitende ernährungsmedizinische Maßnahmen und gegebenenfalls eine osteotrope Therapie sollten zum Erhalt der Knochenmasse eingesetzt werden.

Mixed phase clouds: observations and theoretical advances (overview)

Science.gov (United States)

Korolev, Alexei

2013-04-01

Mixed phase clouds play important role in precipitation formation and radiation budget of the Earth. The microphysical measurements in mixed phase clouds are notoriously difficult due to many technical challenges. The airborne instrumentation for characterization of the microstructure of mixed phase clouds is discussed. The results multiyear airborne observations and measurements of frequency of occurrence of mixed phase, characteristic spatial scales, humidity in mixed phase and ice clouds are presented. A theoretical framework describing the thermodynamics and phase transformation of a three phase component system consisting of ice particles, liquid droplets and water vapor is discussed. It is shown that the Wegener-Bergeron-Findeisen process plays different role in clouds with different dynamics. The problem of maintenance and longevity of mixed phase clouds is discussed.

Argumentellipse in der "weichen" Nachricht im Deutschen und im Slowenischen

Directory of Open Access Journals (Sweden)

Uršula Krevs

1998-12-01

Full Text Available Argumentellipsen werden in der sprachlichen Kommunikation ohne weiteres akzeptiert, obwohl sie allenfalls in gewissem Sinne wegen ihrer Elliptizität abweichend wirken. Im folgenden interessiert uns, wie die Argumentellipse als syntaktische Entität in den Text eingebettet wird und welche Funktionen sie ausübt. Unsere Aufmerksamkeit gilt bier der Textsorte "weiche" Nachricht, die als Abweichung des Grundmusters gilt. Um im Sprachenpaar Deutsch-Slowenisch die in dieser Textsorte realisierten Argumentellipsen vergleichen zu können, wurde die Homogenität in der Thematik angestrebt und die Texte aus dem gleichen Kommunikationsbereich gewählt. So wurden die Korpora zwei Zeitschriften, die sich mit der gleichen Sportart beschäftigen, entnommen: der deutschen Zeitschrift "Rotpunkt" und der slowenischen Zeitschrift  "Grif '.

A case of Melkersson-Rosenthal Syndrome and the Association Between the Orofacial Granulomatous Diseases

Directory of Open Access Journals (Sweden)

Yalcin Bas

2016-01-01

Full Text Available Melkersson%u2013Rosenthal syndrome is characterized by a triad of symptoms consisting recurring facial paralysis unilateral or bilateral, orofacial edema especially on the lips and fissured tongue. The orofacial swelling is characterized by reddish-brown, swollen, nonpruritic lips or edema of the face. The facial palsy is indistinguishable from Bell%u2019s palsy. The exact etiology is unknown. However, genetic and acquired factors have been implicated. There is no satisfactory treatment for Melkersson Rosenthal Syndrome yet. It is rarely seen to be able to observe all these symptoms of the syndrome at the same time and also incomplete cases may be confusing. Here we report a case of male patient and discuss Melkersson-Rosenthal syndrome and its association between orofacial granulomatosis and cheilitis granulomatosa.

Inlegvellen in Schagen? Winterswijk achter de paywall? De grote sprong voorwaarts van TMG en Wegener

NARCIS (Netherlands)

Dr. Piet Bakker

2012-01-01

Onlangs maakte de Telegraaf Media Groep (TMG) en Mecom hun strategische plannen bekend. Piet Bakker lector massamedia en digitalisering, analyseert de voornemens van de uitgevers.'Dichtbij.nl zou wel eens als winnaar uit de bus kunnen komen'.

Sources of history for "a psychology of verbal communication".

Science.gov (United States)

O'Connell, Daniel C; Kowal, Sabine

2011-02-01

There is a standard version of the history of modern mainstream psycholinguistics that emphasizes an extraordinary explosion of research in mid twentieth century under the guidance and leadership of George A. Miller and Noam Chomsky. The narrative is cast as a dramatic shift away from behavioristic principles and toward mentalistic principles based largely on transformational linguistics. A closer view of the literature diminishes the historical importance of behaviorism, shows a prevailing "written language bias" (Linell in The written language bias in linguistics: Its nature, origins and transformations, Routledge, London, 2005, p. 4) in psycholinguistic research, and elevates some theoretical and empirical thinking of the late nineteenth and the early twentieth centuries on language and language use to a far more important role than has heretofore been acknowledged. In keeping with the theoretical and methodological perspective of the present article, it is particularly appropriate that the German philologist Philipp Wegener be "given his due in the annals of linguistic sciences" (Koerner 1991, p. VI*). In his (1885/1991) Untersuchungen über die Grundfragen des Sprachlebens (Investigations regarding the fundamental questions of the life of language; our translation), he began his philological research with the investigation of actual speaking in everyday settings rather than with analyses of purely formal structure. Moreover, he emphasized understanding language and localized this function in the listener. Compatible with Wegener's own investigations is another aspect of speaking that has been most seriously neglected throughout the history of research on the psychology of verbal communication. For him, as well as for Esper (In C. Murchison [Ed.], A handbook of social psychology, Clark University Press, Worchester, MA, 1935), the basic and primary genre of dialogical discourse was not ongoing conversation, but the occasional use of speech in association with

Material characteristics and tribolotical characteristics of finished boundary layers of heavy-duty ceramics under tribological load. Final report; Werkstoffmechanische und tribologische Charakterisierung bearbeiteter und tribologisch beanspruchter Randschichten von Hochleistungskeramiken. Abschlussbericht

Energy Technology Data Exchange (ETDEWEB)

Zeller, R.; Pfeiffer, W.; Hollstein, T.

2000-04-01

kann. Aehnliches gilt fuer das tribologische Verhalten keramischer Bauteile im Falle einer bevorzugt mechanischen Randschichtbeanspruchung. Waehrend polierte Oberflaechen von SiC beim Gleitverschleiss wegen fehlender mikrohydrodynamischer Schmiereffekte zu katastrophalen Adhaesivverschleiss erleiden, bietet Feinlaeppen vor allem innerhalb der Einlaufphase Vorteile. Grob gelaeppte Oberflaechen bieten wegen des in der Einlaufphase reduzierten Traganteils zunaechst geringere Schmiereffekte als feingelaeppte Oberflaechen, die hohen und tiefreichenden bearbeitungsinduzierten Druckeigenspannungen koennen jedoch ueber lange Verschleisswege den Werkstoffabtrag vermindern. (orig.)

Geschichtsbezogene und rechtspolitische Polonica im Bücherbestand Gottfried Lengnichs

Directory of Open Access Journals (Sweden)

Iwona Imańska

2017-11-01

Full Text Available Gottfried Lengnich, der Historiker und Syndikus der Stadt Danzig aus dem 18. Jahrhundert, hinterließ eine Büchersammlung mit über viertausend Büchern, die nach seinem Tode in zwei Auktionen im Juli und November 1774 versteigert werden sollten. Die Auktionskataloge befinden sich in der Staatsbibliothek zu Berlin – Stiftung Preußischer Kulturbesitz und sind auch online zugänglich. Wegen wissenschaftlicher Interessen Lengnichs befanden sich in seiner Bibliothek ca. 600 Polonica-Drucke, die meisten davon waren Abhandlungen im Themenfeld Geschichte, Recht und Politik. Die Analyse dieses Fragments des Bücherbestands Lengnichs bewies, dass der Syndikus eine perfekte Arbeitswerkstatt für sich schuf, indem er die Werke der meisten wichtigsten polnischen und fremden Autoren, die über die Geschichte und die Gesetzgebung Polens und Preußens schrieben, sammelte.

Chirurgische Therapie der Stressharninkontinenz (SUI - Ein Überblick

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Riss P

2009-01-01

Full Text Available Die Stressharninkontinenz der Frau hat ihre höchste Prävalenz um die Zeit der Menopause. Es gibt verschiedene Erklärungsmöglichkeiten für die Kontinenz bei der Frau: die anatomische Lage des Blasenhalses, die Druckübertragung auf die proximale Urethra, eine Degeneration des M. sphincter urethrae, die sogenannte Hängemattentheorie der Unterstützung des Blasenhalses, das Trampolinmodell von P. Petros und schließlich das Konzept der mittleren Urethra. Auf letzterem beruhen die Operationen, bei denen ein Prolene-Band spannungsfrei unter die mittlere Urethra gelegt wird (TVT und Modifikationen. Wegen der Einfachheit der Durchführung und der exzellenten Erfolgsraten haben diese Operationen die bewährte Kolposuspension nach Burch weitgehend verdrängt. Faszienschlingenoperationen sind speziellen Rezidivsituationen vorbehalten.

Recommendations of the Brazilian Society of Rheumatology for the induction therapy of ANCA-associated vasculitis

Directory of Open Access Journals (Sweden)

Alexandre Wagner Silva de Souza

Full Text Available Abstract The purpose of these recommendations is to guide the appropriate induction treatment of antineutrophil cytoplasmic antibody-associated vasculitis (AAV patients with active disease. The recommendations proposed by the Vasculopathies Committee of the Brazilian Society Rheumatology for induction therapy of AAV, including granulomatosis with polyangiitis, microscopic polyangiitis and renal-limited vasculitis, were based on systematic literature review and expert opinion. Literature review was performed using Medline (PubMed, EMBASE and Cochrane database to retrieve articles until October 2016. PRISMA guidelines were used for the systematic review and articles were assessed according to the Oxford levels of evidence. Sixteen recommendations were made regarding different aspects of induction therapy for AAV. The purpose of these recommendations is to serve as a guide for therapeutic decisions by health care professionals in the management of AAV patients presenting active disease.

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.

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.

De lichttechnische eigenschappen van lichtgekleurde wegoppervlakken : artikel Wegen 56 10: 803-824 t/m 803-325.

NARCIS (Netherlands)

Schreuder, D.A.

1982-01-01

Cement concrete road surfaces have, because of their light colour, a double luminance yield as compared with bituminous roads. Savings in electricity amount to at least 20% in illumination costs or 5-10% of total maintenance costs. Light colour improves visibility at night. Road markings in white

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

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D.V. Pomazan

2016-08-01

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

Vorinostat in Treating Patients With Metastatic or Unresectable Solid Tumors or Lymphoma and Liver Dysfunction

Science.gov (United States)

2014-02-21

Adult Grade III Lymphomatoid Granulomatosis; Adult Nasal Type Extranodal NK/T-cell Lymphoma; Anaplastic Large Cell Lymphoma; Angioimmunoblastic T-cell Lymphoma; Cutaneous B-cell Non-Hodgkin Lymphoma; Extranodal Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue; Intraocular Lymphoma; Nodal Marginal Zone B-cell Lymphoma; Primary Central Nervous System Hodgkin Lymphoma; Primary Central Nervous System Non-Hodgkin Lymphoma; Recurrent Adult Burkitt Lymphoma; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Adult Diffuse Mixed Cell Lymphoma; Recurrent Adult Diffuse Small Cleaved Cell Lymphoma; Recurrent Adult Grade III Lymphomatoid Granulomatosis; Recurrent Adult Hodgkin Lymphoma; Recurrent Adult Immunoblastic Large Cell Lymphoma; Recurrent Adult Lymphoblastic Lymphoma; Recurrent Adult T-cell Leukemia/Lymphoma; Recurrent Cutaneous T-cell Non-Hodgkin Lymphoma; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Grade 3 Follicular Lymphoma; Recurrent Mantle Cell Lymphoma; Recurrent Marginal Zone Lymphoma; Recurrent Mycosis Fungoides/Sezary Syndrome; Recurrent Small Lymphocytic Lymphoma; Small Intestine Lymphoma; Splenic Marginal Zone Lymphoma; Stage III Adult Burkitt Lymphoma; Stage III Adult Diffuse Large Cell Lymphoma; Stage III Adult Diffuse Mixed Cell Lymphoma; Stage III Adult Diffuse Small Cleaved Cell Lymphoma; Stage III Adult Hodgkin Lymphoma; Stage III Adult Immunoblastic Large Cell Lymphoma; Stage III Adult Lymphoblastic Lymphoma; Stage III Adult T-cell Leukemia/Lymphoma; Stage III Cutaneous T-cell Non-Hodgkin Lymphoma; Stage III Grade 1 Follicular Lymphoma; Stage III Grade 2 Follicular Lymphoma; Stage III Grade 3 Follicular Lymphoma; Stage III Mantle Cell Lymphoma; Stage III Marginal Zone Lymphoma; Stage III Mycosis Fungoides/Sezary Syndrome; Stage III Small Lymphocytic Lymphoma; Stage IV Adult Burkitt Lymphoma; Stage IV Adult Diffuse Large Cell Lymphoma; Stage IV Adult Diffuse Mixed Cell Lymphoma; Stage

Synovial sarcoma of the foot; Synovialsarkom des Fusses

Energy Technology Data Exchange (ETDEWEB)

Beus, J. [Mainz Univ. (Germany). Klinik und Poliklinik fuer Radiologie; Kreitner, K.F. [Mainz Univ. (Germany). Klinik und Poliklinik fuer Radiologie; Rompe, J.D. [Mainz Univ. (Germany). Klinik und Poliklinik fuer Orthopaedie; Riehle, H.M. [Mainz Univ. (Germany). Inst. fuer Pathologie

1996-09-01

The case of a 29 year-old female patient who had experienced pain in the right midfoot for 5 years which was diagnosed as a degenerative or rheumatic change and treated by physiotherapy and medication. By means of magnetic resonance imaging we identified a soft-tissue tumor of the midfoot. Histology provided the findings of a monophasic fibrous synovial sarcoma. The case history is reported together with a presentation of the disease and its radiological diagnosis. (orig.) [Deutsch] Es wird ueber den Fall einer 29jaehrigen Patientin berichtet, die 5 Jahre lang wegen Schmerzen im rechten Mittelfuss unter der Diagnose degenerativer oder rheumatischer Veraenderungen physikalisch und medikamentoes behandelt wurde. Magnetresonanztomographisch wurde ein Weichteiltumor des Mittelfusses diagnostiziert. Die histologische Untersuchung erbrachte den Befund eines monophasisch-fibroesen Synovialsarkoms. Mit der Kasuistik verbunden ist eine Darstellung des Krankheitsbildes und dessen radiologischer Diagnostik. (orig.)

Instability Rules: The Ten Most Amazing Ideas of Modern Science

Science.gov (United States)

Flowers, Charles

2002-03-01

World-altering discoveries that reveal a universe of uncertainty and constant change Whether probing the farthest reaches of the vast universe or exploring the microscopic world of genetics and the subatomic world of quantum mechanics, Instability Rules is a remarkably informative and engaging look at ten milestone discoveries and their discoverers-a wide range of very human personalities whose insights have dramatically altered our most basic assumptions about human existence during the last century. The stories include Edwin Hubble and the expanding universe, Alfred Wegener and continental drift, Neils Bohr and quantum mechanics, Alan Turing and artificial intelligence, and James Watson and Francis Crick and DNA. Also covering discoveries of the twenty-first century that are already refining these and other ideas, Instability Rules is an exhilarating, sometimes amusing encounter with the defining scientific discoveries of our age.

Rezension von: Barbara Holland-Cunz: Gefährdete Freiheit. Über Hannah Arendt und Simone de Beauvoir. Opladen u.a.: Verlag Barbara Budrich 2012.

Directory of Open Access Journals (Sweden)

Mirjam Dierkes

2012-11-01

Full Text Available Von Barbara Holland-Cunz, die sich mit Simone de Beauvoir und Hannah Arendt bereits einzeln beschäftigt hat, liegt nun ein Band vor, in dem sie sich diesen herausragenden Denkerinnen des 20. Jahrhunderts gemeinsam widmet. In einer beinahe synoptisch anmutenden Herangehensweise fokussiert sie dabei auf den Begriff der Freiheit, der bei beiden von eminenter Bedeutung für das jeweilige Theoriegebäude ist. Holland-Cunz zufolge verstehen beide, bei einigen Unterschieden, Freiheit so, dass sie sich vor allem durch eine in sich eingeschriebene Gefährdung auszeichnet: Diese resultiert aus einem heroisch-emphatischen Gestus, der in paradoxer Weise gepaart wird mit einem abgeklärten Realismus. Über weite Strecken liest sich der Weg zu dieser Deutung, v. a. auch wegen einer teilweise sympathisch unkonventionellen und originellen Herangehensweise, sehr anregend und überzeugend.

Influencing energy consumption in public buildings - methodology and experience; Beeinflussung der Nutzer in oeffentlichen Gebaeuden - Methoden und Erfahrungen

Energy Technology Data Exchange (ETDEWEB)

Muno, P.

2000-07-01

In public buildings energy consumption and the attitude towards it depends in the most diverse fields upon the consumer hinself. Public buildings are to serve as examples for developing strategies to save energy on the consumer's side. Methods and experience of 21 years of energy management are to be reported on through which a reduction of energy consumption of approx. 20% has been achieved. (orig.) [German] Die Energieanwendung, der Umgang mit Energie in oeffentlichen Gebaeuden ist in verschiedensten Bereichen abhaengig vom Verhalten der Nutzer. Dieses Nutzerverhalten in Richtung Energieeinsparung zu beeinflussen und zu aendern, bietet neben der Verringerung des Energieverbrauchs und der CO{sub 2}-Emissionen den Vorteil niedrigerer Energiekosten. Methoden und Erfahrungen dazu liegen vor, d.h. Energieeinsparung durch nichtinvestive Massnahmen. Ein wichtiges Potential, nicht zuletzt wegen der knapper werdenden Energie-Ressourcen und oeffentlichen Mittel. (orig.)

KoelnArena has 18,000 seats; Die KoelnArena mit 18 000 Sitzplaetzen

Energy Technology Data Exchange (ETDEWEB)

Zerres, E.

1999-06-01

The ``Koelnarena`` is a new hall with 18,000 seats for sports events and cultural events, and with a skating rink in the hall center. In view of the many events (up to 180 per year), the skating rink must be glazed throughout the year (except for the summer holidays) in order to avoid the high cost of frequent freezing and thawing. [Deutsch] Die Koelnarena ist eine neue Halle fuer 18 000 Besucher in Koeln-Deutz, mit vielfacher Verwendbarkeit fuer Eishockey und Eistanz, Tennis, Volleyball, Boxen, Schausport, Showkonzerte, Oper und Tanzveranstaltungen. Das Kernstueck dieser MEGA-Halle ist die Eispiste in der Hallenmitte. Wegen der hohen Auslastung der Halle mit bis zu 180 Veranstaltungen pro Jahr muss die Eispiste ganzjaehrig (ausser waehrend der Sommerferien) vereist bleiben, um den sehr grossen Energieaufwand fuer haeufig Eisbildung (und Abtauen.) sowie die Kosten hierfuer zu vermeiden. (orig.)

Mit dem Puppenwagen in die normative Weiblichkeit. Lili Elbe und die journalistische Inszenierung von Transsexualität in Dänemark

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Sabine Meyer

2010-12-01

Full Text Available When the Danish painter Einar Wegener underwent several operations in 1930 in order to subsequently live as Lili Elvenes, the case was of great interest for both the public and the media. In the beginning of 1931 the Danish newspapers Politiken and Ekstra Bladet published a series of efficacious interlinked articles that were staged as a self-contained whole. Along with emphasizing the discursive competence of medicine and legislation this staging focused on the perpetuation of a heteronormative order. Employing the variables sex, gender and desire as well as their interconnections highlights the normalizing processes of (re-construction tied to such a perpetuation. At the same time it discloses staging strategies, which enable an analysis of Lili Elvenes’ agency. By pursuing this notion of agency such an analysis demonstrates how the transsexual subject is embedded in dominant discourses.

Discrete emotions and persuasion: the role of emotion-induced expectancies.

Science.gov (United States)

DeSteno, David; Petty, Richard E; Rucker, Derek D; Wegener, Duane T; Braverman, Julia

2004-01-01

The authors argue that specific emotions can alter the persuasive impact of messages as a function of the emotional framing of persuasive appeals. Because specific emotions inflate expectancies for events possessing matching emotional overtones (D. DeSteno, R. E. Petty, D. T. Wegener, & D. D. Rucker, 2000), the authors predicted that attempts at persuasion would be more successful when messages were framed with emotional overtones matching the emotional state of the receiver and that these changes would be mediated by emotion-induced biases involving expectancies attached to arguments contained in the messages. Two studies manipulating discrete negative emotional states and message frames (i.e., sadness and anger) confirmed these predictions. The functioning of this emotion-matching bias in parallel with emotion-induced processing differences and the limitations of a valence-based approach to the study of attitude change are also considered.

Positive mood can increase or decrease message scrutiny: the hedonic contingency view of mood and message processing.

Science.gov (United States)

Wegener, D T; Petty, R E; Smith, S M

1995-07-01

Currently dominant explanations of mood effects on persuasive message processing (i.e., cognitive capacity and feelings as information) predict that happy moods lead to less message scrutiny than neutral or sad moods. The hedonic contingency view (D. T. Wegener & R. E. Petty, 1994) predicts that happy moods can sometimes be associated with greater message processing activity because people in a happy mood are more attentive than neutral or sad people to the hedonic consequences of their actions. Consistent with this view, Experiment 1 finds that a happy mood can lead to greater message scrutiny than a neutral mood when the message is not mood threatening. Experiment 2 finds that a happy mood leads to greater message scrutiny than a sad mood when an uplifting message is encountered, but to less message scrutiny when a depressing message is encountered.

Interstital lung disease in ANCA vasculitis.

Science.gov (United States)

Alba, Marco A; Flores-Suárez, Luis Felipe; Henderson, Ashley G; Xiao, Hong; Hu, Peiqi; Nachman, Patrick H; Falk, Ronald J; Charles Jennette, J

2017-07-01

Anti-neutrophil cytoplasmic antibodies (ANCA) vasculitides are immune-mediated disorders that primarily affect small blood vessels of the airway and kidneys. Lung involvement, one of the hallmarks of microscopic polyangiitis and granulomatosis with polyangiitis, is associated with increased mortality and morbidity. In recent years, several retrospective series and case reports have described the association of interstitial lung disease (ILD) and ANCA vasculitis, particularly those positive for ANCA specific for myeloperoxidase. In the majority of these patients pulmonary fibrosis occurs concurrently or predates the diagnosis of ANCA vasculitis. More importantly, these studies have shown that ILD has an adverse impact on the long-term prognosis of ANCA vasculitis. This review focuses on the main clinical and radiologic features of pulmonary fibrosis associated with anti-neutrophil cytoplasmic antibodies. Major histopathology features, prognosis and therapeutic options are summarized. Copyright © 2017 Elsevier B.V. All rights reserved.

Clinical usefulness of {sup 123}I-BMIPP myocardial SPECT in collagen disease

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Ito, Kazuki; Zen, Kan [Asahi Univ., Gifu (Japan). Murakami Memorial Hospital; Sugihara, Hiroki (and others)

2000-07-01

This study was designed to evaluate the clinical usefulness of {sup 123}I-BMIPP myocardial SPECT for detecting cardiac involvement in patients with collagen disease. We studied 12 patients with systemic lupus erythematosus (SLE), 8 with progressive systemic sclerosis (PSS), 6 with polymyositis/dermatomyositis (PM/DM) and 3 with allergic granulomatosis and angiitis (AGA). A 111 MBq of {sup 123}I-BMIPP was intravenously injected at rest, and SPECT images were obtained at 15 min after the injection. Seven of 12 SLE, 6 of 8 PSS, 3 of 6 PM/DM and all 3 AGA patients showed an abnormal tracer uptake. The left ventricular ejection fraction was inversely correlated with a BMIPP abnormality. The regional wall motion abnormality was reduced in regions with reduced tracer uptake. These findings suggest that {sup 123}I-BMIPP imaging could be useful for assessment of cardiac involvement in patients with collagen disease. (author)

Marginal zone B-cell lymphoma with multiple extranodal locations in a patient with Sjögren’s syndrome – a diagnostic problem

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Marta Domżalska

2014-09-01

Full Text Available Sjögren’s syndrome is a chronic autoimmune disease characterized by the presence of lymphocytic infiltrates in exocrine glands, mainly salivary and lacrimal glands, which result in xerophthalmia and xerostomia. About half of the patients develop systemic complications, including lymphoproliferative disorders. We report a case of a 27-year-old woman with a diagnosis of Sjögren’s syndrome and a suspicion of respiratory system involvement in the course of granulomatosis with polyangiitis. Histopathological examination of a skin lesion suggested marginal zone B-cell lymphoma. After pathological and immunohistochemical evaluation of all available previous biopsy samples and the medical documentation the diagnosis of extranodal marginal zone B-cell lymphoma stage IV according to the Ann Arbor classification was rendered. The patient was referred to the Department of Haematology and was treated with R-CVP (cyclophosphamide, vincristine, prednisone, rituximab.

Gemcitabine Hydrochloride, Carboplatin, Dexamethasone, and Rituximab in Treating Patients With Previously Treated Lymphoid Malignancies

Science.gov (United States)

2017-05-28

Adult Nasal Type Extranodal NK/T-cell Lymphoma; Anaplastic Large Cell Lymphoma; Angioimmunoblastic T-cell Lymphoma; Cutaneous B-cell Non-Hodgkin Lymphoma; Extranodal Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue; Hepatosplenic T-cell Lymphoma; Intraocular Lymphoma; Nodal Marginal Zone B-cell Lymphoma; Noncutaneous Extranodal Lymphoma; Peripheral T-cell Lymphoma; Recurrent Adult Burkitt Lymphoma; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Adult Diffuse Mixed Cell Lymphoma; Recurrent Adult Diffuse Small Cleaved Cell Lymphoma; Recurrent Adult Grade III Lymphomatoid Granulomatosis; Recurrent Adult Hodgkin Lymphoma; Recurrent Adult Immunoblastic Large Cell Lymphoma; Recurrent Adult Lymphoblastic Lymphoma; Recurrent Adult T-cell Leukemia/Lymphoma; Recurrent Cutaneous T-cell Non-Hodgkin Lymphoma; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Grade 3 Follicular Lymphoma; Recurrent Mantle Cell Lymphoma; Recurrent Marginal Zone Lymphoma; Recurrent Mycosis Fungoides/Sezary Syndrome; Recurrent Small Lymphocytic Lymphoma; Small Intestine Lymphoma; Splenic Marginal Zone Lymphoma; Testicular Lymphoma; Waldenstrom Macroglobulinemia

Systemic Lupus Erythematosus with Hepatosplenic Granuloma: A Rare Case

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Anju Bharti

2014-01-01

Full Text Available Background. Systemic lupus erythematosus (SLE is an autoimmune disease which is known to present with a wide variety of clinical manifestations. Case Report. A 15-year-old male presented with complaints of moderate grade fever and generalized body swelling. There was no history of cough, weight loss, joint pain, oral ulcerations, skin rash, photosensitivity, loss of hair, pain abdomen, jaundice, or any significant illness in the past. Contrast enhanced computerized tomography of the abdomen revealed hypodense lesions in both liver and spleen (without contrast enhancement, suggestive of granulomas along with few retroperitoneal and mesenteric lymph nodes. On the basis of immunological tests and renal biopsy report, SLE with hepatosplenic granulomatosis diagnosis was made. He was given pulse methylprednisolone 500 mg, for 3 days and he showed dramatic improvement clinically. Conclusion. Hepatic and splenic granulomas are not common in SLE, but this should be kept in differential diagnosis.

Ultrastructural characteristics of type A epithelioid cells during BCG-granulomatosis and treatment with lysosomotropic isoniazid.

Science.gov (United States)

Shkurupii, V A; Kozyaev, M A; Nadeev, A P

2006-04-01

We studied BCG-granulomas, their cellular composition, and ultrastructure of type A epithelioid cells in the liver of male BALB/c mice with spontaneous granulomatous inflammation. The animals received free isoniazid or isoniazid conjugated with lysosomotropic intracellularly prolonged matrix (dialdehyde dextran, molecular weight 65-75 kDa). Lysosomotropic isoniazid was accumulated in the vacuolar apparatus of epithelioid cells and produced a stimulatory effect on plastic processes in these cells.

Neoadjuvant chemoradiation therapy with gemcitabine/cisplatin and surgery versus immediate surgery in resectable pancreatic cancer. Results of the first prospective randomized phase II trial

Energy Technology Data Exchange (ETDEWEB)

Golcher, Henriette; Merkel, Susanne; Hohenberger, Werner [University Hospital Erlangen, Department of Surgery, Erlangen (Germany); Brunner, Thomas B. [University Hospital Erlangen, Department of Radiation Oncology, Erlangen (Germany); University Hospital Freiburg, Department of Radiation Oncology, Freiburg (Germany); Witzigmann, Helmut [University Hospital Leipzig, Department of Surgery, Leipzig (Germany); Hospital Dresden-Friedrichstadt, General Surgery, Dresden (Germany); Marti, Lukas [Hospital of Kanton St. Gallen, General Surgery, St. Gallen (Switzerland); Bechstein, Wolf-Otto [University Hospital Frankfurt, Department of Surgery, Frankfurt/Main (Germany); Bruns, Christiane [University Hospital Munich, Department of Surgery - Hospital Campus Grosshadern, Munich (Germany); University Hospital Magdeburg, Department of Surgery, Magdeburg (Germany); Jungnickel, Henry [Hospital Dresden-Friedrichstadt, General Surgery, Dresden (Germany); Schreiber, Stefan [University Hospital Leipzig, Department of Surgery, Leipzig (Germany); Grabenbauer, Gerhard G. [University Hospital Erlangen, Department of Radiation Oncology, Erlangen (Germany); Hospital Coburg, Department of Radiation Oncology, Coburg (Germany); Meyer, Thomas [University Hospital Erlangen, Department of Surgery, Erlangen (Germany); Hospital Ansbach, General Surgery, Ansbach (Germany); Fietkau, Rainer [University Hospital Erlangen, Department of Radiation Oncology, Erlangen (Germany)

2014-09-25

neoadjuvante Radiochemotherapie bereits zum Therapiestandard. Analog wurde der Stellenwert einer Radiochemotherapie beim Pankreaskarzinom in einer randomisierten Phase-II-Studie untersucht. Das praetherapeutische radiologische Staging war Grundlage dieser Studie im Gegensatz zu adjuvanten Therapiestudien, die auf pathohistologischem Staging basieren. Patienten mit resektablem Pankreaskopfkarzinom wurden randomisiert in primaere Operation (Arm A) versus neoadjuvante Radiochemotherapie gefolgt von einer Operation (Arm B). Beide Gruppen erhielten eine adjuvante Chemotherapie. Es waren 254 Patienten erforderlich, um eine Verbesserung des medianen Gesamtueberlebens von 4,33 Monaten zu erfassen. Die Studie wurde wegen zoegerlicher Rekrutierung nach Einschluss von 73 Patienten beendet. Insgesamt konnten 66 Patienten ausgewertet werden. Die ihnen zugeordnete Radiochemotherapie erhielten 29 von 33 Patienten. Alle Patienten bekamen die vollstaendige Bestrahlungstherapie. Wegen der Toxizitaet wurde bei 3 Patienten die Chemotherapie reduziert. Eine Pankreastumorresektion erhielten 23 vs. 19 Patienten (A vs. B). Die R0-Resektionsrate betrug 48 % (A) und 52 % (B, P = 0,81). Bei 30 % (A) versus 39 % (B, P = 0,44) der resezierten Patienten waren keine Lymphknotenmetastasen vorhanden. Die postoperativen Komplikationen waren in beiden Gruppen vergleichbar. Das mediane Gesamtueberleben betrug 14,4 vs. 17,4 Monate (A vs. B; ''Intention-to-treat''-Analyse; P = 0,96). Nach Pankreastumorresektion stieg das Gesamtueberleben auf 18,9 vs. 25,0 Monate (A vs. B; P = 0,79). Diese weltweit erste randomisierte Studie zur neoadjuvanten Radiochemotherapie beim Pankreaskopfkarzinom war in Bezug auf Toxizitaet sowie perioperative Morbiditaet und Mortalitaet gut durchfuehrbar. Die Ergebnisse sind jedoch nicht signifikant, da diese randomisierte Studie vorzeitig wegen mangelnder Rekrutierung beendet werden musste. ISRCTN78805636; NCT00335543. (orig.)

Abscesses after appendectomy due to intraoperative loss of fecaliths; Abszesse nach Appendektomie durch intraoperativ verlorene Appendicolithen

Energy Technology Data Exchange (ETDEWEB)

Hoermann, M.; Kreuzer, S. [Universitaetsklinik fuer Radiodiagnostik, Vienna (Austria). Abt. chirurgische Faecher; Sacher, P. [UniversitaetsSpital Zuerich (Switzerland). Abt. Kinderchirurgie; Eich, G.F. [UniversitaetsSpital Zuerich (Switzerland). Inst. fuer Diagnostische Radiologie

2001-08-01

Objective: Fecaliths appear to predispose a patient to acute appendicitis, abscess formation, and perforation. The number of preoperative radiological evaluations is increasing, although children with suspected perforation still undergo surgery immediately. We report and discuss imaging findings and implications in children with acute appendicitis and fecalithis. Methods: Four children (3 girls, 1 boy; mean age 9 years) underwent surgery for acute appendicitis. Three children underwent sonography and plain radiography before surgery, 1 child was operated without radiological evaluation. After readmission, all 4 children underwent sonography and plain radiography before surgery. Results: In 3 patients a fecalith was diagnosed initially. After uneventful recovery all 4 patients had acute abdominal pain and readmission was necessary. In all 4 patients the escaped fecalith was demonstrated with sonography and plain radiography confirmed surgically. Conclusion: Discussion about the role of imaging in acute appendicitis has concentrated on the diagnostic yield of cross-section techniques. The importance of demonstrating a fecalith, prompting a more thorough intraoperative search has found little attention. The radiologist should also detect and localize a fecalith and should be aware of retained fecaliths as a cause of abscess formation after appendectomy. (orig.) [German] Ziel: Faekolithen praedisponieren Kinder zu akuter Appendizitis und fuehren vermehrt zu Perforation und Abszessen. Wir berichten und diskutieren die Bedeutung der Bildgebung bei Kindern mit Faekolithen und perforierter akuter Appendizitis. Methoden: 4 Kinder (drei Maedchen, ein Junge, Altersmeridian 9 Jahre) wurden wegen perforierter akuter Appendizitis operiert. Drei Kinder erhielten praeoperativ eine Ultraschalluntersuchung und ein Abdomen-Uebersichtsbild. Nach Neuaufnahme wurden nun bei allen Kindern eine Ultraschalluntersuchung und ein Abdomen-Uebersichtsbild angefertigt und neuerlich operiert

Biliary fascioliasis – an uncommon cause of recurrent biliary colics: Report of a case and brief review [Biliäre Fasciolose – eine ungewöhnliche Ursache für wiederholte Gallenkoliken: Bericht eines Falles mit einer kurzen Übersicht

Directory of Open Access Journals (Sweden)

All, Gamal Nasr Ahmed Abdel

2012-05-01

Full Text Available [english]
Biliary parasitosis is one of the important causes of biliary obstruction in endemic areas, however due to migration and travel the disease is known to occur in non endemic zones as well. The spectrum of biliary fascioliasis ranges from recurrent biliary colics to acute cholangitis. The long term complications are gall stones, sclerosing cholangitis and biliary cirrhosis. We describe fascioliasis as a cause of recurrent biliary colics in a young male necessitating multiple hospitalizations over a period of four years. Investigative profile had been non-contributory every time he was hospitalized for his abdominal pain prior to the current presentation. He never had cholangitis due to the worm in the common bile duct. It was only at endoscopic retrograde cholangiopancreatography (ERCP biliary fascioliasis was discovered to be the cause of his recurrent biliary colics. After removal of the live from the common bile duct he became symptom free and is attending our clinic for last 11 months now. Clinical spectrum of biliary fascioliasis is discussed in this report.[german]
In endemischen Gebieten ist der Parasitenbefall von Gallengängen eine häufige Ursache für Gallenkoliken. Aufgrund von Migration und Reisetätigkeiten kommen derartige Erkrankungen auch außerhalb endemischer Gebiete vor. Das Spektrum der Symptome bei Gallengangsfasciolose erstreckt sich von wiederholten Gallenkoliken bis zur akuten Cholangitis. Als Langzeitkomplikationen werden Gallensteine, sklerosierende Cholangitis und biliäre Zirrhose beschrieben. Wir berichten über eine Fasciolose als Ursache für Gallenkoliken bei einem jungen Mann, der über vier Jahre mehrfach wegen Gallenkoliken stationär behandelt wurde. Das Untersuchungsprofil brachte bisher jedes Mal keinen Erfolg, wenn er stationär wegen Bauchschmerzen aufgenommen wurde. Er hatte vorher niemals eine Cholangitis auf der Basis eines Befalls mit dem Leberegel. Nur durch endoskopisch retrograde

Paradoxical effects of radioiodine therapy in functional thyroid autonomy and mild immunothyropathy; Paradoxe Effekte der Radiojodtherapie bei funktioneller Schilddruesenautonomie und milder Immunthyreopathie

Energy Technology Data Exchange (ETDEWEB)

Dunkelmann, S.; Rudolph, F.; Prillwitz, A.; Groth, P.; Schuemichen, C. [Rostock Univ. (Germany). Klinik und Poliklinik fuer Nuklearmedizin

1998-01-01

Aim: To examine all cases with Graves` disease after radioiodine therapy of autonomously functioning thyroid tissue (AFFT) in order to find the cause. Methods: We retrospectively studied 1428 pts who were treated between 11/93 and 3/97 with radioiodine for AFTT and who underwent at least one control examination. Results: 15 (1.1%) of all pts developed Graves` disease 8.4 (4-13) months after radioiodine therapy. There was no direct suggestion of Graves` disease (TRAK negative, no endocrine ophthalmopathy) in any pt at the time of radioiodine therapy. More detailed analysis of anamnestic data, however, revealed evidence that immunothyropathy predated radioiodine therapy in 11 of the 15 pts. Paradoxical effects of radioiodine therapy manifested as an increase in immunothyropathy 14 pts, a deterioration in metabolism in 11 pts and a first occurrence of endocrine ophthalmopathy in 5 pts. Conclusion: Exacerbation of preexisting, functional primarily insignificant immunothyropathia is held responsible in most cases for the observed paradoxical effects after radioiodine therapy, resulting in radiation-induced manifest Graves` disease; however no therapeutical consequences are recommended. (orig.) [Deutsch] Ziel: Erfassung von Pat. mit einer nach Radiojodtherapie wegen einer funktionellen Autonomie aufgetretenen Immunhyperthyreose sowie deren Ursachen. Methoden: Wir untersuchten in einer retrospektiven Studie 1428 Pat., die im Zeitraum 11/93 bis 3/97 wegen einer funktionellen Autonomie mit Radiojod behandelt wurden und die sich mindestens einer Kontrolluntersuchung unterzogen hatten. Ergebnisse: 15 (1,1%) der Pat. entwickelten 8,4 (4-13) Monate nach Radiojodtherapie eine postradiogene Immunhyperthyreose. Bei allen 15 Pat. lag zum Zeitpunkt der Radiojodtherapie kein direkter Hinweis auf eine Immunhyperthyreose vor (TRAK neg., keine endokrine Orbitopathie). Bei einer genauen Analyse anamnestischer Daten fanden sich jedoch bei 11 der 15 Pat. erste Hinweise auf das Vorliegen

MRI enhancing patterns of non-meningioma meningeal lesions

International Nuclear Information System (INIS)

Tao Xiaofeng; Ding Juan; Xiao Xiangsheng; Shi Zengru; Yu Hong; Gu Qian

2004-01-01

Objective: To analyze the MRI appearances of meningeal diseases and to study MRI diagnostic value of enhancing patterns in different meningeal processes. Methods: Sixty-one patients with integrated clinical data, including 27 infectious meningitis, 4 inflammatory meningitis (2 eosinophilic granuloma, 1 Wegener granuloma, and 1 unknown etiological factor), 12 meningeal metastasis, 2 meningeal lymphoma, 8 cerebrovascular disease, and 8 postoperative changes, were reviewed retrospectively. All patients were examined on MRI before and after contrast administration. Results: (1) MR plain scan: positive findings of plain scan were revealed in only 3 cases, including 1 linear meningeal thickening pattern and 2 nodular pattern. (2) MR enhancement: All cases showed 3 kinds of enhancing patterns: 19 dural-arachnoid pattern, 32 pia-arachnoid pattern, and 10 total meninges pattern, respectively. Conclusion: Different meningeal diseases have different MR imaging manifestations. Creating the enhancement patterns of various diseases can have great clinical significance. (authors)

Exploitation of political and technical scopes for free play. Energy conservation in buildings - part of a future-oriented energy policy; Politische und technische Handlungsspielraeume nutzen. Energieeinsparung im Gebaeudebereich - Teil einer zukunftsweisenden Energiepolitik

Energy Technology Data Exchange (ETDEWEB)

Ehm, H. [Bundesministerium fuer Verkehr, Bau- und Wohnungswesen, Berlin (Germany)

1999-09-01

In April of this year, the section of architecture of the University of Kassel conferred on Professor Dr. Herbert Ehm, department head at the federal building ministry, the title of honorary doctor for his achievements in the sector of energy-saving construction. In his address given at the awarding ceremony, entitled 'Energy conservation in buildings - part of a future-oriented energy policy', the recipient underlined the necessity of energy conservation measures in buildings because of the current high energy consumption and outlined approaches. (orig.) [German] Im April dieses Jahres verlieh der Fachbereich Architektur der Universitaet/GSH Kassel Professor Dr. Herbert Ehm, Abteilungsleiter im Bundesbauministerium, die Ehrendoktorwuerde fuer seine Verdienste um das energiesparende Bauen. In seinem Festvortrag 'Energieeinsparung im Gebaeudebereich - Teil einer zukunftsweisenden Energiepolitik' unterstrich der Geehrte wegen des erheblichen Verbrauchs die Notwendigkeit der Sparmassnahmen im Wohnungsbau und skizzierte Loesungswege. (orig.)

Faunal migration into the Late Permian Zechstein Basin

DEFF Research Database (Denmark)

Sørensen, Anne Mehlin; Håkansson, Eckart; Stemmerik, Lars

2007-01-01

Late Permian bryozoans from the Wegener Halvø, Ravnefjeld and Schuchert Formations in East Greenland have been investigated. 14 genera are recognised.      Integration of the new bryozoan data from the Upper Permian of East Greenland with data on the distribution of Permian bryozoans along...... the northern margin of Pangea is used to test hypotheses concerning Late Palaeozoic evolution of the North Atlantic region. During the Permian, the Atlantic rift system formed a seaway between Norway and Greenland from the boreal Barents Shelf to the warm and arid Zechstein Basin. This seaway is considered...... to be the only marine connection to the Zechstein Basin and therefore the only possible migration route for bryozoans to enter the basin. The distribution of Permian bryozoans is largely in keeping with such a connection from the cool Barents Shelf past the East Greenland Basin to the warm Zechstein Basin...

Wäre es Dir lieber, ich würde zu Recht verurteilt? - Sokrates Bund und die Frage der Kontrolle

Directory of Open Access Journals (Sweden)

Katharina Kaiser-Müller

2014-09-01

Full Text Available Die Ablösung von zweifelhafter menschlicher Kontrolle durch eine zuverlässige Software bietet eindrucksvolle Möglichkeiten. Mit der flächendeckenden Einführung von Sokrates Bund zum Schuljahr 2014/2015 wird endlich die Identifzierung von Pisadurchschnittsverderbern möglich. Die AutorInnen diskutieren die Lage ... Die Situation war dramatisch: Ungerecht angeklagt wegen Verführung der Jugend und Gottlosigkeit, sieht Sokrates dem Tod durch den Schierlingsbecher entgegen und antwortet auf die ob des Fehlurteils durchaus verständliche Klage von Xanthippe mit der titelgebenden Frage. Anschließend verweigert er die Flucht und zieht es vor, sich dem formal korrekt gefällten falschen Urteil zu beugen. Eine legendäre Entscheidung, in der Sokrates die Gesetzestreue mit der Begründung, man müsse schlechte Gesetze ändern, dürfe sie aber nicht übertreten, über das eigene Wohl stellt.

Black-Box Search by Unbiased Variation

DEFF Research Database (Denmark)

Lehre, Per Kristian; Witt, Carsten

2012-01-01

The complexity theory for black-box algorithms, introduced by Droste, Jansen, and Wegener (Theory Comput. Syst. 39:525–544, 2006), describes common limits on the efficiency of a broad class of randomised search heuristics. There is an obvious trade-off between the generality of the black-box model...... and the strength of the bounds that can be proven in such a model. In particular, the original black-box model provides for well-known benchmark problems relatively small lower bounds, which seem unrealistic in certain cases and are typically not met by popular search heuristics.In this paper, we introduce a more...... restricted black-box model for optimisation of pseudo-Boolean functions which we claim captures the working principles of many randomised search heuristics including simulated annealing, evolutionary algorithms, randomised local search, and others. The key concept worked out is an unbiased variation operator...

A case study of microphysical structures and hydrometeor phase in convection using radar Doppler spectra at Darwin, Australia

International Nuclear Information System (INIS)

Riihimaki, Laura D.; Comstock, J. M.; Luke, E.; Thorsen, T. J.; Fu, Q.

2017-01-01

To understand the microphysical processes that impact diabatic heating and cloud lifetimes in convection, we need to characterize the spatial distribution of supercooled liquid water. To address this observational challenge, ground-based vertically pointing active sensors at the Darwin Atmospheric Radiation Measurement site are used to classify cloud phase within a deep convective cloud. The cloud cannot be fully observed by a lidar due to signal attenuation. Therefore, we developed an objective method for identifying hydrometeor classes, including mixed-phase conditions, using k-means clustering on parameters that describe the shape of the Doppler spectra from vertically pointing Ka-band cloud radar. Furthermore, this approach shows that multiple, overlapping mixed-phase layers exist within the cloud, rather than a single region of supercooled liquid. Diffusional growth calculations show that the conditions for the Wegener-Bergeron-Findeisen process exist within one of these mixed-phase microstructures.

A case study of microphysical structures and hydrometeor phase in convection using radar Doppler spectra at Darwin, Australia

Science.gov (United States)

Riihimaki, L. D.; Comstock, J. M.; Luke, E.; Thorsen, T. J.; Fu, Q.

2017-07-01

To understand the microphysical processes that impact diabatic heating and cloud lifetimes in convection, we need to characterize the spatial distribution of supercooled liquid water. To address this observational challenge, ground-based vertically pointing active sensors at the Darwin Atmospheric Radiation Measurement site are used to classify cloud phase within a deep convective cloud. The cloud cannot be fully observed by a lidar due to signal attenuation. Therefore, we developed an objective method for identifying hydrometeor classes, including mixed-phase conditions, using k-means clustering on parameters that describe the shape of the Doppler spectra from vertically pointing Ka-band cloud radar. This approach shows that multiple, overlapping mixed-phase layers exist within the cloud, rather than a single region of supercooled liquid. Diffusional growth calculations show that the conditions for the Wegener-Bergeron-Findeisen process exist within one of these mixed-phase microstructures.

Phase transformations in an ascending adiabatic mixed-phase cloud volume

Science.gov (United States)

Pinsky, M.; Khain, A.; Korolev, A.

2015-04-01

Regimes of liquid-ice coexistence that may form in an adiabatic parcel ascending at constant velocity at freezing temperatures are investigated. Four zones with different microphysical structures succeeding one another along the vertical direction have been established. On the basis of a novel balance equation, analytical expressions are derived to determine the conditions specific for each of these zones. In particular, the necessary and sufficient conditions for formation of liquid water phase within an ascending parcel containing only ice particles are determined. The results are compared to findings reported in earlier studies. The role of the Wegener-Bergeron-Findeisen mechanism in the phase transformation is analyzed. The dependence of the phase relaxation time on height in the four zones is investigated on the basis of a novel analytical expression. The results obtained in the study can be instrumental for analysis and interpretation of observed mixed-phase clouds.

Pulmonary berylliosis. Experimental induction. Early detection

International Nuclear Information System (INIS)

Andre, Stephane

1984-01-01

This research thesis reports the study of the biological reactivity of industrial and environmental aerosols, notably beryllium powder aerosols. In order to study beryllium toxicity under its metal form, and the dose-effect relationship and the suspected carcinogen effect, a pulmonary berylliosis has been experimentally induced in rat and monkey. In order to develop means of rapid detection of exposure and sensitisation to beryllium, the author studied early pathological cellular mechanisms occurring during a pulmonary granulomatosis. Quantitative and qualitative modifications have been searched for, more particularly at the level of endo-alveolar cellular populations of macrophages and lymphocytes. The study has also been extended to the sensitisation of lymphocytes with respect to beryllium. After some generalities about beryllium (properties, sources, uses, exposure sources), and an overview of its toxicity (epidemiology, pathologies, experiments, biological mechanisms, detoxification), the author reports the introduction of a chronic pulmonary berylliosis into animals, discusses the various noticed pathologies and their analysis, and reports in situ and in vitro studies [fr

Monoclonal Antibody Therapy Before Stem Cell Transplant in Treating Patients With Relapsed or Refractory Lymphoid Malignancies

Science.gov (United States)

2017-10-10

Adult Nasal Type Extranodal NK/T-cell Lymphoma; Anaplastic Large Cell Lymphoma; Angioimmunoblastic T-cell Lymphoma; Cutaneous B-cell Non-Hodgkin Lymphoma; Extranodal Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue; Hepatosplenic T-cell Lymphoma; Intraocular Lymphoma; Nodal Marginal Zone B-cell Lymphoma; Noncutaneous Extranodal Lymphoma; Peripheral T-cell Lymphoma; Recurrent Adult Burkitt Lymphoma; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Adult Diffuse Mixed Cell Lymphoma; Recurrent Adult Diffuse Small Cleaved Cell Lymphoma; Recurrent Adult Grade III Lymphomatoid Granulomatosis; Recurrent Adult Hodgkin Lymphoma; Recurrent Adult Immunoblastic Large Cell Lymphoma; Recurrent Adult Lymphoblastic Lymphoma; Recurrent Adult T-cell Leukemia/Lymphoma; Recurrent Cutaneous T-cell Non-Hodgkin Lymphoma; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Grade 3 Follicular Lymphoma; Recurrent Mantle Cell Lymphoma; Recurrent Marginal Zone Lymphoma; Recurrent Mycosis Fungoides/Sezary Syndrome; Recurrent Small Lymphocytic Lymphoma; Refractory Hairy Cell Leukemia; Small Intestine Lymphoma; Splenic Marginal Zone Lymphoma; T-cell Large Granular Lymphocyte Leukemia; Testicular Lymphoma; Waldenström Macroglobulinemia

Spectrum of Epstein-Barr virus-related diseases. A pictorial review

International Nuclear Information System (INIS)

Maeda, Eriko; Akahane, Masaaki; Kiryu, Shigeru

2009-01-01

Epstein-Barr virus (EBV) prevails among more than 90% of the adult population worldwide. Most primary infections occur during young childhood and cause no or only nonspecific symptoms; then the virus becomes latent and resides in lymphocytes in the peripheral blood. Inactive latent EBV usually causes no serious consequences, but once it becomes active it can cause a wide spectrum of malignancies: epithelial tumors such as nasopharyngeal and gastric carcinomas; mesenchymal tumors such as follicular dendritic cell tumor/sarcoma; and lymphoid malignancies such as Burkitt lymphoma, lymphomatoid granulomatosis, pyothorax-associated lymphoma, immunodeficiency-associated lymphoproliferative disorders, extranodal natural killer (NK) cell/T-cell lymphoma, and Hodgkin's lymphoma. The purpose of this article is to describe the spectrum of EBV-related diseases and their key imaging findings. EBV-related lymphoproliferative disorders and lymphomas are especially common in immunocompromised patients. Awareness of their clinical settings and imaging spectrum contributes to early detection and early treatment of possibly life-threatening disorders. (author)

[Prostatic granulomas revealing a peripheral T-cell lymphoma].

Science.gov (United States)

Foguem, C; Curlier, E; Rouamba, M-M; Regent, A; Philippe, P

2009-02-01

The presence of granulomas on tissue biopsie has been reported in a wide range of disorders. The clinical presentation and the diagnostic work-up of granulomatosis can be difficult as it is illustrated in the following report. A 59-year-old patient was referred in 2002 for a granulomatous prostatitis. Physical examination was normal. Except for the increase of prostate-specific antigen (which motivated a biopsy), the laboratory results were normal. Thoracic CT-scan disclosed mediastinal lymph nodes. A minor salivary gland biopsy was consistent with the diagnosis of sarcoidosis. In 2004, the patient presented an epidermal necrolysis, and in 2005 the deterioration of general status raised suspicion of a lymphoproliferative disorder. Liver and bone marrow biopsies revealed a granulomatous process. Despite steroid therapy, the patient died. Autopsy discloses a anaplasic T cell lymphoma. This report illustrates the relationship between sarcoidosis and lymphoma as a mode of presentation, a complication, or an accidental but misleading association? The association between anaplastic lymphoma and sarcoidosis is exceptional.

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.