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Sample records for wegener granulomatosis revealed

  1. Pulmonary Localization Revealing Wegener's Granulomatosis

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    Mona Mlika

    2010-01-01

    Full Text Available Wegener's granulomatosis (WG is the most frequent antineutrophil cytoplasmic antibody (ANCA–associated vasculitis. It affects mainly the upper airways, lungs, and kidneys. Two forms are identified: systemic and limited. We describe three cases of limited WG diagnosed during a 7-year period. Our aim is to report three localized forms of WG and to put emphasis on the necessity of differentiating localized from systemic forms because of their different prognoses and manner of management. Our study contained two men and one woman with a mean age of 43 years. All our patients were symptomatic and presented with nonspecific respiratory signs. The cANCA were positive in all patients. The imaging findings consisted of cavitary masses. The diagnosis was based on surgical lung biopsy in all cases. All patients were put on cyclophosphamide and prednisolone. Only one patient presented with renal complications after a 2-year follow-up period. The two other patients did not present complications after, respectively, 1 month and 1 year of follow-up. These case reports put emphasis on a rare form of WG, the limited form. The low number of patients, due to the rarity of this disease, does not allow us to delineate the characteristics and the differences between this form and the systemic form, but we highlight the necessity of future investigations in order to explore the pathogenesis, therapeutic, and prognosis differences between these two subsets.

  2. Granulomatose de Wegener Wegener's granulomatosis

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    Telma Antunes

    2005-07-01

    Full Text Available A granulomatose de Wegener caracteriza-se por vasculite necrosante granulomatosa que acomete preferencialmente vias aéreas superiores, inferiores e rins. Seu diagnóstico é feito associando-se as manifestações clínicas, radiológicas (multiplos nódulos escavados e os achados anatomopatológicos e o anticorpo anticitoplasma de neutrófilos positivo. O tratamento com corticosteróides e ciclofosfamida leva a 90% de remissão da doença em 1 ano.Wegener's granulomatosis is characterized by granulomatous necrotizing vasculitis that primarily affects the airways (upper and lower and the kidneys. The diagnosis is made by analyzing the clinical and radiological manifestations (multiple pulmonary cavitations, together with the pathological findings and results of the test for cytoplasmic-pattern antineutrophil cytoplasmic antibodies. Treatment consists of corticosteroids and cyclophosphamide and leads to remission of the disease within one year in 90% of cases.

  3. Staphylococcus aureus and Wegener's granulomatosis

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    Popa, ER; Stegeman, CA; Kallenberg, CGM; Tervaert, JWC

    2002-01-01

    Wegener's granulomatosis (WG) is a form of systemic vasculitis. It is characterized by granulomatous inflammation in the upper and lower airways, vasculitis and necrotizing glomerulonephritis, and is strongly associated with antineutrophil cytoplasmic antibodies against proteinase 3, Since the etiol

  4. Wegener's granulomatosis--an etiology of acute pancreatitis.

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    Joshipura, Vismit P; Haribhakti, Sanjiv P; Pandya, Sapan C; Soni, Harshad N; Patel, Nitin R

    2007-01-01

    Wegener's granulomatosis is a systemic disease that usually involves the upper respiratory tract and kidneys. We report a 47-year-old man with Wegener's granulomatosis that presented as acute pancreatitis.

  5. Kidney involvement in a wegener granulomatosis case

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    Gioacchino Li Cavoli

    2012-01-01

    Full Text Available Wegener Granulomatosis is a systemic Anti-Neutrophil Cytoplasmic Autoantibody- associated Vasculitis, affecting small-to-medium vessels. Clinical presentation with simultaneous involvement of kidney and upper and lower respiratory tract is unusual. We report an instructive case of WG, analyzing clinical course, laboratory, and radiological features, kidney, lung, and larynx histological pictures. Besides renal biopsy, nephrology team performed larynx and lung biopsies because of unusual clinical presentation, computed tomography chest examination, and relevant malignancy risk regarding following immunosuppressant therapy.

  6. Diagnostic Consideration for Sinonasal Wegener's Granulomatosis Clinically Mistaken for Carcinoma

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    La Rosa, Cristina; Emmanuele, Carmela; Tranchina, Maria Grazia; Ippolito, Massimo; Cosentino, Sebastiano; Saita, Vincenzo; Fraggetta, Filippo

    2013-01-01

    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. PMID:24106630

  7. Deoxyspergualin in relapsing and refractory Wegener's granulomatosis

    DEFF Research Database (Denmark)

    Flossmann, O; Baslund, B; Bruchfeld, A

    2008-01-01

    at entry and prednisolone doses adjusted according to clinical status. Deoxyspergualin, 0.5 mg/kg per day, was self-administered by subcutaneous injection in six cycles of 21 days with a 7-day washout between cycles. Cycles were stopped early for white blood count less than 4000 cells/mm(3). The primary......-threatening (> or = grade 3) treatment-related adverse events occurred in 24 (53%) patients mostly due to leucopaenias. CONCLUSIONS: Deoxyspergualin achieved a high rate of disease remission and permitted prednisolone reduction in refractory or relapsing Wegener's granulomatosis. Adverse events were common but rarely led...

  8. Massive intracerebral hemorrhage associated with Wegener granulomatosis.

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    Ceri, Mevlut; Ortabozkoyun, Levent; Unverdi, Selman; Kirac, Mustafa; Duranay, Murat

    2012-06-01

    Wegener granulomatosis (WG) is a necrotizing granulomatous vasculitis that predominantly affects airways and kidneys. But central nervous system involvement (7-11%) is an uncommon. Massive ICH may occur in the course of WG, and this serious condition is related with high risk of mortality. Therefore, the new treatment strategies may be considered in addition to classical practices in serious organ involvement and recurrent attack. Here, we present an adult patient with WG whose disease was complicated by a massive intracerebral hemorrhage (ICH), which subsequently led to death.

  9. Linfoma de Burkitt en un portador de granulomatosis de Wegener

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    Kryssia Rodríguez-Castro

    2005-10-01

    Full Text Available Se reporta el caso de un paciente costarricense portador de granulomatosis de Wegener, en tratamiento con ciclofosfamida y prednisona, quien desarrolla un linfoma de Burkitt que lo lleva a la muerte. Se menciona la posible relación entre estas dos patologías.This is a report of a costarrican male with Wegener' s granulomatosis undergoing treatment with prednisone and cyclophosphamide, he developed a fatal Burkitt' s lymphoma. The possible relationship between these two disorders is addressed.

  10. Bronchoplastic procedure for an unusual indication--Wegener's granulomatosis.

    LENUS (Irish Health Repository)

    Soo, Alan

    2009-09-01

    Wegener\\'s granulomatosis (WG) is a systemic vasculitic condition that commonly affects the lung and kidneys. With improvement in medical therapy, airway complications are increasingly encountered and are difficult to manage. Here, we present a case whereby a patient presenting with airway complication is successfully treated with surgery.

  11. Wegener's granulomatosis simulated by a T cell lymphoma of the lung

    OpenAIRE

    1991-01-01

    A case of primary T cell lymphoma of the lung associated with antineutrophil cytoplasmic antibody simulated Wegener's granulomatosis, the patient having features compatible with but not diagnostic of Wegener's granulomatosis.

  12. Wegener's granulomatosis in childhood

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    McHugh, K.; Manson, D. (Hospital for Sick Children, Toronto, ON (Canada). Dept. of Diagnostic Imaging); Eberhard, B.A.; Shore, A.; Laxer, R.M. (Hospital for Sick Children, Toronto, ON (Canada). Div. of Rheumatology)

    1991-12-01

    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.).

  13. Sinonasal Wegener's granulomatosis: CT characteristics

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    Benoudiba, F.; Marsot-Dupuch, K.; Rabia, Hadj M.; Lasjaunias, P. [Neuroradiology Department, Bicetre Hospital, 78 rue du General Leclerc, 94275 Le Kremlin Bicetre (France); Cabanne, J. [Department of Internal Medecine, Saint-Antoine Hospital, 184 rue du Faubourg Saint-Antoine, 75012 Paris (France); Bobin, S. [Head and Neck Department, Bicetre Hospital, 78, rue du General Leclerc, 94275 Le Kremlin Bicetre (France)

    2003-02-01

    Wegener's granulomatosis (WG) is a severe and potentially lethal granulomatosis. Even though no specific radiological criteria exist, CT may suggest the correct diagnosis at an early stage. Recent improvement in the prognosis is related to earlier diagnosis, allowing the initiation of efficient and specific treatment before any severe complications occur. We reviewed a series of WG cases in order to establish the CT diagnostic criteria. (orig.)

  14. Wegener's granulomatosis : description of a case with oral manifestation

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    Reboll Ferrer, Rosa María; Zapater Latorre, Enrique; Calabuig Crespo , Consuelo; Basterra Alegría, Jorge

    2010-01-01

    Wegener?s granulomatosis is a multisystemic granulomatous vasculitis that predominantly affects the airways and the kidneys, but may affect any organ. Otorhinolaryngological manifestations may be oral ulcers, gingival swelling or septal perforations that can cause saddle nose deformities, rhinitis, sinusitis and hearing loss. The oral ulcers usually occur when the disease is advanced. Renal involvement is characterized by focal and segmental glomerulosclerosis, and determines the evolutio...

  15. Pancreatic mass as an initial presentation of severe Wegener's granulomatosis

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    Valerieva, Yana; Golemanov, Branimir; Tzolova, Nadezhda; Mitova, Rumiana

    2013-01-01

    Acute pancreatitis or a pancreatic mass is a very rare initial presentation of Wegener's granu-lomatosis. A 62-year-old woman presented with tumor-like pancreatitis and otitis media Abdominal ultrasound and magnetic resonance suggested the presence of pancreatic tumor. Ultrasound-guided fine needle aspiration was negative. Distal pancreatic resection and splenectomy were performed and histopathology proved Wegener's vasculitis of the pancreas and spleen. Azathioprine and steroids were subsequently started and six months later the patient was asymptomatic. Involvement of the pancreas could be considered as a presenting symptom of Wegener's vasculitis. PMID:24714250

  16. Wegener's granulomatosis with unusual cavernous sinus and sella turcica extension

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    Hermann, M.; Bobek-Billewicz, B. [Dept. of Radiology, Medical University of Gdansk (Poland); Bullo, B.; Hermann, A.; Rutkowski, B. [Dept. of Nephrology, Medical University of Gdansk (Poland)

    1999-07-01

    Intracerebral extension of Wegener's granulomatosis (WG) is rare. We present a patient with oculomotor and trochlear nerve palsy with histologically proved WG. An MR examination revealed granulomatous tissue in nasal cavity, paranasal sinuses with meningeal infiltration, and uncommon penetration into cavernous sinus and sella turcica. The MR images before and during pharmacological therapy are presented. (orig.)

  17. [The first clinical description of granulomatosis with polyangiitis (known before as Wegener's granulomatosis)].

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    Mercado, Ulises

    2017-01-01

    Before 1950, cases of necrotizing vasculitis were commonly published in journals of pathology. Most of these cases were designated as polyarteritis nodosa. In 1952, the pathologist Pearl Zeek critically reviewed and summarized the literature dealing with polyarteritis nodosa and first grouped the different types of necrotizing vasculitis. But she omitted some types of not well-characterized vasculitis, among them granulomatosis with polyangiitis (Wegener's granulomatosis).

  18. Incidence of granulomatosis with polyangiitis (Wegener`s) in Greenland and the Faroe Islands

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    Faurschou, M; Helleberg, M; Obel, N

    2013-01-01

    Previous studies suggest that the incidence of granulomatosis with polyangiitis (Wegener's; GPA) increases along a south-north gradient in the Northern Hemisphere with an incidence of 8.0/million/year reported for the population of Northern Norway. In the present study, we assessed the incidence ...

  19. Incidence of granulomatosis with polyangiitis (Wegener`s) in Greenland and the Faroe Islands

    DEFF Research Database (Denmark)

    Faurschou, M; Helleberg, M; Obel, N

    2013-01-01

    Previous studies suggest that the incidence of granulomatosis with polyangiitis (Wegener's; GPA) increases along a south-north gradient in the Northern Hemisphere with an incidence of 8.0/million/year reported for the population of Northern Norway. In the present study, we assessed the incidence ...

  20. Wegener's granulomatosis: description of a case with oral manifestation.

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    Reboll-Ferrer, Rosa Maria; Zapater-Latorre, Enrique; Calabuig-Crespo, Consuelo; Basterra-Alegría, Jorge

    2010-07-01

    Wegener's granulomatosis is a multisystemic granulomatous vasculitis that predominantly affects the airways and the kidneys, but may affect any organ. Otorhinolaryngological manifestations may be oral ulcers, gingival swelling or septal perforations that can cause saddle nose deformities, rhinitis, sinusitis and hearing loss. The oral ulcers usually occur when the disease is advanced. Renal involvement is characterized by focal and segmental glomerulosclerosis, and determines the evolution. The diagnosis is made by clinical symptoms and signs, the presence of c-ANCA and a positive biopsy. The anatomic pathology is characterized by vasculitis, granulomatous inflammation with multinuclear giant cells and necrosis. The prognosis has improved as a result of treatment with immunosuppressants associated with corticosteroids. We report a case of a 53-year-old patient with Wegener's granulomatosis with oral manifestation, which began as chronic mastitis.

  1. An atypical case of Wegener`s granulomatosis complicated by sepsis and coxitis; Ziarniniakowatosc Wegenera o nietypowej lokalizacji powiklana posocznica i ropnym zapaleniem stawu biodrowego

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    Makowski, A.; Faflik, J. [Centrum Zdrowia Matki Polki, Lodz (Poland)

    1993-12-31

    There is presented an atypical case Wegener`s granulomatosis of maxillo-nasal region without bony destruction. The case is unusual because of sepsis and purulent coxitis. The patient responded well to treatment with vincristine and cyclophosphamide. ANCA (anti-neutrophil cytoplasmic antibodies) assays have very good sensitivity and specificity for Wegener`s granulomatosis. (author). 17 refs, 2 figs.

  2. Renal cancer and Wegener's granulomatosis: a case report

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    Bumbasirevic Uros

    2011-12-01

    Full Text Available Abstract Wegener's granulomatosis (WG is a systemic disorder characterized by necrotizing vasculitis involving the respiratory tract, and in most cases, the kidneys. The most common manifestation of WG in the kidneys is segmental necrotizing glomerulonephritis. The presence of a renal mass as a manifestation of WG is rare. We report a patient with WG in whom a CT scan revealed an infiltrating mass in the lower portion of the left kidney. After surgical exploration, we performed an open radical nephrectomy. Histopathology showed clear cell type renal cell carcinoma (RCC. RCC associated with WG has been reported in only a few cases, and in most of them, the diseases started simultaneously, suggesting common pathogenetic pathways. Long-term immunosuppressive treatment is a known risk factor in the development of malignancies, so occurrence of RCC in WG has been proposed as a side effect of cyclophosphamide treatment. Furthermore, it is important to make a differential diagnosis between RCC and pseudotumors in WG as they cannot be distinguished solely on basis of imaging findings. Due to the higher risk of urologic malignancies, more frequent checkups and screening of WG patients should be considered.

  3. Malignancies in Wegener's granulomatosis: incidence and relation to cyclophosphamide therapy in a cohort of 293 patients

    DEFF Research Database (Denmark)

    Faurschou, Mikkel; Sørensen, Inge Juul; Mellemkjaer, Lene

    2008-01-01

    To describe the incidence of malignancies in a cohort of Danish patients with Wegener's granulomatosis (WG) and to investigate the cancer risk associated with cyclophosphamide (CYC) -therapy in WG.......To describe the incidence of malignancies in a cohort of Danish patients with Wegener's granulomatosis (WG) and to investigate the cancer risk associated with cyclophosphamide (CYC) -therapy in WG....

  4. Kaposi's sarcoma following immune suppressive therapy for Wegener's granulomatosis.

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    Deschênes, Isabelle; Dion, Louise; Beauchesne, Claude; de Brum-Fernandes, Artur

    2003-03-01

    The association between Kaposi's sarcoma and infection with human herpesvirus 8 is now well recognized. Immunologic impairment is associated with 2 forms of Kaposi's sarcoma, epidemic [associated with human immunodeficiency virus (HIV) infection] and iatrogenic (associated with immunosuppressive treatment); both forms have become more common during the last decade. We describe an HIV negative 54-year-old man who developed Kaposi's sarcoma 2 months after the beginning of immuno-suppressive therapy for Wegener's granulomatosis (WG). With tapering of medication, complete remission of Kaposi's sarcoma was achieved in one year. To our knowledge, this is the second reported case of iatrogenic Kaposi's sarcoma in a patient with WG.

  5. Meningeal involvement in Wegener's granulomatosis is associated with localized disease.

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    Di Comite, G; Bozzolo, E P; Praderio, L; Tresoldi, M; Sabbadini, M G

    2006-01-01

    Meningeal involvement is a rare occurrence in Wegener's Granulomatosis (WG). A Medline search uncovered only 48 previously reported cases. Here we describe the clinical features of meningeal involvement in WG and to evaluate the association with systemic disease extension. Through a systematic literature review of papers concerning meningeal involvement in WG, we collected and analysed data about sex, age, disease extension, symptoms, cerebrospinal fluid examination, imaging, ANCA and histology about previously reported patients. Headache is almost always the first symptom of meningeal involvement in WG. Later in the course of the disease other abnormalities may develop. Among them cranial nerve palsy, seizures and encephalopathy are the most frequent. Diagnosis is obtained by neuroimaging, which may disclose two distinct patterns of meningeal thickening: diffuse or focal. 62.9% of patients tests positive for ANCA. Histology typically shows necrotizing granulomatosis. Meningeal involvement is by far more frequent in the setting of localized WG. Meningitis is a rare complication of WG. It usually develops in patients with localized disease who are more likely to have destructive lesions of the upper airways. It may be recognized by a constellation of clinical and radiological findings and by histological signs of necrotizing granulomatosis, with little or no vasculitis.

  6. Acute Pancreatitis as the First Presentation of Wegener's Granulomatosis

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    Mohammed Abu-Hilal

    2008-05-01

    Full Text Available Context Wegener’s granulomatosis is a systemic vasculitis with prominent involvement of the respiratory tract and kidney. An association between acute pancreatitis and Wegener's granulomatosis is rarely reported and is even rarer as the first presentation. This can result in diagnostic difficulty and may allow severe pancreatitis to develop with potentially poor outcome. Case report We report a rare case with fatal outcome of vasculitis consistent with Wegener’s granulomatosis presenting as acute pancreatitis in a 20-year-old female. The patient was admitted with worsening abdominal pain associated with nausea and loss of appetite. Accepted causes of acute pancreatitis were excluded and granulomatous vasculitis of the pancreas was confirmed from immunological profile, computed tomography and histology. As the disease progressed the patient experienced cutaneous, pulmonary, renal and severe gut involvement. Thirteen months from diagnosis the patient died of multi-organ failure despite appropriate surgical and immunosuppressive therapy. Conclusion Vasculitic disease of the pancreas is rare but should be considered when other causes have been appropriately ruled out. Careful radiological, immunological and histological diagnosis is necessary and early immunosuppressant therapy in conjunction with advice from immunologists is essential to avoid the poor outcome reported in this and other case reports.

  7. Pulmonary manifestations of wegener granulomatosis: CT findings in 57 patients and a review of the literature

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    Lohrmann, Christian; Uhl, Markus; Kotter, Elmar; Burger, Dieter; Ghanem, Nadir; Langer, Mathias

    2005-03-01

    Wegener granulomatosis is a multisystem disease of unknown cause characterized by a necrotizing granulomatous vasculitis. In comparison to other vasculitides, the lung is the most common organ involved in wegener granulomatosis presenting with a very aggressive airways pathology and chronic relapsing course. Chest radiographs fail to describe the pattern and distribution of thoracic pathology sufficiently, and CT has shown to be more sensitive for detecting lung involvement. We present the CT findings of 57 patients with wegener granulomatosis and a review of the literature.

  8. Cardiopatía valvular en un paciente con granulomatosis de Wegener Valvular cardiopathy in a patient with Wegener's granulomatosis

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    Luis E Silva

    2012-01-01

    Full Text Available La granulomatosis de Wegener es una vasculitis necrotizante autoinmune cuya prevalencia reportada es de 3/100.000 habitantes. Tiene compromiso multisistémico, principalmente el tracto respiratorio superior e inferior, y el sistema nervioso central y renal. La frecuencia del compromiso cardiaco varía según las series estudiadas, pero oscila alrededor de 6%; sin embargo, las manifestaciones clínicas son poco frecuentes. El compromiso valvular se reporta como insuficiencia aórtica o mitral, secundaria a infiltración de las valvas o dilatación de la raíz aórtica. El tratamiento se basa en el control de la enfermedad, el manejo de la falla cardiaca y la intervención quirúrgica de la válvula comprometida según la indicación.Wegener's granulomatosis is an autoimmune necrotizing vasculitis with a reported prevalence of 3/100.000 inhabitants. It is a multisystemic disease, involving mainly the upper and lower respiratory tract, the central nervous system and the kidneys. The frequency of cardiac involvement varies depending on the series studied, but oscillates around 6%; however, the clinical manifestations are rare. The valvular involvement is reported as aortic or mitral insufficiency secondary to infiltration of the leaflets, or as aortic root dilatation. Treatment is based on the control of the disease, the management of heart failure and surgical intervention of the involved valve, as directed.

  9. Diabetes Insipidus as a Complication of Wegener's Granulomatosis and Its Treatment with Biologic Agents

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    Joanna Rosalind Cunnington

    2009-01-01

    Full Text Available Wegener's granulomatosis of the pituitary gland resulting in diabetes insipidus is a rare complication of the disease. Standard treatment for Wegener's granulomatosis involves a combination of prednisolone and cylophosphamide, however biologic agents are now being used in refractory cases. We report three cases of patients with diabetes insipidus as a complication of Wegener's granulomatosis who were treated with biologic agents. All three cases showed clinical response to treatment with biologic agents including rituximab and alemtuzumab and two cases demonstrated improvement in pituitary gland abnormalities by MRI. Clinicians should be aware that diabetes insipidus can present as a complication of Wegener's granulomatosis and that biologic therapies may be effective in refractory cases.

  10. A disease activity score for ENT involvement in granulomatosis with polyangiitis (Wegener's)

    DEFF Research Database (Denmark)

    Del Pero, Marcos Martinez; Chaudhry, Afzal; Rasmussen, Niels

    2013-01-01

    Accurate assessment of disease activity in patients with otorhinolaryngological manifestations of granulomatosis with polyangiitis (Wegener's) (ENT/GPA) is necessary for treatment decisions and clinical trials. We have designed a disease activity score (ENT/GPA DAS) for this purpose....

  11. Wegener's granulomatosis patients show an adequate antibody response to influenza vaccination

    NARCIS (Netherlands)

    Holvast, A.; Stegeman, C. A.; Benne, C. A.; Huckriede, A.; Wilschut, J. C.; Palache, A. M.; Kallenberg, C. G. M.; Bij, M.

    2009-01-01

    Objectives: Wegener's granulomatosis (WG) is a systemic vasculitis characterised by relapsing and remitting disease activity. Immunosuppressive drugs are used to control disease, but increase susceptibility to infection. Therefore, influenza vaccination should be considered in WG patients. This stud

  12. Impaired health-related quality of life in patients treated for Wegener's granulomatosis

    DEFF Research Database (Denmark)

    Faurschou, Mikkel; Sigaard, Lene; Bjørner, Jakob Bue;

    2010-01-01

    To investigate whether patients with Wegener's granulomatosis (WG) experience reduced health-related quality of life (HRQOL) after accomplishment of remission, and to study the influence of WG-associated organ damage on HRQOL.......To investigate whether patients with Wegener's granulomatosis (WG) experience reduced health-related quality of life (HRQOL) after accomplishment of remission, and to study the influence of WG-associated organ damage on HRQOL....

  13. Wegener's granulomatosis: chest computed tomography findings; Granulomatose de Wegener: aspectos na tomografia computadorizada de torax

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    Monteiro, Evelise de Azevedo; Marchiori, Edson; Martins, Erick Malheiro Leoncio [Universidade Federal Fluminense, Niteroi, RJ (Brazil). Dept. de Radiologia]. E-mail: edmarchiori@zipmail.com.br; Souza Junior, Arthur Soares [Faculdade de Medicina de Sao Jose do Rio Preto (FAMERP), SP (Brazil). Hospital de Base; Cerqueira, Elza Maria F.P. de [Universidade Estadual de Campinas, SP (Brazil). Faculdade de Ciencias Medicas. Dept. de Radiologia; Irion, Klaus L. [Pavilhao Pereira Filho, Porto Alegre, RS (Brazil); Araujo Neto, Cesar de [Bahia Univ., Salvador, BA (Brazil). Dept. de Radiologia; Souza, Rodrigo Azeredo de [Universidade Federal, Rio de Janeiro, RJ (Brazil). Faculdade de Medicina. Dept. de Radiologia

    2003-03-01

    Wegeners granulomatosis is an inflammatory systemic necrotizing idiopathic vasculitis affecting mainly small vessels. In this paper we analyzed the computed tomography findings of seven patients with Wegeners granulomatosis. The most common findings were masses and nodules (71.4%), areas of ground glass attenuation (57.1%), consolidation (42.8%), halo sign (42.8%), cavitation (42.8%), interlobular septa thickening (14.2%), bronchial wall thickening (14.2%) and pleural effusion (14.2%). Although radiological findings are mostly nonspecific, computed tomography and particularly high-resolution computed tomography are important tools in the evaluation of disease activity when evaluated in association with bronchoscopy findings and clinical data. (author)

  14. 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.

  15. Kaposi's sarcoma in an elderly man with Wegener's granulomatosis treated with cyclophosphamide and corticosteroids.

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    Erban, S B; Sokas, R K

    1988-05-01

    The association of Kaposi's sarcoma with malignant lymphoreticular diseases and immunosuppressive therapy is well documented. This report describes an elderly man who presented with fulminant Wegener's granulomatosis that responded to treatment with cyclophosphamide and corticosteroids. Rapidly progressing cutaneous Kaposi's sarcoma developed ten weeks after the start of immunosuppressive therapy yet regressed on discontinuation of the corticosteroid therapy, despite continuation of cyclophosphamide therapy. To our knowledge, this is the first reported case of Kaposi's sarcoma occurring in association with Wegener's granulomatosis. The literature on Kaposi's sarcoma in immunosuppressed patients is reviewed.

  16. Wegener's granulomatosis with renal involvement: patient survival and correlations between initial renal function, renal histology, therapy and renal outcome.

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    Andrassy, K; Erb, A; Koderisch, J; Waldherr, R; Ritz, E

    1991-04-01

    Patient survival and renal outcome were followed in 25 patients with biopsy confirmed Wegener's granulomatosis and renal involvement. Fourteen out of 25 patients required dialysis on admission, 11/25 patients did not. All patients were treated with a novel protocol comprising methylprednisolone and cyclophosphamide. The median follow-up observation was 36 months (12-113 months). With the exception of 1 patient (who died from causes not related to Wegener's granulomatosis) all patients are alive. Among the patients initially requiring dialysis (n = 14) 4 are in terminal renal failure after 0, 7, 21 and 38 months respectively. In the nondialysis group (n = 11) only 1 patient subsequently required chronic dialysis 30 months after clinical admission. Renal failure was due to non-compliance with immunosuppressive therapy in at least 2 patients. Percentage of obsolescent glomeruli and the degree of tubulointerstitial lesions, but not active glomerular lesions (crescents, necroses) predicted renal outcome. The major cause of renal functional impairment was relapse of Wegener's granulomatosis usually within 2 years after clinical remission. Therefore prolonged treatment with cyclophosphamide for at least 2 years after clinical remission is recommended. Two patients with initially negative immunohistology had a second renal biopsy which revealed de novo appearance of mesangial IgA deposits.

  17. Persistent expansion of CD4(+) effector memory T cells in Wegener's granulomatosis

    NARCIS (Netherlands)

    Abdulahad, W. H.; van der Geld, Y. M.; Stegeman, C. A.; Kallenberg, C. G. M.

    2006-01-01

    In order to test the hypothesis that Wegener's granulomatosis (WG) is associated with an ongoing immune effector response, even in remission, we examined the distribution of peripheral naive and memory T-lymphocytes in this disease, and analyzed the function-related phenotypes of the memory T-cell p

  18. Plasma levels of soluble endothelial cell protein C receptor in patients with Wegener's granulomatosis

    NARCIS (Netherlands)

    Boomsma, MM; Stearns-Kurosawa, DJ; Stegeman, CA; Raschi, E; Meroni, PL; Kurosawa, S; Tervaert, JWC

    Elevated soluble thrombomodulin (sTM) levels are an accepted marker of endothelial damage. The physiological significance of plasma endothelial protein C receptor (sEPCR) levels is not known. To assess the relevance of this plasma protein in Wegener's granulomatosis (WG), sEPCR levels were measured

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

    Directory of Open Access Journals (Sweden)

    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.

  20. 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...

  1. Dilatation tracheoscopy for laryngeal and tracheal stenosis in patients with Wegener's granulomatosis

    NARCIS (Netherlands)

    Schokkenbroek, Ada A.; Franssen, Casper F. M.; Dikkers, Frederik G.

    2008-01-01

    Wegener's granulomatosis (WG) frequently involves the subglottis and trachea and may compromise the upper airway. The objective of this study is to evaluate retrospectively the effect of treatment of subglottic stenosis (SGS) and tracheal stenosis (TS) by dilatation tracheoscopy (DT) in patients wit

  2. Enhanced endothelium-dependent microvascular responses in patients with Wegener's granulomatosis

    NARCIS (Netherlands)

    Nienhuis, Hans L.A.; De Leeuw, Karina; Smit, Andries J.; Bijzet, Johan; Stegeman, Coen A.; Kallenberg, Cees G.M.; Bijl, Marc

    2007-01-01

    Objective. To assess endothelial cell (EC) function of the cutaneous microcirculation in patients with Wegener's granulomatosis (WG) and to relate EC function to EC activation and presence of atherosclerosis. Methods. We studied 28 WG patients with inactive disease and 28 age and sex matched control

  3. Detection of immune deposits in skin lesions of patients with Wegener's granulomatosis

    NARCIS (Netherlands)

    Brons, RH; de Jong, MCJM; de Boer, NK; Stegeman, CA; Kallenberg, CGM; Tervaert, JWC

    2001-01-01

    Background-Wegener's granulomatosis (WG) is considered a pauci-immune systemic vasculitis based on the absence of immune deposits in renal biopsies of patients with active disease. In animal models of antineutrophil cytoplasmic antibody (ANCA) associated glomerulonephritis, immune deposits along the

  4. Association study with Wegener granulomatosis of the human phospholipase Cγ2 gene

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    Csernok Elena

    2005-02-01

    Full Text Available Abstract Background Wegener Granulomatosis (WG is a multifactorial disease of yet unknown aetiology characterized by granulomata of the respiratory tract and systemic necrotizing vasculitis. Analyses of candidate genes revealed several associations, e.g. with α(1-antitrypsin, proteinase 3 and with the HLA-DPB1 locus. A mutation in the abnormal limb mutant 5 (ALI5 mouse in the region coding for the hydrophobic ridge loop 3 (HRL3 of the phospholipaseCγ2 (PLCγ-2 gene, corresponding to human PLCγ-2 exon 27, leads to acute and chronic inflammation and granulomatosis. For that reason, we screened exons 11, 12 and 13 coding for the hydrophobic ridge loop 1 and 2 (HRL1 and 2, respectively and exon 27 of the PLCγ-2 protein by single strand conformation polymorphism (SSCP, sequencing and PCR/ restriction fragment length polymorphism (RFLP analyses. In addition, we screened indirectly for disease association via 4 microsatellites with pooled DNA in the PLCγ-2 gene. Results Although a few polymorphisms in these distinct exons were observed, significant differences in allele frequencies were not identified between WG patients and respective controls. In addition, the microsatellite analyses did not reveal a significant difference between our patient and control cohort. Conclusion This report does not reveal any hints for an involvement of the PLCγ-2 gene in the pathogenesis of WG in our case-control study.

  5. Case of granulomatosis with polyangiitis (Wegener's granulomatosis) manifested with asymptomatic intracerebral hemorrhage.

    Science.gov (United States)

    Takaoka, Hirokazu; Hashimoto, Atsushi; Nogi, Shinichi; Iwata, Kanako; Futami, Hidekazu; Arinuma, Yoshiyuki; Shimada, Kota; Nakayama, Hisanori; Komiya, Akiko; Furukawa, Hiroshi; Matsui, Toshihiro; Tohma, Shigeto

    2013-01-01

    A 46-year-old man, who had had sinusitis, developed bilateral omalgia, petechiae on his lower extremities and a congested right eye. A blood test detected elevated serum C-reactive protein level. Computed tomography incidentally found an acute lesion of thalamic hemorrhage without neurological symptoms and no specific therapy was given at the time. Thereafter, he developed vertigo, vomiting and pneumonia for which antibiotics were ineffective. He was referred and admitted to our hospital. Further, aural and renal lesions, and presence of serum proteinase 3-antineutrophil cytoplasmic antibody (PR3-ANCA) confirmed his diagnosis of granulomatosis with polyangiitis (Wegener's) (GPA). With corticosteroid and cyclophosphamide therapy, his symptoms disappeared in two months along with faded PR3-ANCA. Afterward he showed neither new cerebral lesion nor symptom. This is a rare case of GPA manifested with asymptomatic intracerebral hemorrhage. It should be noted that GPA could cause various manifestations in central nervous system such as a fatal or an asymptomatic hemorrhagic lesion, which might respond to immunosuppressive therapy.

  6. Limited Wegener's granulomatosis presenting as lung nodules in a patient with rheumatoid arthritis: a case report.

    Science.gov (United States)

    Pai, Sushma; Panda, Mukta

    2008-12-23

    Rheumatoid arthritis has varied pleuroparenchymal manifestations. Wegener's granulomatosis can develop in an established case of rheumatoid arthritis and this association although previously reported is very rare. A 60-year-old lady had been diagnosed with rheumatoid arthritis on the basis of her clinical symptoms and serological tests which were positive RA factor and anti-CCP antibodies. Her rheumatoid arthritis activity had been mild and well controlled with hydroxychloroquine and low dose prednisone. She presented with a productive cough and right-sided pleuritic chest pain. CT scan of the chest showed three lung nodules with increased uptake on PET CT scan, raising concerns for an inflammatory or malignant process. The differential diagnosis included rheumatoid nodules, infections or malignancy. A CT-guided needle biopsy of the largest nodule was undertaken which showed vasculitis typical of Wegener's granulomatosis. Stains and cultures of the biopsy specimen were negative for bacteria, fungi and acid fast bacilli. A panel of serological tests for vasculitis were checked and showed elevated titers of cANCA and anti-proteinase 3 antibodies. Urine analysis and CT scan of paranasal sinuses was normal. Since the upper respiratory tract and the kidneys were spared a diagnosis of limited Wegener's granulomatosis affecting only the lungs was made. Due to the toxicity of cyclophosphamide, her relatively mild disease sparing the kidneys and the underlying rheumatoid arthritis, weekly methotrexate was started and low dose prednisone was continued. She had marked symptomatic improvement and complete resolution of the nodules was documented on subsequent imaging. Wegener's granulomatosis developing in a patient with rheumatoid arthritis is very rare but should be considered as it warrants a different and possibly more aggressive treatment approach.

  7. Incidence of granulomatosis with polyangiitis (Wegener's) in Greenland and the Faroe Islands

    DEFF Research Database (Denmark)

    Faurschou, M; Helleberg, M; Obel, N

    2013-01-01

    Previous studies suggest that the incidence of granulomatosis with polyangiitis (Wegener's; GPA) increases along a south-north gradient in the Northern Hemisphere with an incidence of 8.0/million/year reported for the population of Northern Norway. In the present study, we assessed the incidence...... of GPA in the predominantly Inuit population of Greenland and in the Caucasian population of the Faroe Islands....

  8. Testicular Involvement in Wegener Granulomatosis: Case Report and Review of the literature

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    Mehmet Nuri TURAN

    2012-05-01

    Full Text Available Wegener granulomatosis (WG is a type of necrotising vasculitis that affects mainly the respiratory tract, paranasal sinuses and the kidneys. Urogenital involvement is rare; mainly affecting the prostate gland. Testicular involvement in WG is exceptional. We report a 50-year-old male patient with hematuria, proteinuria and testicular pain and renal failure diagnosed as WG by testicular and kidney biopsy. We also provide a review of the literature.

  9. Granulomatose de Wegener e estenose subglótica: descrição de caso Wegener's granulomatosis and subglottic stenosis: case description

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    Fernanda Guidolin

    2004-06-01

    Full Text Available Descreve-se o caso de um paciente com Granulomatose de Wegener, em tratamento com esteróides e ciclofosfamida, que, apesar do tratamento, evoluiu com dispnéia progressiva e rouquidão. A investigação mostrou tratar-se de uma estenose subglótica, sendo a dispnéia prontamente aliviada com a traqueostomia. São discutidos diagnósticos diferenciais e modalidades de tratamentos de estenose subglótica em portadores de Wegener.We describe a patient with Wegener Granulomatosis being treated with steroids and cyclofosfamide that developed progressive dyspnea and hoarseness despite of treatment. Investigation showed a subglotic stenosis. The dyspnea was relieved by tracheotomy. We discuss the differential diagnosis as well as a number of treatment modalities in patients with Wegener granulomatosis and subglottic stenosis.

  10. A case of exorbitism in association with Wegener's granulomatosis with renal involvement.

    Science.gov (United States)

    Beji, S; Fatma, L Ben; Chebbi, A; Rais, L; Krid, M; Smaoui, W; Maiz, H Ben; Zouaghi, K; Moussa, F Ben

    2012-03-01

    Wegener's granulomatosis (WG) is a necrotizing granulomatous vasculitis involving 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 glomerulonephritis. 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 bilateral exophthalmos. Prompt recognition of this early manifestation is important for the institution of early treatment.

  11. Goodpasture syndrome involving overlap with Wegener's granulomatosis and anti-glomerular basement membrane disease.

    Science.gov (United States)

    Kalluri, R; Meyers, K; Mogyorosi, A; Madaio, M P; Neilson, E G

    1997-11-01

    A 68-year-old Caucasian woman presented to the hospital with nodular pulmonary infiltrates and acute renal failure. Wegener's granulomatosis was initially considered to be most likely because of the presence of increased serum levels of c-anti-neutrophil cytoplasmic antibodies (c-ANCA). A consultation through the Internet after a renal biopsy demonstrated crescentic, necrotizing glomerulonephritis and linear deposits of immunoglobulin G (IgG) and complement C3, typical of anti-glomerular basement membrane (GBM) disease. Hemodialysis was instituted; however, the patient suddenly developed a massive cerebral hemorrhage and died before full therapy could take effect. Postmortem analysis of the patient's sera revealed high titers of IgG against the alpha 3 NC1 domain of type IV collagen. Serologic evidence of both p-ANCA and anti-GBM antibodies are becoming more frequently recognized in the setting of rapidly progressive glomerulonephritis. The patient reported here had the unusual combination of c-ANCA antibodies with anti-GBM disease, and this association raises complex questions regarding the pathogenesis of this type of renal injury.

  12. Polymicrobial Infections in the Early Period of Kidney Transplantation in a Case with Wegener's Granulomatosis

    Directory of Open Access Journals (Sweden)

    Funda COŞKUN

    2011-01-01

    Full Text Available Wegener's granulomatosis (WG is a disorder that causes necrotizing granulomatosis vasculitis particularly of the upper respiratory tract, lung and kidney. A 43-year-old male who had been treated with hemodialysis because of renal insufficiency due to WG underwent live donor renal transplantation. Pulmonary infiltrates were detected on the postoperative 4th day and antibiotic therapy was started with a diagnosis of sepsis and pulmonary infection. Dialysis treatment was also started due to the degradation of renal function for the patient who was intubated during follow-up. Non-invasive mechanical ventilation (BiPAP treatment was started after extubation. The graft and respiratory function improved during clinical follow-up. Resistant hospital infections, causing respiratory failure and systemic complications, were facilitated by a long history of hospitalization before transplantation, the presence of WG and immunosupression and were successfully treated with a multidisciplinary approach.a

  13. Wegener`s granulomatosis and mucoromycosis: A case study and review of literature

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    Sayyed Mojtaba Abtahi

    2012-01-01

    Full Text Available Mucormycosis is a fatal invasive infection which mostly involves diabetic or immunosuppressed patients. Early diagnosis, improving immunosuppression, systemic antifungal therapy, and surgical debridement are necessary for successful treatment. In this case study, we represent a known case of Wegener′s granulomatosis (WG, with concomitant sinusal mucormycosis mimicking vasculitic disease relapse, which was successfully treated with surgical debridement, amphotericine, and intravenous immunoglobuline.

  14. 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......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...

  15. Pituitary involvement in Wegener ' s granulomatosis: a case report and review of the literature

    Institute of Scientific and Technical Information of China (English)

    陶建瓴; 董怡

    2003-01-01

    Wegener ' s Granulomatosis (WG) is a multi-system disorder characterized by necrotizing granulomas in the upper and/or lower respiratory tract, with or without focal necrotizing glomerulonephritis and other systemic vasculitis.1 Clinical manifestations may vary with the site of involvement and the extension of the lesions. Pituitary involvement is rare, and to our knowledge no more than ten cases with this complication have been published in the literature in English. We report here an additional WG case complicated by pan-hypopituitarism.

  16. Increased morbidity from ischemic heart disease in patients with Wegener's granulomatosis

    DEFF Research Database (Denmark)

    Faurschou, Mikkel; Mellemkjaer, Lene; Sørensen, Inge Juul;

    2009-01-01

    OBJECTIVE: Experimental studies indicate that patients with Wegener's granulomatosis (WG) experience accelerated atherosclerosis. The purpose of this study was to investigate whether the occurrence of overt ischemic heart disease (IHD) is increased in WG. METHODS: A total of 293 WG patients were...... MI were found in patients who were > or =50.0 years of age at the time of diagnosis of WG, in male patients, and in patients who received high cumulative doses of cyclophosphamide. CONCLUSION: Compared with the background population, WG patients seem to experience an increased number of both early...

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

    Directory of Open Access Journals (Sweden)

    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.

  18. Granulomatous renal pseudotumor in Wegener's granulomatosis: imaging findings in one case

    Energy Technology Data Exchange (ETDEWEB)

    Verswijvel, G.; Eerens, I.; Oyen, R. [Department of Radiology, University Hospitals. Catholic University of Leuven, Leuven (Belgium); Messiaen, T. [Department of Nephrology, University Hospitals. Catholic University of Leuven, Leuven (Belgium)

    2000-08-01

    Wegener's granulomatosis is a clinico-pathological entity characterized by necrotizing granulomatous angiitis involving the upper- and/or lower respiratory tract and the kidneys. Renal involvement is usually characterized by a rapidly progressive necrotizing glomerulonephritis. A case is presented of a patient who developed renal failure and presented a solitary pseudotumoral lesion in the upper pole of the left kidney. Imaging characteristics on US, CT and MR imaging are discussed. The diagnosis was confirmed with ultrasound-guided needle biopsy. (orig.)

  19. [Systemic diseases--the significance of early diagnosis exemplified by systemic lupus erythematosus and Wegener's granulomatosis].

    Science.gov (United States)

    Türk, H

    1993-12-01

    New therapeutic modalities have shown remarkable advances in the fields of systemic lupus erythematosus and Wegener's granulomatosis. For an optimal clinical outcome therapy has to be started early and must be adapted to disease activity. Concerning these two points early diagnosis is essential. This aim can be reached by a detailed evaluation of the patient's history by focusing on early symptoms and on typical clinical constellations, taking into account that both diseases show a great variability and sometimes even an insidious course. Immunological tests, capillary microscopy, echocardiography and computed tomography can be very helpful in the diagnosis of these diseases.

  20. Prevalence of hearing impairment in patients with rheumatoid arthritis, granulomatosis with polyangiitis (GPA, Wegener's granulomatosis), or systemic lupus erythematosus.

    Science.gov (United States)

    Rahne, Torsten; Clauß, Franziska; Plontke, Stefan K; Keyßer, Gernot

    2017-07-01

    Hearing loss in patients with autoimmune diseases, such as systemic lupus erythematosus (SLE), granulomatosis with polyangiitis (GPA, Wegener's granulomatosis), or rheumatoid arthritis (RA), is controversial. Many studies lack measurements of bone-conduction thresholds to sufficiently differentiate between sensorineural hearing loss and conductive hearing loss. In addition, many studies lack control groups or comparisons to an age-related normal hearing threshold. This study investigates hearing performance with an extended audiological battery using psychoacoustic and objective measures. A total of 22 adults with RA, 16 with GPA, 20 with SLE, and two age- and gender-matched control groups (n = 34 for GPA and RA and n = 42 for SLE) were included. Pure-tone hearing thresholds, speech perception in quiet and noise, tympanometry, and high-resolution otoacoustic emissions were assessed. GPA patients exhibited impaired pure-tone hearing compared to the control group, whereas SLE and RA patients did not. In GPA patients, a larger air-bone gap indicated conductive hearing loss. In addition, speech perception was reduced exclusively in GPA patients. A significant correlation was found between hearing loss and both the cumulative steroid dose and number of organ manifestations in GPA and SLE patients. Our data indicate that GPA and SLE patients are at moderate-to-high risk of conductive hearing loss. In contrast, RA patients are at low risk of disease-associated hearing loss.

  1. Pseudotumor pulmonar como apresentação inicial de granulomatose de Wegener Lung pseudotumor as the initial presentation of Wegener's granulomatosis

    Directory of Open Access Journals (Sweden)

    Marcelo Fouad Rabahi

    2009-04-01

    Full Text Available A granulomatose de Wegener é uma doença inflamatória multissistêmica de etiologia desconhecida, que se caracteriza por vasculite de pequeno e médio calibre. As manifestações clínicas mais comuns envolvem o trato respiratório superior, pulmões e rins e as alterações encontradas nos exames de imagem são geralmente opacidades pulmonares ou nódulos múltiplos, bilaterais e, em 50% dos casos, cavitações. O tratamento é feito com corticoides e imunossupressores. Descreve-se um caso atípico de um homem de 61 anos cuja investigação inicial de um tumor pulmonar unilateral evidenciou, na verdade, Granulomatose de Wegener.Wegener's granulomatosis is a multisystemic inflammatory illness of unknown etiology, characterized by vasculitis of small and medium caliber vessels. The most common clinical manifestations involve the upper respiratory tract, lungs and kidneys. Common alterations in imaging studies include pulmonary opacities and bilateral multiple nodules, cavitations occurring in 50% of the cases. Treatment includes corticosteroids and immunosuppressants. We describe an atypical case of a 61-year-old man initially investigated due to suspicion of a unilateral lung tumor, which proved to be, in fact, a case of Wegener's granulomatosis.

  2. Meningeal involvement in Wegener granulomatosis: case report and review of the literature.

    Science.gov (United States)

    Soriano, A; Lo Vullo, M; Casale, M; Quattrocchi, C C; Afeltra, A

    2012-01-01

    Wegener Granulomatosis (WG) is a multisystem autoimmune disorder characterized by necrotizing granulomatous vasculitis that most commonly involves the upper respiratory tract, lungs, and kidneys. The involvement of the central nervous system (CNS) is infrequent and can cause stroke, cranial nerve abnormalities, cerebrovascular events, seizures, and meningeal involvement. Meningeal involvement is rare and may occur due to local vasculitis, directly spread from adjacent disease in the skull base, paranasal or orbital region. We describe the case of a 20-year-old Caucasian man who was diagnosed with sinonasal WG with frontal focal meningeal involvement. A literature review on diagnosis and treatment of meningeal involvement in course of WG was carried out. The importance of an early diagnosis and treatment of localized WG has been emphasized, in order to avoid the progression to a severe form of disease, especially in younger patients and in paucisymptomatic cases.

  3. Combined CNS and pituitary involvement as a primary manifestation of Wegener granulomatosis.

    Science.gov (United States)

    Spísek, Radek; Kolouchová, Elena; Jensovský, Jirí; Rusina, Robert; Fendrych, Pavel; Plas, Jaroslav; Bartůnková, Jirina

    2006-09-01

    Wegener granulomatosis (WG) is a systemic vasculitis of small and medium vessels. It predominantly affects the upper and/or lower respiratory airway and kidneys. Its pathogenesis is not fully understood. WG relatively frequently affects the nervous system (in 30-50% according to the different studies). Most frequently, it manifests as necrotizing vasculitis that leads to the peripheral neuropathies or to the cranial nerves palsy. Impairment of the central nervous system (CNS) is less frequent and occurs in 2-8% of patients. Three major pathogenetic mechanisms were described: CNS vasculitis, spreading of granulomas from the adjacent anatomical areas (paranasal cavities, orbit etc.), and new formation of granulomas in brain tissue. This case report describes patients in whom WG manifested in the form of localized skin involvement and combined CNS involvement that included pituitary gland. Atypical presentation of WG impedes and slows down the process of diagnosis and emphasizes the need for collaboration between medical specialists.

  4. Cardiopatía valvular en un paciente con granulomatosis de Wegener

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    Luis E. Silva, MD

    2012-01-01

    Full Text Available La granulomatosis de Wegener es una vasculitis necrotizante autoinmune cuya prevalencia reportada es de 3/100.000 habitantes. Tiene compromiso multisistémico, principalmente el tracto respiratorio superior e inferior, y el sistema nervioso central y renal. La frecuencia del compromiso cardiaco varía según las series estudiadas, pero oscila alrededor de 6%; sin embargo, las manifestaciones clínicas son poco frecuentes. El compromiso valvular se reporta como insuficiencia aórtica o mitral, secundaria a infiltración de las valvas o dilatación de la raíz aórtica. El tratamiento se basa en el control de la enfermedad, el manejo de la falla cardiaca y la intervención quirúrgica de la válvula comprometida según la indicación.

  5. [Tracheal resection for post-intubation subglottic stenosis in a patient with granulomatosis with polyangiitis (Wegener)].

    Science.gov (United States)

    Stoica, Radu; Negru, Irina; Matache, Radu; MirunaTodor

    2014-01-01

    Granulomatosis with polyangiitis (GPA or Wegener) is a systemic autoimmune disease with inflammation of small- and medium-size vessels. It can affect practically any organ or system, but renal, respiratory andjoint systems are most frequently damaged. Positive pANCA antibodies can raise the suspicion of diagnosis. Subglottic stenosis is relatively frequent, in a quarter of patients, especially in the third decade women. The case presented is of an 80-year-old woman, recently diagnosed with pulmonary, renal and systemic manifestations of GPA and with a subglottic stenosis rapidly evolving towards endotracheal intubation, tracheostomy with mechanical ventilation and renal failure. Further evolution has been favorable under corticoid therapy. After weaning from the mechanical ventilation and30 days after the suppression of the tracheostomy, the patient developed a tracheal stenosis with mixed etiology, secondary to vasculitis and prolonged intubation with tracheostomy. Tracheal resection with termino-terminal anastomosis was performed in emergency with simple post-operative evolution and without late complications.

  6. [Tracheal resection for post-intubation subglottic stenosis in a patient with granulomatosis with polyanaiitis (Wegener)].

    Science.gov (United States)

    Stoica, Radu; Negru, Irina; Matache, Radu; MirunaTodor

    2014-01-01

    Granulomatosis with polyangiitis (GPA or Wegener) is a systemic autoimmune disease with inflammation of small- and medium-size vessels. It can affect practically any organ or system, but renal, respiratory andjoint systems are most frequently damaged. Positive pANCA antibodies can raise the suspicion of diagnosis. Subglottic stenosis is relatively frequent, in a quarter of patients, especially in the third decade women. The case presented is of an 80-year-old woman, recently diagnosed with pulmonary, renal and systemic manifestations of GPA and with a subglottic stenosis rapidly evolving towards endotracheal intubation, tracheostomy with mechanical ventilation and renal failure. Further evolution has been favorable under corticoid therapy. After weaning from the mechanical ventilation and30 days after the suppression of the tracheostomy, the patient developed a tracheal stenosis with mixed etiology, secondary to vasculitis and prolonged intubation with tracheostomy. Tracheal resection with termino-terminal anastomosis was performed in emergency with simple post-operative evolution and without late complications.

  7. Airway Surgery in Tracheostomised Patients with Wegener Granulomatosis Leading to Subglottic Stenosis.

    Science.gov (United States)

    Altun, Demet; Sivrikoz, Nükhet; Çamcı, Emre

    2015-10-01

    Wegener granulomatosis (WG) is a multisystemic disorder characterised by granulomatous inflammation of the respiratory system. The growing of proliferative tissue towards the larynx and trachea may cause airway obstruction on account of subglottic stenosis. In this situation, the surgical goal is to eliminate the airway obstruction by providing natural airway anatomy. While mild lesions do not require surgical intervention, in fixed lesions, surgical intervention is required, such as tracheostomy, laser resection and dilatation. In tracheostomised patients, granuloma formation surrounding the tracheostomy cannula may occur in the trachea. Inflammation and newly formed granulation tissue result in severe stenosis in the airways. During surgical treatment of such patients, airway management is important. In this case report, we will discuss gas exchange and airway management with jet ventilation (JV) during excision of the granulation tissue with endolaryngeal laser surgery, leading to subglottic stenosis in tracheostomised patients in WG.

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

    Directory of Open Access Journals (Sweden)

    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.

  9. Wegener's Granulomatosis

    Science.gov (United States)

    ... to) Wegener’s, but no specific infection (bacterial, viral, fungal, or other) has been identified. How is Wegener’s ... ANCAs. Some patients with inflammatory bowel disease, rheumatoid arthritis, or SLE may have atypical P-ANCA test ...

  10. [A case of rapidly progressive glomerulonephritis in the course of Wegener's granulomatosis].

    Science.gov (United States)

    Idasiak-Piechocka, I; Oko, A; Łochyńska, K; Woźniak, A; Czekalski, S

    2000-01-01

    Wegener's granulomatosis (WG) is characterized by granulomatous vasculitis of the respiratory tract and glomerulonephritis (GN). Prognosis of this disease is poor and about 20% of untreated patients die after one year from the onset. WG was recognized in 45-year-old patient on the basis of: 1) clinical symptoms (joint pain and swollen, purpura on the skin which appeared one week after respiratory tract infection, ulceration of the tonsils and lingula), 2) results of additional testing (X-chest-ray-infiltrates of both lungs), positive results of the cANCA (titre 1:640) and rapidly progressive renal failure [the increase of serum creatinine level (Pcr) from 123.7 to 707 mumol/l (1.4 to 8.0 mg/dl) during one week]. Renal biopsy revealed extracapillary GN (cellular crescents in 7 out of 8 glomeruli and scattered foci of fibrinoid necrosis of capillary walls in all). At the beginning of the treatment Pcr raised to 884 mumol/l (10 mg/dl) and the patient required hemodialysis. He was treated with methylprednisolone (M) at flash doses of 1000 mg/24 h by three days followed by 125 mg/24 h i.v.--because of peptic ulcer, with cyclophosphamide (C-150 mg/24 h p.p.), with trimetoprim/sulphametoxazole, with pentoxifylline and omeprazol. After six weeks of the treatment in the control kidney biopsy sclerotic changes in 10 out of 13 glomeruli and diffuse interstitial fibrosis were found. However, during the same time, we observed clinical remission of the disease and the decrease of Pcr to 176.8 mumol/l (2 mg/dl). The M dosis was reduced by 5 mg every weeks and the C dosis--to 50 mg (because of the increase of aminotransferase levels) After six months of the treatment Pcr was 132.6 mumol/l (1.5 mg/dl) and CANCA titer was 1:16. In this case of RPGN, despite off the progression of the morphological changes in the kidney, we obtained the clinical remission of the disease and significant decrease of Pcr level. These results suggest that aggressive treatment of WG is justified even in

  11. Estenosis subglótica y granulomatosis con poliangeítis (Wegener) en dos casos: Report of two cases

    OpenAIRE

    Massa,María; Nicholas C. Emery; Bosio, Martín; Finn,Bárbara C; Bruetman,Julio E; Young,Pablo

    2014-01-01

    Granulomatosis with polyangiitis (GPA) or Wegener's disease is characterized by a granulomatous vasculitis of the upper and lower airways and kidney. It involves the lower respiratory tract causing subglottic tracheal stenosis, which occurs in approximately 22% of patients. We report two females aged 40 and 52 years, admitted to the hospital with a subglottic tracheal stenosis. Their symptoms and management are reviewed. The frst patient responded to rituximab. The second patient required a t...

  12. Comprometimento da árvore respiratória na granulomatose de Wegener Laryngeal and tracheobronchial involvement in Wegener's granulomatosis

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    Ascedio Jose Rodrigues

    2012-04-01

    Full Text Available INTRODUÇÃO: A granulomatose de Wegener (GW é uma forma de vasculite sistêmica que envolve primariamente as vias aéreas superiores e inferiores e os rins. As manifestações mais frequentes nas vias aéreas são estenose subglótica e inflamações, estenoses da traqueia e dos brônquios. A visualização endoscópica das vias aéreas é a melhor ferramenta para avaliação, diagnóstico e manejo dessas alterações. OBJETIVOS: Descrever as alterações endoscópicas encontradas na mucosa das vias aéreas de um grupo de pacientes com GW submetido à broncoscopia no Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo (HC-FMUSP e relatar as intervenções terapêuticas broncoscópicas utilizadas em alguns casos. MÉTODOS: Foram estudados 15 pacientes com diagnóstico de GW provenientes do Ambulatório de Vasculites da Disciplina de Pneumologia do HC-FMUSP, encaminhados para a realização de broncoscopia no serviço de Endoscopia Respiratória do HC-FMUSP no período de 2003 a 2007. RESULTADOS: Dos 15 pacientes avaliados, 11 eram mulheres (73,33% com idade média de 34 ± 11,5 anos. Foram encontradas alterações das vias aéreas em 80% dos pacientes, e o achado endoscópico mais frequente foi estenose subglótica (n = 6. Realizou-se broncoscopia terapêutica em três pacientes com estenose subglótica e em outros três com estenose brônquica, todos apresentando bons resultados. CONCLUSÃO: A broncoscopia permite diagnóstico, acompanhamento e tratamento das lesões de vias aéreas na GW, constituindo-se um recurso terapêutico pouco invasivo em casos selecionados.INTRODUCTION: Wegener's granulomatosis (WG is a form of systemic vasculitis that involves primarily the upper and lower airways and the kidneys. The most frequent airway manifestations include subglottic stenosis and inflammation, and tracheal and bronchial stenoses. The endoscopic visualization of the airways is the best tool for assessing, diagnosing and

  13. On the Wegener granulomatosis associated region on chromosome 6p21.3

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    Csernok Elena

    2006-03-01

    Full Text Available Abstract Background Wegener granulomatosis (WG belongs to the heterogeneous group of systemic vasculitides. The multifactorial pathophysiology of WG is supposedly caused by yet unknown environmental influence(s on the basis of genetic predisposition. The presence of anti-neutrophil cytoplasmic antibodies (ANCA in the plasma of patients and genetic involvement of the human leukocyte antigen system reflect an autoimmune background of the disease. Strong associations were revealed with WG by markers located in the major histocompatibility complex class II (MHC II region in the vicinity of human leukocyte antigen (HLA-DPB1 and the retinoid X receptor B (RXRB loci. In order to define the involvement of the 6p21.3 region in WG in more detail this previous population-based association study was expanded here to the respective 3.6 megabase encompassing this region on chromosome 6. The RXRB gene was analysed as well as a splice-site variation of the butyrophilin-like (BTNL2 gene which is also located within the respective region. The latter polymorphism has been evaluated here as it appears as a HLA independent susceptibility factor in another granulomatous disorder, sarcoidosis. Methods 150–180 German WG patients and a corresponding cohort of healthy controls (n = 100–261 were used in a two-step study. A panel of 94 microsatellites was designed for the initial step using a DNA pooling approach. Markers with significantly differing allele frequencies between patient and control pools were individually genotyped. The RXRB gene was analysed for single strand conformation polymorphisms (SSCP and restriction fragment length polymorphisms (RFLP. The splice-site polymorphism in the BTNL2 gene was also investigated by RFLP analysis. Results A previously investigated microsatellite (#1.0.3.7, Santa Cruz genome browser (UCSC May 2004 Freeze localisation: chr6:31257596-34999883, which was used as a positive control, remained associated throughout the whole two

  14. [Subglottic stenosis in Wegener's granulomatosis--a diagnostic and therapeutic problem].

    Science.gov (United States)

    Wierzbicka, Małgorzata; Gaweqcki, Wojciech; Pastusiak, Tomasz; Szyfter, Witold

    2010-01-01

    Wegener's granulomatosis (WG) is a disease caused by necrotising vasculitis of small and middle blood vessels of upper and lower respiratory tract and kidneys of unknown etiology. ENT symptoms develop in more than 90% of patients and tracheobronchial involvement occurs in about 20% of patients, most often as a subglottic stenosis. Subglottic stenosis occurs usually as a late complication of disease, but sometimes it may be an early isolated symptom. It is usually irregular, no longer than 2-4 cm and affects mucosa and submucosa but sometimes also cartilage. The diagnosis is based on clinical symptoms, X-ray of the chest, urine analysis, c-ANCA detection and histological examination of the granulation from the stenosis. The main treatment of subglottic stenosis in WG is a causal immunosuppressive treatment, however an equally important is a preservation of respiratory tract passage, because a dominant symptom in this form of WG is problem with breathing or even dyspnoea. In this publication basing on literature review different methods of treatment of laryngotracheal stenosis and its importance in WG are described and discussed. In literature presently less invasive operations are recommended. The treatment of choice is endoscopic treatment with repeated dilatations and injections of steroid into the stenosis. In big stenosis in immunologically active disease patient sometimes requires tracheotomy and after remission of disease and maturation of the subglottic scar laryngotracheal resection can be considered.

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

    Directory of Open Access Journals (Sweden)

    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.

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

    Directory of Open Access Journals (Sweden)

    Gustavo Adolfo Martín Small

    2009-01-01

    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.

  17. 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

    2004-01-01

    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), r

  18. RELEVANCE OF CLASSIC ANTINEUTROPHIL CYTOPLASMIC AUTOANTIBODY (C-ANCA)-MEDIATED INHIBITION OF PROTEINASE 3-ALPHA-1-ANTITRYPSIN COMPLEXATION TO DISEASE-ACTIVITY IN WEGENER-GRANULOMATOSIS

    NARCIS (Netherlands)

    DOLMAN, KM; STEGEMAN, CA; VANDEWIEL, BA; HACK, CE; BORNE, AEGKV; KALLENBERG, CGM; GOLDSCHMEDING, R

    1993-01-01

    In the sera of patients with Wegener's granulomatosis (WG), C-ANCA can be detected that are directed against proteinase 3 (PR3). We have previously observed that C-ANCA interfere with PR3 proteolytic activity and with complexation of PR3 with its major physiologic inhibitor, alpha1-antitrypsin (alph

  19. The prevalence of the Staphylococcus aureus tst gene among community- and hospital-acquired strains and isolates from Wegener's Granulomatosis patients

    NARCIS (Netherlands)

    Deurenberg, Ruud H; Nieuwenhuis, Rutger F; Driessen, Christel; London, Nancy; Stassen, Frank R; van Tiel, Frank H; Stobberingh, Ellen E; Vink, Cornelis

    2005-01-01

    To allow rapid identification of toxic shock syndrome toxin-1 (TSST-1)-producing Staphylococcus aureus strains, a real-time PCR assay for the detection of the tst gene, which encodes TSST-1, was developed. The assay was applied to S. aureus isolates from patients with Wegener's Granulomatosis (WG),

  20. PREDOMINANCE OF IGG1 AND IGG4 SUBCLASSES OF ANTINEUTROPHIL CYTOPLASMIC AUTOANTIBODIES (ANCA) IN PATIENTS WITH WEGENERS GRANULOMATOSIS AND CLINICALLY RELATED DISORDERS

    NARCIS (Netherlands)

    BROUWER, E.; Tervaert, J.W.C.; HUITEMA, M.G.; VANDERGIESSEN, M.; Limburg, Piet; Kallenberg, Cees; Horst, G.

    1991-01-01

    In view of the supposed hypersensitivity, the elevated levels of IgE, and the occurrence of eosinophilia reported in Wegener's granulomatosis and related conditions, we studied the IgG subclass distribution of ANCA directed against a 29-kD serine protease and and myeloperoxidase (MPO) in 41 untreate

  1. 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)....

  2. 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...... with 19 population controls (n = 3,420). Information on hospitalizations for stroke, PE, and DVT was obtained from the Danish National Hospital Register. The occurrence of vascular events in the GPA cohort was compared with that in the control group by calculation of incidence rate ratios (IRRs). RESULTS......: The median duration of followup was 7.2 years (interquartile range 3.1-11.7 years) in the GPA cohort. Within the first 2 years following the diagnosis of vasculitis, the incidences of PE and DVT were substantially increased among the patients (IRR 25.7 [95% confidence interval (95% CI) 6.9-96] for PE and IRR...

  3. 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

    OBJECTIVE: To uniquely classify children with microscopic polyangiitis (MPA), to describe their demographic characteristics, presenting clinical features, and initial treatments in comparison to patients with granulomatosis with polyangiitis (Wegener's) (GPA). METHODS: The European Medicines Agency...... 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...... to diagnosis was 1.6 months in the MPA group and 2.1 months in the GPA group (ranging to 39 and 73 months, respectively). Patients with MPA were significantly younger than those with GPA (median age 11 years versus 14 years). Constitutional features were equally common between the groups. In patients with MPA...

  4. Tumour necrosis factor-alpha (TNF), lymphotoxin and TNF receptor levels in serum from patients with Wegener's granulomatosis

    DEFF Research Database (Denmark)

    Jónasdóttir, O; Petersen, J; Bendtzen, K

    2001-01-01

    -R), and these receptors are often found in soluble forms (sTNF-R), which can modulate TNFalpha actions. To evaluate the clinical importance of the TNF family of cytokines, the serum levels of TNFalpha, TNFbeta, now termed lymphotoxin (LTalpha), and sTNF-R1 and sTNF-R2 were measured by ELISA in 8 patients with WG during......Wegener's granulomatosis (WG) is a systemic inflammatory disease with vasculitis as the key feature. Abnormal expression of tumour necrosis factor alpha (TNFalpha) is considered of prime pathogenic importance in several inflammatory diseases. The effects of TNFa are mediated by TNF receptors (TNF...... of the relative amounts of TNFalpha and sTNF-R indicated that TNFalpha was mostly bound to its soluble receptors. In WG, the serum levels of sTNF-R1 and sTNF-R2 were dramatically increased (p...

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

    Directory of Open Access Journals (Sweden)

    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.

  6. Progressive renal disease despite immunosuppressive therapy in a patient with Wegener s granulomatosis.

    NARCIS (Netherlands)

    Klein, I.; Vervoort, G.; Steenbergen, E.; Wetzels, J.

    2008-01-01

    We present a patient with Morbus Wegener and crescentic glomerulonephritis. Treatment with cyclophosphamide and prednisolone resulted in the disappearance of signs and symptoms of systemic inflammation. However, renal function deteriorated. Renal biopsy showed evidence of continuing capillary necros

  7. Perda auditiva neurossensorial em pacientes com granulomatose de Wegener: relato de três casos e revisão de literatura Neurosensory hearing loss in patients with Wegener's granulomatosis: a report of three cases and literature review

    Directory of Open Access Journals (Sweden)

    Ana Paula Monteiro Gomides

    2006-06-01

    Full Text Available A granulomatose de Wegener é uma vasculite sistêmica que acomete vasos de pequeno e médio calibres. As manifestações clássicas ocorrem no trato respiratório superior, inferior e rins, mas outros órgãos podem ser envolvidos. O sistema auditivo pode ser freqüentemente acometido nas suas várias porções (orelha externa, média e/ou interna com manifestações diversas. A perda auditiva neurossensorial pode ser conseqüência de alterações na orelha interna e deve ser precocemente reconhecida. O tratamento rápido e efetivo pode evitar danos irreversíveis. Descrevemos três casos de granulomatose de Wegener e hipoacusia sensorial, com respostas diferentes ao tratamento.Wegener's granulomatosis is a primary systemic vasculitis that affects small and medium-sized vessels. The classical manifestations occur in the upper and lower respiratory tract and in the kidneys, but other organs may be involved. The auditory system can be frequently affect in its various portions (outer, middle and/or inner ear, showing different manifestations. Neurosensory hearing loss might be a consequence of changes in the inner ear and should be recognized early. Rapid and effective treatment may prevent irreversible damage. We report herein three cases of Wegener's granulomatosis and sensory hypoacusis, which responded differently to treatment.

  8. Manifestaciones clínicas de la granulomatosis de Wegener y la poliangeítis microscópica en Santiago-Chile, 1990-2001

    OpenAIRE

    Cisternas M,Marcela; Soto S,Lilian; Jacobelli G,Sergio; Marinovic M,María Angélica; Vargas B,Alex; Sobarzo V,Elizabeth; Saavedra M,Jorge; Chauan I,Karina; Meléndez T,Gabriela; Foster B,Carolina; Pacheco R,Daniel; Wainstein G,Eduardo

    2005-01-01

    Background: Systemic vasculitis are a group of heterogeneous diseases characterized by inflammation and necrosis of blood vessel walls. The etiology is not known, but geographic and environmental factors are implicated. Aim: To describe the clinical features of microscopic polyangiitis (MPA) and Wegener's granulomatosis (WG) in a Chilean cohort of patients. Patients and methods: Retrospective review of the medical records of 123 patients with the diagnosis of systemic vasculitis (65 MPA and 5...

  9. Epidemiology and etiology of wegener granulomatosis, microscopic polyangiitis, churg-strauss syndrome and goodpasture syndrome: vasculitides with frequent lung involvement.

    Science.gov (United States)

    Gibelin, Aude; Maldini, Carla; Mahr, Alfred

    2011-06-01

    This review focuses on the epidemiological characteristics and etiologies of four primary systemic vasculitides with frequent lung involvement, namely Wegener granulomatosis (WG), microscopic polyangiitis (MPA), Churg-Strauss syndrome (CSS), and Goodpasture syndrome (GPS). Elucidation of the mechanisms underlying these vasculitides with frequent lung involvement is complicated by their rarity, which hampers the undertaking of large-scale studies; difficulties in classification; and their multifaceted clinical presentations, which infer the existence of several etiologic pathways. Notwithstanding, epidemiological research showed some evidence for international, interethnic, and temporal variations of the frequencies of these four vasculitides; led to the identification of several genetic and environmental risk factors; and provided insight on the extent to which genes and environment might contribute to their development. Available data support the concept that WG, MPA, CSS, and GPS have unique and shared risk determinants. Although the precise causes of these vasculitides are not yet fully understood and the development of prevention strategies is out of our reach at present, current knowledge enables the formulation of etiologic hypotheses to provide caregivers and their patients with valuable information on the nature of these rare entities. © Thieme Medical Publishers.

  10. 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

    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...

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

    Directory of Open Access Journals (Sweden)

    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

  12. 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....

  13. Vasculitis de Wegener: diferentes presentaciones pulmonares en el diagnóstico inicial y durante la evolución de la enfermedad Wegener granulomatosis: different pulmonary abnormalities at initial diagnostic and during the course of disease

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    Marta Hernandorena González

    2009-09-01

    Full Text Available La enfermedad de Wegener es una vasculitis granulomatosa necrotizante, con afectación característica del tracto respiratorio superior, pulmones y riñones. El 90% de los pacientes tienen afectación pulmonar y los hallazgos radiográficos más frecuentes son nódulos o masas bilaterales múltiples que pueden mostrar cavitación. También se puede presentar como consolidación lobar o segmentaria o como hemorragia pulmonar difusa. El reconocimiento precoz de las alteraciones específicas es crítico para realizar un diagnóstico apropiado y para reducir la morbi-mortalidad. Con el objetivo de lograr una mejor caracterización de las distintas manifestaciones torácicas de esta entidad presentamos cuatro casos que muestran sus distintas manifestaciones radiológicas.Wegener granulomatosis is a necrotizing granulomatous vasculitis that affects the upper respiratory tract, lungs, and kidneys. At least 90% of patients have pulmonary involvement, and the typical radiographic findings are bilateral multiple nodules or masslike lesions, some of which show cavitation. Other frequent findings are consolidations or diffuse pulmonary hemorrhage. Early recognition of specific abnormalities is critical for appropriate intervention and reduced morbidity. To better characterize the diversity of thoracic manifestations in this disease we present four cases to show different radiologic findings in this population.

  14. Lesões destrutivas da linha média induzidas por cocaína com ANCA positivo mimetizando a granulomatose de Wegener Cocaine-induced midline destruction lesions with positive ANCA test mimicking Wegener's granulomatosis

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    Letícia Stahelin

    2012-06-01

    Full Text Available O uso crônico de cocaína por inalação pode causar lesões destrutivas de linha média (LDLMIC, que podem ser difíceis de distinguir das lesões da granulomatose de Wegener (GW nos ouvidos, nariz e garganta. Descrevemos o caso de uma paciente de 43 anos admitida com história de dois anos de obstrução nasal e rinorreia. Ela havia recebido o diagnóstico de GW há cinco meses e estava em tratamento com prednisona e ciclofosfamida. Ao exame físico apresentava perfuração de septo nasal e palato. Exames de laboratório mostraram elevação das proteínas de fase aguda e teste p-ANCA positivo. Ensaios ELISA antiproteinase 3 e mieloperoxidase foram negativos. Tomografia computadorizada (TC dos seios paranasais mostrou destruição de septo nasal e palato, bem como sinusite maxilar bilateral. TC de tórax resultou normal. Biópsia da mucosa nasal revelou infiltrado inflamatório sem granuloma ou vasculite. Quando questionada, admitiu ser usuária de cocaína há cinco anos. Os imunossupressores foram suspensos e a paciente não mais fez uso da droga. Ela está sendo monitorada há seis meses e não desenvolveu novas lesões ou sintomas de outros órgãos. O diagnóstico diferencial em pacientes com LDLMIC pode ser desafiador. A avaliação deve incluir pesquisa de uso intranasal de cocaína. Embora o teste de ANCA não diferencie claramente o ANCA encontrado em alguns pacientes com LDLMIC daqueles em pacientes com GW, o envolvimento localizado e os achados de biópsia não típicos de vasculite granulomatosa de pequenos vasos devem ser reconhecidos como características das lesões induzidas por cocaína.Chronic use of cocaine by inhalation may induce midline destructive lesions (CIMDL, which can sometimes be difficult to distinguish from the ear, nose and throat lesions of Wegener's Granulomatosis (WG. We describe the case of a 43-year-old female patient admitted with a two-year history of nasal obstruction and rhinorrhea. She had been

  15. Granulomatosis with Polyangiitis (Wegener's)

    Science.gov (United States)

    ... Cryopyrin-Associated Autoinflammatory Syndrome (CAPS) (Juvenile) Dermatomyositis (Juvenile) Familial Mediterranean Fever (Juvenile) Fibromyalgia Giant Cell Arteritis Glucocorticoid-induced Osteoperosis ...

  16. Granulomatosis with Polyangiitis (Wegener's)

    Science.gov (United States)

    ... Pharyngitis, Adenitis Syndrome (Juvenile) Polymyalgia Rheumatica Psoriatic Arthritis Raynaud's Phenomenon Reactive Arthritis Rheumatoid Arthritis Scleroderma Sjogren's Syndrome Spinal Stenosis Spondyloarthritis Systemic Lupus Erythematosus (Juvenile) Takayasu's ...

  17. 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

    Directory of Open Access Journals (Sweden)

    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.

  18. Clinical analysis of 10 children with Wegener granulomatosis%儿童韦格纳肉芽肿10例临床分析

    Institute of Scientific and Technical Information of China (English)

    袁林; 陈植; 张桂菊; 沈颖

    2009-01-01

    Objective To investigate the characteristics of Wegener granulomatosis in children and to improve its recognition. Methods Ten pediatric patients with Wegener granulomatosis were studied retrospectively by clinical manifestations, serum examination, pathological data, treatment, etc. Results All children had clinical evidence of upper respiratory tract and lungs" involvement; kidney lesion occurred in 6 cases(6/10); joints, skin, eyes and nervous system were also involved with different degree, cANCA (PR3) showed positive in 8 cases; Pathological examination (1 kidney sample, 2 nasal mucosa samples, 2 skin samples) showed granulomatous vasculitis and crescents were found in renal biopsy; 7 cases were treated with corticosteroid plus cyclophosphamide, 1 case was treated with corticosteroid plus methotrexate, and improvement were seen in all of them. Conclusions Wegener granulomatosis in children have diverse clinical manifestations and clinical diagnosis is difficult. The upper and lower respiratory tract and kidney are most commonly involved organs. ANCA inspection is characteristic. Treatment with corticosteroid and immunosuppressive can achieve good outcome.%目的 分析儿童韦格纳肉芽肿的特点,提高对该病的认识.方法 对10例韦格纳肉芽肿患儿的临床表现、辅助检查、病理结果、治疗等资料进行总结分析.结果 10例患儿中上呼吸道、肺脏受累10例,肾脏受累6例,关节、皮肤、眼及神经系统等也有不同程度受累.实验室检查胞浆型抗中性粒细胞胞浆抗体(cANCA)阳性8例;5例行病理检查(肾脏1例,鼻黏膜2例,皮肤2例),均表现血管炎和(或)肉芽肿性改变,肾活检有新月体形成.7例患儿给予糖皮质激素(甲基泼尼松龙冲击)联合环磷酰胺治疗,1例进行糖皮质激素联合氨甲蝶呤治疗,患儿临床表现均有不同程度的改善.结论 儿童韦格纳肉芽肿临床表现多样,误诊率高;主要累及呼吸道及肾脏,ANCA检查有特

  19. [Spontaneous hemothorax revealing Wegener's vasculitis in a pregnant woman].

    Science.gov (United States)

    Serhane, Hind; Yassine, Msougar; Amro, Lamyae

    2016-01-01

    Spontaneous hemothorax is a rare condition. Its causes are multiple but sometimes they remain unknown. In some patients, thoracotomy may be the only means to determine hemothorax origin. Vasculitis have not been reported as a common cause of spontaneous hemothorax. Pregnancy does not appear to have causal or aggravating effect on spontaneous hemothorax or on vasculitis. We here report the peculiar case of a young patient presenting during pregnancy with spontaneous hemothorax secondary to Wegener's vasculitis. The latter was diagnosed by pleural biopsy performed during exploratory thoracotomy and confirmed by ANCA assays.

  20. Clinical pathological of Wegener's granulomatosis in middle-aged and elderly patients%中老年人Wegener's肉芽肿病的临床病理观察

    Institute of Scientific and Technical Information of China (English)

    方芳; 乔旭柏; 蒲纯; 庞建欣; 刘东戈

    2013-01-01

    目的 探讨Wegener's肉芽肿病(WG)的临床病理学特点,提高对本病的认识.方法 回顾性分析1999年2月至2012年7月我院诊治的21例WG患者的临床、病理资料,其中男11例,女10例;年龄45~76岁,平均58.1岁.34个(包括2例尸检)不同部位的标本常规取材、制片,并进行苏木精-伊红(HE)、组织化学染色,13例肾穿标本全部行免疫荧光染色和电镜检查,观察病理形态特征. 结果 从初发到确诊的时间为24 d至11.0个月,平均为5.3个月.眼、鼻和涎腺是本病病初最容易受累的部位,共11例(52.4%);皮肤受累3例(14.3%),17例(81.0%)累及肺脏,15例(71.4%)累及肾脏.18例检查抗中性粒胞浆抗体(ANCA),13例(72.2%)胸型ANCA (cANCA)阳性;3例(16.7%)核周型ANCA(p-ANCA)阳性;2例(11.1%)ANCA阴性.病理学主要表现:(1)7种血管炎,依次为毛细血管炎、急性血管炎、慢性血管炎、坏死性纤维素性血管炎、坏死性肉芽肿性血管炎、非坏死性肉芽肿性血管炎、瘢痕性血管炎;(2)4种肉芽肿性炎,依次为散在分布的巨细胞、栅栏状排列的组织细胞、松散的小肉芽肿及微脓肿周围车轮状排列的组织细胞;(3)2种实质的变性坏死,地图状坏死、中性粒细胞微脓肿.2例尸检3类13种形态的表现均可观察到,活检小标本可见不同种类的形态变化和弥漫性肺出血等次要表现. 结论 Wegener's肉芽肿病有复杂的病理形态学的变化谱系,主要表现为血管炎、肉芽肿性炎和实质的坏死.%Objective To study the clinical pathological features of Wegener's granulomatosis (WG) in middle-aged and elderly patients,and enhance understanding of this disease.Methods Totally 21 patients with WG (11 males,10 females,aged 45 to 76 years,mean age 58.1 years) in our hospial from February 1999 to July 2012 were selected.The clinical and pathological data of WG patients were retrospectively analyzed.34 biopsies including 2 autopsies

  1. Comparing Presenting Clinical Features in 48 Children With Microscopic Polyangiitis to 183 Children Who Have Granulomatosis With Polyangiitis (Wegener's): An ARChiVe Cohort Study.

    Science.gov (United States)

    Cabral, David A; Canter, Debra L; Muscal, Eyal; Nanda, Kabita; Wahezi, Dawn M; Spalding, Steven J; Twilt, Marinka; Benseler, Susanne M; Campillo, Sarah; Charuvanij, Sirirat; Dancey, Paul; Eberhard, Barbara A; Elder, Melissa E; Hersh, Aimee; Higgins, Gloria C; Huber, Adam M; Khubchandani, Raju; Kim, Susan; Klein-Gitelman, Marisa; Kostik, Mikhail M; Lawson, Erica F; Lee, Tzielan; Lubieniecka, Joanna M; McCurdy, Deborah; Moorthy, Lakshmi N; Morishita, Kimberly A; Nielsen, Susan M; O'Neil, Kathleen M; Reiff, Andreas; Ristic, Goran; Robinson, Angela B; Sarmiento, Angelyne; Shenoi, Susan; Toth, Mary B; Van Mater, Heather A; Wagner-Weiner, Linda; Weiss, Jennifer E; White, Andrew J; Yeung, Rae S M

    2016-10-01

    To uniquely classify children with microscopic polyangiitis (MPA), to describe their demographic characteristics, presenting clinical features, and initial treatments in comparison to patients with granulomatosis with polyangiitis (Wegener's) (GPA). The European Medicines Agency (EMA) classification algorithm was applied by computation to categorical data from patients recruited to the ARChiVe (A Registry for Childhood Vasculitis: e-entry) cohort, with the data censored to November 2015. The EMA algorithm was used to uniquely distinguish children with MPA from children with GPA, whose diagnoses had been classified according to both adult- and pediatric-specific criteria. Descriptive statistics were used for comparisons. In total, 231 of 440 patients (64% female) fulfilled the classification criteria for either MPA (n = 48) or GPA (n = 183). The median time to diagnosis was 1.6 months in the MPA group and 2.1 months in the GPA group (ranging to 39 and 73 months, respectively). Patients with MPA were significantly younger than those with GPA (median age 11 years versus 14 years). Constitutional features were equally common between the groups. In patients with MPA compared to those with GPA, pulmonary manifestations were less frequent (44% versus 74%) and less severe (primarily, hemorrhage, requirement for supplemental oxygen, and pulmonary failure). Renal pathologic features were frequently found in both groups (75% of patients with MPA versus 83% of patients with GPA) but tended toward greater severity in those with MPA (primarily, nephrotic-range proteinuria, requirement for dialysis, and end-stage renal disease). Airway/eye involvement was absent among patients with MPA, because these GPA-defining features preclude a diagnosis of MPA within the EMA algorithm. Similar proportions of patients with MPA and those with GPA received combination therapy with corticosteroids plus cyclophosphamide (69% and 78%, respectively) or both drugs in combination with

  2. Trombose em artérias pulmonares pequenas e médias em granulomatose de Wegener: um estudo com microscopia confocal por varredura a laser Thrombosis in small and medium-sized pulmonary arteries in Wegener's granulomatosis: a confocal laser scanning microscopy study

    Directory of Open Access Journals (Sweden)

    Alfredo Nicodemos Cruz Santana

    2010-12-01

    Full Text Available OBJETIVO: A granulomatose de Wegener (GW pode causar dano nas células endoteliais e fenômenos tromboembólicos. Entretanto, poucos estudos analisaram a microcirculação pulmonar - artérias pulmonares de pequeno/médio calibre (APPMC - em pacientes com GW. O objetivo deste estudo foi quantificar trombos de fibrina em amostras de APPMC de pacientes com GW. MÉTODOS: Analisamos 24 APPMC de seis pacientes com GW e 16 APPMC de quatro pacientes controles sem WG. Utilizamos CD34 para a marcação do endotélio em todas as amostras e microscopia confocal a laser para detectar trombos de fibrina intravasculares. Calculamos a área total do vaso, a área livre do lúmen e a área trombótica. RESULTADOS: A média da área total do vaso foi similar no grupo GW e no grupo controle (32.604 µm² vs. 32.970 µm², p = 0,8793. Trombos foram identificados em 22 das 24 APPMC (91,67% no grupo GW, e em nenhuma do grupo controle (p OBJECTIVE: Wegener's granulomatosis (WG can cause endothelial cell damage and thromboembolic events. Nevertheless, there have been few studies on the pulmonary microcirculation-small and medium-sized pulmonary arteries (SMSPA-in patients with WG. The objective of this study was to quantify fibrin thrombi in the SMSPA of patients with WG. METHODS: We analyzed 24 SMSPA samples collected from six patients with WG and 16 SMSPA samples collected from four patients without WG. In all samples, we used the endothelial cell marker CD34 and confocal laser scanning microscopy in order to detect intravascular fibrin thrombi. We calculated the total vessel area, the free lumen area, and the thrombotic area. RESULTS: The mean total vessel area was similar in the WG and control groups (32,604 µm² vs. 32,970 µm², p = 0.8793. Thrombi were present in 22 (91.67% of the 24 WG group samples and in none of the control group samples (p < 0.0001; OR = 297; 95% CI: 13.34-6,612. The mean thrombotic area was greater in the WG group samples than in the

  3. Clinical analysis of patients with Wegener's granulomatosis complicated with pulmonary infection%韦格纳肉芽肿病合并肺部感染的临床分析

    Institute of Scientific and Technical Information of China (English)

    曾平; 张国华; 苏金梅; 张文; 曾小峰

    2011-01-01

    目的 分析韦格纳肉芽肿病合并肺部感染的临床特点和危险因素.方法 回顾性分析北京协和医院1998-2009年收治的韦格纳肉芽肿病合并肺部感染患者的临床资料.采用t检验和Fisher检验进行统计分析.结果 92例韦格纳肉芽肿病患者中合并肺部感染27例(29%).其中首次就诊即合并肺部感染7例占26%,确诊韦格纳肉芽肿病0~6个月内发生肺部感染12例(44%),确诊韦格纳肉芽肿病6个月以上发生肺部感染8例(30%).肺部感染表现为咳嗽、咯痰(89%),咯血(63%),发热、乏力(56%),胸闷、胸痛(33%).主要为细菌感染(59%),真菌感染(37%),结核分枝杆菌感染(37%).WG合并肺部感染组有鼻窦感染( P=0.01)、低蛋白血症(P=0.03)、低免疫球蛋白血症(P=0.007)、接受激素冲击治疗(P=0.002)的比例高于无肺部感染组.结论 韦格纳肉芽肿病合并肺部感染0~6个月内发生率高,临床上表现为咳嗽、咯痰,病原学检查细菌、真菌、结核感染多见,与鼻窦感染、低蛋白血症、低免疫球蛋白血症、接受激素冲击治疗有关.%Objective To investigate the clinical features and risk factors of patient with Wegener's granulomatosis complicated with pulmonary infection.Methods Patients with Wegener's granulomatosis admitted to our hospital in the past 11 years were retrospectively analyzed.Comparisons between groups were performed by t tests or Fisher test.Results Pulmonary infection occurred in 27 cases with an incidence rate of 29%.Twenty-six percent of pulmonary infections occurred at the initial diagnosis,and 44% occurred within 6 months,while 30% occurred later than 6 months.The clinical manifestations of pulmonary infection were productive cough (89%),hemoptysis (63%),fever and fatigue (56%),chest pain and pactoralgia (33%).The most common causative pathogen were bacteria(59% ),fungi(37% ),and tubercle bacillus(37% ).Sinus infection (P=0

  4. Hemorrhagic acneiform lesions in a teenager as the initial presentation of granulomatosis with polyangiitis.

    Science.gov (United States)

    Dosal, Jacquelyn; Good, Erica; Alshaiji, Jasem; De Solo, Santiago; Ricotti, Carlos; Alvarez-Connelly, Elizabeth

    2014-01-01

    A 19-year-old girl presented with hemorrhagic acneiform lesions on the face for several months that was unresponsive to conventional acne treatment. A biopsy revealed a noninfectious suppurative granulomatous dermatitis with hemorrhage, possibly representing a ruptured folliculitis. A second biopsy revealed chronic granulomatous dermal inflammation and hemorrhage with foreign body giant cells non-infectious by stains. No vasculitis was noted in either biopsy. Later in her course she developed a severe sinusitis and eventually presented with severe fevers, rapid weight loss, sinusitis, and cough. Further workup produced the diagnosis of granulomatosis with polyangiitis (Wegener's granulomatosis). She rapidly improved with intravenous steroids and rituximab. To date, acneiform lesions have only been reported in young adult patients and may represent a clinical manifestation of granulomatosis with polyangiitis unique to this age group, as illustrated in our patient.

  5. IL-17R expression on immune cells in peripheral blood of Wegener's granulomatosis patients%IL-17R在韦格纳肉芽肿患者外周血免疫细胞的表达

    Institute of Scientific and Technical Information of China (English)

    姜波; 王红; 刘布骏

    2011-01-01

    Objective To assess whether the receptor of IL- 17 (IL- 17R) expression on peripheral blood immune cells including B lymphocytes and plasma cells of Wegener's granulomatosis (WG) patients is abnormal. Methods Flow cytometry was used to analyze the expression of IL - 17R protein on peripheral blood immune cells of health control (HC) and WG patients. Results There was no difference of IL- 17R expression on PMN, monocytes, total lymphocytes between WG patients and HC. However, IL - 17R expression on B lymphocytes (P - 0.039) and plasma cells (P = 0.043) were much higher in WG patients compared with HC. There was no difference of IL- 17R expression on these cells between active and inactive WG, cyclophosphamide (Cyc) and methotrexate (MTX) treated WG. Conclusion IL~ 17R expression on B lymphocytes and plasma cells are significantly increased in WG patients, but normal on PMN and monocytes.%目的 检测韦格纳肉芽肿(WG)患者外周血免疫细胞特别是B淋巴细胞和浆细胞上IL-17受体(IL-17R)的表达有无异常.方法 流式细胞仪检测正常人(HC)及WG患者外周血免疫细胞表面IL-17R蛋白的表达.结果 IL-17R在WG患者外周血中性粒细胞(PMN)、单核细胞(Mono)、总淋巴细胞(Lym)上的表达与正常人无显著差异,但在B淋巴细胞(CD19+ CD20+ )(P<0.05)和浆细胞(CD19+CD38+)(P<0.05)上的表达显著高于正常人;活动期与非活动期患者、环磷酰胺(Cyc)和甲氨蝶呤(MTX)治疗组患者1L-17R的表达无显著差异.结论 IL-17R在WG患者外周血B淋巴细胞和浆细胞上的表达显著升高,但在中性粒细胞和单核细胞上的表达无明显异常.

  6. 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

    Directory of Open Access Journals (Sweden)

    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

  7. Adolescente femenino con granulomatosis de Wegener fulminante

    Directory of Open Access Journals (Sweden)

    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.

  8. Sclerosing Wegener's granulomatosis in the orbit

    DEFF Research Database (Denmark)

    Ostri, Christoffer; Heegaard, Steffen; Prause, Jan Ulrik

    2008-01-01

    and from two enucleated eyes. RESULTS: All three patients had longstanding inflammation in the orbit and involvement of the paranasal sinuses. Proptosis, impaired ocular motility and reduced visual acuity dominated the clinical picture. All histopathological specimens featured granulomatous inflammation...

  9. Features of coexisting granulomatosis with polyangiitis in a patient with pemphigus vulgaris: a case report.

    Science.gov (United States)

    Cala, Cather M; Sami, Naveed

    2015-01-01

    Pemphigus vulgaris (PV) and granulomatosis with polyangiitis (Wegener's or GPA) are two rare autoimmune disorders. Both can involve the upper airways, and diagnosis can be difficult in the absence of extra-airway symptoms. We report the case of a patient with well-controlled PV but persistent upper respiratory tract symptoms. Further evaluation revealed perforation of the nasal septum and elevated serologies consistent with GPA. The patient improved with rituximab treatments. This case demonstrates that alternative concomitant diagnosis should be considered in patients with symptoms suggestive of recalcitrant PV and/or GPA, since these patients may require more aggressive initial treatment.

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

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    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.

  11. Orofacial Granulomatosis.

    Science.gov (United States)

    Al-Hamad, Arwa; Porter, Stephen; Fedele, Stefano

    2015-07-01

    Orofacial granulomatosis (OFG) is an uncommon chronic inflammatory disorder of the orofacial region. It is characterized by subepithelial noncaseating granulomas and has a spectrum of possible clinical manifestations ranging from subtle oral mucosal swelling to permanent disfiguring fibrous swelling of the lips and face. Etiopathogenesis is unknown. A range of systemic granulomatous disorders, including Crohn disease and sarcoidosis, may cause orofacial manifestations that cannot be distinguished from those of OFG. Treatment of OFG has proven difficult and unsatisfactory, with no single therapeutic model showing consistent efficacy in reducing orofacial swelling and mucosal inflammation.

  12. Orofacial granulomatosis

    Directory of Open Access Journals (Sweden)

    Srinivas Kandula

    2016-01-01

    Full Text Available Orofacial granulomatosis (OFG is a rare disease, usually presenting as a persistent swelling of the soft tissues in the orofacial region, predominantly lips (cheilitis granulomatosa. The treatment of OFG can be challenging as frequent recurrences are very common. Here, we present a case of an Asian female patient with persistent swelling of the upper lip from the past 6 months. After establishing the diagnosis, intralesional triamcinolone injection (10 mg on a weekly schedule was planned for a period of 6 weeks. Complete resolution of the lesion was observed. This article presents a unique case of cheilitis granulomatosa (CG with a brief highlight on the various treatment modalities and a structured algorithm discussing the various differential diagnoses, aiding in formulating an accurate diagnosis and effective treatment.

  13. 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.

  14. [Pulmonar pseudotumor in granulomatosis with polyangiitis (GPA). Pulmonary cancer and/or GPA? Diagnostic implications of pulmonary nodules].

    Science.gov (United States)

    Horta-Baas, Gabriel; Meza-Zempoaltecatl, Esteban; Pérez-Cristóbal, Mario; Barile-Fabris, Leonor Adriana

    2016-01-01

    Granulomatosis with polyangiitis (GPA), formerly known as Wegener's granulomatosis, is a systemic necrotizing vasculitis, which affects small and medium sized blood vessels and is often associated with cytoplasmic anti-neutrophil cytoplasmic antibodies (ANCA). Inflammatory pseudotumor is a rare condition characterized by the appearance of a mass lesion that mimics a malignant tumor both clinically and on imaging studies, but that is thought to have an inflammatory/reactive pathogenesis. We report a patient with a GPA which was originally diagnosed as malignancy.

  15. 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.

  16. Alfred Wegener - en ilisimatoorsuaq

    DEFF Research Database (Denmark)

    Andersen, Jan

    2007-01-01

    Den naturvidenskabelige grønlandsforskning i begyndelsen af 1900-tallet skulle ikke involvere sig med Grønland. Dens projekt handlede om naturen - ikke kulturen. Wegener er kendt for sin kontinentaldriftsteori, der ofte fremstilles som et resultat af Danmark-ekspeditionens videnskabelige arbejde....... Da Wegener som medlem af ekspeditionen opholdt sig i Nordøstgrønland var tanken om kontinentaldrift imidlertid endnu ikke faldet ham ind. Vi har hans egne ord for, at ideen først opstod i 1910....

  17. Large Pyoderma Gangrenosum-Like Ulcers: A Rare Presentation of Granulomatosis with Polyangiitis

    Directory of Open Access Journals (Sweden)

    Basheer Tashtoush

    2014-01-01

    Full Text Available Granulomatosis with polyangiitis (GPA, formerly known as Wegener's granulomatosis (WG, is a rare systemic vasculitis that classically manifests as necrotizing granulomas of the upper and lower respiratory tract, kidneys, and blood vessels; however, it may affect any organ system, including the skin. Cutaneous manifestations occur in up to 45% of patients during the disease course, and are the presenting feature in 9% to 14% of patients. The most common skin lesion specific to GPA is palpable purpura, with the histopathologic correlate of leukocytoclastic vasculitis. However, a wide range of clinical and histologic features may be seen. We herein report a case of a previously healthy 52-year-old Caucasian man who presented with multiple progressively enlarging painful ulcers on his face, upper extremities, back, and abdomen over a two-month period. Skin biopsies revealed pyoderma gangrenosum-like features. Serological tests were positive for PR3/c-ANCA. Six months later, the patient developed recurrent episodes of sinusitis associated with nasal bleeds and eventually nasal septum perforation. Despite aggressive treatment with Cyclophosphamide and steroids over one year, the patient had persistent nonhealing large ulcers and developed multiple lung nodules with cavitary lesions.

  18. 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...

  19. Treatment of orbital inflammation with rituximab in Wegener's granulomatosis

    DEFF Research Database (Denmark)

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

    2012-01-01

    inflammation. All patients were treated with 1000 mg of rituximab administered twice with an interval of 14 days between the infusions. Six months after therapy, a physical examination and a control computerised tomography (CT) scan was performed. RESULTS: All patients had orbital inflammation demonstrated...... 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...... (seven eyes) with visual impairment responded to therapy, and the improvement in visual acuity was sustained throughout follow-up (median duration of follow-up: 17 months; range: 6-18 months). At the time of the control CT-scan, size-reduction of the orbital mass was observed in two patients, while...

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

    DEFF Research Database (Denmark)

    Faurschou, Mikkel; Mellemkjaer, Lene; Sørensen, Inge Juul

    2009-01-01

    .4; 95% CI 1.1, 38) based on two patients, who developed testis cancer >10 years before WG. The overall prevalence of malignancies diagnosed skin cancer occurred with an increased prevalence within this 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...... randomly for each patient from the Danish Central Population Register. Information on malignancies was obtained through the Danish Cancer Registry. Occurrence of malignancies before WG diagnosis among patients and before WG diagnosis of their matched case among controls (reference date) was compared...

  1. Refractory Granulomatosis with Polyangiitis Presenting as Facial Paralysis and Bilateral Sudden Deafness.

    Science.gov (United States)

    Kim, Sang Hoon; Jung, A Ra; Kim, Su Il; Yeo, Seung Geun

    2016-04-01

    Granulomatosis with polyangiitisis [(GPA) or Wegener granulomatosis] is a multi-system disease characterized by granuloma formation and necrotizing vasculitis. GPA classically shows involvement of the respiratory tracts and the renal system. However, locoregional disease is common and may include otologic manifestations. Although otologic involvement can occur during the course of GPA, no report has described facial palsy with sudden sensorineural total deafness with vertigo as the presenting feature of GPA. This case describes a patient with multiorgan involving resistant form of GPA initially presenting with bilateral profound sudden sensorineural hearing loss and left facial paralysis with vertigo. The condition responded well to treatment with rituximab.

  2. Coexistence of granulomatosis with polyangiitis (GPA) and Crohn's disease or multiorgan manifestation of the same disease?

    Science.gov (United States)

    Jóźwiak, Lucyna; Ławnicka, Izabela; Książek, Andrzej

    2016-01-01

    Granulomatosis with polyangiitis (GPA) is a systemic necrotizing vasculitis of unknown aetiology, often related to the antineutrophil cytoplasmic antibody (ANCA). GPA was previously named Wegener's granulomatosis (WG). The disease frequently has multisystemic presentation, targeting mainly the respiratory tract and kidneys, but gastrointestinal involvement is uncommon. Crohn's disease (CD) is an inflammatory bowel disease (IBD) with many extraintestinal manifestations. Clinically, symptoms of WG and CD can mimic each other. In this paper a case of GPA manifested initially by severe multiorgan damage including colitis, regarded to be coexistent CD, is presented. The case illustrates the difficulties in establishing the diagnosis when symptoms of the diseases mimic each other.

  3. Refractory Granulomatosis with Polyangiitis Presenting as Facial Paralysis and Bilateral Sudden Deafness

    Science.gov (United States)

    Kim, Sang Hoon; Jung, A Ra; Kim, Su Il

    2016-01-01

    Granulomatosis with polyangiitisis [(GPA) or Wegener granulomatosis] is a multi-system disease characterized by granuloma formation and necrotizing vasculitis. GPA classically shows involvement of the respiratory tracts and the renal system. However, locoregional disease is common and may include otologic manifestations. Although otologic involvement can occur during the course of GPA, no report has described facial palsy with sudden sensorineural total deafness with vertigo as the presenting feature of GPA. This case describes a patient with multiorgan involving resistant form of GPA initially presenting with bilateral profound sudden sensorineural hearing loss and left facial paralysis with vertigo. The condition responded well to treatment with rituximab. PMID:27144236

  4. GRANULOMATOSIS WITH POLYANGIITIS, WHICH IS PATHOGENETICALLY ASSOCIATED WITH ANTINEUTROPHIL CYTOPLASMIC ANTIBODIES: CLINICAL FEATURES

    Directory of Open Access Journals (Sweden)

    Tatiana Valentinovna Beketova

    2012-01-01

    Full Text Available The author studied in detail the specific features of the clinical manifestations reflecting a granulomatous inflammatory response and necrotizing polyangiitis, by using her experience in following up 70 patients with granulomatosis and polyangiitis (Wegener's, and analyzed their early symptoms, premorbid background, and possible predictors. Granulomatous inflammation and its related clinical manifestations (primarily orbital pseudotumor and subfold granuloma of the larynx was demonstrated to tend over time to occupy a dominant place in the clinical picture of the disease while the proportion of the symptoms apparently related to necrotizing vasculitis was generally decreased. Interpretation of mechanisms for the development of granulomatosis with polyangiitis (Wegener's may be of value for the further elaboration of an optimal treatment strategy.

  5. Occult Renal Granulomatous Inflammatory Lesions in Granulomatosis with Polyangiitis Detected by 18F-FDG PET/CT.

    Science.gov (United States)

    Fu, Zhanli; Liu, Meng; Li, Ziao; Zhang, Jin; Li, Qian

    2017-09-01

    A 33-year-old woman with a 7-month history of granulomatosis with polyangiitis F-FDG PET/CT scan due to low-grade intermittent fever and elevation of serum inflammatory markers, which revealed multiple hypermetabolic lesions in bilateral kidneys. Biopsy of a lesion showed granulomatosis with polyangiitis, consistent with renal involvement of granulomatosis with polyangiitis.

  6. Clinicopathological diagnosis of orofacial granulomatosis.

    Science.gov (United States)

    Afsar, Fatma Sule; Duran, Hatice Demirlendi; Yilmaz, Gungor; Ermete, Murat

    2017-01-01

    Orofacial granulomatosis is a rare chronic inflammatory disorder characterized by persistent or recurrent soft tissue swellings, oral ulceration, and other orofacial features in the absence of an identifiable granulomatous disease. We report a case of a 61-year-old woman with recurrent ulcerations and swellings in her oral mucosa. She was diagnosed as orofacial granulomatosis based upon clinicopathological correlation after exclusion of other granulomatous diseases and showed a favorable response to systemic corticosteroid treatment.

  7. A case of fever of unknown origin: necrotizing sarcoid granulomatosis.

    Science.gov (United States)

    Unlü, G; Onyılmaz, T A; Barış, S A; Turhan, N; Vural, C; Başyiğit, I; Boyacı, H

    2014-01-01

    Necrotizing sarcoid granulomatosis is a rare type of vasculitis; its etiology and pathogenesis are still unknown. The disease primarily affects the lungs, although extra-pulmonary involvement has been reported. The typical symptoms are cough, chest pain, dyspnea, and weight loss; high temperatures have been reported in rare cases. We present the case of a 65-year-old woman who was diagnosed with lymph node tuberculosis, for which she received treatment for six months. The patient experienced no improvement in her symptoms, which included fever, weakness and dyspnea. A re-evaluation of previously collected thoracoscopic biopsy material revealed compatibility with necrotizing sarcoid granulomatosis.

  8. Lymphomatoid granulomatosis mimicking interstitial lung disease.

    Science.gov (United States)

    Braham, Emna; Ayadi-Kaddour, Aïda; Smati, Belhassen; Ben Mrad, Sonia; Besbes, Mohammed; El Mezni, Faouzi

    2008-11-01

    Lymphoid granulomatosis is a rare form of pulmonary angiitis. This case report presents a patient with lymphoid granulomatosis in whom the clinical presentation, radiological features and the partial response to corticosteroid therapy mimicked interstitial lung disease. Lymphoid granulomatosis was only diagnosed at post-mortem examination. The range of reported clinical presentations, diagnostic approaches and outcomes are described.

  9. 韦格纳肉芽肿病合并肥厚性颅内硬脑膜炎三例并文献复习%Hyperthrophic cranial pachymeningitis in Wegener granulomatosis: 3 case report and review of liter-atures

    Institute of Scientific and Technical Information of China (English)

    吴庆军; 苏金梅; 郑文洁; 曾小峰; 张奉春; 赵岩; 唐福林

    2008-01-01

    Objective To study the clinical, laboratory, radiological and pathological findings of patients with hypertrophic cranial pachymeningitis (HCP) in Wegner's granulomatosis (WG) to improve the recognition of the disease, even when it occurs in limited form. Methods Three patients were described and English literatures of biopsy-proven pachymeningitis in WG were reviewed. Results The features of WG-associated pachymeningitis included: ① Frequently occurred early in the course of active limited WG; ② Commonly presented with sever headache and cranial neuropathies in the absence of other meningeal irritative signs; ③ Variable cerebrospinal fluid findings with mild predominantly lymphocytic pleocytosis and elevated protein concentration were major laboratorg findings; ④Elevated ESR and positive serum anti-neutrophilic cytoplasmic antibody (ANCA) could be found in most patients; ⑤ Gadolinium-enhanced brain MRI is very senitive in the detection of pachymeningitis; ⑥A dural biopsy showed granulomatous necrotizing inflammation, giant cell, and evidence of vasculitis;⑦ A favorable response to standard treatment with corticosteroid, cyclophosphamide or other cytotoxic drugs could be observed. Conclusion HCP may be the initial or cardinal manifestation of the limited form of WG. Early diagnosis by ANCA, MRI and dural biopsy may facilitate diagnosis Corticosteroid and immunosupressant are the choices of treatment.%目的 分析以肥厚性颅内硬脑膜炎(HCP)为主要表现的韦格纳肉芽肿病(WG)的临床表现、磁共振成像(MRI)和硬脑膜病理特征.方法 报道3例WG合并HCP,并复习国外文献.结果 HCP多见于局限型WG,常在发病半年内出现,表现为头痛和颅神经病,伴有呼吸道/眶部受累.半数以上患者抗中性粒细胞胞质抗体(ANCA)阳性.脑脊液改变示约1/3有轻度白细胞数增高和2/3有蛋白增高.MRI均显示硬脑膜增厚并强化.硬脑膜活检病理显示肉芽肿炎症(100%)、组织坏死(94

  10. Characteristics of hypertrophic pachymeningitis in patients with granulomatosis with polyangiitis.

    Science.gov (United States)

    Choi, Hyun Ah; Lee, Mi Ji; Chung, Chin-Sang

    2017-04-01

    Hypertrophic pachymeningitis (HP) is an important neurologic complication of granulomatosis with polyangiitis (GPA, formerly Wegener's granulomatosis). The aim of this study is to investigate the clinical features, radiological findings, and diagnostic pitfalls of GPA-related HP. A retrospective chart review was performed to screen patients diagnosed with GPA at Samsung Medical Center between 1997 and 2016. Neurologic manifestation, laboratory findings, neuroimaging data, and clinical course were evaluated in all patients. Characteristics of patients with HP were compared to those of patients without HP. Sixty-five patients with GPA were identified. Twenty-five of these patients had central nervous system involvement. HP (N = 9, 36%) was the second most common radiologic finding. Other neurologic findings included stroke (N = 7, 28%) and granulomatous disease (N = 10, 40%). Patients with HP had lower incidences of systemic manifestations (N = 2, 22.2% vs. N = 38, 67.9%, p = 0.013 in the lung and N = 1, 11.1% vs. N = 28, 50.0%, p = 0.030 in the kidney) than those without HP. Six patients with GPA-related HP were MPO-ANCA positive (66.7%) and two had PR3-ANCA (22.2%). Most of the patients with HP presented with headache (N = 8, 88.9%) at a rate that is similar to those of primary headache disorders (migraine, tension-type, and stabbing) and other secondary headache disorders (postural type and meningitis). Patients with HP rarely had neurologic deficits (N = 3, 37.5%). Different clinical or radiologic features may be observed in GPA-related HP. Early recognition and accurate diagnosis of GPA-related HP are needed in addition to neuroimaging findings.

  11. Wegener′s granulomatosis disease mimicking pulmonary tuberculosis

    Directory of Open Access Journals (Sweden)

    Naveen Pandhi

    2015-01-01

    Full Text Available Wegener′s granulomatosis (WG is an uncommon autoimmune disease with multi-system involvement that manifests as vasculitis, granulomatosis, and necrosis. While its standard form involves the upper and lower respiratory tracts and kidneys, it may essentially involve any organ. We present a case in a young female patient presenting with symptoms of cough with expectoration, fever, dyspnea and chest discomfort, having cavitating lesion in right upper lobe and nodule in left upper lobe with bilateral paranasal sinus involvement on computed tomography. Allergic rhino-sinusitis with pulmonary tuberculosis was suspected, and patient was investigated further. Upon further investigation, renal involvement was detected, and serology revealed cytoplasmic antineutrophil cytoplasmic antibody. WG was suspected and was proven by histopathology of nasal tissue which revealed necrotizing granulomas.

  12. Pulmonary lymphomatoid granulomatosis in a 4-year-old girl

    Energy Technology Data Exchange (ETDEWEB)

    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.)

  13. Lymphomatoid granulomatosis: two cases with skin involvement.

    Science.gov (United States)

    Camisa, C

    1989-04-01

    Lymphomatoid granulomatosis is a systemic disease marked by a polymorphous cellular infiltrate that is both angiocentric and angiodestructive. The predominant organs of involvement are lungs, skin, central nervous system, and kidneys. I describe two cases of lymphomatoid granulomatosis in association with cutaneous manifestations, stressing to the dermatologist the importance of early recognition and diagnosis of this entity.

  14. The relation between Staphylococcus aureus and Wegener's granulomatosis : Current knowledge and future directions

    NARCIS (Netherlands)

    Popa, ER; Tervaert, JWC

    2003-01-01

    To date, in the investigation of the role of S. aureus in WG, we face a paradoxical situation. On the one hand, clinical results obtained from treatment of WG patients with co-trimoxazole and studies assessing the impact of S. aureus on disease relapses strongly suggest that this bacterium contribut

  15. T-CELL REACTIVITY TO PROTEINASE-3 AND MYELOPEROXIDASE IN PATIENTS WITH WEGENERS GRANULOMATOSIS (WG)

    NARCIS (Netherlands)

    BROUWER, E; STEGEMAN, CA; HUITEMA, MG; LIMBURG, PC; KALLENBERG, CGM

    1994-01-01

    T cell-mediated immunity is hypothesized to play an important role in the pathogenesis of granulomatous inflammation and vasculitis as found in patients with WG. The antigenic specificities of those T cells remain, however, unknown. Anti-neutrophil cytoplasmic antibodies (ANCA) present in patients w

  16. Structured clinical assessment of the ear, nose and throat in patients with granulomatosis with polyangiitis (Wegener's)

    DEFF Research Database (Denmark)

    Martinez Del Pero, Marcos; Rasmussen, Niels; Chaudhry, Afzal;

    2013-01-01

    disease cf. 59/131 and 52/131, respectively, in remission). Nasal crusting was the most common nasal complaint recorded (52/144, 36 %) and bloody rhinorrhoea was the most common symptom in patients with disease activity. Rhinoscopy was highly sensitive in diagnosing disease activity (100 %). Subglottic...... stenosis was the most common head and neck manifestation (27/121, 22 %) and 74 % were symptomatic. In conclusion, the pattern and frequency of clinical ENT manifestations in GPA have been described in a large patient cohort. The use of tools readily available in the ENT clinic was essential to assess...

  17. Atypical Endobronchial Carcinoid with Postobstructive Pneumonia Obscuring the Diagnosis of Granulomatosis with Polyangiitis

    Science.gov (United States)

    Ali, Robert; Baldeo, Candice; Onyenekwe, Jesse; Lala, Roshan; Landa, Cristian; Siddiqi, Anwer

    2015-01-01

    Granulomatosis with polyangiitis (GPA), previously termed Wegener's Granulomatosis, is an autoimmune small vessel vasculitis which is highly associated with antineutrophil cytoplasmic antibodies (ANCA) and has varied clinical manifestations. Diagnosis hinges on identifying a combination of clinical features of systemic vasculitis, positive ANCA serology, and histological evidence of necrotizing vasculitis, necrotizing glomerulonephritis, or granulomatous inflammation from a relevant organ biopsy. The American College of Rheumatology has also developed a classification criteria focusing specifically on nasal or oral inflammation, abnormal chest radiograph, and abnormal urinary sediment, along with granulomatous inflammation, which helps to distinguish GPA from other forms of systemic vasculitis. In the case presented below, the diagnosis of GPA was delayed as the patient had a concomitant atypical endobronchial carcinoid which predisposed to postobstructive pneumonia. Fortunately, the papular lesions that developed across her lower limbs prompted further investigations. The return of appropriate serology coincided with progression to alveolar hemorrhage, offering a more complete clinical picture, and when she responded to the combination of steroid, cyclophosphamide, and plasma exchange, the diagnosis of GPA was cinched. PMID:26347844

  18. Infectious complication or exacerbation of granulomatosis with polyangiitis?

    Science.gov (United States)

    Masiak, Anna; Struk-Panfill, Małgorzata; Zdrojewski, Zbigniew

    2015-01-01

    Granulomatosis with polyangiitis (GPA) is a primary, systemic small vessel vasculitis. The respiratory tract is typically involved in the course of the disease. Abnormalities on the chest radiograph are noted in more than 70% patients at some point during their disease history. In some clinical situations it is difficult to distinguish whether symptoms result from the underlying disease or are a symptom of infection. In these clinical situations, chest computed tomography (CT) can be very useful. We present a patient with GPA localized mainly in the respiratory tract with sudden deterioration of the general state and new abnormalities revealed in the CT of the chest.

  19. Granulomatosis with polyangiitis in Tunisia

    Directory of Open Access Journals (Sweden)

    I. Ben Ghorbel

    2017-05-01

    Full Text Available Granulomatosis with polyangiitis (GPA is more frequent in Northern rather than Southern countries. Very few studies have been conducted in Africa. We have performed a retrospective descriptive study including clinical and laboratory profiles of 30 Tunisian GPA patients seen at the department of Internal Medicine of the University Hospital of la Rabta from 2000 to 2014. Mean age at initial GPA diagnosis was 46±12 years, and the average number of months between the onset of symptoms and diagnosis was 25. Seventeen (56% were male, and 13 (44% were female. Ear/nose/throat involvement occurred in 83%. Lung and renal involvement were observed in respectively 70% and 56% followed by mucocutaneous (50%, neurological (50%, ocular (33%, vascular (20%, ureteral (16%, and cardiac involvement in 10%. Cytoplasmic pattern-antineutrophil cytoplasmic antibodies (ANCA was detected in 27 (90% patients. Induction therapy consisted of intravenous cyclophosphamide pulses in 27 patients (90% and oral methotrexate in 3 patients (10%. Trimethoprime-sulfamethoxazole was used in 26 patients (86%. Maintenance therapy consisted of azathioprine in 17 cases and methotrexate in 13 cases. Relapses occurred in 36%. Eighteen patients had favorable outcome and 12 died. Our patients had a distinct phenotype with high prevalence of pleural involvement, lymph node enlargement, sensorimotor neuropathy and ureter stenosis. ENT symptoms were less frequent as inaugural presentation. Overall 2-year survival was 60%.

  20. Granulomatosis with Polyangiitis (GPA) Mimicking Tuberculosis.

    Science.gov (United States)

    Haridas, Vikram; Haridas, Kiran

    2017-03-01

    Granulomatosis with Polyangiitis (GPA) is a rare disease with varied clinical manifestations. We present a case of GPA which manifested initially with symptoms suggestive of meningeal tuberculosis. High index of suspicion and collective review of all clinical features helped in the correct diagnosis. Treatment of this case with rituximab provided significant symptomatic relief. © Journal of the Association of Physicians of India 2011.

  1. 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.

  2. Histopathological features predictive of a clinical diagnosis of ophthalmic granulomatosis with polyangiitis (GPA).

    Science.gov (United States)

    Isa, Hazlita; Lightman, Sue; Luthert, Philip J; Rose, Geoffrey E; Verity, David H; Taylor, Simon R J

    2012-01-01

    The limited form of Granulomatosis with Polyangiitis (GPA), formerly known as Wegener's Granulomatosis (WG) primarily involves the head and neck region, including the orbit, but is often a diagnostic challenge, particularly as it commonly lacks positive anti-neutrophil cytoplasm antibody (ANCA) titres or classical features on diagnostic orbital biopsies. The purpose of this study was to relate biopsy findings with clinical outcome and to determine which histopathological features are predictive of a clinical diagnosis of GPA. Retrospective case series of 234 patients identified from the database of the UCL Institute of Ophthalmology Department of Eye Pathology as having had orbital biopsies of orbital inflammatory disorders performed between 1988 and 2009. Clinical records were obtained for the patients and analysed to see whether patients had GPA or not, according to a standard set of diagnostic criteria (excluding any histopathological findings). Biopsy features were then correlated with the clinical diagnosis in univariate and multivariate analyses to determine factors predictive of GPA. Of the 234 patients, 36 were diagnosed with GPA and 198 with other orbital pathologies. The majority of biopsies were from orbital masses (47%). Histology showed a range of acute and chronic inflammatory pictures in all biopsies, but the presence of neutrophils (PGPA. In a multivariate analysis, only tissue neutrophils (OR=3.6, P=0.01) and vasculitis (OR=2.6, P=0.02) were independently associated with GPA, in contrast to previous reports associating eosinophils and necrosis with the diagnosis. Neutrophil, eosinophil and macrophage infiltration of orbital tissues, together with vasculitis and necrosis, are all associated with a clinical diagnosis of GPA, but only neutrophil infiltration and vasculitis are independently associated with this diagnosis. These features may assist in the establishing the diagnosis of limited GPA among patients with early orbital disease, particularly

  3. Postirradiation pulmonary fibrosis complicated by aspergilloma and bronchocentric granulomatosis

    Energy Technology Data Exchange (ETDEWEB)

    Makker, H.; McConnochie, K.; Gibbs, A.R. (Univ. of Wales College of Medicine, Llandough Hospital, Penarth (UK))

    1989-08-01

    An asthmatic patient requiring cortico-steroid treatment developed a pulmonary aspergilloma in an area of postmastectomy radiation fibrosis. At necropsy bronchocentric granulomatosis was also found. (author).

  4. Orofacial granulomatosis: clinical signs of different pathologies.

    Science.gov (United States)

    Troiano, Giuseppe; Dioguardi, Mario; Giannatempo, Giovanni; Laino, Luigi; Testa, Nunzio Francesco; Cocchi, Roberto; De Lillo, Alfredo; Lo Muzio, Lorenzo

    2015-01-01

    Orofacial granulomatosis (OFG) is an uncommon disease characterized by persistent or recurrent soft tissue enlargement, oral ulceration and a variety of other orofacial features. It could be an oral manifestation of a systemic disease. For a correct differential diagnosis, local and systemic conditions characterized by granulomatous inflammation should be excluded using appropriate clinical and laboratory investigations. In fact, the diagnosis of OFG may be confirmed only by histopathological identification of noncaseating granulomas. The literature from 1943 to 2014 was reviewed with emphasis on the etiology of OFG and on clinical manifestations of systemic pathologies associated with OFG. The precise cause of OFG is still unknown, although several theories have been suggested, such as infection, hereditary factors and allergy. OFG is a disease that has a wide spectrum of presentation, which may include the oral manifestation of a systemic condition such as Crohn's disease, sarcoidosis, granulomatosis with polyangiitis and Melkersson-Rosenthal syndrome.

  5. Refractory optic perineuritis due to granulomatosis with polyangiitis successfully treated with methotrexate and mycophenolate mofetil combination therapy

    Science.gov (United States)

    Kimura, Yoshitaka; Asako, Kurumi; Kikuchi, Hirotoshi; Kono, Hajime

    2017-01-01

    Optic perineuritis is an uncommon inflammatory disorder of the optic sheath that causes visual loss or eye pain. There are few case reports of optic perineuritis associated with granulomatosis with polyangiitis. Herein we report the case of a 37-year-old male with granulomatosis with polyangiitis and who presented with headache, blurred vision in the right eye, diplopia, and numbness in the right forehead. Brain magnetic resonance images (MRI) findings revealed hypertrophic pachymeningitis and refractory optic perineuritis. These were manageable only by means of weekly methotrexate and mycophenolate mofetil combination therapy but not with methotrexate, mycophenolate mofetil, intravenous cyclophosphamide, rituximab, azathioprine, or cyclosporine individually. PMID:28293459

  6. Treatment of Wegener’s granulomatosis

    Directory of Open Access Journals (Sweden)

    G. Valesini

    2011-09-01

    Full Text Available Treatment of Wegener’s granulomatosis, often a life-threatening disease, has greatly improved, considering that before corticosteroids and immunosuppressives were available, the average survival time of patients amounted to no more than 5 months. The management of Wegener’s granulomatosis can be divided in two stages: induction of remission and maintenance of remission. The standard regimen for the induction of remission consists of cyclophosphamide, 2 mg/kg/day orally, in combination with prednisone, 1mg/Kg/day orally, with a gradual tapering once remission has been obtained. To lower the overall cumulative dose, monthly intravenous pulses of cyclophosphamide have been evaluated. Other alternative treatments as high doses of corticosteroids, methotrexate, or plasmapheresis have been proposed, together as prophylaxis with trimethoprim-sulfamethoxazole. To minimize toxicity, for maintenance therapy other drugs are also used such as methotrexate, azathioprine, cyclosporine. Frequent therapeutic changes are needed due to the great variability of the disease; while important aspects are the recognition and treatment of relapse, and include not only the management of resistant disease, but also some particular aspects such as disease in chronic dialysis, renal transplant, pregnancy. Other cytotoxic drugs like leflunomide or mycofenolic mofetil appear to be promising, while new efforts to identify more effective and less toxic therapies include biologic products, such as high-dose immunoglobulin, TNF antagonists and other monoclonal antibodies. Many different kind of clinical trials are going on to better evaluate the real efficacy and safety of these treatments in Wegener’s granulomatosis.

  7. Orofacial granulomatosis in children: think about Crohn's disease.

    Science.gov (United States)

    Lazzerini, Marzia; Martelossi, Stefano; Cont, Gabriele; Bersanini, Chiara; Ventura, Giovanna; Fontana, Massimo; Zuin, Giovanna; Ventura, Alessandro; Taddio, Andrea

    2015-04-01

    The term orofacial granulomatosis is conventionally used to describe patients with granulomatous lesions affecting the orofacial tissues, in absence of intestinal lesions. Lip swelling and facial swelling are the most common clinical signs. Despite the fact that histologically it is not distinguishable from Crohn's disease, and that both diseases have a chronic/recurrent course, the relationship between orofacial granulomatosis and Crohn's disease is still debated. Herein we present five cases of orofacial granulomatosis. All patients presented concomitant Crohn's disease, supporting the hypothesis that orofacial granulomatosis and Crohn's disease may be one single disease. Thalidomide was effective in inducing remission of oral and intestinal symptoms in all five cases and could be considered a valid treatment opportunity for these patients. Orofacial granulomatosis and Crohn's disease may be part of the same disease; both may respond to thalidomide. Copyright © 2014 Editrice Gastroenterologica Italiana S.r.l. Published by Elsevier Ltd. All rights reserved.

  8. WEGENER: World Earthquake GEodesy Network for Environmental Hazard Research

    Science.gov (United States)

    Ozener, Haluk; Zerbini, Susanna; Bastos, Luisa; Becker, Matthias; Meghraoui, Mustapha; Reilinger, Robert

    2013-04-01

    WEGENER is originally the acronym for Working Group of European Geoscientists for the Establishment of Networks for Earth-science Research. It was founded in March 1981 in response to an appeal delivered at the Journées Luxembourgeoises de Geodynamique in December 1980 to respond with a coordinated European proposal to a NASA Announcement of Opportunity inviting participation in the Crustal Dynamics and Earthquake Research Program. WEGENER, during the past 32 years, has always kept a close contact with the Agencies and Institutions responsible for the development and maintenance of the global space geodetic networks with the aim to make them aware of the scientific needs and outcomes of the project which might have an influence on the general science policy trends. WEGENER was serving as Inter-commission Project 3.2, between Commission 1 and Commission 3, of the International Association of Geodesy (IAG) until 2012. Since then, WEGENER project has became the Sub-commission 3.5 of IAG commission 3, namely Tectonics and Earthquake Geodesy. In this study, we briefly review the accomplishments of WEGENER as originally conceived and outline and justify the new focus of the WEGENER consortium. The remarkable and rapid evolution of the present state of global geodetic monitoring in regard to the precision of positioning capabilities (and hence deformation) and global coverage, the development of InSAR for monitoring strain with unprecedented spatial resolution, and continuing and planned data from highly precise satellite gravity and altimetry missions, encourage us to shift principal attention from mainly monitoring capabilities by a combination of space and terrestrial geodetic techniques to applying existing observational methodologies to the critical geophysical phenomena that threaten our planet and society. Our new focus includes developing an improved physical basis to mitigate earthquake, tsunami, and volcanic risks, and the effects of natural and anthropogenic

  9. Orofacial granulomatosis in a patient with Crohn's disease.

    NARCIS (Netherlands)

    Scheur, van de M.R.; Waal, van der RI; Volker - Dieben, H.J.M.; Knol, E.C.; Starink, T.M.; Waal, van der I.

    2003-01-01

    Orofacial granulomatosis encompasses the previously recognized clinical entities Melkersson-Rosenthal syndrome and cheilitis granulomatosa. We report the case of a 39-year-old patient with cheilitis granulomatosa, intestinal Crohn's disease, and optic neuropathy. Cheilitis granulomatosa and optic ne

  10. Isolated neurological involvement of lymphomatoid granulomatosis

    Institute of Scientific and Technical Information of China (English)

    HU Yu-hua; SHAO En-de; WU Jian-liang; MENG Xian-bing

    2010-01-01

    @@ Lymphomatoid granulomatosis (LG) is a rare EpsteinBarr virus (EBV)-associated systemic granulomatous disease that is characterized by an angiocentric and occasionally angiodestructive polymorphic cellular infiltrate. It most frequently affects the lungs, kidneys,and brain.1,2 Usually the lesions are multi-systemic and multifocal. Central nervous system (CNS) involvement occurs in approximately 30% of affected patients, and patients may present with nonspecific neurological symptoms.3 Although CNS lesions usually appear as a secondary metastasis of pulmonary lesions, in rare conditions, it may be the initial or only manifestation of the disease. In this report, we describe two cases presenting with unifocal mass in the brain, histologically confirmed to be LG,

  11. Pediatric Vasculitis Initiative

    Science.gov (United States)

    2016-08-19

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

  12. A Rare Manifestation of Crohn's Disease: Sinonasal Granulomatosis. Report of a Case and Review of Literature

    Directory of Open Access Journals (Sweden)

    Lilia Baili

    2014-10-01

    Full Text Available Crohn’s disease is a granulomatous inflammatory bowel disease. Its pathologic findings include non-contiguous chronic inflammation and non-caseating granulomas, sometimes with extra-intestinal localizations. Sinonasal manifestations of Crohn’s disease are quite rare and only a few cases have been reported up to date in the worldwide literature. They are characterized by chronic mucosal inflammation, obstruction, bleeding and occasionally septal perforation. We report a case of sinonasal granulomatosis revealing Crohn’s disease in a 22-year-old woman and go over the available literature on sinonasal involvement in Crohn’s disease.

  13. Miescher's granulomatosis (granulomatosis disciformis chronica et progressiva in a non-diabetic patient – case report

    Directory of Open Access Journals (Sweden)

    Brzezińska-Wcisło Ligia

    2009-08-01

    Full Text Available Abstract Introduction Necrobiosis lipoidica diabeticorum is a rare disease of unclear etiology, that occurs in about 1% of diabetic patients. Case report We present case of granulomatosis disciformis chronica et progressiva Miescher with good response to systemic corticosteroids therapy. Patient 45 years old woman, with primary yellow-brown areas skin lesions, with foci well separated from surroundings on both lower legs, that occurred 5 years ago. In laboratory tests there was no abnormalities. Because of advance suggestion (after last admit in dermatological ward of observation according to xantogranuloma necrobioticum tests for paraproteinemia were made. Immunoelectrophoresis, IgG, IgM, IgA levels, kappa light chain, lambda heavy chain; were correct, Bence-Johns protein-negative. During hospitalization in Clinic methylprednisolone in dose of 32 mg od, vascular drugs and local steroidotherapy was applied with good therapeutic response. Conclusion We described case of typical clinical and histological characters of necrobiosis lipoidica. without diabetes-granulomatosis disciformis chronica et progressiva Miescher that despite of suspicion of proper diagnosis for a long time was not treat effective.

  14. Naar meer veiligheid op gebiedsontsluitingswegen : aanbevelingen voor wegvakken van de huidige 50- en 80km/uur-wegen.

    NARCIS (Netherlands)

    Dijkstra, A. & Schermers, G.

    2014-01-01

    Gebiedsontsluitingswegen — de huidige 50- en 80km/uur-wegen — behoren nog steeds tot de gevaarlijkste wegen in Nederland. De ontwerprichtlijnen voor deze wegen worden sterk verschillend toegepast. Een kwantitatieve onderbouwing van de richtlijnen en een beter georganiseerd ontwerpproces kan duidelij

  15. Eosinophilic granulomatosis with polyangiitis: an overview

    Directory of Open Access Journals (Sweden)

    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.

  16. 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.)

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

    Directory of Open Access Journals (Sweden)

    "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

  18. 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.

  19. Uncommon inflammatory swelling of the lips: orofacial granulomatosis.

    Science.gov (United States)

    Hafiz, Abdul; Mufeed, Abdulla; Kandasamy, Gopinath; Krishnapillai, Rekha

    2016-01-12

    Orofacial granulomatosis (OFG) is an unusual condition associated with permanent or recurrent swelling of orofacial tissues together with oral mucosal ulceration and a variety of orofacial characteristics. The chronic inflammation inherent to OFG often displays granulomas in the subepithelial stroma. We present a case of OFG and its management. The patient responded to intralesional injections of corticosteroids.

  20. 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

  1. Granulomatosis with polyangiitis: rapidly progressive necrotizing glomerulonephritis in a pediatric patient

    Directory of Open Access Journals (Sweden)

    Luna M

    2014-04-01

    Full Text Available Mariana Luna,1 Victoria Bocanegra,3 Patricia G Vallés1,21Nephrology Division, Pediatric Department, Dr Humberto Notti Pediatric Hospital, Mendoza, Argentina; 2Pathophysiology Area, Pathology Department, School of Medicine, National Cuyo University, Mendoza, Argentina; 3National Council of Scientific and Technical Research of Argentina (CONICET, Buenos Aires, ArgentinaAbstract: Granulomatosis with polyangiitis (GPA is associated with a broad range of clinical manifestations including renal disease. It is a systemic vasculitis that is rarely encountered in children. We present a 14-year-old girl who suffered from pharyngitis 1 week before admittance to hospital. She was admitted for macroscopic hematuria and oliguria, under the possibility of nephritic syndrome. Renal failure with rapidly progressive glomerulonephritis occurred within 24 hours. Immunologic tests showed the presence of type-C anti-neutrophil cytoplasmic antibodies (c-ANCA with antiproteinase 3 specificity and renal biopsy revealed pauci-immune crescentic focal necrotizing glomerulonephritis. Treatment including methylprednisolone and cyclophosphamide intravenous pulses allowed renal recovery after 3 weeks. The clinical, hematological, and biochemical parameters improved substantially, achieving remission. Granulomatosis with polyangiitis, although rare in children, should be considered in the above clinical scenario. This case underlines that knowledge of renal histology diagnosis and early aggressive immunosuppressive therapy are essential for the management of these patients.Keywords: acute renal failure, vasculitis, crescentic pauci-immune glomerulonephritis-Type-C, antineutrophil cytoplasmic antibodies (c-ANCA-macroscopic hematuria

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

    Directory of Open Access Journals (Sweden)

    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.

  3. Esclerite nodular e poliangiite granulomatosa (Wegener mimetizando tuberculose

    Directory of Open Access Journals (Sweden)

    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.

  4. Wegener's thinking about the mechanism: Greenland and Iceland

    Science.gov (United States)

    Jacoby, Wolfgang

    2015-04-01

    Wegener's early Arctic expeditions to Greenland (1906-08 and 1912-13 with a stop in Iceland) suggest a significant affect on his thinking about the mechanism of continental drift till his death in 1930. Beside his specialized work in meteorology and the Arctic, he had a broad general interest in science especially of the earth system as a whole. The drift idea occurred to him in 1910 on the basis of new data on geomorphology (Atlantic seafloor), supported by geophysics, geology and palaeontology. In his 1912 initial public talk and ensuing paper he mentioned something akin to seafloor spreading and refuted the continental relict hypothesis from break-up for the mid-Atlantic ridge. But 1912 he bypassed the tension fractures in Iceland and in Greenland (1912-13) he experienced the rheology of ice, brittle and viscous, when thinking about the drift of SIAL continents through the SIMA mantle (as documented in his diaries). When in 1915 rewriting his 1012 paper as the book "Die Entstehung der Kontinente und Ozeane" he had given up the early idea for that of floating continental rafts. It is tempting to speculate why. Rheology of rocks was clearly described. But some misconceptions distracted him from the correct relationships: (1) Data of the time suggested that sialic rock is more solid than mafic rock which would soften at lower temperature (contrary to present knowledge) and (2) convection in the atmosphere, well known to him, seemed to be no model for convection in the mantle, although rafting continents implied mantle flow. Did the rheologies appear too different to him? Not before the mid twenties (as documented in the 4th edition of his book, 1929) did Wegener admit that mantle convection might be the answer. A great spirit was misled but clearly saw that the phenomenon of drift, based on observations, is not refuted by the lack of an explanation.

  5. Idiopathic orofacial granulomatosis in a young patient: A rare entity

    Directory of Open Access Journals (Sweden)

    Gurumoorthy Kaarthikeyan

    2012-01-01

    Full Text Available The granular enlargement of the gingiva may be the first clinical manifestation of Orofacial granulomatosis, preceding other local or systemic manifestations. The term Idiopathic Orofacial granulomatosis (OFG refers to conditions restricted to the oral region without any identifiable systemic granulomatous diseases. We report a case of nine year old female patient with gingival enlargement as the sole manifestation without any systemic involvement. Laboratory investigations were done to rule out tuberculosis, sarcoidosis, allergic hypersensitivity, Crohn′s disease. The patient was treated by gingivectomy and there is complete remission of the lesion without any recurrence. Thus the early investigations and diagnosis of OFG can help in the prevention of future systemic complications.

  6. A Rare Presentation of a Rare Disease: Pulmonary Lymphomatoid Granulomatosis

    Directory of Open Access Journals (Sweden)

    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.

  7. Granulomatosis with Polyangiitis Presenting as a Parotid Gland Abscess

    Directory of Open Access Journals (Sweden)

    Blenda Dias

    2015-01-01

    Full Text Available Granulomatosis with polyangiitis (GPA is a small-vessel vasculitis consisting of necrotizing granulomatous lesions in airways and focal necrotizing glomerulonephritis. However, it may affect other sites such as the skin, central nervous system, eyes, heart, gastrointestinal tract, and liver. We describe a rare case of GPA in which the initial manifestation was the involvement of the parotid gland mimicking a pyogenic abscess.

  8. 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...

  9. Computed tomographic characteristics of eosinophilic pulmonary granulomatosis in five dogs.

    Science.gov (United States)

    Fina, Caroline; Vignoli, Massimo; Terragni, Rossella; Rossi, Federica; Wisner, Erik; Saunders, Jimmy H

    2014-01-01

    Canine pulmonary eosinophilic granulomatosis is a rare inflammatory pulmonary disease characterized by formation of eosinophilic granulomas that tend to obliterate the normal pulmonary architecture. The purpose of this retrospective study was to describe the CT characteristics of confirmed idiopathic pulmonary eosinophilic granulomatosis in a group of dogs. Five dogs met inclusion criteria. All patients were young adult dogs of variable breeds. No dog had concurrent occult heartworm disease. Computed tomographic characteristics most commonly included pulmonary masses and nodules of variable size, and lesions were most commonly located in the caudal lung lobes. Four dogs had large pulmonary masses with or without additional nodules and one dog had nodular lesions disseminated throughout the entire lung parenchyma. All large eosinophilic granulomas were smoothly margined, heterogeneous pulmonary masses displaying heterogeneous contrast enhancement. A honeycomb-like enhancement pattern was observed in all but one mass and consisted of multiple hyperattenuating rims delineating central hypoattenuating areas, suggestive of bronchiectatic lung with peripheral enhancing airway walls and fluid-filled, necrotic bronchial lumen. One dog had evidence of tracheobronchial lymphadenopathy. Findings indicated that canine eosinophilic pulmonary granulomatosis should be included as a differential diagnosis for dogs with CT characteristics of multiple pulmonary masses and/or nodules in caudal lung lobes, and a honeycomb-like enhancement pattern in masses after intravenous administration of iodinated contrast medium.

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

    Directory of Open Access Journals (Sweden)

    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.

  11. Eosinophilic Granulomatosis with Polyangiitis and Diffuse Gastrointestinal Involvement

    Directory of Open Access Journals (Sweden)

    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.

  12. Recurrence of Intravenous Talc Granulomatosis following Single Lung Transplantation

    Directory of Open Access Journals (Sweden)

    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.

  13. Characteristics and Outcomes of Granulomatosis With Polyangiitis (Wegener) and Microscopic Polyangiitis Requiring Renal Replacement Therapy: Results From the European Renal Association-European Dialysis and Transplant Association Registry

    NARCIS (Netherlands)

    Hruskova, Z.; Stel, V.S.; Jayne, D.; Aasarod, K.; Meester, J. de; Ekstrand, A.; Eller, K.; Heaf, J.G.; Hoitsma, A.J.; Jimenez, C. Martos; Ravani, P.; Wanner, C.; Tesar, V.; Jager, K.J.

    2015-01-01

    BACKGROUND: This study describes the incidence and outcomes of European patients requiring renal replacement therapy (RRT) for kidney failure due to antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). STUDY DESIGN: Cohort study. SETTING & PARTICIPANTS: 12 renal registries providi

  14. 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 pe

  15. Pulmonary angiocentric lymphoma (lymphomatoid granulomatosis) in a donkey.

    Science.gov (United States)

    du Toit, N; Genovese, L M; Dalziel, R G; Smith, S H

    2012-01-01

    A 36-year-old donkey developed dyspnoea, pyrexia, hypoalbuminaemia and oedema. Following continued clinical deterioration the donkey was humanely destroyed. Grossly, there were numerous nodules (5-10mm) scattered throughout the lung. Microscopically, the lung was infiltrated by an angiocentric and bronchocentric to diffuse mixed population of small mature and atypical lymphocytes, histiocytes, plasma cells and fewer eosinophils. The infiltrate was composed of numerous small mature and fewer atypical CD3(+) T lymphocytes. Low numbers of CD20(+) and CD79a(+) B cells, some atypical, accompanied the T cells. These infiltrates were consistent with an angiocentric lymphoma and resembled lymphomatoid granulomatosis, an Epstein-Barr virus (EBV)-associated human tumour. Immunohistochemistry for EBV latent membrane protein and polymerase chain reaction analysis for equine gamma herpesvirus DNA were negative. To the authors' knowledge this is the first case of angiocentric lymphoma reported in a donkey and the first case of lymphomatoid granulomatosis-type disease in an animal in which possible concurrent infection with a gamma herpesvirus has been investigated.

  16. /sup 67/Ga scanning in talc-induced pulmonary granulomatosis

    Energy Technology Data Exchange (ETDEWEB)

    Brown, D.G.; Aguirre, A.; Weaver, A.

    1980-04-01

    We describe a case of pulmonary granulomatosis in a user who habitually injected methylphenidate (Ritalin) intravenously; symptomatic and objective improvement occurred with corticosteroid therapy. A scan of the lungs using raioactive /sup 67/Ga showed an increased concentration of /sup 67/Ga throughout both lungs. There was a reduction in abnormal accumulation of /sup 67/Ga, improvement in the arterial oxygen pressure and the diffusing capacity for carbon monoxide, and a reduction in the infiltrate on the chest x-ray film two months after the institution of therapy. Before treatment the patient's symptoms and arterial deoxygenation increased despite the cessation of her drug abuse, thus raising the question of a self-perpetuating inflammatory process in a case of pulmonary deposition of talc.

  17. Blijvend vlakke wegen effect van variaties in de ondergrond op de voorspelling van langsvlakheid

    NARCIS (Netherlands)

    Gruijters, S.

    2009-01-01

    Dit rapport bevat de resultaten van het onderzoek dat door Deltares TNO is uitgevoerd in de periode 2007-2009 binnen het Delft Cluster onderzoek Blijvend Vlakke Wegen. Het onderzoek is gericht op het opzetten van een methodiek om variaties in de ondergrond te vertalen naar te verwachten restzettings

  18. Role of WEGENER (World Earthquake GEodesy Network for Environmental Hazard Research) in monitoring natural hazards (Invited)

    Science.gov (United States)

    Ozener, H.; Zerbini, S.; Bastos, M. L.; Becker, M. H.; Meghraoui, M.; Reilinger, R. E.

    2013-12-01

    WEGENER was originally the acronym for Working Group of European Geoscientists for the Establishment of Networks for Earth-science Research. It was founded in March 1981 in response to an appeal delivered at the Journées Luxembourgeoises de Geodynamique in December 1980 to respond with a coordinated European proposal to a NASA Announcement of Opportunity inviting participation in the Crustal Dynamics and Earthquake Research Program. WEGENER, during the past 33 years, has always kept a close contact with the Agencies and Institutions responsible for the development and maintenance of the global space geodetic networks with the aim to make them aware of the scientific needs and outcomes of the project which might have an influence on the general science policy trends. WEGENER served as Inter-commission Project 3.2, between Commission 1 and Commission 3, of the International Association of Geodesy (IAG) until 2012. Since then, WEGENER project has become the Sub-commission 3.5 of IAG commission 3, namely Tectonics and Earthquake Geodesy. In this presentation, we briefly review the accomplishments of WEGENER as originally conceived and outline and justify the new focus of the WEGENER consortium. The remarkable and rapid evolution of the present state of global geodetic monitoring in regard to the precision of positioning capabilities (and hence deformation) and global coverage, the development of InSAR for monitoring strain with unprecedented spatial resolution, and continuing and planned data from highly precise satellite gravity and altimetry missions, encourage us to shift principal attention from mainly monitoring capabilities by a combination of space and terrestrial geodetic techniques to applying existing observational methodologies to the critical geophysical phenomena that threaten our planet and society. Our new focus includes developing an improved physical basis to mitigate earthquake, tsunami, and volcanic risks, and the effects of natural and

  19. Mechanisms of Disease : pathogenesis and treatment of ANCA-associated vasculitides

    NARCIS (Netherlands)

    Kallenberg, Cees G M; Heeringa, Peter; Stegeman, Coen A

    2006-01-01

    Wegener's granulomatosis and microscopic polyangiitis are idiopathic systemic vasculitides strongly associated with antineutrophil cytoplasmic autoantibodies (ANCA). In Wegener's granulomatosis, ANCA are mostly directed against proteinase 3 (PR3), whereas in microscopic polyangiitis ANCA are directe

  20. 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.

  1. [Sarcoidosis--granulomatosis: the modern view of the etiology and pathogenesis with clinical cases review].

    Science.gov (United States)

    Kazmirchuk, V E; Tsarik, V V; Mal'tsev, D V; Ishchenko, M I

    2014-01-01

    Diagnostic criteria of sarcoidosis were offered in the 60-ies of XX century, however today the problem of sarcoidosis is difficult for understanding the different specialists and early detection. The development of laboratory diagnostic of viral infections and introduction of polymerase chain reaction (PCR) has greatly improved the level of diagnosis of herpes infections, reveal the previously unknown etiology of many diseases: sarcoidosis (granulomatosis), migraine, multiple sclerosis, cystic prenatal brain damage, convulsions, Hodgkin's disease and others. Sarcoidosis is the set of clinical symptoms (fatigue, shortness of breath, coughing, heaviness in the chest), laboratory, radiological and histopathological data that allow the doctor to diagnose, predict prognosis and treatment policy. Most often, sarcoidosis affects the lungs and thoracic lymph nodes. In the last period for 2011-2013 in 2930 immunologically tested patients the sarcoidosis was confirmed in 146. Primarily these patients were exposed to different diagnosis--COPD, pneumonia, tuberculosis, lung cancer. Among patients with sarcoidosis on the first place in frequency of detection were EBV and HHV-6. We were the first in 2000, described the Epstein-Barr virus as the causative factor of sarcoidosis, and has been hypothesized the immunopathology of sarcoidosis and principles of individual immunotherapy with a resolution of the granulomatous process in 92 % of cases. Subsequently, this association has been and illustrates the relationship to other viruses (HHV-6, HHV-8) demonstrated by other authors.

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

    Directory of Open Access Journals (Sweden)

    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.

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

    Science.gov (United States)

    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. PMID:27293925

  4. DOCK 8 Deficiency, EBV+ Lymphomatoid Granulomatosis, and Intrafamilial Variation in Presentation.

    Science.gov (United States)

    Dimitriades, Victoria R; Devlin, Vincent; Pittaluga, Stefania; Su, Helen C; Holland, Steven M; Wilson, Wyndham; Dunleavy, Kieron; Shah, Nirali N; Freeman, Alexandra F

    2017-01-01

    Dedicator of cytokinesis 8 (DOCK8) deficiency is an autosomal recessive, combined immunodeficiency within the spectrum of hyper-IgE syndromes. Epstein-Barr virus-positive lymphomatoid granulomatosis (LYG) (EBV + LYG) is a rare diagnosis and a previously unreported presentation of DOCK8 deficiency. A 10-year-old girl was initially evaluated for mild eczema and recurrent sinopulmonary infections. She had normal immunoglobulins with elevated IgE, poor polysaccharide response with low switched memory B cells, low CD4 count, and normal mitogen and antigen responses. Despite clinical improvement following immunoglobulin replacement, a prolonged cough prompted a CT scan, which showed nodules. Biopsy identified a Grade 2 EBV + LYG. Due to an inadequate response with chemotherapy, further workup for primary immunodeficiency was performed. With her symptoms of eczema and IgE elevation, along with her brother's history of recurrent sinopulmonary infections and warts, targeted sequencing of DOCK8 was performed revealing compound heterozygous mutations for the two siblings. Both patients were successfully transplanted with resolution of the LYG and warts, respectively. This is the first reported case of LYG in DOCK8 deficiency. The EBV-driven lymphoproliferative disease along with the infection history in the brother led to the diagnosis of DOCK8 deficiency and curative hematopoietic stem cell transplants.

  5. Lymphomatoid Granulomatosis of Central Nervous System and Lung Driven by Epstein Barr Virus Proliferation: Successful Treatment with Rituximab

    Directory of Open Access Journals (Sweden)

    Ruben Fernandez

    2014-02-01

    Full Text Available 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 of the bronchoalveolar lavage was positive for EBV and biopsy of lung node yielded a diagnosis of LYG, grade III. Shortly after initiation of treatment with chemotherapy, neurologic deterioration appeared. Radiologic 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 radiological and clinical improvement. After completion of treatment with immunochemotherapy, an autologous stem cell transplantation was performed. Patient stays in remission one year after diagnosis.

  6. Characterisation of a Swedish cohort with orofacial granulomatosis with or without Crohn's disease.

    Science.gov (United States)

    Gale, G; Östman, S; Rekabdar, E; Torinsson Naluai, Å; Högkil, K; Hasséus, B; Saalman, R; Jontell, M

    2015-01-01

    To compare oral manifestations in a Swedish cohort of patients with orofacial granulomatosis with or without Crohn's disease and to assess NOD2 polymorphisms in the two groups. Twenty-nine patients with orofacial granulomatosis were included. Demographics, disease history, clinical features and concurrent Crohn's disease were recorded. DNA was extracted from buccal swabs and examined for NOD2 variants Arg702Trp, Gly908Arg and Leu1007fsinsC, all previously linked to gastrointestinal Crohn's disease. Twelve of 29 patients were diagnosed with coexisting gastrointestinal Crohn's disease, and of whom 21 were males. Symptom duration was significantly longer for the orofacial granulomatosis group com-pared to the group with coexisting Crohn's disease (P orofacial granulomatosis patients also perceived their overall discomfort, aesthetic problems and social discomfort as more severe. No significant differences in the clinical presentation of oral lesions between the two groups were found. None of the patients with orofacial granulomatosis carried any of the NOD2 variations, whereas four of the 12 patients with coexisting Crohn's disease had a NOD2 variant (Arg702Trp). The two patient groups had similar phenotypic characteristics but seemed to have genotypic differences regarding NOD2. The Swedish cohort differed in their clinical characteristics from patients reported in other geographical regions. © 2014 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  7. Lymphomatoid Granulomatosis: CT and FDG-PET Findings

    Energy Technology Data Exchange (ETDEWEB)

    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.

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

    Directory of Open Access Journals (Sweden)

    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

  9. Long-term outcome of lung transplantation in previous intravenous drug users with talc lung granulomatosis.

    Science.gov (United States)

    Weinkauf, J G; Puttagunta, L; Nador, R; Jackson, K; LaBranche, K; Kapasi, A; Mullen, J; Modry, D L; Stewart, K C; Thakrar, M; Doucette, K; Lien, D C

    2013-01-01

    Talc lung granulomatosis results from the intravenous use of medication intended for oral use. Talc (magnesium silicate) acts as filler in some oral medications; when injected intravenously, it deposits in the lungs leading to airflow obstruction and impaired gas exchange. Allocation of donor lungs to previous intravenous drug users is controversial. After a careful selection process, 19 patients with talc lung granulomatosis have received lung allografts in our program. Long-term survival for these patients is excellent and our results suggest the previous use of intravenous drugs should not necessarily preclude lung transplantation.

  10. Wegener’s granulomatosis and environmental factors in Western Montana

    Directory of Open Access Journals (Sweden)

    Andrew Samuel Zeft

    2010-12-01

    Full Text Available The objective of our study was to determine whether exposure to silica or other environmental factors is associated with developing Wegener’s granulomatosis (WG, in a geographically isolated region of Western Montana. We sought to identify and interview all cases of WG diagnosed during 1993-2006 among residents of a ten-county region of Western Montana, as well as a group of demographically similar controls (n=39 without autoimmune disease. In the interview, we ascertained occupational silica and other exposures (metals, solvents, pesticides, tobacco. We enumerated 32 cases of WG, of whom 27 were included in the case-control study. Overall, a history of silica exposure was not associated with WG (OR = 0.68, 95% CI: 0.13-3.27, although there was a suggestion of increase in risk among persons with relatively recent (OR=2.67, 95% CI: 0.54-17.2, heavy (OR=1.82, 95% CI: 0.09-112.9, and prolonged (OR=1.53, 95% CI: 0.16-20.0 exposures. A history of having worked in the mining industry was associated with WG (six cases including three with no silica exposure, zero controls, lower 95% CI: 1.53. Risk was not associated with occupational or aerial pesticide exposure, but with residential rodenticide use (OR=12.15, 95% CI: 1.54-552. Occupational exposure to metals or solvents was not associated with WG, nor was a history of cigarette smoking. Results of earlier studies of WG support the hypothesis that silica exposure adversely influences the risk of developing WG. Our small study of WG failed to identify an association with silica overall, but the results are compat­ible with an increased risk in persons with relatively heavy, prolonged, and/or recent exposure.

  11. Detailed Follow-up Study of Pediatric Orofacial Granulomatosis Patients.

    Science.gov (United States)

    Haaramo, Anu; Alapulli, Heikki; Aine, Liisa; Saarnisto, Ulla; Tuokkola, Jetta; Ruuska, Tarja; Sipponen, Taina; Pitkäranta, Anne; Kolho, Kaija-Leena

    2017-10-01

    Orofacial granulomatosis (OFG) is a chronic inflammatory condition affecting the orofacial area. Its connection to Crohn disease (CD) is debated. Our aim was to describe a cohort of pediatric patients with OFG in detail, study the long-term behavior of OFG, and evaluate factors predicting CD in patients with OFG. We invited patients diagnosed with OFG at 2 university hospitals, Finland for a follow-up appointment. Patients (n = 29) were examined by a dentist and an otorhinolaryngologist using a structural schema. Orofacial findings were also recorded using digital photographing. Patients filled in questionnaires about general health and special diets. Patients' nutrition was evaluated from food records. The findings were compared between patients with OFG only and OFG with CD. Patients with CD had more findings in the orofacial area (total score for orofacial findings median 11) compared to patients with OFG only (total score median 7.5). There was no statistically significant difference in the type of lesions between these groups, except the upper lip was more often affected in patients with CD (n = 11) than in patients with OFG only (n = 0). Most of the patients had normal otorhinolaryngological findings. All patients with elevated anti-Saccharomyces cerevisiae antibody A levels had CD (n = 6) and they presented with more orofacial findings (total score) than patients with normal levels of anti-S cerevisiae antibody A (P = 0.0311). Long-term follow-up of pediatric-onset patients with OFG shows good prognosis. Patients with OFG do not seem to have otorhinolaryngological comorbidity. Anti-S cerevisiae antibody A may serve as a factor to indicate the possible presence of underlying CD in patients with OFG, but further studies are requested.

  12. Recurrent Septic Arthritis Due to Achromobacter xylosoxidans in a Patient With Granulomatosis With Polyangiitis.

    Science.gov (United States)

    Patel, Payal K; von Keudell, Arvind; Moroder, Philipp; Appleton, Paul; Wigmore, Robin; Rodriguez, Edward K

    2015-12-01

    We report a case of recurrent Achromobacter xylosoxidans infections including bacteremia, sepsis, septic joints and endocarditis in a 72 year old female with granulomatosis with polyangiitis. Achromobacter xylosoxidans is a gram negative bacteria increasingly identified in immunocompromised patients. Surgical and medical therapy may need to be combined.

  13. 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 tubercul......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...

  14. Voriconazole-induced periostitis causing arthralgias mimicking a flare of granulomatosis with polyangiitis.

    Science.gov (United States)

    Gladue, Heather S; Fox, David A

    2013-12-01

    We describe a case of voriconazole-induced periostitis that occurred in a 68-year-old woman with granulomatosis with polyangiitis. Our patient presented with months of severe hip pain limiting her daily activities, which was initially felt to be a flare of her granulomatosis with polyangiitis. However, upon further review, she had an elevated alkaline phosphatase and periostitis on her hip radiograph; voriconazole was held, and within 2 days she had marked improvement in her pain. Although this clinical syndrome is well documented in transplant patients, it is a rare complication in patients with autoimmune disorders. However, it is important because it may cause severe arthralgias that can mimic a flare of rheumatic diseases.

  15. Chronic meningitis with multiple cranial neuropathies: A rare initial presentation of Wegener′s granulomatosis

    Directory of Open Access Journals (Sweden)

    Vikas Gupta

    2013-01-01

    Full Text Available Wegener′s granulomatosis (WG is a systemic necrotizing vasculitis that affects the small blood vessels. It mainly affects the upper and lower respiratory tract and kidneys. Central nervous system (CNS involvement is rare, and has been reported only in about 8% of cases during the course of illness. Initial presentation with neurologic affection, particularly chronic hypertrophic meningitis is very unusual. We report the case of a 34 year old male who presented with chronic hypertrophic meningitis and multiple cranial nerve involvement as the initial manifestation, without respiratory and renal symptoms. This case highlights the difficulties in diagnosing a rare disease with rarer presentation, and at the same time illustrates that Wegener′s granulomatosis should be considered in the differential diagnosis of chronic meningitis.

  16. Granulomatosis with Polyangiitis with Bilateral Facial Palsy and Severe Mixed Hearing Loss

    Directory of Open Access Journals (Sweden)

    Agnieszka Wawrzecka

    2016-01-01

    Full Text Available Granulomatosis with polyangiitis is autoimmune and rare disease. It affects many organs, but the most often affected organs are the nose, lungs, and kidneys. It is part of vasculitis and causes an autoimmune attack by an abnormal type of circulating antibody termed ANCAs against small blood vessels. Disease concerns both men and women with a peak age of presentation in the sixth and seven decades. Typically upper and lower respiratory tract and kidneys are involved. Otitis externa, otitis media, or mastoiditis rarely occurs in granulomatosis with polyangiitis. Deafness is the most dangerous aural complication. Histological examination of biopsy is often not specific. A case of GPA with bilateral otitis media, bilateral deafness, and bilateral facial palsy with fatal course is presented.

  17. Two cases of extrapulmonary onset granulomatosis with polyangiitis which caused diffuse alveolar haemorrhage

    Directory of Open Access Journals (Sweden)

    Halide Kaya

    2014-01-01

    Full Text Available Granulomatosis with polyangiitis (GPA is a rare form of vasculitis. Multidisciplinary therapeutic approach and early diagnosis assume vital importance in management of patients with diffuse alveolar haemorrhage caused by GPA, which is a rare complication. The purpose of this study was to present the diagnostic and therapeutic challenges experienced by clinicians in management of two severe cases of GPA with insidious extrapulmonary manifestations which rapidly progressed into acute kidney injury, alveolar haemorrhage and acute respiratory failure.

  18. Pachymeningitis in Granulomatosis with Polyangiitis: A Case Report and a Review of the Literature

    Directory of Open Access Journals (Sweden)

    Grigorios T. Sakellariou

    2013-01-01

    Full Text Available Central nervous involvement, mainly with symptoms of cranial neuropathies, occurs in 2–8% of patients with granulomatosis with polyangiitis (GPA. Meningeal involvement, with persistent and severe headache as main manifestation and abnormal thickening and enhancement of the dural mater on postcontrast magnetic resonance imaging, is extremely rare. We present a case of pachymeningitis due to limited GPA, providing simultaneously a literature review.

  19. [C-ANCA positive necrotising scleritis and multiple sclerosis compatible with ocular Wegener: treatment with rituximab].

    Science.gov (United States)

    Aldasoro-Cáceres, V; Aldasoro-Cáceres, I; Pérez-Moreiras, J V; Murié-Fernández, M; Ibáñez-Bosch, R

    2014-01-01

    A patient diagnosed with necrotizing scleritis, c-ANCA+ an orbital pseudotumour, and possible multiple sclerosis in 2003 was treated with oral cyclophosphamide and steroids with partial response. Between 2005-2010 she suffered self-limited episodes. In 2010 a first scleral transplant was performed with poor outcome. She was treated with rituximab, and a second graft was performed with good results. At 12 months there was no change in magnetic resonance and the second graft healed. Wegener's disease with limited involvement of the orbit and/or the eye is a rare condition. The histopathology, blood analysis, symptoms and good response to treatment are the key to its diagnosis. Copyright © 2011 Sociedad Española de Oftalmología. Published by Elsevier Espana. All rights reserved.

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

    OpenAIRE

    Antoccia Antonio; Ferrari Francesca; Angelino Giulia; Scarselli Alessia; Folgori Laura; 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...

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

    Directory of Open Access Journals (Sweden)

    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

  2. Pulmonary sarcoid-like granulomatosis induced by aluminum dust: report of a case and literature review

    Institute of Scientific and Technical Information of China (English)

    CAI Hou-rong; CAO Min; MENG Fan-qing; WEI Jing-yi

    2007-01-01

    @@ Awide range of pulmonary pathology is attributed to aluminum dust exposure, including interstitial fibrosis,1-5 desquamative interstitial pneumonia,6 and pulmonary alveolar proteinosis.7 To our knowledge,granulomatous lung disease induced by aluminum dust is rare, only two cases were reported in literatures.8,9 We had the opportunity to make a diagnosis of pulmonary sarcoid-like granulomatosis in a patient exposed to aluminum dust. Herein, we report the clinical history,radiographic and histopathological findings in this patient,and the detailed mineralogical analyses performed on lung tissue obtained by open lung biopsy.

  3. Pulmonary lymphomatoid granulomatosis: Report of a case and review of literature

    Directory of Open Access Journals (Sweden)

    D B Olusina

    2011-01-01

    Full Text Available Lymphomatoid granulomatosis (LYG is a rare angiocentric lymphoproliferative process predominantly affecting the lung. The diagnosis of this condition is often difficult as the physical signs, history, chest x-ray, and routine laboratory investigations are usually non-specific. Nevertheless, it is important to establish a tissue diagnosis, as this lymphoproliferative disorder can be refractory to treatment and even progress to overt lymphoma. We report a case of pulmonary LYG in a 52-year old Nigerian man of Ibo extraction treated in our centre in 2001 and followed up for a year. The difficulty in making diagnosis is highlighted and treatment modality discussed.

  4. A rare case of primary orofacial granulomatosis of gingiva during pregnancy

    Directory of Open Access Journals (Sweden)

    Vaibhavi Joshipura

    2015-01-01

    Full Text Available Orofacial granulomatosis (OFG comprises a group of diseases characterized by noncaseating granulomatous inflammation affecting the soft tissues of the oral and maxillofacial region. It is important to establish the diagnosis accurately because this condition is sometimes a manifestation of many systemic conditions like Crohn's disease or sarcoidosis. The clinical outcome of OFG patients continues to be unpredictable. Current therapies remain unsatisfactory. This article reports a rare case of isolated OFG with langhans type giant cells and inflammatory infiltrate without any systemic involvement, in which the condition was a manifestation of pregnancy. The diagnostic approach to and the treatment of OFG are reviewed.

  5. A rare case of primary orofacial granulomatosis of gingiva during pregnancy.

    Science.gov (United States)

    Joshipura, Vaibhavi; Mahantesha, S; Subbaiah, Shobha Krishna; Lakkasetty, Yogesh T

    2015-01-01

    Orofacial granulomatosis (OFG) comprises a group of diseases characterized by noncaseating granulomatous inflammation affecting the soft tissues of the oral and maxillofacial region. It is important to establish the diagnosis accurately because this condition is sometimes a manifestation of many systemic conditions like Crohn's disease or sarcoidosis. The clinical outcome of OFG patients continues to be unpredictable. Current therapies remain unsatisfactory. This article reports a rare case of isolated OFG with langhans type giant cells and inflammatory infiltrate without any systemic involvement, in which the condition was a manifestation of pregnancy. The diagnostic approach to and the treatment of OFG are reviewed.

  6. Wegener′s granulomatosis presenting as spontaneous pneumothorax in young adult

    Directory of Open Access Journals (Sweden)

    Sunil Kumar

    2014-01-01

    Full Text Available Pulmonary involvement in Wegener′s granulomatosis (WG usually starts with nonspecific symptoms such as cough, dyspnea, hemoptysis, and pleuritis. Spontaneous pneumothorax as initial presentation is extremely rare. Although its real incidence is unknown, according to different classic series, it ranges between 3 and 5% of the cases. In this case, a 28-year-old male presented with complaints of epistaxis and breathlessness, which was diagnosed as WG with pneumothorax on the basis of chest X-ray and computed tomography (CT chest and pathological confirmation by high level of serum cytoplasmic antineutrophil cytoplasmic antibody (ANCA.

  7. CT引导经皮肺切割活检术诊断活动性韦格纳肉芽肿%Percutaneous CT -guided Core Needle Biopsy of Lungs in the Diagnosis of Active Wegener's Granulomatosis

    Institute of Scientific and Technical Information of China (English)

    陈敏; 郭佑民; 张少娟; 杨健; 牛刚; 汪军峰

    2005-01-01

    目的探讨CT引导下经皮肺芯针切割活检术(percutaneous CT-guided core needle biopsy,PTCB)对活动性韦格纳肉芽肿(WG)的诊断价值以及指导临床治疗的意义.方法8例经PTCB行病理组织学检查WG病人,根据其临床资料作BVAS评分,其结果与病理对照研究.结果8例病人经PTCB取得足够的组织学标本,病理结果:①坏死性血管炎(75%),②肉芽性炎症(62.5%),③实质坏死(50%),④肺出血改变(12.5%),⑤纤维化(12.5%).确诊时BVAS平均分数为10分,所有病人确诊后都进行了糖皮质激素联合细胞毒药物冲击治疗,临床有不同程度好转,治疗4周后平均分数为2.5分.结论多部位、多次经皮肺切割活检可以准确的诊断活动性WG,指导临床早期治疗.

  8. 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

  9. Place in therapy of rituximab in the treatment of granulomatosis with polyangiitis and microscopic polyangiitis.

    Science.gov (United States)

    Shah, Shivani; Geetha, Duvuru

    2015-01-01

    Granulomatosis with polyangiitis and microscopic polyangiitis are small vessel vasculitides characterized by circulating antineutrophil circulating antibodies. Standard treatment for active severe disease has consisted of cyclophosphamide with glucocorticoids with or without plasmapheresis, which achieves approximately 75% sustained remission, but carries significant adverse effects such as malignancy, infertility, leukopenia, and infections. The role of B cells in the pathogenesis of anti-neutrophil circulating antibodies-associated vasculitis has been established, and as such, rituximab, a monoclonal anti-CD20 antibody, has been studied in treatment of active granulomatosis with polyangiitis and microscopic polyangiitis (induction) and in maintaining remission. Rituximab has been shown to be effective in inducing remission in several retrospective studies in patients with refractory disease or cyclophosphamide intolerance. The RAVE and RITUXVAS trials demonstrated rituximab is a noninferior alternative to standard cyclophosphamide-based therapy; however, its role in elderly patients and patients with severe renal disease warrants further investigation. Rituximab has been compared with azathioprine for maintaining remission in the MAINRITSAN trial and may be more efficacious in maintaining remission in patients treated with cyclophosphamide induction. Rituximab is not without risks and carries a similar adverse event risk rate as cyclophosphamide in randomized control trials. However, its use can be considered over cyclophosphamide in patients who have relapsing or refractory disease or in young patients seeking to preserve fertility.

  10. Atypical Endobronchial Carcinoid with Postobstructive Pneumonia Obscuring the Diagnosis of Granulomatosis with Polyangiitis

    Directory of Open Access Journals (Sweden)

    Robert Ali

    2015-01-01

    Full Text Available Granulomatosis with polyangiitis (GPA, previously termed Wegener’s Granulomatosis, is an autoimmune small vessel vasculitis which is highly associated with antineutrophil cytoplasmic antibodies (ANCA and has varied clinical manifestations. Diagnosis hinges on identifying a combination of clinical features of systemic vasculitis, positive ANCA serology, and histological evidence of necrotizing vasculitis, necrotizing glomerulonephritis, or granulomatous inflammation from a relevant organ biopsy. The American College of Rheumatology has also developed a classification criteria focusing specifically on nasal or oral inflammation, abnormal chest radiograph, and abnormal urinary sediment, along with granulomatous inflammation, which helps to distinguish GPA from other forms of systemic vasculitis. In the case presented below, the diagnosis of GPA was delayed as the patient had a concomitant atypical endobronchial carcinoid which predisposed to postobstructive pneumonia. Fortunately, the papular lesions that developed across her lower limbs prompted further investigations. The return of appropriate serology coincided with progression to alveolar hemorrhage, offering a more complete clinical picture, and when she responded to the combination of steroid, cyclophosphamide, and plasma exchange, the diagnosis of GPA was cinched.

  11. Polishing surgical metal pieces, granulomatosis and mineralogical analysis.

    Science.gov (United States)

    Catinon, Mickaël; Chemarin, C; Roux, E; Cavalin, C; Rosental, P-A; Thivolet-Bejui, F; Vincent, M

    2016-08-01

    This report describes the case of a 44-year-old man with pulmonary nodules whose histological analysis initially suggested tuberculosis. The Mycobacterium tuberculosis (MT) culture was negative and a questionnaire revealed a professional activity of brushing and polishing surgical instruments without any protection for 7 years.  A mineralogical analysis by optical and electron microscopy was performed on both a healthy lung tissue biopsy and a lung nodule in a paraffin block. Electron microscopy analysis revealed the presence of metal particles (iron oxide, titanium oxide, aluminum oxide and steel) in both samples. This study suggests that mineralogical analysis combined with a questionnaire on dust exposure could help redirect the diagnosis of a dust-related disease.

  12. Liver granulomatosis: a case of Chlamydophila pneumoniae infection.

    Science.gov (United States)

    Yildiz, H; Wieërs, G; Yombi, J C; Marot, J C

    2015-02-01

    An 18-year-old man was referred to the Internal Medicine ward because of a 2-week history of intermittent high fever, weight loss and cough. Clinical examination revealed hepato-splenomegaly and multiple lymph nodes swelling while laboratory tests showed elevated C-reactive protein, gamma glutamyl transferase and lactate dehydrogenase. All serologic testes for auto-immune antibodies, viruses and bacteria were negative except for Chlamydophila pneumoniae. An 18-FDG PET computed tomography scanner showed hypermetabolism in the liver, spleen and lymph nodes. We therefore conducted a liver biopsy that demonstrated non-necrotizing granulomas. We conclude to a C. pneumoniae infection associated with a granulomatous hepatitis. After treatment with Doxyciclin the patient had no more fever, hepatosplenomegaly resolved and blood testes normalized. This case report is to our knowledge the first report of a granulomatous hepatitis associated with C. pneumoniae respiratory infection.

  13. Genetically Distinct Subsets within ANCA-Associated Vasculitis

    NARCIS (Netherlands)

    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; Harper, Lorraine; Hruskova, 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, Mrten; Stegeman, Coen A.; Tesar, Vladimir; Vaglio, Augusto; Wieczorek, Stefan; Wilde, Benjamin; Zwerina, Jochen; Rees, Andrew J.; Clayton, David G.; Smith, Kenneth G. C.

    2012-01-01

    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 i

  14. 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...

  15. Cohort profile: the Welsh Geriatric Registrar-Led Research Network (WeGeN): rationale, design and description

    Science.gov (United States)

    Long, Sara; Butler, John

    2017-01-01

    Purpose Medical trainees are required to undertake audit and quality improvement projects. They must also have an understanding of the principles of research and are encouraged to participate in research projects. However, the constraints of time, a lack of formal training and rotation between different training posts create barriers to audit cycle completion and pursuing research. This leads to trainees being reluctant to undertake research, facilitates poor quality research and risks incomplete audit. Participants The Welsh Geriatricians Network (WeGeN) has been created with the aims of facilitating collaborative, trainee-led research within Geriatric Medicine in Wales, promoting research engagement and improving the research evidence base for older patients. By coordinating collaborative research projects across different sites within Wales, trainees continue existing projects at new sites, allowing completion of projects and establishing the long-term infrastructure and experienced personnel needed for high-quality research data to be gathered. Findings WeGeN has facilitated 4 national audits, all of which are intended for peer review publication. The first project considers the service provision for the older person in the emergency department, the second Parkinson's disease, the third reviews delirium management and the fourth project considers epidemiology of surgical disease in older people. Future plans The objective of this project is to further establish and develop WeGeN as a group which facilitates high-quality research and provides the opportunity for geriatric trainees to engage in research activity. It is anticipated that the establishment of this research platform will provide a blueprint for the development of other such networks in the UK and beyond. PMID:28196947

  16. 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

  17. Granulomatosis de células de Langerhans: Presentación de un caso

    Directory of Open Access Journals (Sweden)

    . 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.

  18. Pronounced Risk of Fractures among Elderly Men Affected by Granulomatosis with Polyangiitis

    DEFF Research Database (Denmark)

    Faurschou, Mikkel; Baslund, Bo; Obel, Niels

    2015-01-01

    OBJECTIVE: It is unknown whether patients affected by granulomatosis with polyangiitis (GPA) are at increased risk of fractures, and whether the fracture risk in GPA varies with age and sex. The aim of the present study was to compare the fracture risk among patients with GPA with that among age......- and sex-matched population controls. METHODS: We established a monocentric cohort of patients treated for GPA at a Danish tertiary care center from 1995 to 2010 (n = 159) and a register-derived GPA cohort identified from the Danish National Hospital Register (n = 402). Each patient was matched with 7...... population controls. The occurrence of fractures among patients was compared with that among controls by calculation of incidence rate ratios (IRR). RESULTS: In the monocentric cohort, an increased fracture risk was observed among men aged ≥ 55 years at the time of first hospitalization for GPA (IRR 3.5, 95...

  19. Risk of Diabetes Mellitus among Patients Diagnosed with Giant Cell Arteritis or Granulomatosis with Polyangiitis

    DEFF Research Database (Denmark)

    Faurschou, Mikkel; Ahlström, Magnus G; Lindhardsen, Jesper

    2017-01-01

    patients diagnosed with giant cell arteritis (GCA) or granulomatosis with polyangiitis (GPA), i.e., patients with relatively common forms of systemic vasculitis. METHODS: We used Danish healthcare registries to identify 1682 patients diagnosed with GCA and 342 patients diagnosed with GPA from 1997 to 2015.......7-10.6) in the GPA cohort. During the first year after diagnosis of vasculitis, the IRR for DM was 7.0 (95% CI 5.2-9.3) among patients with GCA and 10.4 (95% CI 4.4-24) among patients with GPA. IRR for DM were not significantly increased in either cohort during later followup periods. Within the first year......, treatment with high cumulative prednisolone/PRED doses was associated with new-onset DM among the patients with vasculitis. CONCLUSION: Patients diagnosed with GCA or GPA have a markedly increased risk of new-onset DM during early treatment phases....

  20. Granulomatosis with polyangiitis and facial palsy: Literature review and insight in the autoimmune pathogenesis.

    Science.gov (United States)

    Iannella, Giannicola; Greco, Antonio; Granata, Guido; Manno, Alessandra; Pasquariello, Benedetta; Angeletti, Diletta; Didona, Dario; Magliulo, Giuseppe

    2016-07-01

    Granulomatosis with polyangiitis (GPA) is an autoimmune systemic necrotizing small-vessel vasculitis associated with the presence of anti-neutrophil cytoplasmic antibodies (ANCA). Oto-neurological manifestations of ANCA-associated vasculitis according to PR3-ANCA positivity and MPO-ANCA positivity are usually reported. Facial nerve palsy is usually reported during the clinical course of the disease but it might appear as the presenting sign of GPA. Necrotizing vasculitis of the facial nerve 'vasa nervorum' is nowadays the most widely accepted etiopathogenetic theory to explain facial damage in GPA patients. A central role for PR3-ANCA in the pathophysiology of vasculitis in GPA patients with oto-neurological manifestation is reported. GPA requires prompt, effective management of the acute and chronic manifestations. Once the diagnosis of GPA has been established, clinicians should devise an appropriate treatment strategy for each individual patient, based on current clinical evidence, treatment guidelines and recommendations.

  1. [Teutschlaender lipo-calcino-granulomatosis or tumoral calcinosis of Inclan (author's transl)].

    Science.gov (United States)

    Barrière, H; Welin, J; Lenne, Y; Visset, J; Vigier, P

    1977-02-01

    The present report describes a typical case of tumoral calcinosis. Differential diagnosis was initially directed towards sarcoma suggested by fibrous connective tissue surrounding cystlike cavities. The course of this disease led to chronic multiple fistulae with secondary infection in spite of two attempts of surgical excision. This case adds further support to the results of earlier reports showing no specific biological abnormalities. The present sutyd indicates otherwise that tumoral calcinosis and lipo-calcino-granulomatosis of Teutschlaender are the same condition. The pathogenesis of this affection remains enigmatic; there is no evidence that the lipidic excess should be the beginning of the process, but this disease must be considered as a distinctive form of calcinosis. The surgical exeresis is the only possible treatment, even if it doesn't prevent recurrence.

  2. Arthritis in lymphomatoid granulomatosis: Report of a case and review of literature

    Directory of Open Access Journals (Sweden)

    Agarwal Vikas

    2004-02-01

    Full Text Available Lymphomatoid granulomatosis (LG is a rare systemic vasculitis caused by Epstein Barr virus induced transformation of the B-cells in a T-cell rich environment. The predominant clinical presentations are confined to the pulmonary system however; extra-pulmonary manifestations can sometimes be the main feature of the disease. Here in we describe a 52-year-old female who presented with symmetric polyarthritis and generalized stiffness for 7 months and papular lesions over extremities for 3 months duration. She in addition had generalized lymphadenopathy. Histopathological examination of the cutaneous lesions confirmed LG. Patient died despite therapy with cyclophosphamide and prednisolone. This is the first report of LG mimicking rheumatoid arthritis from India.

  3. Fatal Cytomegalovirus Disease after Combination Therapy with Corticosteroids and Rituximab for Granulomatosis with Polyangiitis

    Directory of Open Access Journals (Sweden)

    Talal Hilal

    2015-01-01

    Full Text Available The association of cytomegalovirus (CMV with autoimmune disease is poorly understood with suggested causality and reported viral reactivation coinciding with active inflammation. We report a case of a patient who presented with diffuse alveolar hemorrhage and acute renal failure from rapidly progressive glomerulonephritis ultimately diagnosed with granulomatosis with polyangiitis (GPA. She was acutely managed with plasmapheresis to reduce antibody-mediated end-organ damage, hemodialysis for worsening hyperkalemia and acidosis, and high-dose intravenous methylprednisolone. She was transitioned to oral prednisone and started on weekly rituximab with resultant remission induction over a three-week period at which point she developed reactivation of CMV causing severe fatal lung disease and viremia. The case highlights the multiple factors associated with CMV reactivation in cases of severe systemic inflammatory states and the need for further research to help establish practice guidelines regarding antimicrobial prophylaxis in patients with autoimmune diseases on prolonged courses of corticosteroids and biologic agents.

  4. Place in therapy of rituximab in the treatment of granulomatosis with polyangiitis and microscopic polyangiitis

    Directory of Open Access Journals (Sweden)

    Shah S

    2015-08-01

    Full Text Available Shivani Shah, Duvuru Geetha Department of Medicine, Division of Nephrology, Johns Hopkins University School of Medicine, Baltimore, MD, USA Abstract: Granulomatosis with polyangiitis and microscopic polyangiitis are small vessel vasculitides characterized by circulating antineutrophil circulating antibodies. Standard treatment for active severe disease has consisted of cyclophosphamide with glucocorticoids with or without plasmapheresis, which achieves approximately 75% sustained remission, but carries significant adverse effects such as malignancy, infertility, leukopenia, and infections. The role of B cells in the pathogenesis of anti-neutrophil circulating antibodies-associated vasculitis has been established, and as such, rituximab, a monoclonal anti-CD20 antibody, has been studied in treatment of active granulomatosis with polyangiitis and microscopic polyangiitis (induction and in maintaining remission. Rituximab has been shown to be effective in inducing remission in several retrospective studies in patients with refractory disease or cyclophosphamide intolerance. The RAVE and RITUXVAS trials demonstrated rituximab is a noninferior alternative to standard cyclophosphamide-based therapy; however, its role in elderly patients and patients with severe renal disease warrants further investigation. Rituximab has been compared with azathioprine for maintaining remission in the MAINRITSAN trial and may be more efficacious in maintaining remission in patients treated with cyclophosphamide induction. Rituximab is not without risks and carries a similar adverse event risk rate as cyclophosphamide in randomized control trials. However, its use can be considered over cyclophosphamide in patients who have relapsing or refractory disease or in young patients seeking to preserve fertility. Keywords: rituximab, ANCA-associated vasculitis, GPA, MPA, induction therapy, maintenance therapy

  5. 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.

  6. How Earth works 100 years after Wegener's continental drift theory and IGCP 648

    Science.gov (United States)

    Li, Z. X.; Evans, D. A.; Zhong, S.; Eglington, B. M.

    2015-12-01

    It took half a century for Wegener's continental drift theory to be accepted as a fundamental element of the plate tectonic theory. Another half a century on, we are still unsure of the driving mechanism for plate tectonics: is it dominated by thermal convection, gravitational forces, or by a combination of mechanisms? Nonetheless, breakthroughs in the past decades put us in a position to make a major stride in answering this question. These include: (1) widely accepted cyclic occurrences of supercontinent assembly and break-up (whereas random occurrence of supercontinents was an equal possibility in the 1990s); (2) the discovery of two equatorial and antipodal large low seismic velocity provinces (LLSVPs) that dominate the lower mantle and appear to have been the base for almost all mantle plumes since at the Mesozoic, and of subduction of oceanic slabs all the way to the core-mantle boundary, which together suggesting whole-mantle convection; (3) the recognition of true polar wander (TPW) as an important process in Earth history, likely reflecting Earth's major internal mass redistribution events; and (4) rapidly enhancing computer modelling power enabling us to simulate all aspect of Earth's dynamic inner working. Many new yet often controversial ideas have been proposed, such a possible coupling in time (with an offset) and space between supercontinent cycle and superplume (LLSVP) events which oppose to the idea of static and long-lived LLSVPs, and the orthoversion v.s. introversion or extroversion models for supercontinent transition. To fully utilise these advances as well as the rapidly expanding global geoscience databases to address the question of how Earth works, an UNESCO-IUGS sponsored IGCP project No. 648 was formed to coordinate a global cross-disciplinary effort. We aim to achieve a better understanding of the supercontinent cycle, and examine the relationship between supercontinent cycle and global plume events. We will establish a series of global

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

    Directory of Open Access Journals (Sweden)

    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.

  8. Hémothorax spontané révélant une vascularite de Wegener chez une femme enceinte

    Science.gov (United States)

    Serhane, Hind; Yassine, Msougar; Amro, Lamyae

    2016-01-01

    L’hémothorax spontané demeure une pathologie rare. Ses étiologies sont multiples mais restent parfois inconnues. Chez certains patients, la thoracotomie peut être le seul recours pour déterminer son origine. Les vascularites n’ont pas été rapporté comme cause habituelle des hémothorax spontanés. La grossesse ne semble pas avoir d’effet causal, ni aggravant des hémothorax spontanés, ni des vascularites. Nous rapportons une observation assez particulière d’une jeune patiente, présentant au cours de sa grossesse un hémothorax spontané secondaire à une vasularite de type Wegener, diagnostiquée par la biopsie pleurale faite au cours d’une thoracotomie exploratrice et confirmée par le dosage des ANCA. PMID:28154726

  9. MicroRNA-941 Expression in Polymorphonuclear Granulocytes Is Not Related to Granulomatosis with Polyangiitis

    DEFF Research Database (Denmark)

    Svendsen, Jesper Brink; Baslund, Bo; Cramer, Elisabeth Præstekjær

    2016-01-01

    granulomatosis with polyangiitis (GPA) have lower expression of miR-941 than healthy control donors as a biological cause for higher JMJD3 levels. We found no significant difference in the degree of maturation of PMNs from GPA patients (n = 8) and healthy controls (n = 11) as determined from cell surface...... expression of the neutrophil maturation marker CD16 and gene expression profile of FCGR3B. The expression of PRTN3 and KDM6B mRNAs and miR-941 was not significantly different in GPA patients and healthy controls. Transfection of pre-miR-941 into the neutrophil promyelocyte cell line PLB-985 cells did...... not result in reduction of the KDM6B mRNA level as shown previously in a hepatocellular carcinoma cell line. The amount of PR3 in PMNs from GPA patients and healthy controls was comparable. In conclusion, we found that PRTN3 mRNA, KDM6B mRNA, and miR-941 expression levels in PMNs do not differ between GPA...

  10. 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

    OBJECTIVE: To assess the impact of pre-existing co-morbidities on mortality among patients affected by granulomatosis with polyangiitis (GPA). METHODS: By means of the Danish National Hospital Register, we identified a cohort of patients hospitalized for GPA during 1994-2010 (n = 308). The burden...... 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...... = 246) was 3.9 (95% CI 2.0, 7.5) during years 0-2 and 1.4 (95% CI 0.9, 2.0) from the second year of follow-up onwards. The corresponding MRRs were 13.3 (95% CI 5.8, 31) and 1.9 (95% CI 1.1, 3.6) for patients with a CCI score ⩾1 (n = 62). In a direct comparison, GPA patients with a CCI score ⩾1 were...

  11. Eosinophilic granulomatosis with polyangiitis complicated by cholecystitis: a case report and review of the literature.

    Science.gov (United States)

    Ye, Lu; Lu, Xiaoyong; Xue, Jing

    2016-01-01

    The objectives are to report an atypical case of eosinophilic granulomatosis with polyangiitis (EGPA) associated with cholecystitis and to review the main clinical features, therapy, and prognosis of it. We present a 49-year-old male with non-classic clinical manifestations of EGPA and EGPA-related cholecystitis. EGPA was diagnosed by histology of the gallbladder after cholecystectomy. In addition, 11 cases of EGPA-associated cholecystitis have been reported and were described in details in this article. Gallbladder involvement is very uncommon in EGPA. All cases reviewed showed multiple organs involved as well as obviously elevated eosinophilic granulocyte proportion with inflammatory index, although antineutrophil cytoplasmic antibody may be negative. All patients in this cohort that showed gallbladder involvement were eventually confirmed with EGPA by histology examination after cholecystectomy. The pathological change could be infiltration of inflammatory mononuclear cells of small- and medium-sized vessels. Of the cases, 91.7% responded well to steroid and immunosuppressant therapy. Gallbladder involvement is a very rare comorbid condition in EGPA. However, it is an important symptom or secondary condition to alert physicians the diagnosis of EGPA. Moreover, timely diagnosis and correct administration in the early stage of this disease could obviously improve the prognosis.

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

    Directory of Open Access Journals (Sweden)

    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.

  13. Comparison of the Phenotype and Outcome of Granulomatosis with Polyangiitis Between UK and Japanese Cohorts.

    Science.gov (United States)

    Furuta, Shunsuke; Chaudhry, Afzal N; Arimura, Yoshihiro; Dobashi, Hiroaki; Fujimoto, Shouichi; Homma, Sakae; Rasmussen, Niels; Jayne, David R

    2017-02-01

    There are differences in the frequencies of antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis subgroups between different geographic regions, and we have reported differences in the phenotype of microscopic polyangiitis between Europe and Japan. In this retrospective observational study, we compared phenotypes and outcomes of granulomatosis with polyangiitis (GPA) between the United Kingdom and Japan. We identified 128 UK and 82 Japanese patients with GPA diagnosed between 2000 and 2012. We evaluated baseline characteristics including ANCA status and organ involvement, treatment, patient and renal survival, and time to first relapse. Median age at onset was higher in Japan than in the UK (62.2 yrs vs 57.5 yrs, p UK (61.0% vs 85.2%, p UK (34.1% vs 8.6%, p UK (68.1 μmol/l vs 101.0 μmol/l, p UK (69.5% vs 40.6%, p UK, respectively (p = 0.03). At 60 months the cumulative relapse rates were 37.1% and 68.1% in Japan and the UK, respectively (p UK patients. The relapse-free survival rate was higher in Japan than the United Kingdom.

  14. A clinicopathologic analysis of primary central nervous system lymphomatoid granulomatosis: case report and literature review

    Directory of Open Access Journals (Sweden)

    FU Yong-juan

    2012-10-01

    Full Text Available Objective To investigate the clinical, neuroimaging and histopathological features of primary central nervous system lymphomatoid granulomatosis (LG. Methods The clinical manifestation, neuroimaging, histopathological and biological features of a patient with primary central nervous system LG were presented, and the related literatures were reviewed. Results A 57-year-old male presented with memory impairment, weak in orientation, calculation, apprehension and judgment for 3 months. Magnetic resonance imaging (MRI showed space-occupying lesions in bilateral frontal lobes, with T1WI isointensity and T2WI hyperintensity, and the enhancement was irregular. The lesion was slight expansive with yellow surface and gray-white section in color and soft texture and abundant blood supply. Microscopically, the lesion was characterized by angiocentric and angiodestructive lymphoproliferation, partly showed the structure of LG characterized by T cell predominant proliferation, macrophage infiltration, astrocyte activation, small vessel proliferation and hyalinization, and partly showed the structure of lymphoma characterized by diffuse atypical B cell proliferation, with IgK monoclonal production. Epstein-Barr virus (EBV was negative. Conclusion As a precursor disease of lymphoma, LG should be considered in the differential diagnosis of both diffuse and multifocal lesions of the central nervous system. The relavance between primary central nervous system LG and EBV infection should be further discussed.

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

    Directory of Open Access Journals (Sweden)

    Christian Dejaco

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

  16. Prolonged risk of specific malignancies following cyclophosphamide therapy among patients with granulomatosis with polyangiitis

    DEFF Research Database (Denmark)

    Faurschou, Mikkel; Mellemkjaer, Lene; Voss, Anne

    2015-01-01

    -melanoma skin cancers (NMSCs) and 11 were bladder carcinomas. A high occurrence of NMSC was observed from the second year of follow-up onwards, with a SIR of 7.0 (95% CI 2.3, 16) for cases diagnosed ≥20 years after GPA. The incidence of bladder cancer increased after 5-9, 10-14 and 15-19 years of follow......OBJECTIVE: The long-term cancer risk for patients treated for granulomatosis with polyangiitis (GPA) is not well characterized. We assessed the risk of early and late-occurring cancers among 293 patients diagnosed with GPA from 1973 to 1999 and followed throughout 2010. METHODS: Cancer incidence...... in the cohort was determined by linkage with the Danish Cancer Registry and compared with that in the general population by calculation of standardized incidence ratios (SIRs). RESULTS: The median duration of follow-up was 9.7 years (range 0-36). Seventy-three cancers occurred, of which 30 were non...

  17. Generation of high-resolution wind fields from the dense meteorological station network WegenerNet in South-Eastern Austria

    Science.gov (United States)

    Schlager, Christoph; Kirchengast, Gottfried; Fuchsberger, Jürgen

    2016-04-01

    To investigate weather and climate on a local scale as well as for evaluating regional climate models (RCMs) the Wegener Center at the University of Graz established the long-term field experiment WegenerNet Feldbach region, a dense grid of 153 meteorological stations. The observations of these stations are managed by an automatic WegenerNet Processing system. This system includes a quality check of collected observations and a Data Product Generator (DPG), among other subsystems. Products already implemented in the DPG are gridded weather and climate products, generated from the main parameters temperature, precipitation and relative humidity (Kirchengast et. al., Bull. Amer. Meteor. Soc., 95, 227-242, 2014). Missing elements are gridded wind fields from wind observations. Wind is considered as one of the most difficult meteorological variables to model and depends on many different parameters such as topography and surface roughness. Therefore a simple interpolation can only be performed in case of uniform characteristics of landscape. The presentation introduces our method of generation of wind fields from near real-time observations of the WegenerNet. Purpose of this work is to provide a database with 3D wind fields in a high spatial and time resolution as addition to the existing products, for evaluating convection permitting climate models as well as investigating weather and climate on a local scale. Core of the application is the diagnostic California Meteorological Model (CALMET). This model computes 3D wind fields based on meteorological observational data, a digital elevation model and land use categories. The application generates the required input files from meteorological stations of the WegenerNet Feldbach region and triggers the start of the CALMET model with these input files. In a next step the modeled wind fields are stored automatically every 30 minutes with a spatial resolution of 100 x 100 m in the WegenerNet database. To verify the

  18. 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.

  19. Successful Use of Plasma Exchange in the Treatment of Corticosteroid-Refractory Eosinophilic Granulomatosis with Polyangiitis Associated with Gastrointestinal Manifestations

    Directory of Open Access Journals (Sweden)

    Kohei Tsujimoto

    2016-01-01

    Full Text Available We describe the case of a 33-year-old woman having corticosteroid-refractory eosinophilic granulomatosis with polyangiitis (EGPA who presented with abdominal pain and responded dramatically to plasma exchange. Eosinophilia, asthma history, neuropathy, pulmonary infiltrates, and paranasal sinus abnormalities confirmed the diagnosis of EGPA. Treatment was initiated with 1 g/day of methylprednisolone pulse therapy for 3 days followed by 60 mg/day of intravenous prednisolone without relieving abdominal pain. Then, plasma exchange was performed thrice. Abdominal pain disappeared after the first plasma exchange. Indication of plasma exchange for EGPA remains controversial; however, it may represent a valid option in cases with gastrointestinal involvement.

  20. Genetics and pathophysiology of granulomatosis with polyangiitis (GPA) and its main autoantigen proteinase 3.

    Science.gov (United States)

    Relle, Manfred; Föhr, Bernd; Fasola, Federica; Schwarting, Andreas

    2016-12-01

    Granulomatosis with polyangiitis (GPA) is a severe autoimmune disease and one of the small vessel anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides. Although its etiology and pathophysiology are still widely unknown, it is accepted that infections, environmental factors, epigenetic modifications, and a genetic predisposition provide the basis for this systemic disorder. GPA typically evolves into two phases: an initial phase characterized by ear, nose and throat (ENT) manifestations, such as chronic sinusitis and otitis, ulceration of the oral cavity and pharynx, as well as pulmonary nodules and a severe generalized phase, defined by the occurrence of rapidly progressive glomerulonephritis, pulmonary hemorrhage, and arthritis. ANCAs, directed against the neutrophilic enzymes proteinase 3 and myeloperoxidase, are present in up to 90% of the affected patients in the systemic phase. As the humoral immunity is predominantly directed against neutrophilic antigens, it is apparent that neutrophils play a critical role in GPA both as target and effector cells. Although GPA pathogenesis is not well known, some susceptibility genes and loci have been identified by candidate gene approaches, genome-wide association studies, and meta-analyses, as well as familial association studies. Such genes are CTLA4, PTPN22, COL11A2, SERPINA1, and the MHC class II gene cluster. This review highlights the clinical, pathophysiological, and genetic background of GPA and aims to give an overview of recent efforts to identify GPA susceptibility genes. We point out the genetic basis of the main autoantigen PR3 and why it is so difficult to establish a murine GPA model. Copyright © 2016 Elsevier Ltd. All rights reserved.

  1. IgA antibodies to myeloperoxidase in patients with eosinophilic granulomatosis with polyangiitis (Churg-Strauss).

    Science.gov (United States)

    Oommen, Esha; Hummel, Amber; Allmannsberger, Lisa; Cuthbertson, David; Carette, Simon; Pagnoux, Christian; Hoffman, Gary S; Jenne, Dieter E; Khalidi, Nader A; Koening, Curry L; Langford, Carol A; McAlear, Carol A; Moreland, Larry; Seo, Philip; Sreih, Antoine; Ytterberg, Steven R; Merkel, Peter A; Specks, Ulrich; Monach, Paul A

    2017-01-01

    To determine the prevalence of anti-myeloperoxidase (MPO) antibodies of IgA (IgA anti-MPO) isotype in patients with eosinophilic granulomatosis with polyangiitis (EGPA), and the association of the IgA antibodies with IgG anti-MPO and with disease activity. Serum samples from patients with EGPA followed in a multicenter longitudinal cohort were tested by ELISA for the presence of IgA anti-MPO and IgG anti-MPO antibodies. Sera from 87 healthy controls were used to define a positive test. Sera from 168 patients with EGPA (298 samples) were tested. Frequencies of positive testing for IgA anti-MPO were compared between patients with active EGPA, patients in remission, and controls. IgA anti-MPO was detected in 10 of 168 (6%) patients with EGPA (11 of 298 serum samples) compared to 1 of 87 (1%) healthy controls (p=0.10). All 11 samples testing positive for IgA anti-MPO also tested positive for IgG anti-MPO. Ninety samples tested positive for IgG anti-MPO but negative for IgA. Samples taken during active EGPA were positive for IgA anti-MPO in 6/72 cases (8%), compared to 5/226 (2%) during remission (p=0.03). Among samples taken during moderate or high disease activity, 5/41 were positive (12%, p=0.01 compared to remission). Although IgA anti-MPO antibodies are detectable in some patients with EGPA and may be detectable more frequently during active disease, their presence seems unlikely to provide information beyond what is obtained from conventional IgG anti-MPO.

  2. Azathioprine is effective for oral involvement in Crohn's disease but not for orofacial granulomatosis alone.

    Science.gov (United States)

    Mentzer, A; Goel, R; Elliott, T; Campbell, H; Hullah, E; Patel, P; Challacombe, S; Escudier, M; Sanderson, J D

    2016-04-01

    There have been no previous reports assessing the effectiveness of azathioprine (AZA) in the treatment of orofacial granulomatosis (OFG). This report is a review of patients receiving AZA for active OFG with or without concomitant gut Crohn's disease (CD) in a specialist tertiary referral centre. Clinical response was defined by Global Physician Assessment at 4-, 12- and 24-month follow-up and a standardised oral disease activity score (ODAS). Sixty of 215 patients seen with OFG in our clinic over a 12-year period were treated with AZA. Of these, 22 had concomitant CD. The proportion of patients responding to AZA with a diagnosis of CD/OFG vs. OFG only at 4, 12 and 24 months were 54% vs. 21% (P = 0.03), 59% vs. 21% (P = 0.003) and 41% vs. 24% (P = 0.16), respectively. A statistically significant difference was seen between starting and follow-up ODAS scores at 4 months in the CD/OFG group which was not observed in the OFG only group. Factors predicting a need for AZA included a diagnosis of intestinal CD, sulcal swelling, sulcal ulcers and upper lip involvement. The factor predicting response to treatment was a diagnosis of CD at 12 months of follow-up. No difference in the number of adverse effects was observed between the two groups of patients. AZA is significantly more effective in the treatment of oral disease with a concurrent diagnosis of CD rather than in the treatment of OFG alone. © 2015 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  3. Vasculitis Pregnancy Registry

    Science.gov (United States)

    2016-09-08

    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

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

    Energy Technology Data Exchange (ETDEWEB)

    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.)

  5. MicroRNA-941 Expression in Polymorphonuclear Granulocytes Is Not Related to Granulomatosis with Polyangiitis

    Science.gov (United States)

    Svendsen, Jesper Brink; Baslund, Bo; Cramer, Elisabeth Præstekjær; Rapin, Nicolas; Borregaard, Niels

    2016-01-01

    Jumonji Domain-Containing Protein 3 (JMJD3)/lysine demethylase 6B (KDM6B) is an epigenetic modulator that removes repressive histone marks on genes. Expression of KDM6B mRNA is elevated in leukocytes from patients with ANCA-associated vasculitis (AAV) and has been suggested to be the reason for higher proteinase 3 (PR3) mRNA expression in these cells due to derepression of PRTN3 gene transcription. MicroRNA-941 (miR-941) has been shown to target KDM6B mRNA and inhibit JMJD3 production. We therefore investigated whether polymorphonuclear granulocytes (PMNs) from patients suffering from granulomatosis with polyangiitis (GPA) have lower expression of miR-941 than healthy control donors as a biological cause for higher JMJD3 levels. We found no significant difference in the degree of maturation of PMNs from GPA patients (n = 8) and healthy controls (n = 11) as determined from cell surface expression of the neutrophil maturation marker CD16 and gene expression profile of FCGR3B. The expression of PRTN3 and KDM6B mRNAs and miR-941 was not significantly different in GPA patients and healthy controls. Transfection of pre-miR-941 into the neutrophil promyelocyte cell line PLB-985 cells did not result in reduction of the KDM6B mRNA level as shown previously in a hepatocellular carcinoma cell line. The amount of PR3 in PMNs from GPA patients and healthy controls was comparable. In conclusion, we found that PRTN3 mRNA, KDM6B mRNA, and miR-941 expression levels in PMNs do not differ between GPA patients and healthy controls, and that miR-941 does not uniformly regulate KDM6B mRNA levels by inducing degradation of the transcript. Thus, decreased miR-941 expression in PMNs cannot be part of the pathogenesis of GPA. PMID:27755585

  6. Association between orofacial granulomatosis and Crohn's disease in children: systematic review.

    Science.gov (United States)

    Lazzerini, Marzia; Bramuzzo, Matteo; Ventura, Alessandro

    2014-06-21

    To review pediatric cases of orofacial granulomatosis (OFG), report disease characteristics, and explore the association between OFG and Crohn's disease. We conducted a systematic review according to the PRISMA guidelines. We searched Medline, LILACS, Virtual Health Library, and Web of Knowledge in September 2013 for cases of OFG in the pediatric age range (< 18 years), with no language limitations. All relevant articles were accessed in full text. The manual search included references of retrieved articles. We extracted data on patients' characteristics, disease characteristics, association with other diseases, and treatment. We analyzed the data and reported the results in tables and text. We retrieved 173 reports of OFG in children. Mean age at onset was 11.1 ± 3.8 years (range: 2.0-18 years). Prevalence in males was significant higher than in females (P < 0.001), with a male:female ratio of 2:1. Gastrointestinal signs or symptoms were present in 26.0% of children at the time of OFG diagnosis. Overall, 70/173 (40.4%) children received a concomitant diagnosis of Crohn's disease. In about half (51.4%) of the cases the onset of OFG anticipated the diagnosis of Crohn's disease, with a mean time between the two diagnoses of 13.1 ± 11.6 mo (range: 3-36 mo). Overall, 21/173 (12.1%) of the children with OFG had perianal disease, while 11/173 (6.4%) had a family history of Crohn's disease. Both perianal disease and a family history of Crohn's disease were significantly associated with a higher risk of Crohn's disease diagnosis in children with OFG [relative risk (RR) = 3.10, 95% confidence interval (CI): 2.46-3.90; RR = 2.74, 95%CI: 2.24-3.36, P < 0.0001 for both). Treatment of OFG included steroids (70.8% of children) and other immunosuppressive drugs (42.7%), such as azathioprine, thalidomide and infliximab. High prevalence of Crohn's disease in children with OFG suggests that OFG may be a subtype of Crohn's disease.

  7. WegenerNet climate station network region Feldbach/Austria: From local measurements to weather and climate data products at 1 km-scale resolution

    Science.gov (United States)

    Kabas, T.; Leuprecht, A.; Bichler, C.; Kirchengast, G.

    2010-12-01

    South-eastern Austria is characteristic for experiencing a rich variety of weather and climate patterns. For this reason, the county of Feldbach was selected by the Wegener Center as a focus area for a pioneering observation experiment at very high resolution: The WegenerNet climate station network (in brief WegenerNet) comprises 151 meteorological stations within an area of about 20 km × 15 km (~ 1.4 km × 1.4 km station grid). All stations measure the main parameters temperature, humidity and precipitation with 5 minute sampling. Selected further stations include measurements of wind speed and direction completed by soil parameters as well as air pressure and net radiation. The collected data is integrated in an automatic processing system including data transfer, quality control, product generation, and visualization. Each station is equipped with an internet-attached data logger and the measurements are transferred as binary files via GPRS to the WegenerNet server in 1 hour intervals. The incoming raw data files of measured parameters as well as several operating values of the data logger are stored in a relational database (PostgreSQL). Next, the raw data pass the Quality Control System (QCS) in which the data are checked for its technical and physical plausibility (e.g., sensor specifications, temporal and spatial variability). In consideration of the data quality (quality flag), the Data Product Generator (DPG) results in weather and climate data products on various temporal scales (from 5 min to annual) for single stations and regular grids. Gridded data are derived by vertical scaling and squared inverse distance interpolation (1 km × 1 km and 0.01° × 0.01° grids). Both subsystems (QCS and DPG) are realized by the programming language Python. For application purposes the resulting data products are available via the bi-lingual (dt, en) WegenerNet data portal (www.wegenernet.org). At this time, the main interface is still online in a system in which

  8. 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.

  9. [Hereditary systemic autoinflammatory diseases. Part II: cryopyrin-associated periodic syndromes, pediatric systemic granulomatosis and PAPA syndrome].

    Science.gov (United States)

    Aróstegui, Juan I; Yagüe, Jordi

    2008-03-29

    Hereditary systemic autoinflammatory diseases result from a genetically-based dysregulated inflammatory process, and are clinically characterized by recurrent or persistent systemic inflammatory episodes, which typically occur in the absence of infectious, neoplastic or autoimmune etiology. Elucidation of their molecular basis has enabled the use of genetic analyses to achieve an accurate and definitive diagnosis, and to establish a tailored treatment. The present review is the second and last part of an updated and comprehensive overview of hereditary systemic autoinflammatory diseases, and will introduce persistent, non-periodic autoinflammatory diseases, such as: a) the group of cryopyrin-associated periodic syndromes (CAPS), which includes familial cold-induced autoinflammatory syndrome (FCAS), Muckle-Wells syndrome, and CINCA-NOMID syndrome; b) the group of pediatric systemic granulomatosis, which includes both Blau syndrome and early-onset sarcoidosis, and c) the pyogenic sterile arthritis, pyoderma gangrenosum and acne (PAPA) syndrome.

  10. Depositional evolution of a reef-dominated Upper Permian carbonate platform, Wegener Halvoe Formation, Karstryggen area, East Greenland

    Energy Technology Data Exchange (ETDEWEB)

    Stemmrik, L.

    1996-12-31

    The Wegener Halvoe Formation carbonate platform in the Karstryggen area consists of three third order depositional sequences that formed in response to three Kazanian sea level cycles. Pinning point curves for the sub-aerial exposure surfaces separating the depositional sequences quantify the amplitude of the relative sea level fluctuations in the range of 70-140 m. The Karstryggen platform developed on top of a karstified older Permian carbonate platform and pre-depositional relief was locally exceeding 70 m. The pre-depositional relief influenced deposition in all three sequences. Algal-cement buildups formed over karst pinnacles and merged during time to form a N-S trending barrier dissected by deep fluvially modified karst channels. Peritidal carbonates formed behind the barrier whereas off platform sedimentation was dominated by thin transgressive siliciclastics overlain by shallow marine algal-foraminifer grain-stones in the first two sequences. During the last sea level cycle pro-grading oolitic grain-stones formed basin-wards of the algal-cement buildups. Transgressive systems tracts are thin and often dominated by condensed siliciclastic units in off platform areas and palaeolows. Over palaeotopographic highs it consists of aggrading algal cement-stones. High-stand deposits are limited to palaeotopographic elevated areas and consist of algal cementstones along the basin margin and shallow sub-tidal to inter-tidal carbonates and evaporites in the platform area. Outside these areas carbonate deposition took place during falling sea level, and during deposition of the first two sequences thin laterally extensive units of shallow marine grain-stones were deposited directly on top of deeper marine siliciclastics. During the final sea level fall, thick pro-grading units of oolitic grain-stones were deposited. (au) Appendix no. 3. 28 refs.

  11. Clinical features and diagnosis of primary cerebral lymphomatoid granulomatosis%原发性颅内淋巴瘤样肉芽肿病的临床特点及诊断

    Institute of Scientific and Technical Information of China (English)

    卫华; 李存江; 朴月善; 卢德宏; 周静安

    2014-01-01

    目的:总结原发性颅内淋巴瘤样肉芽肿病的临床特点、影像学及组织病理学特点。方法报告我院收治的1例原发性颅内淋巴瘤样肉芽肿病病例,并对相关文献进行复习。结果患者,女,28岁,癫痫发作3年余,头颅MRI显示散在多发病灶,环形或结节状强化,病灶周围水肿明显。除颅内多发病灶外其他器官未受累。脑组织活检病理学提示淋巴瘤样肉芽肿病。结论原发性颅内淋巴瘤样肉芽肿病影像学有一定特点,但无特异性,因此颅内占位性病变或散在、浸润性病变患者需注意和本病鉴别。单纯颅内淋巴瘤样肉芽肿病预后较好。%Objective To report a case of primary brain lymphomatoid granulomatosis (LYG), and summarize its imaging, histopathological characteristics and prognosis. Methods The clinical, pathological, imaging and laboratory data of the patient were presented, and the pertinent literature were reviewed to discuss the importance of this rare entity. Re-sults A 28-year-old woman had been suffering from epilepsia for more than 3 years. MRI revealed multiple focal in-tra-parenchymal lesions, with heterogeneous enhancement surrounded by perilesional edema in the left frontal and tempo-ral lobe. The focal resection of the lesion in the left frontal was performed and lymphomatoid granulomatosis was diagnosed based on the histopathological examination. Conclusion CNS-LYG is a rare disease that should be considered in the dif-ferential diagnosis of both diffuse and space-occupying cerebral lesions. Primary cerebral LYG appears to have a better prognosis than systemic LYG with CNS localization.

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

    Directory of Open Access Journals (Sweden)

    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

  13. 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

    Directory of Open Access Journals (Sweden)

    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.

  14. Woorden wikken en wegen

    NARCIS (Netherlands)

    Kraaij, W.

    2009-01-01

    Zoals eenieder zich nog wel de roes kan herinneren na de eindexamenuitslag van de middelbare school, zo heeft voor mij ook de eerste kennismaking met het world wide web een bijzondere indruk gemaakt. Het zal ergens in 1993 geweest zijn toen ik aan collega’s van het toenmalige Instituut voor Taal en

  15. Prolonged B cell depletion with rituximab is effective in treating refractory pulmonary granulomatous inflammation in granulomatosis with polyangiitis (GPA).

    Science.gov (United States)

    Henderson, Scott R; Copley, Susan J; Pusey, Charles D; Ind, Philip W; Salama, Alan D

    2014-12-01

    Pulmonary nodule formation is a frequent feature of granulomatosis with polyangiitis (GPA). Traditional induction therapy includes methotrexate or cyclophosphamide, however, pulmonary nodules generally respond slower than vasculitic components of disease. Efficacy of rituximab (RTX) solely for the treatment of pulmonary nodules has not been assessed. In this observational cohort study, we report patient outcomes with RTX in GPA patients with pulmonary nodules who failed to achieve remission following conventional immunosuppression. Patients (n = 5) with persistent pulmonary nodules were identified from our clinic database and retrospectively evaluated. Systemic manifestations, inflammatory markers, disease activity, concurrent immunosuppression, and absolute B cell numbers were recorded pre-RTX and at 6 monthly intervals following treatment. Chest radiographs at each time point were scored by an experienced radiologist, blinded to clinical details. Five patients with GPA and PR3-ANCA were evaluated (2 male, 3 female), mean age 34 (22-52) years. Pulmonary nodules (median 4, range 2-6), with or without cavitation were present in all patients. RTX induced initial B cell depletion (GPA following prolonged B cell depletion.

  16. Orofacial granulomatosis affecting lip and gingiva in a 15-year-old patient: A rare case report

    Directory of Open Access Journals (Sweden)

    Monika Bansal

    2015-01-01

    Full Text Available Orofacial granulomatosis (OFG is a rare disorder affecting the orofacial region, and clinically characterized by diffuse, nontender, soft to firm, painless swelling restricted to one or both lips and intraoral sites such as tongue, gingiva and buccal mucosa. Histologically, OFG is characterized by noncaseating granulomatous inflammation. The early diagnosis of OFG is essential for the better prognosis of the lesion. Delay in diagnosis of OFG results into formation of indurated and permanent swelling of the lip that not only compromises esthetic appearance but also causes impairment in function such as speaking and eating. Early diagnosis of OFG is challenging to the health care professionals due to clinical and histological resemblance to other chronic granulomatous disorders. Thus, dentists may act as a first person to diagnose the lesion and play an important role in the multidisciplinary treatment of granulomatous disorders. Here, we present a case of OFG affecting lips and gingiva in a 15-year-old patient without any identifiable systemic or local causes.

  17. Diffuse alveolar hemorrhage emerging one week after starting high-dose corticosteroid therapy for granulomatosis with polyangiitis (GPA) with systemic lupus erythematosus (SLE).

    Science.gov (United States)

    Fukui, Shoichi; Iwamoto, Naoki; Tsuji, Sosuke; Umeda, Masataka; Nishino, Ayako; Nakashima, Yoshikazu; Suzuki, Takahisa; Horai, Yoshiro; Koga, Tomohiro; Kawashiri, Shin-Ya; Ichinose, Kunihiro; Hirai, Yasuko; Tamai, Mami; Nakamura, Hideki; Origuchi, Tomoki; Kawakami, Atsushi

    2015-01-01

    A 69-year-old man was diagnosed with granulomatosis with polyangiitis (GPA) based on the presence of skin granuloma, refractory otitis media, renal insufficiency and myeloperoxidase-antineutrophil cytoplasmic antibody positivity and slight lung opacity. He was treated with high-dose corticosteroid therapy. Despite the initial improvement of his renal function and a decrease in his C-reactive protein level, he suffered from an alveolar hemorrhage one week after the start of corticosteroid therapy. An anti-dsDNA antibody test was positive and the patient had hypocomplementemia. Elements of both GPA and systemic lupus erythematosus were thought to have affected his clinical course.

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

    Science.gov (United States)

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

    2015-11-01

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

  19. 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,

  20. TNF-alpha bioactivity-inhibiting therapy in ANCA-associated vasculitis : Clinical and experimental considerations

    NARCIS (Netherlands)

    Huugen, Dennis; Tervaert, Jan Willem Cohen; Heeringa, Peter

    2006-01-01

    Wegener's granulomatosis, microscopic polyangiitis, idiopathic necrotizing crescentic glomerulonephritis, and Churg-Strauss syndrome are associated with the presence of ANCA with specificity for myeloperoxidase or proteinase 3. Current therapy consists mainly of corticosteroids and cyclophosphamide,

  1. 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

  2. Pauci-immune necrotizing glomerulonephritis

    NARCIS (Netherlands)

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

    2010-01-01

    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 Churg-St

  3. Vasculitis Illness Perception (VIP) Study

    Science.gov (United States)

    2014-07-11

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

  4. 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...

  5. 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...

  6. 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

  7. 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

  8. Antiproteinase 3-and antimyeloperoxidase-associated vasculitis

    NARCIS (Netherlands)

    Franssen, CFM; Stegeman, CA; Kallenberg, CGM; Gans, ROB; de Jong, PE; Hoorntje, SJ; Tervaert, JWC

    Wegener's granulomatosis, microscopic polyangiitis, and idiopathic pauci-immune necrotizing crescentic glomerulonephritis (NCGN) are strongly associated with antineutrophil cytoplasmic autoantibodies (ANCAs) directed against either proteinase 3 (anti-PR3) or myeloperoxidase (anti-MPO). This has led

  9. Antineutrophil cytoplasmic autoantibodies specific for myeloperoxidase cause glomerulonephritis and vasculitis in mice

    NARCIS (Netherlands)

    Xiao, H; Heeringa, P; Hu, PQ; Liu, Z; Zhao, ML; Aratani, Y; Maeda, N; Falk, RJ; Jennette, JC

    2002-01-01

    Antineutrophil cytoplasmic autoantibodies (ANCAs) are identified in the circulation of approximately 80% of patients with pauci-immune necrotizing and crescentic glomerulonephritis and systemic small vessel vasculitis, such as microscopic polyangiitis and Wegener granulomatosis. The most common

  10. Long-term patient survival in ANCA-associated vasculitis

    NARCIS (Netherlands)

    Flossmann, Oliver; Berden, Annelies; de Groot, Kirsten; Hagen, Chris; Harper, Lorraine; Heijl, Caroline; Hoglund, Peter; Jayne, David; Luqmani, Raashid; Mahr, Alfred; Mukhtyar, Chetan; Pusey, Charles; Rasmussen, Niels; Stegeman, Coen; Walsh, Michael; Westman, Kerstin

    2011-01-01

    Background 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. Objective T

  11. Bilateral orbital cavernous haemangiomas.

    OpenAIRE

    Fries, P D; Char, D. H.

    1988-01-01

    Simultaneous bilateral orbital lesions are rare. The differential diagnosis includes orbital pseudotumour, metastasis, leukaemia, lymphoma, Wegener's granulomatosis, and neurofibromatosis. We report what we believe to be the first case of bilateral orbital cavernous haemangiomas.

  12. Antineutrophil cytoplasmic autoantibody-associated small-vessel vasculitis

    NARCIS (Netherlands)

    Kallenberg, Cees G. M.

    2007-01-01

    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 micro

  13. Increased frequency of circulating IL-21 producing Th-cells in patients with granulomatosis with polyangiitis (GPA).

    Science.gov (United States)

    Abdulahad, Wayel H; Lepse, Nikola; Stegeman, Coen A; Huitema, Minke G; Doornbos-van der Meer, Berber; Tadema, Henko; Rutgers, Abraham; Limburg, Pieter C; Kallenberg, Cees G M; Heeringa, Peter

    2013-01-01

    The present study aimed to explore a possible role for IL-21 producing Th-cells in the immunopathogenesis of granulomatosis with polyangiitis (GPA). Peripheral blood from 42 GPA patients in remission and 29 age-matched healthy controls (HCs) were stimulated in vitro, and the frequencies of IL-21 producing Th-cells were determined by flow cytometry. Since Th17-cells produce a low level of IL-21, IL-17 was also included in the analysis. Given that IL-21 is a hallmark cytokine for T follicular helper cells (T(FH)), we next evaluated the expression of their key transcription factor BCL-6 by RT-PCR and flow cytometry. To investigate the effect of IL-21 on autoantibody-production, PBMCs from GPA patients were stimulated in vitro with BAFF/IL-21 and total IgG and ANCA levels were measured in supernatants. In addition, the expression of IL-21-receptor on B-cells was analyzed. Percentages of IL-21 producing Th-cells were significantly elevated in GPA-patients compared to HCs, and were restricted to ANCA-positive patients. The expression of BCL-6 was significantly higher in ANCA-positive GPA-patients, as compared with ANCA-negative patients and HCs. IL-21 enhanced the production of IgG and ANCA in vitro in stimulated PBMCs from GPA patients. No difference was found in the expression of the IL-21-receptor on B-cells between ANCA-negative patients, ANCA-positive patients, and HCs. The increased frequency of circulating IL-21 producing Th-cells in ANCA-positive GPA patients and the stimulating capacity of IL-21 on ANCA-production suggest a role for these cells in the immunopathogenesis of GPA. Blockade of IL-21 could constitute a new therapeutic strategy for GPA.

  14. 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....

  15. ANCA-associated vasculitis and malignancy

    DEFF Research Database (Denmark)

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

    2013-01-01

    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 infla......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...

  16. [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...

  17. Cardiovascular magnetic resonance for evaluation of heart involvement in ANCA-associated vasculitis. A luxury or a valuable diagnostic tool?

    Science.gov (United States)

    Mavrogeni, Sophie; Markousis-Mavrogenis, George; Kolovou, Genovefa

    2014-01-01

    Antineutrophil cytoplasmic antibody (ANCA)-related vasculitis is a systemic small-vessel vasculitis, including 3 clinical syndromes: granulomatosis with polyangiitis, known as Wegener's granulomatosis (WG), microscopic polyangiitis (MPA) and the Churg-Strauss syndrome (CSS). ANCA-related vasculitis usually presents with severe kidney or pulmonary disease, has a mortality of 28% at 5 years, and also contributes to increased morbidity in vasculitis patients. Cardiac involvement in this entity may have different forms, including coronary vessels, pericarditis, myocarditis, endocarditis, myocardial infarction and subendocardial vasculitis that can contribute to reduced life expectancy. Cardiovascular magnetic resonance using oedema and fibrosis imaging can early reveal, noninvasively and without radiation, heart involvement during vasculitis, undetected by other imaging techniques and guide further risk stratification and treatment of these patients.

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

    Science.gov (United States)

    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.

  19. Poliangeitis amb Granulomatosis eosinofílica i asma al·lèrgica; dues monedes amb una mateixa cara?

    OpenAIRE

    Ramentol Sintas, Marc

    2013-01-01

    La síndrome de Churg-Strauss o Poliangeitis amb Granulomatosis eosinofílica (EGPA), clàssicament s'ha vinculat a un substrat asmàtic d'origen al·lèrgic. No obstant, l'evidència en aquest sentit és equívoca. L'objectiu d'aquest treball és identificar diferències clíniques i serològiques entre pacients amb EGPA i pacients asmàtics al·lèrgics, i determinar el perfil al·lèrgic d'un grup de pacients diagnosticats d'EGPA. Es comparen dos grups de pacients: un grup de diagnosticats d'EGPA, i un altr...

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

    Science.gov (United States)

    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.

  1. Evaluation of automated multi-parametric indirect immunofluorescence assays to detect anti-neutrophil cytoplasmic antibodies (ANCA) in granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA).

    Science.gov (United States)

    Csernok, Elena; Damoiseaux, Jan; Rasmussen, Niels; Hellmich, Bernhard; van Paassen, Pieter; Vermeersch, Pieter; Blockmans, Daniel; Cohen Tervaert, Jan-Willem; Bossuyt, Xavier

    2016-07-01

    The aim of this multicenter EUVAS study was to evaluate the diagnostic performance of multi-parametric indirect immunofluorescence (IIF) assays to detect anti-neutrophil cytoplasmic antibodies (ANCA) in granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA). The study included 912 samples from diseased controls and 249 diagnostic samples from GPA (n=183) and MPA (n=66) patients. The performance of two automated multi-parametric assays [Aklides (Medipan/Generic Assays) and EuroPattern (Euroimmun)] combining IIF on cellular and purified antigen substrates was compared with two manual IIF analyses and with commercially available ELISAs for MPO- and PR3-ANCA (Euroimmun). The area under the curve (AUC) of the receiver operating characteristics (ROC) curve to discriminate AAV from controls was 0.925, 0.848, 0.855 and 0.904 for, respectively, the two manual analyses, Aklides and EuroPattern, and 0.959, 0.921 and 0.886 for, respectively, antigen-specific ELISA, antigen-coated beads, and microdot, respectively. Variation in pattern assignment between IIF methods was observed. The performance of IIF depends on the substrate used and the definition of IIF patterns. The performance of automated IIF is improved by multi-parameter testing (combined IIF and antigen-specific testing). Given the variability between IIF methods, the diagnostic importance of this technique is questioned. Copyright © 2016. Published by Elsevier B.V.

  2. Eosinophilic Granulomatosis With Polyangiitis With Thrombotic Microangiopathy: Is Simultaneous Systemic Lupus Erythematosus Associated With Clinical Manifestations?: A Case Report and Review of the Literature.

    Science.gov (United States)

    Fukui, Shoichi; Iwamoto, Naoki; Tsuji, Sosuke; Umeda, Masataka; Nishino, Ayako; Nakashima, Yoshikazu; Suzuki, Takahisa; Horai, Yoshiro; Koga, Tomohiro; Kawashiri, Shin-Ya; Ichinose, Kunihiro; Hirai, Yasuko; Tamai, Mami; Nakamura, Hideki; Origuchi, Tomoki; Kinoshita, Naóe; Kawakami, Atsushi

    2015-11-01

    Eosinophilic granulomatosis with polyangiitis (EGPA) is one of the antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis, which is characterized by vasculitis of the small to medium-sized vessels. On the contrary, thrombotic microangiopathy (TMA) is a life-threatening condition which can cause ischemic organ injury. Although several case reports have described patients with TMA associated with ANCA-associated vasculitis except for EGPA, there are no previous case reports of EGPA associated with TMA.A 71-year-old Japanese man was diagnosed with EGPA based on his asthma, eosinophilia, lung opacity, refractory sinusitis, and positive myeloperoxidase-ANCA. He was also diagnosed with TMA based on peripheral schizocytes and hemolytic anemia. We performed plasmapheresis and started high-dose corticosteroid therapy; thereafter, he improved promptly. His case also fulfilled the classification criteria of systemic lupus erythematosus (SLE) based on the pleural effusion, renal disorder, anemia, thrombocytopenia, positive antidouble-stranded DNA antibody, and low complement. Elements of SLE were thought to affect his clinical course.We reviewed 11 patients with EGPA or hypereosinophilic syndrome (HES) associated with SLE, including our case. Patients with EGPA or HES associated with SLE had more heart complications than patients with simple EGPA or simple HES did. Patients with EGPA or HES associated with SLE had more pleural effusion than patients with simple SLE did.Clinical manifestations of eosinophilia with SLE or SLE with eosinophilia may differ from simple SLE or simple eosinophilia.

  3. 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.

  4. Ambiguity Revealed

    OpenAIRE

    Subir Bose; Matthew Polisson; Ludovic Renou

    2012-01-01

    We derive necessary and suffcient conditions for data sets composed of state-contingent prices and consumption to be consistent with two prominent models of decision making under ambiguity: variational preferences and smooth ambiguity. The revealed preference conditions for the maxmin expected utility and subjective expected utility models are characterized as special cases.

  5. Ambiguity revealed

    OpenAIRE

    Bayer, Ralph-C; Bose, Subir; Polisson, Matthew; Renou, Ludovic

    2013-01-01

    We derive necessary and sufficient conditions for data sets composed of state-contingent prices and consumption to be consistent with two prominent models of decision making under uncertainty: variational preferences and smooth ambiguity. The revealed preference conditions for subjective expected utility, maxmin expected utility, and multiplier preferences are characterised as special cases. We implement our tests on data from a portfolio choice experiment.

  6. Mathematics revealed

    CERN Document Server

    Berman, Elizabeth

    1979-01-01

    Mathematics Revealed focuses on the principles, processes, operations, and exercises in mathematics.The book first offers information on whole numbers, fractions, and decimals and percents. Discussions focus on measuring length, percent, decimals, numbers as products, addition and subtraction of fractions, mixed numbers and ratios, division of fractions, addition, subtraction, multiplication, and division. The text then examines positive and negative numbers and powers and computation. Topics include division and averages, multiplication, ratios, and measurements, scientific notation and estim

  7. REVEALED ALTRUISM

    OpenAIRE

    Cox, James C; Friedman, Daniel; Sadiraj, Vjollca

    2009-01-01

    This pap er develops a theory of revealed preferences over oneís own and othersímonetary payo§s. We intro duce ìmore altruistic thanî(MAT), a partial ordering over preferences, and interpret it with known parametric mo dels. We also intro duce and illustrate ìmore generous thanî (MGT), a partial ordering over opp ortunity sets. Several recent discussions of altruism fo cus on two player extensive form games of complete information in which the Örst mover (FM) cho oses a more or less gen...

  8. Higher antineutrophil cytoplasmic antibody (C-ANCA) titers are associated with increased overall healthcare use in patients with sinonasal manifestations of granulomatosis with polyangiitis (GPA).

    Science.gov (United States)

    Janisiewicz, Agnieszka M; Klau, Marc H; Keschner, David B; Lehmer, Randy R; Venkat, Kumar V; Medhekar, Swati S; Chang, Parke K; Badran, Karam; Leary, Ryan; Garg, Rohit; Nguyen, Andrew A; Lee, Jivianne T

    2015-01-01

    Granulomatosis with polyangiitis (GPA) is an autoimmune disease characterized by necrotizing granulomatous airway inflammation and vasculitis. Sinonasal involvement occurs in more than 80% cases, with antineutrophil cytoplasmic antibody (C-ANCA) titers used as a marker of disease severity. The purpose of this study was to determine whether C-ANCA levels impact radiographic findings and healthcare use in patients with sinonasal GPA. A retrospective review was performed on GPA patients evaluated in a multidisciplinary rheumatologic/otolaryngologic clinic from 2008 to 2013. Data were collected with respect to age, gender, clinical presentation, C-ANCA titers, Lund-Mackay (LM) scores, surgical interventions, and healthcare use, the latter of which were determined by assessing the number of rheumatology/otolaryngology clinic visits, computed tomography (CT) scans, and email/telephone encounters. A total of 44 patients were identified, 11 male and 33 female. Sinonasal manifestations were evident in 70.4%, with chronic rhinosinusitis (CRS) (41.9%), septal perforation (38.7%), and crusting (32.2%) the most common findings. No significant differences in number of CT scans (p = 0.10) or mean LM scores (p = 0.47) were found between patients with more than or equal to 1:80 and less than 1:80 C-ANCA titers, respectively. However, overall healthcare use was increased in the more than or equal to 1:80 C-ANCA group (n = 28) compared with less than 1:80 (n = 16), with a significantly greater number of rheumatologic/otolaryngologic encounters (mean 121 versus 69.2, p = 0.03) noted. When otolaryngologic healthcare use was specifically examined, the average number of encounters was also higher in more than or equal to 1:80 C-ANCA patients (31.9 versus 22.9), but this difference was not statistically significant (p = 0.16). Sinonasal GPA patients with presenting C-ANCA titers more than or equal to 1:80 demonstrated significantly greater overall healthcare use than their lower C

  9. Revealing Rembrandt

    Directory of Open Access Journals (Sweden)

    Andrew J Parker

    2014-04-01

    Full Text Available The power and significance of artwork in shaping human cognition is self-evident. The starting point for our empirical investigations is the view that the task of neuroscience is to integrate itself with other forms of knowledge, rather than to seek to supplant them. In our recent work, we examined a particular aspect of the appreciation of artwork using present-day functional magnetic resonance imaging (fMRI. Our results emphasised the continuity between viewing artwork and other human cognitive activities. We also showed that appreciation of a particular aspect of artwork, namely authenticity, depends upon the co-ordinated activity between the brain regions involved in multiple decision making and those responsible for processing visual information. The findings about brain function probably have no specific consequences for understanding how people respond to the art of Rembrandt in comparison with their response to other artworks. However, the use of images of Rembrandt’s portraits, his most intimate and personal works, clearly had a significant impact upon our viewers, even though they have been spatially confined to the interior of an MRI scanner at the time of viewing. Neuroscientific studies of humans viewing artwork have the capacity to reveal the diversity of human cognitive responses that may be induced by external advice or context as people view artwork in a variety of frameworks and settings.

  10. Fluoxetin-induced pulmonary granulomatosis.

    Science.gov (United States)

    de Kerviler, E; Trédaniel, J; Revlon, G; Groussard, O; Zalcman, G; Ortoli, J M; Espié, M; Hirsch, A; Frija, J

    1996-03-01

    A patient treated with fluoxetin for a manic depressive disorder developed pulmonary inflammatory nodules with noncaseating giant cell granulomas, interstitial pneumonia and non-necrotizing vasculitis, whilst remaining asymptomatic. A progressive resolution of pulmonary nodules occurred after withdrawal of the offending agent, and the chest radiograph returned to normal in 9 months. The diagnosis was assessed by an open lung biopsy.

  11. Pulmonary vasculitis.

    Science.gov (United States)

    Lally, Lindsay; Spiera, Robert F

    2015-05-01

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

  12. A abordagem cognitiva da metáfora em perspectiva pragmática nos séculos dezoito e dezenove: as contribuições de Lambert e Wegener a discussões atuais

    Directory of Open Access Journals (Sweden)

    Ulrike Agathe Schröder

    2014-01-01

    Full Text Available Desde o surgimento da Teoria Conceptual da Metáfora, há mais de trinta anos, houve uma série de autores que apontaram a falta de contextualização historiográfica, uma vez que a maioria das hipóteses sobre a metáfora conceptual e até boa parte dos exemplos já são antecipadas desde o século dezessete em reflexões filosóficas, antropológicas, psicológicas e linguísticas. O presente trabalho pretende apresentar dois pensadores dos séculos dezoito e dezenove, a saber, o filósofo Johann Heinrich Lambert (1965 e o linguista Philipp Wegener (1991, que não apenas já desenvolvem uma teoria cognitiva da metáfora cotidiana, como também já conseguem integrar aspectos pragmáticos, que atualmente são redescobertos como fatores descuidados pela primeira geração da Linguística Cognitiva. Sendo assim, as duas concepções podem lançar uma ponte a discussões atuais, uma vez que ambas conseguem apontar caminhos para superar tanto o abismo entre o sujeito solipsista e a sociedade, como a dicotomia entre universalismo e relatividade cultural. Os dois autores entendem a metáfora como fenômeno cognitivo e, ao mesmo tempo, intersubjetivo, por ser negociado entre os participantes em situações comunicativas concretas.

  13. Fatal inflammatory hypophysitis.

    Science.gov (United States)

    McIntyre, Elizabeth A; Perros, Petros

    2007-01-01

    A young female patient presented as an acute medical emergency with hypoglycaemia. Investigations revealed panhypopituitarism and an inflammatory pituitary mass. An antibody screen was negative for anti-neutrophil cytoplasmic antibodies with cytoplasmic distribution (cANCA). Pituitary histology showed lymphocytic infiltration and a few Langerhan's cells. The pituitary mass rapidly expanded to involve the optic nerves and led to bilateral blindness. Later, the patient developed diarrhoea, a vasculitis rash, scleritis, and proteinuria. In subsequent investigations cANCA became positive. The patient responded to steroids and cyclophosphamide treatment and remained in partial remission for six months before dying of severe sepsis. This is the first description of Wegener's granulomatosis presenting with acute anterior pituitary failure in the absence of other organ involvement and negative serology.

  14. 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.

  15. 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 polyangiit

  16. Autologous Peripheral Blood Stem Cell Transplantation in Patients With Life Threatening Autoimmune Diseases

    Science.gov (United States)

    2005-06-23

    Purpura, Schoenlein-Henoch; Graft Versus Host Disease; Anemia, Hemolytic, Autoimmune; Rheumatoid Arthritis; Churg-Strauss Syndrome; Hypersensitivity Vasculitis; Wegener's Granulomatosis; Systemic Lupus Erythematosus; Giant Cell Arteritis; Pure Red Cell Aplasia; Juvenile Rheumatoid Arthritis; Polyarteritis Nodosa; Autoimmune Thrombocytopenic Purpura; Takayasu Arteritis

  17. The value of serial ANCA testing during follow-up studies in patients with ANCA-associated vasculitides. A review

    NARCIS (Netherlands)

    Tervaert, JWC

    1996-01-01

    Anti-neutrophil cytoplasmic autoantibodies (ANCA) have specificity for cytoplasmic proteins of myeloid cells. Many antigens recognized by ANCA have already been described. In most patients with primary forms of vasculitis such as Wegener's granulomatosis (WG), the Churg-Strauss Syndrome (CSS) and mi

  18. 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

  19. Prognosis of microscopic polyangiitis with renal involvement: report of 60 Chinese patients

    Institute of Scientific and Technical Information of China (English)

    HU Wei-xin; LIU Zhi-hong; LIU Chun-bei; TANG Zheng; WANG Qing-wen; CHEN Hui-ping; LI Lei-shi

    2005-01-01

    @@ With the widespread clinical application of renal biopsy and serum antineutrophil cytoplasmic antibody (ANCA) testing, the percentage of renal injuries caused by systemic small vessel vasculitis (SVV) including microscopic polyangiitis (MPA)and Wegener's granulomatosis (WG) is on the rise in China.

  20. Granuloma formation in ANCA-associated vasculitides

    NARCIS (Netherlands)

    Lamprecht, Peter; Wieczorek, Stefan; Epplen, Jorg T.; Ambrosch, Petra; Kallenberg, Cees G. M.

    2009-01-01

    Granuloma formation is a key pathologic finding in two of the anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitides: Wegener's granulomatosis (WG) and Churg-Strauss syndrome (CSS). So far, no animal models have been established convincingly reproducing both vasculitic and granuloma

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

    Science.gov (United States)

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

    2000-05-01

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

  2. Correlação anátomo-clínica: granulomatose sistêmica por talco em drogadicto hiv negativo Systemic talc granulomatosis in a HIV-negative intravenous drug addict

    Directory of Open Access Journals (Sweden)

    D.F. da Cunha

    1999-07-01

    Full Text Available É descrita a correlação anatomopatológica de um paciente usuário de drogas injetáveis com talcose sistêmica acometendo pulmões, pleuras, fígado, baço e linfonodos e que desenvolveu hipertensão arterial pulmonar e cor pulmonale. Destaca-se o fato de que apesar do uso crônico de drogas injetáveis e outros comportamentos de alto risco para infecção pelo retrovírus, o paciente era HIV negativo, não apresentava linfocitopenia, nem alterações histopatológicas compatíveis com AIDS à necropsia.Anatomo-pathological correlation in a case of systemic talc granulomatosis affecting lungs, pleura, liver, spleen and mesenteric lymph nodes resulting in pulmonary arterial hypertension and cor pulmonale is described. The patient, a 26-year-old male HIV-negative intravenous drug addict had no lymphopenia or any histopathologic findings at necroscopy compatible with AIDS, despite of a chronic high-risk behavior favoring this illness.

  3. 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

  4. Stijging van het aantal operaties wegens stressincontinentie

    NARCIS (Netherlands)

    Vierhout, M.E.

    2005-01-01

    Since the introduction of the minimally invasive tension-free vaginal tape (TVT) the number of operations performed for treatment of stress urinary incontinence has increased dramatically from over 1600 in 1999 to more than 4200 in 2003. Both gynaecologists and urologists now perform more TVTs and f

  5. Pulmonary lymphomatoid granulomatosis: an immunohistochemical and gene rearrangement study%肺淋巴瘤样肉芽肿病的免疫表型及基因重排研究

    Institute of Scientific and Technical Information of China (English)

    冯瑞娥; 刘鸿瑞; 刘彤华; 陈杰; 凌庆; 师晓华; 钟定荣; 罗玉风; 曹金伶

    2011-01-01

    目的 观察肺淋巴瘤样肉芽肿病的细胞组成、免疫表型和分子生物学改变.方法 回顾性分析北京协和医院9例肺淋巴瘤样肉芽肿病患者的临床病理情况.其中5例为开胸肺活检标本,3例为肺叶切除标本,1例尸检.标本经4%甲醛固定,石蜡包埋,常规切片,HE染色.免疫组织化学EnVision法染色(抗体包括CD20、CD3、CD56),原位杂交检测EB病毒,采用聚合酶链反应进行Ig和TCR基因重排检测.结果 9例肺淋巴瘤样肉芽肿患者,年龄3~59岁,男∶女=3:6.9例患者肺组织内病变分布均显示以血管为中心的淋巴细胞浸润为特点.免疫组织化学显示以CD3阳性的T淋巴细胞占绝对优势,散在不等数量的CD20阳性的B细胞,CD56均为阴性.8例行EB病毒原位杂交,4例阳性细胞数20%,1例为15%.按照WHO的3级分级方法,Ⅰ级为4例,Ⅱ级1例,Ⅲ级4例.6例行基因重排检测,3例显示有Ig基因重排阳性,其中,1例为Ⅱ级病变,2例为Ⅲ级病变.6例TCR重排检测均为阴性.随访时间0.5~6.5年不等,9例患者中3例死亡,2例存活,4例失访.结论 部分肺淋巴瘤样肉芽肿病,特别是Ⅱ、Ⅲ级患者,有B淋巴细胞克隆性增生,提示其为淋巴瘤性病变.%Objective To study the immunophenotype and gene rearrangement pattern of pulmonary lymphomatoid granulomatosis. Methods Nine cases of pulmonary lymphomatoid granulomatosis, included 5 cases of open lung biopsy, 3 cases of lobectomy specimen and 1 case of autopsy, were retrospectively analyzed by immunohistochemistry, in-situ hybridization for Epstein-Barr virus-encoded RNA, immunoglobulin and T-cell receptor gene rearrangement studies. Results The Histologically, all cases showed lymphocytic infiltration surrounding the blood vessels and in the perivascular areas. Most of these lymphoid cells expressed T-cell marker CD3. There were also variable numbers of CD20-positive B cells. The staining for CD56 was negative. According to the WHO

  6. Ocular manifestations of granulomatosis with polyangiitis: an analysis of 164 patients%肉芽肿性多血管炎患者眼部表现临床分析

    Institute of Scientific and Technical Information of China (English)

    陈迪; 闵寒毅; 赵久良; 曾小峰

    2016-01-01

    Objective To review the ophthalmic features of granulomatosis with polyangiitis (GPA).Methods Clinical data of 164 GPA patients diagnosed in Peking Union Medical College Hospital during the past 10 years were retrospectively reviewed.Results Among 164 patients,48 (29.3%) had ocular involvement with a male to female ratio of 1.4:1 and an average age of (45 ± 15) years.Nine patients (5.5%) were first diagnosed at the ophthalmology department.Scleritis (15.9%,26/164),orbit lesions (13.4%,22/164) and peripheral corneal ulcer (9.1%,15/164) were the three most common ophthalmic complications.Twenty-two patients (13.4%) had positive findings in the orbit imaging.Six patients (3.7%,6/164) lost their vision due to GPA.Conclusion The ocular manifestations of GPA are diverse and complicated.GPA may affect every component of the eye,sclera,orbit and cornea are the most frequently affected sites of the eye.%目的 分析肉芽肿性多血管炎(GPA)眼部临床表现的特点.方法 回顾性分析2005年8月至2015年8月北京协和医院确诊的164例GPA患者的眼科临床资料.结果 164例患者中眼部受累者48例(29.3%),男女比例为1.4:1,平均年龄(45±15)岁;首诊于眼科者9例(5.5%);巩膜炎、眼眶病变和边缘性角膜溃疡是最常见的GPA眼部表现,分别为26例(15.9%)、22例(13.4%)、15例(9.1%);眼眶MRI检查阳性者22例(13.4%).GPA致盲率3.7% (6/164).结论 GPA眼部表现复杂多样,可侵犯眼部所有组织,巩膜、眼眶和角膜是其最常累及的部位.

  7. CASO -2016: Sindrome de Churg Strauss: a propósito de dos casos en el Hospital San Vicente de Paúl

    OpenAIRE

    Hu Liang, Huixian; Leitón Chaves, Andrés; Salas Mena, Claudio

    2016-01-01

    Las vasculitis asociadas a anticuerpos citoplasmá-ticos antineutrófilos, clasificadas como vasculitis de pequeños vasos incluyen: granulomatosis con poliangeítis (enfermedad de Wegener), poliangeítis microscópica y la granulomatosis eosinofilica con poliangeítis (GEPA) (Churg Strauss). La enferme-dad de Churg Strauss es una de las vasculitis necro-tizantes sistémicas más raras, pero potencialmente mortal. La incidencia internacional de la enferme-dad es de aproximadamente 2.5 casos por cada 1...

  8. Heat reveals faults

    Energy Technology Data Exchange (ETDEWEB)

    Weinreich, Bernhard [Solarschmiede GmbH, Muenchen (Germany). Engineering Dept.

    2010-07-01

    Gremlins cannot hide from the all-revealing view of a thermographic camera, whereby it makes no difference whether it is a roof-mounted system or a megawatt-sized farm. Just as diverse are the range of faults that, with the growing level of expertise, can now be detected and differentiated with even greater detail. (orig.)

  9. TypeScript revealed

    CERN Document Server

    Maharry, Dan

    2013-01-01

    TypeScript Revealed is a quick 100-page guide to Anders Hejlsberg's new take on JavaScript. With this brief, fast-paced introduction to TypeScript, .NET, Web and Windows 8 application developers who are already familiar with JavaScript will easily get up to speed with TypeScript and decide whether or not to start incorporating it into their own development. TypeScript is 'JavaScript for Application-scale development'; a superset of JavaScript that brings to it an additional object-oriented-like syntax familiar to .NET programmers that compiles down into simple, clean JavaScript that any browse

  10. Cavitary pulmonary nodules in atypical collagen disease and lupoid drug reaction. Report of two cases

    Energy Technology Data Exchange (ETDEWEB)

    Muren, C.; Strandberg, O.

    The case histories of two patients with cavitary pulmonary nodules and the findings at chest radiography are reviewed. The first patient had a connective tissue disease with features common to systematic lupus erythematosus and Wegener's granulomatosis. In the second patient the lung changes developed as part of a drug reaction to carbamezapine and/or phenytoin. The common denominator of the cavitating nodules is probably the presence of granulomas, developing as a sequela of pulmonary vasculitis. (orig.).

  11. Bilateral scleritis and sclerokeratitis associated with IgA nephropathy

    OpenAIRE

    Garza-Leon, Manuel; Flores, Diana; Alarcón-Galván, Gabriela; Sánchez-Martínez, Concepción

    2012-01-01

    Purpose The purpose of this study is to report a case of bilateral nodular scleritis in a patient with final diagnosis of IgA nephropathy. Methods This is an observational case report. Results A male patient, 42 years old, presented with a bilateral nodular scleritis and OD sclerokeratitis. He had a previous history of acute otitis media and developed posterior renal failure and arterial hypertension. Clinical and systemic findings suggest Wegener's granulomatosis. A kidney biopsy was perform...

  12. [Vasculitis : EULAR/ACR guidelines with respect to the clinical cardiological routine].

    Science.gov (United States)

    Maisch, B

    2015-02-01

    In this article the diagnostics, differential diagnosis, laboratory findings, multimodal imaging and treatment of vasculitis of small, medium and large vessels as well as granulomatous and eosinophilic vascular diseases are described in the context of previous and current European League against Rheumatism (EULAR) and American College of Rheumatology (ACR) recommendations. Vasculitis is a syndrome which is part of various clinical disease entities, such as Wegener's granulomatosis, polyangiitis, Churg-Strauss syndrome, polyarteritis nodosa, cryoglobulinemia and other forms of vasculitis.

  13. Revealing the programming process

    DEFF Research Database (Denmark)

    Bennedsen, Jens; Caspersen, Michael Edelgaard

    2005-01-01

    One of the most important goals of an introductory programming course is that the students learn a systematic approach to the development of computer programs. Revealing the programming process is an important part of this; however, textbooks do not address the issue -- probably because the textb......One of the most important goals of an introductory programming course is that the students learn a systematic approach to the development of computer programs. Revealing the programming process is an important part of this; however, textbooks do not address the issue -- probably because...... the textbook medium is static and therefore ill-suited to expose the process of programming. We have found that process recordings in the form of captured narrated programming sessions are a simple, cheap, and efficient way of providing the revelation.We identify seven different elements of the programming...... process for which process recordings are a valuable communication media in order to enhance the learning process. Student feedback indicates both high learning outcome and superior learning potential compared to traditional classroom teaching....

  14. Android Emotions Revealed

    DEFF Research Database (Denmark)

    Vlachos, Evgenios; Schärfe, Henrik

    2012-01-01

    This work presents a method for designing facial interfaces for sociable android robots with respect to the fundamental rules of human affect expression. Extending the work of Paul Ekman towards a robotic direction, we follow the judgment-based approach for evaluating facial expressions to test...... in which case an android robot like the Geminoid|DK –a duplicate of an Original person- reveals emotions convincingly; when following an empirical perspective, or when following a theoretical one. The methodology includes the processes of acquiring the empirical data, and gathering feedback on them. Our...... findings are based on the results derived from a number of judgments, and suggest that before programming the facial expressions of a Geminoid, the Original should pass through the proposed procedure. According to our recommendations, the facial expressions of an android should be tested by judges, even...

  15. Android Emotions Revealed

    DEFF Research Database (Denmark)

    Vlachos, Evgenios; Schärfe, Henrik

    2012-01-01

    This work presents a method for designing facial interfaces for sociable android robots with respect to the fundamental rules of human affect expression. Extending the work of Paul Ekman towards a robotic direction, we follow the judgment-based approach for evaluating facial expressions to test...... in which case an android robot like the Geminoid|DK –a duplicate of an Original person- reveals emotions convincingly; when following an empirical perspective, or when following a theoretical one. The methodology includes the processes of acquiring the empirical data, and gathering feedback on them. Our...... findings are based on the results derived from a number of judgments, and suggest that before programming the facial expressions of a Geminoid, the Original should pass through the proposed procedure. According to our recommendations, the facial expressions of an android should be tested by judges, even...

  16. Chemokine receptor co-expression reveals aberrantly distributed T-H effector memory cells in GPA patients

    NARCIS (Netherlands)

    Lintermans, Lucas L.; Rutgers, Abraham; Stegeman, Coen A.; Heeringa, Peter; Abdulahad, Wayel H.

    2017-01-01

    Background: Persistent expansion of circulating CD4(+) effector memory T cells (TEM) in patients with granulomatosis with polyangiitis (GPA) suggests their fundamental role in disease pathogenesis. Recent studies have shown that distinct functional CD4(+) TEM cell subsets can be identified based on

  17. [Vasculitis: New nomenclature of the Chapel Hill consensus conference 2012].

    Science.gov (United States)

    Holl-Ulrich, K

    2014-11-01

    Within the last years, many advances have been made in the understanding of the etiopathology of vasculitis as well as of different disease courses. The revised 2012 Chapel Hill consensus conference (CHCC) nomenclature reflects current knowledge on the etiopathology in addition to the descriptive principles of vessel size and types of inflammation. The anti-neutrophil cytoplasmic antibody (ANCA)-associated forms of vasculitis have been separated as a group, as opposed to immune complex small vessel vasculitis. When consensus was achieved eponyms have been replaced by systematic names, such as granulomatosis with polyangiitis (Wegener's granulomatosis) or eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome). Moreover, clinically important but less well-known types of vasculitis have now been included in the CHCC nomenclature. This article presents the changes and summarizes the results of important new articles on the clinical picture and morphology of vasculitis.

  18. Puerto Rico Revealed Preference data

    Data.gov (United States)

    National Oceanic and Atmospheric Administration, Department of Commerce — Revealed preference models provide insights into recreational angler behavior and the economic value of recreational fishing trips. Revealed preference data is...

  19. Revealing the Beast Within

    Science.gov (United States)

    2003-07-01

    Deeply Embedded Massive Stellar Clusters Discovered in Milky Way Powerhouse Summary Peering into a giant molecular cloud in the Milky Way galaxy - known as W49 - astronomers from the European Southern Observatory (ESO) have discovered a whole new population of very massive newborn stars . This research is being presented today at the International Astronomical Union's 25th General Assembly held in Sydney, Australia, by ESO-scientist João Alves. With the help of infrared images obtained during a period of excellent observing conditions with the ESO 3.5-m New Technology Telescope (NTT) at the La Silla Observatory (Chile), the astronomers looked deep into this molecular cloud and discovered four massive stellar clusters, with hot and energetic stars as massive as 120 solar masses. The exceedingly strong radiation from the stars in the largest of these clusters is "powering" a 20 light-year diameter region of mostly ionized hydrogen gas (a "giant HII region"). W49 is one of the most energetic regions of star formation in the Milky Way. With the present discovery, the true sources of the enormous energy have now been revealed for the first time, finally bringing to an end some decades of astronomical speculations and hypotheses. PR Photo 21a/03 : Colour Composite of W49A (NTT+SOFI). PR Photo 21b/03 : Radio and Near-Infrared Composite of W49A Giant molecular clouds Stars form predominantly inside Giant Molecular Clouds which populate our Galaxy, the Milky Way. One of the most prominent of these is W49 , which has a mass of a million solar masses. It is located some 37,000 light-years away and is the most luminous star-forming region known in our home galaxy: its luminosity is several million times the luminosity of our Sun. A smaller region within this cloud is denoted W49A - this is one of the strongest radio-emitting areas known in the Galaxy . Massive stars are excessive in all ways. Compared to their smaller and ligther brethren, they form at an Olympic speed and

  20. Chemokine receptor co-expression reveals aberrantly distributed TH effector memory cells in GPA patients.

    Science.gov (United States)

    Lintermans, Lucas L; Rutgers, Abraham; Stegeman, Coen A; Heeringa, Peter; Abdulahad, Wayel H

    2017-06-14

    Persistent expansion of circulating CD4(+) effector memory T cells (TEM) in patients with granulomatosis with polyangiitis (GPA) suggests their fundamental role in disease pathogenesis. Recent studies have shown that distinct functional CD4(+) TEM cell subsets can be identified based on expression patterns of chemokine receptors. The current study aimed to determine different CD4(+) TEM cell subsets based on chemokine receptor expression in peripheral blood of GPA patients. Identification of particular circulating CD4(+) TEM cells subsets may reveal distinct contributions of specific CD4(+) TEM subsets to the disease pathogenesis in GPA. Peripheral blood of 63 GPA patients in remission and 42 age- and sex-matched healthy controls was stained immediately after blood withdrawal with fluorochrome-conjugated antibodies for cell surface markers (CD3, CD4, CD45RO) and chemokine receptors (CCR4, CCR6, CCR7, CRTh2, CXCR3) followed by flow cytometry analysis. CD4(+) TEM memory cells (CD3(+)CD4(+)CD45RO(+)CCR7(-)) were gated, and the expression patterns of chemokine receptors CXCR3(+)CCR4(-)CCR6(-)CRTh2(-), CXCR3(-)CCR4(+)CCR6(-)CRTh2(+), CXCR3(-)CCR4(+)CCR6(+)CRTh2(-), and CXCR3(+)CCR4(-)CCR6(+)CRTh2(-) were used to distinguish TEM1, TEM2, TEM17, and TEM17.1 cells, respectively. The percentage of CD4(+) TEM cells was significantly increased in GPA patients in remission compared to HCs. Chemokine receptor co-expression analysis within the CD4(+) TEM cell population demonstrated a significant increase in the proportion of TEM17 cells with a concomitant significant decrease in the TEM1 cells in GPA patients compared to HC. The percentage of TEM17 cells correlated negatively with TEM1 cells in GPA patients. Moreover, the circulating proportion of TEM17 cells showed a positive correlation with the number of organs involved and an association with the tendency to relapse in GPA patients. Interestingly, the aberrant distribution of TEM1 and TEM17 cells is modulated in CMV

  1. Granulomatosis sarcoidea necrosante con compromiso sistémico Necrotizing sarcoid granulomatosis with systemic involvement

    Directory of Open Access Journals (Sweden)

    María Elena Ceballos

    2006-09-01

    Full Text Available Mujer mapuche de 70 años quien en el curso de 1 año presentó baja de peso, ojo rojo bilateral y úlcera corneal que evolucionó hacia la perforación con herniación uveal y pérdida de la visión. Posteriormente se agregaron lesiones nodulares, violáceas, sensibles, algunas ulceradas en las zonas distales de ambas extremidades inferiores. La TAC de tórax demostró múltiples nódulos pulmonares. Tras un estudio exhaustivo se descartaron la tuberculosis y otras infecciones. Tanto la biopsia de las lesiones cutáneas como la biopsia pulmonar obtenida mediante toracotomía confirmaron la presencia de granulomas necrotizantes no caseificantes y arteritis granulomatosa. Se trató con esteroides y ciclofosfamida con mejoría de su condición general y regresión de las lesiones cutáneas y pulmonares en menos de 1 mes. Se realizó un trasplante de cornea. A los seis meses de seguimiento continúa en tratamiento, no ha presentado recaídas y tiene recuperación parcial de la visiónA 70-year-old mapuche female presented with a 1 year history of weight loss, bilateral red painful eyes and corneal ulcer that evolved into perforation and uveal herniation with complete visual loss. Reddish and painful nodules appeared on the distal aspect of both lower extremities, that ulcerated after months. A thoracic CT scan showed multiple pulmonary nodules. Infectious diseases were ruled out. An open lung biopsy and a skin biopsy were performed and both showed non-caseating necrotizing granulomas and granulomatous arteritis. Steroids and cyclophosphamide were started with complete regression of skin and pulmonary lesions within a month. Corneal transplantation was done. After 6 months follow-up, the patient is on low-dose of steroids, free of disease with partial vision recovery

  2. Bouwmaterialen paalmatrassystemen: Delft Cluster Blijvend Vlakke Wegen, WP3

    NARCIS (Netherlands)

    Van der Meij, R.; Van Ruijven, J.

    2007-01-01

    Dit rapport betreft een literatuurstudie naar bouwmaterialen die in paalmatrassystemen worden gebruikt. De volgende aspecten komen aan bod: • Materialen voor paalmatrassystemen (palen, geotextielen, matrasmateriaal) • Hiaten in kennis (op basis van interviews) Door middel van deze literatuurstudie e

  3. Verkeersonveiligheid op rijkswegen en belangrijke 80 km/uur-wegen.

    NARCIS (Netherlands)

    Janssen, S.T.M.C.

    1993-01-01

    In this project, experience was gained with the collection, processing and presentation of data with respect to road hazard on so-called 'A' and 'N' roads (motorways and arterial rural roads). Parts of the A and N road networks in the Netherlands were charted for three provinces. A distinction was m

  4. 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; an

  5. Bruggen en wegen naar een duurzame Randstad in 2040

    NARCIS (Netherlands)

    d'Haese, N.

    2008-01-01

    Dit rapport werd geschreven door het Dutch Research Institute for Transitions in opdracht van Delft Cluster. Het rapport kadert binnen het PIZZA-project, een samenwerking tussen 6 Bsik-programma’s van de 8 voor ruimte en KSI. Het doel van dit project is een methodiek voor transitiemonitoring te ontw

  6. Decision Making and Revealed Preference

    DEFF Research Database (Denmark)

    de la Rosa, Leonidas Enrique

    If our decision-making processes are to some extent shaped by evolutionary pressures and our environment is different from that to which we adapted, some of our choices will not be in our best interest. But revealed preference is the only tool that we have so far to conduct a normative analysis...

  7. Revealed preference with limited consideration

    NARCIS (Netherlands)

    Demuynck, T.; Seel, C.

    2014-01-01

    We derive revealed preference tests for models where individuals use consideration sets to simplify their consumption problem. Our basic test provides necessary and sufficient conditions for consistency of observed choices with the existence of consideration set restrictions. The same conditions can

  8. [Endobronchial hamartoma revealed by hemoptysis].

    Science.gov (United States)

    Smati, Belhassen; Boudaya, Mohamed Sadok; Mestiri, Taher; Djilani, Habiba; Mezni, Faouzi; Kilani, Tarek

    2005-05-01

    Hamartoma is the most frequent benign tumor of the lung. Its endo bronchial location is rare. We report two cases of endo bronchial hamartoma occurring in 2 men aged 68 and 60 years respectively. The two cases were revealed by hemoptysis. Bronchial fibroscopy showed a bud respectively in the left stump and in the lower left bronchus. Treatment consisted in a pneumonectomy and a lower lobectomy. A histological examination confirmed the diagnosis of endo bronchial hamartoma. Diagnosis of endobronchial hamartoma before surgery is difficult. Pulmonary resections are often necessary because of parenchyma lelions caused bronchial obstruction.

  9. Transparency masters for mathematics revealed

    CERN Document Server

    Berman, Elizabeth

    1980-01-01

    Transparency Masters for Mathematics Revealed focuses on master diagrams that can be used for transparencies for an overhead projector or duplicator masters for worksheets. The book offers information on a compilation of master diagrams prepared by John R. Stafford, Jr., audiovisual supervisor at the University of Missouri at Kansas City. Some of the transparencies are designed to be shown horizontally. The initial three masters are number lines and grids that can be used in a mathematics course, while the others are adaptations of text figures which are slightly altered in some instances. The

  10. Revealing ontological commitments by magic.

    Science.gov (United States)

    Griffiths, Thomas L

    2015-03-01

    Considering the appeal of different magical transformations exposes some systematic asymmetries. For example, it is more interesting to transform a vase into a rose than a rose into a vase. An experiment in which people judged how interesting they found different magic tricks showed that these asymmetries reflect the direction a transformation moves in an ontological hierarchy: transformations in the direction of animacy and intelligence are favored over the opposite. A second and third experiment demonstrated that judgments of the plausibility of machines that perform the same transformations do not show the same asymmetries, but judgments of the interestingness of such machines do. A formal argument relates this sense of interestingness to evidence for an alternative to our current physical theory, with magic tricks being a particularly pure source of such evidence. These results suggest that people's intuitions about magic tricks can reveal the ontological commitments that underlie human cognition. Copyright © 2014 Elsevier B.V. All rights reserved.

  11. Plan competitions reveal entrepreneurial talent

    Energy Technology Data Exchange (ETDEWEB)

    Madison, Alison L.

    2011-05-15

    Monthly economic diversity column for Tri-City Herald business section. Excerpt below: There’s something to be said for gaining valuable real-world experience in a structured, nurturing environment. Take for instance learning to scuba dive in the comfort of my resort pool rather than immediately hanging out with sharks while I figure out little things like oxygen tanks and avoiding underwater panic attacks. Likewise, graduate students are getting some excellent, supportive real-world training through university business plan competitions. These competitions are places where smart minds, new technologies, months of preparation and coaching, and some healthy pre-presentation jitters collide to reveal not only solid new business ideas, but also some promising entrepreneurial talent. In fact, professionals from around our region descend upon college campuses every spring to judge these events, which help to bridge the gap between academics and the real technology and business-driven economy.

  12. [Vasculitis. New nomenclature of the Chapel Hill consensus conference 2012].

    Science.gov (United States)

    Holl-Ulrich, K

    2013-11-01

    In recent years, many advances have been made in our understanding of vasculitis etiopathology as well as of different disease courses. The revised Chapel Hill Consensus Conference (CHCC) 2012 nomenclature reflects current knowledge about etiopathology, in addition to the descriptive principles of vessel size and type of inflammation. Anti-neutrophil cyptoplasmic antibody (ANCA)-associated vasculitides have been classified as a separate group, as opposed to immune complex small vessel vasculitis. In cases where consensus was achieved, eponyms have been replaced by systematic names, such as granulomatosis with polyangiitis (Wegener's) or eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome). Moreover, clinically important but less well-known types of vasculitis have now been included in the CHCC nomenclature. This article presents the changes, focussing on those types that are relevant to the histopathologist, and summarizes the results of important new articles on morphology and clinical picture of vasculitis.

  13. Diffuse abnormalities of the trachea: computed tomography findings

    Energy Technology Data Exchange (ETDEWEB)

    Marchiori, Edson [Universidade Federal Fluminense (UFF), Niteroi, RJ (Brazil). Dept. de Radiologia]. E-mail: edmarchiori@gmail.com; Pozes, Aline Serfaty [Universidade Federal do Rio de Janeiro (UFRJ), RJ (Brazil). Faculdade de Medicina. Dept. de Radiologia; Souza Junior, Arthur Soares [Faculdade de Medicina de Sao Jose do Rio Preto (FAMERP), SP (Brazil). Escola de Medicina. Dept. de Radiologia; Escuissato, Dante Luiz [Universidade Federal do Parana (UFPR), Curitiba, PR (Brazil). Dept. de Radiologia; Irion, Klaus Loureiro [The Cardiothoracic Centre NHS Trust, Liverpool (United Kingdom); Araujo Neto, Cesar de [Universidade Federal da Bahia (UFBA), Salvador, BA (Brazil). Dept. de Radiologia; Barillo, Jorge Luiz; Zanetti, Glaucia [Faculdade de Medicina de Petropolis (FMP), RJ (Brazil). Dept. de Cirurgia Clinica; Souza, Carolina Althoff [University of Ottawa, Ottawa, ON (Canada)

    2008-01-15

    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)

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

    Directory of Open Access Journals (Sweden)

    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.

  15. Etiologic diagnosis of jaw osteonecrosis, other than bisphosphonate and radiotherapy related osteonecrosis.

    Science.gov (United States)

    Magremanne, M; Picheca, S; Reychler, H

    2014-02-01

    Our purpose was to highlight the various etiologies of maxillo-mandibular osteonecrosis, other than radiotherapy and biphosphonate related osteitis that have been abundantly reported. We performed a PubMed search from August 1, 1972 to August 1, 2012 using the following MeSH terms: "osteonecrosis", "bone", "necrosis", "jaw", "maxilla", "mandible", "palate", "oral", "avascular necrosis", NOT "bisphosphonate" NOT "osteoradionecrosis". Most cases of osteonecrosis were iatrogenic. Viral, mycotic, or bacterial infections were less frequent causes. Cocaine abuse, Wegener's granulomatosis, and N/K lymphoma were other etiologies. It is important to identify the various etiologies rapidly to manage this sometimes very mutilating condition adequately.

  16. [Etiologic diagnosis of jaw osteonecrosis, other than bisphosphonate and radiotherapy related osteitis].

    Science.gov (United States)

    Magremanne, M; Picheca, S; Reychler, H

    2014-02-01

    Our purpose was to highlight the various etiologies of maxillo-mandibular osteonecrosis, other than radiotherapy and biphosphonate related osteitis that have been abundantly reported. We performed a PubMed search from August 1, 1972 to August 1, 2012 using the following MeSH terms: "osteonecrosis", "bone", "necrosis", "jaw", "maxilla", "mandible", "palate", "oral", "avascular necrosis", NOT "bisphosphonate" NOT "osteoradionecrosis". Most cases of osteonecrosis were iatrogenic. Viral, mycotic, or bacterial infections were less frequent causes. Cocaine abuse, Wegener's granulomatosis, and N/K lymphoma were other etiologies. It is important to identify the various etiologies rapidly to manage this sometimes very mutilating condition adequately.

  17. Focus groups reveal consumer ambivalence.

    Science.gov (United States)

    1983-01-01

    According to qualitative research, Salvadoreans are ambivalent about the use of contraceptives. Since complete responsibility for management of the CSM project was accepted by the Association Demografica Salvadorena (ADS), the agency which operates the contraceptive social marketing project in El Salvador, in November 1980, the need for decisions in such areas as product price increases, introduction of new condom brands, promotion of the vaginal foaming tablet, and assessment of product sales performance had arisen. The ICSMP funded market research, completed during 1983, was intended to provide the data on which such decisions by ADS could be based. The qualitative research involved 8 focus groups, comprised of men and women, aged 18-45, contraceptive users and nonusers, from the middle and lower socioeconomic strata of the city of San Salvador and other suburban areas. In each group a moderator led discussion of family planning and probed respondents for specific attitudes, knowledge, and behavior regarding the use of contraceptives. To assess attitudes at a more emotional level, moderators asked respondents to "draw" their ideas on certain issues. A marked discrepancy was revealed between respondents' intellectual responses to the issues raised in group discussion, as opposed to their feelings expressed in the drawings. Intellectually, participants responded very positively to family planning practice, but when they were asked to draw their perceptions, ambivalent feelings emerged. Drawings of both the user and the nonuser convey primarily negative aspects for either choice. The user is tense and moody toward her children; the nonuser loses her attractiveness and "dies." Figures also show drawings of some of the attitudes of single and married male participants. 1 drawing shows an incomplete and a complete circle, symbolizing a sterilized man (incomplete) and a nonsterilized man (complete). Another picture depicts a chained man who has lost his freedom

  18. 原发于中枢神经系统的淋巴瘤样肉芽肿:病例报告并文献分析%A clinicopathologic analysis of primary central nervous system lymphomatoid granulomatosis:case report and literature review

    Institute of Scientific and Technical Information of China (English)

    付永娟; 朴月善; 陈莉; 李存江; 卢德宏

    2012-01-01

    Objective To investigate the clinical, neuroimaging and histopathological features of primary central nervous system lymphomatoid granulomatosis (LG). Methods The clinical manifestation, neuroimaging, histopathological and biological features of a patient with primary central nervous system LG were presented, and the related literatures were reviewed. Results A 57-year-old male presented with memory impairment, weak in orientation, calculation, apprehension and judgment for 3 months. Magnetic resonance imaging (MRI) showed space-occupying lesions in bilateral frontal lobes, with TiWI isointensity and T2WI hyperintensity, and the enhancement was irregular. The lesion was slight expansive with yellow surface and gray-white section in color and soft texture and abundant blood supply. Microscopically, the lesion was characterized by angiocentric and angiodestructive lymphoproliferation, partly showed the structure of LG characterized by T cell predominant proliferation, macrophage infiltration, astrocyte activation, small vessel proliferation and hyalinization, and partly showed the structure of lymphoma characterized by diffuse atypical B cell proliferation, with IgK monoclonal production. Epstein-Barr virus (EBV) was negative. Conclusion As a precursor disease of lymphoma, LG should be considered in the differential diagnosis of both diffuse and multifocal lesions of the central nervous system. The relavance between primary central nervous system LG and EBV infection should be further discussed.%目的 探讨原发于中枢神经系统的淋巴瘤样肉芽肿的临床表现、影像学及病理学特点.方法 回顾分析一例原发于中枢神经系统的淋巴瘤样肉芽肿患者的临床及影像学表现、组织学及分子生物学特点,并复习相关文献.结果 男性患者,57岁.表现为记忆力减退3个月,地点定向障碍,计算力和理解判断力下降.影像学检查显示双侧额叶占位性病变,呈等T1、长T2信号,强化不均匀.

  19. Revealing conceptual understanding of international business

    NARCIS (Netherlands)

    Ashley, S.; Schaap, H.; Bruijn, E. de

    2016-01-01

    This study aims to identify an adequate approach for revealing conceptual understanding in higher professional education. Revealing students’ conceptual understanding is an important step towards developing effective curricula, assessment and aligned teaching strategies to enhance conceptual

  20. Revealing conceptual understanding of international business

    NARCIS (Netherlands)

    Ashley, S.M.; Schaap, H.; de Bruijn, E.

    2016-01-01

    This study aims to identify an adequate approach for revealing conceptual understanding in higher professional education. Revealing students’ conceptual understanding is an important step towards developing effective curricula, assessment and aligned teaching strategies to enhance conceptual underst

  1. Revealing conceptual understanding of international business

    NARCIS (Netherlands)

    Sue Ashley; Dr. Harmen Schaap; Prof.Dr. Elly de Bruijn

    2017-01-01

    This study aims to identify an adequate approach for revealing conceptual understanding in higher professional education. Revealing students’ conceptual understanding is an important step towards developing effective curricula, assessment and aligned teaching strategies to enhance conceptual

  2. Revealing conceptual understanding of international business

    NARCIS (Netherlands)

    Ashley, S.M.; Schaap, H.; de Bruijn, E.

    2016-01-01

    This study aims to identify an adequate approach for revealing conceptual understanding in higher professional education. Revealing students’ conceptual understanding is an important step towards developing effective curricula, assessment and aligned teaching strategies to enhance conceptual

  3. 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.

  4. Lymphomatoid granulomatosis of the brain: A case report

    Directory of Open Access Journals (Sweden)

    Edgardo Quinones

    2016-01-01

    Conclusions: This rare lesion in an adult, immunocompetent patient, debuting with motor seizures represents a challenge in terms of diagnosis and treatment. After surgical and medical treatment, the patient had a satisfactory recovery. Clinical features, imaging, differential diagnosis, and pathology are discussed.

  5. Diffuse Large B Cell Lymphoma Mimicking Granulomatosis with Polyangiitis

    Science.gov (United States)

    Horowitz, Netanel; Ben-Itzhak, Ofer; Braun-Moscovici, Yolanda

    2016-01-01

    In a patient with systemic multiorgan disease with overlapping features, the differential diagnosis included infectious diseases, malignancies, and systemic autoimmune or inflammatory diseases. We present an unusual case of a young male with B cell lymphoma who presented with symptoms mimicking systemic vasculitis and review the existing literature. PMID:27293945

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

    Science.gov (United States)

    ... Lotte Strauss as a syndrome consisting of “asthma, eosinophilia [an excessive number of eosinophils in the blood], ... phase of the disease is often marked by eosinophilia, the finding of an excessive number of eosinophils ...

  7. Diffuse Large B Cell Lymphoma Mimicking Granulomatosis with Polyangiitis

    Directory of Open Access Journals (Sweden)

    Mohammad E. Naffaa

    2016-01-01

    Full Text Available In a patient with systemic multiorgan disease with overlapping features, the differential diagnosis included infectious diseases, malignancies, and systemic autoimmune or inflammatory diseases. We present an unusual case of a young male with B cell lymphoma who presented with symptoms mimicking systemic vasculitis and review the existing literature.

  8. 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.

  9. Revealing Conceptual Understanding of International Business

    Science.gov (United States)

    Ashley, Sue; Schaap, Harmen; de Bruijn, Elly

    2017-01-01

    This study aims to identify an adequate approach for revealing conceptual understanding in higher professional education. Revealing students' conceptual understanding is an important step towards developing effective curricula, assessment and aligned teaching strategies to enhance conceptual understanding in higher education. Essays and concept…

  10. 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.

  11. Meningitis granulomatosa, glomerulonefritis rápidamente progresiva y vasculitis

    Directory of Open Access Journals (Sweden)

    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.

  12. [Safety and efficacy of biologics directed against TNF-alpha and CD20 in the therapy of vasculitis and systemic lupus erythematosus].

    Science.gov (United States)

    Walker, Ulrich A; Hasler, Paul

    2008-05-01

    While the inhibition of TNF-alpha has been shown to improve vasculitis in vitro and in animal models, the clinical evidence for the efficacy of TNF-alpha blockade in most forms of vasculitis is mainly based on case reports and case series. Randomised controlled studies have so far not shown superiority of anti-TNF-alpha agents for Wegener's granulomatosis and giant cell arteritis. Moreover, in the context of Wegener's granulomatosis, a higher frequency and severity of infections are to be expected. In refractory cases of Behçet's disease therapy of uveitis and other organ manifestations is promising. Rituximab has achieved good effects in case reports of vasculitis. Results from controlled trials are not available. Observational studies indicate that in refractory systemic lupus erythematosus, and possibly also in several instances of small vessel vasculitis, rituximab can achieve good responses. The increased frequency of severe infections under TNF-alpha blockade requires a stringent benefit and risk assessment in addition to a multidisciplinary analysis of follow-up parameters. A detailed information of the patient regarding symptoms and signs of a possible infection are a prerequisite. Due to the complexity of the field and the danger of morbidity and mortality as a consequence of vasculitis or systemic lupus erythematosus on the one hand, and of the therapy on the other, biologics should only be used to treat these disorders in institutions fully equipped and staffed for this purpose.

  13. 心脏韦格纳肉芽肿1例临床病理观察

    Institute of Scientific and Technical Information of China (English)

    姚庆端; 张金升; 于海易; 王敏; 曲美婷

    2004-01-01

    To investigates the clinical sings and the pathology morphologic changes of the heart tissue of a case with the cardiac Wegener' s granulomatosis. Methods The clinical with was examined ECG and laboratory. The pathology samples were sectioned routine paraffin and stained with HE、PAS、AB-PAS and cellulose staining and observed with light microscope. Results The ECG was manifested acute myocardial infarction in the left lateral wall and the blood examination were of the heart, and the blood examination were manifested lift pedigree ferment of myocardium. The pathology sections were manifested necrosis large area perforation wall of the myocardial and became granuloma diffusible. Endocarditis, necrotic vesselitis and thrombus could be found and that the coronary is not embolism. Similar lesions could be found and alike to the heart in the left lung parenchyma and the peripheral zone of trachea and blood vessel. Histochemical examination with PAS and AB-PAS showed cryptococcus negative in the heart and lungs. Cellulose staining was positive reaction in the vascular wall. Antineutrophil cytoplasmic antibody (ANCA) was negative in the blood from the heart. Conclusion Cardiac Wegener's granulomatosis is rare. Clinical sings the ever so similar to acute myocardial infarction and that is not all but differentiation and prognosis and unfavorable of the ever cuch.

  14. 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

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

    Directory of Open Access Journals (Sweden)

    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.

  16. Saturn's Rings Reveal Unexpected Phenomena

    Institute of Scientific and Technical Information of China (English)

    李颖

    2004-01-01

    Safely in orbit around Saturn, NASA's Cassini spacecraft sent back its first close-up images of the massive planet's rings on July 1, revealing an unexpectedly varied terrain featuring surprisingly sharp edges, braids and delicate ridges.

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

    Directory of Open Access Journals (Sweden)

    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.

  18. Genetic aspects of anti-neutrophil cytoplasmic antibody-associated vasculitis.

    Science.gov (United States)

    Alberici, Federico; Martorana, Davide; Vaglio, Augusto

    2015-04-01

    The genetics of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a complex area of investigation because of the low frequency of AAVs, the rarity of familial cases and the complexity of disease phenotypes. However, recent studies have been able to gather significant numbers of patients, and multicentre collaborative efforts have allowed the performance of two genome-wide association studies (GWASs). Genetic association studies based on candidate gene approaches and the two GWASs have greatly contributed to our current understanding of the genetic basis of AAV. The central role of autoimmunity has been confirmed by the significant association with HLA polymorphisms; interestingly, the three main AAV subtypes are associated with distinct HLA variants, i.e. granulomatosis with polyangiitis (Wegener's GPA) with HLA-DP1, microscopic polyangiitis with HLA-DQ and eosinophilic GPA (Churg-Strauss) with HLA-DRB4. GWASs also revealed that polymorphic variants of genes encoding proteinase 3 (PR3), the predominant antigenic target of ANCA in GPA, and its main inhibitor, alpha-1 antitrypsin, are highly associated with GPA and, even more significantly, with PR3-ANCA positivity (regardless of the clinical diagnosis); this emphasizes the central pathogenic role of PR3 and humoral autoimmunity in PR3-ANCA positive vasculitis. Finally, candidate gene approach studies have shown associations with other variants involved in autoimmunity, such as those belonging to the CTLA-4 and PTPN22 genes, although these findings warrant replication in larger studies. Additional studies are underway to better characterize disease associations within the AAV spectrum, which could provide new pathogenetic clues and possibly new treatment targets.

  19. Integrated Fluorine-18 Fluorodeoxyglucose (18F-FDG) PET/CT Compared to Standard Contrast-Enhanced CT for Characterization and Staging of Pulmonary Tumors Eligible for Surgical Resection

    Energy Technology Data Exchange (ETDEWEB)

    Quaia, E.; Tona, G.; Gelain, F.; Lubin, E.; Pizzolato, R.; Boscolo, E.; Bussoli, L. (Dept. of Radiology, Cattinara Hospital, Univ. of Trieste, Trieste (Italy))

    2008-11-15

    Background: Accurate staging is necessary to determine the appropriate therapy in patients with lung cancer. Few studies have compared integrated fluorine-18 fluorodeoxyglucose (18F-FDG) positron emission tomography/computed tomography (PET/CT) and contrast-enhanced CT in the characterization and staging of pulmonary tumors considered eligible for surgical resection. Purpose: To compare 18F-FDG PET/CT with standard contrast-enhanced CT for the diagnosis and staging of lung neoplasms eligible for surgical resection. Material and Methods: Seventy-six consecutive patients (56 male, 20 female; mean age+-SD, 63.4+-20 years) with 84 pulmonary tumors suspected for malignancy and considered eligible for surgical resection were prospectively enrolled. Seventy-three malignant (65 non-small-cell lung carcinomas, one small-cell lung cancer, two carcinoids, and five metastases) and 11 benign lung tumors (three hamartomas, two sarcoidosis, one amyloidosis, one Wegener granulomatosis, one tuberculosis, and three areas of scarring) were finally diagnosed by histology. Tumor staging was based on the revised American Joint Committee on Cancer. Results: In lesion characterization, the sensitivity and specificity of 18F-FDG PET/CT versus contrast-enhanced CT were 90% vs. 83% and 18% vs. 63% (P<0.05, McNemar test), respectively. In nodal staging, the sensitivity and specificity of 18F-FDG PET/CT versus contrast-enhanced CT were 78% vs. 46% and 80% vs. 93% (P<0.05), respectively. Conclusion: In patients with lung neoplasms considered eligible for surgical resection, 18F-FDG PET/CT versus contrast-enhanced CT revealed higher sensitivity in nodal staging, but lower specificity both in lesion characterization and nodal staging.

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

    Directory of Open Access Journals (Sweden)

    Fermin Blanco Filho

    2001-01-01

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

  1. Endoscopic dacryocystorhinostomy

    Directory of Open Access Journals (Sweden)

    Milojević Milanko

    2010-01-01

    Full Text Available Background/Aim. Intensive epiphora (lacrimal apparatus disease can make difficult daily routine and cause ocular refraction disturbances. In most cases ethiology is unknown, rarely occurs after nose surgical procedures, face fractures, in Wegener granulomatosis, sarcoidosis and malignancies. The aim of this study was to evaluate efficacy of endonasal endoscopic surgical procedure with the conventional surgical instruments in treatment of nasolacrimal obstructions. Methods. This retrospective study included 12 female patients with endonasal endoscopic surgical procedure from Otorhinological and Ophtalmological Departments of Military Medical Academy, Belgrade from September 2007 to April 2009. Preoperative nasal endoscopy was performed in order to reveal concomitant pathological conditions and anatomic anomalies which could make surgical procedure impossible. Computerized tomography was performed only in suspect nose diseases. Surgical endonasal endoscopic procedure was performed by otorhinolaryngologist and ophtalmologist in all patients. The patients had regular controls from 2 to 20 months. Results. A total of 12 female patients, age 34-83 years, were included in our study. Epiphora was a dominant symptom in all patients. In two patients deviation of nasal septum was found, and in other one conha bulosa at the same side as chronic dacryocystitis. All patients were subjected to endonasal dacryocystorhinostomy (DCR by endoscopic surgical technique using conventional instruments. Concomitantly with DCR septoplastics in two patients and lateral lamictetomy in one patient were performed. There were no complications intraoperatively as well as in the immediate postoperative course. In two patients the need for reoperation occurred. Conclusion. Endoscopic DCR is minimally invasive and efficacious procedure for nasolacrymal obstructions performed by otorhinolaryngologist and ophtalmologist. Postoperative recovery is very fast.

  2. REVEAL: Software Documentation and Platform Migration

    Science.gov (United States)

    Wilson, Michael A.; Veibell, Victoir T.

    2011-01-01

    The Research Environment for Vehicle Embedded Analysis on Linux (REVEAL) is reconfigurable data acquisition software designed for network-distributed test and measurement applications. In development since 2001, it has been successfully demonstrated in support of a number of actual missions within NASA's Suborbital Science Program. Improvements to software configuration control were needed to properly support both an ongoing transition to operational status and continued evolution of REVEAL capabilities. For this reason the project described in this report targets REVEAL software source documentation and deployment of the software on a small set of hardware platforms different from what is currently used in the baseline system implementation. This presentation specifically describes the actions taken over a ten week period by two undergraduate student interns and serves as an overview of the content of the final report for that internship.

  3. Omics strategies for revealing Yersinia pestis virulence

    Science.gov (United States)

    Yang, Ruifu; Du, Zongmin; Han, Yanping; Zhou, Lei; Song, Yajun; Zhou, Dongsheng; Cui, Yujun

    2012-01-01

    Omics has remarkably changed the way we investigate and understand life. Omics differs from traditional hypothesis-driven research because it is a discovery-driven approach. Mass datasets produced from omics-based studies require experts from different fields to reveal the salient features behind these data. In this review, we summarize omics-driven studies to reveal the virulence features of Yersinia pestis through genomics, trascriptomics, proteomics, interactomics, etc. These studies serve as foundations for further hypothesis-driven research and help us gain insight into Y. pestis pathogenesis. PMID:23248778

  4. [Mastitis revealing Churg-Strauss syndrome].

    Science.gov (United States)

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

    2014-01-01

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

  5. Revealing the Anatomy of Vote Trading

    CERN Document Server

    Guerrero, Omar A

    2016-01-01

    Cooperation in the form of vote trading, also known as logrolling, is central for law-making processes, shaping the development of democratic societies. Empirical evidence of logrolling is scarce and limited to highly specific situations because existing methods are not easily applicable to broader contexts. We have developed a general and scalable methodology for revealing a network of vote traders, allowing us to measure logrolling on a large scale. Analysis on more than 9 million votes spanning 40 years in the U.S. Congress reveals a higher logrolling prevalence in the Senate and an overall decreasing trend over recent congresses, coincidental with high levels of political polarization. Our method is applicable in multiple contexts, shedding light on many aspects of logrolling and opening new doors in the study of hidden cooperation.

  6. Revealing interference by continuous variable discordant states.

    Science.gov (United States)

    Meda, A; Olivares, S; Degiovanni, I P; Brida, G; Genovese, M; Paris, M G A

    2013-08-15

    In general, a pair of uncorrelated Gaussian states mixed in a beam splitter (BS) produces a correlated state at the output. However, when the inputs are identical Gaussian states the output state is equal to the input, and no correlations appear, as the interference had not taken place. On the other hand, since physical phenomena do have observable effects, and the BS is there, a question arises on how to reveal the interference between the two beams. We prove theoretically and demonstrate experimentally that this is possible if at least one of the two beams is prepared in a discordant, i.e., Gaussian correlated, state with a third beam. We also apply the same technique to reveal the erasure of polarization information. Our experiment involves thermal states and the results show that Gaussian discordant states, even when they show a positive Glauber P-function, may be useful to achieve specific tasks.

  7. Revealed comparative advantage of Russian agricultural exports

    Directory of Open Access Journals (Sweden)

    Natalia Ishchukova

    2013-01-01

    Full Text Available This paper studies the issue of revealed comparative advantage in the case of the Russian foreign trade in agricultural products and foodstuffs. The objective of this study is to analyze specialization and the competitive performance of the Russian agricultural sector and to determine whether or not patterns of comparative advantage for Russia have undergone significant changes over the period 1998–2010.The analysis is performed using several measures of revealed comparative advantage: classical Balassa’s index, Vollrath’s index and Lafay index. Balassa’s index identified a group of products, which has relatively stable comparative advantage during the whole period. Among those products we can count cereals (wheat, barley, etc., their by-products (for example bran of wheat and products of their processing (cereals preparations, etc., as well as oilseeds, vegetable oils and chocolate.Vollrath’s index showed that the number of products that have revealed competitive advantage was steadily growing during the period. Lafay index, used in the analysis by regions, showed that Russia has comparative advantages in relation to CIS countries and Asian countries due to its geographical location and good trade relations.Primary products have the comparative advantage in relation to EU and Asian countries. In relation to the Commonwealth of Independent States and Americas on the contrary the processed products have comparative advantages, while the most of primary products have comparative disadvantage.In relation to the whole world, analysis has also revealed a shift of comparative advantage from by-products (e.g. bran of wheat, sunflower cake etc. in 1998–2001 to primary products in 2002–2010 (wheat, barley, whole cow milk, sunflower seed etc..

  8. Reveal quantum correlation in complementary bases

    OpenAIRE

    Shengjun Wu; Zhihao Ma; Zhihua Chen; Sixia Yu

    2013-01-01

    An essential feature of genuine quantum correlation is the simultaneous existence of correlation in complementary bases. We reveal this feature of quantum correlation by defining measures based on invariance under a basis change. For a bipartite quantum state, the classical correlation is the maximal correlation present in a certain optimum basis, while the quantum correlation is characterized as a series of residual correlations in the mutually unbiased bases. Compared with other approaches ...

  9. Mediastinal Mature Teratoma Revealed by Empyema

    Directory of Open Access Journals (Sweden)

    Mohammed Raoufi

    2016-01-01

    Full Text Available Teratomas are germ cell tumors, manifested with a great variety of clinical features; the most common extragonadal site is the anterior mediastinum. In this case, we report the patient with a large mature mediastinal teratoma with several components of ectodermal and endothermal epithelium. A 24-year-old female patient presented with history of persistent chest pain and progressively aggravating dyspnea for the previous 3 months. A chest X-ray showed a large opacity of the entire left hemithorax. Transcutaneous needle aspiration revealed a purulent fluid. The tube thoracostomy was introduced and the effusion was evacuated. Some weeks later, patient was seen in emergency for persistent cough and lateral chest pain. CT scan revealed a mass of the left hemithorax. The mass showed heterogeneous density, without compressing mediastinum great vessels and left hilar structures. Lipase value was elevated in needle aspiration. The patient underwent a total resection of the mediastinum mass via a left posterolateral thoracotomy. Microscopy revealed a mature teratoma with cystic structures. The patient subsequently made a full recovery. This case provide benign mediastinal teratoma with total atelectasis of left lung and elevated lipase value in needle transcutaneous aspiration; this event is explained by pancreatic component in the cystic tumor. Total removal of the tumor is adequate treatment for this type of teratoma and the prognosis is excellent.

  10. Revealing digital documents. Concealed structures in data

    CERN Document Server

    Voß, Jakob

    2011-01-01

    This short paper gives an introduction to a research project to analyze how digital documents are structured and described. Using a phenomenological approach, this research will reveal common patterns that are used in data, independent from the particular technology in which the data is available. The ability to identify these patterns, on different levels of description, is important for several applications in digital libraries. A better understanding of data structuring will not only help to better capture singular characteristics of data by metadata, but will also recover intended structures of digital objects, beyond long term preservation.

  11. Revealing Cosmic Magnetism with Radio Polarimetry

    CERN Document Server

    Gaensler, Bryan M

    2007-01-01

    While gravitation sustains the on-going evolution of the cosmos, it is magnetism that breaks gravity's symmetry and that provides the pathway to the non-thermal Universe. By enabling processes such as anisotropic pressure support, particle acceleration, and jet collimation, magnetism has for billions of years regulated the feedback vital for returning matter to the interstellar and intergalactic medium. After reviewing recent results that demonstrate the unique view of magnetic fields provided by radio astronomy, I explain how the Square Kilometre Array will provide data that will reveal what cosmic magnets look like, how they formed, and what role they have played in the evolving Universe.

  12. Infections Revealing Complement Deficiency in Adults

    Science.gov (United States)

    Audemard-Verger, A.; Descloux, E.; Ponard, D.; Deroux, A.; Fantin, B.; Fieschi, C.; John, M.; Bouldouyre, A.; Karkowsi, L.; Moulis, G.; Auvinet, H.; Valla, F.; Lechiche, C.; Davido, B.; Martinot, M.; Biron, C.; Lucht, F.; Asseray, N.; Froissart, A.; Buzelé, R.; Perlat, A.; Boutboul, D.; Fremeaux-Bacchi, V.; Isnard, S.; Bienvenu, B.

    2016-01-01

    Abstract Complement system is a part of innate immunity, its main function is to protect human from bacterial infection. As genetic disorders, complement deficiencies are often diagnosed in pediatric population. However, complement deficiencies can also be revealed in adults but have been poorly investigated. Herein, we describe a case series of infections revealing complement deficiency in adults to study clinical spectrum and management of complement deficiencies. A nationwide retrospective study was conducted in French university and general hospitals in departments of internal medicine, infectious diseases enrolling patients older than 15 years old who had presented at least one infection leading to a complement deficiency diagnosis. Forty-one patients included between 2002 and 2015 in 19 different departments were enrolled in this study. The male-to-female ratio was 1.3 and the mean age at diagnosis was 28 ± 14 (15–67) years. The main clinical feature was Neisseria meningitidis meningitis 75% (n = 31/41) often involving rare serotype: Y (n = 9) and W 135 (n = 7). The main complement deficiency observed was the common final pathway deficiency 83% (n = 34/41). Half of the cohort displayed severe sepsis or septic shock at diagnosis (n = 22/41) but no patient died. No patient had family history of complement deficiency. The mean follow-up was 1.15 ± 1.95 (0.1–10) years. Half of the patients had already suffered from at least one infection before diagnosis of complement deficiency: meningitis (n = 13), pneumonia (n = 4), fulminans purpura (n = 1), or recurrent otitis (n = 1). Near one-third (n = 10/39) had received prophylactic antibiotics (cotrimoxazole or penicillin) after diagnosis of complement deficiency. The vaccination coverage rate, at the end of the follow-up, for N meningitidis, Streptococcus pneumonia, and Haemophilius influenzae were, respectively, 90% (n = 33/37), 47% (n = 17/36), and 35

  13. Superstitious perceptions reveal properties of internal representations.

    Science.gov (United States)

    Gosselin, Frédéric; Schyns, Philippe G

    2003-09-01

    Everyone has seen a human face in a cloud, a pebble, or blots on a wall. Evidence of superstitious perceptions has been documented since classical antiquity, but has received little scientific attention. In the study reported here, we used superstitious perceptions in a new principled method to reveal the properties of unobservable object representations in memory. We stimulated the visual system with unstructured white noise. Observers firmly believed that they perceived the letter S in Experiment 1 and a smile on a face in Experiment 2. Using reverse correlation and computational analyses, we rendered the memory representations underlying these superstitious perceptions.

  14. Apocalypto: revealing lost text with XMT

    Science.gov (United States)

    Mills, David; Davis, Graham R.; Lai, Yu-kun; Rosin, Paul

    2014-09-01

    "Can brute-force high-contrast tomography techniques and image processing techniques retrieve textual content from damaged heritage materials?" The Dental Institute at Queen Mary University of London (QMUL) is the leading centre for very high contrast X-Ray Microtomography imaging. The Apocalypto Project is our collaboration with the heritage community and experts in Computer Vision systems in the Computer Science Department at Cardiff University. This collaboration has developed techniques and a workflow that allows us to reveal textual content from moisture-damaged parchment rolls. This article will also present some initial results from burned and heat shrunken parchment rolls, an insect damaged Mamluk cap and a birch bark roll.

  15. Revealing Event Saliency in Unconstrained Video Collection.

    Science.gov (United States)

    Zhang, Dingwen; Han, Junwei; Jiang, Lu; Ye, Senmao; Chang, Xiaojun

    2017-04-01

    Recent progresses in multimedia event detection have enabled us to find videos about a predefined event from a large-scale video collection. Research towards more intrinsic unsupervised video understanding is an interesting but understudied field. Specifically, given a collection of videos sharing a common event of interest, the goal is to discover the salient fragments, i.e., the curt video fragments that can concisely portray the underlying event of interest, from each video. To explore this novel direction, this paper proposes an unsupervised event saliency revealing framework. It first extracts features from multiple modalities to represent each shot in the given video collection. Then, these shots are clustered to build the cluster-level event saliency revealing framework, which explores useful information cues (i.e., the intra-cluster prior, inter-cluster discriminability, and inter-cluster smoothness) by a concise optimization model. Compared with the existing methods, our approach could highlight the intrinsic stimulus of the unseen event within a video in an unsupervised fashion. Thus, it could potentially benefit to a wide range of multimedia tasks like video browsing, understanding, and search. To quantitatively verify the proposed method, we systematically compare the method to a number of baseline methods on the TRECVID benchmarks. Experimental results have demonstrated its effectiveness and efficiency.

  16. Transcriptome classification reveals molecular subtypes in psoriasis

    Directory of Open Access Journals (Sweden)

    Ainali Chrysanthi

    2012-09-01

    Full Text Available Abstract Background Psoriasis is an immune-mediated disease characterised by chronically elevated pro-inflammatory cytokine levels, leading to aberrant keratinocyte proliferation and differentiation. Although certain clinical phenotypes, such as plaque psoriasis, are well defined, it is currently unclear whether there are molecular subtypes that might impact on prognosis or treatment outcomes. Results We present a pipeline for patient stratification through a comprehensive analysis of gene expression in paired lesional and non-lesional psoriatic tissue samples, compared with controls, to establish differences in RNA expression patterns across all tissue types. Ensembles of decision tree predictors were employed to cluster psoriatic samples on the basis of gene expression patterns and reveal gene expression signatures that best discriminate molecular disease subtypes. This multi-stage procedure was applied to several published psoriasis studies and a comparison of gene expression patterns across datasets was performed. Conclusion Overall, classification of psoriasis gene expression patterns revealed distinct molecular sub-groups within the clinical phenotype of plaque psoriasis. Enrichment for TGFb and ErbB signaling pathways, noted in one of the two psoriasis subgroups, suggested that this group may be more amenable to therapies targeting these pathways. Our study highlights the potential biological relevance of using ensemble decision tree predictors to determine molecular disease subtypes, in what may initially appear to be a homogenous clinical group. The R code used in this paper is available upon request.

  17. Symptomatic hypopituitarism revealing primary suprasellar lymphoma

    Directory of Open Access Journals (Sweden)

    M'rabti Hind

    2010-11-01

    Full Text Available Abstract Background The most common cause of hypopituitarism is pituitary adenoma. However, in the case of suprasellar masses different etiologies are possible. We report an unusual case of primary suprasellar lymphoma presented with hypopituitarism. Case presentation A 26 year old woman presented with amenorrhea, galactorrhea and neurological disorders. Also, the laboratory work-up revealed partial hypopituitarism. The magnetic resonance imaging of the head showed a suprasellar mass. A presumptive diagnosis of granulomatous processes was made and the patient was given steroid therapy. Repeated brain MRI detected new lesions in the brain with regression of the suprasellar mass. Stereotactic biopsy of the paraventricular lesion revealed the diagnosis of B-cell lymphoma. Conclusion This case presentation reports a rare cause of hypopituitarism. Primary suprasellar lymphoma is extremely rare and represented a real diagnostic challenge. Besides, suprasellar masses are varied in aetiology and can present diagnostic problems for a radiologist. Also, because of the increased incidence of PCNSL, lymphoma must be kept in mind in the differential diagnosis of lesions in the suprasellar region.

  18. Revealed Quantum Information in Weak Interaction Processes

    CERN Document Server

    Hiesmayr, B C

    2014-01-01

    We analyze the achievable limits of the quantum information processing of the weak interaction revealed by hyperons with spin. We find that the weak decay process corresponds to an interferometric device with a fixed visibility and fixed phase difference for each hyperon. Nature chooses rather low visibilities expressing a preference to parity conserving or violating processes (except for the decay $\\Sigma^+\\longrightarrow p \\pi^0$). The decay process can be considered as an open quantum channel that carries the information of the hyperon spin to the angular distribution of the momentum of the daughter particles. We find a simple geometrical information theoretic interpretation of this process: two quantization axes are chosen spontaneously with probabilities $\\frac{1\\pm\\alpha}{2}$ where $\\alpha$ is proportional to the visibility times the real part of the phase shift. Differently stated the weak interaction process corresponds to spin measurements with an imperfect Stern-Gerlach apparatus. Equipped with this...

  19. Social patterns revealed through random matrix theory

    Science.gov (United States)

    Sarkar, Camellia; Jalan, Sarika

    2014-11-01

    Despite the tremendous advancements in the field of network theory, very few studies have taken weights in the interactions into consideration that emerge naturally in all real-world systems. Using random matrix analysis of a weighted social network, we demonstrate the profound impact of weights in interactions on emerging structural properties. The analysis reveals that randomness existing in particular time frame affects the decisions of individuals rendering them more freedom of choice in situations of financial security. While the structural organization of networks remains the same throughout all datasets, random matrix theory provides insight into the interaction pattern of individuals of the society in situations of crisis. It has also been contemplated that individual accountability in terms of weighted interactions remains as a key to success unless segregation of tasks comes into play.

  20. Revealing effective classifiers through network comparison

    CERN Document Server

    Gallos, Lazaros K

    2014-01-01

    The ability to compare complex systems can provide new insight into the fundamental nature of the processes captured in ways that are otherwise inaccessible to observation. Here, we introduce the $n$-tangle method to directly compare two networks for structural similarity, based on the distribution of edge density in network subgraphs. We demonstrate that this method can efficiently introduce comparative analysis into network science and opens the road for many new applications. For example, we show how the construction of a phylogenetic tree across animal taxa according to their social structure can reveal commonalities in the behavioral ecology of the populations, or how students create similar networks according to the University size. Our method can be expanded to study a multitude of additional properties, such as network classification, changes during time evolution, convergence of growth models, and detection of structural changes during damage.

  1. Neutron Imaging Reveals Internal Plant Hydraulic Dynamics

    Energy Technology Data Exchange (ETDEWEB)

    Warren, Jeffrey [ORNL; Bilheux, Hassina Z [ORNL; Kang, Misun [ORNL; Voisin, Sophie [ORNL; Cheng, Chu-Lin [ORNL; Horita, Jusuke [ORNL; Perfect, Edmund [ORNL

    2013-01-01

    Many terrestrial ecosystem processes are constrained by water availability and transport within the soil. Knowledge of plant water fluxes is thus critical for assessing mechanistic processes linked to biogeochemical cycles, yet resolution of root structure and xylem water transport dynamics has been a particularly daunting task for the ecologist. Through neutron imaging, we demonstrate the ability to non-invasively monitor individual root functionality and water fluxes within Zea mays L. (maize) and Panicum virgatum L. (switchgrass) seedlings growing in a sandy medium. Root structure and growth were readily imaged by neutron radiography and neutron computed tomography. Seedlings were irrigated with water or deuterium oxide and imaged through time as a growth lamp was cycled on to alter leaf demand for water. Sub-millimeter scale resolution reveals timing and magnitudes of root water uptake, redistribution within the roots, and root-shoot hydraulic linkages, relationships not well characterized by other techniques.

  2. [Pneumothorax revealed by postoperative computed tomography].

    Science.gov (United States)

    Ikeda, Shizuka; Katori, Kiyoshi; Fujimoto, Minoru; Nitahara, Keiichi; Higa, Kazuo

    2005-11-01

    We report a case of pneumothorax revealed by postoperative computed tomography. A 39-year-old obese woman (height 153 cm, weight 70 kg) with fractures of the radius, ulna, clavicle, and femur in a traffic accident, was scheduled for osteosynthesis. Anesthesia was induced with thiopental and maintained with 50% nitrous oxide in oxygen and sevoflurane. The Spo2 decreased from 99% to 94% during the surgery. Bilateral chest sounds were symmetrical. The Spo2 increased to 100% after discontinuation of nitrous oxide. Pneumothorax was not evident on a postoperative chest X-ray, but computed tomography of the chest demonstrated right-sided pneumothorax. An ECG electrode had overlapped the fractured rib on the preoperative chest X-ray.

  3. Can Clustering in Genotype Space Reveal "Niches"?

    Science.gov (United States)

    D'Andrea, Rafael; Ostling, Annette

    2016-01-01

    Community ecology lacks the success enjoyed by population genetics to quantify the relative roles played by deterministic and stochastic processes. It has been proposed that clustered patterns of abundance in genotype space provide evidence of selection in microbial communities, since no such clustering would arise in the absence of selection. We critique this test for its unrealistic null hypothesis. We show mathematically and with simulations that point mutations alone lead to clustering in genotype space by causing correlations between abundances of similar genotypes. We also show potential deviations from the mutation-only pattern caused by immigration from a source pool. Clustered patterns in genotype space may still be revealing of selection if analyzed quantitatively but only if neutral and selective regimes can be distinguished once mutation and immigration are included in the null model.

  4. Reward reveals dissociable aspects of sustained attention.

    Science.gov (United States)

    Esterman, Michael; Reagan, Andrew; Liu, Guanyu; Turner, Caroline; DeGutis, Joseph

    2014-12-01

    Although reward is known to have a powerful influence on performance, its effects on the ability to continuously sustain performance over time are poorly understood. The current study examines multiple measures of sustained attention (accuracy and variability) and their decrements over time, while introducing reward in the form of a monetary incentive or the promise of early completion. Compared with unrewarded participants, rewarded participants demonstrated greater overall accuracy and lower reaction time variability. However, rewarded and unrewarded participants displayed nearly identical decrements in performance over time, suggesting that these aspects of sustained attention are far less malleable by enhanced effort. This study helps to resolve conflicting models of sustained attention as it reveals that some aspects of performance are due to motivational lapses whereas others are due to the depletion of cognitive resources that cannot be easily overcome.

  5. Double peaks reveal rare diplomonad sex.

    Science.gov (United States)

    Andersson, Jan O

    2012-02-01

    Diplomonads, single-celled eukaryotes, are unusual in having two nuclei. Each nucleus contains two copies of the genome and is transcriptionally active. It has long been assumed that diplomonads in general and Giardia intestinalis in particular are asexual. Genomic and population genetic data now challenge that assumption and extensive allelic sequence heterogeneity has been reported in some but not all examined diplomonad lineages. Here it is argued, in contrast to common assumptions, that allelic differences indicate recent sexual events, and isolates that have divided asexually for many generations have lost their allelic variation owing to within-cell recombination. Consequently, directed studies of the allelic sequence heterogeneity in diverse diplomonad lineages are likely to reveal details about the enigmatic diplomonad sexual life cycle.

  6. Chemotaxis: new role for Ras revealed

    Institute of Scientific and Technical Information of China (English)

    Jianshe Yan; Dale Hereld; Tian Jin

    2010-01-01

    @@ A recent study of chemotaxis revealed a new role for the proto-oncogene Ras in the social ameba Dictyostelium discoideum.Chemotaxis,the directional movement of cells toward chemokines and other chemoattractants,plays critical roles in diverse physiological processes,such as mobilization of immune cells to fight invading microorganisms,targeting of metastatic cancer cells to specific tissues,and guidance of sperm cells to ova during fertilization.This work,published in the July 26 issue of The Journal of Cell Biology,was conducted in Dr.Devreotes' lab at John Hopkins University and Dr.Parent's lab at National Cancer Institute.This research team demonstrated that RasC functions as an upstream regulator of TORC2 and thereby governs the effects of TORC2-PKB signaling on the cytoskeleton and cell migration.

  7. Licking microstructure reveals rapid attenuation of neophobia.

    Science.gov (United States)

    Monk, Kevin J; Rubin, Benjamin D; Keene, Jennifer C; Katz, Donald B

    2014-03-01

    Many animals hesitate when initially consuming a novel food and increase their consumption of that food between the first and second sessions of access-a process termed attenuation of neophobia (AN). AN has received attention as a model of learning and memory; it has been suggested that plasticity resulting from an association of the novel tastant with "safe outcome" results in a change in the neural response to the tastant during the second session, such that consumption increases. Most studies have reported that AN emerges only an hour or more after the end of the first exposure to the tastant, consistent with what is known of learning-related plasticity. But these studies have typically measured consumption, rather than real-time behavior, and thus the possibility exists that a more rapidly developing AN remains to be discovered. Here, we tested this possibility, examining both consumption and individual lick times in a novel variant of a brief-access task (BAT). When quantified in terms of consumption, data from the BAT accorded well with the results of a classic one-bottle task-both revealed neophobia/AN specific to higher concentrations (for instance, 28mM) of saccharin. An analysis of licking microstructure, however, additionally revealed a real-time correlate of neophobia-an explicit tendency, similarly specific for 28-mM saccharin, to cut short the initial bout of licks in a single trial (compared with water). This relative hesitancy (i.e., the shortness of the first lick bout to 28-mM saccharin compared with water) that constitutes neophobia not only disappeared between sessions but also gradually declined in magnitude across session 1. These data demonstrate that the BAT accurately measures AN, and that aspects of AN-and the processes underlying familiarization-begin within minutes of the very first taste.

  8. Stochastic homothetically revealed preference for tight stochastic demand functions

    OpenAIRE

    Jan Heufer

    2009-01-01

    This paper strengthens the framework of stochastic revealed preferences introduced by Bandyopadhyay et al. (1999, 2004) for stochastic homothetically revealed preferences for tight stochastic demand functions.

  9. Speeding Clouds May Reveal Invisible Black Holes

    Science.gov (United States)

    Kohler, Susanna

    2017-07-01

    Several small, speeding clouds have been discovered at the center of our galaxy. A new study suggests that these unusual objects may reveal the lurking presence of inactive black holes.Peculiar Cloudsa) Velocity-integrated intensity map showing the location of the two high-velocity compact clouds, HCN0.0090.044 and HCN0.0850.094, in the context of larger molecular clouds. b) and c) Latitude-velocity and longitude-velocity maps for HCN0.0090.044 and HCN0.0850.094, respectively. d) and e) spectra for the two compacts clouds, respectively. Click for a closer look. [Takekawa et al. 2017]Sgr A*, the supermassive black hole marking the center of our galaxy, is surrounded by a region roughly 650 light-years across known as the Central Molecular Zone. This area at the heart of our galaxy is filled with large amounts of warm, dense molecular gas that has a complex distribution and turbulent kinematics.Several peculiar gas clouds have been discovered within the Central Molecular Zone within the past two decades. These clouds, dubbed high-velocity compact clouds, are characterized by their compact sizes and extremely broad velocity widths.What created this mysterious population of energetic clouds? The recent discovery of two new high-velocity compact clouds, reported on in a paper led by Shunya Takekawa (Keio University, Japan), may help us to answer this question.Two More to the CountUsing the James Clerk Maxwell Telescope in Hawaii, Takekawa and collaborators detected the small clouds near the circumnuclear disk at the centermost part of our galaxy. These two clouds have velocity spreads of -80 to -20 km/s and -80 to 0 km/s and compact sizes of just over 1 light-year. The clouds similar appearances and physical properties suggest that they may both have been formed by the same process.Takekawa and collaborators explore and discard several possible origins for these clouds, such as outflows from massive protostars (no massive, luminous stars have been detected affiliated

  10. ERYTHEMA NODOSUM REVEALING ACUTE MYELOID LEUKEMIA

    Directory of Open Access Journals (Sweden)

    Chebbi Wafa

    2013-07-01

    Full Text Available Introduction: Erythema nodosum (EN is the most common type of panniculitis. It may be idiopathic or secondary to various etiologies. However, the occurrence of erythema nodosum in malignant hemopathy had rarely been reported. Case report: A 42 year-old woman presented with a four week history of recurrent multiple painful erythematous nodules developed on the lower limbs associated with arthralgia of the ankles and fever. The clinical features of skin lesions with contusiform color evolution allowed establishing the diagnosis of EN. No underlying cause was found. The skin lesions were improved with non-steroidal anti-inflammatory drugs and colchicine. Three months later, the patient consulted for recurrence of EN associated with fever, inflammatory polyarthralgia and hepatosplenomegaly. The peripheral blood count revealed pancytopenia. A bone marrow examination confirmed the diagnosis of acute myeloid leukemia type 2. Initiation of chemotherapy was followed by the complete disappearance of skin lesions of EN. Conclusion: Paraneoplastic erythema nodosum is a rare entity. In the literature, a few cases of association with leukemia have been reported. Exploration for solid neoplasms or hemopathy in case of recurrent EN or resistance to conventional treatment should be systematic

  11. Balance Trees Reveal Microbial Niche Differentiation.

    Science.gov (United States)

    Morton, James T; Sanders, Jon; Quinn, Robert A; McDonald, Daniel; Gonzalez, Antonio; Vázquez-Baeza, Yoshiki; Navas-Molina, Jose A; Song, Se Jin; Metcalf, Jessica L; Hyde, Embriette R; Lladser, Manuel; Dorrestein, Pieter C; Knight, Rob

    2017-01-01

    Advances in sequencing technologies have enabled novel insights into microbial niche differentiation, from analyzing environmental samples to understanding human diseases and informing dietary studies. However, identifying the microbial taxa that differentiate these samples can be challenging. These issues stem from the compositional nature of 16S rRNA gene data (or, more generally, taxon or functional gene data); the changes in the relative abundance of one taxon influence the apparent abundances of the others. Here we acknowledge that inferring properties of individual bacteria is a difficult problem and instead introduce the concept of balances to infer meaningful properties of subcommunities, rather than properties of individual species. We show that balances can yield insights about niche differentiation across multiple microbial environments, including soil environments and lung sputum. These techniques have the potential to reshape how we carry out future ecological analyses aimed at revealing differences in relative taxonomic abundances across different samples. IMPORTANCE By explicitly accounting for the compositional nature of 16S rRNA gene data through the concept of balances, balance trees yield novel biological insights into niche differentiation. The software to perform this analysis is available under an open-source license and can be obtained at https://github.com/biocore/gneiss. Author Video: An author video summary of this article is available.

  12. VISTA Reveals the Secret of the Unicorn

    Science.gov (United States)

    2010-10-01

    A new infrared image from ESO's VISTA survey telescope reveals an extraordinary landscape of glowing tendrils of gas, dark clouds and young stars within the constellation of Monoceros (the Unicorn). This star-forming region, known as Monoceros R2, is embedded within a huge dark cloud. The region is almost completely obscured by interstellar dust when viewed in visible light, but is spectacular in the infrared. An active stellar nursery lies hidden inside a massive dark cloud rich in molecules and dust in the constellation of Monoceros. Although it appears close in the sky to the more familiar Orion Nebula it is actually almost twice as far from Earth, at a distance of about 2700 light-years. In visible light a grouping of massive hot stars creates a beautiful collection of reflection nebulae where the bluish starlight is scattered from parts of the dark, foggy outer layers of the molecular cloud. However, most of the new-born massive stars remain hidden as the thick interstellar dust strongly absorbs their ultraviolet and visible light. In this gorgeous infrared image taken from ESO's Paranal Observatory in northern Chile, the Visible and Infrared Survey Telescope for Astronomy (VISTA [1], eso0949) penetrates the dark curtain of cosmic dust and reveals in astonishing detail the folds, loops and filaments sculpted from the dusty interstellar matter by intense particle winds and the radiation emitted by hot young stars. "When I first saw this image I just said 'Wow!' I was amazed to see all the dust streamers so clearly around the Monoceros R2 cluster, as well as the jets from highly embedded young stellar objects. There is such a great wealth of exciting detail revealed in these VISTA images," says Jim Emerson, of Queen Mary, University of London and leader of the VISTA consortium. With its huge field of view, large mirror and sensitive camera, VISTA is ideal for obtaining deep, high quality infrared images of large areas of the sky, such as the Monoceros R2 region

  13. Revealing the values behind convenience food consumption.

    Science.gov (United States)

    Botonaki, Anna; Mattas, Konstadinos

    2010-12-01

    The increasing importance of convenience in consumer food choices has attracted researchers' interest. In the effort to understand how convenience affects consumers' food preferences, values are believed to play an important role. The present study attempts to examine the way personal values suggested by Schwartz (1992) are associated with behaviour and attitudes regarding convenience food. A number of constructs describing food related attitudes and behaviours were developed and their relationship with personal values was analyzed following the methodology proposed by Brunsø, Scholderer, and Grunert (2004). Data were collected through a questionnaire survey from a random sample of consumers in Thessaloniki city, Greece. The results reveal that convenience food consumption and convenience orientation in the food domain are mainly connected with values that motivate people to seek new experiences, act independently and enhance their own personal interests, while are in conflict with values of conservation and self-transcendence. The opposite holds for other food related attitudes and behaviours like involvement with cooking and variety in diet. The findings seem to be of particular interest not only for marketers of food products, but also for food policy makers.

  14. Myasthenia Revealed Following Laparotomy - A Case Report

    Directory of Open Access Journals (Sweden)

    Abdelilah GHANNAM

    2015-06-01

    Full Text Available Myasthenia (muscle weakness is a rare neuromuscular disease of which respiratory failure is the main complication. The accidental discovery of such disease in the perioperative period is rare and potentially serious.We report a case of a woman who underwent emergency operation for appendiceal peritonitis, and failed repeatedly at weaning from postoperative mechanical ventilation. The usual etiologies such as postoperative respiratory complications, ventilator-associated pneumonia, acute respiratory distress syndrome complicating the septic shock or having no impact on it, and neuromyopathy’s resuscitation were considered, researched, examined or eliminated.Faced with the diagnostic impasse and the obvious weaning failure, another interview revealed signs of muscle fatigue which led to the diagnosis of myasthenia gravis decompensated perioperatively. Once the diagnosis was confirmed by means of a neostigmine test, the specific treatment began, particularly through plasma exchange sessions, and the process of weaning resumed. The result was complete weaning. A three-month follow-up showed a stable patient with no significant muscular disability.

  15. Balance Trees Reveal Microbial Niche Differentiation

    Science.gov (United States)

    Morton, James T.; Sanders, Jon; Quinn, Robert A.; McDonald, Daniel; Gonzalez, Antonio; Vázquez-Baeza, Yoshiki; Navas-Molina, Jose A.; Metcalf, Jessica L.; Hyde, Embriette R.; Lladser, Manuel; Dorrestein, Pieter C.

    2017-01-01

    ABSTRACT Advances in sequencing technologies have enabled novel insights into microbial niche differentiation, from analyzing environmental samples to understanding human diseases and informing dietary studies. However, identifying the microbial taxa that differentiate these samples can be challenging. These issues stem from the compositional nature of 16S rRNA gene data (or, more generally, taxon or functional gene data); the changes in the relative abundance of one taxon influence the apparent abundances of the others. Here we acknowledge that inferring properties of individual bacteria is a difficult problem and instead introduce the concept of balances to infer meaningful properties of subcommunities, rather than properties of individual species. We show that balances can yield insights about niche differentiation across multiple microbial environments, including soil environments and lung sputum. These techniques have the potential to reshape how we carry out future ecological analyses aimed at revealing differences in relative taxonomic abundances across different samples. IMPORTANCE By explicitly accounting for the compositional nature of 16S rRNA gene data through the concept of balances, balance trees yield novel biological insights into niche differentiation. The software to perform this analysis is available under an open-source license and can be obtained at https://github.com/biocore/gneiss. Author Video: An author video summary of this article is available. PMID:28144630

  16. Revealing atropisomer axial chirality in drug discovery.

    Science.gov (United States)

    LaPlante, Steven R; Edwards, Paul J; Fader, Lee D; Jakalian, Araz; Hucke, Oliver

    2011-03-07

    An often overlooked source of chirality is atropisomerism, which results from slow rotation along a bond axis due to steric hindrance and/or electronic factors. If undetected or not managed properly, this time-dependent chirality has the potential to lead to serious consequences, because atropisomers can be present as distinct enantiomers or diastereoisomers with their attendant different properties. Herein we introduce a strategy to reveal and classify compounds that have atropisomeric chirality. Energy barriers to axial rotation were calculated using quantum mechanics, from which predicted high barriers could be experimentally validated. A calculated rotational energy barrier of 20 kcal mol(-1) was established as a suitable threshold to distinguish between atropisomers and non-atropisomers with a prediction accuracy of 86%. This methodology was applied to subsets of drug databases in the course of which atropisomeric drugs were identified. In addition, some drugs were exposed that were not yet known to have this chiral attribute. The most valuable utility of this tool will be to predict atropisomerism along the drug discovery pathway. When used in concert with our compound classification scheme, decisions can be made during early discovery stages such as "hit-to-lead" and "lead optimization," to foresee and validate the presence of atropisomers and to exercise options of removing, further stabilizing, or rendering the chiral axis of interest more freely rotatable via SAR design, thereby decreasing this potential liability within a compound series. The strategy can also improve drug development plans, such as determining whether a drug or series should be developed as a racemic mixture or as an isolated single compound. Moreover, the work described herein can be extended to other chemical fields that require the assessment of potential chiral axes.

  17. Quantitative proteomics reveals cellular targets of celastrol.

    Directory of Open Access Journals (Sweden)

    Jakob Hansen

    Full Text Available Celastrol, a natural substance isolated from plant extracts used in traditional Chinese medicine, has been extensively investigated as a possible drug for treatment of cancer, autoimmune diseases, and protein misfolding disorders. Although studies focusing on celastrol's effects in specific cellular pathways have revealed a considerable number of targets in a diverse array of in vitro models there is an essential need for investigations that can provide a global view of its effects. To assess cellular effects of celastrol and to identify target proteins as biomarkers for monitoring treatment regimes, we performed large-scale quantitative proteomics in cultured human lymphoblastoid cells, a cell type that can be readily prepared from human blood samples. Celastrol substantially modified the proteome composition and 158 of the close to 1800 proteins with robust quantitation showed at least a 1.5 fold change in protein levels. Up-regulated proteins play key roles in cytoprotection with a prominent group involved in quality control and processing of proteins traversing the endoplasmic reticulum. Increased levels of proteins essential for the cellular protection against oxidative stress including heme oxygenase 1, several peroxiredoxins and thioredoxins as well as proteins involved in the control of iron homeostasis were also observed. Specific analysis of the mitochondrial proteome strongly indicated that the mitochondrial association of certain antioxidant defense and apoptosis-regulating proteins increased in cells exposed to celastrol. Analysis of selected mRNA transcripts showed that celastrol activated several different stress response pathways and dose response studies furthermore showed that continuous exposure to sub-micromolar concentrations of celastrol is associated with reduced cellular viability and proliferation. The extensive catalog of regulated proteins presented here identifies numerous cellular effects of celastrol and constitutes

  18. Quadrupole transitions revealed by Borrmann spectroscopy.

    Science.gov (United States)

    Pettifer, Robert F; Collins, Stephen P; Laundy, David

    2008-07-10

    The Borrmann effect-a dramatic increase in transparency to X-ray beams-is observed when X-rays satisfying Bragg's law diffract through a perfect crystal. The minimization of absorption seen in the Borrmann effect has been explained by noting that the electric field of the X-ray beam approaches zero amplitude at the crystal planes, thus avoiding the atoms. Here we show experimentally that under conditions of absorption suppression, the weaker electric quadrupole absorption transitions are effectively enhanced to such a degree that they can dominate the absorption spectrum. This effect can be exploited as an atomic spectroscopy technique; we show that quadrupole transitions give rise to additional structure at the L(1), L(2) and L(3) absorption edges of gadolinium in gadolinium gallium garnet, which mark the onset of excitations from 2s, 2p(1/2) and 2p(3/2) atomic core levels, respectively. Although the Borrmann effect served to underpin the development of the theory of X-ray diffraction, this is potentially the most important experimental application of the phenomenon since its first observation seven decades ago. Identifying quadrupole features in X-ray absorption spectroscopy is central to the interpretation of 'pre-edge' spectra, which are often taken to be indicators of local symmetry, valence and atomic environment. Quadrupolar absorption isolates states of different symmetries to that of the dominant dipole spectrum, and typically reveals orbitals that dominate the electronic ground-state properties of lanthanides and 3d transition metals, including magnetism. Results from our Borrmann spectroscopy technique feed into contemporary discussions regarding resonant X-ray diffraction and the nature of pre-edge lines identified by inelastic X-ray scattering. Furthermore, because the Borrmann effect has been observed in photonic materials, it seems likely that the quadrupole enhancement reported here will play an important role in modern optics.

  19. Frantic activity revealed in dusty stellar factories

    Science.gov (United States)

    2009-01-01

    Thanks to the Very Large Telescope's acute and powerful near-infrared eye, astronomers have uncovered a host of new young, massive and dusty stellar nurseries in nearby galaxy NGC 253. The centre of this galaxy appears to harbour a twin of our own Milky Way's supermassive black hole. ESO PR Photo 02a/09 The Spiral Galaxy NGC 253 Astronomers from the Instituto de Astrofísica de Canarias (Spain) used NACO, a sharp-eyed adaptive optics instrument on ESO's Very Large Telescope (VLT), to study the fine detail in NGC 253, one of the brightest and dustiest spiral galaxies in the sky. Adaptive Optics (AO) corrects for the blurring effect introduced by the Earth's atmosphere. This turbulence causes the stars to twinkle in a way that delights poets, but frustrates astronomers, since it smears out the images. With AO in action the telescope can produce images that are as sharp as is theoretically possible, as if the telescope were in space. NACO revealed features in the galaxy that were only 11 light-years across. "Our observations provide us with so much spatially resolved detail that we can, for the first time, compare them with the finest radio maps for this galaxy -- maps that have existed for more than a decade," says Juan Antonio Fernández-Ontiveros, the lead author of the paper reporting the results [1]. Astronomers identified 37 distinct bright regions, a threefold increase on previous results, packed into a tiny region at the core of the galaxy, comprising just one percent of the galaxy's total size. The astronomers combined their NACO images with data from another VLT instrument, VISIR, as well as with images from the NASA/ESA Hubble Space Telescope and radio observations made by the Very Large Array and the Very Large Baseline Interferometer. Combining these observations, taken in different wavelength regimes, provided a clue to the nature of these regions. "We now think that these are probably very active nurseries that contain many stars bursting from their

  20. Dramatic Outburst Reveals Nearest Black Hole

    Science.gov (United States)

    2000-01-01

    Sgr. The radio observations revealed the presence of a jet escaping from the system at mind-boggling speeds. Only three other galactic X-ray stellar systems have been found to eject material at such speeds. They have been dubbed "microquasars" because, on a smaller scale, they resemble quasars, which lie at the hearts of distant galaxies and also spew out high-velocity jets of particles. In galaxy-core quasars, the black holes are millions of times more massive than the Sun; in the more nearby microquasars the black holes are roughly three to twenty times more massive than the Sun. The extremely high velocity of the jets suggests that their origin lies close to the event horizon of a black hole. Microquasar activity is thought to arise when the black hole in the binary system draws material away from its companion star. The material surrounding the black hole forms a rapidly spinning disk called an accretion disk. This disk is heated by friction to millions of degrees, causing it to emit X-rays. As spiralling gas moves into the gravity well of the black hole, it moves faster and faster. Magnetic fields in the disk are believed to expel the charged subatomic particles at speeds close to that of light. As the charged particles interact with the magnetic fields, they emit radio waves. If some of the material escapes by being magnetically expelled into space, the matter may continue moving at the tremendous speed it had attained near the black hole. After their ejection, the jets of particles expand and cool, fading from astronomers' view. V4641 Sgr excites astronomers because it is close and because it acted so differently from other microquasars. In other microquasars, outbursts have dimmed more slowly over weeks or months rather than hours. "There's something fundamentally different about this one; it's more extreme than any other example," Hjellming said. "And because this system happens to be so close to us, `it is very likely that there are more objects like V4641

  1. Microradiometers Reveal Ocean Health, Climate Change

    Science.gov (United States)

    2013-01-01

    When NASA researcher Stanford Hooker is in the field, he pays close attention to color. For Hooker, being in the field means being at sea. On one such research trip to the frigid waters of the Arctic, with a Coast Guard icebreaker looming nearby and the snow-crusted ice shelf a few feet away, Hooker leaned over the edge of his small boat and lowered a tethered device into the bright turquoise water, a new product devised by a NASA partner and enabled by a promising technology for oceanographers and atmospheric scientists alike. Color is a function of light. Pure water is clear, but the variation in color observed during a visit to the beach or a flight along a coastline depends on the water s depth and the constituents in it, how far down the light penetrates and how it is absorbed and scattered by dissolved and suspended material. Hooker cares about ocean color because of what it can reveal about the health of the ocean, and in turn, the health of our planet. "The main thing we are interested in is the productivity of the water," Hooker says. The seawater contains phytoplankton, microscopic plants, which are the food base for the ocean s ecosystems. Changes in the water s properties, whether due to natural seasonal effects or human influence, can lead to problems for delicate ecosystems such as coral reefs. Ocean color can inform researchers about the quantities and distribution of phytoplankton and other materials, providing clues as to how the world ocean is changing. NASA s Coastal Zone Color Scanner, launched in 1978, was the first ocean color instrument flown on a spacecraft. Since then, the Agency s ocean color research capabilities have become increasingly sophisticated with the launch of the SeaWiFS instrument in 1997 and the twin MODIS instruments carried into orbit on NASA s Terra (1999) and Aqua (2002) satellites. The technology provides sweeping, global information on ocean color on a scale unattainable by any other means. One issue that arises from

  2. Categorisering van wegen en gewenste rijsnelheden : een discussienota ten behoeve van de Werkgroep "Rijsnelheid" van Rijkswaterstaat.

    NARCIS (Netherlands)

    Janssen, S.T.M.C. Roszbach, R. Noordzij, P.C. & Wesemann, P.

    1992-01-01

    The Ministry of Transport and Public Works Working Group `Driving speed' was commissioned to perform an exploration into a desirable speed limit per road category. For this purpose propositions are made for a classification system with accompanying speed limits for the road network in The Netherland

  3. Samenhang tussen ongevallenrisico, snelheid en wegkenmerken op 80km/uur-wegen : een steekproefstudie.

    NARCIS (Netherlands)

    Aarts, L.T. & Schagen, I.NL.G. van

    2007-01-01

    The relation between crash rate, speed and road features on 80 km/h roads; a cross-sectional study. This exploratory study investigates the relation between crash rate, speed, and several features of 80 km/h roads. We carried out this study with driving speed and crash data available to us from anot

  4. De veiligheid van bermen en wegen : een beschouwing van de stand van zaken.

    NARCIS (Netherlands)

    Heijer, T.

    1992-01-01

    By order of the Dienst Verkeerskunde of the Ministry of Transport and Public Works, the current situation around the safety of road side safety constructions in the Netherlands and the possible consequences of a number of future developments were analyzed by SWOV Institute for Road Safety Research.

  5. 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 Barb

  6. Improving air quality (policy) around roads; Naar een beter luchtkwaliteitsbeleid rond wegen

    Energy Technology Data Exchange (ETDEWEB)

    Van Dam, C.E.; Kruyt, H.A.; Dorel, F.G. [Directie Water en Milieu, Provincie Zuid-Holland, The Hague (Netherlands)

    1997-12-01

    Due to the ever increasing traffic in the Netherlands air quality around roads will remain poor, in spite of measures taken to reduce the emissions of both industry and traffic (e.g catalysts). To improve air quality policy, and thereby air quality, it is required (1) to integrate air quality policy with other environmental policy aspects at an early stage; (2) that the legal air quality standards are accounted for during the process of physical planning by municipalities and provinces; (3) that all three organisations who are involved in road-maintenance (state, province, municipality) also report about air pollution levels around those roads; (4) to develop a new mathematical model with which local air pollution can be reliably estimated for future situations (e.g. in 2010); (5) to introduce an air quality standard for fine dust. Although the emphasis in air quality policy should be on reducing emissions, it seems that in addition a remediation policy should be developed and implemented. This policy should aim at reducing the amount of people exposed to too much air pollution as much as possible. 8 refs.

  7. Natuurlijk sturen in Limburg : een kijkgedrag- en snelheidsonderzoek en een verkeerskundige analyse van twee aangepaste wegen.

    NARCIS (Netherlands)

    Vlakveld, W.P. Boele, M.J. Aarts, L.T. & Schermers, G.

    2013-01-01

    Natural traffic calming in the Dutch Province of Limburg : pilot study of two adapted roads. The continuous search for infrastructural measures to further improve road safety motivated and stimulated a number of regions to use more ‘natural’ measures and territorial characteristics. These natural

  8. Een categorie-indeling van wegen benaderd vanuit het aspect verkeersveiligheid.

    NARCIS (Netherlands)

    Janssen, S.T.M.C.

    1977-01-01

    Foreseeing the consequences when manoeuvring is easier when traffic situations are not too complex. This can be achieved by categorization of roads. Within one road category roads must be consistent, continuous, uniform and recognizable in their characteristics. The traffic area can be divided in

  9. De gemiddelde reissnelheid op auto (snel) wegen : kwantificering van vertragingen door diverse oorzaken.

    NARCIS (Netherlands)

    Burgt, G.J. van der Hammendorp, H.J. & Kampen, L.T.B. van

    1972-01-01

    This article discusses the results of a study of car average travelling speeds on highways in the Netherlands. Test runs were made with desired speeds of 80, 110, and 140 km/h after which time losses caused by road and traffic characteristics were analyzed. Recommendations are given for the design o

  10. Snelheid en verkeersonveiligheid op 80 km/uur-wegen : een literatuurstudie.

    NARCIS (Netherlands)

    Oei, H.-l.

    1990-01-01

    The aim of this literature review was to make an inventory of theoretical and empirical knowledge with regard to speed in its relationship to the characteristics of the highway, the traffic and the driver. Results show that speed control has great potential possibilities to improve traffic safety.

  11. 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.

  12. Natural history of chronic hepatitis B: what exactly has REVEAL revealed?

    Science.gov (United States)

    Iloeje, Uchenna H; Yang, Hwai-I; Chen, Chien-Jen

    2012-10-01

    Chronic hepatitis B virus (HBV) infection is a serious public health problem because of its worldwide prevalence and potential to cause adverse consequences. The Risk Evaluation of Viral Load Elevation and Associated Liver Disease/Cancer-Hepatitis B Virus (REVEAL-HBV) study carried out in Taiwan was used to investigate the natural history of chronic hepatitis B. The REVEAL-HBV study has established an HBV viral load paradigm in the natural history of chronic hepatitis B (CHB). Serum HBV DNA level has been shown to be significantly and independently associated with incidence of hepatocellular carcinoma (HCC) and cirrhosis and liver-related mortality across a biological gradient. It is also a major predictor of HBsAg seroclearance. Genetic features including HBV genotype and basal core promoter A1762T/G1764A mutant, and precore G1896A mutant were documented as predictors of HCC risk. Inactive HBV carriers still had an increased risk on HCC development and liver-related mortality compared with HBsAg -seronegatives. Nomograms focusing on facilitating risk communication between patients and clinicians were developed incorporating non-invasive clinical parameters to predict long-term HCC risk. These will hopefully contribute to evidence-based decisions in the clinical management of CHB patients. A somewhat provocative and novel finding from the REVEAL-HBV study is the association of chronic HBV infection in active replication with an increased pancreatic cancer risk especially in women less than 50 years old. This finding will hopefully spur further research in this area seeking confirmatory evidence. Finally, we hope that the REVEAL-HBV study will continue to be a source of data to answer other important questions in chronic hepatitis B research going forward. © 2012 John Wiley & Sons A/S.

  13. 21 CFR 1.21 - Failure to reveal material facts.

    Science.gov (United States)

    2010-04-01

    ... 21 Food and Drugs 1 2010-04-01 2010-04-01 false Failure to reveal material facts. 1.21 Section 1... GENERAL ENFORCEMENT REGULATIONS General Labeling Requirements § 1.21 Failure to reveal material facts. (a) Labeling of a food, drug, device, or cosmetic shall be deemed to be misleading if it fails to reveal...

  14. Invasive pulmonary mycosis due to Chaetomium globosum with false-positive galactomannan test: a case report and literature review.

    Science.gov (United States)

    Capoor, Malini R; Agarwal, Poojan; Goel, Manoj; Jain, Sarika; Shivaprakash, Mandya Rudramurthy; Honnavar, Prasanna; Gupta, Sunita; Chakrabarti, Arunaloke

    2016-03-01

    In this case, the authors report Chaetomium globosum as a cause of invasive pulmonary infection in a patient with Wegener's granulomatosis. Fungal hyphae (KOH and Calcofluor) were seen on direct microscopy of lung biopsy sample and bronchoalveolar lavage (BAL) sample. C. globosum isolated on culture clinched the diagnosis of invasive pulmonary infection by Chaetomium spp. A positive galactomannan of serum and BAL was repeatedly seen and was utilised for follow-up and as prognostic marker in patient management. The patient was successfully treated with liposomal amphotericin B followed by voriconazole. All the Chaetomium infections reported till date since 1980 are reviewed. Chaetomium spp. with its unique ecology has a hidden clinical potential to cause invasive mould infections.

  15. The inspiratory "squawk" in extrinsic allergic alveolitis and other pulmonary fibroses.

    Science.gov (United States)

    Earis, J E; Marsh, K; Pearson, M G; Ogilvie, C M

    1982-01-01

    An inspiratory musical sound ("squawk") was recorded in 14 patients with diffuse pulmonary fibrosis. These were divided into two groups: nine patients suffering from extrinsic allergic alveolitis (seven with bird fancier's lung and two with farmer's lung) and five patients with pulmonary fibrosis due to other causes, including rheumatoid disease, Wegener's granulomatosis, systemic sclerosis, and sarcoidosis. Clinical studies and phonopneumographic analysis of 10 consecutive squawks in each patient showed that the sound in the group with extrinsic allergic alveolitis was of shorter duration, occurred later in inspiration, and tended to be of higher frequency than the sound heard in the other group. Inspiratory crackles were present in all patients and in eight a single loud crackle preceded the squawk. We suggest that squawks, like crackles, result from the opening of airways and that the differences between the squawks in the two groups may reflect the size of the affected airways. PMID:7170682

  16. 以呕吐为首发症状的老年ANCA相关性血管炎两例分析

    Institute of Scientific and Technical Information of China (English)

    孙德珍

    2009-01-01

    @@ ANCA相关性血管炎(ANCA associated vasculitis) 主要包括韦格纳肉芽肿(wegener's granulomatosis,WG)和显微镜下多动脉炎(mycroscopic polyangilitis,MPA).因其与抗中性粒细胞胞浆抗体(ANCA)密切相关,故又称ANCA相关性系统性血管炎[1].本病多见于老年病人,高峰发病年龄为55~70岁[2].MPA是一种主要累及小血管的系统性坏死性血管炎,常表现为坏死性肾小球肾炎和肺毛细血管炎.临床上以血尿和咯血为主要症状.

  17. Imaging appearance of granulomatous lesions of head and neck

    Energy Technology Data Exchange (ETDEWEB)

    Abdel Razek, Ahmed Abdel Khalek, E-mail: arazek@mans.eun.e [Diagnostic Radiology Department, Mansoura Faculty of Medicine, 62 ElNokrsi Street-Meet Hadr, Mansoura (Egypt); Castillo, Mauricio [Department of Radiology, University of North Carolina, Chapel Hill, NC (United States)

    2010-10-15

    We aim to review the imaging appearance of granulomatous lesions of the head and neck. Granulomatous lesions are seen in different regions of the head and neck and a difficult diagnostic challenge for the radiologist. Infective granulomas may be due to bacterial or fungal agents. Non-infective granulomas are Wegener's granulomatosis, sarcoidosis, amyloidosis, chemical granuloma and reparative giant cell granuloma. Familiarity with the clinical presentation and imaging features of these lesions can suggest diagnosis in some cases. CT and MR imaging demonstrate the exact location, extension and effect on surrounding structures. A thorough knowledge of age, gender, common location, clinical features and imaging appearance of granulomatous lesions are important for diagnosis.

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

    OpenAIRE

    María Virginia Paolini; Juan Pablo Ruffino; Diego S. Fernández Romero

    2013-01-01

    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 fre...

  19. [Skin changes in rheumatic diseases].

    Science.gov (United States)

    Dobrić, Ivan

    2005-01-01

    The Intruduction includes those eflorescences that might be useful for diagnostics in rheumatology. Further in the text we have described four groups of rheumatic disorders. The first group: rheumatic diseases (lupus erythematosus, dermatomyositis, systemic scleroderma, the mixed connective tissue disease, allergic vasculitis, polyarteritis) which are the most common from the dermatological point of view. The second group: rheumatic diseases (Wegener's granulomatosis, rheumatoid arthritis, Sjögren, Reiter and Behçet syndrome and Kawasaki's disease) which are rarely of interest to our dermatologists. In this group there is also psoriatic arthritis, which is not rare in dermatology but its diagnostics and treatment belong to rheumatologists' field of expertise. The third group: infections (rheumatic fever, diseminated gonococcal infection, subacute bacterial endocarditis, Lyme disesease). The fourth group: metabolic disorders (gout). The diseases of the first group are described completely. In the second, third and fourth group of the diseases we have included only skin changes.

  20. [Implantable loop recorders of the Reveal family (Medtronic)].

    Science.gov (United States)

    Voss, Frederik

    2016-12-01

    This review explains the implantable loop recorders Medtronic Reveal XT and Medtronic Reveal LINQ. Technical specifications of the two devices are described in great detail. Additional tips for implantation as well as device programming are given including specific considerations of follow-up.

  1. Hypertrophic pachymeningitis: significance of myeloperoxidase anti-neutrophil cytoplasmic antibody.

    Science.gov (United States)

    Yokoseki, Akiko; Saji, Etsuji; Arakawa, Musashi; Kosaka, Takayuki; Hokari, Mariko; Toyoshima, Yasuko; Okamoto, Kouichirou; Takeda, Shigeki; Sanpei, Kazuhiro; Kikuchi, Hirotoshi; Hirohata, Shunsei; Akazawa, Kouhei; Kakita, Akiyoshi; Takahashi, Hitoshi; Nishizawa, Masatoyo; Kawachi, Izumi

    2014-02-01

    The aim of this study was to elucidate the characteristics, pathogenesis and treatment strategy of hypertrophic pachymeningitis that is associated with myeloperoxidase anti-neutrophil cytoplasmic antibody (ANCA). We retrospectively investigated clinical, radiological, immunological and pathological profiles of 36 patients with immune-mediated or idiopathic hypertrophic pachymeningitis, including 17 patients with myeloperoxidase-ANCA, four patients with proteinase 3-ANCA, six patients with other immune-mediated disorders, and nine patients with 'idiopathic' variety. Myeloperoxidase-ANCA-positive hypertrophic pachymeningitis was characterized by: (i) an elderly female predominance; (ii) 82% of patients diagnosed with granulomatosis with polyangiitis (previously known as Wegener's granulomatosis) according to Watts' algorithm; (iii) a high frequency of patients with lesions limited to the dura mater and upper airways, developing headaches, chronic sinusitis, otitis media or mastoiditis; (iv) a low frequency of patients with the 'classical or generalized form' of granulomatosis with polyangiitis involving the entire upper and lower airways and kidney, or progressing to generalized disease, in contrast to proteinase 3-ANCA-positive hypertrophic pachymeningitis; (v) less severe neurological damage according to the modified Rankin Scale and low disease activity according to the Birmingham Vasculitis Activity Score compared with proteinase 3-ANCA-positive hypertrophic pachymeningitis; (vi) increased levels of CXCL10, CXCL8 and interleukin 6 in cerebrospinal fluids, and increased numbers of T cells, neutrophils, eosinophils, plasma cells and monocytes/macrophages in autopsied or biopsied dura mater with pachymeningitis, suggesting TH1-predominant granulomatous lesions in hypertrophic pachymeningitis, as previously reported in pulmonary or renal lesions of granulomatosis with polyangiitis; and (vii) greater efficacy of combination therapy with prednisolone and

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

    Energy Technology Data Exchange (ETDEWEB)

    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.

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

    Directory of Open Access Journals (Sweden)

    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.

  4. Immunoadsorption in Autoimmune Diseases Affecting the Kidney.

    Science.gov (United States)

    Stummvoll, Georg; Aringer, Martin; Handisurya, Ammon; Derfler, Kurt

    2017-09-01

    Autoantibodies play an important role in the pathophysiology of renal involvement in systemic autoimmune diseases, such as systemic lupus erythematosus (SLE), systemic vasculitis, and anti-glomerular basement membrane disease (or Goodpasture syndrome). Direct removal of autoantibodies therefore has been tried in various ways, first by plasma exchange. Today, immunoadsorption is the extracorporeal method that most effectively removes (pathogenic) immune complexes and antibodies. Although past data have shown efficacy and biocompatibility of immunoadsorption in (renal) SLE, it is still an experimental and expensive procedure, and evidence from randomized controlled trials is needed. Nevertheless, immunoadsorption is being used as a rescue therapy in life-threatening situations of SLE patients because of its fast mode of action and its acceptable safety profile. In granulomatosis with polyangiitis (GPA) (or Wegener's granulomatosis), microscopic polyangiitis (MPA), and anti-glomerular basement membrane disease, the current standard is plasma exchange. Immunoadsorption, which probably would reduce the autoantibody burden more effectively, might be an even better more effective option, but sufficient evidence is lacking. Copyright © 2017 Elsevier Inc. All rights reserved.

  5. Systemic Lupus Erythematosus and hearing disorders: Literature review and meta-analysis of clinical and temporal bone findings.

    Science.gov (United States)

    Di Stadio, Arianna; Ralli, Massimo

    2017-01-01

    Objective This literature review and meta-analysis was performed to evaluate the correlations among hearing and vestibular clinical symptoms, temporal bone findings, and pathological mechanisms in patients with systemic lupus erythematosus (SLE). Study design Relevant papers in the literature were retrospectively reviewed. Clinical hearing aspects in patients with SLE and relevant temporal bone studies in the same field were analyzed. Methods PubMed and Google Scholar searches were performed using the following keywords: "auto-immune disease," "systemic lupus erythematosus (SLE)," "hearing loss," "temporal bone study," "vertigo," "dizziness," "tinnitus," "ear symptoms," "treatment," "diagnosis," "symptoms," "etiopathogenesis," "Wegener granulomatosis," "Sjogren," "polyarteritis nodosa," "Cogan syndrome," and "granulomatosis." Also included were reviews in which the following terms were present: "SLE," "temporal bone," and "hearing symptoms." Review and conclusion This literature review and meta-analysis focused on the pathological mechanisms through which SLE can damage inner ear structures and determinate hearing and vestibular symptoms. The main mechanisms involved in inner ear damage include the autoimmune response, deposition of immune complexes in the vessels and, to a lesser extent, cytotoxic damage.

  6. Childhood vasculitis hospitalizations in Spain, 1997-2011.

    Science.gov (United States)

    Villaverde-Hueso, A; Alonso, V; Morales-Piga, A; Hens-Pérez, M; Abaitua, I; Posada-de-la-Paz, M

    2014-05-01

    The aim of this study is to describe the childhood vasculitis hospital burden in Spain (1997-2011), considering type of disease, hospitalization rates and time trends. Data were obtained from the National Discharges Basic Minimum Data Set (National Patient Data Base). Inpatient events of children younger than 15 years of age were analyzed. Principal diagnosis of vasculitis were selected according Ninth Revision of the International Classification of Diseases: Takayasu arteritis, Polyarteritis nodosa, Kawasaki disease, Wegener`s granulomatosis, Churg-Strauss syndrome, and Henoch-Schönlein purpura. A total of 14518 children hospitalizations related to vasculitis were identified in Spain from 1997 to 2011. The average hospitalization rate for children was 13.33±1.71 per 100,000. Henoch-Schönlein purpura and Kawasaki disease were the most common type of vasculitis, hospitalization rates were 11.00 and 3.97 per 100,000 children, respectively. Other vasculitis hospitalizations are much rare in childhood. Average length of stay was 6.04 days and estimated cost per inpatient hospital care was 2,847€. Hospital case fatality rate was 0.05% for overall vasculitis. In conclusion, epidemiological data of childhood vasculitis are useful both to health decision-making and to identify research priorities.

  7. Thoracic vasculitis presenting as surgical problems.

    LENUS (Irish Health Repository)

    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.

  8. High-mobility group box-1 protein (HMGB1) is increased in antineutrophilic cytoplasmatic antibody (ANCA)-associated vasculitis with renal manifestations.

    Science.gov (United States)

    Bruchfeld, Annette; Wendt, Mårten; Bratt, Johan; Qureshi, Abdul R; Chavan, Sangeeta; Tracey, Kevin J; Palmblad, Karin; Gunnarsson, Iva

    2011-01-01

    High-mobility group box 1 (HMGB1) is a nuclear and cytosolic protein that is increasingly recognized as an important proinflammatory mediator actively secreted from monocytes and macrophages and passively released from necrotic cells. In antineutrophilic cytoplasmatic antibody (ANCA)-associated vasculitis (AAV), the kidneys are commonly affected vital organs, characterized by focal necrotizing and/or crescentic pauci-immune glomerulonephritis. The aim of the study was to determine whether HMGB1 serum levels are elevated in AAV with renal manifestations. A total of 30 AAV patients (16 female and 14 male; median age 59 years, range 17-82) with Wegener granulomatosis, microscopic polyangiitis and Churg-Strauss syndrome with available renal biopsies and serum samples were included. In seven cases, serum was also obtained at rebiopsy in remission. HMGB1 was analyzed with Western blot. Birmingham Vasculitis Activity Score (BVAS, version 2003), C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), urinanalysis, creatinine, estimated glomerular filtration rate, sex and age were included in the analysis. Twenty-five episodes of biopsy-proven active disease with BVAS 17.9 ± 4.6 and 13 cases with inactive biopsies and BVAS 2.3 ± 3.7 (P = 0.0001) were identified. CRP, ESR, hematuria and proteinuria were significantly higher in active cases. HMGB1 was significantly elevated (P = 0.01) comparing active with inactive cases (120 ± 48 versus 78 ± 46 ng/mL) and significantly lower in the seven control patients (P = 0.03) at rebiopsy in remission. HMGB1 remained higher in inactive cases compared with historic healthy controls (10.9 ± 10.5 ng/mL). HMGB1 levels did not differ significantly between AAV subgroups. CRP and ESR did not correlate with HMGB1. HMGB1 is significantly increased in AAV with renal involvement. Residual HMGB1 elevation in remission could possibly reflect low-grade inflammatory activity or tissue damage. Future studies may further reveal whether HMGB

  9. [Eosinophilic pneumonia revealing B-cell non-Hodgkin lymphoma].

    Science.gov (United States)

    Fikal, Siham; Sajiai, Hafsa; Serhane, Hind; Aitbatahar, Salma; Amro, Lamyae

    2016-01-01

    The diagnosis of eosinophilic pneumonia is rare and malignant etiology remains exceptional. Eosinophilic pneumonia etiology varies and is mainly dominated by allergic and drug causes. We report the case of a 61-year-old patient with B-cell non-Hodgkin lymphoma revealed by eosinophilic pneumonia. The diagnosis of eosinophilic pneumonia was confirmed by eosinophil count of 56% in bronchoalveolar lavage. Immunohistochemical examination of bone marrow biopsy revealed malignant Small B cells non-Hodgkin lymphoma.

  10. Eye movement monitoring reveals differential influences of emotion on memory

    OpenAIRE

    Lily Riggs; McQuiggan, Douglas A.; Anderson, Adam K.; Ryan, Jennifer D.

    2010-01-01

    Research shows that memory for emotional aspects of an event may be enhanced at the cost of impaired memory for surrounding peripheral details. However, this has only been assessed directly via verbal reports which reveal the outcome of a long stream of processing but cannot shed light on how/when emotion may affect the retrieval process. In the present experiment, eye movement monitoring was used as an indirect measure of memory as it can reveal aspects of online memory processing. For ex...

  11. Eye Movement Monitoring Reveals Differential Influences of Emotion on Memory

    OpenAIRE

    Riggs, Lily; McQuiggan, Douglas A.; Anderson, Adam K.; Ryan, Jennifer D.

    2010-01-01

    Research shows that memory for emotional aspects of an event may be enhanced at the cost of impaired memory for surrounding peripheral details. However, this has only been assessed directly via verbal reports which reveal the outcome of a long stream of processing but cannot shed light on how/when emotion may affect the retrieval process. In the present experiment, eye movement monitoring (EMM) was used as an indirect measure of memory as it can reveal aspects of online memory processing. For...

  12. 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/.

  13. ON THE AXIOMS OF REVEALED PREFERENCE IN FUZZY CONSUMER THEORY

    Institute of Scientific and Technical Information of China (English)

    Irina GEORGESCU

    2004-01-01

    The revealed preference is a central subject in classical consumer theory. Authors like Samuelson, Arrow, Richter, Sen, Uzawa and others have proposed an axiomatic setting of revealed preference theory. Consequently revealed preference axioms WARP and SARP and congruence axioms WCA and SCA have been considered. An important theorem of Sen establishes the equivalence between these axioms provided thefamily of budgets includes all non-empty finite sets of bundles. Fuzzy consumer theory (=fuzzy choice functions) is a topic that appears in a lot of papers.Particularly, Banerjee studies in fuzzy context axioms of revealed preference and congruence extending some results of Arrow and Sen. In this paper we modify the Banerjee definition of a fuzzy choice function (=fuzzy consumer)and we study some fuzzy versions of the axioms of revealed preference and congruence. Banerjee fuzzifies only the range of a consumer; we use a fuzzification of both the domain and the range of a consumer. The axioms WAFRP, SAFRP, WFCA, SFCA generalize to fuzzy consumer theory the well-known axioms WARP, SARP, WCA, SCA. Our main result establishes some connections between WAFRP, SAFRP, WFCA, SFCA extending a significant part of Sen theorem. Generally, we work in a fuzzy set theory based on a continuous t-norm, but some results are obtained for Godel t-norm and others are obtained for Lukasiewicz t-norm.

  14. Revealing physical interaction networks from statistics of collective dynamics

    Science.gov (United States)

    Nitzan, Mor; Casadiego, Jose; Timme, Marc

    2017-01-01

    Revealing physical interactions in complex systems from observed collective dynamics constitutes a fundamental inverse problem in science. Current reconstruction methods require access to a system’s model or dynamical data at a level of detail often not available. We exploit changes in invariant measures, in particular distributions of sampled states of the system in response to driving signals, and use compressed sensing to reveal physical interaction networks. Dynamical observations following driving suffice to infer physical connectivity even if they are temporally disordered, are acquired at large sampling intervals, and stem from different experiments. Testing various nonlinear dynamic processes emerging on artificial and real network topologies indicates high reconstruction quality for existence as well as type of interactions. These results advance our ability to reveal physical interaction networks in complex synthetic and natural systems. PMID:28246630

  15. More on Measuring the Overall Revealed Comparative Advantage

    Directory of Open Access Journals (Sweden)

    Veselin Hadzhiev

    2014-11-01

    Full Text Available The overall revealed comparative advantages approach aims to summarize the divergent expression of comparative advantages by commodity groups and countries. It represents an alternative to the traditional approach of Bella Balassa, Thomas Vollrath and others. The innovativeness of the overall revealed comparative advantages approach preconditions a certain prudence with regard to its use and interpretation. It is necessary to explore the potential of the approach in different conditions and different circumstances. It was found that the approach of overall revealed comparative advantages is resistant to the Euclidean distances and commodity classifications used for metrification. It has the potential to synthesize uniformly divergent changes in specialization by country and commodity groups and is a useful tool for the analysis of comparative advantages.

  16. 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.

  17. Acute Myocarditis in a Patient with Newly Diagnosed Granulomatosis with Polyangiitis

    DEFF Research Database (Denmark)

    Munch, Anne; Sundbøll, Jens; Høyer, Søren;

    2015-01-01

    (MRI) showed findings consistent with myocarditis but the etiology of the apical hypokinesis could not be determined with certainty and may well have been due to a myocardial infarction, a notion supported by a coronary angiogram displaying slow flow in the territory of the left anterior descending...... artery. Finally, an endomyocardial biopsy confirmed the diagnosis of myocarditis. The cardiac symptoms subsided upon treatment with high-dose prednisolone and rituximab....

  18. A young lady presented with limited pulmonary Wegener′s granulomatosis

    Directory of Open Access Journals (Sweden)

    Dey Atin

    2008-01-01

    Full Text Available A 19 year old female college student presented with fever, dry cough, chest pain, blood tinged sputum with subsequent development of polyarthralgia with radiological evidence of bilateral multiple unevenly distributed pulmonary nodular opacities with cavitation. There was no other systemic involvement and the patient was cytoplasmic antineutrophil antibody (c-ANCA positive with more than four times the normal upper limit of anti PR3 antibody. Excellent response to oral steroid with antimicrobial agent "trimethoprim - sulphamethoxazole" was noted.

  19. ID-check: Online concealed information test reveals true identity

    NARCIS (Netherlands)

    Verschuere, B.; Kleinberg, B.

    2016-01-01

    The Internet has already changed people's lives considerably and is likely to drastically change forensic research. We developed a web-based test to reveal concealed autobiographical information. Initial studies identified a number of conditions that affect diagnostic efficiency. By combining these

  20. Natural Disasters that Reveal Cracks in Our Social Foundation

    Science.gov (United States)

    Langer, Nieli

    2004-01-01

    The recent deaths of more than 13,000 French elderly in the European heat wave of 2003 revealed cracks in the social foundation of urban communities, here and abroad. The breakdown occurred in community services, neighborhood networks, and governmental agencies that were responsible for warning of impending dangers to at-risk elderly. This paper…

  1. On galaxy spiral arms' nature as revealed by rotation frequencies

    NARCIS (Netherlands)

    Roca-Fabrega, Santi; Valenzuela, Octavio; Figueras, Francesca; Romero-Gomez, Merce; Velazquez, Hector; Antoja Castelltort, Teresa; Pichardo, Barbara

    2013-01-01

    High-resolution N-body simulations using different codes and initial condition techniques reveal two different behaviours for the rotation frequency of transient spiral arms like structures. Whereas unbarred discs present spiral arms nearly corotating with disc particles, strong barred models (bulge

  2. UTV Expansion Pack: Special-Purpose Rank-Revealing Algorithms

    DEFF Research Database (Denmark)

    Fierro, Ricardo D.; Hansen, Per Christian

    2005-01-01

    This collection of Matlab 7.0 software supplements and complements the package UTV Tools from 1999, and includes implementations of special-purpose rank-revealing algorithms developed since the publication of the original package. We provide algorithms for computing and modifying symmetric rank-r...

  3. Genome-Wide Scan Reveals Mutation Associated with Melanoma

    Science.gov (United States)

    ... Q R S T U V W X Y Z We want to hear from you You are here: News & Events 2017 2016 2015 2014 2013 2012 2011 2010 2009 2008 2007 2006 2005 2004 2003 2002 2001 2000 1999 Spotlight on Research 2012 July 2012 (historical) Genome-Wide Scan Reveals Mutation Associated with Melanoma A team of ...

  4. Nilaja Sun's "No Child"...: Revealing Teaching and Learning through Theater

    Science.gov (United States)

    Hetland, Lois

    2009-01-01

    This article presents an analysis of Nilaja Sun's one-woman play, "No Child" . . ., that applies the Studio Habits of Mind framework to reveal essential features of great teaching artistry and great teaching. The play conveys much about twenty-first century schools and the policies that control them; about respect, equity, justice, and the lack of…

  5. Integrated genomics of Mucorales reveals novel therapeutic targets

    Science.gov (United States)

    Mucormycosis is a life-threatening infection caused by Mucorales fungi. We sequenced 30 fungal genomes and performed transcriptomics with three representative Rhizopus and Mucor strains with human airway epithelial cells during fungal invasion to reveal key host and fungal determinants contributing ...

  6. The Basics of How to Reveal Epilepsy--Part Two

    Science.gov (United States)

    Mittan, Robert J.

    2009-01-01

    In the April 2009 edition of "Exceptional Parent," Part One of this series explored why, for their own emotional well-being, it is so important for parents to tell others about their or their child's epilepsy. This month's installment will discuss the basics of how to reveal epilepsy to others, including some additional advantages one receives in…

  7. Revealing Hanny's Voorwerp : radio observations of IC 2497

    NARCIS (Netherlands)

    Jozsa, G. I. G.; Garrett, M. A.; Oosterloo, T. A.; Rampadarath, H.; Paragi, Z.; van Arkel, H.; Lintott, C.; Keel, W. C.; Schawinski, K.; Edmondson, E.

    2009-01-01

    We present multi-wavelength radio observations in the direction of the spiral galaxy IC 2497 and the neighbouring emission nebula known as "Hanny's Voorwerp". Our WSRT continuum observations at 1.4 GHz and 4.9 GHz reveal the presence of extended emission at the position of the nebulosity, although t

  8. Chemical milling solution reveals stress corrosion cracks in titanium alloy

    Science.gov (United States)

    Braski, D. N.

    1967-01-01

    Solution of hydrogen flouride, hydrogen peroxide, and water reveals hot salt stress corrosion cracks in various titanium alloys. After the surface is rinsed in water, dried, and swabbed with the solution, it can be observed by the naked eye or at low magnification.

  9. Mitochondrial specialization revealed by single muscle fiber proteomics

    DEFF Research Database (Denmark)

    Schiaffino, S; Reggiani, C; Kostrominova, T Y

    2015-01-01

    We have developed a highly sensitive mass spectrometry-based proteomic workflow to examine the proteome of single muscle fibers. This study revealed significant differences in the mitochondrial proteome of the four major fiber types present in mouse skeletal muscle. Here, we focus on Krebs cycle ...... scavenging capacity to cope with the higher levels of reactive oxygen species production....

  10. Thrombotic Microangiopathy Revealing Bone Metastases from an Ethmoid Sinus Carcinoma

    Directory of Open Access Journals (Sweden)

    Mony Chenda Morisse

    2016-08-01

    Full Text Available Cancer-related thrombotic microangiopathy (TMA is a rare entity whose clinical and biological characteristics have been described in various tumors. Here we describe the first case of cancer-related TMA revealing diffuse bone metastases from an ethmoid sinus carcinoma.

  11. Revealing Hanny's Voorwerp : radio observations of IC 2497

    NARCIS (Netherlands)

    Jozsa, G. I. G.; Garrett, M. A.; Oosterloo, T. A.; Rampadarath, H.; Paragi, Z.; van Arkel, H.; Lintott, C.; Keel, W. C.; Schawinski, K.; Edmondson, E.

    We present multi-wavelength radio observations in the direction of the spiral galaxy IC 2497 and the neighbouring emission nebula known as "Hanny's Voorwerp". Our WSRT continuum observations at 1.4 GHz and 4.9 GHz reveal the presence of extended emission at the position of the nebulosity, although

  12. Intracranial hemorrhage revealing pseudohypoparathyroidism as a cause of fahr syndrome.

    Science.gov (United States)

    Swami, Abhijit; Kar, Giridhari

    2011-01-01

    Pseudohypoparathyroidism is an infrequently encountered disease. It is one of the causes of Fahr syndrome which also is a rare clinical entity caused by multiple diseases. A 4-year-old man hospitalized for sudden onset left hemiparesis and hypertension was diagnosed to have right thalamic and midbrain hemorrhage on plain CT scan of the head which also revealed co-existent extensive intracranial calcifications involving the basal ganglia and cerebellum bilaterally. General physical examination revealed features of Albright hereditary osteodystrophy, goitre, hypertension, left hemiparesis, and signs of cerebellar dysfunction. Laboratory findings suggested hypocalcemia, hyperphosphatemia along with high TSH, low FT(4), low FT(3), and high anti-TPO antibody. Though bilateral intracranial calcifications are usually encountered as an incidental radiological finding in the CT scan of brain, in this case, the patient admitted for thalamic and midbrain hemorrhage was on investigation for associated intracranial calcification, and goitre was also found to have coexisting pseudohypoparathyroidism and autoimmune hypothyroidism.

  13. Genome Polymorphisms Between Indica and Japonica Revealed by RFLP

    Institute of Scientific and Technical Information of China (English)

    WANG Song-wen; LIU Xia; XU Cai-guo; SHI Li-li; ZHANG Xin; DING De-liang; WANG Yong

    2007-01-01

    Revealing the genome polymorphisms between indica and japonica subspecies; RFLP markers, which are located across 12 chromosomes of rice, were used to analyze indica-japonica differentiation in different rice varieties. At the same time, genome sequence variations of screened loci were analyzed by bioinformatics method. Twenty-eight RFLP probes, which can classify indica-japonica rice, were confirmed. Subspecies genome polymorphisms of screened loci were found by analyzing the publication of the genome sequences data of rice. The study indicated that these screened markers can be used for classifying indica-japonica subspecies. With the publication of the genome sequences of rice, marker polymorphisms between indica and japonica subspecies can be revealed by genome differentiation.

  14. UTV Expansion Pack: Special-Purpose Rank-Revealing Algorithms

    DEFF Research Database (Denmark)

    Fierro, Ricardo D.; Hansen, Per Christian

    2005-01-01

    This collection of Matlab 7.0 software supplements and complements the package UTV Tools from 1999, and includes implementations of special-purpose rank-revealing algorithms developed since the publication of the original package. We provide algorithms for computing and modifying symmetric rank......-revealing VSV decompositions, we expand the algorithms for the ULLV decomposition of a matrix pair to handle interference-type problems with a rank-deficient covariance matrix, and we provide a robust and reliable Lanczos algorithm which - despite its simplicity - is able to capture all the dominant singular...... values of a sparse or structured matrix. These new algorithms have applications in signal processing, optimization and LSI information retrieval....

  15. Maps of random walks on complex networks reveal community structure.

    Science.gov (United States)

    Rosvall, Martin; Bergstrom, Carl T

    2008-01-29

    To comprehend the multipartite organization of large-scale biological and social systems, we introduce an information theoretic approach that reveals community structure in weighted and directed networks. We use the probability flow of random walks on a network as a proxy for information flows in the real system and decompose the network into modules by compressing a description of the probability flow. The result is a map that both simplifies and highlights the regularities in the structure and their relationships. We illustrate the method by making a map of scientific communication as captured in the citation patterns of >6,000 journals. We discover a multicentric organization with fields that vary dramatically in size and degree of integration into the network of science. Along the backbone of the network-including physics, chemistry, molecular biology, and medicine-information flows bidirectionally, but the map reveals a directional pattern of citation from the applied fields to the basic sciences.

  16. Comparative Genomics Reveals High Genomic Diversity in the Genus Photobacterium

    DEFF Research Database (Denmark)

    Machado, Henrique; Gram, Lone

    2017-01-01

    Vibrionaceae is a large marine bacterial family, which can constitute up to 50% of the prokaryotic population in marine waters. Photobacterium is the second largest genus in the family and we used comparative genomics on 35 strains representing 16 of the 28 species described so far, to understand...... the genomic diversity present in the Photobacterium genus. Such understanding is important for ecophysiology studies of the genus. We used whole genome sequences to evaluate phylogenetic relationships using several analyses (16S rRNA, MLSA, fur, amino-acid usage, ANI), which allowed us to identify two...... misidentified strains. Genome analyses also revealed occurrence of higher and lower GC content clades, correlating with phylogenetic clusters. Pan-and core-genome analysis revealed the conservation of 25% of the genome throughout the genus, with a large and open pan-genome. The major source of genomic diversity...

  17. Systematic toxicological analysis revealing a rare case of captan ingestion.

    Science.gov (United States)

    Gottzein, Anne K; Musshoff, Frank; Madea, Burkhard

    2013-07-01

    This article presents a case of suicide by intoxication with various pharmaceuticals, particularly anticonvulsants, combined with the fungicide captan. A cause of death could not be ascertained at autopsy. However, systematic toxicological analysis (STA) including a screening via solid-phase microextraction (SPME) and gas chromatography-mass spectrometry (GC-MS) for (semi) volatile organic compounds revealed results suggesting a possible cause of death. The effects of captan on the human organism, its metabolism, and distribution will be discussed. Macroscopically, the cause of death was unascertained. STA revealed clonazepam, citalopram, and its metabolites, lamotrigine, levetiracetam, lacosamide, clonazepam, captan, and its metabolite tetrahydrophthalimide (THPI). For the first time, it was detected in human viscera. A quantification of THPI was performed to obtain distribution in the organs. The significance of a complete STA must be emphasized. The presence of THPI would have been missed without previous detection of captan. Consequently, this fatality would not have been investigated satisfactorily.

  18. Benign Cystic Peritoneal Mesothelioma Revealed by Small Bowel Obstruction.

    Science.gov (United States)

    Bray Madoué, Kaimba; Boniface, Moifo; Annick Laure, Edzimbi; Pierre, Herve

    2016-01-01

    Benign cystic peritoneal mesothelioma is a rare tumor which frequently occurs in women of reproductive age. Abdominal pain associated with pelvic or abdominal mass is the common clinical presentation. We report the case of a 22-year-old woman with a pathological proved benign cystic mesothelioma of the peritoneum revealed by a small bowel obstruction and a painful left-sided pelvic mass with signs of psoitis. Contrast enhanced abdominal CT-scan demonstrated a large pelvic cystic mass with mass effect on rectosigmoid and pelvic organs. The patient underwent surgical removal of the tumor. Pathological examination revealed the diagnosis of benign cystic mesothelioma of the peritoneum. The outcome was excellent with a 12-month recoil.

  19. Acting without seeing: eye movements reveal visual processing without awareness.

    Science.gov (United States)

    Spering, Miriam; Carrasco, Marisa

    2015-04-01

    Visual perception and eye movements are considered to be tightly linked. Diverse fields, ranging from developmental psychology to computer science, utilize eye tracking to measure visual perception. However, this prevailing view has been challenged by recent behavioral studies. Here, we review converging evidence revealing dissociations between the contents of perceptual awareness and different types of eye movement. Such dissociations reveal situations in which eye movements are sensitive to particular visual features that fail to modulate perceptual reports. We also discuss neurophysiological, neuroimaging, and clinical studies supporting the role of subcortical pathways for visual processing without awareness. Our review links awareness to perceptual-eye movement dissociations and furthers our understanding of the brain pathways underlying vision and movement with and without awareness.

  20. Revealing how network structure affects accuracy of link prediction

    Science.gov (United States)

    Yang, Jin-Xuan; Zhang, Xiao-Dong

    2017-08-01

    Link prediction plays an important role in network reconstruction and network evolution. The network structure affects the accuracy of link prediction, which is an interesting problem. In this paper we use common neighbors and the Gini coefficient to reveal the relation between them, which can provide a good reference for the choice of a suitable link prediction algorithm according to the network structure. Moreover, the statistical analysis reveals correlation between the common neighbors index, Gini coefficient index and other indices to describe the network structure, such as Laplacian eigenvalues, clustering coefficient, degree heterogeneity, and assortativity of network. Furthermore, a new method to predict missing links is proposed. The experimental results show that the proposed algorithm yields better prediction accuracy and robustness to the network structure than existing currently used methods for a variety of real-world networks.

  1. Intracranial Hemorrhage Revealing Pseudohypoparathyroidism as a Cause of Fahr Syndrome

    Directory of Open Access Journals (Sweden)

    Abhijit Swami

    2011-01-01

    Full Text Available Pseudohypoparathyroidism is an infrequently encountered disease. It is one of the causes of Fahr syndrome which also is a rare clinical entity caused by multiple diseases. A 4-year-old man hospitalized for sudden onset left hemiparesis and hypertension was diagnosed to have right thalamic and midbrain hemorrhage on plain CT scan of the head which also revealed co-existent extensive intracranial calcifications involving the basal ganglia and cerebellum bilaterally. General physical examination revealed features of Albright hereditary osteodystrophy, goitre, hypertension, left hemiparesis, and signs of cerebellar dysfunction. Laboratory findings suggested hypocalcemia, hyperphosphatemia along with high TSH, low FT4, low FT3, and high anti-TPO antibody. Though bilateral intracranial calcifications are usually encountered as an incidental radiological finding in the CT scan of brain, in this case, the patient admitted for thalamic and midbrain hemorrhage was on investigation for associated intracranial calcification, and goitre was also found to have coexisting pseudohypoparathyroidism and autoimmune hypothyroidism.

  2. Dermatomyositis revealing breast cancer: report of a case.

    Science.gov (United States)

    Lamquami, Safae; Errarhay, Sanae; Mamouni, Nisrine; Bouchikhi, Chahrazad; Banani, Abdelaziz

    2015-01-01

    Dermatomyositis (DM) is a rare connective corresponding to an inflammatory disease of skeletal muscles. Paraneoplastic origin must always be sought, primarily gynecological tumor in women, but the investigations are often made difficult by the fact that a primary tumor is often not detectable at the time of the cutaneous manifestations. This approach includes in addition to the monitoring report at regular intervals of 6 to 12 months for two years after diagnosis. We report a case of Dermatomyositis revealing breast cancer.

  3. Monofractal nature of air temperature signals reveals their climate variability

    OpenAIRE

    Deliège, Adrien; Nicolay, Samuel

    2014-01-01

    We use the discrete "wavelet transform microscope" to show that the surface air temperature signals of weather stations selected in Europe are monofractal. This study reveals that the information obtained in this way are richer than previous works studying long range correlations in meteorological stations. The approach presented here allows to bind the H\\"older exponents with the climate variability. We also establish that such a link does not exist with methods previously carried out.

  4. Diabetic acido-ketosis revealing thiamine-responsive megaloblastic anemia.

    Science.gov (United States)

    Bouyahia, O; Ouderni, M; Ben Mansour, F; Matoussi, N; Khaldi, F

    2009-12-01

    Thiamine-responsive megaloblastic anemia (TRMA) is a rare autosomal recessive disorder characterized by megaloblastic anemia, diabetes mellitus and progressive sensorineural deafness. We report the cases of two infants, aged 4 and 5 months, hospitalized for diabetic ketoacidosis requiring insulin therapy. Laboratory tests revealed megaloblasic anemia, thrombocytopenia and normal thiamine level. Neurosensorial investigations showed bilateral deafness and ophthalmic involvement. Treatment with oral thiamine normalized hematological disorders and controlled diabetes; however, thiamine therapy had no impact on neurosensorial disorders.

  5. Revealing Children's Experiences and Emotions through Q Methodology

    OpenAIRE

    Ingunn T. Ellingsen; Arlene Arstad Thorsen; Ingunn Størksen

    2014-01-01

    Over the last two decades, there has been a greater readiness to view children as competent contributors to our understanding of children’s lives and experiences. As a consequence of this, we have witnessed an increased focus on including children in research. When research aims at revealing children’s perspectives, experiences, and emotions, we need to employ methods that are easy-to-use means for obtaining their stories. In this paper, we argue that Q methodology is particularly suitable fo...

  6. Proposal for revealing quantum nonlocality via local contextuality.

    Science.gov (United States)

    Cabello, Adán

    2010-06-04

    Two distant systems can exhibit quantum nonlocality even though the correlations between them admit a local model. This nonlocality can be revealed by testing extra correlations between successive measurements on one of the systems which do not admit a noncontextual model whatever the reduced state of this system is. This shows that quantum contextuality plays a fundamental role in quantum nonlocality, and allows an experimental test of the Kochen-Specker with locality theorem.

  7. De relatie tussen snelheidslimiet en verkeersveiligheid : onwikkeling van de verkeersonveiligheid op 50- en 80km/uur-wegen vergeleken met die op 30- en 60km/uur-wegen.

    NARCIS (Netherlands)

    Braimaister, L. Bos, N.M. Kars, V. & Stipdonk, H.L.

    2013-01-01

    The relation between speed and road safety : the development of road safety on 50 and 80 km/h roads compared with the development on 30 and 60 km/h roads. Since the implementation of the Start-up Programme Sustainable Safety from 1998 onward, the speed limit on a considerable length of urban access

  8. De relatie tussen snelheidslimiet en verkeersveiligheid : onwikkeling van de verkeersonveiligheid op 50- en 80km/uur-wegen vergeleken met die op 30- en 60km/uur-wegen.

    NARCIS (Netherlands)

    Braimaister, L. Bos, N.M. Kars, V. & Stipdonk, H.L.

    2013-01-01

    The relation between speed and road safety : the development of road safety on 50 and 80 km/h roads compared with the development on 30 and 60 km/h roads. Since the implementation of the Start-up Programme Sustainable Safety from 1998 onward, the speed limit on a considerable length of urban access

  9. Genes but not genomes reveal bacterial domestication of Lactococcus lactis.

    Directory of Open Access Journals (Sweden)

    Delphine Passerini

    Full Text Available BACKGROUND: The population structure and diversity of Lactococcus lactis subsp. lactis, a major industrial bacterium involved in milk fermentation, was determined at both gene and genome level. Seventy-six lactococcal isolates of various origins were studied by different genotyping methods and thirty-six strains displaying unique macrorestriction fingerprints were analyzed by a new multilocus sequence typing (MLST scheme. This gene-based analysis was compared to genomic characteristics determined by pulsed-field gel electrophoresis (PFGE. METHODOLOGY/PRINCIPAL FINDINGS: The MLST analysis revealed that L. lactis subsp. lactis is essentially clonal with infrequent intra- and intergenic recombination; also, despite its taxonomical classification as a subspecies, it displays a genetic diversity as substantial as that within several other bacterial species. Genome-based analysis revealed a genome size variability of 20%, a value typical of bacteria inhabiting different ecological niches, and that suggests a large pan-genome for this subspecies. However, the genomic characteristics (macrorestriction pattern, genome or chromosome size, plasmid content did not correlate to the MLST-based phylogeny, with strains from the same sequence type (ST differing by up to 230 kb in genome size. CONCLUSION/SIGNIFICANCE: The gene-based phylogeny was not fully consistent with the traditional classification into dairy and non-dairy strains but supported a new classification based on ecological separation between "environmental" strains, the main contributors to the genetic diversity within the subspecies, and "domesticated" strains, subject to recent genetic bottlenecks. Comparison between gene- and genome-based analyses revealed little relationship between core and dispensable genome phylogenies, indicating that clonal diversification and phenotypic variability of the "domesticated" strains essentially arose through substantial genomic flux within the dispensable

  10. Transcriptome Reveals Cathepsin K in Periodontal Ligament Differentiation.

    Science.gov (United States)

    Yamada, S; Ozaki, N; Tsushima, K; Yamaba, S; Fujihara, C; Awata, T; Sakashita, H; Kajikawa, T; Kitagaki, J; Yamashita, M; Yanagita, M; Murakami, S

    2016-08-01

    Periodontal ligaments (PDLs) play an important role in remodeling the alveolar bond and cementum. Characterization of the periodontal tissue transcriptome remains incomplete, and an improved understanding of PDL features could aid in developing new regenerative therapies. Here, we aimed to generate and analyze a large human PDL transcriptome. We obtained PDLs from orthodontic treatment patients, isolated the RNA, and used a vector-capping method to make a complementary DNA library from >20,000 clones. Our results revealed that 58% of the sequences were full length. Furthermore, our analysis showed that genes expressed at the highest frequencies included those for collagen type I, collagen type III, and proteases. We also found 5 genes whose expressions have not been previously reported in human PDL. To access which of the highly expressed genes might be important for PDL cell differentiation, we used real-time polymerase chain reaction to measure their expression in differentiating cells. Among the genes tested, the cysteine protease cathepsin K had the highest upregulation, so we measured its relative expression in several tissues, as well as in osteoclasts, which are known to express high levels of cathepsin K. Our results revealed that PDL cells express cathepsin K at similar levels as osteoclasts, which are both expressed at higher levels than those of the other tissues tested. We also measured cathepsin K protein expression and enzyme activity during cell differentiation and found that both increased during this process. Immunocytochemistry experiments revealed that cathepsin K localizes to the interior of lysosomes. Last, we examined the effect of inhibiting cathepsin K during cell differentiation and found that cathepsin K inhibition stimulated calcified nodule formation and increased the levels of collagen type I and osteocalcin gene expression. Based on these results, cathepsin K seems to regulate collagen fiber accumulation during human PDL cell

  11. The features of Drosophila core promoters revealed by statistical analysis

    Directory of Open Access Journals (Sweden)

    Trifonov Edward N

    2006-06-01

    Full Text Available Abstract Background Experimental investigation of transcription is still a very labor- and time-consuming process. Only a few transcription initiation scenarios have been studied in detail. The mechanism of interaction between basal machinery and promoter, in particular core promoter elements, is not known for the majority of identified promoters. In this study, we reveal various transcription initiation mechanisms by statistical analysis of 3393 nonredundant Drosophila promoters. Results Using Drosophila-specific position-weight matrices, we identified promoters containing TATA box, Initiator, Downstream Promoter Element (DPE, and Motif Ten Element (MTE, as well as core elements discovered in Human (TFIIB Recognition Element (BRE and Downstream Core Element (DCE. Promoters utilizing known synergetic combinations of two core elements (TATA_Inr, Inr_MTE, Inr_DPE, and DPE_MTE were identified. We also establish the existence of promoters with potentially novel synergetic combinations: TATA_DPE and TATA_MTE. Our analysis revealed several motifs with the features of promoter elements, including possible novel core promoter element(s. Comparison of Human and Drosophila showed consistent percentages of promoters with TATA, Inr, DPE, and synergetic combinations thereof, as well as most of the same functional and mutual positions of the core elements. No statistical evidence of MTE utilization in Human was found. Distinct nucleosome positioning in particular promoter classes was revealed. Conclusion We present lists of promoters that potentially utilize the aforementioned elements/combinations. The number of these promoters is two orders of magnitude larger than the number of promoters in which transcription initiation was experimentally studied. The sequences are ready to be experimentally tested or used for further statistical analysis. The developed approach may be utilized for other species.

  12. Windows PowerShell desired state configuration revealed

    CERN Document Server

    Chaganti, Ravikanth

    2014-01-01

    Desired State Configuration (DSC) is a powerful new configuration management platform that makes it easier than ever to perform cross-platform configuration management of your infrastructure, whether on-premise or in the cloud. DSC provides the management platform and Application Programming Interface (API) that can be used with any programming language. Windows PowerShell Desired State Configuration Revealed will take you through this new technology from start to finish and demonstrates the DSC interfaces through Windows PowerShell. DSC allows you to manage target devices by simply declarin

  13. Linear stability analysis reveals exclusion zone for sliding bed transport

    Directory of Open Access Journals (Sweden)

    Talmon Arnold M.

    2015-06-01

    Full Text Available A bend or any another pipe component disturbs solids transport in pipes. Longitudinal pressure profiles downstream of such a component may show a stationary transient harmonic wave, as revealed by a recent settling slurry laboratory experiment. Therefore the fundamental transient response of the two-layer model for fully stratified flow is investigated as a first approach. A linear stability analysis of the sliding bed configuration is conducted. No stationary transient harmonic waves are found in this analysis, but adaptation lengths for exponential recovery are quantified. An example calculation is given for a 0.1 m diameter pipeline.

  14. Planck revealed bulk motion of Centaurus A lobes

    CERN Document Server

    De Paolis, F; Nucita, A A; Ingrosso, G; Kashin, A L; Khachatryan, H G; Mirzoyan, S; Yegorian, G; Jetzer, Ph; Qadir, A; Vetrugno, D

    2015-01-01

    Planck data towards the active galaxy Centaurus A are analyzed in the 70, 100 and 143 GHz bands. We find a temperature asymmetry of the northern radio lobe with respect to the southern one that clearly extends at least up to 5 degrees from the Cen A center and diminishes towards the outer regions of the lobes. That transparent parameter - the temperature asymmetry - thus has to carry a principal information, i.e. indication on the line-of-sight bulk motion of the lobes, while the increase of that asymmetry at smaller radii reveals the differential dynamics of the lobes as expected at ejections from the center.

  15. Sequencing of 50 human exomes reveals adaptation to high altitude

    DEFF Research Database (Denmark)

    Yi, Xin; Liang, Yu; Huerta-Sanchez, Emilia

    2010-01-01

    represent strong candidates for altitude adaptation, were identified. The strongest signal of natural selection came from endothelial Per-Arnt-Sim (PAS) domain protein 1 (EPAS1), a transcription factor involved in response to hypoxia. One single-nucleotide polymorphism (SNP) at EPAS1 shows a 78% frequency...... difference between Tibetan and Han samples, representing the fastest allele frequency change observed at any human gene to date. This SNP's association with erythrocyte abundance supports the role of EPAS1 in adaptation to hypoxia. Thus, a population genomic survey has revealed a functionally important locus...

  16. Revealing and Characterizing Dark Excitons Through Coherent Multidimensional Spectroscopy

    CERN Document Server

    Tollerud, Jonathan O; Davis, Jeffrey A

    2016-01-01

    Dark excitons are of fundamental importance in a broad range of contexts, but are difficult to study using conventional optical spectroscopy due to their weak interaction with light. We show how coherent multidimensional spectroscopy can reveal and characterize dark states. Using this approach, we identify different types of dark excitons in InGaAs/GaAs quantum wells and determine details regarding lifetimes, homogeneous and inhomogeneous linewidths, broadening mechanisms and coupling strengths. The observations of coherent coupling between bright and dark excitons hint at a role for a multi-step process by which excitons in the barrier can relax into the quantum wells.

  17. How the ``Blues'' reveals the intimacy of music and physics

    Science.gov (United States)

    Gibson, J. Murray

    2013-03-01

    Little do most people know when they hear blues piano - and you'll hear some live in this talk - that physics permeates the style, as it does all of music. Why should you care? By deconstructing blues piano the intimacy of physics, mathematics and music will be revealed in its glory.[1] The exercise says something about how the brains of the music composer and of the listener must be intimately linked to the physical principles of acoustics. And it provides a great vehicle to explain physical phenomena to non-scientists - everything from quantum mechanics to protein structure.

  18. Mediated amperometry reveals different modes of yeast responses to sugars.

    Science.gov (United States)

    Garjonyte, Rasa; Melvydas, Vytautas; Malinauskas, Albertas

    2016-02-01

    Menadione-mediated amperometry at carbon paste electrodes modified with various yeasts (Saccharomyces cerevisiae, Candida pulcherrima, Pichia guilliermondii and Debaryomyces hansenii) was employed to monitor redox activity inside the yeast cells induced by glucose, fructose, sucrose, maltose or galactose. Continuous measurements revealed distinct modes (transient or gradually increasing) of the current development during the first 2 to 3 min after subjection to glucose, fructose and sucrose at electrodes containing S. cerevisiae and non-Saccharomyces strains. Different modes (increasing or decreasing) of the current development after yeast subjection to galactose at electrodes with S. cerevisiae or D. hansenii and at electrodes with C. pulcherrima and P. guilliermondii suggested different mechanisms of galactose assimilation.

  19. [Mucocele of the ethmoid sinus revealed by an epiphora].

    Science.gov (United States)

    Précausta, F; Goga, D; Laure, B

    2013-11-01

    To report a case of anterior ethmoid mucocele with atypical location and ophthalmic symptomatology. A 18-year-old patient presented with epiphora and swelling of the left medial canthus; craniofacial scan revealed an ethmoid mucocele. We describe a surgical technique for the treatment of mucoceles, illustrated with CT and endoscopy images. Mucoceles present with various clinical and radiological manifestations, and in particular may have an ophthalmologic impact. Treatment is surgical, with various techniques available. Epiphora may be an unusual presentation of an ethmoid mucocele, due to lacrimal duct compression. Copyright © 2013 Elsevier Masson SAS. All rights reserved.

  20. Circumstellar Environments of MYSOs Revealed by IFU Spectroscopy

    Science.gov (United States)

    Navarete, F.; Damineli, A.; Barbosa, C. L.; Blum, R. D.

    2015-01-01

    Formation of massive stars (M > 8 M ⊙) is still not well understood and lacks of observational constraints. We observed 7 MYSO candidates using the NIFS spectrometer at Gemini North Telescope to study the accretion process at high angular resolution (~ 50 mas) and very closer to the central star. Preliminary results for 2 sources have revealed circumstellar structures traced by Brackett-Gamma, CO lines and extended H2 emission. Both sources present kinematics in the CO absorption lines, suggesting rotating structures. The next step will derive the central mass of each source by applying a keplerian model for these CO features.

  1. Malar Bone Metastasis Revealing a Papillary Thyroid Carcinoma

    Directory of Open Access Journals (Sweden)

    Ihsen Slim

    2012-01-01

    Full Text Available Papillary thyroid carcinoma is the most common form of differentiated thyroid carcinoma. It is generally confined to the neck with or without spread to regional lymph nodes. Metastatic thyroid carcinomas are uncommon and mainly include lung and bone. Metastases involving oral and maxillofacial region are extremely rare. We described a case of malar metastasis revealing a follicular variant of papillary thyroid carcinoma, presenting with pain and swelling of the left cheek in a 67-years-old female patient with an unspecified histological left lobo-isthmectomy medical history. To our knowledge, this is the first recorded instance of a malar metastasis from a follicular variant of papillary thyroid carcinoma.

  2. Recombination patterns reveal information about centromere location on linkage maps

    DEFF Research Database (Denmark)

    Limborg, Morten T.; McKinney, Garrett J.; Seeb, Lisa W.

    2016-01-01

    , approximate centromere placement is possible by phasing the same data used to generate linkage maps. Assuming one obligate crossover per chromosome arm, information about centromere location can be revealed by tracking the accumulated recombination frequency along linkage groups, similar to half....... mykiss) characterized by low and unevenly distributed recombination – a general feature of male meiosis in many species. Further, a high frequency of double crossovers along chromosome arms in barley reduced resolution for locating centromeric regions on most linkage groups. Despite these limitations......, our method should work well for high‐density maps in species with strong recombination interference and will enrich many existing and future mapping resources....

  3. IVT-seq reveals extreme bias in RNA sequencing

    OpenAIRE

    Kavaklı, Halil; Lahens, Nicholas F.; Zhang, Ray; Hayer, Katharina; Black, Michael B.; Dueck, Hannah; Pizarro, Angel; Kim, Junhyong; Irizarry, Rafael; Thomas, Russell S.; Grant, Gregory R.; Hogenesch, John B.

    2014-01-01

    RESEARCH Open Access IVT-seq reveals extreme bias in RNA sequencing Nicholas F Lahens1, Ibrahim Halil Kavakli2,3, Ray Zhang1, Katharina Hayer4, Michael B Black5, Hannah Dueck6, Angel Pizarro7, Junhyong Kim6, Rafael Irizarry8, Russell S Thomas5, Gregory R Grant4,9 and John B Hogenesch1* Abstract Background: RNA-seq is a powerful technique for identifying and quantifying transcription and splicing events, both known and novel. However, given its recent development and the prol...

  4. Revealing alteration of membrane structures during ischema using impedance spectroscopy

    Directory of Open Access Journals (Sweden)

    Mihaela Gheorghiu

    2002-11-01

    Full Text Available Alterations of membrane structure and function are essential characteristics of cells undergoing ischemia. Noninvasive monitoring of tissue alterations during ischemia and the estimation of the reversibility domain (corresponding to organ capability to fully recover its functions after shifting back to normal blood perfusion are important for biomedical applications allowing better time management during surgical interventions, especially in organ transplantation. Due to it’s capability to reveal inhomogeneities, as well as it’s noninvasive character, impedance spectroscopy was used for continuous monitoring of the progression of excised tissue samples during ischemia. We have developed a fast, noninvasive, automated method for quantitative analysis of impedance spectra of tissue samples, capable of revealing, through characteristic parameters (dispersion amplitudes, time constants and distribution parameters membrane based microscopic processes like the closure ofgap-junctions (a characteristic of the early alterations of ischemic tissues in the reversibility phase. Microscopic and equivalent circuit modeling was used to probe the effect of closure of cell connections and of changes in electrical properties of cell constituents on impedance spectra. We have developed a normalizing procedure emphasizing the pattern of ischemic alterations and enabling the comparison of different data sets.

  5. Microsatellite genotyping reveals a signature in breast cancer exomes.

    Science.gov (United States)

    McIver, L J; Fonville, N C; Karunasena, E; Garner, H R

    2014-06-01

    Genomic instability at microsatellite loci is a hallmark of many cancers, including breast cancer. However, much of the genomic variation and many of the hereditary components responsible for breast cancer remain undetected. We hypothesized that variation at microsatellites could provide additional genomic markers for breast cancer risk assessment. A total of 1,345 germline and tumor DNA samples from individuals diagnosed with breast cancer, exome sequenced as part of The Cancer Genome Atlas, were analyzed for microsatellite variation. The comparison group for our analysis, representing healthy individuals, consisted of 249 females which were exome sequenced as part of the 1,000 Genomes Project. We applied our microsatellite-based genotyping pipeline to identify 55 microsatellite loci that can distinguish between the germline of individuals diagnosed with breast cancer and healthy individuals with a sensitivity of 88.4 % and a specificity of 77.1 %. Further, we identified additional microsatellite loci that are potentially useful for distinguishing between breast cancer subtypes, revealing a possible fifth subtype. These findings are of clinical interest as possible risk diagnostics and reveal genes that may be of potential therapeutic value, including genes previously not associated with breast cancer.

  6. Diffusion tensor imaging reveals evolution of primate brain architectures.

    Science.gov (United States)

    Zhang, Degang; Guo, Lei; Zhu, Dajiang; Li, Kaiming; Li, Longchuan; Chen, Hanbo; Zhao, Qun; Hu, Xiaoping; Liu, Tianming

    2013-11-01

    Evolution of the brain has been an inherently interesting problem for centuries. Recent studies have indicated that neuroimaging is a powerful technique for studying brain evolution. In particular, a variety of reports have demonstrated that consistent white matter fiber connection patterns derived from diffusion tensor imaging (DTI) tractography reveal common brain architecture and are predictive of brain functions. In this paper, based on our recently discovered 358 dense individualized and common connectivity-based cortical landmarks (DICCCOL) defined by consistent fiber connection patterns in DTI datasets of human brains, we derived 65 DICCCOLs that are common in macaque monkey, chimpanzee and human brains and 175 DICCCOLs that exhibit significant discrepancies amongst these three primate species. Qualitative and quantitative evaluations not only demonstrated the consistencies of anatomical locations and structural fiber connection patterns of these 65 common DICCCOLs across three primates, suggesting an evolutionarily preserved common brain architecture but also revealed regional patterns of evolutionarily induced complexity and variability of those 175 discrepant DICCCOLs across the three species.

  7. Social Investment for Sustainability of Groundwater: A Revealed Preference Approach

    Directory of Open Access Journals (Sweden)

    Edna Tusak Loehman

    2014-08-01

    Full Text Available Groundwater is a form of natural capital that is valued for the goods it provides, including ecosystem health, water quality, and water consumption. Degradation of groundwater could be alleviated through social investment such as for water reuse and desalination to reduce the need for withdrawals from groundwater. This paper develops a participatory planning process—based on combining revealed preference with economic optimization—to choose a desired future for sustaining groundwater. Generation of potential groundwater futures is based on an optimal control model with investment and withdrawal from groundwater as control variables. In this model, groundwater stock and aquatic health are included as inter-temporal public goods. The social discount rate expressing time preference—an important parameter that drives optimization—is revealed through the participatory planning process. To implement the chosen future, a new method of inter-temporal pricing is presented to finance investment and supply costs. Furthermore, it is shown that the desired social outcome could be achieved by a form of privatization in which the pricing method, the appropriate discount rate, and the planning period are contractually specified.

  8. Differential metabolism of Mycoplasma species as revealed by their genomes

    Directory of Open Access Journals (Sweden)

    Fabricio B.M. Arraes

    2007-01-01

    Full Text Available The annotation and comparative analyses of the genomes of Mycoplasma synoviae and Mycoplasma hyopneumonie, as well as of other Mollicutes (a group of bacteria devoid of a rigid cell wall, has set the grounds for a global understanding of their metabolism and infection mechanisms. According to the annotation data, M. synoviae and M. hyopneumoniae are able to perform glycolytic metabolism, but do not possess the enzymatic machinery for citrate and glyoxylate cycles, gluconeogenesis and the pentose phosphate pathway. Both can synthesize ATP by lactic fermentation, but only M. synoviae can convert acetaldehyde to acetate. Also, our genome analysis revealed that M. synoviae and M. hyopneumoniae are not expected to synthesize polysaccharides, but they can take up a variety of carbohydrates via the phosphoenolpyruvate-dependent phosphotransferase system (PEP-PTS. Our data showed that these two organisms are unable to synthesize purine and pyrimidine de novo, since they only possess the sequences which encode salvage pathway enzymes. Comparative analyses of M. synoviae and M. hyopneumoniae with other Mollicutes have revealed differential genes in the former two genomes coding for enzymes that participate in carbohydrate, amino acid and nucleotide metabolism and host-pathogen interaction. The identification of these metabolic pathways will provide a better understanding of the biology and pathogenicity of these organisms.

  9. Economic choices reveal probability distortion in macaque monkeys.

    Science.gov (United States)

    Stauffer, William R; Lak, Armin; Bossaerts, Peter; Schultz, Wolfram

    2015-02-18

    Economic choices are largely determined by two principal elements, reward value (utility) and probability. Although nonlinear utility functions have been acknowledged for centuries, nonlinear probability weighting (probability distortion) was only recently recognized as a ubiquitous aspect of real-world choice behavior. Even when outcome probabilities are known and acknowledged, human decision makers often overweight low probability outcomes and underweight high probability outcomes. Whereas recent studies measured utility functions and their corresponding neural correlates in monkeys, it is not known whether monkeys distort probability in a manner similar to humans. Therefore, we investigated economic choices in macaque monkeys for evidence of probability distortion. We trained two monkeys to predict reward from probabilistic gambles with constant outcome values (0.5 ml or nothing). The probability of winning was conveyed using explicit visual cues (sector stimuli). Choices between the gambles revealed that the monkeys used the explicit probability information to make meaningful decisions. Using these cues, we measured probability distortion from choices between the gambles and safe rewards. Parametric modeling of the choices revealed classic probability weighting functions with inverted-S shape. Therefore, the animals overweighted low probability rewards and underweighted high probability rewards. Empirical investigation of the behavior verified that the choices were best explained by a combination of nonlinear value and nonlinear probability distortion. Together, these results suggest that probability distortion may reflect evolutionarily preserved neuronal processing. Copyright © 2015 Stauffer et al.

  10. The microbiome of Brazilian mangrove sediments as revealed by metagenomics.

    Directory of Open Access Journals (Sweden)

    Fernando Dini Andreote

    Full Text Available Here we embark in a deep metagenomic survey that revealed the taxonomic and potential metabolic pathways aspects of mangrove sediment microbiology. The extraction of DNA from sediment samples and the direct application of pyrosequencing resulted in approximately 215 Mb of data from four distinct mangrove areas (BrMgv01 to 04 in Brazil. The taxonomic approaches applied revealed the dominance of Deltaproteobacteria and Gammaproteobacteria in the samples. Paired statistical analysis showed higher proportions of specific taxonomic groups in each dataset. The metabolic reconstruction indicated the possible occurrence of processes modulated by the prevailing conditions found in mangrove sediments. In terms of carbon cycling, the sequences indicated the prevalence of genes involved in the metabolism of methane, formaldehyde, and carbon dioxide. With respect to the nitrogen cycle, evidence for sequences associated with dissimilatory reduction of nitrate, nitrogen immobilization, and denitrification was detected. Sequences related to the production of adenylsulfate, sulfite, and H(2S were relevant to the sulphur cycle. These data indicate that the microbial core involved in methane, nitrogen, and sulphur metabolism consists mainly of Burkholderiaceae, Planctomycetaceae, Rhodobacteraceae, and Desulfobacteraceae. Comparison of our data to datasets from soil and sea samples resulted in the allotment of the mangrove sediments between those samples. The results of this study add valuable data about the composition of microbial communities in mangroves and also shed light on possible transformations promoted by microbial organisms in mangrove sediments.

  11. Piriform spider silk sequences reveal unique repetitive elements.

    Science.gov (United States)

    Perry, David J; Bittencourt, Daniela; Siltberg-Liberles, Jessica; Rech, Elibio L; Lewis, Randolph V

    2010-11-08

    Orb-weaving spider silk fibers are assembled from very large, highly repetitive proteins. The repeated segments contain, in turn, short, simple, and repetitive amino acid motifs that account for the physical and mechanical properties of the assembled fiber. Of the six orb-weaver silk fibroins, the piriform silk that makes the attachment discs, which lashes the joints of the web and attaches dragline silk to surfaces, has not been previously characterized. Piriform silk protein cDNAs were isolated from phage libraries of three species: A. trifasciata , N. clavipes , and N. cruentata . The deduced amino acid sequences from these genes revealed two new repetitive motifs: an alternating proline motif, where every other amino acid is proline, and a glutamine-rich motif of 6-8 amino acids. Similar to other spider silk proteins, the repeated segments are large (>200 amino acids) and highly homogenized within a species. There is also substantial sequence similarity across the genes from the three species, with particular conservation of the repetitive motifs. Northern blot analysis revealed that the mRNA is larger than 11 kb and is expressed exclusively in the piriform glands of the spider. Phylogenetic analysis of the C-terminal regions of the new proteins with published spidroins robustly shows that the piriform sequences form an ortholog group.

  12. Ancient DNA sequence revealed by error-correcting codes.

    Science.gov (United States)

    Brandão, Marcelo M; Spoladore, Larissa; Faria, Luzinete C B; Rocha, Andréa S L; Silva-Filho, Marcio C; Palazzo, Reginaldo

    2015-07-10

    A previously described DNA sequence generator algorithm (DNA-SGA) using error-correcting codes has been employed as a computational tool to address the evolutionary pathway of the genetic code. The code-generated sequence alignment demonstrated that a residue mutation revealed by the code can be found in the same position in sequences of distantly related taxa. Furthermore, the code-generated sequences do not promote amino acid changes in the deviant genomes through codon reassignment. A Bayesian evolutionary analysis of both code-generated and homologous sequences of the Arabidopsis thaliana malate dehydrogenase gene indicates an approximately 1 MYA divergence time from the MDH code-generated sequence node to its paralogous sequences. The DNA-SGA helps to determine the plesiomorphic state of DNA sequences because a single nucleotide alteration often occurs in distantly related taxa and can be found in the alternative codon patterns of noncanonical genetic codes. As a consequence, the algorithm may reveal an earlier stage of the evolution of the standard code.

  13. High resolution FESEM and TEM reveal bacterial spore attachment.

    Science.gov (United States)

    Panessa-Warren, Barbara J; Tortora, George T; Warren, John B

    2007-08-01

    Transmission electron microscopy (TEM) studies in the 1960s and early 1970s using conventional thin section and freeze fracture methodologies revealed ultrastructural bacterial spore appendages. However, the limited technology at that time necessitated the time-consuming process of imaging serial sections and reconstructing each structure. Consequently, the distribution and function of these appendages and their possible role in colonization or pathogenesis remained unknown. By combining high resolution field emission electron microscopy with TEM images of identical bacterial spore preparations, we have been able to obtain images of intact and sectioned Bacillus and Clostridial spores to clearly visualize the appearance, distribution, resistance (to trypsin, chloramphenicol, and heat), and participation of these structures to facilitate attachment of the spores to glass, agar, and human cell substrates. Current user-friendly commercial field emission scanning electron microscopes (FESEMs), permit high resolution imaging, with high brightness guns at lower accelerating voltages for beam sensitive intact biological samples, providing surface images at TEM magnifications for making direct comparisons. For the first time, attachment structures used by pathogenic, environmental, and thermophile bacterial spores could be readily visualized on intact spores to reveal how specific appendages and outer spore coats participated in spore attachment, colonization, and invasion.

  14. Invoking Thomas Kuhn: What Citation Analysis Reveals about Science Education

    Science.gov (United States)

    Loving, Cathleen C.; Cobern, William W.

    This paper analyzes how Thomas Kuhn's writings are used by others, especially science education researchers. Previous research in citation analysis is used to frame questions related to who cites Kuhn, in what manner and why. Research questions first focus on the variety of disciplines invoking Kuhn and to what extent Structure of Scientific Revolutions (SSR) is cited. The Web of Science database provides material from 1982 for this analysis. The science education literature is analyzed using back issues from 1985 of the Journal of Research in Science Teaching and Science Education. An article analysis reveals trends in terms of what Kuhnian ideas are most frequently invoked. Results indicate a wide array of disciplines from beekeeping to law cite Kuhn - especially generic citations to SSR. The science education journal analysis reveals pervasive use of the term paradigm, although use is quite varied. The two areas of research in science education most impacted by Kuhn appear to be conceptual change theory and constructivist epistemologies. Additional uses of Kuhn are discussed. The degree to which Kuhn is invoked in ways supporting the theoretical framework of citation analysis, whether his work is misappropriated, and the impact of Kuhn are discussed.

  15. Membrane Protein Properties Revealed through Data-Rich Electrostatics Calculations.

    Science.gov (United States)

    Marcoline, Frank V; Bethel, Neville; Guerriero, Christopher J; Brodsky, Jeffrey L; Grabe, Michael

    2015-08-04

    The electrostatic properties of membrane proteins often reveal many of their key biophysical characteristics, such as ion channel selectivity and the stability of charged membrane-spanning segments. The Poisson-Boltzmann (PB) equation is the gold standard for calculating protein electrostatics, and the software APBSmem enables the solution of the PB equation in the presence of a membrane. Here, we describe significant advances to APBSmem, including full automation of system setup, per-residue energy decomposition, incorporation of PDB2PQR, calculation of membrane-induced pKa shifts, calculation of non-polar energies, and command-line scripting for large-scale calculations. We highlight these new features with calculations carried out on a number of membrane proteins, including the recently solved structure of the ion channel TRPV1 and a large survey of 1,614 membrane proteins of known structure. This survey provides a comprehensive list of residues with large electrostatic penalties for being embedded in the membrane, potentially revealing interesting functional information.

  16. Revealing Children's Experiences and Emotions through Q Methodology

    Directory of Open Access Journals (Sweden)

    Ingunn T. Ellingsen

    2014-01-01

    Full Text Available Over the last two decades, there has been a greater readiness to view children as competent contributors to our understanding of children’s lives and experiences. As a consequence of this, we have witnessed an increased focus on including children in research. When research aims at revealing children’s perspectives, experiences, and emotions, we need to employ methods that are easy-to-use means for obtaining their stories. In this paper, we argue that Q methodology is particularly suitable for facilitating children’s participation in research. We will give a brief presentation of the methodology and demonstrate its relevance for research with children. We do so by presenting two research examples aiming at revealing children’s experiences and emotions in challenging life circumstances. In the first example, Q methodology was used with five-year-old children where the research topic was divorce. The second example is a study on family perceptions among adolescents in foster home care.

  17. Blue whale earplug reveals lifetime contaminant exposure and hormone profiles.

    Science.gov (United States)

    Trumble, Stephen J; Robinson, Eleanor M; Berman-Kowalewski, Michelle; Potter, Charles W; Usenko, Sascha

    2013-10-15

    Lifetime contaminant and hormonal profiles have been reconstructed for an individual male blue whale (Balaenoptera musculus, Linnaeus 1758) using the earplug as a natural aging matrix that is also capable of archiving and preserving lipophilic compounds. These unprecedented lifetime profiles (i.e., birth to death) were reconstructed with a 6-mo resolution for a wide range of analytes including cortisol (stress hormone), testosterone (developmental hormone), organic contaminants (e.g., pesticides and flame retardants), and mercury. Cortisol lifetime profiles revealed a doubling of cortisol levels over baseline. Testosterone profiles suggest this male blue whale reached sexual maturity at approximately 10 y of age, which corresponds well with and improves on previous estimates. Early periods of the reconstructed contaminant profiles for pesticides (such as dichlorodiphenyltrichloroethanes and chlordanes), polychlorinated biphenyls, and polybrominated diphenyl ethers demonstrate significant maternal transfer occurred at 0-12 mo. The total lifetime organic contaminant burden measured between the earplug (sum of contaminants in laminae layers) and blubber samples from the same organism were similar. Total mercury profiles revealed reduced maternal transfer and two distinct pulse events compared with organic contaminants. The use of a whale earplug to reconstruct lifetime chemical profiles will allow for a more comprehensive examination of stress, development, and contaminant exposure, as well as improve the assessment of contaminant use/emission, environmental noise, ship traffic, and climate change on these important marine sentinels.

  18. NeuCode Proteomics Reveals Bap1 Regulation of Metabolism

    Directory of Open Access Journals (Sweden)

    Joshua M. Baughman

    2016-07-01

    Full Text Available We introduce neutron-encoded (NeuCode amino acid labeling of mice as a strategy for multiplexed proteomic analysis in vivo. Using NeuCode, we characterize an inducible knockout mouse model of Bap1, a tumor suppressor and deubiquitinase whose in vivo roles outside of cancer are not well established. NeuCode proteomics revealed altered metabolic pathways following Bap1 deletion, including profound elevation of cholesterol biosynthetic machinery coincident with reduced expression of gluconeogenic and lipid homeostasis proteins in liver. Bap1 loss increased pancreatitis biomarkers and reduced expression of mitochondrial proteins. These alterations accompany a metabolic remodeling with hypoglycemia, hypercholesterolemia, hepatic lipid loss, and acinar cell degeneration. Liver-specific Bap1 null mice present with fully penetrant perinatal lethality, severe hypoglycemia, and hepatic lipid deficiency. This work reveals Bap1 as a metabolic regulator in liver and pancreas, and it establishes NeuCode as a reliable proteomic method for deciphering in vivo biology.

  19. Ananke: temporal clustering reveals ecological dynamics of microbial communities

    Directory of Open Access Journals (Sweden)

    Michael W. Hall

    2017-09-01

    Full Text Available Taxonomic markers such as the 16S ribosomal RNA gene are widely used in microbial community analysis. A common first step in marker-gene analysis is grouping genes into clusters to reduce data sets to a more manageable size and potentially mitigate the effects of sequencing error. Instead of clustering based on sequence identity, marker-gene data sets collected over time can be clustered based on temporal correlation to reveal ecologically meaningful associations. We present Ananke, a free and open-source algorithm and software package that complements existing sequence-identity-based clustering approaches by clustering marker-gene data based on time-series profiles and provides interactive visualization of clusters, including highlighting of internal OTU inconsistencies. Ananke is able to cluster distinct temporal patterns from simulations of multiple ecological patterns, such as periodic seasonal dynamics and organism appearances/disappearances. We apply our algorithm to two longitudinal marker gene data sets: faecal communities from the human gut of an individual sampled over one year, and communities from a freshwater lake sampled over eleven years. Within the gut, the segregation of the bacterial community around a food-poisoning event was immediately clear. In the freshwater lake, we found that high sequence identity between marker genes does not guarantee similar temporal dynamics, and Ananke time-series clusters revealed patterns obscured by clustering based on sequence identity or taxonomy. Ananke is free and open-source software available at https://github.com/beiko-lab/ananke.

  20. Random field model reveals structure of the protein recombinational landscape.

    Directory of Open Access Journals (Sweden)

    Philip A Romero

    Full Text Available We are interested in how intragenic recombination contributes to the evolution of proteins and how this mechanism complements and enhances the diversity generated by random mutation. Experiments have revealed that proteins are highly tolerant to recombination with homologous sequences (mutation by recombination is conservative; more surprisingly, they have also shown that homologous sequence fragments make largely additive contributions to biophysical properties such as stability. Here, we develop a random field model to describe the statistical features of the subset of protein space accessible by recombination, which we refer to as the recombinational landscape. This model shows quantitative agreement with experimental results compiled from eight libraries of proteins that were generated by recombining gene fragments from homologous proteins. The model reveals a recombinational landscape that is highly enriched in functional sequences, with properties dominated by a large-scale additive structure. It also quantifies the relative contributions of parent sequence identity, crossover locations, and protein fold to the tolerance of proteins to recombination. Intragenic recombination explores a unique subset of sequence space that promotes rapid molecular diversification and functional adaptation.