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Wegener disease

James C Wang, Brittany A Leader, Ryan A Crane, Bernadette L Koch, Matthew M Smith, Stacey L Ishman
Granulomatosis with polyangiitis (GPA, previously known as Wegener's granulomatosis) is an autoimmune systemic small-vessel vasculitis, associated with the presence of anti-neurophil cytoplasmic antibodies with a cytoplasmic staining pattern (c-ANCA). It is characterized by necrotizing granulomas, usually affecting the airways and kidneys. GPA should be considered when patients do not improve despite adequate treatment of otologic symptoms, when patients have unspecific symptoms suggesting systemic disease (e...
April 2018: International Journal of Pediatric Otorhinolaryngology
Edward C Kuan, Kevin A Peng, Lyndon O Gonzalez, Joel A Sercarz
We report a rare case of squamous cell carcinoma (SCC) of the nasal cavity arising in a patient with granulomatosis with polyangiitis (GPA). The patient was a 35-year-old man who had been diagnosed 15 years earlier with GPA and treated medically for sinonasal, pulmonary, and renal involvement. He presented to us with left-sided orbital and cheek pain and nasal obstruction. Endoscopy detected a friable, exophytic mass that involved the left lateral nasal wall and septum. Biopsy analysis identified the mass as an SCC...
January 2018: Ear, Nose, & Throat Journal
Vineeta Shobha, Saba Fathima, Ravi Prakash
Granulomatosis with polyangiitis (GPA) previously known as Wegener's granulomatosis is one of the forms of idiopathic systemic vasculitis. There is very scanty data available on GPA in Asian and Indian population. We studied data of 60 patients from southern India, diagnosed with GPA to describe the physical characteristics, the treatment, and outcome. Patients who fulfilled any two of the four criteria proposed by the American College of Rheumatology, and those with clinical features of GPA with ANCA positivity and histopathological confirmation, were included in the study...
February 28, 2018: Clinical and Experimental Medicine
A I Krukov, E A Kirasirova, O K Piminidi, R A Rezakov, N V Lafutkina, R F Mamedov
The objective of the present study was to optimize the treatment of patients with subglottic laryngeal stenosis. MATERIAL AND METHODS: We have examined a total of 14 patients presenting with subglottic laryngeal stenosis. The etiological factors and underlying conditions of laryngeal stenosis were the prolonged intubation in 4 patients, Wegener granulomatosis in 6 patients, and idiopathic stenosis of the larynx in 4 patients. All the patients underwent balloon dilatation with the application of the video endoscopic technique...
2018: Vestnik Otorinolaringologii
Sandra Tillmann, Hussain M Awwad, Amanda R Eskelund, Giulia Treccani, Juergen Geisel, Gregers Wegener, Rima Obeid
SCOPE: Probiotics may influence one-carbon (C1) metabolism, neurotransmitters, liver function markers, or behavior. METHODS AND RESULTS: Male adult Flinders Sensitive Line rats (model of depression, FSL; n = 22) received Lactobacillus helveticus R0052 and Bifidobacterium longum R0175 (10 9 or 10 10 colony-forming units/d) or vehicle for 10 weeks. The controls, Flinders Resistant Line rats (FRL, n = 8), only received vehicle. C1-related metabolites were measured in plasma, urine, and different tissues...
February 17, 2018: Molecular Nutrition & Food Research
Ali Safavi Naini, Jahangir Ghorbani, Sima Montazer Lotfe Elahi, Mohsen Beigomi
Introduction: Granulomatosis with polyangiitis (GPA; also known as Wegener's granulomatosis) is a primary systemic vasculitis involving the ear, nose and throat system (ENT) and lower respiratory tract. Because of the lack of knowledge regarding the clinical findings of GPA due to the limited number of studies, the current study was designed to investigate the prevalence and nature of the otology manifestations in the disease course. Materials and Methods: In the current prospective study, patients with a diagnosis of GPA from 2012-2016 were included...
November 2017: Iranian Journal of Otorhinolaryngology
Jacques-Eric Gottenberg, Thomas Dörner, Hendrika Bootsma, Valérie Devauchelle-Pensec, Simon J Bowman, Xavier Mariette, Holger Bartz, Marga Oortgiesen, Anthony Shock, Willem Koetse, Catrinel Galateanu, Sabine Bongardt, William A Wegener, David M Goldenberg, Guy Meno-Tetang, Gordana Kosutic, Caroline Gordon
OBJECTIVE: EMBODY 1 (NCT01262365) and EMBODY 2 (NCT01261793) investigated the efficacy and safety of epratuzumab, a CD22-targeted humanized monoclonal IgG1 antibody, in patients with systemic lupus erythematosus (SLE). The studies showed no significant difference from placebo in primary or secondary clinical outcome measures, but did demonstrate B-cell-specific immunological activity. METHODS: Efficacy and safety of epratuzumab were compared between two patient subpopulations randomized in EMBODY 1 & 2: SLE patients with a diagnosis of associated Sjögren's syndrome (aSjS) and without aSjS (non-aSjS patients)...
January 30, 2018: Arthritis & Rheumatology
Darren Patrick Moloney, Liam Chawke, Mairead Therese Crowley, Terence M O'Connor
Hypercalcaemia occurs in many granulomatous diseases. Among them, sarcoidosis and tuberculosis are the most common causes. Other causes include berylliosis, coccidioidomycosis, histoplasmosis, Crohn's disease, silicone-induced granulomas, cat-scratch disease, Wegener's granulomatosis and Pneumocystis carinii pneumonia. Hypercalcaemia in granulomatous disease occurs as a consequence of dysregulated production of 1,25-(OH)2 D3 (calcitriol) by activated macrophages in granulomas. Hypercalcaemia in patients with Mycobacterium tuberculosis infection has been reported in 0%-28% of cases...
January 26, 2018: BMJ Case Reports
Jorn Fierstra, Christiaan van Niftrik, Geoffrey Warnock, Susanne Wegener, Marco Piccirelli, Athina Pangalu, Giuseppe Esposito, Antonios Valavanis, Alfred Buck, Andreas Luft, Oliver Bozinov, Luca Regli
BACKGROUND AND PURPOSE: Increased stroke risk correlates with hemodynamic failure, which can be assessed with (15O-)H2O positron emission tomography (PET) cerebral blood flow (CBF) measurements. This gold standard technique, however, is not established for routine clinical imaging. Standardized blood oxygen-level-dependent (BOLD) functional magnetic resonance imaging+CO2 is a noninvasive and potentially widely applicable tool to assess whole-brain quantitative cerebrovascular reactivity (CVR)...
January 25, 2018: Stroke; a Journal of Cerebral Circulation
O Syniachenko, O Khaniukov, Ye Yehudina, H Taktashov, K Gashynova
The purpose of the study - investigation the separate joint lesion in systemic vasculitis, their X-ray sonographic characteristics, the correlation of the articular syndrome severity with extra-articular manifestations of the diseases, as well as aspects of the arthritis pathogenesis in this category of patients. The study included 525 patients in the ratio of the examined with Henoch-Schonlen purpura, microscopic polyangiitis, cryoglobulinemic vasculitis, polyarteritis nodosa, Takayasu's arteritis, Wegener's granulomatosis with polyangiitis and eosinophilic granulomatosis with polyangiitis Churg-Strauss as a 7:4:3:1:1:1:1...
December 2017: Georgian Medical News
Mariana O Perez, Ricardo M Oliveira, Mauricio Levy-Neto, Valeria F Caparbo, Rosa M R Pereira
OBJECTIVES: To determine the possible association of serum 25-hydroxyvitamin D (25OHD) levels with disease activity and respiratory infection in granulomatosis with polyangiitis patients during two different periods: winter/spring and summer/autumn. METHODS: Thirty-two granulomatosis with polyangiitis patients were evaluated in the winter/spring, and the same patients (except 5) were evaluated in summer/autumn (n=27). The 25OHD levels were measured by radioimmunoassay...
December 2017: Clinics
Marie-Claire Arrieta, Andrea Arévalo, Leah Stiemsma, Pedro Dimitriu, Martha E Chico, Sofia Loor, Maritza Vaca, Rozlyn C T Boutin, Evan Morien, Mingliang Jin, Stuart E Turvey, Jens Walter, Laura Wegener Parfrey, Philip J Cooper, Brett Finlay
BACKGROUND: Asthma is the most prevalent chronic disease of childhood. Recently, we identified a critical window early in the life of both mice and Canadian infants during which gut microbial changes (dysbiosis) affect asthma development. Given geographic differences in human gut microbiota worldwide, we studied the effects of gut microbial dysbiosis on atopic wheeze in a population living in a distinct developing world environment. OBJECTIVE: We sought to determine whether microbial alterations in early infancy are associated with the development of atopic wheeze in a nonindustrialized setting...
November 30, 2017: Journal of Allergy and Clinical Immunology
Rahil Giti, Massood Hosseinzadeh
Flexible fiberoptic bronchoscopy is often the initial technique for diagnosis of lung and bronchial tumors. Many studies have shown the high accuracy rate of bronchial washing and brushing cytology in the evaluation of neoplastic and non-neoplastic bronchopulmonary lesions. The aim of this study is to emphasize the value of the bronchial cytologic findings for diagnosis of non-neoplastic bronchopulmonary lesions. In a cross-sectional study, we retrieved all cases with bronchial washing and brushing cytology from 21 Mach 2014 to 21 December 2015...
October 2017: Acta Medica Iranica
Gokhan Sargin, Taskin Senturk
The first International Chapel Hill Consensus Conference was held in 1994. There have been suggestions about the nomenclature of systemic vasculitis. Important categories were added to the classification of vasculitis, and many changes were made for disease names in the second Chapel Hill Consensus Conference 2012, which were not included in the Chapel Hill Consensus Conference 1994. The new nomenclature was introduced instead of being referred to by many names such as Churg-Strauss and Wegener"s. New categories such as Behçet"s and Cogan etc...
November 2017: Revista Brasileira de Reumatologia
Ires Ghielen, Erwin E H van Wegen, Sonja Rutten, Cees J T de Goede, Marieke Houniet-de Gier, Emma H Collette, Ingrid A L Burgers-Bots, Jos W R Twisk, Gert Kwakkel, Kees Vermunt, Bep van Vliet, Henk W Berendse, Odile A van den Heuvel
BACKGROUND: In Parkinson's disease (PD) patients, fluctuations in symptoms commonly occur after many years of dopamine replacement therapy. The so-called wearing-off phenomenon exists of both motor and non-motor symptoms, such as rigidity and anxiety. Current treatment options are limited and an integrated approach is needed to address the complex interactions between motor and non-motor symptoms. Since wearing-off is eventually inevitable, treatment needs to focus on coping, acceptance and self-efficacy...
December 2017: Journal of Psychosomatic Research
Eunhye Lee, Jynkyun Park, Seung Hong Choi, Sung-Hye Park
Granulomatosis with polyangiitis (GPA), previously known as Wegener's granulomatosis (WG), is a rare systemic disease characterized by necrotizing granulomas and vasculitis that usually presents in the respiratory tract and renal system. Here, we report a case of anti-neutrophil cytoplasmic antibody (ANCA)-negative GPA with diagnostic difficulties. Neurologic involvement in GPA is not uncommon, with up to 50% of patients manifesting with neurologic defects; however, it is usually a late event. This patient was a 50-year-old man whose first manifestation was multiple neurologic defects without respiratory or renal symptoms...
November 14, 2017: Neuropathology: Official Journal of the Japanese Society of Neuropathology
Marius Wegener, Michaela Müller-McNicoll
Nuclear retention of incompletely spliced or mature mRNAs emerges as a novel, previously underappreciated layer of gene regulation, which enables the cell to rapidly respond to stress, viral infection, differentiation cues or changing environmental conditions. Focusing on mammalian cells, we discuss recent insights into the mechanisms and functions of nuclear retention, describe retention-promoting features in protein-coding transcripts and propose mechanisms for their regulated release into the cytoplasm. Moreover, we discuss examples of how aberrant nuclear retention of mRNAs may lead to human diseases...
November 21, 2017: Seminars in Cell & Developmental Biology
Claudia Kohl, Merle Wegener, Andreas Nitsche, Andreas Kurth
Outbreaks of infectious diseases may occur in animal and human populations; this underlines the need for suitable One Health approaches. During outbreak situations, straightforward identification of etiological agents is indispensable for taking countermeasures. A recently published protocol for metagenomic virus detection in clinical specimens (TUViD-VM) was developed for snap-frozen tissues which can be challenging to obtain. Here, we describe the use of RNALater(®) -treated tissue at ambient temperatures for virome sequencing...
2017: Frontiers in Microbiology
A B Turovsky, N A Shostak, M E Artemyev, N K Khuazheva, T P Bessarab, P L Chumakov, A V Artemieva-Karelova
The objective of the present work was to present the results of the clinical analysis of the patient presenting with natural killer (NK)/T-cell lymphoma of the nasal type. We undertook the analysis of the medical documentation concerning the case of interest. It was shown that the development of progressive perforation of the nasal septum and the pronounced destructive changes in the intranasal and adjacent structures following the endonasal surgical interventions made necessary differential diagnostics between the condition under consideration and certain latent disorders (such as Wegener's granulomatosis, leprosy, syphilis, leishmaniasis, dirofilariasis tuberculosis, etc...
2017: Vestnik Otorinolaringologii
E Héron, T Bourcier
Episcleritis is generally a benign disease with a self-limited course, while scleritis is a severe ocular condition due to a risk of impaired vision in one-fifth of patients and its association with systemic diseases in one third of them. Infectious scleritis, representing 8 % of the etiologies, is mainly of herpetic origin (varicella zoster and herpes simplex viruses). A systemic autoimmune disease is observed in roughly 30 % of scleritis patients: inflammatory rheumatisms (15 %), firstly rheumatoid polyarthritis, systemic vasculitides (8 %), mainly granulomatosis with polyangiitis (Wegener's) and polychondritis, and less often inflammatory bowel disease and systemic lupus erythematosus...
October 2017: Journal Français D'ophtalmologie
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