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Iliana Tantcheva-Poór, Vinzenz Oji, Cristina Has
Jüngste Fortschritte der Genforschung haben ihren Weg in die klinische Dermatologie gefunden. Nahezu ein Drittel aller Erbkrankheiten zeigt charakteristische Hautveränderungen. Zudem können Proben menschlicher Haut als Untersuchungsmaterial zur Detektion genetischer Mosaike und der zu Grunde liegenden Defekte eingesetzt werden. Die Diagnose von Genodermatosen bleibt jedoch aufgrund ihrer unterschiedlich ausgeprägten und überlappenden Phänotypen sowie ihrer Seltenheit und Vielzahl neuer Informationen wegen eine Herausforderung für die Kliniker...
October 2016: Journal der Deutschen Dermatologischen Gesellschaft, Journal of the German Society of Dermatology: JDDG
Sarah-Jayne Edmondson, Saina Attaran, Ulrich P Rosendahl
Wegner's granulomatosis (WG) is an autoimmune systemic vasculitis that results in necrotizing granulomas. We report a WG patient with a lung granuloma and aortic root dilatation, who underwent aortic root replacement on cardiopulmonary bypass (CPB). Intraoperatively, the patient suffered an aortic dissection, which was repaired immediately under deep hypothermic circulatory arrest (DHCA). Follow-up imaging showed complete granuloma resolution, despite absence of immunosuppressive therapy. Immune stimulation following CPB is well described; here, the opposite was observed and DHCA effects are discussed...
February 2016: Aorta (Stamford, Conn.)
Zoran Koturović, Miroslav Knežević, Dejan M Rašić
The main purpose of this paper is to provide the information about the incidence and types of pathology of secondary acquired obstructions of the lacrimal excretory outflow system caused by primary lacrimal sac non-neoplastic and neoplastic lesions. After a thorough literature search, 17 case-control studies were found and selected, data were extracted and categorized, to evaluate specific lacrimal sac pathology mimicking inflammation. A total of 3865 histopathologically examined lacrimal sac wall biopsy specimens from 3662 patients, taken during dacryocystorhinostomy for clinically presumed primary chronic dacryocystitis, were analyzed...
October 18, 2016: Bosnian Journal of Basic Medical Sciences
S Lieb, T Littmann, N Plank, J Felixberger, M Tanaka, T Schäfer, S Krief, S Elz, K Friedland, G Bernhardt, J Wegener, T Ozawa, A Buschauer
A set of histamine H1 receptor (H1R) agonists and antagonists was characterized in functional assays, using dynamic mass redistribution (DMR), electric cell-substrate impedance sensing (ECIS) and various signaling pathway specific readouts (Fura-2 and aequorin calcium assays, arrestin recruitment (luciferase fragment complementation) assay, luciferase gene reporter assay). Data were gained from genetically engineered HEK293T cells and compared with reference data from GTPase assays and radioligand binding. Histamine and the other H1R agonists gave different assay-related pEC50 values, however, the order of potency was maintained...
October 14, 2016: Pharmacological Research: the Official Journal of the Italian Pharmacological Society
Rafael Laso-Pérez, Gunter Wegener, Katrin Knittel, Friedrich Widdel, Katie J Harding, Viola Krukenberg, Dimitri V Meier, Michael Richter, Halina E Tegetmeyer, Dietmar Riedel, Hans-Hermann Richnow, Lorenz Adrian, Thorsten Reemtsma, Oliver Lechtenfeld, Florin Musat
The anaerobic formation and oxidation of methane involve unique enzymatic mechanisms and cofactors that are believed to be all specific for C1-compounds. Here we found that an anaerobic thermophilic enrichment culture composed of dense consortia of archaea and bacteria apparently uses partly similar pathways to oxidize the C4-hydrocarbon butane. The archaea, proposed genus Candidatus Syntrophoarchaeum, showed the characteristic autofluorescence of methanogens, and contained highly expressed genes encoding enzymes similar to methyl-coenzyme M reductase (MCR)...
October 17, 2016: Nature
Christian Pagnoux
Antineutrophil cytoplasm antibody (ANCA)-associated vasculitides are small-vessel vasculitides that include granulomatosis with polyangiitis (formerly Wegener's granulomatosis), microscopic polyangiitis, and eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome). Renal-limited ANCA-associated vasculitides can be considered the fourth entity. Despite their rarity and still unknown cause(s), research pertaining to ANCA-associated vasculitides has been very active over the past decades. The pathogenic role of antimyeloperoxidase ANCA (MPO-ANCA) has been supported using several animal models, but that of antiproteinase 3 ANCA (PR3-ANCA) has not been as strongly demonstrated...
September 2016: Eur J Rheumatol
Meret Branscheidt, Juliane Schneider, Patrik Michel, Elissavet Eskioglou, Georg Kaegi, Robert Stark, Urs Fischer, Simon Jung, Marcel Arnold, Maria Wertli, Ulrike Held, Susanne Wegener, Andreas Luft, Hakan Sarikaya
BACKGROUND AND PURPOSE: The impact of excess body weight on prognosis after stroke is controversial. Many studies report higher survival rates in obese patients ("obesity paradox"). Recently, obesity has been linked to worse outcomes after intravenous (IV) thrombolysis, but the number and sample size of these studies were small. Here, we aimed to assess the relationship between body weight and stroke outcome after IV thrombolysis in a large cohort study. METHODS: In a prospective observational multicenter study, we analyzed baseline and outcome data of 896 ischemic stroke patients who underwent IV thrombolysis...
2016: PloS One
Kelly L Keeling, Okki Cho, Denis B Scanlon, Grant W Booker, Andrew D Abell, Kate L Wegener
Constrained α-helical peptides are showing potential as biological probes and therapeutic agents that target protein-protein interactions. However, the factors that determine the optimal constraint locations are still largely unknown. Using the β-integrin/talin protein interaction as a model system, we examine the effect of constraint location on helical conformation, as well as binding affinity, using circular dichroism and NMR spectroscopy. Stapling increased the overall helical content of each integrin-based peptide tested...
October 18, 2016: Organic & Biomolecular Chemistry
Christian Hauptmann, Alexander Wegener, Hendrik Poppe, Mark Williams, Gerald Popelka, Peter A Tass
BACKGROUND: Sound-based tinnitus intervention stimuli include broad-band noise signals with subjectively adjusted bandwidths used as maskers delivered by commercial devices or hearing aids, environmental sounds broadly described and delivered by both consumer devices and hearing aids, music recordings specifically modified and delivered in a variety of different ways, and other stimuli. Acoustic coordinated reset neuromodulation therapy for tinnitus reduction has unique and more stringent requirements compared to all other sound-based tinnitus interventions...
October 2016: Journal of the American Academy of Audiology
Roman Steckiewicz, Marek Rosiak, Przemysław Stolarz, Elżbieta B Świętoń, Marcin Grabowski, Dariusz A Kosior
Reversible complete atrioventricular block in patient with Wegener's granulomatosis - a report on a positive outcome with long term follow-up. Atrioventricular (AV) block is a rare complication of Wegener's granulomatosis (WG), thus there are no standards of management in such cases. We present a case of a patient with a dual-chamber pacemaker (DDD) implanted due to complete AV block in the course of Wegener's granulomatosis (WG). An immunosuppressive therapy resulted in the resolution of non-cardiac and AV conduction disorders...
2016: Wiadomości Lekarskie: Organ Polskiego Towarzystwa Lekarskiego
Alexander Ryabchun, Matthias Kollosche, Michael Wegener, Oksana Sakhno
Volume diffraction gratings (VDGs) are inscribed selectively by diffusive introduction of benzophenone and subsequent UV-holographic structuring into an electroactive dielectric elastomer actuator (DEA), to afford a continuous voltage-controlled grating shift of 17%. The internal stress coupling of DEA and optical domain allows for a new generation of true monolithic tunable elastomer optics with voltage controlled properties.
October 7, 2016: Advanced Materials
Paul M K Gordon, Aneal Khan, Umair Sajid, Nicholas Chang, Varun Suresh, Leo Dimnik, Ryan E Lamont, Jillian S Parboosingh, Steven R Martin, Richard T Pon, Jene Weatherhead, Shelly Wegener, Debra Isaac, Steven C Greenway
Cell-free DNA (cfDNA) has significant potential in the diagnosis and monitoring of clinical conditions. However, accurately and easily distinguishing the relative proportion of DNA molecules in a mixture derived from two different sources (i.e., donor and recipient tissues after transplantation) is challenging. In human cellular transplantation, there is currently no useable method to detect in vivo engraftment, and blood-based non-invasive tests for allograft rejection in solid organ transplantation are either non-specific or absent...
2016: Frontiers in Cardiovascular Medicine
Hamid Tavakkoli, Mehdi Zobeiri, Mansour Salesi, Mohammad Hossein Sanei
Wegener's granulomatosis is an uncommon inflammatory disease that manifests as vasculitis, granulomatosis, and necrosis. It usually involves the upper and lower respiratory tracts and kidneys. Although it may essentially involve any organ, gastrointestinal (GI) involvement is notably uncommon. A 20-year-old male patient presented with epigastric pain, vomiting, hematemesis, and melena. On physical examination, he was pale. There was no abdominal tenderness or organomegaly. Upper GI endoscopy revealed dark blue-colored infiltrative lesions in prepyloric area...
July 2016: Middle East Journal of Digestive Diseases
Zachary S Wallace, Eli M Miloslavsky, Matthew Cascino, Sebastian H Unizony, Na Lu, Gary S Hoffman, Cees G M Kallenberg, Carol A Langford, Peter A Merkel, Paul A Monach, Philip Seo, Robert Spiera, E William St Clair, Ulrich Specks, Paul Brunetta, Hyon K Choi, John H Stone
INTRODUCTION: We investigated the relationships between glucocorticoid (GC) use, disease activity, and changes in body mass index (BMI) in patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). METHODS: We analyzed AAV patients enrolled in the Rituximab in ANCA-Associated Vasculitis (RAVE) trial. GC use, BMI, and disease activity were measured regularly during the trial period. We performed mixed effects regressions to examine the associations of time-dependent cumulative average GC use and disease activity with changes in body mass index (BMI) over time, while adjusting for potential confounders...
October 1, 2016: Arthritis Care & Research
Jiangtian Chen, Wencke Reiher, Christiane Hermann-Luibl, Azza Sellami, Paola Cognigni, Shu Kondo, Charlotte Helfrich-Förster, Jan A Veenstra, Christian Wegener
Feeding and sleep are fundamental behaviours with significant interconnections and cross-modulations. The circadian system and peptidergic signals are important components of this modulation, but still little is known about the mechanisms and networks by which they interact to regulate feeding and sleep. We show that specific thermogenetic activation of peptidergic Allatostatin A (AstA)-expressing PLP neurons and enteroendocrine cells reduces feeding and promotes sleep in the fruit fly Drosophila. The effects of AstA cell activation are mediated by AstA peptides with receptors homolog to galanin receptors subserving similar and apparently conserved functions in vertebrates...
September 2016: PLoS Genetics
Shweta S Duggal, Chirag Shah
No abstract text is available yet for this article.
September 2016: PM & R: the Journal of Injury, Function, and Rehabilitation
Elizabeth Manuely González Revilla, Araceli Abad Fernandez, María Teresa Río Ramirez, Sara Calero Pardo, María Antonia Juretschke Moragues
Granulomatosis with polyangiitis (GPA) is the name that has been used in recent years for Wegener's granulomatosis. This condition is a systemic inflammatory disease characterised by necrotizing vasculitis that affects small and medium-sized blood vessels (capillaries, arterioles, venules and arteries). The granulomatous inflammation affects the respiratory system; it also commonly affects the kidney and can very rarely affect large vessels such as the aorta and the surrounding retroperitoneal tissue. Early diagnosis and treatment is of vital importance because of the high risk of dissection and of obstruction of retroperitoneal structures...
2016: Respiratory Medicine Case Reports
Anne Laure Bernat, Etienne Lefevre, Damien Sène, Philippe Herman, Homa Adle Biassette, Sebastien Froelich
Granulomatosis with polyangitis (Wegener's disease) is a rare ANCA (anti-neutrophil cytoplasmic antibody) positive vasculitis that seldom involves brain and meninges. We herein present a case of a 35-year-old woman presenting with granulomatosis with polyangitis involving the brain and the meninges. The histological diagnosis required a complete resection of the lesion. Despite the possibility of post operative wound infection surgery was mandatory to ascertain the diagnostic and to treat locally.
September 23, 2016: World Neurosurgery
Caroline Winters, Martijn W Heymans, Erwin E H van Wegen, Gert Kwakkel
BACKGROUND: The impact of spontaneous neurobiological recovery is still neglected in designing rehabilitation trials early post stroke. We aimed to investigate the impact of the timing of randomization and prognostic stratification on the required sample sizes that are needed to reveal significant intervention effects on upper limb function at 26 weeks after first-ever ischemic stroke. METHOD: Sample size calculations were based on a cohort study of 159 patients, using the Fugl-Meyer Assessment Upper Extremity and Action Research Arm Test as outcome measures (power = 80 %; two-tailed alpha = 0...
September 26, 2016: Trials
Tim Vanbellingen, Thomas Nyffeler, Tobias Nef, Gert Kwakkel, Stephan Bohlhalter, Erwin E H van Wegen
BACKGROUND: Patients with Parkinson's disease exhibit disturbed dexterity. Validated self-reported outcomes for dexterity in Parkinson's disease are lacking. The aim of this study was to investigate the reliability, content and construct validity of a new Dexterity Questionnaire 24. METHODS: One hundred and three patients with Parkinson's disease completed the Dexterity Questionnaire 24 (score range 24-96), at baseline and four weeks later. The internal consistency was determined...
September 15, 2016: Parkinsonism & related Disorders
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