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Wegener disease

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https://www.readbyqxmd.com/read/29139157/seronegative-granulomatosis-with-polyangiitis-presenting-with-multiple-cranial-nerve-palsies
#1
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
https://www.readbyqxmd.com/read/29102717/nuclear-retention-of-mrnas-quality-control-gene-regulation-and-disease
#2
REVIEW
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 1, 2017: Seminars in Cell & Developmental Biology
https://www.readbyqxmd.com/read/29018436/use-of-rnalater-%C3%A2-preservation-for-virome-sequencing-in-outbreak-settings
#3
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
https://www.readbyqxmd.com/read/28980601/-the-clinical-observation-of-nk-t-cell-lymphoma-of-the-nasal-type
#4
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
https://www.readbyqxmd.com/read/28916114/-scleritis-and-episcleritis
#5
REVIEW
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
https://www.readbyqxmd.com/read/28912820/granulomatosis-with-polyangiitis-with-myocarditis-and-ventricular-tachycardia
#6
Ramy Magdy Hanna, Eduardo Lopez, James Wilson
Granulomatosis with polyangiitis (GPA), previously known as Wegener's granulomatosis, is a pulmonary-renal syndrome affecting small and medium sized blood vessels. The disease has a prevalence in studies ranging from 3 to 15.7 cases per 100,000, with a noted increasing incidence and prevalence in more recent studies. Pulmonary manifestations include hemorrhage, lung cavitary lesions, and pulmonary fibrosis. Within the kidney, GPA is known to cause rapidly progressive pauci-immune crescentic glomerulonephritis...
2017: Case Reports in Medicine
https://www.readbyqxmd.com/read/28881107/saddle-nose-deformity-and-septal-perforation-in-granulomatosis-with-polyangiitis
#7
Annekatrin Coordes, Sonja Maike Loose, Veit M Hofmann, Grant S Hamilton, Frank Riedel, Dirk Jan Menger, Andreas E Albers
BACKGROUND: Patients who have granulomatosis with polyangiitis (GPA, syn. M. Wegener) often develop an external nose deformity which may have devastating psychological effects. Therefore, reconstruction of nasal deformities by rhinoplasty may become necessary to achieve a normal appearance. OBJECTIVE OF REVIEW: The aim of this systematic review was to investigate the efficacy and safety of surgical reconstruction in external nasal deformities and septal perforation in GPA patients...
September 7, 2017: Clinical Otolaryngology
https://www.readbyqxmd.com/read/28863794/immunoadsorption-in-autoimmune-diseases-affecting-the-kidney
#8
REVIEW
Georg Stummvoll, Martin Aringer, Ammon Handisurya, Kurt Derfler
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...
September 2017: Seminars in Nephrology
https://www.readbyqxmd.com/read/28842398/anca-glomerulonephritis-and-vasculitis
#9
J Charles Jennette, Patrick H Nachman
ANCA vasculitis has an associated autoimmune response that produces ANCAs that induce distinct pathologic lesions. Pauci-immune necrotizing and crescentic GN is a frequent component of ANCA vasculitis. ANCA vasculitis is associated with ANCA specific for myeloperoxidase (MPO-ANCA) or proteinase 3 (PR3-ANCA). A diagnosis of ANCA vasculitis should always specify the serotype as MPO-ANCA positive, PR3-ANCA positive, or ANCA-negative. To fully characterize a patient, the serotype also should be accompanied by the clinicopathologic variant if this can be determined: microscopic polyangiitis, granulomatosis with polyangiitis (Wegener), eosinophilic granulomatosis with polyangiitis (Churg-Strauss), or renal-limited vasculitis...
October 6, 2017: Clinical Journal of the American Society of Nephrology: CJASN
https://www.readbyqxmd.com/read/28839290/rare-occupational-cause-of-nasal-septum-perforation-nickel-exposure
#10
Ertugrul Cagri Bolek, Abdulsamet Erden, Cagri Kulekci, Umut Kalyoncu, Omer Karadag
Many etiologies are held accountable for nasal septum perforations. Topical nasal drug usage, previous surgeries, trauma, nose picking, squamous cell carcinoma, some rheumatological disorders such as granulomatosis with polyangiitis (Wegener granulomatosis), some infectious diseases such as syphilis and leprosy are among the causes of the perforations. Occupational heavy metal exposures by inhalation rarely may also cause nasal septum perforation. Here, we present a 29-year-old patient without any known diseases, who is a worker at a metallic coating and nickel-plating factory, referred for investigation of his nasal cartilage septum perforation from an otorhinolaryngology clinic...
August 23, 2017: International Journal of Occupational Medicine and Environmental Health
https://www.readbyqxmd.com/read/28817371/phase-i-ii-trial-of-labetuzumab-govitecan-anti-ceacam5-sn-38-antibody-drug-conjugate-in-patients-with-refractory-or-relapsing-metastatic-colorectal-cancer
#11
MULTICENTER STUDY
Efrat Dotan, Steven J Cohen, Alexander N Starodub, Christopher H Lieu, Wells A Messersmith, Pamela S Simpson, Michael J Guarino, John L Marshall, Richard M Goldberg, J Randolph Hecht, William A Wegener, Robert M Sharkey, Serengulam V Govindan, David M Goldenberg, Jordan D Berlin
Purpose The objectives were to evaluate dosing schedules of labetuzumab govitecan, an antibody-drug conjugate targeting carcinoembryonic antigen-related cell adhesion molecule 5 (CEACAM5) for tumor delivery of 7-ethyl-10-hydroxycamptothecin (SN-38), in an expanded phase II trial of patients with relapsed or refractory metastatic colorectal cancer. Patients and Methods Eligible patients with at least one prior irinotecan-containing therapy received labetuzumab govitecan once weekly at 8 and 10 mg/kg, or two times per week at 4 and 6 mg/km on weeks 1 and 2 of 3-week repeated cycles...
October 10, 2017: Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology
https://www.readbyqxmd.com/read/28816552/the-role-of-biopsy-in-lacrimal-gland-inflammation-a-clinicopathologic-study
#12
Panitee Luemsamran, Jack Rootman, Valerie A White, Nariman Nassiri, Manraj K S Heran
PURPOSE: To determine the causes of lacrimal gland inflammation based on histopathology and systemic evaluation. METHODS: This is a retrospective case series study. From the University of British Columbia Orbit Clinic between January 1976 and December 2008, we reviewed the medical records of 60 patients who presented with inflammatory features of the lacrimal gland (i.e., erythema, edema, or tenderness) in which the diagnoses were not possible clinically and on imaging alone...
August 17, 2017: Orbit
https://www.readbyqxmd.com/read/28802759/sensor-assisted-self-management-in-parkinson-s-disease-a-feasibility-study-of-ambulatory-posture-detection-and-feedback-to-treat-stooped-posture
#13
E E H van Wegen, C J T de Goede, G Kwakkel, J van Kordelaar
INTRODUCTION: A stooped posture is one of the characteristic motor symptoms of patients with Parkinson's disease, and has been linked to impairments in daily activities and quality of life. We aimed to test the efficacy, safety, practical utility and user-friendliness of a posture correction and vibrotactile trunk angle feedback device (the UpRight) in the home setting of patients with Parkinson's disease with a stooped posture. It was hypothesized that ambulatory use of the UpRight would be safe, feasible and result in a less stooped posture, i...
July 25, 2017: Parkinsonism & related Disorders
https://www.readbyqxmd.com/read/28799078/intrachiasmatic-abscess-caused-by-igg4-related-hypophysitis
#14
Georgios F Hadjigeorgiou, Eva Løbner Lund, Lars Poulsgaard, Ulla Feldt-Rasmussen, Åse Krogh Rasmussen, Marianne Wegener, Kåre Fugleholm
INTRODUCTION: Autoimmune hypophysitis is a rare disease of the pituitary, which may affect vision by inflammation and compression of the optic chiasm. However, intrachiasmatic abscess formation has not been previously reported. METHODS: In this study, we report a case of a 29-year-old female with bitemporal hemianopia due to a cystic intrasellar tumor. The patient underwent surgical decompression of the lesion, which was found to be an intrachiasmatic abscess. RESULTS: The histologic findings were consistent with IgG4 hypophysitis...
November 2017: Acta Neurochirurgica
https://www.readbyqxmd.com/read/28771620/a-benign-helminth-alters-the-host-immune-system-and-the-gut-microbiota-in-a-rat-model-system
#15
Laura Wegener Parfrey, Milan Jirků, Radek Šíma, Marie Jalovecká, Bohumil Sak, Karina Grigore, Kateřina Jirků Pomajbíková
Helminths and bacteria are major players in the mammalian gut ecosystem and each influences the host immune system and health. Declines in helminth prevalence and bacterial diversity appear to play a role in the dramatic rise of immune mediated inflammatory diseases (IMIDs) in western populations. Helminths are potent modulators of immune system and their reintroduction is a promising therapeutic avenue for IMIDs. However, the introduction of helminths represents a disturbance for the host and it is important to understand the impact of helminth reintroduction on the host, including the immune system and gut microbiome...
2017: PloS One
https://www.readbyqxmd.com/read/28756634/necrotizing-sarcoid-granulomatosis-a-distinctive-form-of-pulmonary-granulomatous-disease
#16
REVIEW
Georgia Karpathiou, Anna Batistatou, Panagiotis Boglou, Dimitrios Stefanou, Marios E Froudarakis
OBJECTIVES: To define the characteristics of necrotizing sarcoid granulomatosis (NGS) a very rare pulmonary disease hardly recognized by pulmonologists and pathologists. DATA SOURCE: PubMed was searched for the term necrotising or necrotizing sarcoid granulomatosis. STUDY SELECTION: All cases reported in the English literature were included. RESULTS: NGS is presented at all ages (range 8-68 years) with a median age of 42 years old...
July 29, 2017: Clinical Respiratory Journal
https://www.readbyqxmd.com/read/28745688/-risk-factors-for-anemia-in-the-early-stages-of-chronic-kidney-disease
#17
Yu S Milovanov, L V Kozlovskaya, L Yu Milovanova, M M Markina, V V Kozlov, M V Taranova, V V Fomin
AIM: To identify the early markers of anemia in chronic kidney disease (CKD) in patients with chronic glomerulonephritis (CGN) and glomerulonephritis (GN) in systemic diseases. SUBJECTS AND METHODS: Seventy-nine patients with some male preponderance who were aged 21 to 65 years (45.3±11.1 years) and had CKD (CGN and GN) in systemic diseases (systemic lupus erythematosus and Wegener's granulomatosis) in the early stages (Stages I-II) of CKD were examined. GN was diagnosed by a lifetime renal biopsy...
2017: Terapevticheskiĭ Arkhiv
https://www.readbyqxmd.com/read/28741986/serum-ferritin-as-an-activity-marker-for-granulamotosis-with-polyangiitis
#18
Hamit Kucuk, Ozkan Varan, Berna Goker, Berivan Bitik, Mehmet Akif Ozturk, Seminur Haznedaroglu, Abdurrahman Tufan
BACKGROUND: Serum ferritin correlates well with the activities of systemic lupus erythematosus (SLE) and dermatomyositis, but it has not been previously studied in patients with vasculitis. METHODS: Medical records of granulomatosis with polyangiitis (GPA, Wegener's granulomatosis) patients with at least six months of regular follow-up were evaluated. The activity of GPA was assessed with Birmingham Vasculitis Activity Score for Wegener's Granulomatosis (BVAS/WG)...
November 2017: Renal Failure
https://www.readbyqxmd.com/read/28692611/a-national-survey-on-the-use-of-electronic-directly-observed-therapy-for-treatment-of-tuberculosis
#19
Michelle Macaraig, Mark N Lobato, Kara McGinnis Pilote, Donna Wegener
CONTEXT: An increasing number of tuberculosis (TB) programs are adopting electronic directly observed therapy (eDOT), the use of technology to supervise patient adherence remotely. Pilot studies show that treatment adherence and completion were similar with eDOT compared with the standard in-person DOT. OBJECTIVE: In December 2015, the National Tuberculosis Controllers Association administered an online survey to determine the extent to which eDOT is used in the United States...
July 7, 2017: Journal of Public Health Management and Practice: JPHMP
https://www.readbyqxmd.com/read/28631702/-the-frequency-of-ophthalmologic-manifestations-of-granulomatosis-with-polyangiitis-wegener-s-and-their-relationship-to-systemic-diseases
#20
D S Ismailova, P I Novikov, Ya O Grusha, Yu V Abramova, N M Bulanov, E A Makarov
AIM: To estimate the frequency of lesions in the organ of vision in granulomatosis with polyangiitis (GPA) (Wegener's) and to determine their relationship to systemic diseases. SUBJECTS AND METHODS: The retrospective study enrolled 218 patients followed up at the E.M. Tareyev Clinic of Nephrology, Internal and Occupational Diseases, with a diagnosis of GPA. The frequency and association of ophthalmic manifestations with systemic involvement were statistically analyzed using PASW Statistics 18...
2017: Terapevticheskiĭ Arkhiv
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