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IgG 4 Related disease

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https://www.readbyqxmd.com/read/27893674/relapsing-remitting-lesions-in-a-woman-with-progressive-hemifacial-atrophy-and-chronic-hepatitis-b-virus-infection-a-case-report
#1
Ying Zhang, Mingqin Zhu, Xiaozhen Li, Jing Miao, Chenchen Duan, Li Cui, Xuefan Yu
INTRODUCTION: Progressive hemifacial atrophy (PHA) is a rare disorder characterized by unilateral facial atrophy affecting the skin, subcutaneous tissue, and fat, muscle, and osteocartilagenous structures creating a sunken hemiface appearance.Etiopathogenesis of PHA is poorly understood; no definitive treatment is currently available. CLINICAL FINDINGS: We report a 41-year-old woman with PHA who showed an uncharacteristic "relapsing-remitting" evolution of brain lesions and was seropositive for hepatitis B virus (HBV)...
November 2016: Medicine (Baltimore)
https://www.readbyqxmd.com/read/27891673/igg4-related-disease-experience-of-100-consecutive-cases-from-a-specialist-centre
#2
Adrian C Bateman, Emma L Culver
AIMS: To describe the features of 100 consecutive cases referred to a single UK institution, in which a diagnosis of IgG4-related disease (IgG4-RD) was under consideration. METHODS AND RESULTS: The histological features were reviewed by a single histopathologist and cases categorised using the 2012 Boston criteria: Category 1 - histologically highly suggestive of IgG4-RD; 2 - probable histopathological features of IgG4-RD; 3 - insufficient histopathological features of IgG4-RD...
November 28, 2016: Histopathology
https://www.readbyqxmd.com/read/27866757/differential-diagnosis-of-igg4-related-sialadenitis-primary-sj%C3%A3-gren-syndrome-and-chronic-obstructive-submandibular-sialadenitis
#3
X Hong, W Li, X-Y Xie, Z-Y Zhang, Y Chen, Y Gao, X Peng, J-Z Su, Y-Y Zhang, Z Wang, Z-G Cai, L Zhang, Y-Y Liu, J He, L-M Ren, Z-G Li, G-Y Yu
Our aim was to differentiate IgG4-related sialadenitis, primary Sjögren syndrome, and chronic obstructive submandibular sialadenitis by analysing clinical, radiographic, and pathological features. Fifty-five patients, 50, and 50 were enrolled, respectively and their baseline characteristics and serological, sialographic, and pathological findings compared. The male:female ratio for IgG4-related sialadenitis was 1:1.2 for primary Sjögren syndrome 1:15.7, and for chronic obstructive submandibular sialadenitis1:0...
November 17, 2016: British Journal of Oral & Maxillofacial Surgery
https://www.readbyqxmd.com/read/27852547/prevalence-of-igg-4-associated-cholangiopathy-based-on-serum-igg-4-levels-in-patients-with-primary-sclerosing-cholangitis-and-its-relationship-with-inflammatory-bowel-disease
#4
Seyed Alireza Taghavi, Shaghayegh Kaedi Majd, Mahnoosh Sianati, Masood Sepehrimanesh
BACKGROUND/AIMS: Autoimmune cholangiopathy is part of a fibro-inflammatory immunoglobulin G-4 (IgG-4)-related systemic disease that causes biliary tract strictures. Its clinical presentation is quite similar to that of more common diseases such as primary sclerosing cholangitis (PSC) and pancreatobilliary malignancies. The aims of the present study were to evaluate the prevalence of IgG-4-associated cholangiopathy (IAC) in patients diagnosed with PSC and its relationship with inflammatory bowel disease (IBD)...
November 2016: Turkish Journal of Gastroenterology: the Official Journal of Turkish Society of Gastroenterology
https://www.readbyqxmd.com/read/27851820/impact-of-helicobacter-pylori-immunoglobulin-g-levels-and-atrophic-gastritis-status-on-risk-of-metabolic-syndrome
#5
Atsushi Takeoka, Jun Tayama, Hironori Yamasaki, Masakazu Kobayashi, Sayaka Ogawa, Tatsuo Saigo, Masaki Hayashida, Susumu Shirabe
BACKGROUND: Helicobacter pylori (HP) infection is implicated in gastric and extra-gastric diseases. While gastritis-related chronic inflammation represents a known trigger of metabolic disturbances, whether metabolic syndrome (MetS) is affected by gastritis status remains unclear. We aimed to clarify the effect of HP-related gastritis on the risk of MetS. MATERIALS AND METHODS: We retrospectively enrolled patients undergoing screening for MetS between 2014 and 2015...
2016: PloS One
https://www.readbyqxmd.com/read/27802825/mog-igg-in-nmo-and-related-disorders-a-multicenter-study-of-50-patients-part-3-brainstem-involvement-frequency-presentation-and-outcome
#6
Sven Jarius, Ingo Kleiter, Klemens Ruprecht, Nasrin Asgari, Kalliopi Pitarokoili, Nadja Borisow, Martin W Hümmert, Corinna Trebst, Florence Pache, Alexander Winkelmann, Lena-Alexandra Beume, Marius Ringelstein, Oliver Stich, Orhan Aktas, Mirjam Korporal-Kuhnke, Alexander Schwarz, Carsten Lukas, Jürgen Haas, Kai Fechner, Mathias Buttmann, Judith Bellmann-Strobl, Hanna Zimmermann, Alexander U Brandt, Diego Franciotta, Kathrin Schanda, Friedemann Paul, Markus Reindl, Brigitte Wildemann
BACKGROUND: Myelin oligodendrocyte glycoprotein antibodies (MOG-IgG) are present in a subset of aquaporin-4 (AQP4)-IgG-negative patients with optic neuritis (ON) and/or myelitis. Little is known so far about brainstem involvement in MOG-IgG-positive patients. OBJECTIVE: To investigate the frequency, clinical and paraclinical features, course, outcome, and prognostic implications of brainstem involvement in MOG-IgG-positive ON and/or myelitis. METHODS: Retrospective case study...
November 1, 2016: Journal of Neuroinflammation
https://www.readbyqxmd.com/read/27802824/mog-igg-in-nmo-and-related-disorders-a-multicenter-study-of-50-patients-part-4-afferent-visual-system-damage-after-optic-neuritis-in-mog-igg-seropositive-versus-aqp4-igg-seropositive-patients
#7
Florence Pache, Hanna Zimmermann, Janine Mikolajczak, Sophie Schumacher, Anna Lacheta, Frederike C Oertel, Judith Bellmann-Strobl, Sven Jarius, Brigitte Wildemann, Markus Reindl, Amy Waldman, Kerstin Soelberg, Nasrin Asgari, Marius Ringelstein, Orhan Aktas, Nikolai Gross, Mathias Buttmann, Thomas Ach, Klemens Ruprecht, Friedemann Paul, Alexander U Brandt
BACKGROUND: Antibodies against myelin oligodendrocyte glycoprotein (MOG-IgG) have been reported in patients with aquaporin-4 antibody (AQP4-IgG)-negative neuromyelitis optica spectrum disorders (NMOSD). The objective of this study was to describe optic neuritis (ON)-induced neuro-axonal damage in the retina of MOG-IgG-positive patients in comparison with AQP4-IgG-positive NMOSD patients. METHODS: Afferent visual system damage following ON was bilaterally assessed in 16 MOG-IgG-positive patients with a history of ON and compared with that in 16 AQP4-IgG-positive NMOSD patients...
November 1, 2016: Journal of Neuroinflammation
https://www.readbyqxmd.com/read/27799828/serum-total-igg-and-igg4-levels-in-thyroid-eye-disease
#8
Aileen Sy, Rona Z Silkiss
PURPOSE: To investigate the relationship between immunoglobulin G (IgG)4-related disease (IgG4-RD) and thyroid eye disease (TED) with respect to IgG levels. PATIENTS AND METHODS: A retrospective review of total IgG, IgG subclass, and thyroid stimulating immunoglobulin (TSI) levels in 24 patients with TED. RESULTS: Five patients (20.8%) demonstrated serum IgG4 levels consistent with IgG4-RD without any additional systemic disease. Total IgG and IgG subclass levels were found to be an inadequate proxy for TSI elevation...
2016: International Medical Case Reports Journal
https://www.readbyqxmd.com/read/27795522/spontaneous-heparin-induced-thrombocytopenia-hit-syndrome-hit-without-any-heparin-exposure
#9
Shigeki Miyata
Heparin-induced thrombocytopenia (HIT) is a pro-thrombotic side effect of heparin therapy caused by HIT antibodies with platelet-activating properties. Recent advances in understanding of spontaneous HIT syndrome, which can occur even without any heparin exposure despite its clinical and serological characteristics being similar to those of HIT, reveal the following HIT clinical features atypical for an immune-mediated disease. Heparin-naïve patients can develop IgG antibodies as early as day 4, as in a secondary immune response...
2016: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/27788675/mog-igg-in-nmo-and-related-disorders-a-multicenter-study-of-50-patients-part-1-frequency-syndrome-specificity-influence-of-disease-activity-long-term-course-association-with-aqp4-igg-and-origin
#10
Sven Jarius, Klemens Ruprecht, Ingo Kleiter, Nadja Borisow, Nasrin Asgari, Kalliopi Pitarokoili, Florence Pache, Oliver Stich, Lena-Alexandra Beume, Martin W Hümmert, Corinna Trebst, Marius Ringelstein, Orhan Aktas, Alexander Winkelmann, Mathias Buttmann, Alexander Schwarz, Hanna Zimmermann, Alexander U Brandt, Diego Franciotta, Marco Capobianco, Joseph Kuchling, Jürgen Haas, Mirjam Korporal-Kuhnke, Soeren Thue Lillevang, Kai Fechner, Kathrin Schanda, Friedemann Paul, Brigitte Wildemann, Markus Reindl
BACKGROUND: Antibodies to myelin oligodendrocyte glycoprotein (MOG-IgG) have been suggested to play a role in a subset of patients with neuromyelitis optica and related disorders. OBJECTIVE: To assess (i) the frequency of MOG-IgG in a large and predominantly Caucasian cohort of patients with optic neuritis (ON) and/or myelitis; (ii) the frequency of MOG-IgG among AQP4-IgG-positive patients and vice versa; (iii) the origin and frequency of MOG-IgG in the cerebrospinal fluid (CSF); (iv) the presence of MOG-IgG at disease onset; and (v) the influence of disease activity and treatment status on MOG-IgG titers...
September 26, 2016: Journal of Neuroinflammation
https://www.readbyqxmd.com/read/27765766/tula-2-t-cell-ubiquitin-ligand-2-inhibits-the-platelet-fc-receptor-for-igg-iia-fc%C3%AE-riia-signaling-pathway-and-heparin-induced-thrombocytopenia-in-mice
#11
Yuhang Zhou, Shaji Abraham, Stephanie Renna, Leonard C Edelstein, Carol A Dangelmaier, Alexander Y Tsygankov, Satya P Kunapuli, Paul F Bray, Steven E McKenzie
OBJECTIVE: The objective of this study is to investigate the role of T-cell ubiquitin ligand-2 (TULA-2) in the platelet Fc receptor for IgG IIA (FcγRIIA) pathway and in the pathogenesis of heparin-induced thrombocytopenia (HIT). APPROACH AND RESULTS: HIT is a life-threatening thrombotic disease in which IgG antibodies against the heparin-platelet factor 4 complex activate platelets via FcγRIIA. We reported previously differential expression of TULA-2 in human population was linked to FcγRIIA responsiveness...
October 20, 2016: Arteriosclerosis, Thrombosis, and Vascular Biology
https://www.readbyqxmd.com/read/27739273/chronic-tubulo-interstitial-pyelonephritis-with-retroperitoneal-fibrosis
#12
Manish Soneja, S Achintya, Neeraj Nischal, Piyush Ranjan, Surabhi Vyas, Sudheer Arava, S K Sharma
A large proportion of patients with retroperitoneal fibrosis (RPF) have been labelled as having idiopathic RPF historically. Recent reports of a subset of these patients having a fibro-inflammatory condition with characteristic histology and good response to therapy has helped in renewed understanding of an old disease. A 40 year old man presented with vague abdominal discomfort of 5 months duration. Ultrasound examination revealed soft tissue thickening with calcification over lower abdominal aorta. CT scan characterized the lesion as a retroperitoneal mass encasing the aorta with an associated lesion in upper pole of left kidney...
June 2016: Journal of the Association of Physicians of India
https://www.readbyqxmd.com/read/27720730/no-definite-clinical-features-of-igg4-related-disease-in-patients-with-pulmonary-nodular-lymphoid-hyperplasia
#13
Melanie C Bois, Hiroshi Sekiguchi, Jay H Ryu, Eunhee S Yi
Pulmonary nodular lymphoid hyperplasia (PNLH) may show heavy lymphoplasmacytic infiltrates with increased Immunoglobulin G4 (IgG4)-positive plasma cells as in IgG4-related disease (IgG4-RD). However, it is unclear whether PNLH could be a manifestation of IgG4-RD. A recent study reported the presence of Epstein-Barr virus (EBV) in IgG4-related lymphadenopathy. We postulated that a subset of PNLH might represent IgG4-related lung disease (IgG4-RLD) with EBV-positive lymphocytes as has been reported in IgG4-related lymphadenopathy...
October 5, 2016: Human Pathology
https://www.readbyqxmd.com/read/27716337/clinical-and-paraclinical-profile-and-predictors-of-outcome-in-90-cases-of-scrub-typhus-meghalaya-india
#14
Sunuraj Sivarajan, Siddharudha Shivalli, Debomallya Bhuyan, Michael Mawlong, Rittwick Barman
BACKGROUND: India is an integral component of "tsutsugamushi triangle" which depicts a part of the globe endemic to scrub typhus. Owing to frequent outbreaks witnessed in different parts of the country in the recent past, scrub typhus is described as a re-emerging infectious disease in India. The present study aimed to study the clinical and paraclinical profile, complications and predictors of outcome among 90 cases of scrub typhus diagnosed in a hospital of north-eastern India from Sept 2011 to Aug 2012...
October 5, 2016: Infectious Diseases of Poverty
https://www.readbyqxmd.com/read/27668357/efficacy-and-safety-of-rituximab-therapy-in-neuromyelitis-optica-spectrum-disorders-a-systematic-review-and-meta-analysis
#15
Valentina Damato, Amelia Evoli, Raffaele Iorio
Importance: Neuromyelitis optica spectrum disorders (NMOSDs) are autoimmune astrocytopathies characterized by predominant involvement of the optic nerves and spinal cord. In most patients, an IgG autoantibody binding to astrocytic aquaporin 4, the principal water channel of the central nervous system, is detected. Rituximab, a chimeric monoclonal antibody specific for the CD20 B-lymphocyte surface antigen, has been increasingly adopted as a first-line off-label treatment for patients with NMOSDs...
September 26, 2016: JAMA Neurology
https://www.readbyqxmd.com/read/27637985/ibrutinib-for-patients-with-relapsed-or-refractory-chronic-lymphocytic-leukaemia-with-17p-deletion-resonate-17-a-phase-2-open-label-multicentre-study
#16
Susan O'Brien, Jeffrey A Jones, Steven E Coutre, Anthony R Mato, Peter Hillmen, Constantine Tam, Anders Österborg, Tanya Siddiqi, Michael J Thirman, Richard R Furman, Osman Ilhan, Michael J Keating, Timothy G Call, Jennifer R Brown, Michelle Stevens-Brogan, Yunfeng Li, Fong Clow, Danelle F James, Alvina D Chu, Michael Hallek, Stephan Stilgenbauer
BACKGROUND: The TP53 gene, encoding tumour suppressor protein p53, is located on the short arm of chromosome 17 (17p). Patients with 17p deletion (del17p) chronic lymphocytic leukaemia have poor responses and survival after chemoimmunotherapy. We assessed the activity and safety of ibrutinib, an oral covalent inhibitor of Bruton's tyrosine kinase, in relapsed or refractory patients with del17p chronic lymphocytic leukaemia or small lymphocytic lymphoma. METHODS: We did a multicentre, international, open-label, single-arm study at 40 sites in the USA, Canada, Europe, Australia, and New Zealand...
October 2016: Lancet Oncology
https://www.readbyqxmd.com/read/27628523/-pre-endoscopy-point-of-care-test-simtomax-iga-igg-deamidated-gliadin-peptide-for-coeliac-disease-in-iron-deficiency-anaemia-diagnostic-accuracy-and-a-cost-saving-economic-model
#17
Michelle Shui Yee Lau, Peter Mooney, William White, Victoria Appleby, Sulleman Moreea, Ismail Haythem, Joshua Elias, Kiran Bundhoo, Gareth Corbett, Liam Wong, Her Hsin Tsai, Simon Cross, John Hebden, Sami Hoque, David Sanders
BACKGROUND: International guidelines recommend coeliac serology in iron deficiency anaemia, and duodenal biopsy for those tested positive to detect coeliac disease. However, pre-endoscopy serology is often unavailable, thus committing endoscopists to take routine duodenal biopsies. Some endoscopists consider duodenal biopsy mandatory in anaemia to exclude other pathologies. We hypothesise that using a point of care test at endoscopy could fill this gap, by providing rapid results to target anaemic patients who require biopsies, and save costs by biopsy avoidance...
September 15, 2016: BMC Gastroenterology
https://www.readbyqxmd.com/read/27619393/impact-of-low-immunoglobulin-g-levels-on-disease-outcomes-in-patients-with-inflammatory-bowel-diseases
#18
Nicholas Horton, Xianrui Wu, Jessica Philpott, Ari Garber, Jean-Paul Achkar, Aaron Brzezinski, Bret A Lashner, Bo Shen
BACKGROUND: Inflammatory bowel diseases (IBDs) are considered immune-mediated disorders with dysregulated innate and adaptive immunities. Secondary immunogloblin deficiency can occur in IBD and its impact on the disease course of IBD is not clear. AIMS: We sought to determine associations between low IgG/G1 levels and poor clinical outcomes in IBD patients. METHODS: This historic cohort study was performed on IBD patients with obtained IgG/IgG1 levels...
November 2016: Digestive Diseases and Sciences
https://www.readbyqxmd.com/read/27613250/efficacy-safety-tolerability-and-pharmacokinetics-of-a-novel-human-immune-globulin-subcutaneous-20-a-phase-2-3-study-in-europe-in-patients-with-primary-immunodeficiencies
#19
M Borte, G Kriván, B Derfalvi, L Maródi, T Harrer, S Jolles, C Bourgeois, W Engl, H Leibl, B McCoy, D Gelmont, L Yel
A highly concentrated (20%) immunoglobulin (Ig)G preparation for subcutaneous administration (IGSC 20%), would offer a new option for antibody replacement therapy in patients with primary immunodeficiency diseases (PIDD). The efficacy, safety, tolerability and pharmacokinetics of IGSC 20% were evaluated in a prospective trial in Europe in 49 patients with PIDD aged 2-67 years. Over a median of 358 days, patients received 2349 IGSC 20% infusions at monthly doses equivalent to those administered for previous intravenous or subcutaneous IgG treatment...
September 10, 2016: Clinical and Experimental Immunology
https://www.readbyqxmd.com/read/27600386/house-dust-mite-allergens-new-discoveries-and-relevance-to-the-allergic-patient
#20
REVIEW
Wayne R Thomas
PURPOSE OF REVIEW: Recent findings on house dust allergens and their contribution to knowledge that will significantly impact on current and future allergy treatments are appraised. RECENT FINDINGS: Quantitation of IgE binding to a spectrum of allergen components in several independent studies in varying locations has largely affirmed the main components as the groups 1 and 2 and possibly 23 allergens with mid-tier contributions from the groups 4, 5, 7, and 21. Prevalent binding to Der p 23 has been recapitulated sometimes with low titers...
September 2016: Current Allergy and Asthma Reports
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