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Autoimmune myasthenia

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https://www.readbyqxmd.com/read/28923775/immunoadsorption-using-immusorba-tr-and-ph
#1
REVIEW
Ryuichiro Hirano, Noriko Hirata
Immusorba TR (IM-TR) and PH (IM-PH) were developed as immunoadsorbents from nonbiological materials as affinity ligands for removal of pathogenic substances. The immunoadsorbents in IM-TR and IM-PH are immobilized on a polyvinyl alcohol gel with tryptophan and phenylalanine, respectively, as a ligand. IM-TR is mainly clinically applied to autoimmune neurological diseases such as myasthenia gravis, Guillain-Barré syndrome, and multiple sclerosis. IM-PH is also applied to neurological diseases but mainly to rheumatic diseases such as rheumatoid arthritis and systemic lupus erythematosus...
August 30, 2017: Transfusion and Apheresis Science
https://www.readbyqxmd.com/read/28919008/immunoadsorption-in-neurological-disorders
#2
REVIEW
Satoru Oji, Kyoichi Nomura
In recent years, immunoadsorption has been increasingly recognized as an alternative to therapeutic plasma exchange and used for the treatment of neurological disorders such as Guillain-Barré syndrome, chronic inflammatory demyelinating polyneuropathy, myasthenia gravis, neuromyelitis optica spectrum disorders, and multiple sclerosis, as well as autoimmune encephalitis. Unlike therapeutic plasma exchange, which requires fluid replacement with a blood solution such as fresh frozen plasma or albumin, immunoadsorption is a blood purification technique that enables the selective removal of humoral factors from separated plasma through a high-affinity adsorbent with tryptophan or phenylalanine...
August 26, 2017: Transfusion and Apheresis Science
https://www.readbyqxmd.com/read/28916122/myasthenia-gravis-and-crisis-evaluation-and-management-in-the-emergency-department
#3
Jamie Roper, M Emily Fleming, Brit Long, Alex Koyfman
BACKGROUND: Myasthenia gravis (MG) is an uncommon autoimmune disorder affecting the neuromuscular junction and manifesting as muscle weakness. A multitude of stressors can exacerbate MG. When symptoms are exacerbated, muscle weakness can be severe enough to result in respiratory failure, a condition known as myasthenic crisis (MC). OBJECTIVE: This review discusses risk factors, diagnosis, management, and iatrogenic avoidance of MC. DISCUSSION: MC can affect any age, ethnicity, or sex and can be precipitated with any stressor, infection being the most common...
September 12, 2017: Journal of Emergency Medicine
https://www.readbyqxmd.com/read/28914199/clinical-applications-of-intravenous-immunoglobulins-in-child-neurology
#4
Maria Gogou, Efimia Papadopoulou-Alataki, Martha Spilioti, Sofia Alataki, Athanasios Evangeliou
BACKGROUND: While there are guidelines for the use of intravenous immunoglobulins in children with Guillain-Barre syndrome and myasthenia gravis based on high-level evidence studies, data is fewer for the majority of neurologic disorders in this age group. Neuronal antibodies are detected in children with seizures of autoimmune etiology. Intravenous immunoglobulins with their broad immunomodulatory mechanism of action could be ideally effective in different forms of immune-dysregulated intractable epilepsies such as autoimmune epilepsy and autoimmune Rasmussen encephalitis...
September 15, 2017: Current Pharmaceutical Biotechnology
https://www.readbyqxmd.com/read/28912035/specific-removal-of-autoantibodies-by-extracorporeal-immunoadsorption-ameliorates-experimental-autoimmune-myasthenia-gravis
#5
Konstantinos Lazaridis, Ioannis Dalianoudis, Vasiliki Baltatzidi, Socrates J Tzartos
Myasthenia gravis (MG) is caused by autoantibodies, the majority of which target the muscle acetylcholine receptor (AChR). Plasmapheresis and IgG-immunoadsorption are useful therapy options, but are highly non-specific. Antigen-specific immunoadsorption would remove only the pathogenic autoantibodies, reducing the possibility of side effects while maximizing the benefit. We have extensively characterized such adsorbents, but in vivo studies are missing. We used rats with experimental autoimmune MG to perform antigen-specific immunoadsorptions over three weeks, regularly monitoring symptoms and autoantibody titers...
September 6, 2017: Journal of Neuroimmunology
https://www.readbyqxmd.com/read/28893803/pure-red-cell-aplasia-and-myasthenia-gravis-a-patient-having-both-autoimmune-conditions-in-the-absence-of-thymoma
#6
Annelise Aquilina, David James Camilleri, Josanne Aquilina
This is a patient who was presented initially with symptoms of malaise, tiredness and exertional dyspnoea and found to have a severe normocytic normochromic anaemia with low reticulocyte counts. Bone marrow confirmed the diagnosis of pure red cell aplasia (PRCA) and at the time serology for recent parvovirus infection was positive. He was successfully treated with transfusions and intravenous Ig. Six years later, he had a mild relapse of his PRCA and subsequently developed severe dysphagia and dysarthria which were fatigable...
September 11, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28890037/the-association-between-scabies-and-myasthenia-gravis-a-nationwide-population-based-cohort-study
#7
Ren-Jun Hsu, Chien-Yu Lin, Fung-Wei Chang, Chun-Fa Huang, Heng-Chang Chuang, Jui-Ming Liu
Scabies is an infectious inflammatory pruritic skin disease. Cytokine-mediated inflammatory processes contribute to the pathologic mechanism in scabies. Myasthenia gravis (MG) is also an autoimmune disease that is mediated by cytokines. The study aimed to investigate the association between scabies and myasthenia gravis. We conducted a nationwide population-based cohort study utilized data from the National Health Insurance Research Database (NHIRD) of Taiwan. Patients with scabies (n=5429) and control subjects without scabies (n=20,176) were enrolled...
September 7, 2017: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
https://www.readbyqxmd.com/read/28885698/autoimmune-antibodies-to-collagen-xiii-in-myasthenia-gravis-patients
#8
Hongmin Tu, Ritva Pirskanen-Matell, Anne Heikkinen, Tuomo Oikarainen, Juha Risteli, Taina Pihlajaniemi
INTRODUCTION: Myasthenia Gravis (MG) is a neuromuscular junction (NMJ) disorder caused by autoantibodies against NMJ proteins. Collagen XIII is a muscle-derived transmembrane protein required for NMJ maturation. The objective of this study is to explore existence of autoantibodies to collagen XIII in MG patients. METHODS: Seventy MG patient sera and 61 human healthy controls were screened for collagen XIII autoantibodies by enzyme-linked immunosorbent assay (ELISA)...
September 8, 2017: Muscle & Nerve
https://www.readbyqxmd.com/read/28881516/-effectiveness-and-safety-of-rituximab-for-children-with-autoimmune-diseases-of-the-nervous-system
#9
Z Fu, X H Bao, Y Wu, J Zhou, Y H Zhang, Y Zhang, T Y Ji, Y Chen
Objective: To assess the effectiveness and safety of rituximab in Chinese children with autoimmune diseases of the nervous system. Method: An ambispective cohort study enrolled patients with refractory and(or) relapse autoimmune diseases of nervous system from June 2010 to June 2016 in Peking University First Hospital.These patients failed to respond to steroids and(or)intravenous immunoglobulin (IVIG) were treated with rituximab and seen for follow-up visits once every 3 months.The effectiveness was assessed by modified Rankin scale (mRs) and the annualized relapse rate...
September 2, 2017: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/28878127/autoantibody-producing-plasmablasts-after-b-cell-depletion-identified-in-muscle-specific-kinase-myasthenia-gravis
#10
Panos Stathopoulos, Aditya Kumar, Richard J Nowak, Kevin C O'Connor
Myasthenia gravis (MG) is a B cell-mediated autoimmune disorder of neuromuscular transmission. Pathogenic autoantibodies to muscle-specific tyrosine kinase (MuSK) can be found in patients with MG who do not have detectable antibodies to the acetylcholine receptor (AChR). MuSK MG includes immunological and clinical features that are generally distinct from AChR MG, particularly regarding responsiveness to therapy. B cell depletion has been shown to affect a decline in serum autoantibodies and to induce sustained clinical improvement in the majority of MuSK MG patients...
September 7, 2017: JCI Insight
https://www.readbyqxmd.com/read/28875521/chronic-myocarditis-with-a-long-clinical-course-report-of-an-autopsy-case-of-probable-autoimmune-myocarditis
#11
Masayuki Shintaku, Koji Uchiyama, Yohei Kobayashi
The patient was a 54-year-old woman, who died of chronic cardiac insufficiency after a clinical course of 2 years and 4 months. She had complained of myalgia, muscle weakness, and blepharoptosis before the onset of cardiac symptoms, but there was no evidence of myasthenia gravis or collagen-vascular diseases. At autopsy, the heart (280 g) showed marked dilatation of the four chambers and thinning of the ventricular walls. Diffuse and intense lymphocytic infiltration and extensive fibrosis were noted, with the latter being accentuated in the subendocardial and subepicardial zones...
September 6, 2017: Pathology International
https://www.readbyqxmd.com/read/28874083/azathioprine-induced-pancytopenia-and-septic-complications-a-probable-cause-of-death
#12
Bijoy K Panda, Siddhi Umarje, Arundhati Diwan
Azathioprine, an immunosuppressant which is widely used in the management of the autoimmune neuromuscular disorder. Myasthenia gravis is known to cause myelotoxicity. A 55-year-old male recently diagnosed with myasthenia gravis and chronic kidney disease was put on azathioprine (100 mg/d) along with pyridostigmine and prednisolone. When the treatment was initiated, the hematological reports revealed normal levels of blood count. However, approximately within 3 weeks of continuing the prescribed drugs, the patient was readmitted for complaints of loose watery stools, weakness, and giddiness...
January 1, 2017: Journal of Pharmacy Practice
https://www.readbyqxmd.com/read/28869483/obinutuzumab-plus-chlorambucil-in-a-patient-with-severe-myasthenia-gravis-and-chronic-lymphocytic-leukemia
#13
Angela Russell, Megan Yaraskavitch, Daniel Fok, Sameer Chhibber, Lesley Street, Lawrence Korngut
Myasthenia gravis (MG) is an autoimmune disease of the neuromuscular junction, characterized by fatigable weakness of the extraocular, bulbar, and limb musculature; prevalence is estimated at 14 to 32 per 100,000 in North America. Chronic lymphocytic leukemia (CLL) is the most common type of leukemia in adults, resulting from clonal expansion of B-cells in blood, marrow, and secondary lymphoid tissues. The simultaneous presentation of MG and CLL is exceedingly rare. This article presents the case of 71-year-old man diagnosed simultaneously with MG and CLL...
2017: Journal of Neuromuscular Diseases
https://www.readbyqxmd.com/read/28851660/thymoma-a-clinicopathological-correlation-of-1470-cases
#14
Annikka Weissferdt, Neda Kalhor, Justin A Bishop, Se Jin Jang, Jae Ro, Fredrik Petersson, Bingcheng Wu, Gerald Langman, Hollie Bancroft, Yalan Bi, Yunxiao Meng, Filomena Medeiros, Hans Brunnstrom, Dominic Spagnolo, Siaw Ming Chai, Andrew Laycock, Paul E Wakely, Goran Elmberger, Fernando A Soares, Antonio H Campos, Derya Gumurdulu, Isabel Alvarado-Cabrero, Domenico Coppola, Arlene M Correa, David Rice, Reza J Mehran, Boris Sepesi, Garrett Walsh, Larry Kaiser, Cesar A Moran
We present 1470 surgical resections for thymoma identified in the pathology files of 14 institutions from 11 countries with the purpose to determine and correlate a simplified histological classification of thymoma and pathological staging with clinical outcome. The study population was comprised of 720 men and 750 women between the ages of 12 and 86years (average: 54.8years). Clinically 137 patients (17%) had a history of myasthenia gravis, 31 patients (3.8%) of other autoimmune disease, and 55 (6.8%) patients of another neoplastic process...
August 26, 2017: Human Pathology
https://www.readbyqxmd.com/read/28844501/onx-0914-a-selective-inhibitor-of-immunoproteasome-ameliorates-experimental-autoimmune-myasthenia-gravis-by-modulating-humoral-response
#15
Ru-Tao Liu, Peng Zhang, Chun-Lin Yang, Yu Pang, Min Zhang, Na Zhang, Long-Tao Yue, Xiao-Li Li, Heng Li, Rui-Sheng Duan
Accumulating evidence shows that the immunoproteasome participates in the immune response, beyond its initial role in the protein degradation. Here, we tested the effects of the selective immunoproteasome inhibitor, ONX-0914, on experimental autoimmune myasthenia gravis (EAMG). We found that ONX-0914 ameliorated the severity of ongoing EAMG by reducing the autoantibody affinity, accompanied with decreased Tfh cells and antigen presenting cells. Also it reduced the percentage of Th17 cells and inhibited the secretion of IL-17...
August 18, 2017: Journal of Neuroimmunology
https://www.readbyqxmd.com/read/28827490/triple-furrowed-atrophic-tongue-of-myasthenia-gravis
#16
Nathan P Young, Eric J Sorenson, Margherita Milone, C Michel Harper
The authors present a case and image of a patient with refractory tongue weakness and characteristic triple furrowed pattern of atrophy due to autoimmune myasthenia gravis.
September 2017: Journal of Clinical Neuromuscular Disease
https://www.readbyqxmd.com/read/28822831/ifna-as1-regulates-cd4-t-cell-activation-in-myasthenia-gravis-though-hla-drb1
#17
Mengchuan Luo, Xiaofang Liu, Huanyu Meng, Liqun Xu, Yi Li, Zhibin Li, Chang Liu, Yue-Bei Luo, Bo Hu, Yuanyuan Xue, Yu Liu, Zhaohui Luo, Huan Yang
Abnormal CD4(+)T cell activation is known to play roles in the pathogenesis of myasthenia gravis (MG). However, little is known about the mechanisms underlying the roles of lncRNAs in regulating CD4(+) T cell. In this study, we discovered that the lncRNA IFNG-AS1 is abnormally expressed in MG patients associated with quantitative myasthenia gravis (QMG) and the positive anti-AchR Ab levels patients. IFNG-AS1 influenced Th1/Treg cell proliferation and regulated the expression levels of their transcription factors in an experimental autoimmune myasthenia gravis (EAMG)model...
August 16, 2017: Clinical Immunology: the Official Journal of the Clinical Immunology Society
https://www.readbyqxmd.com/read/28801669/balance-between-estrogens-and-proinflammatory-cytokines-regulates-chemokine-production-involved-in-thymic-germinal-center-formation
#18
Nadine Dragin, Patrice Nancy, José Villegas, Régine Roussin, Rozen Le Panse, Sonia Berrih-Aknin
The early-onset form of Myasthenia Gravis (MG) is prevalent in women and associates with ectopic germinal centers (GCs) development and inflammation in the thymus. we aimed to investigate the contribution of estrogens in the molecular processes involved in thymic GCs formation. We examined expression of genes involved in anti-acetylcholine receptor (AChR) response in MG, MHC class II and α-AChR subunit as well as chemokines involved in GC development (CXCL13, CCL21and CXCL12). In resting conditions, estrogens have strong regulatory effects on thymic epithelial cells (TECs), inducing a decreased protein expression of the above molecules...
August 11, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28796013/electroconvulsive-therapy-for-depression-comorbid-with-myasthenia-gravis-a-case-report-and-review-of-the-literature
#19
Mark B Warren, Scott Elder, Norman P Litchfield
OBJECTIVES: Myasthenia gravis (MG) is a rare but well-described autoimmune disease, which is sometimes comorbid with psychiatric illness. There have been several case reports describing the use of electroconvulsive therapy (ECT) for the treatment of core psychopathology in the context of MG. We sought to review the available published data on ECT in MG and add another case example to the literature. METHODS: We performed a PubMed search for relevant articles or case reports in English describing ECT in MG and summarized findings...
August 9, 2017: Journal of ECT
https://www.readbyqxmd.com/read/28782655/on-the-role-of-igg4-in-inflammatory-conditions-lessons-for-igg4-related-disease
#20
REVIEW
David C Trampert, Lowiek M Hubers, Stan F J van de Graaf, Ulrich Beuers
The pathophysiology of immunoglobulin G4-related disease (IgG4-RD) and its most common manifestations, IgG4-associated (sclerosing) cholangitis and autoimmune pancreatitis, remains largely unknown, but IgG4 is presumably involved. IgG4 is a promiscuous antibody, which could be directly pathogenic, fulfill a protective role, or could just be a fortuitous marker of an aberrant inflammatory response. IgG4 antibodies possess exclusive structural and functional characteristics suggesting anti-inflammatory and tolerance-inducing effects...
August 4, 2017: Biochimica et Biophysica Acta
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