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Myasthenia

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https://www.readbyqxmd.com/read/29145329/new-onset-of-myasthenia-gravis-after-intravesical-bacillus-calmette-guerin-a-case-report-and-literature-review
#1
Tsubasa Takizawa, Marenori Kojima, Shigeaki Suzuki, Takashi Osada, Satoshi Kitagawa, Jin Nakahara, Shinichi Takahashi, Norihiro Suzuki
RATIONALE: Recently, drug-related myasthenia gravis (MG) has received attention, because the number of reported cases involving MG associated with immune checkpoint inhibitors, a new immunotherapy, is increasing. We present a case involving the new onset of MG, in which the symptoms started shortly after intravesical Bacillus Calmette-Guerin (BCG) for bladder cancer. PATIENT CONCERNS: A 69-year-old male with bladder cancer developed ptosis and diplopia 4 days after the completion of a treatment regimen with intravesical BCG weekly for 6 weeks...
November 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29143359/indolent-t-lymphoblastic-proliferation-concomitant-to-acinic-cell-carcinoma-mimicking-t-lymphoblastic-lymphoma-case-report-and-literature-review
#2
Hajime Yasuda, Miyuki Tsutsui, Yasunori Ota, Masaru Tanaka, Norio Komatsu
Indolent T-lymphoblastic proliferation (iT-LBP) is a non-clonal benign condition showing extrathymic proliferation of T-lymphoblasts positive for CD3, CD4, CD8, and TdT. Isolated iT-LBP has been observed, but the majority of iT-LBP have been seen in conjunction with other disorders including Castleman disease, hepatocellular carcinoma, follicular dendritic cell tumors, angioimmunoblastic T-cell lymphoma, myasthenia gravis, and acinic cell carcinoma (ACC). The clinical course of iT-LBP is indolent, and no therapy is usually required...
November 15, 2017: Histopathology
https://www.readbyqxmd.com/read/29141361/-effect-of-probucol-on-preventing-contrast-induced-nephropathy-in-patients-undergoing-percutaneous-coronary-intervention
#3
X Q Suo, S C Yang, Z H Ma, T T Sun, W Y Zhang, H L Cong, W H Lin, C Z Lu, F S Tian, N K Fu
Objective: To investigate the preventive effect, possible mechanism and safety of probucol on contrast-induced nephropathy (CIN) after percutaneous coronary intervention (PCI) in patients with coronary heart disease (CHD). Methods: A total of 641 patients with coronary heart disease were consecutively enrolled from Department of Cardiology, in Tianjin Chest Hospital, Tianjin TEDA International Cardiovascular Hospital, Tianjin First Central Hospital, Tianjin Fourth Central Hospital. They were randomly divided into probucol group (n=321) and control group (n=320)...
November 7, 2017: Zhonghua Yi Xue za Zhi [Chinese medical journal]
https://www.readbyqxmd.com/read/29139384/-seronegative-myasthenia-gravis
#4
Oana Catar, Anne-Catherine Aubé-Nathier, Aleksandra Nadaj-Pakleza
No abstract text is available yet for this article.
November 2017: Médecine Sciences: M/S
https://www.readbyqxmd.com/read/29138598/familial-aggregation-of-myasthenia-gravis-in-affected-families-a-population-based-study
#5
Fu-Chao Liu, Chang-Fu Kuo, Lai-Chu See, Hsin-I Tsai, Huang-Ping Yu
Introduction: Myasthenia gravis (MG) is clinically heterogeneous and can be life-threatening if bulbar or respiratory muscles are involved. However, relative contributions of genetic, shared, and nonshared environmental factors to MG susceptibility remain unclear. The aim of this study was to examine the familial aggregation and heritability of MG and the relative risks (RRs) of other autoimmune diseases in the relatives of patients with MG. Methods: A population-based family study using the Taiwan National Health Insurance (NHI) Database was conducted...
2017: Clinical Epidemiology
https://www.readbyqxmd.com/read/29130637/how-chromosomal-deletions-can-unmask-recessive-mutations-deletions-in-10q11-2-associated-with-chat-or-slc18a3-mutations-lead-to-congenital-myasthenic-syndrome
#6
Mathias Schwartz, Damien Sternberg, Sandra Whalen, Alexandra Afenjar, Arnaud Isapof, Brigitte Chabrol, Marie-France Portnoï, Solveig Heide, Boris Keren, Sandra Chantot-Bastaraud, Jean-Pierre Siffroi
A congenital myasthenia was suspected in two unrelated children with very similar phenotypes including several episodes of severe dyspnea. Both children had a 10q11.2 deletion revealed by Single Nucleotide Polymorphisms array or by Next Generation Sequencing analysis. The deletion was inherited from the healthy mother in the first case. These deletions unmasked a recessive mutation at the same locus in both cases, but in two different genes: CHAT and SLC18A3.
November 12, 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/29125188/the-mouse-passive-transfer-model-of-musk-myasthenia-gravis-disrupted-musk-signaling-causes-synapse-failure
#7
REVIEW
Nazanin Ghazanfari, Sofie Trajanovska, Marco Morsch, Simon X Liang, Stephen W Reddel, William D Phillips
While the majority of myasthenia gravis patients express antibodies targeting the acetylcholine receptor, the second most common cohort instead displays autoantibodies against muscle-specific kinase (MuSK). MuSK is a transmembrane tyrosine kinase found in the postsynaptic membrane of the neuromuscular junction. During development, MuSK serves as a signaling hub, coordinating the alignment of the pre- and postsynaptic components of the synapse. Adult mice that received repeated daily injections of IgG from anti-MuSK(+) myasthenia gravis patients developed muscle weakness, associated with neuromuscular transmission failure...
November 10, 2017: Annals of the New York Academy of Sciences
https://www.readbyqxmd.com/read/29125185/thymus-involvement-in-early-onset-myasthenia-gravis
#8
REVIEW
Mélanie A Cron, Solène Maillard, José Villegas, Frédérique Truffault, Muriel Sudres, Nadine Dragin, Sonia Berrih-Aknin, Rozen Le Panse
It has long been established that the thymus plays a central role in autoimmune myasthenia gravis (MG) because of either thymoma or thymic hyperplasia of lymphoproliferative origin. In this review, we discuss thymic changes associated with thymic hyperplasia and their implications in the development of an autoimmune response against the acetylcholine receptor (AChR).The hyperplastic MG thymus displays all the characteristics of tertiary lymphoid organs (TLOs): neoangiogenic processes with high endothelial venule and lymphatic vessel development, chemokine overexpression favoring peripheral cell recruitment, and ectopic germinal center development...
November 10, 2017: Annals of the New York Academy of Sciences
https://www.readbyqxmd.com/read/29125182/circulating-micrornas-as-potential-biomarkers-in-myasthenia-gravis-patients
#9
REVIEW
Anna Rostedt Punga, Tanel Punga
MicroRNAs (miRNAs) are small noncoding RNA molecules that bind to specific mRNA targets and regulate a wide range of important biological processes within cells. Circulating miRNAs are released into the extracellular space and can be measured in most biofluids, including blood serum and plasma. Recently, circulating miRNAs have emerged as easily accessible markers in various body fluids with different profiles and quantities specific for different human disorders, including autoimmune diseases. In myasthenia gravis (MG), diagnostic tests such as titers of serum autoantibodies specific for either the acetylcholine receptor (AChR(+) ) or muscle-specific tyrosine kinase (MuSK(+) ) do not necessarily reflect disease progression, and there is a great need for reliable objective biomarkers to monitor the disease course and therapeutic response...
November 10, 2017: Annals of the New York Academy of Sciences
https://www.readbyqxmd.com/read/29124031/successful-lung-transplantation-in-a-patient-with-myasthenia-gravis
#10
Kangmin Kim, Hyun Joo Lee, Samina Park, Yoohwa Hwang, Young Whan Kim, Young Tae Kim
A 47-year-old man with myasthenia gravis (MG) was admitted for a lung transplant. He had bronchiolitis obliterans after allogeneic hematopoietic stem cell transplantation due to acute myeloid leukemia. MG developed after stem cell transplantation. Bilateral sequential lung transplantations and a total thymectomy were performed. The patient underwent right diaphragmatic plication simultaneously due to preoperatively diagnosed right diaphragmatic paralysis. A tracheostomy was performed and bilevel positive airway pressure (BiPAP) was applied on postoperative days 8 and 9, respectively...
October 2017: Korean Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/29121404/the-association-of-british-neurologists-myasthenia-gravis-guidelines
#11
Jon Sussman, Maria E Farrugia, Paul Maddison, Marguerite Hill, M Isabel Leite, David Hilton-Jones
Myasthenia gravis (MG) is an autoimmune disease of the neuromuscular junction for which many therapies were developed before the era of evidence-based medicine. Despite the basic principles of treatment being well known, patients continue to receive suboptimal treatment. A myasthenia gravis guidelines group was therefore established under the aegis of the Association of British Neurologists. These guidelines attempt to steer a path between evidence-based practice where available and established best practice where evidence is unavailable...
November 9, 2017: Annals of the New York Academy of Sciences
https://www.readbyqxmd.com/read/29114250/a-sensitive-method-for-detecting-peptide-specific-cd4-t-cell-responses-in-peripheral-blood-from-patients-with-myasthenia-gravis
#12
Sapna Sharma, Clas Malmeström, Christopher Lindberg, Sarah Meisel, Karin Schön, Martina Verolin, Nils Yngve Lycke
Myasthenia gravis (MG) is an autoimmune neurological disorder typified by skeletal muscle fatigue and most often production of autoantibodies against the nicotinic acetylcholine receptor (AChR). The present study was undertaken to assess the extent of AChR-peptide recognition in MG patients using co-culturing (DC:TC) of autologous monocyte-derived dendritic cells (moDCs) and highly enriched CD4(+) T cells from the blood as compared to the traditional whole peripheral blood mononuclear cell (PBMC) cultures. We found that the DC:TC cultures were highly superior to the PBMC cultures for detection of reactivity toward HLA-DQ/DR-restricted AChR-peptides...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/29111026/a-retrospective-study-of-acetylcholine-receptor-antibody-positive-ocular-myasthenia-in-the-west-of-scotland
#13
Maria E Farrugia, Marie Cleary, Caroline Carmichael
Ocular myasthenia is the milder end of the myasthenia gravis spectrum but treatment can be challenging especially in older patients. We retrospectively studied all patients on our database with ocular myasthenia (OMG), positive for acetylcholine receptor (AChR) antibodies. We identified 93 patients (64 men and 29 women). The mean age at disease onset was 63y, median 68y. Most (72%) experienced ptosis with diplopia; 19% experienced ptosis alone, while 7.5% complained of diplopia without ptosis. As expected, pyridostigmine was commenced early at diagnosis in the majority (69%) and 20% were still receiving pyridostigmine at final review...
November 15, 2017: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/29109003/prediction-of-postoperative-mechanical-ventilation-after-thymectomy-in-patients-with-myasthenia-gravis-a-myth-or-reality
#14
EDITORIAL
Javier H Campos
No abstract text is available yet for this article.
August 10, 2017: Journal of Cardiothoracic and Vascular Anesthesia
https://www.readbyqxmd.com/read/29104781/the-minor-variant-of-the-single-nucleotide-polymorphism-rs3753381-affects-the-activity-of-a-slamf1-enhancer
#15
L V Putlyaeva, A M Schwartz, A V Klepikova, I E Vorontsov, I V Kulakovskiy, D V Kuprash
The SLAMF1 gene encodes CD150, a transmembrane glycoprotein expressed on the surface of T and B-lymphocytes, NK-cells, dendritic cells, and subpopulations of macrophages and basophils. We investigated the functional regulatory polymorphisms of the SLAMF1 locus associated with autoimmune processes, using bioinformatics and a mutational analysis of the regulatory elements overlapping with polymorphic positions. In the reporter gene assay in MP-1 and Raji B-cell lines, the enhancer activity of the regulatory region of the locus containing the rs3753381 polymorphism demonstrated a twofold increase upon the introduction of the rs3753381 minor variant (G → A) associated with myasthenia gravis...
July 2017: Acta Naturae
https://www.readbyqxmd.com/read/29101274/myasthenia-gravis-impairment-index-responsiveness-meaningful-change-and-relative-efficiency
#16
Carolina Barnett, Vera Bril, Moira Kapral, Abhaya V Kulkarni, Aileen M Davis
OBJECTIVE: To study responsiveness and meaningful change of the Myasthenia Gravis Impairment Index (MGII) and its relative efficiency compared to other measures. METHODS: We enrolled 95 patients receiving prednisone, IV immunoglobulin (IVIg), or plasma exchange (PLEX) and 54 controls. Patients were assessed with the MGII and other measures-including the Quantitative Myasthenia Gravis Score, Myasthenia Gravis Composite, and Myasthenia Gravis Activities of Daily Living-at baseline and 3-4 weeks after treatment...
November 3, 2017: Neurology
https://www.readbyqxmd.com/read/29100295/the-correlation-of-neutrophil-to-lymphocyte-ratio-with-the-presence-and-activity-of-myasthenia-gravis
#17
De-Hao Yang, Mei-Zi Qian, Mao-Mao Wei, Jia Li, Meng-Meng Yu, Xue-Mian Lu, Hong Yang, Hai Lin, Xiang Li, Jun-Yan Zhu, Xu Zhang
Though the pathogenesis of myasthenia gravis (MG) is not fully understood, the role of inflammation has been well appreciated in the development of MG. We aimed to investigate the role of neutrophil-to-lymphocyte ratio (NLR) in MG patients and the relationship between the NLR and the activity of the disease. A total number of 172 MG patients and 207 healthy controls (HC) were enrolled in this study. The MG patients were divided into tertiles according to NLR (low NLR < 1.58, n = 57; intermediate NLR 1.58-2...
September 29, 2017: Oncotarget
https://www.readbyqxmd.com/read/29099348/transient-neonatal-myasthenia-gravis-with-infantile-hypertrophic-pyloric-stenosis-coincidence-or-causation
#18
Aakash Pandita, Deepak Sharma, Srinivas Murki, Tejo Pratap Oleti, Vallamsetty Leelakumar
No abstract text is available yet for this article.
January 1, 2017: Tropical Doctor
https://www.readbyqxmd.com/read/29096852/therapeutic-plasma-exchange-in-a-rare-case-myasthenic-crisis-after-botox-injection
#19
Azita Chegini
BACKGROUND AND AIMS: Botulinum toxin (Botox) injections are used as a cosmetic treatment to decrease wrinkles in face and chin. Being a neurotoxic agent it minimizes muscle activity, while side effects are usually rare. This article subsequently presents one case of these rare effects. CASE: A 30-year-old woman presenting with ptosis, diplopia, dysarthria, dysphagia and muscle weakness was admitted to our hospital. She had no history of disease. For cosmetic reasons, she had three Botox injections during the preceding months...
November 2017: Atherosclerosis. Supplements
https://www.readbyqxmd.com/read/29090020/long-term-treatment-of-refractory-myasthenia-gravis-with-subcutaneous-immunoglobulin
#20
Edina Kovács, Katalin Dankó, Melinda Nagy-Vince, László Csiba, Judit Boczán
No abstract text is available yet for this article.
November 2017: Therapeutic Advances in Neurological Disorders
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