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https://www.readbyqxmd.com/read/29691994/chemical-biochemical-preclinical-and-clinical-studies-of-ganoderma-lucidum-polysaccharide-as-an-approved-drug-for-treating-myopathy-and-other-diseases-in-china
#1
REVIEW
Pengjiao Zeng, Zhihua Guo, Xuan Zeng, Cui Hao, Yiran Zhang, Meng Zhang, Yong Liu, Hui Li, Juan Li, Lijuan Zhang
Ganoderma lucidum is an edible medicinal mushroom known as "Lingzhi" in China and "Reishi or Manetake" in Japan. It is a highly prized vitality-enhancing herb for more than 2000 years. G. lucidum polysaccharide (GLPS) has been identified as one of the major bioactive components and developed into a drug named "Ji 731 Injection" in China since 1973. The large-scale production of the drug began in 1985 and approved by the Chinese FDA as "Polysaccharidum of G. lucidum Karst Injection" (Ling Bao Duo Tang Zhu She Ye) in 2000, which is applied intramuscularly...
April 24, 2018: Journal of Cellular and Molecular Medicine
https://www.readbyqxmd.com/read/29691272/immune-mediated-necrotising-myopathy-a-rare-cause-of-hyperckaemia
#2
Emily Liang, Mandana Rastegar
Immune-mediated necrotising myopathy (IMNM) is a type of inflammatory myopathy characterised by acute or subacute severe proximal muscle weakness, significantly elevated creatine kinase levels, and prominent myofibre necrosis and regeneration with little or no inflammation. A subtype of IMNM identified by anti-HMG-CoA reductase (HMGCR)antibodies has been shown to be associated with statin exposure. Treatment of IMNM consists of immunosuppression with steroids, steroid-sparing agents, intravenous immune globulin and/or biologics...
April 24, 2018: BMJ Case Reports
https://www.readbyqxmd.com/read/29689380/mpa-a-free-accessible-and-efficient-pipeline-for-single-nucleotide-variant-annotation-and-prioritization-for-next-generation-sequencing-routine-molecular-diagnosis
#3
Kevin Yauy, David Baux, Henri Pegeot, Charles Van Goethem, Charly Mathieu, Thomas Guignard, Raul Juntas Morales, Delphine Lacourt, Martin Krahn, Vilma-Lotta Lehtokari, Gisele Bonne, Sylvie Tuffery-Giraud, Michel Koenig, Mireille Cossée
Interpretation of next-generation sequencing data constitutes the main limitation in molecular genetics diagnosis. In diagnosis of myopathies and muscular dystrophies (MMD), another major issue is to efficiently predict pathogenicity of variants identified in large genes, especially TTN, since current in silico prediction tools show limitations to predict and rank the numerous variants of such genes. We propose a unique variant prioritization score called mobidic prioritization algorithm (MPA) based on curated interpretation for previously reported variants, biological assumptions, and splice and missense predictors to prioritize all types of single nucleotide variants...
April 21, 2018: Journal of Molecular Diagnostics: JMD
https://www.readbyqxmd.com/read/29689030/checkpoint-inhibitor-immune-therapy-systemic-indications-and-ophthalmic-side-effects
#4
Lauren A Dalvin, Carol L Shields, Marlana Orloff, Takami Sato, Jerry A Shields
PURPOSE: To review immune checkpoint inhibitor indications and ophthalmic side effects. METHODS: A literature review was performed using a PubMed search for publications between 1990 and 2017. RESULTS: Immune checkpoint inhibitors are designed to treat system malignancies by targeting one of three ligands, leading to T-cell activation for attack against malignant cells. These ligands (and targeted drug) include cytotoxic T-lymphocyte antigen-4 (CTLA-4, ipilimumab), programmed death protein 1 (PD-1, pembrolizumab, nivolumab), and programmed death ligand-1 (PD-L1, atezolizumab, avelumab, durvalumab)...
April 23, 2018: Retina
https://www.readbyqxmd.com/read/29688538/instrumental-compression-force-and-meat-attribute-changes-in-woody-broiler-breast-fillets-during-short-term-storage
#5
X Sun, D A Koltes, C N Coon, K Chen, C M Owens
Woody breast (WB) is a myopathy of the Pectoralis major muscle in broilers that is characterized by increased hardness of the raw fillet. This defect increases economic losses due to associated decreased meat quality and the addition of training skilled laborers to identify problematic fillets. Therefore, the objectives of this study were to determine if compression force (CF) could be used as a non-invasive method of detecting WB and to assess changes in meat attributes during cold storage. Deboned fillets (n = 121) were collected from 8-wk-old broilers, and categorized as normal (NORM, n = 42), mild (MILD, n = 39), and moderate/severe (SEV, n = 40) for WB...
April 23, 2018: Poultry Science
https://www.readbyqxmd.com/read/29687901/thick-filament-protein-network-functions-and-disease-association
#6
Li Wang, Janelle Geist, Alyssa Grogan, Li-Yen R Hu, Aikaterini Kontrogianni-Konstantopoulos
Sarcomeres consist of highly ordered arrays of thick myosin and thin actin filaments along with accessory proteins. Thick filaments occupy the center of sarcomeres where they partially overlap with thin filaments. The sliding of thick filaments past thin filaments is a highly regulated process that occurs in an ATP-dependent manner driving muscle contraction. In addition to myosin that makes up the backbone of the thick filament, four other proteins which are intimately bound to the thick filament, myosin binding protein-C, titin, myomesin, and obscurin play important structural and regulatory roles...
March 13, 2018: Comprehensive Physiology
https://www.readbyqxmd.com/read/29681093/low-level-expression-of-epg5-leads-to-an-attenuated-vici-syndrome-phenotype
#7
Megan A Waldrop, Felecia Gumienny, Daniel Boue, Emily de Los Reyes, Richard Shell, Robert B Weiss, Kevin M Flanigan
Vici syndrome is a multisystem disorder characterized by agenesis of the corpus callosum, oculocutaneous hypopigmentation, cataracts, cardiomyopathy, combined immunodeficiency, failure to thrive, profound developmental delay, and acquired microcephaly. Most individuals are severely affected and have a markedly reduced life span. Here we describe an 8-year-old boy with a history of developmental delay, agenesis of the corpus callosum, failure to thrive, myopathy, and well-controlled epilepsy. He was initially diagnosed with a mitochondrial disorder, based in part upon nonspecific muscle biopsy findings, but mitochondrial DNA mutation analysis revealed no mutations...
May 2018: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/29674613/risk-factors-and-disease-mechanisms-in-myositis
#8
REVIEW
Frederick W Miller, Janine A Lamb, Jens Schmidt, Kanneboyina Nagaraju
Autoimmune diseases develop as a result of chronic inflammation owing to interactions between genes and the environment. However, the mechanisms by which autoimmune diseases evolve remain poorly understood. Newly discovered risk factors and pathogenic processes in the various idiopathic inflammatory myopathy (IIM) phenotypes (known collectively as myositis) have illuminated innovative approaches for understanding these diseases. The HLA 8.1 ancestral haplotype is a key risk factor for major IIM phenotypes in some populations, and several genetic variants associated with other autoimmune diseases have been identified as IIM risk factors...
April 20, 2018: Nature Reviews. Rheumatology
https://www.readbyqxmd.com/read/29674612/autoantibodies-in-myositis
#9
REVIEW
Neil J McHugh, Sarah L Tansley
The discovery of novel autoantigen systems related to idiopathic inflammatory myopathies (collectively referred to as myositis) in adults and children has had major implications for the diagnosis and management of this group of diseases across a wide range of medical specialties. Traditionally, autoantibodies found in patients with myositis are described as being myositis-specific autoantibodies (MSAs) or myositis-associated autoantibodies (MAAs), depending on their prevalence in other, related conditions. However, certain MSAs are more closely associated with extramuscular manifestations, such as skin and lung disease, than with myositis itself...
April 20, 2018: Nature Reviews. Rheumatology
https://www.readbyqxmd.com/read/29673367/comparing-the-importance-of-quality-measurement-themes-in-juvenile-idiopathic-inflammatory-myositis-between-patients-and-families-and-healthcare-professionals
#10
Heather O Tory, Ruy Carrasco, Thomas Griffin, Adam M Huber, Philip Kahn, Angela Byun Robinson, David Zurakowski, Susan Kim
BACKGROUND: A standardized set of quality measures for juvenile idiopathic inflammatory myopathies (JIIM) is not in use. Discordance has been shown between the importance ascribed to quality measures between patients and families and physicians. The objective of this study was to assess and compare the importance of various aspects of high quality care to patients with JIIM and their families with healthcare providers, to aid in future development of comprehensive quality measures. METHODS: Surveys were developed by members of the Childhood Arthritis and Rheumatology Research Alliance (CARRA) Juvenile Dermatomyositis Workgroup through a consensus process and administered to patients and families through the CureJM Foundation and to healthcare professionals through CARRA...
April 19, 2018: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/29670827/vasculitis-and-neurobrucellosis-evaluation-of-nine-cases-using-radiologic-findings
#11
Sule Aydin Turkoglu, Siddika Halicioglu, Fatma Sirmatel, Murside Yildiz, Nebil Yildiz, Serpil Yildiz
Background: Brucellosis is an important multisystemic disease with many different clinical symptoms, and its early diagnosis and treatment are possible. Neurobrucellosis (NB) is a rare but serious finding of brucellosis. Brucella can be seen as meningitis and encephalopathy, and it can cause cranial nerve pathologies, vascular syndromes, myopathy, spinal diseases, and psychiatric disorders. In NB, vascular syndromes secondary to inflammation are rarely seen. Here, we present nine young patients with vascular and nonspecific neuropsychiatric findings who had NB as the etiology of stroke...
April 2018: Brain and Behavior
https://www.readbyqxmd.com/read/29670510/differential-expression-of-several-mirnas-and-the-host-genes-aatk-and-dnm2-in-leukocytes-of-sporadic-als-patients
#12
Katarina Vrabec, Emanuela Boštjančič, Blaž Koritnik, Lea Leonardis, Leja Dolenc Grošelj, Janez Zidar, Boris Rogelj, Damjan Glavač, Metka Ravnik-Glavač
Genetic studies have managed to explain many cases of familial amyotrophic lateral sclerosis (ALS) through mutations in several genes. However, the cause of a majority of sporadic cases remains unknown. Recently, epigenetics, especially miRNA studies, show some promising aspects. We aimed to evaluate the differential expression of 10 miRNAs, including miR-9, miR-338, miR-638, miR-663a, miR-124a, miR-143, miR-451a, miR-132, miR-206, and let-7b, for which some connection to ALS was shown previously in ALS culture cells, animal models or patients, and in three miRNA host genes, including C1orf61 (miR-9), AATK (miR-338), and DNM2 (miR-638), in leukocyte samples of 84 patients with sporadic ALS...
2018: Frontiers in Molecular Neuroscience
https://www.readbyqxmd.com/read/29669494/an-epidemiological-investigation-of-an-idiopathic-myopathy-in-hunting-dogs-in-new-zealand
#13
H Hunt, N J Cave, B D Gartrell, N Cogger, J Petersen, W D Roe
AIM: To conduct an epidemiological investigation of an idiopathic myopathy, known as "Go Slow" (GSM), which was initially recognised in dogs used for pig hunting. A secondary aim was to describe the hunting activities, diet and health of dogs used for pig hunting in New Zealand. METHODS: A retrospective cohort study was conducted between June 2014-June 2017. Cases of GSM in dogs were diagnosed by veterinarians using a combination of clinical history, physical examination findings, serum biochemistry and/or skeletal muscle histology...
April 18, 2018: New Zealand Veterinary Journal
https://www.readbyqxmd.com/read/29669460/current-diagnosis-and-treatment-of-polymyositis-and-dermatomyositis
#14
Hirokazu Sasaki, Hitoshi Kohsaka
Idiopathic inflammatory myopathies (IIMs) are heterogeneous disorders that affect the skeletal muscles. Polymyositis, dermatomyositis, and inclusion body myositis are major IIM subsets. Immune-mediated necrotizing myopathy became recognized as a potentially new IIM subset. Since the new classification criteria published by the International Myositis Classification Criteria Project have higher sensitivity and specificity for IIM classification and subclassification than the previous criteria, they should help precise diagnosis...
April 18, 2018: Modern Rheumatology
https://www.readbyqxmd.com/read/29669168/characterization-of-congenital-myopathies-at-a-korean-neuromuscular-center
#15
Young-Eun Park, Jin-Hong Shin, Hyang-Sook Kim, Chang-Hoon Lee, Dae-Seong Kim
INTRODUCTION: Congenital myopathies are muscle diseases characterized by specific histopathological features, generalized hypotonia from birth, and perinatal complications, although some cases develop during childhood or rarely even in adulthood. We undertook the study to characterize congenital myopathies among patients registered at our institution. METHODS: Clinical, histopathological and genetic features were evaluated in 34 patients recruited for this study...
April 18, 2018: Muscle & Nerve
https://www.readbyqxmd.com/read/29669056/performance-meat-quality-and-pectoral-myopathies-of-broilers-fed-either-corn-or-sorghum-based-diets-supplemented-with-guanidinoacetic-acid
#16
H A Córdova-Noboa, E O Oviedo-Rondón, A H Sarsour, J Barnes, P Ferzola, M Rademacher-Heilshorn, U Braun
One experiment was conducted to evaluate the effects of guanidinoacetic acid (GAA) supplementation in broilers fed corn or sorghum-based diets on live performance, carcass and cut up yields, meat quality, and pectoral myopathies. The treatments consisted of corn or sorghum-based diets with or without the addition of GAA (600 g/ton). A total of 800 one-d-old male Ross 708 broiler chicks were randomly placed in 40 floor pens with 10 replicates (20 birds per pen) per each of the four treatments. At hatch, 14, 35, and 50 d, BW and feed intake were recorded...
April 13, 2018: Poultry Science
https://www.readbyqxmd.com/read/29669035/effect-of-guanidinoacetic-acid-supplementation-on-live-performance-meat-quality-pectoral-myopathies-and-blood-parameters-of-male-broilers-fed-corn-based-diets-with-or-without-poultry-by-products
#17
H A Córdova-Noboa, E O Oviedo-Rondón, A H Sarsour, J Barnes, D Sapcota, D López, L Gross, M Rademacher-Heilshorn, U Braun
Creatine is a nitrogenous compound naturally occurring in animal tissues and is obtained from dietary animal protein or de novo synthesis from guanidinoacetic acid (GAA). The dietary supply of this semi-essential nutrient could be adversely compromised when feeding purely vegetable-based diets. The objective of this experiment was to evaluate the effects of GAA supplementation in broilers fed corn-based diets with or without the inclusion of poultry by-products (PBP) on live performance, carcass and cut up yields, meat quality, pectoral muscle myopathies, differential blood count, blood clinical chemistry, serum GAA and its metabolites...
April 13, 2018: Poultry Science
https://www.readbyqxmd.com/read/29668884/statin-coadministration-increases-the-risk-of-daptomycin-associated-myopathy
#18
Ryan K Dare, Chad Tewell, Bryan Harris, Patty W Wright, Sara L Van Driest, Eric Farber-Eger, George E Nelson, Thomas R Talbot
Background: Daptomycin-associated myopathy has been identified in 2-14% of patients, and rhabdomyolysis is a known adverse effect. Although risk factors for daptomycin-associated myopathy are poorly defined, creatine phosphokinase (CPK) monitoring and temporary discontinuation of HMG-CoA reductase inhibitors, or "statins", has been recommended. Methods: We conducted a single-center, retrospective, matched case-control risk factor analysis in adult and pediatric patients from 2004-2015...
April 16, 2018: Clinical Infectious Diseases: An Official Publication of the Infectious Diseases Society of America
https://www.readbyqxmd.com/read/29668875/giant-biventricular-aneurysms-a-novel-cardiac-phenotype-in-myotubular-centronuclear-myopathy
#19
Francisco José Bermúdez-Jiménez, Juan Jiménez-Jáimez, Mercedes González Molina, Luis Tercedor
No abstract text is available yet for this article.
April 13, 2018: European Heart Journal
https://www.readbyqxmd.com/read/29666206/taurine-supplementation-for-prevention-of-stroke-like-episodes-in-melas-a-multicentre-open-label-52-week-phase-iii-trial
#20
Yutaka Ohsawa, Hiroki Hagiwara, Shin-Ichiro Nishimatsu, Akihiro Hirakawa, Naomi Kamimura, Hideaki Ohtsubo, Yuta Fukai, Tatsufumi Murakami, Yasutoshi Koga, Yu-Ichi Goto, Shigeo Ohta, Yoshihide Sunada
OBJECTIVE: The aim of this study was to evaluate the efficacy and safety of high-dose taurine supplementation for prevention of stroke-like episodes of MELAS (mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes), a rare genetic disorder caused by point mutations in the mitochondrial DNA that lead to a taurine modification defect at the first anticodon nucleotide of mitochondrial tRNALeu(UUR) , resulting in failure to decode codons accurately. METHODS: After the nationwide survey of MELAS, we conducted a multicentre, open-label, phase III trial in which 10 patients with recurrent stroke-like episodes received high-dose taurine (9 g or 12 g per day) for 52 weeks...
April 17, 2018: Journal of Neurology, Neurosurgery, and Psychiatry
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