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https://www.readbyqxmd.com/read/29780824/clinical-histological-and-immunohistochemical-findings-in-inclusion-body-myositis
#1
Leonardo Valente de Camargo, Mary Souza de Carvalho, Samuel Katsuyuki Shinjo, Acary Souza Bulle de Oliveira, Edmar Zanoteli
Sporadic inclusion body myositis (sIBM) is considered the most common acquired myopathy aged over 50 years. The disease is characterized by a particular process of muscle degeneration characterized by abnormal deposit of protein aggregates in association with inflammation. The aim of this study was to present clinical and muscle histopathological findings, including immunostaining for LC3B, p62, α -synuclein, and TDP-43, in 18 patients with sIBM. The disease predominated in males (61%) and European descendants, with onset of clinical manifestations around 59 years old...
2018: BioMed Research International
https://www.readbyqxmd.com/read/29774305/multi-slice-mri-reveals-heterogeneity-in-disease-distribution-along-the-length-of-muscle-in-duchenne-muscular-dystrophy
#2
Stephen M Chrzanowski, Celine Baligand, Rebecca J Willcocks, Jasjit Deol, Ilona Schmalfuss, Donovan J Lott, Michael J Daniels, Claudia Senesac, Glenn A Walter, Krista Vandenborne
Background: Duchenne muscular dystrophy (DMD) causes progressive pathologic changes to muscle secondary to a cascade of inflammation, lipid deposition, and fibrosis. Clinically, this manifests as progressive weakness, functional loss, and premature mortality. Though insult to whole muscle groups is well established, less is known about the relationship between intramuscular pathology and function. Objective: Differences of intramuscular heterogeneity across muscle length were assessed using an ordinal MRI grading scale in lower leg muscles of boys with DMD and correlated to patient's functional status...
September 2017: Acta Myologica: Myopathies and Cardiomyopathies: Official Journal of the Mediterranean Society of Myology
https://www.readbyqxmd.com/read/29763467/natural-disease-history-of-the-dy2j-mouse-model-of-laminin-%C3%AE-2-merosin-deficient-congenital-muscular-dystrophy
#3
S Pasteuning-Vuhman, K Putker, C L Tanganyika-de Winter, J W Boertje-van der Meulen, L van Vliet, M Overzier, J J Plomp, A Aartsma-Rus, M van Putten
Merosin deficient congenital muscular dystrophy 1A (MDC1A) is a very rare autosomal recessive disorder caused by mutations in the LAMA2 gene leading to severe and progressive muscle weakness and atrophy. Although over 350 causative mutations have been identified for MDC1A, no treatment is yet available. There are many therapeutic approaches in development, but the lack of natural history data of the mouse model and standardized outcome measures makes it difficult to transit these pre-clinical findings to clinical trials...
2018: PloS One
https://www.readbyqxmd.com/read/29720800/necrotizing-autoimmune-myopathy-clinicopathologic-study-from-a-single-tertiary-care-centre
#4
Sobiya Mahnaz Ayesha, A K Meena, Navatha Vangala, Liza Rajasekhar, Subhash Kaul, Rupam Borgahain, Megha S Uppin
Background: Idiopathic inflammatory myopathies (IIMs) are a group of chronic, autoimmune disorders which include a new entity, necrotizing autoimmune myopathy (NAM). NAM lacks inflammation and presents with markedly elevated creatinine phosphokinase (CPK) levels. It is associated with connective tissue diseases (CTDs), statin use, malignancies, and most cases are idiopathic. Objectives: The objectives of this study are to describe the clinicopathologic features in muscle biopsy-proven cases of NAM...
January 2018: Annals of Indian Academy of Neurology
https://www.readbyqxmd.com/read/29720222/targeted-lipidomics-analysis-identified-altered-serum-lipid-profiles-in-patients-with-polymyositis-and-dermatomyositis
#5
Joan Raouf, Helena Idborg, Petter Englund, Helene Alexanderson, Maryam Dastmalchi, Per-Johan Jakobsson, Ingrid E Lundberg, Marina Korotkova
BACKGROUND: Polymyositis (PM) and dermatomyositis (DM) are severe chronic autoimmune diseases, characterized by muscle fatigue and low muscle endurance. Conventional treatment includes high doses of glucocorticoids and immunosuppressive drugs; however, few patients recover full muscle function. One explanation of the persistent muscle weakness could be altered lipid metabolism in PM/DM muscle tissue as we previously reported. Using a targeted lipidomic approach we aimed to characterize serum lipid profiles in patients with PM/DM compared to healthy individuals (HI) in a cross-sectional study...
May 2, 2018: Arthritis Research & Therapy
https://www.readbyqxmd.com/read/29707197/case-report-acute-amyopathic-dermatomyositis-presenting-with-isolated-facial-edema
#6
Efthymia Pappa, Marina Gkeka, Asimina Protogerou, Leonidas Marinos, Chariclia Loupa, Constantinos Christopoulos
A 45-year-old Asian man presented with acute-onset periorbital and facial edema associated with pyrexia. Muscle weakness was absent. Initial laboratory investigations showed an inflammatory reaction, while screening for infections was negative. Serum muscle enzyme levels were normal. He was hospitalized and treated empirically with antibiotics and corticosteroids, pending the result of facial skin and muscle biopsy. He showed a good clinical and laboratory response but an attempt to discontinue corticosteroids led to a prompt relapse of facial edema and pyrexia, associated with rising laboratory indices of inflammation...
2018: F1000Research
https://www.readbyqxmd.com/read/29692797/combined-therapies-for-duchenne-muscular-dystrophy-to-optimize-treatment-efficacy
#7
REVIEW
Gonzalo Cordova, Elisa Negroni, Claudio Cabello-Verrugio, Vincent Mouly, Capucine Trollet
Duchene Muscular Dystrophy (DMD) is the most frequent muscular dystrophy and one of the most severe due to the absence of the dystrophin protein. Typical pathological features include muscle weakness, muscle wasting, degeneration, and inflammation. At advanced stages DMD muscles present exacerbated extracellular matrix and fat accumulation. Recent progress in therapeutic approaches has allowed new strategies to be investigated, including pharmacological, gene-based and cell-based therapies. Gene and cell-based therapies are still limited by poor targeting and low efficiency in fibrotic dystrophic muscle, therefore it is increasingly evident that future treatments will have to include "combined therapies" to reach maximal efficiency...
2018: Frontiers in Genetics
https://www.readbyqxmd.com/read/29691609/effect-of-25-hydroxyvitamin-d-deficiency-and-its-interaction-with-prednisone-treatment-on-musculoskeletal-health-in-growing-mdx-mice
#8
Sung-Hee Yoon, Kim S Sugamori, Marc D Grynpas, Jane Mitchell
Duchenne muscular dystrophy (DMD) results from genetic mutations of the gene encoding dystrophin, leading to muscle inflammation and degeneration that is typically treated with glucocorticoids. DMD and its treatment with glucocorticoids result in poor bone health and high risk of fractures. Insufficient levels of 25-hydroxyvitamin D (25-hydroxy D) that may contribute to weakened bone are routinely found in DMD patients. To determine the effect of 25-hydroxy D deficiency, this study examined the effects of low vitamin D dietary intake with and without glucocorticoids on the musculoskeletal system of the Mdx mouse model of DMD...
April 24, 2018: Calcified Tissue International
https://www.readbyqxmd.com/read/29691272/immune-mediated-necrotising-myopathy-a-rare-cause-of-hyperckaemia
#9
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/29651121/classification-of-myositis
#10
REVIEW
Ingrid E Lundberg, Marianne de Visser, Victoria P Werth
The idiopathic inflammatory myopathies (IIMs; also known as myositis) are a heterogeneous group of disorders in which a common feature is chronic inflammation of skeletal muscle, leading to muscle weakness. Other organs are frequently affected in IIMs, such as the skin, joints, lungs, gastrointestinal tract and heart, contributing to morbidity and mortality. Currently, IIMs are most often subclassified into polymyositis, dermatomyositis and inclusion body myositis, but this subclassification has limitations as these subgroups often have overlapping clinical and histopathological features, and outcomes vary within the subgroups; additionally, subgroups without considerable myopathy are not included...
April 12, 2018: Nature Reviews. Rheumatology
https://www.readbyqxmd.com/read/29651119/update-on-outcome-assessment-in-myositis
#11
REVIEW
Lisa G Rider, Rohit Aggarwal, Pedro M Machado, Jean-Yves Hogrel, Ann M Reed, Lisa Christopher-Stine, Nicolino Ruperto
The adult and juvenile myositis syndromes, commonly referred to collectively as idiopathic inflammatory myopathies (IIMs), are systemic autoimmune diseases with the hallmarks of muscle weakness and inflammation. Validated, well-standardized measures to assess disease activity, known as core set measures, were developed by international networks of myositis researchers for use in clinical trials. Composite response criteria using weighted changes in the core set measures of disease activity were developed and validated for adult and juvenile patients with dermatomyositis and adult patients with polymyositis, with different thresholds for minimal, moderate and major improvement in adults and juveniles...
April 12, 2018: Nature Reviews. Rheumatology
https://www.readbyqxmd.com/read/29648516/the-phosphodiesterase-4-inhibitor-roflumilast-reverts-proteolysis-in-skeletal-muscle-cells-of-patients-with-copd-cachexia
#12
Esther Barreiro, Ester Puig-Vilanova, Anna Salazar-Degracia, Sergi Pascual-Guardia, Carme Casadevall, Joaquim Gea
Peripheral muscle weakness and mass loss are characteristic features in severe COPD. We hypothesized that the phosphodiesterase-4 inhibitor roflumilast-induced cAMP may ameliorate proteolysis and metabolism in skeletal muscles of COPD patients with severe muscle wasting. In myogenic precursor cells (isolated from muscle biopsies and cultured up to obtain differentiated myotubes) from 10 severe COPD patients and 10 healthy controls, which were treated with 1 microM roflumilast N-oxide (RNO) for three time-cohorts (1h, 6h, and 24h), genes of antioxidant defense and oxidative stress marker, myogenesis and muscle metabolism, proteolysis (tyrosine release assay) and ubiquitin-proteasome system markers, autophagy, and myosin isoforms were analyzed using RT-PCR and immunoblotting...
April 12, 2018: Journal of Applied Physiology
https://www.readbyqxmd.com/read/29621305/biomarkers-associated-with-low-moderate-and-high-vastus-lateralis-muscle-hypertrophy-following-12-weeks-of-resistance-training
#13
Christopher B Mobley, Cody T Haun, Paul A Roberson, Petey W Mumford, Wesley C Kephart, Matthew A Romero, Shelby C Osburn, Christopher G Vann, Kaelin C Young, Darren T Beck, Jeffrey S Martin, Christopher M Lockwood, Michael D Roberts
We sought to identify biomarkers which delineated individual hypertrophic responses to resistance training. Untrained, college-aged males engaged in full-body resistance training (3 d/wk) for 12 weeks. Body composition via dual x-ray absorptiometry (DXA), vastus lateralis (VL) thickness via ultrasound, blood, VL muscle biopsies, and three-repetition maximum (3-RM) squat strength were obtained prior to (PRE) and following (POST) 12 weeks of training. K-means cluster analysis based on VL thickness changes identified LOW [n = 17; change (mean±SD) = +0...
2018: PloS One
https://www.readbyqxmd.com/read/29618495/fibrinogen-does-not-relate-to-cardiovascular-or-muscle-manifestations-in-copd-cross-sectional-data-from-the-erica-study
#14
Divya Mohan, Julia R Forman, Matthew Allinder, Carmel M McEniery, Charlotte Emma Bolton, John R Cockcroft, William MacNee, Jonathan Fuld, Mellone Marchong, Nichola Sian Gale, Marie Fisk, Sridevi Nagarajan, Joseph Cheriyan, David A Lomas, Peter M A Calverley, Bruce E Miller, Ruth Tal-Singer, Ian B Wilkinson, Michael I Polkey
Cardiovascular and skeletal muscle manifestations constitute important comorbidities in COPD, with systemic inflammation proposed as a common mechanistic link. Fibrinogen has prognostic role in COPD. We aimed to determine whether aortic stiffness and quadriceps weakness are linked in COPD, and whether they are associated with the systemic inflammatory mediator-fibrinogen. Aortic pulse wave velocity (aPWV), quadriceps maximal volitional contraction (QMVC) force and fibrinogen were measured in 729 patients with stable, Global Initiative for Chronic Obstructive Lung Disease (GOLD) stages II-IV COPD...
April 4, 2018: Thorax
https://www.readbyqxmd.com/read/29595876/exercise-mitigates-the-effects-of-hyperhomocysteinemia-on-adverse-muscle-remodeling
#15
Lee J Winchester, Sudhakar Veeranki, Sathnur Pushpakumar, Suresh C Tyagi
Hyperhomocysteinemia (HHcy) is known for causing inflammation and vascular remodeling, particularly through production of reactive oxygen species (ROS) and matrix metalloproteinase-9 (MMP-9) activation. Although its effect on the skeletal muscle is unclear, HHcy can cause skeletal muscle weakness and functional impairment by induction of inflammatory mediators and macrophage mediated injury. Exercise has been shown to reduce homocysteine levels and therefore, could serve as a promising intervention for HHcy...
March 2018: Physiological Reports
https://www.readbyqxmd.com/read/29595280/-idiopathic-inflammatory-myopathies
#16
Jiří Vencovský
Idiopathic inflammatory myopathies form a heterogeneous group of acquired inflammatory diseases afflicting striated muscles. The disease is frequently accompanied by systemic and organ involvement. Dermatomyositis, polymyositis, cancer associated myositis, immune mediated necrotizing myopathy, myositis in overlap syndromes, juvenile myositis and inclusion body myositis are the most frequently encountered subtypes. The basic manifestation is usually painless muscle weakness brought about by inflammation and by other immune changes at the impacted muscles...
2018: Vnitr̆ní Lékar̆ství
https://www.readbyqxmd.com/read/29578426/dysfunction-of-various-organ-systems-induced-by-sepsis-with-underlying-severe-myelomic-diseases-and-prostate-cancer-case-report
#17
L Ratiani, N Intskirveli, L Goliadze, T Chkhikvadze, L Koptonashvili, E Khuchua
A 65-year-old male patient, unconscious, was admitted into the clinic by the Ambulance. From the patient's medical history it was revealed that several hours before the admission in the clinic the following symptoms were present: shortness of breath, fever, hypotonia, consciousness inhibition, because of which emergency brigade was called and was brought by the Emergency Brigade. The history is loaded by chronic pathologies: myeloma disease, prostate cancer, ciliary arrhythmia, heart failure; received several courses of polichemotherapy, last ten days has been treated for pneumonia with antibiotics of ceftriaxone group in outpatient setting...
February 2018: Georgian Medical News
https://www.readbyqxmd.com/read/29534714/lumbar-spine-epidural-abscess-and-facet-joint-septic-arthritis-due-to-streptococcus-agalactiae-a-case-report
#18
Costansia Bureta, Hiroyuki Tominaga, Takuya Yamamoto, Takao Setoguchi, Hideki Kawamura, Satoshi Nagano, Ichiro Kawamura, Masahiko Abematsu, Hironori Kakoi, Yasuhiro Ishidou, Setsuro Komiya
BACKGROUND: Here we report a rare case of lumbar spine epidural abscess and facet joint septic arthritis caused by Streptococcus agalactiae, which had spread to the iliopsoas muscles, leading to urine retention. CASE PRESENTATION: A 68-year-old woman with low back pain experienced a sudden onset of bilateral lower limb weakness, it was followed 14 days later by urine retention. At consultation, magnetic resonance imaging and identification of serum β-hemolytic streptococci provided a diagnosis of Streptococcus agalactiae infection...
March 13, 2018: BMC Surgery
https://www.readbyqxmd.com/read/29460723/model-of-traumatic-spinal-cord-injury-for-evaluating-pharmacologic-treatments-in-cynomolgus-macaques-macaca-fasicularis
#19
Nitin Seth, Heather A Simmons, Farah Masood, William A Graham, Douglas L Rosene, Susan V Westmoreland, Sheila M Cummings, Basia Gwardjan, Ervin Sejdic, Amber F Hoggatt, Dane R Schalk, Hussein A Abdullah, John B Sledge, Shanker Nesathurai
Here we present the results of experiments involving cynomolgus macaques, in which a model of traumatic spinal cord injury (TSCI) was created by using a balloon catheter inserted into the epidural space. Prior to the creation of the lesion, we inserted an EMG recording device to facilitate measurement of tail movement and muscle activity before and after TSCI. This model is unique in that the impairment is limited to the tail: the subjects do not experience limb weakness, bladder impairment, or bowel dysfunction...
February 1, 2018: Comparative Medicine
https://www.readbyqxmd.com/read/29439058/autoimmune-necrotising-myopathy-and-hmgcr-antibodies
#20
Kushan Karunaratne, Dimitri Amiras, Matthew C Pickering, Monika Hofer, Stuart Viegas
Statins lower serum cholesterol concentrations by inhibiting the enzyme 3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR). Muscle side effects are relatively common and include asymptomatic elevation of serum creatine kinase (CK), myalgia, proximal muscle weakness and rhabdomyolysis. More recently, a subset of cases of immune-mediated necrotising myopathy has been found to have antibodies against HMGCR. It is often an aggressive and debilitating myopathy and has a complex pathogenesis characterised by fibre necrosis, usually with minimal associated inflammation...
April 2018: Practical Neurology
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