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https://www.readbyqxmd.com/read/28637129/patient-reported-outcomes-in-gne-myopathy-incorporating-a-valid-assessment-of-physical-function-in-a-rare-disease
#1
Christina Slota, Margaret Bevans, Li Yang, Joseph Shrader, Galen Joe, Nuria Carrillo
BACKGROUND: The aim of this analysis was to evaluate the psychometric properties of three patient reported outcome (PRO) measures characterizing physical function in GNE myopathy: the Human Activity Profile, the Inclusion Body Myositis Functional Rating Scale, and the Activities-specific Balance Confidence scale. METHODS: This analysis used data from 35 GNE myopathy subjects participating in a natural history study. For construct validity, correlational and known-group analyses were between the PROs and physical assessments...
February 7, 2017: Disability and Rehabilitation
https://www.readbyqxmd.com/read/28630772/severe-rhabdomyolysis-due-to-presumed-drug-interactions-between-atorvastatin-with-amlodipine-and-ticagrelor
#2
Iouri Banakh, Kavi Haji, Ross Kung, Sachin Gupta, Ravindranath Tiruvoipati
Atorvastatin and ticagrelor combination is a widely accepted therapy for secondary prevention of ischaemic heart disease. However, rhabdomyolysis is a well-known rare side effect of statins which should be considered when treatments are combined with cytochrome P450 3A4 enzyme inhibitors. We report a case of atorvastatin and ticagrelor associated severe rhabdomyolysis that progressed to multiorgan failure requiring renal replacement therapy, inotropes, intubation, and mechanical ventilation. Despite withdrawal of the precipitating cause and the supportive measures including renal replacement therapy, creatinine kinase increased due to ongoing rhabdomyolysis rapidly progressing to upper and lower limbs weakness...
2017: Case Reports in Critical Care
https://www.readbyqxmd.com/read/28629834/myositis-in-the-head-and-neck-challenges-in-diagnosis-and-management
#3
R Ratansi, G Fabbroni, A Kanatas
Myositis in the head and neck may present with non-specific symptoms, and radiographically may mimic malignancy. Multidisciplinary management is often essential, and we describe the challenges in an effort to raise awareness of the condition.
June 16, 2017: British Journal of Oral & Maxillofacial Surgery
https://www.readbyqxmd.com/read/28628466/evaluation-of-cancer-associated-myositis-and-scleroderma-autoantibodies-in-breast-cancer-patients-without-rheumatic-disease
#4
Ami A Shah, Antony Rosen, Laura K Hummers, Betty J May, Alpana Kaushiva, Richard B S Roden, Deborah K Armstrong, Fredrick M Wigley, Livia Casciola-Rosen, Kala Visvanathan
OBJECTIVES: Systemic sclerosis (scleroderma) and dermatomyositis are two prototypic autoimmune diseases that are strongly associated with malignancy. While specific autoantibodies in these diseases are markers of an increased risk of cancer at scleroderma and dermatomyositis onset, it is not known whether these autoantibodies are biomarkers of cancer risk in patients without rheumatic disease. METHODS: In a matched case-control study of women without rheumatic disease, identified from a familial breast cancer cohort, 50 breast cancer cases and 50 controls were assayed for 3 autoantibodies that are known markers of cancer-associated scleroderma and dermatomyositis: anti-RNA polymerase III, anti-NXP2, and anti-TIF1γ...
June 19, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28623757/heterotopic-mesenteric-and-abdominal-wall-ossification-two-case-reports-in-one-institution
#5
Cátia Ferreira, Carina Gomes, Ana Melo, Nádia Tenreiro, Bruno Pinto, Herculano Moreira, Artur Ribeiro, Paulo Avelar
INTRODUCTION: Heterotopic ossification occurs when bone develops in tissues which usually don't undergo ossification. Heterotopic mesenteric ossification, also known as intra-abdominal myositis ossificans, is a rare and benign form of ossification, usually related with previous abdominal surgery or trauma. PRESENTATION OF CASES: We report two cases of heterotopic ossification both after multiple abdominal surgeries, with intraoperative findings of mesenteric and abdominal wall ossification...
June 8, 2017: International Journal of Surgery Case Reports
https://www.readbyqxmd.com/read/28623559/ifn-%C3%AE-induced-reactive-oxygen-species-and-mitochondrial-damage-contribute-to-muscle-impairment-and-inflammation-maintenance-in-dermatomyositis
#6
Alain Meyer, Gilles Laverny, Yves Allenbach, Elise Grelet, Vanessa Ueberschlag, Andoni Echaniz-Laguna, Béatrice Lannes, Ghada Alsaleh, Anne Laure Charles, François Singh, Joffrey Zoll, Evelyne Lonsdorfer, François Maurier, Olivier Boyer, Jacques-Eric Gottenberg, Anne Sophie Nicot, Jocelyn Laporte, Olivier Benveniste, Daniel Metzger, Jean Sibilia, Bernard Geny
Dermatomyositis (DM) is an autoimmune disease associated with enhanced type I interferon (IFN) signalling in skeletal muscle, but the mechanisms underlying muscle dysfunction and inflammation perpetuation remain unknown. Transcriptomic analysis of early untreated DM muscles revealed that the main cluster of down-regulated genes was mitochondria-related. Histochemical, electron microscopy, and in situ oxygraphy analysis showed mitochondrial abnormalities, including increased reactive oxygen species (ROS) production and decreased respiration, which was correlated with low exercise capacities and a type I IFN signature...
June 16, 2017: Acta Neuropathologica
https://www.readbyqxmd.com/read/28619061/rituximab-versus-cyclophosphamide-for-the-treatment-of-connective-tissue-disease-associated-interstitial-lung-disease-recital-study-protocol-for-a-randomised-controlled-trial
#7
Peter Saunders, Vicky Tsipouri, Gregory J Keir, Deborah Ashby, Marcus D Flather, Helen Parfrey, Daphne Babalis, Elisabetta A Renzoni, Christopher P Denton, Athol U Wells, Toby M Maher
BACKGROUND: Interstitial lung disease (ILD) frequently complicates systemic autoimmune disorders resulting in considerable morbidity and mortality. The connective tissue diseases (CTDs) most frequently resulting in ILD include: systemic sclerosis, idiopathic inflammatory myositis (including dermatomyositis, polymyositis and anti-synthetase syndrome) and mixed connective tissue disease. Despite the development, over the last two decades, of a range of biological therapies which have resulted in significant improvements in the treatment of the systemic manifestations of CTD, the management of CTD-associated ILD has changed little...
June 15, 2017: Trials
https://www.readbyqxmd.com/read/28610606/biologic-therapies-for-refractory-juvenile-dermatomyositis-five-years-of-experience-of-the-childhood-arthritis-and-rheumatology-research-alliance-in-north-america
#8
C H Spencer, K Rouster-Stevens, H Gewanter, G Syverson, R Modica, K Schmidt, H Emery, C Wallace, S Grevich, K Nanda, Y D Zhao, S Shenoi, S Tarvin, S Hong, C Lindsley, J E Weiss, M Passo, K Ede, A Brown, K Ardalan, W Bernal, M L Stoll, B Lang, R Carrasco, C Agaiar, L Feller, H Bukulmez, R Vehe, H Kim, H Schmeling, D Gerstbacher, M Hoeltzel, B Eberhard, R Sundel, S Kim, A M Huber, A Patwardhan
BACKGROUND: The prognosis of children with juvenile dermatomyositis (JDM) has improved remarkably since the 1960's with the use of corticosteroid and immunosuppressive therapy. Yet there remain a minority of children who have refractory disease. Since 2003 the sporadic use of biologics (genetically-engineered proteins that usually are derived from human genes) for inflammatory myositis has been reported. In 2011-2016 we investigated our collective experience of biologics in JDM through the Childhood Arthritis and Rheumatology Research Alliance (CARRA)...
June 13, 2017: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/28606058/risk-factors-of-lower-limb-cellulitis-in-a-level-two-healthcare-facility-in-cameroon-a-case-control-study
#9
Tsi Njim, Leopold Ndemnge Aminde, Valirie Ndip Agbor, Louise Daniele Toukam, Sara Saheb Kashaf, Eric O Ohuma
BACKGROUND: Cellulitis is a common infection of the skin and subcutaneous tissues. It is associated with significant morbidity from necrosectomies and amputations especially in sub-Saharan Africa. We aimed at identifying the risk factors and burden of lower limb cellulitis to inform preventive strategies in Cameroon. METHODS: This was a hospital-based case-control study carried out in the Bamenda Regional Hospital (BRH) between September 2015 and August 2016. Cases were defined as consenting adults admitted to the surgical unit who presented with a localised area of lower limb erythema, warmth, oedema and pain, associated with fever (temperature ≥ 38 °C) and/or chills of sudden onset...
June 12, 2017: BMC Infectious Diseases
https://www.readbyqxmd.com/read/28605526/anti-nxp2-antibody-associated-extensive-subcutaneous-calcinosis-in-adult-onset-myositis
#10
Radjiv Goulabchand, Philippe Guilpain, Catherine Cyteval, Alain Le Quellec
No abstract text is available yet for this article.
June 12, 2017: Rheumatology
https://www.readbyqxmd.com/read/28604554/severe-ocular-myositis-after-ipilimumab-treatment-for-melanoma-a-report-of-2-cases
#11
Anna Pushkarevskaya, Ulf Neuberger, Antonia Dimitrakopoulou-Strauss, Alexander Enk, Jessica C Hassel
Ipilimumab binds and blocks cytotoxic T-lymphocyte-associated antigen-4, causing enhanced T-cell reaction, antitumor response, and significant improvement of the overall survival of patients with metastatic melanoma. Patients treated with ipilimumab can develop immune-related adverse effects, primarily dermatitis, colitis, hepatitis, and hypophysitis. Although, in phase I-III studies, 64.2% of all patients suffered from immune-related adverse effects, ocular adverse effects occurred in 1.3% only. In the cases reported below, 2 patients with metastatic melanoma developed severe ocular myositis after treatment with ipilimumab...
June 9, 2017: Journal of Immunotherapy
https://www.readbyqxmd.com/read/28602176/calpainopathy-with-macrophage-rich-regional-inflammatory-infiltrates
#12
Peter W Schutz, Renata S Scalco, Rita Barresi, Henry Houlden, Matthew Parton, Janice L Holton
Mutations in calpain-3 cause limb girdle muscular dystrophy 2A. Biopsy pathology is typically dystrophic, sometimes characterized by frequent lobulated fibres. More recently calpain mutations have been shown in association with eosinophilic myositis, suggesting that calpain mutations may render muscle susceptible to inflammatory change. We present the case of a 33-year old female with mild proximal muscle weakness and high CK levels (6698 IU/L at presentation). Muscle biopsy showed clusters of fibre necrosis associated with very dense macrophage infiltrates and small numbers of lymphocytes, raising the possibility of an inflammatory myopathy...
April 27, 2017: Neuromuscular Disorders: NMD
https://www.readbyqxmd.com/read/28599620/blackleg-in-cattle-a-case-report-of-fetal-infection-and-a-literature-review
#13
Camila C Abreu, Erin E Edwards, John F Edwards, Philippa M Gibbons, Jeann Leal de Araújo, Raquel R Rech, Francisco A Uzal
Clostridium chauvoei causes blackleg in cattle. The disease has been reported worldwide, and although it can be prevented by vaccination, sporadic cases and occasional outbreaks still occur. We describe a case of blackleg in a 2-y-old, pregnant Gyr cow with in utero transmission to the fetus. The cow had characteristic gross and microscopic lesions of blackleg including widespread necrohemorrhagic and emphysematous skeletal and myocardial myositis, and fibrinous pericarditis. Her uterus contained a near-term, markedly emphysematous fetus with skeletal muscle and myocardial lesions similar to those seen in the dam...
June 1, 2017: Journal of Veterinary Diagnostic Investigation
https://www.readbyqxmd.com/read/28589389/-safety-and-efficacy-of-off-label-use-of-biologic-therapies-in-patients-with-inflammatory-rheumatic-diseases-refractory-to-standard-of-care-therapy-data-from-a%C3%A2-nationwide-german-registry-graid2
#14
F Proft, H Schulze-Koops, M Grunke, E Schrezenmeier, F Halleck, J Henes, L Unger, E Schmidt, C Fiehn, A Jacobi, C Iking-Konert, C Kneitz, R E Schmidt, B Bannert, R E Voll, R Fischer-Betz, I Kötter, H P Tony, J Holle, M Aringer, A Erler, F Behrens, G R Burmester, T Dörner
BACKGROUND: The German Registry of Autoimmune Diseases 2 (GRAID2) is a retrospective, non-interventional, multicenter registry study collecting data from patients with inflammatory, mainly rheumatic diseases refractory to standard of care therapy and treated with an off-label biologic therapy. The retrospective documentation comprised case history, diagnosis, course of disease (including safety and global efficacy). The objective was to evaluate the global clinical outcome and safety of off-label biologic therapy in clinical practice...
June 6, 2017: Zeitschrift Für Rheumatologie
https://www.readbyqxmd.com/read/28589170/immune-and-myodegenerative-pathomechanisms-in-inclusion-body-myositis
#15
REVIEW
Christian W Keller, Jens Schmidt, Jan D Lünemann
Inclusion Body Myositis (IBM) is a relatively common acquired inflammatory myopathy in patients above 50 years of age. Pathological hallmarks of IBM are intramyofiber protein inclusions and endomysial inflammation, indicating that both myodegenerative and inflammatory mechanisms contribute to its pathogenesis. Impaired protein degradation by the autophagic machinery, which regulates innate and adaptive immune responses, in skeletal muscle fibers has recently been identified as a potential key pathomechanism in IBM...
June 2017: Annals of Clinical and Translational Neurology
https://www.readbyqxmd.com/read/28587920/-what-would-you-do-with-an-adult-patient-who-consults-due-to-head-muscle-weakness-and-has-dropped-head
#16
L Paino, N Blasco
The dropped head syndrome, whether due to muscle weakness, rigidity, or ankylosis, is not uncommon in the elderly. It is characterised by a "chin-on-chest" reducible kyphosis, which is secondary to head muscle debility. It may be associated with a neuromuscular group of diseases such as polymyositis, chronic Inflammatory demyelinating polyneuropathy, myasthenia gravis, amyotrophic lateral sclerosis, and inclusion-body myositis. Some cases associated with hypothyroidism and hyperparathyroidism have also been described...
June 3, 2017: Semergen
https://www.readbyqxmd.com/read/28586844/skeletal-muscle-involvement-in-antisynthetase-syndrome
#17
Eri Noguchi, Akinori Uruha, Shigeaki Suzuki, Kohei Hamanaka, Yuko Ohnuki, Jun Tsugawa, Yurika Watanabe, Jin Nakahara, Takashi Shiina, Norihiro Suzuki, Ichizo Nishino
Importance: Antisynthetase syndrome, characterized by myositis, interstitial lung disease, skin rash, arthropathy, and Raynaud phenomenon, is a clinical entity based on the presence of aminoacyl transfer RNA synthetase (ARS) antibodies in patients' serum. However, antisynthetase syndrome is not included in the histological subsets of idiopathic inflammatory myopathies. Objective: To elucidate the clinical features of myositis in patients with antisynthetase syndrome...
June 5, 2017: JAMA Neurology
https://www.readbyqxmd.com/read/28578311/orbital-myositis-presenting-with-only-unilateral-orbital-pain
#18
Saori Utsunomiya, Daisuke Yamamoto, Tsuyoshi Uchiyama
No abstract text is available yet for this article.
June 3, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28572050/myocarditis-in-auto-immune-or-auto-inflammatory-diseases
#19
REVIEW
Cloé Comarmond, Patrice Cacoub
Myocarditis is a major cause of heart disease in young patients and a common precursor of heart failure due to dilated cardiomyopathy. Some auto-immune and/or auto-inflammatory diseases may be accompanied by myocarditis, such as sarcoidosis, Behçet's disease, eosinophilic granulomatosis with polyangiitis, myositis, and systemic lupus erythematosus. However, data concerning myocarditis in such auto-immune and/or auto-inflammatory diseases are sparse. New therapeutic strategies should better target the modulation of the immune system, depending on the phase of the disease and the type of underlying auto-immune and/or auto-inflammatory disease...
May 29, 2017: Autoimmunity Reviews
https://www.readbyqxmd.com/read/28571966/carcinomatous-myositis-secondary-to-gastric-cancer
#20
Diego Rubio Solís, Sandra Sánchez García, Marta López Valcarcel, Serafín Costilla García
No abstract text is available yet for this article.
May 29, 2017: Medicina Clínica
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