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https://www.readbyqxmd.com/read/28550457/treatment-of-juvenile-dermatomyositis-an-update
#1
REVIEW
Charalampia Papadopoulou, Lucy R Wedderburn
The idiopathic inflammatory myopathies of childhood consist of a heterogeneous group of autoimmune diseases characterised by proximal muscle weakness and pathognomonic skin rashes. The overall prognosis of juvenile myositis has improved significantly over recent years, but the long-term outcome differs substantially from patient to patient, suggestive of distinct clinical phenotypes with variable responses to treatment. High doses of corticosteroids remain the cornerstone of therapy along with other immunosuppressant therapies depending on disease severity and response...
May 26, 2017: Paediatric Drugs
https://www.readbyqxmd.com/read/28549557/aberrant-distribution-of-the-trochlear-nerve-a-cadaveric-study-supported-by-immunohistochemistry
#2
Kojiro Takezawa, Grant Townsend, Jim Manavis, Mounir Ghabriel
The trochlear nerve is generally considered to be a purely motor nerve supplying one extraocular muscle, the superior oblique. In the current study, 28 orbits were dissected and in one orbit (3.6%), the trochlear nerve divided into two main branches. The medial branch followed the classical course, entered the superior oblique muscle and was presumed to be motor in function. However, before entering the muscle, it partially fused with the frontal nerve, and gave a bundle of nerve fibres to the frontal nerve...
May 23, 2017: Annals of Anatomy, Anatomischer Anzeiger: Official Organ of the Anatomische Gesellschaft
https://www.readbyqxmd.com/read/28549273/body-weight-supported-bedside-treadmill-training-facilitates-ambulation-in-icu-patients-an-interventional-proof-of-concept-study
#3
Juultje Sommers, Denise C Wieferink, Dave A Dongelmans, Frans Nollet, Raoul H H Engelbert, Marike van der Schaaf
PURPOSE: Early mobilisation is advocated to improve recovery of intensive care unit (ICU) survivors. However, severe weakness in combination with tubes, lines and machinery are practical barriers for the implementation of ambulation with critically ill patients. The aim of this study was to explore the feasibility of Body Weight-Supported Treadmill Training (BWSTT) in critically ill patients in the ICU. METHODS: A custom build bedside Body Weight-Supported Treadmill was used and evaluated in medical and surgical patients in the ICU...
May 13, 2017: Journal of Critical Care
https://www.readbyqxmd.com/read/28548993/muscle-metabolism
#4
Nicolas Tardif, Jonathan Grip, Olav Rooyackers
PURPOSE OF REVIEW: To review the recent findings on metabolic derangements leading to loss of muscle mass and function. RECENT FINDINGS: Several recent studies investigated methods to assess muscle mass and function and its clinical relevance. These are also included. A few studies confirm that a low muscle mass is related to a worse outcome but also a compromised muscle function at discharge is related to long-term survival. A low quality of muscle assessed by the density of muscle from a computed tomography scan is related to mortality...
May 25, 2017: Current Opinion in Critical Care
https://www.readbyqxmd.com/read/28547134/clinical-radiographic-and-biochemical-characteristics-of-adult-hypophosphatasia
#5
T Schmidt, H Mussawy, T Rolvien, T Hawellek, J Hubert, W Rüther, M Amling, F Barvencik
In this study, we report on clinical, radiographic and biochemical characteristics of 38 patients with adult hypophosphatasia. High-resolution peripheral quantitative computed tomography showed alterations of bone microstructure in a subgroup of 14 patients. Pyridoxal-5-phosphate levels correlated with the occurrence of fractures and the number of symptoms. INTRODUCTION: Hypophosphatasia (HPP) is a rare disorder with a wide range of clinical manifestations. A reduced enzymatic activity of alkaline phosphatase (ALP) is the key marker of the disease, causing an accumulation of ALP substrates such as pyridoxal-5-phosphate (PLP)...
May 25, 2017: Osteoporosis International
https://www.readbyqxmd.com/read/28546002/catechins-activate-muscle-stem-cells-by-myf5-induction-and-stimulate-muscle-regeneration
#6
A Rum Kim, Kyung Min Kim, Mi Ran Byun, Jun-Ha Hwang, Jung Il Park, Ho Taek Oh, Hyo Kyeong Kim, Mi Gyeong Jeong, Eun Sook Hwang, Jeong-Ho Hong
Muscle weakness is one of the most common symptoms in aged individuals and increases risk of mortality. Thus, maintenance of muscle mass is important for inhibiting aging. In this study, we investigated the effect of catechins, polyphenol compounds in green tea, on muscle regeneration. We found that (-)-epicatechin gallate (ECG) and (-)-epigallocatechin-3-gallate (EGCG) activate satellite cells by induction of Myf5 transcription factors. For satellite cell activation, Akt kinase was significantly induced after ECG treatment and ECG-induced satellite cell activation was blocked in the presence of Akt inhibitor...
May 22, 2017: Biochemical and Biophysical Research Communications
https://www.readbyqxmd.com/read/28545172/femoral-nerve-block-versus-adductor-canal-block-for-analgesia-after-total-knee-arthroplasty
#7
REVIEW
In Jun Koh, Young Jun Choi, Man Soo Kim, Hyun Jung Koh, Min Sung Kang, Yong In
Inadequate pain management after total knee arthroplasty (TKA) impedes recovery, increases the risk of postoperative complications, and results in patient dissatisfaction. Although the preemptive use of multimodal measures is currently considered the principle of pain management after TKA, no gold standard pain management protocol has been established. Peripheral nerve blocks have been used as part of a contemporary multimodal approach to pain control after TKA. Femoral nerve block (FNB) has excellent postoperative analgesia and is now a commonly used analgesic modality for TKA pain control...
June 1, 2017: Knee Surgery & related Research
https://www.readbyqxmd.com/read/28544788/fibrosing-myopathy-in-systemic-sclerosis-associates-with-higher-mortality
#8
Julie J Paik, Fredrick M Wigley, Ami A Shah, Andrea M Corse, Livia Casciola-Rosen, Laura K Hummers, Andrew L Mammen
OBJECTIVE: To determine if a unique subtype of scleroderma muscle disease exists by comparing the clinical features of systemic sclerosis (SSc) patients with predominant fibrosis on muscle biopsy to those with inflammatory muscle histopathology. METHODS: This retrospective, cross-sectional study included SSc patients with muscle weakness and an available muscle biopsy. Biopsies with fibrosis but without inflammation/necrosis were designated as "fibrosing myopathy" and those with inflammation and/or necrosis were assigned a category of "inflammatory myopathy"...
May 23, 2017: Arthritis Care & Research
https://www.readbyqxmd.com/read/28544784/presynaptic-congenital-myasthenic-syndrome-with-a-homozygous-sequence-variant-in-lama5-combines-myopia-facial-tics-and-failure-of-neuromuscular-transmission
#9
Ricardo A Maselli, Juan Arredondo, Jessica Vázquez, Jessica X Chong, Michael J Bamshad, Deborah A Nickerson, Marian Lara, Fiona Ng, Victoria L Lo, Peter Pytel, Craig M McDonald
Defects in genes encoding the isoforms of the laminin alpha subunit have been linked to various phenotypic manifestations, including brain malformations, muscular dystrophy, ocular defects, cardiomyopathy, and skin abnormalities. We report here a severe defect of neuromuscular transmission in a consanguineous patient with a homozygous variant in the laminin alpha-5 subunit gene (LAMA5). The variant c.8046C>T (p.Arg2659Trp) is rare and has a predicted deleterious effect. The affected individual, who also carries a rare homozygous sequence variant in LAMA1, had muscle weakness, myopia, and facial tics...
May 25, 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28544463/axonal-charcot-marie-tooth-neuropathy-concurrent-with-distal-and-proximal-weakness-by-translational-elongation-of-the-3-utr-in-nefh
#10
Da Eun Nam, Sung-Chul Jung, Da Hye Yoo, Sun Seong Choi, Sung-Yum Seo, Gwang Hoon Kim, Song-Ja Kim, Soo Hyun Nam, Byung-Ok Choi, Ki Wha Chung
Mutations in the NEFH gene encoding the heavy neurofilament protein are usually associated with neuronal damage and susceptibility to amyotrophic lateral sclerosis (ALS). Recently, frameshift variants in NEFH (p.Asp1004Glnfs*58 and p.Pro1008Alafs*56) have been reported to be the underlying cause of axonal Charcot-Marie-Tooth disease type 2CC (CMT2CC). The frameshift mutation resulted in a stop loss and translation of a cryptic amyloidogenic element (CAE) encoded by the 3' UTR. This study also identified a de novo c...
May 24, 2017: Journal of the Peripheral Nervous System: JPNS
https://www.readbyqxmd.com/read/28543791/the-association-between-eccentric-hip-abduction-strength-and-hip-and-knee-angular-movements-in-recreational-male-runners-an-explorative-study
#11
René B K Brund, Sten Rasmussen, Rasmus O Nielsen, Uwe G Kersting, Uffe Laessoe, Michael Voigt
Weak hip abductors may be related with increased hip adduction and knee abduction angular movement, which may be risk factors of lower extremity injuries. Since the role of eccentric hip abduction strength (EHAS) on hip adduction angular movement and the knee abduction angular movement (KABD) remains unclear and the purpose of the present study was to explore the association between EHAS and hip- and knee angular movement. In 100 healthy male recreational runners, EHAS was quantified using an isokinetic dynamometer, while hip- and knee angular movement were collected using pressure sensitive treadmill and Codamotion active marker system...
May 24, 2017: Scandinavian Journal of Medicine & Science in Sports
https://www.readbyqxmd.com/read/28543406/novel-murine-xenograft-model-for-the-evaluation-of-stem-cell-therapy-for-profound-dysphagia
#12
Maggie A Kuhn, Amanda B Black, M Tausif Siddiqui, Jan A Nolta, Peter C Belafsky
OBJECTIVES/HYPOTHESIS: Dysphagia is common and costly. Treatments are limited and innovative therapies are required. The tongue is essential for safe, effective swallowing and is a natural target for regenerative therapy. Muscle-derived stem cells (MDSCs) hold potential to restore dynamic function, and their application in the damaged tongue is appealing. We examined the safety and efficacy of human MDSC implantation into a novel mouse tongue model. STUDY DESIGN: Animal study...
May 23, 2017: Laryngoscope
https://www.readbyqxmd.com/read/28542133/protein-disulfide-isomerase-mediated-apoptosis-and-proliferation-of-vascular-smooth-muscle-cells-induced-by-mechanical-stress-and-advanced-glycosylation-end-products-result-in-diabetic-mouse-vein-graft-atherosclerosis
#13
Suning Ping, Shuying Liu, Yuhuan Zhou, Ziqing Li, Yuhuang Li, Kefeng Liu, Adham Sa Bardeesi, Linli Wang, Jingbo Chen, Lie Deng, Jingjing Wang, Hong Wang, Dadi Chen, Zhengyu Zhang, Puyi Sheng, Chaohong Li
Protein disulfide isomerase (PDI) involves cell survival and death. Whether PDI mediates mechanical stretch stress (SS) and/or advanced glycosylation end products (AGEs) -triggered simultaneous increases in proliferation and apoptosis of vascular smooth muscle cells (VSMCs) is unknown. Here, we hypothesized that different expression levels of PDI trigger completely opposite cell fates among the different VSMC subtypes. Mouse veins were grafted into carotid arteries of non-diabetic and diabetic mice for 8 weeks; the grafted veins underwent simultaneous increases in proliferation and apoptosis, which triggered vein graft arterializations in non-diabetic or atherosclerosis in diabetic mice...
May 25, 2017: Cell Death & Disease
https://www.readbyqxmd.com/read/28541423/grip-strength-and-inflammatory-biomarker-profiles-in-very-old-adults
#14
Antoneta Granic, Karen Davies, Carmen Martin-Ruiz, Carol Jagger, Thomas B L Kirkwood, Thomas von Zglinicki, Avan Aihie Sayer
Background: weak grip strength (GS) and chronic inflammation have been implicated in the aetiology of sarcopenia in older adults. Given the interrelationships between inflammatory biomarkers, a summary variable may provide better insight into the relationship between inflammation and muscle strength. This approach has not been investigated in very old adults (aged ≥85) who are at highest risk of muscle weakness. Methods: we used mixed models to explore the prospective association between GS over 5 years in 845 participants in the Newcastle 85+ Study, and inflammatory components identified by principal component analysis (PCA)...
May 25, 2017: Age and Ageing
https://www.readbyqxmd.com/read/28540637/importance-of-human-peritoneal-mesothelial-cells-in-the-progression-fibrosis-and-control-of-gastric-cancer-inhibition-of-growth-and-fibrosis-by-tranilast
#15
Hiroto Saito, Sachio Fushida, Shinichi Harada, Tomoharu Miyashita, Katsunobu Oyama, Takahisa Yamaguchi, Tomoya Tsukada, Jun Kinoshita, Hidehiro Tajima, Itasu Ninomiya, Tetsuo Ohta
BACKGROUND: Scirrhous gastric cancer is an intractable disease with a high incidence of peritoneal dissemination and obstructive symptoms (e.g., ileus, jaundice, and hydronephrosis) arising from accompanying marked fibrosis. Microenvironmental interactions between cancer cells and cancer-associated fibroblasts are the suggested cause of the disease. We elucidated the mechanisms of tumor growth and fibrosis using human peritoneal mesothelial cells (HPMCs) and investigated the effects of tranilast treatment on cells and a xenograft mouse model of fibrosis...
May 24, 2017: Gastric Cancer
https://www.readbyqxmd.com/read/28540249/liquid-biopsies-for-bladder-cancer
#16
EDITORIAL
Douglas G Ward, Richard T Bryan
The development of accurate urinary biomarkers for the non-invasive detection of urothelial bladder cancer (UBC) could transform patient pathways by reducing reliance on cystoscopy, and the identification of highly prognostic (or even predictive) biomarkers could better guide patient management. A number of approaches are being utilised to address these challenges in both urinary- and plasma-borne tumour DNA (tDNA), so-called "liquid biopsies". Next generation sequencing (NGS) and droplet digital PCR (ddPCR) allow detection of very low levels of such tDNA amongst a large excess of non-tumour DNA, the former permitting large mutation panels to be assessed and the latter potentially identifying ultrarare mutant alleles yet restricted for multiplexing...
April 2017: Translational Andrology and Urology
https://www.readbyqxmd.com/read/28538253/immune-myopathy-with-perimysial-pathology-associated-with-interstitial-lung-disease-and-anti-ej-antibodies
#17
Patrick M Kwon, Lan Zhou, Rajeev Motiwala, Leslie D Kerr, Susan C Shin
OBJECTIVES: We report a case of immune myopathy with perimysial pathology associated with anti-glycyl-transfer RNA synthetase (anti-EJ) antibody and an excellent treatment response. METHODS: Chart review. RESULTS: A 36-year-old woman presented with 3 months of fatigue, weight loss, progressive weakness in a scapuloperoneal distribution, and dysphagia. Nerve conduction studies, electromyography, and ultrasound suggested an irritable myopathy...
June 2017: Journal of Clinical Neuromuscular Disease
https://www.readbyqxmd.com/read/28538252/concurrent-paraspinous-myopathy-and-myasthenia-gravis
#18
Alissa E Romano, Zaid Al-Qudah, Henry J Kaminski, Bashar Katirji, Karim Salame
Paraspinous myopathy is a rare neuromuscular disorder characterized by selective involvement of the cervical, thoracic, or lumbar muscles. Leading clinical features include a bent spine or dropped head (antecollis). In myasthenia gravis (MG), patients may have camptocormia secondary to neuromuscular junction dysfunction of the paraspinal muscles, and this condition usually responds to acetylcholinesterase inhibitors or immunosuppressive treatments. However, concomitant MG and paraspinous myopathy with histologic and electrophysiologic evidence of myopathic changes of the paraspinal muscles has only been reported twice in the literature...
June 2017: Journal of Clinical Neuromuscular Disease
https://www.readbyqxmd.com/read/28535487/nerve-excitability-changes-related-to-muscle-weakness-in-chronic-progressive-external-ophthalmoplegia
#19
Antoine Gueguen, Claude Jardel, Marc Polivka, S Veronica Tan, Françoise Gray, Catherine Vignal, Anne Lombès, Olivier Gout, Hugh Bostock
OBJECTIVE: To explore potential spreading to peripheral nerves of the mitochondrial dysfunction in chronic progressive external ophthalmoplegia (CPEO) by assessing axonal excitability. METHODS: CPEO patients (n=13) with large size deletion of mitochondrial DNA and matching healthy controls (n=22) were included in a case-control study. Muscle strength was quantified using MRC sum-score and used to define two groups of patients: CPEO-weak and CPEO-normal (normal strength)...
April 26, 2017: Clinical Neurophysiology: Official Journal of the International Federation of Clinical Neurophysiology
https://www.readbyqxmd.com/read/28533552/physical-activity-for-paediatric-rheumatic-diseases-standing-up-against-old-paradigms
#20
REVIEW
Bruno Gualano, Eloisa Bonfa, Rosa M R Pereira, Clovis A Silva
Over the past 50 years it has become clear that physical inactivity is associated with chronic disease risk. For several rheumatic diseases, bed rest was traditionally advocated as the best treatment, but several levels of evidence support the imminent paradigm shift from the prescription of bed rest to physical activity in individuals with paediatric rheumatic diseases, in particular juvenile systemic lupus erythematosus, juvenile idiopathic arthritis, juvenile fibromyalgia, and juvenile dermatomyositis. Increasing levels of physical activity can alleviate several symptoms experienced by patients with paediatric rheumatic diseases, such as low aerobic fitness, pain, fatigue, muscle weakness and poor health-related quality of life...
May 23, 2017: Nature Reviews. Rheumatology
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