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Muscle Disease

Yongbin Lu, Zhiyuan Cheng, Yaxue Zhao, Xiaoyu Chang, Cynthia Chan, Yana Bai, Ning Cheng
BACKGROUND AND AIMS: Our study aims to evaluate the efficacy and safety of long-term treatment of statins for coronary heart disease (CHD). METHODS: Efficacy outcomes included changes in blood lipids, risk of CHD mortality and all-cause mortality. Safety outcomes were evaluated by the risk of adverse events (AE). Bayesian network meta-analysis was used to compare the direct and indirect effects between different statins. RESULTS: The systematic review showed that levels of blood lipids decreased during statin treatment...
October 14, 2016: Atherosclerosis
C Dolfus, J-P Simon, G Landemore, F Leroy, F Chapon
INTRODUCTION: The clinical and histological presentations of the adult form of Pompe disease may be atypical. In such cases, identifying histological signs that point to the diagnosis would be crucial to avoid a delay in care. The aim of our study was to investigate the presence of rimmed vacuoles and acid phosphatase positivity in muscle biopsies of patients with late-onset Pompe disease. MATERIAL AND METHODS: We retrospectively studied muscle biopsies of all cases of the adult form of Pompe disease diagnosed at the University Hospital of Caen...
2016: Folia Neuropathologica
Anand Pandey, Vipin Gupta, Shailendra P Singh, Vijendra Kumar, Rajesh Verma
A trophic ulcer is a pressure ulcer caused by external trauma to a part of the body that is compromised due to disease, vascular insufficiency, or loss of afferent nerve fibers. Spinal dysraphism (ie, neural tube defects [NTD]) such as meningomyelocele is a risk factor for developing these ulcers in adults and pediatric patients. Information regarding the occurrence of trophic ulcers in pediatric patients with NTD is lacking. A review of the English-language literature on skin/neuropathic ulcers in patients with NTDs, irrespective of study design, published between 1975 and 2014, was undertaken using the PubMed database...
December 2015: Ostomy/wound Management
Bobby A Tajudeen, Zachary D Taylor, James Garritano, Harrison Cheng, Aidan Pearigen, Adria J Sherman, Fernando Palma-Diaz, Pratik Mishra, Siddharth Bhargava, Julianna Pesce, Irene Kim, Christine Sebastian, Ali Razfar, Asael Papour, Oscar Stafsudd, Warren Grundfest, Maie St John
BACKGROUND: Head and neck squamous cell carcinomas (HNSCCs) are debilitating diseases for which a patient's prognosis depends heavily on complete tumor resection. Currently, the surgeon's fingers determine the location of tissue margins. This study evaluated the diagnostic utility of a novel imaging modality, dynamic optical contrast imaging (DOCI), in the detection of HNSCC. This system generates contrast by illuminating the tissue with pulsed light and detecting variations in endogenous fluorophore lifetimes...
October 20, 2016: Cancer
Rajeev B Tajhya, Xueyou Hu, Mark R Tanner, Redwan Huq, Natee Kongchan, Joel R Neilson, George G Rodney, Frank T Horrigan, Lubov T Timchenko, Christine Beeton
Myoblasts are mononucleated precursors of myofibers; they persist in mature skeletal muscles for growth and regeneration post injury. During myotonic dystrophy type 1 (DM1), a complex autosomal-dominant neuromuscular disease, the differentiation of skeletal myoblasts into functional myotubes is impaired, resulting in muscle wasting and weakness. The mechanisms leading to this altered differentiation are not fully understood. Here, we demonstrate that the calcium- and voltage-dependent potassium channel, KCa1...
October 20, 2016: Cell Death & Disease
Matthias Boentert, Hélène Prigent, Katalin Várdi, Harrison N Jones, Uwe Mellies, Anita K Simonds, Stephan Wenninger, Emilia Barrot Cortés, Marco Confalonieri
Pompe disease is an autosomal-recessive lysosomal storage disorder characterized by progressive myopathy with proximal muscle weakness, respiratory muscle dysfunction, and cardiomyopathy (in infants only). In patients with juvenile or adult disease onset, respiratory muscle weakness may decline more rapidly than overall neurological disability. Sleep-disordered breathing, daytime hypercapnia, and the need for nocturnal ventilation eventually evolve in most patients. Additionally, respiratory muscle weakness leads to decreased cough and impaired airway clearance, increasing the risk of acute respiratory illness...
October 17, 2016: International Journal of Molecular Sciences
Liang Gao, Gülce Sila Gülcüler, Lieke Golbach, Helena Block, Alexander Zarbock, Ana Martin-Villalba
Integrin activation is crucial for regulation of leukocyte rolling, adhesion and trans-vessel migration during inflammation and occurs by engagement of myeloid cells through factors presented by inflamed vessels. However, endothelial-dependent mechanisms of myeloid cell recruitment are not fully understood. Here we show using an autoperfused flow chamber assay of whole blood neutrophils and intravital microscopy of the inflamed cremaster muscle that CD95 mediates leukocyte slow rolling, adhesion and transmigration upon binding of CD95-ligand (CD95L) that is presented by endothelial cells...
October 20, 2016: ELife
Amanda Faria Assoni, Giuliana Castello, Marcos Valadares, Melinda Beccari, Juliana Gomes, Mayra Pelatti, Miguel Mitne-Neto, Valdemir Melechco Carvalho, Mayana Zatz
Duchenne muscular dystrophy (DMD) is a lethal X-linked disorder caused by null mutations in the dystrophin gene. Although the primary defect is the deficiency of muscle dystrophin, secondary events, including chronic inflammation, fibrosis and muscle regeneration failure are thought to actively contribute to disease progression. Despite several advances, there is still no effective therapy for DMD. Therefore, the potential regenerative capacities, as well as immune-privileged properties of Mesenchymal Stromal Cells (MSCs), have been the focus of intense investigation in different animal models aiming the treatment of these disorders...
October 20, 2016: Stem Cells and Development
Colin Crist
Skeletal muscle is the most abundant tissue in our body, is responsible for generating the force required for movement and is also an important thermogenic organ. Skeletal muscle is an enigmatic tissue because, while on one hand, skeletal muscle regeneration after injury is arguably one of the best studied stem cell dependent regenerative processes, on the other hand, skeletal muscle is still subject to many degenerative disorders with few therapeutic options in the clinic. It is important to develop new regenerative medicine based therapies for skeletal muscle...
October 20, 2016: Journal of Pathology
Yan Xiong, Xiangfang Tang, Qingshi Meng, Hongfu Zhang
Ammonia has been considered the contaminant primarily responsible for respiratory disease in poultry. Even though it can cause tracheal lesions, its adverse effects on the trachea have not been sufficiently studied. The present study investigated tracheal changes in Arbor Acres broilers (Gallus gallus) induced by high concentration of ammonia using isobaric tag for relative and absolute quantification (iTRAQ)-based proteome analysis. In total, 3,706 proteins within false discovery rate of 1% were identified, including 119 significantly differentially expressed proteins...
October 17, 2016: Science China. Life Sciences
Avadhesh Kumar, Shalini Kaushal, Neelu Verma, Deepti Chandra
The treatment of gingival recession defects is indicated for esthetic and functional reasons to reduce root sensitivity, to remove muscle pull, to create or augment keratinized tissue, and to prevent disease progression. The presence of sufficient amount of periosteum adjacent to gingival recession defects makes it a suitable graft. The adult human periosteum is a highly vascular connective tissue with immense regenerative potential. It contains fibroblasts, osteogenic progenitor cells, and stem cells as a result of which it has the ability to differentiate into fibroblast, osteoblast, chondrocytes, adipocytes, and skeletal myocytes...
September 2016: Journal of Oral Biology and Craniofacial Research
Kazuhiko Yoshimatsu, Gakuji Osawa, Hajime Yokomizo, Yuki Yano, Sachiyo Okayama, Akiko Sakuma, Masaya Satake, Yasufumi Yamada, Shinichi Asaka, Takebumi Usui, Kentaro Yamaguchi, Shunichi Shiozawa, Takeshi Shimakawa, Takao Katsube, Yoshihiko Naritaka
A 78-year-old man visited our hospital with a prolapsed hemorrhoid. He was referred to the dermatology unit due to the thickness and redness of the perianal skin. He was diagnosed as having extra mammary Paget's disease by skin biopsy. After a biopsy of the anal polyp was performed to investigate the primary site, he was diagnosed with early anal canal cancer with Pagetoid spread and underwent a radical operation. Abdominoperineal resection with skin(D2 prx D3 lymphadenectomy) was performed with perineal reconstruction using a gracilis muscle graft...
October 2016: Gan to Kagaku Ryoho. Cancer & Chemotherapy
Can-Zhao Liu, Xiang-Yu Li, Ren-Hong Du, Min Gao, Ming-Ming Ma, Fei-Ya Li, Er-Wen Huang, Hong-Shuo Sun, Guan-Lei Wang, Yong-Yuan Guan
BACKGROUND: Previous research has demonstrated that ClC-3 is responsible for volume-regulated Cl(-)current (ICl.vol) in vascular smooth muscle cells (VSMCs). However, it is still not clear whether and how ClC-3 is transported to cell membranes, resulting in alteration ofICl.vol.Methods and Results:Volume-regulated chloride current (ICl.vol) was recorded by whole-cell patch clamp recording, and Western blotting and co-immunoprecipitation were performed to examine protein expression and protein-protein interaction...
October 19, 2016: Circulation Journal: Official Journal of the Japanese Circulation Society
Feng Hao, Fuqiang Zhang, Daniel Dongwei Wu, Dong An, Jing Shi, Guohong Li, Xuemin Xu, Mei-Zhen Cui
Vascular smooth muscle cell (SMC) migration is an essential step involved in neointimal formation in restenosis and atherosclerosis. Lysophosphatidic acid (LPA) is a bioactive component of oxidized low density lipoprotein and is produced by activated platelets, implying that LPA influences vascular remodeling. Our previous study revealed that matricellular protein CCN1, a prominent extracellular matrix (ECM) protein, mediates LPA-induced SMC migration in vitro. Here we examined the role of CCN1 in LPA-induced neointimal formation...
October 19, 2016: American Journal of Physiology. Cell Physiology
M Lu, D S Fan
Objective: To investigate whether or not the involvement degree of extensor and flexor muscles in patients with Kennedy's Disease (KD) was different by measuring the diameter of biceps and triceps brachii muscles using Magnetic Resonance Imaging (MRI). Methods: All the subjects were outpatients or hospitalized patients from Department of Neurology, the Third Hospital of Peking University, 2011-2015. Subjects included 30 KD patients and 30 amyotrophic lateral sclerosis (ALS) patients. The right upper arms of all subjects were examined by muscle MRI...
October 11, 2016: Zhonghua Yi Xue za Zhi [Chinese medical journal]
Pushpa Raj Joshi, Thekla Apitz, Stephan Zierz
During physical activity in McArdle patients, little or no lactate is released in the skeletal muscle. However, excessive ammonia production has frequently been reported in these patients. Production of ammonia is catalysed by AMP deaminase (AMPD) and adenylate kinase (AK). The activities of AMPD and AK along with housekeeping enzyme phosphoglucoisomerase (PGI) were measured in 11 genetically confirmed McArdle patients and compared with 27 healthy controls. The AMPD and AK activities were not significantly different in patients and controls...
October 20, 2016: Neurological Research
Michel van Schaardenburgh, Martin Wohlwend, Øivind Rognmo, Erney J R Mattsson
PURPOSE: Mitochondria are essential for energy production in the muscle cell and for this they are dependent upon a sufficient supply of oxygen by the circulation. Exercise training has shown to be a potent stimulus for physiological adaptations and mitochondria play a central role. Whether changes in mitochondrial respiration are seen after exercise in patients with a reduced circulation is unknown. The aim of the study was to evaluate the time course and whether one session of calf raise exercise stimulates mitochondrial respiration in the calf muscle of patients with peripheral vascular disease...
2016: PloS One
Renata Rubinsztajn, Tadeusz Przybyłowski, Marta Maskey-Warzęchowska, Krzysztof Karwat, Ryszarda Chazan
: Body composition disorders are observed in chronic obstructive pulmonary disease (COPD) patients and have a significant impact the general condition and outcome in this disease. AIM: The aim of the study was to assess body composition in relation to airway obstruction severity in patients with COPD. MATERIALS AND METHODS: The study group consisted of 143 patients (58F, 85M) in the middle age The group was divided to two groups: group A FEV1<50% predicted and group B FEV1 ≥50% predicted; both groups were compared...
October 19, 2016: Polski Merkuriusz Lekarski: Organ Polskiego Towarzystwa Lekarskiego
Ademar Francisco de Oliveira, Gêssyca Adryene de Menezes Silva, Débora Milenna Xavier Almeida
Amyotrophic lateral sclerosis is a progressive and fatal neurodegenerative disease characterized by the degeneration of motor neurons, which are the central nervous system cells that control voluntary muscle movements. The excessive salivation (sialorrhea) is present in approximately 50% of amyotrophic lateral sclerosis cases. Thus, some alternative therapeutic methods are sought, such as anticholinergic drugs and surgery. Recently the use of botulinum toxin applied at a midpoint of the salivary glands, often guided by ultrasound, have demonstrated positive results...
July 2016: Einstein
Xiaojing Wang, Fan Ping, Cuijuan Qi, Xinhua Xiao
BACKGROUND: Autoimmune polyglandular syndrome type 2 (APS-2), also known as Schmidt's syndrome, is an uncommon disorder characterized by the coexistence of Addison's disease with thyroid autoimmune disease and/or type 1 diabetes mellitus. Addison's disease as the obligatory component is potentially life-threatening. Unfortunately, the delayed diagnosis of Addison's disease is common owing to its rarity and the nonspecific clinical manifestation. METHODS: Here we reported a case of 38-year-old female patient who presented with 2 years' history of Hashimoto's thyroiditis and received levothyroxine replacement...
October 2016: Medicine (Baltimore)
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