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进行性肌营养不良症(Progressive muscular dystophy)是一种遗传性进行性的随意肌疾病。其中有数种病型;Duchenne型(简称DMD)是一种X连锁隐性遗传性疾病。病理特征为细胞膜结构和通透性异常,使随意肌细胞内代谢产物不断渗出,最后导致肌纤维的变性坏死,部份肌纤维为结缔组织和脂肪所充填而形成假性肥大,临床可见腰、骨盆部伸直肌和胸肌、肩胛、上臂部肌群呈对称性进行性萎缩,而腓肠肌呈假性肥大。由于随意肌的萎缩无力,患儿步态不
Progressive muscular dystrophy (Progressive muscular dystophy) is a hereditary progressive disorder of the voluntary muscles. There are several types of disease; Duchenne type (referred to as DMD) is a X-linked recessive genetic disease. Pathological features of the abnormal cell membrane structure and permeability, so that the constant intracellular metabolites in muscle cells constantly exudation, and finally lead to degeneration and necrosis of muscle fibers, some muscle fibers for the formation of connective tissue and fat filling and the formation of pseudo-hypertrophy, clinically visible waist and pelvis Department of extensor and pectoral muscles, scapula, upper arm muscles symmetrical progressive atrophy, while the gastrocnemius muscle was false hypertrophy. Due to atrophy of voluntary muscles, children with gait not