【摘要】 目的　研究骨骼肌营养不良症 (SMD)的病理学、免疫组织化学及超微结构改变与疾病进展的关系。方法　选取 2 0例 SMD患者的骨骼肌组织 ,按常规制作石蜡切片、半薄切片和超薄切片 ,采用光镜和电镜系统观察。结果　 2 0例 SMD分为三种类型 :单纯性肌营养不良 8例 ,多为局灶性的病变 ;进行性肌营养不良 10例 ,多为弥漫性的病变并伴有大量的细胞器变性 ;神经源性肌营养不良 2例 ,可见为变性的神经所支配区域骨骼肌的损伤。骨骼肌受损伤时 ,Myosin首先发生变性。结论　病理改变及超微结构改变可判断骨骼肌营养不良的进展程度 ;Myosin的丢失程度可早期预测疾病的进展程度。更多还原

【Abstract】 Objective To study the relationship between pathological, immunohistochemical and ultrastructural changes of skeletal myodystrophy (SMD) and the development of the disease.Methods SMD tissue of 20 cases were routinely processed,the paraffin sections,the semi thin sections and the ultrasthin sections were observed by light microscopy and electron microscopy.Results 20 cases with SMD tissue were divided into three groups: Simple SMD for 8 cases, major changes were regional; Progressive SMD for 10 ca ses, the pathological changes were diffuse with large amount of degeneration of cell organs; SMD derived from nerve injury for 2 cases, pathological changes of the part controlled by the nerve were observed. While SMD was injured, myosin got deneration first.Conclusion The pathological and ultrastructure changes could be used to judge the progressive degree of myodystrophin. The amount of lost myosin could forecast the progression of the disease.更多还原