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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。
Objective To study the pathological, immunohistochemical and ultrastructural changes of skeletal muscle malnutrition (SMD) and its relationship with disease progression. Methods Twenty skeletal muscle samples from SMD patients were selected and paraffin sections, semi-thin sections and ultra-thin sections were made according to the conventional method. The specimens were observed under light and electron microscope. Results 20 cases of SMD were divided into three types: 8 cases of simple muscular dystrophy, mostly focal lesions; progressive muscular dystrophy in 10 cases, mostly diffuse lesions accompanied by a large number of organelles degeneration; nerve 2 cases of muscular dystrophy, showing the degeneration of nerve-dominated area of skeletal muscle injury. Myosin first degenerates when skeletal muscle is damaged. Conclusion Pathological changes and ultrastructural changes can determine the degree of skeletal muscle malnutrition progress; Myosin loss can be an early prediction of disease progression.