脂质沉积症骨骼肌纤维的电镜观察

Transmission electron microscopic observation on skeletal muscle fibers of lipoidosis

观察脂质沉积症骨骼肌纤维的超微病理变化特征。活检肌组织取自一例16岁少女,透射电镜制样观察。镜下显示病变的肌纤维内堆积有大量脂滴,肌节模糊,肌丝紊乱,Z线断裂,线粒体嵴变态成网管状,胞质内同时伴有糖原颗粒增多,提示脂质的不正常沉积可导致肌纤维内超微结构的病理改变。

To observe the uhrastructural change of a case of lipoid storage disease （lipoidose）of skeletal muscle fibers. The muscle biopsy specimen was taken from a patient of 16-year-old girl and uhrathin sections were observed under the transmission electron microscope. There were a large number of lipid droplet deposit in the sareoplasm, associated with disarrangement of sarcomeres and myofilaments. In addition, the glycogen granules in the sarcoplasm were a bit abundant than normal skeletal muscle. It is suggested that the abnormally deposit of lipoids in myofrils could lead to the pathophysiological changes of lipoidosis of skeletal muscle fiber.