McArdle病的超微结构观察

本文报道两例Ｖ型糖元沉积性肌病的肌活检超微形态改变，光镜下见肌纤维中呈现的ＰＡＳ染色阳性的颗粒状沉积物质，肌磷酸化酶染色阴性。电镜检查可区别为三种形态学改变：（一）肌膜下及肌原纤维间有多量游离糖颗粒沉积；（二）在完全破坏的纤维中，糖元颗粒聚集成圆球形和不规则形；（三）出现众多的空泡，其间见残存的细胞碎片和散在稀疏的糖元粒沉积。

Ultrastructure of Muscle Biopsy in McArdle Disease

Electron microscopical observations of the muscle biopsies in 2 cases with McArdle disease (glycogenosis V) is reported. Three types of changes could be distinguished.Accumulations of free glycogen granules under sarcolemma and between myofibrils were most outstanding,as mentioned by most authors.We found,however,ball like and irregularly shaped dense aggregations of glycogen granules,which were without membrane bound.Thirdly,numerous vacuoles were seen,in which there were destroyed cell fragments and sparse glycogen particles.The latter two changes occurred in those areas where myofibrils underwent severe destruction.