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假肥大型进行性肌营养不良,又称Duchenne氏病(简称DMD),系属性连隐性遗传性疾病,多发生于男性儿童。本文对24例DMD患者腓肠肌活体组织进行了光镜及电镜观察,并作了有关血清生化测定;此外对三例患儿母亲及一例患儿未发病的兄弟作了同样观察。一、观察结果:1.腓肠肌肌纤维有不同程度变性及灶性坏死,肌膜有灶性缺损(见图),肌浆网扩张,线粒体固缩或肿胀,也可见部份肌节破坏。2.可见极少数新生肌纤维,有肌细丝,未形成完整的肌节,肌核个别有坏死固缩;有的核变大,核仁及常染色质明显,但细胞质内或核周区域内核蛋白体极少。3.小血管及毛细血管未见
Duchenne muscular dystrophy, also known as Duchenne’s disease (referred to as DMD), Department of inheritance with occult genetic disease, occurs in men and women. In this paper, 24 cases of DMD gastrocnemius muscle tissue was observed by light and electron microscopy, and made the serum biochemical determination; in addition to three children with mothers and a case of children without the disease made the same observation. First, the observation results: 1. Gastrocnemius muscle fibers have varying degrees of degeneration and focal necrosis, myofascial fissure (see photo), sarcoplasmic reticulum dilatation, mitochondria shrink or swelling, but also some of the damage can be seen. 2. Visible very few newborn muscle fibers, there are muscle filaments, did not form a complete sarcomere, muscle nucleus individual necrosis; Some nuclei become larger, clear nucleoli and chromatin, but the cytoplasm or perinuclear region of the inner core Very few protein body. No small blood vessels and capillaries