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进行性肌营养不良患者的肌浆膜异常得到了多方面证实.生化异常包括血清肌酸激酶(CK)增加,肌腺环酶(muscle adenyl cyclase)对肾上腺素的敏感性降低及Na-K-Mg-ATP酶减少且对哇巴因(Quabain)反应发生改变.形态学异常已观察到与肌浆膜相邻接的肌纤维稀疏区(病灶),电镜发现肌浆膜崩解,肌浆变性;电子细胞化学研究证实过氧化酶可经肌浆膜崩解处进入.为了进一步研究该病早期肌浆膜异常,作者对6例婴幼儿患者作了肌浆膜超微结构研究并与6名正常儿童(对照组)作了对比.6例患儿中,2例为无症状婴儿:其中1例为2个月
Skeletal muscle abnormalities in patients with progressive muscular dystrophy have been demonstrated in several ways: Biochemical abnormalities include an increase in serum creatine kinase (CK), a decrease in the sensitivity of the muscle adenyl cyclase to epinephrine, and a decrease in Na-K- Mg-ATPase decreased and Quabain reaction changed Morphological abnormalities Muscle sparsity adjacent to sarcoplasmic reticulum (lesion) was observed. The sarcoplasmic reticulum and sarcoplasmic reticulum were found by electron microscopy. Electron cytochemistry studies have confirmed that peroxidase can enter through the sarcoplasmic disintegration in order to further study the disease early sarcoplasmic abnormalities, the authors of 6 cases of infants and young children made sarcoplasmic membrane ultrastructure study and 6 normal Children (control group) were compared, 6 of 6 children were asymptomatic infants: one of 2 children