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有关特发性扩张型心肌病(IDC)的病因学或IDC 的病理机制的了解很少.已知某些 IDC 病例可发生在同一家族成员中。本文介绍了5个家庭中的IDC 病人(每个家庭中2例)的心肌活检结果和经电镜发现的超微结构变化及临床征象.10例均有晚期心肌病的临床表现.其中2家庭中分别为2例男病人,2家庭中分别为2例女病人,1家庭中男女各1例.平均年龄16岁(13～21岁).62例经年龄配对确诊为晚期心肌病而无家族史的病人做为对照.另设607例老年性充血性心衰或心肌缺血病人和30例正常人做对照。结果除正常对照者外,所有受检者显示有心肌肥厚延长,核深染与不规则.家族性心肌病患者的心肌在电镜下,除见中度心肌肥厚及胞核轻度增大外,常有脂色素
Little is known about the etiology of idiopathic dilated cardiomyopathy (IDC) or the pathology of IDC, and some IDC cases are known to occur in the same family. This article presents the results of myocardial biopsy and ultrastructural changes and clinical signs of IDC patients in 5 families (2 in each family) found by electron microscopy and clinical manifestations of 10 patients with advanced cardiomyopathy, of which 2 families Respectively, 2 male patients, 2 family were 2 female patients, 1 family of 1 male and 1 female, with an average age of 16 years (13 to 21 years old) .62 cases of late cardiomyopathy by age paired with no family history Of patients as a control.Another set of 607 cases of senile congestive heart failure or myocardial ischemia and 30 normal controls. Results Except for the normal controls, all the subjects showed prolonged cardiac hypertrophy, deep-stained nuclei and irregular nuclei.In patients with familial cardiomyopathy, myocardial hypertrophy and mild nuclear enlargement were observed under electron microscope, Often fat pigment