Csanády M
Szent-Györgyi Albert Orvostudományi Egyetem, Szeged, II. sz. Belgyógyászati Klinika.
Orv Hetil. 1993 Mar 7;134(10):507-11.
The author surveys the literature and gives a state of art of familial dilatative cardiomyopathy including his own experience. In the past two decades, III familial dilatative cardiomyopathy cases have been encountered in 30 families in their own material, which means a 21.5 percent incidence rate as concerns the total number of dilatative cardiomyopathy patients. The inheritance pattern was autosomal dominant in the majority of the cases. Both cardiomegaly (20 vs 29.6 ys) and the complaints (29.9 vs 37.6 ys) displayed an earlier onset in familial dilatative cardiomyopathy than in the nonfamilial form. The characters of the signs and symptoms did not differ otherwise between the two groups. Significant differences were observed between the two groups in the progression of the disease: the five-year survival rate after the onset of the symptoms was 23.1 percent in the familial cases and 52.1 percent in the non-familial form (p = 0.026).
作者查阅了文献，并结合自身经验，对家族性扩张型心肌病的现状进行了阐述。在过去二十年中，作者自身收集的资料显示，在30个家族中发现了III例家族性扩张型心肌病病例，这意味着在所收集的全部扩张型心肌病患者中，发病率为21.5%。大多数病例的遗传模式为常染色体显性遗传。与非家族性扩张型心肌病相比，家族性扩张型心肌病的心脏扩大（20岁对29.6岁）和症状出现（29.9岁对37.6岁）都更早。两组在体征和症状特点上没有其他差异。两组在疾病进展方面存在显著差异：症状出现后的五年生存率，家族性病例为23.1%，非家族性病例为52.1%（p = 0.026）。
