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[施泰纳特综合征与心肌。心肌的全基因表达]

[Steinert's syndrome and the myocardium. Total gene expression by the myocardium].

作者信息

Schmitt J, Schmidt C l

出版信息

J Genet Hum. 1975 Mar;23(1):59-64.

PMID:51906
Abstract

As revealed by a very thorough cardiological study of Steinert's disease in 13 cases, it would appear that the incidence of cardiac involvement observed in this disease is not merely frequent; it is, in fact, usual, systematic, and forms an integral part of the pathological picture in the same way as peripheral muscular dystrophy. One is aware of the diagnostic interest of this fact in the juvenile or abortive forms of the disease. The cardiac involvement is an indication of the complete expression of the gene towards striated muscle tissue, whether skeletal or myocardial. Finally, it opens new perspectives on the prophylactic attitude to be adopted for such patients; regular and systematic cardiological checks, moderation in the prescription of digitalis and anti-arythmia drugs, implantation of a cardiac pacemaker before an auriculo-ventricular syncopal block occurs.

摘要

一项针对13例斯坦纳特病的详尽心脏病学研究表明,该疾病中心脏受累的发生率似乎不仅常见;事实上,它是普遍、系统性的,并且与周围肌肉营养不良一样,构成了病理表现的一个组成部分。人们认识到这一事实在该疾病的青少年或顿挫型中的诊断意义。心脏受累表明基因在骨骼肌或心肌等横纹肌组织中完全表达。最后,它为针对此类患者应采取的预防措施开辟了新的前景;定期进行系统性心脏病检查,谨慎使用洋地黄和抗心律失常药物,在发生房室性晕厥性传导阻滞之前植入心脏起搏器。

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