Kearns-Sayre syndromes an absolute indication for prophylactic implantation of definitive pacemaker?

H M Barragán-Campos, C F Barrera-Ramírez, P Iturralde Torres, H Ilarraza-Lomelí, M C Avila-Casado, B Estañol, J Dorantes, J Oseguera
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Abstract

Kearns-Sayre syndrome is a mitochondrial cytopathy characterized by chronic progressive external ophthalmoplegia, retinitis pigmentosa and heart block, the last of which determines the survival of these patients. The case of a 23 year old man with Kearns-Sayre syndrome, conduction disturbances and mitral valve prolapse is presented. The characteristics of this syndrome are described and the criteria for prophylactic installation of a pacemaker discussed.

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卡恩斯-塞尔综合征是预防性植入决定性起搏器的绝对指征?
Kearns-Sayre综合征是一种线粒体细胞病变,以慢性进行性眼外麻痹、视网膜色素变性和心脏传导阻滞为特征,后者决定了这些患者的生存。病例的23岁男子卡恩斯-塞尔综合征,传导障碍和二尖瓣脱垂提出。本文描述了该综合征的特点,并讨论了预防性安装起搏器的标准。
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