Abstract

DE CASTRO JUNIOR, Hélio; MONTES PENA, Felipe; RIBEIRO, Mário Luiz  and  DE ANDRADE MARTINS, Wolney. Kearns-Sayre syndrome: Case report. Insuf. card. [online]. 2011, vol.6, n.2, pp.92-95. ISSN 1852-3862.

Kearns-Sayre syndrome The Kearns-Sayre syndrome (KSS) is a mitochondriopathies characterized by multiorgan dysfunction that typically develops before the age of twenty. The diagnostic criteria most widely accepted in the literature are a triad: progressive external ophthalmoplegia, pigmentary retinopathy and disorders of cardiac conduction. KSS prognosis is related to the number of tissues affected and the severity of the alterations. In this article we report on a patient who presented 18 clinical features consistent with the Kearns-Sayre syndrome.

Keywords : Mitochondrial diseases; Kearns-Sayre syndrome; Congenital complete atrioventricular block; Heart failure.

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