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Near-fatal Kearns-Sayre Syndrome

A Case Report and Review of Clinical Manifestations

Division of Pediatric Cardiology and Internal Medicine, University of Virginia School of Medicine, Charlottesville, Virginia

Nancy A. Ayres, MD

Division of Pediatric Cardiology and Internal Medicine, University of Virginia School of Medicine, Charlottesville, Virginia

T. Duncan Sellers, MD

Division of Pediatric Cardiology and Internal Medicine, University of Virginia School of Medicine, Charlottesville, Virginia

John P. DiMarco, MD

Division of Pediatric Cardiology and Internal Medicine, University of Virginia School of Medicine, Charlottesville, Virginia

The Kearns-Sayre syndrome is a relatively uncommon condition which occurs in childhood and is characterized by neurologic dysfunction and abnormalities in atrioventricular (AV) conduction. The high mortality rate is directly attrib utable to the cardiovascular manifestations of this syndrome. We report a case, with documentation of a "near-fatal" episode, and review the pertinent clinical features of the syndrome.

Clinical Pediatrics, Vol. 22, No. 12, 822-825 (1983)
DOI: 10.1177/000992288302201208


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