Chawla Sanjay, Coku Jorida, Forbes Thomas, Kannan Sujatha
Pediatrics, Children's Hospital of Michigan, 3901 Beaubien Blvd., Detroit, MI 48201, USA.
Pediatr Cardiol. 2008 May;29(3):659-62. doi: 10.1007/s00246-007-9040-z. Epub 2007 Aug 29.
Kearns-Sayre syndrome (KSS) is a rare mitochondrial disorder characterized by large-scale deletions of mitochondrial DNA. Neuromuscular and cardiac conduction systems are most commonly involved in these patients. Here, we discuss a 10-year-old patient with diabetes mellitus who presented in complete heart block leading to the diagnosis of KSS. The cardiovascular complications of this syndrome are reviewed and discussed.