Kearns-Sayre syndrome presenting as complete heart block.
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.
Chawla
Sanjay
S
Pediatrics, Children's Hospital of Michigan, 3901 Beaubien Blvd., Detroit, MI 48201, USA.
Coku
Jorida
J
Forbes
Thomas
T
Kannan
Sujatha
S
D001706:Biopsy; D002304:Cardiac Pacing, Artificial; D002648:Child; D003922:Diabetes Mellitus, Type 1 / Q000150:complications; D003937:Diagnosis, Differential; D004562:Electrocardiography; D005260:Female; D006327:Heart Block / Q000175:diagnosis* / Q000209:etiology / Q000628:therapy; D006801:Humans; D028361:Mitochondrial Diseases / Q000150:complications / Q000175:diagnosis*; D018482:Muscle, Skeletal / Q000473:pathology