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October 1989

Hyperglycemic Acidosis With Mortality in Kearns-Sayre Syndrome

Author Affiliations

Department of Pediatrics Division of Pediatric Cardiology University of Miami PO Box 016960 (R-76) Miami, FL 33101

Am J Dis Child. 1989;143(10):1135-1136. doi:10.1001/archpedi.1989.02150220023010

Sir.—Kearns-Sayre syndrome (KSS) is a rare multisystemic disorder, usually described as the clinical triad of chronic progressive ophthalmoplegia, retinitis pigmentosa, and cardiomyopathy.1-3 There is a pathologic degeneration of the central nervous system (ie, spongiform degeneration of the brain) and skeletal and pancreatic abnormalities.4,5 Sudden death in patients with KSS has been primarily attributed to the cardiac conduction defects and cardiomyopathy.3

Recently, the association of chemical diabetes and hyperglycemic acidotic coma has been documented in the ophthalmology literature.6 We describe three patients with KSS who we believed died secondary to complications related to KSS and hyperglycemic coma. We would like to bring this potentially devastating problem to the attention of the pediatrician.

Patient Reports.—Patient 1.—A 14-year-old boy was first seen at 10 years of age with bilateral ptosis, proximal skeletal myopathy, and external ophthalmoplegia. At that time, the results of his cardiac examination were

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