Hyperglycemic acidotic coma and death in Kearns-Sayre syndrome

Ophthalmology. 1986 Mar;93(3):391-6. doi: 10.1016/s0161-6420(86)33744-8.


This paper presents the clinical and metabolic findings in two young boys with long-standing Kearns-Sayre syndrome. Following short exposure to oral prednisone, both boys developed lethargy, increasing somnolence, polydipsia, polyphagia, and polyuria. Both presented in the emergency room with profound coma, hypotension, severe hyperglycemia, and acidosis. Nonketotic lactic acidosis was present in one and ketosis without a known serum lactate level was present in the other. Respiratory failure rapidly ensued and both patients expired in spite of efforts at resuscitation. We believe these two cases represent a newly described and catastrophic metabolic-endocrine failure in the Kearns-Sayre syndrome.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Acidosis / chemically induced
  • Acidosis / etiology
  • Administration, Oral
  • Child
  • Child, Preschool
  • Coma / chemically induced
  • Coma / etiology
  • Electrocardiography
  • Humans
  • Hyperglycemia / chemically induced
  • Hyperglycemia / etiology
  • Kearns-Sayre Syndrome / complications
  • Kearns-Sayre Syndrome / drug therapy*
  • Kearns-Sayre Syndrome / pathology
  • Kearns-Sayre Syndrome / physiopathology
  • Male
  • Ophthalmoplegia / drug therapy*
  • Prednisone / adverse effects


  • Prednisone