Fatal metabolic acidosis, hyperglycemia, and coma after steroid therapy for Kearns-Sayre syndrome.
Two adolescent boys with Kearns-Sayre syndrome (progressive external ophthalmoplegia, heart block, elevated CSF protein, and ragged-red muscle fibers) developed lethargy, increasing somnolence, polydipsia, polyphagia, and polyuria after a brief course of steroid therapy. Both had hyperglycemia and acidosis. Nonketotic, lactic acidosis was present in one and ketosis in the other. Severe respiratory failure developed, and both patients died. Postmortem revealed fatty infiltration of the pancreas in addition to a diffuse spongiform encephalopathy.
R G
RG
Curless
J
J
Flynn
B
B
Bachynski
J B
JB
Gregorios
P
P
Benke
R
R
Cullen
D000138:Acidosis / Q000139:chemically induced*; D002648:Child; D003128:Coma / Q000139:chemically induced*; D003643:Death; D006801:Humans; D006943:Hyperglycemia / Q000139:chemically induced*; D007625:Kearns-Sayre Syndrome / Q000188:drug therapy*; D008297:Male; D009886:Ophthalmoplegia / Q000188:drug therapy*; D011241:Prednisone / Q000009:adverse effects* / Q000627:therapeutic use
D011241:Prednisone