Fatal malignant hyperthermia-like syndrome with rhabdomyolysis complicating the presentation of diabetes mellitus in adolescent males.
OBJECTIVE
This report describes a new fatal syndrome observed in adolescent males at the initial presentation of diabetes mellitus. The features include hyperglycemic hyperosmolar coma complicated by a malignant hyperthermia-like picture with fever, rhabdomyolysis, and severe cardiovascular instability.


METHODS
Case series.


METHODS
Pediatric intensive care units of 3 tertiary care facilities in the United States.


METHODS
Six adolescent males, 5/6 obese with acanthosis nigricans, 4/6 black.


RESULTS
Four of 6 patients died. Four of 6 patients did not have significant ketosis. Six of 6 patients had increased temperature after the administration of insulin.


CONCLUSIONS
The underlying etiology of this syndrome remains unclear. Possibilities include an underlying metabolic disorder such as a fatty acid oxidation defect, an unrecognized infection, exposure to an unknown toxin, or a genetic predisposition to malignant hyperthermia. Evaluation for all these possibilities and empiric treatment with dantrolene should be considered for this type of patient until this syndrome is better characterized.
Hollander
Abby S
AS
Division of Pediatric Endocrinology and Metabolism, Washington University School of Medicine and St Louis, Children's Hospital, St Louis, MO 63110, USA. hollander@kids.wustl.edu
Olney
Robert C
RC
Blackett
Piers R
PR
Marshall
Bess A
BA
D000293:Adolescent; D003620:Dantrolene / Q000627:therapeutic use; D003924:Diabetes Mellitus, Type 2 / Q000175:diagnosis* / Q000188:drug therapy / Q000628:therapy; D017809:Fatal Outcome; D006801:Humans; D006944:Hyperglycemic Hyperosmolar Nonketotic Coma / Q000175:diagnosis / Q000188:drug therapy / Q000628:therapy; D008297:Male; D008305:Malignant Hyperthermia / Q000175:diagnosis* / Q000188:drug therapy / Q000628:therapy; D012206:Rhabdomyolysis / Q000175:diagnosis* / Q000188:drug therapy / Q000628:therapy; D013577:Syndrome; D016896:Treatment Outcome
D003620:Dantrolene