• Abby S Hollander, Robert C Olney, Piers R Blackett, and Bess A Marshall.
• 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
• Pediatrics. 2003 Jun 1; 111 (6 Pt 1): 1447-52.

ObjectiveThis 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.DesignCase series.SettingPediatric intensive care units of 3 tertiary care facilities in the United States.PatientsSix adolescent males, 5/6 obese with acanthosis nigricans, 4/6 black.ResultsFour 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.ConclusionsThe 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.

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