Abstract:
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. DESIGN:Case series. SETTING:Pediatric intensive care units of 3 tertiary care facilities in the United States. PATIENTS: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.
journal_name
Pediatricsjournal_title
Pediatricsauthors
Hollander AS,Olney RC,Blackett PR,Marshall BAdoi
10.1542/peds.111.6.1447subject
Has Abstractpub_date
2003-06-01 00:00:00pages
1447-52issue
6 Pt 1eissn
0031-4005issn
1098-4275journal_volume
111pub_type
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