Approach to management of malignant hyperthermia-like syndrome in pediatric diabetes mellitus.
BACKGROUND
Hyperglycemic hyperosmolar nonketotic syndrome (HHNS) is usually associated with type 2 diabetes mellitus and is rare in children. However, a fatal malignant hyperthermia-like syndrome (MHLS) with rhabdomyolysis associated with new-onset diabetes mellitus and HHNS in adolescents has been described.


METHODS
Case series.


RESULTS
A 16-yr-old obese male (case A) and a 10-yr-old mid-pubertal nonobese female (case B) presented within a 6-month period with emesis, altered mental status, blood glucose >1600 mg/dL, and laboratory evidence of rhabdomyolysis. Case A developed fever after initiation of insulin therapy, along with refractory hypotension and multiorgan failure. He died 14 hrs after admission. Case B developed fever before insulin therapy, was treated with dantrolene, and made a full recovery. Metabolic workup showed evidence of short-chain acyl-CoA dehydrogenase (SCAD) deficiency.


CONCLUSIONS
We report two cases of malignant hyperthermia-like syndrome associated with HHNS in adolescents. Their respective fluid management and clinical courses are described. Dantrolene therapy should be initiated immediately after this syndrome is recognized. We believe it is unlikely insulin is the sole trigger for MHLS. Case B is unique in that there was evidence of SCAD deficiency, a metabolic defect that we propose could lead to MHLS. We recommend that all patients with HHNS and MHLS be evaluated for an underlying metabolic disorder.
Kilbane
Brendan J
BJ
Department of Pediatrics, Cincinnati Children's Hospital Medical Center and University of Cincinnati, Cincinnati, OH, USA.
Mehta
Sanjeev
S
Backeljauw
Philippe F
PF
Shanley
Thomas P
TP
Crimmins
Nancy A
NA
D000293:Adolescent; D001741:Black or African American; D044926:Butyryl-CoA Dehydrogenase / Q000172:deficiency; D002648:Child; D003620:Dantrolene / Q000627:therapeutic use; D003924:Diabetes Mellitus, Type 2 / Q000150:complications*; D017809:Fatal Outcome; D005260:Female; D005440:Fluid Therapy; D006801:Humans; D006944:Hyperglycemic Hyperosmolar Nonketotic Coma / Q000209:etiology / Q000628:therapy*; D008297:Male; D008305:Malignant Hyperthermia / Q000209:etiology* / Q000628:therapy*; D009765:Obesity / Q000150:complications; D012206:Rhabdomyolysis / Q000209:etiology; D013577:Syndrome; D016896:Treatment Outcome
D044926:Butyryl-CoA Dehydrogenase; D003620:Dantrolene