PEDIATRICS Vol. 71 No. 1 January 1983, pp. 118-119
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Malignant Hyperthermia in a Child with Duchenne Muscular Dystrophy

HOWARD M. KELFER MD1, WILLIAM D. SINGER MD1, and ROBERT N. REYNOLDS MD1

1 Departments of Pediatrics (Neurology) and Anesthesiology, Boston Floating Hospital, New England Medical Center, Boston

Patients with Duchenne muscular dystrophy (DMD) are susceptible to numerous adverse intraoperative and postoperative side effects of anesthetic agents. These include: hyperthermia and hyperkalemia,1,2 systemic acidosis,3 cardiac abnormalities (tachycardia, arrhythmia, arrest),2-5 rhabdomyolysis,2-6 as well as death.2,5 These clinical and laboratory findings are similar to those associated with malignant hyperthermia (MH).7,8 Until this time no one has confirmed the association of MH, as reflected by these clinical phenomena, in a patient with DMD. We present a patient who manifested many features of MH immediately following confirmatory muscle biopsy for DMD under general anesthesia. In vitro response to testing of a muscle biopsy specimen was consistent with a diagnosis of malignant hyperthermia.




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