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To the Editor: Malignant hyperthermia (MH) is an autosomal dominant condition in which certain anesthetics trigger calcium dysregulation in skeletal muscle, resulting in a catastrophic, life-threatening hypermetabolic syndrome.¹ More than 50% of families with MH have mutations in the gene encoding the ryanodine receptor (RYR1).² In a porcine model of MH, nonanesthetic, stress-induced deaths have been reported in pigs homozygous for the Arg614Cys mutation in the RYR1 gene,³ but this phenomenon has not been reported in humans with MH mutations. To our knowledge, we report the first case of nonanesthetic, stress-induced hyperpyrexic death in an individual with a history of MH.

Report of a Case

At age 12 years, the patient underwent general anesthesia with sevoflurane for reduction of a humerus fracture. Fifteen minutes after induction, he was found to have an abrupt increase in end tidal CO₂ (>70 mm Hg), heart rate (150/min), and temperature (36.7°C to 39.4°C). Diaphoresis, but not . . . [Full Text of this Article]

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