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.
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