title: Unexpected MH deaths without exposure to inhalation anesthetics in pediatric patients.
abstract: Children, later found to have ryanodine receptor type one variants (RYR1), died without exposure to inhalation anesthetics. Family members with the same RYR1 variants had contracture tests consistent with susceptibility to malignant hyperthermia or in vitro testing showed increased sensitivity to RYR1 agonist.
authors.affiliation: Department of Anesthesiology, North American Malignant Hyperthermia Registry of the Malignant Hyperthermia Association of the United States, University of Pittsburgh Medical Center, Pittsburgh, PA, USA. brandombw@anes.upmc.edu
authors.forename: Barbara W
authors.initials: BW
authors.lastname: Brandom
authors.forename: Sheila M
authors.initials: SM
authors.lastname: Muldoon
mesh_terms: D000768:Anesthesia, General; D018685:Anesthetics, Inhalation / Q000009:adverse effects*; D002648:Child; D002675:Child, Preschool; D005838:Genotype; D006221:Halothane; D006801:Humans; D008305:Malignant Hyperthermia / Q000235:genetics / Q000401:mortality*; D009467:Neuromuscular Depolarizing Agents / Q000009:adverse effects; D012206:Rhabdomyolysis / Q000139:chemically induced; D019837:Ryanodine Receptor Calcium Release Channel / Q000235:genetics; D013390:Succinylcholine / Q000009:adverse effects
chemical_list: D018685:Anesthetics, Inhalation; D009467:Neuromuscular Depolarizing Agents; D019837:Ryanodine Receptor Calcium Release Channel; D013390:Succinylcholine; D006221:Halothane
keywords: anesthesiology; molecular medicine; muscular diseases; pediatrics; premature mortality; ryanodine receptor calcium release channel