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  • Title: [Malignant hyperthermia as a complication of anesthesia: predisposition is hereditary].
    Author: Snoeck MM, Gielen MJ, Sengers RC, Padberg GW, Iles DE, Booij LH.
    Journal: Ned Tijdschr Geneeskd; 1997 Mar 29; 141(13):616-9. PubMed ID: 9190536.
    Abstract:
    The frequency of malignant hyperthermia in the Netherlands is about 1 in 200,000 anaesthesias. Five times a year, an anaesthetic procedure will be complicated by a malignant hyperthermic metabolic disturbance, which can cause death if treatment is not instituted rapidly, by the administration of dantrolene. Suxamethonium and all the anaesthetic vapours can trigger such a reaction. Malignant hyperthermia patients are healthy patients who have a mutation of the ryanodine receptor gene RYR. Predisposition to malignant hyperthermia is inherited as an autosomal dominant condition. So far a genetic malignant hyperthermia test is not available because of genetic heterogeneity. The in-vitro contracture test in skeletal muscle is currently used as a diagnostic test for malignant hyperthermia. Patients who are likely to be at risk based on a clinical grading score, and family members with at least a 25% chance of inheriting malignant hyperthermia, are eligible for this test.
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