Malignant hyperthermia: pathophysiology, clinical presentation, and treatment.
Malignant hyperthermia (MH) was first described as an inherited highly lethal disorder in 1960. There has since been significant progress in the clinical management, identification of MH susceptible (MHS) persons, and understanding of the underlying pathophysiology. When patients are known to be MHS prior to surgery, an MH episode can easily be avoided by the use of safe nontriggering anesthetic agents. Current MH mortality is <10%, but many experts believe this can be significantly reduced by improved MH preparedness. MH is triggered in humans by an MH triggering anesthetic agent, which causes the release of calcium from the sarcoplastic reticulum of the skeletal muscle cell at an uncontrolled rate resulting in a hypermetabolic state. Recent molecular genetic studies have shown that MH is related to an abnormal ryanodine receptor that controls the release of calcium from the sarcoplastic reticulum. This article reviews the current understanding of the pathophysiology, diagnosis, clinical presentation, and treatment of MH.
McCarthy
E Jane
EJ
University of Maryland, School of Nursing, Baltimore, USA. mcca003@son.umaryland.edu
D001706:Biopsy; D002110:Caffeine / Q000493:pharmacokinetics; D004198:Disease Susceptibility; D005819:Genetic Markers; D006221:Halothane / Q000493:pharmacokinetics; D006801:Humans; D008305:Malignant Hyperthermia / Q000175:diagnosis / Q000188:drug therapy / Q000235:genetics / Q000503:physiopathology; D009119:Muscle Contraction / Q000187:drug effects; D018482:Muscle, Skeletal / Q000187:drug effects
D005819:Genetic Markers; D002110:Caffeine; D006221:Halothane