National Organization for Rare Disorders, Inc.
It is possible that the main title of the report Malignant Hyperthermia is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.
Malignant hyperthermia (MH) is a dominantly inherited disorder of skeletal muscle that predisposes susceptible individuals to a life threatening adverse reaction (fulminant MH event) upon exposure to potent volatile anesthetics (halothane, isoflurane, sevoflurane, desflurane, etc.) and the skeletal muscle relaxant succinylcholine.
The anesthetic drugs trigger an uncontrolled calcium (Ca2+) release from the sarcoplasmic reticulum (SR) through the ryanodine receptor (RYR1) causing a rapid and sustained rise in myoplasmic Ca2+. The high intracellular Ca2+ activates Ca2+ pumps at the SR and the sarcolemma to reuptake calcium into SR or to transport it into the extracellular space respectively. The energetic cost to regain cellular Ca2+ control causes a need for ATP, which in turn produces heat. Muscle membrane integrity is compromised leading to hyperkalemia and rhabdomyolysis.1 If not treated promptly, by withdrawing the anesthetic and administering dantrolene, mortality can be greater than 70%.2 In some individuals, fulminant MH events can be induced by stress, exercise and high environmental temperatures in the absence of anesthetics.3 Pediatric patients may be at greater risk.4
Malignant Hyperthermia Association of the United States
1 North Main St
PO Box 1069
Sherburne, NY 13460
North American Malignant Hyperthermia Registry
Penn State University
Dept. of Anesthesiology
P.O. Box 850
Hershey, PA 17033-0850
NIH/National Institute of General Medical Sciences
45 Center Drive MSC 6200
Bethesda, MD 20892-6200
Malignant Hyperthermia Investigation Unit
Toronto General Hospital
200 Elizabeth Street
CCRW-2, Room ES3-403A
Ontario, M5G 2C4
Genetic and Rare Diseases (GARD) Information Center
PO Box 8126
Gaithersburg, MD 20898-8126
Neuroleptic Malignant Syndrome Information Service, Inc.
PO Box 1069
11 East State Street
Sherburne, NY 13460
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It is possible that the title of this topic is not the name you selected. Please check the Synonyms listing to find the alternate name(s) and Disorder Subdivision(s) covered by this report
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Last Updated: 6/27/2013
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