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    Malignant Hyperthermia

    Important
    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.

    Synonyms

    • Hyperpyrexia

    Disorder Subdivisions

    • None

    General Discussion

    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

    Resources

    Malignant Hyperthermia Association of the United States
    1 North Main St
    PO Box 1069
    Sherburne, NY 13460
    USA
    Tel: (607)674-7901
    Fax: (607)674-7910
    Email: info@mhaus.org
    Internet: http://www.mhaus.org

    North American Malignant Hyperthermia Registry
    Penn State University
    Dept. of Anesthesiology
    P.O. Box 850
    Hershey, PA 17033-0850
    USA
    Tel: (412)692-6390
    Fax: (412)692-8658
    Tel: (888)274-7899
    Email: bwb@pitt.edu
    Internet: http://www.mhreg.org

    NIH/National Institute of General Medical Sciences
    45 Center Drive MSC 6200
    Bethesda, MD 20892-6200
    Tel: (301)496-7301
    Fax: (301)402-0224
    Email: info@nigms.nih.gov
    Internet: http://www.nigms.nih.gov/

    Malignant Hyperthermia Investigation Unit
    Toronto General Hospital
    200 Elizabeth Street
    CCRW-2, Room ES3-403A
    Toronto
    Ontario, M5G 2C4
    Canada
    Tel: 4163403128
    Fax: 4163404960
    Email: info@mhacanada.org
    Internet: http://www.mhacanada.org

    Genetic and Rare Diseases (GARD) Information Center
    PO Box 8126
    Gaithersburg, MD 20898-8126
    Tel: (301)251-4925
    Fax: (301)251-4911
    Tel: (888)205-2311
    TDD: (888)205-3223
    Internet: http://rarediseases.info.nih.gov/GARD/

    Neuroleptic Malignant Syndrome Information Service, Inc.
    PO Box 1069
    11 East State Street
    Sherburne, NY 13460
    Tel: (607)674-7920
    Fax: (607)674-7910
    Email: info@nmsis.org
    Internet: http://www.nmsis.org

    For a Complete Report:

    This is an abstract of a report from the National Organization for Rare Disorders (NORD). A copy of the complete report can be downloaded free from the NORD website for registered users. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational therapies (if available), and references from medical literature. For a full-text version of this topic, go to www.rarediseases.org and click on Rare Disease Database under "Rare Disease Information".

    The information provided in this report is not intended for diagnostic purposes. It is provided for informational purposes only. NORD recommends that affected individuals seek the advice or counsel of their own personal physicians.

    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

    This disease entry is based upon medical information available through the date at the end of the topic. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder.

    For additional information and assistance about rare disorders, please contact the National Organization for Rare Disorders at P.O. Box 1968, Danbury, CT 06813-1968; phone (203) 744-0100; web site www.rarediseases.org or email orphan@rarediseases.org

    Last Updated: 6/27/2013
    Copyright 1984, 1988, 1989, 1990, 1992, 1999, 2005, 2009, 2013 National Organization for Rare Disorders, Inc.

    WebMD Medical Reference from the National Organization for Rare Disorders

    Last Updated: May 28, 2015
    This information is not intended to replace the advice of a doctor. Healthwise disclaims any liability for the decisions you make based on this information.

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