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Malignant Hyperthermia

National Organization for Rare Disorders, Inc.

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.

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