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Wednesday, January 7, 2009
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Malignant hyperthermiaDefinitionMalignant hyperthermia is an inherited disease that causes a rapid rise in body temperature (fever) and severe muscle contractions when the affected person receives general anesthesia. This condition is not the same as hyperthermia due to medical emergencies such as heat stroke. Alternative NamesHyperthermia - malignant; Hyperpyrexia - malignantCausesMalignant hyperthermia is an autosomal dominant trait, meaning it requires only one parent carrying the disease for a child to inherit the condition. It may be associated with muscular diseases such as multiminicore myopathy and central core disease. One form of malignant hyperthermia is caused by a defect in the ryanodine receptor (RYR1) gene SymptomsMalignant hyperthermia is often noted for the first time after a patient is given anesthesia during a surgical procedure. Symptoms include:
Exams and TestsThere may be a family history of malignant hyperthermia or unexplained death during anesthesia. Tests that may be done include:
TreatmentFor an episode of malignant hyperthermia, a cooling blanket can help reduce fever. The use of a drug called dantrolene during episodes of malignant hyperthermia has greatly reduced the number of deaths. Fluids given by IV and mouth, as well as certain medications, are essential for maintaining kidney function during an acute episode. Support GroupsMalignant Hyperthermia Association of the United States - www.mhaus.org Outlook (Prognosis)Repeated episodes or untreated episodes can cause kidney failure. Untreated episodes can be fatal. Possible Complications
When to Contact a Medical ProfessionalIf you know that you or a member of your family has had problems with general anesthesia, or if you know you have a family history of malignant hyperthermia, be sure to notify both the surgeon and the anesthesiologist before having any surgery. PreventionIf you or anyone in your family has malignant hyperthermia, it is very important to tell your doctor, especially before having surgery with general anesthesia. The use of appropriate anesthetic medication can prevent the complications of malignant hyperthermia during surgery. It is important to avoid stimulant drugs such as cocaine, amphetamine (speed) and ecstasy as they may produce malignant hyperthermia-like problems in susceptible individuals. Genetic counseling is recommended for anyone with a family history of myopathy, muscular dystrophy, or malignant hyperthermia. Vicario S. Heat illness. In: Marx J, ed. Rosen’s Emergency Medicine: Concepts and Clinical Practice. 6th ed. St. Louis, Mo: Mosby; 2006:chap 139.
Review Date: 7/23/2008 Reviewed By: A.D.A.M. Editorial Team: David Zieve, MD, MHA, Greg Juhn, MTPW, David R. Eltz. Previously reviewed by Chad Haldeman-Englert, MD, Division of Human Genetics, Children's Hospitalof Philadelphia, Philadelphia, PA. Review provided by VeriMed HealthcareNetwork (7/25/2007). The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed physician should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. Copyright A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited. |