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Potentially life-threatening emergency
Malignant hyperthermia
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Potentially life-threatening emergency

Malignant hyperthermia

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Contributors: Joon B. Kim BS, Michael W. Winter MD, Jamie Adams MD, Abhijeet Waghray MD
Other Resources UpToDate PubMed

Synopsis

Malignant hyperthermia (MH) is a hypermetabolic response to certain inhaled anesthetic drugs with sudden, intense temperature elevation (hyperthermia) and muscle rigidity. Sustained contraction of skeletal muscles generates more heat than the body can dissipate. Other signs include hyperkalemia, tachycardia, tachypnea, acidosis, elevated carbon dioxide, increased oxygen consumption, and rhabdomyolysis. MH crisis constitutes a medical emergency. Incidence in the general population is estimated at 1:100 000 anesthetics administered.

Monitoring core temperature during surgical use of inhaled anesthetics can identify early MH.

MH crisis can be treated by immediately stopping the triggering anesthetic, administering dantrolene, treating hyperkalemia (hyperventilating), and lowering body temperature. Failure to treat MH crisis immediately leads to severe organ damage and death.

A predisposition to MH crisis is associated with an autosomal dominant pattern of inheritance caused by mutations in RYR1 and CACNA1S genes. Genetic skeletal muscle receptor abnormalities allow excessive calcium accumulation in response to anesthetic agents. Central core disease, King-Denborough syndrome, and central nuclear myopathy are also conditions that predispose patients to MH crisis.

For more information, see OMIM.

Codes

ICD10CM:
T88.3XXA – Malignant hyperthermia due to anesthesia, initial encounter

SNOMEDCT:
405501007 – Malignant Hyperthermia

Look For

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

Best Tests

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Management Pearls

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Therapy

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Drug Reaction Data

Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.

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References

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Last Reviewed: 06/20/2019
Last Updated: 06/20/2019
Copyright © 2019 VisualDx®. All rights reserved.
Potentially life-threatening emergency
Malignant hyperthermia
Print 1 Images
Malignant hyperthermia : Hyperkalemia, Myoglobinuria, Rigidity, Tachycardia, Creatine kinase elevated, Diaphoresis, CO2 elevated, Tachypnea, Hyperthermia
Copyright © 2019 VisualDx®. All rights reserved.