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Malignant hyperthermia
Malignant hyperthermia (MH) or malignant hyperpyrexia is a rare life-threatening condition
that is usually triggered by exposure to certain drugs used for general anesthesia —
specifically the volatile anesthetic agents and succinylcholine, a neuromuscular blocking
agent. In susceptible individuals, these drugs can induce a drastic and uncontrolled increase
in oxidative metabolism in skeletal muscle, which overwhelms the body's capacity to supply
oxygen, remove carbon dioxide, and regulate body temperature, eventually leading
to circulatory collapse and death if not immediately treated.
Susceptibility to MH is often inherited as an autosomal dominant disorder, for which there
are at least 6 genetic loci of interest, most prominently the ryanodine receptor gene (RYR1).
MH susceptibility is phenotypically and genetically related to central core disease (CCD), an
autosomal dominant disorder characterized both by MH signs and myopathy. MH is usually
revealed upon or shortly after exposure to certain general anesthetic agents. There is no
simple, straightforward test to diagnose the condition. Treatment with dantrolene and other
drugs is usually initiated when MH is strongly suspected. Dantrolene and the avoidance of
triggering agents in susceptible people have markedly reduced the mortality from this
condition.
Causes
Malignant hyperthermia is inherited. Only 1 parent has to carry the disease for a child to
inherit the condition. It may occur with some other inherited muscle diseases, such as
multiminicore myopathy and central core disease.
Bleeding
Blood clotting studies (PT, or prothombin time; PTT, or partial thrombloplastin time)
Muscle biopsy
Urine myoglobin (muscle protein)
Treatment
During an episode of malignant hyperthermia, a medicine called dantrolene is often given.
Wrapping the person in a cooling blanket can help reduce fever and the risk of serious
complications. To preserve kidney function during an episode, the person may receive fluids
through a vein. Repeated or untreated episodes can cause kidney failure. Untreated episodes
can be fatal.
Amputation
Swelling of the hands and feet and problems with blood flow and nerve function
(compartment syndrome)
Death
Kidney failure
Prevention
Tell your health care provider if you or anyone in your family has malignant hyperthermia,
especially before having surgery with general anesthesia. Avoid stimulant drugs such as
cocaine, amphetamine (speed), and ecstasy. These drugs may cause problems similar to
malignant hyperthermia in people who are prone to this condition. Genetic counselling is
recommended for anyone with a family history of myopathy, muscular dystrophy, or
malignant hyperthermia.