Malignant hyperthermia

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  1. Pharmacogenetic disorder of the skeletal muscle disorder caused by inhalational anesthetics; it is inherited disorder that is inherited autosomal dominant and is characterized by excessive release of Ca from the sarcoplasmic reticulum in skeletal muscle in response to general anesthetics and muscle relaxants (especially succinylcholine)
  2. incidence 1:15,000 episodes of general anesthesia;
  3. Disease associated with malignant hyperthermia
    1. Definite association :Central Core Disease
    2. Possible Association: Duchenne Muscular Dystrophy, King-Denborough Syndrome, Other myopathies, Becker Muscular Dystrophy, Periodic Paralysis, Myotonia Congenita

Schwartz-Jampel Syndrome, Fukuyama Type of Congenital Muscular Dystrophy, Mitochondrial Myopathy, Sarcoplasmic Reticulum Adenosine Triphosphate Deficiency Coincidental Association: Neuroleptic Malignant Syndrome, Sudden Infant Death Syndrome, Osteogenesis Imperfecta, Glycogen Storage Disease, Lymphomas, Heat Stroke

  1. Clinically: muscle rigidity, fever followed by rhabdomyolysis, myoglobinuric renal failure and autonomic instability, increased end tidal PCO2; mortality is 80% if untreated; muscle biopsies from patients with this disease show abnormally strong contracture when exposed to caffeine
  2. Treatment:

The volatile agent is stopped and 100% oxygen is given The patient is manually hyperventilated. A clean breathing system is used. Surgery should be aborted. Unconsciousness is maintained with an appropriate sedative-hypnotic Dantrolene 1mg/kg is given intravenously, and then 1 – 2.5mg/kg every 10 minutes until MH is under control (to a maximum dose of 10mg/kg). Measure core temperature. Intravenous fluids are given to maintain a steady urinary output, insulin and dextrose may be given to reverse hyperkalaemia. Haemodiafiltration may be necessary in resistant cases. Cardiac arrhythmias are treated as appropriate. The patient is cooled by tepid sponging, cooled fluids, cooling blanket, cool nasogastric lavage etc. as necessary. The patient should be admitted into a High Dependency Unit for postoperative care.

  • Note: dantrolene works by blocking the release of Ca by the sarcoplasmic reticulum
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