[Physiologic, biochemical and genetic aspects of malignant hyperthermia]

Malignant hyperthermic syndrome (MHS) is based on a metabolic dysfunction of the skeletal muscle. It is characterized by an elevation of muscle metabolism and rigidity, accompanied by an increase of arterial pCO2, lactate and potassium plasma concentration, and body temperature. In sensitive individuals, MHS can be evoked pharmacologically. To identify substances that evoke this syndrome or those useful for its therapy, MHS is modelled in pigs. The primary defect attributed to MHS is the impairment of sarcoplasmic calcium homeostasis based on a dysfunction of one of calcium ion channels. In some cases, genetic mapping has shown that MHS is related to changes in 19 chromosome (in humans). Abnormal function of the ion channel is probably not sufficient for the expression of MHS. The syndrome manifests only when several modifying factors coincide.