Johnson C, Edleman K J
Department of Anesthesia, Hutzel Hospital, Wayne State University, Detroit, MI 48201.
J Perinatol. 1992 Mar;12(1):61-71.
Malignant hyperthermia (MH) is a rare genetic myopathy that was first described as a fatal complication of general anesthesia in 1960. It is estimated to affect approximately 1 in 15,000 pediatric patients and 1 in 40,000 adult middle-aged patients. The mode of transmission is genetic: the severest form is autosomal dominant, and the less severe, autosomal recessive. Thus, both men and women can have MH, although there is a slightly higher incidence in the male pediatric population. Malignant hyperthermia is usually triggered by halogenated anesthetic agents with or without depolarizing muscle relaxants. The classic diagnostic triad consists of skeletal muscle rigidity, metabolic acidosis, and elevated body temperature. The definitive diagnosis is suspected susceptible individuals is revealed by exposing an intact muscle fiber to caffeine and halothane in varying concentrations. An abnormal contracture response is hypothesized to be the result of an increase in the release of calcium ion from the sarcoplasmic reticulum in response to neuronal stimulation leading to a hypermetabolic state. The mainstay of treatment is dantrolene, given either prophylactically in susceptible patients or immediately whenever a malignant hyperthermic episode is suspected.
恶性高热（MH）是一种罕见的遗传性肌病，于1960年首次被描述为全身麻醉的致命并发症。据估计，每15000名儿科患者中约有1人受其影响，每40000名成年中年患者中约有1人受其影响。其遗传方式为：最严重的形式是常染色体显性遗传，较轻的是常染色体隐性遗传。因此，男性和女性都可能患恶性高热，尽管在男性儿科人群中的发病率略高。恶性高热通常由卤化麻醉剂引发，无论是否使用去极化肌松药。典型的诊断三联征包括骨骼肌强直、代谢性酸中毒和体温升高。对于疑似易感个体的确诊，是通过将完整的肌纤维暴露于不同浓度的咖啡因和氟烷来实现的。异常挛缩反应被认为是由于神经元刺激导致肌浆网中钙离子释放增加，从而引发高代谢状态的结果。治疗的主要药物是丹曲林，对于易感患者可预防性给药，或在怀疑发生恶性高热发作时立即给药。
