Nelson T E
Department of Anesthesiology, Wake Forest University School of Medicine, Winston-Salem, NC 27157-1009, USA.
Biosci Rep. 2001 Apr;21(2):169-79. doi: 10.1023/a:1013696124358.
Malignant hyperthermia (MH) is a pharmacogenetic disease which predisposes to the trigger of a life-threatening, hypermetabolic syndrome by potent inhaled anesthetics and by depolarizing skeletal muscle relaxants. Heat production in the anesthetized MH can be profound with 5-fold increases in oxygen consumption. The trigger anesthetics cause an abnormal, sustained rise in myoplasmic calcium levels. Possible mechanisms by which continuous release of calcium from skeletal muscle sarcoplasmic reticulum stores can produce the profound hyperthermia are discussed. Mutations in the gene coding the ryanodine receptor calcium release channel have been found in MH families and these mutant channels may be the functional basis for MH.
恶性高热（MH）是一种药物遗传学疾病，易因强效吸入麻醉剂和去极化骨骼肌松弛剂引发危及生命的高代谢综合征。麻醉状态下的恶性高热患者产热剧烈，耗氧量可增加5倍。引发麻醉的药物会导致肌浆钙水平异常持续升高。本文讨论了骨骼肌肌浆网钙储存持续释放钙从而产生严重高热的可能机制。在恶性高热家族中已发现编码兰尼碱受体钙释放通道的基因突变，这些突变通道可能是恶性高热的功能基础。
