Ellinas Herodotos, Albrecht Meredith A
Department of Anesthesiology, Medical College of Wisconsin, 9200 West Wisconsin Avenue, Milwaukee, WI 53226, USA.
Department of Anesthesiology, Medical College of Wisconsin, 9200 West Wisconsin Avenue, Milwaukee, WI 53226, USA.
Anesthesiol Clin. 2020 Mar;38(1):165-181. doi: 10.1016/j.anclin.2019.10.010.
Malignant hyperthermia (MH) is a rare but potentially lethal skeletal muscle disorder affecting calcium release channels. It is inherited in a mendelian autosomal dominant pattern with variable penetration. The initial clinical manifestations are of a hypermetabolic state with increased CO2 production, respiratory acidosis, increased temperature, and increased oxygen demands. If diagnosed late, MH progresses to multi-organ system failure and death. Current data suggest that mortality has improved to less than 5%. The gold standard for ruling out MH is the contracture test. Genetic testing is also available. MH-susceptible individuals should be clearly identified for safe administration of future anesthetics.
恶性高热（MH）是一种罕见但可能致命的骨骼肌疾病，影响钙释放通道。它以孟德尔常染色体显性模式遗传，具有可变的外显率。最初的临床表现为高代谢状态，伴有二氧化碳产生增加、呼吸性酸中毒、体温升高和氧需求增加。如果诊断延迟，MH会进展为多器官系统衰竭并导致死亡。目前的数据表明死亡率已降至5%以下。排除MH的金标准是挛缩试验。也可进行基因检测。应明确识别MH易感个体，以便安全给予未来的麻醉剂。
