The current status of malignant hyperthermia  

作　　者：Lukun Yang Timothy Tautz Shulin Zhang Alla Fomina Hong Liu 

机构地区：[1]Department of Anesthesiology,the Fifth Affiliated Hospital of Sun Yat-sen University,Zhuhai,Guangdong 519000,China [2]Department of Anesthesiology and Pain Medicine,University of California Davis Health,Sacramento,CA 95817,USA [3]Department of Pathology and Laboratory Medicine,College of Medicine,University of Kentucky,Lexington,KY 40506,USA [4]Department of Physiology and Membrane Biology,University of California Davis Health,Sacramento,CA 95817,USA

出　　处：《The Journal of Biomedical Research》2020年第2期75-85,共11页生物医学研究杂志（英文版）

基　　金：supported by the Department of Anesthesiology and Pain Medicine and NIH grant(No.UL1 TR001860)of the University of California Davis Health.

摘　　要：Malignant hyperthermia(MH) is a rare and life-threatening pharmacogenetic disorder triggered by volatile anesthetics, the depolarizing muscle relaxant succinylcholine, and rarely by strenuous exercise or environmental heat. The exact prevalence of MH is unknown, and it varies from 1:16 000 in Denmark to 1:100 000 in New York State. The underlying mechanism of MH is excessive calcium release from the sarcoplasmic reticulum(SR),leading to uncontrolled skeletal muscle hyper-metabolism. Genetic mutations in ryanodine receptor type 1(RYR1)and CACNA1 S have been identified in approximately 50% to 86% and 1% of MH-susceptible(MHS) individuals,respectively. Classic clinical symptoms of MH include hypercarbia, sinus tachycardia, masseter spasm,hyperthermia, acidosis, muscle rigidity, hyperkalemia, myoglobinuria, and etc. There are two types of testing for MH: a genetic test and a contracture test. Contracture testing is still being considered as the gold standard for MH diagnosis. Dantrolene is the only available drug approved for the treatment of MH through suppressing the calcium release from SR. Since clinical symptoms of MH are highly variable, it can be difficult to establish a diagnosis of MH. Nevertheless, prompt diagnosis and treatments are crucial to avoid a fatal outcome. Therefore, it is very important for anesthesiologists to raise awareness and understand the characteristics of MH. This review summarizes epidemiology, clinical symptoms, diagnosis and treatments of MH and any new developments.

关 键 词：malignant hyperthermia general anesthesia DANTROLENE ryanodine receptor 

分 类 号：R614[医药卫生—麻醉学]