The current status of malignant hyperthermia

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

作者机构：Department of Anesthesiologythe Fifth Affiliated Hospital of Sun Yat-sen UniversityZhuhaiGuangdong 519000China Department of Anesthesiology and Pain MedicineUniversity of California Davis HealthSacramentoCA 95817USA Department of Pathology and Laboratory MedicineCollege of MedicineUniversity of KentuckyLexingtonKY 40506USA Department of Physiology and Membrane BiologyUniversity of California Davis HealthSacramentoCA 95817USA 

出 版 物：《The Journal of Biomedical Research》 (生物医学研究杂志（英文版）)

年 卷 期：2020年第34卷第2期

页      面：75-85页

核心收录：

学科分类：0710[理学-生物学] 1002[医学-临床医学] 100217[医学-麻醉学] 10[医学] 

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

主　　题：malignant hyperthermia general anesthesia dantrolene ryanodine receptor 

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.