国际麻醉学与复苏杂志   2023, Issue (6): 0-0
    
中国大陆与中国台湾地区恶性高热病例的对比分析
周阳, 李正迁, 曲音音, 杨禄坤, 韩永正, 张静, 韩彬, 王军, 郭向阳1()
1.北京大学第三医院
Comparison of malignant hyperthermia cases in Chinese mainland and China's Taiwan region
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摘要:

目的 对比分析中国大陆与中国台湾地区恶性高热(malignant hyperthermia, MH)病例的临床资料,探讨MH的早期诊断、治疗措施及效果,以及特效药丹曲林的使用情况。 方法 分别检索万方数据、中国生物医学文献服务系统、中国知网及台湾学术期刊在线数据库,收集1976年1月至2022年4月报道的所有MH病例,对比分析中国大陆与中国台湾地区MH病例的流行病学特征、发病特点、临床表现、治疗措施、丹曲林使用情况及预后转归等。 结果 中国大陆报道的MH病例共140例,中国台湾地区报道的MH病例共12例,均以青少年为主,男性多于女性,主要集中于口腔科手术(唇腭裂修补术)、骨科手术(先天性脊柱侧弯矫形术)等。MH病例的典型临床表现为体温快速升高、心率异常增快、肌紧张或肌肉强直、PETCO2异常升高等。所有病例以临床诊断为主,少数进行了肌肉活检或基因检测。治疗措施主要为降温、呼吸循环支持、利尿、纠酸、碱化尿液等。中国大陆在缺少丹曲林的情况下,少数病例联合应用血液净化治疗,MH患者总存活率为54.3%;中国台湾地区丹曲林应用率为83.3%,MH患者存活率为100%。 结论 与中国台湾地区患者相比,中国大陆患者丹曲林应用较少,MH患者存活率较低,但随着对MH相关知识的重视及了解增多、新版专家共识的发布以及国产注射用丹曲林钠的上市应用,中国大陆MH病例的救治水平有望得以提升。

关键词: 恶性高热; 中国大陆; 中国台湾地区; 丹曲林; 回顾性研究
Abstract:

Objective To comparatively analyze the clinical data of malignant hyperthermia (MH) cases in Chinese mainland and China's Taiwan region, in order to explore the early diagnosis, treatment measures and effectiveness of MH, and the use of dantrolene. Methods All MH cases reported from January 1976 to April 2022 were collected by searching Wanfang Data, SinoMed, CNKI, and Taiwan Scholar Journal Database. The epidemiological characteristics, the characteristics of onset, clinical manifestations, treatment measures, dantrolene usage and outcomes of MH cases in Chinese mainland and China's Taiwan region were analyzed. Results There were 140 MH cases reported in Chinese mainland and 12 cases in China′s Taiwan region, where most of the patients were adolescents, with more boys than girls, and the disease was often developed during oral surgery (cleft lip and palate repair), and orthopedic surgery (orthopedic surgery for congenital scoliosis). The typical clinical manifestations of MH patients included rapid increases in body temperature, abnormal increases in heart rate, muscular tension or muscle rigidity, and abnormal high end‑tidal carbon dioxide partial pressure (PETCO2). The patients were mainly clinically diagnosed, while muscle biopsy or genetic testing was performed in several cases. Treatment measures were mainly cooling, respiratory and circulatory support, diuresis, correction of acidosis, and alkalinization of the urine. In Chinese mainland, when dantrolene was not available, a few patients underwent blood purification treatment, and the overall survival rate of MH patients was 54.3%. The application rate of dantrolene in China′s Taiwan region was 83.3%, with a MH survival rate of 100%. Conclusions Compared with patients in China's Taiwan region, dantrolene is less commonly used for MH patients in Chinese mainland, with a lower survival rate. With the increasing attention and understanding of MH, the proposal of a new version of the consensus of China, and the application of domestic dantrolene sodium for injection use, the treatment effectiveness of MH cases in Chinese mainland is expected to be improved.

Key words: Malignant hyperthermia; Chinese mainland; China's Taiwan region; Dantrolene; Retrospective study