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2015年至2020年中国疑似恶性高热的临床特征:来自中国恶性高热急救援助小组的一项回顾性研究

Clinical Features of Suspected Malignant Hyperthermia in China from 2015 to 2020: A Retrospective Study from China Malignant Hyperthermia Emergency Assistance Group.

作者信息

Tan Lingcan, Teng Yi, Yu Hong, Liu Chunyuan, Xiao Kun, Yin Jin, Zuo Yunxia, Zhu Tao, Deng Xiaoqian

机构信息

Department of Anesthesiology, West China Hospital, Sichuan University, Chengdu, 610041, People's Republic of China.

Department of Anesthesiology, Chongqing Liangping District People's Hospital, Chongqing, 405200, People's Republic of China.

出版信息

J Multidiscip Healthc. 2022 Dec 29;15:3005-3013. doi: 10.2147/JMDH.S387208. eCollection 2022.

Abstract

PURPOSE

Malignant hyperthermia (MH) is a rare but fatal pharmacogenetic disorder, triggered by inhalational anesthetics or succinylcholine. Since the first nonprofit academic organization China Malignant Hyperthermia Emergency Assistance WeChat-based Group (CMHEA Group) was established in 2015, they have actively participated in the diagnosis and treatment of MH patients. Based on the CMHEA Group, the aim of the study was to retrospectively analyze the characteristics of suspected MH in China from 2015 to 2020.

METHODS

We conducted a retrospective analysis of the suspected MH patients from 2015 to 2020, for analyzing the current clinical diagnosis, treatment and prognosis of MH in China.

RESULTS

A total of 58 suspected MH cases occurred from 2015 to 2020, of these, 36 cases were collected with detailed data. The MH clinical grading score of 36 patients ranged from 33 to 73, with a median of 55. Abnormal hyperthermia and hypercarbia were the most common early signs of MH. Four patients were confirmed carrying six different potential MH-causative mutations. Of the total 58 cases, 14 patients (24.1%) received dantrolene and the whole mortality rate was 53.4%. Compared to the patients not receiving dantrolene treatment, the survival rate of patients receiving dantrolene treatment was significantly higher than that of patients not receiving dantrolene (78.6% vs 36.4%, p = 0.007).

CONCLUSION

The current main diagnostic methods of suspected MH in China are still clinical diagnosis. Hence, it is critical to keep dantrolene for immediate accessibility with the introduction of domestic dantrolene to China. The WeChat group model has played an important but limited role in quick diagnosis and treatment of MH.

摘要

目的

恶性高热(MH)是一种罕见但致命的药物遗传疾病,由吸入性麻醉剂或琥珀酰胆碱引发。自2015年首个非营利性学术组织中国恶性高热急救援助微信小组(CMHEA小组)成立以来,他们积极参与MH患者的诊断和治疗。基于CMHEA小组,本研究的目的是回顾性分析2015年至2020年中国疑似MH的特征。

方法

我们对2015年至2020年的疑似MH患者进行了回顾性分析,以分析中国目前MH的临床诊断、治疗及预后情况。

结果

2015年至2020年共发生58例疑似MH病例,其中36例收集到详细数据。36例患者的MH临床分级评分在33至73之间,中位数为55。异常高热和高碳酸血症是MH最常见的早期症状。4例患者被确认携带6种不同的潜在MH致病突变。在58例病例中,14例患者(24.1%)接受了丹曲林治疗,总死亡率为53.4%。与未接受丹曲林治疗的患者相比,接受丹曲林治疗的患者生存率显著高于未接受丹曲林治疗的患者(78.6%对36.4%,p = 0.007)。

结论

中国目前疑似MH的主要诊断方法仍是临床诊断。因此,随着国产丹曲林引入中国,确保丹曲林随时可及至关重要。微信小组模式在MH的快速诊断和治疗中发挥了重要但有限的作用。

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