Department of Cardiology, Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Charité University Medicine, Campus Virchow Klinikum, Augustenburger Platz 1, 13353, Berlin, Germany.
Department of Anesthesiology, The Second Clinical Medical College, Yangtze University, Jingzhou, 434020, China.
BMC Anesthesiol. 2021 Apr 16;21(1):119. doi: 10.1186/s12871-021-01328-3.
Malignant hyperthermia is a rare but life-threatening pharmacogenetic muscle disorder characterized by abnormal hypermetabolic reactions and commonly triggered in susceptible individuals by volatile anesthetics or succinylcholine, or both. Unfortunately, the specific medicine dantrolene is not readily available in many countries including China. The aim of this study was to find the characteristics of malignant hyperthermia under the situation that dantrolene is not readily available.
The cases of malignant hyperthermia reported on the most commonly used databases in China from 1985 to 2020 were analyzed. The inclusion criteria were the MH episodes only related to anesthesia. The exclusion criteria were dubious MH episodes only caused by Ketamine administration or MH episodes irrelevant to anesthesia. Independent samples t-test and Pearson's chi-squared test were applied to assess the difference between the survived and death cases.
Ninety-two cases of malignant hyperthermia reported on the most commonly used databases in China from 1985 to 2020 were analyzed. Median (IQR [range]) age was 18.5 (11.8-37.0 [0-70.0]) years. Compared with the survived cases, the death cases had higher maximum end-tidal partial pressure of CO (P = 0.033), the maximum arterial partial pressure of CO2 (P = 0.006), temperature first measured when the patient was first discovered abnormal (P = 0.012), and maximum temperature (P < 0.001). Besides, the death cases had less minimum pH (P < 0.001) and higher potassium (P < 0.001) and were more likely to have coagulation disorders (p = 0.018). Concerning treatment, cases used furosemide (P = 0.024), mannitol (P = 0.029), blood purification treatment (P = 0.017) had the advantage on the outcome. Creatine phosphokinase, myoglobin, and MB isoenzyme of creatine phosphokinase differed greatly among cases during the first week. 43 (46.7%) cases had congenital diseases. 12 (13.0%) cases were reported with abnormal laboratory test results or abnormal signs that are possibly relevant before anesthesia.
In countries that dantrolene is not readily available, early warning, diagnosis, and prompt effective therapies are crucial for MH patients to survive.
恶性高热是一种罕见但危及生命的遗传性肌肉疾病,其特征为异常的高代谢反应,通常在易感个体中由挥发性麻醉剂或琥珀酰胆碱触发,或两者同时触发。不幸的是,包括中国在内的许多国家都无法轻易获得专门用于治疗恶性高热的药物丹曲林钠。本研究旨在寻找在无法轻易获得丹曲林钠的情况下恶性高热的特征。
对中国 1985 年至 2020 年最常用数据库中报告的恶性高热病例进行分析。纳入标准为仅与麻醉相关的 MH 发作。排除标准为仅由氯胺酮给药引起的可疑 MH 发作或与麻醉无关的 MH 发作。采用独立样本 t 检验和 Pearson 卡方检验评估存活组与死亡组之间的差异。
对中国 1985 年至 2020 年最常用数据库中报告的 92 例恶性高热病例进行分析。中位(IQR [范围])年龄为 18.5(11.8-37.0 [0-70.0])岁。与存活组相比,死亡组的呼气末二氧化碳分压最高(P=0.033)、动脉血二氧化碳分压最高(P=0.006)、患者首次发现异常时的体温最高(P=0.012)、最高体温(P<0.001)。此外,死亡组的最小 pH 值较低(P<0.001)、钾较高(P<0.001),更可能出现凝血功能障碍(p=0.018)。关于治疗,使用速尿(P=0.024)、甘露醇(P=0.029)、血液净化治疗(P=0.017)的病例在预后方面具有优势。肌酸磷酸激酶、肌红蛋白和肌酸磷酸激酶同工酶在发病后第一周差异较大。43 例(46.7%)有先天性疾病。12 例(13.0%)在麻醉前有异常实验室检查结果或可能相关的异常体征的报告。
在无法轻易获得丹曲林钠的国家,早期预警、诊断和及时有效的治疗对恶性高热患者的存活至关重要。