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恶性高热的非典型症状:RYR1基因中一种罕见的致病突变。

Atypical symptoms of malignant hyperthermia: A rare causative mutation in the RYR1 gene.

作者信息

Wang Qiao Ling, Fang Yu, Jin Shuo Guo, Liang Jing Tao, Ren Yi Feng

机构信息

Department of Ministry of Science, Hospital of Chengdu University of Traditional Chinese Medicine, Chengdu, Sichuan, 610072, China.

出版信息

Open Med (Wars). 2022 Feb 2;17(1):239-244. doi: 10.1515/med-2021-0396. eCollection 2022.

Abstract

Malignant hyperthermia (MH) is an autosomal dominant genetic condition of the skeletal muscle triggered by inhaled general anesthetic agents or succinylcholine and associated with a hypermetabolic state and skeletal muscle rigidity. Tachycardia, increased carbon dioxide production, hypercarbia, hyperthermia, acidosis, hyperkalemia, cardiac arrhythmias, muscle rigidity, and rhabdomyolysis are common symptoms of MH. As the progression of the syndrome could be rapid or less evident, even experienced physicians have difficulty in diagnosing MH, which can lead to delays in treatment and increased mortality. We report a rare case of a 36-year-old man, who underwent open reduction and internal fixation of the left clavicle after inhaled anesthetics. The patient developed dyspnea, hypotension, unremitting hyperthermia, tachycardia, and elevated serum myoglobin, and finally died of pyemia and disseminated intravascular coagulation. We reviewed the process of disease development, summarized the steps of diagnosis, and improved genetic testing. Exome sequencing revealed a new mutation c.8519G>A (p.arg2840 GLN) in the RYR1 gene that could be associated with MH. The gene mutation was also found in his daughter's genetic test. This case emphasized the importance of the awareness of MH and its atypical clinical symptoms. The presence of dyspnea, hypotension, unremitting hyperthermia, tachycardia, and raised myoglobin in serum might further strengthen the clinical diagnosis of suspected MH.

摘要

恶性高热(MH)是一种常染色体显性遗传的骨骼肌疾病,由吸入性全身麻醉剂或琥珀酰胆碱引发,与高代谢状态和骨骼肌强直相关。心动过速、二氧化碳生成增加、高碳酸血症、高热、酸中毒、高钾血症、心律失常、肌肉强直和横纹肌溶解是MH的常见症状。由于该综合征的进展可能迅速或不太明显,即使是经验丰富的医生也难以诊断MH,这可能导致治疗延误和死亡率增加。我们报告了一例罕见病例,一名36岁男性在吸入麻醉剂后接受了左锁骨切开复位内固定术。患者出现呼吸困难、低血压、持续高热、心动过速和血清肌红蛋白升高,最终死于脓血症和弥散性血管内凝血。我们回顾了疾病发展过程,总结了诊断步骤,并改进了基因检测。外显子组测序在RYR1基因中发现了一个新的突变c.8519G>A(p.arg2840 GLN),该突变可能与MH相关。在其女儿的基因检测中也发现了该基因突变。本病例强调了认识MH及其非典型临床症状的重要性。出现呼吸困难、低血压、持续高热、心动过速和血清肌红蛋白升高可能会进一步加强对疑似MH的临床诊断。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ccb0/8812711/58678f0881a5/j_med-2021-0396-fig001.jpg

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