恶性高热的临床和形态学特征：一例来自实践的罕见病例

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现代全身麻醉最严重的并发症之一是恶性高热，表型表现为高碳酸血症、窦性心动过速、骨骼肌代谢亢进和横纹肌溶解或在使用吸入麻醉剂和非去极化肌肉松弛剂进行全身麻醉后。恶性高热症最常见的初始迹象是呼气结束时二氧化碳浓度突然上升。这种药物遗传性疾病的非典型形式比暴发性形式更常见。在俄罗斯恶性高热症的问题目前仍未得到解决。

作者描述了一例19岁女孩的恶性高热，她在使用西维兰麻醉下接受了鼻腔呼吸障碍的手术。由于出现了临床诊断的恶性高热，患者在离开麻醉状态1小时25分钟后死亡。法医尸检证实了这一诊断。使用观察性和选择性的组织学染色来描述骨骼肌的形态学变化。

所提出的观察值是由于雷电引起的恶性高热症的罕见性和伴随这些病理变体的高死亡率。这个专家案例展示了一个合格的、全面的组织学检查，包括使用基因测试，可以正确制定诊断，并在必要时对执法当局的问题作出合理的回应。

作者简介

Nikolai M. Anichkov

Saint-Petersburg State Pediatric Medical University

Email: anichkov@bk.ru
ORCID iD: 0000-0003-1834-7881
SPIN 代码: 5222-7003

MD, Dr. Sci. (Med.), Professor

俄罗斯联邦, 2, Litovskay street, Saint-Peterburg, 194100

Elena Yu. Kalinina

Saint-Petersburg State Pediatric Medical University

Email: drkalinina@yandex.ru
ORCID iD: 0000-0001-7077-3584
SPIN 代码: 1176-5739

MD, Cand. Sci. (Med.)

俄罗斯联邦, 2, Litovskay street, Saint-Peterburg, 194100

Zlata V. Davydova

Saint-Petersburg State Pediatric Medical University

编辑信件的主要联系方式.
Email: zlata.davydova@rambler.ru
ORCID iD: 0000-0002-6673-8230
SPIN 代码: 7016-7086

MD, Cand. Sci. (Med.)

俄罗斯联邦, 2, Litovskay street, Saint-Peterburg, 194100

Ekaterina V. Shcherbakova

Bureau of Forensic Medical Examination of the Leningrad region

Email: maestrovody@mail.ru
ORCID iD: 0000-0002-3818-1535
SPIN 代码: 7685-0130
俄罗斯联邦, Saint Petersburg

Oraz D. Yagmurov

St. Petersburg Bureau of Forensic Medical Examination

Email: oraz.yagmurov@gmail.com
ORCID iD: 0000-0003-1822-6043
SPIN 代码: 7765-8978

MD, Dr. Sci. (Med.)

俄罗斯联邦, Saint Petersburg

参考

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  3. Yang L, Tautz T, Zhang S, et al. The current status of malignant hyperthermia. J Biomed Res. 2020;34(2):75–85. doi: 10.7555/JBR.33.20180089
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  5. Chang L, Daly C, Miller DM, et al. Permeabilised skeletal muscle reveals mitochondrial deficiency in malignant hyperthermia-susceptible individuals. Br J Anaesth. 2019;122(5):613–621. doi: 10.1016/j.bja.2019.02.010
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  7. Lie JT, Holley KF, Kampa WR, et al. New histochemical method for morphologic diagnosis of early stages of myocardial ischemia. Proc Mayo Clin.1971;46(5):319–327.

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2. Fig. 1. Massive plethora of blood vessels with the development of sludge and loss of outline of red blood cells with the formation of homogeneous yellow-brown masses (intravascular hemolysis). Hematoxylin-eosin, ×100.

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3. Fig. 2. Wave-like deformation and fragmentation of cardiomyocytes with the presence of contracture sites. Hematoxylin-eosin, ×200.

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4. Fig. 3. Morphological picture of rhabdomyolysis in malignant hyperthermia: а ― loss of cross-striation and fibrous structure, focal tortuosity, homogenization of individual muscle fibers with areas of enlightenment (Hematoxylin-eosin, ×200); b ― positively stained fuchsinophilic areas (GOFP stain, ×200).

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