Successful sedation with remimazolam and alfentanil in a child susceptible to malignant hyperthermia: a case report.

IF 2.3 3区医学 Q2 ANESTHESIOLOGY

BMC Anesthesiology Pub Date : 2025-04-23 DOI:10.1186/s12871-025-03076-0

Kailai Zhu, Shuangwei Wu, Xianglin Hao, Chuanguang Wang

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引用次数: 0

Abstract

Background: Malignant hyperthermia (MH) is a life-threatening autosomal-dominant disorder caused by mutations in the ryanodine receptor 1 (RYR1) gene, leading to calcium dysregulation in skeletal muscle. Patients with genetically confirmed MH susceptibility must strictly avoid volatile anesthetics and succinylcholine. Intravenous sedation presents a viable alternative, yet evidence supporting remimazolam use in pediatric MH patients remains scarce.

Case presentation: We report the first case of a 1-year-old male patient with genetically confirmed MH susceptibility undergoing orchidopexy under remimazolam-alfentanil sedation combined with caudal block. The patient had no MH manifestations intraoperatively or postoperatively and recovered uneventfully.

Conclusion: This case demonstrates the feasibility of remimazolam-based sedation in genetically confirmed pediatric MH patients, supporting its safety profile in this population. Further multicenter studies are needed to establish standardized protocols.

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雷马唑仑和阿芬太尼成功镇静儿童易患恶性高热：1例报告。

背景：恶性高热症（MH）是一种危及生命的常染色体显性疾病，由ryanodine受体1 （RYR1）基因突变引起，导致骨骼肌钙失调。遗传上证实MH易感性的患者必须严格避免使用挥发性麻醉剂和琥珀酰胆碱。静脉镇静是一种可行的替代方案，但支持小儿MH患者使用雷马唑仑的证据仍然很少。病例介绍：我们报告了第一例1岁的男性患者，遗传上证实MH易感性，在雷马唑仑-阿芬太尼镇静联合尾侧阻滞下接受兰花切除术。患者术中及术后均无MH表现，恢复平稳。结论：该病例证明了雷马唑仑为基础的镇静在基因确认的儿童MH患者中的可行性，支持其在该人群中的安全性。需要进一步的多中心研究来建立标准化的方案。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

BMC Anesthesiology ANESTHESIOLOGY-

CiteScore

3.50

自引率

4.50%

发文量

349

审稿时长

>12 weeks

期刊介绍： BMC Anesthesiology is an open access, peer-reviewed journal that considers articles on all aspects of anesthesiology, critical care, perioperative care and pain management, including clinical and experimental research into anesthetic mechanisms, administration and efficacy, technology and monitoring, and associated economic issues.