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我如何治疗儿童热性感染相关性癫痫综合征OA

How I treat pediatric febrile infection-related epilepsy syndrome

中文摘要英文摘要

热性感染相关性癫痫综合征(febrile infection-related epilepsy syndrome,FIRES)主要累及既往健康的儿童及青少年,常伴随严重的神经功能损伤,遗留药物难治性癫痫、认知障碍等远期后遗症.该文基于2例儿童FIRES病例,结合国际、国内指南和临床实践经验,重点展示急性期与慢性期不同炎症阶段下的治疗策略及其升级路径.结合持续脑电图监测及脑脊液炎症因子谱变化,系统阐述以快速终止癫痫持续状态-靶向细胞因子免疫治疗-多学科协同支持为核心的分阶段治疗思路,并重点强调托珠单抗、阿那白滞素及生酮饮食的积极应用,以期为临床医师在FIRES中的治疗决策提供可操作参考.

Febrile infection-related epilepsy syndrome(FIRES)mainly affects previously healthy children and adolescents,often leading to severe neurological impairment and long-term sequelae such as drug-resistant epilepsy and cognitive dysfunction.Based on two pediatric FIRES cases,combined with international and domestic guidelines as well as clinical experience,this paper highlights therapeutic strategies and escalation pathways tailored to different inflammatory phases in the acute and chronic stages.Incorporating continuous electroencephalogram monitoring and cerebrospinal fluid inflammatory cytokine profile changes,a phase-specific treatment approach is systematically described,centered on the rapid termination of status epilepticus,targeted cytokine immunotherapy,and multidisciplinary collaborative support,and the active use of tocilizumab,anakinra,and the ketogenic diet is emphasized,to provide practical guidance for clinicians in managing FIRES.

邓小鹿;彭镜

中南大学湘雅医院儿童医学中心/湖南省儿童脑发育障碍性疾病研究中心,湖南 长沙 410008中南大学湘雅医院儿童医学中心/湖南省儿童脑发育障碍性疾病研究中心,湖南 长沙 410008

热性感染相关性癫痫综合征癫痫持续状态诊断免疫治疗儿童

Febrile infection-related epilepsy syndromeStatus epilepticusDiagnosisImmunotherapyChild

《中国当代儿科杂志》 2026 (6)

659-664,6

国家自然科学基金(82471488)湖南省自然科学基金(2026JJ80167)中南大学教改课题(2025jy203).

10.7499/j.issn.1008-8830.2512090

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