儿童重症肌无力危象治疗的研究进展

Research Advances in the Treatment of Myasthenic Crisis in Children

摘要: 儿童重症肌无力危象(Myasthenic Crisis, MC)是MG患者最严重的临床状态，以进行性呼吸困难而需要依赖呼吸支持为临床特点。儿童患者因发育差异在发病机制、临床表现及治疗策略上与成人存在显著不同。目前治疗证据多源于成人研究或小样本儿科数据，亟待针对性探索。本文对儿童重症肌无力危象的病理生理机制、诊断标准、药物治疗(如胆碱酯酶抑制剂、免疫调节治疗、生物制剂、静脉免疫球蛋白治疗)、非药物治疗(如血浆置换)、重症监护管理以及新兴疗法(如补体抑制剂、FcRn拮抗剂)等方面进行归纳综述。

Abstract: Myasthenic Crisis (MC) in children is the most severe clinical state of MG patients, characterized clinically by progressive dyspnea and reliance on respiratory support. Due to developmental differences, children patients have significant differences from adults in terms of pathogenesis, clinical manifestations and treatment strategies. At present, most of the treatment evidence comes from adult studies or small sample pediatric data, and targeted exploration is urgently needed. This review summarizes the pathophysiological mechanisms, diagnostic criteria, drug treatments (such as cholinesterase inhibitors, immunomodulatory therapy, biologics, intravenous immunoglobulin therapy), non-drug treatments (such as plasma exchange), intensive care management, and emerging therapies (such as complement inhibitors, FcRn antagonists) of myasthenic crisis in children.