治疗性血浆置换辅助治疗儿童重症肌无力临床观察  

Effect of therapeutic plasma exchange on myasthenia gravis patients in children

作　　者：周益平[1] 崔云[1] 缪惠洁[1] 王斐[1] 史婧奕[1] 单怡俊 张育才[1] Zhou Yiping;Cui Yun;Miao Huijie;Wang Fei;Shi Jingyi;Shan Yijun;Zhang Yucai(Department of Critical Care Medicine,Shanghai Children′s Hospital,Shanghai Jiao Tong University,Shanghai 200062,China)

机构地区：[1]上海交通大学附属儿童医院重症医学科,200062

出　　处：《中国小儿急救医学》2021年第11期972-976,共5页Chinese Pediatric Emergency Medicine

基　　金：上海市科委"科技创新行动计划"临床医学领域项目(18411951000)。

摘　　要：目的探讨治疗性血浆置换(therapeutic plasma exchange,TPE)在辅助治疗儿童重症肌无力(myasthenia gravis,MG)中的作用。方法回顾性总结2016年1月2019年12月收入上海交通大学附属儿童医院重症监护病房(PICU)的MG患儿,经乙酰胆碱酯酶抑制剂、糖皮质激素或静脉丙种球蛋白(IVIG)等治疗效果不良基础上进行TPE治疗,血浆置换量为50~70 mL/(kg·次),每例2~3次。比较TPE前后临床症状与抗乙酰胆碱抗体(AChR-Ab)水平变化。结果共收治MG患儿7例,其中全身型4例(肌无力危象合并呼吸衰竭1例),眼肌型3例。AChR-Ab从TPE前1.66(0.99,3.33)nmol/L下降至0.66(0.40,10.97)nmol/L(Z=-2.545,P=0.011),下降50.55%。7例患儿出院时肌无力、眼睑下垂症状部分或完全缓解。TPE前后循环免疫复合物、补体C3及CD4^(+)T细胞、CD8^(+)T细胞、NK细胞水平无显著变化(均P>0.05)。TPE过程中血浆过敏2例,低血压1例,处理后恢复。血纤维蛋白原水平下降,TPE前后分别为1.90(1.40,2.40)g/L和1.10(1.00,1.30)g/L(Z=-3.092,P=0.002)。结论TPE可降低常规治疗效果不佳MG患儿的AChR-Ab水平,缓解肌无力症状,是MG患儿可选治疗措施。TPE时应注意纤维蛋白原降低等不良反应。Objective To investigate the effects of therapeutic plasma exchange(TPE)as adjuvant therapy in children with myasthenia gravis(MG)in pediatric intensive care unit(PICU).Methods A retrospective study was conducted in 7 children with MG admitted to PICU at Shanghai Children′s Hospital from January 2016 to December 2019.TPE was performed on unsatisfactory effect of acetylcholinesterase inhibitors,glucocorticoids or IVIG.The TPE dose was 50-70 mL/kg for 2 to 3 times for each case.The clinical symptoms,anti-acetylcholine antibody(AChR-Ab)level and prognosis were measured before and after TPE.Results Seven children with myasthenia gravis admitted to PICU from January 2016 to December 2019,including 4 cases of systemic myasthenia gravis(1 case of myasthenia crisis with respiratory failure)and 3 cases of ocular myasthenia gravis.The AChR-Ab level decreased from 1.66(0.99,3.33)nmol/L before TPE to 0.66(0.40,10.97)nmol/L after TPE(Z=-2.545,P=0.011).The symptoms of muscle weakness and blepharoptosis were partially or completely relieved in 7 cases.There were no significantly changes in the levels of circulating immune complex,complement C3,CD4^(+),CD8^(+)and NK cells before and after TPE(all P>0.05).During the process of TPE,2 cases had mild rash,and 1 case had hypotensive shock,which were recovered after timely treatment.After TPE,the fibrin levelsdecreased from 1.90(1.40,2.40)g/L to 1.10(1.00,1.30)g/L(Z=-3.092,P=0.002).Conclusion TPE reduce the AChR-Ab levels and improve the short-term symptoms in children with myasthenia gravis who have failed conventional treatment.TPE may be an optional therapy for pediatric severe MG.

关 键 词：血浆置换 重症肌无力 抗乙酰胆碱抗体 儿童 

分 类 号：R746.1[医药卫生—神经病学与精神病学]