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.
作者
周益平
崔云
缪惠洁
王斐
史婧奕
单怡俊
张育才
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)
出处
《中国小儿急救医学》
CAS
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)。
关键词
血浆置换
重症肌无力
抗乙酰胆碱抗体
儿童
Therapeutic plasma exchange
Myasthenia gravis
Anti-acetylcholine antibody
Child
