蛋白A免疫吸附治疗儿童难治性抗N-甲基-D-门冬氨酸受体脑炎的回顾性分析  

Retrospective analysis of protein A column immunoadsorption in the treatment of refractory anti-N-methyl-D-aspartate receptor encephalitis in children

作　　者：王莹[1] 马乐[1] 郭张妍 杜彦强 张华[1] 王卫凯[2] 王义[1] Wang Ying;Ma Le;Guo Zhangyan;Du Yanqiang;Zhang Hua;Wang Weikai;Wang Yi(National Children's Regional Medical Center(Northwest),Children's Hospital Affiliated to Xi'an Jiaotong University,Department of Intensive Care Unit of Xi'an Children's Hospital,Xi'an710003,China;Children Emergency Center Sencond Department,Gansu Maternal and Child Health Hospital(Gansu Provincial Central Hospital),Lanzhou 730050,China)

机构地区：[1]国家儿童区域医疗中心(西北)西安交通大学附属儿童医院,西安市儿童医院儿童重症医学科,710003 [2]甘肃省妇幼保健院(甘肃省中心医院)儿童急救中心二部,兰州730050

出　　处：《中国小儿急救医学》2025年第1期33-37,共5页Chinese Pediatric Emergency Medicine

基　　金：陕西省科技厅自然科学基金(2021JM-560);西安市科技局(21YXYJ0009);西安市卫生健康委面上培训项目(2023ms10);国家儿童区域医疗中心医联体项目(2022E05);甘肃省科技厅重大专项(22ZD6FA034)。

摘　　要：目的评价蛋白A免疫吸附(PA-IA)治疗儿童难治性抗N-甲基-D-门冬氨酸受体(NMDAR)脑炎的安全性和有效性。方法回顾性收集2022年1月至2023年6月西安市儿童医院儿童重症医学科收治的8例一线治疗无效的抗NMDAR脑炎患儿的临床资料,分析PA-IA治疗前后患儿临床特征、改良Rankin评分(mRS)和抗NMDAR抗体滴度(血清和脑脊液)以及血IgG、补体等免疫学指标。同时详细记录治疗期间发生的所有不良事件。结果8例患儿中,男5例,女3例,年龄5~12岁,每例免疫吸附6~10次,共进行75例次PA-IA治疗。8例患儿PA-IA治疗后mRS评分下降;血IgG水平、脑脊液细胞数、脑脊液蛋白水平、血补体C3和C4水平、血和脑脊液相关抗体滴度均较治疗前降低;差异有统计学意义(P<0.001)。75例次治疗中,仅1例患儿治疗过程中发生破膜,停止治疗,更换血浆分离器后继续治疗,无其他不良反应发生。结论PA-IA治疗可有效改善儿童难治性抗NMDAR脑炎临床症状及神经功能,安全性良好,但其在抗NMDAR脑炎患儿中的确切疗效仍需进一步验证。Objective To evaluate the safety and efficacy of immunoadsorption with protein A column(PA-IA)in refractory anti-N-methyl-D-aspartate receptor(NMDAR)encephalitis in children.MethodsThe clinical data of 8 children with anti-NMDAR encephalitis who failed to receive first-line treatment from January 2022 to June 2023 in PICU of Xi'an Children's Hospital were retrospectively collected.Clinical features,modified Rankin score(mRS),anti-NMDAR antibody titers in serum and cerebrospinal fluid(CSF),cells count in CSF,protein level in CSF,serum IgG,complement C3,C4 level were analyzed before and after immunoadsorption therapy.All adverse events that occurred during treatment were recorded in detail.ResultAmong the 8 children,there were 5 males and 3 females,aged 5 to 12 years old,and each patient was immunized 6 to 10 times,and 75 times of PA-IA treatment were performed.The mRS score decreased in 8 children after PA-IA treatment.Blood IgG level,CSF cell number,protein level in CSF,blood complement C3 and C4 levels,and antibody titers related in blood and CSF were all lower than those before PA-IA treatment.The differences were all statistically significant(P<0.001).During 75 times of treatment,only 1 case of membrane rupture occurred during treatment,the treatment was stopped and continued after the plasma separator was replaced,with no other adverse reactions.ConclusionPA-IA could effectively improve the clinical symptoms and neurological function of children with refractory anti-NMDAR encephalitis with good safety,but its exact efficacy in children with anti-NMDAR encephalitis needs to be verified by prospective studies with larger samples.

关 键 词：蛋白A免疫吸附 儿童 抗N-甲基-D-门冬氨酸受体脑炎 

分 类 号：R742[医药卫生—神经病学与精神病学]