儿童IgA血管炎相关神经系统损害的临床分析  

Clinical analysis of neurological damage associated with IgA vasculitis in children

作　　者：李芳芳 李崇巍[2] 郑荣秀[1] LI Fang-fang;LI Chong-wei;ZHENG Rong-xiu(Department of Pediatrics,General Hospital,Tianjin Medical University,Tianjin 300052,China;Department of Rheumatology&Immunology,Tianjin Children′s Hospital,Tianjin 300134,China)

机构地区：[1]天津医科大学总医院儿科,天津300052 [2]天津市儿童医院风湿免疫科,天津300134

出　　处：《天津医科大学学报》2023年第6期662-665,共4页Journal of Tianjin Medical University

摘　　要：目的:总结Ig A血管炎引起的神经系统损害,探讨其危险因素、治疗及转归。方法:选择天津市儿童医院2016年1月至2022年1月收治的Ig A血管炎患儿1879例,其中出现神经系统损害的患儿34例(A组),随机抽取同期非神经系统损害病例72例(B组),对两组临床表现、实验室检查、治疗及转归进行回顾性分析。结果:IgA血管炎相关神经系统损害发生率为1.8%,A组和B组均有典型紫癜样皮疹,A组出现消化道症状、消化道出血、皮疹在非典型部位的发生率分别为97.1%、32.4%、67.6%,B组分别为72.2%、8.3%、40.3%,差异存在统计学意义(χ^(2)=9.571、9.895、6.922,均P<0.05),但尿常规、尿微量白蛋白、尿转铁蛋白、血Ig、便钙卫蛋白等无统计学差异(均P>0.05)。A组中16例脑电图显示背景活动慢波;15例神经电生理显示周围神经损害;2例头颅MRI提示大脑后部可逆性脑病综合征;1例头颅MRI提示静脉窦血栓。A组均接受糖皮质激素治疗,其中强化治疗19例,大剂量甲强龙冲击治疗8例,使用环磷酰胺15例,静脉应用丙种球蛋白10例,7例采用了甲强龙冲击联合环磷酰胺治疗,5例应用甲强龙冲击联合环磷酰胺及丙种球蛋白治疗。B组仅3例应用甲强龙冲击治疗,2例应用环磷酰胺,其中1例应用甲强龙冲击联合环磷酰胺治疗,其余68例仅予常规剂量糖皮质激素或对症治疗后好转。两组患儿出院时病情均好转,出院后随访6个月均未遗留后遗症。结论:Ig A血管炎罕见出现神经系统损害,出现消化道症状、消化道出血和皮疹发生在非典型部位的患儿更容易发生神经系统损害。Objective:To summarize the neurological damage caused by IgA vasculitis and explore the associated risk factors,treatment,and outcomes.Methods:A retrospective analysis was conducted on 1879 cases of IgA vasculitis admitted to Tianjin Children′s Hospital between January 2016 and January 2022.Among these cases,34 children with neurological damage were categorized into group A,while 72 cases without neurological damage were randomly selected for group B.Clinical manifestations,laboratory examinations,treatments,and outcomes were retrospectively analyzed for both groups.Results:The incidence of IgA vasculitis-related neurological damage was found to be 1.8%.A typical purpura rash was observed in both group A and group B.The incidence of gastrointestinal symptoms,gastrointestinal bleeding,and rash was significantly higher in group A(97.1%,37.4%and 67.6%respectively)compared to group B(72.2%,8.3%,and 40.3%respectively,χ^(2)=9.571,9.895,6.922,all P<0.05).However,there were no significant differences between the two groups in terms of urine routine,microalbumin,transferrin,blood Ig levels,and fecal calprotectin(all P>0.05).In group A children,16 EEGs showed background activity slow waves,15 neuroelectrophysiology tests indicated peripheral nerve damage,2 cases had head MRI findings suggestive of posterior brain reversible encephalopathy syndrome,and 1 child had head MRI findings suggestive of venous sinus thrombosis.All children in group A received glucocorticoid treatment,with 19 cases undergoing intensive treatment,8 cases received highdose methylprednisolone pulse therapy,15 cases received cyclophosphamide,and 10 cases were treated with intravenous gamma globulin(IVIG),7 cases were treated with methylprednisolone pulse therapy combined with cyclophosphamide,and 5 cases were treated with methylprednisolone pulse therapy combined with cyclophosphamide and IVIG.In group B,only 3 children received methylprednisolone pulse therapy and 2 children were treated with cyclophosphamide,while 1 case received both treatments.Th

关 键 词：IgA血管炎 神经系统损害 大脑后部可逆性脑病综合征 静脉窦血栓 

分 类 号：R725.9[医药卫生—儿科]