摘要
目的分析儿童特发性膜性肾病(IMN)的临床病理特点,并探讨其治疗方案。方法回顾性分析2013年1月至2023年12月首都医科大学附属首都儿童医学中心确诊为IMN的15例患儿的临床病理特点及治疗效果。结果①15例患儿中男10例,女5例,年龄5~15岁,中位年龄10.26岁,临床表现为肾炎性肾病综合征11例(73.33%),单纯性肾病综合征1例(6.67%),肾炎综合征2例(13.33%),单纯蛋白尿1例(6.67%),起病时2例(13.33%)伴肾功能不全。②病理分型显示,8例(53.33%)表现为典型膜性肾病,7例(46.67%)表现为不典型膜性肾病。光镜下以系膜细胞、基质轻度增生(93.33%)及基底膜增厚(86.67%)为主。免疫荧光均以IgG、C3沉积为主。电镜下电子致密物以上皮下、基底膜内沉积为主,部分可见系膜区、内皮下沉积。③治疗后完全缓解8例(53.33%),部分缓解5例(33.33%),未缓解2例(13.33%)。其中泼尼松联合环孢素治疗8例,完全缓解4例,部分缓解4例,完全缓解率为50.00%,有效率为100.00%。结论儿童IMN多见于男童,发病年龄以10岁以上居多,临床表现以肾炎性肾病综合征为主,病理表现典型膜性肾病及不典型膜性肾病比例相当。泼尼松联合环孢素疗效明确,未来需扩大样本量进一步验证不同治疗方法的疗效差异。
Objective To analyze the clinicopathological features and treatment of idiopathic membranous nephropathy(IMN)in children.Method A retrospective analysis of the clinical and pathological characteristics and treatment outcomes of 15 children diagnosed with IMN at the Capital Center For Children’s Health,Capital Medical University,from January 2013 to December 2023.Result①Among the 15 cases,10 were boys and 5 were girls,aged 5-15 years,with a median age of 10.26 years.The clinical manifestations were nephritic type nephrotic syndrome(NS)in 11 cases(73.33%),simple type nephrotic syndrome in 1 case(6.67%),nephritis syndrome in 2 cases(13.33%),and simple proteinuria in 1 case(6.67%).②Pathological classifi cation revealed typical membranous nephropathy in 8 cases(53.33%)and atypical membranous nephropathy in 7 cases(46.67%).Light microscopy primarily showed mild mesangial cell and matrix proliferation(93.33%)and basement membrane thickening(86.67%).Immunofl uorescence predominantly exhibited IgG and C3 deposition.Electron microscopy demonstrated electrondense deposits mainly in the subepithelial and intramembranous regions,with some observed in the mesangial and subendothelial areas.③Eight cases(53.33%)had complete remission,fi ve cases(33.33%)had partial remission,and two cases(13.33%)had no remission.Among the 8 cases treated with prednisone combined with cyclosporine(CSA),4 achieved complete remission,4 achieved partial remission,resulting in a complete remission rate of 50.00%and an overall response rate of 100.00%.Conclusion IMN in children is more common in males,with a higher incidence in those over 10 years of age.The primary clinical manifestation is nephritic type nephrotic syndrome,and the proportions of typical and atypical membranous nephropathy are comparable.Prednisone combined with cyclosporine shows promising effi cacy.However,further studies with larger sample sizes are needed to validate the differences in treatment effi cacy.
作者
林媛
陈朝英
李华荣
Lin Yuan;Chen Chaoying;Li Huarong(Department of Nephrology,Capital Center For Children’s Health,Capital Medical University,Beijing 100020,China)
出处
《中国医刊》
2026年第2期194-198,共5页
Chinese Journal of Medicine
基金
北京市属医院科研培育计划项目(PX2023050)。
关键词
特发性膜性肾病
病理
治疗
儿童
Idiopathic membranous nephropathy
Pathology
Treatment
Children
