溶血尿毒综合征合并IgA肾病的临床病理学观察  

Hemolytic uremic syndrome complicated with IgA nephropathy:a clinicopathological study

作　　者：姚兴凤[1] 王响 伏利兵[1] 陈植[2] 张楠[1] 李雪倩 刘小荣[2] 何乐健[1] Yao Xingfeng;Wang Xiang;Fu Libing;Chen Zhi;Zhang Nan;Li Xueqian;Liu Xiaorong;He Lejian(Department of Pathology,Beijing Children′s Hospital,Capital Medical University,National Center for Children′s Health Beijing 100045;Department of Reanal Medicine,Beijing Children′s Hospital,Capital Medical University,National Center for Children′s Health)

机构地区：[1]国家儿童医学中心,首都医科大学附属北京儿童医院病理科,100045 [2]国家儿童医学中心,首都医科大学附属北京儿童医院肾病科,儿童慢性肾脏病与血液净化北京市重点实验室,100045

出　　处：《中华病理学杂志》2021年第8期910-914,共5页Chinese Journal of Pathology

基　　金：北京市科学技术委员会首都临床特色应用研究(Z161100000516106);北京市科学技术委员会重点项目(D181100000118006)。

摘　　要：目的探讨溶血尿毒综合征合并IgA肾病患儿的临床及病理特征、治疗、预后及发病机制。方法分析首都医科大学附属北京儿童医院确诊的2例溶血尿毒综合征合并IgA肾病患者的临床表现、肾脏穿刺标本的病理组织形态(光镜、免疫荧光、透射电镜)、治疗及预后,并复习相关文献。结果患者临床表现为微血管性溶血性贫血、血小板减少、急性肾功能损害三联征伴血尿、蛋白尿,抗H因子抗体阳性。病理形态同时具备溶血尿毒综合征肾损伤和IgA肾病的组织学特点,包括光镜:肾小球系膜细胞和基质增生伴内皮细胞增生、毛细血管腔狭窄,小动脉增厚、狭窄,伴有小球缺血性改变及毛细血管腔瘤样扩张;免疫荧光:肾小球系膜区弥漫性IgA沉积;电镜检查:肾小球系膜细胞及内皮细胞增生、基底膜内疏松层增宽,同时系膜区块状电子致密物沉积,伴小节段的基底膜皱缩。2例患儿对血浆置换及激素治疗反应好,分别随访5年7个月及8个月,反复尿蛋白及潜血阳性。结论溶血尿毒综合征合并IgA肾病罕见,确诊主要依靠组织病理学,需光镜、免疫荧光、电镜三者相结合。血浆置换及激素治疗是有效治疗方式,长期预后不理想,可能与病理分级及继发因素相关。二者合并发生可能由前驱感染因素或继发因素导致。Objective To investigate the clinicopathologic characteristics,treatments,outcomes and mechanisms of hemolytic uremic syndrome(HUS)complicated with IgA nephropathy(IgAN).Methods The clinical manifestations,treatments,prognosis and histopathological features of renal biopsy tissues were analyzed in two cases of HUS complicated with IgAN from Beijing Children′s Hospital,Capital Medical University using light microscopy,immunofluorescence detection and electron microscopy.The related literatures were also reviewed.Results The clinical manifestations were microvascular hemolytic anemia,thrombocytopenia,acute renal impairment with hematuria,proteinuria,and positive anti-H factor antibody.Histological findings confirmed presence of both HUS and IgAN.Histological features included glomerular mesangial and stromal hyperplasia with endothelial cell proliferation,capillary stenosis,arteriolar thickening,and glomerular ischemia and capillary dilatation.Immunofluorescence detection showed diffuse IgA deposition in the glomerular mesangial matrix.Electron microscopy showed proliferation of mesangial and endothelial cells,thickening of the inner layer of the glomerular basement membrane,deposition of massive electronic densification in the mesangial region,and shrinkage of the segmental basement membrane.The two children were very responsive to plasma exchange and steroid treatments.However,their urine protein and occult blood tests remained continuously positive during the follow-up of 5 years 7 months and 8 months respectively.Conclusions HUS complicated with IgAN is rare.The diagnosis relies on various pathological examinations,which require the combination of light microscopy,immunofluorescence detection and electron microscopy.Plasma exchange and steroid treatments are effective.However,the long-term prognosis is concerning and may relate to pathological grade and secondary factors.The mechanism of connecting HUS and IgAN is unknown,but may be caused by prodromal or secondary factors.

关 键 词：溶血尿毒症综合征 肾小球肾炎 IGA 电子显微镜检查 

分 类 号：R725[医药卫生—儿科] R726.9[医药卫生—临床医学]