中国实用儿科杂志

中国实用儿科杂志

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儿童C3肾小球肾炎6例临床与病理分析

  

  1. 吉林大学第一医院小儿肾病科,吉林  长春  130021
  • 出版日期:2017-05-06 发布日期:2017-05-10

Clinical and pathological analysis of C3 glomerulonephritis in 6 children

  1. Department of Pediatrics，the First Hospital of Jilin University，Changchun  130021，China
  • Online:2017-05-06 Published:2017-05-10

摘要：

目的    分析6例儿童C3肾小球肾炎的临床表现、病理特征及治疗反应，旨在提高儿科医生对此病的认识和处理。方法　对2010年9月至2016年6月在吉林大学第一医院小儿肾病科确诊为C3肾小球肾炎且有完整临床资料的6例患儿的临床表现、病理改变、疗效及预后进行回顾性分析。结果　（1）临床表现及实验室检查：以血尿为首发症状2例，以血尿和蛋白尿为首发症状4例；其中表现为肾炎综合征3例，肾病综合征1例；6例患儿补体C3均降低；补体C4均正常；（2）病理特点：6例患儿免疫荧光均可见补体C3强阳性沉积；光镜下表现为系膜增生性肾小球肾炎5例，表现为毛细血管内增生性肾小球肾炎1例；电镜下3例可见电子致密物沉积， 3例未见电子致密物沉积；（3）治疗及预后；均予肾炎常规治疗，2例有新月体形成者加用糖皮质激素治疗。随访6～42个月，预后较好。结论　本组C3肾小球肾炎患儿以血尿和（或）蛋白尿为主要表现，免疫荧光见C3强阳性沉积，光镜表现多以系膜增生性肾小球肾炎为主，电镜可表现为电子致密物沉积，短期预后较好。

关键词: 儿童, C3肾小球肾炎, 肾脏病理, 治疗与随访

Abstract:

Objective　To analyze the clinical characteristics，pathological features and treatment responses of 6 pediatric patients with C3 glomerulonephritis（C3GN），in order to improve the understanding and treatment of this disease for pediatricians. Methods　Analyze the clinical manifestations，pathological features，therapies，prognosis of patients who were diagnosed with C3 glomerulonephritis from September，2010 to June，2016 retrospectively. Results　Clinical characteristics and laboratory examination：2 patients’ first symptom was hematuria；4 patients’ first symptom was hematuria and proteinuria；3 patients presented as acute nephritic syndrome，one presented as nephrotic syndrome. All the patients showed that the level of serum complement C3 was reduced，while sernm complement C4 was normal. Pathological character：6 patients showed strong positive complement C3 deposition under immunofluorescence. Lightmicroscopy showed mesangial proliferative glomerulonephritis in 5 cases，1 case was diagnosed as endocapillary proliferative glomerulonephritisin，and 3 patients presented electrondense depositionin under electron microscope. Treatment and prognosis：after conventional treatment，2 patients who were with crescent were treated with glucocorticoid .After a follow-up from 6 months to 42 months ，the prognosis was pretty good. Conclusion　Children with C3GN are usually presented with hematuria and （or）proteinuria，characterized by strong positive C3 deposition. Lightmicroscopy always shows mesangial proliferative glomerulonephritis. Electron microscope show electron dense deposition，and short-term prognosis is pretty good.

Key words: child, C3 glomerulonephritis, renal pathology, treatment and follow-up