目的分析毛细血管内增生性肾炎致肾病综合征患儿的临床表现,病理改变和治疗。方法2002-06—2004-08,对苏州大学附属儿童医院肾内科收治的毛细血管内增生性肾炎致肾病综合征6例进行回顾分析。结果(1)6例患儿的临床表现均为肾炎型肾病综合征;(2)其病理特点为:在毛细血管内增生的基础上有细胞新月体形成,可见IgG、C3的颗粒状沉积物沿毛细血管袢连续排列,且发现“不典型驼峰”和部分足突融合;(3)治疗:6例均予激素治疗。其中2例治疗4周内完全缓解;4例未能在4周内达到完全缓解者,2例加用环磷酰胺,2例予雷公藤,均缓解。结论(1)病理改变与临床表现关系密切,伴新月体者肉眼血尿时间较长,伴足突融合者蛋白尿程度重,持续时间长;(2)给予足量激素,或联合应用免疫抑制剂,可取得较好疗效,但长期预后尚待进一步观察。 Objective To analyze the clinical manifestations, pathological changes and treatment of children with nephrotic syndrome caused by capillary proliferative glomerulonephritis. Methods From June 2002 to August 2008, 6 cases of nephrotic syndrome caused by capillary proliferative glomerulonephritis were admitted to Department of Nephrology, Children’s Hospital Affiliated to Soochow University. Results (1) The clinical manifestations of 6 children were all nephritis nephrotic syndrome. (2) The pathological features were: the formation of crescentic cells on the basis of hyperplasia of capillaries, and the granular deposition of IgG and C3 Continuous arrangement of capillaries along the capillaries, and found “atypical hump” and part of foot process fusion; (3) treatment: 6 cases were treated with hormones. Two of them were completely relieved within 4 weeks of treatment; 4 patients failed to achieve complete remission within 4 weeks, 2 patients received cyclophosphamide and 2 patients were treated with Tripterygium wilfordii, both of which were relieved. Conclusions (1) The pathological changes are closely related to the clinical manifestations. The gross hematuria of the crescent with crescent body is longer, and proteinuria with foot process fusion is more severe and lasts longer. (2) Sufficient hormone or combined immunosuppression Agents, can achieve better efficacy, but the long-term prognosis remains to be further observed.