ëϸѪ���������������������ۺ���6���ٴ�����

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。

Clinical analysis of 6 cases of nephrotic syndrome due to EPGN

??AbstractObjectiveTo analyze the clinical manifestation,pathological changes and treatment of 6 patients with nephrotic syndrome(NS) due to endocapillary proliferative glomerulonephritis(EPGN)MethodsThe data of patients with NS due to EPGN admitted to our department from 2002 to 2004 were retrospectively analyzed.Results(1)All the 6 patients were manifested clinically by nephritis nephrotic syndrome.(2)The pathological features showed that cell crescent was formed on the basis of endocapillary proliferation;the granular deposition of IgG and C3 was found along the blood capillary,and ??nontypical hump?? and partial foot process fusion were also detected.(3)Treatment:6 cases were treated with methyprednisolone.Two cases completely alleviated,4 cases weren??t.Among these 4 cases,2 cases were treated with cyclohosphamide,and 2 cases were treated with common three-wing-nut Root,all of them being cured.Conclusion(1)There is close relation ship between pathological changes and clinical manifestations.Those with crescent were presented with long duration of hematuria and those with foot process fusion have long duration of severe proteinuria.(2)Enough doses of steroid should be given once diagnosis is confirmed.A satisfactory effect could be achieved through combination with immunosuppressant if there is no responses after 4 weeks of steroid.But long term prognosis is yet to be investigated.