原发性肾病综合征患儿539例病理类型及随访

目的分析原发性肾病综合征（PNS）患儿的病理类型、治疗及随访情况。方法纳入2005年1月至2009年12月在湖南省儿童医院肾内科住院的诊断为PNS,进行肾脏病理学检查且随访时间≥6个月的患儿,根据年龄分为〈2岁组、～5岁组、～10岁组和〉10岁组,对不同年龄组肾脏病理类型、对糖皮质激素的反应、转归及随访资料进行分析。结果共纳入539例PNS患儿,男性402例,女性137例,男女比例为2.9∶1;〈2岁组159例（29.5%）,～5岁组269例（49.9%）,～10岁组84例（15.6%）,〉10岁组27例（5.0%）（P〈0.01）。①微小病变274例（50.8%）,局灶节段性肾小球硬化79例（14.6%）,系膜增生性肾小球肾炎173例（32.1%）,膜增生性肾小球肾炎6例（1.1%）,膜性肾病4例（0.74%）,增生硬化性肾小球肾炎2例（0.37%）,脂蛋白肾病1例（0.18%）。不同年龄组间肾脏病理类型的分布差异有统计学意义（P〈0.05）。②对糖皮质激素敏感和依赖的PNS患儿以微小病变为主（分别为62.4%和69.6%）,对糖皮质激素耐药的PNS患儿以系膜增生性肾小球肾炎为主（48.4%）。③539例中239例完全缓解,75例部分缓解,61例无效,158例症状控制,尿蛋白（-）,但仍在服用泼尼松。6例患儿分别因对糖皮质激素耐药,病情不能缓解,合并严重感染或进展至终末期肾病放弃治疗而死亡。结论儿童PNS的发病高峰年龄、肾脏病理类型以及对糖皮质激素耐药的发生率可能已发生改变,并且在不同年龄患儿间存在差异。

Pathological and follow-up studies in 539 children with primary nephrotic syndrome

Objective To study the feature of treatment,pathology and follow-up in children with primary nephrotic syndrome（PNS）.Methods Children with PNS hospitalized in Department of Nephrology of Hunan Children＇s Hospital from January 2005 to December 2009 who had been followed-up longer than 6 months were included.PNS patients were grouped into 2 years,-5 years,-10 years and 10 years groups according to age.The information of treatment,renal pathological lesion and follow-up data in children with PNS were analyzed.Results Five hundred and thirty-nine children（including 402 boys and 137 girls）with PNS were investigated in the study.The ratio of boys to girls was 2.9.Among them,159（29.5%）were under 2 years of age,269（49.9%）aged 2-5 years,84（15.6%）aged 5-10 years,and 27（5.0%）aged over 10 years.①Among them 274（50.8%）patients showed minimal change disease,79（14.7%）patients showed focal segment glomerulosclerosis,173（32.1%）patients showed mesangioproliferative glomerulonephritis,6（1.1%）patients showed membranoproliferative glomerulonephritis,and 7（1.3%）patients showed others＇s pathologic lesion.The proportional distribution of pathologic lesion type in kidney among 4 groups（〈2 years,-5 years,-10 years and〉 10 years）was signifiantly different（P〈0.05）.②The major pathology pattern of children with steroid sensitive and resistant PNS was minimal change disease（62.4% and 69.6%,respectively）.The major pathology pattern of children with steroid dependant PNS was mesangioproliferative glomerulonephritis（48.4%）.③239/539 patients were with complete remission,75 with partial remission,61 invalid,158 with symptomatic control,urine protein（-）,but still taking prednisone.Six cases died due to glucocorticoid resistance with severe infection or progression to end-stage renal disease to give up medical treatment.Conclusions In recent years,the age of onset,sex,pathologic lesion type and response to steroid therapy of children with PNS may change,and there existed significant differences in patients with different ages.