单中心28年儿童原发性肾病综合征临床与病理分析

Clinicopathological analysis of primary nephrotic syndrome in children based on 28 years renal biopsy data： a single center

目的 分析儿童原发性肾病综合征（PNS）临床与病理的关系及病理构成的变迁情况.方法 回顾性分析1984年1月至2011年12月在中山大学附属第一医院小儿肾病中心行肾活检、年龄≤14岁的PNS患儿的临床和病理资料,并分为1984年至1991年、1992年至2001年和2002年至2011年3个阶段进行比较分析.结果 共363例PNS患儿纳入研究,病理类型构成：微小病变（MCD） 168例（46.3％）,局灶节段性肾小球硬化（FSGS）77例（21.2％）,系膜增生性肾小球肾炎（MsPGN） 76例（20.9％）,膜增生性肾小球肾炎（MPGN）14例（3.9％）,膜性肾病（MN）12例（3.3％）,硬化性肾小球肾炎（CSGN）3例（0.8％）,新月体肾炎（CrGN）2例（0.6％）,其他11例（3.0％）.363例患儿中单纯型NS 205例（56.5％）,其中64.9％病理为MCD.肾炎型NS158例（43.5％）,第2阶段肾炎型NS病理类型以MsPGN为主（占36.4％）,第3阶段以FSGS最多见（占39.6％）.频复发（FR） NS 180例（49.6％）、激素依赖（SD） NS 49例（13.5％）、SD/FRNS的病理类型以MCD最为多见（62.2％）,其次为MsPGN（13.3％）和FSGS（13.3％）.激素耐药NS（SRNS） 172例（47.4％）,其中54.1％（93/172例）为原发耐药NS （PSRNS）,45.9％（79/172例）为继发耐药NS（SSRNS）.PSRNS和SSRNS的病理类型比较,差异无统计学意义（P＞0.05）.SRNS第2阶段最常见的病理类型为MsPGN,占41.5％,而第3阶段为FSGS,占41.5％.108例（29.8％）患儿肾组织无免疫复合物沉积,病理类型主要为MCD（63.9％）.96例（26.4％）肾组织免疫荧光以IgM沉积为主,其中SRNS 46例（47.9％）,SD/FRNS 36例（37.5％）.结论 儿童PNS病理类型主要为MCD、MsPGN和FSGS,临床和病理有一定的联系.近20年来,FSGS病例数增加,MsPGN的病例数减少.肾炎型NS、SD/FRNS、SRNS建议及早行肾活检,必要时重复肾活检.

Objective To analyze the relationship between pathology and clinical features and the changing tendency of pathologic constitution in children with primary nephrotic syndrome （PNS）.Methods A retrospective study was done at pediatric renal biopsy database of the First Affiliated Hospital of Sun Yat-Sen University starting from Jan.1984 to Dec.2011.All of these patients were diagnosed as PNS and under 14 years old.The cases were categorized into 3 periods：period 1 （1984 to 1991）,period 2 （1992 to 2001）,and period 3 （2002 to 2011）.Results A total of 363 patients were investigated in this study.Renal pathology revealed that there were 168 （46.3％） patients with minimal change disease（MCD）,77 cases （21.2％） with focal and segmental glomerulosclerosis （FSGS）,76 cases （20.9％）with mesangioproliferative glomerulonephritis （MsPGN）,14 cases （3.9％） with membranoproliferative glomerulonephritis（MPGN）,12 cases（3.3％） with membranous glomerulopathy（MN）,3 cases（0.8％） with chronic sclerosing glomerulonephritis （CSGN）,2 cases （0.6％） with crescentic glomerulonephritis （CrGN） and other 11 cases （3.0％）.Two hundred and five cases（56.5％） showed simple NS and 64.9％ of them were MCD.One hundred and fifty-eight cases （43.5％） showed nephritic NS in which the most common pathological type was MsPGN （36.4％） in the second period and FSGS（39.6％） in the third period.There were 180 cases （49.6％） that frequently relapse with NS （FRNS）and 49（13.5％） steroid-dependent NS（SDNS）.MCD（62.2％） occupied the largest proportion of FR/SDNS,followed by MsPGN （13.3 ％） and FSGS （13.3 ％）.One hundred and seventy-two cases （47.4％） showed steroid resistant （SR）and 54.1％ （93/172 cases） of them were primary steroid resistant（PSR）.No significant difference was found between pathological type of PSRNS and secondary steroid resistant NS （SSRNS） （P ＞ 0.05）.The most common pathological type of SRNS was MsPGN （41.5％） in period 2 and FSGS （41.5％） in period 3.There was no immune complex present in 108 cases（29.8％） in which the main pathological type was MCD （63.9％）.Ninety-six cases （26.4％）mainly presented with IgM,of which 47.9％ were SRNS and 37.5％ were SD/FRNS.Conclusions The main pathological types are MCD,MsPGN and FSGS in children＇s PNS.There is a relationship between clinic and pathology.FSGS cases have been increasing while MsPGN cases have been decreasing in recent 20 years.It is advised to perform the renal biopsy early for nephritic NS,SD/FRNS and SRNS and repeat the biopsy if necessary.