单中心儿童肾活检1579例临床和病理类型分析

摘要

Objective To investigate the clinical features and pathological characteristics as well as their associations with secular trend of the disease of 1 579 pediatric renal biopsies. Methods The clinical and pathological data were retrospectively analyzed in 1 579 children with renal diseases with biopsy performed from 1989 to 2012 in our hospital. The changes of clinical and pathological type distribution were characterized in every 5 years historically in different age groups ( - 1, -3, - 6, -12, -18 years ) and genders. Results ①The mean age of 1 579 children was ( 9. 3 ±3.2) years ( 4 months to 18 years ). The ratio of males to females was 1. 92. Primary glomerulonephritis ( PGN ), secondary glomerulonephritis ( SGN ) and inherited nephropathy accounted for 60. 1％ , 31. 2％ and 8. 3％ of the total cases. The PGN and SGN mainly included nephritic syndrome ( NS ), isolated hematuria, acute glomerulonephritis ( AGN ) and Henoch-schonlein purpura nephritis ( HSPN ), HBV-associatied glomerulonephritis( HBVGN ), lupus nephritis( LN ), respectively. The pathological classification of PGN mainly included minimal change disease/mild disease ( MCD/ML ), IgAN and mesangial proliferative glomerulonephritis ( MsPGN ). ② Male patients dominated in NS, HBVGN and Alport syndrome, however, female dominated in isolated hematuria, rapidly progressive glomerulonephritis( RPGN ), LN, ANCA-associated glomerulonephritis and thin basement membrane disease. The proportions of NS, isolated hematuria, AGN, CGN, HBVGN, LN and hemolytic uremic syndrome significantly changed with age. ③The clinical patterns of PGN were significantly correlated with the distribution of pathological types. The main pathological patterns of NS were MCD/ML, IgMN and IgAN. The main pathological patterns of hematuria were MCD/ML, IgAN and MsPGN. The main pathological patterns of hematuria with proteinuria were IgAN and MsPGN. The main pathological patterns of RPGN and chronic glomerulonephritis( CGN ) were crescentic glomerulonephritis( CreGN ) and sclerosing glomerulonephritis( SGN ), respectively. ④ The spectrum of NS, HSPN, HBVGN, IgAN was changed in 23 years. Conclusions There were some relationships between the clinical and pathological features to gender and age in children. Renal biopsy is valuable in the diagnosis and treatment in children with renal diseases.%目的 了解肾穿刺患儿病理特点及其与临床表现的关系,以及疾病谱变迁.方法 调取华中科技大学同济医学院附属同济医院儿科1989至2012年行肾活检病例的临床分类和病理学分型资料,依据年龄(<1、～3、～6、～12和～18岁)和性别分组进行构成比的比较和分析,以2001年为时间节点分为2个阶段分析疾病谱的变化趋势.结果 1 579例肾活检患儿进入分析,平均肾穿刺年龄(9.3±3.2)岁,男女比例1.92:1.①肾活检患儿中原发性肾小球疾病949例(60.1%),继发性肾小球疾病493例(31.2%),遗传性肾脏疾病130例(8.2%);原发性肾小球疾病中肾病综合征、单纯血尿、急性肾炎分别占44.8%、26.2%和15.3%;继发性肾小球肾炎中紫癜性肾小球肾炎、HBV相关性肾炎(HBVGN)和狼疮性肾炎(LN)分别占55.4%、22.9%和18.4%;遗传性肾脏疾病中薄基底膜病和Alport综合征分别占50.0%和46.2%.②949例原发性肾小球疾病的病理类型以轻微病变/微小病变(24.8%)、IgA肾病(21.0%)和系膜增生性肾小球肾炎(19.1%)为主;女性新月体性肾小球肾炎构成比显著高于男性.③肾病综合征构成比随年龄增长呈逐渐减少趋势,单纯血尿构成比在～12岁组最高(31.0%),急性肾炎和慢性肾炎构成比随年龄增长呈逐渐增加趋势;HBVGN主要分布于～3岁组(71.4%),构成比随年龄增长而呈下降趋势.LN主要见于～18岁组,溶血尿毒综合征主要分布于～3岁和～6岁组.④原发性肾小球疾病主要病理类型:肾病综合征为微小病变/轻微病变(31.1%),急性肾炎为毛细血管内增生性肾小球肾炎(28.3%),慢性肾炎为硬化性肾炎(59.4%).⑤2002至2012年肾病综合征、紫癜性肾炎、IgA肾病构成比较1989至2001年显著增高,急性肾炎、HBVGN构成比显著下降.结论 肾活检患儿肾小球疾病临床和病理类型与年龄、性别有一定相关性,23年间某些肾小球疾病的构成比发生变化.