从复旦大学附属儿科医院31年肾脏病理及临床资料反思肾活检指征

目的 了解复旦大学附属儿科医院肾脏风湿科肾脏疾病的病理学类型及临床特点，并对肾活检指征进行反思。方法 回顾性分析1979至2009年肾活检病理学分型和临床资料，并以10年为一个间期分3个阶段进行分析比较。结果 31年中肾活检1 633例，其中1 419例满足入选条件进入分析。①原发性、继发性和遗传性肾脏疾病分别占63.9%（907例）、23.2%（329例）和12.1%（172例）。②原发性肾脏疾病中，IgA肾病（26.6%，241/907例）、微小病变病（23.0%，209/907例）和轻微病变（18.1%，164/907例）所占比例较高，局灶节段肾小球硬化仅占3.0%（27/907例）；继发性和遗传性肾脏疾病分别以紫癜性肾炎（47.1%，155/329例）和薄基膜肾病（80.8%，139/172例）所占比例最高。③31年间肾活检病理学类型构成比的变化趋势为IgA肾病、轻微病变和紫癜性肾炎在各阶段所占比例逐渐增加，系膜增生性肾小球肾炎及HBV相关性肾炎所占比例逐渐减少。④肾活检临床表现以血尿（38.8%，551/1 419例）和原发性肾病综合征（30.9%，439/1 419例）多见。血尿中单纯性镜下血尿占14.5%（206/1 419例），其病理类型主要为薄基膜肾病（52.9%，109/206例）和轻微病变（23.3%，48/206例）；原发性肾病综合征初发为单纯性肾病的患儿中激素依赖及频复发占11.1%（157例），其病理学类型主要为微小病变（61.8%，97例）和轻微病变（17.2%，27例）。结论 肾活检病理学的构成比中仍以原发性肾脏疾病多见，主要为IgA肾病和微小病变，临床表现以血尿和原发性肾病综合征为主。肾活检对于单纯性镜下血尿患儿意义相对有限，临床上可密切随访尿蛋白和尿微量蛋白，如有异常再考虑行肾活检。而对于原发性肾病综合征激素依赖及频复发，特别是初发表现为单纯性肾病的患儿，临床上应关注糖皮质激素的不良反应，如出现严重不良反应，需加用免疫抑制剂特别是环孢素A和他克莫司前应考虑行肾活检。

Time to reflect our renal biopsy indications: a thirty-one-year review of renal biopsy histopathology

Objective To identify the patterns of peadiatric renal biopsy histopathology and its variation in the past 31 year in a single center and implication on modification to the clinical indications. Methods A retrospective study was done on all renal biopsies performed from January 1979 to December 2009. All the cases were recruited consecutively from Department of Nephrology and Rheumatism in Children's Hospital of Fudan University and were categorized into 3 periods: period Ⅰ (1979 to 1989), period Ⅱ (1990 to 1999) and period Ⅲ (2000 to 2009). Results A total of 1 419 renal biopsies were performed in 31 years. Mean age was (8.08±3.46) years (6 months-18 years). Major clinical presentations were haematuria (38.8%, 551/1 419), followed by primary nephrotic syndrome (30.9%, 439/1 419) and renal manifestations secondary to systemic diseases (23.8%, 338/1 419). Primary glomerulonephritis (PGN) accounted for 63.9% (907/1 419) of the total patients, secondary glomerulonephritis (SGN) 23.2% (329/1 419) and hereditary glomerulonephritis (HGN) 12.1% (172/1 419). Common causes of PGN were IgAN (26.6%, 241/907) (98 out of 241 were diffuse proliferative type) and MCD (23.0%, 209/907) (120 out of 209 with IgM deposition). FSGS only accounted for 3.0% (27/907). The percentage of IgAN was relatively high and that of FSGS was low. In SGN, HSN (47.1%, 155/329) (72 out of 155 were focal segmental proliferative type) ranked first and followed by LN (28.6%, 94/329) (43 out of 94 were diffuse proliferative LN). In HGN, thin basement membrane nephropathy (TBMN) accounted for 80.8% (139/172) and Alport syndrome accounted for 17.4% (30/172). During 31 years, the composition of PGN decreased while that of HGN increased MsPGN and HBV-GN were on the top in period Ⅰ and decreased in period Ⅱ and Ⅲ. IgAN and HSN had a rise in composition. Most haematuria with proteinuria patients had IgAN (42.6%, 84/197) while most isolated microscopic haematuria had TBMN (52.9%, 109/206). Most primary nephrotic syndrome patients especially steroid dependent and frequently relaps (SDFR) had MCD (61.8%, 97/157). Conclusions It is time for us to reflect the renal biopsy indication. For SDFR, the renal biopsy should be performed for those who are starting to use immunosuppressive drugs such as cyclosporin A or tacrolimus. The indication of renal biopsy for isolated microscopic hematuria should be stricter.