儿童肾病综合征肾组织病理免疫荧光分型和病理类型与激素疗效关系

Pathological patterns of renal biopsy with immunofluorescence relate to the therapeutic effects of corticosteroid in childhood nephrotic syndrome

目的分析肾组织病理免疫荧光分型和病理类型及激素疗效的相关性。方法回顾性分析1990年1月至2015年12月临床诊断为肾病综合征(NS)并行肾穿刺活检患儿的肾组织病理及临床随访资料。结果 252例NS患儿的年龄分布为0.8~15岁,男女比例为3.94:1。肾组织病理免疫荧光类型分布为:IgA为主型13例(5.16%),Ig M为主型78例(30.95%),补体为主型19例(7.54%),IgG+A+M型17例(6.75%),Ig G+A+M+补体型22例(8.73%),IgA+补体型2例(0.79%),Ig M+补体型55例(21.83%),无免疫复合物型46例(18.25%);肾组织病理类型中轻微病变(MCNS)157例(62.3%),系膜增生性肾小球肾炎(Ms PGN)35例(13.89%),局灶节段肾小球硬化(FSGS)60例(23.81%)。使用激素4周内完全缓解167例(77.31%),部分缓解31例(14.35%),激素无效18例(8.33%)。8种不同病理免疫荧光分型患儿的病理类型分布差异有统计学意义(χ~2=31.308,P=0.001),但激素疗效差异无统计学意义(P>0.05)。结论不同病理免疫荧光分型的病理类型分布有差异,提示免疫荧光沉积情况对NS临床评估可能有一定价值。

Objective To explore the relationship of pathological patterns of renal biopsy with immunofluorescence the therapeutic effects of corticosteroid in childhood nephrotic syndrome. Method Renal biopsy materials and clinical data of the children diagnosed with nephrotic syndrome in the Department of Pediatric Nephrology of Shanghai Xinhua Hospital from January, 1990 to December, 2015 were retrospectively reviewed and analyzed. Results The renal pathological patterns by immunofluorescence of 252 patients showed that IgA was presented in 13 cases (5.16%), IgM in 78 cases(30.95%), complement in 19 cases (7.54%), IgG+A+M in 17 cases(6.75%), IgG+A+M+complement in 22 cases (8.73%),IgA+complement in 2 cases (0.79%), IgM+complement in 55 cases (21.83%), and there was no immune complex present in 46cases (18.25%). The renal pathological patterns were found that 157 (62.3%) minimal change nephropathy syndrome (MCNS),35 (13.89%) mesangioproliferative glomerulonephritis (MsPGN), 60 (23.81%) focal and segmental glomerulosclerosis (FSGS).In the 4 weeks of oral administration of prednisone, complete remission was achieved in 167 cases (77.31%), partial remission was achieved in 31 cases (14.35%), and no remission was shown in 18 cases (8.33). There was statistically difference in the distribution of renal pathological patterns among the 8 patterns by immunofluorescence (χ2=31.308,P=0.001). The therapeutic effects of corticosteroid on the 8 patterns by immunofluorescence have no significant difference (P>0.05). Conclusions The distribution of renal pathological patterns among the 8 patterns of immunofluorescence is significantly different.