Huang Jian-ping, Zhang Jing-jing, Liu Jing-cheng, Chen Ying-nan, Yao Yong, Xiao Hui-jie, Yang Ji-yun
Department of Pediatrics, Peking University First Hospital, Beijing 100034, China.
Zhonghua Er Ke Za Zhi. 2004 Jul;42(7):516-9.
To investigate the clinical and pathological characteristics of focal segmental glomerulosclerosis (FSGS) in children.
The data of 38 children,aged from one and half to 15 years, 25 boys and 13 girls, with primary FSGS were studied retrospectively.
Majority of the cases in this study were school-aged children. The average age of initial onset was 8.9 +/- 3.68 years. The ratio of boys to girls was 1.92. The clinical manifestation included isolated proteinuria in 3 cases, proteinuria and hematuria in 1 and nephrotic syndrome in 34 (simple type in 16 and nephritic type in 18). Of 38 cases, 24 (63%) presented with hematuria, 11 (29%) with hypertension and 7 (18%) with decreased creatinine clearance. The pathologic classification included perihilar variant in 17 cases, peripheral variant in 14 and tip variant in 7. The predominant clinical feature of children with tip variant was simple type of nephrotic syndrome (86%). Microscopic hematuria was not common (29%). Blood pressure and renal function were normal. The children with diffuse mesangial hypercellularity superimposed on changes of FSGS (in 21 of 38 cases) were more likely to have hematuria (76%) and less simple nephrotic syndrome (30%). The initial treatment response to prednisone in 34 cases with nephrotic syndrome showed sensitive in 12 cases, resistant in 21 and unknown in 1. Transition from sensitive to resistant occurred in six of 12 children. Three of 4 cases with non-nephrotic syndrome showed no response and the remaining one had unknown response. It was found that 44% of children who received cyclophosphamide and 83% of children who received pulse methylprednisolone and pulse cyclophosphamide or cyclosporin A in addition to oral steroids had complete or partial remission. Correlation analysis showed that the level of proteinuria after treatment was correlated directly with renal tubulointerstitial lesion and renal function (Pr = 0.48, P < 0.05; Pr = 0.45, P < 0.05).
FSGS was common in school-aged children. The predominant presenting feature was nephrotic syndrome. Hematuria was common. Hypertension and renal insufficiency were less frequently seen. The renal biopsy showed multiple variants. Pulse methylprednisolone and pulse cyclophosphamide or cyclosporin A treatments showed relatively good response.
探讨儿童局灶节段性肾小球硬化（FSGS）的临床及病理特征。
回顾性分析38例年龄在1.5至15岁之间的原发性FSGS患儿的数据，其中男25例，女13例。
本研究中大多数病例为学龄儿童。平均起病年龄为8.9±3.68岁。男女比例为1.92。临床表现包括3例孤立性蛋白尿，1例蛋白尿和血尿，34例肾病综合征（单纯型16例，肾炎型18例）。38例中，24例（63%）出现血尿，11例（29%）出现高血压，7例（18%）肌酐清除率下降。病理分类包括17例系膜周围型，14例外周型，7例顶端型。顶端型患儿的主要临床特征为单纯型肾病综合征（86%）。镜下血尿不常见（29%）。血压和肾功能正常。38例中有21例在FSGS改变基础上合并弥漫性系膜细胞增生，这些患儿更易出现血尿（76%），单纯肾病综合征较少（30%）。34例肾病综合征患儿对泼尼松的初始治疗反应显示，12例敏感，21例耐药，1例反应不明。12例敏感患儿中有6例转为耐药。4例非肾病综合征患儿中3例无反应，其余1例反应不明。发现接受环磷酰胺治疗的患儿中有44%，接受静脉注射甲泼尼龙及静脉注射环磷酰胺或环孢素A加口服类固醇治疗的患儿中有83%达到完全或部分缓解。相关性分析显示，治疗后蛋白尿水平与肾小管间质病变及肾功能直接相关（Pr = 0.48，P < 0.05；Pr = 0.45，P < 0.05）。
FSGS在学龄儿童中常见。主要表现为肾病综合征。血尿常见。高血压和肾功能不全较少见。肾活检显示多种类型。静脉注射甲泼尼龙及静脉注射环磷酰胺或环孢素A治疗反应相对较好。
