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目的 :检测肾病综合征 (NS)患儿肾小球足细胞中nephrin、podocin、α actinin及WT1的表达和分布特征 ,以探讨这些分子在蛋白尿发生中的可能作用。 方法 :用免疫荧光染色 ,激光扫描共聚焦显微镜采集图像及图像分析的方法 ,检测 2 5例NS患儿 [其中肾脏病理为微小病变 (MCD) 2例 ,系膜增生性肾小球肾炎 (MsPGN) 17例 ,局灶节段性肾小球硬化 (FSGS) 5例 ,新月体性肾小球肾炎 (CREGN) 1例 ]、9例单纯性血尿患儿及 9例对照肾组织中nephrin、podocin、α actinin及WT1的表达。 结果 :(1)NS患儿肾小球中podocin的表达量 (82 9± 2 1 5 )与单纯性血尿组 (10 3 6± 2 0 2 )及对照组 (110 8± 15 4 )比较差异显著 (P =0 0 12 ,P =0 0 0 1) ;WT1的表达量 (6 1 3± 9 2 3)与对照组 (6 9 7± 9 2 7)比较差异也显著 (P =0 0 37) ;nephrin和α actinin表达量 (12 9 3± 2 2 6 ,10 4 3± 19 0 )与单纯性血尿组(132 1± 7 4 0 ,94 9± 13 0 )及对照组 (133 9± 8 5 ,10 3 6± 15 0 )比较差异均不显著 (P >0 0 5 )。 (2 )对照组肾组织中nephrin和podocin的染色均见于肾小球毛细血管壁 ,呈均匀、线状分布 ;α actinin主要沿肾小球毛细血管壁呈点状分布 ;WT1的染色主要集中于肾小球细胞核。 (3)病理表现为MsPG
Objective: To investigate the expression and distribution of nephrin, podocin, α actinin and WT1 in glomerular podocytes of children with nephrotic syndrome (NS) to explore the possible role of these molecules in the pathogenesis of proteinuria. Methods: Immunofluorescence staining, laser scanning confocal microscope acquisition of images and image analysis methods were used to detect 25 cases of NS children [including 2 cases of small pathological renal disease (MCD), mesangial proliferative glomerulonephritis (MsPGN ) In 17 cases, 5 cases of focal segmental glomerulosclerosis (FSGS), 1 case of crescentic glomerulonephritis (CREGN)], 9 cases of simple hematuria and 9 cases of control renal tissue nephrin, podocin, α actinin and WT1 expression. Results: (1) The expression of podocin in glomeruli of NS children (82 9 ± 2 1 5) was significantly lower than that of simple hematuria group (103 6 ± 202) and control group (110 8 ± 15 4) (P = 0 0 12, P = 0 0 0 1). The expression level of WT1 (6 1 3 ± 9 2 3) was also significantly different from that of the control group (6 9 7 ± 9 2 7) 37). The expression levels of nephrin and α actinin were significantly higher in simple hematuria group (132 1 ± 7 4 0, 94 9 ± 13 0) than those in control group (12 9 3 ± 2 2 6, 10 4 3 ± 19 0) ± 8 5, 10 3 6 ± 15 0) (P> 0.05). (2) The nephrin and podocin staining in the control group were found in the glomerular capillary wall, showing a uniform, linear distribution; α actinin mainly along the glomerular capillary wall was dotted distribution; WT1 staining mainly concentrated in Glomerular nucleus. (3) pathological manifestations of MsPG