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目的观察乙型肝炎病毒相关性膜性肾病(HBV-MN)患儿肾小球足细胞数量的变化及意义。方法以 Wilms 瘤蛋白1(WT1)为足细胞核的特征性标记,通过免疫组织化学方法对19例经肾穿刺活检确诊为 HBV-MN 患儿及8例同期肾活检诊断为薄基底膜病(TBMD)的患儿(对照组)肾脏病理切片的足细胞进行特异性染色,采用 Weibel-Gomez 点计数方法测定单位肾小球足细胞绝对数目(Npodo)、足细胞相对密度(Nv)及单个肾小球毛细血管簇体积(V),比较两组间差异,并分析其与血清免疫学检查、蛋白尿程度及肾组织病理改变等指标间的关系。结果①19例 HBV-MN 患儿的超微病理结果均显示存在不同程度的足突融合,3例有足突微绒毛化,4例出现足细胞肿胀,1例足细胞空泡变性,2例可见足细胞崩解;②HBV-MN 患儿 Npodo 及 Nv 均较对照组显著下降(P<0.01);③ Npodo 的减少与肾小球中 HBsAg 的沉积强度相关,HBsAg 免疫荧光强度≥(++)患儿的平均 Npodo 明显低于荧光强度<(++)者(p<0.01);但 Npodo 及 Nv 的下降均与肾脏病变病理分期无显著相关性;④Nv 减少与蛋白尿程度相关,24 h尿蛋白定量<2 g患儿的 Nv 明显高于>2g 者(P<0.05);⑤肾小球足细胞数量与血清补体 C_3,水平呈正相关(r=0.548,P=0.028),而与血清白蛋白水平无显著相关性(r=-0.037,P=0.891)。结论 HBV-MN 患儿均存在不同程度的足细胞损伤、缺失,Npodo 减少可能在 HBV-MN 的发病中起重要作用。
Objective To observe the changes of glomerular podocyte in children with hepatitis B virus associated nephropathy (HBV-MN) and its significance. Methods Wilms tumor cell line 1 (WT1) was used as a marker of podocyte nucleus. Nineteen cases of HBV-MN diagnosed by renal biopsy and 8 cases of simultaneous renal biopsy were diagnosed as thin basement membrane disease (TBMD) by immunohistochemistry (Control group), and Weibel-Gomez point counting method was used to determine the absolute number of glomerular podocytes (Npodo), podocyte relative density (Nv) and single kidney size The volume of capillary capillaries (V) was compared between the two groups, and the relationship between them was analyzed with serum immunological examination, proteinuria and renal pathological changes. Results ① The pathological findings of 19 cases of HBV-MN children showed different degrees of foot process fusion, 3 cases of foot process microvilli, 4 cases of podocyte swelling, 1 cases of podocyte degeneration, 2 cases were seen Podocyte disintegration; ②Npodo and Nv in HBV-MN children were significantly lower than those in the control group (P <0.01); ③The decrease of Npodo was correlated with the deposition intensity of HBsAg in glomerulus, and the immunofluorescence intensity of HBsAg ≥ (P <0.01). However, the decrease of Npodo and Nv was not significantly correlated with the pathological stage of nephropathy. (4) The decrease of Nv was correlated with the level of proteinuria. The 24 h urinary protein There was a positive correlation between the number of glomerular podocyte and the level of serum complement C_3 (r = 0.548, P = 0.028), but not with serum albumin There was no significant correlation (r = -0.037, P = 0.891). Conclusion There are varying degrees of podocyte injury and loss in children with HBV-MN. The decrease of Npodo may play an important role in the pathogenesis of HBV-MN.