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Alport综合征患者肾小球基底膜超微结构改变与层粘连蛋白的异常分布
其他题名Correlation between ultrastructural changes of glomerular basement membrane and abnormal distribution of laminins in patients with Alport' s syndrome
黄巍; 王晨; 郑欣; 鄂洁; 王素霞; 刘刚
关键词肾炎 遗传性 肾小球基底膜 层粘连蛋白 Glomerular Basement Membrane Nephritis Laminin Hereditary
刊名北京大学学报(医学版)
2009
DOI10.3969/j.issn.1671-167X.2009.06.004
41期:6页:630-634
文章类型Journal Article
摘要目的:观测Alport综合征患者肾小球基底膜(GBM)超微结构改变与GBM上层粘连蛋白α1(laminin α1)和α5(laminin α5)分布的关系.方法:1998年1月至2008年7月在北京大学第一医院经肾活检病理确诊为Alport综合征的患者20例,另外选择6例肾肿瘤切除术后患者的正常肾组织作为对照.在透射电镜下测量GBM厚度以及增厚和撕裂的范围.对肾组织石蜡切片进行laminin αl、laminin α5免疫组织化学染色,对其在GBM的分布进行半定量评分:0分为0%,1分为≤25%,2分为25%~50%,3分为50%~75%,4分为≥175%.结果:透射电镜下观察Alport综合征患者的GBM均出现不同程度的增厚和撕裂分层.免疫组织化学显示,正常对照组的laminin α1主要在肾小球系膜区,GBM无显色;laminin α5则沿着GBM均匀显色.Alport患者的laminin α1在肾小球系膜区的分布低于对照组,主要在GBM上显色;laminin α5在GBM上的分布低于对照组;GBM上laminin α1与laminin α5半定最评分呈高度负相关(r=-0.83,P<0.001).GBM增厚及撕裂范围与laminin α1的半定量评分均呈正相关(分别为r=0.76,P<0.001;r=0.56,P=0.015),与laminin α5的半定量评分均呈负相关(分别为r=-0.59,P=0.010;r=-0.53,P=0.025).结论:Alport综合征患者GBM中异常分布的laminin α1和laminin α5与GBM增厚和撕裂的超微病理改变有关. Objective:To analyse the relationship of ultrastructural changes of glomerular basement membrane (GBM) and glomerular distributions of laminin α1 and laminin α5 in patients with Alport' s syndrome. Methods: Twenty patients with Alport' s syndrome were recruited. The thickness of GBM and the extension of thickening and splitting GBM were measured under transmission electron microscope. Normal renal tissues from 6 nephrectomies of renal carcinoma were taken as controls. Paraffin embedded sections of formalin-fixed renal tissue were processed for immunohistochemistry with monoclonal antibodies to laminin α1 and laminin α5. Their distributions in GBM were evaluated by a semiquantitative scale of positive extension; absent, 0≤25% , 1; 25%-50% , 2; 50%-75% , 3;≥75% , 4. Results: There were a variety of degrees of thickening or splitting GBM in patients with Alport' s syndrome. Laminin al was positive in glomerular mesangial area and absolutely negative in GBM and laminin α5 was evenly positive in GBM in normal tissue. In Alport' s syndrome, laminin α1 was much weaker in glomerular mesangial area, but strongly positive in GBM; laminin α5 in GBM was prominently reduced. There was a high negative correlation of semiquantitative scores between laminin al and laminin α5 (r =-0. 83, P<0. 001). The extension of thickening or splitting GBM was positively correlated with scores of laminin al in GBM ( r = 0. 76, P<0.001; r = 0. 56, P=0. 015 ) , and was negatively correlated with scores of laminin α5 in GBM ( r =-0. 59, P =0. 010; r=-0. 53, P =0.025). Conclusion: Abnormal distribution of laminin al and laminin α5 in GBM is correlated with GBM thickening and splitting in human Alport' s syndrome.
语种中文
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文献类型期刊论文
条目标识符http://ir.bjmu.edu.cn/handle/400002259/45850
Collection北京大学第一临床医学院_肾脏内科
作者单位北京大学第一医院肾内科,北京大学肾脏病研究所,北京,100034
Recommended Citation
GB/T 7714
黄巍,王晨,郑欣,等. Alport综合征患者肾小球基底膜超微结构改变与层粘连蛋白的异常分布[J]. 北京大学学报(医学版),2009,41(6):630-634.
APA 黄巍,王晨,郑欣,鄂洁,王素霞,&刘刚.(2009).Alport综合征患者肾小球基底膜超微结构改变与层粘连蛋白的异常分布.北京大学学报(医学版),41(6),630-634.
MLA 黄巍,et al."Alport综合征患者肾小球基底膜超微结构改变与层粘连蛋白的异常分布".北京大学学报(医学版) 41.6(2009):630-634.
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