表现为肾病综合征或大量蛋白尿的薄基底膜肾病

THIN BASEMENT MEMBRANE NEPHROPATHY WITH SEVEREPROTEINURIA OR NEPHROTIC SYNDROME

目的：了解薄基底膜肾病的临床病理特点。方法：通过透射电镜观察，图象分析仪测量基底膜的厚度，并结合临床资料，分析研究了５例表现为大量蛋白尿的薄基底膜肾病的临床病理特点。结果：５例病人临床均以大量蛋白尿伴镜下血尿为主要特点，光镜及免疫荧光检查无明显病理改变，电镜下基底膜弥漫性变薄伴上皮足突节段性或广泛性融合，是其主要的病理改变及诊断依据。结论：薄基底膜肾病临床上除多数表现为血尿外，也可表现为大量蛋白尿甚至肾病综合征。

Objective:To investigate the clinicopathological features of thin basement membrane nephropathy(TBMN). Methods: 5 patients of TBMN with severe proteinuria were studied by electron microscopy, image analysis combining with clinical data. Results: The main clinical manifestation of 5 patients was severe proteinuria with microscopic hematuria. There were no obvious pathological changes by light microscopy and immunofluorescence. By electron microscopy, thinning of glomerular basement membrane(GBM), with segmental or extensive fusion of epithelial foot processes, was its main pathological change and also diagnostic evidence. Conclusion: Although hematuria was its predominant findings, TBMN also manifested severe proteinuria, even nephrotic syndrome.