薄基底膜肾病临床和病理的相关性研究

The Relationship between the Clinical Symptoms and Pathology of Thin Basement Membrane Nephropathy

目的探讨薄基底膜肾病(TBMN)的临床表现和病理特点以及二者的相关性.方法选取42例经肾穿刺病理检查确诊为TBMN的患者,对其临床及病理资料进行分析.结果 42例患者中,28例临床表现为单纯性血尿,6例血尿合并有少量蛋白尿,4例少量血尿合并有中等量蛋白尿,4例以肾病综合征为表现.肾组织光学显微镜检:42例患者中17例表现为肾小球系膜轻度增生,11例为局灶肾小球系膜增生,5例为毛细血管内增生,8例为肾小球轻微病变,1例为肾小球局灶硬化.免疫荧光检查:26例患者免疫荧光阴性,14例IgM±～+,2例IgM++.电子显微镜下42例患者肾小球基底膜均弥漫性变薄,厚度均<250 nm.血尿、蛋白尿的严重程度与肾小球基底膜厚度呈负相关(r分别为-0.92、-0.98,P<0.01)结论 TBMN临床上多表现为单纯性血尿,也可伴蛋白尿,少数以肾病综合征为表现.确诊有赖于肾活检电镜下肾小球基底膜厚度的测定,研究TBMN的临床特点及病理改变的相关性后发现,其血尿、蛋白尿的严重程度与肾小球基底膜厚度呈负相关,而病程、红细胞变形率与肾小球基底膜厚度无明显相关关系.

Objective To explore the clinical presentations and pathological figures of the Thin Basement Membrane Nephropathy（TBMN） and the relationship between the them.Method 42 cases of TBMN which were diagnosed with renal biopsy were selected,and the clinical and pathological data were analyzed.Results In 42 cases of TBMN,there were 28 cases of isolated hematuria,6 cases of hematuria associated with a small amount of proteinuria,4 cases of a small amount of hematuria associated with a moderate amount of proteinuria,4 cases of nephrotic syndrome.The microscopic examinations showed that 17 cases with glomerulose mensangial slight hyperplasia,11 cases with focal glomerulose mensangial hyperplasia,5 cases with intercapillary hyperplasia,8 cases with slight glomerulopathy,and 1 case with glomerulose focal cirrhosis.The immunofluorescence examination indicated that 26 cases with negative immunofluorescence,14 cases with feeble positive immunofluorescence IgM, and 6 case with strong positive IgM.All the GBM width became diffusely thin,and the average GBM was less than 250nm.The severe degree of haematuria and albuminuria was negatively correlated with the thickness of renal glomerular basement membrane（r =-0.92,-0.98,P 0.01）.Conclusions The common clinical presentations of thin basement membrane nephropathy are isolated hematuria,some accompanied with proteuria, few patients presented as nephropathy syndrome.The diagnosis depends on the GBM thickness determination through electromicroscope.The severe degree of haematuria and albuminuria is negatively correlated with the thickness of renal glomerular basement membrane,there is no obvious correlation between the course of disease,red blood cell dysmorphosis rate and the GBM sickness.