【摘要】 目的探讨薄基底膜肾病(TBMN)的临床表现和病理特点。方法对54例临床表现为孤立性血尿、经肾活检证实的TBMN患儿进行分析。所有患儿行肾穿刺,分别进行光镜、免疫荧光和电镜观察及基底膜厚度测定。采用间接免疫荧光法检测皮肤基底膜(EBM)Ⅳ型胶原α1、α5和肾小球基底膜(GBM)Ⅳ型胶原α1、α3和α5链表达。结果所有病例肾小球基底膜弥漫性均匀性变薄,无基底膜分层、撕裂等改变,部分病例存在节段性足突消失。4段基底膜厚度分别为(170．7±45．0)nm、(175．6±47．9)nm、(181．8±36．3)nm和(170．0±32．9)nm。EBM和 GBMⅣ型胶原α链检测,发现异常2例。结论薄基底膜肾病主要表现为孤立性血尿,确诊有赖肾活检电镜基底膜厚度测定,须与Alport综合征鉴别。更多还原

【Abstract】 Objective To explore the clinical manifestation and pathological characteristics of thin basement membrane nephropathy (TBMN) confirmed by renal biopsy in 54 patients who manifested with isolated hematuria. Methods Renal biopsy was carried out in all patients (20 males and 34 females) aging between 3-16 years. Thirty-two of them had family history. The average course of disease before biopsy was 2.3 years. Specimens obtained by nephrocentesis in all patients were examined by light microscopy, immunofluorescence and electron microscopy, respectively. The thickness of glomerular basement membrane (GBM) was measured under electron microscopy. The synthetic levels of α1 and α5 chains for epidermal basement membrane (EBM) type IV collagen and α1,α3,α5 chain for glomerular basement membrane (GBM) type IV collagen were detected by indirect immunofluorescence. Results The diffusely and uniformly thinned GBM was seen in all patients without lamellation and segment, but segmental effacement of podocyte existed in some of them. The thickness of four parts of basement membrane was 170. 7±45.0, 175.6±47.9, 181.8±36.3 and 170.0±32.9 nm. Three patients showed abnormal a chain of type IV collagen on both EBM and GBM. Patients were followed up for 2 months to 3 years. Hematuria disappeared in 5 of them, and there was a conversion from continuous hematuria to intermittent hematuria in other 3 cases. The hematuria persisted in the remaining patients but with a stable renal function and a normal blood pressure. Conclusions It was suggested that isolated hematuria is the main manifestation of TBMN, and renal biopsy and measurement of the thickness of GBM under electron microscopy should be necessary for the diagnosis of TBMN. However, the differential diagnosis with Alport syndrome is indispensable.更多还原