膜性肾病合并薄基底膜肾病患者临床病理分析  

Idiopathic membranous nephropathy in association with thin basement membrane nephropathy： A clinico- pathological study

作　　者：刘林昌[1] 王素霞[1] 章友康[1] 才智勇[1] 郑欣[1] 刘刚[1] 柴立军[1] 李月红[2] 邹万忠[1] 

机构地区：[1]北京大学第一医院肾内科北京大学肾脏病研究所,北京100034 [2]天津市南开医院肾内科

出　　处：《临床肾脏病杂志》2010年第7期304-307,F0002,共5页Journal Of Clinical Nephrology

基　　金：基金项目：国家自然科学基金（30670982）

摘　　要：目的探讨膜性肾病（MN）合并薄基底膜肾病（TBMN）的临床病理特点。方法选择我院经肾脏活体组织检查（简称：肾活检）确诊为I期MN合并TBMN（I期MN-TBMN）患者7例，分析其临床病理特点，并与同期的I期MN及单纯的TBMN进行比较。结果7例I期MN—TBMN患者占同期所有I期MN的1．49％（7／471例），均表现为持续性变形性镜下血尿、轻中度蛋白尿[（2．09±0．78）g／24h]和正常肾功能[SCr（65．30±14．09）／2mol／L]；电镜超微结构显示肾小球基底膜（GBM）弥漫性变薄[GBM厚度（205．96±45．94）nm]伴上皮下块状电子致密物沉积，免疫荧光显示Ⅳ型胶原a3、a5链呈线条样沿GBM分布与正常肾组织相同。与同期原发性I期MN组相比，I期MN-TBMN组血尿发生率显著升高（P=0．015），蛋白尿程度显著降低（P=0．019）。随访时间（22．3±16．4）个月，I期MN—TBMN组较I期MN组血白蛋白水平显著增高（P=0．045），尿蛋白阴性患者比例显著增高（P＝0．017）。结论少数原发性I期MN可与TBMN同时存在，I期MN-TBMN与原发性I期MN相比较血尿发生率升高，蛋白尿程度降低，临床相对较轻，预后相对较好。Objective To investigate the clinical-pathological characteristics of idiopathic membranous nephropathy（MN）in association with thin basement membrane nephropathy（TBMN）. Methods Renal biopsies collected from the files of our institute between Jan. 2003 to Dec. 2009 were investigated. Seven patients were diagnosed as stage I MN coexisted with TBMN（ stage I MN- TBMN）. The elinico-pathological features of patients with stage I MN-TBMN were analyzed and compared with those of patients with stage I MN （13 cases）and TBMN （14 cases） respectively. Results Stage I MN-TBMN was found in 7（1.49%）of 471 cases of native stage I MN,including 1 male and 6 females. Persistent microscopic hematuria was found in all patients, the level of urine protein was（2. 09 ± 0. 78）g/24 h,and serum creatinine was（65. 30± 14. 09） μmot/L. Under the electron microscopy, diffuse thinning of glomerular basement membrane （GBM） with a thickness of（205. 96 ± 45. 94）nm and subepithelial deposits were identified in stage I MN-TBMN. The distribution of collagen IV a3, a5 chains in these patients was the same as that in normal renal tissue Patients with stage I MN-TBMN had a higher frequency of hernaturia and lower degree of proteinuria than those with stage I MN （13 cases） （P〈0. 05）. After a follow-up period of （22. 3 ± 16. 4）months, the serum albumin level in stage I MN-TBMN group was significantly higher than in stage I MN group（P = 0. 045）, the percent of negative proteinuria （〈0. 15 g/24 h）in stage I MN-TBMN group（4/6 cases, 66. 7%） was higher than in stage I MN group（1/13 cases,7. 69%）. Conclusions A few of stage I MN could coexist with TBMN. Patients with stage I MN-TBMN showed a higher frequency of hematuria and lower degree of proteinuria than those with stage I MN. It seemed that there was a better prognosis of stage I MN-TBMN than stage I MN.

关 键 词：肾病 膜性 肾小球基底膜 显微镜检查 电子 

分 类 号：R735.2[医药卫生—肿瘤]