Ⅲ型胶原肾小球病2例报道及文献复习  

Two cases of TypeⅢcollagen glomerulopathy and literature review

作　　者：喻芳 朱雪婧[1] 袁曙光[1] 龚再良 许向青[1] 刘虹[1] 李军[1] 孙林[1] 刘伏友[1] YU Fang;ZHU Xuejing;YUAN Shuguang;GONG Zailiang;XU Xiangqing;LIU Hong;LI Jun;SUN Lin;LIU Fuyou(Department of Nephrology,Second Xiangya Hospital,Central South University,Institute of Nephrology,Central South University,Changsha 410011;Department of Nephrology,Xiangya Changde Hospital,Changde Hunan 415000,China)

机构地区：[1]中南大学湘雅二医院肾内科,中南大学肾脏病研究所,长沙410011 [2]湘雅常德医院肾内科,湖南常德415000

出　　处：《中南大学学报（医学版）》2020年第7期869-873,共5页Journal of Central South University ：Medical Science

基　　金：国家自然科学基金(81300600);湖南省自然科学基金(2018JJ3728,2018JJ2596);中南大学自由探索计划(2012QNZT146)。

摘　　要：回顾性分析2例Ⅲ型胶原肾小球病患者,临床表现为肾病综合征、蛋白尿、高血压及肾功能减退。1例患者行基因检测,结果显示补体因子H相关蛋白5(complement factor H-related protein 5,CFHR5)基因疑似发生致病突变。肾组织病理检查显示系膜基质增生、内皮插入伴基底膜双轨形成。免疫组织化学显示Ⅲ型胶原阳性。电子显微镜显示大量40~70 nm胶原沉积。1例应用血管紧张素Ⅱ受体阻滞剂(angiotensinⅡreceptor blocker,ARB)治疗,肾功能维持正常,尿蛋白稳定在轻到中度水平;1例因其他系统疾病并发急性肾损伤后行血液透析。Ⅲ型胶原肾小球病临床表现不典型,光镜病理表现多样,主要通过电子显微镜及免疫组织化学检查确诊。In this paper,2 cases of collagen TypeⅢglomerulopathy were analyzed.The clinical manifestations mainly included nephrotic syndrome,proteinuria,hypertension and renal dysfunction.One patient showed that the complement factor H-related protein 5(CFHR5)gene was likely a disease-causing mutation.The pathological examination of renal tissues showed hyperplasia of mesangial matrix,sub-endothelial insertion,and double-track formation.Immunohistochemistry of TypeⅢcollagen was positive.Electron microscopy revealed that massive collagen fibers(40-70 nm in diameter)deposited in the mesangial matrix and basement membrane.As for the follow-up results,the normal renal function had kept steady and the proteinuria was moderate in 1 case treated with angiotensinⅡreceptor blocker.Due to other system disease,another case developed into acute kidney injury and then received hemodialysis.The clinical manifestations of collagen TypeⅢglomerulopathy was atypical,the light microscope pathological features were various,and the disease was mainly diagnosed by electron microscopy and immunohistochemistry.

关 键 词：Ⅲ型胶原肾小球病 肾组织病理学 免疫组织化学 电子显微镜 

分 类 号：R692.6[医药卫生—泌尿科学]