家族性IgA肾病——777例中国IgA肾病回顾性调查分析  

Familial IgA nephropathy in Chinese patients

作　　者：吕继成[1] 张宏[1] 陈育青[1] 刘刚[1] 王海燕[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,100034

出　　处：《中华肾脏病杂志》2004年第1期5-7,共3页Chinese Journal of Nephrology

基　　金：首都医学发展科研基金资助项目(ZD199910)

摘　　要：目的报道中国家族性IgA肾病(FIgAN)发生情况以及临床病理特征,提高对家族性IgA肾病的认识。方法利用肾脏疾病数据库IgA肾病子库中我院1988～2001年收集的777例IgA肾病患者,进行家族史调查,以及部分家庭成员中行尿常规肾功能检查确定FIgAN发病情况,比较家族性与家族史阴性的IgA肾病患者临床表型的差异。结果(1)在调查的777例IgA肾病患者中,35例患者同时合并薄基底膜肾病,另外742例患者中,65例IgA肾病患者家族史阳性,占8.7%,其中10例(1.3%)患者为FIgAN,55例(7.4%)为可疑家族性IgA肾病。(2)与家族史阴性的患者相比,FIgAN患者在发病年龄、性别、肾穿时的血压、血尿、蛋白尿、肾功能上差异无显著性意义。结论FIgAN在中国人并非少见,加强家系调查特别是在IgA肾病患者亲属中常规进行尿检查将有助于发现该病。家族性IgA肾病诊断应强调同时电镜检查以除外薄基底膜肾病和早期Alport综合征。初步研究提示,本组患者与家族史阴性的IgAN患者相比,其临床病理表现不具有特征性。Objects To report the prevalence of familial IgA nephropathy (FIgAN) in a center in northern China and describe its clinical and pathological characterization. Methods Seven hundred and seventy seven IgA nephropathy patients proved by biopsy were from Renal Disease Database. All the patients were investigated by detailed family history and the relatives were also checked by urine and blood analysis in some patients. The clinical presentation and renal lesions were compared between FIgAN and the IgA nephropathy patients without a family history of renal disease. Results Thirty five IgA nephropathy patients accompanied thin basement membrane nephropathy. Among the other 742 patients,sixty five (8 7%) patients had positive family history of renal disease.Of above 65 patients,10 cases(1 3%) were proved to be FIgAN,the other 55 patients(7 4%) were defined as suspected familial IgA nephropathy.A comparison between familial IgAN and the patients with negative family history of renal disease showed no significant difference in age, sex, serum creatinine, creatinine clearance, blood pressure, serum IgA at the time of renal biopsy. Conclusions The present study provides new data on the interesting field of familial IgA nephropathy. Familial IgA nephropathy is not rare in Chinese, nearly 8 7%IgAN patients cluster in kindred with more than two probably affected relatives. For not all the patients′relatives were screened by urinary analysis, some patients may remain asymptomatic in lifetime. An accurate family history and urinalysis in all family members of IgAN patient is recommended. Moreover electron microscope is essential in the diagnosis of FIgAN which has ever been underestimated, for some patients accompanied hereditay disease such as thin basement membrane nephropathy. There are no special clinical and pathological features in familial IgA nephropathy in our patients.

关 键 词：家族性IGA肾病 病理特征 电镜检查 肾功能 肾组织活检 

分 类 号：R692.3[医药卫生—泌尿科学]