家族性IgA肾病临床特点研究  

Clinical characteristics of familial IgA nephropathy

作　　者：谢静远[1] 王朝晖[1] 章晓炎[1] 沈平雁[1] 李娅[1] 任红[1] 王伟铭[1] 陈楠[1] 

机构地区：[1]上海交通大学医学院附属瑞金医院肾脏科,上海200025

出　　处：《中国实用内科杂志》2014年第3期258-261,共4页Chinese Journal of Practical Internal Medicine

基　　金：国家重点基础研究发展计划(2012CB517604);国家自然科学基金(81000295;81370015;81070568;81170634);中华医学会临床医学科研专项基金(13030280413);国家临床重点专科建设项目

摘　　要：目的总结家族性IgA肾病患者临床及预后特点。方法回顾性分析上海交通大学医学院附属瑞金医院1989--2010年间619例肾活检证实的IgAN患者，其中家族性IgA肾病患者78例（占12．6％）。收集所有患者肾活检时及随访过程中临床数据，对比家族性IgA肾病及散发性IgA肾病患者临床特征，多元COX回归检测基础临床参数与预后相关性以发现肾脏病进展的独立危险因子。结果家族性IgA肾病较散发性IgA肾病患者肾活检时高血压发生率低（33．3％对48．8％，P=0．01）、尿蛋白少[1n（uP）-0．18±1．05对0．26±1．07，P=0．001]、血白蛋白高[（36±5．9）g／L对（33．9±8．1）g/L，P=0．006]、危险评分低（一0．38±1．31对0．06±1．32，P=0．006）。肾脏病家族史是IgA肾病患者终末期肾病（ESRD）的独立危险因素[HR=0．34（0．14～0．81），P=0．015]。结论家族性IgA肾病患者较散发性IgA肾病患者肾活检时临床严重程度较轻，可能与家族性IgA肾病患者早期诊断有关。提示应重视对家族性IgA肾病患者的早期诊断和筛查。Objective To summarize the clinical features and prognosis of patients with familial IgA nephropathy （FIgAN）. Methods A total of 619 biopsy-proven IgAN patients were recruited,of which 78 cases （ 12. 6% ） had familial history of kidney diseases. Clinical data of all patients were recorded at the time of renal biopsy and during follow up. Clinical charac- teristics were compared between FIgAN patients and sporadic IgA nephropathy （SIgAN） patients. Multiple COX regression model was built up to test the association between baseline clinical parameters and prognosis and to discover independent the risk factors of prognosis of renal diseases. Results We found at the time of renal biopsy, FIgAN patients had more fre- quent rate of hypertension （33.3 % vs 48.8 % ,P =0. 01 ） ,lower proteinuria （In [ UP ] -0. 18±1.05 vs 0. 26 ± 1.07, P = 0. 001 ） ,higher serum albumin （ [ 3.6±0. 59 ] g/L vs [ 3.39 ± 0. 811 g/L, P = 0. 006 ） and lower risk score （ - 0. 38 ± 1.31 vs 0.06 ± 1.32, P = 0.006 ） , as compared with SIgAN patients. Famihal history of kidney diseases （ HR = 0. 34 [ 0. 14 -0. 81 ] , P = 0. 015 ） was a significant independent risk factor of end-stage renal disease. Conclusion We find fe- wer serious clinical manifestations and relatively better prognosis in FIgAN patients than in SIgAN patients, which may be related to early diagnosis of FIgAN patients. Therefore we should pay more attention to early diagnosis and screening of FI- gAN patients.

关 键 词：家族性IGA肾病 散发性IgA肾病 终末期肾病 

分 类 号：R692.6[医药卫生—泌尿科学]