1. Increased Lifetime Risk of ESRD in Familial IgA Nephropathy
- Author
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Yuanmeng Jin, Qinjie Weng, Weiming Wang, Jun Tong, Zhengying Fang, Nan Chen, Manman Shi, Wenjie Wei, Shuwen Yu, Wen Du, Zijin Chen, Xiaoxia Pan, Kiryluk Krzysztof, Jingyuan Xie, Yan Ouyang, and Zhaohui Wang
- Subjects
medicine.medical_specialty ,030232 urology & nephrology ,Renal function ,Late onset ,single-generation ,Urine ,Disease ,030204 cardiovascular system & hematology ,urologic and male genital diseases ,End stage renal disease ,Nephropathy ,03 medical and health sciences ,0302 clinical medicine ,Clinical Research ,Internal medicine ,gender ,medicine ,end-stage renal disease ,business.industry ,multiple-generation ,Hazard ratio ,lifetime risk ,medicine.disease ,Confidence interval ,Nephrology ,familial IgA nephropathy ,Corrigendum ,business - Abstract
Introduction Familial IgA nephropathy (IgAN) has been widely reported. However, its clinicohistologic characteristics and long-term prognosis are not clear. Methods A total of 348 familial IgAN cases from 167 independent families were recruited and their clinicohistologic characteristics as well as lifetime risk of end-stage renal disease (ESRD) were compared to 1116 sporadic IgAN patients from the same geographic region. Results Of all familial IgAN patients, 60 (17%) came from 32 single-generation (SG; all affected individuals are siblings) families, whereas 286 (82%) came from 134 multiple-generation (MG; affected individuals were present in at least 2 consecutive generations) families. The lifetime ESRD risk was significantly higher in familial patients than sporadic ones after adjusting by gender (hazard ratio [HR]=1.40, 95% confidence interval [CI]: 1.12–1.74, P = 0.004), with 5 years younger in median ESRD age (60 years vs. 65 years in familial and sporadic cases separately). Interestingly, among familial patients, we found cases from SG families (vs. MG families: HR = 2.62, 95% CI: 1.59–4.31, P < 0.001) or with early onset (onset age, Graphical abstract
- Published
- 2021
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