Increased Risk of End-Stage Renal Disease in Familial IgA Nephropathy

医学 肾病 内科学 无症状的 胃肠病学 优势比 蛋白尿 肾活检 队列 肾小球肾炎 肾功能 内分泌学 糖尿病
作者
Francesco Paolo Schena,Giuseppina Cerullo,Michele Rossini,Salvatore Giovanni Lanzilotta,Christian D’Altri,Carlo Manno
出处
期刊:Journal of The American Society of Nephrology 卷期号:13 (2): 453-460 被引量:68
标识
DOI:10.1681/asn.v132453
摘要

ABSTRACT. Primary IgA nephropathy (IgAN) is characterized by recurrent episodes of macroscopic hematuria accompanied by upper respiratory tract infections or persistent asymptomatic microscopic hematuria with or without proteinuria. IgAN may involve one or more members of a family. Three generations of a cohort of 110 patients with biopsy-proven IgAN, living in Southern Italy, were checked for urinalysis, and the relative risk (RR) of developing the disease was evaluated. A total of 19 unrelated familial, 37 suspected, and 54 sporadic cases of IgAN were identified. Renal survival was estimated by the Kaplan-Meier method for censored data and compared by use of the log-rank test. More than 50% of the patients with IgAN clustered in kindred with more than two probably affected relatives. In 19 unrelated IgAN families, 8 had single-generation (SG) and 11 multigenerational (MG) involvement showing a prevalent vertical transmission of the trait. The RR was 16 times higher in first-degree relatives (odds ratio [OR], 16.4; 95% confidence interval [CI], 5.7 to 47.8; P < 0.0001) and >2 times higher, even if NS, in second-degree relatives (OR, 2.4; 95 % CI, 0.7 to 7.9; P = 0.145). The clinical and histologic picture of familial and sporadic IgAN appeared to be similar. The 20-yr renal survival rate from the apparent onset of the disease was significantly poorer in patients with familial (41%) than in patients with sporadic (94%) IgAN (P = 0.003). Furthermore, 15-yr renal survival from the time of renal biopsy was significantly worse in familial IgAN (P = 0.02); end-stage renal disease was present in 64% of familial and only in 8% of patients with sporadic IgAN. Finally, renal survival was significantly worse in patients belonging to families with SG rather than with MG involvement (P = 0.03). These data show, for the first time, that familial IgAN may be considered a nonbenign disease that occurs frequently in first-degree relatives. Familial IgAN has a poorer outcome than sporadic IgAN. Therefore, an accurate family history and urinalysis in all family members is urgently recommended in clinical practice. This procedure might avoid late referral of subjects with persistent and underestimated urinary abnormalities and late diagnosis of the disease.
最长约 10秒,即可获得该文献文件

科研通智能强力驱动
Strongly Powered by AbleSci AI
更新
大幅提高文件上传限制,最高150M (2024-4-1)

科研通是完全免费的文献互助平台,具备全网最快的应助速度,最高的求助完成率。 对每一个文献求助,科研通都将尽心尽力,给求助人一个满意的交代。
实时播报
答案是Cyndi完成签到,获得积分10
1秒前
charm12发布了新的文献求助10
1秒前
拥挤而独行完成签到,获得积分10
1秒前
香蕉觅云应助foredoom007采纳,获得10
1秒前
1秒前
2秒前
小杨发布了新的文献求助10
2秒前
zzzzzz完成签到,获得积分20
2秒前
2秒前
白菜发布了新的文献求助10
2秒前
2秒前
3秒前
Avicii发布了新的文献求助12
3秒前
中和皇极发布了新的文献求助10
4秒前
kayyu完成签到,获得积分20
4秒前
闹闹发布了新的文献求助10
4秒前
5秒前
迈安纳发布了新的文献求助10
5秒前
月月发布了新的文献求助10
5秒前
阿钉发布了新的文献求助10
6秒前
6秒前
耶律遗风发布了新的文献求助10
7秒前
朱文琛完成签到,获得积分10
7秒前
8秒前
8秒前
8秒前
小虫子发布了新的文献求助20
8秒前
8秒前
小M发布了新的文献求助10
8秒前
完美世界应助金灶沐采纳,获得10
9秒前
9秒前
万能图书馆应助XDM采纳,获得10
9秒前
骆子军发布了新的文献求助20
9秒前
10秒前
10秒前
研友_VZG7GZ应助屁王采纳,获得10
11秒前
MapleLeaf完成签到 ,获得积分10
11秒前
冷艳后妈发布了新的文献求助10
11秒前
白菜完成签到,获得积分10
12秒前
yuyuyu998发布了新的文献求助10
12秒前
高分求助中
The late Devonian Standard Conodont Zonation 2000
The Lali Section: An Excellent Reference Section for Upper - Devonian in South China 1500
Nickel superalloy market size, share, growth, trends, and forecast 2023-2030 1000
Smart but Scattered: The Revolutionary Executive Skills Approach to Helping Kids Reach Their Potential (第二版) 1000
Mantiden: Faszinierende Lauerjäger Faszinierende Lauerjäger 800
PraxisRatgeber: Mantiden: Faszinierende Lauerjäger 800
A new species of Coccus (Homoptera: Coccoidea) from Malawi 500
热门求助领域 (近24小时)
化学 医学 生物 材料科学 工程类 有机化学 生物化学 物理 内科学 纳米技术 计算机科学 化学工程 复合材料 基因 遗传学 催化作用 物理化学 免疫学 量子力学 细胞生物学
热门帖子
关注 科研通微信公众号,转发送积分 3245464
求助须知:如何正确求助?哪些是违规求助? 2889085
关于积分的说明 8256869
捐赠科研通 2557437
什么是DOI,文献DOI怎么找? 1386114
科研通“疑难数据库(出版商)”最低求助积分说明 650285
邀请新用户注册赠送积分活动 626541