Epidemiology, Outcomes, and Complement Gene Variants in Secondary Thrombotic Microangiopathies

医学 血栓性血小板减少性紫癜 非典型溶血尿毒综合征 内科学 病因学 血栓性微血管病 移植 入射(几何) 胃肠病学 补体系统 免疫学 抗体 血小板 疾病 光学 物理
作者
Alexis Wérion,P Storms,Ysaline Zizi,Claire Beguin,J. A. Bernards,J Cambier,Karin Dahan,Daan Dierickx,Nathalie Godefroid,Pascale Hilbert,Catherine Lambert,Elena Levtchenko,Thomas Meyskens,Xavier Poiré,Lambert van den Heuvel,Kathleen Claes,Johann Morelle
出处
期刊:Clinical Journal of The American Society of Nephrology [American Society of Nephrology]
卷期号:18 (7): 881-891 被引量:5
标识
DOI:10.2215/cjn.0000000000000182
摘要

Background The identification of complement defects as major drivers of primary atypical hemolytic uremic syndrome (HUS) has transformed the landscape of thrombotic microangiopathies (TMAs), leading to the development of targeted therapies and better patient outcomes. By contrast, little is known about the presentation, genetics, and outcomes of TMA associated with specific diseases or conditions, also referred to as secondary TMA. Methods In this study, we assessed the relative incidence, clinical and genetic spectra, and long-term outcomes of secondary TMA versus other TMAs in consecutive patients hospitalized with a first episode of TMA from 2009 to 2019 at two European reference centers. Results During the study period, 336 patients were hospitalized with a first episode of TMA. Etiologies included atypical HUS in 49 patients (15%), thrombotic thrombocytopenic purpura (TTP) in 29 (9%), shigatoxin-associated HUS in 70 (21%), and secondary TMA in 188 (56%). The main causes of secondary TMA were hematopoietic stem-cell transplantation ( n =56, 30%), solid-organ transplantation ( n =44, 23%), and malignant hypertension ( n =25, 13%). Rare variants in complement genes were identified in 32 of 49 patients (65%) with atypical HUS and eight of 64 patients (13%) with secondary TMA; pathogenic or likely pathogenic variants were found in 24 of 49 (49%) and two of 64 (3%) of them, respectively ( P < 0.001). After a median follow-up of 1157 days, death or kidney failure occurred in 14 (29%), eight (28%), five (7%), and 121 (64%) patients with atypical HUS, TTP, shigatoxin-associated HUS, and secondary TMA, respectively. Unadjusted and adjusted Cox regressions showed that patients with secondary TMA had the highest risk of death or kidney failure (unadjusted hazard ratio [HR], 3.35; 95% confidence interval [CI], 1.85 to 6.07; P < 0.001; adjusted HR, 4.11; 95% CI, 2.00 to 8.46; P < 0.001; considering atypical HUS as reference). Conclusions Secondary TMAs represent the main cause of TMA and are independently associated with a high risk of death and progression to kidney failure.
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
1秒前
小马甲应助高兴的小甜瓜采纳,获得10
1秒前
WNL完成签到,获得积分10
1秒前
科研通AI2S应助长亮采纳,获得10
2秒前
Hey发布了新的文献求助10
2秒前
WWXWWX应助科研文献搬运工采纳,获得30
3秒前
3秒前
Liu发布了新的文献求助10
3秒前
4秒前
稳如老狗发布了新的文献求助10
4秒前
小黄加油鸭完成签到,获得积分20
4秒前
茕凡桃七完成签到,获得积分10
5秒前
5秒前
校长发布了新的文献求助10
6秒前
6秒前
wmm完成签到 ,获得积分10
6秒前
孝顺的尔丝完成签到,获得积分10
6秒前
明亮的翠风完成签到,获得积分10
6秒前
7秒前
1476194342发布了新的文献求助10
8秒前
9秒前
后来完成签到,获得积分10
9秒前
10秒前
派大星和海绵宝宝完成签到,获得积分10
10秒前
10秒前
11秒前
科研通AI2S应助Charlieite采纳,获得10
11秒前
咿呀完成签到,获得积分10
12秒前
lingxu完成签到,获得积分10
12秒前
May完成签到,获得积分10
13秒前
长亮完成签到,获得积分20
13秒前
fangjie发布了新的文献求助10
14秒前
15秒前
赘婿应助友好的向梦采纳,获得10
15秒前
ivying0209完成签到,获得积分10
15秒前
1476194342完成签到,获得积分10
15秒前
彤彤完成签到 ,获得积分10
16秒前
16秒前
cfy完成签到,获得积分10
16秒前
xin完成签到 ,获得积分10
16秒前
高分求助中
Evolution 10000
Sustainability in Tides Chemistry 2800
The Young builders of New china : the visit of the delegation of the WFDY to the Chinese People's Republic 1000
юрские динозавры восточного забайкалья 800
English Wealden Fossils 700
Foreign Policy of the French Second Empire: A Bibliography 500
Chen Hansheng: China’s Last Romantic Revolutionary 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 3147351
求助须知:如何正确求助?哪些是违规求助? 2798580
关于积分的说明 7829767
捐赠科研通 2455324
什么是DOI,文献DOI怎么找? 1306666
科研通“疑难数据库(出版商)”最低求助积分说明 627883
版权声明 601567