C3 glomerulopathy in children: a European longitudinal study evaluating outcome.

IF 2.6 3区 医学 Q1 PEDIATRICS Pediatric Nephrology Pub Date : 2024-11-26 DOI:10.1007/s00467-024-06587-z
Andrea Cappoli, Tanja Kersnik-Levart, Valeria Silecchia, Gema Ariceta, Ann Christin Gjerstad, Gianmarco Ghiggeri, Dieter Haffner, Nele Kanzelmeyer, Elena Levtchenko, Andrea Pasini, Aoife Waters, Juan Cruz Len Aguilera, Licia Peruzzi, Marina Noris, Elena Bresin, Antonio Gargiulo, Francesco Emma, Marina Vivarelli
{"title":"C3 glomerulopathy in children: a European longitudinal study evaluating outcome.","authors":"Andrea Cappoli, Tanja Kersnik-Levart, Valeria Silecchia, Gema Ariceta, Ann Christin Gjerstad, Gianmarco Ghiggeri, Dieter Haffner, Nele Kanzelmeyer, Elena Levtchenko, Andrea Pasini, Aoife Waters, Juan Cruz Len Aguilera, Licia Peruzzi, Marina Noris, Elena Bresin, Antonio Gargiulo, Francesco Emma, Marina Vivarelli","doi":"10.1007/s00467-024-06587-z","DOIUrl":null,"url":null,"abstract":"<p><strong>Background: </strong>C3 glomerulopathy is a rare clinical entity characterized by dysregulation of the alternative complement pathway in glomerular disease. Studies defining the natural history of C3G in the pediatric population are scarce.</p><p><strong>Methods: </strong>Patients included in this retrospective study were diagnosed between 2011 and 2020 in 12 European pediatric nephrology units. Data were collected from baseline, 6 months, 12 months and at the last follow-up. Complete remission (CR) was defined as a urinary protein creatinine ratio (UPCR) < 0.3 mg/mg with normal estimated glomerular filtration rate (eGFR). Partial remission was defined as a decrease in UPCR to 0.3 and 3 mg/mg with normal eGFR. Lack of remission was defined as non-response.</p><p><strong>Results: </strong>A total of 108 pediatric patients were included. Complete remission was achieved in 71/108 patients (65.7%), with probability of CR of 50% at 1.8 years and of 78% at 7 years. At presentation by univariate analysis the predictive factors at presentation associated with CR included eGFR (p = 0.028), UPCR (p = 0.004), serum C3 levels (p = 0.018), elevated plasma sC5b9 levels, defined as > 400 ng/ml, (p = 0.037), the presence of endocapillary proliferation (p = 0.017), and the absence of dense deposits on electron microscopy (p = 0.032). By multivariate analysis a low UPCR at presentation (p < 0.001) and the presence of endocapillary proliferation (p < 0.01) remained positively associated with CR.</p><p><strong>Conclusions: </strong>Our data confirm that C3G has a more benign outcome in children compared to previous reports in adults, and suggest that endocapillary proliferation and the degree of proteinuria at onset are the most relevant prognostic factors.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":""},"PeriodicalIF":2.6000,"publicationDate":"2024-11-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Pediatric Nephrology","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1007/s00467-024-06587-z","RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q1","JCRName":"PEDIATRICS","Score":null,"Total":0}
引用次数: 0

Abstract

Background: C3 glomerulopathy is a rare clinical entity characterized by dysregulation of the alternative complement pathway in glomerular disease. Studies defining the natural history of C3G in the pediatric population are scarce.

Methods: Patients included in this retrospective study were diagnosed between 2011 and 2020 in 12 European pediatric nephrology units. Data were collected from baseline, 6 months, 12 months and at the last follow-up. Complete remission (CR) was defined as a urinary protein creatinine ratio (UPCR) < 0.3 mg/mg with normal estimated glomerular filtration rate (eGFR). Partial remission was defined as a decrease in UPCR to 0.3 and 3 mg/mg with normal eGFR. Lack of remission was defined as non-response.

Results: A total of 108 pediatric patients were included. Complete remission was achieved in 71/108 patients (65.7%), with probability of CR of 50% at 1.8 years and of 78% at 7 years. At presentation by univariate analysis the predictive factors at presentation associated with CR included eGFR (p = 0.028), UPCR (p = 0.004), serum C3 levels (p = 0.018), elevated plasma sC5b9 levels, defined as > 400 ng/ml, (p = 0.037), the presence of endocapillary proliferation (p = 0.017), and the absence of dense deposits on electron microscopy (p = 0.032). By multivariate analysis a low UPCR at presentation (p < 0.001) and the presence of endocapillary proliferation (p < 0.01) remained positively associated with CR.

Conclusions: Our data confirm that C3G has a more benign outcome in children compared to previous reports in adults, and suggest that endocapillary proliferation and the degree of proteinuria at onset are the most relevant prognostic factors.

查看原文
分享 分享
微信好友 朋友圈 QQ好友 复制链接
本刊更多论文
儿童 C3 肾小球病:一项评估结果的欧洲纵向研究。
背景:C3肾小球病是一种罕见的临床疾病,其特点是肾小球疾病中替代性补体途径失调。界定 C3G 在儿科人群中自然病史的研究很少:这项回顾性研究中的患者于 2011 年至 2020 年期间在 12 个欧洲儿科肾病科确诊。研究收集了基线、6个月、12个月和最后一次随访的数据。完全缓解(CR)定义为尿蛋白肌酐比值(UPCR):共纳入 108 名儿童患者。71/108名患者(65.7%)获得了完全缓解,1.8年和7年时CR概率分别为50%和78%。通过单变量分析,发病时与 CR 相关的预测因素包括 eGFR(p = 0.028)、UPCR(p = 0.004)、血清 C3 水平(p = 0.018)、血浆 sC5b9 水平升高(定义为 > 400 ng/ml)(p = 0.037)、存在毛细血管内增殖(p = 0.017)以及电镜下无致密沉积(p = 0.032)。通过多变量分析,C3G 患者发病时的 UPCR 较低(p 结论:UPCR 越低,C3G 的发病率越高:我们的数据证实,与之前的成人报告相比,C3G 在儿童中的良性预后更佳,并表明毛细血管内膜增生和发病时的蛋白尿程度是最相关的预后因素。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
求助全文
约1分钟内获得全文 去求助
来源期刊
Pediatric Nephrology
Pediatric Nephrology 医学-泌尿学与肾脏学
CiteScore
4.70
自引率
20.00%
发文量
465
审稿时长
1 months
期刊介绍: International Pediatric Nephrology Association Pediatric Nephrology publishes original clinical research related to acute and chronic diseases that affect renal function, blood pressure, and fluid and electrolyte disorders in children. Studies may involve medical, surgical, nutritional, physiologic, biochemical, genetic, pathologic or immunologic aspects of disease, imaging techniques or consequences of acute or chronic kidney disease. There are 12 issues per year that contain Editorial Commentaries, Reviews, Educational Reviews, Original Articles, Brief Reports, Rapid Communications, Clinical Quizzes, and Letters to the Editors.
期刊最新文献
Concurrent use of continuous kidney replacement therapy during extracorporeal membrane oxygenation: what pediatric nephrologists need to know-PCRRT-ICONIC practice points. Furosemide stress test to predict acute kidney injury progression in critically ill children. A better future for children with STEC-hemolytic uremic syndrome: news from Argentina. Acetaminophen induced high anion gap metabolic acidosis: a potentially under-recognized consequence from a common medication. Malnutrition management in children with chronic kidney disease.
×
引用
GB/T 7714-2015
复制
MLA
复制
APA
复制
导出至
BibTeX EndNote RefMan NoteFirst NoteExpress
×
×
提示
您的信息不完整,为了账户安全,请先补充。
现在去补充
×
提示
您因"违规操作"
具体请查看互助需知
我知道了
×
提示
现在去查看 取消
×
提示
确定
0
微信
客服QQ
Book学术公众号 扫码关注我们
反馈
×
意见反馈
请填写您的意见或建议
请填写您的手机或邮箱
已复制链接
已复制链接
快去分享给好友吧!
我知道了
×
扫码分享
扫码分享
Book学术官方微信
Book学术文献互助
Book学术文献互助群
群 号:481959085
Book学术
文献互助 智能选刊 最新文献 互助须知 联系我们:info@booksci.cn
Book学术提供免费学术资源搜索服务,方便国内外学者检索中英文文献。致力于提供最便捷和优质的服务体验。
Copyright © 2023 Book学术 All rights reserved.
ghs 京公网安备 11010802042870号 京ICP备2023020795号-1