Haemolytic uremic syndrome as a cause of chronic kidney disease stage 5 in children is in retreat: results from the Polish Registry of Kidney Replacement Therapy in children (2000-2023).

IF 2.6 3区 医学 Q1 PEDIATRICS Pediatric Nephrology Pub Date : 2024-11-16 DOI:10.1007/s00467-024-06584-2
Ilona Zagożdżon, Maria Szczepańska, Jacek Rubik, Katarzyna Zachwieja, Anna Musielak, Monika Bratkowska, Irena Makulska, Katarzyna Niwińska, Beata Leszczyńska, Beata Bieniaś, Katarzyna Taranta-Janusz, Hanna Adamczyk-Kipigroch, Aleksandra Żurowska
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Abstract

Background: Haemolytic uremic syndrome (HUS) is a life-threatening disease with a historically poor prognosis in children receiving maintenance kidney replacement therapy (KRT). This study aimed to analyse the incidence and outcome of chronic kidney disease stage 5 (CKD5) due to Escherichia coli-HUS (STEC-HUS) and complement-mediated HUS (CM-HUS) in children, compared with controls with non-HUS CKD5 over the last 24 years.

Methods: The study included 1488 children undergoing KRT in Poland between 2000 and 2023. Thirty-nine patients with CM-HUS and 18 with STEC-HUS were identified and analysed for incidence, KRT modality and survival.

Results: The incidence rate of CKD5 was 0.09 cases/million age-related population (marp) for STEC-HUS and 0.23/marp for CM-HUS, while no new cases have been observed in recent years. CKD5 due to CM-HUS developed significantly earlier from initial HUS manifestation than in STEC-HUS (median 0.2 vs. 9.8 years). CM-HUS was associated with younger age at initiation of KRT compared to STEC-HUS and non-HUS controls (median 6.0 years vs. 10.9 and 10.9 years), with higher risk of death (Hazard Ratio 1.92, 95% confidence interval 0.9-4.13) and worse 5-year kidney graft survival at 77%, 93% and 90%, respectively (p < 0.001).

Conclusions: In recent years, both CM-HUS and STEC-HUS have become increasingly rare causes of CKD5 in children. CKD5 due to CM-HUS in the eculizumab era and due to STEC-HUS after improving supportive treatment is exceptional. Children on KRT due to STEC-HUS had a significantly better survival, shorter waiting time for kidney transplantation and better kidney graft survival compared to the CM-HUS group.

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溶血性尿毒症综合征作为儿童慢性肾病 5 期的病因正在消退:波兰儿童肾脏替代疗法登记处(2000-2023 年)的结果。
背景:溶血性尿毒症(HUS)是一种危及生命的疾病,在接受维持性肾脏替代治疗(KRT)的儿童中,其预后历来较差。本研究旨在分析过去 24 年中儿童因大肠杆菌-HUS(STEC-HUS)和补体介导的 HUS(CM-HUS)导致慢性肾病五期(CKD5)的发病率和预后,并与非 HUS CKD5 的对照组进行比较:研究纳入了 2000 年至 2023 年期间在波兰接受 KRT 治疗的 1488 名儿童。确定了 39 名 CM-HUS 患者和 18 名 STEC-HUS 患者,并对其发病率、KRT 方式和存活率进行了分析:STEC-HUS和CM-HUS的CKD5发病率分别为0.09例/百万年龄相关人口(marp)和0.23例/marp,近年来未发现新病例。与 STEC-HUS 相比,CM-HUS 引起的 CKD5 从最初的 HUS 表现开始明显提前(中位数为 0.2 年对 9.8 年)。与 STEC-HUS 和非 HUS 对照组相比,CM-HUS 开始接受 KRT 治疗的年龄更小(中位数为 6.0 岁 vs. 10.9 岁和 10.9 岁),死亡风险更高(危险比为 1.92,95% 置信区间为 0.9-4.13),5 年肾移植存活率更低,分别为 77%、93% 和 90%(P 结论:CM-HUS 和非 HUS 肾移植存活率均低于 STEC-HUS:近年来,CM-HUS 和 STEC-HUS 已逐渐成为儿童 CKD5 的罕见病因。在依库珠单抗时代,CM-HUS 导致的 CKD5 和 STEC-HUS 在改善支持治疗后导致的 CKD5 都非常罕见。与 CM-HUS 组相比,因 STEC-HUS 而接受 KRT 治疗的儿童生存率明显更高,肾移植等待时间更短,肾移植存活率更高。
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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
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