首页 > 最新文献

Kidney International Reports最新文献

英文 中文
APOL1 G1/G2 Variants Associated With CKD in Afro-Brazilians 与非裔巴西人CKD相关的APOL1 G1/G2变异
IF 5.7 2区 医学 Q1 UROLOGY & NEPHROLOGY Pub Date : 2025-12-01 DOI: 10.1016/j.ekir.2025.09.026
Celia M.B. Souza , Bibiana S.O. Fam , Giovanna C. Giudicelli , Nathan Araujo Cadore , Mariléa F. Feira , Mayara Jorgens Prado , Thayne W. Kowalski , Osvaldo Alfonso P. Artigalás , Renan B. Lemes , Maíra Ribeiro Rodrigues , Mauro R.S. Junior , William C. Silva , Maicon D. Torely , Franciele M. Barbosa , Alexandre da Costa Pereira , Lygia V. Pereira , Tábita Hünemeier , Francisco V. Veronese , Fernanda S.L. Vianna
{"title":"APOL1 G1/G2 Variants Associated With CKD in Afro-Brazilians","authors":"Celia M.B. Souza , Bibiana S.O. Fam , Giovanna C. Giudicelli , Nathan Araujo Cadore , Mariléa F. Feira , Mayara Jorgens Prado , Thayne W. Kowalski , Osvaldo Alfonso P. Artigalás , Renan B. Lemes , Maíra Ribeiro Rodrigues , Mauro R.S. Junior , William C. Silva , Maicon D. Torely , Franciele M. Barbosa , Alexandre da Costa Pereira , Lygia V. Pereira , Tábita Hünemeier , Francisco V. Veronese , Fernanda S.L. Vianna","doi":"10.1016/j.ekir.2025.09.026","DOIUrl":"10.1016/j.ekir.2025.09.026","url":null,"abstract":"","PeriodicalId":17761,"journal":{"name":"Kidney International Reports","volume":"10 12","pages":"Pages 4289-4293"},"PeriodicalIF":5.7,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145652074","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
NELL-1–Positive Membranous Nephropathy Following Inhalational Mercury Exposure 吸入汞暴露后的nell -1阳性膜性肾病
IF 5.7 2区 医学 Q1 UROLOGY & NEPHROLOGY Pub Date : 2025-12-01 DOI: 10.1016/j.ekir.2025.10.009
Yagmur Tahillioglu , Ozge Hurdogan , Ahmet Burak Dirim , Ayse Serra Artan , Abdulkadir Celik , Egemen Cebeci , Ozgur Akin Oto , Yasemin Ozluk , Halil Yazici , Aydin Turkmen , Savas Ozturk
{"title":"NELL-1–Positive Membranous Nephropathy Following Inhalational Mercury Exposure","authors":"Yagmur Tahillioglu , Ozge Hurdogan , Ahmet Burak Dirim , Ayse Serra Artan , Abdulkadir Celik , Egemen Cebeci , Ozgur Akin Oto , Yasemin Ozluk , Halil Yazici , Aydin Turkmen , Savas Ozturk","doi":"10.1016/j.ekir.2025.10.009","DOIUrl":"10.1016/j.ekir.2025.10.009","url":null,"abstract":"","PeriodicalId":17761,"journal":{"name":"Kidney International Reports","volume":"10 12","pages":"Pages 4306-4307"},"PeriodicalIF":5.7,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145651710","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Response To the Letter to the Editor Entitled “First Report of Daratumumab Therapy in Refractory THSD7A-Positive Membranous Nephropathy” 对题为“Daratumumab治疗难治性thsd7a阳性膜性肾病的第一份报告”的致编辑的回复
IF 5.7 2区 医学 Q1 UROLOGY & NEPHROLOGY Pub Date : 2025-12-01 DOI: 10.1016/j.ekir.2025.10.012
Fatima Ayub , John Arthur , Md Rajibul Hasan , Luis A. Juncos , Joseph Hunter Holthoff
{"title":"Response To the Letter to the Editor Entitled “First Report of Daratumumab Therapy in Refractory THSD7A-Positive Membranous Nephropathy”","authors":"Fatima Ayub , John Arthur , Md Rajibul Hasan , Luis A. Juncos , Joseph Hunter Holthoff","doi":"10.1016/j.ekir.2025.10.012","DOIUrl":"10.1016/j.ekir.2025.10.012","url":null,"abstract":"","PeriodicalId":17761,"journal":{"name":"Kidney International Reports","volume":"10 12","pages":"Pages 4316-4317"},"PeriodicalIF":5.7,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145651854","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Effect of Baseline Dialysis and Plasma Exchange on Renal Prognosis in Patients With Antineutrophil Cytoplasmic Autoantibody–Associated Vasculitis 基线透析和血浆置换对抗中性粒细胞细胞质自身抗体相关性血管炎患者肾脏预后的影响
IF 5.7 2区 医学 Q1 UROLOGY & NEPHROLOGY Pub Date : 2025-12-01 DOI: 10.1016/j.ekir.2025.09.005
Maxime Vignac , Dorian Nezam , François Grolleau , Pauline Morel , Dimitri Titeca-Beauport , Stanislas Faguer , Alexandre Karras , Justine Solignac , Noémie Jourde-Chiche , François Maurier , Hamza Sakhi , Khalil El Karoui , Rafik Mesbah , Pierre Louis Carron , Vincent Audard , Didier Ducloux , Romain Paule , Jean-François Augusto , Julien Aniort , Aurélien Tiple , Benjamin Terrier

Introduction

The identification of prognostic factors for renal failure in antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV) remains a challenge. The benefit of plasma exchange (PLEX) has been questioned, and the target population remains to be defined. We investigated the outcome of patients requiring renal replacement therapy (RRT) at baseline and factors associated with their prognosis at 1 year.

Methods

This retrospective multicenter study evaluated the 1-year composite end point of death or end-stage kidney disease (ESKD) in patients with biopsy-proven renal AAV involvement.

Results

Of the 394 patients included, 105 (26.6%) were on dialysis at baseline. Of these, 60 (57.1%) reached the composite end point compared with 29 patients (10.0%) who were not on RRT at baseline (P < 0.001). On multivariate analysis, age and sex were not associated with the composite outcome (P = 0.945 and P = 0.154, respectively); however, myeloperoxidase (MPO)-ANCA was (odds ratio [OR]: 3.60; 95% confidence interval [CI]: 1.79–7.60), as was a high baseline histologic renal risk score (OR: 1.29; 95% CI 1.17–1.44). The most strongly associated factor remained the need for dialysis at baseline (OR: 10.91; 95% CI: 5.52–22.70). Of the 91 patients surviving after requiring dialysis at baseline, 45 were weaned from RRT (49.5%) at 1 year, and PLEX was independently associated with a reduced risk of the composite outcome (OR: 0.23, 95% CI: 0.05–0.80).

Conclusion

MPO-ANCA, need for dialysis, and high histological renal risk score at baseline were associated with the 1-year composite end point of death or ESKD. Almost half of the patients on dialysis at baseline were off dialysis at 1 year, with a better prognosis in those who had received PLEX.
抗中性粒细胞胞浆自身抗体(ANCA)相关性血管炎(AAV)肾功能衰竭的预后因素鉴定仍然是一个挑战。血浆置换(PLEX)的益处一直受到质疑,目标人群仍有待确定。我们调查了基线时需要肾脏替代治疗(RRT)的患者的预后以及与1年预后相关的因素。方法:本回顾性多中心研究评估活检证实肾AAV累及患者的1年复合终点死亡或终末期肾病(ESKD)。结果纳入的394例患者中,105例(26.6%)在基线时接受透析治疗。其中,60例(57.1%)达到了复合终点,而基线时未接受RRT治疗的29例(10.0%)达到了复合终点(P < 0.001)。多因素分析显示,年龄和性别与综合结局无关(P = 0.945和P = 0.154);然而,髓过氧化物酶(MPO)-ANCA(优势比[OR]: 3.60; 95%可信区间[CI]: 1.79-7.60)和高基线组织学肾危险评分(OR: 1.29; 95% CI 1.17-1.44)也是如此。最强烈的相关因素仍然是在基线时需要透析(OR: 10.91; 95% CI: 5.52-22.70)。在基线时需要透析后存活的91例患者中,45例(49.5%)在1年后从RRT中断奶,PLEX与复合结局风险降低独立相关(OR: 0.23, 95% CI: 0.05-0.80)。结论mpo - anca、透析需求和基线时较高的肾组织学风险评分与1年死亡或ESKD的综合终点相关。几乎一半的基线透析患者在1年后停止透析,接受PLEX的患者预后较好。
{"title":"Effect of Baseline Dialysis and Plasma Exchange on Renal Prognosis in Patients With Antineutrophil Cytoplasmic Autoantibody–Associated Vasculitis","authors":"Maxime Vignac ,&nbsp;Dorian Nezam ,&nbsp;François Grolleau ,&nbsp;Pauline Morel ,&nbsp;Dimitri Titeca-Beauport ,&nbsp;Stanislas Faguer ,&nbsp;Alexandre Karras ,&nbsp;Justine Solignac ,&nbsp;Noémie Jourde-Chiche ,&nbsp;François Maurier ,&nbsp;Hamza Sakhi ,&nbsp;Khalil El Karoui ,&nbsp;Rafik Mesbah ,&nbsp;Pierre Louis Carron ,&nbsp;Vincent Audard ,&nbsp;Didier Ducloux ,&nbsp;Romain Paule ,&nbsp;Jean-François Augusto ,&nbsp;Julien Aniort ,&nbsp;Aurélien Tiple ,&nbsp;Benjamin Terrier","doi":"10.1016/j.ekir.2025.09.005","DOIUrl":"10.1016/j.ekir.2025.09.005","url":null,"abstract":"<div><h3>Introduction</h3><div>The identification of prognostic factors for renal failure in antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV) remains a challenge. The benefit of plasma exchange (PLEX) has been questioned, and the target population remains to be defined. We investigated the outcome of patients requiring renal replacement therapy (RRT) at baseline and factors associated with their prognosis at 1 year.</div></div><div><h3>Methods</h3><div>This retrospective multicenter study evaluated the 1-year composite end point of death or end-stage kidney disease (ESKD) in patients with biopsy-proven renal AAV involvement.</div></div><div><h3>Results</h3><div>Of the 394 patients included, 105 (26.6%) were on dialysis at baseline. Of these, 60 (57.1%) reached the composite end point compared with 29 patients (10.0%) who were not on RRT at baseline (<em>P</em> &lt; 0.001). On multivariate analysis, age and sex were not associated with the composite outcome (<em>P</em> = 0.945 and <em>P</em> = 0.154, respectively); however, myeloperoxidase (MPO)-ANCA was (odds ratio [OR]: 3.60; 95% confidence interval [CI]: 1.79–7.60), as was a high baseline histologic renal risk score (OR: 1.29; 95% CI 1.17–1.44). The most strongly associated factor remained the need for dialysis at baseline (OR: 10.91; 95% CI: 5.52–22.70). Of the 91 patients surviving after requiring dialysis at baseline, 45 were weaned from RRT (49.5%) at 1 year, and PLEX was independently associated with a reduced risk of the composite outcome (OR: 0.23, 95% CI: 0.05–0.80).</div></div><div><h3>Conclusion</h3><div>MPO-ANCA, need for dialysis, and high histological renal risk score at baseline were associated with the 1-year composite end point of death or ESKD. Almost half of the patients on dialysis at baseline were off dialysis at 1 year, with a better prognosis in those who had received PLEX.</div></div>","PeriodicalId":17761,"journal":{"name":"Kidney International Reports","volume":"10 12","pages":"Pages 4199-4206"},"PeriodicalIF":5.7,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145651923","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
New RCTs Advance Incremental Dialysis With Clinically-Guided HD and HDF-Based Prescriptions 新的随机对照试验推进了临床指导的HD和基于hdf处方的增量透析
IF 5.7 2区 医学 Q1 UROLOGY & NEPHROLOGY Pub Date : 2025-12-01 DOI: 10.1016/j.ekir.2025.10.002
Mariana Murea , Andrea K. Viecelli
{"title":"New RCTs Advance Incremental Dialysis With Clinically-Guided HD and HDF-Based Prescriptions","authors":"Mariana Murea ,&nbsp;Andrea K. Viecelli","doi":"10.1016/j.ekir.2025.10.002","DOIUrl":"10.1016/j.ekir.2025.10.002","url":null,"abstract":"","PeriodicalId":17761,"journal":{"name":"Kidney International Reports","volume":"10 12","pages":"Pages 4129-4131"},"PeriodicalIF":5.7,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145651759","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
From Dialysis to Destinations: Safe Travel Strategies for Patients With CKD 从透析到目的地:CKD患者的安全旅行策略
IF 5.7 2区 医学 Q1 UROLOGY & NEPHROLOGY Pub Date : 2025-12-01 DOI: 10.1016/j.ekir.2025.09.035
Seong Geun Kim , Chia-Ter Chao , Kornchanok Vareesangthip , Winston Fung , Mariko Miyazaki , Sayaka Ishigaki , Jungeon Kim , Donghyung Lee , Hyeong-Cheon Park , Young-Ki Lee , Kyung Don Yoo , Korean Society of Nephrology Disaster Preparedness and Response Committee
Chronic kidney disease (CKD) and end-stage kidney disease (ESKD) represent growing global health burdens, driven by aging populations and increasing prevalence of diabetes and hypertension. Patients with ESKD face significant lifestyle limitations, particularly regarding mobility and travel, because of treatment schedules, medical complexity, and heightened vulnerability to complications. Air and long-distance travel pose distinct risks—cardiovascular stress, infection, and logistical challenges—that demand tailored assessment and preparation. This review outlines key travel-related complications in CKD and patients on dialysis, including cardiovascular and thromboembolic events, infection, jet lag, and vascular access risks. It further examines aeromedical considerations, such as hypobaric hypoxemia and venous thromboembolism (VTE), and evaluates airline-specific medical clearance protocols in major global carriers. Detailed recommendations are provided for hemodialysis and peritoneal dialysis (PD) patients, including pre-travel assessment, medication and supply management, international dialysis coordination, and inflight precautions.
慢性肾脏疾病(CKD)和终末期肾脏疾病(ESKD)代表着日益增长的全球健康负担,这是由人口老龄化和糖尿病和高血压患病率增加所驱动的。由于治疗计划、医疗复杂性和对并发症的易感性,ESKD患者面临着严重的生活方式限制,特别是在行动和旅行方面。航空和长途旅行带来了不同的风险——心血管压力、感染和后勤挑战——需要量身定制的评估和准备。这篇综述概述了CKD和透析患者主要的旅行相关并发症,包括心血管和血栓栓塞事件、感染、时差反应和血管通路风险。它进一步审查了航空医学方面的考虑因素,如低压低氧血症和静脉血栓栓塞(VTE),并评估了全球主要航空公司的航空公司特定医疗许可协议。为血液透析和腹膜透析(PD)患者提供了详细的建议,包括旅行前评估、药物和供应管理、国际透析协调和机上预防措施。
{"title":"From Dialysis to Destinations: Safe Travel Strategies for Patients With CKD","authors":"Seong Geun Kim ,&nbsp;Chia-Ter Chao ,&nbsp;Kornchanok Vareesangthip ,&nbsp;Winston Fung ,&nbsp;Mariko Miyazaki ,&nbsp;Sayaka Ishigaki ,&nbsp;Jungeon Kim ,&nbsp;Donghyung Lee ,&nbsp;Hyeong-Cheon Park ,&nbsp;Young-Ki Lee ,&nbsp;Kyung Don Yoo ,&nbsp;Korean Society of Nephrology Disaster Preparedness and Response Committee","doi":"10.1016/j.ekir.2025.09.035","DOIUrl":"10.1016/j.ekir.2025.09.035","url":null,"abstract":"<div><div>Chronic kidney disease (CKD) and end-stage kidney disease (ESKD) represent growing global health burdens, driven by aging populations and increasing prevalence of diabetes and hypertension. Patients with ESKD face significant lifestyle limitations, particularly regarding mobility and travel, because of treatment schedules, medical complexity, and heightened vulnerability to complications. Air and long-distance travel pose distinct risks—cardiovascular stress, infection, and logistical challenges—that demand tailored assessment and preparation. This review outlines key travel-related complications in CKD and patients on dialysis, including cardiovascular and thromboembolic events, infection, jet lag, and vascular access risks. It further examines aeromedical considerations, such as hypobaric hypoxemia and venous thromboembolism (VTE), and evaluates airline-specific medical clearance protocols in major global carriers. Detailed recommendations are provided for hemodialysis and peritoneal dialysis (PD) patients, including pre-travel assessment, medication and supply management, international dialysis coordination, and inflight precautions.</div></div>","PeriodicalId":17761,"journal":{"name":"Kidney International Reports","volume":"10 12","pages":"Pages 4162-4173"},"PeriodicalIF":5.7,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145651860","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
DMX-200 in Patients With Primary Focal Segmental Glomerulosclerosis: Results of the Phase 2 ACTION2 Trial DMX-200用于原发性局灶节段性肾小球硬化患者:2期ACTION2试验的结果
IF 5.7 2区 医学 Q1 UROLOGY & NEPHROLOGY Pub Date : 2025-12-01 DOI: 10.1016/j.ekir.2025.09.044
Hiddo J.L. Heerspink , Alessia Fornoni , Lesley A. Inker , Irene L. Noronha , Hernán Trimarchi , Muh Geot Wong , Alisha Smith , Robert Shepherd , Carl W. White , David Fuller , Jonathan Barratt
{"title":"DMX-200 in Patients With Primary Focal Segmental Glomerulosclerosis: Results of the Phase 2 ACTION2 Trial","authors":"Hiddo J.L. Heerspink ,&nbsp;Alessia Fornoni ,&nbsp;Lesley A. Inker ,&nbsp;Irene L. Noronha ,&nbsp;Hernán Trimarchi ,&nbsp;Muh Geot Wong ,&nbsp;Alisha Smith ,&nbsp;Robert Shepherd ,&nbsp;Carl W. White ,&nbsp;David Fuller ,&nbsp;Jonathan Barratt","doi":"10.1016/j.ekir.2025.09.044","DOIUrl":"10.1016/j.ekir.2025.09.044","url":null,"abstract":"","PeriodicalId":17761,"journal":{"name":"Kidney International Reports","volume":"10 12","pages":"Pages 4272-4276"},"PeriodicalIF":5.7,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145651995","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Donor-Derived Bartonella quintana Infections in 2 Kidney Transplant Recipients 2例肾移植受者供体源性金塔纳巴尔通体感染
IF 5.7 2区 医学 Q1 UROLOGY & NEPHROLOGY Pub Date : 2025-12-01 DOI: 10.1016/j.ekir.2025.09.030
Yanis Tamzali , Marie Dirix , Nadia Arzouk , Anne Scemla , Jean-Michel Correas , Victoria Donciu , Isabelle Podglajen , Jean-Paul Duong-Van Huyen , Jerome Tourret , Nathalie Chavarot
{"title":"Donor-Derived Bartonella quintana Infections in 2 Kidney Transplant Recipients","authors":"Yanis Tamzali ,&nbsp;Marie Dirix ,&nbsp;Nadia Arzouk ,&nbsp;Anne Scemla ,&nbsp;Jean-Michel Correas ,&nbsp;Victoria Donciu ,&nbsp;Isabelle Podglajen ,&nbsp;Jean-Paul Duong-Van Huyen ,&nbsp;Jerome Tourret ,&nbsp;Nathalie Chavarot","doi":"10.1016/j.ekir.2025.09.030","DOIUrl":"10.1016/j.ekir.2025.09.030","url":null,"abstract":"","PeriodicalId":17761,"journal":{"name":"Kidney International Reports","volume":"10 12","pages":"Pages 4304-4305"},"PeriodicalIF":5.7,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145651709","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Impact of Age-3 Urine Screening on Diagnosis and Treatment Timing in Alport Syndrome 3岁尿液筛查对Alport综合征诊断和治疗时机的影响
IF 5.7 2区 医学 Q1 UROLOGY & NEPHROLOGY Pub Date : 2025-12-01 DOI: 10.1016/j.ekir.2025.09.022
Hideaki Kitakado , Shingo Ishimori , Shuhei Aoyama , Yuka Kimura , Yuta Inoki , Chika Ueda , Yu Tanaka , Tomoko Horinouchi , Tomohiko Yamamura , Nana Sakakibara , China Nagano , Kandai Nozu

Introduction

Alport syndrome is an inherited kidney disease that leads to end-stage kidney disease (ESKD) due to pathogenic variants in COL4A3/4/5, which encode type IV collagen. Promptly identifying patients with Alport syndrome and starting treatment with a renin–angiotensin system inhibitor (RAS-I) is important for delaying progression to ESKD. In Japan, public urine screening is available for all children aged 3 years.

Methods

Patients genetically diagnosed with Alport syndrome at our department between August 2015 and May 2024, who were aged ≤ 18 years, were included in the study. We evaluated their clinical and genetic characteristics and identified the circumstances under which abnormal urine findings were first detected, with a focus on the role of urine screening at the age of 3 years (age-3 urine screening).

Results

A total of 356 patients with Alport syndrome were eligible for this study. The most common setting for detecting urine abnormalities for the first time was during age-3 urine screening (n = 113, 31.7%). The inherited forms were as follows: X-linked (XL) female (43.3%), XL male (30.1%), autosomal dominant (AD) (19.5%), and autosomal recessive (AR) (6.2%) Alport syndrome. In addition, 60.2% of these patients already met the criteria for RAS-I treatment at the time of urine screening.

Discussion

Our study showed that approximately 30% of patients with Alport syndrome had the opportunity to be diagnosed through age-3 urine screening, and among them, more than half were already eligible for RAS-I treatment. Urine screening may be an optimal method for contributing to a delay in the progression to ESKD in patients with Alport syndrome.
alport综合征是一种遗传性肾脏疾病,由于编码IV型胶原蛋白的COL4A3/4/5致病性变异导致终末期肾脏疾病(ESKD)。及时识别Alport综合征患者并开始使用肾素-血管紧张素系统抑制剂(RAS-I)治疗对于延缓ESKD进展非常重要。在日本,所有3岁儿童都可以进行公共尿液检查。方法选取2015年8月至2024年5月在我科就诊的年龄≤18岁遗传诊断为Alport综合征的患者作为研究对象。我们评估了他们的临床和遗传特征,并确定了首次发现异常尿液的情况,重点关注3岁时尿液筛查的作用(3岁尿液筛查)。结果共有356例Alport综合征患者符合本研究的条件。首次发现尿液异常的最常见情况是在3岁尿液筛查期间(n = 113, 31.7%)。遗传形式为:x连锁(XL)型女性(43.3%)、XL型男性(30.1%)、常染色体显性(AD)型(19.5%)、常染色体隐性(AR)型(6.2%)。此外,60.2%的患者在尿液筛查时已经符合RAS-I治疗标准。我们的研究表明,大约30%的Alport综合征患者有机会通过3岁尿液筛查被诊断出来,其中一半以上的患者已经符合RAS-I治疗的条件。尿液筛查可能是延迟Alport综合征患者进展为ESKD的最佳方法。
{"title":"Impact of Age-3 Urine Screening on Diagnosis and Treatment Timing in Alport Syndrome","authors":"Hideaki Kitakado ,&nbsp;Shingo Ishimori ,&nbsp;Shuhei Aoyama ,&nbsp;Yuka Kimura ,&nbsp;Yuta Inoki ,&nbsp;Chika Ueda ,&nbsp;Yu Tanaka ,&nbsp;Tomoko Horinouchi ,&nbsp;Tomohiko Yamamura ,&nbsp;Nana Sakakibara ,&nbsp;China Nagano ,&nbsp;Kandai Nozu","doi":"10.1016/j.ekir.2025.09.022","DOIUrl":"10.1016/j.ekir.2025.09.022","url":null,"abstract":"<div><h3>Introduction</h3><div>Alport syndrome is an inherited kidney disease that leads to end-stage kidney disease (ESKD) due to pathogenic variants in <em>COL4A3</em>/<em>4</em>/<em>5</em>, which encode type IV collagen. Promptly identifying patients with Alport syndrome and starting treatment with a renin–angiotensin system inhibitor (RAS-I) is important for delaying progression to ESKD. In Japan, public urine screening is available for all children aged 3 years.</div></div><div><h3>Methods</h3><div>Patients genetically diagnosed with Alport syndrome at our department between August 2015 and May 2024, who were aged ≤ 18 years, were included in the study. We evaluated their clinical and genetic characteristics and identified the circumstances under which abnormal urine findings were first detected, with a focus on the role of urine screening at the age of 3 years (age-3 urine screening).</div></div><div><h3>Results</h3><div>A total of 356 patients with Alport syndrome were eligible for this study. The most common setting for detecting urine abnormalities for the first time was during age-3 urine screening (<em>n</em> = 113, 31.7%). The inherited forms were as follows: X-linked (XL) female (43.3%), XL male (30.1%), autosomal dominant (AD) (19.5%), and autosomal recessive (AR) (6.2%) Alport syndrome. In addition, 60.2% of these patients already met the criteria for RAS-I treatment at the time of urine screening.</div></div><div><h3>Discussion</h3><div>Our study showed that approximately 30% of patients with Alport syndrome had the opportunity to be diagnosed through age-3 urine screening, and among them, more than half were already eligible for RAS-I treatment. Urine screening may be an optimal method for contributing to a delay in the progression to ESKD in patients with Alport syndrome.</div></div>","PeriodicalId":17761,"journal":{"name":"Kidney International Reports","volume":"10 12","pages":"Pages 4234-4240"},"PeriodicalIF":5.7,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145651850","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Barriers to and Facilitators of Phosphate Control in Children With CKD CKD儿童磷酸盐控制的障碍和促进因素
IF 5.7 2区 医学 Q1 UROLOGY & NEPHROLOGY Pub Date : 2025-12-01 DOI: 10.1016/j.ekir.2025.09.045
Louise McAlister , Vanessa Shaw , Pearl Pugh , Triona Joyce , Evelien Snauwaert , Fionna Bathgate , Charlotte Holt , Caroline Anderson , An Desloovere , José Renken-Terhaerdt , Maria Rosa Grassi , Sevcan Bakkaloğlu , Gulsah Sahin , Rukshana Shroff , Kelly Lambert

Introduction

Managing mineral and bone disorder in children with chronic kidney disease (CKD) requires control of serum phosphate levels. However, hyperphosphatemia is common, particularly in adolescents, reflecting suboptimal adherence to phosphate-binder medications and a reduced phosphate diet. We explored phosphate-related knowledge and adherence barriers in children, and their caregivers, using a sequential explanatory mixed-methods study design.

Methods

Children aged 8 to 18 years with CKD stages 4 and 5, on dialysis or post-transplantation, and caregivers, were recruited from 3 UK pediatric kidney centers. The Phosphate Understanding and Knowledge Assessment questionnaire was used to assess knowledge. Online focus groups explored real-world challenges to phosphate control.

Results

Forty-eight children and 43 caregivers were recruited; 44 (92%) children and 33 (75%) caregivers completed the questionnaire. Median knowledge scores were 64.3% (interquartile range, 55.3–78.6) for children and 72.7% (interquartile range, 64.3–85.7) for caregivers (P = 0.04). Older children scored higher (P = 0.01, R2 = 0.13), but knowledge did not correlate with serum phosphate. Dietary restriction was perceived as more challenging than using phosphate-binders (59% children; 71% caregivers). Forty-six participants, including 30 child-caregiver dyads, joined focus groups. The following 5 themes were identified encapsulating the experiences of families: practical advice and support are valued; personalized strategies are preferred to facilitate sense-making; the social environment of the child and family is disrupted; education and self-management skills can influence success; and the journey requires acceptance, adaptation, and perseverance.

Conclusions

In pediatric CKD, poor adherence to phosphate advice originates more from social and practical barriers than knowledge deficits. Our findings can inform personalized strategies to improve adherence in real-world settings.
治疗慢性肾脏疾病(CKD)患儿的矿物质和骨骼紊乱需要控制血清磷酸盐水平。然而,高磷血症很常见,特别是在青少年中,这反映了对磷酸盐结合剂药物和减少磷酸盐饮食的不理想依从性。我们采用顺序解释性混合方法研究设计,探讨了儿童及其照顾者中与磷酸盐相关的知识和依从性障碍。方法从英国3个儿科肾脏中心招募年龄在8 - 18岁的CKD 4期和5期、透析或移植后的儿童及其护理人员。采用磷酸盐理解与知识评估问卷进行知识评估。在线焦点小组探讨了现实世界中磷酸盐控制的挑战。结果共纳入48名儿童和43名护理人员;44名(92%)儿童和33名(75%)看护人完成了问卷。儿童的知识得分中位数为64.3%(四分位数范围为55.3 ~ 78.6),照顾者的知识得分中位数为72.7%(四分位数范围为64.3 ~ 85.7)(P = 0.04)。年龄较大的儿童得分较高(P = 0.01, R2 = 0.13),但知识与血清磷酸盐无关。饮食限制被认为比使用磷酸盐粘合剂更具挑战性(59%的儿童;71%的看护人)。46名参与者,包括30对照顾孩子的夫妇,加入了焦点小组。确定了以下5个主题,概括了家庭的经验:重视实际的建议和支持;个性化策略是促进意义形成的首选;儿童和家庭的社会环境被破坏;教育和自我管理技能可以影响成功;这段旅程需要接受、适应和坚持。结论:在儿童CKD中,磷酸盐建议依从性较差的主要原因是社会和实践障碍,而不是知识缺陷。我们的研究结果可以为个性化策略提供信息,以提高现实环境中的依从性。
{"title":"Barriers to and Facilitators of Phosphate Control in Children With CKD","authors":"Louise McAlister ,&nbsp;Vanessa Shaw ,&nbsp;Pearl Pugh ,&nbsp;Triona Joyce ,&nbsp;Evelien Snauwaert ,&nbsp;Fionna Bathgate ,&nbsp;Charlotte Holt ,&nbsp;Caroline Anderson ,&nbsp;An Desloovere ,&nbsp;José Renken-Terhaerdt ,&nbsp;Maria Rosa Grassi ,&nbsp;Sevcan Bakkaloğlu ,&nbsp;Gulsah Sahin ,&nbsp;Rukshana Shroff ,&nbsp;Kelly Lambert","doi":"10.1016/j.ekir.2025.09.045","DOIUrl":"10.1016/j.ekir.2025.09.045","url":null,"abstract":"<div><h3>Introduction</h3><div>Managing mineral and bone disorder in children with chronic kidney disease (CKD) requires control of serum phosphate levels. However, hyperphosphatemia is common, particularly in adolescents, reflecting suboptimal adherence to phosphate-binder medications and a reduced phosphate diet. We explored phosphate-related knowledge and adherence barriers in children, and their caregivers, using a sequential explanatory mixed-methods study design.</div></div><div><h3>Methods</h3><div>Children aged 8 to 18 years with CKD stages 4 and 5, on dialysis or post-transplantation, and caregivers, were recruited from 3 UK pediatric kidney centers. The Phosphate Understanding and Knowledge Assessment questionnaire was used to assess knowledge. Online focus groups explored real-world challenges to phosphate control.</div></div><div><h3>Results</h3><div>Forty-eight children and 43 caregivers were recruited; 44 (92%) children and 33 (75%) caregivers completed the questionnaire. Median knowledge scores were 64.3% (interquartile range, 55.3–78.6) for children and 72.7% (interquartile range, 64.3–85.7) for caregivers (<em>P</em> = 0.04). Older children scored higher (<em>P</em> = 0.01, <em>R</em><sup>2</sup> = 0.13), but knowledge did not correlate with serum phosphate. Dietary restriction was perceived as more challenging than using phosphate-binders (59% children; 71% caregivers). Forty-six participants, including 30 child-caregiver dyads, joined focus groups. The following 5 themes were identified encapsulating the experiences of families: practical advice and support are valued; personalized strategies are preferred to facilitate sense-making; the social environment of the child and family is disrupted; education and self-management skills can influence success; and the journey requires acceptance, adaptation, and perseverance.</div></div><div><h3>Conclusions</h3><div>In pediatric CKD, poor adherence to phosphate advice originates more from social and practical barriers than knowledge deficits. Our findings can inform personalized strategies to improve adherence in real-world settings.</div></div>","PeriodicalId":17761,"journal":{"name":"Kidney International Reports","volume":"10 12","pages":"Pages 4252-4263"},"PeriodicalIF":5.7,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145651856","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
期刊
Kidney International Reports
全部 Acc. Chem. Res. ACS Applied Bio Materials ACS Appl. Electron. Mater. ACS Appl. Energy Mater. ACS Appl. Mater. Interfaces ACS Appl. Nano Mater. ACS Appl. Polym. Mater. ACS BIOMATER-SCI ENG ACS Catal. ACS Cent. Sci. ACS Chem. Biol. ACS Chemical Health & Safety ACS Chem. Neurosci. ACS Comb. Sci. ACS Earth Space Chem. ACS Energy Lett. ACS Infect. Dis. ACS Macro Lett. ACS Mater. Lett. ACS Med. Chem. Lett. ACS Nano ACS Omega ACS Photonics ACS Sens. ACS Sustainable Chem. Eng. ACS Synth. Biol. Anal. Chem. BIOCHEMISTRY-US Bioconjugate Chem. BIOMACROMOLECULES Chem. Res. Toxicol. Chem. Rev. Chem. Mater. CRYST GROWTH DES ENERG FUEL Environ. Sci. Technol. Environ. Sci. Technol. Lett. Eur. J. Inorg. Chem. IND ENG CHEM RES Inorg. Chem. J. Agric. Food. Chem. J. Chem. Eng. Data J. Chem. Educ. J. Chem. Inf. Model. J. Chem. Theory Comput. J. Med. Chem. J. Nat. Prod. J PROTEOME RES J. Am. Chem. Soc. LANGMUIR MACROMOLECULES Mol. Pharmaceutics Nano Lett. Org. Lett. ORG PROCESS RES DEV ORGANOMETALLICS J. Org. Chem. J. Phys. Chem. J. Phys. Chem. A J. Phys. Chem. B J. Phys. Chem. C J. Phys. Chem. Lett. Analyst Anal. Methods Biomater. Sci. Catal. Sci. Technol. Chem. Commun. Chem. Soc. Rev. CHEM EDUC RES PRACT CRYSTENGCOMM Dalton Trans. Energy Environ. Sci. ENVIRON SCI-NANO ENVIRON SCI-PROC IMP ENVIRON SCI-WAT RES Faraday Discuss. Food Funct. Green Chem. Inorg. Chem. Front. Integr. Biol. J. Anal. At. Spectrom. J. Mater. Chem. A J. Mater. Chem. B J. Mater. Chem. C Lab Chip Mater. Chem. Front. Mater. Horiz. MEDCHEMCOMM Metallomics Mol. Biosyst. Mol. Syst. Des. Eng. Nanoscale Nanoscale Horiz. Nat. Prod. Rep. New J. Chem. Org. Biomol. Chem. Org. Chem. Front. PHOTOCH PHOTOBIO SCI PCCP Polym. Chem.
×
引用
GB/T 7714-2015
复制
MLA
复制
APA
复制
导出至
BibTeX EndNote RefMan NoteFirst NoteExpress
×
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
×
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
Book学术官方微信
Book学术文献互助
Book学术文献互助群
群 号:604180095
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