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Clinical Presentation and Outcomes of Antineutrophil Cytoplasmic Autoantibody–Negative Pauci-Immune Glomerulonephritis 抗中性粒细胞胞浆自身抗体阴性的包囊免疫肾小球肾炎的临床表现和预后
IF 5.7 2区 医学
Kidney International Reports Pub Date : 2025-05-01 DOI: 10.1016/j.ekir.2025.02.032
Lauren Floyd , Anamay Shetty , Adam D. Morris , Krešimir Galešić , Mohamed Elsayed , Grace Lavery , Amrita Dhutia , Sorcha O’Brien , Sinead Stoneman , Allyson Egan , Mark A. Little , Vojtech Kratky , Zdenka Hruskova , Vladimir Tesar , Marek Kollar , Anke von Bergwelt-Baildon , Ulf Schönermarck , Eveline Y. Wu , Lauren Blazek , Vimal K. Derebail , Matija Crnogorac
{"title":"Clinical Presentation and Outcomes of Antineutrophil Cytoplasmic Autoantibody–Negative Pauci-Immune Glomerulonephritis","authors":"Lauren Floyd ,&nbsp;Anamay Shetty ,&nbsp;Adam D. Morris ,&nbsp;Krešimir Galešić ,&nbsp;Mohamed Elsayed ,&nbsp;Grace Lavery ,&nbsp;Amrita Dhutia ,&nbsp;Sorcha O’Brien ,&nbsp;Sinead Stoneman ,&nbsp;Allyson Egan ,&nbsp;Mark A. Little ,&nbsp;Vojtech Kratky ,&nbsp;Zdenka Hruskova ,&nbsp;Vladimir Tesar ,&nbsp;Marek Kollar ,&nbsp;Anke von Bergwelt-Baildon ,&nbsp;Ulf Schönermarck ,&nbsp;Eveline Y. Wu ,&nbsp;Lauren Blazek ,&nbsp;Vimal K. Derebail ,&nbsp;Matija Crnogorac","doi":"10.1016/j.ekir.2025.02.032","DOIUrl":"10.1016/j.ekir.2025.02.032","url":null,"abstract":"<div><h3>Introduction</h3><div>Antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis is a rare, complex autoimmune condition. Although ANCAs have a pathogenic role, they are considered a suboptimal biomarker of disease activity. Previous studies suggest differences in clinical phenotypes and outcomes in those without detectable circulating autoantibody. This study aimed to investigate the clinical presentation, histopathological findings, treatment practices, and outcomes of patients with ANCA-negative pauci-immune glomerulonephritis (PIGN).</div></div><div><h3>Methods</h3><div>A retrospective, multicenter cohort study was conducted from 2002 to 2022 and included those with biopsy-proven PIGN. We aimed to investigate differences in presentation, clinical outcomes, and treatment practices of patients with ANCA-negative PIGN when compared with ANCA-positive controls.</div></div><div><h3>Results</h3><div>In total, 132 ANCA-negative and 127 ANCA-positive patients were included. ANCA-negative patients were younger (<em>P</em> &lt; 0.001), more commonly presented with renal-limited disease (<em>P</em> &lt; 0.001), had worse estimated glomerular filtration rate at diagnosis (<em>P</em> &lt; 0.02) and higher rates of proteinuria (<em>P</em> &lt; 0.01). Controlling for age, sex, ethnicity, and recruiting center, ANCA-negative patients had lower rates of relapse (<em>P</em> &lt; 0.001) and higher rates of end-stage kidney disease (ESKD) at 1 and 3 years (<em>P</em> &lt; 0.001). Standard remission induction and maintenance therapies were used less often in ANCA-negative patients.</div></div><div><h3>Conclusion</h3><div>The precise pathophysiology and factors contributing to the clinical phenotype of ANCA-negative PIGN remain unclear and potentially represent a distinct disease entity. Adverse outcomes may result from delays in diagnosis, advanced disease at presentation, and less intense immunosuppressive treatment. Current classification criteria inadequately address ANCA-negative disease and collaborative research, which includes ANCA-negative patients in trials is needed.</div></div>","PeriodicalId":17761,"journal":{"name":"Kidney International Reports","volume":"10 5","pages":"Pages 1450-1459"},"PeriodicalIF":5.7,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143922973","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
Longitudinal Lipid Trajectories and Progression of CKD in Children 儿童CKD的纵向脂质轨迹和进展
IF 5.7 2区 医学
Kidney International Reports Pub Date : 2025-05-01 DOI: 10.1016/j.ekir.2025.02.007
Uwe Querfeld , Marietta Kirchner , Francesca Mencarelli , Karolis Azukaitis , Aysun Bayazit , Ali Duzova , Anke Doyon , Nur Canpolat , Ipek Kaplan Bulut , Lukasz Obrycki , Justine Bacchetta , Rukshana Shroff , Dusan Paripovic , Cengiz Candan , Jerome Harambat , Alev Yilmaz , Harika Alpay , Jun Oh , Hakan Erdogan , Claus P. Schmitt , Franz Schaefer
{"title":"Longitudinal Lipid Trajectories and Progression of CKD in Children","authors":"Uwe Querfeld ,&nbsp;Marietta Kirchner ,&nbsp;Francesca Mencarelli ,&nbsp;Karolis Azukaitis ,&nbsp;Aysun Bayazit ,&nbsp;Ali Duzova ,&nbsp;Anke Doyon ,&nbsp;Nur Canpolat ,&nbsp;Ipek Kaplan Bulut ,&nbsp;Lukasz Obrycki ,&nbsp;Justine Bacchetta ,&nbsp;Rukshana Shroff ,&nbsp;Dusan Paripovic ,&nbsp;Cengiz Candan ,&nbsp;Jerome Harambat ,&nbsp;Alev Yilmaz ,&nbsp;Harika Alpay ,&nbsp;Jun Oh ,&nbsp;Hakan Erdogan ,&nbsp;Claus P. Schmitt ,&nbsp;Franz Schaefer","doi":"10.1016/j.ekir.2025.02.007","DOIUrl":"10.1016/j.ekir.2025.02.007","url":null,"abstract":"<div><h3>Introduction</h3><div>There are discrepant findings regarding the effect of dyslipidemia on disease progression in adult patients with chronic kidney disease (CKD).</div></div><div><h3>Methods</h3><div>In a prospective cohort study of children with stage 3 to 5 (predialysis) CKD, triglycerides (TGs), total cholesterol (CHOL), low-density lipoprotein cholesterol (LDL-C), and high-density lipoprotein cholesterol (HDL-C) were measured semiannually. We investigated whether CKD progression is associated with serum lipid levels at baseline and with lipid trajectories during follow-up. CKD progression was defined as the time to a composite event of 50% reduction in estimated glomerular filtration rate (eGFR), eGFR &lt; 10 ml/min per 1.73 m<sup>2</sup>, or start of kidney replacement therapy. By semiparametric group-based trajectory modeling (GBTM), 2 trajectories were defined for each lipid, termed “high” and “low.”</div></div><div><h3>Results</h3><div>A total of 681 patients aged 12.2 ± 3.3 years with a mean eGFR of 26.9 ± 11.6 ml/min per 1.73 m<sup>2</sup> were included. Kidney diagnosis was classified as congenital anomalies of the kidneys and urinary tracts (CAKUT) in 69%, glomerulopathy in 8.4%, and other disorders in 22.6% of patients. During a median of 5.1 years of follow-up, 59% of patients reached the composite end point. Kidney survival was significantly different for HDL-C (<em>P</em> = 0.0128), but not for other lipid trajectories in the Kaplan-Meier analysis. There was no significant association of any of the lipid trajectories with CKD progression in Cox proportional hazard models. Variables consistently associated with CKD progression in models for each lipid at baseline and for lipid trajectories included age, a diagnosis other than CAKUT, eGFR at baseline, albuminuria, the serum albumin level, and diastolic blood pressure (BP).</div></div><div><h3>Conclusions</h3><div>These data do not support an important role for lipids in the progression of CKD in children.</div></div>","PeriodicalId":17761,"journal":{"name":"Kidney International Reports","volume":"10 5","pages":"Pages 1393-1403"},"PeriodicalIF":5.7,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143923078","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
Comprehensive Splice Pattern Analysis for Previously Reported OCRL Splicing Variants and Their Phenotypic Contributions 先前报道的OCRL剪接变异及其表型贡献的综合剪接模式分析
IF 5.7 2区 医学
Kidney International Reports Pub Date : 2025-05-01 DOI: 10.1016/j.ekir.2025.02.023
Rini Rossanti , Eri Okada , Nana Sakakibara , Ryota Suzuki , Yuta Inoki , Yuta Ichikawa , Yu Tanaka , Hideaki Kitakado , Chika Ueda , Atsushi Kondo , Yuya Aoto , China Nagano , Tomoko Horinouchi , Tomohiko Yamamura , Shingo Ishimori , Kandai Nozu
{"title":"Comprehensive Splice Pattern Analysis for Previously Reported OCRL Splicing Variants and Their Phenotypic Contributions","authors":"Rini Rossanti ,&nbsp;Eri Okada ,&nbsp;Nana Sakakibara ,&nbsp;Ryota Suzuki ,&nbsp;Yuta Inoki ,&nbsp;Yuta Ichikawa ,&nbsp;Yu Tanaka ,&nbsp;Hideaki Kitakado ,&nbsp;Chika Ueda ,&nbsp;Atsushi Kondo ,&nbsp;Yuya Aoto ,&nbsp;China Nagano ,&nbsp;Tomoko Horinouchi ,&nbsp;Tomohiko Yamamura ,&nbsp;Shingo Ishimori ,&nbsp;Kandai Nozu","doi":"10.1016/j.ekir.2025.02.023","DOIUrl":"10.1016/j.ekir.2025.02.023","url":null,"abstract":"<div><h3>Introduction</h3><div>Two distinct phenotypes of Dent disease-2 and Lowe syndrome are caused by <em>oculocerebrorenal syndrome of Lowe</em> (<em>OCRL</em>) abnormality. Previous genetic studies demonstrated that truncating variants in exons 1 to 7 results in Dent disease-2 and in exons 8 to 24, result in Lowe syndrome. Recently, we successfully identified a functional <em>OCRL</em> isoform, whose altered initiation codons (Met187 and Met206) in exon 8 can affect the <em>OCRL</em>-truncating variant phenotypes. However, the association between <em>OCRL</em> splicing variants and phenotypes is poorly understood.</div></div><div><h3>Methods</h3><div>We performed a detailed splicing pattern analysis of previously reported 28 <em>OCRL</em> splicing variants obtained from the Human Gene Mutation Database. We assessed the variant consequences at the mRNA level using an <em>in vitro</em> splicing assay with a minigene system, and examined their compatibility with <em>in silico</em> algorithms and correlation with disease phenotypes.</div></div><div><h3>Results</h3><div>Aberrant splicing was confirmed in all 27 variants, except for 1, in which splicing could not be experimentally confirmed in the minigene system, and therefore could not be concluded with certainty. Splicing variants in <em>OCRL</em> exons 1 to 7 resulted in Dent disease-2, and in exons 9 to 24 resulted in Lowe syndrome. In 1 case, c.561-2 A &gt; G in exon 8 demonstrated Dent disease-2.</div></div><div><h3>Conclusion</h3><div>This study provides significant data on the pathogenicity of <em>OCRL</em> splicing variants and genotype-phenotype correlations. In c.561-2 A &gt; G, the latter altered initiation codon of the OCRL isoform (Met206) was preserved, potentially indicating the Dent disease-2 phenotype. This result supports our recent finding regarding the altered initiation codons in exon 8 of the OCRL isoform.</div></div>","PeriodicalId":17761,"journal":{"name":"Kidney International Reports","volume":"10 5","pages":"Pages 1509-1517"},"PeriodicalIF":5.7,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143923095","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
Gene-Environment Interactions Among Black Adults With Kidney Failure – Not A Straight Forward Relationship 黑人成年人肾衰竭的基因-环境相互作用-不是直接的关系
IF 5.7 2区 医学
Kidney International Reports Pub Date : 2025-05-01 DOI: 10.1016/j.ekir.2025.03.038
Jessica L. Harding , Stephen O. Pastan
{"title":"Gene-Environment Interactions Among Black Adults With Kidney Failure – Not A Straight Forward Relationship","authors":"Jessica L. Harding ,&nbsp;Stephen O. Pastan","doi":"10.1016/j.ekir.2025.03.038","DOIUrl":"10.1016/j.ekir.2025.03.038","url":null,"abstract":"","PeriodicalId":17761,"journal":{"name":"Kidney International Reports","volume":"10 5","pages":"Pages 1332-1334"},"PeriodicalIF":5.7,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143923135","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
Urinary Dickkopf-Related Protein 3—A Potential Long-Term Biomarker for Progressive CKD in Children 尿dickkopf相关蛋白3-A可能是儿童进行性CKD的长期生物标志物
IF 5.7 2区 医学
Kidney International Reports Pub Date : 2025-05-01 DOI: 10.1016/j.ekir.2025.02.003
Katalin Dittrich , Emilia Marczak , Richard Wagner , Wieland Kiess , Lea Maria Merz
{"title":"Urinary Dickkopf-Related Protein 3—A Potential Long-Term Biomarker for Progressive CKD in Children","authors":"Katalin Dittrich ,&nbsp;Emilia Marczak ,&nbsp;Richard Wagner ,&nbsp;Wieland Kiess ,&nbsp;Lea Maria Merz","doi":"10.1016/j.ekir.2025.02.003","DOIUrl":"10.1016/j.ekir.2025.02.003","url":null,"abstract":"","PeriodicalId":17761,"journal":{"name":"Kidney International Reports","volume":"10 5","pages":"Pages 1582-1586"},"PeriodicalIF":5.7,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143923164","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
The Utility of Myeloperoxidase Immunostaining to Characterize Immune Deposits in Patients With Crescentic Glomerulonephritis 髓过氧化物酶免疫染色在新月形肾小球肾炎患者免疫沉积中的应用
IF 5.7 2区 医学
Kidney International Reports Pub Date : 2025-05-01 DOI: 10.1016/j.ekir.2025.03.001
Rachelle Gietzen , Tiffany N. Caza , Alejandro Best-Rocha , Christopher P. Larsen
{"title":"The Utility of Myeloperoxidase Immunostaining to Characterize Immune Deposits in Patients With Crescentic Glomerulonephritis","authors":"Rachelle Gietzen ,&nbsp;Tiffany N. Caza ,&nbsp;Alejandro Best-Rocha ,&nbsp;Christopher P. Larsen","doi":"10.1016/j.ekir.2025.03.001","DOIUrl":"10.1016/j.ekir.2025.03.001","url":null,"abstract":"<div><h3>Introduction</h3><div>Pauci-immune crescentic glomerulonephritis (GN) is nearly synonymous with antineutrophil cytoplasmic antibody (ANCA)–associated disease. Cases with immune complex deposition create a diagnostic conundrum leading to suspicion for concurrent infection or autoimmune disease. Small case series have demonstrated myeloperoxidase (MPO) in the immune deposits in patients with membranous nephropathy (MN) and ANCA-associated disease. However, the specificity of MPO staining to characterize immune deposits in crescentic GN has not been thoroughly evaluated.</div></div><div><h3>Methods</h3><div>We performed MPO immunostaining of 143 kidney biopsies, including pauci-immune crescentic GN (<em>n</em> = 15), ANCA with immune-complex crescentic GN (<em>n</em> = 20), MN without crescents (<em>n</em> = 24), endocarditis-associated crescentic GN (<em>n</em> = 25), hydralazine-associated crescentic GN (<em>n</em> = 11), and concurrent crescentic GN and MN without phospholipase A2 receptor (PLA2R) (<em>n</em> = 38) and with PLA2R (<em>n</em> = 10). MPO immunohistochemistry (IHC) was evaluated for positivity, character, and location of MPO immune deposits by 4 blinded pathologists.</div></div><div><h3>Results</h3><div>In patients with dual crescentic GN and MN without PLA2R, 84.2% were MPO-IHC positive. Crescentic GN with mesangial IgG was MPO-IHC positive in 40%. Crescentic GN related to hydralazine exposure was MPO-IHC positive in 72.7%. All cases with pauci-immune crescentic GN, endocarditis-associated cases, and MN cases with known antigens were negative for MPO.</div></div><div><h3>Conclusion</h3><div>Our study demonstrated that glomerular immune deposits in patients with crescentic GN with positive MPO serology demonstrated MPO positivity in the pattern of immune deposits in the majority of cases. Glomerular immune complexes in patients with MPO-positive crescentic GN therefore represent MPO-IgG immune complexes and should be thought of as one disease rather than a second disease process.</div></div>","PeriodicalId":17761,"journal":{"name":"Kidney International Reports","volume":"10 5","pages":"Pages 1460-1467"},"PeriodicalIF":5.7,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143922901","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
Kidney Transplantation Improves Survival in Antineutrophil Cytoplasmic Antibody–Associated Vasculitides With End-Stage Kidney Disease 肾移植可提高抗中性粒细胞细胞质抗体相关血管增生终末期肾病患者的生存率
IF 5.7 2区 医学
Kidney International Reports Pub Date : 2025-05-01 DOI: 10.1016/j.ekir.2025.02.001
Benoît Brilland , Jean-François Augusto , Thomas Jouve , Noémie Jourde-Chiche , Cécile Couchoud , Renal Epidemiology and Information Network (REIN) registry
{"title":"Kidney Transplantation Improves Survival in Antineutrophil Cytoplasmic Antibody–Associated Vasculitides With End-Stage Kidney Disease","authors":"Benoît Brilland ,&nbsp;Jean-François Augusto ,&nbsp;Thomas Jouve ,&nbsp;Noémie Jourde-Chiche ,&nbsp;Cécile Couchoud ,&nbsp;Renal Epidemiology and Information Network (REIN) registry","doi":"10.1016/j.ekir.2025.02.001","DOIUrl":"10.1016/j.ekir.2025.02.001","url":null,"abstract":"<div><h3>Introduction</h3><div>Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) frequently leads to end-stage kidney disease (ESKD). Although kidney transplantation (KT) is considered the optimal treatment for ESKD, its survival benefit in patients with AAV remains understudied. This study aimed to determine the impact of KT on survival in waitlisted patients with AAV-induced ESKD (AAV-ESKD).</div></div><div><h3>Methods</h3><div>We conducted a retrospective analysis of patients with AAV-ESKD registered in the French Renal Epidemiology and Information Network (REIN) registry and waitlisted for KT between 2002 and 2022. KT was treated as a time-dependent variable to avoid immortal time bias. Survival was assessed using Kaplan-Meier analysis and Cox proportional hazards models, adjusting for key demographic and clinical factors. Subgroup analyses were conducted based on vasculitis type, age, sex, and year of ESKD onset.</div></div><div><h3>Results</h3><div>Of 1165 patients with AAV-ESKD, 468 (40%) were waitlisted, and 318 of these (68%) received a transplant. After a median follow-up of 61 months after waitlisting, KT was associated with a 53% reduction in mortality risk (adjusted hazard ratio [HR] = 0.47 [0.31–0.73], <em>P</em> &lt; 0.001). This benefit was consistent across subgroups. Patient survival at 10 years was 72% for transplant recipients versus 28% for nontransplanted patients (<em>P</em> &lt; 0.001). Sensitivity analyses, after excluding recipients of living donors and patients removed from the waitlist supported the robustness of these findings. Within 2 years from ESKD onset, 24% of waitlisted patients were transplanted. Graft failure probability was 22% at 10 years posttransplant.</div></div><div><h3>Conclusion</h3><div>KT is associated with a significant survival benefit in waitlisted patients with AAV-ESKD compared with waiting on dialysis. These findings emphasize the importance of timely transplant evaluation and improved access to KT for this population.</div></div>","PeriodicalId":17761,"journal":{"name":"Kidney International Reports","volume":"10 5","pages":"Pages 1415-1427"},"PeriodicalIF":5.7,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143922970","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
Nephrotic Syndrome Thromboprophylaxis With Direct Oral Anticoagulants or Vitamin K Antagonists 直接口服抗凝剂或维生素K拮抗剂预防肾病综合征血栓形成
IF 5.7 2区 医学
Kidney International Reports Pub Date : 2025-05-01 DOI: 10.1016/j.ekir.2025.02.028
Edouard Cubilier , Youcef Chergui , Cyril Garrouste , Ines Ramos , Carole Philipponnet , Alba Atenza , Clarisse Greze , Julien Aniort , Charlotte Uro-Coste , Anne-Elisabeth Heng
{"title":"Nephrotic Syndrome Thromboprophylaxis With Direct Oral Anticoagulants or Vitamin K Antagonists","authors":"Edouard Cubilier ,&nbsp;Youcef Chergui ,&nbsp;Cyril Garrouste ,&nbsp;Ines Ramos ,&nbsp;Carole Philipponnet ,&nbsp;Alba Atenza ,&nbsp;Clarisse Greze ,&nbsp;Julien Aniort ,&nbsp;Charlotte Uro-Coste ,&nbsp;Anne-Elisabeth Heng","doi":"10.1016/j.ekir.2025.02.028","DOIUrl":"10.1016/j.ekir.2025.02.028","url":null,"abstract":"<div><h3>Introduction</h3><div>Nephrotic syndrome (NS) is a pathological state of the glomerular filtration barrier associated with an increased venous and arterial thrombotic risk. Current guidelines suggest heparin-based or vitamin K antagonist (VKA) regimens for thromboprophylaxis in such patients. Although widely prescribed for other indications, direct oral anticoagulants (DOACs) are not recommended in NS because of limited pharmacological and safety reports. This study aimed to compare DOACs and VKAs for thromboprophylaxis in NS, specifically regarding thrombotic events (TEs) and bleeding events (BEs).</div></div><div><h3>Methods</h3><div>We conducted a retrospective monocentric analysis of recorded NS episodes that required prophylactic anticoagulation between January 2006 and December 2023. We included 133 NS episodes of which 51 were treated with DOACs and 82 with VKAs. The primary endpoint was a composite endpoint, including thrombosis occurrence and major or clinically significant BEs during thromboprophylaxis. The secondary endpoints consisted of relevant features potentially involved when each primary endpoint was considered independently.</div></div><div><h3>Results</h3><div>Patient characteristics, underlying NS etiology, personal thrombotic and bleeding risk factors, and biological parameters were globally similar in both groups. The primary endpoint appeared similar in both groups (<em>P</em> = 0.481). The secondary endpoints were mostly hypothesis-generating because of the low TE (<em>n</em> = 2) and BE (<em>n</em> = 7) occurrences.</div></div><div><h3>Conclusion</h3><div>This study provides reassuring clinical data on DOAC use in NS thromboprophylaxis compared with VKAs, the recommended therapy, and calls for confirmation in randomized controlled trials (RCTs) and larger pharmacological studies.</div></div>","PeriodicalId":17761,"journal":{"name":"Kidney International Reports","volume":"10 5","pages":"Pages 1468-1475"},"PeriodicalIF":5.7,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143923065","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
Scoping Review of Kidney Patients and Providers Perspectives on Disaster Management 肾病患者和提供者在灾害管理方面的观点的范围综述
IF 5.7 2区 医学
Kidney International Reports Pub Date : 2025-05-01 DOI: 10.1016/j.ekir.2025.02.030
Elie Fadel , Shreya Udupa , Isabelle Ethier , Alessia N. Paparella , Lindsay Hales , Caroline Stigant , Laura Horowitz , Catherine Weber , Shaifali Sandal
{"title":"Scoping Review of Kidney Patients and Providers Perspectives on Disaster Management","authors":"Elie Fadel ,&nbsp;Shreya Udupa ,&nbsp;Isabelle Ethier ,&nbsp;Alessia N. Paparella ,&nbsp;Lindsay Hales ,&nbsp;Caroline Stigant ,&nbsp;Laura Horowitz ,&nbsp;Catherine Weber ,&nbsp;Shaifali Sandal","doi":"10.1016/j.ekir.2025.02.030","DOIUrl":"10.1016/j.ekir.2025.02.030","url":null,"abstract":"<div><h3>Introduction</h3><div>Patients with kidney disease are uniquely vulnerable to disasters and the need to understand stakeholder experiences to improve disaster preparedness has been highlighted. We aimed to explore the existing literature capturing patient and provider perspectives, identify research gaps, and develop research priorities in disaster management.</div></div><div><h3>Methods</h3><div>This was a scoping review of the empirical literature that has explored the lived experience and preparedness of patients, caregivers, or healthcare professionals during natural or human-caused disasters. A content analysis using an inductive approach was conducted.</div></div><div><h3>Results</h3><div>Of the 11,146 titles and abstracts screened, 22 met our inclusion criteria and emerged from Japan (<em>n</em> = 8), USA (<em>n</em> = 8), Syria (<em>n</em> = 2), Turkey (<em>n</em> = 2), China (<em>n</em> = 1), and Europe (<em>n</em> = 1, related to the Russian invasion of Ukraine). The outcomes examined were variable focusing on the following 4 aspects of disaster management: (i) identifying patient-level issues (preparedness, personal challenges, and psychosocial impact); (ii) damage assessment (infrastructure and equipment, personnel, and patient outcomes); (iii) response assessment (hemodialysis treatments delivered or missed, delivery of other kidney replacement therapies, and identifying practice gaps); and (iv) system assessment (examining capabilities and addressing surge capacity). The studies were at risk of survivor bias and most only used an investigator-designed survey for data collection. There was a dearth of evidence capturing the perspectives of caregivers, and pediatric and other vulnerable patients.</div></div><div><h3>Conclusion</h3><div>The literature examining patient and provider perspectives or experiences is scarce and at risk of bias. Methodological, population, outcome, process, and impact priorities are proposed to guide future research initiatives and generate evidence to inform context and disaster-specific relief efforts.</div></div>","PeriodicalId":17761,"journal":{"name":"Kidney International Reports","volume":"10 5","pages":"Pages 1346-1359"},"PeriodicalIF":5.7,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143923074","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
Treatment of Nephrotic Syndrome With Antinephrin Antibodies Using Plasmapheresis, Rituximab, and Mycophenolate Mofetil 血浆置换、利妥昔单抗和霉酚酸酯联合抗肾上腺素抗体治疗肾病综合征
IF 5.7 2区 医学
Kidney International Reports Pub Date : 2025-05-01 DOI: 10.1016/j.ekir.2025.02.017
Keiichi Takizawa , Yoko Shirai , Yuko Kajiho , Shoichiro Kanda , Motohiro Kato , Hiroyuki Abe , Kenichiro Miura , Motoshi Hattori , Yutaka Harita
{"title":"Treatment of Nephrotic Syndrome With Antinephrin Antibodies Using Plasmapheresis, Rituximab, and Mycophenolate Mofetil","authors":"Keiichi Takizawa ,&nbsp;Yoko Shirai ,&nbsp;Yuko Kajiho ,&nbsp;Shoichiro Kanda ,&nbsp;Motohiro Kato ,&nbsp;Hiroyuki Abe ,&nbsp;Kenichiro Miura ,&nbsp;Motoshi Hattori ,&nbsp;Yutaka Harita","doi":"10.1016/j.ekir.2025.02.017","DOIUrl":"10.1016/j.ekir.2025.02.017","url":null,"abstract":"","PeriodicalId":17761,"journal":{"name":"Kidney International Reports","volume":"10 5","pages":"Pages 1591-1593"},"PeriodicalIF":5.7,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143923097","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
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