Maria G. Pucci , Mowa Ayibiowu , Jad Fadlallah , Aghna Wasim , Nathaniel Edwards , Madeline Li , Doris Howell , Susan Bartlett , John D. Peipert , Samantha Anthony , Istvan Mucsi , Janine Farragher
{"title":"Assessing Social Participation Among Kidney Transplant Recipients Using PROMIS Computer Adaptive Testing","authors":"Maria G. Pucci , Mowa Ayibiowu , Jad Fadlallah , Aghna Wasim , Nathaniel Edwards , Madeline Li , Doris Howell , Susan Bartlett , John D. Peipert , Samantha Anthony , Istvan Mucsi , Janine Farragher","doi":"10.1016/j.ekir.2025.05.023","DOIUrl":"10.1016/j.ekir.2025.05.023","url":null,"abstract":"<div><h3>Introduction</h3><div>Social participation is a valued aspect of quality of life among kidney transplant recipients; however, few validated measures exist to assess it. This study aimed to explore the reliability and validity of the Patient-Reported Outcomes Measurement Information System (PROMIS) Ability to Participate in Social Roles and Activities (PROMIS-SP), administered as a computer adaptive test (CAT), among kidney transplant recipients.</div></div><div><h3>Methods</h3><div>This was a cross-sectional study involving a convenience sample of adult recipients from Toronto, Canada. Participants completed the PROMIS-SP CAT and legacy measures of social participation on an electronic data capture platform. Reliability of the PROMIS-SP CAT was determined using standard error of measurement (SEM) and test-retest reliability using intraclass correlation coefficient. Convergent validity was assessed by calculating Spearman’s correlation between the PROMIS-SP CAT and legacy measures, and construct validity was assessed using known group comparisons.</div></div><div><h3>Results</h3><div>We recruited 284 participants with a mean (SD) age of 53 (14) years and a median of 5.5 years since kidney transplantation; 61% were male, 53% were White, and 30% had diabetes. The mean (SD) PROMIS T-score was 51 (9). Reliability (<em>r</em> = 0.93) and test-retest reliability (intraclass correlation coefficient = 0.97) of the PROMIS-SP CAT were excellent. Strong correlations were observed between PROMIS-SP CAT and social difficulty inventory (SDI) (rho = −0.65), SDI “everyday living” (rho = −0.68), and EuroQol-5 Dimensions-5 Levels (EQ5D5L) “usual activities” (rho = −0.66). PROMIS-SP CAT scores were significantly different between known groups in the expected direction.</div></div><div><h3>Conclusion</h3><div>Our results support the validity and reliability of PROMIS-SP CAT among kidney transplant recipients, and suggest that this tool can be used to identify recipients with restricted social participation.</div></div>","PeriodicalId":17761,"journal":{"name":"Kidney International Reports","volume":"10 8","pages":"Pages 2708-2719"},"PeriodicalIF":5.7,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144756951","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}
Felicitas E. Hengel , Michelle C.Z. Chong , Wing Yin Leung , Silke Dehde , Anne Mühlig , Matthias Janneck , Henry H.L. Wu , Tobias B. Huber , Arvind Ponnusamy , Nicola M. Tomas
{"title":"Non-invasive Diagnosis of Antinephrin–Associated Podocytopathy","authors":"Felicitas E. Hengel , Michelle C.Z. Chong , Wing Yin Leung , Silke Dehde , Anne Mühlig , Matthias Janneck , Henry H.L. Wu , Tobias B. Huber , Arvind Ponnusamy , Nicola M. Tomas","doi":"10.1016/j.ekir.2025.05.005","DOIUrl":"10.1016/j.ekir.2025.05.005","url":null,"abstract":"<div><h3>Introduction</h3><div>Circulating autoantibodies against the podocyte surface protein nephrin have recently been described in patients with podocytopathies, that is, minimal change disease, primary focal segmental glomerulosclerosis, and childhood idiopathic nephrotic syndrome. Their high specificity for podocytopathies in combination with a strong correlation with disease activity hold the potential for a non-invasive diagnosis, but prospective data are lacking.</div></div><div><h3>Methods</h3><div>Here, we describe 3 patients with contraindications or unwillingness for a kidney biopsy, hampering a timely histological diagnosis and choice of appropriate therapy.</div></div><div><h3>Results</h3><div>In all patients, antinephrin autoantibodies were detected by quantitative immunoprecipitation, prompting the initiation of adequate treatment. These interventions induced a decrease in antinephrin autoantibody levels and clinical remission.</div></div><div><h3>Conclusion</h3><div>Our study highlights the potential of antinephrin autoantibody measurement for a noninvasive diagnosis of antinephrin-associated podocytopathy.</div></div>","PeriodicalId":17761,"journal":{"name":"Kidney International Reports","volume":"10 8","pages":"Pages 2800-2804"},"PeriodicalIF":5.7,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144757345","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}
Marco Allinovi , Maxime Teisseyre , Matteo Accinno , Cecilia Finocchi , Vincent L.M. Esnault , Marion Cremoni , Tommaso Mazzierli , Daniela Lazzarini , Micaela Anna Casiraghi , Céline Fernandez , Kévin Zorzi , Vesna Brglez , Lorenzo Cosmi , Andrea Matucci , Leonardo Caroti , Giulia Antognoli , Calogero Lino Cirami , Alessandra Vultaggio , Barbara Seitz-Polski
{"title":"Anti-Rituximab Antibodies Occurrence and Clinical Outcomes in Patients With Primary Membranous Nephropathy","authors":"Marco Allinovi , Maxime Teisseyre , Matteo Accinno , Cecilia Finocchi , Vincent L.M. Esnault , Marion Cremoni , Tommaso Mazzierli , Daniela Lazzarini , Micaela Anna Casiraghi , Céline Fernandez , Kévin Zorzi , Vesna Brglez , Lorenzo Cosmi , Andrea Matucci , Leonardo Caroti , Giulia Antognoli , Calogero Lino Cirami , Alessandra Vultaggio , Barbara Seitz-Polski","doi":"10.1016/j.ekir.2025.04.059","DOIUrl":"10.1016/j.ekir.2025.04.059","url":null,"abstract":"<div><h3>Introduction</h3><div>Rituximab is a first-line treatment for primary membranous nephropathy (pMN), with proven efficacy and safety. The use of monoclonal antibodies such as rituximab can lead to the formation of antidrug antibodies that may interfere with the therapeutic response. In pMN, anti-rituximab antibodies (ARAs) have been shown to neutralize the cytotoxicity of rituximab, thereby increasing the risk of relapse of nephrotic syndrome. However, the kinetics of ARAs over time and the effect of ARA titer on prognosis are unclear.</div></div><div><h3>Methods</h3><div>This retrospective international multicenter study included 74 patients with pMN treated with rituximab. Here we aimed to clarify the correlation between ARAs and clinical outcome, as well as to evaluate the most appropriate timing of ARA detection.</div></div><div><h3>Results</h3><div>Overall, 35 out of 74 patients (47%) developed ARAs after a median of 9 (interquartile range: 6–12) months following rituximab administration. ARA monitoring at month-9, month-12 and before rituximab readministration identified 88% of patients with ARAs. Clinical remission rate at 6 and 12 months after rituximab administration was significantly lower in patients with ARAs (31% vs. 56%, <em>P</em> = 0.03 and 54% vs. 87%, <em>P</em> = 0.0017, respectively). ARAs were associated with a significantly higher rate of relapse (63% vs. 29%, <em>P</em> = 0.036) and a higher rate of B-cell reconstitution at 6 months (74.2% vs. 50%, <em>P</em> = 0.048). Notably, relapse occurred earlier in patients with ARAs (22 months vs. 32 months, <em>P</em> = 0.01).</div></div><div><h3>Conclusion</h3><div>The development of ARAs represents one of the most important prognostic factors in pMN, being significantly associated with a reduced remission rate and a higher relapse rate after rituximab therapy. Alternative therapies with obinutuzumab or ofatumumab should be considered for these patients.</div></div>","PeriodicalId":17761,"journal":{"name":"Kidney International Reports","volume":"10 8","pages":"Pages 2621-2629"},"PeriodicalIF":5.7,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144757417","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}
Alice Tata , Guillaume Rocha , Marguerite Hureaux , Alice S. Serafin , Esther Porée , Lucie Menguy , Nicolas Goudin , Nicolas Cagnard , Lilian Gréau , Marc Fila , Luis Briseño-Roa , Jean-Philippe Annereau , Sophie Saunier , Alexandre Benmerah
{"title":"Prostaglandin Analogs and Eupatilin as Treatments for Nephronophthisis","authors":"Alice Tata , Guillaume Rocha , Marguerite Hureaux , Alice S. Serafin , Esther Porée , Lucie Menguy , Nicolas Goudin , Nicolas Cagnard , Lilian Gréau , Marc Fila , Luis Briseño-Roa , Jean-Philippe Annereau , Sophie Saunier , Alexandre Benmerah","doi":"10.1016/j.ekir.2025.04.060","DOIUrl":"10.1016/j.ekir.2025.04.060","url":null,"abstract":"<div><h3>Introduction</h3><div>Primary cilia (PCs) are sensory antennae that are present on the majority of quiescent vertebrate cells where they mediate key signaling during development and in response to environmental stimuli. Defects in PCs result in a group of heterogeneous inherited disorders with overlapping phenotypes, called ciliopathies. Nephronophthisis is an autosomal recessive tubulointerstitial kidney ciliopathy with > 25 identified genes called <em>NPHP</em>. Presently, no treatment exists beyond supportive care and kidney transplant, underscoring the need for novel therapies.</div></div><div><h3>Methods</h3><div>Using a phenotypic screening approach in cultured cell lines, we previously identified prostaglandin analogues as candidate therapeutic molecules based on their ability to rescue ciliogenesis defects in kidney tubular cells from patients with <em>NPHP1</em> . Here, we investigated the potential beneficial effects of ROCK inhibitor and Eupatilin, similarly identified by other groups in different <em>NPHP</em> contexts, in kidney cells from patients with <em>NPHP1</em> and those with <em>IQCB1/NPHP5</em> as well as in a zebrafish <em>nphp</em> mutant line (<em>traf3ip1/ift54</em>).</div></div><div><h3>Results</h3><div>Eupatilin partially rescued <em>NPHP1</em>-associated ciliogenesis defects. Transcriptomic analyses pointed out that cell cycle progression was inhibited by Eupatilin, likely explaining its broad effects on cilia assembly. Interestingly, though ciliary defects also observed in <em>NPHP5</em> patient cells were rescued by both prostaglandins and Eupatilin, only prostaglandin analogues were able to reduce pronephric cysts size in the used <em>nphp</em> zebrafish model.</div></div><div><h3>Conclusion</h3><div>Our study indicates that these molecules can show beneficial effects across genetic contexts and shed light on their potential as therapeutic interventions for nephronophthisis.</div></div>","PeriodicalId":17761,"journal":{"name":"Kidney International Reports","volume":"10 8","pages":"Pages 2821-2835"},"PeriodicalIF":5.7,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144757295","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}
Erik Hansson , Rebekah A.I. Lucas , Jason R. Glaser , Ilana Weiss , Ulf Ekström , Magnus Abrahamson , Catharina Wesseling , David H. Wegman , Kristina Jakobsson
{"title":"Understanding Changes in Serum Creatinine During Work in Heat","authors":"Erik Hansson , Rebekah A.I. Lucas , Jason R. Glaser , Ilana Weiss , Ulf Ekström , Magnus Abrahamson , Catharina Wesseling , David H. Wegman , Kristina Jakobsson","doi":"10.1016/j.ekir.2025.04.047","DOIUrl":"10.1016/j.ekir.2025.04.047","url":null,"abstract":"","PeriodicalId":17761,"journal":{"name":"Kidney International Reports","volume":"10 8","pages":"Pages 2860-2863"},"PeriodicalIF":5.7,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144757289","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}
Christin Findeisen , Maria Papazian , Linda Pöschla , Anastasia Ertel , Wenjun Jin , Nydia Panitz , Elena Hantmann , Paul Coucke , Firdous Abdulwahab , Lama AlAbdi , Fawzan S. Alkuraya , May Salem , Hamad Alzaidan , Kai-Uwe Eckardt , Søren T. Christensen , Alexandre Benmerah , Sophie Saunier , Jan Halbritter , Ria Schönauer
{"title":"Structure-Activity Analysis Reveals Perturbed Cilia-Jun N-Terminal Kinase Signaling in MAPKBP1-Associated Kidney Disease","authors":"Christin Findeisen , Maria Papazian , Linda Pöschla , Anastasia Ertel , Wenjun Jin , Nydia Panitz , Elena Hantmann , Paul Coucke , Firdous Abdulwahab , Lama AlAbdi , Fawzan S. Alkuraya , May Salem , Hamad Alzaidan , Kai-Uwe Eckardt , Søren T. Christensen , Alexandre Benmerah , Sophie Saunier , Jan Halbritter , Ria Schönauer","doi":"10.1016/j.ekir.2025.05.049","DOIUrl":"10.1016/j.ekir.2025.05.049","url":null,"abstract":"<div><h3>Introduction</h3><div>Nephronophthisis (NPH) is a renal ciliopathy characterized by chronic tubulointerstitial fibrosis. Despite discovery of multiple disease genes, mechanisms of NPH-associated kidney degeneration remain poorly understood. In this study, we present details of clinical and molecular mechanisms of <em>MAPKBP1</em> (<em>NPHP20</em>) loss-of-function.</div></div><div><h3>Methods</h3><div>This study was a systematic clinical and <em>in vitro</em> analysis of all published and newly identified cases using overexpression systems, patient fibroblasts, and mitogen-activated protein kinase binding protein 1 (MAPKBP1) knock down cells.</div></div><div><h3>Results</h3><div>We demonstrated that <em>MAPKBP1</em>-NPH follows a distinct natural history, characterized by predominantly nonsyndromic kidney disease and exceptionally slow progression. Furthermore, we showed that endogenous MAPKBP1 is lost from ciliary basal bodies in patients with <em>NPHP20</em> and in <em>MAPKBP1</em> knock down, accompanied by shortened primary cilia. Overexpression of MAPKBP1 patient variants revealed impaired microtubule, centrosomal, and basal body localization. We propose that the activation status of Jun N-terminal kinase (JNK) determines the switch between centriolar association or dissociation of MAPKBP1 via distinct protein domains. Importantly, we found that JNK activation leads to the disassembly of cilia concomitantly with MAPKBP1 dissociation from the basal body. Downstream, we observed that impaired trafficking of phosphorylated JNK upon loss of MAPKBP1 and pharmacological disassembly of the JNK-target, actin, restores ciliary length in patients with <em>NPHP20</em>. Overall, MAPKBP1-associated molecular alterations appeared to be relatively modest, in line with late onset kidney function decline in patients with <em>NPHP20</em>.</div></div><div><h3>Conclusion</h3><div>In summary, we propose alterations in cilia-related JNK pathways as a novel mechanism in the development of NPH. Thus, a more detailed investigation of JNK signaling and involved protein interactions are promising for the discovery of novel targets for urgently needed treatment strategies in NPH.</div></div>","PeriodicalId":17761,"journal":{"name":"Kidney International Reports","volume":"10 8","pages":"Pages 2836-2851"},"PeriodicalIF":5.7,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144757292","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}
Aqeeb Ur Rehman , Stefania Renzi , David Andres Ballesteros Castro , Lilia Cervantes , René Clavero , Bianca Davidson , Mariano Rafael Farias , Vicente Campolo Girard , Rolando Claure-Del Granado , Gonzalo Ramirez-Guerrero , Darío Jiménez , Cristian Paul Leon Rabanal , Alejandra Molano Trivino , Thiago Reis , Lilia Rizo-Topete , Olynka Vega-Vega , Andrea K. Viecelli , Krissia Kamile Singer Wallbach-Massai , Marina Wainstein , Sabine Karam , Javier A. Neyra
{"title":"Barriers and Facilitators to Renal Replacement Therapy for Acute Kidney Injury in Latin America: Insights From an Expert Roundtable","authors":"Aqeeb Ur Rehman , Stefania Renzi , David Andres Ballesteros Castro , Lilia Cervantes , René Clavero , Bianca Davidson , Mariano Rafael Farias , Vicente Campolo Girard , Rolando Claure-Del Granado , Gonzalo Ramirez-Guerrero , Darío Jiménez , Cristian Paul Leon Rabanal , Alejandra Molano Trivino , Thiago Reis , Lilia Rizo-Topete , Olynka Vega-Vega , Andrea K. Viecelli , Krissia Kamile Singer Wallbach-Massai , Marina Wainstein , Sabine Karam , Javier A. Neyra","doi":"10.1016/j.ekir.2025.06.030","DOIUrl":"10.1016/j.ekir.2025.06.030","url":null,"abstract":"","PeriodicalId":17761,"journal":{"name":"Kidney International Reports","volume":"10 8","pages":"Pages 2515-2519"},"PeriodicalIF":5.7,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144757275","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}
{"title":"Topiramate and Kidney Protection: A Serendipitous Connection?","authors":"Silvio Borrelli , Antonio Russo","doi":"10.1016/j.ekir.2025.06.012","DOIUrl":"10.1016/j.ekir.2025.06.012","url":null,"abstract":"","PeriodicalId":17761,"journal":{"name":"Kidney International Reports","volume":"10 8","pages":"Pages 2530-2532"},"PeriodicalIF":5.7,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144757339","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}