Kidney International Reports最新文献

{"title":"WCN25-561 SALT WON’T BREAK THIS BRAIN: A CASE OF SEVERE HYPERNATREMIA AND OVERCORRECTION WITHOUT NOTICEABLE CHANGES IN MENTAL STATUS","authors":"Roy Rajan ∗ , Andrew Yanik , Charles Hopley , Clay Block , Thomas Kaneko","doi":"10.1016/j.ekir.2024.11.073","DOIUrl":"10.1016/j.ekir.2024.11.073","url":null,"abstract":"","PeriodicalId":17761,"journal":{"name":"Kidney International Reports","volume":"10 2","pages":"Page S2"},"PeriodicalIF":5.7,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143135902","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Response to “Refining Urgent-Start Peritoneal Dialysis: Definitions, Techniques, and Efficacy for Broader Adoption”","authors":"George Tsihlis , Keiren Pirabhahar , Frederika Sciberras , MaryAnn Nicdao , Laraine Aw , Alvie Agoo , Vincent Lee , Jennifer Li , Lukas Kairaitis , Kamal Sud , Jan Swinnen , Katrina Chau","doi":"10.1016/j.ekir.2024.11.1367","DOIUrl":"10.1016/j.ekir.2024.11.1367","url":null,"abstract":"","PeriodicalId":17761,"journal":{"name":"Kidney International Reports","volume":"10 2","pages":"Pages 625-626"},"PeriodicalIF":5.7,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143160027","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Improving Transparency and Relevance of the Clinical Practice Guidelines in Nephrology","authors":"David J. Tunnicliffe , Martin Howell , Vincent Lee , Jonathan C. Craig","doi":"10.1016/j.ekir.2024.11.031","DOIUrl":"10.1016/j.ekir.2024.11.031","url":null,"abstract":"","PeriodicalId":17761,"journal":{"name":"Kidney International Reports","volume":"10 2","pages":"Pages 299-301"},"PeriodicalIF":5.7,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143160845","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"COL4A5 Intronic Variants at Third to Fifth Nucleotides Cause Alport Syndrome","authors":"Hideaki Kitakado ,&nbsp;Tomoko Horinouchi ,&nbsp;Shuhei Aoyama ,&nbsp;Yuka Kimura ,&nbsp;Yuta Inoki ,&nbsp;Yu Tanaka ,&nbsp;Chika Ueda ,&nbsp;Yuya Aoto ,&nbsp;Nana Sakakibara ,&nbsp;China Nagano ,&nbsp;Tomohiko Yamamura ,&nbsp;Shingo Ishimori ,&nbsp;Rini Rossanti ,&nbsp;Masafumi Matsuo ,&nbsp;Kandai Nozu","doi":"10.1016/j.ekir.2024.11.016","DOIUrl":"10.1016/j.ekir.2024.11.016","url":null,"abstract":"<div><h3>Introduction</h3><div>Alport syndrome (AS) is an inherited kidney disease caused by variants in the <em>COL4A3</em>, <em>COL4A4,</em> or <em>COL4A5</em> genes, resulting in type IV collagen abnormalities. Although autosomal dominant variants in <em>COL4A3</em> and <em>COL4A4</em> are increasingly being diagnosed, X-linked AS (XLAS) caused by <em>COL4A5</em> variants predominates. Single nucleotide substitutions in introns positioned at first and second from the last nucleotide (called a consensus sequence) of exons always cause aberrant splicing. However, whether intronic variants at the third to fifth positions from the last nucleotide of exons can cause aberrant splicing is unclear.</div></div><div><h3>Methods</h3><div>We identified 11 intronic variants positioned at the third, fourth, and fifth nucleotides from the exon 3′ end in <em>COL4A5</em> from our AS cohort (January 2006–July 2022). We conducted <em>in vitro</em> splicing assays using minigenes and <em>in silico</em> splicing analysis using commercial splicing prediction software and evaluated mRNA sequences obtained from patients’ samples when available.</div></div><div><h3>Results</h3><div>All 11 patients showed aberrant splicing patterns in the minigene splicing assays. <em>In vivo</em> analysis of 6 patients corroborated these findings. The commercial splicing prediction software accurately predicted splicing changes in 10 variants.</div></div><div><h3>Conclusions</h3><div>This study shows that 11 intronic variants at the third to fifth positions in <em>COL4A5</em> introns cause aberrant splicing. This finding highlights the importance of evaluating such variants for the diagnosis and prognosis of XLAS. Further investigation is warranted to confirm the pathogenicity of these variants and their effect on the prognosis of the kidney in XLAS.</div></div>","PeriodicalId":17761,"journal":{"name":"Kidney International Reports","volume":"10 2","pages":"Pages 516-521"},"PeriodicalIF":5.7,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143160854","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The Role of Mitochondria in Diabetic Kidney Disease and Potential Therapeutic Targets","authors":"Masanobu Takasu ,&nbsp;Seiji Kishi ,&nbsp;Hajime Nagasu ,&nbsp;Kengo Kidokoro ,&nbsp;Craig R. Brooks ,&nbsp;Naoki Kashihara","doi":"10.1016/j.ekir.2024.10.035","DOIUrl":"10.1016/j.ekir.2024.10.035","url":null,"abstract":"<div><div>Diabetic kidney disease (DKD) is recognized worldwide as a leading cause of end-stage renal failure. Although therapies that target glomerular hemodynamics and can inhibit disease progression have been developed, there is currently no fundamental cure for the disease. Mitochondria play an important role in cellular respiration, producing adenosine triphosphate (ATP) by oxidative phosphorylation, and are essential for renal function, especially in proximal tubular cells (PTCs). In diabetic conditions, maintaining mitochondrial health is vital for preserving renal function. Under diabetic conditions, excessive reactive oxygen species (ROS) can damage mitochondrial DNA (mtDNA), leading to renal dysfunction. Strategies targeting mitochondrial function, such as AMP-activated protein kinase (AMPK) activation and modulation of nitric oxide (NO) availability, are promising for suppressing diabetic nephropathy. The immune response to DKD, initiated by detecting damage- and pathogen-associated molecular patterns, has a significant impact on the progression of DKD, including leakage of mtDNA and RNA, leading to inflammation through various pathways. This contributes to renal impairment characterized by hyperfiltration, endothelial dysfunction, and albuminuria. Mitochondrial energy metabolism and dynamics induced by hyperglycemia precede the onset of albuminuria and histological changes in the kidneys. The increased mitochondrial fission and decreased fusion that occur under diabetic conditions result in ATP depletion and exacerbate cellular dysfunction. Therapeutic strategies focused on restoring mitochondrial function are promising for slowing the progression of DKD and reduce the adverse effects on renal function. Sodium-glucose cotransporter-2 inhibitors (SGLT2is) and glucagon-like peptide-1 (GLP-1) receptor agonists, already in clinical use, have been shown to be protective for mitochondria, and nuclear factor erythroid 2-related factor 2 (Nrf2) activation and mitochondrial dynamics are promising drug discovery targets for further research.</div></div>","PeriodicalId":17761,"journal":{"name":"Kidney International Reports","volume":"10 2","pages":"Pages 328-342"},"PeriodicalIF":5.7,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143161285","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"WCN25-3713 MOLECULAR PROFILING FROM THE RENAL AL AMYLOID INVOLVEMENT AND NEOD00 (RAIN) TRIAL SHOW HETEROGENEITY IN GENE EXPRESSION","authors":"Felix Eichinger ∗ ,&nbsp;Cindy Varga ,&nbsp;Viji Nair ,&nbsp;Abhijit Naik ,&nbsp;Samih Nasr ,&nbsp;Agnes Fogo ,&nbsp;Denis Toskic ,&nbsp;Matthias Kretzler ,&nbsp;Raymond Comenzo","doi":"10.1016/j.ekir.2024.11.099","DOIUrl":"10.1016/j.ekir.2024.11.099","url":null,"abstract":"","PeriodicalId":17761,"journal":{"name":"Kidney International Reports","volume":"10 2","pages":"Page S17"},"PeriodicalIF":5.7,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143135899","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Nearing the Finish Line: Steady Progress in the Development of Complement Inhibitors for Glomerular Disease","authors":"Joshua M. Thurman","doi":"10.1016/j.ekir.2024.11.1370","DOIUrl":"10.1016/j.ekir.2024.11.1370","url":null,"abstract":"","PeriodicalId":17761,"journal":{"name":"Kidney International Reports","volume":"10 2","pages":"Pages 302-305"},"PeriodicalIF":5.7,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143161286","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Impact of Different CKD Definitions on Long-Term Renal Function and Mortality in a Population-Based Cohort Study","authors":"Delal Dalga ,&nbsp;Aurélie Huber ,&nbsp;Anne Dufey ,&nbsp;Peter Vollenweider ,&nbsp;Pedro Marques-Vidal ,&nbsp;Sophie de Seigneux ,&nbsp;Belen Ponte ,&nbsp;Lena Berchtold","doi":"10.1016/j.ekir.2024.11.027","DOIUrl":"10.1016/j.ekir.2024.11.027","url":null,"abstract":"<div><h3>Introduction</h3><div>The adoption of age or individualized body surface area (i-BSA) estimated glomerular filtration rate (eGFR) thresholds could influence the prevalence and prognosis of chronic kidney disease (CKD). This longitudinal study with up to 15 years of follow-up in the general population, compares different eGFR thresholds for CKD definition: standard, corrected to i-BSA, and age-stratified. For each, we assessed the prevalence of CKD and the combined impact on rapid renal function decline (RRFD) and mortality.</div></div><div><h3>Methods</h3><div>Patients were classified as CKD according to the presence of significant albuminuria and/or different eGFR thresholds as follows: (i) &lt; 60ml/min per 1.73 m²; (ii) &lt; 60ml/min corrected to i-BSA; (iii) stratified by age, that is, &lt; 75, &lt; 60 and &lt; 45 ml/min per 1.73 m² if aged &lt; 40 years, 40 to 65 years, and &gt; 65 years, respectively. We performed adjusted Cox regression analyses to predict RRFD and global mortality.</div></div><div><h3>Results</h3><div>We analyzed 4952 participants (54% women; mean age: 52 years). Age-stratified definition resulted in 24 of 677 participants aged &lt; 40 years reclassified as CKD, with no adverse outcomes; whereas 55 of 713 participants aged &gt; 65 years were reclassified as non-CKD, with 12 deaths and 1 RRFD. After multivariate adjustment, the CKD group had a poorer prognosis compared with the non-CKD group independently of the definition used; hazard ratio (HR) and 95% confidence interval (CI) were 2.23 (1.59–3.12), 2.06 (1.46–2.90), and 1.64 (1.13–2.38) for the standard, corrected to i-BSA, and age-stratified definitions, respectively.</div></div><div><h3>Conclusion</h3><div>In our study, classification of CKD by age or i-BSA does not appear to improve prediction of RRFD and mortality.</div></div>","PeriodicalId":17761,"journal":{"name":"Kidney International Reports","volume":"10 2","pages":"Pages 386-395"},"PeriodicalIF":5.7,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143161288","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Patients With Immunoglobulin A Nephropathy Show Abnormal Frequencies of B Cell Subsets, Unconventional T Cells, and High Levels of Galactose-Deficient IgA1–Coated Gut Bacteria","authors":"Micaela Gentile ,&nbsp;Nina Goerlich ,&nbsp;I-Ju Lo ,&nbsp;N. Eric Olson ,&nbsp;Mark McConnell ,&nbsp;Johannes Pospiech ,&nbsp;Tobias Bohnenpoll ,&nbsp;Philipp Skroblin ,&nbsp;Olivier Radresa ,&nbsp;Uwe Andag ,&nbsp;Kirk N. Campbell ,&nbsp;Kristin Meliambro ,&nbsp;Luis Sanchez-Russo ,&nbsp;Alberto Verlato ,&nbsp;Enrico Fiaccadori ,&nbsp;Seunghee Kim-Schulze ,&nbsp;Maria Lanau ,&nbsp;M. Loreto Fernandez-Lorente ,&nbsp;Miguel Fribourg ,&nbsp;Joaquin Manrique ,&nbsp;Paolo Cravedi","doi":"10.1016/j.ekir.2024.11.007","DOIUrl":"10.1016/j.ekir.2024.11.007","url":null,"abstract":"<div><h3>Introduction</h3><div>Mucosal inflammation is involved in the pathophysiology of immunoglobulin-A nephropathy (IgAN); however, peripheral immune phenotype analyses of patients with IgAN often do not include unconventional T cells, the major subset in mucosal immunity.</div></div><div><h3>Methods</h3><div>We measured serum total IgA, galactose-deficient IgA1 (gd-IgA1), secretory IgA (SIgA), B cell-activating factor (BAFF), and A proliferation-inducing ligand (APRIL) in 66 patients with IgAN and 30 healthy controls (HCs). We also quantified the total IgA and gd-IgA1 in stool supernatant along with the same coated on bacteria. In 35 patients and 14 controls, we performed extensive phenotyping using cytometry by Time-of-Flight (CyTOF) of circulating immune cells, including unconventional T cells (mucosal associated invariant T [MAIT] cells, γδ T, and natural killer [NK] T cells). The results were validated using RNAseq data from a larger cohort of 179 patients with IgAN, 140 patients with minimal change disease, and 91 HCs.</div></div><div><h3>Results</h3><div>Patients with IgAN had higher circulating levels of total IgA, gd-IgA1, and APRIL, and higher IgA and gd-IgA1-coated gut bacteria than controls, whereas serum levels of SIgA and BAFF did not differ between groups. Patients with IgAN showed more class-switched memory (CSM) and double negative (DN) B cells than controls. MAIT cells and γδ T cells were significantly lower, and CD4⁻CD8⁻ NK T cells were significantly higher in patients with IgAN than in HCs. We validated the significant decrease in MAIT cells in an independent cohort of patients with IgAN.</div></div><div><h3>Conclusion</h3><div>The data indicate that patients with IgAN have increased circulating CSM and DN B cells associated with abnormal T cell immunity, involving defects in unconventional T cell frequency. This may suggest putative alterations at mucosal sites because of cell migration leading to altered IgA production.</div></div>","PeriodicalId":17761,"journal":{"name":"Kidney International Reports","volume":"10 2","pages":"Pages 475-488"},"PeriodicalIF":5.7,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143161353","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Validating the SONG-PKD Pain Instrument, a Core Outcome Measure for Pain in ADPKD","authors":"Rosanna Cazzolli ,&nbsp;Angela Ju ,&nbsp;Patrizia Natale ,&nbsp;Armando Teixeira-Pinto ,&nbsp;Martin Howell ,&nbsp;Allison Jaure ,&nbsp;Ronald D. Perrone ,&nbsp;Eva Burnette ,&nbsp;Niek F. Casteleijn ,&nbsp;Arlene Chapman ,&nbsp;Jonathan C. Craig ,&nbsp;Sarah Eastty ,&nbsp;Ron T. Gansevoort ,&nbsp;Tess Harris ,&nbsp;Marie C. Hogan ,&nbsp;Shigeo Horie ,&nbsp;Bertrand Knebelmann ,&nbsp;Richard Lee ,&nbsp;Karine Manera ,&nbsp;Reem A. Mustafa ,&nbsp;Yeoungjee Cho","doi":"10.1016/j.ekir.2024.11.015","DOIUrl":"10.1016/j.ekir.2024.11.015","url":null,"abstract":"<div><h3>Introduction</h3><div>Pain is a critically important outcome in autosomal dominant polycystic kidney disease (ADPKD); however, it is infrequently and inconsistently reported in clinical trials. This study aimed to validate the Standardized Outcomes in Nephrology-Polycystic Kidney Disease (SONG-PKD) Pain measure, which includes 3 items related to pain (frequency, severity, and impact on life participation) measured on a 5-point Likert scale, in adults with ADPKD.</div></div><div><h3>Methods</h3><div>A total of 316 adults with ADPKD from 21 countries participated online. The median (interquartile range) age of participants was 56 (44–66) years, 219 (69%) were female, and 222 (70%) had a university degree or higher. Participants completed a demographic questionnaire, brief medical history, and 4 pain measures at baseline. The pain measures were readministered 2 days later. Internal consistency was evaluated with Cronbach’s alpha. Test-retest reliability was assessed using intraclass correlation coefficient (ICC), and convergent validity was assessed using Spearman’s rho. Known groups comparisons for patients with or without a history of kidney complications were performed using a Mann-Whitney rank sum test.</div></div><div><h3>Results</h3><div>The SONG-PKD Pain measure demonstrated high internal consistency (0.94, 95% confidence interval [CI]: 0.93–0.95) and test-retest reliability (0.92, 95% CI: 0.90–0.94). There was a high convergence of SONG-PKD Pain with the Brief Pain Inventory-Short Form (BPI-SF; 0.84, 95% CI: 0.80–0.87) and a visual analog scale (VAS; 0.84, 95% CI: 0.81–0.87). There was a significant difference in the median scores of patients with and without a history of complications (4.0 vs. 0.0, <em>P</em> &lt; 0.001).</div></div><div><h3>Conclusion</h3><div>SONG-PKD Pain instrument is a brief and simple measure that has demonstrated strong psychometric properties.</div></div>","PeriodicalId":17761,"journal":{"name":"Kidney International Reports","volume":"10 2","pages":"Pages 447-456"},"PeriodicalIF":5.7,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143161394","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}