Nephron最新文献

{"title":"The Physiology and Pathophysiology of Branched-Chain Amino Acids in the Kidney.","authors":"Louis J Delinois, Samaneh DiMartino, Sian E Piret","doi":"10.1159/000551377","DOIUrl":"10.1159/000551377","url":null,"abstract":"<p><strong>Background: </strong>Kidneys require large amounts of energy to maintain function and are highly metabolically active. Acute kidney injury (AKI) and diseases including chronic kidney disease (CKD), diabetic kidney disease (DKD), and polycystic kidney disease (PKD) are strongly associated with metabolic disturbances.</p><p><strong>Summary: </strong>While most research to date has focused on glucose and fatty acid metabolism, the catabolism of the branched-chain amino acids (BCAAs) leucine, isoleucine, and valine is an emerging area of importance across different kidney pathologies. BCAAs can be used in protein synthesis or catabolized to provide tricarboxylic acid (TCA) cycle intermediates. BCAAs and their metabolites can also act as signaling molecules. Disturbances of BCAA catabolism have recently been described in AKI, CKD, DKD, and PKD, driven by both transcriptional and posttranslational mechanisms. This results in accumulation of BCAAs in the kidneys and the loss of a source of TCA cycle intermediates. In addition, accumulated BCAAs, especially leucine, can activate mechanistic target of rapamycin complex 1 (mTORC1) signaling. In addition to the described disturbances in BCAA catabolism, recent preclinical studies have shown that reactivation of BCAA catabolism could be a potential therapeutic strategy.</p><p><strong>Key messages: </strong>This review will describe the process of BCAA catabolism and its disturbances in AKI, CKD, DKD, and PKD.</p>","PeriodicalId":18998,"journal":{"name":"Nephron","volume":" ","pages":"1-9"},"PeriodicalIF":1.8,"publicationDate":"2026-03-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147365872","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Water Consumption and Chronic Hemodialysis Therapy: Where Do We Stand? Where Must We Go? Review of Clinical Practices.","authors":"Charles Chazot, Romain Vial, Alain Birbes, Catherine Lasseur, Hafsah Hachad, Maxime Espi, Jocelyne Rey, Fabrice Huré, Isabelle Ethier, Maryvonne Hourmant","doi":"10.1159/000551009","DOIUrl":"10.1159/000551009","url":null,"abstract":"<p><strong>Background: </strong>Chronic hemodialysis therapy is a life-saving procedure requiring large amounts of water whereas this natural resource availability declines with increasing temperatures and the dialysis needs are far from being covered in many world areas.</p><p><strong>Summary: </strong>Reducing water consumption in hemodialysis relies on the moto \"reduce, reuse, and recycle.\" Because of a significant amount of water is discarded to the drain by the procedure, the efficiency of the reverse osmosis (RO) of the water treatment system is a key factor of water consumption during the hemodialysis session. Along decades, the RO devices have become more efficient, with a decrease of the rejected part of water from around 70 to below 30%. The optimization of the maintenance and disinfection procedures of the water loop, as well as the practice of acid concentrate delivery to the dialysis machine, can reduce significantly water needs. Reducing dialysate flow is also effective, even with convective modalities, but more studies are needed to confirm long-term patient safety. The co-decision with the patient on dialysis modality choice including alternatives to hemodialysis must take into account the environmental impact after appropriate information. Other aspects of water sparing are presented including, among others, the reuse of RO water reject and the spent dialysate recycling.</p><p><strong>Key message: </strong>Green dialysis including the water preservation must become a \"top of the list\" priority for all the dialysis stakeholders to preserve the access and the future of this tremendous progress of medical history.</p>","PeriodicalId":18998,"journal":{"name":"Nephron","volume":" ","pages":"1-11"},"PeriodicalIF":1.8,"publicationDate":"2026-03-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147365813","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Living Kidney Donation: An Update.","authors":"Ana González Rinne, Anna Manonelles, Carla Burballa, Esther González Monte","doi":"10.1159/000551271","DOIUrl":"10.1159/000551271","url":null,"abstract":"<p><strong>Background: </strong>Living donor kidney transplantation offers superior long-term outcomes compared to deceased donor transplantation. However, ensuring long-term donor safety remains the primary objective of the clinical assessment process. Pre-donation risk evaluation can be challenging, particularly in specific borderline scenarios where evidence is limited.</p><p><strong>Summary: </strong>This review explores several critical aspects of the decision-making process in living kidney donor (LKD) assessment. It addresses how to evaluate potential donors with complex medical or psychosocial profiles, including those with obesity, advanced age, psychiatric histories, or past substance use. The review also emphasizes the importance of using accurate and reliable tools for donor evaluation, such as measured glomerular filtration rate, particularly in cases with borderline kidney function, and discusses the growing role of genetic testing as it becomes more accessible. Furthermore, it considers how socioeconomic, cultural, and religious factors can influence both the willingness to donate and the likelihood of being selected as a donor. Lastly, the review underscores the essential role of long-term follow-up in safeguarding donor health and optimizing outcomes.</p><p><strong>Key messages: </strong>To safely expand the pool of eligible LKDs, it is essential to use precise evaluation tools, ensure and enable long-term follow-up, and promote research to improve risk stratification and guide decision-making. In this review, we update information in the evaluation of LKDs highlighting gaps in current knowledge and practice.</p>","PeriodicalId":18998,"journal":{"name":"Nephron","volume":" ","pages":"1-13"},"PeriodicalIF":1.8,"publicationDate":"2026-03-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147348611","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Characterization of Kidney and Liver Cystic Phenotype Associated with <italic>GANAB</italic> Using Advanced Imaging Biomarkers.","authors":"Fadi George Munairdjy Debeh, Marie Therese Bou Antoun, Ahmad Ghanem, Vineetha Rangarajan, Abdul Hamid Borghol, Stefan Paul, Dana Hanna, Bassel AlKhatib, Nay Nader, Besher Shami, Adriana Gregory, Hana Yang, Rachel S Schauer, Ziad Zoghby, Marie C Hogan, Neera K Dahl, Christian Hanna, Timothy L Kline, Peter C Harris, Fouad T Chebib","doi":"10.1159/000551274","DOIUrl":"10.1159/000551274","url":null,"abstract":"<p><strong>Background: </strong>Monoallelic pathogenic variants in GANAB cause autosomal dominant cystic kidney and liver disease, but quantitative imaging phenotypes remain incompletely defined.</p><p><strong>Methods: </strong>We performed a retrospective study of 16 individuals with GANAB variants and available abdominal imaging. Deep learning-based cyst segmentation quantified kidney and liver volumes and cyst metrics, including height-adjusted total kidney volume (htTKV), height-adjusted total liver volume, total cyst number (TCN), and height-adjusted total cyst volume.</p><p><strong>Results: </strong>Hepatic involvement was common, with polycystic liver disease present in most individuals with varying severity (liver TCN range 22-219). Kidney involvement was more heterogeneous (htTKV range 153-858 mL/m; kidney TCN range 3-42). Individuals with kidney TCN <20 had preserved kidney function and slower annual estimated glomerular filtration rate (eGFR) decline (median -1.68 mL/min/1.73 m2) compared with those with kidney TCN ≥20 (-2.8 mL/min/1.73 m2/year); no individual progressed to kidney failure during follow-up. Hypertension occurred in 50%. Intracranial aneurysms were identified in 3 of 6 screened individuals, including two from a family with known aneurysmal disease.</p><p><strong>Conclusions: </strong>Quantitative imaging reveals a phenotypic spectrum in ADPKD-GANAB, ranging from liver-predominant cystic disease with minimal kidney involvement to a phenotype with higher kidney cyst burden and faster eGFR decline. Establishing robust genotype-phenotype relationships in this rare disease will require larger, aggregated cohorts with standardized imaging and systemic extrarenal screening.</p>","PeriodicalId":18998,"journal":{"name":"Nephron","volume":" ","pages":"1-16"},"PeriodicalIF":1.8,"publicationDate":"2026-03-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13135869/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147348646","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Comparative Effectiveness of Lipid-Lowering Therapies in Increasing High-Density Lipoprotein Cholesterol Levels in Patients with Chronic Kidney Disease.","authors":"Mohammad Abdullah Al Zubair Naim, Fridtjof Thomas, Elani Streja, Robert Lowell Davis, Kamyar Kalantar-Zadeh, Keiichi Sumida, Csaba P Kovesdy","doi":"10.1159/000551276","DOIUrl":"10.1159/000551276","url":null,"abstract":"<p><strong>Background: </strong>In patients with chronic kidney disease (CKD), lower and subfunctional high-density lipoprotein cholesterol (HDL-C) is associated with poor cardiovascular outcomes. Notwithstanding such poor outcomes, the primary therapeutic target in patients with CKD is low-density lipoprotein cholesterol (LDL-C), and the comparative effectiveness of commonly used lipid-lowering therapies (LLTs) in changing HDL-C levels in patients with non-dialysis-dependent CKD (NDD-CKD) remains unclear.</p><p><strong>Methods: </strong>In this retrospective cohort study, using a target trial emulation framework, we examined a nationwide cohort of 3,562,882 US Veterans with normal kidney function enrolled between October 2004 and September 2006 and identified 247,270 incident CKD patients eligible for de novo LLT exposure, occurring during longitudinal follow-up until October 2019. We defined de novo LLT initiation using pharmacy dispensation data and followed patients for up to 1 year. We compared the intraindividual slopes of HDL-C levels in de novo fibrates and niacin users with those in statin users, using mixed-effects models adjusted for baseline and time-varying covariates. We also compared the odds of having a clinically meaningful (>10% from baseline) increase in HDL-C following LLT initiation.</p><p><strong>Results: </strong>A total of 38,223 patients with incident CKD initiated de novo LLT (statin [n = 35,284], fibrate [n = 1,805], and niacin [n = 1,134]). The mean (standard deviation) age was 67.3 (10.5) years; 95.0% were men, and 20.6% were Black. Compared to statin users, the multivariable-adjusted annualized intraindividual increase of HDL-C was significantly higher following fibrate {1.15 mg/dL/year (95% confidence interval [CI]: 0.43, 1.87); p = 0.002} and niacin monotherapy (2.51 mg/dL/year [95% CI: 1.62, 3.41]; p < 0.001). Furthermore, niacin (OR: 1.37 [95% CI: 1.07, 1.75]; p = 0.012) was more likely than statins to provide a clinically meaningful elevation in HDL-C. Our findings were consistent in several sensitivity analyses.</p><p><strong>Conclusion: </strong>Among patients with NDD-CKD, de novo prescriptions of fibrates or niacin are associated with a greater increase in HDL-C levels compared to statins. Further studies are warranted to investigate whether such differences have meaningful effects on clinical outcomes.</p>","PeriodicalId":18998,"journal":{"name":"Nephron","volume":" ","pages":"1-15"},"PeriodicalIF":1.8,"publicationDate":"2026-02-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147317379","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Comparative Evaluation of Five Prognostic Scoring Systems in Pauci-Immune Necrotizing and Crescentic Glomerulonephritis.","authors":"Saliha Yıldırım, Betül Öğüt, Emre Yaşar, Veysel Baran Tomar, Hatice Şahin, Ebru Gök Oğuz, İpek Işık Gönül, Mehmet Deniz Ayli, Galip Güz","doi":"10.1159/000550772","DOIUrl":"10.1159/000550772","url":null,"abstract":"<p><strong>Introduction: </strong>Pauci-immune necrotizing and crescentic glomerulonephritis (PiNCGN) is a leading cause of rapidly progressive kidney failure. Several prognostic tools - Berden classification, Mayo Clinic Chronicity Score (MCCS), Percentage of ANCA Crescents Score (PACS), ANCA Renal Risk Score (ARRS), and the improved ANCA Kidney Risk Score (AKRiS) - have been developed to predict renal outcomes, but data on their performance in anti-neutrophil cytoplasmic antibody (ANCA)-negative patients remain scarce. This study evaluated the prognostic value of these scoring systems in a PiNCGN cohort.</p><p><strong>Methods: </strong>We retrospectively analyzed 100 patients with biopsy-proven PiNCGN. Demographic, laboratory, and histopathological data were collected, and patients were categorized according to all five risk scores. Outcomes included all-cause mortality and end-stage kidney disease (ESKD), defined as initiation of dialysis or kidney transplantation. The Kaplan-Meier survival and log-rank tests were applied to assess prognostic discrimination.</p><p><strong>Results: </strong>Of 100 patients, 86 were ANCA-positive and 14 ANCA-negative. Median age was 58.5 years; 41% were male. Induction therapy consisted of glucocorticoids with cyclophosphamide or rituximab, followed by azathioprine, mycophenolate, or rituximab for maintenance. Over a median follow-up of 12 months, 52 patients died and 21 progressed to ESKD. In ANCA-positive patients, ARRS and AKRiS best predicted ESKD. In ANCA-negative patients, AKRiS additionally predicted both mortality and ESKD. Other scores demonstrated limited utility.</p><p><strong>Conclusion: </strong>ARRS and AKRiS provided the most consistent prognostic stratification in PiNCGN, with AKRiS uniquely retaining value in ANCA-negative patients. These findings highlight the potential clinical utility of composite scoring systems across the spectrum of PiNCGN, although prospective multicenter validation remains warranted.</p>","PeriodicalId":18998,"journal":{"name":"Nephron","volume":" ","pages":"1-9"},"PeriodicalIF":1.8,"publicationDate":"2026-02-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146259050","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Rare Kidney Disease Conference Proceedings: Palm Springs, December 2024.","authors":"Niloufar Ebrahimi, Amir Abdipour, Raad Chowdhury, Mehrbod Vakhshoori, Giv Heidari-Bateni, Pranav S Garimella, Silvi Shah, Sayna Norouzi","doi":"10.1159/000549596","DOIUrl":"10.1159/000549596","url":null,"abstract":"","PeriodicalId":18998,"journal":{"name":"Nephron","volume":" ","pages":"1-12"},"PeriodicalIF":1.8,"publicationDate":"2026-02-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146181213","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Endothelial Glycocalyx: From Microscope Stage to Bedside.","authors":"Sara Conti, Michael S Goligorsky, Susanna Tomasoni","doi":"10.1159/000550816","DOIUrl":"10.1159/000550816","url":null,"abstract":"<p><strong>Background: </strong>It sounds paradoxical that a tiny component of the cellular mantle known as glycocalyx is also the most ubiquitous, more so than mitochondria or nuclei, endowing glycocalyx coverage to every cell with no exceptions. Despite a relatively short history, this organelle has received exponentially growing attention and number of publications as reflected in the last decade. This cellular mantle consists of 4 transmembrane proteins and 6 GPI-anchored proteins, all of which are decorated with glycosaminoglycans like heparan, chondroitin and dermatan sulfates, and hyaluronic acid. All this has been exhaustively reviewed.</p><p><strong>Summary: </strong>Our goal here will be to briefly sketch the important common features of glycocalyx, especially of endothelial cells, and to focus on clinically pertinent practical aspects of this structure. We will describe visual and biochemical detection challenges, highlight the usefulness of identifying and quantifying glycocalyx fragments in biological fluids while broadening the spectrum of specimens for diagnostic purposes, and discuss how these parameters may provide valuable clues regarding their therapeutic relevance as pharmacological targets.</p><p><strong>Key message: </strong>The glycocalyx represents a clinically valuable target and advances in its detection can enhance diagnostic precision and strengthen its translational relevance.</p>","PeriodicalId":18998,"journal":{"name":"Nephron","volume":" ","pages":"1-10"},"PeriodicalIF":1.8,"publicationDate":"2026-02-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146125744","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The Impact of Air Pollution on Kidney Function: A Two-Sample Mendelian Randomization Study.","authors":"Hao Wu, Zixian Yu, Ziyun Yu, Yan Du, Lu Li","doi":"10.1159/000550247","DOIUrl":"10.1159/000550247","url":null,"abstract":"<p><strong>Background: </strong>Chronic kidney disease (CKD) represents a major global health challenge that leads to considerable morbidity and mortality. The influence of particulate matter (PM)_2.5, PM_2.5-10, PM₁₀, nitrogen oxides (NO_X), nitrogen dioxide (NO₂) on renal diseases has raised concerns. However, the etiological relation remains uncertain. This study aimed to establish genetic support for causal links between environmental exposures and renal impairment.</p><p><strong>Methods: </strong>A two-sample Mendelian randomization (MR) study was launched based on genome-wide association study. The inverse variance weighted method served as the primary analysis, complemented by four other approaches (MR-Egger, MR-Weighted median, weighted mode, and simple mode). The results were reinforced by MR-Egger regression, MR-PRESSO, and leave-one-out analysis.</p><p><strong>Results: </strong>The MR analysis revealed that exposed to PM_2.5-10 significantly increases the risk of developing CKD (OR: 1.631; 95% confidence interval [CI]: 1.161, 2.290), eGFR_crea (β: -0.013; 95% CI: -0.021, -0.004), and eGFR_cys (β: -0.024; 95% CI: -0.039, -0.008). NO_x exposure correlated with declining eGFR_cys (β: -0.016; 95% CI: -0.029, -0.003). NO₂ correlated with increased urine albumin-to-creatinine ratio levels (β: 0.039; 95% CI: 0.009, 0.069). However, no significant association was found between exposure to PM_2.5 or PM₁₀ and impaired renal function, with no evidence of significant heterogeneity or horizontal pleiotropy.</p><p><strong>Conclusion: </strong>By employing MR analysis, we found that elevated levels of air pollutants, specifically PM_2.5-10, can causally worsen kidney function. These results facilitate a deeper comprehension of the pathogenesis and treatment of air pollution-induced renal damage.</p>","PeriodicalId":18998,"journal":{"name":"Nephron","volume":" ","pages":"1-13"},"PeriodicalIF":1.8,"publicationDate":"2026-01-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146086479","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Circadian Rhythms and Glucocorticoid Dynamics in the Kidney Matrix.","authors":"Charles Gyasi, Rachel Lennon, Rebecca Preston","doi":"10.1159/000550773","DOIUrl":"10.1159/000550773","url":null,"abstract":"<p><strong>Background: </strong>The kidney matrix, once viewed as a static scaffold, is now recognised as a dynamic microenvironment that undergoes continual remodelling in response to physiological cues. Emerging evidence demonstrates that this remodelling follows circadian patterns driven by molecular clocks within specific kidney cell types.</p><p><strong>Summary: </strong>This review synthesises recent advances on circadian regulation of the kidney matrisome, with emphasis on glomerular compartments. Circadian clocks in the glomerulus coordinate the timing of matrix turnover to preserve structural integrity, maintain filtration, and promote repair. Disruption of these rhythms contributes to maladaptive matrix accumulation, fibrosis, and kidney disease progression.</p><p><strong>Key messages: </strong>Finally, we discuss mechanistic insights and translational opportunities, including chronotherapy and clock-targeted interventions. Understanding circadian control of glomerular matrix dynamics provides a framework for linking temporal biology to kidney health and disease.</p>","PeriodicalId":18998,"journal":{"name":"Nephron","volume":" ","pages":"1-10"},"PeriodicalIF":1.8,"publicationDate":"2026-01-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12975147/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146086442","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}