Nephron[JOURNAL]
Sun
200 papers
Molecular Insights into the Immunogenetic Pathways of Diabetic Kidney Disease.
Yu C, Xiong C, Xie K … +4 more , Chen Z, Liu E, Wang L, Gao J
Nephron · 2026 Apr · PMID 41926562 · Publisher ↗
BACKGROUND: Diabetic kidney disease (DKD), a major microvascular complication of diabetes, is increasingly recognized as a chronic inflammatory disorder driven by immune dysregulation. SUMMARY: This review examines key i...
BACKGROUND: Diabetic kidney disease (DKD), a major microvascular complication of diabetes, is increasingly recognized as a chronic inflammatory disorder driven by immune dysregulation. SUMMARY: This review examines key immunogenetic pathways in DKD, focusing on genetic variants affecting innate and adaptive immunity. We explore how polymorphisms in genes regulating immune cell function, cytokine production, and inflammatory signaling contribute to renal damage. Recent evidence demonstrates that these immunogenetic factors significantly influence DKD development and progression. KEY MESSAGES: The pathogenesis of DKD is profoundly influenced by an individual's genetic predisposition to immune dysregulation. Numerous genetic variants can exacerbate inflammatory responses, promoting disease development. A deeper understanding of these immunogenetic pathways not only clarifies the pathophysiology of DKD but also opens avenues for novel biomarker discovery and the development of targeted, personalized immunomodulatory therapies.
Beyond Oral Anticoagulants Two Case Reports of Biopsy-Proven Heparin-Induced Anticoagulant-Related Nephropathy.
Islami T, Gregorini M, Grignano MA … +17 more , Serpieri N, Sepe V, Pattonieri EF, Margiotta E, Soccio G, Portalupi V, Stea ED, Reseghetti E, Moscardino S, De Mauri A, Castoldi F, Delvino P, D Apos Ambrosio G, Minutillo P, Verga L, Cavagna L, Rampino T
Nephron · 2026 Apr · PMID 41926555 · Publisher ↗
INTRODUCTION: Anticoagulant-related nephropathy (ARN) is an emerging and often unrecognized form of acute kidney injury. Histologically characterized by glomerular hemorrhage and intratubular erythrocyte casts, ARN is a...
INTRODUCTION: Anticoagulant-related nephropathy (ARN) is an emerging and often unrecognized form of acute kidney injury. Histologically characterized by glomerular hemorrhage and intratubular erythrocyte casts, ARN is a serious complication associated with increased morbidity and progression to chronic kidney disease. Although it has been described in association with oral anticoagulants, the literature regarding ARN induced by non-oral agents, such as low-molecular weight heparin (LMWH), remains extremely scarce. This report aims to fill this gap, highlighting the importance of early diagnosis in complex clinical settings. CASE PRESENTATIONS: We present two cases of biopsy-proven ARN induced by LMWH in patients with concomitant active renal disease. The first case is a 53-year-old man with ANCA-associated vasculitis who, after starting enoxaparin for deep vein thrombosis, experienced worsening renal function despite improvement in his pulmonary and serological vasculitis markers. A second renal biopsy was crucial to diagnose the superimposed ARN, leading to a change in therapy and renal recovery. The second case is a 73-year-old man with chronic kidney disease who developed an acute kidney injury initially attributed to post-infectious glomerulonephritis. The initiation of prophylactic LMWH was followed by further, severe deterioration of renal function requiring dialysis. In this case as well, a second biopsy revealed the presence of superimposed ARN. The discontinuation of anticoagulant therapy led to the resolution of hematuria and the cessation of dialysis. In both cases, the diagnosis was confirmed by positive Perl's staining and immunohistochemical findings of tubular damage. CONCLUSIONS: These cases demonstrate that ARN is not an exclusive complication of oral anticoagulants but can also be induced by LMWH. ARN can mask or overlap with other renal pathologies, making diagnosis difficult. Renal biopsy, sometimes repeated, remains the gold standard for an accurate differential diagnosis. This makes it possible to avoid inappropriate therapy escalation and to guide correct clinical management. Greater awareness of this clinical entity is necessary in all anticoagulated patients who develop acute kidney injury.
A Study Using Point-of-Care Creatinine Testing in a Nigerian Primary Health Care Centre: Malaria as the Leading Cause of Acute Kidney Injury Particularly in Children.
Ugwem-Ikuru Y, Laurent-Ordu V, Emem-Chioma P … +3 more , Erekosima I, Lewis D, Poulikakos D
Nephron · 2026 Mar · PMID 41911078 · Full text
BACKGROUND: Acute kidney injury (AKI) worsens outcomes in low- and middle-income countries, largely due to delayed diagnosis and limited access to renal replacement therapy. Its true epidemiology remains unclear, partly...
BACKGROUND: Acute kidney injury (AKI) worsens outcomes in low- and middle-income countries, largely due to delayed diagnosis and limited access to renal replacement therapy. Its true epidemiology remains unclear, partly due to delayed biochemical testing. In a prior phase of our work, we evaluated point-of-care creatinine (POC Cr) technology and its use in implementing a clinical algorithm to select patients at risk of AKI in a Nigerian hospital emergency department. In this study, POC Cr was used in a large primary care health center in Nigeria. METHODS: The study was conducted at Ozuoba Model Comprehensive Primary Health Care Centre in Nigeria, where renal function tests are rarely available and external laboratory results typically take over 48 h. POC Cr testing was introduced for high-risk adults using the clinical algorithm developed in the previous phase of this programme and for children with suspected severe illness based on clinical judgement or reduced urine output. Adjusted POC Cr values were calculated (POC Cr - 27.2 µmol/L), and AKI was staged using KDIGO criteria, with baseline creatinine defined as 100 µmol/L for adults and age-specific norms for children. RESULTS: A total of 424 patients were tested using POC Cr, comprising 301 adults and 123 children. Malaria was the most frequent diagnosis, accounting for 293 cases (61.1%). The median adjusted POC Cr across the entire cohort was 72.8 µmol/L (interquartile range [IQR]: 36). Among adult patients, AKI was diagnosed in 2 out of the 301 individuals (0.6%), one stage 1 and one stage 2, both of whom had malaria. In the paediatric group, the median age was 5 years (IQR: 7), with females comprising 65% of the cohort. Malaria was diagnosed in 69.9% of the children. AKI was identified in 70 out of 123 children (56.9%), with AKI stage 1 in 25 (20.3%), stage 2 in 26 (21.2%), and stage 3 in 19 children (15.4%). Among the 70 paediatric AKI cases, 49 (70%) had malaria. The highest prevalence of AKI was seen in children under 5 years of age, with the incidence declining steadily and reaching zero beyond age 12. CONCLUSION: Our study found that more than half of paediatric patients diagnosed with malaria at a primary health care level had AKI, highlighting AKI as a common complication of malaria in young children. These findings emphasise the need for further research to support informed potential updates to WHO malaria treatment guidelines to incorporate the KDIGO definition of AKI, particularly in the context of paediatric care in low-resource settings.
The Role of Combining Exercise with Pharmacologic Management of Chronic Kidney disease: From Sodium-Glucose Co-Transporter-2 and Glucagon-Like Peptide-1 to Broader Therapeutic Strategies.
Wilkinson TJ, Biddle G, James E
Nephron · 2026 Mar · PMID 41843705 · Publisher ↗
BACKGROUND: Given the exceptional evolution of pharmacological approaches to chronic kidney disease (CKD) management and their potential interactions with lifestyle, in this review, we describe the intersection of exerci...
BACKGROUND: Given the exceptional evolution of pharmacological approaches to chronic kidney disease (CKD) management and their potential interactions with lifestyle, in this review, we describe the intersection of exercise and pharmacotherapy and the possible role of exercise training as an adjunct management option to optimise glucose-lowering therapies (GLTs) in people living with diabetes and CKD. SUMMARY: Exercise remains a cornerstone intervention for individuals living with CKD and diabetes, providing well-established benefits for cardiovascular health, metabolic regulation, and preservation of physical function. While GLTs, including sodium-glucose co-transporter-2 inhibitors and glucagon-like peptide-1 receptor agonists, offer significant promise, these pharmacologic advances must be contextualised within a broader lifestyle framework to achieve optimal outcomes. Future research should prioritise strategies that integrate pharmacotherapy with structured exercise programs, while accounting for patient-specific factors such as comorbidities, frailty, and functional limitations. Implementation science will be critical to translate these findings into routine care, leveraging multidisciplinary teams, digital health tools, and behavioural support. Ultimately, success will depend on integrative, person-centred care models that align pharmacologic and lifestyle interventions to enhance quality of life, reduce disease burden, and improve long-term outcomes for people with CKD and diabetes. KEY MESSAGES: Exercise and GLTs share complementary mechanisms, such as improvements in insulin sensitivity, inflammation, and body composition. Yet, the synergistic potential of combining these interventions warrants further investigation through high-quality trials and mechanistic studies. Current evidence is encouraging but insufficient to confidently guide clinical practice.
A Service Evaluation of Anti-Xa Measurements in Patients with Kidney Impairment in a Tertiary Centre.
Gurumurthy G, Haysom R, Lemma M … +5 more , Aziz N, Hartemink J, Begum Y, Thachil J, Parker K
Nephron · 2026 Mar · PMID 41812002 · Full text
BACKGROUND: Pre-emptive dose reduction of low-molecular-weight heparins (LMWHs) is often utilised in those with chronic kidney disease (CKD) to prevent bioaccumulation. We report on the association of a pre-emptive dose...
BACKGROUND: Pre-emptive dose reduction of low-molecular-weight heparins (LMWHs) is often utilised in those with chronic kidney disease (CKD) to prevent bioaccumulation. We report on the association of a pre-emptive dose reduction on anti-Xa range and its correlation with clinical outcomes. METHODS: We undertook a retrospective service evaluation of patients with CKD (eGFR < 30 mL/min/1.73 m2) receiving therapeutic dose dalteparin. The primary exposure was dalteparin anti-Xa trough and peak. Primary outcomes were ISTH-defined clinically relevant bleeding, thrombosis, and all-cause mortality within 90 days of LMWH initiation. A multivariate Cox proportional hazards model was employed to assess the relationship between anti-Xa levels and the incidence of bleeding and mortality. RESULTS: A total of 103 patients were identified over a 2-year period. Seventy-eight (75.7%) had anti-Xa monitoring done. Trough anti-Xa distribution was within-target in 58 (75.6%). Patients on dialysis had a higher incidence of bleeding (19 vs. 12, p < 0.05). Patients with bleeding had significantly higher median anti-Xa trough (0.26 vs. 0.13 U/mL, p < 0.01). The median time to bioaccumulation was 19 days. In multivariate Cox models, only anti-Xa trough remained an independent association with bleeding (HR: 1.47 per 0.1 U/mL, 95% CI: 1.05-2.15; p < 0.05). No associations with mortality were identified. CONCLUSION: In this report, trough anti-Xa measurement of dalteparin is independently associated with bleeding in patients with CKD. Further prospective, larger studies are warranted to validate these results before it can be universally recommended in clinical practice.
Erratum.
Nephron · 2026 · PMID 41805748 · Publisher ↗
In the article by Imberti and Benigni entitled "Renal Endowment in Men and Women: Start from the Beginning" [Nephron. 2025;149:207-212; https://doi.org/10.1159/000542411], the following statement was mistakenly left out...
In the article by Imberti and Benigni entitled "Renal Endowment in Men and Women: Start from the Beginning" [Nephron. 2025;149:207-212; https://doi.org/10.1159/000542411], the following statement was mistakenly left out of the Acknowledgements section:This review is based on lectures given by A.B. for the CME course of the DOKI project: "Gender Differences in Renal Disease: Focus on Diabetes and Obesity," Garachico - Tenerife (Canary Islands, Spain, December 1-2, 2023), an initiative of the DOKI project (Diabetes, Obesity and the Kidney) - PN: 101079207 - Twinning project, awarded by the European Commission.
Proceedings of the 15th Annual UAB-UCSD O'Brien Center Symposium: Changing Paradigms in Acute Kidney Injury - From Mechanisms to Management.
Neyra JA, Vasiliu M
Nephron · 2026 Mar · PMID 41805690 · Full text
Abstract loading — click title to view on PubMed.
Water Consumption and Chronic Hemodialysis Therapy: Where Do We Stand? Where Must We Go? Review of Clinical Practices.
Chazot C, Vial R, Birbes A … +7 more , Lasseur C, Hachad H, Espi M, Rey J, Huré F, Ethier I, Hourmant M
Nephron · 2026 · PMID 41785219 · Publisher ↗
BACKGROUND: 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 be...
BACKGROUND: 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. SUMMARY: 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. KEY MESSAGE: 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.
The Physiology and Pathophysiology of Branched-Chain Amino Acids in the Kidney.
Delinois LJ, DiMartino S, Piret SE
Nephron · 2026 Mar · PMID 41785211 · Publisher ↗
BACKGROUND: 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),...
BACKGROUND: 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. SUMMARY: 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. KEY MESSAGES: This review will describe the process of BCAA catabolism and its disturbances in AKI, CKD, DKD, and PKD.
Living Kidney Donation: An Update.
González Rinne A, Manonelles A, Burballa C … +1 more , González Monte E
Nephron · 2026 Mar · PMID 41774598 · Publisher ↗
BACKGROUND: 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 assess...
BACKGROUND: 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. SUMMARY: 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. KEY MESSAGES: 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.
Characterization of Kidney and Liver Cystic Phenotype Associated with <italic>GANAB</italic> Using Advanced Imaging Biomarkers.
Munairdjy Debeh FG, Bou Antoun MT, Ghanem A … +17 more , Rangarajan V, Borghol AH, Paul S, Hanna D, AlKhatib B, Nader N, Shami B, Gregory A, Yang H, Schauer RS, Zoghby Z, Hogan MC, Dahl NK, Hanna C, Kline TL, Harris PC, Chebib FT
Nephron · 2026 Mar · PMID 41774594 · Full text
BACKGROUND: Monoallelic pathogenic variants in GANAB cause autosomal dominant cystic kidney and liver disease, but quantitative imaging phenotypes remain incompletely defined. METHODS: We performed a retrospective study...
BACKGROUND: Monoallelic pathogenic variants in GANAB cause autosomal dominant cystic kidney and liver disease, but quantitative imaging phenotypes remain incompletely defined. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.
Comparative Effectiveness of Lipid-Lowering Therapies in Increasing High-Density Lipoprotein Cholesterol Levels in Patients with Chronic Kidney Disease.
Naim MAAZ, Thomas F, Streja E … +4 more , Davis RL, Kalantar-Zadeh K, Sumida K, Kovesdy CP
Nephron · 2026 Feb · PMID 41758740 · Publisher ↗
BACKGROUND: 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 pri...
BACKGROUND: 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. METHODS: 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. RESULTS: 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. CONCLUSION: 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.
Comparative Evaluation of Five Prognostic Scoring Systems in Pauci-Immune Necrotizing and Crescentic Glomerulonephritis.
Yıldırım S, Öğüt B, Yaşar E … +6 more , Tomar VB, Şahin H, Gök Oğuz E, Gönül İI, Ayli MD, Güz G
Nephron · 2026 · PMID 41719207 · Publisher ↗
INTRODUCTION: 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 (...
INTRODUCTION: 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. METHODS: 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. RESULTS: 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. CONCLUSION: 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.
Rare Kidney Disease Conference Proceedings: Palm Springs, December 2024.
Ebrahimi N, Abdipour A, Chowdhury R … +5 more , Vakhshoori M, Heidari-Bateni G, Garimella PS, Shah S, Norouzi S
Nephron · 2026 · PMID 41678440 · Publisher ↗
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Endothelial Glycocalyx: From Microscope Stage to Bedside.
Conti S, Goligorsky MS, Tomasoni S
Nephron · 2026 · PMID 41642739 · Publisher ↗
BACKGROUND: 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 exce...
BACKGROUND: 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. SUMMARY: 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. KEY MESSAGE: The glycocalyx represents a clinically valuable target and advances in its detection can enhance diagnostic precision and strengthen its translational relevance.
Circadian Rhythms and Glucocorticoid Dynamics in the Kidney Matrix.
Gyasi C, Lennon R, Preston R
Nephron · 2026 · PMID 41610090 · Full text
<p>Background: 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 t...
<p>Background: 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. Summary: 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. Key Messages: 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>.
The Impact of Air Pollution on Kidney Function: A Two-Sample Mendelian Randomization Study.
Wu H, Yu Z, Yu Z … +2 more , Du Y, Li L
Nephron · 2026 · PMID 41610085 · Publisher ↗
BACKGROUND: Chronic kidney disease (CKD) represents a major global health challenge that leads to considerable morbidity and mortality. The influence of particulate matter (PM), PM, PM, nitrogen oxides (NO), nitrogen dio...
BACKGROUND: Chronic kidney disease (CKD) represents a major global health challenge that leads to considerable morbidity and mortality. The influence of particulate matter (PM), PM, PM, nitrogen oxides (NO), nitrogen dioxide (NO) 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. METHODS: 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. RESULTS: The MR analysis revealed that exposed to PM significantly increases the risk of developing CKD (OR: 1.631; 95% confidence interval [CI]: 1.161, 2.290), eGFR (β: -0.013; 95% CI: -0.021, -0.004), and eGFR (β: -0.024; 95% CI: -0.039, -0.008). NO exposure correlated with declining eGFR (β: -0.016; 95% CI: -0.029, -0.003). NO 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 or PM and impaired renal function, with no evidence of significant heterogeneity or horizontal pleiotropy. CONCLUSION: By employing MR analysis, we found that elevated levels of air pollutants, specifically PM, can causally worsen kidney function. These results facilitate a deeper comprehension of the pathogenesis and treatment of air pollution-induced renal damage.
Effects of <sc>n</sc>-Acetyl-<sc>l</sc>-Cysteine on the Progression of Kidney Dysfunction in Acadian Variant Fanconi Syndrome: A Case Series.
Al-Qadi M, Jose C, Gaudet J … +1 more , Thibeault Y
Nephron · 2026 · PMID 41553940 · Full text
<p>Introduction: Acadian variant Fanconi syndrome (AVFS) is an autosomal recessive disease caused by a mutation in the NDUFAF6 gene which results in mitochondrial dysfunction and oxidative damage to the kidneys. It prese...
<p>Introduction: Acadian variant Fanconi syndrome (AVFS) is an autosomal recessive disease caused by a mutation in the NDUFAF6 gene which results in mitochondrial dysfunction and oxidative damage to the kidneys. It presents as a slowly progressive chronic kidney disease and proximal tubular dysfunction that, in contrast to typical Fanconi syndrome, leads to end-stage renal disease. It is diagnosed clinically and is present only in Acadian people. The objective of this study was to evaluate the effect of <sc>n</sc>-acetyl-<sc>l</sc>-cysteine (NAC), an antioxidant, on the progression of loss of renal function in patients with AVFS. Case Presentation: Chart reviews were conducted on 4 active cases of AVFS not on kidney replacement therapy. Data on age, estimated glomerular filtration rate (eGFR), creatinine, medications, and blood pressure were collected. Progression of eGFR was evaluated in each patient before and after NAC prescription, and the projected time to kidney replacement therapy (eGFR = 10 mL/min/1.73 m2) with and without the use of NAC was calculated for each case. NAC had a positive effect on slowing the rate of eGFR loss in each patient. The projected need for kidney replacement therapy was delayed by an average of 14 years in the treated patients who otherwise would have required it by an estimated mean age of 28 years. Conclusion: This study suggests that NAC has a beneficial effect on slowing the progression of kidney disease in patients with AVFS. This may be due to the modulation of oxidative stress by NAC. Further studies are needed to evaluate the potential benefits of NAC in other chronic kidney conditions. </p>.
Cystic Fibrosis beyond Childhood: A Case Report of Recurrent Heat-Induced Electrolyte Disturbances Unmasking a Late Diagnosis.
Roldán D, León-Póo M, López-Melero E … +1 more , Shabaka A
Nephron · 2026 · PMID 41543998 · Publisher ↗
INTRODUCTION: Cystic fibrosis (CF) is an autosomal recessive genetic disorder caused by pathogenic variants in the CFTR gene, leading to impaired chloride and bicarbonate transport across epithelial tissues. While typica...
INTRODUCTION: Cystic fibrosis (CF) is an autosomal recessive genetic disorder caused by pathogenic variants in the CFTR gene, leading to impaired chloride and bicarbonate transport across epithelial tissues. While typically diagnosed in infancy due to classic respiratory and gastrointestinal symptoms, atypical forms can present later in life with subtle or isolated manifestations. This case represents a rare monosymptomatic and atypical adult-onset presentation of CF, with electrolyte disturbances as the sole clinical manifestation. CASE PRESENTATION: We present the case of a 31-year-old man who developed recurrent episodes of profound electrolyte imbalance and acute kidney injury following intense physical activity in high environmental temperatures. He exhibited syncope, muscle cramps, and signs of severe dehydration. Laboratory tests revealed hyponatremia, hypokalemia, hypochloremia, hemoconcentration, and elevated creatinine levels, with urine findings consistent with extrarenal salt loss. A similar episode had occurred 6 years earlier. In the absence of an identifiable cause, CF was suspected. Sweat chloride testing confirmed the diagnosis in two separate samples. Genetic analysis revealed compound heterozygosity for a pathogenic CFTR variant and a variant of uncertain significance. Screening for other common manifestations of CF was negative. CONCLUSION: This case underscores the diagnostic challenge of adult-onset CF, particularly in regions where neonatal screening was not historically implemented. Electrolyte disturbances, especially in the context of heat stress, may represent the only clinical clue in patients with residual CFTR function. Prompt recognition and diagnosis are essential for initiating appropriate monitoring and treatment, including the potential use of CFTR modulator therapies that can significantly improve quality of life and long-term outcomes.
Metabolic Demand, Renal Mass, and Glomerular Filtration Rate Changes in Men and Women: Lessons from Living Kidney Donors and Recipients.
González-Rinnea A, Porrini E
Nephron · 2026 · PMID 41528951 · Publisher ↗
BACKGROUND: The determinants of glomerular filtration rate (GFR) and its changes over time are multiple and diverse. Nephron endowment is the starting point for GFR. Low renal endowment due to maternal undernutrition or...
BACKGROUND: The determinants of glomerular filtration rate (GFR) and its changes over time are multiple and diverse. Nephron endowment is the starting point for GFR. Low renal endowment due to maternal undernutrition or premature birth as well as the loss of renal mass because of surgical procedures have a major impact on GFR. Eventually low renal mass may lead to organ damage when combined with metabolic syndrome and obesity or diabetes. The kidney has a major role in maintaining homeostasis. Changes in metabolic demand have a major influence in renal function. Increments in metabolic demand may determine a compensatory increase in GFR to cope with the new metabolic status. Clear examples of these conditions are pregnancy and obesity. Also, GFR may vary in response to the stimulation of renal reserve and the ageing process. All these aspects are different in men and women and may explain gender differences in renal function in health and disease. SUMMARY: In general, women have lower renal mass and lower metabolic demand. However, the study of these aspects in humans is complex. A living donor has two healthy kidneys and after nephrectomy, one of them remains in the same subject undergoing mechanisms of compensation whereas the other is transplanted in a patient with CKD that might have different body size, sex or age, than the donor. This makes the living kidney donation a unique setting to study the determinants of GFR. In this review, we take advantage of data from living kidney donors and recipients to understand diverse determinants of GFR, focusing on gender differences in renal function. KEY MESSAGES: In health and disease, several factors influence GFR like renal mass, the presence or absence of renal reserve, metabolic demand, the capacity of the kidney to adapt to it and the effect of ageing and senescence. In all of them, gender differences play a relevant role, making differences between men and women a factor to consider in the analysis of GFR.
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