Yaping Zhang, Yinghui Wang, Jingyan Yang, Gang Liu
{"title":"Melatonin Protects Against Diabetic Kidney Disease via the SIRT1/NLRP3 Signalling Pathway.","authors":"Yaping Zhang, Yinghui Wang, Jingyan Yang, Gang Liu","doi":"10.1111/nep.70073","DOIUrl":"https://doi.org/10.1111/nep.70073","url":null,"abstract":"<p><strong>Aim: </strong>Diabetic kidney disease (DKD) is a leading cause of end-stage renal disease. Melatonin, a hormone with anti-oxidative and anti-inflammatory properties, has demonstrated potential in mitigating diabetic complications; however, its specific effects and mechanisms in DKD remain unclear. This study aimed to investigate the protective effects of melatonin on podocyte injury and renal damage in DKD and to elucidate its underlying mechanisms.</p><p><strong>Methods: </strong>In vitro, podocytes were exposed to high glucose (HG) and advanced glycation end products (AGEs) to simulate a diabetic environment. The effects of melatonin on podocyte viability, cytoskeletal organisation, oxidative stress markers (MDA, SOD, CAT, and GSH), and NLRP3 signalling activation were assessed. In vivo, a DKD mouse model was treated with melatonin, and renal function, histopathology, oxidative stress, and inflammation were evaluated. The role of SIRT1 in mediating the effects of melatonin was also investigated.</p><p><strong>Results: </strong>Melatonin improved podocyte viability, alleviated cytoskeletal disorganisation, reduced oxidative stress (decreased MDA, increased SOD, CAT, and GSH), and inhibited activation of the NLRP3 signalling pathway in podocytes and renal tissues. In DKD mice, melatonin reduced proteinuria, improved renal histopathology, and suppressed NLRP3-mediated inflammation. Mechanistically, melatonin upregulated SIRT1 expression, which inhibited NLRP3 activation and downstream inflammatory responses.</p><p><strong>Conclusion: </strong>Melatonin protects against DKD by enhancing podocyte viability, reducing oxidative stress, and suppressing NLRP3-mediated inflammation through SIRT1 upregulation. These findings highlight the potential of melatonin as a therapeutic agent for DKD.</p>","PeriodicalId":520716,"journal":{"name":"Nephrology (Carlton, Vic.)","volume":"30 7","pages":"e70073"},"PeriodicalIF":0.0,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144532849","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Three Years Outcome Comparison of Remote Monitoring Versus Conventional Automated Peritoneal Dialysis in Incident APD Patients: A Retrospective Study From Taiwan.","authors":"Mu-Chi Chung, Tung-Min Yu, Laing-You Wu, Ching-Ching Hsiao, Ya-Wen Chuang, Ming-Ju Wu, Cheng-Hsu Chen, Chi-Jung Chung","doi":"10.1111/nep.70061","DOIUrl":"https://doi.org/10.1111/nep.70061","url":null,"abstract":"<p><strong>Aim: </strong>The efficacy of the remote monitoring of automated peritoneal dialysis (RM-APD) with Sharesource platform versus conventional APD outcomes in East Asia remains limited.</p><p><strong>Methods: </strong>This study analysed data from incident APD patients at Taichung Veterans General Hospital between 2010 and 2021. Patients were categorised into RM-APD (n = 77, initiated March 2019-Dec 2021) and conventional APD groups (n = 100, initiated Jan 2010-Dec 2017). Follow-up for both groups extended to censoring, studied outcomes, or the end of the observation period (Dec 2018 for conventional APD and Dec 2022 for RM-APD), with a maximum follow-up of 3 years. Outcomes included death, technique survival, peritonitis incidence and hospitalisation rates using Cox proportional hazard models.</p><p><strong>Results: </strong>Among 452 patients undergoing peritoneal dialysis, 177 were included after exclusions. Mean age was 49 years in RM-APD and 46 years in conventional APD groups. After mean follow-up of 2.05 years, no significant differences were found in mortality, technique survival rates, or hospitalisation rates between groups. However, RM-APD showed significantly lower peritonitis incidence compared to conventional APD (HR = 0.39, 95% CI: 0.17-0.90, p < 0.05). The conventional APD group had 3 relapse peritonitis and 2 transfer to HD, while the RM-APD group had none.</p><p><strong>Conclusions: </strong>RM-APD using Sharesource platform significantly reduces peritonitis incidence compared to conventional APD in Taiwan. While mortality and technique survival were similar, improved peritonitis outcomes suggest RM-APD can enhance care quality and safety for incident APD patients in clinical practice. Further prospective studies are warranted to validate these findings.</p>","PeriodicalId":520716,"journal":{"name":"Nephrology (Carlton, Vic.)","volume":"30 7","pages":"e70061"},"PeriodicalIF":0.0,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144532850","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Application of Estimated Glomerular Filtration Rate Decline Rate to Optimise Timing of Pre-Emptive Arteriovenous Fistula Creation for Chronic Kidney Disease Patients.","authors":"Panuwat Chuemor, Kittipan Rerkasem, Apichat Tantraworasin, Jiraporn Khorana, Theerachai Thammathiwat","doi":"10.1111/nep.70029","DOIUrl":"https://doi.org/10.1111/nep.70029","url":null,"abstract":"<p><strong>Aim: </strong>Establishing a functional pre-emptive arteriovenous fistula (pAVF) before the commencement of haemodialysis requires careful patient selection and precise timing. Our study aimed to create an eGFR (estimated glomerular filtration rate) slope-based prediction model to estimate the time before the onset of haemodialysis for individual patients.</p><p><strong>Methods: </strong>In this prognostic prediction study, we retrospectively analysed past eGFR data from chronic kidney disease patients who underwent pAVF surgery at Naresuan University Hospital and Maharaj Nakorn Chiangmai Hospital in Thailand.</p><p><strong>Results: </strong>Seventy-one patients who underwent pAVF creation between January 1st, 2016, and December 31st, 2022, were included. 75% achieved successful AVF cannulation for their initial haemodialysis, with 5% not needing dialysis. Past eGFR decline rate (eGFRr), eGFR at referral for AVF (eGFRbase), age ≥ 80 years systolic blood pressure, body mass index, and serum calcium levels have been identified as significant factors in estimating the time remaining before the initiation of haemodialysis (Tr). The model estimates the probability of successful pAVF use versus the need for a catheter at the first haemodialysis session. The model's discriminative performance, evaluated via area under the receiver operating characteristic curve (AuROC), yielded a value of 0.96 (95% CI: 0.92, 1.00) and reliable results as confirmed by the calibration plot.</p><p><strong>Conclusion: </strong>Our prediction model helps estimate Tr for individual patients, enabling timely referral for creation of a pAVF. Implementation in clinical practice has the potential to enhance the utilisation of pAVF and reduce the risk of catheter-dependent haemodialysis. However, a prospective validation study is required to confirm the accuracy of the model before its clinical application.</p>","PeriodicalId":520716,"journal":{"name":"Nephrology (Carlton, Vic.)","volume":"30 7","pages":"e70029"},"PeriodicalIF":0.0,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144532845","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Zheng Xi Kog, Jiashen Cai, Li Choo Ng, Zhihua Huang, Lydia Lim, Felicia Loo, Hanis Bte Abdul Kadir, Jia Liang Kwek, Jason Choo, Lina Choong, Chieh Suai Tan, Cynthia C Lim
{"title":"Hospital Readmissions for All-Causes and Fluid Overload in Severely Reduced Kidney Function Not Requiring Dialysis-A Retrospective Cohort Study.","authors":"Zheng Xi Kog, Jiashen Cai, Li Choo Ng, Zhihua Huang, Lydia Lim, Felicia Loo, Hanis Bte Abdul Kadir, Jia Liang Kwek, Jason Choo, Lina Choong, Chieh Suai Tan, Cynthia C Lim","doi":"10.1111/nep.70071","DOIUrl":"https://doi.org/10.1111/nep.70071","url":null,"abstract":"<p><strong>Aim: </strong>Chronic kidney disease is associated with frequent readmissions for ambulatory-sensitive conditions such as fluid overload. There is a paucity of literature to identify individuals at high risk of fluid overload or all-cause readmissions.</p><p><strong>Methods: </strong>We performed a single-centre retrospective cohort study involving 783 patients with an estimated glomerular filtration rate of 11-30 mL/min/1.73 m<sup>2</sup> hospitalised for fluid overload between 2015 and 2017. Multivariable logistic regression analysis was performed to evaluate associations between the 30-day fluid overload-related and all-cause readmissions and various sociodemographic factors, comorbidities and healthcare utilisation.</p><p><strong>Results: </strong>The 30-day readmission rate for fluid overload and all causes were 10.6% and 26.8%, respectively. Fluid overload readmissions were associated with atherosclerotic cardiovascular disease (ASCVD; adjusted odds ratio [aOR] 1.81, 95% CI 1.08-3.03), atrial fibrillation (AF; aOR 1.93, 95% CI 1.13-3.30), higher serum potassium (aOR 1.61, 95% CI 1.14-2.26) and use of high-dose intravenous furosemide during the index hospitalisation (aOR 1.66, 95% CI 1.02-2.67). In contrast, prior nephrology consult (aOR, 0.51, 95% CI 0.29-0.89) and renin-angiotensin system (RAS) blocker prescription at discharge (aOR 0.61, 95% CI 0.38-0.99) were associated with reduced risk of readmission for fluid overload. More frequent emergency department visits (aOR 1.21, 95% CI 1.04-1.40) and higher LACE score (aOR 1.09, 95% CI 1.01-1.18) were independently associated with 30-day readmission for all causes. Hypertension (aOR 0.62, 95% CI 0.42, 0.93), antidepressant use (aOR 0.40, 95% CI 0.16-0.99) and statin prescription at discharge (aOR 0.53, 95% CI 0.35-0.81) were associated with reduced risk for all-cause readmissions.</p><p><strong>Conclusion: </strong>Factors related to comorbidity burden (ASCVD, AF, more frequent emergency department visits and higher LACE score) and disease severity (higher serum potassium and need for high-dose intravenous furosemide) can identify individuals at increased risk of readmission. Further research is required to evaluate the impact of modifiable factors (nephrology consult, RAS blocker prescription at discharge and statin prescription at discharge) to reduce fluid overload-related and all-cause readmissions.</p>","PeriodicalId":520716,"journal":{"name":"Nephrology (Carlton, Vic.)","volume":"30 7","pages":"e70071"},"PeriodicalIF":0.0,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144532847","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Bente Ponsaerts, Evangelos Ntalianis, Everton J Santana, František Sabovčik, Amaryllis Van Craenenbroeck, Maarten Naesens, Tatiana Kuznetsova, Nicholas Cauwenberghs
{"title":"Longitudinal Changes and Predictive Value of the Renal Resistive Index in the General Population.","authors":"Bente Ponsaerts, Evangelos Ntalianis, Everton J Santana, František Sabovčik, Amaryllis Van Craenenbroeck, Maarten Naesens, Tatiana Kuznetsova, Nicholas Cauwenberghs","doi":"10.1111/nep.70068","DOIUrl":"10.1111/nep.70068","url":null,"abstract":"<p><strong>Aim: </strong>Haemodynamic imaging markers like the renal-resistive index (RRI) provide insights into cardiovascular-renal interactions. However, longitudinal epidemiological data on the RRI's natural history, correlates and predictive value are lacking. We investigated factors associated with longitudinal RRI changes (ΔRRI) and its predictive value for cardiovascular events in the community.</p><p><strong>Methods: </strong>In this prospective observational community-based cohort study, 594 adults underwent renal ultrasonography for RRI assessment and were followed for major cardiovascular disease incidence for 9.1 ± 2.3 years. A subset of 135 (22.7%) had a second RRI measurement after 8.1 ± 1.6 years. Stepwise regression identified clinical correlates of ΔRRI, while multivariable Cox models assessed the association between major cardiovascular events and baseline RRI.</p><p><strong>Results: </strong>Over 8 years, mean RRI increased from 0.60 ± 0.05 to 0.63 ± 0.06 (p < 0.001). Greater ΔRRI was independently associated with older age, higher pulse pressure and diabetes mellitus at baseline and with greater increase in pulse pressure during follow-up. Baseline RRI was associated with major cardiovascular events in univariate analysis, but this association was mostly explained by age and was no longer significant after adjustment for confounders (p = 0.51). Neither the highest RRI tertile (HR: 1.22 (0.51 to 2.94), p = 0.65) nor an RRI above 0.70 (HR: 1.39 (0.70 to 2.76), p = 0.35) predicted major cardiovascular events after full adjustment.</p><p><strong>Conclusion: </strong>This study provides the first longitudinal description of the RRI's natural history in a general adult population. RRI increased stronger over time with ageing, higher pulsatility and diabetes mellitus. However, its limited predictive value for cardiovascular events disfavours its use for risk stratification in the community.</p>","PeriodicalId":520716,"journal":{"name":"Nephrology (Carlton, Vic.)","volume":"30 7","pages":"e70068"},"PeriodicalIF":0.0,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144532848","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Correspondence on \"New Onset of Primary Membranous Nephropathy After COVID-19 mRNA Vaccination in Affected Sjogren's Syndrome\".","authors":"Hinpetch Daungsupawong, Viroj Wiwanitkit","doi":"10.1111/nep.70074","DOIUrl":"https://doi.org/10.1111/nep.70074","url":null,"abstract":"","PeriodicalId":520716,"journal":{"name":"Nephrology (Carlton, Vic.)","volume":"30 7","pages":"e70074"},"PeriodicalIF":0.0,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144532846","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Alyssa Pradhan, Melanie Wyld, Susan Wan, Rebecca Davis, Kushani Jayasinghe, Kate Wyburn
{"title":"Risk Factors for the Development of BK Polyomavirus and Treatment Outcomes in Kidney Transplant Recipients: An 8-Year Retrospective Cohort Study.","authors":"Alyssa Pradhan, Melanie Wyld, Susan Wan, Rebecca Davis, Kushani Jayasinghe, Kate Wyburn","doi":"10.1111/nep.70058","DOIUrl":"10.1111/nep.70058","url":null,"abstract":"<p><strong>Background: </strong>BKPyV-DNAemia occurs in up to 30% of kidney transplant recipients (KTRs), with graft-threatening BKPyV-nephropathy in up to 10%. Risk factors for BKPyV-DNAemia, BKPyV-nephropathy, and associated graft loss are incompletely described. We sought to determine the prevalence, risk factors for, and long-term impact of BKPyV-DNAemia.</p><p><strong>Methods: </strong>A single-centre retrospective study of adult KTRs between 2010 and 2018. We used logistic regression to determine odds ratios (OR) of BKPyV-DNAemia, and survival analysis to assess the impact of BKPyV-DNAemia on graft and patient survival.</p><p><strong>Results: </strong>Of 522 patients, 100 (19%) developed BKPyV-DNAemia and 43 (8.2%) developed BKPyV-nephropathy, resulting in the loss of two grafts. Factors associated with the development of BKPyV-DNAemia were non-Caucasian ethnicity (OR 1.76, CI 0.98-3.16), pre-transplant diabetes (OR 2.06, CI 1.02-4.14) and HLA mismatch of 3/6 or 4/6 (OR 2.37, CI 1.06-5.56) and HLA mismatch of 5/6 and 6/6 (OR 2.53, CI 1.20-5.63). Additionally, a greater than 25 mg per day prednisolone dose following acute transplant and acute rejection in the first month post-transplant was associated with an increased risk of BKPyV-DNAemia (OR 3.06, CI 1.66-6.06 and OR 2.36, CI 1.16-4.75 respectively). Over a 10-year follow-up, the development of BKPyV-DNAemia and BKPyV-nephropathy was not associated with reduced graft or patient survival.</p><p><strong>Conclusion: </strong>While BKPyV-DNAemia and BKPyV-nephropathy remain prevalent in KTR, there were low rates of associated graft loss and no demonstrable impact on long-term graft or patient survival.</p>","PeriodicalId":520716,"journal":{"name":"Nephrology (Carlton, Vic.)","volume":"30 6","pages":"e70058"},"PeriodicalIF":0.0,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12133353/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144218570","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Economic Evaluation of Rituximab Versus Corticosteroid-Cyclophosphamide or Cyclosporine in Patients With Membranous Nephropathy in Republic of Korea.","authors":"Heejung Choi, Yoon Cho, Minjeong Lee, Ji-Woo Yun, Hankil Lee, Inwhee Park","doi":"10.1111/nep.70063","DOIUrl":"https://doi.org/10.1111/nep.70063","url":null,"abstract":"<p><strong>Background: </strong>Cyclic corticosteroid-cyclophosphamide or cyclosporine is a well-known membranous nephropathy (MN) treatment but has high risks of adverse drug reactions (ADRs). Rituximab has a non-inferior effect compared to previous treatments, with fewer ADRs. However, the high cost of rituximab is a pharmacoeconomic disincentive.</p><p><strong>Methods: </strong>We conducted a cost-minimisation analysis to evaluate the relative and absolute costs of rituximab versus corticosteroid-cyclophosphamide or cyclosporine in patients with MN over 2 years using a decision-tree model based on ADRs from two pivotal trials (RI-CYCLO and MENTOR). We included costs of medication, time, transportation, and ADRs. Deterministic sensitivity analysis and threshold analysis were performed to assess the uncertainty of the model input parameters and estimate the appropriate price of rituximab.</p><p><strong>Results: </strong>The total expected cost for rituximab was $4132, and $2684 for the comparators, with an expected incremental cost of $1448. Despite a 2.8 times higher medication cost, rituximab reduced ADR costs by 96.3% ($223), time costs by 31.1% ($280), and transportation costs by 46.8% ($180). A 46.9% price reduction of rituximab would make it an economically favourable option for treating MN compared to comparators.</p><p><strong>Conclusions: </strong>If the price of rituximab is reduced, it can be a good alternative to corticosteroid-cyclophosphamide or cyclosporine for MN in the Republic of Korea.</p>","PeriodicalId":520716,"journal":{"name":"Nephrology (Carlton, Vic.)","volume":"30 6","pages":"e70063"},"PeriodicalIF":0.0,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144259917","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Qiang Liu, Qian Wang, Hu Tan, Weina Zhang, Beining Wang, Tiantian Jin, Li Zhang, Yan Gao
{"title":"Renal-Limited Thrombotic Microangiopathy Induced by Fruquintinib and Tislelizumab: A Case Report.","authors":"Qiang Liu, Qian Wang, Hu Tan, Weina Zhang, Beining Wang, Tiantian Jin, Li Zhang, Yan Gao","doi":"10.1111/nep.70067","DOIUrl":"https://doi.org/10.1111/nep.70067","url":null,"abstract":"<p><p>Thrombotic microangiopathy (TMA) is a rare but potentially severe condition induced by cancer treatments, including angiogenesis inhibitors and immune checkpoint inhibitors. This case report presents the first documented instance of renal-limited TMA potentially triggered by fruquintinib and tislelizumab in a patient with metastatic rectal cancer. A 60-year-old woman with stage IIIB rectal cancer developed nephrotic syndrome following treatment with fruquintinib and tislelizumab. She had no prior history of kidney disease but presented with lower limb oedema and proteinuria. Renal biopsy-confirmed TMA with focal segmental glomerulosclerosis. Following discontinuation of the drugs and management of hypertension, her renal function improved and proteinuria resolved. This case underscores the importance of monitoring renal function in patients with cancer receiving fruquintinib and immune checkpoint inhibitors, as TMA may develop even after short-term exposure. Early detection through renal biopsy and prompt withdrawal of the causative agents may prevent irreversible renal damage. Further research is required to better understand the pathophysiology of TMA in this context and to inform management strategies.</p>","PeriodicalId":520716,"journal":{"name":"Nephrology (Carlton, Vic.)","volume":"30 6","pages":"e70067"},"PeriodicalIF":0.0,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144319198","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"The First Observation and Diagnosis of Nail-Patella Syndrome Using LV-SEM: GBM Abnormalities Mimicking Alport Syndrome.","authors":"Hiroki Ito, Katsuya Ishiyama, Takuo Hirose, Shigemitsu Sato, Risa Ishikawa, Akari Endo, Ikuko Oba-Yabana, Tomoyoshi Kimura, Wako Yumura, Takefumi Mori","doi":"10.1111/nep.70062","DOIUrl":"https://doi.org/10.1111/nep.70062","url":null,"abstract":"<p><p>We report the case of a 32-year-old man with a family history of nail patella syndrome (NPS) who presented with severe kidney dysfunction, hematuria, and proteinuria. A renal biopsy was performed to rule out other kidney diseases and obtain information on the possibility of future renal transplantation, as the renal morphology was relatively preserved and abnormal urinary findings indicative of nephritis. The scarcity of viable glomeruli for conventional transmission electron microscopy (TEM) prompted us to use low-vacuum scanning electron microscopy (LV-SEM) on light microscopy samples. LV-SEM analysis revealed characteristic glomerular basement membrane (GBM) alterations, including a coarse meshwork structure and a ragged pattern, similar to the GBM changes observed in Alport syndrome. These findings, combined with genetic testing results, confirmed the diagnosis of NPS-associated nephropathy. This case represents the first reported use of LV-SEM for observing and diagnosing NPS-associated nephropathy, demonstrating its potential as a valuable diagnostic tool wherein sample availability or quality limits the conventional TEM.</p>","PeriodicalId":520716,"journal":{"name":"Nephrology (Carlton, Vic.)","volume":"30 6","pages":"e70062"},"PeriodicalIF":0.0,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144259918","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}