Nephrology最新文献

{"title":"Fibrillary Glomerulonephritis: Clinicopathological Characteristics and Outcome-Case Series From a Multicentre Australasian Cohort.","authors":"Muralikrishna Gangadharan Komala, Angela Bayly, Adrian Y S Lee, Brian Nankivell, Levina Neill, Seethalakshmi Viswanathan","doi":"10.1111/nep.70022","DOIUrl":"10.1111/nep.70022","url":null,"abstract":"<p><strong>Aim: </strong>Fibrillary glomerulonephritis (FGN) is a rare deposition disease with unclear aetiology. There are limited case series of FGN described in the literature. Here, we describe the clinicopathological characteristics and outcomes of a series of 26 patients with FGN diagnosed at an Australian tertiary centre for renal diseases over a decade.</p><p><strong>Method(s): </strong>The present study includes 26 patients with biopsy-proven FGN diagnosed between January 2011 and December 2021.</p><p><strong>Results: </strong>The average age at presentation was 60 years, with a female predominance. The mean creatinine at presentation was 205 μmol/L. Most of the patients had significant proteinuria, with an average 24-h urine protein of 3.76 g. Associated conditions included four patients with autoimmune disease, one patient with malignancy, and two patients with Hepatitis C infection. Serum electrophoresis demonstrated monoclonality in three patients, although immunofluorescence did not reveal clonal restriction on the renal biopsy. Most patients had mesangial expansion, with an increase in mesangial cellularity and variable degrees of capillary wall thickening. An established membranoproliferative pattern was seen in 10 patients. The median follow-up period was 33 months. Three patients received therapy targeted at FGN. End-stage kidney disease developed in 10 patients, with 6 patients dying during the follow-up period, mostly due to additional cardiovascular disease or sepsis.</p><p><strong>Conclusion: </strong>This case series of FGN demonstrates that a significant proportion of patients progress towards end-stage kidney disease. The mortality is significant although the cause of death is due to additional conditions rather than directly due to FGN.</p>","PeriodicalId":19264,"journal":{"name":"Nephrology","volume":"30 4","pages":"e70022"},"PeriodicalIF":2.4,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11949550/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143730906","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":"Effectiveness of Semaglutide in Overweight or Obese Patients With IgA Nephropathy Without Diabetes.","authors":"Feng-Lei Si, Chen Tang, Pei Chen, Wan-Yin Hou, Hong-Yu Yang, Ji-Cheng Lv, Su-Fang Shi, Xu-Jie Zhou, Li-Jun Liu, Hong Zhang","doi":"10.1111/nep.70023","DOIUrl":"https://doi.org/10.1111/nep.70023","url":null,"abstract":"","PeriodicalId":19264,"journal":{"name":"Nephrology","volume":"30 4","pages":"e70023"},"PeriodicalIF":2.4,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143753651","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":"Correction to \"Comment on: The Comprehensive Incidence and Risk Factors of Fracture in Kidney Transplant Recipients: A Meta-Analysis\".","authors":"","doi":"10.1111/nep.70026","DOIUrl":"https://doi.org/10.1111/nep.70026","url":null,"abstract":"","PeriodicalId":19264,"journal":{"name":"Nephrology","volume":"30 4","pages":"e70026"},"PeriodicalIF":2.4,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143764425","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":"Correction to \"Role of lncRNA-MEG8/miR-296-3p Axis in Gestational Diabetes Mellitus\".","authors":"","doi":"10.1111/nep.70032","DOIUrl":"https://doi.org/10.1111/nep.70032","url":null,"abstract":"","PeriodicalId":19264,"journal":{"name":"Nephrology","volume":"30 4","pages":"e70032"},"PeriodicalIF":2.4,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143796039","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":"Long non-coding RNA Growth Arrest Specific 5 Regulates the Podocyte Function in Nephrotic Syndrome Development via microRNA-144-5p/Phosphatase And Tensin Homolog.","authors":"Xinyi Zhang, Huan Tian, Chang Lu, Shen-Ping Xie, Jing-Sheng Ma, Huai-Zhou Chen, Dong-E Tang, Yong Dai, Qiang Yan, Wei Xian","doi":"10.1111/nep.70024","DOIUrl":"https://doi.org/10.1111/nep.70024","url":null,"abstract":"<p><strong>Aim: </strong>This research examined the role and possible regulatory mechanisms of lncRNA GAS5 in the occurrence and progression of primary nephrotic syndrome (PNS) to provide biomarkers for early screening of PNS in the clinic.</p><p><strong>Methods: </strong>RT-qPCR was employed to assess the expression levels of GAS5 and miR-144-5p. ROC analysis was conducted to evaluate their predictive capabilities for PNS. The interaction between GAS5 and miR-144-5p was confirmed using a dual-luciferase assay. Following this, GAS5 overexpression plasmids, along with co-transfected plasmids, were introduced into podocytes to examine their impact on the inflammatory factors, oxidative stress index, cell proliferation and apoptosis. Furthermore, we performed GO and KEGG enrichment analyses, along with PPI analysis, on the target genes of miR-144-5p to speculate on its potential functions and to identify critical genes.</p><p><strong>Results: </strong>The expression levels of GAS5 were decreased while miR-144-5p levels were elevated in PNS patients. The diagnostic approach of serum GAS5 combined with miR-144-5p improved the accuracy of identification. GAS5 was observed to inhibit inflammation and oxidative stress responses and the apoptosis of MPC-5 cells, and enhance cell proliferation. However, the overexpression of miR-144-5p counteracted the effect of GAS5 on podocyte function. Enrichment analysis suggested the miR-144-5p target genes could affect podocyte structure, homeostasis and cell growth. PTEN and STAT3 are identified as critical regulatory targets.</p><p><strong>Conclusion: </strong>The sponging effect of GAS5 on miR-144-5p caused changes in PTEN mRNA expression and could potentially prevent or mitigate PNS. GAS5 is expected to become a potential target for treating PNS.</p>","PeriodicalId":19264,"journal":{"name":"Nephrology","volume":"30 4","pages":"e70024"},"PeriodicalIF":2.4,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143743648","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":"Acute Kidney Injury in Hospitalised Cancer Patients: Incidence, Risk Factors and Outcomes.","authors":"Yanting Shi, Genwen Chen, Zhihui Lu, Hao Wang, Jiarui Xu, Yang Li, Jie Teng","doi":"10.1111/nep.70025","DOIUrl":"https://doi.org/10.1111/nep.70025","url":null,"abstract":"<p><strong>Aim: </strong>Acute kidney injury (AKI) is a common complication in cancer patients and significantly impacts their treatment and prognosis. To better understand the epidemiology and clinical implications of AKI in hospitalised cancer patients, this study was designed to determine the incidence of AKI, identify risk factors for AKI and assess the impact of AKI on in-hospital outcomes.</p><p><strong>Methods: </strong>Retrospective analysis of 68 379 cancer admissions in 2019. AKI incidence, risk factors (demographics, comorbidities and clinical characteristics), and impact on in-hospital mortality and length of stay were assessed. Logistic regression was employed to identify the risk factors for AKI. Survival analysis was conducted using the Cox proportional hazards model, with log-rank statistics used to assess survival outcome.</p><p><strong>Results: </strong>Of the 68 379 eligible cancer admissions, 7734 AKI cases were recognised with an incidence rate of 11.3%. The highest rates were observed in renal cancer (40.1%), ureter cancer (27.9%) and multiple myeloma (16.1%). Clinical risk factors such as age > 50 years, body mass index < 18.5 kg/m², and hyperuricemia were significantly associated with hospital-acquired AKI compared to the non-AKI group (p < 0.001). In cases of severe community-acquired AKI, significant differences in hypertension, anaemia and leukocyte elevation were also observed (p < 0.001). The mortality rate was notably higher in AKI patients, especially in the severe AKI subgroup. The length of stay was markedly prolonged in patients with hospital-acquired and severe AKI, further underscoring the clinical burden of this complication.</p><p><strong>Conclusion: </strong>Hospitalised cancer patients experience a high incidence of AKI. Identifying and mitigating risk factors may improve patient outcomes.</p>","PeriodicalId":19264,"journal":{"name":"Nephrology","volume":"30 4","pages":"e70025"},"PeriodicalIF":2.4,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143796009","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 Role of IL-10-Producing Regulatory B Cells in Children With Primary Nephrotic Syndrome.","authors":"Baohui Yang, Xiongjun Tan, Shifang Dong, Mo Wang, Daoqi Wu, Gaofu Zhang, Xuelan Chen, Mei Wang, Haiping Yang, Qiu Li","doi":"10.1111/nep.70008","DOIUrl":"https://doi.org/10.1111/nep.70008","url":null,"abstract":"<p><strong>Aim: </strong>This study aimed to investigate the contribution of interleukin (IL)-10-producing regulatory B cells (B10 cells) to the pathogenesis of PNS.</p><p><strong>Methods: </strong>The percentages of B10 cells, CD19⁺CD24^hiCD27⁺ B cells, CD19⁺CD24^hiCD38^hi B cells, CD19⁺CD24^hiCD27⁺IL-10⁺ B cells, CD19⁺CD24^hiCD38^hiIL-10⁺ B cells, Th17 cells, and regulatory T (Treg) cells within the peripheral blood mononuclear cells (PBMCs) from healthy subjects and PNS patients with active disease or in remission were analysed by flow cytometry.</p><p><strong>Results: </strong>The percentages and IL-10 production of circulating B10 cells and the two subsets were decreased in children with active PNS and returned to normal levels in PNS patients in remission. B10 cells and their subsets were negatively correlated with the Th17/Treg ratio. The percentages of B10pro+B10 cells, CD19⁺CD24^hiCD27⁺IL-10⁺ B cells, CD19⁺CD24^hiCD38^hiIL-10⁺ B cells, Th17 cells, Treg cells, and the Th17/Treg ratio did not significantly differ between groups after CpG and CD40L stimulation. However, in the presence of CpG and anti-CD40L mAb, the percentages of B10pro+B10 cells, CD19⁺CD24^hiCD27⁺IL-10⁺ B cells, CD19⁺CD24^hiCD38^hiIL-10⁺ B cells, and Treg cells were significantly reduced, whereas the Th17 cell percentage and the Th17/Treg ratio were significantly increased. Furthermore, the Th17/Treg ratio correlated negatively, whereas the percentages of B10 cells and their subsets correlated positively with serum immunoglobulin G (IgG) concentration.</p><p><strong>Conclusion: </strong>Our study demonstrates that the numbers of B10 cells and their ability to produce IL-10 are decreased, leading to an imbalance in the Th17/Treg ratio and low IgG levels, which may then contribute to the pathogenesis of PNS.</p>","PeriodicalId":19264,"journal":{"name":"Nephrology","volume":"30 3","pages":"e70008"},"PeriodicalIF":2.4,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143573156","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":"A Case of Atypical Hemolytic Uremic Syndrome With a Complement Factor I Mutation Triggered by a Femoral Neck Fracture.","authors":"Toshiki Kano, Hiroaki Io, Yu Sasaki, Masahiro Muto, Sayaka Muto, Kei Ogiwara, Arisa Ikeda, Hiroyuki Iwasaki, Yusuke Suzuki","doi":"10.1111/nep.70010","DOIUrl":"10.1111/nep.70010","url":null,"abstract":"<p><p>Atypical hemolytic uremic syndrome is a thrombotic microangiopathy caused by the abnormal activation of the alternative complement pathway. Mutations in complement-related genes and autoantibodies against complement regulators are involved in the pathogenesis of this condition; the frequency of, and prognosis of patients harbouring, each genetic mutation varies based on the region and race. Complement factor I (CFI) mutations have been observed in 4%-8% of cases in Europe; however, they have not yet been reported in Japan. We present the first Japanese case of atypical hemolytic uremic syndrome in a patient harbouring a CFI mutation. An 83-year-old female patient presented with severe acute kidney injury, thrombocytopenia, and hemolytic anaemia following a femoral neck fracture. Plasma exchange and haemodialysis were initiated, resulting in improved kidney function and platelet count. However, the platelet count decreased when plasma exchange was discontinued. Therefore, we administered ravulizumab, an anti-complement 5 monoclonal antibody, which led to the maintenance of stable kidney function and platelet count. Genetic analysis revealed a CFI mutation, and the patient was treated with ravulizumab for 2 years without relapse. Individuals diagnosed with atypical hemolytic uremic syndrome harbouring CFI mutations experience poor outcomes, including low rates of remission, high rates of mortality, and progression to end-stage kidney disease. Our case serves as a crucial example demonstrating how prompt identification and appropriate management can lead to better patient outcomes.</p>","PeriodicalId":19264,"journal":{"name":"Nephrology","volume":"30 3","pages":"e70010"},"PeriodicalIF":2.4,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11861886/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143502886","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":"Challenges of Managing Lupus Nephritis in an Emerging Nephrology Centre: A Fijian Cohort Study.","authors":"Vishal Kumar, Yogeshni Chandra, Sabiha Khan, Mai Ling Perman, Angus Ritchie, Shilpanjali Jesudason, Anis Ta'eed","doi":"10.1111/nep.70012","DOIUrl":"https://doi.org/10.1111/nep.70012","url":null,"abstract":"<p><strong>Aim: </strong>To characterise the epidemiology and outcomes of Lupus Nephritis (LN) in Fiji.</p><p><strong>Methods: </strong>All adult LN cases diagnosed from 2016 to 2020 at the national referral hospital were included. Treatment response, kidney failure, dialysis dependence and death were reported.</p><p><strong>Results: </strong>From 33 cases, a crude annual incidence of 2.44 (95% CI 1.73-3.43) per 100,000 population and an age-standardised incidence of 2.37 (95% CI 0.65-4.09) per 100,000 population was derived. The median age was 25.7 years (IQR 19.5-32) with a predominance of indigenous iTaukei ethnicity (67%). Kidney biopsy with adequate tissue was performed in 24 patients (73%), revealing LN class III in 10 patients (42%) and class IV in 14 patients (58%). Twenty-eight patients (85%) underwent induction immunosuppression, with complete and partial response in 12 patients (43%) and 2 patients (7%) at 12 months, respectively. No factor was found to be significantly associated with complete response at 12 months. At 2 years, 13 patients (39%) had developed kidney failure, 6 of whom commenced dialysis, and 13 patients (39%) had died. The risk of dialysis dependence or death was associated with suboptimal adherence to therapy (OR 12.0, 95% CI 1.23-117, p = 0.028) and 12-month complete response (OR 0.08, 95% CI 0.01-0.54, p = 0.005).</p><p><strong>Conclusion: </strong>Fiji has a high incidence of LN and nearly half of our cohort had either died or were dialysis dependent within 2 years of diagnosis. These results will inform targeted healthcare strategies that can be implemented in Fiji and neighbouring Pacific Island countries.</p>","PeriodicalId":19264,"journal":{"name":"Nephrology","volume":"30 3","pages":"e70012"},"PeriodicalIF":2.4,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143597294","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":"Plasma Potassium Negatively Correlates With Sodium Chloride Cotransporter Abundance and Phosphorylation in Urinary Extracellular Vesicles From Patients With Chronic Kidney Disease.","authors":"Aihua Wu, Martin J Wolley, David Vesey, Andrew S Terker, Paul A Welling, Robert A Fenton, Michael Stowasser","doi":"10.1111/nep.70017","DOIUrl":"10.1111/nep.70017","url":null,"abstract":"<p><strong>Aim: </strong>Using urinary extracellular vesicles (uEVs), we have demonstrated the functional 'renal-K switch' mechanism (the WNK-SPAK-NCC pathway) in both healthy subjects and those with primary aldosteronism. The close relationship between blood pressure and CKD has led to the hypothesis that high potassium intake may be reno-protective through the same mechanism. This study used uEVs to evaluate whether plasma potassium negatively correlates with NCC and its phosphorylation (pNCC) in patients with CKD.</p><p><strong>Methods: </strong>Morning blood and second morning urine were collected on a single occasion between 8 and 11 AM from patients with various CKD stages. Plasma potassium levels were assessed by a local pathology laboratory. uEVs were obtained by progressive ultracentrifugation, and NCC and pNCC were analysed by western blotting.</p><p><strong>Results: </strong>Correlation analyses among 23 patients with CKD revealed the abundance of NCC (R² = 0.46, p = 0.0003) and pNCC (R² = 0.30, p = 0.0067) strongly and negatively correlate with plasma potassium. The negative correlations persist among 18 patients who did not receive SGLT2 inhibitors or K-binders (NCC: R² = 0.5, p = 0.002; pNCC: R² = 0.30, p = 0.03) and the negative trends remain among 5 patients who received either SGLT2 inhibitors or K-binders (NCC: R² = 0.64, p = 0.11; pNCC: R² = 0.42, p = 0.24).</p><p><strong>Conclusion: </strong>In patients with CKD, there are negative correlations between NCC and pNCC in uEVs and plasma potassium, which appear independent of eGFR. This suggests that the mechanism at play is distinct from the overall kidney function, and potassium supplement within a safe level may assist in natriuresis and improve cardiovascular outcomes.</p>","PeriodicalId":19264,"journal":{"name":"Nephrology","volume":"30 3","pages":"e70017"},"PeriodicalIF":2.4,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11907743/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143625079","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}