Journal of Nephrology最新文献

{"title":"Peripheral artery disease in chronic kidney disease: an underestimated comorbidity.","authors":"Sidar Copur, Lasin Ozbek, Carlo Basile, Mehmet Kanbay","doi":"10.1093/joneph/aajaf014","DOIUrl":"https://doi.org/10.1093/joneph/aajaf014","url":null,"abstract":"<p><p>Chronic kidney disease (CKD), defined as an estimated glomerular filtration rate (eGFR) below 60 mL/min/1.73 m2 or the presence of clinical, imaging, or histopathological features of kidney injury over a 3-month period, has been linked to several comorbidities including cardiovascular diseases, such as myocardial infarction, cerebrovascular diseases, renal artery stenosis and peripheral artery disease. A pro-inflammatory and pro-fibrotic state, together with the accumulation of uremic toxins and impaired calcium-phosphorus homeostasis leading to mineral and bone disorders, appear to constitute the underlying pathophysiology of peripheral artery disease in CKD patients. Despite such association and the deleterious effects of peripheral artery disease, the association between CKD and peripheral artery disease is generally underestimated in clinical practice, with physicians being less likely to initiate pharmacotherapy or offer interventional treatments to patients with CKD. Many therapeutic options, including lifestyle modifications, methods for cardiovascular risk reduction such as anti-platelet or lipid-lowering drugs, targeted pharmacotherapies, and endovascular or surgical interventions are available for the management of peripheral artery disease. However, the diagnostic and/or therapeutic process in CKD patients is not as straightforward as it is in the general population, with misleading outcomes and higher treatment-related complications. Aim of this narrative review was to discuss the epidemiology, risk factors, underlying pathophysiology, and diagnostic and therapeutic approaches toward peripheral artery disease in CKD patients.</p>","PeriodicalId":16542,"journal":{"name":"Journal of Nephrology","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2026-05-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147839172","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":"An interferon-NETosis-IL-6-epithelial-mesenchymal transition axis defines the early therapeutic window in autosomal dominant polycystic kidney disease.","authors":"Chen Juanlu, Lihan Chen, Pin-Syuan Chen","doi":"10.1093/joneph/aajag029","DOIUrl":"https://doi.org/10.1093/joneph/aajag029","url":null,"abstract":"","PeriodicalId":16542,"journal":{"name":"Journal of Nephrology","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2026-05-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147839158","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":"Pale blue inclusions on light microscopy as a clue to light chain proximal tubulopathy.","authors":"Shoko Ochiai, Masao Kikuchi, Koichi Kaikita, Shouichi Fujimoto","doi":"10.1093/joneph/aajag003","DOIUrl":"https://doi.org/10.1093/joneph/aajag003","url":null,"abstract":"","PeriodicalId":16542,"journal":{"name":"Journal of Nephrology","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2026-05-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147839232","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":"Mycophenolate mofetil-induced oral ulcers in a pediatric kidney transplant recipient.","authors":"Miroslava Brndiarova, Jakub Zieg, Nikola Halacova, Milos Jesenak","doi":"10.1093/joneph/aajaf045","DOIUrl":"https://doi.org/10.1093/joneph/aajaf045","url":null,"abstract":"","PeriodicalId":16542,"journal":{"name":"Journal of Nephrology","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2026-04-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147774159","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":"Natural history of patients with familial focal segmental glomerulosclerosis associated with TRPC6 variants.","authors":"Heidi Sarrasin, Daniel Sidler, Deborah Bartholdi, Christiane Zweier, Vincenzo Girardi, Bruno Vogt, Federica Bocchi","doi":"10.1093/joneph/aajaf054","DOIUrl":"https://doi.org/10.1093/joneph/aajaf054","url":null,"abstract":"<p><strong>Background: </strong>Pathogenic variants in the TRPC6 gene have been identified in families affected by adult-onset autosomal dominant focal segmental glomerulosclerosis (FSGS). Although the exact mechanisms leading to kidney disease remain unclear, growing evidence suggests a role of the TRPC6 channel not only in genetic forms of FSGS but also in acquired forms of glomerular diseases. This highlights TRPC6 as a promising target for therapeutic intervention.</p><p><strong>Methods: </strong>This single-centre cohort study at the University Hospital in Bern, Switzerland, included patients from families with (likely) pathogenic TRPC6 variants. Patients' family history, as well as clinical and genetic data were obtained through interviews and medical records. The study aimed to analyse the renal and extra-renal disease phenotype, its evolution, and explore potential genotype-phenotype correlations.</p><p><strong>Results: </strong>Nine individuals from four unrelated families were included. Most patients presented in adulthood with signs of structural nephropathy. Notably, the initial presentation involved sub-nephrotic range proteinuria rather than nephrotic syndrome, with progression to kidney failure over the course of several years. Four out of nine patients exhibited multi-organ (> 3) involvement with unclear genotype-phenotype correlation. Notably, among the four TRPC6 variants identified, we report a novel variant (p.(Trp680*)), which expands the current spectrum of TRPC6 mutations. Additionally, another variant (p.(Arg175Trp)) was associated with infantile onset of disease characterised by steroid-resistant nephrotic syndrome.</p><p><strong>Conclusion: </strong>This study contributes to a broader understanding of the genotype-phenotype variability in TRPC6-associated FSGS and expands the mutational spectrum by identifying a novel TRPC6 variant, underscoring the importance of genetic analysis in guiding patient prognosis and personalised management strategies.</p>","PeriodicalId":16542,"journal":{"name":"Journal of Nephrology","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2026-04-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147774188","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":"Efficacy, safety, and biomarker changes of B-cell activating factor and A proliferation-inducing ligand-targeted therapies in IgA nephropathy: a systematic review and meta-analysis of randomized controlled trials.","authors":"Rafael Dos Santos Borges, Rodrigo Lara Santos, Luiza Haikal de Paula, Jefferson Manoel Borges Martins, Pedro Antônio Machado Gomes de Sousa, Giovanni Gosch Berton, Ana Cristina Simões E Silva","doi":"10.1093/joneph/aajaf002","DOIUrl":"https://doi.org/10.1093/joneph/aajaf002","url":null,"abstract":"<p><strong>Background: </strong>IgA nephropathy (IgAN) is the most common primary glomerular disease worldwide. B-cell activating factor (BAFF) and A proliferation-inducing ligand (APRIL) are cytokines involved in B-cell activation and survival, contributing to the pathogenesis of IgAN. This meta-analysis aimed to evaluate the efficacy, safety, and biomarkers of BAFF- or APRIL-targeted therapies in patients with IgAN.</p><p><strong>Methods: </strong>We searched PubMed, Scopus, and the Cochrane Library for randomized controlled trials (RCTs) comparing BAFF- or APRIL-targeted drugs with placebo in adults with IgAN, published up to December 2024 and written in English. Efficacy outcomes were the mean percent change in the 24-hour urine protein-to-creatinine ratio (UPCR) and the mean change in the estimated glomerular filtration rate (eGFR) from baseline. Safety included the incidence of adverse events. The biomarkers we used were changes in serum Gd-IgA1, IgG, IgA, and IgM from baseline. The risk of bias and the certainty of evidence were assessed using RoB v2.0 and the Grading of Recommendations, Assessment, Development, and Evaluations (GRADE). We used the R software version 4.2.1 for statistics.</p><p><strong>Results: </strong>Four Phase II RCTs, including 331 patients, were included, with three studies having a low risk of bias, while there were some concerns regarding one study. Compared to placebo, BAFF- or APRIL-targeted drugs significantly reduced 24-hour UPCR (mean difference [MD] -38.94%; 95% confidence interval [CI] -58.98 to -18.90; P = 0.0001; I² = 0%) and significantly improved the eGFR (MD 7.05 mL/min/1.73 m²; 95% CI 3.83 to 10.27; P < 0.0001; I² = 0%). Adverse events did not significantly differ between the study groups. BAFF- or APRIL-targeted drugs significantly decreased serum Gd-IgA1, IgG, IgA, and IgM compared with placebo, indicating lower immune complex formation and response to treatment.</p><p><strong>Conclusion: </strong>BAFF- or APRIL-targeted therapies appear to be effective and safe in reducing proteinuria in patients with IgAN.</p><p><strong>Prospero registration id: </strong>CRD42024598157.</p>","PeriodicalId":16542,"journal":{"name":"Journal of Nephrology","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2026-04-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147774242","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":"Kidney and inferior vena cava abnormalities with leg thromboses.","authors":"Rachel S M Lee, Lawrence P McMahon, Limy Wong","doi":"10.1093/joneph/aajag014","DOIUrl":"https://doi.org/10.1093/joneph/aajag014","url":null,"abstract":"","PeriodicalId":16542,"journal":{"name":"Journal of Nephrology","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2026-04-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147774249","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 decline in kidney function during treatment for malignancy: a single center retrospective cohort study.","authors":"Miho Murashima, Kodai Suzuki, Yuki Miyaguchi, Takahisa Kasugai, Maki Hiratsuka, Takayuki Hamano","doi":"10.1093/joneph/aajag055","DOIUrl":"https://doi.org/10.1093/joneph/aajag055","url":null,"abstract":"","PeriodicalId":16542,"journal":{"name":"Journal of Nephrology","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2026-04-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147774114","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":"Squamous epithelial metaplasia of the bladder in a pediatric patient: a nephrology image.","authors":"Nadide Melike Sav, Yusuf Senoglu, Sinem Kantarcıoğlu Coşkun","doi":"10.1093/joneph/aajag040","DOIUrl":"https://doi.org/10.1093/joneph/aajag040","url":null,"abstract":"","PeriodicalId":16542,"journal":{"name":"Journal of Nephrology","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2026-04-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147774198","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":"Atypical preeclampsia with nephrotic syndrome before 20 weeks of gestation: clinical features and angiogenic biomarker analysis.","authors":"Tomo Suzuki, Mea Aso, Daisuke Ichikawa, Yugo Shibagaki","doi":"10.1093/joneph/aajag042","DOIUrl":"https://doi.org/10.1093/joneph/aajag042","url":null,"abstract":"","PeriodicalId":16542,"journal":{"name":"Journal of Nephrology","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2026-04-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147774177","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}