Journal of Nephrology最新文献

{"title":"Lessons for the clinical nephrologist: encapsulating peritoneal sclerosis immediately following intra-abdominal infection without peritonitis.","authors":"Tzu-Hsien Yang, Chien-Chou Chen, Shih-Hua Lin","doi":"10.1007/s40620-025-02428-w","DOIUrl":"https://doi.org/10.1007/s40620-025-02428-w","url":null,"abstract":"","PeriodicalId":16542,"journal":{"name":"Journal of Nephrology","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2025-09-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145199607","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":"Holistic needs assessment for hospitalized patients with kidney disease: a prospective cross-sectional study.","authors":"Cynthia Hsin-Ya Chao, Ka-Wai Tam, Chien-Ling Su, Yun-Yun Chou, Tzu-Tung Kuo, Juliana Tze-Wah Kao","doi":"10.1007/s40620-025-02427-x","DOIUrl":"https://doi.org/10.1007/s40620-025-02427-x","url":null,"abstract":"<p><strong>Background: </strong>Kidney disease is a chronic health burden that considerably impairs patients' quality of life, necessitating a systematic evaluation of their holistic needs. This study employed the Taiwanese version of the Sheffield Profile for Assessment and Referral for Care (SPARC-T) questionnaire to identify unmet needs among patients with kidney disease.</p><p><strong>Methods: </strong>In this prospective, cross-sectional study, the patients completed the SPARC-T questionnaire upon admission to the nephrology ward. Scores exceeding predefined thresholds in the psychological, depression, religious and spiritual, or social domains triggered referral to a psychiatry department, social works, or a discharge planning team. Demographic information and SPARC-T scores were analyzed using logistic regression.</p><p><strong>Results: </strong>A total of 490 patients completed the questionnaire between January 2023 and June 2024. Of these, 47 patients (9.59%) had scores exceeding the threshold in at least one SPARC-T domain. The most frequently reported concerns were depression (6.94%) and spiritual distress (5.51%). Multivariate logistic regression revealed that female patients (odds ratio = 2.21, 95% confidence interval: 1.09 to 4.47) and individuals from low-income groups (odds ratio = 5.56, 95% confidence interval: 1.48 to 20.95) were significantly more likely to report higher distress scores.</p><p><strong>Conclusions: </strong>Many patients with kidney disease, particularly women and those belonging to low-income groups, experienced unmet needs that extended beyond physical symptoms. Routine use of the SPARC-T is recommended to support patient-centered care by addressing psychological, spiritual, and social concerns alongside clinical management.</p>","PeriodicalId":16542,"journal":{"name":"Journal of Nephrology","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2025-09-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145192060","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":"Comprehensive analysis of sparsentan-related adverse events: latest insights from VigiAccess and FAERS.","authors":"Hongxuan Fan, Yafen Yang, Jiahui Li, Zhuolin Huang, Boda Zhou","doi":"10.1007/s40620-025-02412-4","DOIUrl":"https://doi.org/10.1007/s40620-025-02412-4","url":null,"abstract":"<p><strong>Background: </strong>Sparsentan represents a major advancement in the treatment of proteinuric kidney diseases, offering a promising option to improve patient outcomes and slow disease progression. Evaluation of its safety profile is essential to support its long-term integration into clinical practice.</p><p><strong>Methods: </strong>This study employed a retrospective descriptive analysis combined with four advanced statistical methods to evaluate adverse events related to sparsentan. The data, sourced from the WHO's VigiAccess database, was queried in November 2024 to retrieve adverse event reports associated with sparsentan. The Food and Drug Administration Adverse Event Reporting System (FAERS) database has also been utilized to conduct an in-depth analysis of adverse events associated with sparsentan.</p><p><strong>Results: </strong>A total of 1476 adverse events associated with sparsentan were reported in VigiAccess until the end of November 2024. The analysis revealed that the ten most frequently reported adverse events included dizziness, fatigue, product use in unapproved indication, hypotension, nausea, peripheral swelling, headache, blood pressure decrease, pruritus, wrong technique in product usage process.</p><p><strong>Conclusions: </strong>While the majority of adverse events were mild and self-limiting, there were instances of severe events that could have led to hospitalization or even fatalities. It is crucial to actively prioritize primary safety research on sparsentan, with a particular focus on cohort event monitoring, to better understand and establish causal relationships between the treatment and reported adverse events.</p>","PeriodicalId":16542,"journal":{"name":"Journal of Nephrology","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2025-09-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145192035","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":"Membranous nephropathy preceding Bing-Neel syndrome: successful treatment with tirabrutinib.","authors":"Kanako Watanabe-Kusunoki, Daigo Nakazawa, Hiroyuki Ohigashi, Takahiro Tsuji, Tatsuya Atsumi","doi":"10.1007/s40620-025-02401-7","DOIUrl":"https://doi.org/10.1007/s40620-025-02401-7","url":null,"abstract":"<p><p>We report a unique case of membranous nephropathy and tubulointerstitial nephritis preceding neurological manifestations of Bing-Neel syndrome, a rare variant of Waldenström macroglobulinemia. A 46-year-old man presented with nephrotic syndrome, and kidney biopsy revealed membranous nephropathy with phospholipase A2 receptor positivity and IgG4 predominance. Despite initial partial remission with corticosteroids and cyclosporine, neurological symptoms emerged alongside nephrotic syndrome relapse two years after onset. Diagnostic workup revealed serum monoclonal IgM kappa, abnormal lymphoplasmacytic cells in bone marrow and cerebrospinal fluid, and MYD88 L265P mutation in the latter, confirming Bing-Neel syndrome. Immunostaining of the initial kidney biopsy demonstrated IgM-kappa-positive lymphoplasmacytic infiltration consistent with the monoclonal protein. After bendamustine-rituximab therapy showed partial effectiveness, tirabrutinib, a Bruton tyrosine kinase inhibitor, achieved complete remission of both renal and neurological manifestations with sustained response over three years. Immunofluorescence staining revealed increased nuclear factor (NF)-κB and STAT3 expression in infiltrating immune cells and elevated NF-κB in surrounding tubular epithelial cells, suggesting activation of these pathways downstream of the MYD88 mutation in kidney pathology. Our findings suggest a potential contribution of Bruton's tyrosine kinase signaling to both kidney and neurological pathology in Bing-Neel syndrome, with tirabrutinib achieving clinical improvement in both organ manifestations.</p>","PeriodicalId":16542,"journal":{"name":"Journal of Nephrology","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2025-09-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145149485","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":"Exploring vascular access survival in prevalent thrice-weekly in-centre nocturnal haemodialysis patients.","authors":"Katherine L Hull, Ann Bugeja, Matthew P M Graham-Brown, Lindsay Reid, Aiden J Smith, Brigit C van Jaarsveld, James O Burton","doi":"10.1007/s40620-025-02431-1","DOIUrl":"https://doi.org/10.1007/s40620-025-02431-1","url":null,"abstract":"<p><strong>Background: </strong>This study explores vascular access complications in patients established on in-centre nocturnal haemodialysis (INHD) compared to conventional haemodialysis.</p><p><strong>Methods: </strong>This was a retrospective cohort study; patients acted as their own control. Data were collected from three centres. Adults established on INHD (intervention) preceded by usual daytime haemodialysis (control) were eligible. Data were collected between 01/01/2009 and 12/31/2021. The data collection period was up to 12 months for both control and intervention periods. The primary outcome was a composite of outcomes related to vascular access complications: hospitalisation, intervention, change in vascular access modality, change in dialysis modality and death. The primary outcome was evaluated by time-to-event rate in days using Kaplan-Meier plots. Statistical significance was accepted at a P < 0.05.</p><p><strong>Results: </strong>One hundred forty-five individuals were included: median age was 52.0 years (IQR 36.0-65.0), 71.0% (n = 103) were male, and 57.2% (n = 83) were White. The primary outcome occurred in 24.1% (n = 35) during the intervention and in 25.5% (n = 37) during the control period (P = 0.875). The 12-month vascular access survival probability was 73.4% (95%CI 65.8-81.0%) for the intervention and 70.6% (95%CI 62.4%-78.8%) for the control period. During the intervention period, arteriovenous grafts were associated with lower vascular access survival (P < 0.001). Regular vitamin K antagonist was associated with a lower 12-month vascular access survival for both the intervention (P = 0.044) and the control periods (P < 0.001).</p><p><strong>Conclusion: </strong>There does not appear to be an increased risk to vascular access events for INHD compared to daytime haemodialysis. Vascular access type and regular anticoagulation were associated with a reduced vascular access survival probability.</p>","PeriodicalId":16542,"journal":{"name":"Journal of Nephrology","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2025-09-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145149514","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":"Differences in chronic kidney disease management based on identification and diagnosis in a population-based observational study.","authors":"Michael Olszewski, Karl Bjurström, Markus Lingman, Dan Henrohn, Poyan Shojaiyan, Magnus Garell, Björn Agvall","doi":"10.1007/s40620-025-02414-2","DOIUrl":"https://doi.org/10.1007/s40620-025-02414-2","url":null,"abstract":"<p><strong>Background: </strong>Chronic kidney disease (CKD) affects 6-10% of adults and often remains undiagnosed until advanced stages, leading to inadequate management. This study compared diagnosed, proxy-diagnosed, and undiagnosed CKD patients regarding prevalence, clinical assessment, nephroprotective treatment, healthcare utilization, and mortality.</p><p><strong>Methods: </strong>This retrospective observational study analyzed Region Halland's healthcare data for adults meeting KDIGO CKD-confirmed criteria for the year 2019. Patients were categorized as diagnosed CKD (ICD-coded), proxy-diagnosed CKD (CKD-related diagnoses), or undiagnosed CKD (meeting CKD criteria without an ICD CKD diagnosis).</p><p><strong>Results: </strong>Of 20,488 CKD patients, 21% had diagnosed CKD, 18% proxy-diagnosed CKD, and 61% undiagnosed CKD. Mean ages were 76.4, 62.4, and 81.8 years, respectively (p < 0.001). Blood pressure follow-up was carried out in diagnosed CKD (88%) versus 67% and 80% in the proxy-diagnosed and undiagnosed groups. eGFR was tested in 66% overall (73% diagnosed, 53% proxy-diagnosed, 66% undiagnosed), while urine albumin-to-creatinine ratio (UACR) testing was performed in 27% overall (50%, 20%, and 21%, respectively). Renin-angiotensin system inhibitors were prescribed to 45% overall (51%, 28%, and 47%, respectively). The adjusted hospitalization risk was 2.71 (CI: 2.59-2.84) in diagnosed CKD and 1.38 (CI: 1.31-1.46) in proxy-diagnosed CKD. Adjusted all-cause mortality hazard ratios were 2.22 (CI: 1.95-2.52) and 1.31 (CI: 1.08-1.60), respectively. Stratified sensitivity analyses by CKD stage confirmed these associations, though the strength varied.</p><p><strong>Conclusions: </strong>Patients with complex comorbidities, more advanced CKD, and frequent hospitalizations are more likely to be diagnosed with CKD and receive better follow-up care. Proxy-diagnosed CKD was common and associated with suboptimal management. These findings emphasize the need for consistent and accurate CKD identification to improve outcomes and optimize care.</p>","PeriodicalId":16542,"journal":{"name":"Journal of Nephrology","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2025-09-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145176019","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":"Iptacopan/LNP023 and rituximab as rescue therapy in a patient with systemic lupus erythematosus-associated atypical haemolytic uraemic syndrome.","authors":"Francesco Benvenuti, Marta Proglio, Manuela Rigno, David Sorbo","doi":"10.1007/s40620-025-02425-z","DOIUrl":"https://doi.org/10.1007/s40620-025-02425-z","url":null,"abstract":"<p><p>Systemic lupus erythematosus (SLE) is rarely associated with atypical haemolytic uraemic syndrome, leading to a poor prognosis with multi-system involvement. The pathogenesis seems to be driven by hyperactivation of alternate pathways of complement cascade and patients should be treated with prompt administration of complement inhibitors and immunosuppressive therapy. Iptacopan/LNP023, a novel, orally administered C3 convertase inhibitor that has shown efficacy in C3 glomerulopathy and paroxysmal nocturnal haemoglobinuria, is going to be tested in patients with atypical haemolytic syndrome. We present a case of a 59 year-old patient with mild systemic lupus erythematosus developing atypical haemolytic uraemic syndrome with renal, myocardial and neurological involvement, which was refractory to synthetic immunosuppressive, eculizumab and plasma exchange therapies. The syndrome rapidly subsided after iptacopan/LPN023 administration associated with a single rituximab cycle, followed by tapering of glucocorticoids, immunosuppressive therapy and plasma exchange. Unfortunately, kidney failure occurred requiring twice weekly haemodialysis sessions. To the best of our knowledge, this is the first case of SLE-aHUS successfully treated with iptacopan/LPN023, in association with standard SLE immunosuppression.</p>","PeriodicalId":16542,"journal":{"name":"Journal of Nephrology","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2025-09-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145137824","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":"Effects of sodium bicarbonate therapy on cognitive function in chronic kidney disease (ancillary study): a randomized controlled trial.","authors":"Angelina Dixon, Zhiying You, Rebecca Richey, Seth Furgeson, Jessica Kendrick","doi":"10.1007/s40620-025-02429-9","DOIUrl":"https://doi.org/10.1007/s40620-025-02429-9","url":null,"abstract":"","PeriodicalId":16542,"journal":{"name":"Journal of Nephrology","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2025-09-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145137815","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":"Socioeconomic factors and access to home dialysis and early kidney transplantation across Europe.","authors":"Jan Dominik Kampmann, Vianda S Stel, Leah Sejrup Christensen, Anneke Kramer, Patrik Finne","doi":"10.1007/s40620-025-02424-0","DOIUrl":"https://doi.org/10.1007/s40620-025-02424-0","url":null,"abstract":"","PeriodicalId":16542,"journal":{"name":"Journal of Nephrology","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2025-09-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145092072","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":"Medullary sponge kidney: in-depth phenotyping for a better understanding of functional and structural abnormalities.","authors":"Corentin Tournebize, Aurélie De Mul, Nadia Abid, Aurélie Portefaix, Sophie Pacaud, Maxime Schleef, Laurence Derain-Dubourg, Olivier Rouviere, Sandrine Lemoine","doi":"10.1007/s40620-025-02413-3","DOIUrl":"https://doi.org/10.1007/s40620-025-02413-3","url":null,"abstract":"<p><strong>Background: </strong>Medullary sponge kidney is an entity characterized by pre-calyceal dilatation of the renal tubules, whose pathophysiology is unknown. Tubular anomalies have been described, suggesting impaired medullary function. To better characterize these patients, tools for assessing medullary function and structure are needed. The latter can be evaluated with functional magnetic resonance imaging (fMRI), using blood-oxygen-level-dependent imaging, which quantifies tissue oxygenation, and diffusion-weighted-imaging and T1-mapping sequences which allow fibrosis assessment. The aim of this study was to deeply phenotype medullary sponge kidney patients.</p><p><strong>Methods: </strong>We carried out fMRI, measured glomerular filtration rate (mGFR) by iohexol clearance, and metabolic assessment of urolithiasis in patients with medullary sponge kidney and in healthy controls. The primary endpoint was the comparison of R2*, inversely proportional to oxygen content, measured by blood-oxygen-level-dependent MRI. Secondary endpoints included comparison of T1 and apparent diffusion coefficient, comparison of GFR between medullary sponge kidney patients and controls, and the correlations between fMRI, GFR and biological abnormalities in medullary sponge kidney.</p><p><strong>Results: </strong>Twenty patients with medullary sponge kidney were included, as well as 13 controls. We observed a higher R2* cortex-to-medulla ratio in medullary sponge kidney patients compared to controls (0.60 vs. 0.55; p = 0.04). No difference was observed for T1 and apparent diffusion coefficient cortex-to-medulla ratio. mGFR was significantly lower in medullary sponge kidney patients (90 ml/min/1.73m² vs 78 ml/min/1.73m²; p = 0.008) although estimated GFR did not differ. Medullary cysts were visible on MRI in 60% of medullary sponge kidney patients.</p><p><strong>Conclusion: </strong>We identified impaired renal oxygenation in patients with medullary sponge kidney. We did not find evidence of kidney fibrosis in medullary sponge kidney. GFR estimation was not accurate in medullary sponge kidney patients. MRI can visualize medullary cystic appearance of medullary sponge kidney.</p>","PeriodicalId":16542,"journal":{"name":"Journal of Nephrology","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2025-09-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145092067","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}