Journal of Nephrology最新文献

{"title":"Nephrology in space: from fluid conservation to cosmic kidney disease.","authors":"Marco Pizzarelli, Francesco Pizzarelli","doi":"10.1093/joneph/aajag163","DOIUrl":"https://doi.org/10.1093/joneph/aajag163","url":null,"abstract":"","PeriodicalId":16542,"journal":{"name":"Journal of Nephrology","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2026-09-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148881021","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-term outcome of pediatric anti-complement factor H antibody-associated atypical hemolytic uremic syndrome receiving abbreviated plasma exchange: a retrospective study from a single center.","authors":"Pujitha Vallabhaneni, Lesa Dawman, Amit Rawat, Ritambhra Nada, Prateek Bhatia, Thakurvir Singh, Aravind Sekar, Karalanglin Tiewsoh","doi":"10.1093/joneph/aajag189","DOIUrl":"https://doi.org/10.1093/joneph/aajag189","url":null,"abstract":"<p><strong>Background: </strong>Anti-complement factor H antibody- (anti-CFH Ab) associated atypical hemolytic uremic syndrome (aHUS) is a known cause of pediatric complement-mediated thrombotic microangiopathy. In our center, plasma exchange (PEX) until remission paired with immunosuppression continues to be the mainstay of therapy, due to limited access to eculizumab. We wanted to study the long-term outcome of this cohort.</p><p><strong>Methods: </strong>We conducted a retrospective cohort study of children (<12 years) with Anti-CFH Ab-associated aHUS admitted between January 2017 and December 2024 at our center. Clinical, laboratory, treatment, and outcome data were retrieved from medical records. The primary outcome was renal recovery, defined as eGFR >90 mL/min/1.73 m2 with normal blood pressure, no significant proteinuria, and absence of hematuria at last follow-up or at 5 years.</p><p><strong>Results: </strong>Of 66 records screened, 40 children were eligible. Median age at presentation was 7 years (interquartile range [IQR] 5,8). Median anti-CFH Ab titer was 306 AU/mL (IQR 214-426). Low C3 was noted in 55% of subjects. All patients received PEX (median 11 cycles; IQR 7.25, 20) and immunosuppression. Median follow-up was 24 months (IQR 7-53). At last follow-up, 21 (52.5%) achieved complete renal recovery, while 19 (47.5%) had CKD stage 2-4. Relapse incidence was 7.1 per 100 person-years, usually within 6 months of onset. No baseline clinical, laboratory, or treatment variable except duration of dialysis independently predicted renal recovery. Persistent proteinuria was observed up to 6 months but subsequently improved.</p><p><strong>Conclusions: </strong>PEX paired with immunosuppression remains effective for anti-CFH Ab-associated aHUS, but further studies are needed to refine protocols and evaluate complement inhibitors for improved long-term outcomes.</p>","PeriodicalId":16542,"journal":{"name":"Journal of Nephrology","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148865537","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":"IMPROVE kidney care: perspectives from marginalised people with CKD and risk factors for CKD on access to, and experience of, kidney care services: a cross-sector collaborative exploration, employing qualitative approaches.","authors":"Rebekah Cheung Judge, Roseline Agyekum, Anjolaoluwa Awe, Muhammad Bojang, Kate Bramham, Stephanie Hanson, Imani Henry Bailey, Lina Johansson, Adam Kamenetzky, Ritika Karamchandani, Jordanna Nunes, Nupur Yogarajah, Shone Surendran, Nadine Fontaine-Palmer","doi":"10.1093/joneph/aajag052","DOIUrl":"https://doi.org/10.1093/joneph/aajag052","url":null,"abstract":"<p><strong>Background: </strong>Access to, and experience of, chronic kidney disease (CKD) care is inequitable-with barriers to accessing quality care for marginalised groups. We conducted an exploratory study employing qualitative approaches to understand the factors that influence access to, and experience of, healthcare services for marginalised people with CKD and at risk of CKD.</p><p><strong>Methods: </strong>An exploratory study employing qualitative approaches was conducted as a cross-sector collaboration between kidney care services and an activist, antiracist community-based research and social justice organisation (Mabadiliko Community Interest Company (CIC)). Two groups were recruited: 1) those with risk factors for CKD or early-stage CKD, and 2) people who presented late to kidney care services. Semi-structured interviews were co-designed with people with lived experience and conducted by Mabadiliko CIC. Thematic analysis was undertaken, with themes refined by participants.</p><p><strong>Results: </strong>Twenty interviews were undertaken with a diverse cohort of participants. Knowledge and awareness of CKD was limited, and compounded by a lack of delivery of accessible, culturally congruent information. Significant barriers to accessing kidney care exist for marginalised people, including people who are from global majority ethnic backgrounds, Disabled people, and/or people experiencing material hardship. These barriers are compounded by interpersonal discrimination and paternalistic power dynamics within healthcare interactions.</p><p><strong>Conclusion: </strong>This study captures the experiences of marginalised people at different stages of their journey with CKD, in accessing and engaging with kidney care services. Participants faced a complex array of challenges, highlighting opportunities for multi-level intervention. We outline recommendations to address these issues, co-developed with participants.</p>","PeriodicalId":16542,"journal":{"name":"Journal of Nephrology","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148865477","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 a telehealth-supported home-based exercise program on functional capacity, sarcopenia risk, and patient-reported outcomes in non-dialysis stage 4-5 chronic kidney disease.","authors":"Büşra Yıldırım Uz, Gökçe Yağmur Güneş Gencer, Feyza Bora","doi":"10.1093/joneph/aajag172","DOIUrl":"https://doi.org/10.1093/joneph/aajag172","url":null,"abstract":"<p><strong>Background: </strong>Evidence on the effects of 12-week telehealth-supported home-based exercise in advanced non-dialysis chronic kidney disease (CKD) remains limited. This study evaluated its effects on functional capacity, sarcopenia risk, and patient-reported outcomes in patients with stage 4-5 CKD.</p><p><strong>Methods: </strong>In this prospective, parallel-group randomized controlled trial (October 2024-July 2025), 54 non-dialysis patients aged ≥45 years with stage 4-5 CKD were randomized 1:1 to a 12-week telehealth-supported home-based exercise program plus usual nephrology care or usual care alone. Complete-case analyses included 51 participants (exercise group: n = 25 [stage 4, n = 21; stage 5, n = 4]; control group: n = 26 [stage 4, n = 20; stage 5, n = 6]). The primary outcome was change in 6-minute walk test distance. Secondary outcomes included physical performance, sarcopenia risk, fatigue, mood symptoms, sleep quality, and health-related quality of life.</p><p><strong>Results: </strong>Fifty-one participants completed the study (exercise group, n = 25; control group, n = 26). Compared with controls, the exercise group showed a greater improvement in 6-minute walk test distance (+29.41 ± 35.63 m vs -19.27 ± 33.62 m; P < .001), which remained significant after covariate adjustment. Significant improvements were also observed in sarcopenia risk, fatigue, mood symptoms, sleep quality, physical performance, and quality of life. Among intervention participants, 40% used the web-based platform and 60% received video-based support.</p><p><strong>Conclusions: </strong>A 12-week telehealth-supported home-based exercise program significantly improved functional capacity and multiple patient-reported outcomes in patients with non-dialysis stage 4-5 CKD.</p>","PeriodicalId":16542,"journal":{"name":"Journal of Nephrology","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2026-08-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148860519","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":"Burden of diagnosed and undetected hyperkalemia in Spain: Clinical characteristics, management, healthcare resource utilization, and costs.","authors":"José Luis Gorriz, Roberto Alcázar, Patricia de Sequera, Enrique Morales, Miquel Blasco, Marco Montomoli, Alberto Ortiz","doi":"10.1093/joneph/aajag234","DOIUrl":"https://doi.org/10.1093/joneph/aajag234","url":null,"abstract":"<p><strong>Background: </strong>Management of hyperkalemia (HK) is challenging and suboptimal. This study analyzed the prevalence of HK and undetected HK, as well as management, clinical outcomes, healthcare resource utilization (HCRU), and costs.</p><p><strong>Methods: </strong>Observational study comprising cross-sectional and retrospective analyses using secondary data from electronic health records (608,514 adults) from BIG-PAC database. The population comprised adults (n=7006) with a diagnostic code for HK, potassium >5.0 mmol/L and/or treatment for HK from January 2013 to December 2020, and follow-up ≥2 years. Index date was defined as the first HK event and undetected HK when diagnosis and/or treatment were not observed despite potassium >5.0 mmol/L at index date.</p><p><strong>Results: </strong>The prevalence of HK was 1.15%, of which 0.77% was undetected. Patients with undetected HK (vs diagnosed HK) were younger and less frequently had atherosclerotic cardiovascular disease (20.0% vs 24.1%; p<0.001) and chronic kidney disease (10.8% vs 42.4%; p<0.001). In undetected HK, 58.7% had mild, 35.8% moderate, and 5.4% severe HK. Regarding outcomes, 10.9% and 18.8% (p<0.001) died, and annual hospitalization rates were 15.6% and 33.1% (p<0.001), respectively. Outcomes were more common in diagnosed HK vs undetected HK. Annual costs per patient were higher in diagnosed HK vs undetected HK (€7615 [6381] vs €5005 [4619], USD9241.6 [7743.9] vs USD6074.1 [5605.6]; p<0.001).</p><p><strong>Conclusions: </strong>Around 1% of patients in Spain had HK; of these, two-thirds were undetected, including patients with moderate-to-severe HK. Despite lower comorbidity burden, these patients experienced substantial clinical events and suboptimal use of cardio-renal protective therapies.</p>","PeriodicalId":16542,"journal":{"name":"Journal of Nephrology","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2026-08-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148850867","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":"Renal denervation in patients with uncontrolled hypertension and renal artery stenosis assessed by invasive physiology.","authors":"Domenico Tavella, Gabriele Pesarini, Alessia Gambaro, Federico Vigo, Enrico Galuppi, Matteo Casal, Aurora Trevisanello, Laura Trento, Matteo Piscopo, Alessandro Sarai, Giulio Previdi, Verdiana Galli, Concetta Gangemi, Valeria Ferrero, Roberto Scarsini, Simone Fezzi, Giovanni Gambaro, Manuel Ferraro, Flavio L Ribichini","doi":"10.1093/joneph/aajag056","DOIUrl":"10.1093/joneph/aajag056","url":null,"abstract":"<p><strong>Background: </strong>Renal artery stenosis is usually considered as a contraindication for renal denervation. Whether renal denervation can be safe and effective in the presence of a renal artery stenosis once secondary renovascular hypertension has been ruled-out has never been investigated to date. We report our experience with radiofrequency-based renal denervation in patients with moderate-to-severe renal artery stenosis.</p><p><strong>Methods: </strong>Patients with uncontrolled hypertension eligible for renal denervation referred to our Center were evaluated for this study. Renal artery stenosis >50% as evaluated by quantitative angiography analysis underwent physiological evaluation (ratio of the distal pressure to aortic pressure: Pd/Pa). Those with Pd/Pa ≥ 0.90 underwent renal denervation, as did patients with normal kidney arteries. Patients with Pd/Pa < 0.90 were treated with renal artery stenting. Office systolic and diastolic blood pressure (BP) and estimated glomerular filtration rate (eGFR) were assessed and compared among patients with normal renal arteries (Group 1), those with non-hemodynamically significant renal artery stenosis (Group 2), and patients undergoing renal artery stenting (Group 3).</p><p><strong>Results: </strong>All patients with at least one-year of follow-up were included (n = 139). Similar systolic BP reduction was reported in Groups 1 (n = 114) and 2 (n = 25) : -5.76 ± 3.50, -5.86 ± 3.74, -14.46 ± 3.6, -22.79 ± 27.12 mmHg and -14.93 ± 26.53, -11.29 ± 27.32, -17.38 ± 22.72 and -19.21 ± 23.70 mmHg at 3, 6, 12, and 24 months, respectively. Kidney function remained stable. The patients in group 3 (n = 9) showed significant BP lowering after renal artery stenting.</p><p><strong>Conclusions: </strong>Physiologic assessment of renal artery stenosis distinguished hemodynamically relevant lesions, responsible for renovascular hypertension and suitable to undergo revascularization, from lesions without significant hemodynamic impact, which may be encountered in patients with essential hypertension. An accurate algorithm for stenosis assessment allows the selection of the most appropriate treatment (ie, renal artery stenting or renal denervation).</p>","PeriodicalId":16542,"journal":{"name":"Journal of Nephrology","volume":" ","pages":"959-966"},"PeriodicalIF":2.6,"publicationDate":"2026-08-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148163468","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":"Beyond the 10-year barrier: ultrastructural insights into long-term tunneled catheter function from calcified specimens-a report on 4 cases.","authors":"Yueqi Cao, Bo Li, Haiyan Chen, Ruining Zhang, Lihua Wang","doi":"10.1093/joneph/aajag016","DOIUrl":"10.1093/joneph/aajag016","url":null,"abstract":"<p><p>Tunneled-cuffed catheters (TCCs) remain a primary vascular access modality for patients requiring maintenance hemodialysis, yet catheter dysfunction is a frequent and challenging complication. Although the factors contributing to catheter dysfunction are well recognized, the ultrastructural changes of fibrin sheaths in long-term indwelling catheters have not been systematically documented in the literature. Herein, we present 4 cases of malfunctioning subcutaneous TCCs of varying dwelling durations. Catheter exchange was performed under digital subtraction angiography guidance, and fibrin sheath specimens were collected for analysis. Ultrastructural assessment was conducted via scanning electron microscopy, supplemented with energy-dispersive X-ray spectroscopy and hematoxylin-eosin staining. Fibrous tissue hyperplasia was observed in all 4 specimens. Specimens from the 2 catheters with longer dwelling durations exhibited myofiber degeneration within the fibrin sheaths, accompanied by tissue calcification. In contrast, fibrin sheaths from the 2 catheters with shorter dwelling durations were predominantly characterized by exudates and inflammatory cellular infiltration. Fibrin sheath hyperplasia is a consistent pathological feature in long-term hemodialysis catheters. These findings suggest that prolonged catheter dwelling time correlates with progressively more severe pathological changes in the fibrin sheath.</p>","PeriodicalId":16542,"journal":{"name":"Journal of Nephrology","volume":" ","pages":"1052-1057"},"PeriodicalIF":2.6,"publicationDate":"2026-08-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148258507","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 rare cause of kidney disease in two siblings: transaldolase deficiency.","authors":"Lala Gurbanova, Bahar Büyükkaragöz, Buket Dalgic","doi":"10.1093/joneph/aajag030","DOIUrl":"10.1093/joneph/aajag030","url":null,"abstract":"<p><p>Transaldolase is a key enzyme in the pentose phosphate pathway, an alternative pathway for glucose metabolism. Transaldolase deficiency leads to the accumulation of metabolites such as polyols and seven-carbon sugars, particularly in urine. Transaldolase deficiency is a rare disorder with multisystem involvement, including liver dysfunction, hepatosplenomegaly, anemia, thrombocytopenia, endocrine abnormalities and dysmorphic features. We now report on two siblings with kidney involvement due to transaldolase deficiency. Kidney dysfunction is common in transaldolase deficiency and may progress to chronic kidney disease (CKD). The disease may have an early onset, but it can also progress without showing any sign of kidney impairment until adulthood. Transaldolase deficiency should be considered among the causes of proteinuria and CKD of unknown origin, and these patients should be carefully monitored for kidney function. Although the disease has been generally reported to manifest with tubular dysfunction, one of our patients had both tubular and glomerular involvement, which started at an earlier age than in her younger sister.</p>","PeriodicalId":16542,"journal":{"name":"Journal of Nephrology","volume":" ","pages":"1058-1062"},"PeriodicalIF":2.6,"publicationDate":"2026-08-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148338953","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":"Effect of hemodialysis and fluid removal on lung function in patients with kidney failure-a pre-post study.","authors":"Ana Carolina Lúcio Garcia Pires, Augusto Homem Mansur, Gustavo Dutra, Gabryela Mariane Costa, Milene Rossi, Bruna Godinho Corrêa, Jessica Puchalski Trettim, Eduardo Ribes Kohn, Maristela Bohlke","doi":"10.1093/joneph/aajag057","DOIUrl":"10.1093/joneph/aajag057","url":null,"abstract":"<p><strong>Background: </strong>Fluid overload may worsen pulmonary mechanics in patients with kidney failure. We evaluated the acute impact of a single hemodialysis (HD) session on lung volumes, flows, respiratory muscle strength, and the prevalence of preserved ratio impaired spirometry.</p><p><strong>Methods: </strong>A pre-post observational study was conducted at a university hospital dialysis unit. Forced expiratory volume in one second (FEV1), peak expiratory flow, and FEV1 to forced vital capacity (FVC) ratio (FEV1/FVC) were measured by spirometry, while maximum inspiratory pressure and maximum expiratory pressure were measured by manovacumetry. Evaluations were performed before and after the HD session. The association between changes in respiratory volumes and pressures and the volume of ultrafiltration (UF) removed during the HD session was evaluated using adjusted linear regression.</p><p><strong>Results: </strong>The study included 104 patients (57% male), with a mean age of 54.7 (15.8) years. Both maximum expiratory pressure (71.3 ± 39.6 to 79.7 ± 35.4% predicted) and maximum inspiratory pressure (64.5 ± 29.0 to 69.9 ± 32.1% predicted) significantly increased from before to after the HD session (P < .001 for both). The ratio (FEV1/FVC) was 80.1 (77.1 to 83.14) % before and 83.5 (80.7 to 86.3) % after the HD session (P = .02). The proportion of participants meeting preserved ratio impaired spirometry criteria significantly decreased from 56.0% to 43.5% after HD (P < .001). The UF volume was positively correlated with changes in % FEV1/FVC ratio (β = 1.84, P = .04) and FEV1 (β = .09, P = .006).</p><p><strong>Conclusion: </strong>HD was associated with acute improvements in respiratory muscle strength and in spirometry pattern (reduction in preserved ratio impaired spirometry prevalence). The magnitude of improvement in FEV1/FVC and FEV1 correlated with UF volume, suggesting a role of fluid removal. Further studies should confirm these findings longitudinally and investigate clinical outcomes.</p>","PeriodicalId":16542,"journal":{"name":"Journal of Nephrology","volume":" ","pages":"967-973"},"PeriodicalIF":2.6,"publicationDate":"2026-08-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148411653","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":"Mainstreaming of clinical genetic testing in nephrology: special considerations in resource-limited settings.","authors":"Neveen A Soliman","doi":"10.1093/joneph/aajag109","DOIUrl":"10.1093/joneph/aajag109","url":null,"abstract":"","PeriodicalId":16542,"journal":{"name":"Journal of Nephrology","volume":" ","pages":"1068-1070"},"PeriodicalIF":2.6,"publicationDate":"2026-08-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148603849","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}