Journal of Nephrology最新文献

{"title":"Potentially reversible severe cardiac involvement in thrombotic microangiopathies with malignant hypertension.","authors":"Marco Allinovi, Silvia Menale, Valentina Querin, Leonardo Caroti, Giulia Antognoli, Calogero Lino Cirami, Niccolò Marchionni, Valentina Scheggi","doi":"10.1007/s40620-025-02334-1","DOIUrl":"https://doi.org/10.1007/s40620-025-02334-1","url":null,"abstract":"<p><strong>Background: </strong>Thrombotic microangiopathy (TMA) represents a pathological response to endothelial damage, caused by genetic or acquired factors. It includes conditions like thrombotic thrombocytopenic purpura, atypical hemolytic uremic syndrome (aHUS), and secondary TMAs. Malignant hypertension can both result from and trigger TMA. This study aims to identify risk factors for severe cardiac involvement, defined as a left ventricular ejection fraction ≤ 50%, in patients with concomitant TMA and malignant hypertension, and to investigate the evolution of cardiac anomalies post-TMA remission.</p><p><strong>Methods: </strong>We retrospectively collected data of 33 patients with TMA and malignant hypertension, admitted to our hospital between January 2014 and December 2023. Patients were followed up to monitor kidney function, blood pressure, and hematological parameters, and those with severe cardiac involvement at admission underwent repeated echocardiograms. Genetic testing for complement factors was performed to identify aHUS cases.</p><p><strong>Results: </strong>Severe cardiac involvement was diagnosed in 9 patients, all of whom exhibited left ventricular hypertrophy and varying degrees of diastolic dysfunction and valve regurgitation. Patients with severe cardiac involvement had significantly higher interventricular septal and posterior wall thickness at admission. Over a mean follow up of 42 months, all patients with severe cardiac involvement showed recovery of left ventricular systolic function. Patients with severe cardiac involvement had a higher incidence of respiratory failure [5/9 (56%) vs 2/24 (8%) patients, p = 0.003] and kidney failure [7/9 (78%) vs 8/24 (33%) patients, p = 0.022].</p><p><strong>Conclusions: </strong>Severe cardiac involvement in TMA associated with malignant hypertension is common but largely reversible. Early identification and tailored treatment can lead to improved outcomes. This study highlights the importance of comprehensive cardiac assessment in managing TMA patients with malignant hypertension.</p>","PeriodicalId":16542,"journal":{"name":"Journal of Nephrology","volume":" ","pages":""},"PeriodicalIF":2.7,"publicationDate":"2025-07-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144637309","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 hemolytic uremic syndrome is not always complement mediated: lesson for the clinical nephrologist.","authors":"Andrea Spasiano, Francesca Menni, Martina Ambrogio, Alessandro Naticchia, Nicola Panocchia, Pietro Manuel Ferraro, Gianluigi Ardissino","doi":"10.1007/s40620-025-02359-6","DOIUrl":"https://doi.org/10.1007/s40620-025-02359-6","url":null,"abstract":"","PeriodicalId":16542,"journal":{"name":"Journal of Nephrology","volume":" ","pages":""},"PeriodicalIF":2.7,"publicationDate":"2025-07-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144626593","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":"Alcohol consumption and incidence of decline in glomerular filtration rate and of proteinuria: the Osaka Kenko Innovation (TOKI) study.","authors":"Yuko Nakamura, Naoko Otsuki, Qinyan Li, Maki Shinzawa, Isao Matsui, Miyae Yamakawa, Asuka Oyama, Hiroshi Toki, Ryohei Yamamoto","doi":"10.1007/s40620-025-02339-w","DOIUrl":"https://doi.org/10.1007/s40620-025-02339-w","url":null,"abstract":"<p><strong>Background: </strong>Although excessive alcohol consumption is a critical factor for non-communicable diseases, its clinical relevance to chronic kidney disease is controversial.</p><p><strong>Methods: </strong>This retrospective cohort study, including 80,765 men and 88,507 women aged 40-74 years who underwent annual health checkups in Japan between April 2012 and March 2017, assessed a dose-dependent association between alcohol consumption (rare, occasional, and daily drinkers with ≤ 19, 20-39, 40-59, and ≥ 60 g/day) and incidence of ≥ 30% decline in estimated glomerular filtration rate (eGFR), eGFR < 60 ml/min/1.73 m² and presence of proteinuria (dipstick urinary protein ≥ 1 +), using Cox proportional hazards models adjusted for clinically relevant factors.</p><p><strong>Results: </strong>The incidence of ≥ 30% eGFR decline was observed in 1231 (1.5%) men and 1291 (1.5%) women during the median observation period of 2.8 and 2.9 years, respectively. In men, daily drinkers consuming ≥ 40 g/day of ethanol were at significantly high risk for ≥ 30% eGFR decline (adjusted hazard ratio [95% confidence interval] of rare, occasional, and daily drinkers with ≤ 19, 20-39, 40-59, and ≥ 60 g/day: 1.00 [reference], 1.05 [0.87, 1.27], 0.99 [0.80, 1.21], 1.05 [0.88, 1.26], 1.23 [1.01, 1.51], 1.61 [1.22, 2.11], respectively). Similar dose-dependent associations with incidence of eGFR < 60 ml/min/1.73 m² and proteinuria were observed in men. Contrary to men, alcohol consumption was not associated with eGFR decline and proteinuria in women.</p><p><strong>Conclusion: </strong>Men with alcohol consumption ≥ 40 g/day were at a high risk of eGFR decline and development of proteinuria.</p>","PeriodicalId":16542,"journal":{"name":"Journal of Nephrology","volume":" ","pages":""},"PeriodicalIF":2.7,"publicationDate":"2025-07-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144618633","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":"Impact of SGLT2i on bone health in patients with lupus nephritis: randomized double blinded placebo-controlled clinical trial.","authors":"Mohamed Hosney Badawi, Eman Nagy, Ehab Wahba Wafa, Amr El-Husseini, Nourelsabah Mohamed, Mohamed Abo El-Ghar, Wael Ibrahim Mortada, Kareem Ahmed Nabieh, Mohamed Abd El-Kader Sobh","doi":"10.1007/s40620-025-02351-0","DOIUrl":"https://doi.org/10.1007/s40620-025-02351-0","url":null,"abstract":"<p><strong>Background: </strong>Patients with systemic lupus erythematosus (SLE) commonly experience osteoporosis and are at increased risk of bone fractures. Sodium-glucose co-transporter 2 inhibitors (SGLT2i) have shown promising cardiovascular and renal benefits in patients with kidney disease; however emerging evidence suggests that they may negatively impact bone health. This study aims to investigate the impact of SGLT2i on bone metabolism in patients with lupus nephritis (LN).</p><p><strong>Methods: </strong>This is a randomized, double-blinded placebo-controlled clinical trial, including 84 adult patients with biopsy-proven LN and estimated glomerular filtration rate (eGFR) > 30 ml/min/1.73m². Patients were randomized 1:1 and stratified according to age and gender to Dapagliflozin (10 mg daily) or placebo groups. Serum creatinine, eGFR, urinary protein-creatinine, calcium-creatinine, and phosphorus-creatinine ratios were measured at baseline and after 12 months. Bone health was assessed at the same time points using bone formation markers (bone-specific alkaline phosphatase, procollagen type 1 N-terminal propeptide) and bone resorption markers (tartrate-resistant acid phosphatase 5b and Sclerostin). Moreover, quantitative computed tomography was used to assess volumetric bone mineral density.</p><p><strong>Results: </strong>Participants had a mean age of 38 ± 7 years, with no significant baseline differences between groups. By the end of the study, there were no significant differences in kidney function or bone turnover biomarkers between the dapagliflozin and placebo groups after adjusting for baseline values. Only the lumber spine 3 (L3) T score of the quantitative computed tomography parameters was significantly better in the dapagliflozin group compared to the placebo group (P = 0.017). In a linear mixed model analysis, dapagliflozin was associated with a significant unadjusted decline in lumbar spine bone mineral density over one year, but this effect was attenuated and became non-significant after adjusting for age, steroid dose, and gender.</p><p><strong>Conclusions: </strong>Dapagliflozin did not significantly affect bone and mineral metabolism in patients with lupus nephritis over a 12-month period. These findings suggest that dapagliflozin is unlikely to have adverse effects on bone health in patients with lupus nephritis, though further research is warranted to confirm these results over longer periods in a larger cohort.</p>","PeriodicalId":16542,"journal":{"name":"Journal of Nephrology","volume":" ","pages":""},"PeriodicalIF":2.7,"publicationDate":"2025-07-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144618636","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":"Supporting people with chronic kidney disease to self-manage their condition: understanding the lived experiences, needs and requirements, and barriers and facilitators.","authors":"Courtney J Lightfoot, Thomas J Wilkinson, Matthew P M Graham-Brown, Alice C Smith","doi":"10.1007/s40620-025-02349-8","DOIUrl":"https://doi.org/10.1007/s40620-025-02349-8","url":null,"abstract":"<p><strong>Background: </strong>Self-management has been identified as an essential component in the effective management of patients with chronic kidney disease (CKD). To effectively develop interventions that support patients with CKD to self-manage, it is crucial to understand their experiences and the factors that may influence their ability to self-manage. This study explored awareness, attitudes and participation with self-management in people living with non-dialysis CKD to understand factors influencing self-management behaviours.</p><p><strong>Methods: </strong>Semi-structured interviews were conducted with 22 individuals living with non-dialysis CKD. Topics explored included perspectives and experiences of self-management, health and lifestyle behaviours, healthcare professional support, and self-management support, including future interventional approaches. Data were audio recorded and transcribed verbatim. Thematic analysis was used to analyse the data and to identify and report themes.</p><p><strong>Results: </strong>Six themes were identified encompassing perspectives, barriers and facilitators of self-management: \"perceptions and experiences of (self-)managing CKD\", \"perceived needs and requirements for self-management education and support\", \"knowledge and capability-related factors\", \"skills and opportunity-related factors\", \"confidence and motivational-related factors\" and \"social support\".</p><p><strong>Conclusion: </strong>Participants perceived that their CKD was not a significant problem, given the lack of concern from their doctor. Despite reporting a lack of awareness and understanding of CKD and its management, participants expressed interest in learning more and implementing appropriate self-management strategies. It was perceived that information and support were provided when it was almost too late, and not when it could potentially have the greatest impact. Perceived barriers and facilitators must be considered when developing interventions to support self-management for people with CKD.</p>","PeriodicalId":16542,"journal":{"name":"Journal of Nephrology","volume":" ","pages":""},"PeriodicalIF":2.7,"publicationDate":"2025-07-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144618637","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":"Impact of almond milk fortified with vitamin D3-loaded soy protein nanoparticles on serum 25(OH)D3 concentrations in adults with CKD: a pilot double-blind randomized controlled trial.","authors":"Li-Ying Kuo, Blanca Nuria Casta Neda, Yangyi E Luo, Jiyan Aslan Ceylan, Juan E Andrade, Jeanette M Andrade","doi":"10.1007/s40620-025-02353-y","DOIUrl":"https://doi.org/10.1007/s40620-025-02353-y","url":null,"abstract":"<p><strong>Background: </strong>Chronic kidney disease (CKD) impairs the activation of vitamin D, leading to deficiencies that exacerbate disease progression and metabolic complications. This pilot study investigated the effects of consuming almond milk fortified with vitamin D₃ nanoparticles on 25(OH)D₃ among adults with advanced stages of CKD.</p><p><strong>Methods: </strong>A 21-day, double-blind randomized controlled pilot study was conducted. Participants (n = 18) were randomly assigned to consume 4 oz (118 ml) of almond milk daily with 4000 IU of vitamin D₃ dispersed in soy protein nanoparticles (intervention group), or almond milk without vitamin D (control group). The primary outcome was the change in serum 25(OH)D₃ levels, while secondary outcomes included changes in serum calcium and parathyroid hormone (PTH).</p><p><strong>Results: </strong>From baseline to end of intervention, there was a significant decrease in serum 25(OH)D3 in the control group (49.2 ± 23.3 nmol/L to 44.5 ± 25.6 nmol/L; p < 0.05). In the intervention group, there was a significant increase in 25(OH)D3 (33.0 ± 15.8 nmol/L to 36.4 ± 15.8 nmol/L; p < 0.05). A significant time × treatment interaction (p < 0.01) was observed, with the intervention group showing a marked increase in serum 25(OH)D₃. No differences between groups were observed in serum calcium and PTH.</p><p><strong>Conclusion: </strong>The consumption of almond milk fortified with vitamin D₃ dispersed in soy protein nanoparticles increased 25(OH)D₃ in adults with advanced stages of CKD over 21-days. Future research should explore the metabolism and long-term efficacy of nanoparticle-based delivery of vitamin D₃ in larger, more diverse populations.</p>","PeriodicalId":16542,"journal":{"name":"Journal of Nephrology","volume":" ","pages":""},"PeriodicalIF":2.7,"publicationDate":"2025-07-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144618635","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":"Approach to the prevention of atypical hemolytic uremic syndrome recurrence using eculizumab in a pregnant kidney transplant recipient with complement factor H gene mutation: a case report and literature review.","authors":"Somaya Zahran, Tiina Podymow, Shaifali Sandal, Thomas Nodzynski, Catherine Matte, Marcelo Cantarovich","doi":"10.1007/s40620-025-02342-1","DOIUrl":"https://doi.org/10.1007/s40620-025-02342-1","url":null,"abstract":"<p><p>Eculizumab, a monoclonal antibody targeting C5, is used to treat atypical hemolytic uremic syndrome (aHUS) and prevent recurrence post-kidney transplant (KTx). However, clinical experience with its use during pregnancy in kidney transplant recipients remains limited. We report a case of a 36-year-old woman with a history of aHUS and a high-risk complement factor H gene mutation who received a KTx and was treated with maintenance eculizumab post-transplant for the prevention of aHUS recurrence, and who later became pregnant. Eculizumab maintenance was continued during pregnancy. The patient delivered a healthy infant who developed normally; allograft function was well-preserved with no recurrence of thrombotic microangiopathy during pregnancy or in the 8 years postpartum. We reviewed the literature for similar cases and herein propose a structured approach to managing pregnant KTx recipients with a history of aHUS receiving eculizumab.</p>","PeriodicalId":16542,"journal":{"name":"Journal of Nephrology","volume":" ","pages":""},"PeriodicalIF":2.7,"publicationDate":"2025-07-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144618634","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":"Development and validation of personalized risk prediction models for patients with IgA nephropathy: a nationwide multicenter cohort study.","authors":"Keita Hirano, Tatsuyoshi Ikenoue, Tomohisa Seki, Sho Komukai, Hirosuke Nakata, Takashi Yasuda, Yoshinari Yasuda, Keiichi Matsuzaki, Tetsuya Kawamura, Takashi Yokoo, Shoichi Maruyama, Hitoshi Suzuki, Yusuke Suzuki, Shingo Fukuma","doi":"10.1007/s40620-025-02338-x","DOIUrl":"https://doi.org/10.1007/s40620-025-02338-x","url":null,"abstract":"<p><strong>Background: </strong>Effective prediction of immunoglobulin A nephropathy (IgAN) progression is crucial for early intervention and management. We aimed to develop and validate distinct IgAN prediction models for clinical and research applications.</p><p><strong>Methods: </strong>We analyzed data from the Japanese Nationwide Retrospective Cohort Study in IgAN (n = 1174) gathered over 10 years. The models were developed and tested using data from general physicians in primary care, specialists in tertiary care hospitals, and researchers at academic research institutes. Three tailored prediction models (Primary Care, Tertiary Care, and Research Institute Models) were created to address the unique needs of different clinical environments. The primary outcome was a composite renal event defined as a 1.5-fold increase in serum creatinine level or progression to kidney failure. The predictive performance was assessed using C-statistics.</p><p><strong>Results: </strong>In the derivation cohort, the primary care model included predictors such as estimated glomerular filtration rate < 45 mL/min/1.73 m², proteinuria ≥ 0.5 g/day, and non-use of corticosteroids, achieving a C-statistic of 0.796 (95% confidence interval [CI] 0.686-0.895). The tertiary care model showed a C-statistic of 0.807 (95% CI 0.713-0.886), using predictors such as glomerular number and histological severity. The research institute model, incorporating 38 variables, demonstrated a C-statistic of 0.802 (95% CI 0.686-0.906).</p><p><strong>Conclusions: </strong>The prediction models for primary and tertiary care settings provided effective tools for forecasting renal outcomes in IgAN patients and are competitive with more complex machine learning-based models used in research. These models can help guide clinical decisions in various healthcare settings.</p>","PeriodicalId":16542,"journal":{"name":"Journal of Nephrology","volume":" ","pages":""},"PeriodicalIF":2.7,"publicationDate":"2025-07-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144608569","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":"Immunotherapy-induced acute interstitial nephritis with neutrophil infiltrate, looking like pyelonephritis.","authors":"Mathis Michel, Adrien Flahault, Antoine Max, Jerome Olagne, Hervé Sartelet","doi":"10.1007/s40620-025-02332-3","DOIUrl":"https://doi.org/10.1007/s40620-025-02332-3","url":null,"abstract":"","PeriodicalId":16542,"journal":{"name":"Journal of Nephrology","volume":" ","pages":""},"PeriodicalIF":2.7,"publicationDate":"2025-07-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144608570","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":"Lumasiran at birth changes the trajectory of primary hyperoxaluria type 1: same disease, different outcomes in two affected siblings.","authors":"Licia Peruzzi, Marta Leporati","doi":"10.1007/s40620-025-02325-2","DOIUrl":"https://doi.org/10.1007/s40620-025-02325-2","url":null,"abstract":"<p><p>Lumasiran, an RNA interference therapeutic, demonstrated effectiveness in clinical trials, leading to approval for primary hyperoxaluria type 1 management in all age groups. To date, little is known about its use in newborns. This study assesses, for the first time, the oxalate and glycolate metabolism in a newborn affected by primary hyperoxaluria type 1 treated at birth. His older brother, also affected by primary hyperoxaluria type 1, experienced severe disease progression and significant comorbidities. These challenges informed the decision to initiate immediate treatment for the younger sibling. The child was treated at 6 h of life with lumasiran 6 mg/kg subcutaneously, in combination with pyridoxin 10 mg/kg/day. Lumasiran 6 mg/kg was repeated at 30 and 60 days, then was reduced to 3 mg/kg every month. Intravenous hyperhydration (240 mL/kg/day) was maintained for 16 days, together with oral water and potassium citrate (500 mg in 500 mL/day) in addition to breastfeeding. Although gycolate oxidase inhibition was started immediately after birth in the absence of previous deposits, it showed a latency of at least 15 days. Over this period of time, dangerous levels of blood and urinary oxalate were reached, due to the physiological low glomerular filtration rate in the perinatal period, as demonstrated by the increasing levels of endogenous oxalate production until day 6. Blood oxalate supersaturation 30 days after the first dose of treatment was never reached again. No adverse events occurred. In this report, early treatment with lumasiran, coupled with hyperhydration and supportive therapy, was able to ensure the absence of primary hyperoxaluria type 1 symptoms throughout the 24 months of follow-up.</p>","PeriodicalId":16542,"journal":{"name":"Journal of Nephrology","volume":" ","pages":""},"PeriodicalIF":2.7,"publicationDate":"2025-07-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144591476","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}