Pediatric Nephrology最新文献

{"title":"Broken signals: when the mineralostat alters the cardiovascular system.","authors":"Emily Akerman, Amélie Cifuentes, Guillaume Courbon","doi":"10.1007/s00467-026-07283-w","DOIUrl":"10.1007/s00467-026-07283-w","url":null,"abstract":"<p><p>Patients with kidney disease face a markedly increased risk of developing cardiovascular disease (CVD) which manifests in multiple forms, including: left ventricular hypertrophy, heart failure, atherosclerosis, and vascular inflammation, all of which are associated in clinical studies to well-characterized morbidity and mortality reported throughout disease progression. These patients also exhibit disturbances in mineral metabolism and in the network of signals that regulate mineral balance, that we collectively refer to as the \"mineralostat.\" This review aims to summarize the links between mineralostat alterations and its potential implications for the onset and progression of CVD, as disruptive signals are transmitted to the heart and vascular system. Over the past decade, studies using sophisticated preclinical models have identified new proteins and signaling pathways of the mineralostat as candidates contributing to CVD. Here, we discuss the pathophysiological conditions reported to disrupt the mineralostat, the hormones involved in mineral metabolism, and the minerals. We also summarize the mechanisms through which each of these factors may promote CVD, with attention to their relevance for patients.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"4271-4279"},"PeriodicalIF":2.5,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147777694","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Discontinuation of mycophenolate mofetil as maintenance therapy after rituximab treatment in childhood-onset complicated frequently relapsing or steroid-dependent nephrotic syndrome.","authors":"Taishi Nada, Koichi Kamei, Keigo Sasaki, Kentaro Nishi, Yu Kamigaki, Toru Uchimura, Aya Inaba, Masao Ogura, Shuichi Ito","doi":"10.1007/s00467-026-07397-1","DOIUrl":"10.1007/s00467-026-07397-1","url":null,"abstract":"<p><strong>Background: </strong>Mycophenolate mofetil (MMF) as maintenance therapy after rituximab treatment is effective in preventing relapses in children with complicated frequently relapsing or steroid-dependent nephrotic syndrome. However, once sustained remission has been achieved by MMF, the outcomes and risk of relapse after discontinuing MMF remain unclear.</p><p><strong>Methods: </strong>We conducted a two-center, retrospective study of patients with childhood-onset frequently relapsing or steroid-dependent nephrotic syndrome who discontinued MMF because of sustained remission for ≥ 2 years on maintenance MMF after rituximab treatment. Relapse, additional treatment, and risk factors for relapses after MMF discontinuation were analyzed.</p><p><strong>Results: </strong>Sixty patients were enrolled. After tapering or discontinuation of MMF, 35 (58%) patients relapsed, and the median time from tapering to relapse was 276 days. The 50% relapse-free survival was 1.5 years using the Kaplan-Meier method. After relapses, 30 (86%) patients required reinitiation of immunosuppressants or additional rituximab treatment. The history of initial steroid-resistant nephrotic syndrome, relapses < 10 times before the first rituximab treatment with immunosuppressants, and a longer period between the last B-cell recovery and tapering MMF were significantly associated with a lower risk of relapse in a multivariate analysis using the Cox proportional hazards model (all p < 0.05).</p><p><strong>Conclusions: </strong>Discontinuation of MMF may be considered in selected patients with sustained remission after rituximab treatment, particularly those with identified low-risk factors for relapse. The timing of discontinuation should be individualized according to these factors and patient-specific considerations because resumption of immunosuppressants or additional rituximab treatment is frequently required after relapse following MMF discontinuation.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"4423-4432"},"PeriodicalIF":2.5,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148227443","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Reversed cortico-medullary differentiation in kidneys on fetal magnetic resonance imaging - a case series.","authors":"Monika Bekiesinska-Figatowska, Jaroslaw Madzik, Marcin Ring, Agata Skorka, Marta Smyk, Ewa Obersztyn","doi":"10.1007/s00467-025-06938-4","DOIUrl":"10.1007/s00467-025-06938-4","url":null,"abstract":"<p><p>The reversed cortico‑medullary differentiation in fetal kidneys on ultrasound has been described in the literature, but there have been no descriptions of such a finding on fetal magnetic resonance imaging (MRI) so far. The authors present three unrelated fetuses with hyperechoic kidneys on ultrasound (US) and reversed signal intensity of their cortex and pyramids on SSFSE/T2WI and FIESTA images on magnetic resonance imaging (MRI). All of them shared the same deletion of the long arm of chromosome 17 in the 17q12 region, responsible for the expression of clinical features of renal cysts and diabetes (RCAD) syndrome. All of them had multiple tiny kidney cysts on US after birth. This specific finding on fetal MRI may point at this specific genetic condition.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"77-79"},"PeriodicalIF":2.6,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12685989/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144964115","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Progression of the estimated glomerular filtration rate in asphyxiated neonates undergoing therapeutic hypothermia during the first 10 days of life.","authors":"Karel Allegaert, Julia Macente, Djalila Mekahli, John van den Anker, Pieter Annaert, Anne Smits","doi":"10.1007/s00467-025-06957-1","DOIUrl":"10.1007/s00467-025-06957-1","url":null,"abstract":"<p><strong>Background: </strong>Serum creatinine (Scr) centile values were recently described in a cohort of 1136 (near)-term neonates that underwent therapeutic hypothermia (TH) because of moderate to severe hypoxic-ischemic encephalopathy. Recent methodological progress enables conversion of these Scr centiles to estimated glomerular filtration rate (eGFR) values.</p><p><strong>Methods: </strong>Scr centiles in the TH dataset during the first 10 days of life were converted to eGFR values, using the Schwartz formula, with the Smeets k-value (0.31) and fixed body length (50 cm) to generate postnatal reference eGFR values, centiles, and an equation for median eGFRs. These findings were compared to published eGFR data in term controls.</p><p><strong>Results: </strong>A polynomial function was estimated: <math><mrow><mi>eGFR</mi> <mfenced><mfrac><mi>mL</mi> <mi>min</mi></mfrac> <mo>∙</mo> <mn>1.73</mn> <msup><mrow><mi>m</mi></mrow> <mn>2</mn></msup> </mfenced> <mo>=</mo> <mn>9.1667</mn> <mo>+</mo> <mn>7.1173</mn> <mo>-</mo> <mn>0.3439</mn> <msup><mrow><mi>x</mi></mrow> <mn>2</mn></msup> <mo>,</mo> <mrow><mo>(</mo> <mi>x</mi> <mo>=</mo> <mi>days</mi> <mo>)</mo></mrow> </mrow> </math> for eGFR in TH neonates. The median eGFR increases 2- to threefold over the first week (day 1: 16.1; day 2: 19.4; day 7: 41.2 mL/min∙1.73 m²), while the polynomial function does not fully reflect the interindividual variability in eGFR values (intra-day variability is also 2- to threefold). Patterns in acute kidney injury (AKI) TH cases differ significantly from non-AKI TH cases. Based on pooling of published eGFR data, this was compared to a function in healthy term neonates: <math><mrow><mi>eGFR</mi> <mfenced><mfrac><mi>mL</mi> <mi>min</mi></mfrac> <mo>∙</mo> <mn>1.73</mn> <msup><mrow><mi>m</mi></mrow> <mn>2</mn></msup> </mfenced> <mo>=</mo> <mn>14.2167</mn> <mo>+</mo> <mn>6.7644</mn> <mo>-</mo> <mn>0.3901</mn> <msup><mrow><mi>x</mi></mrow> <mn>2</mn></msup> <mrow><mo>(</mo> <mi>x</mi> <mo>=</mo> <mi>days</mi> <mo>)</mo></mrow> </mrow> </math> (day 1: 20; day 2: 26; day 7: 42 mL/min/1.73 m²).</p><p><strong>Conclusions: </strong>Based on a pooled dataset in TH cases, we converted Scr centiles to eGFR centiles. Based on median values, this resulted in a polynomial function in TH cases, compared to healthy term neonates. This eGFR function enables precision pharmacotherapy for GFR-cleared drugs in this vulnerable population.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"233-238"},"PeriodicalIF":2.5,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12686099/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145086795","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Gut microbiota-targeted therapies in pediatric chronic kidney disease: gaps and opportunities.","authors":"Arpana Iyengar, Balamurugan Ramadass, Shruthi Venkatesh, Robert H Mak","doi":"10.1007/s00467-025-06789-z","DOIUrl":"10.1007/s00467-025-06789-z","url":null,"abstract":"<p><p>Given the complex relationship between the gut microbiome and chronic kidney disease (CKD), exploring the potential role and scope of microbiota-targeted therapies in pediatric CKD is highly relevant. We aim to provide an overview of gut-targeted therapeutic strategies, including nutritional interventions (fiber, phytochemicals, fermented foods, and traditional Chinese medicines), probiotics, synbiotics, oral absorbents, and fecal microbial transplantation. Enhancing physical activity and preventing constipation are additional strategies that may promote gut microbiome health. In a uremic environment, gut microbiota-targeted therapies could potentially rebalance the gut microbiota, improve gut barrier function, decrease uremic toxin concentrations, enhance the production of short-chain fatty acids (SCFA), and reduce inflammation. While research in adult CKD patients has provided insights into these approaches, there are limited data in children with CKD. This review aims to summarize potential targeted therapies for restoring a balanced gut microbiota, emphasizing the need for studies that evaluate their effects on clinical outcomes in pediatric CKD.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"33-43"},"PeriodicalIF":2.5,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144041019","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Correlation between polymorphisms of the aquaporin-1 gene and peritoneal function in children on chronic peritoneal dialysis.","authors":"Jiani Yao, Chunyan Wang, Xiaoyan Fang, Jing Chen, Zhiqing Zhang, Jiaojiao Liu, Jialu Liu, Rufeng Dai, Xiaotian Chen, Yihui Zhai, Hong Xu, Qian Shen","doi":"10.1007/s00467-025-06959-z","DOIUrl":"10.1007/s00467-025-06959-z","url":null,"abstract":"<p><strong>Background: </strong>Aquaporins (AQPs) are a class of proteins that transport water molecules across membranes, which can promote water transport in cells. We aimed to explore the correlation between different polymorphisms of AQP1 and peritoneal function in children on peritoneal dialysis (PD).</p><p><strong>Methods: </strong>Children who underwent PD at the Children's Hospital of Fudan University from January 1, 2014, to December 31, 2023, were included. The AQP1 genotypes of the four polymorphisms were rs2075574 (TT, CT, CC), rs1049305 (GG, CG, CC), rs10253374 (TT, CT, CC) and rs17159702 (TT, CT, CC).</p><p><strong>Results: </strong>A total of 187 children on chronic PD were included in the study. We found that the TT group with rs2075574 exhibited a lower baseline peritoneal equilibration test (PET) ultrafiltration level than the CC group (302 ± 129 vs. 408 ± 168 ml/m², P = 0.015). For rs1049305, the CC group had a higher pKT/V than both the GG (2.71 ± 1.25 vs. 2.27 ± 0.79, P = 0.04) and CG groups (2.71 ± 1.25 vs. 2.24 ± 0.88, P = 0.03). Additionally, at 12-month follow-up, the CC (410 ± 160 ml/m², P = 0.04) and CG (393 ± 174 ml/m², P = 0.04) groups of rs1049305 showed higher PET ultrafiltration than the GG group (239 ± 288 ml/m²). No significant correlation was observed between the four genotypes and adverse events.</p><p><strong>Conclusions: </strong>AQP1 rs2075574 and rs1049305 polymorphisms might be associated with ultrafiltration and urea transport in children with PD.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"193-202"},"PeriodicalIF":2.5,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145150533","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"B-cell-targeting therapies in podocytopathies.","authors":"Carolina Bigatti, Paolo Cravedi, Andrea Angeletti","doi":"10.1007/s00467-026-07297-4","DOIUrl":"10.1007/s00467-026-07297-4","url":null,"abstract":"<p><p>Podocytopathies, including minimal change disease and focal segmental glomerulosclerosis, represent the leading causes of nephrotic syndrome in children and adolescents. Although traditionally considered T-cell-mediated disorders, growing evidence over the last decade has reshaped this paradigm, highlighting a central role for B-cells, plasma cells, and humoral immune mechanisms in podocyte injury. The identification of pathogenic autoantibodies, particularly anti-nephrin IgG, and the involvement of complement activation have provided a strong mechanistic rationale for B-cell-targeted therapies. Rituximab, an anti-CD20 monoclonal antibody, has become an established steroid-sparing agent in steroid-dependent and frequently relapsing nephrotic syndrome, significantly reducing relapse rates and cumulative steroid exposure in pediatric patients. However, its efficacy is limited in steroid-resistant disease and in settings characterized by persistent autoantibody production or post-transplant recurrence. These limitations have prompted the exploration of next-generation anti-CD20 agents, such as obinutuzumab, which achieve deeper and more sustained B-cell depletion, as well as therapies targeting long-lived plasma cells, including anti-CD38 monoclonal antibodies. Emerging clinical data suggest that combined B-cell and plasma cell targeting may induce durable remission in refractory podocytopathies and recurrent post-transplant focal segmental glomerulosclerosis, while maintaining an acceptable safety profile. In parallel, novel strategies targeting the BAFF/APRIL axis and cellular therapies such as CAR-T cells are under investigation. This review summarizes current evidence on B-cell-directed therapies in podocytopathies, mostly in pediatrics, discusses unmet clinical needs, and outlines future perspectives toward precision, mechanism-based immunotherapy.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"4259-4269"},"PeriodicalIF":2.5,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13672453/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147717639","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Neonatal acute kidney injury: Reassessing diagnostic criteria for acute kidney injury in neonates with hypoxic-ischaemic encephalopathy undergoing therapeutic hypothermia.","authors":"Þórdís Kristinsdóttir, Milan Chromek, Mats Blennow, Katarina Robertsson Grossmann","doi":"10.1007/s00467-026-07469-2","DOIUrl":"10.1007/s00467-026-07469-2","url":null,"abstract":"<p><strong>Background: </strong>Acute kidney injury (AKI) remains common in neonates with hypothermia-treated hypoxic-ischaemic encephalopathy (HIE). Although the modified neonatal KDIGO (nKDIGO) criteria form the diagnostic standard, their reliance on serum creatinine (SCr) increase and urine output (UO) is particularly challenging in early onset neonatal AKI. The Gupta definition, based on insufficient SCr-decline, has been proposed to identify neonates with clinically significant AKI uncaptured by nKDIGO.</p><p><strong>Methods: </strong>This retrospective cohort included 55 near-term/term neonates with hypothermia-treated HIE in Stockholm (2007-2009). Applying nKDIGO and Gupta criteria, infants were categorised as nKDIGO-AKI, Gupta-AKI, or no AKI. Primary outcome was AKI-incidence, secondary outcomes included mortality, organ support, length of stay, and signs of possible impaired kidney function at 10-12 years.</p><p><strong>Results: </strong>In our cohort, 31 (56%) suffered AKI: 17 (31%) fulfilled both definitions, 7 (13%) Gupta-only, and 7 (13%) nKDIGO-only. Need for inotropic support was more common among infants with nKDIGO-AKI compared to those without nKDIGO-AKI (p = 0.03). In a subanalysis, a significantly larger proportion of infants with Gupta-AKI needed inotropic support compared to those without Gupta-AKI (p = 0.03). Gupta-AKI was associated with a significantly higher cystatin C at 10-12 years (p = 0.03) compared to those without Gupta-AKI.</p><p><strong>Conclusions: </strong>The Gupta definition identified additional infants with AKI beyond nKDIGO. For both definitions, a larger proportion of infants needed inotropic support in the AKI-group compared to those without AKI. Children with a history of Gupta-AKI had a significantly higher cystatin C in early adolescence compared to those without. Incorporating Gupta criteria may improve identification of infants with significant AKI.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"4657-4668"},"PeriodicalIF":2.5,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13672522/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148550109","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Rapid early renal response to add-on belimumab in lupus nephritis.","authors":"Jhao-Jhuang Ding, Jing-Long Huang, Min-Hua Tseng","doi":"10.1007/s00467-026-07439-8","DOIUrl":"10.1007/s00467-026-07439-8","url":null,"abstract":"","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"4713-4715"},"PeriodicalIF":2.5,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148302647","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Reply to 'Can clinician preferences alone guide implementable CDS tool design to measure pediatric CKD risk after preterm birth?'","authors":"Keia Sanderson, Christine E Kistler, T Michael O'Shea, Jennifer E Flythe","doi":"10.1007/s00467-026-07511-3","DOIUrl":"10.1007/s00467-026-07511-3","url":null,"abstract":"","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"4733-4734"},"PeriodicalIF":2.5,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148721550","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}