Pediatric Nephrology最新文献

{"title":"Management dilemma in choosing evolving treatments in neutropenic septic shock.","authors":"H David Humes, Kera Luckritz, Stephen Gorga, Katie Plomaritas, Sara Hoatlin, Michael Humes, Lenar Yessayan","doi":"10.1007/s00467-025-06798-y","DOIUrl":"10.1007/s00467-025-06798-y","url":null,"abstract":"<p><p>How does a physician decide to use a recently FDA-approved life-saving device in a desperately ill child in which little prior clinical experience is available? This report presents a pediatric patient with neutropenic septic shock and multiorgan failure (MOF) with a 95% chance of death and the availability of a therapeutic device with a completely new approach to treat sepsis. This device, called the selective cytopheretic device (SCD), is a first-in-class autologous immune cell directed therapy. The SCD, when integrated into an extracorporeal blood circuit, has been shown to bind activated neutrophils and monocytes. With a simple pharmacologic maneuver within the device, the bound cells in real time are immunomodulated from a highly pro-inflammatory state to a less inflammatory phenotype. These transformed cells are then released back into the systemic circulation thereby tempering the systemic hyperinflammatory disorder. Since this cell directed therapy focuses on neutrophils, the processing of these cells in a neutropenic state may be a substantive risk resulting in further immunosuppression. On the other hand, the immunomodulation of the circulating neutrophils and monocytes, although sparse, may be beneficial to disrupt the dysregulated inflammatory state responsible for ongoing tissue damage and organ dysfunction. Prior clinical SCD trials excluded patients with neutropenia so that no prior clinical experience was available to make a difficult decision. This report presents the way the medical team approached these issues and made a therapeutic plan that resulted in a positive clinical outcome for the patient.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"3577-3583"},"PeriodicalIF":2.6,"publicationDate":"2025-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12484358/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144340339","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":"Clinical characteristics of patients with SALL1-related disorder.","authors":"Yoshitaka Asagai, Yu Tanaka, Hiroaki Hanafusa, China Nagano, Tomoko Horinouchi, Shingo Ishimori, Hiroshi Kaito, Kazumoto Iijima, Kandai Nozu, Naoya Morisada","doi":"10.1007/s00467-025-06878-z","DOIUrl":"10.1007/s00467-025-06878-z","url":null,"abstract":"<p><strong>Background: </strong>The Spalt-like transcription factor 1 (SALL1) gene is essential for kidney development. Pathogenic SALL1 variants cause Townes-Brocks syndrome 1 (TBS1), which typically presents with imperforate anus, dysplastic ears, and digital anomalies. However, clinical features vary widely. Some patients present only with dysplastic ears and hearing loss (HL) or with congenital anomalies of the kidney and urinary tract (CAKUT), resembling branchio-oto-renal syndrome (BORS), a presentation referred to as Townes-Brocks branchio-oto-renal-like (TBS BOR-like) syndrome. In this study, we aimed to describe the clinical characteristics of patients with SALL1-related disorders in the Japanese population.</p><p><strong>Methods: </strong>We analyzed phenotypes of a nationwide cohort comprising 1108 families with chronic kidney disease (CKD) or mild urinary anomalies, using genetic testing conducted from 2010 to 2024.</p><p><strong>Results: </strong>We identified SALL1 variants in 14 families (20 individuals): seven frameshift, four nonsense, one missense, one exon 2 deletion, and one whole-gene deletion. Ten variants were novel. The median age at diagnosis was 16 years (male:female = 13:7). Dysplastic ears were observed in 45%, HL in 40%, digital anomalies in 40%, and anorectal malformations in 25%. Based on clinical features, eight individuals were diagnosed with TBS1, four with TBS BOR-like syndrome, and seven with non-syndromic CAKUT. One case lacked detailed clinical data. Most variants were truncating and located in exon 2.</p><p><strong>Conclusions: </strong>SALL1-related disorders exhibit broad phenotypic variability. Some cases present with atypical features overlapping with TBS BOR-like syndrome or isolated CAKUT, rather than with typical TBS1. These findings enhance the understanding and diagnosis of SALL1-related disorders.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"3407-3414"},"PeriodicalIF":2.6,"publicationDate":"2025-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12484339/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144626809","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":"Growth after pediatric and neonatal acute kidney injury: a meta-analysis.","authors":"Michelle C Starr, Mital Patel, Faizeen Zafar, Melissa S Zhou, Russell Griffin, Annabel Biruete, Vedran Cockovski, Rasheed Gbadegesin, Dana Y Fuhrman, Katja M Gist, Cherry Mammen, Shina Menon, Catherine Morgan, Cara L Slagle, Scott Sutherland, Michael Zappitelli, Danielle E Soranno","doi":"10.1007/s00467-025-06801-6","DOIUrl":"10.1007/s00467-025-06801-6","url":null,"abstract":"&lt;p&gt;&lt;strong&gt;Background: &lt;/strong&gt;Acute kidney injury (AKI) occurs commonly in critically ill children. The impact of AKI on pediatric growth outcomes has been sparsely described.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Objective: &lt;/strong&gt;To compare growth in children with a history of AKI compared to those without AKI. We hypothesized that children with AKI would have worse growth compared to those without AKI.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Data sources: &lt;/strong&gt;A convenience sample of existing prospective and retrospective cohorts of children with AKI who had already collected or were able to collect data on growth parameters before and after an episode of AKI.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Study eligibility criteria: &lt;/strong&gt;There are &lt; 5 studies in the published literature on growth in children with AKI. These investigators were contacted, and additional studies were added by contacting primary investigators of studies of childhood AKI in which data on growth parameters was able to be collected.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Participants and interventions: &lt;/strong&gt;Children from existing cohorts evaluating AKI (exposure) during childhood. Each included cohort had previously received local IRB approval per institutional guidelines. As our study was a meta-analysis and only used cohort-level data, no IRB approval was required for this report.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Study appraisal and synthesis methods: &lt;/strong&gt;Growth parameters (length and weight z-scores) before and after an episode of AKI were compared using a meta-means analysis. MOOSE guidelines were used. Data were pooled using a random-effects model. Hedges g was calculated, and Higgins I&lt;sup&gt;2&lt;/sup&gt; statistic was used to define variability due to between-cohort heterogeneity.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Results: &lt;/strong&gt;We included 3,586 children from 17 existing cohorts of AKI in various populations, including infants, children with cardiac disease, solid organ transplant and critically ill children without cardiac disease with follow-up from 12 months to 11 years after AKI. At most distant follow-up, those with AKI had lower length z-score than those without AKI (mean difference -0.37 [95%CI -0.52, -0.22, p &lt; 0.001]) and lower weight z-score (mean difference of -0.29 [95%CI -0.43, -0.15, p &lt; 0.001]). This difference was most striking in infants, as those with AKI had impaired growth (both length z-score and weight z-score) after AKI compared to those without AKI.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Limitations: &lt;/strong&gt;The analysis included only a convenience sample of observational cohorts of children, study selection could have been biased, and we did not evaluate the relationship between decreased kidney function (e.g., chronic kidney disease) after AKI in these cohorts and its relationship to poor growth.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Conclusions and implications of key findings: &lt;/strong&gt;This meta-analysis found that children with AKI have impaired growth after AKI. These findings were most striking in infants. We suggest focusing on growth outcomes in both clinical care and research investigating the impact","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"3379-3389"},"PeriodicalIF":2.6,"publicationDate":"2025-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12484312/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144004880","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":"Unmasking fluid overload in children on peritoneal dialysis: a multimodal diagnostic approach.","authors":"Bahriye Atmis, Ikbal Turker, Derya Cevizli, Cagla Cagli Piskin, Faruk Ekinci, Dincer Yildizdas, Aysun K Bayazit","doi":"10.1007/s00467-025-06825-y","DOIUrl":"10.1007/s00467-025-06825-y","url":null,"abstract":"<p><strong>Background: </strong>This study aimed to assess fluid status in pediatric patients on peritoneal dialysis by combining ultrasonography and bioimpedance spectroscopy (BIS). It specifically focused on examining the changes in volume status following a 2-h dwell time ultrafiltration exchange and evaluating the reliability of these techniques.</p><p><strong>Methods: </strong>Thirteen pediatric patients on peritoneal dialysis were enrolled in this study, and their hydration status was assessed clinically. In addition, 56 lung ultrasound measurements, inferior vena cava (IVC) collapsibility index assessments, and BIS evaluations were performed both before and after a 2-h dwell exchange using 2.27%/2.5% dextrose dialysate.</p><p><strong>Results: </strong>The mean age of the patients was 8.6 ± 4.1 years, and eight of them (61.5%) were male. The IVC collapsibility index significantly increased (26.3 ± 10.0% vs. 44.4 ± 9.4%; p < 0.001), and the total number of B-lines significantly decreased (median 22 vs. 11.5; p < 0.001) after a 2-h dwell exchange using 2.27%/2.5% dextrose dialysate. A positive correlation was observed between the total number of B-lines and fluid overload measured using BIS both pre-dialysis (r = 0.504, p = 0.006) and post-dialysis (r = 0.528, p = 0.004). A significant reduction in the total number of B-lines was observed across all hydration groups after dialysis (p < 0.001). The area under the receiver-operating characteristic curve (AUC) for the total number of B-lines in predicting severe overhydration was 0.685 (p = 0.097) when assessed using BIS and 0.740 (p = 0.181) when assessed by weight.</p><p><strong>Conclusion: </strong>Our results highlight marked changes in fluid status parameters from pre- to post-dialysis, underscoring the clinical value of combining lung ultrasonography and BIS for monitoring fluid overload in pediatric patients undergoing peritoneal dialysis.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"3505-3511"},"PeriodicalIF":2.6,"publicationDate":"2025-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12484372/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144554127","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":"Investigation of normalized protein catabolic rate as a marker of nutritional status in infants and children receiving chronic hemodialysis: a longitudinal cohort study.","authors":"Nicole Salach, Celina Brunson, Anqing Zhang, Kristen Sgambat","doi":"10.1007/s00467-025-06859-2","DOIUrl":"10.1007/s00467-025-06859-2","url":null,"abstract":"<p><strong>Background: </strong>Children with kidney failure are at risk for compromised nutritional status due to a variety of challenges. Normalized protein catabolic rate (NPCR) below 1 g/kg/day has been associated with weight loss in adolescent patients on chronic hemodialysis. We sought to establish NPCR as a marker of nutritional status in patients on hemodialysis under age 13 years.</p><p><strong>Methods: </strong>A longitudinal retrospective cohort study was conducted to investigate NPCR as a marker of a composite indicator of compromised nutritional status (MINI) in infants and children (0-12 years old) who received chronic hemodialysis between 2002 and 2023. Generalized linear mixed effect models were applied to explore associations between MINI and NPCR across the study cohort (0-12 years) and after age stratification (0-3 years and 4-12 years).</p><p><strong>Results: </strong>The analysis included 758 observations of 58 patients with median age 8 (IQR 3,11) years. Compromised nutritional status was identified in 35/58 patients at 235/758 time points according to the composite definition of MINI. In children 0-12 years, NPCR < 1.2 was associated with approximately twofold increased odds of MINI (p = 0.04), adjusted for time on dialysis and Kt/V. After stratifying by age, children ages 4-12 years with NPCR < 1.2 had approximately fourfold higher odds of MINI (p = 0.003).</p><p><strong>Conclusions: </strong>NPCR < 1.2 g/kg/d was associated with a composite indicator of compromised nutritional status in this single-center retrospective analysis of the largest sample of hemodialysis patients under the age 13 to date. Multicenter studies are needed to verify these findings.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"3485-3493"},"PeriodicalIF":2.6,"publicationDate":"2025-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144476154","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":"Toward lean body weight- and ECV-based dosing of burosumab: eliminating apparent sex differences in XLH therapy.","authors":"Guido Filler, Funmbi Babalola","doi":"10.1007/s00467-025-06823-0","DOIUrl":"10.1007/s00467-025-06823-0","url":null,"abstract":"","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"3585-3587"},"PeriodicalIF":2.6,"publicationDate":"2025-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144143248","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":"Response to: \"Toward better outcomes in pediatric septic shock: the role of practical risk models\".","authors":"Yu Fang, Wei Lin","doi":"10.1007/s00467-025-06927-7","DOIUrl":"10.1007/s00467-025-06927-7","url":null,"abstract":"","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"3595-3596"},"PeriodicalIF":2.6,"publicationDate":"2025-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144784949","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":"Risk factors for predicting acute kidney injury in children with septic shock: a retrospective cohort study.","authors":"Yu Fang, Weihong Zheng, Kepei Chen, Qiqi Gao, Wenwen Jin, Wei Hu, Yu Chen, Zhenlang Lin, Guoquan Pan, Wei Lin","doi":"10.1007/s00467-025-06834-x","DOIUrl":"10.1007/s00467-025-06834-x","url":null,"abstract":"<p><strong>Background: </strong>Acute kidney injury (AKI) is a prevalent and severe complication of septic shock in children, yet data on its risk factors remain scarce. This study aims to identify key predictors for AKI in this population and develop a clinical model for early risk assessment.</p><p><strong>Methods: </strong>We conducted a retrospective analysis of clinical data from 180 children diagnosed with septic shock at a large tertiary hospital in China over 10 years. Multivariate analysis was performed to identify independent risk factors for AKI. Based on the results of the multivariate analysis, a clinical predictive nomogram for assessing the risk of sepsis-associated AKI (SA-AKI) in children with septic shock was established and validated using the \"rms\" package in R 4.3.0 software.</p><p><strong>Results: </strong>The incidence of AKI in children with septic shock was 44.4%, with significant predictors identified as greater height (95% CI 1.01-1.04), positive random proteinuria (95% CI 1.17-13.09), elevated procalcitonin levels (95% CI 1.00-1.04), base excess (95% CI 0.85-0.99), increased blood urea nitrogen levels (95% CI 1.03-1.22), and prolonged prothrombin time by ≥ 3 s (95% CI 1.13-11.43). Early use of antibiotics (95% CI 0.03-0.77) demonstrated a protective effect. The developed clinical predictive nomogram's ROC curve AUC was 0.895 (95% CI 0.836-0.955), with a sensitivity of 77.1% and specificity of 88.9%. It outperformed individual variables in predicting SA-AKI, and demonstrated good calibration and clinical utility as shown by the calibration and DCA curve. Internal validation by the bootstrap resampling method (1000 times) confirmed the model's accuracy with an AUC of 0.895 (95% CI 0.893-0.896).</p><p><strong>Conclusions: </strong>Recognizing these risk factors facilitates timely interventions for pediatric patients with septic shock. The nomogram serves as a valuable tool for clinicians, improving the management of AKI and potentially enhancing patient outcomes.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"3563-3575"},"PeriodicalIF":2.6,"publicationDate":"2025-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144266852","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":"Tubular proteinuria due to hereditary endocytic receptor disorder of the proximal tubule: Dent disease and chronic benign proteinuria.","authors":"Nana Sakakibara, Kandai Nozu","doi":"10.1007/s00467-025-06745-x","DOIUrl":"10.1007/s00467-025-06745-x","url":null,"abstract":"<p><p>The proximal tubule has a highly efficient endocytic pathway dedicated to reabsorbing albumin and low-molecular-weight proteins that have passed through the glomerular filtration barrier. This pathway is dependent on multi-ligand receptors: megalin and cubilin. Abnormalities in genes associated with endocytosis in the proximal tubule can lead to tubular proteinuria, where the urine contains albumin and low-molecular-weight proteins. Dent disease is a hereditary X-linked disorder characterized by low-molecular-weight proteinuria, hypercalciuria, nephrocalcinosis, nephrolithiasis, and progressive kidney dysfunction, often leading to CKD stage 5. CLCN5 is the gene responsible for Dent disease-1 and encodes the voltage-gated chloride channel ClC-5. Meanwhile, OCRL is the causative gene of Dent disease-2 and encodes phosphatidylinositol 4,5-bisphosphate 5-phosphatase, and its variants are also associated with Lowe syndrome. ClC-5 and OCRL are essential to the endocytic machinery, and their loss affects endosomal acidification and trafficking, resulting in disruption of megalin and cubilin recycling. CUBN, which encodes cubilin, was originally identified as the causative gene of Imerslund-Gräsbeck syndrome, a disorder of megaloblastic anemia associated with proteinuria. However, recently, a biallelic C-terminal variant of CUBN was shown to be responsible for isolated proteinuria without kidney dysfunction. This proteinuria is recognized as a new disease concept called chronic benign proteinuria (proteinuria, chronic benign: PROCHOB), which contradicts the common belief that proteinuria is harmful and ultimately leads to kidney damage. This article deepens the understanding of genetic tubular proteinuria and its origins, focusing on the role of megalin- and cubilin-mediated endocytosis in the proximal tubule.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"3367-3377"},"PeriodicalIF":2.6,"publicationDate":"2025-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12484296/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143753767","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":"Impact of a standardized care pathway and a dedicated multidisciplinary posterior urethral valve clinic on 5-year kidney and bladder outcomes.","authors":"Mandy Rickard, Armando J Lorenzo, Cal Robinson, Samer Maher, Jin Kyu Kim, Adree Khondker, Mirriam Mikhail, Beverly Miranda, Rodrigo Romao, Joao Pippi Salle, Michael Chua, Aseel Al-Dmour, Nithiakishna Selvathesan, Joana Dos Santos","doi":"10.1007/s00467-025-06864-5","DOIUrl":"10.1007/s00467-025-06864-5","url":null,"abstract":"<p><strong>Background: </strong>Posterior urethral valves (PUV) are a rare congenital condition leading to chronic kidney disease (CKD) and bladder dysfunction. Traditional management is often reactive and non-standardized. In 2019, we implemented a dedicated multidisciplinary PUV clinic with a standardized, proactive management pathway to optimize kidney and bladder outcomes. This study evaluates its impact over 5 years.</p><p><strong>Methods: </strong>We conducted a retrospective review of our prospectively maintained PUV database, including patients diagnosed before 24 months and managed exclusively at our institution. Outcomes were compared between patients managed in the PUV clinic (aPUV) to patients before implementation (bPUV). Primary outcomes included kidney function (serum creatinine, eGFR, CKD progression, and kidney replacement therapy). Secondary outcomes assessed bladder function, including medications, catheterization, and urinary tract infections.</p><p><strong>Results: </strong>A total of 196 patients were analyzed (bPUV: 133; aPUV: 63). The aPUV cohort had earlier interventions and a higher rate of primary urinary diversion (54% vs. 11%; p < 0.01). The aPUV group had lower nadir serum creatinine (21 vs. 29 μmol/l; p < 0.01), reduced CKD progression (12% vs. 27%; p = 0.02), and fewer patients requiring kidney replacement therapy (3% vs. 20%; p < 0.01). Earlier bladder therapy initiation led to reduced hydronephrosis and timelier catheterization.</p><p><strong>Conclusion: </strong>A standardized PUV clinic and proactive management approach significantly improved kidney and bladder outcomes at 5 years. If sustained, this strategy may delay CKD progression and reduce the need for kidney replacement therapy. Longer follow-up is needed to assess long-term impact.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"3459-3467"},"PeriodicalIF":2.6,"publicationDate":"2025-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144485369","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}