Pediatric Nephrology最新文献

{"title":"The impact of deportation and disparities on pediatric kidney health: a humanitarian call to action.","authors":"Rupesh Raina, Jieji Hu, Kenji Ishikura, Sampson Antwi, Arsheya Ahuja, Erica Bjornstad, Melvin Bonilla-Felix","doi":"10.1007/s00467-025-06749-7","DOIUrl":"10.1007/s00467-025-06749-7","url":null,"abstract":"","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"3015-3018"},"PeriodicalIF":2.6,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143730825","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":"The unique challenges of childhood-onset systemic lupus erythematosus and lupus nephritis patients: a proposed framework for an individualized transitional care plan.","authors":"Thomas Renson, Liz Lightstone, Coziana Ciurtin, Claire Gaymer, Stephen D Marks","doi":"10.1007/s00467-024-06654-5","DOIUrl":"10.1007/s00467-024-06654-5","url":null,"abstract":"<p><p>Childhood-onset systemic lupus erythematosus (cSLE) is a severe lifelong and life-threatening autoimmune disease with multi-organ involvement. Compared to those with adult-onset disease, cSLE patients have more aggressive disease with a higher prevalence of early lupus nephritis (LN) causing worse kidney and patient outcomes. The transfer of adolescent patients to adult healthcare poses several major challenges, from a disease as well as a psychosocial perspective. Transitional care even in tertiary centers can be heterogenous, suboptimal, and often even non-existent. In this comprehensive review of the literature, we synthesize the obstacles adolescents and young adults (AYA) with systemic lupus erythematosus (SLE) and LN face and how these challenges impact the transfer to adult health care. Finally, we propose a framework for a structured and individually modifiable transitional care plan, tailored to the unique needs of this population and taking into account their social and cultural background. This framework includes suggestions for the timing of the preparatory phase and the transfer itself, the composition of the transitional care team, increasing transition readiness and treatment adherence, and establishing a supportive network of peers. Efficient transitional care will optimize long-term patient outcomes.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"3045-3053"},"PeriodicalIF":2.6,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12401761/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143625873","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 urinary extravasation in posterior urethral valves: clinical implications of urinomas and ascites on kidney function.","authors":"Adree Khondker, Joana Dos Santos, Samer Maher, Michael Chua, Armando J Lorenzo, Mandy Rickard","doi":"10.1007/s00467-025-06984-y","DOIUrl":"https://doi.org/10.1007/s00467-025-06984-y","url":null,"abstract":"<p><strong>Background: </strong>Urinary extravasation (UE) in patients with posterior urethral valves (PUV), in the form of urinomas or urinary ascites, is thought to represent a pop-off mechanism. Previous literature has proposed a kidney protective mechanism, although this remains controversial. Here, we performed a matched comparison to assess the effect of UE on kidney outcomes.</p><p><strong>Methods: </strong>We retrospectively reviewed our PUV database, including all patients diagnosed < 3 months of age with at least 1-year follow-up. We collected demographics, management, vesicoureteral reflux (VUR) status, and kidney function parameters. UE was defined as postnatal urinoma or urinary ascites. We performed both unadjusted and propensity-matched comparisons of patients with and without UE. Matching was used to balance age, diversion status, urinary tract infection history, presence of VUR, and nadir creatinine. The primary outcomes were 1-, 3-, and 5-year kidney outcomes.</p><p><strong>Results: </strong>Of the 138 patients meeting inclusion criteria, 27 (20%) had UE (23 urinoma, 4 urinary ascites). The median age at presentation was 5 days. Six patients (26%) required percutaneous drainage, and the median time to resolution was 21 days. Patients with UE had significantly higher initial creatinine levels but no difference in nadir values. Of those with available data, 18 (18%) and 10 (14%) had chronic kidney disease (CKD) at 3 and 5 years. Overall, there was no difference in 3- and 5-year rates of CKD between patients with and without a history of UE.</p><p><strong>Conclusions: </strong>In a matched comparison, UE was not associated with long-term adverse or beneficial effects on kidney function. This study provides further evidence that the presence of UE may not be a relevant prognostic factor in children with PUV.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2025-09-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145200642","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":"Is home best when it comes to pediatric hemodialysis?","authors":"Sarah J Swartz, Nicholas G Larkins","doi":"10.1007/s00467-025-06982-0","DOIUrl":"https://doi.org/10.1007/s00467-025-06982-0","url":null,"abstract":"","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2025-09-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145200625","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":"Letter to the Editor: Long-term kidney outcomes in patients with Kabuki syndrome.","authors":"Zoha Mirza, Laiba Fiaz, Muhammad Irfan","doi":"10.1007/s00467-025-06976-y","DOIUrl":"https://doi.org/10.1007/s00467-025-06976-y","url":null,"abstract":"","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2025-09-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145186471","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":"New prognostic markers for IgA nephropathy in children.","authors":"Chiara Casuscelli, Elisa Longhitano, Giovanni Conti, Veronica Maressa, Silvia Di Carlo, Claudia Spinella, Luigi Peritore, Vincenzo Calabrese, Domenico Santoro","doi":"10.1007/s00467-025-06956-2","DOIUrl":"https://doi.org/10.1007/s00467-025-06956-2","url":null,"abstract":"<p><p>IgA nephropathy (IgAN) is the most common glomerulonephritis worldwide, with significant implications for adults and children. The disease progresses variably, from asymptomatic hematuria to severe glomerulonephritis, and around 10-20% of children diagnosed in childhood develop stage 5 chronic kidney disease (CKD 5) within 20 years. Identifying reliable prognostic markers is crucial for early intervention and long-term management. The International IgAN Prediction Tool combines clinical, laboratory, and histological data and has been adapted for pediatric use. It is highly accurate in predicting CKD 5. Markers such as the Oxford MEST-C score, proteinuria, eGFR, and blood pressure assist in risk stratification. Emerging biomarkers, including the IgA/C3 ratio, galactose-deficient IgA1, soluble CD89, and urinary cytokines like IL-6 and TGF-β1, show potential for predicting disease progression. Although promising, further research is needed to validate these biomarkers in pediatric populations and refine predictive tools for IgAN progression. These advances will guide targeted therapies and improve long-term renal outcomes in children.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2025-09-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145138291","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":"Early fluid status and severe intraventricular hemorrhage or death in extremely preterm infants.","authors":"Lucinda J Weaver, Samuel J Gentle, Arie Nakhmani, Fazlur Rahman, Namasivayam Ambalavanan, Vivek V Shukla, Christine Stoops, David Askenazi, Colm P Travers","doi":"10.1007/s00467-025-06962-4","DOIUrl":"https://doi.org/10.1007/s00467-025-06962-4","url":null,"abstract":"<p><strong>Background: </strong>Measures of early postnatal fluid balance may be associated with severe intraventricular hemorrhage (sIVH) and/or death in extremely preterm infants in the first postnatal week.</p><p><strong>Methods: </strong>A single-center, retrospective cohort study including actively treated inborn infants weighing ≥ 400 g and 22-27 weeks' gestation from 2014-2021. Longitudinal mixed effect models compared daily fluid balance covariates including serum sodium, percent weight change, total fluid intake, urine output, and fluid balance (daily weight - birth weight /birth weight × 100) among infants with and without sIVH or death, during the first seven postnatal days. Multiple regression and machine learning models were developed to predict sIVH and/or death. Variables that were incorporated into the models included measures of fluid balance, gestational age, birth weight, antenatal corticosteroids, multiples, and sex.</p><p><strong>Results: </strong>We included 932 infants with mean ± SD gestational age of 25w2d ± 11d and birth weight of 746 ± 212 g of whom 195 (20.9%) had sIVH and/or death. Lower percentage weight change (p < 0.001), higher total fluid intake (p = 0.007), higher sodium (p = 0.007), and positive early fluid balance (p < 0.001) were associated with sIVH and/or death even after adjustment for baseline characteristics. The area under the receiver-operating curve (AUC) for regression models predicting sIVH and/or death incorporating baseline characteristics improved after adding fluid balance measures from 0.75 to 0.80, while the AUC for machine learning models improved from 0.72 to 0.84.</p><p><strong>Conclusions: </strong>In extremely preterm infants, early fluid status measures were associated with risk of sIVH and/or death. The addition of fluid status measures improves the performance of models predicting sIVH and/or death.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2025-09-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145131615","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":"A 1-year-old boy with MIRAGE syndrome and nephrotic syndrome, whose kidney histopathology revealed membranous nephropathy-like findings: a case report.","authors":"Shingo Ishimori, Aya Imaide, Tomoyuki Yokota, Satoshi Narumi, Norishige Yoshikawa","doi":"10.1007/s00467-025-06971-3","DOIUrl":"https://doi.org/10.1007/s00467-025-06971-3","url":null,"abstract":"<p><p>MIRAGE syndrome is a rare multisystem disorder caused by gain-on-function SAMD9 variants. Kidney biopsies in some MIRAGE syndrome patients have shown glomerular sclerosis or interstitial nephritis. A boy with genetically confirmed MIRAGE syndrome, who showed microhematuria and nephrotic range proteinuria, underwent kidney biopsy at 18 months, revealing diffuse mesangial proliferation and partial segmental lobular accentuation associated with mesangial cell proliferation with neither crescentic lesions nor sclerotic glomeruli. Immunofluorescence staining showed diffuse granular deposition of IgG along the glomerular basement membrane and dominant deposition of IgG in the mesangial matrix. Electron microscopy revealed diffuse subepithelial electron-dense deposits. Treatment with prednisolone for membranous nephropathy-like findings was initiated but the proteinuria persisted. At 21 months of age, the patient died of multi-organ failure as a result of severe gastrointestinal infection and necrotizing enterocolitis. This is the first case with histologically membranous nephropathy-like findings and marked mesangial proliferation in a child with MIRAGE syndrome with nephrotic syndrome.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2025-09-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145125672","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":"How can we make novel treatments of glomerular diseases available for children as early and safely as possible?","authors":"Kjell Tullus","doi":"10.1007/s00467-025-06963-3","DOIUrl":"https://doi.org/10.1007/s00467-025-06963-3","url":null,"abstract":"","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2025-09-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145125555","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":"Comparison of different equations for estimating the glomerular filtration rate in pediatric kidney transplant recipients.","authors":"Paphawadee Sukboonthong, Julaporn Pooliam, Maturin Jantongsree, Achra Sumboonnanonda, Anirut Pattaragarn, Suroj Supavekin, Nuntawan Piyaphanee, Kraisoon Lomjansook, Yarnarin Thunsiribuddhichai, Intraparch Tinnabut, Nuttiporn Khueankong, Thanaporn Chaiyapak","doi":"10.1007/s00467-025-06942-8","DOIUrl":"https://doi.org/10.1007/s00467-025-06942-8","url":null,"abstract":"<p><strong>Background: </strong>Accurate glomerular filtration rate estimation (eGFR) is essential for managing pediatric kidney transplant recipients. Given the physiology of pediatric patients receiving adult-donor kidneys, identifying the most appropriate plasma creatinine (PCr)-based formula-pediatric or adult-specific-is crucial.</p><p><strong>Methods: </strong>This cross-sectional study included pediatric kidney transplant recipients (age 1-18 years) who received adult-donor kidneys. We compared agreement thresholds of various pediatric and adult PCr-based GFR equations with CKiD 2012 combined PCr‒cystatin C (PCr-CystC) equation via intraclass correlation coefficients (ICCs), concordance correlation coefficients (CCCs), total deviation index (TDI), P30 performance metric (P30), Bland-Altman plots, and receiver-operating characteristic (ROC) analysis. Correlation between CKiD under 25 (U25) PCr-CystC and reference CKiD 2012 equation was also evaluated.</p><p><strong>Results: </strong>One hundred twenty samples were collected from 23 recipients (mean age = 14.2 ± 3.4 years) and donors (mean age = 31.7 ± 10.0 years). Schwartz-Lyon equation demonstrated the highest performance with the reference (ICC = 0.913, CCC = 0.911, TDI = 14.0 mL/min/1.73 m², P30 = 99.2%). U25 (ICC = 0.922, CCC = 0.882, P30 = 93.3%), full age spectrum (FAS)-height (ICC = 0.897, CCC = 0.877, P30 = 96.7%), and Bedside Schwartz equations (ICC = 0.850, CCC = 0.819, P30 = 89.2%) showed comparable performance. Bland-Altman plots revealed proportional bias (p < 0.05), leading to ROC analysis, which identified eGFR < 70 mL/min/1.73 m² for Schwartz-Lyon, U25, and FAS-height, and < 60 mL/min/1.73 m² for Bedside Schwartz as optimal agreement thresholds, beyond which each equation showed increased bias. Subgroup analyses also showed better performance in patients aged 10-18 years. Additionally, U25 PCr-CystC equation showed excellent agreement with the reference (ICC = 0.993, CCC = 0.990, P30 = 100%).</p><p><strong>Conclusions: </strong>Schwartz-Lyon equation demonstrated the highest performance among PCr-based equations with the reference in pediatric kidney transplant recipients, particularly when eGFR was < 70 mL/min/1.73 m² and in patients aged 10-18 years. U25 PCr-CystC equation showed best overall agreement with the reference and should be preferred where CystC measurement is feasible.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2025-09-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145125474","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}