Pediatric Nephrology最新文献

{"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":"10.1007/s00467-025-06976-y","url":null,"abstract":"","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"261"},"PeriodicalIF":2.5,"publicationDate":"2026-12-01","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":"Impact of maternal health on neonatal and long-term kidney outcomes.","authors":"Nivedita Kamath, Saudamini Nesargi, Divya Bajpai, Pravin Singarayar, Enrico Vidal, Valerie A Luyckx","doi":"10.1007/s00467-025-06771-9","DOIUrl":"10.1007/s00467-025-06771-9","url":null,"abstract":"<p><p>The increasing prevalence of non-communicable diseases like chronic kidney disease signals the need for a deeper understanding of the impact of the intra-uterine milieu on developmental programming and its impact on health outcomes through the lifespan of an individual. Maternal health in the pre-gestational and gestational phases, including nutrition, exposure to drugs, environmental toxins, infectious and non-infectious diseases, and socio-economic conditions influence the overall development of the fetus as well as the fetal kidney. The small, vulnerable newborn, born from an adverse developmental environment, is predisposed to low nephron number and is at risk for acute kidney injury in the neonatal period. Developmental programming has far-reaching consequences, including a higher risk for cardio-kidney-metabolic diseases, including hypertension and chronic kidney disease, and pregnancy complications, which perpetuates an intergenerational cycle of non-communicable disease risk. This risk can be mitigated by optimizing the care of individuals in the reproductive age group, identifying high-risk pregnancies early, and providing optimal treatment and monitoring. Care of the small vulnerable neonate includes prevention of acute kidney injury and life-long surveillance and modulation of cardio-kidney-metabolic risk. The review focuses on highlighting the influence of maternal health in the pre-gestational and gestational phases on kidney health from the neonatal period to adulthood.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"45-59"},"PeriodicalIF":2.5,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144209107","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":"Commentary on \"Ultra-rare severe kidney dysplasia mimicking salt-wasting tubulopathy associated with TFCP2L1 gene variants\".","authors":"Noaman Khan, Sameed Ahmad, Muneeb Ullah","doi":"10.1007/s00467-025-06900-4","DOIUrl":"10.1007/s00467-025-06900-4","url":null,"abstract":"","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"257"},"PeriodicalIF":2.5,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144668080","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":"Adequacy and safety of pediatric native kidney biopsy using 16- and 18-gauge needles.","authors":"Petr Ananin, Anastasiia Milovanova, Kirill Kulikov, Ekaterina Stolyarevich, Alexey Tsygin","doi":"10.1007/s00467-025-06973-1","DOIUrl":"10.1007/s00467-025-06973-1","url":null,"abstract":"<p><strong>Background: </strong>The native kidney biopsy is an important diagnostic procedure in pediatric nephrology. Recent meta-analyses did not find the size of the needle as a risk factor for bleeding complications, but they were predominantly based on adult studies. There are few papers comparing the safety and core adequacy in pediatric native kidney biopsy.</p><p><strong>Methods: </strong>We present a large single-center retrospective study performed in a tertiary pediatric nephrology center. Data of children who received a real-time ultrasound-guided native kidney biopsy with a 16- or an 18-gauge needle from 2018 to 2024 were analyzed.</p><p><strong>Results: </strong>Overall, 1040 children (644 boys) were included, with a median age of 10.25 (6.6; 14.23) years. One hundred three (9.9%) patients experienced bleeding complications. Perinephric hematoma was reported in 86 (8.3%) cases, gross hematuria in 18 (1.7%), and 3 (0.3%) children required transfusion. Multivariate regression analysis revealed the needle size (OR for 16-gauge 2.06, 95% CI 1.22-3.47, p = 0.007) as a risk factor for complications in the overall cohort and in children under 12 years old. The needle size did not affect complication rates in children aged 12-18 years. Inadequate kidney cores were reported in 37 (4.5%) cases; OR for 18-gauge needles (OR 5.08, 95% CI 1.07-24.21, p = 0.041) was found.</p><p><strong>Conclusions: </strong>Use of a 16-gauge needle reduces the risk of obtaining an inadequate core in comparison with an 18-gauge. An 18G needle has a safety advantage over a 16G needle in children younger than 12 years. A 16G needle is as safe as an 18G needle and should be used for native kidney biopsy in children older than 12 years.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"151-156"},"PeriodicalIF":2.5,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145125714","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":"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":"203-216"},"PeriodicalIF":2.5,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12686102/pdf/","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":"OA","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":"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":"239-247"},"PeriodicalIF":2.5,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12685979/pdf/","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":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Impact of kidney maturation on aminoglycoside exposure in term-born pediatric patients: an in silico study.","authors":"Felix J Meigel, Ana Catalina Alvarez-Elías, Rasha Hussein, Doris H Fuertinger","doi":"10.1007/s00467-026-07420-5","DOIUrl":"10.1007/s00467-026-07420-5","url":null,"abstract":"<p><strong>Background: </strong>Kidney function determines aminoglycoside clearance in early life, but its maturation is insufficiently reflected in weight- and age-based dosing. Using in silico studies, we evaluate how kidney function maturation and growth influence aminoglycoside exposure and associated toxicity risks across pediatric development.</p><p><strong>Methods: </strong>We performed an in silico pharmacokinetic study using a two-compartment model parameterized from pediatric data. Age-homogeneous virtual term-born pediatric cohorts (1 day to 12 years; total N = 10,000) were generated from WHO growth standards and reference values for measured glomerular filtration rates (mGFR). Primary analyses simulated guideline gentamicin dosing (4 mg/kg every 24 h in neonates, 7 mg/kg every 24 h in infants/children) and assessed peak (8-12, 15-20 mg/L) and trough (< 1, < 0.5 mg/L) targets on days 1-10. Amikacin and tobramycin were evaluated in secondary analyses.</p><p><strong>Results: </strong>Weight and mGFR exhibited different maturation trajectories. Despite guideline-based weight-normalized gentamicin dosing, substantial variation in target attainment was observed. Peak target attainment increased from 34.2% to 70.0%. Trough target attainment increased from < 10% to > 90%, peaking around 2 years of age. Marked age-related heterogeneity persisted within infants: trough target attainment increased > 65% in one year. Sensitivity analyses indicated that exposure was more responsive to changes in glomerular filtration than to weight.</p><p><strong>Conclusions: </strong>Glomerular filtration maturation is a dominant driver of aminoglycoside exposure in early life. Standard weight-based dosing does not ensure target attainment across the pediatric age range. This supports the development of physiology-informed, model-based dosing strategies accounting for glomerular filtration maturation to improve efficacy while reducing toxicity risks.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"4513-4523"},"PeriodicalIF":2.5,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13672625/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148397248","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":"New therapeutic hope for rare podocytopathies.","authors":"Yanqin Zhang, Fang Wang, Jie Ding","doi":"10.1007/s00467-026-07442-z","DOIUrl":"10.1007/s00467-026-07442-z","url":null,"abstract":"<p><p>Podocytopathies are kidney diseases caused by podocyte injury or dysfunction that drives proteinuria or nephrotic syndrome. A significant expansion in understanding of the complex causes and mechanisms of podocyte injury, as well as potential therapeutic approaches, has been achieved over the past two decades with rapid advances in genomics in both research and clinical practice. Podocytopathies associated with monogenic mechanisms account for approximately 30% of cases with steroid-resistant nephrotic syndrome (SRNS) or focal segmental glomerulosclerosis lesions (FSGS), with even higher proportions in pediatric patients. Podocytopathies related to each gene mutation are rare. However, recent developments in these rare podocytopathies contribute to an optimistic outcome for the future to eventually avoid kidney failure. This review therefore focuses on aspects of new advances of therapies in rare podocytopathies with monogenic causes.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"4355-4367"},"PeriodicalIF":2.5,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13672428/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148391328","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":"Serum lactate and capillary refill time as predictors of acute kidney injury after pediatric cardiovascular surgery: a prospective cohort study.","authors":"Vanesa A Ochoa Jimenez, Inés E Gómez Hernández, Ana María Portilla Buenaventura, Alejandro Padilla Guzman, Camilo José Cuadros Mateus, Christian Pinilla-Medina, Gustavo Adolfo Cruz Suarez, Carlos Alberto González Amador, Juan Fernando Vélez Moreno, María Del Pilar Duque Guevara, Jesús Aldemar Aguirre Recalde, Laura Torres Canchala, Jessica M Forero Delgadillo, Jaime Manuel Restrepo","doi":"10.1007/s00467-026-07303-9","DOIUrl":"10.1007/s00467-026-07303-9","url":null,"abstract":"<p><strong>Background: </strong>Acute kidney injury (AKI) is a frequent complication after pediatric cardiovascular surgery (CS) and is associated with higher morbidity and mortality. Early, modifiable predictors are needed to improve outcomes.</p><p><strong>Methods: </strong>We conducted a prospective cohort study at a high-complexity center in Cali, Colombia (November 2020-July 2022). Children aged 0-60 months undergoing CS and admitted to the pediatric cardiovascular ICU were included. AKI was defined and staged according to KDIGO criteria. Serum lactate and capillary refill time (CRT) were measured preoperatively, at ICU admission, and at 6, 12, and 24 h postoperatively. Univariate and multivariate Cox proportional hazards models were used to assess associations with AKI.</p><p><strong>Results: </strong>Of 127 patients, 14.17% developed AKI, 66.7% of which were severe. The 28-day mortality was 11.8% overall and 44.4% among patients with AKI. In the multivariate analysis, elevated serum lactate was associated with higher risk of AKI at 24 h postoperatively (HR 1.61; 95%CI 1.24-2.08). Prolonged CRT at ICU admission was also a significant predictor (HR 1.80; 95%CI 1.03-3.15). Higher surgical complexity (RACHS-1), vasopressor use, and low systolic blood pressure were also associated with AKI.</p><p><strong>Conclusions: </strong>In this cohort, postoperative tissue perfusion markers-elevated serum lactate and prolonged CRT-were associated with AKI following pediatric CS. These accessible bedside measures may support early risk stratification and targeted kidney protective strategies.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"4677-4688"},"PeriodicalIF":2.5,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13672414/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148562668","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":"Anti-nephrin antibodies in pediatric nephrotic syndrome: a new paradigm.","authors":"Anne K Mühlig, Felicitas E Hengel, Jun Oh, Nicola M Tomas, Tobias B Huber","doi":"10.1007/s00467-026-07292-9","DOIUrl":"10.1007/s00467-026-07292-9","url":null,"abstract":"<p><p>Circulating anti-nephrin antibodies have recently been identified as mediators in many cases of childhood nephrotic syndrome. After binding of the antibody to nephrin, a key component of the slit diaphragm, different intracellular signaling mechanisms lead to foot process effacement. These new insights into the pathophysiology of nephrotic syndrome may have the potential to influence clinical treatment strategies. First, the measurement provides a pathomechanism-specific serological biomarker of a previously clinically diagnosed renal disease. Second, it may have additional value for diagnosis and prognostication, as anti-nephrin antibodies are more common in steroid-sensitive forms and less frequently associated with multidrug-resistant forms. Furthermore, anti-nephrin autoantibodies have the potential to be used as biomarkers for disease activity and to estimate the risk of relapse before kidney transplantation, after conduction of appropriate clinical trials. Additionally, the detection of autoantibodies strengthens the role of B-cell targeting therapies and provides a potential rationale for further multicenter, prospective, randomized clinical trials using B-cell depletion to potentially reduce corticosteroid use and relapse rates in children. In the future, more targeted therapies such as the interference with downstream effects of antibody binding or the depletion of autoantigen-specific antibody producing cells might become possible. Finally, the detection of anti-nephrin autoantibodies provides an explanation for patients and their families with a previously elusive \"idiopathic\" disease in antibody positive patients. In summary, the identification of anti-nephrin antibodies in nephrotic syndrome now opens up new questions and paves the way for further experimental and clinical studies to improve diagnostic and therapeutic approaches.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"4281-4290"},"PeriodicalIF":2.5,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13672670/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147777743","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}