Pediatric Nephrology最新文献

{"title":"Feasibility and comparability in pediatric donation after circulatory death kidney transplantation.","authors":"Gaohua Cao, Yulong Yu, Kunsheng Wu","doi":"10.1007/s00467-026-07365-9","DOIUrl":"10.1007/s00467-026-07365-9","url":null,"abstract":"","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"4727-4728"},"PeriodicalIF":2.5,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148387946","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":"Hypogammaglobulinemia and B cell repopulation after rituximab in childhood nephrotic syndrome.","authors":"Cal H Robinson, Sophia Gorgiev, Nowrin Aman, Tonny H M Banh, Josefina Brooke, Valentina Bruno, Vaneet Dhillon, Mackenzie Garner, Christoph Licht, Ashlene McKay, Damien Noone, Rachel Pearl, Seetha Radhakrishnan, Keisha Rasool, Nithiakishna Selvathesan, Chia Wei Teoh, Jovanka Vasilevska-Ristovska, Rulan S Parekh","doi":"10.1007/s00467-026-07450-z","DOIUrl":"10.1007/s00467-026-07450-z","url":null,"abstract":"<p><strong>Background: </strong>Rituximab is increasingly used to treat childhood nephrotic syndrome, although its long-term effects are poorly understood. Our study aims to evaluate the incidence and risk factors for hypogammaglobulinemia, hematological complications, B cell repopulation, and relapses after rituximab treatment.</p><p><strong>Methods: </strong>We included children (1-18 years of age) with nephrotic syndrome who were enrolled in a prospective cohort study (Greater Toronto Area, Canada) and received rituximab from June 2018 to December 2023. We collected all immunoglobulin, hematology, and microbiology results from our institution. The primary outcome was hypogammaglobulinemia (IgG ≥ 2 standard deviations below the age-specific mean), classified as severe (IgG < 3 g/L) and/or prolonged (≥ 12 months). Secondary outcomes included other immunoglobulin and hematological parameters, B cell depletion, and relapses.</p><p><strong>Results: </strong>Seventy children with nephrotic syndrome received rituximab treatment. Pre-existing hypogammaglobulinemia was present in 46% of the patients. After rituximab, 68% of the patients had any, 32% had severe, and 46% had prolonged hypogammaglobulinemia. Younger age (odds ratio [OR] 1.13, 95% CI 1.01-1.29), European ethnicity (OR 6.37, 95% CI 1.74-30.83), lower pre-treatment IgG (OR 1.63, 95% CI 1.24-2.31) and longer maintenance immunosuppression duration (OR 1.27, 95% CI 1.03-1.61) were risk factors for prolonged hypogammaglobulinemia post-rituximab. Post-rituximab, 16% of the children had anemia, 21% had neutropenia, and 3% had thrombocytopenia. Twelve children (17%) developed documented infections. All children experienced complete B cell depletion post-rituximab, with B cell repopulation at median 6.3 months. Older age, prior calcineurin inhibitor treatment, and steroid sensitivity were associated with longer time-to-B cell repopulation.</p><p><strong>Conclusions: </strong>Prolonged hypogammaglobulinemia occurs in half of children with nephrotic syndrome post-rituximab. Younger age, European ethnicity, lower pre-treatment IgG, and longer maintenance immunosuppression use are associated with higher prolonged hypogammaglobulinemia risk.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"4597-4607"},"PeriodicalIF":2.5,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148422915","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":"Prenatal body fluid analysis in the evaluation of CAKUT.","authors":"Thomas Simon, Bénédicte Buffin-Meyer, Julie Klein, Stéphane Decramer, Joost P Schanstra","doi":"10.1007/s00467-026-07375-7","DOIUrl":"10.1007/s00467-026-07375-7","url":null,"abstract":"<p><p>Congenital anomalies of the kidney and urinary tract (CAKUT) are the leading cause of chronic kidney disease in children. Although prenatal ultrasound enables early detection, its ability to predict postnatal renal outcome, particularly in bilateral cases, remains limited due to substantial phenotypic variability and the inability of imaging to capture ongoing disease processes. Genetic testing improves etiological classification but shows weak genotype-phenotype correlations and limited prognostic value, reflecting the multifactorial nature of CAKUT and prompting investigation of molecular biomarkers in prenatal body fluids. This review summarizes current prenatal prognostic strategies in CAKUT, including imaging, genetics, and downstream molecular approaches such as transcriptomics, metabolomics, and proteomics, with particular emphasis on peptide-based profiling. Among these, prenatal peptide signatures derived from fetal urine or amniotic fluid have demonstrated strong prognostic performance for predicting postnatal renal outcome and may capture early molecular processes related to tissue remodeling and inflammation. However, translation into clinical practice remains challenging. Validation requires large prospective multicenter studies that are difficult to conduct in rare diseases and often lack sustained funding. In addition, implementation will likely rely on mass spectrometry-based assays in clinical laboratories. Despite these hurdles, integrating molecular peptide profiling with improved and standardized imaging approaches may enhance prenatal counselling and clinical decision-making in CAKUT.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"4311-4323"},"PeriodicalIF":2.5,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13672403/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148163971","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":"Touch contamination and associated peritonitis in children undergoing maintenance peritoneal dialysis.","authors":"Rebecca G Same, Natalie K Grills, Sreejata Dutta, Alicia M Neu, Bradley A Warady, Brandy Begin","doi":"10.1007/s00467-026-07455-8","DOIUrl":"10.1007/s00467-026-07455-8","url":null,"abstract":"<p><strong>Background: </strong>Infections are a leading cause of hospitalization and death in children receiving maintenance peritoneal dialysis (PD). Touch contamination is an established risk factor for development of peritonitis, but data regarding optimal management of touch contamination are limited.</p><p><strong>Methods: </strong>We conducted a retrospective cohort study using data submitted to the Standardizing Care to Improve Outcomes in Pediatric End Stage Kidney Disease (SCOPE) collaborative between 10/1/2011 and 7/31/2025 to evaluate touch contamination in children. Reported touch contamination events and their management, including administration of prophylactic antibiotics and transfer set changes, were evaluated. Univariate and multivariable generalized linear mixed modeling were used to assess relationships between demographic factors and episodes of touch contamination and between management of touch contamination events and temporally associated peritonitis. We also evaluated the microbiology of touch contamination-associated peritonitis and compared it to peritonitis not associated with touch contamination.</p><p><strong>Results: </strong>Of 2685 children included, 846 (32%) reported at least one touch contamination. In a multivariable model, younger age and non-Hispanic Black race were associated with increased rates of touch contamination. Administration of prophylactic antibiotics following touch contamination was associated with a lower prevalence of peritonitis (23% vs. 38%). Most episodes of touch contamination-associated peritonitis were Gram-positive (46%) or culture negative (24%).</p><p><strong>Conclusions: </strong>Touch contaminations are common in children undergoing maintenance PD. Administration of prophylactic antibiotics following touch contamination is associated with decreased risk of peritonitis.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"4589-4596"},"PeriodicalIF":2.5,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13672569/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148422998","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":"Genetic insights into congenital and infantile nephrotic syndrome: predicting severity and informing care.","authors":"Ewelina D Hejenkowska, Paul Kruszka, Agnieszka Swiatecka-Urban","doi":"10.1007/s00467-026-07407-2","DOIUrl":"10.1007/s00467-026-07407-2","url":null,"abstract":"<p><p>Nephrotic syndrome (NS) is the most common chronic glomerular disorder in pediatric populations, yet its manifestation during the neonatal or infantile period often portends a severe, treatment-resistant monogenic form. While recent studies reveal marked heterogeneity in the clinical course and severity of congenital and infantile NS, critical gaps remain in understanding its underlying mechanisms. This review synthesizes current evidence on the genetic landscape of the early-onset NS, highlighting how advances in next-generation sequencing and molecular diagnostics have refined genotype-phenotype correlations. We discuss how these insights are reshaping disease classification, enabling early prognostic stratification, and informing personalized management strategies. Emphasis is placed on the transformative role of genetic testing in guiding clinical care and uncovering novel therapeutic avenues for this complex pediatric kidney disorder.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"4335-4353"},"PeriodicalIF":2.5,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13410343/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148339568","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":"Thiamin deficiency in children with chronic kidney disease on peritoneal dialysis and its association with dialysis duration and transport peritoneal membrane status.","authors":"Wipawee Suwanboriboon, Thanaporn Chaiyapuk, Intraparch Tinnabut, Gornmigar Sanpawitayakul, Chatchawan Srisawat, Sarawut Junnu, Sompong Liammongkolkul, Kwanjai Chotipanang, Hathaichanok Rukprayoon, Phakwan Laohathai, Narumon Densupsoontorn","doi":"10.1007/s00467-025-06847-6","DOIUrl":"10.1007/s00467-025-06847-6","url":null,"abstract":"<p><strong>Background: </strong>Patients with chronic kidney disease (CKD) stage 5D receiving peritoneal dialysis (PD) are at risk for thiamin deficiency (TD). This study compared the proportion of TD in pediatric CKD patients undergoing PD with that in healthy controls and evaluated the associations of various factors with TD in CKD patients.</p><p><strong>Methods: </strong>Thirty-two patients with CKD stage 5D and 34 healthy children were recruited. The participants reported their consumption of foods containing antithiamin factors and completed a 3-day food record to assess their intake of thiamin, energy, and macronutrients. The medical records of the CKD group were reviewed. Thiamin status was assessed via an erythrocyte transketolase activity assay, where the thiamin pyrophosphate effect was determined.</p><p><strong>Results: </strong>Thirteen percent of participants in the CKD group had TD, whereas 29% of the healthy controls did (p = 0.093). The CKD group had significantly greater total thiamin intake per 1,000 kcal of energy due to thiamin supplementation (2.14 [1.83, 2.99] vs. 0.87 [0.59, 1.14] mg/1,000 kcal; p < 0.001), despite inadequate dietary thiamin intake. A longer PD duration (in months) and a high-transport peritoneal membrane status were significantly associated with poorer thiamin status (β = + 0.59, p < 0.001, and β = + 0.38, p = 0.013, respectively). In contrast, greater total thiamin intake was correlated with improved thiamin status (β = -0.35, p = 0.022).</p><p><strong>Conclusions: </strong>Thiamin deficiency was observed in 13% of pediatric CKD patients on PD and 29% of healthy controls. In CKD patients, TD was associated with longer PD duration (in months), high-transport peritoneal membrane status, and low total thiamin intake.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"177-184"},"PeriodicalIF":2.5,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12686079/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144476155","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":"Developmental origins of disease - Effects of iron deficiency in the rat developing kidney and beyond.","authors":"Anthony Babu, Whitley N Hulse, Matthew W Harer, Keri A Drake, Pamela J Kling","doi":"10.1007/s00467-025-06762-w","DOIUrl":"10.1007/s00467-025-06762-w","url":null,"abstract":"<p><p>Iron is an essential cofactor in metabolic and developmental processes. Iron deficiency (ID) is the most common micronutrient deficiency in pregnancy, especially impacting medically underserved populations worldwide. Iron deficiency (ID) in pregnancy predisposes neonates to poor iron status, i.e., congenital ID and associated adverse effects. The role of congenital ID on human kidney development is unstudied, but impaired fetal kidney development is possible. Both vascular and global nutrient restriction rat models report impaired fetal kidney development, as well as induce hypertension, supporting the developmental origins of health and disease (DOHaD) hypothesis. This review compiles findings from 17 published studies in rats examining congenital or early postnatal ID, showing the same. The review compares histological and physiological findings in both congenital and postnatal ID, placing these in the context of recent knowledge describing molecular mechanistic pathways regulating nephrogenesis. Findings in rat early-life ID include lower kidney iron levels, lower glomerular generations and estimated glomerular numbers, larger maculae densa size, interstitial fibrosis, and prolonging active glomerulogenesis past normal temporal cessation. Additionally, several physiological studies in rat congenital ID promote altered renin-angiotensin signaling and hypertension with maturation, especially in males. Key findings of morphological kidney maldevelopment, altered renin-angiotensin signaling, and hypertension in early-life ID underscore the urgent need for future mechanistic data in animals such as rats. The long-term goal would be to leverage understanding from these data into either preventative or early therapeutic strategies in children.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"23-32"},"PeriodicalIF":2.5,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12686096/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144049450","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":"Ambulatory arterial stiffness index among children and adolescents with chronic kidney disease: a report from the Chronic Kidney Disease in Children Study.","authors":"Susan M Halbach, Jennifer Roem, Michael F Schneider, Joshua Samuels, Susan L Furth, Bradley A Warady, Joseph T Flynn","doi":"10.1007/s00467-026-07461-w","DOIUrl":"10.1007/s00467-026-07461-w","url":null,"abstract":"<p><strong>Background: </strong>To quantify the association between ambulatory arterial stiffness index (AASI) and hypertensive status, and to identify additional predictors of AASI, including body mass index (BMI), proteinuria, and mineral metabolism, in children and adolescents with chronic kidney disease (CKD).</p><p><strong>Methods: </strong>We analyzed data collected from 654 participants during 1283 study visits in the Chronic Kidney Disease in Children (CKiD) prospective cohort study. AASI was calculated from ambulatory blood pressure monitoring (ABPM) data as 1 minus the slope coefficient from a least squares linear regression of diastolic blood pressure (BP) on systolic BP. Hypertensive status was determined by clinic BP and ABPM. Linear regression with generalized estimating equations to account for repeated AASI measurements over time within the same participant were used to quantify the relationship between AASI and hypertensive status.</p><p><strong>Results: </strong>At their first successful ABPM visit, the median age was 12 years, 61% were male, 15% had obesity and the median eGFR was 49 ml/min/1.73 m². Over half (57%) reported current antihypertensive therapy use with RAAS inhibitors, yet 42% met criteria for abnormal ABPM (elevated mean total/awake/sleep DBP/SBP per updated guidelines). Masked hypertension (MH) was common (27%) and associated with significantly higher AASI compared to being normotensive (difference in mean AASI: + 0.051, 95% CI: 0.032, 0.071). Male sex and higher BMI were associated with higher AASI, but the presence of proteinuria, abnormal serum calcium or phosphate were not.</p><p><strong>Conclusion: </strong>AASI is associated with MH and BMI in children and adolescents with CKD and may be a useful non-invasive measure of vascular stiffness in this population.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"4669-4676"},"PeriodicalIF":2.5,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13672448/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148562664","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":"Variants and variability - a cohort of Omani children sheds more light on autosomal recessive polycystic kidney disease.","authors":"Kathrin Burgmaier, Max C Liebau","doi":"10.1007/s00467-026-07490-5","DOIUrl":"10.1007/s00467-026-07490-5","url":null,"abstract":"","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"4249-4251"},"PeriodicalIF":2.5,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13672658/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148584883","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":"Screening of pediatric patients with congenital anomalies of the kidney and urinary tract for associated congenital heart disease in a tertiary care setting.","authors":"Sahar S Sheta, Rana Hendawy, Esraa Fathy, Shaimaa Sayed","doi":"10.1007/s00467-026-07454-9","DOIUrl":"10.1007/s00467-026-07454-9","url":null,"abstract":"<p><strong>Background: </strong>Congenital anomalies of the kidney and urinary tract (CAKUT) are among the most common birth defects and represent a leading cause of chronic kidney disease (CKD) in children. Congenital heart disease (CHD) is the most frequent severe congenital malformation worldwide. Given the shared mesodermal embryologic origin of both the renal and cardiovascular systems, an association between CAKUT and CHD has been proposed. We aimed to screen pediatric patients diagnosed with CAKUT for the presence of associated CHD.</p><p><strong>Methods: </strong>This cross-sectional analytical study included 300 pediatric patients with CAKUT. Demographic data, type of CAKUT and kidney function status were collected. All patients underwent cardiac evaluation and echocardiography to detect structural heart defects and assess cardiac function. CAKUT types and their association with CHD were analyzed.</p><p><strong>Results: </strong>The mean age of the cohort was 6.42 years, with a male predominance (68.7%). CHD was detected in 7% of patients, with atrial septal defect (33%) and ventricular septal defect (23%) being the most frequent lesions. 28.5% of these patients with CHD needed surgical intervention. The most common CAKUT subtype was vesicoureteral reflux (27.7%), and it was the most common CAKUT subtype associated with CHD. Fractional shortening impairment was strongly associated with advanced CKD.</p><p><strong>Conclusions: </strong>The coexistence of CHD among children with CAKUT could emphasize the need for cardiac evaluation and consider the echocardiographic screening in this population. Moreover, the observed link between advanced CKD stage and cardiac dysfunction supports the necessity of integrated nephro-cardiac care in these patients.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"4609-4616"},"PeriodicalIF":2.5,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13672461/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148422946","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}