Pediatric Nephrology最新文献

{"title":"Evaluation of the comparative efficacy and safety of obinutuzumab and other follow-up strategies after rituximab failure in refractory nephrotic syndrome.","authors":"Haoyu Zha, Chaoying Chen, Juan Tu, Ling Wan","doi":"10.1007/s00467-026-07445-w","DOIUrl":"10.1007/s00467-026-07445-w","url":null,"abstract":"<p><strong>Background: </strong>Refractory nephrotic syndrome (RNS) in children remains difficult to manage, particularly in patients who fail to maintain sustained remission after rituximab (RTX). Optimal follow-up treatment strategies after RTX failure remain unclear. This study aimed to evaluate the efficacy of different follow-up treatment strategies after RTX failure.</p><p><strong>Methods: </strong>This retrospective study included children with RNS who failed to maintain sustained remission after a single course of RTX. According to subsequent treatment, patients were divided into three groups: anti-CD20-free maintenance therapy (ACF), RTX retreatment (RTX-R), and obinutuzumab treatment (OBZ). Patients in the ACF group received corticosteroids and/or other immunosuppressants without additional anti-CD20 biologics, whereas those in the RTX-R and OBZ groups received RTX or OBZ therapy respectively.</p><p><strong>Results: </strong>Twenty-eight children were included: 10 in the ACF group, 8 in the RTX-R group, and 10 in the OBZ group. At 6 months, the OBZ group demonstrated significantly higher relapse-free survival compared to both the RTX-R group (100% vs. 37.5%, P < 0.01) and the ACF group (100% vs. 30%, P < 0.01), with no difference between the RTX and ACF groups. At 52 weeks, relapse-free survival remained highest in the OBZ group (80.0%), compared with 25.0% in the RTX-R group and 30.0% in the ACF group. OBZ induced sustained peripheral B-cell depletion, with B cells remaining undetectable throughout the scheduled 6-month follow-up. No serious adverse effects were observed in three groups.</p><p><strong>Conclusion: </strong>Obinutuzumab may be an effective and safe follow-up option for children with RNS who fail to maintain sustained remission after RTX.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"4561-4572"},"PeriodicalIF":2.5,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148405451","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 oral health indices and parental awareness in children with and without idiopathic nephrotic syndrome. A case-control study.","authors":"Sradha Madhu, Malini V, Jeby Jose Olickal, Anil Mathew","doi":"10.1007/s00467-026-07463-8","DOIUrl":"10.1007/s00467-026-07463-8","url":null,"abstract":"<p><strong>Background: </strong>Nephrotic syndrome (NS) is the most common pediatric kidney disorder and may adversely affect oral health. This study compared oral health status, parental awareness, and dietary habits in children with NS with those of healthy controls.</p><p><strong>Methods: </strong>A case-control study was conducted among children aged 5-14 years in a tertiary care center in Kerala, India, including 151 children with NS diagnosed per International Study of Kidney Diseases in Children criteria and 151 age-matched healthy controls. Oral health was assessed by evaluating decayed, missing and filled teeth (DMFT to indicate permanent dentition and dmft to indicate primary dentition), oral hygiene, gingival health and enamel defects.</p><p><strong>Results: </strong>Children with NS had significantly poorer oral hygiene (OHI-S: 0.70 ± 0.64 vs. 0.29 ± 0.54; p < 0.001) and greater gingival inflammation (GI: 0.39 ± 0.49 vs. 0.11 ± 0.31; p < 0.001). DMFT scores were higher in controls (1.48 ± 2.12 vs. 0.81 ± 1.72; p = 0.002). Age correlated positively with DMFT (NS: r = 0.268; non-NS: r = 0.401) and inversely with dmft (NS: r =  - 0.344; non-NS: r =  - 0.583; all p < 0.001). Children with NS had higher odds of infrequent dental visits (OR = 3.47, 95% CI 2.00-6.03) and regular brushing (OR = 3.47, 95% CI 2.00-6.03).</p><p><strong>Conclusions: </strong>Children with nephrotic syndrome (NS) have poorer oral hygiene and gingival health despite lower sugar intake and reported oral practices, underscoring the influence of dentition stage and the need for integrated dental care and targeted oral health education in pediatric NS management.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"4573-4579"},"PeriodicalIF":2.5,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148412631","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":"Urinary periostin as a novel non-invasive biomarker for kidney scarring in pediatric vesicoureteral reflux patients.","authors":"Reza Mohammadi Farsani, Amirreza Shamshirgaran, Seyyed Mohammad Ghahestani, Hossein Amirzargar, Fateme Guitynavard, Hossein Chivaee, Fardin Asgari, Mohammad Hadi Samadi, Parsa Nikoofar, Seyed Mohammad Kazem Aghamir","doi":"10.1007/s00467-025-06953-5","DOIUrl":"10.1007/s00467-025-06953-5","url":null,"abstract":"<p><strong>Background: </strong>Vesicoureteral reflux (VUR) is a common pediatric urological condition associated with kidney scarring, which can lead to hypertension, proteinuria, and chronic kidney disease. Current diagnostic methods, such as 99mTc-dimercaptosuccinic acid (DMSA) scans, are costly, involve radiation, and fail to detect early fibrosis. Urinary periostin, an extracellular matrix protein upregulated in kidney fibrosis, holds promise as a non-invasive biomarker for kidney scarring in VUR patients. This study aimed to evaluate urinary periostin as a biomarker for kidney scarring in children with VUR and compare its diagnostic performance with DMSA scans.</p><p><strong>Methods: </strong>This prospective case-control study enrolled 60 children (35 males and 25 females) aged between 1 and 140 months (with mean (SD) of 43.7 (34.45)) with VUR (30 with kidney scarring [Scar+] and 30 without [Scar-]). Urinary periostin levels were measured via ELISA and corrected with urine creatinine. DMSA scans confirmed scarring. Receiver operating characteristic (ROC) analysis assessed periostin's diagnostic accuracy, and logistic regression identified predictors of scarring.</p><p><strong>Results: </strong>Urinary periostin levels were significantly higher in Scar+ patients (27.4 ± 6.64 ng/mL) than in Scar- patients (18.1 ± 4.63 ng/mL, p < 0.001). ROC analysis yielded an area under the curve of 0.869, with a 21.5 ng/mL cutoff showing 80.0% sensitivity and 76.6% specificity. Periostin (OR 1.44, p < 0.001) was the only independent predictor of scarring. Periostin levels correlated with VUR grade (r = 0.63, p < 0.001).</p><p><strong>Conclusions: </strong>Urinary periostin is a highly sensitive and specific non-invasive biomarker for detecting kidney scarring in VUR, potentially reducing reliance on DMSA scans.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"109-118"},"PeriodicalIF":2.5,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145177139","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":"Performance of GFR estimation equations in pediatric hematology-oncology patients.","authors":"Tyler J Benning, Asmaa Ferdjallah, Laura M Dinnes, Timothy S Larson, Silvia M Titan, Christian Hanna","doi":"10.1007/s00467-025-06857-4","DOIUrl":"10.1007/s00467-025-06857-4","url":null,"abstract":"","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"249-251"},"PeriodicalIF":2.5,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144326414","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":"Kidney disease and surveillance testing in children with Bardet-Biedl syndrome: an administrative data study.","authors":"Gal Finer, Sarah E Ward, Jeremy Pomeroy, Erica Davis, Sriram Ramgopal","doi":"10.1007/s00467-026-07470-9","DOIUrl":"10.1007/s00467-026-07470-9","url":null,"abstract":"","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"4721-4723"},"PeriodicalIF":2.5,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13672443/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148448530","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 childhood and parental smoking and substance use on nephrotic syndrome outcomes: a prospective cohort study.","authors":"Tanay Joshi, Cal H Robinson, Nowrin Aman, Tonny H M Banh, Josefina Brooke, Valentina Bruno, Rahul Chanchlani, Vaneet Dhillon, Mackenzie Garner, Sophia Gorgiev, Leo Levin, Christoph Licht, Ashlene M McKay, Rachel Pearl, Seetha Radhakrishnan, Nithiakishna Selvathesan, Chia Wei Teoh, Jovanka Vasilevska-Ristovska, Hubert Wong, Rulan S Parekh","doi":"10.1007/s00467-026-07480-7","DOIUrl":"10.1007/s00467-026-07480-7","url":null,"abstract":"<p><strong>Background: </strong>The impact of environmental exposures such as household and adolescent smoking, alcohol use, and drug use on childhood nephrotic syndrome outcomes is unknown. Our objective was to characterize tobacco, alcohol, and recreational drug use and longitudinal associations.</p><p><strong>Methods: </strong>Analyzing data from Insight into Nephrotic Syndrome: Investigating Genes, Health, and Therapeutics, a prospective cohort, we included children (1-18 years) diagnosed with nephrotic syndrome from 1996 to 2023. Tobacco, alcohol, and recreational drug use were self-reported at annual study visits, treated as time-varying exposures. We evaluated prevalence and quantity of exposure to each substance and associations with relapse rate, steroid-sparing medication initiation, and hospitalizations using generalized linear mixed models.</p><p><strong>Results: </strong>We included 732 children with nephrotic syndrome (total of 2065 study visits). Median age at diagnosis was 3.8-years (IQR 2.7-6.3), 461 (73%) were male, and 692 (96%) were steroid-sensitive at diagnosis. Exposure to a household tobacco smoker was reported in 114 (16%) participants. Eight adolescents (1%) reported personal tobacco smoking, 33 (5%) used alcohol, and 10 (1%) used recreational drugs at any time. Personal or household tobacco smoking was not associated with relapse rate (adjusted rate ratio 1.22, 95% CI 0.96-1.54), steroid-sparing medication initiation (adjusted odds ratio 0.97, 95% CI 0.66-1.42), or hospitalization (adjusted odds ratio 0.66, 95% CI 0.31-1.37). Youth alcohol use was also not associated with nephrotic syndrome outcomes.</p><p><strong>Conclusions: </strong>Up to 5% of youth with nephrotic syndrome use tobacco, alcohol, or recreational drugs, and 16% are exposed to a household smoker. While this study was underpowered to evaluate the independent impact of personal tobacco and recreational drug use, no clear association between substance exposure and longitudinal outcomes was identified.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"4703-4712"},"PeriodicalIF":2.5,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148584894","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":"Chronic hyponatraemia with a reset osmostat: when abnormal is normal.","authors":"Hannah Butaye, Lotte Hoskens, Detlef Bockenhauer","doi":"10.1007/s00467-026-07449-6","DOIUrl":"10.1007/s00467-026-07449-6","url":null,"abstract":"<p><p>Hyponatraemia is one of the most common electrolyte abnormalities encountered in clinical practice, seen in up to 30% of hospitalised children. A key part of the evaluation of hyponatraemia concerns the assessment of volume status. Whereas hypovolaemia indicates a deficit in salt, euvolaemic hyponatraemia is most commonly associated with excess water. An important yet often forgotten differential diagnosis in chronic euvolaemic hyponatraemia is a reset osmostat, where patients regulate their plasma osmolality around a set point below the normal range. Here we present a case of an adolescent with chronic hyponatraemia and how we arrived at a diagnosis of a reset osmostat through simple diagnostic tests.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"4409-4411"},"PeriodicalIF":2.5,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148353044","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":"Obinutuzumab as a primary anti-CD20 agent in pediatric frequently relapsing and steroid-dependent nephrotic syndrome.","authors":"Ancuta Caliment, Emmanuelle Andlauer, Sara Macchi, Olivier Niel","doi":"10.1007/s00467-026-07425-0","DOIUrl":"10.1007/s00467-026-07425-0","url":null,"abstract":"<p><strong>Background: </strong>Idiopathic nephrotic syndrome (INS) is the leading glomerular disease in children, often responding well to steroids. However, frequently relapsing nephrotic syndrome (FRNS) or steroid-dependent nephrotic syndrome (SDNS) require the introduction of steroid-sparing agents to mitigate steroid toxicity.</p><p><strong>Methods: </strong>This retrospective, pilot study including 6 patients with FRNS or SDNS assessed the efficacy and safety of obinutuzumab as a first line of anti-CD20 therapy.</p><p><strong>Results: </strong>Using one dose of obinutuzumab, complete remission was achieved in all 6 patients within 11 months. Remission rate was 100% up to 12 months post-injection, and 80% at both 24 and 56 months. Infusion-related reactions occurred in 83% of the patients, primarily mild and transient; one patient required immunoglobulin supplementation but no severe infections were noted.</p><p><strong>Conclusions: </strong>These preliminary results suggest that obinutuzumab is a promising first-line anti-CD20 therapy for FRNS and SDNS in children, offering sustained remission with a good safety profile.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"4525-4530"},"PeriodicalIF":2.5,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148405463","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":"Genetic mutation patterns in Indonesian children with primary steroid-resistant nephrotic syndrome.","authors":"Reza Fahlevi, Partini Pudjiastuti Trihono, Dina Muktiarti, Eka Laksmi Hidayati, Pustika Amalia Wahidayat, Cut Nurul Hafifah, Widya Eka Nugraha, Mentari Kasih","doi":"10.1007/s00467-026-07327-1","DOIUrl":"10.1007/s00467-026-07327-1","url":null,"abstract":"<p><strong>Background: </strong>Approximately 10-30% of pediatric cases of steroid-resistant nephrotic syndrome (SRNS) have pathogenic monogenic variants, particularly in primary SRNS. Identifying these variants aids in predicting outcomes, guiding treatment, and enabling genetic counseling. In Indonesia, data on genetic mutation patterns in pediatric SRNS are still limited.</p><p><strong>Methods: </strong>A cross-sectional study was conducted using whole-exome sequencing (WES) to detect variants of genetic origin in children diagnosed with primary SRNS at Indonesia's National Referral Hospital, Cipto Mangunkusumo. We assessed the relationship between genetic findings and clinical features, including age at onset, treatment response, kidney biopsy, and kidney function.</p><p><strong>Results: </strong>Among 60 children with primary SRNS, 9 subjects (15%) harbored pathogenic or likely pathogenic variants across 8 genes (CUBN, AVIL, TRPC6, INF2, COL4A4, LAMA5, INVS, and FGA). Variants included stop-gained (n = 4), missense (n = 4), splice site (n = 1), and frameshift (n = 1), with both autosomal recessive and dominant inheritance observed. Two subjects had compound heterozygous variants. Most variant-positive subjects were male (6/9), had disease onset before age 3, and showed diverse histology (FSGS, MCD, or mesangial/membranoproliferative lesions). Four showed extrakidney features: peripheral neuropathy, high myopia, microcephaly with short stature, and hearing loss. While 53.3% responded to cyclosporin, over half remained in non-remission.</p><p><strong>Conclusions: </strong>This study reveals the genetic and phenotypic heterogeneity of primary SRNS in Indonesian children. The identification of diverse pathogenic variants underscores the utility of WES, even in cases responsive to cyclosporin. Genetic testing should be integrated into routine SRNS evaluation, especially in atypical presentations or early-onset of the disease.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"4531-4545"},"PeriodicalIF":2.5,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148412601","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":"Informative censoring of the \"prolonged hypogammaglobulinemia\" outcome by relapse-triggered rituximab redosing.","authors":"Nashmia Faraz, Haider Imran, Abdul Basit","doi":"10.1007/s00467-026-07526-w","DOIUrl":"10.1007/s00467-026-07526-w","url":null,"abstract":"","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"4739-4740"},"PeriodicalIF":2.5,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148930437","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}