Pediatric Nephrology最新文献

{"title":"Association of intrapatient tacrolimus variability and concentration-to-dose ratio with outcomes in pediatric kidney transplantation.","authors":"Maral Baghai Arassi, Nora Fisch, Manuel Feißt, Kai Krupka, Britta Höcker, Alexander Fichtner, Nele Kanzelmeyer, Jens König, Anette Melk, Jun Oh, Lars Pape, Lutz T Weber, Marcus Weitz, Burkhard Tönshoff","doi":"10.1007/s00467-025-06872-5","DOIUrl":"https://doi.org/10.1007/s00467-025-06872-5","url":null,"abstract":"<p><strong>Background: </strong>Data on the relevance of tacrolimus intrapatient variability (TacIPV) and concentration-to-dose ratio (C/D ratio) as an approximation of tacrolimus metabolism for predicting outcomes in pediatric kidney transplant (pKTx) recipients are scarce.</p><p><strong>Methods: </strong>We conducted a multicenter retrospective study of 255 pKTx recipients from the CERTAIN registry. TacIPV was quantified as the coefficient of variation (CV%) during months 6-12 post-transplant. In addition, the C/D ratio, corrected for body surface area, was calculated for the first 6 months post-transplant. Cutoffs were determined by minimization of log-rank P values: 23% for TacIPV and 1.0 for C/D ratio. Rejection episodes were classified according to the Banff criteria in the period following marker quantification.</p><p><strong>Results: </strong>A total of 13,159 tacrolimus trough blood levels were analyzed, with a median of 52 (IQR, 41-63) measurements per patient. High TacIPV (> 23%) during months 6-12 post-transplant was associated with an increased risk of rejection beyond 12 months post-transplant (hazard ratio (HR) 1.04, 95% CI 1.01-1.06, P = 0.002; Kaplan-Meier analysis P = 0.002). Similarly, a low C/D ratio (< 1.0), i.e., rapid tacrolimus metabolism, during the first 6 months was associated with a higher risk of rejection between months 6 and 12 (inverse HR 3.13, 95% CI 1.01-9.09, P = 0.04; Kaplan-Meier analysis P = 0.011).</p><p><strong>Conclusions: </strong>This largest to date multicenter study determines pediatric-specific cutoff values for TacIPV and tacrolimus C/D ratio as a predictive marker for graft rejection. Patients with these risk factors should be closely monitored and their immunosuppressive therapy adjusted accordingly.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2025-07-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144675452","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":"Critique on \"Risk profile of promoters and inhibitors of kidney stone formation in paediatric inflammatory bowel disease\".","authors":"Abdul Aziz, Hamza Nasir, Jawad Ahmad","doi":"10.1007/s00467-025-06908-w","DOIUrl":"https://doi.org/10.1007/s00467-025-06908-w","url":null,"abstract":"","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2025-07-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144668081","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":"https://doi.org/10.1007/s00467-025-06900-4","url":null,"abstract":"","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2025-07-18","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":"Continuous kidney replacement therapy with CARPEDIEM^® of premature and low birth weight neonates from the French registry.","authors":"Jennifer Battista, Sergio Eleni Dit Trolli, Bruno Ranchin, Justine Bacchetta, Julien Frederic Baleine, Danièle De Luca, Stéphane Decramer, Carole Enoch, Camille Faudeux, Marc Fila, Giulia Regiroli, Claus Peter Schmitt, Julie Bernardor","doi":"10.1007/s00467-025-06896-x","DOIUrl":"https://doi.org/10.1007/s00467-025-06896-x","url":null,"abstract":"<p><strong>Background: </strong>Acute kidney injury (AKI) affects 30% of hospitalized pediatric patients, with high mortality in neonates. The Cardio-Renal Pediatric Dialysis Emergency Machine^® (CARPEDIEM^®) is a continuous kidney replacement therapy (CKRT) device designed for infants weighing 2.5-9.9 kg.</p><p><strong>Methods: </strong>We retrospectively evaluated the technical feasibility, efficacy regarding solute and fluid removal, tolerability and patient outcomes of CKRT with CARPEDIEM^® in preterm and low birth weight (LBW) neonates (< 2.5 kg) with AKI, treated in six French pediatric intensive care units.</p><p><strong>Results: </strong>Ten neonates with a median gestational age of 31 [interquartile 29-32] (range 25-38) weeks and a birth weight of 1.1 [IQ 1.0-1.7] (0.6-2.0) kilograms received continuous veno-venous hemofiltration (CVVH) during 22 sessions. CVVH was initiated at a median age of 6 [2-12] (1-72) days and a weight of 1.9 [1.5-2.4] (1.3-2.8) kg. All CVVH sessions achieved efficient blood purification. At CVVH initiation fluid overload was 29 [21-39] (11-68)% and improved until the end of treatment to 16[8-18] (0-40)% (p = 0.04). Thrombocytopenia, requiring platelet transfusion, and hypotensive episodes were the main complications observed in 14 and 13 sessions. No deaths occurred during the CARPEDIEM^® treatment but all except one neonate died 6 [1-9] (1-63) days later, mainly due to multi-organ failure or ethical considerations linked to severe brain injury.</p><p><strong>Conclusion: </strong>CVVH using CARPEDIEM^® is technically feasible and effective in neonates with a birth weight below 2.5 kg with AKI and multi-organ dysfunction with the potential to improve clinical management. Further studies are needed to define adequate timing, dosing, and the impact on patient outcome.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2025-07-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144643134","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 Zahid and Rehman.","authors":"Barbora Piteková, Ivan Hric, Jakub Zieg, Eva Baranovičová, Patrik Konopásek, Jakub Gécz, Paul J Planet, Viktor Bielik","doi":"10.1007/s00467-025-06871-6","DOIUrl":"https://doi.org/10.1007/s00467-025-06871-6","url":null,"abstract":"","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2025-07-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144643136","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":"Anti-CD19 chimeric antigen receptor T-cell therapy in a highly sensitized patient with focal and segmental glomerulosclerosis.","authors":"Isabella Guzzo, Marco Becilli, Andrea Cappoli, Pietro Merli, Raffaella Labbadia, Francesca Del Bufalo, Nicola M Tomas, Manuela Colucci, Tobias B Huber, Mattia Algeri, Marco Andreani, Francesco Emma, Franco Locatelli","doi":"10.1007/s00467-025-06884-1","DOIUrl":"https://doi.org/10.1007/s00467-025-06884-1","url":null,"abstract":"<p><strong>Background: </strong>High panel reactive antibody (PRA) titers are a significant challenge for patients undergoing kidney transplantation. Currently, no desensitization protocol has proven effective in preventing mid- and long-term graft loss. In the present study, we used anti-CD19 chimeric antigen receptor (CAR) T-cell therapy in an attempt to reduce PRA in a highly sensitized patient. The role of this therapy in preventing focal segmental glomerulosclerosis (FSGS) recurrence was also evaluated.</p><p><strong>Methods: </strong>An 18-year-old girl with primary kidney failure secondary to FSGS failed a first kidney transplant at age 4 years due to disease recurrence. Despite being listed in a special program for hyperimmune patients, she had not been offered a new kidney. She received a single infusion of anti-CD19 CAR T cells after lymphodepletion therapy. Anti-human leukocyte antigens (HLA) antibody titers were monitored before therapy and monthly thereafter.</p><p><strong>Results: </strong>PRA titers decreased progressively during the first 6 months after CAR T-cell therapy. Unexpectedly, after 5.5 months, the patient was offered a cadaveric kidney that was fully matched for HLA-A, HLA-B, HLA-C, HLA-DR, and HLA-DQ at the antigen level. However, she developed an early relapse of FSGS for which she started plasmapheresis, which prevented further monitoring of PRA titers.</p><p><strong>Conclusions: </strong>This case shows that a single infusion of anti-CD19 CAR T cells can induce a durable reduction of anti-HLA antibodies in highly sensitized patients. However, profound B-cell depletion did not prevent FSGS relapse.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2025-07-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144643133","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":"Language preference and acute care utilization in the first year after pediatric kidney transplant.","authors":"Yaritzy Michelle Astudillo, Sonia Solomon, Debora Matossian","doi":"10.1007/s00467-025-06868-1","DOIUrl":"https://doi.org/10.1007/s00467-025-06868-1","url":null,"abstract":"","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2025-07-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144643135","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":"Retrospective analysis of glucocorticoid therapy in pediatric immunoglobulin A nephropathy: Kidney outcomes and efficacy.","authors":"Heyan Wu, Zhengkun Xia, Lidan Zhang","doi":"10.1007/s00467-025-06845-8","DOIUrl":"https://doi.org/10.1007/s00467-025-06845-8","url":null,"abstract":"<p><strong>Background: </strong>The efficacy of glucocorticoid (GC) in the management of immunoglobulin A nephropathy (IgAN) remains highly controversial. The study was conducted to analyze the efficacy and kidney outcomes of GC in the treatment of pediatric IgAN.</p><p><strong>Methods: </strong>Using the follow-up data of children with chronic kidney disease from the Department of Pediatrics at Jinling Hospital between January 2000 and December 2020, we selected children with primary IgAN who were ≤ 18 years old, confirmed by kidney biopsy, and had undergone regular follow-up for more than 2 years. Patients who had previously used other immunosuppressive agents or had not received renin-angiotensin system blocker (RASB) treatment were excluded. The selected patients were divided into two groups based on their prior treatment regimens: the GC + RASB group and the RASB group. The primary outcome was a composite of a 40% decrease in estimated glomerular filtration rate (eGFR) from baseline, kidney failure, or death due to kidney disease.</p><p><strong>Results: </strong>A total of 374 patients (149 females) were enrolled, with 230 in the GC + RASB group and 144 in the RASB group. At baseline, the GC + RASB group had lower albumin and higher creatinine levels (all P < 0.05). From 6 months of treatment, the GC + RASB group showed higher urinary protein remission rates (P < 0.05), but hematuria relief was similar between groups. Adverse events, including centripetal obesity, were more frequent in the GC + RASB group (P = 0.001). After a median follow-up of 130.97 months, the GC + RASB group had fewer endpoint events (5.22% vs. 11.11%, P = 0.035) and higher cumulative kidney event-free survival rates, particularly in patients with eGFR > 50 ml/min/1.73 m² and 24 h-UP ≥ 1 g/d (all P < 0.05).</p><p><strong>Conclusions: </strong>GC therapy reduced the risk of progression to kidney failure in children with initial eGFR > 50 ml/min/1.73 m² and proteinuria ≥ 1 g/d. No additional kidney event-free survival benefit was observed in children with eGFR ≤ 50 ml/min/1.73 m² or proteinuria < 1 g/d.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2025-07-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144637711","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":"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":"https://doi.org/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":""},"PeriodicalIF":2.6,"publicationDate":"2025-07-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","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":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The determinants of fluid accumulation in critically ill children: a prospective single-center cohort study.","authors":"Shannon Mohoric, Rashid Alobaidi, Tegan McGraw, Ari R Joffe","doi":"10.1007/s00467-025-06875-2","DOIUrl":"https://doi.org/10.1007/s00467-025-06875-2","url":null,"abstract":"<p><strong>Background: </strong>Fluid accumulation (FA) is associated with morbidity and mortality in intensive care. We aimed to determine sources of FA in critically ill children admitted to pediatric intensive care.</p><p><strong>Methods: </strong>Prospective cohort study of children in a university affiliated tertiary pediatric intensive care unit. Primary outcome was to describe contributors to fluid intake. Secondary outcomes were independent associations between fluid intake and FA > 5%, ventilator-free days, and intensive-care-free days.</p><p><strong>Results: </strong>Of patients admitted to intensive care, 99/120 (83%) met eligibility criteria. Median total fluid intake was median [interquartile range (IQR)] 46.9 [30.3, 72.1] ml/kg/day, and median [IQR] fluid output was 26.3 [15.1, 49.8] ml/kg/day. The largest contributors to fluid intake were maintenance (37.4%; IQR 20.0, 57.3), nutrition (23.2%; IQR 6.8, 58.1), medications (7.8%; IQR 2.9, 21.8), and resuscitative fluid (4.2%; IQR 0, 18). Children with peak FA > 5% versus FA ≤ 5% had higher total fluid intake (67.8 vs. 30.3 ml/kg/day; odds ratio (OR) 1.09 [95% confidence interval (CI), 1.06, 1.14)] and output [36.9 vs. 19.5 ml/kg/day; OR 1.04 (95% CI, 1.02, 1.06)], and higher volumes of maintenance, nutrition, and medications, but not resuscitative fluid. Total fluid intake was independently associated with FA > 5% (OR 1.09; 95% CI 1.05, 1.14; p < 0.001). At 28 days, peak FA% was independently associated with fewer intensive-care-free days [Effect Size - 0.30 (95% CI - 0.45, - 0.16), p < 0.001)].</p><p><strong>Conclusions: </strong>Higher fluid intake, rather than reduced output, was the predominant factor in FA, with maintenance fluid being the largest source of intake. Future research should evaluate the impact of optimized maintenance fluid calculations.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2025-07-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144626810","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}