Pediatric Nephrology最新文献

{"title":"Impact of early volume expansion on severe long-term kidney sequelae in children with Shiga toxin-producing Escherichia coli hemolytic uremic syndrome.","authors":"Paula A Coccia, Laura F Alconcher, Alejandro Balestracci, Ana Paula Spizzirri, Laura A Garcia Chervo, Iliana Principi, Aida Vazquez, Flavia B Ramirez, Elsa M Isern, Mariana Saurit, Gimena Ramirez, Maria Graciela Perez Y Gutierrez, Marta I Giordano, Adriana Aralde, Veronica S Selva, Nahir E Quijada, Jorge Montero, Andrea M Exeni, Sabrina Bronfen, Romina S Escalante, María Carolina Bettendorff, Diego B Ripeau, Diana Massó, Viviana M Ratto, Cecilia Cordoba, Jose Ojeda","doi":"10.1007/s00467-026-07465-6","DOIUrl":"10.1007/s00467-026-07465-6","url":null,"abstract":"<p><strong>Background: </strong>Early fluid management may influence disease severity and long-term kidney outcomes in children with Shiga toxin-producing Escherichia coli hemolytic uremic syndrome (STEC-HUS). We evaluated the association between early volume expansion and severe long-term kidney sequelae.</p><p><strong>Methods: </strong>We conducted a multicenter retrospective cohort study including children with STEC-HUS and severe acute kidney injury, defined by the need for dialysis for at least 48 h. Kidney outcome at follow-up was classified according to estimated glomerular filtration rate (GFR) as normal kidney function or chronic kidney disease stage 1 versus chronic kidney disease stages 2-5.</p><p><strong>Results: </strong>A total of 232 children were included. Median age was 2.34 years (interquartile range 1.6-4). At the end of follow-up (median 4.8 years, interquartile range 3.2-6.6), 72 patients (31%) had kidney sequelae corresponding to chronic kidney disease stages 2-5. Early volume expansion was performed in 102 patients (44%). These patients required dialysis for a shorter duration, had higher estimated GFR at follow-up, and less frequently developed chronic kidney disease stages 2-5. Lack of early volume expansion was independently associated with higher odds of kidney sequelae (odds ratio 2.46, 95% confidence interval 1.25-5.03), as was longer dialysis duration (odds ratio 1.10 per day, 95% confidence interval 1.05-1.15). Estimated GFR at follow-up was inversely correlated with dialysis duration; this association was not evident in patients who received early volume expansion but remained significant in those who did not.</p><p><strong>Conclusions: </strong>Early volume expansion and shorter dialysis duration were associated with long-term improved kidney outcomes.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"4627-4635"},"PeriodicalIF":2.5,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148536596","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-onset lipoprotein glomerulopathy in a 23-month-old boy with homozygous APOE Kyoto variant.","authors":"Julia S Donald, Hugh J McCarthy, Ali Moghimi, Deborah Lewis","doi":"10.1007/s00467-026-07458-5","DOIUrl":"10.1007/s00467-026-07458-5","url":null,"abstract":"<p><p>Lipoprotein glomerulopathy is a rare, inherited kidney disorder caused by pathogenic APOE variants that leads to obstruction of glomerular capillaries by lipoprotein thrombi, steroid-resistant nephrotic syndrome, and progressive chronic kidney disease. Immunosuppressive therapy is ineffective; instead, lipid-lowering measures reduce intraglomerular lipoprotein deposition and preserve kidney function. We describe a 23-month-old boy presenting with nephrotic syndrome and severe anaemia. Family history of kidney disease, parental consanguinity, and ultrasound showing enlarged echogenic kidneys led to early renal biopsy, which confirmed lipoprotein glomerulopathy. Genetic analysis revealed homozygosity for the APOE Kyoto variant and prompted discontinuation of prednisolone. Initiation of lipid-lowering therapy resulted in rapid clinical improvement. This case highlights the phenotype associated with homozygous APOE Kyoto variant and demonstrates how prompt kidney biopsy and genetic testing in atypical paediatric nephrotic syndrome can enable targeted treatment.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"4417-4421"},"PeriodicalIF":2.5,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148405448","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":"Renal maturation lays the physiological basis for precision aminoglycoside dosing in pediatrics.","authors":"Kun Cheng, Si-Yu Chen, Li Yang","doi":"10.1007/s00467-026-07489-y","DOIUrl":"10.1007/s00467-026-07489-y","url":null,"abstract":"","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"4737-4738"},"PeriodicalIF":2.5,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148763472","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 spectrum, genetic variants, and outcomes of autosomal recessive polycystic kidney disease in Omani children: two-center experience.","authors":"Mohamed S Al Riyami, Khadija Al Wahaibi, Hiba Ahmed, Maryam Ourdoucen, Maryam Al Azani, Anisa Al Maskari, Asma Alfar, Badria Al Ghaithi, Suliman Al Saidi, Intisar Al Alawi, Naifain Al Kalbani, Anwar Al-Omairi","doi":"10.1007/s00467-026-07418-z","DOIUrl":"10.1007/s00467-026-07418-z","url":null,"abstract":"<p><strong>Background: </strong>Autosomal recessive polycystic kidney disease (ARPKD) is a rare inherited disorder characterized by kidney and hepatic involvement, often presenting in early life. Data from Arab populations are limited, particularly in Oman, where consanguinity is common and may influence genotype-phenotype correlations.</p><p><strong>Methods: </strong>This retrospective study included Omani children (0-13 years) diagnosed with ARPKD between January 2011 and December 2024 at two national tertiary centers. Clinical features, genetic variants, kidney and hepatic outcomes, and mortality were analyzed. Kidney and hepatic severity were compared by genotype, and kidney and patient survival were assessed using Kaplan-Meier analysis.</p><p><strong>Results: </strong>A total of 114 patients were included; 58.9% presented before 6 months, and 64% had parental consanguinity. Hypertension occurred in 55.3%, 60% developed chronic kidney disease (CKD), and 12.3% progressed to kidney failure (KF). Hepatic complications were frequent, including hepatomegaly (65.8%) and portal hypertension (20.2%). Genetic testing (n = 81) revealed a limited PKHD1 variant spectrum predominantly c.107C > T, c.406A > G (novel), and c.4870C > T with no truncating mutations identified. 107C > T homozygosity was associated with more severe kidney and hepatic disease, while c.4870C > T correlated with milder kidney involvement. Overall mortality was 10.5% and was associated with early presentation (p < 0.05). Kaplan-Meier analysis showed high survival, with median survival not reached; estimated 5- and 10-year survival rates were 96.2% and 82.6%, respectively.</p><p><strong>Conclusions: </strong>ARPKD in Omani children typically presents early and is strongly associated with high rates of consanguinity and recurrent PKHD1 founder variants, with heterogeneous kidney and hepatic outcome. Early diagnosis, genetic counseling, and multidisciplinary care is essential in this setting.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"4433-4447"},"PeriodicalIF":2.5,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148272542","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":"Vancomycin exposure in critically ill children undergoing high intensity continuous renal replacement therapy.","authors":"Victor Belliard, Naim Bouazza, Baptiste Grenier, Mathilde Grapin, Valérie Nivet-Antoine, Noémie de Cacqueray, Jean-Marc Treluyer, Romain Berthaud, Sylvain Renolleau, Mehdi Oualha, Agathe Béranger","doi":"10.1007/s00467-026-07452-x","DOIUrl":"10.1007/s00467-026-07452-x","url":null,"abstract":"<p><strong>Background: </strong>This study aimed to describe vancomycin concentrations in critically ill children undergoing continuous renal replacement therapy (CRRT) and to identify factors impacting vancomycin concentrations.</p><p><strong>Methods: </strong>Monocentric retrospective study. Children under 18 years of age, who underwent CRRT from 2020 to 2023 and received vancomycin with at least one concentration measurement on CRRT were included. Patients receiving ECMO were excluded. Demographic, clinical and biological data were collected from the patients' medical records, including CRRT modalities and flow rate parameters. Regarding vancomycin, we collected dosing regimen, times and duration of infusions, duration of treatment, times of sampling, and plasma concentrations. Associations between vancomycin concentrations and each covariate were analyzed using a linear mixed modelling approach including a random effect on patient and adjusted on the dosing regimen and infusion modality.</p><p><strong>Results: </strong>Forty-two patients were included with a total of 512 vancomycin samples. Dialysate and effluent flow rates were set at 63.1 (0-308) mL/h/kg and 65.5 (0-327) mL/h/kg, respectively, corresponding to a high intensity. Vancomycin underexposure was more frequent for patients on CRRT than off CRRT (n = 172/320, 54% versus n = 71/192, 37%, p < 0.001), with lower concentrations on CRRT compared to off CRRT (18.5 (0.5-89.9) vs. 21.3 (0.5-73) mg/L, p < 0.001). The factors decreasing vancomycin exposure were a lower body weight, higher effluent flow rate (EFR), residual urine output and lower hematocrit.</p><p><strong>Conclusions: </strong>Within the limitations of this retrospective study, lacking standardized vancomycin administration and therapeutic drug monitoring (TDM) protocols, the present study suggests that critically ill children receiving vancomycin and on CRRT are at higher risk of underexposure than those off CRRT. Vancomycin concentrations are impacted by CRRT flow rates, particularly effluent flow rate (EFR), reflecting CRRT intensity, with more frequent underexposure observed at very high clearance rates. Considering the large inter- and intraindividual variability and the narrow therapeutic range, TDM and population pharmacokinetic modelling could be helpful to optimize and individualize vancomycin dosing in children undergoing CRRT.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"4501-4511"},"PeriodicalIF":2.5,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148405389","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":"Which patients should switch from rituximab to obinutuzumab in refractory nephrotic syndrome?","authors":"Shuichiro Fujinaga, Takahiro Ono, Aya Saito","doi":"10.1007/s00467-026-07540-y","DOIUrl":"10.1007/s00467-026-07540-y","url":null,"abstract":"","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"4741"},"PeriodicalIF":2.5,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148875409","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 commentary on \"Ultra-rare severe kidney dysplasia mimicking salt-wasting tubulopathy associated with TFCP2L1 gene variants\".","authors":"Manuel Vaqueiro Graña, Sara Gómez-Conde, Leire Madariaga","doi":"10.1007/s00467-025-06948-2","DOIUrl":"10.1007/s00467-025-06948-2","url":null,"abstract":"","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"259-260"},"PeriodicalIF":2.5,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12685987/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145040786","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":"Kidney health outcomes in children born very prematurely compared to full-term counterparts: a systematic review and meta-analysis.","authors":"Vaia Dokousli, Anastasia Stoimeni, Nikolaos Gkiourtzis, Despoina Samourkasidou, Vera Karatisidou, Nikolaos Charitakis, Kali Makedou, Christos Tsakalidis, George Koliakos, Despoina Tramma","doi":"10.1007/s00467-025-06797-z","DOIUrl":"10.1007/s00467-025-06797-z","url":null,"abstract":"&lt;p&gt;&lt;strong&gt;Background: &lt;/strong&gt;Advances in neonatal care have improved survival rates in preterm neonates. However, concerns persist regarding the long-term kidney implications of prematurity. Nephrogenesis is disrupted, particularly in those born very preterm (≤ 32 weeks of gestation), increasing the risk of early kidney dysfunction and hypertension later in life.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Objectives: &lt;/strong&gt;This systematic review and meta-analysis aimed to evaluate kidney health outcomes in former very preterm children and adolescents compared to full-term peers.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Data sources: &lt;/strong&gt;A systematic literature search was conducted in MEDLINE/PubMed, Scopus, and Web of Science from their earliest available records to October 9, 2024.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Study eligibility criteria: &lt;/strong&gt;We included observational studies comparing kidney health parameters between children/adolescents born very preterm (gestational age - GA ≤ 32 weeks) and their full-term counterparts (gestational age &gt; 36 weeks or birth weight &gt; 2000 g) within the age range of 6 to 18 years.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Participants and interventions: &lt;/strong&gt;Children and adolescents aged 6-18 years born very preterm were compared to their full-term counterparts. The analyzed kidney function markers included serum Cystatin C, serum creatinine (sCr), estimated glomerular filtration rate (eGFR) based on sCr (Cr-eGFR), and blood pressure (systolic and diastolic, SBP/DBP).&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Study appraisal and synthesis methods: &lt;/strong&gt;The Newcastle-Ottawa Scale was used to assess study quality. The mean difference with 95% confidence intervals was used for continuous outcomes. Statistical significance was set at p &lt; 0.05. Sensitivity, subgroup and meta-regression analyses were conducted for further exploration of the outcomes. Statistical analyses were performed using R software (Version 4.3.2).&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Results: &lt;/strong&gt;Thirteen studies (16 reports; 2,112 participants) were included. Very preterm children and adolescents had higher serum Cystatin C (0.05 mg/L; 95%CI: 0.02-0.08), lower Cr-eGFR (-11.87 mL/min/1.73 m&lt;sup&gt;2&lt;/sup&gt;; 95%CI: -22.44 to -1.31), and higher SBP (1.96 mmHg; 95%CI: 0.21-3.71). Sensitivity analysis confirmed Cystatin C findings but rendered Cr-eGFR and SBP differences non-significant. Subgroup analysis showed a significant GA effect on sCr (p &lt; 0.0001), though the ≥ 28 weeks subgroup included only two studies.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Limitations: &lt;/strong&gt;Considerable heterogeneity across studies persisted despite sensitivity and subgroup analyses. The lack of randomized controlled trials and longitudinal studies limits result interpretation, while non-significant meta-regression findings hinder full explanation of heterogeneity. Insufficient data prevented the assessment of additional kidney function parameters.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Conclusions and implications of key findings: &lt;/strong&gt;Cystatin C was elevated in very preterm individuals compared to full-term peers, reinforcing its role ","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"61-72"},"PeriodicalIF":2.5,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12686000/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144143246","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":"From survival to surveillance: the long-term kidney legacy of preterm birth.","authors":"Enrico Vidal, Daniele Trevisanuto","doi":"10.1007/s00467-025-06909-9","DOIUrl":"10.1007/s00467-025-06909-9","url":null,"abstract":"","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"17-21"},"PeriodicalIF":2.5,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144708414","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":"PROGRESS Study: Longitudinal analysis of antioxidant status and its relationship with heat shock proteins in children with chronic kidney disease.","authors":"Zeynep Nagehan Yuruk Yildirim, Sebahat Usta Akgul, Harika Alpay, Bagdagul Aksu, Fatma Savran Oguz, Aysel Kiyak, Nurver Akinci, Sevgi Yavuz, Gul Ozcelik, Asuman Gedikbasi, Ibrahim Gokce, Nese Ozkayin, Nurdan Yildiz, Cemile Pehlivanoglu, Nilufer Goknar, Seha Saygili, Sebahat Tulpar, Nuran Kucuk, Ilmay Bilge, Mehmet Tasdemir, Ayse Agbaş, Ahmet Dirican, Sevinc Emre, Ahmet Nayir, Alev Yilmaz","doi":"10.1007/s00467-026-07488-z","DOIUrl":"10.1007/s00467-026-07488-z","url":null,"abstract":"<p><strong>Background: </strong>Chronic kidney disease (CKD) progresses via complex mechanisms, including oxidative stress and impaired antioxidant defenses. We aimed to investigate longitudinal changes in serum and urinary levels of superoxide dismutase (SOD), catalase, and glutathione in children with CKD, and to assess their relationship with heat shock proteins (HSPs), which we have previously reported to be altered in this population.</p><p><strong>Methods: </strong>A total of 117 children with CKD and 56 healthy children were enrolled in the study. The CKD group was followed prospectively for 24 months. Serum and urinary levels of SOD, catalase, and glutathione were measured using ELISA at baseline, 12 months, and 24 months. Longitudinal changes and associations with kidney outcomes and CKD progression were analyzed using mixed-effects and logistic regression models.</p><p><strong>Results: </strong>Serum SOD and catalase levels, as well as urinary SOD, catalase, and glutathione levels were higher in the CKD group than in the control group. Serum and urinary SOD and catalase levels steadily decreased over time, similarly to urinary HSP levels. Most antioxidant biomarkers were not significantly associated with longitudinal kidney outcomes. However, higher longitudinal urinary catalase levels were modestly associated with better kidney function over time, and higher baseline urinary catalase was independently associated with a lower risk of CKD progression. In contrast, higher baseline serum SOD levels were associated with an increased risk of progression. Antioxidant biomarkers showed strong correlations with serum and urinary HSP levels.</p><p><strong>Conclusion: </strong>Although urinary catalase consistently associates with kidney function and CKD progression, most antioxidant biomarkers alone have limited predictive value. Children with CKD show altered antioxidant defenses that decline over time, and their correlations with HSPs suggest that they may act together in response to oxidative stress.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"4689-4702"},"PeriodicalIF":2.5,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148592932","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}