Pediatric Nephrology最新文献

{"title":"Unveiling long-standing low-molecular-weight proteinuria: A proximal tubular mitochondrial enzyme impairment caused by EHHADH mutation.","authors":"Hiroshi Tanaka, Toshiyuki Imasawa, Koji Tsugawa, Yuta Inoki, Morito Endo, Kandai Nozu","doi":"10.1007/s00467-025-07020-9","DOIUrl":"https://doi.org/10.1007/s00467-025-07020-9","url":null,"abstract":"<p><p>The energy requirement of renal proximal tubular cells depends on mitochondrial fatty acid β-oxidation. Enoyl-CoA hydratase-l-3-hydroxyacyl-CoA dehydrogenase (EHHADH) is a crucial enzyme in mitochondrial fatty acid oxidation, and mutations in EHHADH reportedly cause Fanconi syndrome. Here, we report the case of a 28-year-old Japanese woman with a long-term history of low-molecular-weight proteinuria (LMWP) who exhibited a missense mutation in the EHHADH gene. She was diagnosed with LMWP at 4 years of age. The search for the CLCN5 gene was unremarkable, and kidney biopsy revealed no significant tubulointerstitial changes. The LMWP persisted, and glucosuria gradually developed thereafter. Unexpectedly, next-generation sequencing identified a heterozygous missense mutation in the EHHADH gene. Her father, who harbored LMWP, also had the same mutation. Furthermore, immunostaining of the stored kidney specimens showed decreased immunoreactivity for cytochrome c oxidase subunit 4 in the proximal tubules, suggesting an underlying mitochondrial impairment.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2025-10-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145336744","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 transplantation in childhood cancer survivors.","authors":"Abigail S Kane, Wendy C Bravo","doi":"10.1007/s00467-025-06985-x","DOIUrl":"https://doi.org/10.1007/s00467-025-06985-x","url":null,"abstract":"<p><p>Advancements in pediatric cancer treatment protocols have significantly improved long-term survival. This has been accompanied by a growing recognition of morbidity and mortality associated with late effects of treatment, including kidney disease. Surviving cancer in childhood implies exposure to multiple nephrotoxic insults, some of which carry a greater risk for the development of chronic kidney disease and progression to kidney failure than others. In childhood cancer survivors who develop kidney failure, a kidney transplant is a potential life-prolonging treatment option. However, the optimal timing of transplant in relation to remission, particularly when weighing morbidity associated with increased time on dialysis against the risk of cancer recurrence associated with transplant immunosuppression, remains controversial. This review explores nephrotoxicity and kidney-related complications of cancer treatment, kidney disease screening guidelines, and considerations for kidney transplant in childhood cancer survivors including timing from remission to transplant, cancer recurrence in the setting of post-transplant immunosuppression, and transplant outcomes.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2025-10-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145329696","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":"Neutrophil extracellular traps drive peritoneal inflammation and tissue remodeling in pediatric peritoneal dialysis.","authors":"Charlotte Maria Dücker, Martin Herrmann, Susanne Boettcher, Sarah Bauer-Carmona, Raphael-Sebastian Schild, Lavinia Schönfeld, Laia Pagerols Raluy, Konrad Reinshagen, Claus Peter Schmitt, Maria Bartosova Medvid, Michael Boettcher","doi":"10.1007/s00467-025-07003-w","DOIUrl":"https://doi.org/10.1007/s00467-025-07003-w","url":null,"abstract":"<p><strong>Background: </strong>Peritoneal dialysis (PD) sustains children with chronic kidney disease stage 5 (CKD5) but promotes peritoneal membrane remodeling. Neutrophil extracellular traps (NETs) orchestrate antimicrobial defense and sterile inflammation; their involvement in PD-induced transformation is unknown.</p><p><strong>Methods: </strong>Forty-five children were enrolled in the International Pediatric Peritoneal Biobank. Peritoneal biopsies taken at PD initiation and after ≥ 12 months of low-glucose-degradation-product PD were compared with surgical biopsies from non-uremic peers. Histomorphometry quantified microvessel density, submesothelial thickness, leukocyte infiltration, collagen I/III, and NET markers (citrullinated histone H3, neutrophil elastase, myeloperoxidase). Dialysate and plasma collected every 2 months for 18 months were assayed for cell-free DNA, NET proteins, DNase1, and DNase1L3.</p><p><strong>Results: </strong>After chronic PD, the peritoneum displayed doubled microvessel density, tripled submesothelial thickness, and marked immune-cell infiltration (all p < 0.01). NET structures were prominent in tissue, while dialysate and plasma concentrations of cell-free DNA, citrullinated histone H3, neutrophil elastase, and myeloperoxidase increased two- to fourfold versus baseline (p < 0.05). DNase1 levels correlated with membrane thickness (r = 0.46, p = 0.003) and DNase1L3 with vascular density (r = 0.51, p = 0.001), suggesting limited compensatory NET clearance.</p><p><strong>Conclusions: </strong>Chronic PD elicits NET-driven sterile inflammation that parallels structural remodeling of the pediatric peritoneum. Supplementing PD fluids with exogenous NET-degrading enzymes may preserve membrane integrity and prolong PD suitability in children.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2025-10-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145313398","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":"Reassessing predictive markers for steroid sensitivity in pediatric nephrotic syndrome.","authors":"Muhammed Umer Javed, Muhammad Dawood Ghaffar","doi":"10.1007/s00467-025-07016-5","DOIUrl":"https://doi.org/10.1007/s00467-025-07016-5","url":null,"abstract":"","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2025-10-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145313304","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":"Improvisation, innovation, inequity: peritoneal dialysis in low-resource settings.","authors":"Francisco E Anacleto, Melvin Bonilla-Félix","doi":"10.1007/s00467-025-07015-6","DOIUrl":"https://doi.org/10.1007/s00467-025-07015-6","url":null,"abstract":"","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2025-10-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145313316","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":"Comparing German medical guidelines with recommendations by large language models using the example of hemolytic uremic syndrome (TMA).","authors":"Adrian Gieser, Samipa Pudasaini, Dominik Müller","doi":"10.1007/s00467-025-07008-5","DOIUrl":"https://doi.org/10.1007/s00467-025-07008-5","url":null,"abstract":"","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2025-10-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145313310","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":"Randomized cross-over comparison of double mini-PET with standard versus adapted dwell volumes and dwell times in children on chronic peritoneal dialysis.","authors":"Ariane Zaloszyc, Betti Schaefer, Maria Bartosova Medvid, Alberto Edefonti, Sara Testa, Fabio Paglialonga, Rukshana Shroff, Armelle Doutey, Johan Vande Walle, Thomas Lavaux, Ludovic Glady, Joris Delanghe, Matthijs Oyaert, Laura Friebus, Ivan Damgov, Michel Fischbach, Claus Peter Schmitt","doi":"10.1007/s00467-025-06993-x","DOIUrl":"https://doi.org/10.1007/s00467-025-06993-x","url":null,"abstract":"<p><strong>Background: </strong>Automated peritoneal dialysis (APD) consists of dwells with the same dwell volume and time. New cyclers allow modification of time and volume to prescribe adapted APD (AAPD), i.e., a series of short, small dwells followed by long, large dwells. Safety, efficacy, and underlying mechanisms of AAPD in children are uncertain.</p><p><strong>Methods: </strong>Two double mini-PET were performed in randomized sequence. The standard test consisted of two identical cycles (fill volume 1000 ml/m², 75 min) and the adapted test of a short, small cycle (600 ml/m² BSA, 30 min) followed by a long, large cycle (1400 ml/m², 120 min). Solute and water fluxes were quantified together with intraperitoneal pressure (IPP). Nine pediatric PD patients (5-21 years) were treated per protocol.</p><p><strong>Results: </strong>Residual dialysate volume was 422 ± 190 ml/m² BSA. There were no differences in ultrafiltration rates, glucose uptake, and creatinine, urea, and electrolyte clearances with the adapted and standard double mini-PET, despite identical cumulative dialysate volume and time. IPP varied by 1.7 ± 3.4 (range -2 to 9) cm H₂O with a drained volume of 1123 ± 386 and 1159 ± 210 ml/m² BSA for each standard dwell. IPP decreased from 1.9 with small volume to 1.0 cm H₂O /m²/100 ml with large volume dwells (p < 0.001) and was above 14 cm H₂O in 21 out of 63 measurements.</p><p><strong>Conclusion: </strong>Within the limitation of small patient numbers, this proof-of-concept study suggests similar ultrafiltration and clearance rates with a single adapted versus standard double mini-PET. High residual dialysate volumes and high IPPs highlight the challenges of AAPD prescription in children.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2025-10-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145308877","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":"Cautious interpretation of AKI predictors in pediatric pneumonia: A call for methodological rigor.","authors":"Hua Zhao","doi":"10.1007/s00467-025-07010-x","DOIUrl":"https://doi.org/10.1007/s00467-025-07010-x","url":null,"abstract":"","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2025-10-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145302415","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":"\"Why won't the ammonia go down?\": ammonia management while on continuous kidney replacement therapy.","authors":"Michelle C Starr, Jason Burnham, Michelle Voivoidas, Amy Wilson","doi":"10.1007/s00467-025-07006-7","DOIUrl":"https://doi.org/10.1007/s00467-025-07006-7","url":null,"abstract":"<p><strong>Background: </strong>Kidney replacement therapy (KRT) is commonly used to treat critically ill children for a variety of reasons, including hyperammonemia. KRT management in children with hyperammonemia not due to inborn errors of metabolism is challenging.</p><p><strong>Case presentation: </strong>We report a complex case of hyperammonemia in a 17-year-old critically ill female patient, emphasizing the challenges of management in a pediatric intensive care setting. Despite the initiation of kidney replacement therapy (KRT) and progressive increases in the prescribed dialytic dose, the patient's ammonia levels continued to escalate. This prompted a reevaluation of her metabolic needs, with a focus on optimizing glucose delivery to facilitate ammonia metabolism and dialytic clearance. Adjustments to increase the delivered glucose, along with careful monitoring of glucose removal during KRT, ultimately led to the stabilization of her ammonia levels.</p><p><strong>Conclusion: </strong>This case underscores the intricate interplay between metabolic support and dialytic strategies in the management of hyperammonemia. The use of a glucose delivery calculator is proposed. This case highlights the need for individualized, dynamic approaches in critically ill pediatric patients.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2025-10-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145308874","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":"Major contributors to musculoskeletal pain among children receiving hemodialysis.","authors":"Doaa Mosad Mosa, Mohamed Taman, Abobakr Abdelgalil, Doaa Shokry Alemam, Mohamed M Zedan, Mai S Korkor","doi":"10.1007/s00467-025-06964-2","DOIUrl":"https://doi.org/10.1007/s00467-025-06964-2","url":null,"abstract":"<p><strong>Background: </strong>Children who are undergoing hemodialysis are vulnerable to musculoskeletal (MSK) abnormalities. These conditions can potentially lead to functional impairments and diminished health-related quality of life. Hence, this study was to explore the prevalence of MSK pain in children receiving hemodialysis and the factors associated with this MSK pain.</p><p><strong>Methods: </strong>This cross-sectional study was conducted on a group of children undergoing hemodialysis at the nephrology unit, Mansoura University Children's Hospital, from December 2022 to January 2024. Patient demographics, clinical data, musculoskeletal findings, physical function, quality of life assessment parameters, laboratory data, and Dual Energy X-ray Absorptiometry (DEXA) results were collected. All patients were classified into two groups: patients with MSK pain and those without any MSK pain.</p><p><strong>Results: </strong>The total number of cases with MSK pain was 32 (64%). The most frequent musculoskeletal manifestations were: osteoporosis/osteopenia (64%), myalgia (64%), cramps (60%), arthralgia/arthritis (32%), and regional pain (32%). MSK pain was independently associated with longer dialysis duration, the presence of comorbidity, childhood health assessment questionnaire results, pediatric quality of life inventory, hemoglobin level, serum uric acid, calcium × phosphate product, and DEXA (Z-score).</p><p><strong>Conclusions: </strong>Involvement of the MSK system is a common morbidity in children with hemodialysis. Calcium × phosphate product (p = 0.026) and vitamin D level (p = 0.003) were the most significant factors associated with MSK pain in multivariate regression analysis.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2025-10-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145308875","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}