Pediatric Nephrology最新文献

{"title":"Acute kidney injury in paediatric kidney transplant recipients.","authors":"Barian Mohidin, Stephen D Marks","doi":"10.1007/s00467-025-06655-y","DOIUrl":"10.1007/s00467-025-06655-y","url":null,"abstract":"<p><p>Acute kidney injury (AKI) in paediatric kidney transplant recipients is common. Infection including urinary tract infection (UTI) and rejection are the most common causes in children. Surgical complications often cause AKI early post-transplant, whereas BK polyomavirus nephropathy rarely occurs in the first month post-transplant. Understanding kidney physiology helps to appreciate the sensitivity of the allograft to AKI, more so than native kidneys. Although the cause of AKI is often multi-factorial, there may be an underlying process that is treatable. Eliciting the aetiology, in this regard, is of paramount importance. Pre-renal and post-renal causes of allograft dysfunction are important to distinguish from intrinsic kidney disease. Clinical information and examination of fluid balance, urine dipstick testing, blood tests, bladder and kidney transplant ultrasound, and kidney transplant biopsy remain vital assessment tools in narrowing the differential diagnosis. A careful prescribed and recreational drug history is always warranted as many drugs including supplements are nephrotoxic. Additional causes such as allograft rejection, recurrent disease, and calcineurin inhibitor toxicity need to be considered in cases of allograft dysfunction, which would not affect the native kidneys. Early detection and assessment of AKI is crucial in promoting recovery. Significant progress has been made in specific pathologies over the last 20 years, which has improved kidney allograft survival rates considerably. Research into identifying AKI biomarkers to assist early diagnosis, before the serum creatinine rises, is ongoing.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"2161-2175"},"PeriodicalIF":2.6,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143059922","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 incidence and outcome of acute kidney injury during pediatric kidney tumor treatment-a national cohort study.","authors":"Paulien A M A Raymakers-Janssen, Gerrit van den Berg, Marc R Lilien, Inge A van Kessel, Alida F W van der Steeg, Marc H W A Wijnen, Mieke I Triest, Sophie E van Peer, Marjolijn C J Jongmans, Harm van Tinteren, Geert O Janssens, Marta Fiocco, Roelie M Wösten-van Asperen, Marry M van den Heuvel-Eibrink","doi":"10.1007/s00467-025-06684-7","DOIUrl":"10.1007/s00467-025-06684-7","url":null,"abstract":"<p><strong>Background: </strong>Acute kidney injury (AKI) is a serious complication of pediatric cancer treatment that is suggested to increase the risk of chronic kidney disease (CKD). Children with a kidney tumor may be at particular risk. This study aimed to determine the incidence and risk factors of AKI and its association with CKD during pediatric kidney tumor treatment.</p><p><strong>Methods: </strong>We analyzed data from a prospective national cohort of patients ≤ 18 years old diagnosed with a kidney tumor between 2015 and 2021 in the Princess Máxima Center for Pediatric Oncology in the Netherlands. AKI was defined according to KDIGO criteria. CKD was assessed 1 year post-treatment based on proteinuria and/or decreased estimated glomerular filtration rate (eGFR).</p><p><strong>Results: </strong>Of 147 patients, we observed AKI in 104 patients (71%) during therapy. AKI occurred most often within 48 h after tumor nephrectomy (88/104), while the rest had non-nephrectomy-related AKI from multifactorial causes. Sixteen patients experienced more than one AKI episode, and 92/104 episodes were reversible. Patients who developed AKI had a higher eGFR prior to surgery compared to those who did not develop AKI. CKD was observed in 16/120 patients (13%). Risk factors for developing CKD included the occurrence of at least 1 AKI event, the use of a > 3-drug regimen, and a lower eGFR at the start of treatment.</p><p><strong>Conclusion: </strong>The high incidence of AKI and its association with early CKD highlights the need for early detection, prevention, and intervention strategies during pediatric kidney tumor treatment.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"2393-2401"},"PeriodicalIF":2.6,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143449886","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 Dickkopf-related protein 3 as a novel biomarker for kidney function decline in children with Alport syndrome.","authors":"Jan Boeckhaus, Burkhard Tönshoff, Lutz T Weber, Lars Pape, Kay Latta, Henry Fehrenbach, Baerbel Lange-Sperandio, Matthias Kettwig, Sabine König, Ulrike John-Kroegel, Jutta Gellermann, Matthias Galiano, Sima Jami, Dennis Pieper, Gry Helene Dihazi, Angelika Hafke, Stefan Kohl, Max C Liebau, Jens König, Dieter Haffner, Oliver Gross, Manuel Wallbach","doi":"10.1007/s00467-025-06696-3","DOIUrl":"10.1007/s00467-025-06696-3","url":null,"abstract":"<p><strong>Background: </strong>Chronic kidney disease (CKD) seriously affects the well-being and shortens the life expectancy of children and adolescents, but its progression is challenging to predict. Therefore, there is an urgent need for biomarkers that can identify children at risk of faster CKD progression. Alport syndrome (AS) is the most common monogenetic glomerular kidney disease. Urinary Dickkopf-related protein 3 (DKK3) is associated with a decline in estimated glomerular filtration rate (eGFR) in adults and children with advanced CKD. However, its potential for early detection of CKD and its prognostic value in children with AS remain unknown.</p><p><strong>Methods: </strong>Urine samples from 49 children enrolled in the EARLY PRO-TECT Alport trial were analyzed to evaluate whether DKK3 could identify children with AS to be at risk for faster CKD progression.</p><p><strong>Results: </strong>DKK3 levels in patients with AS were higher than those of healthy individuals reported in the literature. DKK3 levels were more elevated in patients with later stages of AS. Furthermore, children who were not treated with renin angiotensin system inhibitors (RASi) had higher DKK3 levels than treated children. Children with above-average DKK3 levels were more likely to have increased albuminuria after 2 years of follow-up than children with below-average DKK3 levels.</p><p><strong>Conclusion: </strong>Urinary DKK3 is significantly elevated in children at early stages of AS. There was a potential association between higher DKK3 levels, worsening albuminuria, and a decline in kidney function. These findings suggest that DKK3 may be a prognostic marker for predicting the risk of kidney damage in children with AS.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"2205-2213"},"PeriodicalIF":2.6,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143190020","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":"A cost-effective innovation in anaemia management for paediatric patients with haemodialysis-dependent chronic kidney disease.","authors":"Rebecca Preston, Demetria Theodorou, Kate Sinnott, Dean Wallace, Amrit Kaur","doi":"10.1007/s00467-025-06680-x","DOIUrl":"10.1007/s00467-025-06680-x","url":null,"abstract":"<p><strong>Background: </strong>Paediatric patients undergoing haemodialysis typically require intravenous (IV) iron therapy to replenish iron stores. Upon establishing our home haemodialysis service, the need for an efficient IV iron administration method prompted exploration beyond the conventional use of iron sucrose, which is associated with anaphylaxis and requires frequent infusions. Ferric carboxymaltose has a favourable safety profile and corrects iron deficiency with less frequent infusions. We aimed to establish if ferric carboxymaltose was a viable alternative in this patient group.</p><p><strong>Methods: </strong>This single-centre, uncontrolled retrospective cohort study assessed the effectiveness of ferric carboxymaltose in maintaining laboratory parameters (haemoglobin level, transferrin saturation and reticulocyte haemoglobin content) within target range in our home haemodialysis population. Secondly, we conducted a comparative analysis to establish maintenance efficacy of ferric carboxymaltose, versus iron sucrose over a 12-month period. Finally, we performed a cost-effectiveness analysis of IV iron therapy, considering cost per dose and per month of treatment.</p><p><strong>Results: </strong>Following ferric carboxymaltose infusion, we observed significant increases in haemoglobin level, transferrin saturation and reticulocyte haemoglobin content, which was maintained at 3-month post-infusion. Ferric carboxymaltose demonstrated comparable efficacy to iron sucrose in maintaining laboratory parameters. Strikingly, ferric carboxymaltose treatment was associated with significantly decreased number of infusions per month (~ tenfold) and a significant cost-saving (~ fivefold).</p><p><strong>Conclusions: </strong>This study underscores the clinical efficacy and economic benefits of ferric carboxymaltose as a viable treatment for iron deficiency anaemia in paediatric patients who are haemodialysis-dependent and highlights the potential for significant improvements in healthcare delivery, in terms of reducing frequency of hospital visits for this patient population.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"2353-2361"},"PeriodicalIF":2.6,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143537617","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":"Voices of Resilience - integrating psychosocial support and music for children with kidney disease.","authors":"Alison Lap-Tak Ma, Fanny Tsz-Wai Ho, Eugene Yu-Hin Chan","doi":"10.1007/s00467-025-06766-6","DOIUrl":"10.1007/s00467-025-06766-6","url":null,"abstract":"","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"2413"},"PeriodicalIF":2.6,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143772889","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":"A \"Trial within a Cohort\" platform for pediatric clinical trials on idiopathic nephrotic syndrome: scope, objectives, and design of the retrospective-prospective cohort PIN'SNP.","authors":"Claire Bahans, Olivia Boyer, Olivier Dunand, Cyrielle Parmentier, Bruno Ranchin, Gwenaëlle Roussey, Charlotte Samaille, Stéphanie Tellier, Isabelle Vrillon, Evgenia Preka, Théo Mériguet, Astrid Dubrasquet, Lydia Ichay, Stéphanie Clavé, Julie Bernardor, Elodie Merieau, Claire Dossier, Vincent Guigonis","doi":"10.1007/s00467-025-06676-7","DOIUrl":"10.1007/s00467-025-06676-7","url":null,"abstract":"<p><strong>Background: </strong>Idiopathic nephrotic syndrome (INS) in children is the most common glomerular disease and is characterized by recurrent relapses. There is no community consensus on the treatment of relapsing forms of nephrotic syndrome in children, unlike that for the initial presentation. To date, available treatments only enable relapsing patients to be maintained in remission, rather than modifying the course of the disease; therefore, more therapeutic trials are needed. The Société de Néphrologie Pédiatrique (SNP) decided to implement within its French centers a national coordinated long-term clinical research program for children treated for INS based on a Trials within Cohorts (TwiCs) model. The aim of this paper is to describe the PIN'SNP cohort and research program as well as the TwiCs design adapted to INS research in the French regulatory system.</p><p><strong>Methods: </strong>This retrospective-prospective, multicenter research program will rely on a dynamic prospective cohort of children followed for an INS, known as the PIN'SNP cohort (i) to identify cases treated within SNP centers, (ii) to describe their clinical and epidemiological characteristics, and (iii) to provide a platform to nest prospective trials, and thus facilitate inclusion of patients in these future trials.</p><p><strong>Conclusions: </strong>The PIN'SNP cohort is the first French national pediatric platform dedicated to the implementation of randomized nested trials along with longitudinal and observational studies on INS in children. The adaptation of the TwiCs design to inform all eligible patients/parents to each nested trial will facilitate methodological robustness and ethical acceptability and reinforce communication between investigators and participants.</p><p><strong>Trial registration: </strong>number NCT04207580.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"2225-2238"},"PeriodicalIF":2.6,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143542840","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":"A 15-year experience highlighting the spectrum of Alport kidney disease in the pediatric population and novel genetic variants in COL4A3-5.","authors":"Nastja Andrejašič, Anja Blejc Novak, Mirjam Močnik, Nataša Marčun Varda, Špela Stangler Herodež, Danijela Krgović, Andrej Zupan, Anamarija Meglič","doi":"10.1007/s00467-025-06683-8","DOIUrl":"10.1007/s00467-025-06683-8","url":null,"abstract":"<p><strong>Background: </strong>Alport kidney disease (AKD) presents one of the most prevalent genetic kidney disorders, characterized by a complex genetic background and diverse clinical manifestations. This study aimed to review the clinical and genetic features of pediatric patients with COL4A3-5 variants and identify novel genetic variants.</p><p><strong>Methods: </strong>Data were collected retrospectively at a national level from pediatric patients up to 19 years old, who underwent genetic testing between 2008 and 2023. Patients with pathogenic and likely pathogenic COL4A3-5 variants were included. Their clinical, laboratory, and genetic characteristics were presented.</p><p><strong>Results: </strong>Over 15 years, 85 children and adolescents tested positive for pathogenic or likely pathogenic COL4A3-5 variants. Increasing incidence was noted as genetic testing became more prevalent. One patient (1.2%) progressed to kidney failure and six (7%) had extrarenal involvement. Pathogenic or likely pathogenic variants in COL4A3, COL4A4, and COL4A5 genes were found in 14 (16.4%), 34 (40.0%), and 37 (43.6%) patients, respectively. Patients were diagnosed with autosomal, X-linked, and digenic AKD in 55.2%, 43.6%, and 1.2%, respectively. Eight novel variants were recorded, and their associated phenotype presented.</p><p><strong>Conclusions: </strong>This study expands the genetic and clinical background of pediatric patients with AKD, presenting on a spectrum from mild hematuria to progressive chronic kidney disease. Genetic confirmation and risk stratification in the pediatric population are critical to ensure timely care and potentially slow down the progression of kidney disease.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"2215-2223"},"PeriodicalIF":2.6,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143190041","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":"Thick skin and thicker arteries: case report on a rare cause of hypertension.","authors":"Georgie Mathew, Srinivasavaradan Govindarajan, Swathi Kiran Shiri, Deepthi Rv, Gurijala Phaneendra Rao, Indira Agarwal","doi":"10.1007/s00467-025-06691-8","DOIUrl":"10.1007/s00467-025-06691-8","url":null,"abstract":"<p><p>Renovascular hypertension is the second leading cause of hypertension. Twenty-seven genes have been attributed to monogenic renovascular hypertension at present. We present a 15-year-old boy with facial dysmorphism, thick skin and renovascular hypertension with a novel gain-of-function variant in SMAD4 gene suggesting Myhre syndrome. Hypertension was controlled with three anti-hypertensive agents and vascular intervention is planned. It is characterized by a multisystem connective tissue disorder associated with progressive fibrosis of skin, cardiovascular, musculoskeletal, gastrointestinal and respiratory systems. This case highlights the need for recognizing syndromic features in children with renovascular hypertension.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"2197-2199"},"PeriodicalIF":2.6,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143047440","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":"Increased urinary podocyte mRNA loss in healthy early-term infants.","authors":"Kong Li, Xueou Liu, Lulu Zhang, Fangrui Ding","doi":"10.1007/s00467-024-06639-4","DOIUrl":"10.1007/s00467-024-06639-4","url":null,"abstract":"","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"2403-2405"},"PeriodicalIF":2.6,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142871787","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":"Listening to the multidisciplinary care team: exploring the pediatric palliative care needs in advanced chronic kidney disease.","authors":"Vanessa Nenner, Hendrik Napierala, Maria Agnes Jonas, Nina Kubiak, Julia Thumfart","doi":"10.1007/s00467-025-06728-y","DOIUrl":"10.1007/s00467-025-06728-y","url":null,"abstract":"<p><strong>Background: </strong>Pediatric palliative care (PPC) aims to improve the quality of life for children with life-limiting conditions, such as advanced chronic kidney disease (CKD), from the time of diagnosis. However, PPC is not commonly integrated into routine pediatric nephrology care. This study explores the perspectives and experiences of healthcare providers (HCPs) to better understand the experiences and specific barriers related to PPC integration for children and adolescents with advanced CKD.</p><p><strong>Methods: </strong>We conducted a qualitative study with 23 HCPs, including nurses, psychologists, social workers, and physicians from seven German pediatric nephrology centers, analyzing semi-structured focus groups and individual interviews using structured content analysis.</p><p><strong>Results: </strong>Five main categories emerged from the analysis, revealing HCPs' perceptions of CKD as a life-limiting condition, HCPs' moral distress in addressing end-of-life issues, and barriers to PPC integration. Although HCPs reported comprehensive multidisciplinary support for end-of-life situations, a lack of interprofessional communication occasionally hindered coordinated care. HCPs rarely addressed CKD's life-limiting nature proactively. A fear of diminishing hope led HCPs to avoid conversations about prognosis unless in response to a therapeutic crisis. PPC was mostly reserved for end-of-life cases, as HCPs associated PPC with terminal care and expressed concerns over distressing families.</p><p><strong>Conclusions: </strong>This study highlights the gap between guidelines recommending early integration of PPC and daily nephrology practice, which tends to introduce PPC late in the course of the disease. Training for nephrology teams could improve the quality of life for children with advanced CKD and their families by promoting early integration of primary PPC principles.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"2341-2351"},"PeriodicalIF":2.6,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143516130","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}