Pediatric Nephrology最新文献

{"title":"Tubular proteinuria due to hereditary endocytic receptor disorder of the proximal tubule: Dent disease and chronic benign proteinuria.","authors":"Nana Sakakibara, Kandai Nozu","doi":"10.1007/s00467-025-06745-x","DOIUrl":"10.1007/s00467-025-06745-x","url":null,"abstract":"<p><p>The proximal tubule has a highly efficient endocytic pathway dedicated to reabsorbing albumin and low-molecular-weight proteins that have passed through the glomerular filtration barrier. This pathway is dependent on multi-ligand receptors: megalin and cubilin. Abnormalities in genes associated with endocytosis in the proximal tubule can lead to tubular proteinuria, where the urine contains albumin and low-molecular-weight proteins. Dent disease is a hereditary X-linked disorder characterized by low-molecular-weight proteinuria, hypercalciuria, nephrocalcinosis, nephrolithiasis, and progressive kidney dysfunction, often leading to CKD stage 5. CLCN5 is the gene responsible for Dent disease-1 and encodes the voltage-gated chloride channel ClC-5. Meanwhile, OCRL is the causative gene of Dent disease-2 and encodes phosphatidylinositol 4,5-bisphosphate 5-phosphatase, and its variants are also associated with Lowe syndrome. ClC-5 and OCRL are essential to the endocytic machinery, and their loss affects endosomal acidification and trafficking, resulting in disruption of megalin and cubilin recycling. CUBN, which encodes cubilin, was originally identified as the causative gene of Imerslund-Gräsbeck syndrome, a disorder of megaloblastic anemia associated with proteinuria. However, recently, a biallelic C-terminal variant of CUBN was shown to be responsible for isolated proteinuria without kidney dysfunction. This proteinuria is recognized as a new disease concept called chronic benign proteinuria (proteinuria, chronic benign: PROCHOB), which contradicts the common belief that proteinuria is harmful and ultimately leads to kidney damage. This article deepens the understanding of genetic tubular proteinuria and its origins, focusing on the role of megalin- and cubilin-mediated endocytosis in the proximal tubule.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"3367-3377"},"PeriodicalIF":2.6,"publicationDate":"2025-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12484296/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143753767","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":"Successful living donor kidney transplantation in a 14-year-old boy with Fontan circulation.","authors":"Satoshi Okada, Koichi Kamei, Kentaro Nishi, Masao Ogura, Hiroshi Ono, Seisuke Sakamoto","doi":"10.1007/s00467-025-06826-x","DOIUrl":"10.1007/s00467-025-06826-x","url":null,"abstract":"<p><p>We report successful living donor kidney transplantation (KT) in a 14-year-old boy with Fontan circulation. He was diagnosed with complete transposition of the great arteries (type 3) at birth. After Blalock-Taussig shunt placement, he developed acute kidney injury, which recovered to stage 4 chronic kidney disease. At 2 years old, he underwent a single-stage Fontan procedure to reduce the number of surgeries. At 7 years old, he started peritoneal dialysis. At 13 years old, the feasibility of KT was evaluated using preoperative cardiac catheterization with fluid loading, revealing that adequate systemic perfusion could be maintained during KT. Following a multidisciplinary preoperative assessment, KT was successfully performed under meticulous hemodynamic monitoring. At 1-year post-KT, the patient showed good kidney function (estimated glomerular filtration rate: 78 mL/min/1.73 m²). This case demonstrates that selected patients with Fontan circulation can safely undergo KT through appropriate preoperative assessment and perioperative management.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"3403-3406"},"PeriodicalIF":2.6,"publicationDate":"2025-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144199800","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":"Challenges in blood pressure measurement in children with obesity: focus on the cuff.","authors":"Kleo Evripidou, Athanasia Chainoglou, Vasilios Kotsis, Stella Stabouli","doi":"10.1007/s00467-025-06678-5","DOIUrl":"10.1007/s00467-025-06678-5","url":null,"abstract":"<p><p>Childhood obesity is a well-known risk factor for primary hypertension (HTN), especially during puberty. Validated automated oscillometric devices based on universal protocols using proper cuffs according to arm sizes must be selected for blood pressure (BP) measurement in clinical practice. Recent recommendations have addressed the importance of accurate BP measurement in patients with obesity. The American Heart Association (AHA) suggests using a cone-shaped cuff, while the European Society of Hypertension (ESH) emphasizes the significance of cuff characteristics based on arm size. The applicability of cone-shaped cuffs over cylindrical cuffs has been studied in adults with obesity. Most studies focused on the upper arm's shape and provided evidence that the use of cylindrical cuffs may overestimate BP in individuals with obesity, while the conical cuff was proposed as a proper alternative. However, current validation studies for BP measurement devices have not included the arm's size as a recruiting criterion, which may compromise the accuracy of devices in individuals with obesity. This review discusses the role of arm characteristics in cuff selection and the potential role of conical cuffs as an alternative to commonly used cylindrical ones in BP measurement in pediatric patients with obesity.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"3359-3366"},"PeriodicalIF":2.6,"publicationDate":"2025-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12484095/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143190053","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":"Measuring and estimating the GFR in children: state of the art in 2025.","authors":"Hans Pottel, George J Schwartz","doi":"10.1007/s00467-025-06724-2","DOIUrl":"10.1007/s00467-025-06724-2","url":null,"abstract":"<p><p>Glomerular filtration rate (GFR) can be measured directly, or estimated from biomarkers like serum creatinine and cystatin C, or both. Measuring GFR in children is cumbersome, as it requires the intravenous injection of an exogenous filtration marker like iohexol, and several blood samples to determine the concentration-time decay curve. Serum creatinine (SCr) measurement is inexpensive and is part of the routine biochemical blood tests that are commonly requested in daily clinical practice. SCr-based estimated GFR is therefore still the most widely used test to obtain information on kidney function, although SCr varies with age and sex during childhood and GFR remains nearly constant over the 2-18-year age range. These issues are partially resolved by factoring SCr by height, or rescaling SCr by the median of healthy subjects, making interpretation of SCr and eGFR more straightforward. Cystatin C has become an interesting alternative kidney biomarker, and estimating GFR from cystatin C has therefore become more important. The aim of this review is to show recent advances in measuring and estimating GFR in children.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"3347-3357"},"PeriodicalIF":2.6,"publicationDate":"2025-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143605988","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":"Post-infectious glomerulonephritis among children before and during the COVID-19 pandemic.","authors":"Eman Nooreddeen, Faten Almuteri, Mona Hamza Syrafi, Ahmed Alarabi, Khamisa Almokali, Mesbah Alshumrani, Sultan A Alharbi, Suzan Ali Alhazmi, Mossab Elkheir Imery Omer, Abdullah Zeid Alsuheili, Aeshah Alyahiwi, Sawsan Albatati, Saeed Alzabali, Osama Safdar, Khalid Alhasan, Jameela A Kari, Enas M Ghulam, Amer Alshengeti, Ibrahim Sandokji","doi":"10.1007/s00467-025-06837-8","DOIUrl":"10.1007/s00467-025-06837-8","url":null,"abstract":"<p><strong>Background: </strong>Post-infectious glomerulonephritis (PIGN) is a common cause of glomerulonephritis in children worldwide. The coronavirus disease 2019 (COVID-19) pandemic impacted the epidemiology of several infectious diseases; however, little is known about its effect on the epidemiology of PIGN. This study compared the epidemiology, clinical presentations, and outcomes of childhood PIGN before and during the COVID-19 pandemic.</p><p><strong>Methods: </strong>This multicenter case control study involved six Saudi Arabian centers. Children aged 0-18 years diagnosed with PIGN from 2016 to 2023 were included. The clinical, laboratory, and outcome data of children diagnosed before and during the COVID-19 pandemic (2016-2019 and 2020-2023) were compared.</p><p><strong>Results: </strong>A total of 141 patients were enrolled. Patients who presented during the pandemic were younger, and the clinical presentation of PIGN was milder, particularly regarding the rate of oliguria, and the severity of proteinuria in patients with acute post-streptococcal glomerulonephritis. A lower median C3 level was found in the pre-COVID-19 pandemic cohort. More patients with high anti-streptolysin O antibody levels were noted before the pandemic, while more cases had positive GAS throat cultures during the pandemic.</p><p><strong>Conclusions: </strong>PIGN incidence decreased during the early COVID-19 pandemic (2020-2022), followed by a resurgence of cases with an altered seasonality pattern. During the pandemic, children with PIGN were younger and had milder disease severity.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"3415-3421"},"PeriodicalIF":2.6,"publicationDate":"2025-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144234714","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":"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":"3555-3562"},"PeriodicalIF":2.6,"publicationDate":"2025-11-01","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}

{"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":"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":"3447-3457"},"PeriodicalIF":2.6,"publicationDate":"2025-11-01","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":"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":"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":"3513-3519"},"PeriodicalIF":2.6,"publicationDate":"2025-11-01","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":"Acute interstitial nephritis associated with ondansetron.","authors":"Olga Charnaya, Elizabeth Goswami","doi":"10.1007/s00467-025-06816-z","DOIUrl":"10.1007/s00467-025-06816-z","url":null,"abstract":"<p><p>A 16-year-old male presented with seizure to an outside hospital and was found to have severe kidney dysfunction of unknown chronicity. He had dilute urine throughout the hospitalization and a negative serological work-up for acute glomerulonephritis. Exposure history was notable for 1-2 doses of ibuprofen and ondansetron use for nausea for the 3-4 days prior to the admission. All prior medications were discontinued, and he was started on levetiracetam for seizure management. Kidney function markedly improved on hospital day 4, and a planned kidney biopsy was cancelled. Kidney function continued to normalize after discharge. He experienced nausea again 10 days after discharge and took another dose of ondansetron with increased creatinine noted 1 day later. After discontinuation of ondansetron, his kidney function returned to and has remained normal.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"3399-3401"},"PeriodicalIF":2.6,"publicationDate":"2025-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12331191/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144199799","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":"Bilateral renal vein thrombosis in a preterm with Netherton syndrome.","authors":"Ana Roche-Gomez, Diana Voskanyan, Joanna Śladowska-Kozłowska, Giulia Bassanese, Claus Peter Schmitt","doi":"10.1007/s00467-025-06821-2","DOIUrl":"10.1007/s00467-025-06821-2","url":null,"abstract":"<p><p>Netherton syndrome (NS) is a rare autosomal recessive syndrome caused by mutations in the serine protease inhibitor of the Kazal type 5 (SPINK5) gene. Patients are characterized by the classic triad of congenital ichthyosiform erythroderma, a hair shaft abnormality termed trichorrhexis invaginata and atopic diathesis. These children require meticulous fluid and salt control from the first day of life to prevent kidney damage due to the defective skin barrier.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"3391-3393"},"PeriodicalIF":2.6,"publicationDate":"2025-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12484293/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144226150","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}