Pediatric Nephrology最新文献

{"title":"Critical insights on post-infectious glomerulonephritis among children before and during the COVID-19 pandemic.","authors":"Sadeeq Khan, Abdur Rehman, Waheed Khan","doi":"10.1007/s00467-025-06886-z","DOIUrl":"10.1007/s00467-025-06886-z","url":null,"abstract":"","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"3591"},"PeriodicalIF":2.6,"publicationDate":"2025-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144529202","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":"Evidence-based risk stratification for neonatal acute kidney injury: a call to action.","authors":"Tahagod Mohamed, Nicole Asdell, Xia Ning, Jason G Newland, Matthew W Harer, Cara L Slagle, Michelle C Starr, John D Spencer, Francis P Wilson, David T Selewski, Jonathan L Slaughter","doi":"10.1007/s00467-025-06752-y","DOIUrl":"10.1007/s00467-025-06752-y","url":null,"abstract":"","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"3335-3339"},"PeriodicalIF":2.6,"publicationDate":"2025-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143720452","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":"Effect of losartan on uric acid metabolism in children with proteinuric kidney disease: crossover randomized controlled clinical trial.","authors":"Laura Beaudoin, Luciana Meni Battaglia, Sandra Mariel Martin, Ismael Toledo, Alejandro Balestracci","doi":"10.1007/s00467-025-06754-w","DOIUrl":"10.1007/s00467-025-06754-w","url":null,"abstract":"<p><strong>Background: </strong>Low uric acid (UA) levels are desirable in kidney disease. Enalapril is the most used reno-protective drug; losartan has a similar antihypertensive and antiproteinuric effect, but also induces hyperuricosuria due to tubular urate transporter 1 inhibition. As this effect has not been demonstrated in paediatrics, we assessed if losartan reduces serum UA in children, owing to an increase in its urinary excretion, compared to enalapril.</p><p><strong>Methods: </strong>Single-centre, open-label, crossover randomized trial. Patients aged 3-12 years with proteinuric kidney disease and estimated glomerular filtration rate ≥ 30 ml/min/1.73 m², were assigned to receive enalapril or losartan for 30 days. Then, all patients received 15-days of enalapril to washout the effect of losartan in those who received it. Subsequently, they were switched to the opposite treatment modality.</p><p><strong>Results: </strong>Forty patients were included (36 CKD stage 1, 4 stage 2), median age 8.58 years; median serum UA 4 mg/dL (IQR, 3.5-5.1). Losartan significantly increased median UA urinary fractional excretion from 7% (IQR 6-8.27) to 8.9% (IQR 6.3-11) (p < 0.001) and significantly reduced its median serum level from 4.2 mg/dL (IQR 3.4-4.9) to 3.6 mg/dL (IQR 2.9-4.5) (p < 0.001). Median urinary excretion [pre 6.65% (IQR 5-8.41) vs. post 7% (IQR 5.5-8.3), p = 0.61)] and median serum values [pre 4.2 mg/dL (IQR 3.6-5.1) vs. post 4.1 mg/dL (IQR 3.4-5), p = 0.42)] were comparable with enalapril. The decrease in serum UA levels post-losartan correlated with the increase in its urinary excretion (r = -0.33; p = 0.036).</p><p><strong>Conclusions: </strong>Losartan significantly increased UA urinary excretion along with the consequent reduction in its serum levels.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"3495-3503"},"PeriodicalIF":2.6,"publicationDate":"2025-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144507323","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":"In vitro measurements of ultrafiltration precision in hemofiltration and hemodialysis devices used in infants, Part 2: Comparison of PrisMax and CARPEDIEM with previous data on NIDUS, Prismaflex and Aquarius.","authors":"Jean Crosier, Denise Colosimo, Rachel Hansen, Heather J Lambert, Malcolm G Coulthard, Zaccaria Ricci","doi":"10.1007/s00467-025-06788-0","DOIUrl":"10.1007/s00467-025-06788-0","url":null,"abstract":"<p><strong>Background: </strong>We sought to determine in vitro whether the PrisMax and CARPEDIEM hemofiltration and hemodialysis devices can reliably deliver ultrafiltration (UF) control that is sufficiently precise to treat infants.</p><p><strong>Methods: </strong>We have previously measured the precision of UF control of the Prismaflex, Aquarius and NIDUS devices by in vitro testing with a bag of saline set up as a dummy patient, and comparing the differences between the UF set and displayed by the devices, and the actual fluid removal or addition measured by precise weighing. Here we have tested the PrisMax (updated version of Prismaflex) and the CARPEDIEM using the same method.</p><p><strong>Results: </strong>The variances of the setting vs. actual errors, and display vs. actual errors after 15 min of 'treatment' with the PrisMax and CARPEDIEM were similar, but were significantly larger than in the NIDUS, and much smaller than in the Prismaflex. However, after a 4-h 'treatment session', the cumulative errors were still within ± 9 mL for these devices, compared with a maximum error of 2.6 mL in the NIDUS, and a deviation of -37.5 mL in the Prismaflex.</p><p><strong>Conclusions: </strong>The PrisMax and the CARPEDIEM have adequate precision to be used in infants. The only device with UF error below 3 ml in 4 h is the volumetrically-controlled NIDUS. We recommend that regulatory bodies should introduce UF precision-testing for devices intended for use in infants.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"3549-3554"},"PeriodicalIF":2.6,"publicationDate":"2025-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12484341/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144584490","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":"Urinary DKK3 in Alport syndrome: early marker or epiphenomenon?","authors":"Iqra Nasir, Fida Hussain, Muhammad Ibrahim","doi":"10.1007/s00467-025-06879-y","DOIUrl":"10.1007/s00467-025-06879-y","url":null,"abstract":"","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"3589"},"PeriodicalIF":2.6,"publicationDate":"2025-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144507324","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":"IPNA webinars: an effective teaching platform advancing patient care in pediatric nephrology worldwide.","authors":"Eugene Yu-Hin Chan, Sharon Teo, Michelle López, Abdullahi Mudi, Pankaj Hari, Olivia Boyer, John D Mahan, Dieter Haffner, Elena Levtchenko","doi":"10.1007/s00467-025-06775-5","DOIUrl":"10.1007/s00467-025-06775-5","url":null,"abstract":"","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"3341-3345"},"PeriodicalIF":2.6,"publicationDate":"2025-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144047432","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":"Toward better outcomes in pediatric septic shock: the role of practical risk models.","authors":"Ziwei Zhao, Hujie Dong, Huihua Zhu","doi":"10.1007/s00467-025-06907-x","DOIUrl":"10.1007/s00467-025-06907-x","url":null,"abstract":"","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"3593-3594"},"PeriodicalIF":2.6,"publicationDate":"2025-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144855952","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":"Epidemiology and outcome of pediatric acute kidney injury-multicenter observational study from a low-middle-income country.","authors":"Uma Ali, Amol Madave, Kinnari Vala, Sadhana Zope, Manoj Matnani, Jyoti Singhal, Anupama Mauskar, Poonam Wade, Radha Ghildiyal, Jyoti Sharma, Madhulika Chakravarthi, Puneet Chhajed, Nivedita Pande, Nisha Krishnamurthy, Aarthi Prasanna, Kiran Sathe, Atul Deokar, Manish Arya, Vaibhav Keskar, Pawan Deore","doi":"10.1007/s00467-025-06856-5","DOIUrl":"10.1007/s00467-025-06856-5","url":null,"abstract":"<p><strong>Background: </strong>The epidemiology and outcome of acute kidney injury (AKI) in low-middle-income countries (LMICs) differ from those in high-income countries due to differences in type and severity of non-renal systemic illness and variability in nephrology-care facilities. There is a paucity of multicenter studies from LMICs. This multicenter observational study was undertaken to study the epidemiology of pediatric AKI in a LMIC and analyze the significance of associated sample characteristics and interventions on outcomes, namely renal recovery and mortality.</p><p><strong>Methods: </strong>Children (1 month-18 years) diagnosed with AKI, based on KDIGO criteria, seen in 10 centers, over 30 months, were included. Data collected included hospital type, city, patient demographics, illness characteristics, pre-existing diseases, AKI profile, interventions including mechanical ventilation (MV), vasoactive drugs (VADs), nephrotoxic drugs, radiocontrast exposure, and recent surgery. Use of kidney replacement therapy (KRT), modality, renal recovery, and patient survival was assessed.</p><p><strong>Results: </strong>Non-renal systemic illness accounted for 79% of cases. Majority were infections. Pre-existing illness was present in 55%, with 29% having kidney disease. AKI was diagnosed at admission in 68%, with 40% in KDIGO stage 3; 50% had severe AKI. MV and VADs were used in 42% and 46%, respectively. KRT was required in 29%, most receiving acute peritoneal dialysis (58%). Complete recovery (CR) was seen in 44%, while 29.6% died. Pre-existing kidney disease and KRT negatively impacted CR. VAD use was linked to mortality, and CR was associated with survival.</p><p><strong>Conclusions: </strong>Non-renal systemic infection was the leading cause of AKI characterized by early, rapid progression, severe in 50%, high need for KRT, CR in less than 50% and high mortality.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"3539-3547"},"PeriodicalIF":2.6,"publicationDate":"2025-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144541800","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":"Management dilemma in choosing evolving treatments in neutropenic septic shock.","authors":"H David Humes, Kera Luckritz, Stephen Gorga, Katie Plomaritas, Sara Hoatlin, Michael Humes, Lenar Yessayan","doi":"10.1007/s00467-025-06798-y","DOIUrl":"10.1007/s00467-025-06798-y","url":null,"abstract":"<p><p>How does a physician decide to use a recently FDA-approved life-saving device in a desperately ill child in which little prior clinical experience is available? This report presents a pediatric patient with neutropenic septic shock and multiorgan failure (MOF) with a 95% chance of death and the availability of a therapeutic device with a completely new approach to treat sepsis. This device, called the selective cytopheretic device (SCD), is a first-in-class autologous immune cell directed therapy. The SCD, when integrated into an extracorporeal blood circuit, has been shown to bind activated neutrophils and monocytes. With a simple pharmacologic maneuver within the device, the bound cells in real time are immunomodulated from a highly pro-inflammatory state to a less inflammatory phenotype. These transformed cells are then released back into the systemic circulation thereby tempering the systemic hyperinflammatory disorder. Since this cell directed therapy focuses on neutrophils, the processing of these cells in a neutropenic state may be a substantive risk resulting in further immunosuppression. On the other hand, the immunomodulation of the circulating neutrophils and monocytes, although sparse, may be beneficial to disrupt the dysregulated inflammatory state responsible for ongoing tissue damage and organ dysfunction. Prior clinical SCD trials excluded patients with neutropenia so that no prior clinical experience was available to make a difficult decision. This report presents the way the medical team approached these issues and made a therapeutic plan that resulted in a positive clinical outcome for the patient.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"3577-3583"},"PeriodicalIF":2.6,"publicationDate":"2025-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12484358/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144340339","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":"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":"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":"3407-3414"},"PeriodicalIF":2.6,"publicationDate":"2025-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12484339/pdf/","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":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}