Pediatric Nephrology最新文献

{"title":"Recent progress in xenotransplantation and its application to pediatric kidney disease.","authors":"Keita Morimoto, Shuichiro Yamanaka, Takashi Yokoo","doi":"10.1007/s00467-025-06664-x","DOIUrl":"10.1007/s00467-025-06664-x","url":null,"abstract":"<p><p>Patients with kidney failure require dialysis or kidney transplantation. Kidney transplantation offers great benefits, including reduced mortality; however, many patients who wish to undergo kidney transplantation are unable to do so due to a shortage of donor organs. This shortage is a global issue, and xenotransplantation has emerged as a potential solution. The history of xenotransplantation is characterized by overcoming the immunological challenge of hyperacute rejection. Recently, breakthroughs such as gene-edited pigs and novel immunosuppressants have successfully lowered rejection rates. Recent clinical studies have reported transplants in patients diagnosed with brain death, and in March 2024, a gene-edited pig kidney was transplanted into a patient with kidney failure at Massachusetts General Hospital, marking the first instance of a gene-edited xenotransplantation into a living patient. Our research focuses on applying xenotransplantation in pediatric and obstetric fields, specifically exploring fetal therapy using pig fetal kidneys. We have long been researching the development of a novel kidney replacement therapy involving the transplantation of fetal pig kidneys. Fetal pig kidneys have the advantage of not requiring vascular anastomosis and are less likely to be rejected compared to adult pig kidneys. Currently, we are advancing nonhuman primate studies aimed at clinical trials of pig fetal kidney transplant therapy for fetuses diagnosed with Potter syndrome, characterized by bilateral kidney agenesis. We sincerely hope that xenotransplantation will soon become a viable treatment option for adult, pediatric, and fetal patients with kidney failure.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"3037-3044"},"PeriodicalIF":2.6,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143067071","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":"Comprehensive genetic analysis and genotype-phenotype correlations in pediatric patients with atypical hemolytic uremic syndrome.","authors":"Jie Ni, Zhi Chen, Chen Ling, Nan Zhou, Yue Xi, Dan Wu, Hejia Zhang, Xiaorong Liu","doi":"10.1007/s00467-025-06814-1","DOIUrl":"10.1007/s00467-025-06814-1","url":null,"abstract":"<p><strong>Background: </strong>Atypical hemolytic uremic syndrome (aHUS) is a rare disease caused by the dysregulation of the alternative pathway. The objective of this study was to evaluate the genetic background and genotype-phenotype correlations in pediatric patients with aHUS.</p><p><strong>Methods: </strong>This retrospective study enrolled 116 pediatric patients from 2013 to 2023 in China. Here, we screened rare and common variants of atypical hemolytic uremic syndrome predisposing genes, as well as reported the clinical characteristics and extrarenal manifestations.</p><p><strong>Results: </strong>Genetic mutations were identified in 20% of patients. Factor H autoantibodies were detected in 53% of patients, with a homozygous CFHR1 deletion observed in 50% of them. The variant of CFHR5 p.V170M (7% vs. 0, P = 0.009, adjusted P-value = 0.036) was enriched in aHUS patients. No significant difference in the frequencies of CFH-H3 and CD46ggaac at-risk haplotypes was observed between aHUS patients and healthy controls. CFH was the most common mutation and was associated with the poorest prognosis, with a 1-year kidney survival rate of 45% after disease onset in the absence of complement blockade. Among patients with factor H autoantibodies, those with a homozygous CFHR1 deletion exhibited a significantly higher relapse rate.</p><p><strong>Conclusions: </strong>Chinese children with aHUS present a low proportion of genetic mutations. Kidney outcomes significantly differ according to genetic backgrounds in the pre-complement blockade era. Homozygous CFHR1 homozygous deletion increases the risk of relapse in patients with factor H autoantibodies.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"3219-3231"},"PeriodicalIF":2.6,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144234751","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":"Letter to the Editors re: The gut microbiome and metabolome in children with a first febrile urinary tract infection: a pilot study.","authors":"Komal Zahid, Abdur Rehman","doi":"10.1007/s00467-025-06867-2","DOIUrl":"10.1007/s00467-025-06867-2","url":null,"abstract":"","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"3331"},"PeriodicalIF":2.6,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144512269","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":"Relationship between clinical and pathologic findings and the presence of genetic variants in patients with steroid-resistant nephrotic syndrome.","authors":"Koichi Kamei, Kandai Nozu, Tomoko Horinouchi, Nana Sakakibara, Kentaro Nishi, Naoya Fujita, Shuichiro Fujinaga, Hiroshi Kaito, Aya Inaba, Riku Hamada, Yuko Shima, Takayuki Okamoto, Junya Hashimoto, Masaki Yamamoto, Yoshimitsu Gotoh, Yusuke Okuda, Hirotsugu Kitayama, Junya Fujimura, Shingo Ishimori, Naohiro Kamiyoshi, Norishige Yoshikawa","doi":"10.1007/s00467-025-06842-x","DOIUrl":"10.1007/s00467-025-06842-x","url":null,"abstract":"<p><strong>Background: </strong>Genetic analysis, crucial in determining treatment strategies for steroid-resistant nephrotic syndrome (SRNS), can be performed in limited facilities and requires a long time. Predicting the presence or absence of genetic variants by clinical and pathologic features is preferable.</p><p><strong>Methods: </strong>In this multicenter, retrospective study, we compared the clinical or pathologic features between the patients with and without genetic variants in children with SRNS and evaluated the efficacy of immunosuppressive treatment and long-term kidney outcomes.</p><p><strong>Results: </strong>Fifty-three patients in 17 institutes were included, and 11 patients (21%) showed genetic variants. Two patients with a family history of nephrotic syndrome harbored genetic variants. Serum albumin level at onset was significantly lower in patients without genetic variants (p = 0.001). The receiver operating characteristic curve analysis showed that a cutoff value of serum albumin level of 2.3 g/dL at onset had a sensitivity and specificity of 82% and 90%, respectively, in predicting genetic variants. Patients with asymptomatic proteinuria at onset were more likely to harbor genetic variants (p = 0.05). None of the pathologic features was significantly different between the two groups. Mesangial proliferation and diffuse foot process effacement were observed more in patients without genetic variants, although statistically insignificant. Immunosuppressive treatment was less effective, and the 5-year kidney survival was poorer (31% and 78%, p = 0.03) in patients with genetic variants than in those without genetic variants.</p><p><strong>Conclusions: </strong>Higher serum albumin levels at onset can predict the presence of genetic variants. Pathologic features might have limited utility in predicting them.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"3111-3120"},"PeriodicalIF":2.6,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144234715","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":"Advanced hemodynamic monitoring: feasibility of leveraging non-invasive electrocardiometry in critically ill pediatric patients requiring continuous kidney replacement therapy.","authors":"Anna M Lang, Ayse Akcan-Arikan, Christin Silos, Jack F Price, Srivaths Poyyapakkam, Joey Alge, Sameer Thadani","doi":"10.1007/s00467-025-06860-9","DOIUrl":"10.1007/s00467-025-06860-9","url":null,"abstract":"<p><strong>Background: </strong>Hemodynamic instability occurs in children receiving continuous kidney replacement therapy (CKRT). Electrocardiometry can help characterize hemodynamics beyond traditional blood pressure (BP) and heart rate (HR). We aimed to assess the feasibility and correlations of hemodynamic measurements obtained using electrocardiometry in children receiving CKRT.</p><p><strong>Methods: </strong>Prospective single-center observational study of pediatric patients receiving CKRT between 11/2019 and 3/2021. Patients who received extracorporeal membranous oxygenation, ventricular assist device, pacemaker, apheresis, no invasive BP, and COVID-19 were excluded. Electrocardiometry measured cardiac index (CI), HR, stroke volume variability (SVV), stroke volume index (SVI), and systemic vascular resistance index (SVRI) continuously; data were aggregated into 1-h epochs, and correlation coefficients were computed using Spearman's rank test.</p><p><strong>Results: </strong>Seventeen patients with a median age of 43 months (IQR 13-122). Median weight and fluid overload at CKRT start were 13.9 kg (IQR 8.79-29.80) and 14.4% (IQR 2.4-25.6%) + 171.46 mL/kg (IQR 31.10-307.41), respectively. All measurements obtained via ICON were of high quality and no adverse events were identified. CI had a negative correlation with SVRI (r =  - 0.67) and had a positive correlation with SVI (r = 0.83) and mean arterial pressure (MAP) (r = 0.63). HR did not correlate with any hemodynamic variables, while MAP only correlated with SVI (r = 0.63).</p><p><strong>Conclusions: </strong>Electrocardiometry can assess the hemodynamic profile of children receiving CKRT. Compensatory cardiovascular changes remain intact in children receiving CKRT, as evidenced by correlations between SVI, SVRI, CI, and MAP. Future studies should investigate how this technology could enable more individualized CKRT prescriptions and improve patient outcomes.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"3263-3271"},"PeriodicalIF":2.6,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144340338","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":"Adaption and validation of a glomerular filtration rate estimation equation for Chinese children: a cross-sectional analysis of pooled data.","authors":"Ruohua Yan, Xiaowen Wang, Chao Zhang, Chen Wang, Yaguang Peng, Lin Huang, Hans Pottel, Fang Wang, Xiaoxia Peng","doi":"10.1007/s00467-025-06818-x","DOIUrl":"10.1007/s00467-025-06818-x","url":null,"abstract":"<p><strong>Background: </strong>This study aimed to adapt the existing Q-based equation for glomerular filtration rate (GFR) estimation using serum creatinine (SCr) to Chinese children by modification of certain parameters, and to establish the pediatric reference intervals (RIs) for estimated GFR in China accordingly.</p><p><strong>Methods: </strong>The Q-based equation had the form eGFR = M/(SCr/Q), where the parameter M was estimated from 328 children with measured GFR in Wuhan Children's Hospital, and the parameter Q was fitted among 11,713 healthy children volunteers who had SCr recruited from 11 provinces of China. The Q-based equation was applied to 60,524 inpatients in Beijing Children's Hospital to assess the impact of estimated GFR on the clinical diagnosis of acute kidney injury (AKI). Then, the Q-based equation was used to estimate GFR in a large representative population of healthy Chinese children for RI establishment.</p><p><strong>Results: </strong>The parameter M was estimated separately in children aged less than 2 years (100.2) and 2 years or above (107.3). The parameter Q was modeled as a linear function of age in boys (19.5 + 3.2Age) and girls (23.6 + 2.2Age), respectively. The GFR estimated by the Q-based equation could well identify AKI, with a significantly higher risk of in-hospital mortality in AKI patients than non-AKI patients (OR, 5.69; 95% CI, 4.80-6.74). The RI of estimated GFR for children aged 2 years or above ranged from 82.5 to 145.2 ml/min/1.73 m², comparable to that for young adults (80 to 140 ml/min/1.73 m²).</p><p><strong>Conclusions: </strong>The Q-based equation is simple and practical for GFR estimation in Chinese children.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"3169-3180"},"PeriodicalIF":2.6,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144192098","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: \"Is sclerostin a true mediator of diabetic nephropathy, or just a surrogate of altered bone metabolism?\"","authors":"Dina E Sallam, Yasmine Ibrahim Mahmoud Elhenawy, Aya Mohamed Abdullah Ahmed, Sara Ibrahim Abdelfattah Taha, Eman Mohamed Elsayed","doi":"10.1007/s00467-025-06925-9","DOIUrl":"10.1007/s00467-025-06925-9","url":null,"abstract":"","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"3329-3330"},"PeriodicalIF":2.6,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144768917","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 Zahid and Rehman.","authors":"Barbora Piteková, Ivan Hric, Jakub Zieg, Eva Baranovičová, Patrik Konopásek, Jakub Gécz, Paul J Planet, Viktor Bielik","doi":"10.1007/s00467-025-06871-6","DOIUrl":"10.1007/s00467-025-06871-6","url":null,"abstract":"","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"3333-3334"},"PeriodicalIF":2.6,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12401757/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144643136","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":"A population pharmacokinetic approach to compare ⁵¹Cr-EDTA and ^{99 m}Tc-DTPA clearances in measuring renal glomerular filtration rate in oncopediatrics.","authors":"Matthieu Gracia, Victoire Ankaoua, Mathieu Alonso, Marlène Pasquet, Etienne Chatelut","doi":"10.1007/s00467-025-06828-9","DOIUrl":"10.1007/s00467-025-06828-9","url":null,"abstract":"<p><strong>Background: </strong>⁵¹Cr-EDTA and ^{99 m}Tc-DTPA clearances are two reference methods of determining the glomerular filtration rate. The study aimed to compare these two radioisotopic clearance measurements using an original approach based on population pharmacokinetics in oncopediatrics.</p><p><strong>Methods: </strong>Plasma concentrations of ⁵¹Cr-EDTA and ^{99 m}Tc-DTPA obtained from, respectively, 40 and 19 children treated with nephrotoxic chemotherapy were simultaneously analyzed while accounting for three covariates (i.e., serum creatinine, plasma cystatin C, body weight) previously described as being related to the pediatric glomerular filtration rate. A binary covariate \"GFR measurement\" (MES = 0 or 1, respectively, for ⁵¹Cr-EDTA or ^{99 m}Tc-DTPA) was added to the model.</p><p><strong>Results: </strong>Analysis revealed a non-significant bias (± 95% CI) of - 0.9% ± 11.4% between the two measurements (with ^{99 m}Tc-DTPA clearance overestimated).</p><p><strong>Conclusions: </strong>This result confirmed that both radioisotopic clearances are equivalent in pediatrics, as has been reported in the literature on adults based on intrapatient comparisons. The value of the population approach in comparing the pharmacokinetics of two different compounds is thus demonstrated.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"3163-3168"},"PeriodicalIF":2.6,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12401762/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144174460","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":"Utilization of anti-CD20 antibodies for treatment of childhood nephrotic syndrome, 2010 to 2022.","authors":"Michelle R Denburg, Kathryn Hirabayashi, Amy Goodwin Davies, Hanieh Razzaghi, Vikas R Dharnidharka, Bradley P Dixon, Joseph T Flynn, Caroline A Gluck, Mark M Mitsnefes, William E Smoyer, Susan L Furth, Christopher B Forrest","doi":"10.1007/s00467-025-06811-4","DOIUrl":"10.1007/s00467-025-06811-4","url":null,"abstract":"<p><strong>Background: </strong>A growing body of evidence supports the efficacy of the type I anti-CD20 monoclonal antibody, rituximab, in the management of children with frequently relapsing or steroid-dependent nephrotic syndrome. We examined temporal trends and described current patterns in the use of anti-CD20 antibodies and other corticosteroid-sparing drug therapies in a large multi-institutional population of children with nephrotic syndrome.</p><p><strong>Methods: </strong>Data came from PEDSnet, a clinical research network that aggregates electronic health record data at several children's healthcare organizations in the United States. Patients with at least one inpatient, emergency, or outpatient physician encounter between January 2010 and November 2022 who met our published computable phenotype algorithm for nephrotic conditions were included. Children with systemic lupus erythematosus or congenital/genetic nephrotic diagnoses were excluded. Treatments were measured from nephrotic syndrome diagnosis to kidney transplant or most recent encounter.</p><p><strong>Results: </strong>Among 6,892,137 patients across 6 centers, 2962 met criteria for nephrotic conditions (0.4 per 1000 patients). 852 (28.8%) had at least one native kidney biopsy. Nearly half of the population was exposed to at least one steroid-sparing agent, most of whom had exposure to multiple agents. 524 (17.7%) patients were exposed to rituximab, and utilization of rituximab increased over the 12-year study period. Similar trends were observed for mycophenolate and tacrolimus. Concurrently, use of cyclosporine and cyclophosphamide decreased.</p><p><strong>Conclusion: </strong>Use of rituximab to manage nephrotic syndrome has steadily increased, and tacrolimus, mycophenolate, and rituximab are currently the most commonly used steroid-sparing agents for childhood nephrotic syndrome.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"3121-3127"},"PeriodicalIF":2.6,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12378964/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144234716","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}