Pediatric NephrologyPub Date : 2025-09-01Epub Date: 2025-02-13DOI: 10.1007/s00467-025-06697-2
Asheeta Gupta, Kushani Jayasinghe, Amar Majmundar, Nina Mann, Rajiv Sinha, Matthew G Sampson, Catherine Quinlan
{"title":"Next-generation nephrology: part 1-an aid for genetic and genomic testing in pediatric nephrology.","authors":"Asheeta Gupta, Kushani Jayasinghe, Amar Majmundar, Nina Mann, Rajiv Sinha, Matthew G Sampson, Catherine Quinlan","doi":"10.1007/s00467-025-06697-2","DOIUrl":"10.1007/s00467-025-06697-2","url":null,"abstract":"<p><p>Genetic and genomic testing has transformed the practice of clinical nephrology. Yet nephrologists have identified genetic literacy and access to kidney genetic care models as critical barriers to utilising this technology in their practice. To accelerate the implementation of genomic medicine in clinical nephrology, we provide a two-part evidence-based educational review. This inaugural segment highlights the importance of genomic testing for kidney disease and the steps a nephrologist can take to understand and apply this technology to one's own clinical practice. The second part delineates approaches to operationalising a kidney genetics service based on existing models, reported barriers, and the authors' experiences in developing kidney clinic models for different settings.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"2759-2777"},"PeriodicalIF":2.6,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143409613","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}
Pediatric NephrologyPub Date : 2025-09-01Epub Date: 2025-05-22DOI: 10.1007/s00467-025-06809-y
Ling Li, Hui-Ling Shu, Shu-Dong Cui, Xiao-Lin Miao, Xiao-Qing Chen, Jing-Jing Pan, Yang Yang
{"title":"Risk factors of acute kidney injury in extremely low gestational age neonates (ELGANs) - a retrospective case-control study.","authors":"Ling Li, Hui-Ling Shu, Shu-Dong Cui, Xiao-Lin Miao, Xiao-Qing Chen, Jing-Jing Pan, Yang Yang","doi":"10.1007/s00467-025-06809-y","DOIUrl":"10.1007/s00467-025-06809-y","url":null,"abstract":"<p><strong>Background: </strong>Neonatal acute kidney injury (AKI) is associated with poor clinical outcomes. Consequently, this study was designed to explore the prenatal and postnatal risk factors in Chinese extremely low gestational age neonates (ELGANs).</p><p><strong>Methods: </strong>This retrospective study included all ELGANs (born between 23-0/7 and 27-6/7 weeks of gestation) hospitalized from January 2019 to December 2022. These premature babies were divided into the AKI group (n = 39) and the non-AKI group (n = 76) according to the Kidney Disease: Improving Global Outcomes (KDIGO) criteria.</p><p><strong>Results: </strong>AKI most frequently occurred in the first 10 days of life (median: 8th day, quartile range: 6th - 10th day). Most infants (61.54%) manifested stage 1 AKI. Lower gestational age (GA) was the major independent risk factor for AKI [adjusted OR (aOR): 0.40, 95% CI: 0.22-0.67]. Moreover, the lower the GA, the higher the risk of AKI. When the interactive effect between GA and nonsteroidal anti-inflammatory drugs (NSAIDs) was considered, NSAIDs administration would greatly enhance the risk of AKI (interaction term, aOR: 3.94, 95% CI: 1.26-13.92). The levels of serum creatinine and urea nitrogen in the AKI group generally returned to normal upon discharge and continued until 6 months after follow-up.</p><p><strong>Conclusion: </strong>When using NSAIDs in the ELGANs population, great attention should be paid to dynamically monitor kidney function.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"2981-2991"},"PeriodicalIF":2.6,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144120564","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":"Telitacicept use in children with IgA vasculitis nephritis: preliminary observations.","authors":"Jing Wang, Jingjing Cui, Jiani Chen, Yabin Liao, Mifeng Yang, Junyu Lin, Xiran Yang, Bo Zhao","doi":"10.1007/s00467-025-06709-1","DOIUrl":"10.1007/s00467-025-06709-1","url":null,"abstract":"<p><strong>Objectives: </strong>To investigate the safety and efficacy of telitacicept in children with immunoglobulin (Ig) A vasculitis nephritis (IgAVN).</p><p><strong>Methods: </strong>This is a case series of children with biopsy-confirmed IgAVN who received glucocorticoid with or without a second immunosuppressant and presented with urinary protein levels > 0.3 g/day/kg. The patients were administered telitacicept subcutaneously weekly as an adjunct to the standard treatment. The laboratory indices were assessed before and 24 weeks post-treatment, and the treatment's efficacy and side effects were analyzed.</p><p><strong>Results: </strong>Seven patients (six males and one female) were enrolled in the study. The median age and body weight of patients were 13 years and 48 kg, respectively. The median disease duration was 29 months. After treatment with telitacicept (80 or 160 mg administered per week) until the 24th week, the 24-h urinary protein levels, percentage of CD19 + B cells, and IgA and IgM levels were decreased compared to the baseline values (P < 0.05). Serum albumin and complement C4 levels increased significantly in response to the treatment (P < 0.05). The serum creatinine, blood urea, serum total protein, IgG, and complement C3 levels and urine erythrocyte counts were not significantly different (P > 0.05) from the respective pre-treatment values. The enrolled patients did not experience any drug reactions.</p><p><strong>Conclusions: </strong>The combination of telitacicept and the standard treatment showed good clinical effects in children with IgAVN. This treatment showed good safety and had good tolerance.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"2829-2836"},"PeriodicalIF":2.6,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143557541","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}
Pediatric NephrologyPub Date : 2025-09-01Epub Date: 2025-02-28DOI: 10.1007/s00467-025-06711-7
Asheeta Gupta, Kushani Jayasinghe, Amar Majmundar, Nina Mann, Rajiv Sinha, Matthew G Sampson, Catherine Quinlan
{"title":"Next-generation nephrology: part 2-mainstreaming genomics in nephrology, a global perspective.","authors":"Asheeta Gupta, Kushani Jayasinghe, Amar Majmundar, Nina Mann, Rajiv Sinha, Matthew G Sampson, Catherine Quinlan","doi":"10.1007/s00467-025-06711-7","DOIUrl":"10.1007/s00467-025-06711-7","url":null,"abstract":"<p><p>Kidney genetic services are being created worldwide, revolutionising the way in which we manage families with suspected monogenic kidney disease. There is potential to learn from one another, whether one is just embarking on this journey or within an established kidney genetics service model with aspirations to optimise it further. This concluding portion of our two-part educational review explores the global efforts to integrate genomics into nephrology. We discuss key considerations for establishing kidney genetics services and share insights from successful implementation in Australia, India, the United Kingdom (UK) and the United States (US), through case studies. Widespread integration of genomics within nephrology still faces barriers including limited genomics education among clinicians, high costs and ethical concerns. Educational strategies including workshop-based, online resources and clinical decision tools are aiming to address the genomic literacy gap among nephrologists. Multidisciplinary kidney genetics clinic models comprising nephrologists, geneticists, clinical scientists and counsellors are proving to be an effective model of delivering this diagnostic tool. Data of how kidney genetics clinics can foster collaboration with registries to facilitate research and shared learning to optimise care for patients are becoming evident. We also explore the importance of equitable access to genomics services across diverse populations, advocating for policies that address disparities in access to healthcare and genetic data representation. We hope to highlight the importance of upskilling the nephrology workforce to fully leverage the advances in genomic medicine and ensure comprehensive, accessible and personalised care for patients with genetic kidney diseases.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"2779-2793"},"PeriodicalIF":2.6,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143524074","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}
Pediatric NephrologyPub Date : 2025-09-01Epub Date: 2025-05-14DOI: 10.1007/s00467-025-06794-2
Abdulsalam S Alrashdi, Jasir N Alshammari, Sulaiman K Abdullah, Sulaiman I Alqannas, Hassan Faqeehi, Sawsan Albatati, Khawla A Rahim, Ibrahim A Sandokji, Abdulkarim S Alanazi, Saeed M Alzabali
{"title":"Outcomes of continuous kidney replacement therapy in pediatric patients: the impact of timing and predictors of mortality.","authors":"Abdulsalam S Alrashdi, Jasir N Alshammari, Sulaiman K Abdullah, Sulaiman I Alqannas, Hassan Faqeehi, Sawsan Albatati, Khawla A Rahim, Ibrahim A Sandokji, Abdulkarim S Alanazi, Saeed M Alzabali","doi":"10.1007/s00467-025-06794-2","DOIUrl":"10.1007/s00467-025-06794-2","url":null,"abstract":"<p><strong>Background: </strong>While critically ill children receiving continuous kidney replacement therapy (CKRT) are at increased risk of mortality, few studies have examined the predictors of mortality in this population. This study aimed to evaluate CKRT outcomes, focusing on predictors of mortality.</p><p><strong>Methods: </strong>This cohort study included children aged 0-14 years who received CKRT. It collected baseline, clinical, and laboratory data. Descriptive analyses were performed. Least absolute shrinkage and selection operator (LASSO) regression was used to select the best predictors of mortality. A multivariable logistic regression model was constructed and validated with 1000 bootstraps.</p><p><strong>Results: </strong>This study included 113 children who received CKRT, of whom 83 (73.5%) survived and 30 (26.6%) died. Children admitted to the intensive care unit with a higher Pediatric Risk of Mortality III score, sepsis, longer intubation, or hypoalbuminemia (< 30 g/dL) were more likely to die. Multifactorial acute kidney injury was more common in those who died than in those who survived (83.3% vs. 31.3%, p < 0.01). As the exclusive indication for CKRT, fluid overload was more common in those who died than in those who survived (26.7% vs. 6.0%, p = 0.01). LASSO and multivariable regression models identified hemodynamic instability, as evidenced by inotropic support use, and abnormal coagulation, as evidenced by not using heparin anticoagulation, as independent predictors of mortality. Initiating CKRT late (> 48 h) was associated with mortality in the univariate but not the multivariate analysis.</p><p><strong>Conclusions: </strong>Hemodynamic instability was the best predictor of mortality in critically ill children receiving CKRT.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"2973-2980"},"PeriodicalIF":2.6,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144003843","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":"Impact of organ dysfunction on outcomes in pediatric hypernatremia: a retrospective observational study.","authors":"Ayumi Mihara, Kentaro Nishi, Itaru Hayakawa, Hiroki Kato, Norihiko Tsuboi, Masao Ogura, Hirotaka Chiba, Kentaro Ide, Shotaro Matsumoto, Kenji Ishikura, Koichi Kamei, Akira Ishiguro","doi":"10.1007/s00467-025-06768-4","DOIUrl":"10.1007/s00467-025-06768-4","url":null,"abstract":"<p><strong>Background: </strong>Hypernatremia is associated with increased mortality risk in pediatric patients. However, its impact on outcomes remains unclear. This study aimed to clarify the clinical backgrounds of contemporary children with hypernatremia and identify prognostic factors, including the impact of organ dysfunction on mortality.</p><p><strong>Methods: </strong>We conducted a retrospective observational study of children under 18 years of age with hypernatremia (serum sodium ≥ 150 mmol/L) at a tertiary pediatric hospital in Japan between December 2021 and May 2023. Patient characteristics, clinical course, mortality rate, and occurrence of intracranial hemorrhage were analyzed. Kaplan-Meier and multivariable Cox proportional hazard analyses assessed 180-day survival and mortality risk factors.</p><p><strong>Results: </strong>Out of 9,208 children admitted, 116 (1.2%) with hypernatremia were finally analyzed; 91% had underlying chronic diseases and 91% had hospital-acquired hypernatremia. Before onset, 85% had medication related to hypernatremia, 80% received intravenous fluids, and 63% were managed with nil per os. Survival rates were 87.9%, 83.6%, and 81.9% at 30, 90, and 180 days from the onset of hypernatremia, respectively. Cox proportional hazard analysis reveal that four or more organ dysfunctions (odds ratio 5.83, 95%CI 1.92-17.7, P = 0.002) significantly correlated with death, after adjusting for intravenous fluids and peak sodium level. Two patients (2%) developed new subdural hematomas after the onset of hypernatremia.</p><p><strong>Conclusions: </strong>Pediatric hypernatremia predominantly occurs in medically complex children and is mostly hospital-acquired. The number of organ dysfunctions is a strong predictor of mortality in children with hypernatremia.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"2945-2956"},"PeriodicalIF":2.6,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144027831","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}
Pediatric NephrologyPub Date : 2025-09-01Epub Date: 2025-04-24DOI: 10.1007/s00467-025-06783-5
Yiman Zhai, Yuying Zhang, Hao Gou
{"title":"Pharmacological treatment of pediatric nocturnal enuresis: a systematic review and network meta-analysis.","authors":"Yiman Zhai, Yuying Zhang, Hao Gou","doi":"10.1007/s00467-025-06783-5","DOIUrl":"10.1007/s00467-025-06783-5","url":null,"abstract":"<p><strong>Background: </strong>Nocturnal enuresis (NE) is a socially stigmatizing and stressful childhood condition. Many drugs have been applied for NE treatments. We aimed to assess the efficacy of multiple drugs in pediatric NE through a network meta-analysis (NMA).</p><p><strong>Methods: </strong>Under PRISMA guidelines and the PROSPERO registration number CRD42024581022, we systematically searched the Cochrane Library, Embase, PubMed, and Web of Science from inception to September 9, 2024, for randomized controlled trials (RCTs). Studies involving patients aged 5 to 18 and diagnosed with NE and using at least one drug treatment were included. The NIH Quality Assessment Tools were used to assess the quality of the included studies. Bayesian NMA was organized with R software 4.3.3.</p><p><strong>Results: </strong>Twenty-three RCTs with 1658 participants were enrolled. The complete and partial response rates of combination therapy were higher than those of monotherapy. SUCRA ranking showed that desmopressin (DES) plus propiverine (75.24%), DES plus solifenacin (68.83%), and DES plus tolterodine (66.46%) ranked among the top six in terms of complete response rate. None of the four included interventions significantly improved the relapse rate of NE. All treatments yielded few adverse events. Moreover, combination therapy with DES (RR [95%CrI] = 3.55 [2.28, 5.64]) or without DES (RR [95%CrI] = 3.74 [1.19, 12.06]) seemed to be superior to DES monotherapy in terms of complete response rate. Small number/sample size and inconsistency among included trials might impair the strength of evidence.</p><p><strong>Conclusions: </strong>Combination therapy may be superior to monotherapy in NE management. The most frequently used combination therapy is desmopressin plus anticholinergic agents. All drugs seemed to be safe.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"2795-2806"},"PeriodicalIF":2.6,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144037647","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}
Pediatric NephrologyPub Date : 2025-09-01Epub Date: 2025-03-25DOI: 10.1007/s00467-025-06736-y
Robin Raschke, Clarkson Crane, Robert Sheets, Noureddin Nourbakhsh, Nadine Benador, Elizabeth Ingulli, Katayoon Shayan, Peter Yorgin, Caitlin Carter
{"title":"Incomplete concordance between laboratory and pathologic findings on post-induction kidney biopsy in pediatric patients with proliferative lupus nephritis.","authors":"Robin Raschke, Clarkson Crane, Robert Sheets, Noureddin Nourbakhsh, Nadine Benador, Elizabeth Ingulli, Katayoon Shayan, Peter Yorgin, Caitlin Carter","doi":"10.1007/s00467-025-06736-y","DOIUrl":"10.1007/s00467-025-06736-y","url":null,"abstract":"<p><strong>Background: </strong>Proliferative lupus nephritis (LN) is associated with increased risk of progression to kidney failure. After initial kidney biopsy, the utility and timing of subsequent biopsies is unknown. There is known discordance between the laboratory parameters used to diagnose LN and the histopathologic classification. We explore the utility of a subsequent kidney biopsy in guiding treatment of LN to determine the factors that warrant follow-up kidney biopsy.</p><p><strong>Methods: </strong>We conducted a single center retrospective cohort study of 30 SLE patients who underwent serial kidney biopsy for LN. Subjects were stratified based on their Childhood Arthritis and Rheumatology Research Alliance (CARRA) renal response into complete renal response (CRR) and incomplete renal response (IRR) groups at the time of second biopsy.</p><p><strong>Results: </strong>Among 30 patients with LN, 11/18 in CRR group and 11/12 in IRR group had persistent proliferative nephritis at 1 ± 0.3 years after initial biopsy. Only SLEDAI score was associated with an increased risk of persistent proliferative nephritis (p = 0.03). Initial CARRA response category was associated with outcome at last follow-up (mean 4.5 years), with 11/18 CRR and 3/12 IRR achieving CRR at last follow-up at mean 4.5 years (p < 0.001). Kidney biopsy directly impacted clinical decision in 7/18 CRR patients in the CRR group who had therapy escalated or reduction withheld due to biopsy findings.</p><p><strong>Conclusions: </strong>Available laboratory markers in LN are insufficient to identify children with ongoing proliferative nephritis. Follow-up kidney biopsy may be warranted for children with CRR at 1 year after initial biopsy.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"2845-2854"},"PeriodicalIF":2.6,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143710762","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}