Agathe Bikupe Nkoy, Floreen Maluwenze Mumaka, Therance Tobo Matoka, Ange Ngonde, Ernest Kiswaya Sumaili, Justine Busanga Bukabau, Veerle Labarque, Lambertus P van den Heuvel, Arend Bökenkamp, Etienne Cavalier, Elena Levtchenko, Pierre Delanaye, Hans Pottel, Pépé Mfutu Ekulu
{"title":"Performance of creatinine and cystatin C-based equations to estimate glomerular filtration rate in African children with sickle cell anemia.","authors":"Agathe Bikupe Nkoy, Floreen Maluwenze Mumaka, Therance Tobo Matoka, Ange Ngonde, Ernest Kiswaya Sumaili, Justine Busanga Bukabau, Veerle Labarque, Lambertus P van den Heuvel, Arend Bökenkamp, Etienne Cavalier, Elena Levtchenko, Pierre Delanaye, Hans Pottel, Pépé Mfutu Ekulu","doi":"10.1007/s00467-025-06986-w","DOIUrl":"https://doi.org/10.1007/s00467-025-06986-w","url":null,"abstract":"<p><strong>Background: </strong>Serum creatinine (SCr), the most used biomarker to evaluate glomerular filtration rate (GFR), might be inaccurate in children with sickle cell anemia (SCA). In this context, cystatin C (SCys) could be of interest. This study evaluated the performance of commonly used SCr- and SCys-based estimated GFR (eGFR) equations in African children with SCA.</p><p><strong>Methods: </strong>This cross-sectional study included 109 steady-state children with SCA aged 3-18 years, from the Democratic Republic of Congo. Measured GFR (mGFR) was obtained using iohexol plasma clearance. eGFR was calculated using commonly used SCr- and SCys-based equations in children. The performance of these equations was evaluated by calculating the bias, precision, and accuracy within 30% (P30) of mGFR.</p><p><strong>Results: </strong>The mean age of participants was 9.9 ± 4.2 years, and 48.6% were female. The median mGFR was 142 (IQR 119-169) mL/min/1.73 m<sup>2</sup>. Of the equations studied, the FAS-Age SCr had the lowest bias (0.9 mL/min/1.73 m<sup>2</sup>). However, the 95% limit of agreement was very wide (-80.3 to + 81.6). SCr failed to rise in an age-dependent manner, reflecting a progressive loss of muscle mass or increased tubular secretion. All SCys-based equations underestimated GFR and failed to detect hyperfiltration, but there was no age-related change in bias.</p><p><strong>Conclusion: </strong>These data show that all common eGFR equations using SCr or SCys poorly predict mGFR in African children with SCA. SCr-based equations potentially miss a decline in kidney function, which suggests that SCys could be the preferred marker in this population.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2025-10-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145308876","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}
Cal H Robinson, Simon Carter, Nowrin Aman, Valentina Bruno, Shelby Chan, Brian Cuthbertson, Mackenzie Garner, Eddy Fan, Christoph Licht, Ashlene M McKay, Seetha Radhakrishnan, Veronique Rowley, Chia Wei Teoh, Jovanka Z Vasilevska-Ristovska, Anna Heath, Rulan S Parekh
{"title":"Establishing core outcomes and minimal clinically important differences for childhood steroid sensitive nephrotic syndrome clinical trials: results from a Delphi consensus process.","authors":"Cal H Robinson, Simon Carter, Nowrin Aman, Valentina Bruno, Shelby Chan, Brian Cuthbertson, Mackenzie Garner, Eddy Fan, Christoph Licht, Ashlene M McKay, Seetha Radhakrishnan, Veronique Rowley, Chia Wei Teoh, Jovanka Z Vasilevska-Ristovska, Anna Heath, Rulan S Parekh","doi":"10.1007/s00467-025-06960-6","DOIUrl":"https://doi.org/10.1007/s00467-025-06960-6","url":null,"abstract":"<p><strong>Background: </strong>Few randomized controlled trials are conducted in childhood nephrotic syndrome, and substantial global practice variation exists. Trials have not reported consistent outcomes, patient-reported outcomes, or justified minimal clinically important differences (MCID). Our aim was to establish consensus on core outcomes, assessment timepoints, MCIDs, and desirability of outcome ranking (DOOR) endpoints for future childhood steroid-sensitive nephrotic syndrome research.</p><p><strong>Methods: </strong>We conducted a two-stage Delphi consensus survey of international healthcare providers and Canadian patients and caregivers with experience in childhood nephrotic syndrome. Respondents rated potential outcomes using Likert scales and assigned MCID values for relapse and remission endpoints. Following the surveys, we held workshops for healthcare providers and patients/caregivers in Toronto, Canada, with facilitated discussions. Qualitative data was analyzed thematically to explore perspectives on childhood nephrotic syndrome clinical trial outcomes and MCIDs.</p><p><strong>Results: </strong>Eighty-one participants (45 providers, 36 patients/caregivers, 63% Canadian) responded to the surveys. Three disease outcomes (relapse rate, relapse-free survival, and relapse occurrence) met consensus criteria as core outcomes. Median MCID values were a 25% absolute difference between two treatments in relapse risk by 1-year (for relapse prevention) choices and 10% absolute difference in remission by 2 weeks (for relapse treatment). Consensus was achieved for five core patient-reported outcomes (pain, physical symptoms, physical function and mobility, life participation, and social function) and DOOR endpoints for nephrotic syndrome relapse and remission.</p><p><strong>Conclusions: </strong>Consensus exists among surveyed participants on core disease and patient-reported outcomes for childhood nephrotic syndrome research. Findings can improve the quality and reporting of future trials in this population.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2025-10-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145293293","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":"Randomized controlled trial on efficacy and safety of torsemide versus furosemide in patients with nephrotic syndrome.","authors":"Tanvi Bindal, Aditi Sinha, Menka Yadav, Jitendra Meena, Pankaj Hari, Arvind Bagga","doi":"10.1007/s00467-025-06990-0","DOIUrl":"https://doi.org/10.1007/s00467-025-06990-0","url":null,"abstract":"<p><strong>Background: </strong>Torsemide is preferred to furosemide in adults with heart failure for its higher bioavailability and longer half-life. Evidence of benefit in children is limited to uncontrolled studies.</p><p><strong>Methods: </strong>This single-center, open-label, randomized controlled trial compared the superiority of oral therapy with torsemide administered at a dose of 0.25-0.5 mg/kg twice daily, to furosemide 1-1.5 mg/kg twice daily for 48-h, in inducing diuresis in patients, 3-18 years-old, with nephrotic syndrome and moderate to severe edema without hypovolemia (CTRI/2023/10/058799). Secondary outcomes included proportions of patients with adequate and inadequate diuresis, treatment failure, treatment-emergent adverse events or serious adverse events (TEAE, SAE), percentage weight loss, natriuresis and free water clearance.</p><p><strong>Results: </strong>Of 173 patients screened, 25 patients each, with no significant baseline differences, were randomized to receive torsemide or furosemide. Nine patients in each group received 4 doses per protocol; others received fewer doses due to treatment failure or TEAE. On intention-to-treat analyses, patients in the torsemide and furosemide groups had similar urine output over 48-h (mean difference, MD 0.42; 95% CI -0.39,1.23 ml/kg/hr) and comparable proportions of patients had adequate diuresis (MD 20%; -3.7, 43.7%), inadequate diuresis (MD 16%; -41, 9.5%), treatment failure (MD -4%; -17.1, 9.1%), and TEAE or SAE. There were no differences in weight loss (MD 0.82; -2.2, 3.8%), natriuresis (MD -2.9; -74.4, 68.5 mEq/day) and free water clearance (MD 150.1; -266.1, 567.2 ml/day). Per-protocol analyses confirmed no intergroup differences.</p><p><strong>Conclusions: </strong>Torsemide is not superior to furosemide in inducing diuresis in children with nephrotic syndrome with moderate to severe edema.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2025-10-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145286651","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}
Myung Hyun Cho, Hee Gyung Kang, Yo Han Ahn, Seong Heon Kim, Eujin Park, Joo Hoon Lee, Jiwon Jung, Min Hyun Cho, Hee Sun Baek, Jae Il Shin, Keum Hwa Lee, Kyoung Hee Han, Heeyeon Cho, Ji Yeon Song, Ji Hyun Kim, Eun Mi Yang
{"title":"Factors influencing serum uric acid levels and their impact on cardiovascular risk in patients with pediatric chronic kidney disease.","authors":"Myung Hyun Cho, Hee Gyung Kang, Yo Han Ahn, Seong Heon Kim, Eujin Park, Joo Hoon Lee, Jiwon Jung, Min Hyun Cho, Hee Sun Baek, Jae Il Shin, Keum Hwa Lee, Kyoung Hee Han, Heeyeon Cho, Ji Yeon Song, Ji Hyun Kim, Eun Mi Yang","doi":"10.1007/s00467-025-06961-5","DOIUrl":"https://doi.org/10.1007/s00467-025-06961-5","url":null,"abstract":"<p><strong>Background: </strong>Elevated serum uric acid levels are commonly observed in chronic kidney disease (CKD) and are associated with cardiovascular disease (CVD). Research on the role of serum uric acid in pediatric CKD is limited. This study aims to investigate factors influencing serum uric acid levels and their association with CVD risk factors in children with CKD.</p><p><strong>Methods: </strong>We used baseline data from the KoreaN cohort study for Outcome in patients with Pediatric Chronic Kidney Disease. Overall, 338 children with CKD were included.</p><p><strong>Results: </strong>The median age of participants was 9.8 years, and their mean estimated glomerular filtration rate (eGFR) was 63.5 mL/min/1.73 m<sup>2</sup>. The median serum uric acid level was 5.8 mg/dL. Multivariable regression analysis indicated that male sex, older age, and low eGFR were associated with higher serum uric acid levels. In children with CKD stage III or higher, glomerulopathy as the etiology of CKD was also associated with elevated serum uric acid levels. Serum uric acid levels showed a U-shaped relationship with the prevalence of hypertension and left ventricular hypertrophy, with the lowest prevalence occurring at levels between 5.5 and 7.5 mg/dL, even after adjustment for covariates, with the lowest odds ratio in this range.</p><p><strong>Conclusions: </strong>In pediatric CKD, higher serum uric acid levels were associated with male sex, older age, and lower eGFR, suggesting that these factors should be considered in clinical management. Additionally, the U-shaped relationship between serum uric acid levels and cardiovascular risk of hypertension and left ventricular hypertrophy underscores the importance of maintaining serum uric acid within an optimal range.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2025-10-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145293268","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 history and future landscape for paediatric ABO incompatible kidney transplantation.","authors":"Anshuman Saha, Rajiv Sinha, Stephen D Marks","doi":"10.1007/s00467-025-06996-8","DOIUrl":"https://doi.org/10.1007/s00467-025-06996-8","url":null,"abstract":"<p><p>In the context of limited donor availability and long waiting times for deceased donor transplantation, ABO-incompatible kidney transplantation (ABOiKT) does give a ray of hope for children with kidney failure. The initial challenge of hyperacute rejection secondary to naturally occurring anti-A or anti-B antibodies has been overcome with advances in desensitisation and immunosuppressive protocols. Younger children have immunological advantages due to lower baseline isohaemagglutinin titres and a more adaptable immune system. Pre-transplant desensitisation strategies have evolved from invasive surgical procedures, including splenectomy, to less invasive and more acceptable medical treatments, combining plasmapheresis or immunoadsorption with intravenous rituximab and/or low-dose intravenous immunoglobulin. Post-transplant management includes close monitoring for antibody-mediated rejection, infectious complications, and continuation of standard maintenance immunosuppressive regimens. Graft outcomes remain comparable to ABO compatible kidney transplantation (ABOcKT) but the risk of infectious complications, requirement for intensive desensitisation procedures, and lack of standardised desensitisation protocols are challenging. Further refinement of protocols through information gained from multicentre collaboration and long-term outcome data is the current requirement to establish the position of ABOiKT in the management ladder for children with kidney failure.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2025-10-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145286685","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}
Sibel Yel, Neslihan Günay, Pelin Abdal Yıldırım, Belde Kasap Demir, Eren Soyaltın, Aslıhan Kara, Metin Kaya Gürgöze, Hülya Nalçacıoğlu, Arife Uslu Gökçeoğlu, Aslı Kavaz Tufan, İsmail Dursun, Muammer Hakan Poyrazoğlu
{"title":"Multicenter evaluation of ambulatory blood pressure in children with CAKUT: distinctive profiles in the cystic dysplasia subgroup.","authors":"Sibel Yel, Neslihan Günay, Pelin Abdal Yıldırım, Belde Kasap Demir, Eren Soyaltın, Aslıhan Kara, Metin Kaya Gürgöze, Hülya Nalçacıoğlu, Arife Uslu Gökçeoğlu, Aslı Kavaz Tufan, İsmail Dursun, Muammer Hakan Poyrazoğlu","doi":"10.1007/s00467-025-07001-y","DOIUrl":"https://doi.org/10.1007/s00467-025-07001-y","url":null,"abstract":"<p><strong>Background: </strong>Hypertension (HT) is an important risk factor in patients with congenital anomalies of the kidney and urinary tract (CAKUT), independent of the existing anomaly in childhood. This study aimed to investigate masked hypertension and/or blood pressure profiles in different subgroups with a normal glomerular filtration rate (GFR).</p><p><strong>Methods: </strong>The study included participants from seven different pediatric nephrology centers in Türkiye. Ambulatory blood pressure monitoring (ABPM) was performed on patients aged 5-18 years, diagnosed with CAKUT, with a GFR above 100 ml/min/m<sup>2</sup>, and normal office blood pressure measurements. Validated Mobil-O-Graph or Spacelabs devices were used in all centers.</p><p><strong>Results: </strong>In total, 118 healthy control data and 322 patients with CAKUT were evaluated, consisting of 73 (22.7%) with agenesis, 79 (24.5%) with cystic dysplasia, 92 (28.6%) with vesicoureteral reflux, 18 (5.6%) with UPJ (ureteropelvic junction) obstruction, and 60 (18.6%) with other conditions. In all CAKUT patients, daytime systolic blood pressure (SBP) and both day and nighttime diastolic blood pressure (DBP) loads were significantly higher compared to the healthy control group (p < 0.05). Nocturnal hypertension was identified in 58 (18%) of 322 children, whereas none of the control group presented hypertension. Children with cystic dysplasia had the highest nighttime hypertension proportions (22.7%) when compared to other subgroups. All data revealed higher total systolic-diastolic SDS and total mean SDS in the cystic dysplasia subgroup (p < 0.05).</p><p><strong>Conclusions: </strong>The findings underscore the importance of blood pressure monitoring in the follow-up of patients with CAKUT, especially those with cystic dysplasia, even in the absence of GFR decline.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2025-10-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145280841","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}
Anna S Nidhiry, Dechu Puliyanda, Cindy Blifeld, Helen Pizzo
{"title":"A rare case of hyperuricemia and acute kidney injury in a kidney transplant patient.","authors":"Anna S Nidhiry, Dechu Puliyanda, Cindy Blifeld, Helen Pizzo","doi":"10.1007/s00467-025-06975-z","DOIUrl":"https://doi.org/10.1007/s00467-025-06975-z","url":null,"abstract":"<p><p>Hyperuricemia is a common finding in kidney transplant recipients, often associated with calcineurin inhibitor or diuretic use, obesity, metabolic syndrome, dyslipidemia, high purine intake, and reduced allograft function. We report a unique case of hyperuricemia leading to acute kidney injury associated with the use of cimetidine for treatment of mosaic warts in a 12-year-old female pediatric kidney transplant patient. With the reduction in the patient's serum uric acid levels, there was concurrent marked improvement in her eGFR. Subsequently, her uric acid remained low, suggesting the cessation of cimetidine maintained her baseline uric acid level. This case highlights the significance of monitoring uric acid levels post-transplant and the importance of attention to potential drug interactions.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2025-10-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145280861","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":"CKD<sub>u</sub> in children-a commentary on evidence, insights, and prioritizing prevention.","authors":"Benjamin Russell, Tahagod Mohamed, John D Mahan","doi":"10.1007/s00467-025-07002-x","DOIUrl":"https://doi.org/10.1007/s00467-025-07002-x","url":null,"abstract":"","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2025-10-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145280894","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":"Blood in urine: an early and constant finding for STEC-HUS diagnosis.","authors":"Alejandro Balestracci, Laura F Alconcher","doi":"10.1007/s00467-025-07000-z","DOIUrl":"https://doi.org/10.1007/s00467-025-07000-z","url":null,"abstract":"","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2025-10-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145280864","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}