Pediatric NephrologyPub Date : 2026-10-01Epub Date: 2026-04-24DOI: 10.1007/s00467-026-07318-2
Mehmet Besrek, Nadir Yalcin, Izzet T Akbasli
{"title":"Extracorporeal circuit-related hemodilution in pediatric CKRT: the dilution factor concept.","authors":"Mehmet Besrek, Nadir Yalcin, Izzet T Akbasli","doi":"10.1007/s00467-026-07318-2","DOIUrl":"10.1007/s00467-026-07318-2","url":null,"abstract":"","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"3649-3650"},"PeriodicalIF":2.5,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147777678","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 : 2026-10-01Epub Date: 2026-03-31DOI: 10.1007/s00467-026-07284-9
Yanting Lu, Yujun Luo
{"title":"Comment on \"The impact of urine pH on lithogenic risk profile in children with urolithiasis\".","authors":"Yanting Lu, Yujun Luo","doi":"10.1007/s00467-026-07284-9","DOIUrl":"10.1007/s00467-026-07284-9","url":null,"abstract":"","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"3663"},"PeriodicalIF":2.5,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147581456","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":"Predictive factors for hypertensive encephalopathy in hospitalized pediatric patients: a retrospective case-control study.","authors":"Pannawat Sengsomwong, Surachai Likasitwattanakul, Nuntawan Piyaphanee, Thanaporn Chaiyapak, Kraisoon Lomjansook, Suroj Supavekin, Anirut Pattaragarn","doi":"10.1007/s00467-026-07300-y","DOIUrl":"10.1007/s00467-026-07300-y","url":null,"abstract":"<p><strong>Background: </strong>Hypertensive emergency in children often exhibits neurological symptoms indicative of hypertensive encephalopathy (HE). The risk factors concerning HE development remain unclear, motivating the objective of this study to identify risk factors and formulate an equation for forecasting HE in hospitalized pediatric patients.</p><p><strong>Methods: </strong>This retrospective case-control study focused on pediatric patients aged 1-18 years diagnosed with hypertension from 2011 to 2021. Logistic regression analysis was utilized to identify variables associated with HE. A HE predictive equation was developed based on significant factors, with sensitivity, specificity, and predictive values assessed using receiver operating characteristics curves.</p><p><strong>Results: </strong>Three hundred thirty-two patients with mean age 9.3 years were recruited. 12.3% developed HE. Univariable analysis revealed risk factors for HE, including central nervous system symptoms, peak systolic and diastolic blood pressure z-score (Z-SBP and Z-DBP), corticosteroid and calcineurin inhibitor use, and leukemia/lymphoma. Logistic regression formed the equation predicting HE occurrence as follows: 2.162 (vomiting) + 2.921 (headache/dizziness) + 2.363 (leukemia/lymphoma) + 1.807 (corticosteroid) + 0.783 (peak Z-SBP). The equation demonstrated robust correlation with predicted probability of developing HE and had AUC of 0.95. A cutoff score of 4 showed high sensitivity (97.6%) and negative predictive value (99%), identifying 98% of HE cases.</p><p><strong>Conclusions: </strong>This study pinpointed key risk factors and introduced an accurate predictive equation, underscoring the significance of assessing multiple factors beyond blood pressure levels for HE prediction in hypertensive pediatric patients.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"3423-3431"},"PeriodicalIF":2.5,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147819184","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":"Ambulatory blood pressure abnormalities, obstructive sleep apnea, and end-organ damage in children with sickle cell disease.","authors":"Harshitha S, Athira Puthukara, Amber Kumar, Abhishek Goyal, Tanya Sharma, Bhavna Dhingra, Narendra Chaudhary, Shikha Malik, Girish Chandra Bhatt","doi":"10.1007/s00467-026-07342-2","DOIUrl":"10.1007/s00467-026-07342-2","url":null,"abstract":"<p><strong>Background: </strong>Many individuals with sickle cell disease (SCD) survive into adulthood, posing a challenge in managing and preventing damage to vital organs such as the heart and kidneys. Studies on hypertension in this population have shown conflicting results, and limited studies on polysomnography highlight a gap in research. This study aims to investigate the prevalence of ambulatory blood pressure abnormalities, obstructive sleep apnea (OSA), and end-organ damage in these children and examine the relationship between these factors.</p><p><strong>Methods: </strong>Fifty-nine children aged 5-18 years with SCD (homozygous and compound heterozygous) were evaluated through history, laboratory tests, ambulatory blood pressure monitoring (ABPM), and polysomnography; estimated glomerular filtration rate (eGFR) by creatinine and cystatin C, echocardiography including left ventricular mass index (LVMI), carotid intimal medial thickness (CIMT), and flow-mediated dilation (FMD) were also assessed.</p><p><strong>Results: </strong>Ambulatory hypertension, masked hypertension, and non-dipping pattern were observed in 13.6%, 11.9%, and 66.1% of participants, respectively. End-organ damage was noted in 71% and was associated with higher frequency of vaso-occlusive crisis (p = 0.003). OSA was identified in 66.1% of participants, and they had lower hemoglobin (p = 0.004). Higher CIMT and LVMI Z-scores were observed in children with OSA (p = 0.004 and p = 0.016, respectively). A negative correlation was observed between FMD and apnea-hypopnea index (AHI) (p = 0.009). Proteinuria was reported in 32.2% and AHI correlated significantly with urine protein-creatinine ratio (r = 0.3; p = 0.02). Although eGFR by both methods showed poor correlation, agreement improved at lower eGFR.</p><p><strong>Conclusions: </strong>Children with SCD show a high prevalence of ABPM abnormalities, OSA, and early vascular dysfunction.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"3579-3589"},"PeriodicalIF":2.5,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147960197","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}
C Federico Moral-Ortega, Yasmine Ghattas, Rachel S Kronenfeld, Chryso Katsoufis, Wacharee Seeherunvong, Jayanthi Chandar, Carolyn Abitbol, Marissa DeFreitas, Fernando F Corrales-Medina
{"title":"Safety and clinical outcomes of antithrombotic therapy in children and young adults with kidney failure on dialysis: a 10-year single-center retrospective cohort study.","authors":"C Federico Moral-Ortega, Yasmine Ghattas, Rachel S Kronenfeld, Chryso Katsoufis, Wacharee Seeherunvong, Jayanthi Chandar, Carolyn Abitbol, Marissa DeFreitas, Fernando F Corrales-Medina","doi":"10.1007/s00467-026-07523-z","DOIUrl":"https://doi.org/10.1007/s00467-026-07523-z","url":null,"abstract":"<p><strong>Background: </strong>Pediatric and young adult patients with kidney failure on dialysis are at increased risk of developing thrombotic events (TE). Antithrombotic therapy in this population is challenging due to a concomitant elevated risk of bleeding and the lack of pediatric and young adult-specific data.</p><p><strong>Methods: </strong>We conducted a 10-year single-center retrospective cohort study of patients < 23 years of age with kidney failure on dialysis to describe antithrombotic therapy use and evaluate associated safety and clinical outcomes.</p><p><strong>Results: </strong>Among 77 patients with kidney failure on dialysis, 33 (42.9%) received antithrombotic therapy. Common treatment indications included acute TE, secondary thromboprophylaxis due to prior thrombosis, and primary thromboprophylaxis in the setting of an identified prothrombotic laboratory abnormality. Low molecular weight heparins (LMWHs) were the most used agents, followed by aspirin and direct oral anticoagulants (DOACs). Bleeding complications occurred in 3 patients (9.1%), including 2 clinically relevant non-major events and 1 minor event. No use of reversal agents or transfusion was required. Recurrent TE were reported in 5 patients (15.2%). Four of them had documented non-adherence, and one occurred after discontinuation of antithrombotic therapy.</p><p><strong>Conclusions: </strong>Antithrombotic therapy in pediatric and young adult patients with kidney failure on dialysis appears to be safe and may be associated with relatively low thrombotic recurrence rates, particularly when adherence is maintained and therapy is closely monitored. This observation is especially relevant given the limited age-specific evidence and the absence of standardized guidance for antithrombotic management in this population.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":""},"PeriodicalIF":2.5,"publicationDate":"2026-09-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148891257","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}
Manon Estienne, Marina Charbit, Claudia Pasqualini, Mehdi Oualha, Klervie Loiselet, Jean-Paul Duong Van Huyen, Sabine Sarnacki, Véronique Minard-Colin, Christophe Chardot
{"title":"Kidney transplantation in children with extended ilio-caval thrombosis.","authors":"Manon Estienne, Marina Charbit, Claudia Pasqualini, Mehdi Oualha, Klervie Loiselet, Jean-Paul Duong Van Huyen, Sabine Sarnacki, Véronique Minard-Colin, Christophe Chardot","doi":"10.1007/s00467-026-07472-7","DOIUrl":"https://doi.org/10.1007/s00467-026-07472-7","url":null,"abstract":"<p><strong>Background: </strong>Kidney transplantation requires the anastomosis of the graft's kidney vein to the recipient's iliac vein or inferior vena cava (IVC). The venous reconstruction can be challenging in case of extended ilio-caval thrombosis.</p><p><strong>Methods: </strong>We report the case of a child with Denis Drash syndrome, with right Wilms tumor and intra-caval tumoral extension, left nodules of nephroblastomatosis on a horseshoe kidney, proteinuria, and progressive kidney failure, resulting in bilateral nephrectomy and iliac veins and vena cava thrombosis extended to the retro-hepatic IVC. At the age of 7 years, 32 months after the end of the oncological treatment, she underwent kidney transplantation. Venous reconstruction was achieved by using the donor IVC as a venous conduit anastomosed to the recipient's retro-hepatic IVC at the level of the hepatic veins.</p><p><strong>Results: </strong>Postoperative course was uneventful, and the child is alive and well, free of tumor and with normal kidney function, 5 years after the end of oncological treatment, and 32 months after transplantation.</p><p><strong>Conclusions: </strong>This technique appears as a safe and physiological alternative to previously described venous anastomoses to the portal system or pelvic varices, preventing chronic venous hypertension of the graft.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":""},"PeriodicalIF":2.5,"publicationDate":"2026-09-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148891318","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":"Cystinosis-associated metabolic bone disease: pathogenesis and outcome.","authors":"Anne Schön, Malgorzata Szaroszyk, Dieter Haffner","doi":"10.1007/s00467-026-07518-w","DOIUrl":"https://doi.org/10.1007/s00467-026-07518-w","url":null,"abstract":"<p><p>Cystinosis-associated metabolic bone disease (CMBD) is a severe complication in patients with infantile nephropathic cystinosis, which is a rare inherited lysosomal storage disease due to pathogenic variants in the CTNS gene that results in an accumulation of cystine in all organs. The kidneys are the organs primarily affected, resulting in renal Fanconi syndrome at around the age of 6-12 months. Without adequate treatment with the cystine-depleting agent cysteamine, this is followed by progressive chronic kidney disease (CKD) and kidney failure in the second decade of life. Patients usually present with progressive disproportionate short stature, rickets, and bone pain in the first 2 years of life. This may be followed by muscle weakness in childhood, and scoliosis and fractures in adulthood, especially if treatment is inadequate. Early diagnosis and treatment are crucial, and this is facilitated by a positive family history, screening programs and care at specialized centers. Therapeutic measures include physical activity, physiotherapy, adequate calcium and phosphate intake, vitamin D supplements, a combination of phosphate supplements and active vitamin D, correction of metabolic acidosis, and, if necessary, growth hormone therapy and surgical correction of persisting leg deformities. Unfortunately, current treatments cannot always prevent progressive bone loss and related skeletal comorbidity. There is increasing evidence for an underlying intrinsic osteoblast and osteoclast defect in cystinosis. Cystine crystal accumulation in macrophages (e.g., in bone and muscle) may result in an inflammatory state promoting increased osteoclast activity and other alterations in myokines and osteokines. Future interventions may target these factors to improve outcomes in patients with cystinosis.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":""},"PeriodicalIF":2.5,"publicationDate":"2026-09-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148892270","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":"Immunosuppression in children with primary mitochondrial diseases after kidney transplantation: insight from case series of RMND1-related disease.","authors":"Sze Wa Wong, Fred Tomlin, Jelena Stojanovic","doi":"10.1007/s00467-026-07527-9","DOIUrl":"https://doi.org/10.1007/s00467-026-07527-9","url":null,"abstract":"<p><p>While successful kidney transplantation has been reported in pediatric patients with primary mitochondrial diseases, immunosuppression regimen and its effect on systemic disease were not described. We present four pediatric patients with genetically confirmed RMND1 disease in a quaternary nephrology center. They presented at a very young age and progressed rapidly to stage 5 chronic kidney disease. All underwent successful kidney transplantation. Their allograft function remained stable throughout the follow-up period, and they did not manifest any major systemic deterioration.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":""},"PeriodicalIF":2.5,"publicationDate":"2026-09-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148890978","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}
Annick Massart, Emilie Desclée, Amélie Dendooven, Nathalie Ghorra, Hans de Fijter, Dominique Trouet
{"title":"Clinical improvement of acquired partial lipodystrophy in an adolescent with C3 glomerulopathy treated with pegcetacoplan.","authors":"Annick Massart, Emilie Desclée, Amélie Dendooven, Nathalie Ghorra, Hans de Fijter, Dominique Trouet","doi":"10.1007/s00467-026-07504-2","DOIUrl":"https://doi.org/10.1007/s00467-026-07504-2","url":null,"abstract":"<p><p>C3 glomerulopathy and acquired partial lipodystrophy (APL) are rare disorders associated with dysregulation of the alternative complement pathway and occasionally coexist. We report a 14-year-old girl who developed nephritic-nephrotic syndrome due to C3 glomerulonephritis concomitant with APL. Despite treatment with mycophenolate mofetil and tacrolimus, kidney function deteriorated, proteinuria increased, and she developed marked faciotruncal lipoatrophy with a 10-kg weight loss. Pegcetacoplan, a C3/C3b inhibitor, was initiated. Five weeks later, serum C3 levels were normal. Creatinine clearance subsequently improved from 25 to 133 mL/min, while proteinuria progressively declined from 3.2 to 0.15 g/g. Within 40 weeks, the patient recovered her usual body weight with marked clinical improvement in subcutaneous fat distribution. This observation suggests that pegcetacoplan may represent a novel therapeutic option for APL.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":""},"PeriodicalIF":2.5,"publicationDate":"2026-09-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148888162","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":"Training programs for families of children on home peritoneal dialysis: a scoping review.","authors":"Joanne Jones, Amanda Ullman, Amy E Mitchell","doi":"10.1007/s00467-026-07509-x","DOIUrl":"https://doi.org/10.1007/s00467-026-07509-x","url":null,"abstract":"<p><strong>Background: </strong>Peritoneal dialysis (PD) is the preferred home dialysis treatment for children awaiting kidney transplant worldwide. There is significant variance in clinical outcomes across centers, which may be related to PD training for caregivers. This scoping review provides an overview of existing research regarding characteristics of training provided to caregivers of children receiving home PD.</p><p><strong>Methods: </strong>Searches were conducted via PubMed, Embase, CENTRAL, PsycINFO, CINHAL and ProQuest electronic databases (2005-2025). Studies in children under 21 years of age were included. Characteristics of training programs were summarized and compared against the International Society of Peritoneal Dialysis (ISPD) pediatric guidelines. Implementation, clinical and service outcomes were reported. Relationships between training program characteristics and the child and family were also reviewed.</p><p><strong>Results: </strong>Twelve articles met inclusion criteria. Overall, there was poor alignment with existing guidelines. Training programs were reported in most articles (n = 9; 75%); however, few (n = 2; 16%) clearly defined training with objectives and criteria. Most studies reported the use of training tools (n = 10; 83%), training ratios (n = 9; 75%), and nurses as trainers (n = 9; 75%). Learning styles assessment and training hours per day were underreported, and no publications discussed tools to assess health literacy. The most frequently reported clinical outcome was peritonitis rates (n = 10; 83%). Family or child level outcomes were absent from the data.</p><p><strong>Conclusion: </strong>Pediatric PD training practices varied significantly among studies, and alignment with existing guideline recommendations was limited. The relationship between caregiver training and child and family outcomes warrants further investigation.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":""},"PeriodicalIF":2.5,"publicationDate":"2026-09-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148881241","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}