Subspecialty recognition and certification in Pediatric Nephrology remain heterogeneous across Europe and globally, resulting in variations in training standards and professional mobility. To address this gap, the European Society for Paediatric Nephrology (ESPN) launched the European Board Certification in 2020 as a pan-European and curriculum-based assessment of knowledge and clinical reasoning in Pediatric Nephrology. We performed a descriptive and analytical evaluation of candidates who applied for the ESPN Board Certification in Pediatric Nephrology between 2020 and 2025. Candidate characteristics, examination performance, and factors associated with success were analyzed. The exam consisted of 100 case-based multiple-choice questions developed according to a predefined blueprint. Multivariable logistic regression was used to identify factors independently associated with passing. Additionally, a cross-sectional survey assessed candidates’ perceptions of the examination and its professional impact. A total of 349 pediatric nephrologists from 52 countries and four continents completed the examination. Median age was 38.2 years (IQR 34.7–43.5), and 59.9

BACKGROUND:Continuous kidney replacement therapy (CKRT) has emerged as a valuable treatment option in critically ill neonates and infants with acute kidney injury (AKI) requiring dialysis. In this population, we apply artificial intelligence (AI) to identify factors influencing mortality and short-term adverse kidney outcomes. METHODS:The study involved neonates and infants included in the EurAKId Registry (NCT02960867), who underwent CKRT treatment. Using the AI XGBoost models, we identified key clinical factors associated with short-term outcomes: mortality before hospital discharge, as well as proteinuria at discharge. We considered the patients' clinical characteristics, anthropometric features, and CKRT technical settings. RESULTS:The study comprised 95 patients: 31.6% neonates and 68.4% infants with a median age at hospital admission of 1 month (interquartile range, IQR 0-7 months). Ten children were born prematurely. The overall mortality rate was 47.3% and did not differ significantly between neonates and infants (53.3% vs 44.4%, respectively, P = .422). The XGBoost model for predicting mortality had the accuracy of 59.53% ± 0.96% and AUC of 0.64 ± 0.11. Lower urine output at CKRT initiation, a greater rise in serum creatinine (SCr), longer time to dialysis initiation, and lower blood pressure were associated with increased risk of mortality. Proteinuria at hospital discharge was present in 30.6% of survivors. The XGBoost model for predicting proteinuria had the accuracy of 79.11% ± 2.46% and AUC (0.74 ± 0.04). Higher SCr concentrations at hospital admission and at CKRT start, as well as primary kidney disease were the most important risk factors for proteinuria. CONCLUSION:We propose the XGBoost models for identifying factors associated with short-term outcomes of CKRT in neonates and infants. Lower urine output at CKRT start, more severe AKI progression and longer time to CKRT initiation might be important risk factors for mortality in infants and neonates. Primary kidney disease and related biochemical parameters are strong predictors of proteinuria at hospital discharge.

BACKGROUND:Carotid-femoral pulse wave velocity (PWV) is the gold standard measure of central arterial stiffness and a predictor of cardiovascular events in adults, with growing relevance in pediatric populations. Although PWV increases naturally with age, cardiometabolic risk factors may accelerate this process. This study compared carotid-femoral PWV between non-overweight and overweight/obese adolescents, evaluated its progression from prepubertal age to early adolescence, and identified independent determinants of arterial stiffness. METHODS:This longitudinal study included 118 participants from the Generation XXI cohort (Porto, Portugal) assessed at ages 8-9 and 13-14 years. Carotid-femoral PWV was measured at both time points using a validated portable device. Anthropometric data, office blood pressure, and fasting blood samples for lipid profile, glucose, and insulin were collected. Body mass index (BMI) classification followed World Health Organization references. RESULTS:PWV increased significantly from childhood to adolescence (5.01 ± 0.47 to 6.30 ± 1.05 m/s; p < 0.001). At 13-14 years, overweight/obese adolescents had significantly higher PWV than non-overweight peers. PWV at 13-14 years correlated positively with BMI, BMI z-score, systolic and diastolic blood pressure, and fasting glucose. In adjusted models, PWV at 8-9 years (β = 0.39; p = 0.039) and BMI z-score at 13-14 years (β = 0.33; p < 0.001) remained independent predictors of PWV at 13-14 years. CONCLUSIONS:Arterial stiffness in early adolescence reflects both vascular tracking from childhood and concurrent adiposity. These findings support early vascular screening and weight management interventions during key developmental periods to optimize long-term cardiovascular health.

Renal tubular dysgenesis (RTD) is a rare disorder characterized by impaired development of the renal tubules. It is often a fatal condition that should be considered in the differential diagnosis of neonatal kidney failure. RTD can be classified as primary (linked to deleterious variants in genes encoding renin-angiotensin system (RAS) proteins) or secondary to an underlying cause. In this case report, we present a late preterm female neonate born at 35 weeks by elective cesarean section due to oligohydramnios and fetal growth restriction. At birth, she exhibited hypotonia and features consistent with Potter sequence and developed persistent anuric kidney failure, fluid-responsive hypotension, and respiratory distress requiring non-invasive ventilation. Kidney ultrasound revealed no significant abnormalities, leading to a presumptive diagnosis of RTD, which was confirmed by histopathology. Karyotype analysis revealed 46,XX,dup(1)(q24.1q25.1), which was further confirmed by whole exome sequencing. The chromosomal abnormality did not involve RAS genes, and the remaining workup was unremarkable. Despite intensive medical management, the patient died on day 20 of life. The aim of this case report was to raise awareness of this severe kidney disorder, highlighting its atypical presentation, which lacked major cardiovascular dysfunction, showed no identifiable classic etiology despite thorough investigation, and revealed a de novo chromosomal abnormality. These findings suggest the involvement of alternative pathophysiologic mechanisms in RTD.

Rare diseases affect fewer than 1 in 2000 individuals, but approximately 150 rare kidney diseases account for about 10

Continuous kidney replacement therapy (CKRT) has recently become the preferred kidney replacement modality for children with acute kidney injury (AKI). We hypothesise that CKRT technical parameters and treatment settings in addition to the clinical characteristics of patients may influence the circuit lifetime in children. The study involved children included in the EurAKId registry (NCT 02960867), who underwent CKRT treatment. We analysed patient characteristics and CKRT parameters. The primary end point was mean circuit lifetime (MCL). Secondary end points were number of elective circuit changes and occurrence of dialysis-related complications. The analysis was composed of 247 children who underwent 37,562 h of CKRT (median 78, IQR 37–165 h per patient). A total of 1357 circuits were utilised (3, IQR 2–6 per patient). MCL was longer in regional citrate anticoagulation (RCA), compared to heparin (HA) and no anticoagulation (NA) (42, IQR 32-58 h; 24, IQR 14-34 h; 18, IQR 12-24 h, respectively, p < 0.001). RCA was associated with longer MCL regardless of the patient’s age or dialyser surface. In multivariate analysis, MCL correlated with dialyser surface area (beta = 0.14, p = 0.016), left internal jugular vein vascular access site (beta = -0.37, p = 0.027), and the use of HA (beta = -0.14, p = 0.038) or NA (beta = -0.37, p < 0.001) vs. RCA. RCA was associated with the highest ratio of elective circuit changes and the lowest incidence of complications. Anticoagulation modality, dialyser surface, and vascular access site influence MCL. RCA should be considered when choosing first-line anticoagulation for CKRT in children. Further efforts should focus on developing guidelines and clinical practice recommendations for paediatric CKRT.

BackgroundCongenital anomalies of the kidney and urinary tract (CAKUT) are defined as structural malformations of the kidney and/or urinary tract. Heat shock proteins (HSPs) are expressed in the kidney in response to cellular changes, such as thermal, hemodynamic, osmotic, inflammatory, and mechanical stresses. This study aimed to assess uHSP70 levels during acute urinary tract infections (UTI) and non-infection periods in patients with CAKUT, and to evaluate whether uHSP70 is elevated in CAKUT subtypes.MethodsAmong patients with CAKUT, 89 patients with UTI (CAKUT-A), 111 without UTI (CAKUT-B), and 74 healthy children were included in the study. uHSP70 levels were measured using enzyme-linked immunosorbent assay (ELISA).ResultsuHSP70 level was significantly higher in the CAKUT-A group than in the CAKUT-B and healthy control groups (p < 0.0001). Moreover, the level of uHSP70 was significantly higher in the CAKUT-B group than in the control group (p < 0.0001), but was not different between the CAKUT subtypes (p > 0.05).ConclusionUrine HSP70 can also be used to predict UTI in patients with CAKUT. Moreover, uHSP70 levels were higher in children with CAKUT during the non-infectious period than in healthy controls. This suggests that children with CAKUT are at risk of chronic non-infectious damage.

BackgroundThere is a lack of information on the current healthcare systems for children with kidney diseases across Europe. The aim of this study was to explore the different national approaches to the organization and delivery of pediatric nephrology services within Europe.MethodsIn 2020, the European society for Paediatric Nephrology (ESPN) conducted a cross-sectional survey to identify the existing pediatric nephrology healthcare systems in 48 European countries covering a population of more than 200 million children.ResultsThe reported three most important priorities in the care of children with kidney diseases were better training of staff, more incentives for physicians to reduce staff shortages, and more hospital beds. Positive achievements in the field of pediatric nephrology included the establishment of new specialized pediatric nephrology centers, facilities for pediatric dialysis and transplant units in 18, 16, and 12 countries, respectively. The most common problems included no access to any type of dialysis (12), inadequate transplant programs for all ages of children (12), lack of well-trained physicians and dialysis nurses (12), inadequate reimbursement of hospitals for expensive therapies (10), and lack of multidisciplinary care by psychologists, dieticians, physiotherapists, social workers and vocational counsellors (6). Twenty-five of 48 countries (52%) expected to have a shortage of pediatric nephrologists in the year 2025, 63% of clinical nurses and 56% of dialysis nurses. All three groups of health care professionals were expected to be lacking in 38% of countries. Prenatal assessment and postnatal management of renal malformations by a multidisciplinary team including obstetricians, geneticists, pediatricians, and pediatric surgeons was available in one third of countries.ConclusionsOur study shows that there are still very marked differences in pediatric health care systems across the European countries and highlights the need need for appropriate services for children with kidney disease in all European countries.

INTRODUCTION:Recent findings suggest that the four-variable Kidney Failure Risk Equation (KFRE) may be useful in predicting the likelihood of allograft failure in adult kidney transplant (KT) recipients. However, research on its application in pediatric patients is lacking. This study aimed to assess the accuracy of the four-variable KFRE for prediction of the two-year and five-year risk of allograft failure in a cohort of pediatric KT recipients. METHODS:A retrospective observational study of patients undergoing KT in a tertiary pediatric nephrology unit between 2007 and 2017 was conducted. The KFRE risk scores were determined using data collected one-year post-transplantation. Discrimination and calibration properties of the four-variable KFRE were assessed through the area under the receiver operating characteristic curves (AUC) and calibration plots. RESULTS:Fifty-nine patients with a median age of 12.4 (9.1-15.7) years at KT were included. Eleven (18.6%) were living donor recipients. The median estimated glomerular filtration rate one-year post-transplantation was 62.0 (49.0-75.0) mL/min/1.73 m2. One (1.7%) and three (5.1%) patients experienced allograft failure within two and five years following the one-year post-transplantation date, respectively. The four-variable KFRE showed excellent and very good discrimination for the two-year and five-year risks, respectively (AUC 0.966, 95% confidence interval (CI) 0.914-1.000; AUC 0.887, 95% CI 0.732-1.000). Calibration plots demonstrated imprecise calibration. CONCLUSION:The four-variable KFRE shows promise in predicting kidney failure progression in pediatric KT recipients with a functioning allograft one-year post-transplantation. Larger-scale studies are essential to confirm its predictive accuracy and establish more definitive conclusions.

Abstract Background and Aims Emerging evidence suggests that the 4-variable Kidney Failure Risk Equation (KFRE) can be used to predict the risk of graft failure in adult transplant recipients. However, research studies in pediatric patients are lacking. This study aimed to validate the 4-variable KFRE (age, sex, estimated glomerular filtration rate [eGFR], and urine albumin-to-creatinine ratio [uACR]) for prediction of the 2- and 5-year risk of graft failure in a group of pediatric transplant patients. Method A retrospective observational study involving 59 pediatric patients who underwent kidney transplant between January 2007 and December 2017 was conducted. The KFRE risk scores were calculated with data collected at 1-year post-transplantation. The area under the receiver operating characteristic curves (AUC) and calibration plots were used to assess the discrimination and calibration properties of the 4-variable KFRE in predicting the risk of graft failure at 2 and 5 years from the point of the 1-year post-transplantation measurements. Results Among the 59 patients, 41 (69.5%) were male, with median age of 12.8 years (interquartile range 9.2-15.7) at the time of the kidney transplant. Eleven (18.6%) were living donor recipients. Median eFGR at 1-year post-transplantation was 62.0 mL/min/1.73 m2 (50.0-76.5); 1 (1.7%) and 3 (5.1%) patients developed graft failure within 2 and 5 years from the point of the 1-year post-transplantation measurements, respectively. The 4-variable KFRE showed excellent discrimination for the 2-year risk (AUC 0.966, 95% confidence interval [CI] 0.914-1.000) and very good discrimination for the 5-year risk (AUC 0.887, 95% CI 0.732-1.000). Calibration plots however showed imprecise calibration. A discernible trend was apparent in this sample, suggesting that living donor recipients have increased probability of experiencing graft failure in the first 5 years (p = 0.069). Conclusion The 4-variable KFRE may be of interest in predicting kidney failure progression in pediatric kidney transplant recipients using data at 1-year post-transplant, however to substantiate these findings and ensure the robustness of conclusions, larger-scale studies should be conducted.

OBJECTIVES:Type 1 diabetes mellitus is considered a state of chronic low-grade inflammation and activation of the innate immune system, which is regulated by several proinflammatory cytokines and other acute-phase reactants. Arterial stiffness, a dynamic property of the vessels evaluated by the determination of pulse wave velocity (PWV), is increased in diabetic patients and is associated with microvascular and macrovascular complications of diabetes and higher cardiovascular risk. In the present study, we aimed to compare the proinflammatory state and arterial stiffness in diabetic and non-diabetic adolescents, and to characterize the association between these two parameters. METHODS:Twenty-three type 1 diabetic patients, aged 12-16 years, followed at a tertiary center, and 23 adolescents nonoverweighted healthy controls, from a Portuguese birth-cohort, were included in the present analysis. Anthropometry, blood pressure, glycemic control data, and lipid parameters were collected. Arterial stiffness was evaluated by carotid-femoral pulse wave velocity. Proinflammatory cytokines' concentrations (TNF-α, IL-1β, IL-6, IL-10, IFN-γ, and GM-CSF) were quantified by multiplex immunoassays using a Luminex 200 analyzer. RESULTS:There were no statistically significant differences between the proinflammatory cytokines' concentrations in the two groups. PWV [6.63 (6.23-7.07) vs. 6.07 (5.15-6.65) m/s, p=0.015] was significantly higher in the diabetic group. PWV was negatively correlated with GM-CSF (ρ=-0.437, p=0.037) in the diabetic group. A linear association was found between diabetes duration and PWV (with PWV increasing by 0.094 m/s (95 % confidence interval, 0.019 to 0.169) per month of disease duration). In the diabetic group, HbA1c was negatively correlated with IL-10 (ρ=-0.473, p=0.026). Negative correlations were also found between IL-10 and total, HDL, and LDL cholesterol only in the diabetic group. CONCLUSIONS:Diabetic adolescent patients present higher PWV, when compared to their healthy counterparts, even though we could not find differences in the levels of several proinflammatory cytokines between the two groups. The negative correlation found between IL-10 and HbA1c might translate a protective counterbalance effect of this anti-inflammatory cytokine, which might also explain the negative correlations found with blood lipids. Further studies are needed to better clarify the association between arterial stiffness and the proinflammatory milieu of diabetes.

Background Prenatal diagnosis (PND) of aortic coarctation (AoCo) has been associated with a significant improvement in early results, but there is limited information on the long-term cardiovascular outcome. Methods We studied 103 patients with simple AoCo, operated in the neonatal period, with a median follow-up of 8,5 years (2 to 23,7 years), with 47% followed for over 10 years. PND was made in 35%. The primary aim was to determine the short and long-term cardiovascular impact of PND of AoCo. Results Neonates with PND had less preoperative neonatal complications, with only 2,8% incidence of a composite preoperative severe morbidity course, compared to 28% in the postnatal group. PND patients underwent surgery 8 days earlier and had a shorter length of stay in ICU. PND did not impact the incidence of post-operative complications. On the long-term, prevalence of hypertension, left ventricular hypertrophy and rate of recoarctation were not influenced by PND. The PND group had mean 24 h diastolic BP 9 mmHg lower and mean daytime diastolic BP 11 mmHg lower. In the final multivariable model, PND was the single independent variable correlating with daytime diastolic BP. Conclusion PND of AoCo effectively leads to a better pre-operative course with less pre-operative morbidity. We found no significant differences in immediate post-operative cardiovascular outcomes. A better initial course of patients with PND does not have a major long-term impact on cardiovascular outcomes, nevertheless, at late follow-up PND patients had lower diastolic BP values on ambulatory monitoring, which may have an impact on long-term cardiovascular risk.