Introduction In current practice, fluid volumes administered to children following kidney transplant vary widely. Up to 52% of children experience fluid overload-related complications. Current fluid guidelines are not evidence-based and the optimal amount of fluid for children after transplant is not known. The aim of Randomised multiple centre trial of conservative versus LIberal fluid adMInisTration for children receiving a kidney tranSplant (LIMITS) is to determine whether relative limitation of fluid volume administered to children receiving kidney transplants is superior to liberal fluid volume administration.Methods and analysis LIMITS is a pragmatic, open-label, UK-based, multicentre randomised controlled trial, with an internal pilot phase and integrated economic evaluation. A total of 140 children receiving kidney transplants will be randomised to receive either conservative postoperative fluid administration (maximum of 150 mL/m2/hour for no longer than 18 hours, followed by a fixed daily target of maximum 1.5 L/m2/day thereafter) versus the comparator of liberal postoperative fluid administration (fluid volume administered to replace urine output and insensible losses for at least 48 hours with target urine output >2 mL/kg/hour). The primary outcome is mean days at home in the first 30 days after kidney transplant. The primary outcome will be analysed using a mixed linear regression model adjusted for donor type (living vs deceased donor) and participant weight (<20 kg and ≥20 kg pretransplant) as fixed effects and transplant centre as a random effect. Cost-effectiveness will also be evaluated.Ethics and dissemination The trial received Health Research Authority approval on 20 August 2025 (REC reference: 25/EE/0161, IRAS project ID: 354370). Findings will be presented to academic groups via national and international conferences and peer-reviewed journals. The patient and public involvement group will play an important part in disseminating the study findings to the public domain.Trial registration number ISRCTN21516608.

Background. There is a lack of robust evidence regarding immunosuppressive therapy in children and adolescents after kidney transplantation (KTx), and as such, international practice is highly variable. Recent clinical practice recommendations advocating individualized immunosuppressive strategies that incorporate newer agents are often not implemented. This can potentially contribute to reduced patient and renal allograft survival. Methods. The guideline was developed between January 1, 2024, and December 12, 2025, according to the Guidance Manual of the German Association of Scientific Medical Societies by the German Societies for Pediatric Nephrology, Nephrology, Transplantation, and Pediatrics, the German Kidney Association, the International Pediatric Transplant Association, the European Society for Pediatric Nephrology and the Members of the Cooperative European Pediatric Renal Transplant Initiative. Results. This evidence- and consensus-based guideline provides up-to-date, state-of-the-art recommendations for immunosuppressive therapy after KTx in pediatric kidney transplant recipients. It is based on the best available evidence and the consensus of the relevant German Medical Societies, Members of the Cooperative European Paediatric Renal Transplant Initiative, and the working group on transplantation of the European Society for Paediatric Nephrology, and the International Pediatric Transplant Association. Conclusions. The formal consensus reached is particularly significant in cases of weak or inconclusive evidence and where recommendations are based solely on expert opinion.

Adrenocortical carcinoma (ACC) in children is a rare and aggressive malignancy. Despite representing a small fraction of pediatric adrenal tumors, ACC poses distinct challenges due to its heterogeneous biological behavior, frequent hormone secretion, and the limited availability of standardized, pediatric-specific treatment protocols. Complete surgical resection remains the cornerstone of curative intent therapy, and complete adrenalectomy offers the best chance for long-term survival. Given the aggressiveness of ACC, particularly in advanced disease, adjuvant therapies are often considered to improve outcomes. The aim of this consensus meeting was to bring together international experts to develop international guidance based on the best available evidence and collective clinical experience to harmonize long-term management. A modified 3-step Delphi consensus method was used to finalize the statements from an international panel of pediatric ACC experts from 4 continents (Europe, the Americas, and Asia, and Oceania). An electronic survey was developed by the steering committee (ENSAT Kids) and distributed to the panel. Agreement and disagreement were rated using a 6-point Likert scale with the opportunity to provide structured feedback. Consensus was defined a priori as ≥75% agreement. This Delphi process yielded consensus statements for therapy guidance, comprising 12 surgical statements and 68 medical treatment statements, organized by strategy and disease stage. These statements are intended to support clinicians in delivering more consistent and standardized therapeutic strategies for pediatric ACC. Future efforts should prioritize the design and conduct of high-quality, collaborative research studies in order to provide more evidence and therapy improvements.

Phaeochromocytomas and paragangliomas (PPGL) are rare neuroendocrine tumours that arise not only in adulthood but also in childhood and adolescence. Up to 70-80% of childhood PPGL are hereditary, accounting for a higher incidence of metastatic and/or multifocal PPGL in paediatric patients than in adult patients. Key differences in the tumour biology and management, together with rare disease incidence and therapeutic challenges in paediatric compared with adult patients, mandate close expert cross-disciplinary teamwork. Teams should ideally include adult and paediatric endocrinologists, oncologists, cardiologists, surgeons, geneticists, pathologists, radiologists, clinical psychologists and nuclear medicine physicians. Provision of an international Consensus Statement should improve care and outcomes for children and adolescents with these tumours.

OBJECTIVES:Childhood-onset systemic lupus erythematosus (cSLE), representing 15%-20% of individuals with SLE, has been difficult to study globally due to differences between registries. This initiative, supported by Childhood Arthritis Rheumatology Research Alliance (CARRA) and Paediatric Rheumatology European Society (PReS), aims to create Core and Expanded cSLE Datasets to standardise and enhance research worldwide. METHODS:21 international cSLE experts and 4 patients participated in a Delphi process (questionnaires, 2 topic-specific focus groups and 3 virtual consensus meetings) to create 2 standardised cSLE datasets. The Core cSLE Dataset was designed to include data essential to meaningful clinical research across many settings. The Expanded cSLE Dataset was designed for centres able to consistently collect data to address broader research questions. Final data items for the Core and Expanded datasets were determined by consensus defined as >80% agreement) using an adapted nominal group technique and voting. RESULTS:The resulting Core cSLE Dataset contains 46 items, including demographics, clinical features, laboratory results, medications and significant adverse events. The Expanded cSLE Dataset adds 26 additional items and includes patient-reported outcomes. Consensus was also achieved regarding the frequency and time points for data collection: baseline, quarterly follow-up visits, annually and flare visits. CONCLUSION:Standardised Core and Expanded cSLE Datasets for registry-based international cSLE research were defined through the consensus of global experts and patient/caregiver representatives, endorsed by CARRA and PReS. These datasets incorporate disease-specific and patient-specific features, optimised for diverse settings to facilitate international collaborative research for children and adolescents with SLE worldwide.

BackgroundElectronic (e-)alerts for rising serum creatinine values are increasingly used as clinical indicators of acute kidney injury (AKI). The aim of this study was to investigate to what degree AKI episodes, as identified using e-alerts, correlated with coding for AKI in the hospital record for a national cohort of hospitalised children and examine whether coding corresponded with 30-day mortality after an AKI episode.MethodsA cross-section of AKI episodes based on alerts issued for children under 18 years in England during 2017 were linked to hospital records. Multivariable logistic regression was used to examine patient and clinical factors associated with AKI coding. Agreement between coding and 30-day mortality was examined at hospital level.Results6272 AKI episodes in 5582 hospitalised children were analysed. Overall, coding was poor (19.7%). Older age, living in the least deprived quintile (odds ratio (OR) 1.4, 95% Confidence Interval (CI) 1.1, 1.7) and higher peak AKI stage (stage 1 reference; stage 2 OR 2.0, 95% CI 1.7, 2.4; stage 3 OR 8.6, 95% CI 7.1, 10.6) were associated with higher likelihood of coding in the hospital record. AKI episodes during birth admissions were less likely to be coded (OR 0.4, 95% CI 0.3, 0.5). No correlation was seen between coding and 30-day mortality.ConclusionsThe proportion of AKI alert-identified episodes coded in the hospital record is low, suggesting under-recognition and underestimation of AKI incidence. Understanding the reasons for inequalities in coding, variation in coding between hospitals and how alerts can enhance clinical recognition is needed.

Pediatric anti-phospholipid syndrome (APS) is a rarely diagnosed multisystem autoimmune inflammatory disorder resulting in recurrent vascular thrombosis with serious clinical implications. We highlight a case of an adolescent female who presented with Catastrophic APS (CAPS) in hypertensive emergency and end organ damage. She developed reduced kidney function, fluid overload and neurological manifestations. Neuroimaging and kidney biopsy confirmed micro-angiopathic thrombosis. Her blood results showed high titers of triple positive anti-phospholipid antibodies with negative serology for other immunological conditions. She was managed with dual anti-coagulation and immunosuppression and remains under pediatric subspecialist follow-up for ongoing multisystem involvement. APS should be considered as a differential diagnosis in children presenting with sepsis and multi-organ dysfunction. Pediatric focused diagnostic criteria and long-term management is needed to cater to needs of the pediatric population.

Childhood-onset systemic lupus erythematosus (cSLE) is a severe lifelong and life-threatening autoimmune disease with multi-organ involvement. Compared to those with adult-onset disease, cSLE patients have more aggressive disease with a higher prevalence of early lupus nephritis (LN) causing worse kidney and patient outcomes. The transfer of adolescent patients to adult healthcare poses several major challenges, from a disease as well as a psychosocial perspective. Transitional care even in tertiary centers can be heterogenous, suboptimal, and often even non-existent. In this comprehensive review of the literature, we synthesize the obstacles adolescents and young adults (AYA) with systemic lupus erythematosus (SLE) and LN face and how these challenges impact the transfer to adult health care. Finally, we propose a framework for a structured and individually modifiable transitional care plan, tailored to the unique needs of this population and taking into account their social and cultural background. This framework includes suggestions for the timing of the preparatory phase and the transfer itself, the composition of the transitional care team, increasing transition readiness and treatment adherence, and establishing a supportive network of peers. Efficient transitional care will optimize long-term patient outcomes.

Atypical haemolytic uraemic syndrome (aHUS) is a rare kidney disease characterized by thrombotic microangiopathy. This study presents the first analysis of UK patients enrolled in the Global aHUS Registry, focusing on patient characteristics and disease natural history prior to treatment initiation (n = 172; 74 paediatric, 98 adult). Mean age at first aHUS manifestation was 23.6 years overall (4.9 years for paediatric patients, 37.8 years for adults). Additional thrombotic microangiopathy events occurred in 57.0% of patients between initial clinical suspicion and registry enrolment. Potential precipitating factors were recorded in 14.0% of patients. Of 115 patients at active sites, 90.4% had genetic data recorded, with 73.8% undergoing "complete" genetic testing (results entered for C3, CD46, CFH, CFB and CFI, as a minimum). Of those with genetic data available, 52.9% had an identified pathogenic variant. Gastrointestinal involvement was the most common extra-renal manifestation, presenting in 22.2% of patients. End-stage kidney disease (ESKD) was present in 8.7% at baseline. ESKD-free survival probability at five years was 0.80 for paediatric patients and 0.57 for adults. ESKD-free survival was negatively influenced by CFH, C3, or CFI variants. This study highlights the historically poor prognosis for untreated patients with aHUS. The UK population of the Global aHUS Registry represents a valuable research cohort with comprehensive demographic data and high genetic characterization. These findings underscore the importance of early aHUS identification and intervention to prevent ESKD and improve patient outcomes.

Background and hypothesis Knowledge regarding access to first kidney transplantation (KT) and subsequent KT in patients commencing kidney replacement therapy (KRT) in childhood is limited.Methods Using European Renal Association (ERA) Registry data, we investigated European patients who started KRT below 20 years of age between 1978 and 2019. Access and determinants to first, second, and third KT were assessed using multivariable Cox regression.Results Totals of 12 623, 4077, and 1186 patients were included while awaiting first, second, and third KT, at median ages of 13.8 (IQR: 7.5-17.4), 20.9 (IQR: 16.5-26.1), and 26.6 (IQR: 20.3-32.8) years, respectively. During the study period, overall access was 87.8%, 72.7%, and 60.5% for first, second, and third KT, respectively, and median time to each KT was 0.9 (IQR: 0.2-2.1), 1.9 (0.6-4.5), and 2.6 (IQR: 1.0-5.3) years. Younger age at KRT initiation (aHR 0-4 vs. 10-14 years: 0.54; 95%CI: 0.51-0.57) and female sex (HR: 0.94; 95%CI: 0.90-0.98) were associated with lower access to first KT. KT candidates between 15 and 19 years had lower access to first and second KT (aHR: 0.69; 95%CI: 0.66-0.73, and aHR: 0.70; 95%CI: 0.61-0.81) compared to 10-14 year-olds. Compared to CAKUT, glomerulonephritis patients had lower access to KT (aHR: 0.75; 95%CI: 0.71-0.80 for first, aHR: 0.89; 95%CI: 0.81-0.98 for second, and aHR: 0.80; 95%CI: 0.66-0.97 for third KT). Similarly, patients with primary renal diseases with high risk of recurrence, had lower chances of receiving a first and second KT (aHR: 0.80; 95%CI: 0.76-0.85 for first, aHR: 0.86; 95%CI: 0.78-0.95 for second KT). Access to re-transplantation was also higher with previous pre-emptive KT and previous graft survival exceeding 5 years.Conclusion Our study highlights KT access disparities particularly for females, the youngest recipients, high-risk age (15-19 years), and diseases with recurrence risk. Notably, pre-emptive transplants and enduring previous grafts offer advantages regarding re-transplantation.