OBJECTIVES: Pancreatic involvement in inflammatory bowel disease (IBD) represents a heterogeneous group of conditions. Our study assessed the prevalence of hyperlipasemia and pancreatic imaging involvement in children with newly diagnosed IBD. We also evaluated predictors of pancreatic changes on magnetic resonance imaging in this population. METHODS: This retrospective study analysed the standard magnetic resonance enterography (MRE) protocol with additional sequences for pancreatic assessment. The serum activity of lipase, aminotransferases, and total immunoglobulin G (IgG) and its subclasses concentrations were measured at diagnosis, as well as body mass index percentile (pBMI) and IBD activity. RESULTS: Data from 68 children were analysed. Increased IgG concentrations were observed in 25 patients (36.6%) for total IgG and in 25 patients (36.6%) for IgG1. Among 27 patients (39.7%) with increased lipase, 8 (29.6%) had increased IgG concentration and 10 (37.1%) had increased IgG1 concentration; both IgG and IgG1 concentration were elevated in 7 (87.5%) patients. MRE revealed pancreatic involvement in 20.59%. Patients with pancreatic involvement on MRE had higher total IgG (p = 0.012) and IgG1 (p = 0.024) levels and lipase activity 3x higher than the normal limit (p = 0.04). Total IgG and IgG1 concentrations were significantly associated with MRE findings and their combination yielded the highest discriminatory performance. IgG subclasses proved to be useful in a multivariate model incorporating IgG1 and IgG2 concentrations (0.75; 95% confidence interval: 0.59-0.92). CONCLUSION: Structural pancreatic involvement was observed in one-fifth of our patients. They had significantly higher total IgG and IgG1 levels. The IgG1 + IgG2 model performed best in prediction pancreatic MRE changes.
OBJECTIVES: Paediatric liver transplantation (pLT) is associated with excellent long-term survival and quality of life. As a growing proportion of recipients survive into adulthood, long-term follow-up after transfer from paediatric to adult care is increasingly important. We aimed to describe patient and graft survival, retention in care, adherence, medical complications and achievement of an ideal outcome after transfer of care. METHODS: This prospective observational study included all pLT recipients who entered our centre's transition programme between January 2010 and December 2024. Prespecified outcomes included patient and graft survival, retention in adult care, attendance and medication adherence, a composite non-adherence outcome, post-transfer medical complications and the proportion achieving an ideal outcome at predefined timepoints. RESULTS: Sixty patients were included (53.3% male; median age at pLT 5 [2.1-10.4] years; median age at transfer of care 19.8 [interquartile range (IQR) 18.9-24] years). Patient survival at 1, 5, 10 and 15 years after transfer of care was 98%, 96%, 93% and 93%, while graft survival was 98%, 94%, 91% and 75%, respectively. At the time of transfer of care, 55% of patients fulfilled criteria for an ideal outcome; this decreased to 41.6% at 5 years and 25% at 10 years. Medication non-adherence was documented in 20%, and composite non-adherence in 25%. The tacrolimus Medication Level Variability Index was significantly associated with composite non-adherence (p = 0.007) and was higher in patients who developed chronic rejection than in those who did not (p = 0.05). CONCLUSIONS: Despite excellent long-term survival, pLT recipients reaching adulthood face increasing challenges over time, particularly regarding adherence. These descriptive findings support continued, individualised follow-up after transfer to adult healthcare.
OBJECTIVES: The contemporary European Society for Pediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN)/North American Society for Pediatric Gastroenterology, Hepatology and Nutrition (NASPGHAN) framework broadens the definition of cystic fibrosis hepatobiliary involvement (CFHBI), but the biological interpretation of individual multiparametric ultrasound parameters within this framework remains unclear. We evaluated the biological associations of shear wave elastography (SWE), shear wave dispersion (SWD), and attenuation imaging (ATI) with histopathological phenotypes in children with cystic fibrosis (CF). METHODS: In this ambispective cohort study, 119 children with CF underwent hepatobiliary assessment, including SWE, SWD, ATI, Doppler ultrasonography, clinical and laboratory evaluation. Histopathological assessment was performed when clinically indicated. CFHBI was classified according to the ESPGHAN/NASPGHAN framework, with the Debray criteria used as a secondary exploratory comparison. Exploratory phenotype-oriented analyses evaluated ultrasound parameters according to corresponding histopathological phenotypes. RESULTS: Exploratory phenotype-oriented analyses demonstrated significantly higher ATI values in histopathologically defined steatosis (p < 0.001), with good discriminatory performance (AUC 0.813) and an optimal threshold of 0.655 dB/cm/MHz. In contrast, SWE was not associated with histopathologically defined fibrosis (p = 0.151), whereas SWD showed no significant phenotype-specific associations. Secondary exploratory analyses demonstrated significant overall CFHBI discrimination by all three ultrasound parameters, with the highest AUC observed for SWD. The ESPGHAN/NASPGHAN and Debray classifications demonstrated significant disagreement (McNemar p < 0.001), with 22.7% of patients classified as Debray-negative but ESPGHAN/NASPGHAN-positive. CONCLUSIONS: Multiparametric ultrasound should be interpreted within the contemporary ESPGHAN/NASPGHAN CFHBI framework rather than as a single diagnostic tool for hepatobiliary involvement. The distinct biological associations of SWE, ATI, and SWD support a phenotype-oriented interpretation of individual ultrasound parameters, whereas overall CFHBI discrimination should be interpreted as a secondary exploratory finding requiring prospective multicenter validation.
Abstract Objective Paediatric inflammatory bowel disease (IBD) surgery is delivered across heterogeneous settings, with limited paediatric‐specific evidence guiding organisation of care, perioperative management, and operative strategy. We characterised contemporary international practice. Methods An international cross‐sectional centre‐level survey was conducted within the Paediatric IBD Surgery Network. A 28‐item questionnaire addressing organisation of care, perioperative management, procedural volumes, and surgical practice was distributed electronically. Analyses were descriptive and performed per question. Results Twenty‐six of 36 invited centres from 14 countries responded (72%). University or teaching hospitals represented 96% of centres, and paediatric surgeons accounted for 69% of respondents. Regular multidisciplinary team meetings were reported by 85%. Use of an enhanced recovery after surgery protocol, structured nutritional screening, and deferral of elective surgery for nutritional optimisation whenever feasible were reported by 50%, 54%, and 42% of centres, respectively. Medication withholding varied, from corticosteroids in 69% of centres to anti‐tumour necrosis factor (anti‐TNF) agents in 31% and individual newer biologic or small‐molecule classes in 15–19%. Formal national centralisation was reported as recommended or mandatory by 42%. Median annual luminal Crohn's disease surgery volume per centre was two procedures (interquartile range [IQR] 1–5) in 2018, 2.5 (IQR 2–5) in 2022, and 2.5 (IQR 1–5) in 2024. Median annual ileal pouch–anal anastomosis volume in children with ulcerative colitis was 1 (IQR 0.1–3.5). Conclusions Considerable variation was observed in perioperative optimisation and medication management, integration of medical and surgical therapy, centralisation, and operative strategy. These findings provide a baseline for international benchmarking, consensus development, and prospective collaborative research.
