Background. The aims of the study were to determine the incidence rate of pediatric inflammatory bowel disease (PIBD) and its trends for the period of 20022010 and to assess the geographical distribution of PIBD in Slovenia. Materials and Methods. Medical records of patients (018 years) with newly diagnosed IBD during the study period were retrospectively reviewed. Results. The mean incidence rate for IBD in 20022010 was 7.6 per 100,000 children and adolescents per year, 4.5 for Crohns disease (CD), 2.9 for ulcerative colitis (UC), and 0.2 for IBD-unclassified, respectively. The incidence rate increased from 5.8 per 100,000 per year in 20022004 to 8.6 in 20052007 and remained stable afterwards. Statistically significant difference in the incidence rate between the Northeastern and Southwestern parts of the country was observed (). Conclusion. This nationwide study demonstrates that Slovenia is among the European countries with the highest PIBD incidence. During the study period a substantial rise of PIBD incidence was observed during the first half of the study and it seems to have stabilized in the second half. The significant difference in PIBD incidence between Northeastern and Southwestern parts of the country merits further exploration of the possible environmental factors. Background. The aims of the study were to determine the incidence rate of pediatric inflammatory bowel disease (PIBD) and its trends for the period of 20022010 and to assess the geographical distribution of PIBD in Slovenia. Materials and Methods. Medical records of patients (018 years) with newly diagnosed IBD during the study period were retrospectively reviewed. Results. The mean incidence rate for IBD in 20022010 was 7.6 per 100,000 children and adolescents per year, 4.5 for Crohns disease (CD), 2.9 for ulcerative colitis (UC), and 0.2 for IBD-unclassified, respectively. The incidence rate increased from 5.8 per 100,000 per year in 20022004 to 8.6 in 20052007 and remained stable afterwards. Statistically significant difference in the incidence rate between the Northeastern and Southwestern parts of the country was observed (). Conclusion. This nationwide study demonstrates that Slovenia is among the European countries with the highest PIBD incidence. During the study period a substantial rise of PIBD incidence was observed during the first half of the study and it seems to have stabilized in the second half. The significant difference in PIBD incidence between Northeastern and Southwestern parts of the country merits further exploration of the possible environmental factors.

Background and Aims: Data on upadacitinib therapy in children with ulcerative colitis (UC) or unclassified inflammatory bowel disease (IBD-U) are scarce. We aimed to evaluate the effectiveness and safety of upadacitinib as an induction therapy in pediatric UC or IBD-U. Methods: In this multicenter retrospective study, children treated with upadacitinib for induction of remission of active UC or IBD-U from 30 centers worldwide were enrolled. Demographic, clinical, and laboratory data, as well as adverse events (AEs), were recorded at Week 8 post-induction. Results: One hundred children were included (90 UC and 10 IBD-U, median age 15.6 [interquartile range 13.3–17.1] years). Ninety-eight were previously treated with biologic therapies, and 76 were treated with ≥2 biologics. At the end of the 8-week induction period, clinical response, clinical remission, and corticosteroid-free clinical remission (CFR) were observed in 84%, 62%, and 56% of the children, respectively. Normal C-reactive protein and fecal calprotectin (FC) <150 mcg/g were achieved in 75% and 50%, respectively. Combined CFR and FC remission was observed in 18/46 (39%) children with available data at 8 weeks. Adverse events were recorded in 37 children, including 1 serious AE of an appendiceal neuroendocrine tumor. The most frequent AEs were hyperlipidemia (n = 13), acne (n = 12), and infections (n = 10, 5 of whom with herpes viruses). Conclusions: Upadacitinib is an effective induction therapy for refractory pediatric UC and IBD-U. Efficacy should be weighed against the potential risks of AEs.

Background: Partial enteral nutrition (PEN) coupled with the Crohn’s disease (CD) exclusion diet (CDED) was shown to be effective in inducing clinical remission in paediatric CD. There are currently no robust data on the endoscopic outcomes of PEN. The aim of this study was to evaluate the clinical and endoscopic rates of remission after PEN combined with a modified CDED (mCDED) adjusted to the local cuisine in comparison with exclusive enteral nutrition (EEN) for the induction of remission. Methods: Between June 2017 and February 2021, a prospective cohort study on children with active CD, treated with PEN + mCDED or EEN, was performed at a single tertiary centre. Results: During the study period, 54 patients were screened and 15 were excluded according to the exclusion criteria, with six patients excluded in the first two days due to intolerance of the enteral formula. Fourteen patients were included in the PEN and 19 in the EEN group. They were assessed at Weeks 0, 1, 3 and 6, using clinical and laboratory parameters. Endoscopy was performed at Weeks 0 and 6. Clinical remission rates per protocol analysis were 84.6% in the PEN group and 81.3% in the EEN group (p = 0.99). At Week 6, an endoscopic response (a decline in the Simple Endoscopic Score for CD (SES-CD) > 50%) was observed in 84.6% of patients on PEN and in 68.8% on EEN treatment (p = 0.41). Endoscopic remission (SES-CD ≤ 2) was achieved in 53.8% of patients in the PEN group and in 50.0% in the EEN group (p = 0.99), while the mucosal healing rates (SES-CD = 0) were 38.5% with PEN and 43.8% with EEN (p = 0.99). A significant decline in the clinical and endoscopic activity scores was observed in both groups. Conclusion: Our study suggests that PEN + mCDED could be effective in inducing endoscopic remission and mucosal healing in active paediatric CD patients. Here, we present an analysis of the data from our cohort of patients and our real-world experience with PEN + mCDED.

Background and Objectives Current data on ustekinumab therapy in children with ulcerative colitis (UC) or unclassified inflammatory bowel disease (IBDU) are limited. We aimed to evaluate the effectiveness and safety of ustekinumab in pediatric UC and IBDU. Methods This multicenter retrospective study included 16 centers affiliated with the IBD Interest and Porto groups of ESPGHAN. Children with UC or IBDU treated with ustekinumab were enrolled. Demographic, clinical, laboratory, endoscopic, and imaging data as well as adverse events were recorded. Analyses were all based on the intention-to-treat principle. Results Fifty-eight children (39 UC and 19 IBDU, median age 14.5 [IQR 11.5–16.5] years) were included. All had failed biologic therapies, and 38 (66%) had failed two or more biologics. Corticosteroid-free clinical remission (CFR) was observed in 27 (47%), 33 (57%), and 37 (64%) children at 16, 26, and 52 weeks, respectively. Normalization of C-reactive protein and calprotectin < 150 μg/g were achieved in 60% and 52%, respectively, by 52 weeks. Endoscopic and radiologic remissions were reached in 8% and 23%, respectively. The main predictors of CFR were diagnosis of UC compared with IBDU (hazard ratio [HR] 2.2, 95% CI 1.03–4.85; p = 0.041) and no prior vedolizumab therapy (HR 2.1, 95% CI 1.11–4.27; p = 0.023). Ustekinumab serum levels were not associated with disease activity. Adverse events were recorded in six (10%) children, leading to discontinuation of the drug in three. Conclusion Based on these findings, ustekinumab appears as an effective therapy for pediatric refractory UC and IBDU. The potential efficacy should be weighed against the risks of serious adverse events.

Background and Aim: Acute severe colitis [ASC] is associated with significant morbidity in paediatric patients with ulcerative colitis [UC]. Most outcome studies in ASC since tumour necrosis factor alpha [TNFα] antagonists became available have focused on the first year after admission. The aim of this study was to characterise the longer-term outcomes of paediatric patients admitted with ASC.Methods: This retrospective study was conducted in 25 centres across Europe and North America. Data on patients with UC aged 70 mm/h, and albumin <2.5 g/dL, were significantly associated with 5-year colectomy risk.Conclusions: High colectomy rates were demonstrated in paediatric UC patients admitted with ASC. Additional studies are required to determine whether intensification of anti-TNFα treatment, close therapeutic drug monitoring, and use of new drugs alter this outcome.

Background and Aims: Ulcerative proctitis [UP] is an uncommon presentation in paediatric patients with ulcerative colitis. We aimed to characterize the clinical features and natural history of UP in children, and to identify predictors of poor outcomes. Methods: This was a retrospective study involving 37 sites affiliated with the IBD Porto Group of ESPGHAN. Data were collected from patients aged <18 years diagnosed with UP between January 1, 2016 and December 31, 2020. Results: We identified 196 patients with UP (median age at diagnosis 14.6 years [interquartile range, IQR 12.5–16.0]), with a median follow-up of 2.7 years [IQR 1.7–3.8]. The most common presenting symptoms were bloody stools [95%], abdominal pain [61%] and diarrhoea [47%]. At diagnosis, the median paediatric ulcerative colitis activity index [PUCAI] score was 25 [IQR 20–35], but most patients exhibited moderate–severe endoscopic inflammation. By the end of induction, 5-aminosalicylic acid administration orally, topically or both resulted in clinical remission rates of 48%, 48%, and 73%, respectively. The rates of treatment escalation to biologics at 1, 3, and 5 years were 10%, 22%, and 43%, respectively. In multivariate analysis, the PUCAI score at diagnosis was significantly associated with initiation of systemic steroids, or biologics, and subsequent acute severe colitis events and inflammatory bowel disease-associated admission, with a score ≥35 providing an increased risk for poor outcomes. By the end of follow-up, 3.1% of patients underwent colectomy. Patients with UP that experienced proximal disease progression during follow-up [48%] had significantly higher rates of a caecal patch at diagnosis and higher PUCAI score by the end of induction, compared to those without progression. Conclusion: Paediatric patients with UP exhibit high rates of treatment escalation and proximal disease extension.