1. Infantile and Very Early Onset Inflammatory Bowel Disease: A Multicenter Study.
- Author
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Guz-Mark A, Aloi M, Scarallo L, Bramuzzo M, Escher JC, Alvisi P, Henderson P, Hojsak I, Lev-Tzion R, El-Matary W, Schwerd T, Granot M, Sladek M, Strisciuglio C, Müller KE, Olbjørn C, Tzivinikos C, Yerushalmy-Feler A, Huysentruyt K, Norsa L, Viola I, de Ridder L, Shouval DS, Lega S, Lionetti P, Catassi G, and Assa A
- Subjects
- Humans, Male, Female, Retrospective Studies, Child, Preschool, Infant, Adolescent, Child, Inflammatory Bowel Diseases genetics, Inflammatory Bowel Diseases diagnosis, Inflammatory Bowel Diseases epidemiology, Inflammatory Bowel Diseases therapy, Follow-Up Studies, Crohn Disease diagnosis, Crohn Disease epidemiology, Crohn Disease therapy, Crohn Disease genetics, Crohn Disease surgery, Colitis, Ulcerative diagnosis, Colitis, Ulcerative therapy, Colitis, Ulcerative genetics, Colitis, Ulcerative surgery, Colitis, Ulcerative epidemiology, Age of Onset
- Abstract
Objectives: This study described disease characteristics and long-term outcomes in patients diagnosed with very early onset inflammatory bowel disease (VEOIBD) (diagnosed before 6 years of age) and infantile-IBD (before 2 years)., Methods: Cases from 21 centers worldwide diagnosed with VEOIBD (2008-2018), with minimum 2 years of follow-up, were retrospectively reviewed., Results: The cohort included 243 patients (52% males, median follow-up of 5.8 [range 2-18] years, including 69 [28%]) with infantile-IBD. IBD subtypes included Crohn's disease (CD), ulcerative colitis (UC), or IBD-unclassified (IBDU) in 30%, 59%, and 11%, respectively. Among patients with CD, 94% had colonic involvement, and among patients with UC/IBDU, 75% had pancolitis. Patients with infantile-IBD presented with higher rates of IBDU, lower hemoglobin and albumin levels, and higher C-reactive protein, and had lower response rates to first-induction therapy and corticosteroids therapy (P < .05 for all). Colectomy and diversion surgeries were performed in 11% and 4%, respectively, with no significant differences between age groups. Corticosteroid-free remission rates were 74% and 78% after 3 and 5 years, respectively, and 86% at end of follow-up. Genetic testing was performed in 96 (40%) patients. Among tested population, 15 (16%) were identified with monogenic disease. This group demonstrated lower response rates to induction therapies, higher rates of surgical intervention, and higher rates of major infections (P < .05 for all)., Conclusions: Patients with VEOIBD, including infantile-IBD, exhibit low rate of complications and surgical interventions at the long term. Patients with monogenic IBD are at risk for more severe disease course., (Copyright © 2024 by the American Academy of Pediatrics.)
- Published
- 2024
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