TY - JOUR
T1 - Very Early Onset Inflammatory Bowel Disease
T2 - A Clinical Approach With a Focus on the Role of Genetics and Underlying Immune Deficiencies
AU - Ouahed, Jodie
AU - Spencer, Elizabeth
AU - Kotlarz, Daniel
AU - Shouval, Dror S
AU - Kowalik, Matthew
AU - Peng, Kaiyue
AU - Field, Michael
AU - Grushkin-Lerner, Leslie
AU - Pai, Sung-Yun
AU - Bousvaros, Athos
AU - Cho, Judy
AU - Argmann, Carmen
AU - Schadt, Eric
AU - Mcgovern, Dermot P B
AU - Mokry, Michal
AU - Nieuwenhuis, Edward
AU - Clevers, Hans
AU - Powrie, Fiona
AU - Uhlig, Holm
AU - Klein, Christoph
AU - Muise, Aleixo
AU - Dubinsky, Marla
AU - Snapper, Scott B
N1 - © 2019 Crohn’s & Colitis Foundation. Published by Oxford University Press. All rights reserved. For permissions, please e-mail: [email protected].
PY - 2019/12/3
Y1 - 2019/12/3
N2 - Very early onset inflammatory bowel disease (VEO-IBD) is defined as IBD presenting before 6 years of age. When compared with IBD diagnosed in older children, VEO-IBD has some distinct characteristics such as a higher likelihood of an underlying monogenic etiology or primary immune deficiency. In addition, patients with VEO-IBD have a higher incidence of inflammatory bowel disease unclassified (IBD-U) as compared with older-onset IBD. In some populations, VEO-IBD represents the age group with the fastest growing incidence of IBD. There are contradicting reports on whether VEO-IBD is more resistant to conventional medical interventions. There is a strong need for ongoing research in the field of VEO-IBD to provide optimized management of these complex patients. Here, we provide an approach to diagnosis and management of patients with VEO-IBD. These recommendations are based on expert opinion from members of the VEO-IBD Consortium (www.VEOIBD.org). We highlight the importance of monogenic etiologies, underlying immune deficiencies, and provide a comprehensive description of monogenic etiologies identified to date that are responsible for VEO-IBD.
AB - Very early onset inflammatory bowel disease (VEO-IBD) is defined as IBD presenting before 6 years of age. When compared with IBD diagnosed in older children, VEO-IBD has some distinct characteristics such as a higher likelihood of an underlying monogenic etiology or primary immune deficiency. In addition, patients with VEO-IBD have a higher incidence of inflammatory bowel disease unclassified (IBD-U) as compared with older-onset IBD. In some populations, VEO-IBD represents the age group with the fastest growing incidence of IBD. There are contradicting reports on whether VEO-IBD is more resistant to conventional medical interventions. There is a strong need for ongoing research in the field of VEO-IBD to provide optimized management of these complex patients. Here, we provide an approach to diagnosis and management of patients with VEO-IBD. These recommendations are based on expert opinion from members of the VEO-IBD Consortium (www.VEOIBD.org). We highlight the importance of monogenic etiologies, underlying immune deficiencies, and provide a comprehensive description of monogenic etiologies identified to date that are responsible for VEO-IBD.
U2 - 10.1093/ibd/izz259
DO - 10.1093/ibd/izz259
M3 - Article
C2 - 31833544
SN - 1078-0998
JO - Inflammatory Bowel Diseases
JF - Inflammatory Bowel Diseases
ER -