Very Early Onset Inflammatory Bowel Disease: A Clinical Approach With a Focus on the Role of Genetics and Underlying Immune Deficiencies

Ouahed, Jodie; Spencer, Elizabeth; Kotlarz, Daniel; Shouval, Dror S.; Kowalik, Matthew; Peng, Kaiyue; Field, Michael; Grushkin-Lerner, Leslie; Pai, Sung-Yun; Bousvaros, Athos; Cho, Judy; Argmann, Carmen; Schadt, Eric; Mcgovern, Dermot P. B.; Mokry, Michal; Nieuwenhuis, Edward; Clevers, Hans; Powrie, Fiona; Uhlig, Holm; Klein, Christoph; Muise, Aleixo; Dubinsky, Marla und Snapper, Scott B. (2020): Very Early Onset Inflammatory Bowel Disease: A Clinical Approach With a Focus on the Role of Genetics and Underlying Immune Deficiencies. In: Inflammatory Bowel Diseases, Bd. 26, Nr. 6: S. 820-842

Abstract

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.

• Dokumententyp: Zeitschriftenartikel
• Fakultät: Medizin
• Themengebiete: 600 Technik, Medizin, angewandte Wissenschaften > 610 Medizin und Gesundheit
• ISSN: 1078-0998
• Sprache: Englisch