Early Onset IBD: Endoscopic Features
Very early onset IBD (VEOIBD) is an emerging form of pediatric IBD with distinct features in the pathogenesis and in the phenotype. The clinical pattern is often associated with isolated unclassified pancolitis, increased severity, aggressive progression, more resistant to many of standard therapy. Genetic susceptibility plays a important role in VEOIBD and above all infantile IBD can be the first isolated presentation of a primary immunodeficiency. Genetic screening is mandatory to recognize underlying genetic defect that require targeted treatment as allogeneic hematopoietic stem cell transplantation.
KeywordsVery early onset inflammatory bowel disease (VEOIBD) Infantile IBD unclassified (IBD-U) Primary immunodeficiency (PID) Genetic
- 2.Silverberg MS, Satsangi J, Ahmad T, Arnott ID, Bernstein CN, Brant S, et al. Toward an integrated clinical, molecular and serological classification of inflammatory bowel disease: report of a Working Party of the 2005 Montreal World Congress of Gastroenterology. Can J Gastroenterol. 2005;19(Suppl A):5A–36A.CrossRefGoogle Scholar
- 7.Snapper SB. Very-early-onset inflammatory bowel disease. Gastroenterol Hepatol (N Y). 2015;11:554–6.Google Scholar
- 19.Tegtmeyer D, Seidl M, Gerner P, Baumann U, Klemann C. IBD due to PID: inflammatory bowel disease caused by primary immunodeficiencies—clinical presentations, review of literature, and proposal of a rational diagnostic algorithm. Pediatr Allergy Immunol. 2017;28(5):412–29. https://doi.org/10.1111/pai.12734.CrossRefPubMedGoogle Scholar