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Semin Pediatr Surg. 2017 Dec;26(6):356-359. doi: 10.1053/j.sempedsurg.2017.10.004. Epub 2017 Oct 10.

Very early onset inflammatory bowel disease.

Author information

1
Harvard Medical School, Department of Pediatrics, 175 Cambridge St, Suite 567, Boston, MA 02114. Electronic address: cjmoran@partners.org.

Abstract

Very early onset inflammatory bowel disease (VEO-IBD) represents a unique and growing subset of patients with inflammatory bowel disease (IBD). Some VEO-IBD patients present with immunodeficiency and possess loss of function genetic mutations involving immune pathways that cause their IBD. A search for Mendelian causes of IBD is likely most beneficial when the presentation involves extra-intestinal autoimmunity or involves intestinal histopathology that is atypical for IBD. While a subset of these young patients will have highly aggressive courses (and likely present with immunodeficiency), the majority of patients with VEO-IBD appear to have disease courses similar to that of their older counterparts. Most notably, many of these young children will require long courses of immunosuppression simply as a result of the profoundly early presentation-thus increasing their long-term risks of cancer and opportunistic infections.

KEYWORDS:

Immunodeficiency; Very early onset IBD

[Indexed for MEDLINE]

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