title: Maintaining intestinal health: the genetics and immunology of very early onset inflammatory bowel disease.
abstract: Inflammatory bowel disease (IBD) is a multifactoral disease caused by dysregulated immune responses to commensal or pathogenic microbes in the intestine, resulting in chronic intestinal inflammation. An emerging population of patients with IBD occurring before the age of 5 represent a unique form of disease, termed Very Early Onset (VEO)-IBD, which is phenotypically- and genetically-distinct from older-onset IBD. VEO-IBD is associated with increased disease severity, aggressive progression and poor responsiveness to most conventional therapies. Further investigation into the causes and pathogenesis of VEO-IBD will help improve treatment strategies, and may lead to a better understanding of the mechanisms that are essential to maintain intestinal health or provoke the development of targeted therapeutic strategies to limit intestinal disease. Here we discuss the phenotypic nature of VEO-IBD, the recent identification of novel gene variants associated with disease, and functional immunologic studies interrogating the contribution of specific genetic variants to the development of chronic intestinal inflammation.
authors.affiliation: Division of Gastroenterology, Hepatology and Nutrition, Children's Hospital of Philadelphia, Philadelphia, PA, 19104, USA.
authors.forename: Judith R
authors.initials: JR
authors.lastname: Kelsen
authors.affiliation: Division of Gastroenterology, Hepatology and Nutrition, Children's Hospital of Philadelphia, Philadelphia, PA, 19104, USA.
authors.forename: Robert N
authors.initials: RN
authors.lastname: Baldassano
authors.affiliation: Jill Roberts Institute for Research in IBD, Joan and Sanford I. Weill Department of Medicine, Division of Gastroenterology and Hepatology, and Department of Microbiology and Immunology, Weill Cornell Medical College, Cornell University, New York, NY, 10021 USA.
authors.forename: David
authors.initials: D
authors.lastname: Artis
authors.affiliation: Jill Roberts Institute for Research in IBD, Joan and Sanford I. Weill Department of Medicine, Division of Gastroenterology and Hepatology, and Department of Microbiology and Immunology, Weill Cornell Medical College, Cornell University, New York, NY, 10021 USA.
authors.forename: Gregory F
authors.initials: GF
authors.lastname: Sonnenberg