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Natural History of Very Early Onset Inflammatory Bowel Disease in North America: A Retrospective Cohort Study
Inflammatory Bowel Diseases
  • Basavaraj Kerur, University of Massachusetts Chan Medical School
  • Eric I. Benchimol, Children's Hospital of Eastern Ontario, Ottawa
  • Karoline Fiedler, Hospital for Sick Children University of Toronto
  • Marisa Stahl, Children's Hospital Denver
  • Jeffrey Hyams, Connecticut Children's Medical Center
  • Michael Stephens, Mayo Clinic
  • Ying Lu, Cohen Children’s Medical Center
  • Marian Pfefferkorn, Riley Children's Hospital
  • Raza Alkhouri, University at Buffalo, The State University of New York
  • Jennifer Strople, Ann & Robert H. Lurie Children's Hospital of Chicago
  • Judith Kelsen, University of Pennsylvania Perelman School of Medicine
  • Leah Siebold, University of Pittsburgh Medical Center, Children's Hospital of Pittsburgh
  • Alka Goyal, Children's Mercy
  • Joel R. Rosh, Atlantic Health
  • Neal Leleiko, Hasbro Children's Hospital
  • Johan Van Limbergen, Universiteit van Amsterdam
  • Anthony L. Guerrerio, Johns Hopkins School of Medicine
  • Ross Maltz, Nationwide Children's
  • Lina Karam, Texas Children's Hospital Houston
  • Eileen Crowley, Western University
  • Anne Griffiths, Hospital for Sick Children University of Toronto
  • Melvin B. Heyman, UCSF Benioff Children‘s Hospital
  • Mark Deneau, The University of Utah
  • Keith Benkov, Icahn School of Medicine at Mount Sinai
  • Joshua Noe, Medical College of Wisconsin
  • Dedrick Mouton, Monroe Carell Jr. Children's Hospital at Vanderbilt
  • Helen Pappa, St. Louis Children's Hospital
  • Joseph A. Galanko, The University of North Carolina at Chapel Hill
  • Scott Snapper, Children's Hospital Boston
Document Type
Article
Publication Date
3-1-2021
URL with Digital Object Identifier
10.1093/ibd/izaa080
Abstract

Background: The incidence of very early onset inflammatory bowel disease (VEOIBD) is increasing, yet the phenotype and natural history of VEOIBD are not well described. Methods: We performed a retrospective cohort study of patients diagnosed with VEOIBD (6 years of age and younger) between 2008 and 2013 at 25 North American centers. Eligible patients at each center were randomly selected for chart review. We abstracted data at diagnosis and at 1, 3, and 5 years after diagnosis. We compared the clinical features and outcomes with VEOIBD diagnosed younger than 3 years of age with children diagnosed with VEOIBD at age 3 to 6 years. Results: The study population included 269 children (105 [39%] Crohn's disease, 106 [39%] ulcerative colitis, and 58 [22%] IBD unclassified). The median age of diagnosis was 4.2 years (interquartile range 2.9-5.2). Most (94%) Crohn's disease patients had inflammatory disease behavior (B1). Isolated colitis (L2) was the most common disease location (70% of children diagnosed younger than 3 years vs 43% of children diagnosed 3 years and older; P = 0.10). By the end of follow-up, stricturing/penetrating occurred in 7 (6.6%) children. The risk of any bowel surgery in Crohn's disease was 3% by 1 year, 12% by 3 years, and 15% by 5 years and did not differ by age at diagnosis. Most ulcerative colitis patients had pancolitis (57% of children diagnosed younger than 3 years vs 45% of children diagnosed 3 years and older; P = 0.18). The risk of colectomy in ulcerative colitis/IBD unclassified was 0% by 1 year, 3% by 3 years, and 14% by 5 years and did not differ by age of diagnosis. Conclusions: Very early onset inflammatory bowel disease has a distinct phenotype with predominantly colonic involvement and infrequent stricturing/penetrating disease. The cumulative risk of bowel surgery in children with VEOIBD was approximately 14%-15% by 5 years. These data can be used to provide anticipatory guidance in this emerging patient population.

Citation Information
Basavaraj Kerur, Eric I. Benchimol, Karoline Fiedler, Marisa Stahl, et al.. "Natural History of Very Early Onset Inflammatory Bowel Disease in North America: A Retrospective Cohort Study" Inflammatory Bowel Diseases Vol. 27 Iss. 3 (2021) p. 295 - 302
Available at: http://works.bepress.com/eileen-crowley/5/