Basavaraj Kerur1, Eric I Benchimol2, Karoline Fiedler3, Marisa Stahl4, Jeffrey Hyams5, Michael Stephens6, Ying Lu7, Marian Pfefferkorn8, Raza Alkhouri9, Jennifer Strople10, Judith Kelsen11, Leah Siebold12, Alka Goyal13, Joel R Rosh14, Neal LeLeiko15, Johan Van Limbergen16, Anthony L Guerrerio17, Ross Maltz18, Lina Karam19, Eileen Crowley20, Anne Griffiths3, Melvin B Heyman21, Mark Deneau22, Keith Benkov23, Joshua Noe24, Dedrick Mouton25, Helen Pappa26, Joseph A Galanko27, Scott Snapper28, Aleixo M Muise3, Michael D Kappelman27. 1. University of Massachusetts Medical School (UMMS), Worcester, MA, United States. 2. Children's Hospital of Eastern Ontario, Ottawa, ON, Canada. 3. The Hospital for Sick Children, Toronto, ON, Canada. 4. Children's Hospital Colorado, Denver, CO, United States. 5. Connecticut Children's Medical Center, Hartford, CT, United States. 6. Center for Individualized Medicine, Mayo Clinic, Rochester, MN, United States. 7. Cohen Children's Medical Center of New York, New York, NY, United States. 8. Riley Children's Hospital, Indianapolis, IN, United States. 9. University at Buffalo, Buffalo, NY, United States. 10. Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, IL, United States. 11. Children's Hospital of Philadelphia, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, United States. 12. UPMC Children's Hospital of Pittsburgh, Pittsburgh, PA, United States. 13. Children's Mercy, Kansas City, MO, United States. 14. Goryeb Children's Hospital/Atlantic Health System, Morristown, NJ, United States. 15. Hasbro Children's Hospital, Providence, RI, United States. 16. Amsterdam University Medical Centres, Emma Children's Hospital, Amsterdam, Netherlands. 17. Johns Hopkins University School of Medicine Baltimore, MD, United States. 18. Nationwide Children's Medical Center, Columbus, OH, United States. 19. Texas Children's Hospital, Houston, TX, United States. 20. Children's Hospital, London Health Science Centre, University of Western Ontario, London, Ontario, Canada. 21. UCSF Benioff Children's Hospital, San Francisco, CA, United States. 22. University of Utah/Intermountain Primary Children's Hospital, Salt Lake City, UT, United States. 23. Icahn School of Medicine at Mount Sinai, New York, NY, United States. 24. Medical College of Wisconsin, Milwaukee, WI, United States. 25. Monroe Carell Jr. Children's Hospital at Vanderbilt, Nashville, TN, United States. 26. Cardinal Glennon Children's Hospital, St. Louis, MO, United States. 27. University of North Carolina in Chapel Hill, Chapel Hill, NC, United States. 28. Children's Hospital Boston, Boston, MA, United States.
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.
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.
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