title: Clinical Presentation and Five-Year Therapeutic Management of Very Early-Onset Inflammatory Bowel Disease in a Large North American Cohort.
abstract: OBJECTIVE
  To evaluate the presentation, therapeutic management, and long-term outcome of children with very early-onset (VEO) (≤ 5 years of age) inflammatory bowel disease (IBD).
  
  
  METHODS
  Data were obtained from an inception cohort of 1928 children with IBD enrolled in a prospective observational registry at multiple centers in North America.
  
  
  RESULTS
  One hundred twelve children were ≤ 5 years of age with no child enrolled at <1 year of age. Of those, 42.9% had Crohn's disease (CD), 46.4% ulcerative colitis (UC), and 10.7% had IBD-unclassified. Among the children with CD, children 1-5 years of age had more isolated colonic disease (39.6%) compared with 6- to 10-year-olds (25.3%, P = .04), and 11- to 16-year-olds (22.3%, P < .01). The change from a presenting colon-only phenotype to ileocolonic began at 6-10 years. Children 1-5 years of age with CD had milder disease activity (45.8%) at diagnosis compared with the oldest group (28%, P = .01). Five years postdiagnosis, there was no difference in disease activity among the 3 groups. However, compared with the oldest group, a greater proportion of 1- to 5-year-olds with CD were receiving corticosteroids (P < .01) and methotrexate (P < .01), and a greater proportion of 1- to 5-year-olds with UC were receiving mesalamine (P < .0001) and thiopurine immunomodulators (P < .0002).
  
  
  CONCLUSIONS
  Children with VEO-CD are more likely to have mild disease at diagnosis and present with a colonic phenotype with change to an ileocolonic phenotype noted at 6-10 years of age. Five years after diagnosis, children with VEO-CD and VEO-UC are more likely to have been administered corticosteroids and immunomodulators despite similar disease activity in all age groups. This may suggest development of a more aggressive disease phenotype over time.
authors.affiliation: Division of Pediatric Gastroenterology and Nutrition, Johns Hopkins Children's Center, Baltimore, MD. Electronic address: moliva@jhmi.edu.
authors.forename: Maria
authors.initials: M
authors.lastname: Oliva-Hemker
authors.affiliation: Johns Hopkins University, Baltimore, MD.
authors.forename: Susan
authors.initials: S
authors.lastname: Hutfless
authors.affiliation: Johns Hopkins University, Baltimore, MD.
authors.forename: Elie S
authors.initials: ES
authors.lastname: Al Kazzi
authors.affiliation: Connecticut Children's Medical Center, Hartford, CT.
authors.forename: Trudy
authors.initials: T
authors.lastname: Lerer
authors.affiliation: Children's Hospital of Eastern Ontario, Ottawa, Ontario, Canada.
authors.forename: David
authors.initials: D
authors.lastname: Mack
authors.affiliation: Hasbro Children's Hospital, Providence, RI.
authors.forename: Neal
authors.initials: N
authors.lastname: LeLeiko
authors.affiliation: Hospital for Sick Children, Toronto, Ontario, Canada.
authors.forename: Anne
authors.initials: A
authors.lastname: Griffiths
authors.affiliation: Medical College of Wisconsin, Milwaukee, WI.
authors.forename: Jose
authors.initials: J
authors.lastname: Cabrera
authors.affiliation: Izaak Walton Killam Health Center, Halifax, Nova Scotia, Canada.
authors.forename: Anthony
authors.initials: A
authors.lastname: Otley
authors.affiliation: Dayton Children's Hospital, Dayton, OH.
authors.forename: James
authors.initials: J
authors.lastname: Rick
authors.affiliation: Children's Hospital, Boston, MA.
authors.forename: Athos
authors.initials: A
authors.lastname: Bousvaros
authors.affiliation: Goryeb Children's Hospital, Morristown, NJ.
authors.forename: Joel
authors.initials: J
authors.lastname: Rosh
authors.affiliation: Children's Hospital of Philadelphia, Philadelphia, PA.
authors.forename: Andrew
authors.initials: A
authors.lastname: Grossman
authors.affiliation: Children's Hospital Medical Center, Cincinnati, OH.
authors.forename: Shehzad
authors.initials: S
authors.lastname: Saeed
authors.affiliation: Cleveland Clinic, Cleveland, OH.
authors.forename: Marsha
authors.initials: M
authors.lastname: Kay
authors.affiliation: Nationwide Children's Hospital, Columbus, OH.
authors.forename: Ryan
authors.initials: R
authors.lastname: Carvalho
authors.affiliation: Children's Hospital of Pittsburgh, Pittsburgh, PA.
authors.forename: David
authors.initials: D
authors.lastname: Keljo
authors.affiliation: Riley Hospital for Children, Indianapolis, IN.
authors.forename: Marian
authors.initials: M
authors.lastname: Pfefferkorn
authors.affiliation: Mayo Clinic, Rochester, MN.
authors.forename: William
authors.initials: W
authors.lastname: Faubion
authors.affiliation: University of North Carolina, Chapel Hill, NC.
authors.forename: Michael
authors.initials: M
authors.lastname: Kappelman
authors.affiliation: University of Minnesota, Minneapolis, MN.
authors.forename: Boris
authors.initials: B
authors.lastname: Sudel
authors.affiliation: Penn State Hershey Children's Hospital, Hershey, PA.
authors.forename: Marc E
authors.initials: ME
authors.lastname: Schaefer
authors.affiliation: Cohen Children's Medical Center of New York, New Hyde Park, NY.
authors.forename: James
authors.initials: J
authors.lastname: Markowitz
authors.affiliation: Connecticut Children's Medical Center, Hartford, CT.
authors.forename: Jeffrey S
authors.initials: JS
authors.lastname: Hyams
mesh_terms: D000293:Adolescent; D017668:Age of Onset; D002648:Child; D002675:Child, Preschool; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D015212:Inflammatory Bowel Diseases / Q000175:diagnosis* / Q000628:therapy; D008297:Male; D009656:North America; D010641:Phenotype; D011379:Prognosis; D011446:Prospective Studies; D012042:Registries