Gastroenterology

Gastroenterology

Volume 135, Issue 4, October 2008, Pages 1114-1122
Gastroenterology

Clinical—Alimentary Tract
Definition of Phenotypic Characteristics of Childhood-Onset Inflammatory Bowel Disease

https://doi.org/10.1053/j.gastro.2008.06.081Get rights and content

Background & Aims: Childhood-onset inflammatory bowel disease (IBD) might be etiologically different from adult-onset IBD. We analyzed disease phenotypes and progression of childhood-onset disease and compared them with characteristics of adult-onset disease in patients in Scotland. Methods: Anatomic locations and behaviors were assessed in 416 patients with childhood-onset (276 Crohn's disease [CD], 99 ulcerative colitis [UC], 41 IBD type unclassified [IBDU] diagnosed before seventeenth birthday) and 1297 patients with adult-onset (596 CD, 701 UC) IBD using the Montreal classification. Results: At the time of diagnosis in children, CD involved small bowel and colon (L3) in 51% (138/273), colon (L2) in 36%, and ileum (L1) in 6%; the upper gastrointestinal (GI) tract (L4) was also affected in 51%. In 39%, the anatomic extent increased within 2 years. Behavioral characteristics progressed; 24% of children developed stricturing or penetrating complications within 4 years (vs 9% at diagnosis; P < .0001; odds ratio [OR], 3.32; 95% confidence interval [CI], 1.86–5.92). Compared with adults, childhood-onset disease was characterized by a “panenteric” phenotype (ileocolonic plus upper GI [L3+L4]; 43% vs 3%; P < .0001; OR, 23.36; 95% CI, 13.45–40.59) with less isolated ileal (L1; 2% vs 31%; P < .0001; OR, 0.06; 95% CI, 0.03–0.12) or colonic disease (L2; 15% vs 36%; P < .0001; OR, 0.31; 95% CI, 0.21–0.46). UC was extensive in 82% of the children at diagnosis, versus 48% of adults (P < .0001; OR, 5.08; 95% CI, 2.73–9.45); 46% of the children progressed to develop extensive colitis during follow-up. Forty-six percent of children with CD and 35% with UC required immunomodulatory therapy within 12 months of diagnosis. The median time to first surgery was longer in childhood-onset than adult-onset patients with CD (13.7 vs 7.8 years; P < .001); the reverse was true for UC. Conclusions: Childhood-onset IBD is characterized by extensive intestinal involvement and rapid early progression.

Section snippets

Subjects

We recruited 416 children with IBD diagnosed before their seventeenth birthday from all the pediatric gastroenterology centers in Scotland as part of an ongoing childhood-onset IBD genetics project from July 2002. Children were investigated according to the ESPGHAN “Porto”-criteria for diagnosis of IBD.13, 14 Detailed patient demographics are presented in Table 1. We also recruited 1297 patients with adult-onset IBD from the Gastroenterology Department at Western General Hospital, Edinburgh

CD at diagnosis

CD affected the small as well as large bowel (L3 ± L4) in 50.5%, the colon (L2 ± L4) in 36.3%, and the ileum (L1 ± L4) in 5.9%. More than half of children (50.9%) were affected by CD proximal to the terminal ileum at diagnosis (any L4). Follow-up of ≥2 years was completed for 196 of 273 patients (71.8%). Follow-up data of ≥4 years were available for 132 of 273 patients (48.4%; Table 3).

CD location

The location of disease changed over time. Detailed analysis of the 196 childhood-onset CD patients who had ≥2

Discussion

The present study represents not only a detailed application of the Montreal classification of IBD in a large cohort of patients with childhood-onset disease, but also provides new robust data on disease evolution, as well as need for immunomodulation and surgery in childhood-onset disease. Rigorous follow-up of 416 children with IBD in the 3 pediatric gastroenterology centers in Scotland has allowed us to document these aspects, notably the progression of disease location and behavior. Data

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    J.V.L. and R.K.R. contributed equally to this manuscript. J.S. and D.C.W. are to be considered joint senior authors.

    Johan Van Limbergen is funded by a Research Training Fellowship from Action Medical Research, The Gay-Ramsay-Steel-Maitland or Stafford Trust and the Hazel M Wood Charitable Trust. Elaine R Nimmo is supported by a Wellcome Trust Programme Grant (072789/Z/03/Z). Financial assistance was also provided by Schering–Plough and the GI/Nutrition Research Fund, Child Life and Health, University of Edinburgh.

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