Abstract

Introduction: In the present study, we analyze the presentation, diagnosis and outcome of IBD with onset before the age of two. Methods: Clinical and biological data were reviewed from the charts of fifteen patients presenting with colitis between 1981 and 2003. Results: Sex ratio (male/female) is 10/5. Four children have a family history of IBD. Median age at onset is 5 months (15d. -24 mo.). Mean follow up time is 6.5 years (5 mo. - 18 yrs). All children presented with bloody diarrhea and fever, with aphtous pharyngeal and/or anoperineal lesions in 5 cases. Weight loss was present in 10 cases. Five children had eczema. No liver, pancreas or extra-digestive disease was diagnosed at presentation or after follow-up. Five children have Crohn’s disease (CD). Terminal ileum disease was present in 2 cases at onset and in a 3rd at follow up, all with associated pancolitis. 2 had left sided colitis. Diagnosis was made at onset in cases with ileum disease. Indeterminate colitis (IC) was the initial diagnosis for the others with definitive diagnosis of CD established after 1 to 7 years. Four children have ulcerative colitis (UC) presenting as pancolitis in 2 cases and left sided colitis in 2 cases. Finally six children have indeterminate pancolitis, with short follow-up (less than 1 year) in four cases. All CD and IC children started disease before the age of one year, whereas all UC were older than one year at onset. Serological markers (ASCA, pANCA) were not contributive for differential diagnosis. Maintenance azathioprine (AZA) therapy is successful in 3 cases of CD and 3 cases of IC while one child with IC and one child with UC are doing well with salicylate maintenance therapy. Seven children (2 CD, 2 IC and 3 UC) had colectomy for steroid resistant disease. Only one of these children received immunosuppressant drugs and was shown to be resistant to treatment. Conclusion: The incidence of early onset IBD is steadily increasing. CD is more frequent before one year of age. The course of disease is rather severe but outcome on AZA therapy is good on short term follow up and early introduction is justified. Long-term outcome and the need for surgery have to be assessed.

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