Increased Incidence of Inflammatory Bowel Disease After Hirschsprung Disease: A Population-based Cohort Study
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Read the article on pubmed.ncbi.nlm.nih.gov ↗Article · Apr 2021 · 1 min read
In brief
In brief
Population-based study across three Canadian provinces found that children with Hirschsprung disease have a 12-fold increased risk of developing inflammatory bowel disease compared to the general population, with over 2.5% ultimately diagnosed with IBD. Crohn's disease was more common than ulcerative colitis, and males were disproportionately affected.
Written by the GCMD Library team from the article.
Objective: To determine the frequency with which inflammatory bowel disease (IBD) is diagnosed in persons with Hirschsprung disease in population-based datasets from 3 Canadian provinces.
Study design: In study I, Ontario data were used to assess the incidence of IBD in a birth cohort of children with Hirschsprung disease relative to children without Hirschsprung disease. In study II, a case-control design was used in Alberta and Manitoba to determine the frequency of previously diagnosed Hirschsprung disease in persons with IBD, compared with the frequency of Hirschsprung disease in matched controls. Validated algorithms for Hirschsprung disease and IBD were applied to each provincial health registry.
Results: In study I, of the 716 children diagnosed with Hirschsprung disease in Ontario since 1991, 18 (2.5%) ultimately developed IBD (168.8 per 100 000 person-years), compared with 7109 of 3 377 394 children without Hirschsprung disease (0.2%, 14.2 per 100 000 person-years). The percentage of males with post-Hirschsprung disease IBD was 77.8%. The incidence rate ratio was 11.9 (95% CI, 7.5-18.8). In study II, the OR of having had Hirschsprung disease before a diagnosis of IBD compared with controls was 74.9 (95% CI, 17.1-328.7) in Alberta and 23.8 (95% CI, 4.6-123) in Manitoba. Crohn's disease was more common after Hirschsprung disease than ulcerative colitis.
Conclusions: IBD can emerge in more than 2% of patients with Hirschsprung disease and, like Hirschsprung disease itself, is more common in males. IBD is much more common after a diagnosis of Hirschsprung disease than in the general population.
DOI: 10.1016/j.jpeds.2021.01.060