Clinical characteristics and validation of diagnosis in individuals with Hirschsprung disease and Inflammatory Bowel Disease.
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In brief
In brief
Population-based cohort study validated 18 cases with both Hirschsprung disease and IBD, finding median IBD onset at age 21 with distribution similar to non-Hirschsprung IBD patients (56% Crohn's, 33% ulcerative colitis). Clinical course paralleled typical IBD with 28% requiring surgical intervention.
Written by the GCMD Library team from the article.
Abstract
Background/purpose
Hirschsprung disease has previously been reported to be associated with inflammatory bowel disease (IBD). The aim was to clinically confirm the diagnosis and to describe characteristics among individuals with both conditions in a national populationbased cohort.
Methods
Nationwide, population-based cohort study, including all individuals with a Hirschsprung disease diagnosis and an IBD diagnosis registered between 1964 and 2016, in which clinical data were collected from the medical records of 18 validated cases with both Hirschsprung disease and IBD. The medical record of each individual in the study cohort was reviewed for age at IBD diagnosis, type of aganglionosis, type of surgical treatment, subtype of IBD, and treatment for IBD.
Results
Median age at follow up was 34 years (range 19–66), and 3 of 18 indivduals (17%) were females. Median age at first diagnosis of IBD was 21 years (range 10–46). Six patients had ulcerative colitis, ten had Crohn's disease and two had unclassified IBD. Most of the patients had pharmacological treatment for IBD and 5 (28%) individuals had surgical treatment.
Conclusion
Hirschsprung disease and IBD was clinically confirmed in 18 cases. Age at IBD onset and subtype of IBD is similar to IBD patients without Hirschsprung disease. Five individuals had undergone surgical treatment for IBD.