Pediatr Gastroenterol Hepatol Nutr.  2018 Jan;21(1):34-42. 10.5223/pghn.2018.21.1.34.

Higher Morbidity of Monogenic Inflammatory Bowel Disease Compared to the Adolescent Onset Inflammatory Bowel Disease

• Kim KY
• Lee EJ
• Kim JW
• Moon JS
• Jang JY
• Yang HR
• Ko JS

• KMID: 2401771
• DOI: http://doi.org/10.5223/pghn.2018.21.1.34

Abstract

PURPOSE
Monogenic inflammatory bowel disease (IBD) patients do not respond to conventional therapy and are associated with a higher morbidity. We summarized the clinical characteristics of monogenic IBD patients and compared their clinical outcomes to that of non-monogenic IBD patients.
METHODS
We performed a retrospective cohort study of all children <18 years old who were diagnosed with IBD between 2005 and 2016. A total of 230 children were enrolled. Monogenic IBD was defined as a presentation age less than 6 years old with confirmation of a genetic disorder. We subdivided the groups into monogenic IBD (n=18), non-monogenic very early-onset IBD (defined as patients with a presentation age <6 years old without a confirmed genetic disorder, n=12), non-monogenic IBD (defined as all patients under 18 years old excluding monogenic IBD, n=212), and severe IBD (defined as patients treated with an anti-tumor necrosis factor excluding monogenic IBD, n=92). We compared demographic data, initial pediatric Crohn disease activity index/pediatric ulcerative colitis activity index (PCDAI/PUCAI) score, frequency of hospitalizations, surgical experiences, and height and weight under 3rd percentile among the patients enrolled.
RESULTS
The initial PCDAI/PUCAI score (p < 0.05), incidence of surgery per year (p < 0.05), and hospitalization per year (p < 0.05) were higher in the monogenic IBD group than in the other IBD groups. Additionally, the proportion of children whose weight and height were less than the 3rd percentile (p < 0.05 and p < 0.05, respectively) was also higher in the monogenic IBD group.
CONCLUSION
Monogenic IBD showed more severe clinical manifestations than the other groups.

Keyword

Inflammatory bowel disease; Very early onset; Immunologic deficiency syndromes; Interleukin-10; Crohn disease

MeSH Terms

Adolescent*
Child
Cohort Studies
Colitis, Ulcerative
Crohn Disease
Hospitalization
Humans
Immunologic Deficiency Syndromes
Incidence
Inflammatory Bowel Diseases*
Interleukin-10
Necrosis
Retrospective Studies
Interleukin-10

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