Higher Morbidity of Monogenic Inflammatory Bowel Disease Compared to the Adolescent Onset Inflammatory Bowel Disease
10.5223/pghn.2018.21.1.34
- Author:
Kwang Yeon KIM
1
;
Eun Joo LEE
;
Ju Whi KIM
;
Jin Soo MOON
;
Ju Young JANG
;
Hye Ran YANG
;
Jae Sung KO
Author Information
1. Department of Pediatrics, Seoul National University College of Medicine, Seoul, Korea. mjschj@snu.ac.kr
- Publication Type:Original Article
- Keywords:
Inflammatory bowel disease;
Very early onset;
Immunologic deficiency syndromes;
Interleukin-10;
Crohn disease
- MeSH:
Adolescent;
Child;
Cohort Studies;
Colitis, Ulcerative;
Crohn Disease;
Hospitalization;
Humans;
Immunologic Deficiency Syndromes;
Incidence;
Inflammatory Bowel Diseases;
Interleukin-10;
Necrosis;
Retrospective Studies
- From:Pediatric Gastroenterology, Hepatology & Nutrition
2018;21(1):34-42
- CountryRepublic of Korea
- Language:English
-
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.
