|
KMID : 1038820180210010034
|
|
Pediatric Gastroenterology, Hepatology & Nutrition
2018 Volume.21 No. 1 p.34 ~ p.42
|
|
|
Higher Morbidity of Monogenic Inflammatory Bowel Disease Compared to the Adolescent Onset Inflammatory Bowel Disease
|
|
Kim Kwang-Yeon
Lee Eun-Joo
Kim Ju-Whi
Moon Jin-Soo
Jang Ju-Young
Yang Hye-Ran
Ko Jae-Sung
|
|
|
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
|
|
|
FullTexts / Linksout information
|
|
|
|
|
|
Listed journal information
|
|
 
|
|