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Published on: September 20, 2018
Higher Morbidity of Monogenic Inflammatory Bowel Disease Compared to the Adolescent Onset Inflammatory Bowel Disease
Kwang Yeon Kim1, Eun Joo Lee1, Ju Whi Kim1
1Department of Pediatrics, Seoul National University College of Medicine, Seoul, Korea.
Insights
Monogenic inflammatory bowel disease (IBD) in children presents with more severe symptoms and worse outcomes. This genetic form of IBD requires distinct management compared to non-monogenic IBD.
Area of Science:
- Pediatric Gastroenterology
- Genetics
- Immunology
Background:
- Monogenic inflammatory bowel disease (IBD) often presents with severe symptoms and poor response to standard treatments.
- Understanding the distinct clinical characteristics of monogenic IBD is crucial for effective patient management.
- Genetic factors play a significant role in the pathogenesis of early-onset IBD.
Purpose of the Study:
- To summarize the clinical characteristics of pediatric patients with monogenic IBD.
- To compare the clinical outcomes of monogenic IBD with non-monogenic IBD.
- To identify differences in disease severity and treatment response.
Main Methods:
- Retrospective cohort study of children under 18 diagnosed with IBD between 2005 and 2016.
- Monogenic IBD defined by presentation before age 6 with confirmed genetic disorder.
- Comparison of demographic data, disease activity scores (PCDAI/PUCAI), hospitalizations, surgeries, and growth parameters.
Main Results:
- Monogenic IBD patients exhibited higher initial disease activity scores (PCDAI/PUCAI).
- Increased incidence of surgery and hospitalizations per year was observed in the monogenic IBD group.
- A greater proportion of children with monogenic IBD had height and weight below the 3rd percentile.
Conclusions:
- Monogenic IBD is associated with more severe clinical manifestations compared to other forms of IBD.
- Genetic IBD presents with significant challenges in growth and requires intensive management.
- These findings highlight the need for early genetic diagnosis in severe early-onset IBD.
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.
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