1. Clinical features of very early-onset inflammatory bowel disease in Japan: a retrospective single-center study
- Author
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Masaaki Usami, Ichiro Takeuchi, Reiko Kyodo, Yuri Hirano, Kosuke Kashiwagi, Hiroki Fujikawa, Hirotaka Shimizu, Toshinao Kawai, and Katsuhiro Arai
- Subjects
monogenic inflammatory bowel disease ,japan ,phenotype ,disease severity ,therapeutics ,Medicine ,Diseases of the digestive system. Gastroenterology ,RC799-869 - Abstract
Background/Aims Very early-onset inflammatory bowel disease (VEO-IBD), defined as IBD diagnosed in patients younger than 6 years, is a challenge for pediatric gastroenterologists. Although there have been reports regarding VEO-IBD in Western countries, those in Asia are still lacking. This study aimed to investigate the clinical features of Japanese VEO-IBD patients. Methods Patients with VEO-IBD diagnosed between 2006 and 2019 were evaluated retrospectively. The disease phenotypes were classified into ulcerative colitis type (UC-type) and Crohn’s disease type (CD-type), and the clinical features and courses were compared between the phenotypes. Results Overall, 54 VEO-IBD patients (19 patients with UC-type and 35 patients with CD-type) were evaluated. The median age at onset was 18 months. One patient had severe combined immunodeficiency (SCID), and 9 patients had monogenic IBD. Monogenic IBD was more prevalent in the CD-type patients with perianal disease (CD-type (PD)). The age at onset was significantly lower in the CD-type group (P
- Published
- 2022
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