{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,22]],"date-time":"2026-09-22T18:15:30Z","timestamp":1790100930717,"version":"4.0.1"},"reference-count":22,"publisher":"Korean Association for the Study of Intestinal Diseases","issue":"4","license":[{"start":{"date-parts":[[2022,6,13]],"date-time":"2022-06-13T00:00:00Z","timestamp":1655078400000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"funder":[{"DOI":"10.13039\/100007786","name":"National Center for Child Health and Development","doi-asserted-by":"publisher","award":["2019A-3"],"award-info":[{"award-number":["2019A-3"]}],"id":[{"id":"10.13039\/100007786","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Ministry of Health, Labor and Welfare, Japan"}],"content-domain":{"domain":["irjournal.org"],"crossmark-restriction":true},"short-container-title":["Intest Res"],"published-print":{"date-parts":[[2022,10,31]]},"abstract":"<jats:p>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\u2019s 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&lt;0.05). The most common initial symptom was bloody stools (70%), followed by diarrhea (63%), weight loss (24%), fever (20%), and perianal disease (20%). Excluding patients with SCID and monogenic IBD, 23 out of 44 patients (52%) required biologics. The biologics were switched in 11 out of 44 patients (25%), and the majority of these patients (82%) were in the CD-type group. Overall, 9 patients (20%) required intestinal resection or ostomy placement.Conclusions: CD-type tends to occur at an earlier age, and monogenic IBD occurs significantly more frequently in CD-type (PD). Disease severity and treatment should be individualized, owing to the disease heterogeneity.<\/jats:p>","DOI":"10.5217\/ir.2021.00142","type":"journal-article","created":{"date-parts":[[2022,6,10]],"date-time":"2022-06-10T06:30:15Z","timestamp":1654842615000},"page":"475-481","update-policy":"https:\/\/doi.org\/10.5217\/crossmark_policy","source":"Crossref","is-referenced-by-count":14,"title":["Clinical features of very early-onset inflammatory bowel disease in Japan: a retrospective single-center 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