Clinical features of very early-onset inflammatory bowel disease in Japan: a retrospective single-center study
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 aim...
| Main Authors: | , , , , , , , , |
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| Format: | Article |
| Language: | English |
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Korean Association for the Study of Intestinal Diseases
2022-10-01
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| Series: | Intestinal Research |
| Subjects: | |
| Online Access: | http://irjournal.org/upload/pdf/ir-2021-00142.pdf |
| _version_ | 1811191210539220992 |
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| author | Masaaki Usami Ichiro Takeuchi Reiko Kyodo Yuri Hirano Kosuke Kashiwagi Hiroki Fujikawa Hirotaka Shimizu Toshinao Kawai Katsuhiro Arai |
| author_facet | Masaaki Usami Ichiro Takeuchi Reiko Kyodo Yuri Hirano Kosuke Kashiwagi Hiroki Fujikawa Hirotaka Shimizu Toshinao Kawai Katsuhiro Arai |
| author_sort | Masaaki Usami |
| collection | DOAJ |
| description | 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<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. |
| first_indexed | 2024-04-11T15:02:36Z |
| format | Article |
| id | doaj.art-4f3d19b512d24295914b71f96d04ce8a |
| institution | Directory Open Access Journal |
| issn | 1598-9100 2288-1956 |
| language | English |
| last_indexed | 2024-04-11T15:02:36Z |
| publishDate | 2022-10-01 |
| publisher | Korean Association for the Study of Intestinal Diseases |
| record_format | Article |
| series | Intestinal Research |
| spelling | doaj.art-4f3d19b512d24295914b71f96d04ce8a2022-12-22T04:16:54ZengKorean Association for the Study of Intestinal DiseasesIntestinal Research1598-91002288-19562022-10-0120447548110.5217/ir.2021.00142964Clinical features of very early-onset inflammatory bowel disease in Japan: a retrospective single-center studyMasaaki Usami0Ichiro Takeuchi1Reiko Kyodo2Yuri Hirano3Kosuke Kashiwagi4Hiroki Fujikawa5Hirotaka Shimizu6Toshinao Kawai7Katsuhiro Arai8 Center for Pediatric Inflammatory Bowel Disease, Division of Gastroenterology, National Center for Child Health and Development, Tokyo, Japan Center for Pediatric Inflammatory Bowel Disease, Division of Gastroenterology, National Center for Child Health and Development, Tokyo, Japan Center for Pediatric Inflammatory Bowel Disease, Division of Gastroenterology, National Center for Child Health and Development, Tokyo, Japan Center for Pediatric Inflammatory Bowel Disease, Division of Gastroenterology, National Center for Child Health and Development, Tokyo, Japan Center for Pediatric Inflammatory Bowel Disease, Division of Gastroenterology, National Center for Child Health and Development, Tokyo, Japan Center for Pediatric Inflammatory Bowel Disease, Division of Gastroenterology, National Center for Child Health and Development, Tokyo, Japan Center for Pediatric Inflammatory Bowel Disease, Division of Gastroenterology, National Center for Child Health and Development, Tokyo, Japan Division of Immunology, National Center for Child Health and Development, Tokyo, Japan Center for Pediatric Inflammatory Bowel Disease, Division of Gastroenterology, National Center for Child Health and Development, Tokyo, JapanBackground/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<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.http://irjournal.org/upload/pdf/ir-2021-00142.pdfmonogenic inflammatory bowel diseasejapanphenotypedisease severitytherapeutics |
| spellingShingle | Masaaki Usami Ichiro Takeuchi Reiko Kyodo Yuri Hirano Kosuke Kashiwagi Hiroki Fujikawa Hirotaka Shimizu Toshinao Kawai Katsuhiro Arai Clinical features of very early-onset inflammatory bowel disease in Japan: a retrospective single-center study Intestinal Research monogenic inflammatory bowel disease japan phenotype disease severity therapeutics |
| title | Clinical features of very early-onset inflammatory bowel disease in Japan: a retrospective single-center study |
| title_full | Clinical features of very early-onset inflammatory bowel disease in Japan: a retrospective single-center study |
| title_fullStr | Clinical features of very early-onset inflammatory bowel disease in Japan: a retrospective single-center study |
| title_full_unstemmed | Clinical features of very early-onset inflammatory bowel disease in Japan: a retrospective single-center study |
| title_short | Clinical features of very early-onset inflammatory bowel disease in Japan: a retrospective single-center study |
| title_sort | clinical features of very early onset inflammatory bowel disease in japan a retrospective single center study |
| topic | monogenic inflammatory bowel disease japan phenotype disease severity therapeutics |
| url | http://irjournal.org/upload/pdf/ir-2021-00142.pdf |
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