{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,21]],"date-time":"2026-05-21T18:10:00Z","timestamp":1779387000198,"version":"3.53.1"},"reference-count":53,"publisher":"MDPI AG","issue":"5","license":[{"start":{"date-parts":[[2026,5,11]],"date-time":"2026-05-11T00:00:00Z","timestamp":1778457600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Children"],"abstract":"<jats:p>Background\/Objectives: Very early onset inflammatory bowel disease (VEO-IBD), frequently associated with monogenic defects, is increasingly recognized worldwide but remains poorly characterized in Vietnam. This study aimed to characterize the clinical phenotypes and genetic spectrum of Vietnamese children with VEO-IBD. Methods: We conducted a retrospective cohort study at a tertiary pediatric referral center in Vietnam from July 2016 to January 2026. Clinical, laboratory, endoscopic, histopathological, genetic, and treatment data were systematically collected and analyzed. Monogenic variants were identified using next-generation sequencing and classified according to ACMG criteria. Results: Thirty-six children were included, with a median age at onset of 7.5 months, and 72.2% presenting before 24 months. Crohn\u2019s disease predominated (72.2%). Disease burden was high, with growth impairment in 75.0% and anemia in 91.7%. Extraintestinal manifestations were frequent, particularly recurrent infections (72.2%), dermatitis (44.4%), and oral ulcers (44.4%). Perianal disease occurred in 58.3%, with early complications including perianal ulcer (44.4%), perianal abscess (30.6%) and fistulas (33.3%). Inflammatory markers were markedly elevated, and disease activity indices indicated moderate-to-severe disease at diagnosis. Genetic testing was performed in 91.7% of patients, identifying monogenic etiologies in 30.3%. Identified variants involved genes related to immune regulation (IL10RA\/IL10RB, FOXP3, XIAP), autoinflammation (TNFAIP3), host defense (CYBB), and epithelial function (MYO5B). Conclusions: Monogenic etiologies account for a substantial proportion of VEO-IBD and are associated with distinct clinical phenotypes and therapeutic implications. Early integration of genomic testing with clinical phenotyping is essential to improve diagnostic precision and enable pathway-based treatment, supporting precision medicine in pediatric IBD.<\/jats:p>","DOI":"10.3390\/children13050666","type":"journal-article","created":{"date-parts":[[2026,5,21]],"date-time":"2026-05-21T16:46:18Z","timestamp":1779381978000},"page":"666","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Clinical Phenotypes and Genetic Findings in Very-Early-Onset Inflammatory Bowel Disease: A Vietnamese Pediatric Cohort Study"],"prefix":"10.3390","volume":"13","author":[{"ORCID":"https:\/\/orcid.org\/0009-0009-9804-7387","authenticated-orcid":false,"given":"Manh Cuong","family":"Nguyen","sequence":"first","affiliation":[{"name":"Department of Pediatrics, Military Hospital 103, Vietnam Military Medical University, Hanoi 100000, Vietnam"},{"name":"Department of Pediatrics, Hanoi Medical University, Hanoi 100000, Vietnam"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0000-5993-5837","authenticated-orcid":false,"given":"Thi Viet Ha","family":"Nguyen","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Hanoi Medical University, Hanoi 100000, Vietnam"},{"name":"Department of Gastroenterology, National Children\u2019s Hospital, Hanoi 100000, Vietnam"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Loi","family":"Nguyen","sequence":"additional","affiliation":[{"name":"Department of Gastroenterology, National Children\u2019s Hospital, Hanoi 100000, Vietnam"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thuy Ha","family":"Dang","sequence":"additional","affiliation":[{"name":"Department of Gastroenterology, National Children\u2019s Hospital, Hanoi 100000, Vietnam"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0000-9702-425X","authenticated-orcid":false,"given":"Tam","family":"Tran","sequence":"additional","affiliation":[{"name":"Department of Medicine, Washington University School of Medicine, Saint Louis, MO 63110, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thi Van Anh","family":"Nguyen","sequence":"additional","affiliation":[{"name":"Department of Immunology-Allergy-Rheumatology, National Children\u2019s Hospital, Hanoi 100000, Vietnam"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ngoc Thach","family":"Hoang","sequence":"additional","affiliation":[{"name":"Department of Pathology, National Children\u2019s Hospital, Hanoi 100000, Vietnam"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ngoc Quynh Le","family":"Nguyen","sequence":"additional","affiliation":[{"name":"Stem Cells Center, National Children\u2019s Hospital, Hanoi 100000, Vietnam"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0000-6344-9479","authenticated-orcid":false,"given":"Thi Minh Phuong","family":"Do","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Hanoi Medical University, Hanoi 100000, Vietnam"},{"name":"Department of Gastroenterology, National Children\u2019s Hospital, Hanoi 100000, Vietnam"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Van Tinh","family":"Nguyen","sequence":"additional","affiliation":[{"name":"Department of Gastroenterology, National Children\u2019s Hospital, Hanoi 100000, Vietnam"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hai Yen","family":"Vu","sequence":"additional","affiliation":[{"name":"Department of Gastroenterology, National Children\u2019s Hospital, Hanoi 100000, Vietnam"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thi Ngoc Hong","family":"Nguyen","sequence":"additional","affiliation":[{"name":"Department of Gastroenterology, National Children\u2019s Hospital, Hanoi 100000, Vietnam"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thi Thu Trang","family":"Nguyen","sequence":"additional","affiliation":[{"name":"Department of Gastroenterology, National Children\u2019s Hospital, Hanoi 100000, Vietnam"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thi Cam Van","family":"Le","sequence":"additional","affiliation":[{"name":"Department of Gastroenterology, National Children\u2019s Hospital, Hanoi 100000, Vietnam"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0004-6078-5839","authenticated-orcid":false,"given":"Thi Khanh Ngoc","family":"Bui","sequence":"additional","affiliation":[{"name":"Department of Nutrition, National Children\u2019s Hospital, Hanoi 100000, Vietnam"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thi Thuy Hang","family":"Le","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Military Hospital 103, Vietnam Military Medical University, Hanoi 100000, Vietnam"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0006-5494-4500","authenticated-orcid":false,"given":"Minh Dien","family":"Tran","sequence":"additional","affiliation":[{"name":"National Children\u2019s Hospital, Hanoi 100000, Vietnam"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2026,5,11]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"1147","DOI":"10.1053\/j.gastro.2021.12.282","article-title":"Twenty-first Century Trends in the Global Epidemiology of Pediatric-Onset Inflammatory Bowel Disease: Systematic 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