Abstract: Patients with very early-onset inflammatory bowel disease（VEO-IBD）have early disease onset，limited environ-mental exposure，and strong family history. Most cases are due to single gene defect. This entity is different from pediatric and adolescent IBD in pathogenesis and natural disease history.VEO-IBD patients have severe inflammatory manifestations and extensive colitis with systemic involvement.Patients suffer from high mortality.In order to improve outcome and prognosis，prompt precise diagnosis and treatment should be carried out.

Key words: very early -onset inflammatory bowel disease, monogenic diseases, genetic sequencing, precision diagnosis and treatment

摘要： 极早发型炎性肠病（very early-onset inflammatory bowel disease，VEO-IBD）患儿发病早、环境暴露时间有限、具有较强家族史，且多由于单基因缺陷所导致。该病与儿童及青少年 IBD 发病机制、疾病自然病程不同，VEO-IBD 患儿临床症状重，以炎性反应为主，多表现为广泛性结肠病变，伴发严重全身表现，具有较高的病死率。针对VEO-IBD，需要尽早开展精准诊断及治疗策略，以改善患儿治疗结局及预后。

关键词: 极早发型炎性肠病, 单基因疾病, 基因检测, 精准诊治