极早发型炎性肠病的诊断及治疗策略

Diagnosis and treatment strategy of very early-onset infla-mmatory bowel disease

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VEOIBD,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。

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.