极早发型与儿童及青少年炎症性肠病治疗策略

Strategy of treatment for very-early-onset and pediatric inflammatory bowel disease

炎症性肠病(inflammatory bowel disease,IBD)可见于各年龄段,若患者发病年龄小于6岁,可称为极早发型炎症性肠病(very early-onset inflammatory bowel disease,VEO-IBD)。VEO-IBD多由单基因突变所致,与儿童及青少年IBD发病机制不同;两者临床表现与疾病自然病程亦有不同,VEO-IBD发病时症状重,以炎症反应为主,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;两者治疗策略亦存在不同。文章总结了VEO-IBD与儿童及青少年IBD的治疗进展,阐述了两者治疗策略选取的不同。

Very-early-onset inflammatory bowel disease refers to the diseases whose have onset is before six years of age in children. Most of them have monogenic disorders. Pediatric inflammatory bowel diseases have different symptoms, natural history and pathogenesis. VEO-IBD patients usually have severe diseases on onset, with predominant inflammation, especially colitis, which are complicated with systemic involvements, causing high mortality. Different treatment strategys should be applied. This review demonstrates advances in strategy for treatment of very early-onset and pediatric inflammatory bowel disease with a focus on the difference between these two disorders.