The role of the innate and adaptive immune system in pediatric inflammatory bowel disease.
The role of the innate and adaptive immune system in pediatric inflammatory bowel disease.
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DOI：
10.1097/mib.0b013e318281f590
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发表时间：
2013-08
影响因子：
4.9
通讯作者：
Denson LA
中科院分区：
文献类型：
--
作者：
Denson LA
最近的转化研究为小儿发病的炎症性肠病（IBD）的发病机制提供了新的见解。登记研究已经确定了不同的临床表型与发病年龄的增加，这导致了临床表型系统的修订，现在称为巴黎分类系统。据认为，有婴儿（年龄<1岁），非常早发病（VEO，1-10岁），和早发性（EO，10-17岁）的疾病形式。在婴儿和VEO形式中发现了影响抗微生物和抗炎途径的罕见基因突变，而在EO疾病中鉴定的遗传途径与成人发病的IBD相似。婴儿型和VEO型的发病率增加表明了重要的环境影响。这可能最终通过肠道植物群的改变（生态失调）和对植物群的免疫应答失调来表达，这被认为是粘膜炎症的关键触发因素。这些数据最终将指导新的疾病致病模型，这将为个体患者的治疗和其高危家庭成员的疾病预防提供信息。
Recent translational studies have provided new insights into the pathogenesis of pediatric-onset Inflammatory Bowel Disease (IBD). Registry studies have identified distinct clinical phenotypes with increasing age of onset; this has led to a revision of the clinical phenotyping system, now termed the Paris classification system. It is recognized that there are infantile (age <1 years), very early onset (VEO, age 1-10), and early onset (EO, age 10-17) forms of disease. Rare genetic mutations affecting anti-microbial and anti-inflammatory pathways have been discovered in infantile and VEO forms, while genetic pathways identified in EO disease have been similar to adult-onset IBD. An increasing incidence in the infantile and VEO forms has suggested an important environmental influence. This is likely ultimately expressed via alterations in the enteric flora (dysbiosis) and dysregulated immune responses to the flora which are recognized as a critical trigger for mucosal inflammation. These data should ultimately guide new pathogenic models of disease which will inform both therapy in individual patients, and disease prevention in their at-risk family members.