How does knowledge from translational research impact our clinical care of pediatric inflammatory bowel disease patients?
How does knowledge from translational research impact our clinical care of pediatric inflammatory bowel disease patients?
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DOI：
10.1007/s11894-012-0258-4
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发表时间：
2012-06
影响因子：
--
通讯作者：
Denson, Lee A
中科院分区：
文献类型：
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作者：
Denson, Lee A
最近的转化研究为儿科炎症性肠病 (IBD) 的发病机制、疾病行为和治疗反应提供了新的见解。登记研究已经确定了随着发病年龄的增加而出现的不同临床表型；这导致了临床表型系统的修订，现在称为巴黎分类系统。人们认识到存在婴儿期（年龄<2岁）、极早发型（VEO，2-10岁）和早发型（EO，10-17岁）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 EO 疾病中发现的基因途径与成人发病的 IBD 相似。诊断后不久测量的遗传和血清学模式已被证明与更激进的限制行为有关；这些模式现在可以在临床上用于帮助预测病程。最近，已经开发出临床和遗传模型，如果经过验证，可以用来预测治疗反应。
Recent translational studies have provided new insights into pathogenesis, disease behavior, and treatment responses in pediatric 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 <2 years), very early onset (VEO, age 2-10), 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. Genetic and serologic patterns measured soon after diagnosis have been shown to be associated with more aggressive stricturing behavior; these patterns may now be used clinically to help predict disease course. More recently, clinical and genetic models have been developed that, if validated, could be used to predict treatment responses.