Hyams Jeffrey S
Division of Digestive Diseases, Hepatology, and Nutrition, Connecticut Children's Medical Center, University of Connecticut School of Medicine, Hartford, Conn., USA.
Dig Dis. 2014;32(4):337-44. doi: 10.1159/000358133. Epub 2014 Jun 23.
Similar to adults, there is heterogeneous phenotypic expression of inflammatory bowel disease (IBD) in children. Thus, a classification system for disease characteristics is obligatory if one seeks to understand and eventually change the natural history of IBD. Extrapolation of adult clinical trial results to children also depends upon comparable classifications of disease. Features that can differentiate IBD in children from adults include more extensive and severe disease at presentation, frequent corticosteroid dependency, change in location and behavior over time, and the implications of disease for growth and sexual maturation. In contrast to the Montreal classification where all patients <17 years were grouped together, the Paris classification recognizes the different expression of pediatric IBD between those patients aged <10 years and those 10-17 years of age. The recent identification of monogenic disorders in very young children (<2 years) with severe IBD-like disease has further clouded the issue of where appropriate pediatric age guidelines should be drawn, though it is clear these infantile-onset cases should not be grouped with older children. The Paris classification recognizes the importance of upper tract disease on natural history by dividing it into L4a and L4b (proximal and distal to the ligament of Treitz, respectively), while the Montreal system groups all upper-tract patients together. Complicated disease behavior in the Montreal system mandated a single category preventing the concomitant designation as stricturing and penetrating, whereas the Paris classification recognizes that both stricturing and penetrating behavior may occur at the same or different times. Growth delay is recognized only in the Paris classification as a serious manifestation of IBD in children affecting therapeutic decisions. As our understanding of the basic molecular mechanisms of disease pathogenesis in IBD changes over time, it is likely that the IBD classification will change as well. A single classification system that reflects both pediatric and adult disease is needed.
与成人相似，儿童炎症性肠病（IBD）也存在异质性表型表达。因此，如果想要了解并最终改变IBD的自然病程，那么疾病特征分类系统必不可少。将成人临床试验结果外推至儿童也依赖于可比的疾病分类。儿童IBD与成人IBD的不同特征包括：发病时病情更广泛、更严重，频繁依赖皮质类固醇，病变部位和行为随时间变化，以及疾病对生长和性成熟的影响。与蒙特利尔分类法将所有17岁以下患者归为一组不同，巴黎分类法认识到10岁以下和10 - 17岁儿童IBD的不同表现。最近在患有严重IBD样疾病的幼儿（<2岁）中发现单基因疾病，进一步模糊了合适的儿科年龄指南应如何制定的问题，不过显然这些婴儿期发病的病例不应与大龄儿童归为一组。巴黎分类法通过将上消化道疾病分为L4a和L4b（分别为屈氏韧带近端和远端）来认识其对自然病程的重要性，而蒙特利尔系统则将所有上消化道患者归为一组。蒙特利尔系统中复杂的疾病行为被归为单一类别，防止同时被指定为狭窄型和穿透型，而巴黎分类法认识到狭窄和穿透行为可能在同一时间或不同时间出现。生长发育迟缓仅在巴黎分类法中被视为影响治疗决策的儿童IBD的严重表现。随着我们对IBD疾病发病机制基本分子机制的理解随时间变化，IBD分类可能也会改变。需要一个能反映儿科和成人疾病的单一分类系统。
