Abstract：
Inflammatory bowel diseases (IBD), including Crohn\'s disease and ulcerative colitis, are a heterogeneous group of multifactorial pathologies, often polygenic, due to a dysregulated immune response in a genetically susceptible host. In children under 6 years of age, a significant proportion of IBD, named \"very early onset inflammatory bowel diseases\" (VEO-IBD), are monogenic disorders in more than one third of cases. Over 80 genes have been linked to VEO-IBD and pathological descriptions are sparce. In this clarification, we describe the clinical aspects of monogenic VEO-IBD and the main causative genes, as well as the various histological patterns observed in intestinal biopsies. The management of a patient with VEO-IBD should be a coordinated effort by a multidisciplinary team including pediatric gastroenterologists, immunologists, geneticists, and of course pediatric pathologists.
摘要：
炎症性肠病（IBD），包括克罗恩病和溃疡性结肠炎，是一组异质的多因素病理，通常是多基因的，由于遗传易感宿主的免疫反应失调。6岁以下儿童，很大一部分IBD,命名为“非常早发性炎症性肠病”（VEO-IBD），超过三分之一的病例是单基因疾病。已经有超过80个基因与VEO-IBD相关，并且病理描述很少。在这个澄清中，我们描述了单基因VEO-IBD的临床方面和主要致病基因，以及在肠道活检中观察到的各种组织学模式。VEO-IBD患者的管理应由包括儿科胃肠病学家在内的多学科团队协调工作。免疫学家,遗传学家,当然还有儿科病理学家.
