The differences between adults and children in inflammatory bowel disease (IBD) phenotype, severity, complications, co-morbidities, and response to the therapy resulted in the extraction of paediatric IBD. It has been revealed that the substantial role in the development of IBD in children under 6 years of age plays a single genetic mutation (monogenic IBD). On the other hand, in older children and adolescents IBD is usually associated with number of interactions between susceptibility loci (polygenic IBD). Until now there have been described about 60 monogenic defects which affect the variety of immune mechanisms in IBD pathogenesis including epithelial barrier, function of neutrophil granulocytes and phagocytes, T- and B-cell selection and activation, immune inhibitory mechanisms, or apoptosis. Il-10 is an anti-inflammatory cytokine which modulates innate and adaptive immunity affecting expression of pro-inflammatory molecules and function of the variety of immune cells. Patients with identified defects in Il-10 pathway manifest with life-threating colitis with perianal lesions which occurs within first months of life. Allogenic hematopoietic stem cell transplantation is curative therapy in children with Il-10 signalling defects. Clinical awareness of Il-10 signalling defects enables early recognition and prompt management of the disease.

中文翻译：

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小儿炎症性肠病中的白细胞介素 10 和白细胞介素 10 受体：从长凳到床边课程

成人和儿童在炎症性肠病 (IBD) 表型、严重程度、并发症、合并症和对治疗的反应方面的差异导致了儿童 IBD 的提取。已经揭示，在 6 岁以下儿童 IBD 发展中的重要作用是单一基因突变（单基因 IBD）。另一方面，在年龄较大的儿童和青少年中，IBD 通常与易感位点（多基因 IBD）之间的相互作用数量相关。到目前为止，已经描述了大约 60 种单基因缺陷，这些缺陷会影响 IBD 发病机制中的多种免疫机制，包括上皮屏障、中性粒细胞和吞噬细胞的功能、T 细胞和 B 细胞的选择和激活、免疫抑制机制或细胞凋亡。Il-10 是一种抗炎细胞因子，可调节先天性和适应性免疫，影响促炎分子的表达和各种免疫细胞的功能。在 Il-10 通路中发现缺陷的患者在出生后的头几个月内会出现危及生命的结肠炎并伴有肛周病变。同种异体造血干细胞移植是治疗 Il-10 信号缺陷儿童的治疗方法。对 Il-10 信号缺陷的临床认识能够早期识别和及时管理疾病。同种异体造血干细胞移植是治疗 Il-10 信号缺陷儿童的治疗方法。对 Il-10 信号缺陷的临床认识能够早期识别和及时管理疾病。同种异体造血干细胞移植是治疗 Il-10 信号缺陷儿童的治疗方法。对 Il-10 信号缺陷的临床认识能够早期识别和及时管理疾病。