Progress in the pathogenesis and treatment of very early-onset inflammatory bowel disease caused by deficiency of interleukin-10 signaling pathway
10.3760/cma.j.cn101070-20221212-01403
- VernacularTitle:白细胞介素10信号通路缺陷引起极早发型炎症性肠病发病机制及治疗的研究进展
- Author:
Tianhao WU
1
;
Heng ZHANG
;
Yongjun FANG
Author Information
1. 南京医科大学附属儿童医院血液肿瘤科，南京 210008
- Keywords:
Interleukin-10;
Very early onset inflammatory bowel disease;
Single gene disease;
Immunity;
Therapy
- From:
Chinese Journal of Applied Clinical Pediatrics
2024;39(3):228-231
- CountryChina
- Language:Chinese
-
Abstract:
Very early onset inflammatory bowel disease (VEO-IBD) in children refers to an IBD with the onset age of less than 6 years old, clinically characterized by recurrent colitis, perianal lesions, and nutrient absorption disorders.Different from adults, single gene mutation plays an important role in the pathogenesis of VEO-IBD.To date, about 70 single gene defects have been identified involving the pathogenesis of VEO-IBD, including epithelial barrier, neutrophil and phagocyte function, immune cell selection and activation, immunosuppressive mechanism, or apoptosis.Interleukin-10 (IL-10) is an anti-inflammatory cytokine that regulates innate and adaptive immunity, influences the expression of pro-inflammatory molecules and the function of multiple immune cells, and plays a vital role in the development and progression of IBD.Patients with defects in the IL-10 signaling pathway (IL-10 or IL-10 receptor deficiency) may develop life-threatening colitis as early as childhood.This article reviews the progress in the pathogenesis and treatment of VEO-IBD caused by IL-10 signaling pathway defects.
