Nonclassic Inflammatory Bowel Disease in Young Infants Immune Dysregulation, Polyendocrinopathy, Enteropathy, X-Linked Syndrome, and Other Disorders

作者：Chandrakasan Shanmuganathan; Venkateswaran Suresh; Kugathasan Subra^*

来源：Pediatric Clinics of North America, 2017, 64(1): 139-+.

DOI：10.1016/j.pcl.2016.08.010

• Very early onset IBD (VEO-IBD)
• IPEX
• IL-10 defects
• Inflammasome
• Epithelial barrier defects
• Neutrophil defects
• T cell or B cell defects

This article discusses non-classical forms of inflammatory bowel disease (IBD) mainly occurs in infants and very young children. Defects in every aspect of the immune system, such as neutrophils, T-cell and B-cell lymphocytes, and macrophages are associated with IBD in infants. Also, non lympho-hematopoietic defects with primary defects in enterocytes can also lead to IBD-like manifestations. Clinical vignettes are presented and the genetic origins and possible management strategies are outlined. Early evaluation of these patients is important because identification of underlying immune defects would facilitate the use of better-targeted therapy for the specific genetic defect.

• 出版日期2017-2