A Systematic Review of Monogenic Inflammatory Bowel Disease.

ABSTRACT:

Background & aims

Advances in genomic technologies have led to increasing reports of monogenic inflammatory bowel disease (IBD). Here, we systematically review the literature to determine the clinical features, genetic profile, and previously used treatment strategies in monogenic IBD.

Methods

A systematic review of MEDLINE articles published between January 2000 and December 2020 was conducted. A total of 750 individual monogenic IBD cases were identified from 303 eligible articles.

Results

The most frequently reported monogenic IBD genes were IL10RA/B, XIAP, CYBB, LRBA, and TTC7A. In total, 63.4% of patients developed IBD before 6 years of age, 17.4% developed IBD between ages 10 and 17.9 years, and 10.9% developed IBD after age 18. There was a substantial differenc

SUBMITTER: Nambu R 

PROVIDER: S-EPMC8448782 | biostudies-literature | 2022 Apr

REPOSITORIES: biostudies-literature