Is Early-onset Inflammatory Bowel Disease a Primary Immune Deficiency?

Rachna Shanbhag

doi:10.5005/jp-journals-10081-1310

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Is Early-onset Inflammatory Bowel Disease a Primary Immune Deficiency?

Rachna Shanbhag

Keywords : Inflammatory bowel disease, Primary immune deficiency disease, Very early-onset IBD

Citation Information : Shanbhag R. Is Early-onset Inflammatory Bowel Disease a Primary Immune Deficiency?. Pediatr Inf Dis 2021; 3 (3):125-127.

DOI: 10.5005/jp-journals-10081-1310

License: CC BY-NC 4.0

Published Online: 25-01-2022

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Abstract

Pediatric-onset IBDs (PIBDs) are a group of genetically heterogeneous diseases with variable severity. Depending on the age of onset, inflammatory bowel disease (IBD) can be classified as pediatric-onset (<17 years), early-onset (<10 years), very early-onset (<6 years), infant/toddler-onset (0–2 years), and neonatal-onset IBD (<28 days). Due to the advancement of molecular science and sequencing technologies, several monogenic defects have been identified in very early-onset IBD (VEO-IBD) over the last few decades. Inflammatory bowel disease manifesting at a very young age is more likely to be a monogenic defect. Pediatric-onset IBDs have been found to be associated with as many as 60 monogenic defects with most presenting below 6 years of age and some presenting before 1 year of age. The current article discusses early-onset IBD in detail with new concepts and a clinical and laboratory approach to these patients has been provided.

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