The Role of Monogenic Disease in Children with Very Early Onset Inflammatory Bowel Disease

Overview

Journal Curr Opin Pediatr

Specialty Pediatrics

Date 2017 Jul 13

PMID 28700415

Citations 17

Authors

Judith R Kelsen

Robert N Baldassano

Affiliations

Soon will be listed here.

Abstract

Purpose Of Review: Inflammatory bowel disease (IBD) is a multifactorial disease caused by dysregulated immune responses to commensal or pathogenic intestinal microbes, resulting in chronic intestinal inflammation. Patients diagnosed with IBD occurring before the age of 5 are a unique population, known as very early onset (VEO)-IBD and can be phenotypically and genetically distinct from older-onset IBD. We aim to review the clinical presentation of children with VEO-IBD and recent discoveries that point to genomic drivers of disease that may impact our therapeutic decisions.

Recent Findings: VEO-IBD is increasing in incidence and is associated with more severe disease, aggressive progression and poor response to most conventional therapies. This article will review the advances in sequencing technology that have led to identification of novel gene variants associated with disease and potentially new targeted therapeutic options.

Summary: Children with VEO-IBD may present with a different phenotype and more severe disease than older children and adults. Identification of the causal gene or pathways, these children may allow for true precision medicine with targeted therapy and improved disease course.

Citing Articles

Infantile inflammatory bowel disease in three Syrian infants: a case series.

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A Roadmap for the Human Gut Cell Atlas.

Zilbauer M, James K, Kaur M, Pott S, Li Z, Burger A Nat Rev Gastroenterol Hepatol. 2023; 20(9):597-614.

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Colorectal Cancer associated with pediatric inflammatory bowel disease: a case series.

Kim M, Ko J, Shin M, Hahn J, Moon S, Kim H BMC Pediatr. 2021; 21(1):504.

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The Growing Need to Understand Very Early Onset Inflammatory Bowel Disease.

Zheng H, de la Morena M, Suskind D Front Immunol. 2021; 12:675186.

PMID: 34122435 PMC: 8187749. DOI: 10.3389/fimmu.2021.675186.

Positioning Biologic Therapies in the Management of Pediatric Inflammatory Bowel Disease.

Breton J, Kastl A, Conrad M, Baldassano R Gastroenterol Hepatol (N Y). 2021; 16(8):400-414.

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