A recent retrospective study (JR Kelsen et al. Inflamm Bowel Dis 2020; 26: 909-18) compares children diagnosed with inflammatory bowel disease at different age points and their outcomes.  During a 9 year study span (2008-16), there were 229 subjects diagnosed as very-early onset (<6 years, VEO), 221 diagnosed as intermediate onset (6-10 years), and 521 diagnosed as older onset (> 10 years)

Key findings:

• VEO-IBD patients were significantly more likely to have had a diverting ileostomy and colectomy than the older patients.  Diverting ileostomy rates: 12.2%, 4.1%, and 1.2% respectively.  Colectomy rates: 7.4%, 4.1%, and 1.7% respectively.
• Ileocecal resections were significantly higher in the older-onset IBD population. In the older group, these resections were noted in 64/521 (12.2%) compared to 1/229 (0.4%) in the VEO group and 10/221 (4.5%) in the intermediate group.
• VEO-IBD patients had higher medication failure rates at 1 year into treatment and were more frequently readmitted to the hospital. For infliximab (IFX), failure rates were 62.4% for VEO subjects compared to 14.6% for older-onset subjects.  For adalimumab, the respective rates were 53.2% vs. 7.2%.
• Targeted therapy was successfully used almost exclusively in the VEO-IBD population

My take: Children with VEO-IBD have a more severe disease course than older children.  Since monogenetic disorders occur in ~8% of the VEO population, targeted therapies are more likely; however; ~2% of older children also have a monogenetic disorder and as such, targeted therapy could be important in this group as well.

Related review article: J Ouahed et al. Very Early Onset Inflammatory Bowel Disease: A Clinical Approach With a Focus on the Role of Genetics and Underlying Immune Deficiencies. Inflamm Bowel Dis 2020; 26: 820-842.  This is a useful review.  A couple of key points:

• “There are no quality studies assessing the use of nutritional approaches in VEO-IBD”
• Stem Cell Transplantation NOT efficacious in these disorders (per Table 3): TTC7A, STXBP2, IKBKG (NEMO)

Related blog posts:

• Patterns and Puzzles with VEO-IBD This is a very good review and the image on this day has one of my favorite patient t-shirts.
• Underlyling Genetic Disease in Pediatric IBD
• VEO-IBD -Useful Position Paper
• More IBD Cases Than Ever in Young Canadian Children | gutsandgrowth
• Expanding VEO Variants
• Why the Genetics of Inflammatory Bowel Diseases Matter Now
• IBD and Immune-Mediated Diseases | gutsandgrowth
• More IBD Cases Than Ever in Young Canadian Children | gutsandgrowth
• IBD Shorts: September 2019 NCF4 defects
• Just the Beginning: Mutations in Very Early Onset … – gutsandgrowth
• Exome Sequencing in VEO-IBD: More Data | gutsandgrowth