A Slice of FPIES (food protein-induced enterocolitis syndrome): Insights from 441 children with FPIES as provided by caregivers in the International FPIES Association.

Food-protein induced enterocolitis syndrome (FPIES) is a non-IgE-mediated gastrointestinal food allergy. There is little known about demographics, food triggers and risk factors for FPIES.We characterized demographics, food triggers, risk factors, and management in a cohort of 441 children with FPIES.We retrospectively analyzed surveys completed by 410 caregiver-members of the International FPIES Association (I-FPIES), both at a conference for families affected by FPIES and online.The majority of children were female (50.7%), white (86.2%), and atopic (54.8%), with median age two years (range two months-19 years). The most common food groups avoided were grains (60.0%), cow's milk (52.4%), vegetables (42.7%), and fruits (38.0%). Avocado was the most commonly avoided fruit. Avoiding avocado was associated with increased likelihood of avoiding banana (P< 0.001). 69.4% avoided at least two food groups due to FPIES. 20.3% had a first-degree relative with FPIES. Having a first-degree relative with FPIES was associated with increased likelihood of avoiding multiple food groups (P=0.035).Grains was the most common food group avoided, consistent with recent literature. Avocado avoidance was higher than previously reported, and this is the first report of an association between avocado and banana avoidance in FPIES. Avoiding multiple food groups due to FPIES was more common than previously reported. A large proportion of children in our cohort had first-degree relatives with FPIES and, furthermore, having a first-degree relative with FPIES was significantly associated with the affected child avoiding multiple food groups.