Food protein-induced enterocolitis syndrome in Spanish children: Results from a multicentre prospective study

Food protein-induced enterocolitis syndrome (FPIES) is a non-immunoglobulin E (IgE)-mediated gastrointestinal food allergy characterized by delayed repetitive vomiting, often accompanied by pallor, lethargy, diarrhoea, dehydration and occasionally hypotension. Its pathophysiology remains unclear and diagnosis relies on clinical criteria. We aimed to describe the clinical characteristics, trigger profile, diagnostic pathway and management of FPIES cases in Spain.

Prospective multicentre registry study conducted in Spain, including children born in 2019 diagnosed with acute or chronic FPIES according to international consensus criteria.

Thirty-two FPIES episodes were recorded in 28 children; 18/28 (64%) were male. Most cases were acute (26/28, 92.8%), with mild-to-moderate severity (26/28, 92.8%). Cow's milk (CM) (19/32, 59.4%), hen's egg (5/32, 15.6%) and fish (3/32, 9.4%) were the most common food triggers, and most children reacted to a single food. Almost a third (6/19) of the CM-FPIES cases presented during exclusive breastfeeding. Despite 16/28 (57.1%) patients experiencing two or more vomiting episodes prior to diagnosis, median diagnostic delay was only 6.5 days (interquartile range 0.25-13.5). Diagnostic oral food challenge was performed in 6/26 (23%) of acute FPIES and 1/2 (50%) of chronic FPIES. Acute management included intravenous fluids, ondansetron and oral rehydration. In CM-FPIES, extensively hydrolyzed formulas were the main choice, while rice hydrolysates and elemental formulas were used less frequently.

Most children reacted to a single offending food. CM remains the most frequent trigger, although hen's egg and fish are also relevant. Improved clinical awareness may have contributed to earlier diagnosis.