Food Protein-Induced Enterocolitis Syndrome: Data From a Multicenter Retrospective Study in Spain

Abstract

To describe the clinical presentation and management practices of food protein-induced enterocolitis syndrome (FPIES).The study included 120 patients (0.5 months to 12 years of age) diagnosed with FPIES in 2017 across 37 hospitals in Spain.This study involved a retrospective chart review of patients diagnosed with FPIES using the 2017 international consensus criteria. Age, clinical symptoms, implicated foods, oral food challenge, laboratory values, skin prick testing, number of episodes before diagnosis, and management approach were collected and analyzed for comparison.The researchers in this study demonstrated that 92.5% (n = 111) of FPIES cases had an acute presentation (2–4 hours), with the majority (76.7%) considered mild to moderate in severity. The most common food trigger was cow’s milk (36.7%), followed by fish (32.5%), hen’s egg (10.8%), and rice (10%). Acute FPIES frequently presented with vomiting (100%), pallor (89.2%), and lethargy (88.3%); whereas chronic FPIES presented with diarrhea (77.8%) or bloody stools (44%). Although 96% of the study population had immunoglobulin E or skin prick testing performed, only 15.7% of patients had positive test results. The acute management of FPIES involved intravenous fluids (46.7%), ondansetron (34.2%), and intravenous corticosteroids (5%). Additionally, extensively hydrolyzed formula was used in 69.8% of cow’s milk FPIES cases, whereas amino acid–based formulas were used in 20.5%. Interestingly, 50% of rice FPIES had a severe course compared with 20% for cow’s milk, 21% for fish, and 7.6% for hen’s egg (P = .032). Delays in diagnosis were common, with 58.5% of patients having at least 2 to 3 episodes before diagnosis. Median age of diagnosis for cow’s milk FPIES was 5 months of age, in contrast to fish and hen’s egg (14.5 and 13.8 months, respectively).FPIES requires further characterization and standardization of practice management. In this expansive description of FPIES in a multicenter Spanish cohort, cow’s milk remained the most common trigger, but fish and egg were also recognized as important triggers.This is the largest European FPIES series published using recent international criteria for diagnosis. This observational study demonstrates the gaps in standardization of diagnosis and management. This study also suggests cultural and dietary factors may affect disease presentation because fish is rarely a trigger for FPIES in the United States, whereas it is the second most common trigger in Spain. This study also highlights the need for future reports to identify risk factors and noninvasive biomarkers to improve diagnostic delays.