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Food-protein induced enterocolitis syndrome — Clinical Guidelines

Overview

Food protein-induced enterocolitis syndrome (FPIES) is a non-IgE mediated hypersensitivity reaction primarily affecting infants, characterized by gastrointestinal symptoms such as vomiting, diarrhea, and dehydration, often triggered by cow's milk or soy proteins 1 11.

Diagnosis

Clinical Criteria: Projectile vomiting, diarrhea, dehydration, lethargy, and failure to thrive 1 11.

Diagnostic Tests: Open food challenge (OFC) is definitive 1 2.

Laboratory Findings: Elevated C-reactive protein (CRP), eosinophilia, and increased absolute neutrophil count (ANC) post-OFC 6 8 10.

Methemoglobin Levels: Elevated methemoglobin levels may aid in diagnosis, particularly in neonates 2 4.

Management

Avoidance of Trigger Foods: Strict elimination of causative food proteins (e.g., cow's milk, soy) 1 3.

Supportive Care: Rehydration, electrolyte management, and monitoring for growth retardation 1 3.

Growth Monitoring: Regular assessment of growth parameters due to potential for growth retardation 3.

Special Populations

Pediatrics: Chronic management focuses on dietary exclusion and growth monitoring 1 3.

Neonates: Consider elevated methemoglobin levels as a diagnostic clue 2 4.

Key Recommendations

Perform Open Food Challenge (OFC) for Diagnosis: Essential for confirming FPIES diagnosis 1 2.

(Evidence: Strong)

Monitor Growth Parameters Closely: Regular assessments are crucial due to risk of growth retardation 3.

(Evidence: Moderate)

Avoid Trigger Foods: Strict dietary exclusion of identified food allergens is critical 1 3.

(Evidence: Strong)

References

1 Monti G, Zarini V, Castagno E, Bondone C. Phenotypes and follow-up of chronic cow's milk food protein-induced enterocolitis syndrome: A 16-year prospective observational study. Annals of allergy, asthma & immunology : official publication of the American College of Allergy, Asthma, & Immunology 2025. link 2 Sanjurjo-Jimenez I, Menéndez Hernando C, Hidalgo Montes I, Chacón Aguilar R, González González C, López Carnero J et al.. Neonatal metabolic acidosis caused by food proteininduced enterocolitis syndrome: a case report. Archivos argentinos de pediatria 2023. link 3 Bulsa K, Standowicz M, Baryła-Pankiewicz E, Czaja-Bulsa G. Chronic Milk-Dependent Food Protein-Induced Enterocolitis Syndrome in Children from West Pomerania Region. Nutrients 2021. link 4 Makita E, Kuroda S, Sato H, Itabashi K, Kawano A, Matsuura M et al.. Comparison of methemoglobin levels in food protein-induced enterocolitis syndrome and other gastrointestinal diseases in neonates. Allergologia et immunopathologia 2020. link 5 Ichimura S, Kakita H, Asai S, Mori M, Takeshita S, Ueda H et al.. A Rare Case of Fetal Onset, Food Protein-Induced Enterocolitis Syndrome. Neonatology 2019. link 6 Shimomura M, Ito Y, Tanaka H, Meguro T, Kimura M. Increased serum cortisol on oral food challenge in infants with food protein-induced enterocolitis syndrome. Pediatrics international : official journal of the Japan Pediatric Society 2018. link 7 Kimura M, Shimomura M, Morishita H, Meguro T, Seto S. Eosinophilia in infants with food protein-induced enterocolitis syndrome in Japan. Allergology international : official journal of the Japanese Society of Allergology 2017. link 8 Kimura M, Shimomura M, Morishita H, Meguro T. Prognosis of infantile food protein-induced enterocolitis syndrome in Japan. Pediatrics international : official journal of the Japan Pediatric Society 2017. link 9 Kimura M, Ito Y, Shimomura M, Morishita H, Meguro T, Adachi Y et al.. Cytokine profile after oral food challenge in infants with food protein-induced enterocolitis syndrome. Allergology international : official journal of the Japanese Society of Allergology 2017. link 10 Kimura M, Ito Y, Tokunaga F, Meguro T, Shimomura M, Morishita H et al.. Increased C-reactive protein and fever in Japanese infants with food protein-induced enterocolitis syndrome. Pediatrics international : official journal of the Japan Pediatric Society 2016. link 11 Feuille E, Nowak-Węgrzyn A. Definition, etiology, and diagnosis of food protein-induced enterocolitis syndrome. Current opinion in allergy and clinical immunology 2014. link 12 Meisel H. Antibodies from egg yolk of immunized hens against a bioactive caseinopeptide (beta-casokinin-10). Biological chemistry Hoppe-Seyler 1994. link

Food-protein induced enterocolitis syndrome

Overview

Diagnosis

Management

Special Populations

Key Recommendations

References

Original source