Abstract
Food Protein-Induced Enterocolitis Syndrome (FPIES) is a heterogeneous disorder that predominantly affects infants and small children. Pathophysiology is poorly understood.
The syndrome is classified as a non-IgE food allergy. Dominant localization of immunopathology reaction and clinical manifestation is gastrointestinal, but also the cellular innate immune system appears to be activated during the systemic FPIES reaction.
Acute FPIES is typically represented by repetitive vomiting delayed 1-4 hours after the ingestion of the trigger food, as well as lethargy and eventually hypotension. In a few hours it can be followed by profuse diarrhea.
Infants are endangered by dehydration, metabolic disorder and hypovolemic shock. Chronic FPIES emerge when formula feeding is continuous and trigger food is not recognized.
FPIES is often misdiagnosed as gastroenteritis or sepsis. Any food protein can trigger FPIES.
Globally the most common FPIES triggers are cow milk, rice, soya and egg. Fish has been reported as a frequent trigger for FPIES in Europe recent few years, especially in Spain, Italy and Greece.
Fish and shellfish triggers have been overlooked for a long time.