Enterocolitis syndrome, food protein-induced P78.3; A09.9

Food protein-induced enterocolitis syndrome, also known as FPIES, is a rare, sometimes acute and severe, non-IgE-mediated gastrointestinal food hypersensitivity.

No sex preference

Aetiopathogenetically, an intolerance reaction to food proteins (which has not been defined in detail so far) forms the basis.

The following foods are the most common triggers: milk or dairy products, soya, cereals; more rarely: rice, oats or fish or egg products.

The disease usually affects very young infants. Very rarely teenagers or adults are affected.

Often after the first contact with the triggering food a profuse, repetitive vomiting occurs 1-4 hours after ingestion, often accompanied by diarrhoea with the risk of dehydration, weight loss, possibly accompanied by fever. Anaphylactic reactions up to severe shock are possible.

If left untreated, the condition leads to weight loss and growth disorders in infants.

In individual cases, tolerance can develop over the course of years (proven in cases of hypersensitivity to fish proteins).

Frequently marked blood eosinophilia. The severity of eosinophilia is correlated with vomiting, bloody stools and diarrhoea. For example, in larger Japanese collectives, patients with diarrhoea only showed lower levels of eosinophilia than those with complete symptoms. CRP is elevated especially in febrile FPIES. No detection of specific IgE antibodies.

The diagnosis is made through an elimination diet with a non-allergenic special food.

Diagnosis verification: should be done by oral provocation. Always perform under stationary clinical conditions!

S.a. proctocolitis, food protein induced

1. González-Delgado P et al (2016) Clinical and immunological characteristics of a pediatric population with food protein-induced enterocolitis syndrome (FPIES) to fish. Pediatric Allergy Immunol 27:269-275.
2. Järvinen KM et al (2013) Food protein-induced enterocolitis syndrome (FPIES): current management strategies and review of the literature. J Allergy Clin Immunol Pract 1:317-322.
3. Kimura M et al (2016) Increased C-reactive protein and fever in Japanese infants with food protein-induced enterocolitis syndrome. Pediatr Int 58:826-830.
4. Kimura M et al (2017) Eosinophilia in infants with food protein-induced enterocolitis syndrome in Japan. Allergol Int 66:310-316.
5. Leonard SA et al (2015) Food protein-induced enterocolitis syndrome. Pediatr Clin North Am 62:1463-1477.