Clinical Manifestations of Food Protein-Induced Enterocolitis Syndrome

Shikha K. Mane; Sami L. Bahna

Disclosures

Curr Opin Allergy Clin Immunol. 2014;14(3):217-221. 

In This Article

Abstract and Introduction

Abstract

Purpose of review To raise awareness among healthcare providers about the clinical and laboratory findings in acute and chronic food protein-induced enterocolitis syndrome (FPIES).

Recent findings FPIES can be caused by trivial exposure or rare foods.

Summary FPIES is a non-IgE-mediated reaction that usually presents with acute severe repetitive vomiting and diarrhea associated with lethargy, pallor, dehydration, and even hypovolemic shock. Manifestations resolve usually within 24–48 h of elimination of the causative food. In chronic cases, symptoms may include persistent diarrhea, poor weight gain, failure to thrive, and improvement may take several days after the food elimination. In the acute cases, laboratory evaluation may reveal thrombocytosis and neutrophilia, peaking about 6 h postingestion. Depending on the severity, metabolic acidosis and methemoglobinemia may occur. In chronic cases, anemia, hypoalbuminemia and eosinophilia may be seen. Radiologic evaluation or other procedures, such as endoscopy and gastric juice analysis may show nonspecific abnormal findings. The diagnosis is based on clinical manifestations. Further studies looking at the phenotypes of FPIES are needed to identify clinical subtypes, and to understand the predisposing factors for developing FPIES compared with immediate-type, IgE-mediated gastroenteropathies.

Introduction

Food-protein induced enterocolitis syndrome (FPIES) is a non-IgE-mediated reaction affecting predominantly infants and children. Adult cases have been recently reported but are rare.[1] A majority of cases occur during infancy, particularly with the early introduction of additional foods. With the general recommendation of delaying introduction of solid foods until 4–6 months, solid-food FPIES presents later than when caused by cow's milk or soybean formulas.

FPIES is characterized by an abnormal response to an ingested food resulting in gastrointestinal inflammation and increased intestinal mucosal permeability.[2] Although sensitization is a prerequisite, some cases apparently occurred following the first exposure that might indicate that the initial sensitizing exposure can be trivial.[3,4] The amount of food required to provoke symptoms has varied widely, reflecting the degree of hypersensitivity in individual patients. The threshold dose could be a normal serving size or a very minute quantity. Although the route of exposure is primarily ingestion, in some patients oral mucosal contact can be significant.[5]

The clinical presentation of FPIES is primarily vomiting and diarrhea, which can be acute or chronic. Comparisons of clinical findings in selected studies are shown in Table 1 .[6,7,8,9,10,11,12] This review will focus on the clinical and laboratory findings in FPIES. The key features in acute and chronic FPIES are shown in Table 2 .[2,13]

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