Clinical presentation and referral characteristics of food protein-induced enterocolitis syndrome in the United Kingdom

Abstract Background Food protein-induced enterocolitis syndrome (FPIES) is a pediatric non–IgE-mediated allergic reaction to foods. The diagnosis of FPIES is clinical, with children presenting typically 2 to 4 hours after ingestion of a food protein. The most striking symptoms are vomiting, lethargy, and pallor. Misdiagnosis and delay in presentation to an allergist or gastroenterologist are common. Objectives To investigate the pathway of patients with FPIES presenting to a specialist clinic in the United Kingdom to ascertain whether they experienced delays or misdiagnoses and to investigate their symptoms and triggers. Methods All patients with FPIES presenting over a 3-year period (2010–2013) in a tertiary pediatric allergy clinic in London were analyzed retrospectively. This was performed by searching electronically for all patients with a diagnosis of FPIES and manually reviewing paper notes. Presenting symptoms and management pathways were collated. Results Fifty-four patients were identified, with an average age of onset at 8 months. They initially presented to medical professionals other than an allergist or gastroenterologist. The most frequent presenting symptom was vomiting followed by signs suggesting shock or hypotension and diarrhea. Differential diagnoses included gastroenteritis, sepsis, and surgical abnormalities. The main eliciting foods were cow's milk, fish, egg, soy, and wheat. Conclusion In the United Kingdom, FPIES typically has its onset at 8 months. Patients experience a delay of 12 months in the diagnosis of FPIES and frequently have multiple episodes and interim diagnoses. A great need remains for enhanced education of medical practitioners dealing with children about the varied presentations of FPIES.