153 Atypical Food-Protein Induced Enterocolitis Syndrome

Abstract

<h3></h3> Food-protein induced enterocolitis syndrome ( FPIES ) is a non-IgE mediated gastrointestinal hypersensitivity, categorised into two major phenotypes: acute and chronic. Acute FPIES manifests as profuse, repetitive vomiting, 1-4 hours after exposure to a triggering food, sometimes with diarrhoea 5-10 hours later, leading to dehydration and lethargy. In chronic FPIES symptoms develop over days or weeks with intermittent emesis, progressive diarrhoea, abdominal distension and failure to thrive. The pathophysiology of FPIES remains obscure, but there is evidence of profound activation of innate cells, eosinophils, and pan-T-cell activation. Most FPIES cases are induced by cow’s milk proteins and soy in infants, although other single or multiple foods can be triggers, including solid foods, where there are significant geographical differences. The incidence ranges from 0.015 to 0.5% There is a significant lack of awareness among professionals about FPIES and significant delay of diagnosis. There are some adult patients, too. Diagnosis is purely clinical, and there are diagnostic criteria for patients presenting with possible PFIES and diagnostic criteria for the interpretation of oral food challenges ( OFC ), as shown here. The most important aspect of management in patients with FPIES is the avoidance of triggering food. The natural course is favourable, and there are no reports of long-term complications in children with FPIES and it is largely a self-limiting, generally benign disorder of childhood. Patients with FPIES usually have undetectable specific IgE to triggering food, except in rare cases of atypical FPIES. In addition to classic acute FPIES symptoms, children with atypical FPIES can also have symptoms consistent with an acute IgE-mediated hypersensitivity such as urticaria, angioedema, flushing, and wheezing, and are likely to have a more protracted course. Rarely, patients with IgE-mediated food allergies develop non-IgE-mediated food allergies and vice versa. Our patient is a child who had been avoiding cow’s milk due to immediate cutaneous reaction on ingestion, with positive specific IgE and skin prick-to-prick test. After ten months of avoidance, and negative new specific IgE, she was challenged for resolution. She tolerated cow’s milk during the 2 hour observation period, but then developed delayed profuse vomiting. FPIES was confirmed with another, FPIES designed oral food challenge, and she has been advised to continue using an extensive hydrolysate formula. Due to the known possibility of developing FPIES to other proteins, their cautious introduction is advised. Further follow-up will be taken, considering OFC every 18-24 months.