Abstract

Food protein-induced enterocolitis syndrome (FPIES) is a non-immunoglobin E-mediated food hypersensitivity disorder. However, little is known about the clinical features of FPIES in patients with Down syndrome (DS). Medical records of children with DS diagnosed at our hospital between 2000 and 2019 were retrospectively reviewed. Among the 43 children with DS, five (11.6%) were diagnosed with FPIES; all cases were severe. In the FPIES group, the median age at onset and tolerance was 84 days and 37.5 months, respectively. Causative foods were cow’s milk formula and wheat. The surgical history of colostomy was significantly higher in the FPIES group than in the non-FPIES group. A colostomy was performed in two children in the FPIES group, both of whom had the most severe symptoms of FPIES, including severe dehydration and metabolic acidosis. The surgical history of colostomy and postoperative nutrition of formula milk feeding may have led to the onset of FPIES. Therefore, an amino acid-based formula should be considered for children who undergo gastrointestinal surgeries, especially colostomy in neonates or early infants. When an acute gastrointestinal disease is suspected in children with DS, FPIES should be considered. This may prevent unnecessary tests and invasive treatments.

Highlights

  • Food protein-induced enterocolitis syndrome (FPIES) is a non-immunoglobin E (IgE)mediated food hypersensitivity disorder that primarily affects formula-fed infants and young children [1,2]

  • In Cases 4 and 5 (Table 4), surgery for colostomy and postoperative nutrition of formula milk feeding may have led to the onset of FPIES

  • An amino acid-based formula should be considered for children who undergo gastrointestinal surgeries, especially colostomy in neonates or early infants

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Summary

Introduction

Food protein-induced enterocolitis syndrome (FPIES) is a non-immunoglobin E (IgE)mediated food hypersensitivity disorder that primarily affects formula-fed infants and young children [1,2]. The clinical manifestation of FPIES is characterized by profuse and repetitive vomiting, usually occurring within a few hours of feeding, accompanied by lethargy and pallor; diarrhea may occur within 24 h. Symptoms usually resolve hours after the elimination of the causative food from the diet. Infants who consume foods such as cow’s milk or soy-based formula daily may experience chronic weight loss and failure to thrive [3]. The true incidence of FPIES is not known, large population-based cohort studies from Israel and Spain have reported the cumulative incidence of cow’s milk

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