title: Definition, etiology, and diagnosis of food protein-induced enterocolitis syndrome.
abstract: OBJECTIVE
  Food protein-induced enterocolitis syndrome (FPIES) is a poorly understood non-IgE-mediated food hypersensitivity, primarily affecting infants and toddlers. There are few data regarding pathophysiology of FPIES that suggest local intestinal imbalance between TNF-α and TGF-β. Patients frequently present with multiple reactions, which are characterized by projectile, repetitive emesis, dehydration, lethargy, and failure to thrive. Despite the severity of presentation, the diagnosis is frequently delayed, and patients often undergo extensive and invasive evaluation prior to reaching the diagnosis.
  
  
  RESULTS
  Reviews published in the last year provide a general approach to diagnosis and management of FPIES and aim to increase awareness and understanding of FPIES among general pediatricians.
  
  
  CONCLUSIONS
  Multicenter studies are necessary to reevaluate and modify the oral food challenge criteria. Research on the pathophysiology of FPIES reactions is necessary to provide insight into the evidence-based approach to diagnosis and management of FPIES. Registries are needed to understand the phenotype, triggers, and prevalence of FPIES.
authors.affiliation: Department of Pediatrics, Jaffe Food Allergy Institute, Division of Allergy and Immunology, Icahn School of Medicine at Mount Sinai, New York, New York, USA.
authors.forename: Elizabeth
authors.initials: E
authors.lastname: Feuille
authors.forename: Anna
authors.initials: A
authors.lastname: Nowak-Węgrzyn
mesh_terms: D002675:Child, Preschool; D004044:Dietary Proteins / Q000009:adverse effects*; D004760:Enterocolitis / Q000145:classification / Q000175:diagnosis* / Q000209:etiology* / Q000378:metabolism; D005512:Food Hypersensitivity / Q000145:classification / Q000175:diagnosis / Q000209:etiology / Q000378:metabolism; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D013577:Syndrome; D016212:Transforming Growth Factor beta / Q000378:metabolism; D014409:Tumor Necrosis Factor-alpha / Q000378:metabolism
chemical_list: D004044:Dietary Proteins; D016212:Transforming Growth Factor beta; D014409:Tumor Necrosis Factor-alpha