Food protein-induced enterocolitis syndrome

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Food Protein-Induced Enterocolitis Syndrome (FPIES) is a severe systemic response to food protein that typically occurs 1 to 4 hours after the ingestion of the causative food and frequently develops in the first few years of life.[1][2][3][4] In the severe form, patients will vomit until dehydration and until a shock-like state, which occurs in 15% of patients.[5][6] FPIES occurs primarily in young infants, but can exist in older children and adults.

In the severe form, symptoms include vomiting, diarrhea, and acidosis.[1][2][3] Laboratory studies during the acute episode shows an elevated white blood cell count with a left shift and elevated platelet count and methemoglobinemia.[7] Endoscopy may reveal a mixed Eosinophilic and neutrophilic infiltrate but is not required to make the diagnosis. The exact mechanism is unclear, but it is hypothesized to be a T cell driven disorder.[8]

There is a variant of FPIES and it manifests as chronic emesis, diarrhea, and failure to thrive.[9] Upon re-exposure to the offending food after a period of elimination, a subacute syndrome can present with repetitive emesis and dehydration.

Milk and soy protein are the most common trigger foods, but other foods have been reported including rice, oat, chicken, peanut, potato, fish, and egg.[3][10][11][12] A similar condition also has been reported in adults, most often related to crustacean shellfish ingestion. There are also cases of FPIES being transmitted through foods in breast milk in rare occasions.[13]

Diagnosis is primarily based on history as specific IgE and skin prick tests are typically negative.[9] In one study, atopy patch tests was found to be helpful.[14]

The prevalence of FPIES in a population study in Israel was 0.3%.[15] Studies have found 90% of the children outgrew it by 3 years of age.[15][16]


  1. ^ a b Powell GK. Food protein-induced enterocolitis of infancy: differential diagnosis and management. Comprehensive therapy 1986; 12:28-37
  2. ^ a b Sicherer SH, Eigenmann PA, Sampson HA. Clinical features of food protein-induced enterocolitis syndrome. The Journal of pediatrics 1998; 133:214-9.
  3. ^ a b c Mehr S, Kakakios A, Frith K, Kemp AS. Food protein-induced enterocolitis syndrome: 16-year experience. Pediatrics 2009; 123:e459-64.
  4. ^ Boyce JA, Assa'ad A, Burks AW, Jones SM, Sampson HA, Wood RA, et al. Guidelines for the Diagnosis and Management of Food Allergy in the United States: Summary of the NIAID-Sponsored Expert Panel Report. The Journal of allergy and clinical immunology 2010; 126:1105-18.
  5. ^ Nowak-Wegrzyn A, Muraro A. Food protein-induced enterocolitis syndrome. Current opinion in allergy and clinical immunology 2009; 9:371-7.
  6. ^ Andrews T, Tsarouhas N, Spergel J. Food allergy presenting as a "septic"-appearing infant. Pediatr Emerg Care 2004; 20:677-9.
  7. ^ Anand RK, Appachi E. Case report of methemoglobinemia in two patients with food protein-induced enterocolitis. Clinical pediatrics 2006; 45:679-82.
  8. ^ Mori F, Barni S, Cianferoni A, Pucci N, de Martino M, Novembre E. Cytokine expression in CD3+ cells in an infant with food protein-induced enterocolitis syndrome (FPIES): case report. Clinical & developmental immunology 2009; 2009:679381.
  9. ^ a b Boyce JA, Assa'ad A, Burks AW, Jones SM, Sampson HA, Wood RA, et al. Guidelines for the diagnosis and management of food allergy in the United States: report of the NIAID-sponsored expert panel. The Journal of allergy and clinical immunology 2010; 126:S1-58.
  10. ^ Sicherer SH. Food protein-induced enterocolitis syndrome: case presentations and management lessons. The Journal of allergy and clinical immunology 2005; 115:149-56.
  11. ^ Levy Y, Danon YL. Food protein-induced enterocolitis syndrome--not only due to cow's milk and soy. Pediatric allergy and immunology : official publication of the European Society of Pediatric Allergy and Immunology 2003; 14:325-9.
  12. ^ Ruffner MA, Ruymann K, Barni S, Cianferonia A, Brown-Whitehorn T, Spergel J. Food Protein-induced Enterocolitis Syndrome: Insights from Review of a Large Referral Population. The Journal of Allergy and Clinical Immunology: In Practice 2013; 4:343-9.
  13. ^ Monti G, Castagno E, Liguori SA, Lupica MM, Tarasco V, Viola S, et al. Food protein-induced enterocolitis syndrome by cow's milk proteins passed through breast milk. The Journal of allergy and clinical immunology 2011; 127:679-80.
  14. ^ Fogg MI, Brown-Whitehorn TA, Pawlowski NA, Spergel JM. Atopy patch test for the diagnosis of food protein-induced enterocolitis syndrome. Pediatric allergy and immunology : official publication of the European Society of Pediatric Allergy and Immunology 2006; 17:351-5.
  15. ^ a b Katz Y, Goldberg MR, Rajuan N, Cohen A, Leshno M. The prevalence and natural course of food protein-induced enterocolitis syndrome to cow's milk: a large-scale, prospective population-based study. The Journal of allergy and clinical immunology 2011; 127:647-53 e1-3.
  16. ^ Hwang JB, Sohn SM, Kim AS. Prospective follow-up oral food challenge in food protein-induced enterocolitis syndrome. Archives of Disease in Childhood 2009; 94:425-8.

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