Food protein-induced enterocolitis syndrome

Food Protein-Induced Enterocolitis Syndrome (FPIES) is a severe systemic response to food protein that typically occurs 1 to 48 hours after the ingestion of the causative food and frequently develops in the first few years of life.[1][2][3][4][5] In the severe form, patients will vomit until dehydration and until a shock-like state, which occurs in 15% of patients.[6] FPIES has mainly been documented in young infants, but can exist in older children and adults. Birch pollen may also trigger symptoms, similar to Oral Allergy Syndrome (OAS).

Signs and symptoms

In the severe form, symptoms include abdominal pain, vomiting, diarrhea (that usually develops 3-6 hours after ingesting the offending food), and acidosis.[1][2][3] Additional symptoms include headache, pallor, lethargy, constipation, and abdominal swelling (distension).[7][5] Laboratory studies during the acute episode shows an elevated white blood cell count with a left shift and elevated platelet count and methemoglobinemia.[8] 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. There is a variant of FPIES and it manifests as chronic emesis, diarrhea, and failure to thrive. Upon re-exposure to the offending food after a period of elimination, a subacute syndrome can present with repetitive emesis and dehydration.[9]

Diagnosis

Diagnosis is primarily based on history as specific IgE and skin prick tests are typically negative[9] and the exclusion of other disorders that present similar clinical features, such as infectious gastroenteritis, celiac disease, inflammatory bowel disease, and eosinophilic gastroenteritis, among others.[10]

Treatment

Avoid feeding affected individuals the foods known to trigger an allergic response. Cow's milk, soy, and cereal grains are the most common trigger foods, but other foods have been reported including eggs, meats (poultry, beef, pork), seafood (fish, shrimp, mollusks), peanut, potatoes, nuts, and fruits (apple, pear, banana, peach, watermelon).[1][2][3] There are also cases of FPIES being transmitted through foods in breast milk in rare occasions.[11]

References
  1. Nowak-Węgrzyn A, Chehade M, et al. (2017). "International consensus guidelines for the diagnosis and management of food protein-induced enterocolitis syndrome: Executive summary-Workgroup Report of the Adverse Reactions to Foods Committee, American Academy of Allergy, Asthma & Immunology". J. Allergy Clin. Immunol. 139 (4): 1111–1126.e4. doi:10.1016/j.jaci.2016.12.966. PMID 28167094.
  2. Nowak-Węgrzyn A, Jarocka-Cyrta E, Moschione Castro A (2017). "Food Protein-Induced Enterocolitis Syndrome" (PDF). J Investig Allergol Clin Immunol. 27 (1): 1–18. doi:10.18176/jiaci.0135. PMID 28211341.
  3. Michelet M, Schluckebier D, Petit LM, Caubet JC (2017). "Food protein-induced enterocolitis syndrome - a review of the literature with focus on clinical management". J Asthma Allergy. 10: 197–207. doi:10.2147/JAA.S100379. PMC 5499953. PMID 28721077.
  4. Mehr S, Kakakios A, Frith K, Kemp AS (2009). "Food protein-induced enterocolitis syndrome: 16-year experience". Pediatrics. 123 (3): e459–64. doi:10.1542/peds.2008-2029. PMID 19188266.
  5. Ruffner, MA (November 2014). "Food Protein-Induced Enterocolitis Syndrome: Insights From Review of a Large Referral Population" (PDF). Pediatrics. 134: S157. Archived from the original (PDF) on 19 July 2018.
  6. Nowak-Wegrzyn A, Muraro A. Food protein-induced enterocolitis syndrome. Current Opinion in Allergy and Clinical Immunology 2009; 9:371-7.
  7. "Food Protein-Induced Enterocolitis Syndrome". National Organization for Rare Disorders (NORD). Archived from the original on 19 February 2017.
  8. Anand RK, Appachi E. Case report of methemoglobinemia in two patients with food protein-induced enterocolitis. Clinical pediatrics 2006; 45:679-82.
  9. Boyce JA, Assa'ad A, Burks AW, Jones SM, et al. (2010). "Guidelines for the diagnosis and management of food allergy in the United States: report of the NIAID-sponsored expert panel". J. Allergy Clin. Immunol. 126 (6 Suppl): S1–58. doi:10.1016/j.jaci.2010.10.007. PMC 4241964. PMID 21134576.
  10. Feuille E, Nowak-Węgrzyn A (2015). "Food Protein-Induced Enterocolitis Syndrome, Allergic Proctocolitis, and Enteropathy". Curr Allergy Asthma Rep. 15 (8): 50. doi:10.1007/s11882-015-0546-9. PMID 26174434.
  11. 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.
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