Food protein-induced enterocolitis syndrome (FPIES) is a systemic, non IgE-mediated response to a specific trigger within food – most likely food protein. FPIES presents in two different forms: an acute form and a chronic form. In its acute form, FPIES presents with vomiting that usually begins 1 to 4 hours after trigger food ingestion (can be 30 minutes to 6 or more hours). Vomiting is often followed by a paleness to the skin, lethargy, and potentially watery, perhaps blood-tinged diarrhea. In the severe form of acute FPIES, a person will vomit until dehydration and until a shock-like state, which occurs in 15% of patients.[1] In its chronic form, which can be difficult to diagnose until a person has already met diagnostic criteria for acute FPIES, after repeated or regular ingestion of the trigger food, the person presents with chronic or episodic vomiting, failure to thrive, and watery, perhaps blood-tinged diarrhea. FPIES can potentially develop at any age but seems most commonly to develop within the first few years of life.[2][3][4][5][6] FPIES has mainly been documented in young infants, but can exist in older children and adults. Some people develop both FPIES and an IgE-mediated type of reaction to the same food, and having FPIES can increase a person's risk of also developing IgE-mediated food allergies.[7]

Signs and symptoms

In the severe form, symptoms include abdominal pain, profuse vomiting, lethargy, potentially diarrhea, and even shock.[2][3][4] Additional symptoms could potentially include – but are not limited to – headache, pallor, lethargy, constipation, and abdominal swelling (distension).[8][6] Laboratory studies might reveal hypoalbuminemia, anemia, eosinophilia, and an elevated white blood cell count with a left shift. Over half of patients experiencing an acute FPIES reaction may develop thrombocytosis (platelets >500x109/L). In both chronic and acute FPIES, both methemoglobinemia and metabolic acidosis (mean pH around 7.03 in one study) have been reported.[9] Endoscopy may reveal a gastric erythema, edema, mucosal friability, and gastric antral erosions. The exact mechanism is unclear, but it is hypothesized to be a T cell driven disorder. Upon re-exposure to the offending food after a period of elimination, a subacute syndrome can present with repetitive emesis and dehydration.[10]

Diagnosis

Diagnosis is primarily based on history as specific IgE and skin prick tests are typically negative[10] and the exclusion of other disorders that present similar clinical features, such as infectious gastroenteritis, celiac disease, inflammatory bowel disease, necrotizing enterocolitis, food protein-induced enteropathy, food protein-induced proctocolitis, and eosinophilic gastroenteritis, among others.[11]

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).[2][3][4] The list of potential food triggers is varied and can be somewhat region specific. There are also cases of FPIES being transmitted through foods in breast milk in rare occasions.[12] During an acute FPIES episode, ondansetron or infacol may be used to control symptoms in children over 6 months of age. Many breastfeeding mothers either eliminate the food from their diet although this is not always necessary or switch to an extensively hydrolyzed or elemental formula if there is a concern about cow's milk being an offending culprit. Some children tolerate soy based formulas if they have FPIES to cow's milk but many do not. Most infants diagnosed with FPIES outgrow it by the time they reach school age or sometime within their school-aged years.[13]

References

  1. ↑ Nowak-Wegrzyn A, Muraro A. Food protein-induced enterocolitis syndrome. Current Opinion in Allergy and Clinical Immunology 2009; 9:371-7. PMID: 19474706
  2. 1 2 3 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. hdl:10044/1/48017. PMID 28167094.
  3. 1 2 3 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.
  4. 1 2 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.
  5. ↑ 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. S2CID 17465758.
  6. 1 2 Ruffner, MA (November 2014). "Food Protein-Induced Enterocolitis Syndrome: Insights From Review of a Large Referral Population" (PDF). Pediatrics. 134: S157. doi:10.1542/peds.2014-1817PP. PMID 25363948. S2CID 46053309. Archived from the original (PDF) on 19 July 2018.
  7. ↑ Nowak-Węgrzyn, Anna; Chehade, Mirna; Groetch, Marion E.; Spergel, Jonathan M.; Wood, Robert A.; Allen, Katrina; Atkins, Dan; Bahna, Sami; Barad, Ashis V.; Berin, Cecilia; Whitehorn, Terri Brown (2017-04-01). "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". Journal of Allergy and Clinical Immunology. 139 (4): 1111–1126.e4. doi:10.1016/j.jaci.2016.12.966. hdl:10044/1/48017. ISSN 0091-6749. PMID 28167094.
  8. ↑ "Food Protein-Induced Enterocolitis Syndrome". National Organization for Rare Disorders (NORD). Archived from the original on 19 February 2017.
  9. ↑ Anand RK, Appachi E. Case report of methemoglobinemia in two patients with food protein-induced enterocolitis. Clinical pediatrics 2006; 45:679-82.
  10. 1 2 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.
  11. ↑ 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. S2CID 6651513.
  12. ↑ 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. PMID: 21146866
  13. ↑ Cherian S, Varshney P (April 2018). "Food Protein-Induced Enterocolitis Syndrome (FPIES): Review of Recent Guidelines". Curr Allergy Asthma Rep. 18 (4): 28. doi:10.1007/s11882-018-0767-9. PMID 29623454. S2CID 4705873.
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