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Food protein-induced enterocolitis syndrome

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Food protein-induced enterocolitis syndrome (FPIES) is a systemic, non IgE-mediated response to a specific trigger within food, most likely food protein. FPIES may present either as an acute or chronic form. In its acute form, FPIES presents with vomiting that typically begins 1 to 4 hours after trigger food ingestion, alongside paleness of the skin, lethargy, and potentially blood-tinged diarrhea. In the severe form of acute FPIES, continued vomiting may cause severe dehydration or hypotensive shock-like state, requiring hospitalization. In its chronic form, chronic exposure to trigger foods results in chronic or episodic vomiting, poor weight gain, failure to thrive, and watery or blood-tinged diarrhea[1]. FPIES can potentially develop at any age, from infancy to adulthood, but most commonly develops within the first few years of life and resolves in early childhood[1][2][3][4]. Atypical FPIES presents with evidence of specific IgE-sensitization via positive specific serum or skin IgE testing to trigger foods; atypical FPIES may prolong time to disease resolution or increase risk of conversion to IgE-mediated food allergy.[5]

Epidemiology

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To date, various studies have estimated FPIES incidence to be between 0.015% and 0.7%. However, establishing the true prevalence of FPIES has been hindered by the scarcity of population-level epidemiological studies, the relatively recent establishment of uniform diagnostic criteria (established in 2017), and under-diagnosis due to disease rarity and lack of awareness.[6] A 2019 United States population-level survey estimated a FPIES prevalence of 0.51% in children and 0.22% in adults.[7] Similar prevalences in children have been found in population-level estimates from Israeli and Spanish studies.[8]

The underlying pathophysiology of FPIES is not understood at this time, though it is generally understood to be non-IgE mediated. One study found that in patients with non-IgE mediated food allergy, Th2 lymphoproliferative responses were similar to that of patients with IgE-mediated allergies, suggesting an underlying T-cell mechanism of action.[9] Another study found elevated IL-17 markers, elevated innate inflammatory markers, and increased T-cell activation after FPIES reaction.[10]

Signs and symptoms

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In the severe form, symptoms include abdominal pain, profuse vomiting, lethargy, potentially diarrhea, and even shock.[1][2][3] Additional symptoms could potentially include – but are not limited to – headache, pallor, lethargy, constipation, and abdominal swelling (distension).[11][4] 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.[12] 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.[13]

Diagnosis

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Diagnosis is primarily based on history as specific IgE and skin prick tests are typically negative[13] 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.[14]

Treatment

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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] 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.[15] 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.[16]

References

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  1. ^ a b c d 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.
  2. ^ a b c 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. ^ a b c 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. ^ a b 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.
  5. ^ Anvari, Sara; Ruffner, Melanie A.; Nowak-Wegrzyn, Anna (April 2024). "Current and future perspectives on the consensus guideline for food protein-induced enterocolitis syndrome (FPIES)". Allergology International. 73 (2): 188–195. doi:10.1016/j.alit.2024.01.006. PMID 38326194.
  6. ^ Anvari, Sara; Ruffner, Melanie A.; Nowak-Wegrzyn, Anna (April 2024). "Current and future perspectives on the consensus guideline for food protein-induced enterocolitis syndrome (FPIES)". Allergology International. 73 (2): 188–195. doi:10.1016/j.alit.2024.01.006. PMID 38326194.
  7. ^ Nowak-Wegrzyn, Anna; Warren, Christopher M.; Brown-Whitehorn, Terri; Cianferoni, Antonella; Schultz-Matney, Fallon; Gupta, Ruchi S. (October 2019). "Food protein–induced enterocolitis syndrome in the US population–based study". Journal of Allergy and Clinical Immunology. 144 (4): 1128–1130. doi:10.1016/j.jaci.2019.06.032. PMC 7923683. PMID 31288044.
  8. ^ Katz, Yitzhak; Goldberg, Michael R.; Rajuan, Nelly; Cohen, Adi; Leshno, Moshe (March 2011). "The prevalence and natural course of food protein–induced enterocolitis syndrome to cow's milk: A large-scale, prospective population-based study". Journal of Allergy and Clinical Immunology. 127 (3): 647–653.e3. doi:10.1016/j.jaci.2010.12.1105. PMID 21377033.
  9. ^ Morita, Hideaki; Nomura, Ichiro; Orihara, Kanami; Yoshida, Koichi; Akasawa, Akira; Tachimoto, Hiroshi; Ohtsuka, Yoshikazu; Namai, Yoshiyuki; Futamura, Masaki; Shoda, Tetsuo; Matsuda, Akio; Kamemura, Norio; Kido, Hiroshi; Takahashi, Takao; Ohya, Yukihiro (February 2013). "Antigen-specific T-cell responses in patients with non–IgE-mediated gastrointestinal food allergy are predominantly skewed to TH2". Journal of Allergy and Clinical Immunology. 131 (2): 590–592.e6. doi:10.1016/j.jaci.2012.09.005. PMID 23083674.
  10. ^ Berin, M. Cecilia; Lozano-Ojalvo, Daniel; Agashe, Charuta; Baker, Mary Grace; Bird, J. Andrew; Nowak-Wegrzyn, Anna (September 2021). "Acute FPIES reactions are associated with an IL-17 inflammatory signature". Journal of Allergy and Clinical Immunology. 148 (3): 895–901.e6. doi:10.1016/j.jaci.2021.04.012. PMC 8675150. PMID 33891982.
  11. ^ "Food Protein-Induced Enterocolitis Syndrome". National Organization for Rare Disorders (NORD). Archived from the original on 19 February 2017.
  12. ^ Anand RK, Appachi E. Case report of methemoglobinemia in two patients with food protein-induced enterocolitis. Clinical pediatrics 2006; 45:679-82.
  13. ^ a b 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.
  14. ^ 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.
  15. ^ 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
  16. ^ 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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