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. In the severe form, patients will vomit until dehydration and until a shock-like state, which occurs in 15% of patients. 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. Additional symptoms include headache, pallor, lethargy, constipation, and abdominal swelling (distension). Laboratory studies during the acute episode shows an elevated white blood cell count with a left shift and elevated platelet count and methemoglobinemia. 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.
Diagnosis is primarily based on history as specific IgE and skin prick tests are typically negative 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.
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). There are also cases of FPIES being transmitted through foods in breast milk in rare occasions.
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