Table 1.

Diagnostic criteria for Food Protein-Induced Enterocolitis Syndrome (FPIES)

Acute FPIES Diagnostic Criteria The diagnosis of FPIES requires that the patient meet the major criterion and at least 3 minor criteria. If only a single episode has occurred, a diagnostic OFC should be strongly considered to confirm the diagnosis, especially because viral gastroenteritis is so common in this age group. Furthermore, although not a criterion for diagnosis, it is important to recognize that acute FPIES reactions will typically completely resolve over a matter of hours compared with the usual several-day time course of gastroenteritis. The patient should be asymptomatic and growing normally when the offending food is eliminated from the diet. Major criterion: Vomiting in the 1- to 4 hours after ingestion of the suspect food and the absence of classic IgE-mediated allergic skin or respiratory symptoms. Minor criteria: 1.A second (or more) episode of repetitive vomiting after eating the same suspect food 2.Repetitive vomiting episode after 1–4 hours after eating a different food 3.Extreme lethargy with any suspected reaction 4.Marked pallor with any suspected reaction 5.Need for Emergency Departed visit with any suspected reaction 6.Need for intravenous fluid administration with any suspected reaction 7.Diarrhea within 24 hours (usually 5–10 hours) 8.Hypotension 9.Hypothermia

Chronic FPIES Diagnostic Criteria Severe presentation: when the offending food is ingested on a regular basis (e.g., infant formula); intermittent but progressive vomiting and diarrhea (occasionally with blood) develop, sometimes with dehydration and metabolic acidosis. Milder presentation: lower doses of the problem food (e.g., solids food or food allergens in breast milk) lead to intermittent vomiting and/or diarrhea, usually with poor weight gain/failure to thrive but without dehydration or metabolic acidosis. The most important criterion for chronic FPIES diagnosis is resolution of the symptoms within days after elimination of the offending food(s) and acute recurrence of symptoms when the food is reintroduced, onset of vomiting in 1–4 hours, diarrhea within 24 hours (usually 5–10 hours). Without confirmatory OFC, the diagnosis of chronic FPIES remains presumptive.

Diagnostic criteria for the interpretation of OFCs Major criterion: Vomiting in the 1- to 4 hours period after ingestion of the suspect foods and the absence of classic IgE-mediated allergic skin or respiratory symptoms. Minor criteria: 1.Lethargy 2.Pallor 3.Diarrhea within 5–10 hours after food ingestion 4.Hypotension 5.Hypothermia 6.Increased neutrophil count of ≥1500 neutrophils above the baseline count.

The OFC is considered diagnostic of FPIES, i.e., positive, if the major criterion is met with at least 2 minor criteria. However, two important remarks need to be considered: (1) With the rapid use of ondansetron, many of the minor criteria, such as repetitive vomiting, pallor, and lethargy may be averted; and (2) Not all facilities performing challenges have the ability to perform neutrophil counts in a timely manner

Abbreviations: OFC Oral Food Challenge, FPIES Food Protein-Induced Enterocolitis Syndrome