Food Protein-Induced Enterocolitis Syndrome

From Practice to Theory

Stefano Miceli Sopo; Monica Greco; Serena Monaco; Salvatore Tripodi; Mauro Calvani

Expert Rev Clin Immunol. 2013;9(8):707-715.

Second Criterion: 'Exposure to the Incriminated Food Elicited Repetitive Vomiting and/or Diarrhea Within 4 h Without Any Other Cause for the Symptoms'

In 2012 Leonard et al. suggested that food ingestion has to be repetitive and that the elicited symptoms are generic gastrointestinal, not specifically vomiting and/or diarrhea. However, we do not agree with these changes. In fact, as it is stated in the fifth criterion (see below), at least two episodes are necessary to make the definitive diagnosis. Moreover, FPIES has to be suspected (not diagnosed) even if only a one episode occurred, so the need for repetitive ingestion specified by Leonard et al.^[1] could divert doctors' attention from a possible diagnosis of the syndrome. We would rather focus on symptom duration, which is usually short in time. In fact, patients take 6−8 h to recover and at the end of all, they appear as if nothing wrong has happened. This last one is a unique characteristic of FPIES, and it can not be found in other diseases, not IgE-mediated which could gloss over this syndrome as acute gastroenteritis but also surgical abdomen and sepsis. We would also highlight that vomiting is undoubtely the prevalent symptom accompanied by hyporeactivity and pallor, as opposed to diarrhea, which is a secondary and inconstant aspect of the syndrome. It would be inaccurate to make a FPIES diagnosis if the patient presents with diarrhea without vomiting, although the conjunctions 'and/or' do not seem to underline the difference.

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Abstract
Sometimes You Need to Be Lucky – Bridget's Story
Sometimes You Need to Be Lucky – Ernst's Story
From Practice to Theory
FPIES Definition and Diagnostic Criteria
First Criterion: 'Age Younger Than 9 Months at Initial Presentation'
Second Criterion: 'Exposure to the Incriminated Food Elicited Repetitive Vomiting and/or Diarrhea Within 4 h Without Any Other Cause for the Symptoms'
Third Criterion: 'Symptoms Limited to the GI Tract'
Fourth Criterion: 'Avoidance of the Offending Protein From the Diet Resulted in Resolution of Symptoms'
Fifth Criterion: 'A Standardized Food Challenge or Isolated Re-exposure Elicited the Typical Symptoms'
The Diagnostic Delay and the Increase of New Diagnoses: Is It a Contradiction?
Katie's Story: She Was Misunderstood
FPIES Differential Diagnosis
Most Frequent Culprit Foods
Diet Therapy, Single and Multiple
Victor's Story: Second Episode
FPIES Prognosis
Micol: The Chameleon FPIES
Acute Management of FPIES
At Last…FPIES Register
Expert Commentary & Five Year View
References
Sidebar

Table 1. Acute and chronic FPIES clinical manifestations.
Acute FPIES	Chronic FPIES
Important and repetitive vomiting (constant)	Regurgitation, small amounts of vomit
Lethargy (frequent)	Lethargy
Hyporeactivity (frequent)	Failure to thrive
Pallor (frequent)	Dehydration
Diarrhea (inconstant and tardive)	Diarrhea, sometimes bloody
Dehydration (rare)	Abdominal distention
Hypothermia (rare)	Hypotension (rare)

Table 2. Diagnostic criteria of FPIES.
Powell, 1986²	Leonard et al., 2012¹	Miceli Sopo et al., 2013
Less than 9 months of age at initial presentation (reaction)	Less than 9 months of age at initial diagnosis	Less than 2 years of age at first presentation (frequent feature but not mandatory)
Exposure to the incriminated food elicits repetitive vomiting and/or diarrhea within 4 h without any other cause for the symptoms	Repeated exposure to causative food elicits gastrointestinal symptoms without alternative cause	Exposure to the incriminated food elicits repetitive and important vomiting, pallor, hyporeactivity and lethargy within 2–4 h. Diarrhea may be present, much less frequently and later. The symptoms last a few hours, usually less than 6 h
Symptoms limited to the GI tract	Absence of symptoms that may suggest an IgE-mediated reaction	Absence of symptoms that may suggest an IgE-mediated reaction
Avoidance of the offending protein from the diet results in resolution of symptoms	Removal of causative food results in resolution of symptoms	Avoidance of the offending protein from the diet results in resolution of symptoms
A standardized food challenge or isolated re-exposure elicits the typical symptoms	Re-exposure or oral food challenge elicits typical symptoms within 4 h	Re-exposure or oral food challenge elicits typical symptoms within 2−4 h. Two typical episodes are needed to deliver the definitive diagnosis

Box 1. Clues in favor of a specific IgE role in food protein-induced enterocolitis syndrome pathogenesis.
There are some cases with positive specific IgEs against the culprit food, even if their levels are low. In one child,, during an episode of acute FPIES caused by rice, a plasmatic cytokinic trend similar to those of IgE-mediated allergic reactions was observed, with an increase of IL-4 and a decrease of IFN-γ [10]. The fact that symptoms arise 2 h after the ingestion of the culprit food is not a typical feature either of classical IgE-mediated forms (the reaction usually appears long before 2 h) or of classical non-IgE-mediated forms (which usually manifest long after 2 h). Few patients (with positive specific IgE research) present a shift in symptoms from those of FPIES to those of gastrointestinal anaphylaxis [11]. Regarding CM FPIES, there is a report about the capability of tolerating the culprit food if well-cooked (baked) [12].