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.

Victor's Story: Second Episode

Victor is part of the abovementioned 2% patients suffering from multiple FPIES in our casistic; his story was not ended. When he was six months old he ate corn at home and at the second administration he showed his fourth FPIES episode. After a time, an OFC was performed in DH with lentils, potato and semolina wheat and Victor presented his fifth episode caused by wheat. At home, his mother gave him chickpeas and Victor had his sixth adverse reaction. In summary, if we had followed Sicherer's suggestions^[17] this baby would not have shown three of his six FPIES episodes. However, as we have already said, the probability of encountering a child affected by multiple FPIES is quite low and considering our 55 clinical cases, Victor is the only one. So, we struck a compromise that somehow takes into account the suggestion of Sicherer.^[17] In fact, we suggest patients perform a DH OFC with a mix of cereals, legumes and poultry, since the probability that the child tolerates such foods is very high. In this way we avoid an enlarged diet in children who actually do not need it. This was, for example, the advice given to Bridget. We did not propose the same management to Ernst as, when he came to our attention, he had already eaten these foodstuffs without any problem. Moreover, especially for CM FPIES, in case of lack of breast-milk, there was a strong recommendation to give an extensively hydrolyzed casein formula instead of cow milk and to avoid soy milk ingestion. Nowadays, this prescription is declining and this is mainly due to the publication by Katz et al.,^[4] which reported that the majority of 43 children suffering from CM FPIES tolerated soy milk. In conclusion, it is certain that the culprit food has to be excluded from patient's diet until he spontaneously reaches tolerance, but it is not certain that further dietary restrictions are needed. So, in an attempt to improve the quality of life of the child with FPIES, we perform OFCs with a mixture of other foods hypothetically involved, without deleting them from the diet prejudicially. In the case of fish FPIES, we suggest OFCs with other types of fish, while in case of CM or hen's egg FPIES we propose challenges with the baked food. Of course, ours is a working hypothesis and studies are in progress to demonstrate its validity.

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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].