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.

Sometimes You Need to Be Lucky – Ernst's Story

Since his birth, Ernst was exclusively breast-fed; he started weaning when he was five months old. At 7 months he had already eaten cooked codfish twice, without showing any clinical problem. One day, 2 h after the ingestion of 50 g of steamed sole, Ernst started to vomit repetitively, he became hypotonic, hyporeactive and shortly after he started to suffer from diarrhea. Five hours later the baby recovered completely. After some time, Ernst's aunt, who was a doctor, suggested his parents give him fish again and consequently Ernst ate a homogenized sole baby food. After 4 h symptoms arose again and they were even more serious compared to those previously reported. When the baby was ten months old his parents decided to try again, giving Ernst a sea bream homogenized baby food, and unfortunately the baby fell sick showing all the symptoms mentioned before except diarrhea. As the third attempt failed as well, Ernst's parents brought their child to an allergist. SPTs performed with the culprit food (sole and sea bream) were negative and FPIES was diagnosed without needing to perform any OFC. The allergist recommended that Ernst keep eating everything he ate before (he had already eaten milk, legumes, cereals and poultry, without any problems) except fish, molluscs and crustaceans. Nevertheless, the allergist pointed out that the chances were that the baby was not allergic to every sea creature. Therefore, it could be possible to perform other OFCs with molluscs and crustaceans, but also with codfish, swordfish and tuna, since there was a discrete possibility of passing him without any adverse reaction.

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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].