Introduction
Food protein-induced enterocolitis syndrome (FPIES) is a non
IgE-mediated food allergy. Its incidence is estimated between
0.015-0.7%,1–4 corresponding to 19-31% of food
allergies diagnosed in infants and children.3,5,6 In
the absence of biomarkers, the diagnosis of FPIES is based on clinical
presentation. International diagnosis criteria have recently been
proposed to improve its diagnosis.7 Acute FPIES is
defined by typical repetitive vomiting starting 1 to 4 hours after
ingestion of the culprit food, in association with at least 3 minor
criteria.7 These include some other episode of
repetitive vomiting after eating the same or different foods, lethargy,
pallor, the need for an emergency department visit or intravenous fluid
support; diarrhea, hypotension or hypothermia.7Chronic FPIES occurs when the food is regularly consumed, and is mainly
reported with cow’s milk (CM) and soy formula ingestion. The diagnosis
of chronic FPIES is based on the presence of intermittent emesis,
chronic diarrhea, poor weight gain or failure to thrive, which improve
after several days to weeks of exclusion of the offending food. After a
period of avoidance, acute typical symptoms occur upon
reexposure.8 Severe forms of acute FPIES may lead to
dehydration, and hypovolemic shock is reported in 15% to 33% of acute
FPIES cases.9–11 IgE sensitization to the culprit
food is unusual but may be observed in atypical
FPIES.7 Although oral food challenges (OFC) are not
necessary for diagnosis when the typical symptoms are present, they are
useful in doubtful cases to confirm the diagnosis.7The offending foods depend on geographic origins.8 The
most frequent culprit foods are CM in Europe and North
America,6,12–18 soya, rice and grains in North
America and Australia,2,4,10,17,19 and fish in
Mediterranean countries.13,20 Resolution of FPIES is
expected by school age in the majority of cases.8 OFCs
are performed to assess tolerance to the food in question, generally
12-18 months after the last reaction.7
In this study we aimed to describe FPIES in a large population of French
children, using international diagnosis criteria, and to describe its
natural history.7