Delayed presentation of food protein-induced enterocolitis syndrome (FPIES) to okra in a toddler

While FPIES reactions can theoretically be caused by any food, cow’s milk is known to be the most prominent trigger [2]. A large 2013 North American study discovered a 67% incidence rate of cow’s milk allergy amongst patients with FPIES [10]. Within the same study, a combination of numerous grains was found to be the second most prevalent at 57%, followed by 41% of FPIES allergies triggered by soy [10]. Fruits also have been known to trigger FPIES, such as bananas, apples, and strawberries, but they are uncommon. Less than 10% of cases reported in the American study have been linked to fruits [6, 10, 11]. While FPIES triggers can vary in frequency depending on the region of study and the participants observed, fruits are typically dwarfed by more dominant triggers such as cow’s milk in the United States and fish in European countries like Greece, Spain, and Italy [11]. To our knowledge, okra has never been formally described as a trigger of FPIES, but as a fruit it should be given clinical consideration as a culprit food if delayed symptoms of vomiting, lethargy, diarrhea,, and in severe cases, hypotension and hypothermia follow its ingestion [3, 8, 12]. The inclusion of okra as a recognized potential FPIES trigger can help reduce the risk of developing chronic FPIES and accelerate the proper diagnosis and appropriate treatment to limit complications associated with dehydration.

While many triggering foods of FPIES have been described, knowledge about the natural history of FPIES remains incomplete. Most FPIES reactions have been found to occur after the first few introductions of the offending food, with studies reporting that 61% to 75% of FPIES reactions occur after the first 2–3 consumptions of the allergen, while others generically report reactions within the first month of introduction to the food trigger [7, 11, 13]. Most current literature discussing FPIES do not focus specifically on the initial presentation of a reaction and lack clarity around the circumstances of the first presentation. In this case, the patient tolerated okra almost daily for a year until she experienced her first FPIES reaction. This notable deviation from the widely-held view of FPIES natural history suggests a need to more broadly consider the disease in patients who once tolerated the food. Due to the variability of FPIES and its prognosis, all foods that elicit characteristic symptoms of the syndrome should be considered as potential triggers attributable to FPIES to reduce potential delays in treatment and management of reactions. It should also be mentioned that different cultures and geographic regions have distinct practices for food introduction [15]. Thus, unique food triggers may reflect regional practices of early food introduction. Overall, FPIES has a good outcome with many studies reporting a 60% rate of resolution for any food [16].

Treatment of FPIES involves removing the known allergen from the diet to avoid subsequent reactions [8]. If ingestion of the food were to occur and evoke a mild reaction of 1–2 episodes of vomiting, parents should offer oral fluids for rehydration [6]. For severe reactions with more than three episodes, lethargy, and features of moderate to severe dehydration, patients should seek medical intervention including intravenous fluids for rehydration [6, 13]. Serotonin release in the gastrointestinal tract in response to the activation of the purinergic pathway offers a possible explanation for the vomiting observed in patients with FPIES. Furthermore, Ondansetron, a serotonin receptor antagonist, has proven effective in the treatment of FPIES-induced vomiting. [17] Oral ondansetron can be offered for mild cases managed at home, with a repeat dose offered if vomiting occurs within ten minutes of the initial dose. IM and IV ondansetron are reserved for severe cases managed in the hospital setting. [6, 13]. Oral food challenges according to published guidelines, may be attempted 12–18 months after the last reaction to determine whether tolerance to the trigger food has developed and FPIES has resolved [13, 14]. Patient education and support are critical to early recognition and prompt management of FPIES to prevent the development of complications related to dehydration in affected patients.

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