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Ask the Expert - Food Protein-Induced Enterocolitis Syndrome (FPIES)

Q: A 6-month-old female presented to our clinic for evaluation of multiple episodes of recurrent vomiting, pallor and lethargy when exposed to solid foods... What is the likely diagnosis and what management is recommended?

She had been exclusively breast fed for the first 4 months of her life. At 2 months of age, she was found to have flakes of blood and somewhat loose stools. At that time, her mother removed all dairy from her own diet and the patient’s flakes of blood and loose stool resolved.

At 4 months of age, she was given rice cereal mixed with a cow’s milk based formula. This was her first exposure to either of these food groups. She tolerated small amounts of this mixture for several days but then developed a mild rash around her mouth and loose stool. She returned to exclusive breast feeding for one month.

At 5 months of age, she was given the same rice cereal but this time mixed with breast milk. About one hour after eating a few bites of this mixture, she developed multiple episodes of vomiting and became pale and lethargic. The vomiting resolved within about 30 minutes but the pallor and lethargy lasted for several hours. She then breast fed, slept for a few hours and was fine when she awoke. The family discussed the reaction with their primary care doctor who felt that the child likely had gastroenteritis and did not feel that there needed to be any changes in the child’s diet.

She was again exclusively breastfed until a few days prior to our evaluation. At that time, she was fed oatmeal cereal mixed with water. Two hours later, she developed multiple episodes of vomiting. She became lethargic and her parents noted some discoloration around her lips. Her parents also noticed that her heart was racing. The lethargy lasted for about 3 hours, at which time she recovered on her own. Again the symptoms were discussed with the primary doctor who told the family that the patient likely had gastroenteritis again. Secondary to the family’s concern that this was not infectious but possibly food related, the primary care doctor referred the child to our allergy clinic.

Her past medical history included only mild eczema controlled with intermittent moisturizers and over-the-counter hydrocortisone. She had no history of infections or respiratory problems. She had no hospitalizations. She had received all of her immunizations without any adverse reactions. She was born at the 20th percentile for weight which initially decreased to the 5th percentile but had been stable on the 5th percentile for the past several months. She did not have issues with chronic emesis or diarrhea. She did not have any known drug allergies. Her family history was significant for allergic rhinitis in both of her parents and several other relatives. There was no family history of food allergies.

On exam, she was a normal appearing child. She was in no distress. She was awake, alert, and developmentally appropriate. Her mucous membranes were moist and her fontanelle was open, soft, and flat. Her lungs were clear to auscultation bilaterally without wheezes, rhochi, or rales. She had no increased work of breathing. She had no hepatosplenomegaly or other abdominal masses. Her cardiovascular exam showed a regular rate and rhythm with no murmurs. She had good pulses and perfusion in all extremities. Her skin was clear without any rashes. She had no clubbing or cyanosis.

On skin prick testing to food allergens in our office, she had a positive skin prick test to egg. Tests to rice, milk, oat, and wheat were negative by skin prick test and by serum allergen specific IgE levels.

What is the diagnosis, and what management is recommended?

A. This patient’s symptoms are consistent with a severe non-IgE-mediated reaction, Food Protein Induced Enterocolitis Syndrome (FPIES). FPIES is a cell-mediated food hypersensitivity characterized by vomiting, diarrhea, and lethargy when exposed to food proteins...Read more

Though most people associate adverse food reactions with symptoms seen in an IgE mediated allergic reaction including urticaria, angioedema, wheezing, and low blood pressure, not all food hypersensitivity is mediated by this immune mechanism. This patient’s symptoms are consistent with a much less known but equally severe reaction, Food Protein Induced Enterocolitis Syndrome (FPIES). FPIES is a cell-mediated, non-IgE antibody associated food hypersensitivity characterized by vomiting, diarrhea, and lethargy when exposed to food proteins. It can progress to dehydration and shock requiring fluid resuscitation in up to 20% of patients. Patients can appear septic and often have a full septic work-up, including lumbar puncture, performed during their acute symptoms. They improve much quicker than a patient with a significant infection. In fact, they often return to baseline several hours after the offending agent had been removed from their diet and if dehydrated, had appropriate fluid resuscitation. The diagnosis of FPIES is frequently missed which can lead children with this disease to have unnecessary future reactions. FPIES is believed to be a T-cell mediated hypersensitivity and skin tests and specific blood IgE levels are frequently negative though there are some children with both an IgE and non-IgE mediated component to their disease.

In one study of 66 patients, the most common presenting symptom was vomiting (100%), followed by lethargy (85%), pallor (67%), and diarrhea (24%). Low body temperature (<36oC in 24%) and thrombocytosis (>500 x 109 in 64%) were also noted in this study.

FPIES usually presents at one of two points in a child’s life. Classically, FPIES symptoms first occur at 1-2 months of age in response to soy or cow’s milk formulas. A second peak occurs between 4 and 12 months upon the introduction of solid foods. The most commonly implicated foods are milk, soy, and grains but there is a long list of foods that have been implicated including eggs, peas, beans, legumes, squash, sweet potatoes, chicken and turkey.

FPIES differs from proctocolitis; another non-IgE mediated food hypersensitivity, in that there is rarely blood noted in the stool. During an acute reaction to a food, children with FPIES can have an elevated blood polymorphonuclear leukocyte count but eosinophilia, which is more often seen with proctocolitis or IgE mediated food allergy, is not common. It differs from dietary protein enteropathy in that it does not have chronic diarrhea or failure to thrive; the symptoms associated with FPIES are acute with exposure to the offending food and resolve relatively quickly once the food is removed from the diet and the necessary resuscitation had occurred.

At this time, management of FPIES involves restriction of possible triggering foods for the first few years of life. It appears that avoiding exposure to high risk foods until the child is 1-2 years old may increase the likelihood of being able to tolerate these foods as a preschooler. Tolerance to the triggering foods develops in most children by 3 years of age but can vary between children significantly. It is very important to have children with this diagnosis followed by a dietician to assist in what can be a restrictive diet.

FPIES is an important diagnosis for allergists, pediatricians and family practitioners to recognize. Failure to diagnose FPIES can lead to unnecessary additional investigations, including imaging, septic evaluations, and surgical consults. It also puts the child at risk for being exposed to high risk foods and possible future reactions. Most of the children with this diagnosis followed in our clinic have had reactions to at least two foods, and several had reacted to more than five foods, before there was any investigation of what was causing the reactions. One of the children we follow had reactions to eight different foods before being seen in our clinic! The fact that none of the symptoms were consistent with an IgE mediated response kept the primary care doctor from believing that food hypersensitivity was the diagnosis. Another child had more than one septic work up, including lumbar puncture, before the association between food exposure and shock was noted. Most health care providers know symptoms consistent with an IgE mediated reaction but it is also important that symptoms related other forms of food hypersensitivity such as FPIES are recognized early.

 
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