Manifestations of Food Protein-Induced Enterocolitis Syndrome
Manifestations of Food Protein-Induced Enterocolitis Syndrome
The first episode of FPIES usually occurs suddenly and progresses rapidly in an alarming manner. Symptoms occur when the causative food is ingested intermittently, which often facilitates identification of the offending food. The typical course of an acute event is persistent projectile vomiting, pallor, followed by diarrhea that can lead to lethargy and dehydration. Resolution of symptoms usually occurs within 24–48 h. The patient appears normal between exposures and returns to baseline once the offending food is eliminated.
Vomiting is by far the most prominent symptom, being reported in more than 95% of the cases. It usually occurs 0.5–6 h postingestion (average 2 h) and is characterized by frequent projectile episodes, every 10–15 min, and can reach more than 20 episodes in some cases. Lethargy and pallor have been reported in 40–100% of cases. However, in a retrospective study based on review of medical records, out of 462 pediatric cases, only 5% were documented to have lethargy and pallor. Loose or watery diarrhea occurs in 20–50% of patients, usually about 6 h after ingestion of the causative food, but can be more delayed up to 16 h. Bloody diarrhea has been reported in 4–11% of cases, but as high as 45% in one study.
Very severe symptoms can occur, ranging from 5 to 24% of cases, as intractable protracted vomiting, lethargy, pallor, hypotension, dehydration, and hypothermia with temperature less than 36°. Such a presentation is often misdiagnosed as IgE-mediated anaphylaxis. Recovery occurs with prompt management, primarily by intravenous fluids, though complete clinical resolution may take 2–3 days. Acute symptoms recur on exposure to the causative food.
We encountered an infant who had three acute FPIES episodes: the first was at 5 months of age, the symptoms occurred 30 min after chewing on a cellophane wrapper; the second was after ingestion of a tablespoon of pureed sweet potato; and the third was after a few sips of rice cereal mixed with breast milk. It was realized that in the first episode, the wrapper was covering a rice cake. This case was peculiar in that it occurred in an exclusively breastfed infant and by noningestant oral contact with a trivial quantity of rice allergen.
Although milk and some other common foods are the most implicated, some cases have been caused by less allergenic foods. Recently, an infant was reported of having multiple episodes of FPIES to boiled egg yolk, which is known to be much less allergenic than egg white. However, contamination with the traces of egg white protein could not be ruled out.
We recently saw an 11-month-old female infant with a history of eczema who was exclusively breastfed until 6 weeks of age. Excessive fussiness, irritability, and intermittent diarrhea with blood and mucus were reported few days after supplementation with cow's milk formula. She continued to have symptoms after switching to soybean formula. At 4 months, feeding extensively hydrolyzed casein formula (Nutramigen, Mead Johnson) resulted in marked improvement in eczema and gastrointestinal symptoms. By 6 months of age, rice cereal, pureed mixed vegetables, and fruits were tolerated. One month later, the patient began to have intermittent episodes of vomiting and diarrhea with blood and mucus. All solid foods were eliminated from the infant's diet for 3 weeks and symptoms resolved. Although exclusively on Nutramigen, the infant ate 1/4 cup of cooked steamed rice and after 30 min developed explosive vomiting and lethargy. All solid foods were eliminated and symptoms resolved within 1–2 days. An upper gastrointestinal series showed significant gastroesophageal reflux, and endoscopy showed healthy mucosa with normal biopsy findings. Because of poor weight gain, caloric intake was increased and the patient was switched to amino acid formula (Neocate, Nutricia). During our evaluation, the medical history revealed rice and green peas as the most suspected and were eliminated. Skin prick testing was significantly positive to cow's milk, egg white, egg yolk, and peanut, but negative to green peas. Total IgE was 16 IU/ml and specific IgE to egg white was 4.12 KU/l (class 3). We recommended strict elimination trial of cow's milk, egg, and peanut as a probable cause of eczema. This case emphasizes a couple of points. First, FPIES can present with mild, chronic symptoms that can resolve with the feeding of a hypoallergenic formula. Second, in FPIES patients with associated atopy, positive allergy tests (skin prick or specific IgE) are more likely relevant to the atopic disease rather than to FPIES.
Acute Food-Protein Induced Enterocolitis Syndrome
The first episode of FPIES usually occurs suddenly and progresses rapidly in an alarming manner. Symptoms occur when the causative food is ingested intermittently, which often facilitates identification of the offending food. The typical course of an acute event is persistent projectile vomiting, pallor, followed by diarrhea that can lead to lethargy and dehydration. Resolution of symptoms usually occurs within 24–48 h. The patient appears normal between exposures and returns to baseline once the offending food is eliminated.
Vomiting is by far the most prominent symptom, being reported in more than 95% of the cases. It usually occurs 0.5–6 h postingestion (average 2 h) and is characterized by frequent projectile episodes, every 10–15 min, and can reach more than 20 episodes in some cases. Lethargy and pallor have been reported in 40–100% of cases. However, in a retrospective study based on review of medical records, out of 462 pediatric cases, only 5% were documented to have lethargy and pallor. Loose or watery diarrhea occurs in 20–50% of patients, usually about 6 h after ingestion of the causative food, but can be more delayed up to 16 h. Bloody diarrhea has been reported in 4–11% of cases, but as high as 45% in one study.
Very severe symptoms can occur, ranging from 5 to 24% of cases, as intractable protracted vomiting, lethargy, pallor, hypotension, dehydration, and hypothermia with temperature less than 36°. Such a presentation is often misdiagnosed as IgE-mediated anaphylaxis. Recovery occurs with prompt management, primarily by intravenous fluids, though complete clinical resolution may take 2–3 days. Acute symptoms recur on exposure to the causative food.
Selected Cases
We encountered an infant who had three acute FPIES episodes: the first was at 5 months of age, the symptoms occurred 30 min after chewing on a cellophane wrapper; the second was after ingestion of a tablespoon of pureed sweet potato; and the third was after a few sips of rice cereal mixed with breast milk. It was realized that in the first episode, the wrapper was covering a rice cake. This case was peculiar in that it occurred in an exclusively breastfed infant and by noningestant oral contact with a trivial quantity of rice allergen.
Although milk and some other common foods are the most implicated, some cases have been caused by less allergenic foods. Recently, an infant was reported of having multiple episodes of FPIES to boiled egg yolk, which is known to be much less allergenic than egg white. However, contamination with the traces of egg white protein could not be ruled out.
We recently saw an 11-month-old female infant with a history of eczema who was exclusively breastfed until 6 weeks of age. Excessive fussiness, irritability, and intermittent diarrhea with blood and mucus were reported few days after supplementation with cow's milk formula. She continued to have symptoms after switching to soybean formula. At 4 months, feeding extensively hydrolyzed casein formula (Nutramigen, Mead Johnson) resulted in marked improvement in eczema and gastrointestinal symptoms. By 6 months of age, rice cereal, pureed mixed vegetables, and fruits were tolerated. One month later, the patient began to have intermittent episodes of vomiting and diarrhea with blood and mucus. All solid foods were eliminated from the infant's diet for 3 weeks and symptoms resolved. Although exclusively on Nutramigen, the infant ate 1/4 cup of cooked steamed rice and after 30 min developed explosive vomiting and lethargy. All solid foods were eliminated and symptoms resolved within 1–2 days. An upper gastrointestinal series showed significant gastroesophageal reflux, and endoscopy showed healthy mucosa with normal biopsy findings. Because of poor weight gain, caloric intake was increased and the patient was switched to amino acid formula (Neocate, Nutricia). During our evaluation, the medical history revealed rice and green peas as the most suspected and were eliminated. Skin prick testing was significantly positive to cow's milk, egg white, egg yolk, and peanut, but negative to green peas. Total IgE was 16 IU/ml and specific IgE to egg white was 4.12 KU/l (class 3). We recommended strict elimination trial of cow's milk, egg, and peanut as a probable cause of eczema. This case emphasizes a couple of points. First, FPIES can present with mild, chronic symptoms that can resolve with the feeding of a hypoallergenic formula. Second, in FPIES patients with associated atopy, positive allergy tests (skin prick or specific IgE) are more likely relevant to the atopic disease rather than to FPIES.
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