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jj Diaz, B Espin, O Segarra; Gastrointestinal Allergy Working Group of the Spanish Society of Pediatric Gastroenterology, Hepatology and Nutrition (SEGHNP). J Pediatr Gastroenterol Nutr. 2019;68(2):232–236 To describe the clinical presentation and management practices of food protein-induced enterocolitis syndrome (FPIES). The study included 120 patients (0.5 months to 12 years of age) diagnosed with FPIES in 2017 across 37 hospitals in Spain. This study involved a retrospective chart review of patients diagnosed with FPIES using the 2017 international consensus criteria. Age, clinical symptoms, implicated foods, oral food challenge, laboratory values, skin prick testing, number of episodes before diagnosis, and management approach were collected and analyzed …

KY Wang, J Lee, A Cianferoni. J Allergy Clin Immunol Pract. 2019;7(2):444–450 To describe experience with oral food challenges (OFCs) for food protein-induced enterocolitis syndrome (FPIES), using a protocol that includes ingestion of one-third of the goal food serving size with 4 hours of observation, followed by home titration to goal dose. A total of 119 subjects underwent 169 OFCs at Children’s Hospital of Philadelphia to 19 different foods between 2014 and 2017. Patients with food protein-induced allergic proctocolitis were excluded. Patients with intermittent food exposures and delayed emesis within 1 to 4 hours and/or diarrhea within 5 to 10 hours were classified …

S Mehr, K Frith, EH Barnes, DE Campbell. J Allergy Clin Immunol. 2017;140(5):1323–1330 To prospectively evaluate the incidence of acute food protein-induced enterocolitis syndrome (FPIES) in Australian children and to identify the clinical characteristics of infants with FPIES. Acute FPIES cases (based on a standardized previously published definition, details of which are found and referenced in the article) were reported by using monthly surveillance reports emailed to pediatric practitioners. Anonymous detailed questionnaires were completed for new suspect cases, and follow-up with reporting clinicians was undertaken to confirm the case definition and to complete missing data. The final inclusion criteria were met by 235 children

Hereditary fructose intolerance (HFI) symptoms develop at first introduction of fruit during weaning. We report on an infant with suspected HFI who presented with repeated episodes of vomiting and hypotension after ingestion of fruit-containing meals. The first episode occurred at age 4 months. Despite negative genetic testing for HFI, strict avoidance of fruit ingestion resulted in lack of recurrence of symptoms. Oral-fructose-tolerance testing conducted with an apple mousse did not determine hypoglycemia or fructosuria but caused severe hypotension. Allergy evaluations were negative, and the history was diagnostic for fruit-induced food protein–induced enterocolitis syndrome. Because this non-immunoglobulin E–mediated gastrointestinal food hypersensitivity manifests as profuse, repetitive vomiting, often with diarrhea, leading to acute dehydration and lethargy, it may be misinterpreted as HFI. We advise pediatricians to consider food protein–induced enterocolitis syndrome in the differential diagnosis when there is a suspicion of HFI.

S Miceli Sopo, G Bersani, S Monaco. Allergy. 2017;72(4):545–551 This study looked at the effectiveness of parenteral ondansetron in resolving acute symptoms of food protein–induced enterocolitis syndrome (FPIES). This study examined patients aged 4 months to 14 years with a positive oral food challenge (OFC) for FPIES. This was a retrospective case series of OFCs done to either definitively diagnose FPIES or to assess for the resolution of FPIES. …

R Goswami, AB Blazquez, R Kosoy, A Rahman, A Nowak-Wʁgrzyn, MC Berin. J Allergy Clin Immunol. 2017;139(6):1885–1896.e9 Food protein-induced enterocolitis syndrome (FPIES) is a non-immunoglobulin E–mediated food allergy in infants, and its pathophysiology is poorly understood. The investigators characterized allergen-responsive cells in the peripheral blood of subjects undergoing supervised food challenge for FPIES. Patients 1 to 21 years of age previously diagnosed with FPIES underwent oral food challenge (OFC) in a clinical research unit to determine resolution. During OFC, the challenge food was administered in 3 equal portions over 30 minutes, and OFC results were deemed positive if emesis and/or diarrhea occurred in concert with an …

Objective. The goal was to examine the demographic characteristics, causative foods, clinical features, treatments, and outcomes for children presenting with acute food protein-induced enterocolitis syndrome. Methods. This was a retrospective study of children with food protein-induced enterocolitis syndrome who presented to the Children's Hospital at Westmead (Sydney, Australia) over 16 years. Results. Thirty-five children experienced 66 episodes of food protein-induced enterocolitis syndrome. The mean age at initial presentation was 5.5 months. Children frequently experienced multiple episodes before a correct diagnosis was made. Twenty-nine children reacted to 1 food, and 6 reacted to 2 foods. Causative foods for the 35 children were rice (n = 14), soy (n = 12), cow's milk (n = 7), vegetables and fruits (n = 3), meats (n = 2), oats (n = 2), and fish (n = 1). In the 66 episodes, vomiting was the most common clinical feature (100%), followed by lethargy (85%), pallor (67%), and diarrhea (24%). A temperature of 500 × 109 cells per L was recorded for 63% of episodes with blood count results. Only 2 of the 19 children who presented to an emergency department with their initial reactions were discharged with correct diagnoses. Additional investigations of food protein-induced enterocolitis syndrome episodes presenting to the hospital were common, with 34% of patients undergoing abdominal imaging, 28% undergoing a septic evaluation, and 22% having a surgical consultation. Prognosis was good, with high rates of resolution for the 2 most common food triggers (ie, rice and soy) by 3 years of age. Conclusions. Misdiagnosis and delays in diagnosis for children with food protein-induced enterocolitis syndrome were common, leading many children to undergo unnecessary, often painful investigations. Decreased body temperature and thrombocytosis emerge as additional features of the syndrome.

SM Sopo, A Battista, M Greco, S Monaco. Int Arch Allergy Immunol. 2014;64(2):137–139 Previous reports suggest that ondansetron can be beneficial to hasten resolution of acute food protein–induced enterocolitis syndrome (FPIES) reactions. This study describes additional cases of successful treatment of acute FPIES reactions with intramuscular ondansetron in pediatric patients. Five children aged 13 months to 4 years with a known diagnosis of …

Background. Infantile food protein-induced enterocolitis syndrome (FPIES) is a severe, cell-mediated gastrointestinal food hypersensitivity typically provoked by cow’s milk or soy. Solid foods are rarely considered a cause.Objective. To describe the clinical characteristics and natural history of FPIES provoked by solid foods.Methods. Patients with FPIES induced by solid foods were identified and their clinical course compared with a control group with FPIES caused by cow’s milk and/or soy evaluated over the same time period.Results. Fourteen infants with FPIES caused by grains (rice, oat, and barley), vegetables (sweet potato, squash, string beans, peas), or poultry (chicken and turkey) were identified. Symptoms were typical of classical FPIES with delayed (median: 2 hours) onset of vomiting, diarrhea, and lethargy/dehydration. Eleven infants (78%) reacted to >1 food protein, including 7 (50%) that reacted to >1 grain. Nine (64%) of all patients with solid food–FPIES also had cow’s milk and/or soy-FPIES. Initial presentation was severe in 79% of the patients, prompting sepsis evaluations (57%) and hospitalization (64%) for dehydration or shock. The diagnosis of FPIES was delayed, after a median of 2 reactions (range: 2–5). Thirty patients with typical cow’s milk- and/or soy-FPIES were identified for comparison. Overall, 48% of the 44 infants with FPIES were reactive to >1 food protein, and the risk for multiple food hypersensitivity approached 80% in the infants with solid food or soy-induced FPIES. None of the patients developed FPIES to maternally ingested foods while breastfeeding unless the causal food was fed directly to the infant.Conclusions. Cereals, vegetables, and poultry meats, typically regarded as of low allergenic potential, must be considered in the evaluation of FPIES, particularly in infants previously diagnosed with FPIES to cow’s milk or soy, and as an initial cause in patients who have been exclusively breastfed. Infants with FPIES are at risk for multiple dietary protein hypersensitivities during an apparent period of immunologic susceptibility. Pediatricians should consider FPIES in the differential diagnosis of shock and sepsis.

MA Ruffner, K Ruymann, S Barni, A Cianferoni, T Brown-Whitehorn, JM Spergel. J Allergy Clin Immunol Pract. 2013;1(4):343–349 The goal of this study was to describe the demographic characteristics, clinical symptoms, and allergy test results in a large cohort of patients with food protein–induced enterocolitis syndrome (FPIES). Patient charts were reviewed from The Children’s Hospital of Philadelphia, a large referral hospital with patients primarily from Pennsylvania, New Jersey, and Delaware. This study was a retrospective chart review of electronic medical records from 2007 to 2012. Originally, 992 patients were identified as having “allergic gastroenteritis and colitis” according to International Classification of Diseases, Ninth Revision , coding. …

Mehr SS, Kakakios AM, Kemp AS. Arch Dis Child . 2009;94(3):220–223 PURPOSE OF THE STUDY. To examine the characteristics of children presenting with food protein-induced enterocolitis syndrome (FPIES) attributable to rice and to determine whether there were any differences from those presenting with cow's milk and/or soy FPIES. STUDY POPULATION. Retrospective study of 31 children presenting with FPIES (14 with rice and 17 with milk/soy) to the Children's Hospital at Westmead, Australia, during …

Mehr S, Kakakios A, Frith K, Kemp AS. Pediatrics. 2009;123(3). Available at: www.pediatrics.org/cgi/content/full/123/3/e459 PURPOSE OF THE STUDY. To investigate possible patterns in demographic features, causative foods, clinical features, treatments at presentation, and outcomes in children diagnosed with food protein-induced enterocolitis syndrome (FPIES). STUDY POPULATION. Retrospective chart review of 35 children who presented with acute FPIES at a tertiary medical center in New South Wales, Australia, between 1992 and 2007. METHODS. Diagnosis was made by pediatric allergists after referral to the allergy clinic (74%) or from the emergency department (ED) (26%), using previously published criteria. Cases were identified by codes signifying allergic and dietetic gastroenteritis and colitis or by …

Y Katz, MR Goldberg, N Rajuan, A Cohen, M Leshno. J Allergy Clin Immunol. 2011;127(3):647–653 To prospectively determine the prevalence, clinical characteristics, and natural history of food protein–induced enterocolitis (FPIES) in association with cow's milk protein (CMP). In this birth-cohort study, 13 019 of 13 234 newborns (98.4%) born over a 2-year period from June 2004 to June 2006 were enrolled. Information on reactions to CMP were obtained for all infants, and those with probable …

Hwang JB, Sohn SM, Kim AS. Arch Dis Child. 2009;94(6):425–428 PURPOSE OF THE STUDY. To determine tolerance rates to cow's milk and soy for infants affected by food protein-induced enterocolitis syndrome (FPIES). STUDY POPULATION. Twenty-three patients (7 female and 16 male) with infantile FPIES were prospectively followed. METHODS. Infants with a diagnosis of FPIES were diagnosed by positive oral food challenges for milk or soy formula at 36 days of age (SD: 14 days; range: 13–58 days). These infants were prospectively followed until >2 years of age. They underwent ≥2 follow-up oral challenges. The first follow-up oral challenges …

Fogg MI, Brown-Whitehorn TA, Pawlowski NA, Spergel JM. Pediatr Allergy Immunol. 2006;17:351–355 PURPOSE OF THE STUDY. This prospective study was undertaken to determine if the atopy patch test (APT) is able to predict the results of the oral food challenge (OFC) for food protein–induced enterocolitis syndrome (FPIES). The APT involves placement of food in a Finn chamber (metal cap) left on the skin for 48 hours and evaluated for rash in the subsequent days after removal. STUDY POPULATION. Nineteen patients aged 5 to 30 months who had suspected FPIES on the basis of clinical history. METHODS. The infants underwent APT to the suspected foods. After APT was performed, the subjects underwent OFC to determine if FPIES was present. The results of APT and OFC were compared and used to calculate sensitivity and specificity of the APT. RESULTS. APT predicted the results of OFCs in 28 of 33 instances. There were 16 cases of FPIES confirmed by OFCs. In all 16 cases of FPIES, the APT result was positive to the suspected food. However, the APT was positive in 5 instances in which the OFC result was negative. All 12 patients with a negative APT result had a negative OFC result to the suspected food. CONCLUSIONS. APT seems to be a promising diagnostic tool for the diagnosis of FPIES. REVIEWER COMMENTS. FPIES is a non–immunoglobulin E (IgE)-mediated food allergy in which affected infants develop gastrointestinal symptoms hours after ingestion of the offending food. Current allergy skin and serum tests are not useful for diagnosing this disorder, because they test for food-specific IgE levels that are often negative in FPIES. A diagnostic OFC is the gold standard. The role for APT in diagnosing other non–IgE-mediated food hypersensitivities has been investigated. The results of this study suggest that APT may have some utility in guiding the diagnosis and management of FPIES.