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The allergic march can often result in a child with one food allergy phenotype developing other allergic manifestations as they grow older. In this grand round, we describe the case of a child diagnosed with Eosinophilic Esophagitis at an early age, who subsequently developed IgE-mediated allergy to milk and egg. Due to the onset of allergic rhinitis, an additional complication of their teenage years was the development of Pollen Food Syndrome, which further impacted upon the already complex dietary management. These multiple allergies had a consequent effect on nutritional status and provoked considerable psycho-social issues which greatly affected the patient's relationship with food. This case illustrates that for complex patients, a cohesive approach and shared decision making is essential for their diagnosis and management. Working with not only the patient, but their whole family, and considering how best to support them, necessitates a flexible approach. Regular follow-up is important to address changing psycho-social and dietary needs as well as monitoring the resolution of allergies and response to pharmacotherapy. Complex patients need the input of a comprehensive multidisciplinary team, with good access to dietetic and psychological support in order to optimize the quality of life of these individuals and their families.

Objective Food protein-induced enterocolitis syndrome (FPIES) is a non–immunoglobulin E-mediated food allergy with potential risk of malnutrition related to the early onset of disease, frequent avoidance of cow's milk, and the possibility of multiple food triggers. This publication is aimed at providing an evidence-based, practical approach to the dietary management of FPIES. Data Sources This is a narrative review summarizing information from national and international guidelines, retrospective studies, population studies, review articles, case reports, and case series to evaluate for nutritional risk and develop guidance for risk reduction in children with FPIES. Study Selections We have included retrospective clinical cohort studies, population-based studies, case reports, and case studies. We did not exclude any studies identified owing to the small number of studies addressing the nutritional management of individuals with FPIES. Results Children with FPIES are at risk of malnutrition owing to suboptimal oral intake, limited food choices, and knowledge deficits related to feeding. In particular, children with 3 or more FPIES triggers seem to be at increased risk for poor weight gain and developing food aversion. Caregivers of children with FPIES also report a high degree of psychosocial burden. Conclusion Appropriate dietary management entails the following 3 essential components: supporting normal growth and development, avoidance of allergens, and advancement of complementary foods. Education to avoid the trigger food and assisting caregivers in creating an individualized, well-designed complementary feeding plan to meet the infant's nutritional needs for optimal growth and development are essential management strategies.

Background: Oral food challenges (OFC) in food protein‐induced enterocolitis syndrome (FPIES) are performed to confirm a diagnosis, test for development of tolerance, and find safe alternatives. Objective: We aimed to define OFC outcomes and identify safer test strategies. Methods: OFCs performed in children with FPIES over a 5-year period were reviewed. Results: A total of 160 OFCs were performed in 59 children (median age, 2.3 years). The most commonly tested foods were hen's egg, fish, and cow's milk. Sixty-six OFC results (41.3%) were positive. Twelve (18.2%) reactions were mild, 18 (27.3%) were moderate, and 36 (54.5%) were severe. Intravenous fluid, ondansetron, and corticosteroids were administered in 83.3, 72.7, and 66.7% of the patients, respectively; one patient required hospitalization. A reaction was most likely with fish (odds ratio [OR] 2.878 [95% confidence interval {CI}, 1.279‐6.473]; p = 0.011), and least likely with cow's milk (OR 0.268 [95% CI, 0.082‐0.872]; p = 0.029). Of the 36 OFCs with egg yolk, 23 patients had a failed OFC, and of the 17 OFCs with egg white (all tolerant to egg yolk), only 2 patients had a failed result. Interestingly, two patients tolerated baked whole egg but not egg yolk. In cow's milk FPIES, two patients could consume fermented milk but not fresh milk. Of the 11 patients with anchovy-triggered FPIES, 6 tolerated sea bass, and 5 tolerated trout, whereas 4 patients with sea bass FPIES tolerated trout. Conclusion: The search for alternative food species, processing, or subdivision of a trigger food is common practice in FPIES, which reflects the expectations of children and their families. The experience gained can be put into practice and will contribute to the understanding of the disease mechanism.

Introduction and objective: The oral food challenge (OFC) is the gold standard to diagnose food allergy (FA); however, it is not a procedure free from the risk of having significant allergic reactions, even life-threatening.The aims of our study were to evaluate the frequency of positive OFCs performed in chil-dren with a suspected diagnosis of IgE- and non-IgE–mediated (food protein–induced enterocolitis syndrome (FPIES)) FA and how the failed challenges were managed.Materials and methods: A retrospective chart review was done on all children who have had OFCs in a tertiary-care pediatric allergy unit from 2017 to 2019.Results: 682 patients were enrolled and 2206 challenges were performed: 2058 (93%) for IgE-mediated FA and 148 (7%) for FPIES. There were 262 (11.8%) challenge failures. The transfer to the emergency department was required 3 times (1.1%). None of the failed challenges resulted in death or hospitalization and 13.3% challenges did not require any treatment.Conclusions: Our findings confirm that food challenges can be performed safely in a specialized setting by well-trained personnel; all food challenge reactions, even the most serious, were reversible, thanks to a prompt recognition and treatment that generally did not worsen over time.

Objective Although food protein–induced enterocolitis syndrome (FPIES) was first described approximately 50 years ago and research is increasing, there are still considerable unmet needs in FPIES. This article catalogs the areas of progress and areas for further research. Data Sources Through our personal experiences in caring for patients with FPIES, our personal research, and a review of the existing FPIES literature as indexed in PubMed, we explored what is known and what is needed in FPIES. Study Selections The studies that have improved the knowledge of FPIES, defined phenotypes, allowed for better-informed management of FPIES, and laid the groundwork for further research. Results Further research is needed in the areas of prevalence, natural history, trigger foods, threshold doses, how and when to perform oral food challenges, and immunopathogenesis of this disorder. Development of a biomarker and determination of the best method to treat reactions is also needed. Furthermore, FPIES has a substantial psychosocial and economic impact on families, and more research is needed in developing and implementing ameliorating strategies. Conclusion By partnering together, health care providers, advocacy organizations, and families can continue to advance our understanding and improve the care of patients and families living with FPIES.

Objective Food protein-induced enterocolitis syndrome (FPIES) is typically diagnosed based on a characteristic clinical history; however, an oral food challenge (OFC) may be necessary to confirm the diagnosis or evaluate for the development of tolerance. FPIES OFC methods vary globally, and there is no universally agreed upon protocol. The objective of this review is to summarize reported FPIES OFC approaches and consider unmet needs in diagnosing and managing FPIES. Data Sources PubMed database was searched using the keywords food protein-induced enterocolitis syndrome, oral food challenge, cow milk allergy, food allergy, non-immunoglobulin E–mediated food allergy and FPIES. Study Selections Primary and review articles were selected based on relevance to the diagnosis of FPIES and the FPIES OFC. Results We reviewed the history of FPIES and the evolution and variations in the FPIES OFC. A summary of current literature suggests that most patients with FPIES will react with 25% to 33% of a standard serving of the challenged food, there is little benefit to offering a divided dose challenge unless there is suspicion of specific immunoglobulin E to the food being challenged, reactions typically appear within 1 to 4 hours of ingestion, and reactions during OFC rarely result in emergency department or intensive care unit admission. Conclusion International standardization in the FPIES OFC approach is necessary with particular attention to specific dose administration across challenged foods, timing between the patient’s reaction and offered OFC to verify tolerance, patient safety considerations before the OFC, and identification of characteristics that would indicate home reintroduction is appropriate.

Objective Food protein-induced enterocolitis syndrome (FPIES) is a condition with heterogeneous features (ie, age at presentation, severity, food triggers, comorbidities) and is not as rare as initially believed. In the last few years, the first population-based epidemiologic study, few prospective birth cohort evaluating FPIES prevalence, and several larger (>100 patients) studies have been published, making epidemiologic estimation more reliable. In this review, we report on the available data on the epidemiology of FPIES. Data Sources PubMed review using the following words: FPIES, epidemiology, and prevalence. Study Selections The review focused on the population-based epidemiologic study, few prospective birth cohort evaluating FPIES prevalence, and several larger (>100 patients) studies. Results We identified 8 population or cohort studies. Conclusion FPIES is not rare in both children and adults and may affect as many as 900,000 people in the United States alone. Most children and adult with FPIES seem to react to 1 to 2 foods; however, they may need further diet restriction owing to high level of comorbidity with immunoglobulin E–mediated food allergies and eosinophilic esophagitis. Globally, cow's milk, rice/oat, and seafood seem to be the most common triggers.

The most common types of non-IgE-mediated food allergy are food protein-induced enterocolitis syndrome (FPIES) and food protein-induced allergic proctocolitis (FPIAP). FPIES presents with delayed refractory emesis, while FPIAP presents with hematochezia in otherwise healthy infants. Acute management of FPIES includes rehydration or ondansetron, or both. No acute management is required for FPIAP. Long-term management of both disorders includes avoidance of the trigger food. The prognosis for both conditions is a high rate of resolution within a few years’ time.

In 2014, drug-induced enterocolitis syndrome (DIES) was described for the first time. It is still a poorly known disease with symptoms that typically resemble those of food protein-induced enterocolitis syndrome (FPIES). To date, six more cases of DIES have been described and new clinical diagnostic criteria have been proposed based on those in the international guidelines for FPIES. In this paper, the authors describe three more cases of DIES. In addition, similarities and differences with FPIES have been deeply analyzed. To date, several unanswered questions need to be addressed, but clinicians must be instructed how to identify DIES, in order to make an allergy workup and give definite therapeutic indications to patients, especially in children where DIES seems to be more frequent.

BACKGROUND Food protein-induced enterocolitis syndrome (FPIES) is a non-IgE-mediated food allergy causing severe acute gastrointestinal symptoms and lethargy, mainly affecting infants and young children. There are geographic variations in its clinical features. This study aimed to describe the clinical characteristics and management of FPIES in Swedish children. METHODS The study included children who presented with acute FPIES during 2008-2017. All Swedish pediatric departments (n = 32) were invited to report their known patients. Data were collected through chart reviews and interviews with parents. RESULTS Eighteen pediatric departments contributed, and 113 patients were included. Most had a family history of atopy (74%), and 51% had an atopic disease. Common trigger foods were cow's milk (26%), fish (25%), oat (22%), and rice (8%). Most patients (85%) reacted to a single food. The median age at first reaction was 3.9 months for cow's milk and 6.0 months for other foods (p

Background: Food protein-induced enterocolitis syndrome (FPIES) is a non-IgE-mediated food allergy characterized by gastrointestinal symptoms, mainly protracted and delayed vomiting. Diagnosis is based on clinical history, and it can be challenging as symptoms are delayed and the causative food is often not very suspicious. Objective: This case report highlights the importance of having a high degree of suspicion to reach a correct diagnosis. Materials and methods: We report an unusual case of FPIES due to zucchini. During the follow-up. Two oral food challenges (OFC) were carried out to evaluate tolerance to the food involved. Results: The first OFC was positive and in the second the child tolerated the food without problems. Conclusions: In this case, the OFC was essential to identify the offending food and to verify that the child had overcome the disease.

Cow’s milk allergy is a common food allergy among infants. Symptoms of cow’s milk allergy are wide-ranging and depend on the mechanism involved. There are immunoglobulin E (IgE)-mediated, non–IgE-mediated, and mixed mechanisms of food allergy. Symptoms of IgE-mediated cow’s milk allergy may be mild or may progress to anaphylaxis, which can be life-threatening. Non–IgE-mediated allergy includes food protein–induced allergic proctocolitis (FPIAP), food protein–induced enterocolitis syndrome, food protein–induced enteropathy, and Heiner syndrome (pulmonary hemosiderosis). These diagnoses comprise about half of all cow’s milk allergies. The most common manifestation of cow’s milk allergy in infants is FPIAP. FPIAP is commonly seen in healthy, full-term infants who present with rectal bleeding and are otherwise well-appearing. This can occur in both formula-fed and exclusively breastfed infants. Food proteins secreted in maternal breast milk can contribute to the development of these symptoms. Maternal cow’s milk elimination diet is often successful in helping resolve symptoms. A period of reintroduction of cow’s milk resulting in re-emergence of symptoms in stable asymptomatic infants is an excellent diagnostic tool to confirm a cow’s milk allergy. Preterm infants are susceptible to food allergy, as demonstrated from several case reports of necrotizing enterocolitis–like illnesses that responded clinically to cow’s milk elimination. Further study is needed about food allergy in the preterm infant population.

Food protein-induced enterocolitis syndrome is still a mysterious disease, pathogenically poorly characterized, although the first FPIES case has been described in 1967. Mainly, food protein-induced enterocolitis syndrome diagnosis is based on clinical history. The oral food challenge remains the gold standard to confirm the diagnosis, especially in particular situations. Although there are no diagnostic laboratory or imaging tests which are specific for diagnosis, they could, however, sometimes be helpful to rule out clinical conditions which are similar to food protein-induced enterocolitis syndrome reactions. The purpose of this review is to define the clinical features of FPIES and to summarize the current available tools for the diagnosis of FPIES. This review is intended to be a practical guide for the clinician facing a patient with food protein-induced enterocolitis syndrome avoiding delayed diagnosis with unnecessary laboratory tests and detrimental treatments. Moreover, it highlights the unmet needs in diagnosis that require urgent attention from the scientific community to improve the management of patients with FPIES.

Background: Food protein‐induced enterocolitis syndrome (FPIES) is a rare non-IgE mediated food allergy. Objective: To delineate the differences in the spectrum of culprit foods, remission patterns, and predictors among varying cultures. Methods: We reviewed demographics, culprit foods, outcomes, and predictors in 81 children with a diagnosis of FPIES who were followed up between 2015 and 2020. Results: Eighty-one patients (55.6% boys) were enrolled, including 72 with acute FPIES and 9 with chronic FPIES. Hen’s egg was the most common culprit food (36.6%), followed by fish (26.9%), and cow’s milk (21.5%). Interestingly, cow’s milk was significantly prevalent in chronic FPIES cases (p = 0.006). The most common clinical symptoms were vomiting (100%), pallor (63.4%), and lethargy (55.9%). Emergency department visits were noted in 39 patients (41.9%), of whom 37 (39.8%) were treated with intravenous (IV) fluid. The subjects were followed up for a median (interquartile range) of 19.4 months (12.3‐41.2 months), and 26 subjects (32.1%) achieved tolerance. The median (interquartile range) age at tolerance was 2.5 years (2.1‐3.2 years). With regard to the culprit foods, hen’s egg was observed more frequently in the subjects with resolved FPIES cases (p = 0.008), whereas fish FPIES cases were high in the persistent group (p = 0.001). IgE sensitization of the culprit food was found to be an independent risk factor for the persistence of FPIES (odds ratio 4.855 [95% confidence interval, 1.131‐20.844]; p = 0.034). Conclusion: In our cohort, unlike other published series, hen’s egg and fish were the two most common culprit foods. Fish differed from other culprit foods, with significantly delayed onset and persistence, and may create a model that allows for the understanding of the disease.

Food protein‐induced enterocolitis syndrome (FPIES) is a non‐IgE-mediated food allergy that primarily affects the gastrointestinal tract. The underlying pathophysiology of FPIES has yet to be fully elucidated; however, FPIES is believed to be secondary to intestinal inflammation after exposure to a food antigen, which thereby leads to increased permeability and fluid shifting into the intestinal lumen. FPIES is categorized into acute and chronic forms. Acute FPIES is characterized by repetitive vomiting that occurs 1‐4 hours after food ingestion. Severe vomiting may progress to dehydration, lethargy, and pallor, which potentially leads to hypovolemic shock. In some patients, diarrhea may present within 24-hours of food ingestion. Patients are clinically well between acute episodes. Chronic FPIES presents with intermittent vomiting and/or diarrhea, followed by failure to thrive. FPIES characteristically presents in infancy, with resolution of the disease typically occurring by school age. However, analysis of recent data indicates that FPIES may persist into adulthood. In addition, late- or adult-onset FPIES has also been reported. The diagnosis of FPIES is based on clinical history; however, oral food challenge currently remains the criterion standard for diagnosis. Management of FPIES requires strict avoidance of food triggers, and treatment requires rapid fluid rehydration. Currently, there are no reliable biomarkers to diagnose FPIES; however, investigations to better understand the role of the innate immune system have been promising. Future studies are needed to better understand the true prevalence and pathophysiology of FPIES.

Introduction: Food protein-induced enterocolitis syndrome (FPIES) is a gastrointestinal syndrome due to a non-IgE mediated food hypersensitivity. The most common triggers are cow's milk and soy. Fish is one of the most frequently reported causes in Spain.The objective of this study is to describe the clinical characteristics of patients diagnosed with (FPIES) in a paediatric allergy clinic. Material and methods: A retrospective descriptive study was carried out by reviewing medical records of patients diagnosed with FPIES in the Paediatric Allergy Unit of the Miguel Servet Children's Hospital from the years 2007 to 2017. Results: A total of 135 patients were diagnosed during the study period, of whom 45% were male and 55% were female. The mean age at diagnosis was 11 ± 1.5 months and the mean age of improvement was 2 years and 6 months ± 2.5 years (n = 83). A personal history of atopy was observed in 31.9%. The main trigger foods were: white fish (41.4%), cow's milk (25.1%), and egg (15.5%). A conversion to IgE-mediated allergy was seen in 4.4% of patients.There was vomiting in 81.5% of the cases, with a mean of 1.75 ± 1.1 h of latency, as well as diarrhoea in 41.5%, with a mean of 7.86 ± 15.16 h of latency, and decline in 30.4% with a mean latency of 3.81 ± 11.57 h. Discussion: In our series, the most frequent trigger of the FPIES was fish. It was manifested mainly by late vomiting and a tolerance was reached mostly at 2 years 6 months. Resumen: Introducción: La enterocolitis inducida por proteínas de la dieta, o también conocida como food protein-induced enterocolitis syndrome (FPIES), es un síndrome gastrointestinal de hipersensibilidad alimentaria no mediada por IgE. Los desencadenantes más comunes son la leche de vaca y de soja. El pescado es una de las causas reportadas con más frecuencia en España.El objetivo de esta investigación es describir las características clínicas de los pacientes diagnosticados de FPIES en nuestra consulta de alergología pediátrica. Material y métodos: Estudio descriptivo retrospectivo, realizado mediante revisión de historias clínicas de los pacientes diagnosticados con FPIES en la Unidad de Alergología Pediátrica del Hospital Infantil Miguel Servet desde 2007 a 2017. Resultados: Desde enero de 2007 hasta diciembre de 2017 fueron diagnosticados 135 pacientes: 45% hombres y 55% mujeres. La edad media al diagnóstico fue de 11 ± 1,5 meses y la edad media de superación 2 años y 6 meses ± 2,5 años (n = 83). Un 31,9% presentó antecedentes personales de atopia. Los principales alimentos desencadenantes fueron: pescado blanco (41,4%), leche de vaca (25,1%) y huevo (15,5%). Un 4,4% presentó conversión a alergia IgE mediada.El 81,5% de los casos presentó vómitos, con una media de 1,75 ± 1,1 horas de latencia; diarreas en un 41,5%, con una media de 7,86 ± 15,16 horas de latencia; decaimiento en el 30,4% con una media de 3,81 ± 11,57 horas de latencia. Discusión: En nuestra serie el desencadenante más frecuente fue el pescado. Se manifestó principalmente por vómitos tardíos, y alcanzó una tolerancia en su mayoría hacia los 2 años 6 meses.

Purpose of review First described in the mid 20th century, it was just in the last decade that diagnostic and treatment guidelines for food protein-induced enterocolitis syndrome (FPIES) were established. Awareness of the diagnosis is improving, and epidemiologic data are emerging. Recent findings Recent studies suggest that FPIES may affect as many as 0.5% of children worldwide. FPIES in adults is usually triggered by seafood and may be more common than previously thought. Many patients with FPIES have other allergic disorders. Summary With refined diagnostic criteria and improved awareness, FPIES is now diagnosed with increasing frequency, and epidemiologic data are emerging. FPIES appears to be increasing in prevalence, and the frequent association with other allergic disorders suggests a shared predisposition or immune mechanism that remains to be elucidated.

Food protein-induced enterocolitis syndrome (FPIES) is a non-IgE-mediated food allergy that manifests with projectile, repetitive emesis that can be followed by diarrhea and may be accompanied by lethargy, hypotonia, hypothermia, hypotension, and metabolic derangements. FPIES usually starts in infancy although onset at older ages is being increasingly recognized. FPIES is not rare, with the cumulative incidence of FPIES in infants estimated to be 0.015% to 0.7%, whereas the population prevalence in the US infants was 0.51%. FPIES diagnosis is challenging and might be missed because of later (1-4 hours) onset of symptoms after food ingestion, lack of typical allergic skin and respiratory symptoms, and food triggers that are perceived to be hypoallergenic. Diagnosis is based on the recognition of symptoms because there are no biomarkers of FPIES. The pathophysiology remains obscure although activation of the innate immune compartment has been detected. Management relies of avoidance of food triggers, treatment of accidental exposures, and periodic re-evaluations with supervised oral food challenges to monitor for resolution. There are no strategies to accelerate development of tolerance in FPIES. Here we review the most important current concepts in epidemiology, pathophysiology, diagnosis, and management of FPIES.

Food protein induced enterocolitis syndrome (FPIES) can present with diarrhea, hypovolemia and electrolyte imbalance in infancy. We present a case of life-threatening methemoglobinemia in a 1-month-old infant, a rare complication of FPIES triggered by cow ’s milk protein intake. A previously healthy 1-month-old boy presented with lethargy, increased work of breathing and 2-day history of vomiting. Review of systems revealed a 3-week history of diarrhea. He was lethargic, shocky and dusky, and was intubated for persistent hypoxia. His blood work revealed severe acidemia with pH of 6.95 and methemoglobin level of 66% (normal range < 3%). His methemoglobin level and clinical status normalized following volume resuscitation, packed red blood cell transfusion and prompt intravenous methylene blue administration. Further investigations revealed a diagnosis of FPIES which was managed with a hypoallergenic formula. Methemoglobinemia should be considered in young infants presenting with severe vomiting and diarrhea, secondary to dietary protein intolerance syndromes. Prompt management with methylene blue and fluid resuscitation can result in excellent prognosis, along with specific ongoing management for FPIES. Int J Clin Pediatr. 2020;9(2):35-40 doi: https://doi.org/10.14740/ijcp366

As the incidence of cow ’s milk protein allergy (CMPA) has increased in the last decades in both breastfed and formula fed infants, possible pitfalls in its diagnosis and management are also increasing. This is especially evident in non-IgE milk allergy due to the considerable delay between the appearance of clinical symptoms after ingestion of the allergen, and the non-specific gastrointestinal symptoms that characterize it. The misdiagnosis could often be combined with inappropriate use of either partially hydrolyzed or amino acid-based infant formula for the management of symptoms. The aim of the paper is to present two cases to illustrate common pitfalls in diagnosis and management of CMPA with divergent gastrointestinal syndrome manifestations: food protein-induced allergic proctocolitis and food protein-induced enterocolitis syndrome, in an effort to increase awareness of these conditions and to guide clinicians in day-to-day practice when facing suspected cases of CMPA. Int J Clin Pediatr. 2020;9(3):98-102 doi: https://doi.org/10.14740/ijcp375

Breastfeeding is currently recommended as the optimal source of nutrition to infants. However, there are several studies that have shown clinical IgE- and non-IgE-mediated reactions to foods in exclusively breastfeeding infants, specifically to cow's milk, egg, peanut, and fish. Literature suggests that antigenic food proteins present in human milk can be found in substantial enough amounts to elicit clinical reactions in some, already-sensitized infants, including anaphylaxis, eczema exacerbation, and non-IgE-mediated gastrointestinal food-allergic syndromes. Diagnosis of food allergy in a breastfed infant and identification of the trigger foods in the mother's diet can be especially challenging in infants with delayed symptoms, such as eczema and gastrointestinal symptoms. Management is further complicated in infants with atopic dermatitis, who have increased caloric needs and therefore in whom nutrition is an extremely important factor for growth and development. One needs to balance possible benefits with risks of further food sensitization through the skin when foods are eliminated from their diets. We review here the literature on clinical presentation and evidence for food allergy in exclusively breastfed infants, including the presence of food antigens in human milk. Incorporating clinical experience and the available data, which largely come from case reports and small, nonrandomized studies performed in referral centers with several limitations, we propose a novel algorithm to diagnosis and management, with emphasis on nutritional considerations.

The prevalence of allergy is increasing across the world, and asthma is the most common chronic disease in children. Exclusive breastfeeding does decrease the occurrence of eczema in the first 2 years of life, and a longer duration of breastfeeding decreases wheezing in the first 2 years of life. A longer duration of breastfeeding protects against asthma even up to 5 years of age. Food allergies in breastfeeding infants do occur, although the exact timing and mechanism(s) of sensitization are poorly understood. Maternal elimination diets in pregnancy and/or lactation do not prevent atopic disease, but infant avoidance of foods and maternal elimination diet may be useful in infants with diagnosed food allergies and specifically food protein induced enterocolitis syndrome. An expert panel of the American Academy of Pediatrics now recommends early introduction (4 to 6 months of age) of infant-safe forms of peanuts to decrease the risk for peanut allergy in at-risk infants.

BACKGROUND: Food protein-induced enterocolitis syndrome (FPIES) is a frequently misdiagnosed, serious, non-IgE mediated food allergy, and the precise mechanism of disease is unknown. Acute FPIES typically presents with repetitive, profuse vomiting approximately 1-4 hours post ingestion of a food trigger. Chronic FPIES is considered less common and less well characterized. OBJECTIVE: We aimed to better describe FPIES and identify factors that may influence FPIES development through use of a self-reported, caregiver’s survey. METHODS: FPIES and allergy-free infant caregivers completed a survey regarding lifestyle factors that may influence allergy acquisition such as: antibiotic usage and delivery mode. FPIES caregivers reported symptoms, number of food triggers, type of FPIES, and symptoms from breastmilk ingestion. FPIES infants were compared to allergy-free infants to identify factors potentially associated with FPIES. RESULTS: Infant and prenatal maternal antibiotic usage was higher in FPIES infants compared to allergy-free infants (43.8% vs 20.6% and 48.8% vs 23.57%, respectively, p< 0.05). When compared to infants with ACUTE FPIES alone, infants described as BOTH acute and chronic FPIES reported earlier onset of symptoms, more non-specific symptoms and symptoms triggered by breast milk, more antibiotic exposure, and more food triggers (p< 0.05). CONCLUSION. Antibiotic usage was significantly higher in FPIES infants when compared to allergy-free infants. Work is needed to elucidate the role of antibiotic usage in the etiology of FPIES. Infants reported to have BOTH acute and chronic FPIES were significantly different from infants with ACUTE FPIES alone highlighting the need to more closely examine these different subtypes of FPIES.

Characteristics of chronic milk-dependent food protein-induced enterocolitis syndrome (FPIES) in children from the region of Western Pomerania were studied. Prospectively, 55 children were diagnosed at a median of 2.2 months. The open food challenges (OFC), morphologies, milk-specific IgE (sIgE) (FEIA method, CAP system), and skin prick tests (SPTs) were examined. Vomiting and diarrhea escalated gradually but quickly led to growth retardation. Of the infants, 49% had BMI &lt, 10 c, 20% BMI &lt, 3 c, 25% had anemia, and 15% had hypoalbuminemia. During the OFCs we observed acute symptoms that appeared after 2–3 h: vomiting diarrhea and pallor. A total of 42% children required intravenous hydration. Casein hydrolysates or amino acids formulae (20%) were used in treatment. In 25% of children, SPT and milk sIgE were found, in 18%—other food SPTs, and in 14% allergy to other foods. A transition to IgE-dependent milk allergy was seen in 3 children. In the twelfth month of life, 62% of children had tolerance to milk, and in the twenty-fifth month—87%. Conclusions. Chronic milk-dependent FPIES resolves in most children. By the age of 2 children are at risk of multiple food sensitization, and those who have milk sIgE are at risk to transition to IgE-mediated milk allergy. Every OFC needs to be supervised due to possible severe reactions.

Food-protein-induced enterocolitis syndrome (FPIES)was notrecognized as a specific disorder until the 1970s(1). While the European Academy of Allergy and Clinical Immunology (EAACI) Guidelines addressed FPIES in 2014(2),it was only in 2017 that the first international consensus guidelines foritsdiagnosis and treatment werepublished (3). Acute FPIES usually presents with repetitive vomiting, diarrhea associated with pallor, lethargy, dehydration, hypotension and even shock in most severe cases.

This study aimed to investigate the clinical characteristics of severe food protein-induced enterocolitis syndrome (FPIES) in patients with Down syndrome. We report the cases of three infants with Down syndrome who were diagnosed with FPIES. All patients presented with hypovolemic shock, metabolic acidosis, and acute kidney injury after introducing a milk-based formula. They required aggressive fluid resuscitation and alternative nutrition. All three patients survived without any complications after the treatment. FPIES may cause hypovolemic shock in infants with Down syndrome and these patients need prompt fluid resuscitation.

Background: Oral food challenges remain the most reliable method for allergy confirmation. Although consensus guidelines have been published to unify Immunoglobulin E (IgE)-mediated challenges, this does not exist for non-IgE mediated gastrointestinal allergies outside of Food Protein Induced Enterocolitis Syndrome. We therefore set out to establish the use of home introduction protocols (HIP) for confirmation of food allergy for milk, soya, egg and wheat using a ladder approach in children with non-IgE mediated allergy.Materials and Methods: Patients with suspected non-IgE mediated gastrointestinal allergies (0–16 years) were recruited following symptom improvement on an elimination diet. All children had skin prick or specific IgE tests to rule out IgE-mediated allergies prior to suggestion the HIP. Number of trials and outcome was documented. HIPs were developed using a published ladder approach for cow's milk as baseline and final dose was calculated based on guidelines for food protein induced enterocolitis syndrome and portions for age from the National Diet and Nutrition Survey. First foods were baked/highly processed and every 4th day patients moved to a more unprocessed/unheated food.Results: From 131 recruited patients, 117 (89.3%) followed the HIP for food allergens. No adverse events were documented. In more than 50% of cases one attempt at the HIP was sufficient to establish allergy status, but many required 2–5 attempts before the outcome was clear. About half of the children were fully tolerant to foods they initially eliminated: 36, 26 and 30% were partially tolerant to milk, soya, and egg and only 15% achieved partial tolerance to wheat. Wheat was the allergen introduced earliest, followed by soya, cow's milk and egg.Conclusions: This study indicates that home HIPs are safe in non-IgE mediated gastrointestinal food allergy and that the ladder approach may be useful in re-introducing allergens in children at home with non-IgE mediated gastrointestinal allergies. From this study we can also conclude that tolerance to processed/baked allergens was observed in many children. Further studies should be performed on the HIP and ideally reintroduction should occur pre-defined time intervals.

Acute food protein-induced enterocolitis syndrome (FPIES) is a non-IgE-mediated food allergy characterized by the onset of delayed repetitive and projectile vomiting accompanied by other clinical manifestations such as pallor, hypotonia, lethargy, hypothermia and/or diarrhea (1). Acute FPIES can be classified as classic or atypical based on the absence or presence of sensitization to the trigger food (2).

Food protein-induced enterocolitis syndrome (FPIES) is a nonimmunoglobulin E cell-mediated food allergy, which occurs predominantly in infants and young children. The most commonly incriminated triggers are cow's milk (CM), soy, and grains. Acute FPIES can be potentially life-threatening and culminate in shock requiring fluid resuscitation in at least 15% of the cases. To our knowledge, there have been no reports in literature of cardiorespiratory arrest induced by acute FPIES. We describe the first case of cardiorespiratory arrest occurred after accidental ingestion of a CM-based formula in a 5-month-old infant with previous diagnosis of FPIES to CM.

Food protein-induced enterocolitis syndrome (FPIES) is considered a rare, non-IgE-mediated food allergy that typically presents in infancy. As there is not a specific biological marker of the disease, diagnosis of FPIES is based on typical symptoms that improve once the offending food is removed from the diet. The most common causative foods are cow’s milk, soy, and rice. For years, we have thought that the typical symptoms and the triggering foods were the same around the world. The epidemiological data are scarce and variable but recent studies suggests that FPIES is not as rare as it is thought but rather misdiagnosed. The clinical symptoms and the offending foods vary depending on the geographic area. Perhaps because different phenotypes might exist due to dietary habits, race, or ethnicity. FPIES symptoms and causative foods are not the same around the world. It is important to know our population in order to reach a correct and early diagnosis of our patients.

Food protein-induced enterocolitis syndrome (FPIES) is a non-IgE-mediated gastrointestinal food allergic disorder that has gained a major interest the past decade. FPIES prevalence, which still needs to be accurately determine in different populations, appears to be higher than previously thought (ie up to 0.7% in infants in the 1st year of life). FPIES to seafood in adults is also increasingly reported; limited data suggest that adult FPIES is most commonly triggered by shellfish, tends to affect females more than men, is characterized by a significant delay in diagnosis and a prolonged course. The first international consensus guidelines on diagnosis and management of FPIES have been published in 2017, proposing new diagnostic criteria as well as new criteria for a positive oral food challenge. However, there is a need to develop new biomarkers to improve the diagnosis and management of FPIES patients, and this requires a better understanding of the pathophysiology. Recently, the role of T cells has been questioned and a major role of innate immune cells has been suggested in acute FPIES. Regarding the treatment of acute FPIES reaction, ondansetron has emerged as an adjunct to intravenous rehydration in moderate-severe reactions and as a first-line treatment in mild reactions. Important information regarding the nutritional management of FPIES patients that might be complex has also been provided in the international guidelines. In this review, we discuss recent advances regarding all those different aspects.

One of non-IgE-mediated disorders that pediatricians and allergologists have to deal with is food protein-induced enterocolitis syndrome (Food Protein Induced Enterocolitis Syndrome, FPIES). Cow milk and soy proteins are the most common cause of FPIES. Other foods that can cause FPIES include a wide range of solid food stuffs, such as grains, vegetables, fruits, and poultry. Food-borne enterocolitis is usually accompanied by acute recurring vomiting and diarrhea, lethargy, pallor, dehydration, and even hypovolemic shock. FPIES often occurs after the first introduction of complementary foods containing trigger products, usually not accompanied by fever or a significant increase in the level of C-reactive protein, and generally has a good prognosis. Depending on the severity of the disease, metabolic acidosis and meth-hemoglobinemia may develop. In chronic cases anemia, hypoalbuminemia and eosinophilia may occur. In acute cases laboratory evaluation may reveal thrombocytosis and neutrophilia, peaking 6 hours after a meal. Manifestations of FPIES usually disappear within 24-48 hours after elimination of the causative food. Radiological evaluation and other methods like endoscopy and gastric juice analysis can yield nonspecific results. Data on the incidence of FPIES is limited, and approximate assessment of affected children rate varies from 1.5 to 30 per 10,000. Further studies are needed to identify clinical subtypes and predisposing factors for the development of FPIES compared to immediate-type IgE-mediated gastroenteropathy.

Background Food protein–induced enterocolitis syndrome (FPIES) is an infrequent non–IgE-mediated gastrointestinal allergic disorder that occurs mostly in infants and young children. FPIES food triggers vary among different geographic locations, and the condition is still underdiagnosed and underrecognized. Objective To identify the triggers, characteristics, and management of FPIES in a pediatric US population of 74 children presenting to a tertiary center during a 3-year period. Methods We performed a retrospective electronic record review of all pediatric patients with a diagnosis of FPIES who presented to Texas Children's Hospital emergency centers and clinics. Results Most of our patients were white, and 65% had a positive family history of atopy. The median age at the first FPIES episode was 5 months (interquartile range, 4-6 months), and the median age at diagnosis was 11 months (interquartile range, 7-16 months). Grains (88%), cow's milk (49%), and vegetables (43%) were the most common food triggers in our cohort. Of the fruits, banana (24%) and avocado (16%) were predominantly reported. More than half of our patients experienced FPIES to multiple food triggers. Conclusion In our cohort, rice (53%) was the most common individual food trigger, surpassing cow's milk and soybean, previously reported as the most prevalent FPIES triggers in the United States. Banana (24%) and avocado (16%) rates were also much higher than in other studied populations, likely a reflection of different dietary and weaning habits in our area. Time from disease presentation to diagnosis was delayed, potentially because of difficulties in disease recognition. We noted a significant percentage of multiple-food FPIES in contrast to other populations.

Food protein-induced enterocolitis syndrome (FPIES) is a non-IgE-mediated food allergy that has been well-characterized clinically, yet it is still poorly understood. Acute FPIES is characterized by vomiting 1-4 h and/or diarrhea within 24 h after ingestion of a culprit food. Chronic FPIES is the result of chronic exposure to an offending food that can result in chronic watery diarrhea, intermittent vomiting, and failure to thrive. FPIES typically presents in infancy and self-resolves by school age in most patients. Adult-onset FPIES is rare, but it has been reported. Cow's milk and soy are the most common triggering foods in infants in the US, and as solids are introduced in the diet, FPIES reactions to grains (rice, oat) increase in prevalence. Variability in common trigger foods exists depending on the geographical origin-for example, fish is a frequent trigger in Spanish and Italian patients. Heavy reliance on a detailed history is required for the diagnosis as physical exam findings, laboratory tests, and/or imaging studies are suggestive and not specific for FPIES. Oral food challenges remain the gold standard for confirming diagnosis, and the challenge protocol may be for an individual depending on risk of reaction, prior reaction severity, and positive-specific IgE status. The recent development of diagnostic criteria in 2017 will serve to increase recognition of the disorder and allow for early implementation of management strategies. Acute management during reactions includes IV hydration, anti-emetics, and IV corticosteroids. Reaction prevention strategies include strict food avoidance until the physician deems a food reintroduction challenge clinically appropriate. Future efforts in FPIES research should be aimed at elucidating the underlying disease mechanisms and possible treatment targets.

Background Food protein–induced enterocolitis syndrome (FPIES) is a non–IgE-mediated food allergy that is diagnosed based on clinical findings, but can be confirmed with oral food challenge (OFC). OFC is more often performed to assess the development of tolerance. Most studies describing OFCs in FPIES are limited in size. Objective We sought to describe our experience with OFCs using our FPIES protocol. Patients were given one-third of serving size with a 4-hour observation period, followed by home titration to full dose. Methods We conducted a retrospective chart review of patients who underwent OFC via the FPIES protocol from 2014 to 2017. Data regarding the history of reaction, age at the time of challenge, and reactions during challenge or with home introduction were collected. Results A total of 169 OFCs were completed under the FPIES protocol, in 119 patients to 19 different foods. Thirty challenges (18%) were positive, with 17 challenges (10%) during initial challenge and 13 (7.7%) during home dosing. Most reactions during initial challenge required intravenous fluids (IVF), but hypotension was uncommon. One hundred thirty-nine (82%) OFCs were negative with home introduction, indicating tolerance to the challenged foods. The mean age of passing a challenge to milk, soy, and grain was earlier than that of other solid foods. Conclusions: Our data suggest that our FPIES OFC protocol is safe. Early administration of IVF may prevent the development of hypotension. It is difficult to stratify the risk of severe or delayed reaction based on patient characteristics, and more data are needed to identify those appropriate for home introduction.

Objective The aim of the study was to describe clinical, epidemiological, and management characteristics of food protein-induced enterocolitis syndrome (FPIES) cases in Spain. Patients and methods Multicenter observational retrospective study. FPIES cases diagnosed in specialized units in Spain over 12 months in 2017 (January-December) according to the recently published international diagnostic criteria were included. Results One hundred twenty patients (53.3% boys) were included. The majority were acute cases (111) with mild-to-moderate severity (76.7%). Triggering foods were cow's milk (48/120), fish (38), egg (13), rice (12), and soy (1). The majority (84.2%) of the patients had FPIES to 1 food only. In addition to vomiting (100%), pallor (89.2%), and altered behavior (88.3%) were most frequently observed in acute forms. On the contrary, diarrhea (70%), abdominal distension (33.3%), and blood in stools (44.4%) were more frequently observed in chronic cases. Oral challenge was performed in 18.9% of the acute forms compared to 44.4% of the chronic forms. The most common treatment was intravenous fluids followed by ondansetron. Corticosteroids were used in 6 patients (5 with acute symptoms and 1 chronic). Seven patients were treated with antibiotics for suspicion of infection. Most cases of cow's milk FPIES were treated with extensively hydrolyzed formulas (69.8%). Conclusions FPIES is not uncommon in our units. Unlike other published series, fish and egg are important triggers in our country. A greater knowledge and diffusion of the international consensus criteria will allow a better characterization of the cases and a standardization of their management.

Background: Data on the prevalence and clinical course of food protein-induced enterocolitis syndrome (FPIES) vary between populations and according to the culprit food. Objective: To evaluate the incidence, clinical characteristics, and remission patterns of FPIES among children in a Greek pediatric allergy referral center. Methods: We retrospectively studied children with acute FPIES. Data on age, sex, type of reaction, the implicated food, and oral food challenge (OFC) outcomes at baseline and upon reevaluation were analyzed. Results: Between October 2010 and March 2017, 72 (38 males) out of 15,114 subjects who had been referred to our department due to any reported allergic symptoms were diagnosed with acute FPIES. The most commonly implicated foods were cow’s milk (CM) (45.8%), fish (34.7%), rice (9.7%), egg (6.9%), and chicken (2.8%). The mean age at diagnosis was 7.1/19.3/9.1/18.7/8.7 months for those with CM/fish/rice/egg/chicken FPIES, respectively. Sixty-nine OFCs were performed, of which 8 were diagnostic and 61 for tolerance evaluation. The type of culprit food was significantly associated with the outcome of the tolerance OFCs. OFCs to fish resulted positive at a significantly higher rate (12/22; 54.5%) than OFCs to CM (4/29; 13.7%), rice (1/5; 20%), egg (0/3; 0%), and chicken (0/2; 0%) (p = 0.01). The time period between diagnosis and tolerance acquisition was prolonged in the fish FPIES cases (74.8 months; 95% CI: 57.9–91.6) compared to that with other foods such as CM (20.7 months; 95% CI: 17.3–24.1), rice (31.8 months; 95% CI: 21.9–41.7), and egg (24.3 months; 95% CI: 10.7–37.9), as shown in a Kaplan-Meier analysis (log-rank, p < 0.001). When the fish FPIES children were assessed for tolerance, OFCs were significantly more often positive than in CM FPIES children (52 vs. 18.1%; p = 0.03), despite the fact that the children were challenged at an older age (fish: 70.4 months, 95% CI: 58.3–82.5, vs. CM: 26.57 months, 95% CI: 21.1–32, p < 0.001). Conclusions: Acute FPIES had a low incidence in our population. CM and fish were the two most frequent elicitors. Significantly delayed presentation and prolonged remission was noted for FPIES caused by fish.

Background: Food protein-induced enterocolitis syndrome (FPIES) is a non-IgE-mediated food allergy. The pathological mechanism of FPIES is intestinal inflammation, and cell-mediated hypersensitivity is presumed to play an important role in its development. Case Report: The first case in which significant fetal intestinal distension suggested fetal onset of FPIES is reported. A 2,334-g male was born at 34 weeks by vaginal delivery. Results: In utero, he had significant intestinal distension on ultrasonography and MRI. A few hours after the first feeding, he produced bloody stool and showed abdominal distension. In this case, FPIES was not only caused by cow’s milk protein diagnosed clinically and by an allergen-specific lymphocyte stimulation test, but also by breast milk diagnosed by oral food challenge. The clinical course and laboratory results strongly suggested not only fetal sensitization but also fetal onset. Conclusion: This report might be helpful for prompt diagnosis and treatment and, furthermore, lead to elucidation of the pathogenesis and pathophysiology of FPIES.

Food protein-induced enterocolitis syndrome (FPIES) is a rare non-immunoglobulin E-mediated food allergy with necrotizing enterocolitis (NEC)-like symptoms which requires differential diagnosis as treatments differ.To evaluate the clinical, laboratory, and radiologic findings that differentiate FPIES from NEC in preterm and term infants.Clinical features, comorbidities, and laboratory and radiologic findings of neonates with presumed NEC were reviewed retrospectively and compared between FPIES and NEC in preterm and term infants who were admitted to the neonatal intensive care unit at Seoul National University Bundang Hospital between May 2003 and February 2020.A total of 10 of 150 (6.7%) preterm and 17 of 38 (44.7%) term infants with presumed NEC were confirmed to have FPIES; the remainder had NEC by modified Bell's criteria. Demographics and comorbidities were similar between these groups. Symptoms such as hematochezia, shock, leukocytosis, peripheral eosinophilia, and feeding of extensively hydrolyzed milk formula or elemental formula after discharge were significantly different between the 2 groups in term infants (P.05), but not in preterm infants. On abdominal ultrasonography, pneumatosis intestinalis was more common among preterm FPIES (44.4%) than NEC cases (21.6%) (P =.04). Among the preterm infants, 4 FPIES (40%) and 25 NEC (17.9%) cases required surgery (P =.10).Differentiating FPIES in neonates suspected of having NEC is important as dietary elimination of the triggering milk protein can be recommended instead of prolonged fasting and antibiotic treatment, which are indicated for NEC, in both term and preterm infants.

Acute food protein-induced enterocolitis syndrome ('FPIES') is a potentially severe type of non IgE-mediated food allergy affecting mainly infants usually when foods are introduced [1]. Acute FPIES triggered by multiple unrelated foods ('multiple food FPIES') has been reported in up to two thirds of patients, particularly in the USA [2,3]. FPIES reactions are often traumatic experiences for parents and weaning leads to significant anxiety, as there is no test to identify safe new foods. This has led to complex weaning recommendations in children with FPIES in an attempt to support parents [4]. However, evidence suggests that multiple food FPIES is rare in other regions, such as Southern Europe [5,6], which questions the applicability of such weaning advice in this population. Studies including a detailed dietary history in children with FPIES are lacking.