Management Of Food Protein-induced Enterocolitis Syndrome Research Articles

Current status and future directions in Food Protein-Induced Enterocolitis Syndrome (FPIES): An NIAID Workshop Report

Food Protein-Induced Enterocolitis (FPIES) is a non-IgE mediated GI food allergy characterized by delayed, protracted vomiting, accompanied by lethargy and pallor, usually 1-4 hours following ingestion of the food allergen. The pathophysiology of FPIES remains unknown and currently there are no diagnostic biomarkers available to assess disease activity or its resolution. Over the last two decades, FPIES has become increasingly recognized in both pediatric and adult patients. Forty years later after the initial FPIES description, the first international classification of diseases (ICD-10) code for FPIES was established and the first international consensus guidelines for diagnosis and management of FPIES was published. On June 22, 2022, the National Institute of Allergy and Infectious Diseases (NIAID) held its first virtual multidisciplinary workshop on FPIES. Various clinical and translational aspects of FPIES, as well as the important areas of unmet needs were discussed as priorities for future research during this 2-day virtual workshop. The following report provides a summary of content of the workshop, including updated literature on the topic areas, as well as providing a critical commentary on the state of FPIES.

Vivre avec le syndrome d’entérocolite induite par les protéines alimentaires (SEIPA) : impact sur la qualité de vie des patients et de leurs familles

The food protein-induced enterocolitis syndrome (FPIES) is a non-IgE-mediated food allergy, often underdiagnosed despite an increasing prevalence. This study aims to assess the impact of FPIES on the quality of life and identify risk factors for psychosocial distress. We collected data from a cohort of 48 pediatric patients with FPIES, recruited by private and hospital allergists through telephone interviews with families. Quality of life, assessed through QUALIN and Peds QL questionnaires, is generally preserved, but psychopathological aspects and sociability are affected. Parental concerns are significant, with 61% experiencing anxiety during the food diversification period. Regarding emotional well-being, 85% of parents express concerns, while FPIES induces stress in 75% of them. Family activity limitations affect 62% of families. FPIES impacts the social life of children (54%) and has a significant financial impact on 57% of families. Negative influences on quality of life include a history of hospitalization, the number of emergency room visits for FPIES, cow's milk as a triggering food, and FPIES to multiple foods. These results emphasize the importance of considering psychosocial dimensions in the overall management of FPIES.

Food protein-induced enterocolitis syndrome in adults: review and practice recommendations

Food protein-induced enterocolitis syndrome (FPIES) is an allergic disorder that manifests as reproducible gastrointestinal symptoms within hours of ingestion of the causative food, which can progress to dehydration and hypotension. Historically, FPIES has been recognized as a disease affecting the pediatric population but it can also develop de novo in adults. The pathophysiology is not well understood; however, the local adaptive immune system and gene expression linked to innate immune activation are implicated. Adult-onset FPIES has some differences with pediatric FPIES. Vomiting may be absent, while abdominal pain is the most common manifestation. A clear predominance in women occurs, being seafood the most common trigger, although many other foods have also been implicated. Diagnosis of adult-onset FPIES is based on a thorough clinical history but in many cases, it should be followed by an oral food challenge (OFC), due to the absence of vomiting in some patients and the lack of confirmatory diagnostic test. The first-line treatment for acute FPIES reactions is fluid replacement, by the oral route in mild to moderate reactions or via the intravenous route in severe reactions. Ondansetron may be effective in shortening the duration of emesis. Management of patients after diagnosis includes dietary advice and follow-up with supervised OFC at regular intervals to monitor for resolution. Tolerance to the trigger food in children is commonly achieved, a finding not so common in adult-onset FPIES. The aim of this article is to review the most important current concepts in epidemiology, pathophysiology, diagnosis, and management of FPIES.

Clinical presentation and management of food protein-induced enterocolitis syndrome in 113 Swedish children.

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. 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. 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 3.9months for cow's milk and 6.0months for other foods (p<0.001, range 1.0month to 9years). Repetitive vomiting (100%), lethargy (86%), and pallor (61%) were common symptoms; 40% had diarrhea. Sixty percent visited the emergency department, and 27% of all patients were hospitalized. Most patients were diagnosed clinically (81%). Specific IgE for the trigger food was positive in 4/89 tested patients (4%), and skin prick test for the trigger food was positive in 1/53 tested patients (2%). In our Swedish study of 113 children, cow's milk, fish, and oat were the commonest trigger foods. Most patients reacted to a single food, and IgE sensitization was rare.

Food Protein-Induced Enterocolitis Syndrome

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 supervisedoral food challenges to monitor for resolution. There are no strategies to accelerate development of tolerance in FPIES.Herewe review the most important current concepts in epidemiology,pathophysiology, diagnosis, and management of FPIES.

Food Protein-Induced Enterocolitis Syndrome: Proposals for New Definitions.

Acute food protein-induced enterocolitis syndrome (FPIES) is a non-IgE-mediated allergy and is characterized by repetitive profuse vomiting episodes, often in association with pallor, lethargy, and diarrhea, presenting within 1–4 h from the ingestion of a triggering food. In 2017, the international consensus guidelines for the diagnosis and management of FPIES were published. They cover all aspects of this syndrome, which in recent decades has attracted the attention of pediatric allergists. In particular, the consensus proposed innovative diagnostic criteria. However, the diagnosis of acute FPIES is still currently discussed because the interest in this disease is relatively recent and, above all, there are no validated panels of diagnostic criteria. We propose some ideas for reflection on the diagnostic and suspicion criteria of acute FPIES with exemplary stories of children certainly or probably suffering from acute FPIES. For example, we believe that new definitions should be produced for mild forms of FPIES, multiple forms, and those with IgE-mediated symptoms. Moreover, we propose two clinical criteria to suspect acute FPIES and to refer the child to the diagnostic oral food challenge.

Food protein-induced enterocolitis syndrome: guidelines summary and practice recommendations.

Food protein-induced enterocolitis syndrome (FPIES) is a poorly understood non-IgE gastrointestinal-mediated food allergy that predominantly affects infants and young children. Cells of the innate immune system appear to be activated during an FPIES reaction. Acute FPIES typically presents between one and 4 hours after ingestion of the trigger food, with the principal symptom being profuse vomiting, and is often accompanied by pallor and lethargy. Additional features can include hypotension, hypothermia, diarrhoea, neutrophilia and thrombocytosis. In Australia, the most commonly reported foods responsible for FPIES are (in descending order) rice, cow's milk, egg, oats and chicken. Most children with FPIES react to only one food trigger, and thus, avoidance of multiple foods is often not indicated. FPIES is often misdiagnosed as sepsis or gastroenteritis. However, a diagnosis of FPIES is favoured if there is rapid resolution of symptoms within hours of presentation, an absence of fever, and a lack of a significant rise in C-reactive protein at presentation. Diagnosis is often hampered by the lack of awareness of FPIES, absence of reliable biomarkers, the non-specific nature of the presenting symptoms, and the delay between allergen exposure and symptoms. Although some national peak allergy bodies have attempted to improve the diagnosis and management of FPIES, up until 2017 there were no internationally agreed guidelines for its diagnosis and management.

Food Protein-Induced Enterocolitis Syndrome.

Food protein-induced enterocolitis syndrome (FPIES) is a non-IgE-, cell-mediated food allergy of unknown prevalence and pathophysiology. Onset is typically during the first year of life; seafood-induced FPIES may start in adulthood. Acute FPIES manifests within 1-4 hours after ingestion with repetitive emesis, pallor, and lethargy progressing to dehydration and hypovolemic shock in 15% of cases. Chronic FPIES manifests with intermittent emesis, watery diarrhea, and poor growth progressing to dehydration and hypovolemic shock over a period of days to weeks. Chronic FPIES has been only reported in infants aged less than 3 months fed with cow milk (CM) or soy formula. The most common triggers are CM, soy, rice, and oat. Diagnosis of FPIES relies on recognition of a pattern of clinical symptoms and may be missed owing to the absence of typical allergic symptoms (eg, urticaria, wheezing) and delayed onset in relation to food ingestion. Physician-supervised food challenge is recommended if diagnosis or the trigger food is not clear and to evaluate for resolution. Testing for food-specific IgE is usually negative, although a subset of patients, usually with CM-induced FPIES may develop sensitization to foods. Such atypical FPIES tends to have a more prolonged course. Despite the potential severity of the reactions, no fatalities have been reported, and FPIES has a favorable prognosis. In most cases, FPIES resolves by age 3-5 years, although persistence of CM-induced FPIES and soy FPIES into adulthood has been reported. The first international consensus guidelines on diagnosis and management of FPIES were published in 2017. Given that the pathophysiology of FPIES is poorly understood, there are no diagnostic biomarkers and no therapies to accelerate resolution. These unmet needs warrant future investigations to improve the care of patients with FPIES.

What is New in the Diagnosis and Management of Food Protein-Induced Enterocolitis Syndrome?

Purpose of Review Many aspects of food protein-induced enterocolitis syndrome (FPIES) remain poorly studied, such as its pathophysiology, dietary management, biomarkers for diagnosis and onset of tolerance. Research in the last decade has steadily advanced our knowledge of the condition. We review the recent literature in relation to the diagnosis and management of FPIES.

Definition, etiology, and diagnosis of food protein-induced enterocolitis syndrome

Purpose of reviewFood protein-induced enterocolitis syndrome (FPIES) is a poorly understood non-IgE-mediated food hypersensitivity, primarily affecting infants and toddlers. There are few data regarding pathophysiology of FPIES that suggest local intestinal imbalance between TNF-α and TGF-β. Patients frequently present with multiple reactions, which are characterized by projectile, repetitive emesis, dehydration, lethargy, and failure to thrive. Despite the severity of presentation, the diagnosis is frequently delayed, and patients often undergo extensive and invasive evaluation prior to reaching the diagnosis.Recent findingsReviews published in the last year provide a general approach to diagnosis and management of FPIES and aim to increase awareness and understanding of FPIES among general pediatricians.SummaryMulticenter studies are necessary to reevaluate and modify the oral food challenge criteria. Research on the pathophysiology of FPIES reactions is necessary to provide insight into the evidence-based approach to diagnosis and management of FPIES. Registries are needed to understand the phenotype, triggers, and prevalence of FPIES.