Management of acute food protein-induced enterocolitis syndrome emergencies at home and in a medical facility

ObjectiveAcute food protein-induced enterocolitis syndrome (FPIES) is characterized by delayed repetitive vomiting after ingestion of a trigger food, and severe reactions may lead to dehydration, hypotension, and shock. We provide recommendations on management of FPIES emergencies in a medical facility and at home.Data SourcesThis review summarizes the literature on clinical context, pathophysiology, presentation, and treatment of FPIES emergencies.Study SelectionsWe referred to the 2017 International Consensus Guidelines for the Diagnosis and Management of FPIES and performed a literature search identifying relevant recent primary articles and review articles on clinical management.ResultsManagement of FPIES emergencies in a medical facility is based on severity of symptoms and involves rehydration, ondansetron, and corticosteroids. A proactive approach for reactions occurring at home involves prescribing oral ondansetron and providing an individualized treatment plan based on the evolution of symptoms and severity of past reactions. A better understanding of the pathophysiology of FPIES and randomized trials on ondansedron and cocorticosteroid use could lead to more targeted treatments.ConclusionChildren with FPIES are at risk for severe symptoms constituting a medical emergency. Management of FPIES emergencies is largely supportive, with treatment tailored to the symptoms, severity of the patient’s condition, location of reaction, and reaction history.     

Current understanding of the immune mechanisms of food protein-induced enterocolitis syndrome

Food protein-induced enterocolitis syndrome (FPIES) is an under-recognized and frequently misdiagnosed non-IgE-mediated food hypersensitivity disorder, characterized by severe vomiting and/or diarrhea. Despite the potential severity of acute reactions, FPIES can be considered self-limiting as avoidance of the incriminating allergen(s) leads to resolution of symptoms. Symptoms typically begin in the first month of life in association with failure to thrive and may progress to acidemia and shock. Although FPIES is well established as a distinct clinical entity, its pathophysiology has not yet been clearly defined and requires further characterization. Several immunologic alterations have been reported in FPIES, suggesting the involvement of antigen-specific T cells and their production of proinflammatory cytokines that regulate the permeability of the intestinal barrier. Humoral immune responses may also be involved in the pathomechanism of FPIES. The aim of this article is to delineate the immunological characteristics of this disorder based on the existing reports and to review the possible pathophysiologic basis of this disease.     

Current understanding of the immune mechanisms of food protein-induced enterocolitis syndrome

Primary prevention of allergy is a laudable goal, but one that has unfortunately proven difficult to achieve. Many different strategies have been reported to date, but unequivocal supporting data for any single strategy does not exist. Any successful strategy must lead to immunomodulation and must be encountered very early on life, likely in utero. Reports of early bacterial and farm animal exposures lend supportive data to the concept of immune regulation via early fetal exposure, howeve attempts at clinical applications of this, such as probiotics has not been completely successful. One practical, clinical method for achieving a similar immune modulation to these exposures would be providing atopic women with allergy immunotherapy while pregnant (or perhaps even preconception). Allergy immunotherapy is associated with favorable immune modulation and some data suggest that these changes if produced in mother can influence the atopic status of offspring.     

Food protein-induced enterocolitis syndrome,from practice to theory

Food protein-induced enterocolitis syndrome (FPIES) is an allergic disease, probably non-IgE-mediated, with expression predominantly in the GI tract. The most characteristic symptom is repeated, debilitating vomiting. It occurs 2?6 h after ingestion of culprit food and is usually accompanied by pallor and lethargy. There may be diarrhea, and in 10?20% of cases, severe hypotension. These symptoms resolve completely within a few hours. The food most frequently involved is cow’s milk, followed by rice, but many other foods may be involved. The prognosis is generally good in a few years. In this review the authors try to cope, with the help of some case histories, with the practical clinical aspects of FPIES. The authors also try to provide a management approach based on current knowledge, and finally, to point out the aspects of FPIES that are still controversial.     

Expression of transforming growth factor beta1, transforming growth factor type I and II receptors, and TNF-alpha in the mucosa of the small intestine in infants with food protein-induced enterocolitis syndrome.

BACKGROUND: TNF-alpha secreted by activated T cells is known to increase intestinal permeability, whereas transforming growth factor (TGF) beta has the ability to protect the epithelial barrier. OBJECTIVE: We determined the expression of TGF-beta1, its receptors, and TNF-alpha on the mucosa of small intestine to investigate their roles in the pathogenesis of food protein-induced enterocolitis syndrome (FPIES). METHODS: Twenty-eight infants diagnosed with FPIES by means of clinical criteria and challenge test results were included. Immunohistochemical stains for TGF-beta1, type 1 and 2 TGF-beta receptors, and TNF-alpha on duodenal biopsy specimens were performed. RESULTS: TGF-beta1 expression was generally depressed in patients. Expression of type 1 TGF-beta receptor was significantly lower in the patients who had villous atrophy compared with expression in those patients who did not (P <.001) and negatively correlated with the severity of atrophy (r = -0.59, P <.001). Expression of type 2 TGF-beta receptor showed no significant difference between the patients with or without villous atrophy. The immunoreactivity for both TGF-beta receptors on lamina proprial cells was slight or negative. TNF-alpha expression was detected on both epithelial and lamina proprial cells and was significantly greater in the patients who had villous atrophy compared with that in the patients who did not (P <.01). CONCLUSION: Our results suggest that decreased countering activity of TGF-beta1 against T-cell cytokines is implicated in the pathogenesis of FPIES. The significantly lower expression of type 1 TGF-beta receptor compared with type 2 receptor suggests the differential contribution of each receptor to the diverse biologic activities of TGF-beta in the intestinal epithelium.     

Emerging triggers of food protein–induced enterocolitis syndrome: Lessons from a pediatric cohort of 74 children in the United States

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.

Genetic analysis of HAV strains recovered from patients with acute hepatitis from Southern Italy

Southern Italy is an endemic area for HAV infection contributing to the majority of Italian hepatitis A cases. Using molecular analysis, HAV strains have been classified in distinct genotypes and subgenotypes. To characterize HAV wild-type strains circulating in Southern Italy, sequence analysis of VP3-VP1 and VP1/2A junction regions of HAV isolates recovered from 25 patients with acute hepatitis during 2000 and 2001 was carried out. HAV isolates showed a degree of identity, after pairwise comparison with one another, ranging from 91.9-100% in the VP3-VP1 junction region and 89.9-100% in the VP1/2A junction region. All strains belonged to genotype I, with 84% (21/25) of samples clustering in subgenotype IA and 16% (4/25) in subgenotype IB. Cocirculation of subgenotypes IA and IB was observed among isolates from 2000, whereas all strains from 2001 were subgenotype IA. In addition, the subgenotype IA strains formed different clusters, one of which was related closely to some Cuban strains, showing a percent similarity of 98.8% in the 168-base pair segment encompassing the VP1/2A junction and the same amino acid substitution. The latter finding suggests that this subgenotype variant circulates also in the Mediterranean area. The results of the phylogenetic analysis confirm the genetic heterogeneity among HAV strains in Western Europe.     

Adherence, enterotoxigenicity, invasiveness and serogroups in Campylobacter jejuni and Campylobacter coli strains from adult humans with acute enterocolitis

Two hundred Campylobacter jejuni and Campylobacter coli strains from the same number of adult Swedish patients with acute enterocolitis were tested regarding adherence to and invasiveness in HEp-2 cells and for enterotoxigenicity by the CHO-cell assay. The serogroup characteristics, heat-stable and heat-labile, for each strain were also investigated. Eighty-four percent of the strains were classified as C. jejuni and 16 percent as C. coli. All of the strains were adherent to HEp-2 cells, 39% were invasive and 31.5% enterotoxigenic. We found significantly more invasive strains in the non-enterotoxigenic group than in the enterotoxigenic one. There would seem to be no correlation between enterotoxigenicity or invasiveness and serogroup. The results of this study suggest the existence of multiple mechanisms for C. jejuni- and C. coli-induced diarrhoea and that the mechanisms may differ from one strain to another.     

A novel variant of the infectious bronchitis virus resulting from recombination events in Italy and Spain

Infectious bronchitis is considered to be one of the most devastating diseases in poultry. Control of its spread is typically attempted through biosecurity measures and extensive vaccination. However, the remarkable genetic and antigenic variability of the virus, which originate from both mutations and recombination events, represents an unsolved challenge for this disease. The present study reports on the emergence and spread of recombinant clusters detected in Italy and Spain between 2012 and 2014. A total of 36 Spanish and Italian infectious bronchitis virus (IBV) field strains were investigated and genetically characterized using phylogenetic, molecular, recombination and selection pressure analyses of the complete S1 gene. Based on the partial S1 sequencing, 27 IBV strains originating from Spain and nine from Italy were initially classified as being closely related to the Guandong/Xindadi (XDN) genotype. Phylogenetic analysis of the complete S1 gene revealed that the XDN strains formed a homogeneous clade with the Spanish IBV isolates within the QX genotype, whereas there was higher variability within the Italian strains. Recombination analysis determined that these strains belonged to four groups, which originated from independent recombination events between the QX and 793B IBV genotypes. Our data support the hypothesis of two different scenarios: firstly, in Spain, the large and homogeneous clade probably originated from a single offspring of the recombinant founder, which became dominant and spread throughout the country. Secondly, the nine Italian recombinants, which are characterized by three different recombination patterns, probably represent less fitted strains, because they were less viable with respect to their recombinant parents.