Oral desensitization in egg acute food protein-induced enterocolitis syndrome.

OBJECTIVE: Strict avoidance of trigger food is the primary management of food protein-induced enterocolitis syndrome (FPIES). No published data are available on active induction of tolerance with oral desensitization (OD) in FPIES. CASE REPORT: We carried out an OD in a 9 and a half years old boy with persistent acute egg FPIES. OD was performed with increasing doses of raw egg every week, starting with an initial dose of 0.2 ml. The boy presented mild and transient gastrointestinal adverse reactions when the 4 ml dose was reached. He could tolerate a whole raw egg in less than 14 months. CONCLUSIONS: Even though randomized controlled clinical trials on patients including various phenotypes of FPIES are needed, our experience is encouraging about the possible efficacy and safety of OD in this food allergy.

The practical dietary management of food protein-induced enterocolitis syndrome.

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.

Alternative Fish Species for Nutritional Management of Children with Fish-FPIES-A Clinical Approach.

In the Mediterranean region, fish is a common cause of food protein-induced enterocolitis syndrome (FPIES) in children. No laboratory tests specific to FPIES are available, and oral food challenge (OFC) is the gold standard for its diagnosis and testing for achievement of tolerance. Children with FPIES to fish are usually advised to avoid all fish, regardless of the species. Fish are typically classified into bony and cartilaginous, which are phylogenetically distant species and therefore contain less cross-reacting allergens. The protein ß-parvalbumin, considered a pan-allergenic, is found in bony fish, while the non-allergenic α-parvalbumin is commonly found in cartilaginous fish. Based on this difference, as a first step in the therapeutic process of children with FPIES caused by a certain fish in the bony fish category (i.e., hake, cod, perch, sardine, gilthead sea bream, red mullet, sole, megrim, sea bass, anchovy, tuna, swordfish, trout, etc.), an OFC to an alternative from the category of cartilaginous fish is suggested (i.e., blue shark, tope shark, dogfish, monkfish, skate, and ray) and vice versa. Regarding the increased mercury content in some sharks and other large species, the maximum limit imposed by the European Food Safety Authority (EFSA) for weekly mercury intake must be considered. An algorithm for the management of fish-FPIES, including alternative fish species, is proposed.

Recomendaciones para el diagnóstico y el tratamiento de los síntomas posquirúrgicos persistentes en la enfermedad de Hirschsprung / Recommendations for the diagnosis and treatment of persistent postsurgical symptoms in Hirschsprung disease

La enfermedad de Hirschsprung ocurre en 1 de cada 5000 nacimientos. La falla de migración de las células ganglionares desde la cresta neural en dirección cefalocaudal genera su ausencia en parte o todo el colon. Se manifiesta con falta de eliminación de meconio, distensión abdominal y dificultades en la evacuación. Luego del tratamiento quirúrgico, existen complicaciones a corto y largo plazo. El objetivo de esta publicación es describir las principales causas de síntomas persistentes en los pacientes operados por enfermedad de Hirschsprung y presentar un algoritmo diagnóstico-terapéutico factible de ser realizado en nuestro medio

Hirschsprung disease is characterized by the lack of migration of intrinsic parasympathetic ganglia from neural crest and consequently absence of them at varying length of the bowel, resulting in functional obstruction. The incidence is 1 per 5000 births. After surgery, short term and long term comorbidity commonly occurs. The aim of this article is to revise the main causes of ongoing symptoms after surgery in Hirschsprung disease patients and to show a diagnostic and therapeutic algorithm that can be developed in our community

Cooking influence in tolerance acquisition in egg-induced acute food protein enterocolitis syndrome

Background: Few studies on the age of resolution of Food Protein Induced Enterocolitis Syndrome (FPIES) induced by solid foods are available. In particular, for FPIES induced by egg, the mean age of tolerance acquisition reported in the literature ranges from 42 to 63 months. Objective: We have assessed whether the age of tolerance acquisition in acute egg FPIES varies depending on whether the egg is cooked or raw. Methods: We conducted a retrospective and multicentric study of children with diagnosis of acute egg FPIES seen in 10 Italian allergy units between July 2003 and October 2017. The collected data regarded sex, presence of other allergic diseases, age of onset of symptoms, kind and severity of symptoms, cooking technique of the ingested egg, outcome of the allergy test, age of tolerance acquisition. Results: Sixty-one children with acute egg FPIES were enrolled, 34 (56%) males and 27 (44%) females. Tolerance to cooked egg has been demonstrated by 47/61 (77%) children at a mean age of 30.2 months. For 32 of them, tolerance to raw egg has been demonstrated at a mean age of 43.9 months. No episodes of severe adverse reaction after baked egg ingestion have been recorded. Conclusions: It is possible to perform an OFC with baked egg, to verify the possible acquisition of tolerance, at about 30 months of life in children with acute egg FPIES

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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.

Cooking influence in tolerance acquisition in egg-induced acute food protein enterocolitis syndrome.

BACKGROUND: Few studies on the age of resolution of Food Protein Induced Enterocolitis Syndrome (FPIES) induced by solid foods are available. In particular, for FPIES induced by egg, the mean age of tolerance acquisition reported in the literature ranges from 42 to 63 months. OBJECTIVE: We have assessed whether the age of tolerance acquisition in acute egg FPIES varies depending on whether the egg is cooked or raw. METHODS: We conducted a retrospective and multicentric study of children with diagnosis of acute egg FPIES seen in 10 Italian allergy units between July 2003 and October 2017. The collected data regarded sex, presence of other allergic diseases, age of onset of symptoms, kind and severity of symptoms, cooking technique of the ingested egg, outcome of the allergy test, age of tolerance acquisition. RESULTS: Sixty-one children with acute egg FPIES were enrolled, 34 (56%) males and 27 (44%) females. Tolerance to cooked egg has been demonstrated by 47/61 (77%) children at a mean age of 30.2 months. For 32 of them, tolerance to raw egg has been demonstrated at a mean age of 43.9 months. No episodes of severe adverse reaction after baked egg ingestion have been recorded. CONCLUSIONS: It is possible to perform an OFC with baked egg, to verify the possible acquisition of tolerance, at about 30 months of life in children with acute egg FPIES.

Prevalence, Risk Factors, and Prognosis of Postoperative Complications after Surgery for Hirschsprung Disease.

BACKGROUND: Although most of patients do well after surgery for Hirschsprung disease (HSCR), there are complications in some instances that impact social aspects and quality of life. The aim of this study was to explore the prevalence, risk factors, and prognosis of these complications, providing guidance for surgeons and healthcare personnel. METHODS: A cohort of patients (N = 229) was retrospectively reviewed in the aftermath of surgery for HSCR. All medical data and operative notes were assessed. Early and late postoperative complications were solicited by questionnaire, using logistic regression and the Cox proportional hazards regression model for analysis. RESULTS: A total of 181 patients qualified for the study. Enterocolitis and soiling/incontinence constituted the most frequent complications, whether early or late in the postoperative period. Risk factors for developing enterocolitis included low weight, low-level IgA, preoperative enterocolitis, and lengthy aganglionic segment in the early term; whereas preoperative enterocolitis and diet control impacted complications emerging later. Risk factors in early soiling/incontinence were low weight, operative age of < 2 months, low IgA level, and lengthy aganglionic segment. Lengthy aganglionic segment, operative age of < 2 months, and toilet training were factors long-term. Prognostic factors included diet control and toilet training. CONCLUSION: Enterocolitis and soiling/incontinence remain the most frequent complications after surgery for HSCR. Risk factors in early and late postoperative periods differed, with diet control and toilet training contributing favorably to enterocolitis and soiling/incontinence, respectively.

Non-IgE-mediated gastrointestinal food allergies in children.

Non-IgE-mediated gastrointestinal food allergic disorders (non-IgE-GI-FA) including food protein-induced enterocolitis syndrome (FPIES), food protein-induced enteropathy (FPE), and food protein-induced allergic proctocolitis (FPIAP) are relatively uncommon in infants and young children, but are likely under-diagnosed. Non-IgE-GI-FA have a favorable prognosis, with majority resolving by age 3-5 years. Diagnosis relies on the recognition of symptoms pattern in FPIAP and FPIES and biopsy in FPE. Further studies are needed for a better understanding of the pathomechanism, which will lead eventually to the development of diagnostic tests and treatments. Limited evidence supports the role of food allergens in subsets of constipation, gastroesophageal reflux disease, irritable bowel syndrome, and colic. The immunologic pathomechanism is not fully understood and empiric prolonged avoidance of food allergens should be limited to minimize nutrient deficiency and feeding disorders/food aversions in infants.

Severe Food Protein-Induced Enterocolitis Syndrome to Cow's Milk in Infants.

Cow's milk is the most common cause of food-protein-induced enterocolitis syndrome (FPIES). The aim of this study was to examine the clinical features and treatment outcomes of infants with severe FPIES to cow's milk. We reviewed all infants ≤ 12 months of age who were hospitalized and diagnosed with severe FPIES to cow's milk between 1 January 2011 and 31 August 2014 in a tertiary Children's Medical Center in China. Patients' clinical features, feeding patterns, laboratory tests, and treatment outcomes were reviewed. A total of 12 infants met the inclusion criteria. All infants presented with diarrhea, edema, and hypoalbuminemia. Other main clinical manifestations included regurgitation/vomiting, skin rashes, low-grade fever, bloody and/or mucous stools, abdominal distention, and failure to thrive. They had clinical remission with resolution of diarrhea and significant increase of serum albumin after elimination of cow's milk protein (CMP) from the diet. The majority of infants developed tolerance to the CMP challenge test after 12 months of avoidance. In conclusion, we reported the clinical experience of 12 infants with severe FPIES to cow's milk, which resulted in malnutrition, hypoproteinemia, and failure to thrive. Prompt treatment with CMP-free formula is effective and leads to clinical remission of FPIES in infants.

Collagenous enterocolitis manifesting as watery diarrhoea and iron-deficiency anaemia.

A 56-year-old Caucasian woman presented with epigastric pain, watery diarrhoea, bloating and flatulence following treatment with duloxetine and venlafaxine for anxiety and depression. Abdominal examination was benign. Blood work revealed haemoglobin of 96 g/L (115-160 g/L), iron 6 µmol/L (10-33 µmol/L), transferrin saturation 0.08 (0.20-0.55), ferritin 26 µg/L (15-180 µg/L), albumin 46 g/L (35-50 g/L), pre-albumin 293 mg/L (170-370 mg/L), total IgA 2.64 g/L (0.78-3.58 g/L) and anti-tTG IgA 5 units (<20 units). Faecal occult blood tests were 3/3 positive and stool cultures were negative. CT enterography was normal. Colonic biopsy revealed collagenous colitis, while duodenal biopsy showed collagenous sprue with blunted to completely flattened villi and markedly thickened subepithelial collagen table entrapping capillaries and lymphocytes. The patient started a gluten-free diet, loperamide and ferrous gluconate. Her symptoms resolved and a faecal immunochemical test performed 6 months later was negative.

Gastrointestinal food allergies.

Gastrointestinal food allergies present during early childhood with a diverse range of symptoms. Cow's milk, soy and wheat are the three most common gastrointestinal food allergens. Several clinical syndromes have been described, including food protein-induced enteropathy, proctocolitis and enterocolitis. In contrast with immediate, IgE-mediated food allergies, the onset of gastrointestinal symptoms is delayed for at least 1-2 hours after ingestion in non-IgE-mediated allergic disorders. The pathophysiology of these non-IgE-mediated allergic disorders is poorly understood, and useful in vitro markers are lacking. The results of the skin prick test or measurement of the food-specific serum IgE level is generally negative, although low-positive results may occur. Diagnosis therefore relies on the recognition of a particular clinical phenotype as well as the demonstration of clear clinical improvement after food allergen elimination and the re-emergence of symptoms upon challenge. There is a significant clinical overlap between non-IgE-mediated food allergy and several common paediatric gastroenterological conditions, which may lead to diagnostic confusion. The treatment of gastrointestinal food allergies requires the strict elimination of offending food allergens until tolerance has developed. In breast-fed infants, a maternal elimination diet is often sufficient to control symptoms. In formula-fed infants, treatment usually involves the use an extensively hydrolysed or amino acid-based formula. Apart from the use of hypoallergenic formulae, the solid diets of these children also need to be kept free of specific food allergens, as clinically indicated. The nutritional progress of infants and young children should be carefully monitored, and they should undergo ongoing, regular food protein elimination reassessments by cautious food challenges to monitor for possible tolerance development.

Nutritional management of food protein-induced enterocolitis syndrome.

PURPOSE OF REVIEW: To summarize the latest information on the nutritional management of food protein-induced enterocolitis syndrome (FPIES), focusing on the foods implicated and how to avoid these whilst maintaining a nutritionally sound diet. RECENT FINDINGS: A number of foods are implicated in FPIES such as milk, soy and grains, particularly rice. The number of foods implicated in FPIES per individual differs, but the majority of reported cases have two or fewer food triggers involved. SUMMARY: FPIES is a complex presentation of non-IgE-mediated food allergy. Dietary management is complicated as both common food allergens as well as atypical food allergens can trigger FPIES. Sound nutritional advice is required to ensure appropriate food avoidance, adequate consumption of other foods and sufficient nutritional intake to maintain and ensure growth and development.

Food protein-induced enterocolitis syndrome due to banana: an uncommon entity.

Food protein-induced enterocolitis syndrome is a non-IgE-mediated food allergy that typically occurs within the first year of age and it is often misdiagnosed for its rarity. This syndrome is usually caused by milk or soy in formula-fed infants, but it can also be associated to solid food proteins, fruit proteins included. We describe and discuss the first case of an infant with mild acute/lateform ofFPIES due to banana only.

Food protein-induced enterocolitis syndrome: 16-year experience.

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 <36 degrees C at presentation was recorded for 24% of episodes. A platelet count of >500 x 10(9) 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.