Author: V. Dimov, M.D., Allergist/Immunologist and Assistant Professor at University of Chicago
Reviewer: S. Randhawa, M.D., Allergist/Immunologist and Assistant Professor at NSU
Food Protein–Induced Enterocolitis Syndrome (FPIES) is a non–IgE-mediated food allergy that most commonly affects infants. Cow’s milk and soy FPIES resolve in most patients by 3 years of age, patients with solid food FPIES and/or detectable food specific IgE levels may have more protracted courses.
One approach to FPIES diagnosis is follow-up challenges every 18 to 24 months in patients without recent reactions.
Food Protein–Induced Enterocolitis Syndrome Oral Food Challenge Protocol. Source: Food protein–induced enterocolitis syndrome: an update on natural history and review of management. JACI, 08/2011.
- Basic requirements: physician supervision, secure intravenous (IV) access, immediate availability of fluid resuscitation
- Baseline vital signs and peripheral neutrophil count (repeat CBC-Diff in 6 hours)
- Gradual (during 1 hour) administration of food protein 0.06 – 0.6 g/kg body weight in 3 equal doses, generally not to exceed total 3 g of protein or 10 g of total food (100 mL of liquid) for an initial feeding
- If no reaction in 2–3 hours, administer an age-appropriate serving of the food followed by several hours of observation
- Fluid resuscitation: 20 mL/kg IV boluses of normal saline
- Steroids: methylprednisolone, 1 mg/kg IV (maximum, 60 – 80 mg)
- Most patients (more than 50%) with positive challenge results require treatment with intravenous fluids and steroids
An FPIES OFC is considered a high-risk procedure and should be conducted in a setting where intravenous access can be secured and rapid fluid resuscitation given if a reaction occurs. Although an inpatient setting is the most appropriate for FPIES OFCs, outpatient settings equipped with resuscitation capabilities with access to a laboratory (for neutrophil counts and stool analysis) can be used.
For the first serving, doses are administered in 3 portions during 45– 60 minutes for a total of 0.06 to 0.6 g/kg of food protein, starting at a lower dose in patients with a history of severe reactions.
The total amount does not generally exceed 3 to 6 g or 10 to 20 g of total food weight (100 mL of total liquid). If the patient is asymptomatic after 2 to 3 hours, then an age-appropriate serving is usually given as a second serving and the patient is observed for several more hours. First-line therapy in the event of a positive challenge result is rapid intravenous hydration of normal saline given in 20 mL/kg boluses. Intravenous corticosteroids may also be used in severe reactions. Epinephrine should be available.
The challenge is considered positive if typical symptoms and laboratory findings are present:
- Emesis (onset, 1–3 hours)
- Diarrhea (onset, 2–10 hours; mean, 5 hours)
- Lethargy (onset, 1–3 hours)
- Laboratory findings:
Elevated neutrophil count (more than 3500/mcL, peaks at 6 hours)
Frank or occult fecal blood
Fecal leukocytes, red blood cells, and/or eosinophils
Gastric juice leukocytosis (>10 leukocytes per high-power field, at 3 hours)
A complete blood count with differential should be measured prior to challenge, and approximately 6 hours after challenge if there are symptoms. If diarrhea is present, stool guaiac test should be performed and stool samples tested for fecal leukocytes, red blood cells, and eosinophils.
References:
Food Protein–Induced Enterocolitis Syndrome Oral Food Challenge Protocol. Source: Food protein–induced enterocolitis syndrome: an update on natural history and review of management. JACI, 08/2011, http://goo.gl/LlwRU
Food Protein-Induced Enterocolitis Syndrome: Consensus Recommendations for Diagnosis and Treatment Japanese Research Group for Neonatal, Infantile Allergic Disorders, 2011, http://goo.gl/TmdFo
What is FPIES (Food Protein Induced Enterocolitis Syndrome)? http://goo.gl/fTP3O
Food protein-induced enterocolitis syndrome (FPIES) - AAAAI Ask the Expert http://goo.gl/J9aS5
Oral food challenges for diagnosis and management of food allergies - UpToDate http://goo.gl/LtRvE
Published: 05/12/2012
Updated: 08/09/2012
Showing posts with label FPIES. Show all posts
Showing posts with label FPIES. Show all posts
Food Protein Induced Enterocolitis Syndrome (FPIES)
Author: V. Dimov, M.D., Allergist/Immunologist and Assistant Professor, University of Chicago
Reviewer: S. Randhawa, M.D., Allergist/Immunologist, Fort Lauderdale, FL
What is FPIES?
FPIES is a rare (incidence 0.34%) non-IgE mediated condition (negative skin tests and sIgE) in breast fed infants. Food Protein Induced Enterocolitis Syndrome (FPIES) is a non-IgE mediated immune reaction in the gastrointestinal system to one or more specific foods, commonly characterized by profuse vomiting and diarrhea. Poor growth may occur with continual ingestion of the offending foods. Upon removing the problem food, all FPIES symptoms generally subside.
Pathology: What causes FPIES?
The mechanism of FPIES remains undefined, although tumor necrosis factor alpha is thought to play a role. Mediators released by T lymphocytes result in inflammation and vascular leakage in this non-IgE-mediated reaction. TNFα has also been implicated in food protein-induced enterocolitis (FPIES).
Adult non-IgE reactions to shellfish may be the same process as pediatric FPIES. Milk protein enteropathy looks similar to celiac disease, but it usually self-limiting.
Deficit in TGF-beta (and its receptors) may be important in development of FPIES. Increased TNF-alpha also seems to be important for FPIES.
Clinical features: When to suspect FPIES?
FPIES presents with vomiting and diarrhea, and may progress to shock. It starts early in infancy.
In a large birth cohort from Israel, the cumulative incidence for FPIES was 0.34%.
The most common symptoms were:
- recurrent vomiting (100%)
- lethargy (77%)
- diarrhea (25%)
- pallor (14%)
- bloody diarrhea (4.5%)
All patients had FPIES within the first 6 months of life.
By the age of 3 years, 90% of the patients had recovered. There is 50% resolution per year for milk-induced FPIES.
There were no concomitant reaction to soy.
The prevalence of FPIES is significant, and its clinical presentation is distinct from that of IgE-cow's milk allergy (CMA).
Most patients with FPIES recover, although a proportion might convert to IgE-CMA. The likelihood for a cross-reactivity to soy in this population was less than previously estimated.
How to diagnose FPIES?
Unfortunately, there is no diagnostic test for FPIES such as skin testing, serum specific IgE (sIgE) or patch testing.
Food challenges in FPIES
Diagnostic tests are lacking in food protein-induced enterocolitis (FPIES), a non–immunoglobulin E, presumably T cell–mediated food allergy disorder. FPIES typically presents in infancy with profuse vomiting and diarrhea 2 to 4 hours after ingestion of the allergen, combined with a left-shift in peripheral blood leukocytes, occasionally causing profound dehydration, hypotension, and lethargy. Chronic exposure results in failure to thrive and hypoalbuminemia. FPIES is elicited commonly by milk and soy protein, oat, rice, and other foods. Oral food challenge (OFC) is performed to follow tolerance development in FPIES, but protracted emesis and dehydration necessitate fluid resuscitation in up to 50% of reactive challenges. Therefore, OFC to confirm the diagnosis of FPIES can be associated with high risk (Annals of Allergy, Asthma & Immunology, 109:3, 221-222, Sep 2012, http://buff.ly/P4zn6d).
References
FPIES: Food Protein Induced Enterocolitis Syndrome. Patient Information: http://bit.ly/qad8q
The prevalence and natural course of food protein-induced enterocolitis syndrome to cow's milk:
A large-scale, prospective population-based study. J Allergy Clin Immunol. 2011 Mar;127(3):647-653.e3.
Related reading
Understanding FPIES Through Moms Sharing Their Stories. Nutricia Neocate blog, 2011.
International Association for Food Protein Enterocolitis (IAFFPE)
Food protein-induced enterocolitis syndrome (FPIES) - AAAAI Ask the Expert addresses clinical questions, 2012.
Possible food protein-induced enterocolitis syndrome (FPIES) - AAAAI Ask the Expert, 2013. http://bit.ly/16lcfY8
Published: 06/28/2010
Updated: 03/16/2013
Reviewer: S. Randhawa, M.D., Allergist/Immunologist, Fort Lauderdale, FL
What is FPIES?
FPIES is a rare (incidence 0.34%) non-IgE mediated condition (negative skin tests and sIgE) in breast fed infants. Food Protein Induced Enterocolitis Syndrome (FPIES) is a non-IgE mediated immune reaction in the gastrointestinal system to one or more specific foods, commonly characterized by profuse vomiting and diarrhea. Poor growth may occur with continual ingestion of the offending foods. Upon removing the problem food, all FPIES symptoms generally subside.
Pathology: What causes FPIES?
The mechanism of FPIES remains undefined, although tumor necrosis factor alpha is thought to play a role. Mediators released by T lymphocytes result in inflammation and vascular leakage in this non-IgE-mediated reaction. TNFα has also been implicated in food protein-induced enterocolitis (FPIES).
Adult non-IgE reactions to shellfish may be the same process as pediatric FPIES. Milk protein enteropathy looks similar to celiac disease, but it usually self-limiting.
Deficit in TGF-beta (and its receptors) may be important in development of FPIES. Increased TNF-alpha also seems to be important for FPIES.
Clinical features: When to suspect FPIES?
FPIES presents with vomiting and diarrhea, and may progress to shock. It starts early in infancy.
In a large birth cohort from Israel, the cumulative incidence for FPIES was 0.34%.
The most common symptoms were:
- recurrent vomiting (100%)
- lethargy (77%)
- diarrhea (25%)
- pallor (14%)
- bloody diarrhea (4.5%)
All patients had FPIES within the first 6 months of life.
By the age of 3 years, 90% of the patients had recovered. There is 50% resolution per year for milk-induced FPIES.
There were no concomitant reaction to soy.
The prevalence of FPIES is significant, and its clinical presentation is distinct from that of IgE-cow's milk allergy (CMA).
Most patients with FPIES recover, although a proportion might convert to IgE-CMA. The likelihood for a cross-reactivity to soy in this population was less than previously estimated.
How to diagnose FPIES?
Unfortunately, there is no diagnostic test for FPIES such as skin testing, serum specific IgE (sIgE) or patch testing.
Food challenges in FPIES
Diagnostic tests are lacking in food protein-induced enterocolitis (FPIES), a non–immunoglobulin E, presumably T cell–mediated food allergy disorder. FPIES typically presents in infancy with profuse vomiting and diarrhea 2 to 4 hours after ingestion of the allergen, combined with a left-shift in peripheral blood leukocytes, occasionally causing profound dehydration, hypotension, and lethargy. Chronic exposure results in failure to thrive and hypoalbuminemia. FPIES is elicited commonly by milk and soy protein, oat, rice, and other foods. Oral food challenge (OFC) is performed to follow tolerance development in FPIES, but protracted emesis and dehydration necessitate fluid resuscitation in up to 50% of reactive challenges. Therefore, OFC to confirm the diagnosis of FPIES can be associated with high risk (Annals of Allergy, Asthma & Immunology, 109:3, 221-222, Sep 2012, http://buff.ly/P4zn6d).
References
FPIES: Food Protein Induced Enterocolitis Syndrome. Patient Information: http://bit.ly/qad8q
The prevalence and natural course of food protein-induced enterocolitis syndrome to cow's milk:
A large-scale, prospective population-based study. J Allergy Clin Immunol. 2011 Mar;127(3):647-653.e3.
Related reading
Understanding FPIES Through Moms Sharing Their Stories. Nutricia Neocate blog, 2011.
International Association for Food Protein Enterocolitis (IAFFPE)
Food protein-induced enterocolitis syndrome (FPIES) - AAAAI Ask the Expert addresses clinical questions, 2012.
Possible food protein-induced enterocolitis syndrome (FPIES) - AAAAI Ask the Expert, 2013. http://bit.ly/16lcfY8
Published: 06/28/2010
Updated: 03/16/2013
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