Food Protein-Induced Enterocolitis Syndrome

National Organization for Rare Disorders, Inc.

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Important
It is possible that the main title of the report Food Protein-Induced Enterocolitis Syndrome is not the name you expected.

Disorder Subdivisions

  • None

General Discussion

Summary
Food protein-induced enterocolitis syndrome (FPIES) is an uncommon disorder characterized by an allergic reaction to food that affects the gastrointestinal system. The term enterocolitis specially refers to inflammation of the small and large intestines. Individuals with FPIES experience profuse vomiting and diarrhea that usually develops approximately 2-6 hours after ingesting the offending food. Additional symptoms include pallor, lethargy, and abdominal swelling (distension). Symptoms can be severe and can potentially cause acute dehydration and/or hypovolemic shock. The most common triggers for an episode are milk, soy, and rice, but the disorder has been associated with a wide range of food proteins. Many children develop a tolerance to the offending foods by the age of three, however, in some cases, the disorder persists. Removal of the offending food should lead to a complete resolution of symptoms. The exact, underlying immune system mechanisms that are involved in the development of FPIES are unknown.

Introduction

Several different gastrointestinal disorders in children are believed to be caused by an abnormal immunologic reaction to dietary proteins. They are generally classified into three groups: IgE-mediated (as in classic food allergies), non-IgE-mediated, or mixed (a combination of both). IgE stands for immunoglobulin E, an antibody that the immune system creates in response to an allergic reaction and is often implicated in food allergies. Food specific IgE antibodies are typically not involved in FPIES. The disorder is presumed to be cell-mediated. Many researchers consider FPIES the severe end of a spectrum or continuum of disease involving non-IgE-mediated gastrointestinal food allergy disorders. This spectrum also includes proctocolitis and food-protein induced enteropathy.

Supporting Organizations

Digestive Disease National Coalition

507 Capitol Court, NE
Suite 200
Washington, DC 20002
Tel: (202)544-7497
Fax: (202)546-7105
Email: ddnc@hmcw.org
Website: http://www.ddnc.org

FPIES Foundation

Main Street, PO Box 304
Stewartville, MN 55976
Email: contact@thefpiesfoundation.org
Website: http://fpiesfoundation.org/

Food Allergy Research & Education

7925 Jones Branch Drive, Suite 1100
McLean, VA 22102
USA
Tel: 703-691-3179
Fax: (703)691-2713
Tel: (800)929-4040
Email: faan@foodallergy.org
Website: http://www.foodallergy.org/

International FPIES Association

319 Richmond Avenue
Point Pleasant Beach, NJ 08742
Tel: 908-910-4419
Fax: 732-751-4568
Email: contact@fpies.org
Website: http://www.fpies.org

NIH/National Institute of Allergy and Infectious Diseases

NIAID Office of Communications and Government Relations
5601 Fishers Lane, MSC 9806
Bethesda, MD 20892-9806
Tel: (301)496-5717
Fax: (301)402-3573
Tel: (866)284-4107
Email: ocpostoffice@niaid.nih.gov
Website: http://www.niaid.nih.gov/

For a Complete Report

This is an abstract of a report from the National Organization for Rare Disorders (NORD). For a full-text version of this report, go to www.rarediseases.org and click on Rare Disease Database under "Rare Disease Information".

The information provided in this report is not intended for diagnostic purposes. It is provided for informational purposes only.

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This disease entry is based upon medical information available through the date at the end of the topic. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder.

Last Updated:  3/22/2016
Copyright  2016 National Organization for Rare Disorders, Inc.