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Food Protein-Induced Enterocolitis Syndrome

National Organization for Rare Disorders, Inc.

Important

It is possible that the main title of the report Food Protein-Induced Enterocolitis Syndrome is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.

Synonyms

  • FPIES
  • dietary protein enterocolitis

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.

Resources

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

Internet: http://www.ddnc.org



NIH/National Institute of Allergy and Infectious Diseases

Office of Communications and Government Relations

6610 Rockledge Drive, MSC 6612

Bethesda, MD 20892-6612

Tel: (301)496-5717

Fax: (301)402-3573

Tel: (866)284-4107

TDD: (800)877-8339

Email: ocpostoffice@niaid.nih.gov

Internet: http://www.niaid.nih.gov/



Food Allergy Research & Education

7925 Jones Branch Drive, Suite 1100

McLean, VA 22102

USA

Fax: (703)691-2713

Tel: (800)929-4040

Email: faan@foodallergy.org

Internet: http://www.foodallergy.org/



International Association for Food Protein Enterocolitis

2372 Highway 9 South

Howell, NJ 07731

Tel: (908)910-4419

Fax: (732)751-4568

Email: contact@iaffpe.org

Internet: http://www.iaffpe.org



For a Complete Report

This is an abstract of a report from the National Organization for Rare Disorders (NORD). A copy of the complete report can be downloaded free from the NORD website for registered users. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational therapies (if available), and references from medical literature. For a full-text version of this topic, 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. NORD recommends that affected individuals seek the advice or counsel of their own personal physicians.

It is possible that the title of this topic is not the name you selected. Please check the Synonyms listing to find the alternate name(s) and Disorder Subdivision(s) covered by this report

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.

For additional information and assistance about rare disorders, please contact the National Organization for Rare Disorders at P.O. Box 1968, Danbury, CT 06813-1968; phone (203) 744-0100; web site www.rarediseases.org or email orphan@rarediseases.org

Last Updated:  5/14/2013

Copyright  2013 National Organization for Rare Disorders, Inc.

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