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Refractory Sprue

National Organization for Rare Disorders, Inc.

Important
It is possible that the main title of the report Refractory Sprue 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

  • refractory celiac disease
  • intractable celiac sprue

Disorder Subdivisions

  • type I refractory sprue
  • type II refractory sprue

General Discussion

Refractory sprue (RS) is a complex autoimmune disorder much like the more common celiac sprue but, unlike celiac sprue, it is resistant or unresponsive to six months of treatment with a strict gluten-free diet. Gliadin, a component of the wheat storage protein gluten, together with similar proteins in barley and rye, are the villains that trigger the immune reaction in celiac sprue. The diagnosis of RS is made by exclusion, especially of any other disorder that can affect the huge number of thread-like projections that line the interior of the intestine (intestinal villi), such as intestinal lymphoma, Crohn's disease, small intestinal bacterial overgrowth or hypogammaglobulinemia.

The intestinal villi are the means by which the gut absorbs fluids and nutrients. In celiac sprue and refractory sprue, these villi shrink and shrivel (atrophy) affecting the absorption of nutrients via the intestines. In celiac sprue, treatment by means of a strict gluten-free diet is usually sufficient to overcome the disorder. However, refractory sprue is just that: refractory or stubbornly resistant to treatment. Only a small percentage of the people with celiac sprue will develop RS, and these patients are almost invariably 30 years of age or older. However, as yet, it is impossible to predict which patient of those with celiac sprue will develop RS.

Resources

Association of Gastrointestinal Motility Disorders, Inc.
12 Roberts Drive
Bedford, MA 01730
Tel: (781)275-1300
Fax: (781)275-1304
Email: gimotility@gmx.com
Internet: http://www.agmd-gimotility.org

Gluten Intolerance Group of North America
31214 124th Ave SE
Auburn, WA 98092
Tel: (253)833-6655
Fax: (253)833-6675
Email: info@gluten.net
Internet: http://www.gluten.net

Crohn's and Colitis Foundation of America
386 Park Avenue South
17th Floor
New York, NY 10016-7374
USA
Tel: (212)685-3440
Fax: (212)779-4098
Tel: (800)932-2423
Email: info@ccfa.org
Internet: http://www.ccfa.org

Celiac Sprue Association
P.O. Box 31700
Omaha, NE 68131-0700
USA
Tel: (402)558-0600
Fax: (402)643-4108
Tel: (877)272-4272
Email: celiacs@csaceliacs.org
Internet: http://www.csaceliacs.org

NIH/National Institute of Diabetes, Digestive & Kidney Diseases
Office of Communications & Public Liaison
Bldg 31, Rm 9A06
31 Center Drive, MSC 2560
Bethesda, MD 20892-2560
Tel: (301)496-3583
Email: NDDIC@info.niddk.nih.gov
Internet: http://www2.niddk.nih.gov/

International Foundation for Functional Gastrointestinal Disorders
700 W. Virginia St., 201
Milwaukee, WI 53217
USA
Tel: (414)964-1799
Fax: (414)964-7176
Tel: (888)964-2001
Email: iffgd@iffgd.org
Internet: http://www.iffgd.org

Celiac Disease Foundation
20350 Ventura Blvd
Ste 240
Woodland Hills, CA 91364
USA
Tel: (818)716-1513
Fax: (818)267-5577
Email: cdf@celiac.org
Internet: http://www.celiac.org

Academy of Nutrition and Dietetics
120 South Riverside Plaza, Suite 2000
Chicago, IL 60606-6995
Tel: (312)899-0400
Fax: (312)899-4899
Tel: (800)877-1600
Email: knowledge@eatright.org
Internet: http://www.eatright.org

Genetic and Rare Diseases (GARD) Information Center
PO Box 8126
Gaithersburg, MD 20898-8126
Tel: (301)251-4925
Fax: (301)251-4911
Tel: (888)205-2311
TDD: (888)205-3223
Internet: http://rarediseases.info.nih.gov/GARD/

National Foundation for Celiac Awareness
P.O. Box 544
224 South Maple Street
Ambler, PA 19002
Tel: (215)325-1306
Fax: (215)643-1707
Email: info@celiaccentral.org
Internet: http://www.CeliacCentral.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:  2/7/2013
Copyright  2006, 2010, 2013 National Organization for Rare Disorders, Inc.

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