WAGR Syndrome

Important
It is possible that the main title of the report WAGR 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

WAGR Complex
Wilms' Tumor-Aniridia-Genitourinary Anomalies-Mental Retardation Syndrome
Wilms' Tumor-Aniridia-Gonadoblastoma-Mental Retardation Syndrome
Chromosome 11p deletion syndrome
11p deletion syndrome

Disorder Subdivisions

Aniridia-Wilms' Tumor Association
AWTA
Aniridia-Ambiguous Genitalia-Mental Retardation
AGR Triad
Aniridia-Wilms' Tumor-Gonadoblastoma

General Discussion

WAGR syndrome is a rare genetic syndrome in which there is a predisposition to several conditions, including certain malignancies, distinctive eye abnormalities, and/or mental retardation. "WAGR" is an acronym for the characteristic abnormalities associated with the syndrome. The acronym stands for (W)ilms' Tumor, the most common form of kidney cancer in children; (A)niridia, partial or complete absence of the colored region of the eye(s) (iris or irides); (G) Genitourinary abnormalities, such as undescended testicles or hypospadias in males, or internal genital or urinary anomalies in females; and Mental (R)etardation. A combination of two or more of these conditions is usually present in most individuals with WAGR syndrome. The clinical picture varies, depending upon the combination of associated abnormalities.

WAGR syndrome is caused by defects (mutations) of adjacent genes on a region of chromosome 11 (11p13). In most cases, such genetic changes (e.g., deletions at band 11p13) occur spontaneously during early embryonic development (de novo) for unknown reasons (sporadic). In very rare cases, the mutation may be inherited as the result of a rearrangement of parts of two chromosomes, which causes the loss of some genetic material (translocation) or other heritable genetic abnormality. The presence of more than one type of chromosomal makeup within an individual (mosaic deletion) resulting in WAGR syndrome has also been reported.

Resources

March of Dimes Birth Defects Foundation
1275 Mamaroneck Avenue
White Plains, NY 10605
Tel: (914)428-7100
Fax: (914)997-4763
Tel: (888)663-4637
Email: Askus@marchofdimes.com
Internet: http://www.marchofdimes.com

Ambiguous Genitalia Support Network
P.O. Box 313
Clements, CA 95227-0313
USA
Email: agsn@inreach.com

American Kidney Fund, Inc.
6110 Executive Boulevard
Suite 1010
Rockville, MD 20852
USA
Tel: (301)881-3052
Fax: (301)881-0898
Tel: (800)638-8299
Email: helpline@kideyfund.org
Internet: http://www.kidneyfund.org

Candlelighters Childhood Cancer Foundation
P.O. Box 498
Kensington, MD 20895-0498
Tel: (301)962-3520
Fax: (301)962-3521
Tel: (800)366-2223
Email: staff@candlelighters,org
Internet: http://www.candlelighters.org

National Kidney Foundation
30 East 33rd Street
New York, NY 10016
Tel: (212)889-2210
Fax: (212)689-9261
Tel: (800)622-9010
Email: info@kidney.org
Internet: http://www.kidney.org

American Cancer Society, Inc.
1599 Clifton Road NE
Atlanta, GA 30329
USA
Tel: (404)320-3333
Tel: (800)227-2345
Internet: http://www.cancer.org

National Eye Research Foundation
910 Skokie Boulevard
Suite 207A
Northbrook, IL 60062
Tel: (847)564-4652
Fax: (847)564-0807
Tel: (800)621-2258
Email: nerf1955@aol.com
Internet: http://www.nerf.org

Glaucoma Research Foundation
490 Post Street
Suite 1427
San Francisco, CA 94102
Tel: (415)986-3162
Fax: (415)986-3763
Tel: (800)826-6693
Email: info@glaucoma.org
Internet: http://www.glaucoma.org

NIH/National Eye Institute
Building 31 Rm 6A32
31 Center Dr MSC 2510
Bethesda, MD 20892-2510
United States
Tel: (301)496-5248
Fax: (301)402-1065
Email: 2020@nei.nih.gov
Internet: http://www.nei.nih.gov/

International WAGR Syndrome Association
PO Box 392
Allen Park, MI 48101
USA
Email: ReachingOut@wagr.org
Internet: http://www.wagr.org and http://health.groups.yahoo.com/group/WAGR/

MUMS (Mothers United for Moral Support, Inc) National Parent-to-Parent Network
150 Custer Court
Green Bay, WI 54301-1243
USA
Tel: (920)336-5333
Fax: (920)339-0995
Tel: (877)336-5333
Email: mums@netnet.net
Internet: http://www.netnet.net/mums/

Aniridia Network
17 Sandmartin Crescent
Colchester, Essex, CO3 8WQ
UK
Tel: 44 07779 859 624
Email: Hannah@aniriddia.org
Internet: http://www.aniridia.org

Friends of Cancer Research
2231 Crystal Drive
Suite 200
Arlington, VA 22202
Tel: (703)302-1503
Fax: (703)302-1568
Email: info@focr.org
Internet: http://www.focr.org

Wellness Community
919 18th Street N.W.
Suite 54
Washington, DC 20006
Tel: (202)659-9709
Fax: (202)659-9301
Tel: (888)793-9355
Email: help@thewellnesscommunity.org
Internet: http://www.thewellnesscommunity.org

For a Complete Report:

This is an abstract of a report from the National Organization for Rare Disorders, Inc. ® (NORD). A copy of the complete report can be obtained for a small fee by visiting the NORD website. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational treatments (if available), and references from medical literature. For a full-text version of this topic, see http://www.rarediseases.org/search/rdblist.html

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: 8/13/2007
Copyright 1994, 1999, 2007 National Organization for Rare Disorders, Inc.

WebMD Medical Reference from the National Organization of Rare Disorders

Last Updated: August 13, 2007

This information is not intended to replace the advice of a doctor. Healthwise disclaims any liability for the decisions you make based on this information.