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

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• WAGR Complex
• Wilms' Tumor-Aniridia-Genitourinary Anomalies-Mental Retardation Syndrome
• Wilms' Tumor-Aniridia-Gonadoblastoma-Mental Retardation Syndrome
• Chromosome 11p deletion syndrome
• 1p deletion syndrome

Disorder Subdivisions

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

General Discussion

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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

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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: 3018813052

Fax: 3018810898

Tel: 8006388299

Email: helpline@kindeyfund.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: 4043203333

Tel: 8002272345

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: 3014965248

Fax: 3014021065

Email: 2020@nei.nih.gov

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



International WAGR Syndrome Association

2063 Regina

Lincoln Park, MI 48146

USA

Tel: 3133814302

Fax: 7752953693

Email: ReachingOut@wagr.org

Internet: http://www.wagr.org



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

For a Complete Report

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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.