Diseases & Conditions


WAGR Syndrome

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

Disorder Subdivisions

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

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.

Organizations related to WAGR Syndrome
  • Ambiguous Genitalia Support Network
    P.O. Box 313
    Clements CA 95227-0313
    Phone #: N/A
    800 #: --
    e-mail: [email protected]
    Home page: N/A
  • American Cancer Society, Inc.
    1599 Clifton Road NE
    Atlanta GA 30329
    Phone #: 404-320-3333
    800 #: 800-227-2345
    e-mail: N/A
    Home page: http://www.cancer.org
  • American Kidney Fund, Inc.
    6110 Executive Boulevard
    Rockville MD 20852
    Phone #: 301-881-3052
    800 #: 800-638-8299
    e-mail: [email protected]
    Home page: http://www.kidneyfund.org
  • Aniridia Network
    17 Sandmartin Crescent
    Colchester, Essex None CO3 8WQ
    Phone #: 44 -077-79 859 624
    800 #: N/A
    e-mail: [email protected]
    Home page: http://www.aniridia.org
  • Candlelighters Childhood Cancer Foundation
    P.O. Box 498
    Kensington MD 20895-0498
    Phone #: 301-962-3520
    800 #: 800-366-2223
    e-mail: [email protected],org
    Home page: http://www.candlelighters.org
  • Friends of Cancer Research
    2231 Crystal Drive
    Arlington VA 22202
    Phone #: 703-302-1503
    800 #: N/A
    e-mail: [email protected]
    Home page: http://www.focr.org
  • Glaucoma Research Foundation
    490 Post Street
    San Francisco CA 94102
    Phone #: 415-986-3162
    800 #: 800-826-6693
    e-mail: [email protected]
    Home page: http://www.glaucoma.org
  • International WAGR Syndrome Association
    PO Box 392
    Allen Park MI 48101
    Phone #: N/A
    800 #: --
    e-mail: [email protected]
    Home page: http://www.wagr.org
  • MUMS (Mothers United for Moral Support, Inc) National Parent-to-Parent Network
    150 Custer Court
    Green Bay WI 54301-1243
    Phone #: 920-336-5333
    800 #: 877-336-5333
    e-mail: [email protected]
    Home page: http://www.netnet.net/mums/
  • Madisons Foundation
    PO Box 241956
    Los Angeles CA 90024
    Phone #: 310-264-0826
    800 #: N/A
    e-mail: [email protected]
    Home page: http://www.madisonsfoundation.org
  • March of Dimes Birth Defects Foundation
    1275 Mamaroneck Avenue
    White Plains NY 10605
    Phone #: 914-428-7100
    800 #: 888-663-4637
    e-mail: [email protected]
    Home page: http://www.marchofdimes.com
  • NIH/National Eye Institute
    Building 31 Rm 6A32
    Bethesda MD 20892-2510
    Phone #: 301-496-5248
    800 #: --
    e-mail: [email protected]
    Home page: http://www.nei.nih.gov/
  • National Eye Research Foundation
    910 Skokie Boulevard
    Northbrook IL 60062
    Phone #: 847-564-4652
    800 #: 800-621-2258
    e-mail: [email protected]
    Home page: http://www.nerf.org
  • National Kidney Foundation
    30 East 33rd Street
    New York NY 10016
    Phone #: 212-889-2210
    800 #: 800-622-9010
    e-mail: [email protected]
    Home page: http://www.kidney.org
  • Wellness Community
    919 18th Street N.W.
    Washington DC 20006
    Phone #: 202-659-9709
    800 #: 888-793-9355
    e-mail: [email protected]
    Home page: 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