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Genetic and Rare Diseases Information Center (GARD)

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WAGR syndrome


Other Names for this Disease

  • 11p deletion syndrome
  • Chromosome 11p deletion syndrome
  • WAGR Complex
  • Wilms tumor, Aniridia, Genitourinary anomalies, mental Retardation syndrome
  • Wilms Tumor-Aniridia-Gonadoblastoma-Mental Retardation syndrome
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.

Overview

WAGR syndrome is a genetic syndrome in which there is a predisposition to several conditions, including certain malignancies, distinctive eye abnormalities, and/or mental retardation.[1] WAGR is an acronym for Wilms tumor, Aniridia, Genitourinary anomalies (such as undescended testicles or hypospadias in males, or internal genital or urinary anomalies in females), mental Retardation syndrome.[1][2] A combination of two or more of these conditions is usually present in most individuals with WAGR syndrome.[1] The syndrome is due to a microdeletion in the 11p13 region of chromosome 11.[2] In most cases, this genetic change occurs spontaneously during early embryonic development (de novo) for unknown reasons (sporadic). Only rarely is the mutation inherited.[1]
Last updated: 3/11/2009

References

  1. Trout K. WAGR Syndrome. National Organization for Rare Disorders (NORD). 2007; http://www.rarediseases.org/search/rdbdetail_abstract.html?disname=WAGR%20Syndrome. Accessed 3/11/2009.
  2. WAGR syndrome. Orphanet. March 2006; http://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=893. Accessed 3/11/2009.
Your Questions Answered
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Please contact us with your questions about WAGR syndrome. We will answer your question and update these pages with new resources and information.

Basic Information

  • Genetics Home Reference (GHR) contains information on WAGR syndrome. This website is maintained by the National Library of Medicine.
  • The National Human Genome Research Institute's (NHGRI) mission encompasses a broad range of studies aimed at understanding the structure and function of the human genome and its role in health and disease. Click on the link to view the information page on this topic.
  • The National Organization for Rare Disorders (NORD) is a federation of more than 130 nonprofit voluntary health organizations serving people with rare disorders. Click on the link to view information on this topic.

In Depth Information

  • Medscape Reference provides information on this topic. Click on the link to view this information. You may need to register to view the medical textbook, but registration is free.
  • The Online Mendelian Inheritance in Man (OMIM) is an catalog of human genes and genetic disorders. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine. 
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs.  Access to this database is free of charge.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss WAGR syndrome. Click on the link to view a sample search on this topic.

Insurance Issues

  • The Social Security Administration (SSA) created the Compassionate Allowance Initiative to speed up the processing of disability claims for applicants with certain conditions. Click on the link to view the Compassionate Allowance information for kidney cancer, a cancer associated with this condition. More information about applying for Social Security disability benefits is available online.
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Other Names for this Disease
  • 11p deletion syndrome
  • Chromosome 11p deletion syndrome
  • WAGR Complex
  • Wilms tumor, Aniridia, Genitourinary anomalies, mental Retardation syndrome
  • Wilms Tumor-Aniridia-Gonadoblastoma-Mental Retardation syndrome
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.