Health / Medical Topics

    Wilms tumor-aniridia-genitourinary anomalies-mental retardation syndrome

    Pronunciation

    Definition 1

    A rare, genetic disorder that is present at birth and has two or more of the following symptoms: Wilms tumor (a type of kidney cancer); little or no iris (the colored part of the eye); defects in the sexual organs and urinary tract (the organs that make urine and pass it from the body); and below average mental ability. This syndrome occurs when part of chromosome 11 is missing. Also called WAGR syndrome. (NCI Dictionary)

    Definition 2

    A very rare congenital condition involving the complex of Wilms' tumor, aniridia, genitourinary abnormalities, and mental retardation. Wilms Tumor-Aniridia-Genitourinary Anomalies-Mental Retardation (WAGR) syndrome involves deletions of several adjacent genes in chromosome region 11p13. Two or more of the four conditions must be present for an individual to be diagnosed with WAGR Syndrome. The clinical picture varies, depending upon the combination of abnormalities. (NCI Thesaurus)

    Definition 3

    A contiguous gene syndrome associated with hemizygous deletions of chromosome region 11p13. The condition is marked by the combination of Wilms' tumor (W), ANIRIDIA (A), genitourinary abnormalities (G), and MENTAL RETARDATION (R). (NLM, Medical Subject Headings)




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