WAGR Syndrome

**Semantic type:** Disease or Syndrome

**Definition:** A syndrome characterized by a predisposition for Wilms tumor, aniridia, genitourinary anomalies, and developmental delay. This is a contiguous gene syndrome due to deletion in the vicinity of chromosome 11p13 in a region containing the WT1 and PAX6 genes.

**Synonyms:** - 11p Partial Monosomy Syndrome - WAGR syndrome - Wagr Syndrome - Wilms Tumor, Aniridia, Genitourinary Anomalies and Developmental Delay Syndrome - Wilms Tumor-Aniridia-Genitourinary Anomalies and Impaired Intellectual Development Syndrome - Wilms Tumor-Aniridia-Genitourinary Anomalies-Intellectual Disability Syndrome - Wilms Tumor-Aniridia-Genitourinary Anomalies-Mental Retardation Syndrome - Wilms Tumor-Aniridia-Genitourinary Anomalies-Mental Retardation Syndrome - Wilms tumor-aniridia-genitourinary anomalies-mental retardation syndrome