Published online September 30, 2005
PEDIATRICS Vol. 116 No. 4 October 2005, pp. 984-988 (doi:10.1542/10.1542/peds.2004-0467)
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REVIEW ARTICLE

WAGR Syndrome: A Clinical Review of 54 Cases

Bernard V. Fischbach, MD*, Kelly L. Trout, RN, BSN, Julia Lewis, MD*, Catherine A. Luis and Mohammed Sika, PhD*

* Department of Medicine, Division of Nephrology, Vanderbilt Medical Center, Nashville, Tennessee

WAGR syndrome is a rare genetic disorder characterized by a de novo deletion of 11p13 and is clinically associated with Wilms’ tumor, aniridia, genitourinary anomalies, and mental retardation (W-A-G-R). Although the genotypic defects in WAGR syndrome have been well established, the large variety of phenotypic manifestations of the syndrome has never been reported. We report on 54 cases of WAGR syndrome to demonstrate both the classical clinical signs and nonclassical manifestations found in a large population of individuals with this disorder. An understanding of WAGR syndrome and its clinical findings can provide important insight regarding the functions of the involved genetic region. Recommendations for diagnosis, evaluation, and surveillance of patients with WAGR syndrome are also presented.

Key Words: WAGR syndrome • Wilms’ tumor • aniridia • FSGS • WT1 • PAX6 • genitourinary abnormalities • mental retardation • Frasier syndrome • Denys-Drash syndrome

Abbreviations: WAGR, Wilms’ tumor, aniridia, genitourinary anomalies, and mental retardation • FSGS, focal segmental glomerulosclerosis • DDS, Denys-Drash syndrome

Accepted Jun 4, 2005.


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