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WAGR syndrome, Wilms' tumor, aniridia, gonadoblastoma, mental retardation: a review apropos of 2 cases

Mochon, M.C.; Blanc, J.F.; Plauchu, H.; Philip, T.

Pediatrie 42(4): 249-252

1987

ISSN/ISBN: 0031-4021
PMID: 2823214
Document Number: 303708
From 41 reviewed cases and 2 personal observations of the WAGR syndrome, the main symptoms and their relative frequency are described: aniridia, mental retardation, Wilms' tumor. Sexual anomalies and gonadoblastoma are less frequent. The chromosomic micro-deletion can be shown by using highly sophisticated cytogenetic techniques, or suspected by blood enzymatic dosage (mainly catalase). Kidney echographic follow-up is highly recommended.

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