title: [WAGR syndrome, Wilms' tumor, aniridia, gonadoblastoma, mental retardation: a review apropos of 2 cases].
abstract: 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.
authors.affiliation: Service de pédiatrie, Hôpital Lucien-Hussel, Vienne, France.
authors.forename: M C
authors.initials: MC
authors.lastname: Mochon
authors.forename: J F
authors.initials: JF
authors.lastname: Blanc
authors.forename: H
authors.initials: H
authors.lastname: Plauchu
authors.forename: T
authors.initials: T
authors.lastname: Philip
mesh_terms: D002869:Chromosome Aberrations; D002871:Chromosome Banding; D025063:Chromosome Disorders; D002880:Chromosomes, Human, Pair 11; D012734:Disorders of Sex Development / Q000235:genetics; D004407:Dysgerminoma / Q000235:genetics*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008607:Intellectual Disability / Q000235:genetics*; D007498:Iris / Q000002:abnormalities*; D007621:Karyotyping; D008297:Male; D013577:Syndrome; D009396:Wilms Tumor / Q000235:genetics*
vernacular_title: Syndrome WAGR, Wilms, aniridie, gonadoblastome, retard mental: revue générale à propos de deux observations.