title: [Wiedemann-Beckwith syndrome heredity pattern (author's transl)].
abstract: The authors report a sibship of four members (two male and two female)displaying the entire clinical picture of the Wiedemann-Beckwith syndrome. There was no consanguinity and no antecedents could be found in a three-generation search. On this occasion, a review is made of what is known about the Wiedemann-Beckwith syndrome concerning its hereditary pattern, which can currently be considered as autosomal dominant with variable expressivity and incomplete penetrance, instead of recessive as it was formerly accepted This heredity pattern must be known in order to provide an accurate genetic advise.
mesh_terms: D000015:Abnormalities, Multiple / Q000235:genetics*; D000303:Adrenal Cortex Diseases / Q000235:genetics; D002869:Chromosome Aberrations; D025063:Chromosome Disorders; D005260:Female; D006554:Hernia, Umbilical / Q000235:genetics; D006801:Humans; D006965:Hyperplasia; D007223:Infant; D007668:Kidney / Q000473:pathology; D008260:Macroglossia / Q000235:genetics; D008297:Male; D010179:Pancreas / Q000473:pathology
vernacular_title: "L'hérédité du syndrome de wiedemann-beckwith" a propos d'une fratrie de quatre membres atteints.