title: The spectrum of the behavioral phenotype in boys and adolescents 47,XXY (Klinefelter syndrome).
abstract: The behavioral phenotype of 47,XXY (Klinefelter syndrome) includes increased risks for developmental delays, language-based learning disabilities, executive dysfunction/ADHD, and socialemotional difficulties. However there is significant variability between individuals with 47,XXY, and many children and adolescents have minimal or no behavioral features while others have quite significant involvement. This paper describes behavioral features in a cohort of 57 children and adolescents with 47,XXY, including results on standardized measures of behavior (BASC-2), attention (Conner's Rating Scales), and social skills (Social Responsiveness Scale). A subset was directly assessed for autism spectrum disorders using the ADOS and ADIR. We discuss our results within the context of previous literature, including implications for genetic counseling, recommendations for care, and areas for future research.
authors.affiliation: University of Colorado School of Medicine, Department of Pediatrics, and Child Development Unit, The Children's Hospital, Aurora, CO 80045, USA. Tartaglia.nicole@tchden.org
authors.forename: Nicole
authors.initials: N
authors.lastname: Tartaglia
authors.forename: Lisa
authors.initials: L
authors.lastname: Cordeiro
authors.forename: Susan
authors.initials: S
authors.lastname: Howell
authors.forename: Rebecca
authors.initials: R
authors.lastname: Wilson
authors.forename: Jennifer
authors.initials: J
authors.lastname: Janusz
mesh_terms: D000293:Adolescent; D001289:Attention Deficit Disorder with Hyperactivity / Q000523:psychology; D002648:Child; D002659:Child Development Disorders, Pervasive / Q000523:psychology; D003071:Cognition; D006801:Humans; D007713:Klinefelter Syndrome / Q000523:psychology*; D007805:Language Development Disorders / Q000523:psychology; D008297:Male; D012919:Social Behavior; D055815:Young Adult