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DOI: 10.1177/002221949602900208 The Psychoeducational Profile of Boys with Klinefelter SyndromeJoanne Rovet is an associate professor of pediatrics and psychology at the University of Toronto and a senior scientist in the Research Institute at the Hospital for Sick Children. Her primary research focuses on the neuropsychological sequelae of pediatric endocrine disorders, with a view to understanding the role of hormones in human brain development and brain functioning.
Charles Netley is a professor of psychology at Lakehead University, Thunder Bay, Ontario. He specializes in clinical and development psychology. Dr. Netley was the principal investigator of the psychological component of this project.
Maureen Keenan, BSc, held a studentship at the Hospital for Sick Children in 1993.
John Bailey is a pediatric endocrinologist who is professor emeritus in pediatrics at the University of Toronto. He is a former chief and current consultant in the Division of Endocrinology at the Hospital for Sick Children.
Donald Stewart is a retired pediatrician, formerly chief of the outpatient service at the Hospital for Sick Children, and an associate professor (emeritus), who was the principal investigator of the medical component of this project. Address: Joanne Rovet, The Hospital for Sick Children, 555 University Ave., Toronto, Ontario, Canada M5G 1X8. Klinefelter syndrome (KS) affects about 1 in 900 males due to an extra X chromosome. Although there are no obvious physical features associated with childhood KS, many boys demonstrate a cognitive deficit in verbal processing. The first section of this article integrates the extant literature on intelligence and achievement outcomes in boys with KS. The second section presents our findings from a 20-year study involving one of the largest unselected cohorts of boys with KS. We followed 36 boys with KS and 33 sibling controls from 6 until 20 years of age. Boys with KS are shown to demonstrate a verbal cognitive deficit and significant underachievement in reading and spelling, as well as in arithmetic. These problems, which are evident from early school years, increase with age such that by late adolescence, boys with KS are four to five grade levels behind. In addition, we also found that they were most likely to have a generalized type of learning disability, with very few boys indicating a pure reading or pure arithmetic problem. They also showed deficits in written language skills and acquisition of knowledge-based subject material were also problematic. Despite significant underachievment and frequent grade failure, many boys with KS had completed high school, and a few were also pursuing postsecondary educations. The discussion section examines how their language-based disability affects comprehension and learning, leading to underachievement.
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