Psychiatric Characteristics in a Self-Selected Sample of Boys With Klinefelter Syndrome
Psychiatric Characteristics in a Self-Selected Sample of Boys With Klinefelter Syndrome
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DOI：
10.1542/peds.2008-1954
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发表时间：
2009-05-01
期刊：
影响因子：
8
通讯作者：
van Engeland, Herman
中科院分区：
文献类型：
--
作者：
Bruining, Hilgo;Swaab, Hanna;van Engeland, Herman
背景Klinefelter综合征是最常见的染色体非整倍体，患病率为1/700。Klinefelter综合征与认知障碍和语言问题广泛相关。以前没有研究系统地调查了儿童和青少年中Klinefelter综合征与精神疾病的关系。据我们所知，唯一可用的数据是来自成年克氏综合征患者的精神病学调查。探讨Klinefelter综合征患儿的精神病发病程度。通过荷兰Klinefelter协会和2所大学医学中心纳入了51例6至19岁的Klinefelter综合征受试者。通过使用结构化和标准化的评估程序对样本进行筛选，涵盖了所有精神问题和障碍。此外，所有男孩都接受了语言障碍的正式评估。语言障碍（65% [51人中的33人]）是最常见的障碍，其次是注意力缺陷障碍（63% [51人中的32人]）和自闭症谱系障碍（27% [51人中的14人]）。行为障碍在自闭症谱系障碍和精神障碍的病例中最为明显（12% [6/51]）。患有克氏综合征的儿童似乎有社交和语言发展问题的风险，以及情绪和行为调节问题。这反映在目前选定的样本中适用的广泛的精神病分类中。医护人员应该意识到，当面对Klinefelter综合征的儿童时，精神问题的先验可能性增加。儿科2009; 123：e865-e870
BACKGROUND. Klinefelter syndrome is the most frequent chromosomal aneuploidy with a prevalence of 1 in 700. Klinefelter syndrome has been widely associated with cognitive impairment and language problems. No previous studies have systematically investigated the association of Klinefelter syndrome with psychiatric disorders in children and adolescents. To our knowledge, the only data available are from psychiatric inventories of adults with Klinefelter syndrome.OBJECTIVE. To explore the extent of psychiatric morbidity in children with Klinefelter syndrome.METHOD. Fifty-one subjects with Klinefelter syndrome aged 6 to 19 years were included through the Dutch Klinefelter association and 2 university medical centers. The sample was screened by using structured and standardized assessment procedures covering the full range of psychiatric problems and disorders. In addition, all boys were formally evaluated for the presence of a language disorder.RESULTS. A wide range of classifications could be applied, with language disorder (65% [33 of 51]) as the most prevalent disorder, followed by attention-deficit disorders (63% [32 of 51]) and autism spectrum disorder (27% [14 of 51]). Behavioral impairment was most evident among cases classified as autism spectrum disorder and psychotic disorder (12% [6 of 51]).CONCLUSIONS. Children with Klinefelter syndrome seem to be at risk for problems in social and language development, as well as for problems in regulation of emotion and behavior. This is reflected in the broad spectrum of psychiatric classifications applicable in the present selected sample. Health care professionals should be aware of an increased a priori possibility of psychiatric problems when confronted with a child with Klinefelter syndrome. Pediatrics 2009; 123: e865-e870