Kiley-Brabeck Karen, Sobin Christina
Children's Evaluation Center, Newton, MA 02464, USA.
Appl Neuropsychol. 2006;13(4):258-68. doi: 10.1207/s15324826an1304_7.
The 22q11 Deletion Syndrome (22q11DS) is among the most frequent gene deletion disorders, occurring once in every 6,000 live births. Descriptive reports have suggested marked social differences in affected children. Empirical studies are needed to verify possible social skills deficits among children with 22q11DS, and also to examine possible associations between their frequently reported executive function deficits and social anomalies. Fifty-two parents of affected children (n = 52) and participating control siblings (n=26) completed the Social Skills Rating System (SSRS) and Behavior Inventory of Executive Function (BRIEF). When compared with control siblings, children with 22q11DS had significantly lower SSRS ratings for Cooperation, Assertion, Responsibility, and Self-Control. Affected children had significantly higher BRIEF ratings for Initiation, Planning, Working Memory, and Monitoring. In affected children, global Social Skill was negatively correlated with BRIEF Global Composite scores. Initiation and Monitoring significantly predicted Social Skill. Children with 22q11DS have marked differences in social skill development which are associated with executive dysfunction.
22q11缺失综合征（22q11DS）是最常见的基因缺失疾病之一，每6000例活产中就有1例发生。描述性报告表明，受影响儿童存在明显的社会差异。需要进行实证研究来验证22q11DS患儿可能存在的社交技能缺陷，并研究他们经常报告的执行功能缺陷与社会异常之间可能存在的关联。52名受影响儿童的父母（n = 52）和参与研究的对照兄弟姐妹（n = 26）完成了社会技能评定系统（SSRS）和执行功能行为量表（BRIEF）。与对照兄弟姐妹相比，22q11DS患儿在合作、 assertiveness（自信）、责任和自我控制方面的SSRS评分显著较低。受影响儿童在启动、计划、工作记忆和监控方面的BRIEF评分显著较高。在受影响儿童中，总体社交技能与BRIEF总体综合得分呈负相关。启动和监控显著预测社交技能。22q11DS患儿在社交技能发展方面存在明显差异，这与执行功能障碍有关。
