Hartshorne Timothy S, Nicholas Jude, Grialou Tina L, Russ Joanna M
Central Michigan University. Mount Pleasant, MI 48859, USA.
Child Neuropsychol. 2007 Jul;13(4):333-44. doi: 10.1080/09297040600850944.
This study addressed the presence of executive dysfunction in children with CHARGE syndrome, a genetic disorder with multiple physical anomalies and severe challenging behaviors. Ninety-eight children were included in the study. More than half received clinically significant scores on the Behavior Rating Inventory of Executive Function (BRIEF; Gioia et al., 2000) scales of Shift, Monitor, and the Behavioral Regulation Index, with additional high scores on Inhibit and the Global Executive Composite. Associations were found with the age the child first walked, scores on the Autism Behavior Checklist (ABC; Krug et al., 1993), and being classified as deafblind. Difficulties with making transitions and flexible problem solving, monitoring their work and their effect on others, and acting on impulse, may be related to the behavioral difficulties exhibited by children with CHARGE. Interventions targeting improved self-regulation may help to manage this challenging behavior.
本研究探讨了CHARGE综合征患儿执行功能障碍的情况，CHARGE综合征是一种伴有多种身体异常和严重挑战性行为的遗传性疾病。该研究纳入了98名儿童。超过半数儿童在执行功能行为评定量表（BRIEF；焦亚等人，2000年）的转换、监控及行为调节指数量表上获得了具有临床意义的分数，在抑制和总体执行功能综合量表上也获得了高分。研究发现这些情况与儿童首次行走的年龄、自闭症行为 Checklist（ABC；克鲁格等人，1993年）的得分以及被归类为聋盲有关。在进行转换和灵活解决问题、监控自己的行为及其对他人的影响以及冲动行事方面存在困难，可能与CHARGE综合征患儿表现出的行为困难有关。针对改善自我调节的干预措施可能有助于管理这种具有挑战性的行为。
