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脆性X综合征中的焦虑、注意力问题、多动及异常行为清单

Anxiety, attention problems, hyperactivity, and the Aberrant Behavior Checklist in fragile X syndrome.

作者信息

Wheeler Anne, Raspa Melissa, Bann Carla, Bishop Ellen, Hessl David, Sacco Pat, Bailey Donald B

机构信息

Carolina Institute for Developmental Disabilities, University of North Carolina at Chapel Hill, Chapel Hill, North Carolina.

出版信息

Am J Med Genet A. 2014 Jan;164A(1):141-55. doi: 10.1002/ajmg.a.36232. Epub 2013 Nov 25.

Abstract

Behavior problems are a common challenge for individuals with fragile X syndrome (FXS) and constitute the primary clinical outcome domain in trials testing new FXS medications. However, little is known about the relationship between caregiver-reported behavior problems and co-occurring conditions such as anxiety and attention problems. In this study, 350 caregivers, each with at least one son or daughter with full-mutation FXS, rated one of their children with FXS using the Aberrant Behavior Checklist-Community Version (ABC-C); the Anxiety subscale of the Anxiety, Depression, and Mood Scale; and the Attention/Hyperactivity Items from the Symptom Inventories. In addition to examining family consequences of these behaviors, this study also sought to replicate psychometric findings for the ABC-C in FXS, to provide greater confidence for its use in clinical trials with this population. Psychometric properties and baseline ratings of problem behavior were consistent with other recent studies, further establishing the profile of problem behavior in FXS. Cross-sectional analyses suggest that selected dimensions of problem behavior, anxiety, and hyperactivity are age related; thus, age should serve as an important control in any studies of problem behavior in FXS. Measures of anxiety, attention, and hyperactivity were highly associated with behavior problems, suggesting that these factors at least coincide with problem behavior. However, these problems generally did not add substantially to variance in caregiver burden predicted by elevated behavior problems. The results provide further evidence of the incidence of problem behaviors and co-occurring conditions in FXS and the impact of these behaviors on the family.

摘要

行为问题是脆性X综合征(FXS)患者常见的挑战,并且在测试新型FXS药物的试验中构成主要的临床结局领域。然而,对于照料者报告的行为问题与诸如焦虑和注意力问题等共病情况之间的关系,人们知之甚少。在本研究中,350名照料者,每人至少有一个患有完全突变型FXS的儿子或女儿,使用异常行为检查表社区版(ABC-C)、焦虑、抑郁和情绪量表的焦虑分量表以及症状清单中的注意力/多动项目对其一名患有FXS的子女进行了评分。除了研究这些行为对家庭的影响外,本研究还试图重复FXS中ABC-C的心理测量学结果,以便更有信心将其用于该人群的临床试验。问题行为的心理测量学特性和基线评分与其他近期研究一致,进一步确立了FXS中问题行为的特征。横断面分析表明,问题行为、焦虑和多动的特定维度与年龄相关;因此,在任何关于FXS问题行为的研究中,年龄都应作为一个重要的对照因素。焦虑、注意力和多动的测量与行为问题高度相关,表明这些因素至少与问题行为同时出现。然而,这些问题通常并没有显著增加由行为问题加剧所预测的照料者负担的方差。结果进一步证明了FXS中问题行为和共病情况的发生率以及这些行为对家庭的影响。

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