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本文引用的文献

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Issues concerning self-report data and population-based data sets involving people with intellectual disabilities.涉及智障人士的自报数据和基于人群的数据集的问题。
Intellect Dev Disabil. 2013 Oct;51(5):333-48. doi: 10.1352/1934-9556-51.5.333.
2
Linking social behaviour and anxiety to attention to emotional faces in Williams syndrome.将社会行为和焦虑与注意力联系起来,以了解威廉姆斯综合征患者对情绪面孔的关注。
Res Dev Disabil. 2013 Dec;34(12):4608-16. doi: 10.1016/j.ridd.2013.09.042. Epub 2013 Oct 24.
3
The interplay between anxiety and social functioning in Williams syndrome.威廉姆斯综合征中焦虑与社会功能之间的相互作用。
J Autism Dev Disord. 2014 May;44(5):1220-9. doi: 10.1007/s10803-013-1984-7.
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What does Williams syndrome reveal about the determinants of social behavior?威廉姆斯综合征对社会行为的决定因素有何启示?
Front Hum Neurosci. 2013 Jun 28;7:321. doi: 10.3389/fnhum.2013.00321. Print 2013.
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Differences in Social Vulnerability among Individuals with Autism Spectrum Disorder, Williams Syndrome, and Down Syndrome.自闭症谱系障碍、威廉姆斯综合征和唐氏综合征患者的社会脆弱性差异。
Res Autism Spectr Disord. 2013 Aug 1;7(8):931-937. doi: 10.1016/j.rasd.2013.04.009.
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Sensitivity of the autonomic nervous system to visual and auditory affect across social and non-social domains in williams syndrome.威廉姆斯综合征患者在社会和非社会领域对视觉和听觉情感的自主神经系统敏感性。
Front Psychol. 2012 Sep 14;3:343. doi: 10.3389/fpsyg.2012.00343. eCollection 2012.
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Genetic and environmental vulnerabilities in children with neurodevelopmental disorders.儿童神经发育障碍的遗传和环境脆弱性。
Proc Natl Acad Sci U S A. 2012 Oct 16;109 Suppl 2(Suppl 2):17261-5. doi: 10.1073/pnas.1121087109. Epub 2012 Oct 8.
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Children with Williams Syndrome: Language, Cognitive, and Behavioral Characteristics and their Implications for Intervention.患有威廉姆斯综合征的儿童:语言、认知和行为特征及其对干预的启示
Perspect Lang Learn Educ. 2011 Oct 1;18(3):98-107. doi: 10.1044/lle18.3.98.
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Social brain development in williams syndrome: the current status and directions for future research.威廉姆斯综合征中的社会大脑发育:现状与未来研究方向
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Duplication of GTF2I results in separation anxiety in mice and humans.GTF2I 基因重复导致小鼠和人类出现分离焦虑。
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刻画威廉姆斯综合征焦虑、社交和认知表型之间的关联与分离。

Characterizing associations and dissociations between anxiety, social, and cognitive phenotypes of Williams syndrome.

作者信息

Ng Rowena, Järvinen Anna, Bellugi Ursula

机构信息

Laboratory for Cognitive Neuroscience, Salk Institute for Biological Studies, USA; University of Minnesota, Twin Cities, Institute of Child Development, USA.

Laboratory for Cognitive Neuroscience, Salk Institute for Biological Studies, USA.

出版信息

Res Dev Disabil. 2014 Oct;35(10):2403-15. doi: 10.1016/j.ridd.2014.06.010. Epub 2014 Jun 26.

DOI:10.1016/j.ridd.2014.06.010
PMID:24973548
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC4112021/
Abstract

Williams syndrome (WS) is a neurogenetic disorder known for its "hypersocial" phenotype and a complex profile of anxieties. The anxieties are poorly understood specifically in relation to the social-emotional and cognitive profiles. To address this gap, we employed a Wechsler intelligence test, the Brief Symptom Inventory, Beck Anxiety Inventory, and Salk Institute Sociability Questionnaire, to (1) examine how anxiety symptoms distinguish individuals with WS from typically developing (TD) individuals; and (2) assess the associations between three key phenotypic features of WS: intellectual impairment, social-emotional functioning, and anxiety. The results highlighted intensified neurophysiological symptoms and subjective experiences of anxiety in WS. Moreover, whereas higher cognitive ability was positively associated with anxiety in WS, the opposite pattern characterized the TD individuals. This study provides novel insight into how the three core phenotypic features associate/dissociate in WS, specifically in terms of the contribution of cognitive and emotional functioning to anxiety symptoms.

摘要

威廉姆斯综合征(WS)是一种神经遗传性疾病,以其“高度社交”的表型和复杂的焦虑症状而闻名。人们对这些焦虑症状与社会情感和认知特征之间的具体关系了解甚少。为了填补这一空白,我们采用了韦氏智力测验、简明症状量表、贝克焦虑量表和索尔克研究所社交能力问卷,来(1)研究焦虑症状如何区分威廉姆斯综合征患者与正常发育(TD)个体;以及(2)评估威廉姆斯综合征三个关键表型特征之间的关联:智力障碍、社会情感功能和焦虑。结果突出了威廉姆斯综合征患者神经生理症状的加剧和焦虑的主观体验。此外,虽然较高的认知能力与威廉姆斯综合征患者的焦虑呈正相关,但在正常发育个体中则呈现相反的模式。本研究为威廉姆斯综合征中三个核心表型特征如何关联/分离提供了新的见解,特别是在认知和情感功能对焦虑症状的影响方面。