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本文引用的文献

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Int J Dev Disabil. 2020 Sep 10;68(4):399-415. doi: 10.1080/20473869.2020.1817717. eCollection 2022.
2
Pragmatic Profiles of Adults with Fragile X Syndrome and Williams Syndrome.脆性X综合征和威廉姆斯综合征成人的实用特征
Brain Sci. 2022 Mar 13;12(3):385. doi: 10.3390/brainsci12030385.
3
Evaluating quality of life in families with Williams Syndrome patients.评估有威廉姆斯综合征患者的家庭的生活质量。
Health Qual Life Outcomes. 2021 Apr 14;19(1):121. doi: 10.1186/s12955-021-01704-0.
4
A meta-analysis of prosody in autism, Williams syndrome, and Down syndrome.自闭症、威廉姆斯综合征和唐氏综合征的韵律分析的荟萃分析。
J Commun Disord. 2021 Jan-Feb;89:106055. doi: 10.1016/j.jcomdis.2020.106055. Epub 2020 Nov 22.
5
An Exploration of Social Cognition in Children with Different Degrees of Genetic Deletion in Williams Syndrome.探讨不同程度遗传缺失的威廉姆斯综合征儿童的社会认知。
J Autism Dev Disord. 2021 May;51(5):1695-1704. doi: 10.1007/s10803-020-04656-4.
6
Williams syndrome: recent advances in our understanding of cognitive, social and psychological functioning.威廉姆斯综合征:认知、社会和心理功能理解的最新进展。
Curr Opin Psychiatry. 2019 Mar;32(2):60-66. doi: 10.1097/YCO.0000000000000477.
7
Neurodevelopmental Disorders Affecting Sociability: Recent Research Advances and Future Directions in Autism Spectrum Disorder and Williams Syndrome.影响社交能力的神经发育障碍:自闭症谱系障碍和威廉姆斯综合征的最新研究进展和未来方向。
Curr Neurol Neurosci Rep. 2018 Oct 17;18(12):94. doi: 10.1007/s11910-018-0902-y.
8
Autism Spectrum Symptomatology in Children with Williams Syndrome Who Have Phrase Speech or Fluent Language.患有威廉姆斯综合征且具有短语言语或流利语言的儿童的自闭症谱系症状。
J Autism Dev Disord. 2018 Sep;48(9):3037-3050. doi: 10.1007/s10803-018-3555-4.
9
Language and emotional abilities in children with Williams syndrome and children with autism spectrum disorder: similarities and differences.威廉姆斯综合征患儿与自闭症谱系障碍患儿的语言和情感能力:异同点
Pediatric Health Med Ther. 2016 Jul 22;7:89-97. doi: 10.2147/PHMT.S66347. eCollection 2016.
10
Explicit Oral Narrative Intervention for Students with Williams Syndrome.针对威廉姆斯综合征学生的明确口头叙事干预
Front Psychol. 2018 Jan 15;8:2337. doi: 10.3389/fpsyg.2017.02337. eCollection 2017.

威廉姆斯综合征患者的实用技能:家庭的看法。

Pragmatic skills in people with Williams syndrome: the perception of families.

机构信息

Faculty of Psychology, University Complutense of Madrid, Campus de Somosaguas, 28223, Pozuelo de Alarcón, Spain.

出版信息

Orphanet J Rare Dis. 2024 Mar 1;19(1):95. doi: 10.1186/s13023-024-03016-0.

DOI:10.1186/s13023-024-03016-0
PMID:38429809
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC10908058/
Abstract

BACKGROUND

One of the most challenging linguistic areas in people with Williams Syndrome throughout their evolutionary stage is the development of pragmatic skills. The research conducted so far highlights specific problems concerning adaptation to the linguistic context and interlocutors, language comprehension, as well as other aspects interfering with verbal communication. However, until now, most scientific evidence has been based on personal assessments of this group. In a complementary manner, the goal of this study was to discover the level of pragmatic skills of people with Williams Syndrome from the point of view of the families. The sample consisted of 34 families belonging to the Williams Syndrome Association of Spain. The assessment instrument was the pragmatic awareness questionnaire, which includes 26 items related to different aspects that are part of the pragmatic area on a Likert-type scale.

RESULTS

The results indicate that, families consider there to be a regular to low level in all the areas assessed. The strong points seem to lie in the paralinguistic aspects, while the weakest factors are those related to the understanding of figurative language.

CONCLUSIONS

Therefore, it is necessary to continue insisting on the importance of language intervention in this group throughout its development to improve its level of linguistic competence.

摘要

背景

在整个进化阶段,患有威廉姆斯综合征的人群中最具挑战性的语言领域之一是语用技能的发展。迄今为止进行的研究强调了与语言环境和对话者适应、语言理解以及其他干扰言语交际的方面有关的具体问题。然而,到目前为止,大多数科学证据都基于对该群体的个人评估。作为一种补充方式,本研究的目的是从家庭的角度发现患有威廉姆斯综合征人群的语用技能水平。样本由 34 个来自西班牙威廉姆斯综合征协会的家庭组成。评估工具是语用意识问卷,其中包括 26 个与语用领域相关的项目,采用李克特量表的形式。

结果

结果表明,家庭认为所有评估领域的水平都在正常到低水平之间。强项似乎在于副语言方面,而最弱的因素则与对隐喻语言的理解有关。

结论

因此,有必要继续强调在该群体的整个发展过程中进行语言干预的重要性,以提高其语言能力水平。