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NIH Toolbox Cognition Battery Feasibility in Individuals With Williams Syndrome. 美国国立卫生研究院工具箱认知电池在威廉姆斯综合征患者中的可行性。
IF 2.1 4区 医学 Q1 EDUCATION, SPECIAL Pub Date : 2022-11-01 DOI: 10.1352/1944-7558-127.6.473
Emma E Condy, Lindsey Becker, Cristan Farmer, Aaron J Kaat, Colby Chlebowski, Beth A Kozel, Audrey Thurm

The NIH Toolbox Cognition Battery (NIHTB-CB) was developed for epidemiological and longitudinal studies across a wide age span. Such a tool may be useful for intervention trials in conditions characterized by intellectual disability (ID), such as Williams syndrome (WS). Three NIHTB-CB tasks, including two executive functioning (Flanker, Dimensional Change Card Sort) and one episodic memory (Picture Sequence Memory) task, were given to 47 individuals with WS, ages 4 to 50, to evaluate feasibility (i.e., proportion of valid administrations) in this population. Findings indicated that NIHTB-CB tests showed good feasibility. Flanker and DCCS age-corrected scores were negatively correlated with age and showed floor effects, indicating these scores may not be useful for quantifying performance on these NIHTB-CB tests in ID.

美国国立卫生研究院工具箱认知电池(NIHTB-CB)是为流行病学和纵向研究而开发的,跨越广泛的年龄跨度。这种工具可能对以智力残疾(ID)为特征的干预试验有用,例如威廉姆斯综合征(WS)。对47名年龄在4 - 50岁的WS患者进行三项NIHTB-CB任务,包括两项执行功能(侧卫,维度变化卡片排序)和一项情景记忆(图片序列记忆)任务,以评估该人群的可行性(即有效给药的比例)。结果表明,NIHTB-CB试验具有良好的可行性。Flanker和DCCS年龄校正分数与年龄呈负相关,并表现出最低效应,表明这些分数可能不适用于量化ID在这些NIHTB-CB测试中的表现。
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引用次数: 0
Working Memory Training in Youth With Autism, Fragile X, and Intellectual Disability: A Pilot Study. 自闭症、脆性X和智力残疾青年的工作记忆训练:一项试点研究。
IF 2.1 4区 医学 Q1 EDUCATION, SPECIAL Pub Date : 2022-09-01 DOI: 10.1352/1944-7558-127.5.369
Catrina A Calub, Songpoom Benyakorn, Shuai Sun, Ana-Maria Iosif, Lauren H Boyle, Marjorie Solomon, David Hessl, Julie B Schweitzer

This pilot study sought to identify potential markers of improvement from pre-post treatment in response to computerized working memory (WM) training for youth (ages 8-18) with autism spectrum disorder (ASD) and comorbid intellectual disability (ID) in a single arm, pre-post design. Participants included 26 children with ASD and 18 with comorbid ASD and fragile X syndrome (ASD+FXS). Analyses were adjusted for age and IQ. The ASD group demonstrated greater improvement on WM training relative to the ASD+FXS group. Participants improved on WM and far transfer outcomes, however, there were no significant group differences in improvement except for repetitive behavior. Higher hyperactivity/impulsivity ratings predicted lower performance on visuospatial WM. Findings suggest cognitive training may be beneficial for youth with ASD and ID, warranting further exploration.

这项试点研究试图在一项单臂、前后设计中,为患有自闭症谱系障碍(ASD)和共病智力残疾(ID)的青少年(8-18岁)确定针对计算机化工作记忆(WM)训练的治疗前后改善的潜在标志物。参与者包括26名ASD儿童和18名患有ASD和脆性X综合征(ASD+FXS)的儿童。分析根据年龄和智商进行了调整。与ASD+FXS组相比,ASD组在WM训练方面表现出更大的改善。参与者在WM和远迁移结果方面有所改善,然而,除了重复行为外,在改善方面没有显著的组间差异。较高的多动/冲动评分预测视觉空间WM的表现较低。研究结果表明,认知训练可能对患有ASD和ID的年轻人有益,值得进一步探索。
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引用次数: 0
The Strengths and Positive Influences of Children With Fetal Alcohol Spectrum Disorders. 胎儿酒精中毒综合症患儿的优势和积极影响。
IF 1.9 4区 医学 Q1 EDUCATION, SPECIAL Pub Date : 2022-09-01 DOI: 10.1352/1944-7558-127.5.355
Carson Kautz-Turnbull, Tangeria R Adams, Christie L M Petrenko

People with disabilities have not been adequately represented in strengths-based research. This study is the first to examine strengths and positive influences of young children with fetal alcohol spectrum disorder (FASD). Thirty adoptive and relative caregivers of children with FASD reported their children's strengths and positive influences and completed measures on family functioning. Using a conversion mixed design, we described themes in strengths and influences, degree of caregiver positivity and relationships with child and family functioning. Caregivers reported wide-ranging strengths and positive influences. Frequency of adaptive strengths correlated with measures of family functioning, but thematic strengths and positive influences did not. Strengths and positive influences are distinct from measures of functioning and are not well captured in deficit-focused research.

在以优势为基础的研究中,残疾人并没有得到充分的体现。本研究首次考察了胎儿酒精谱系障碍(FASD)幼儿的优势和积极影响。30 名 FASD 患儿的收养者和亲属照顾者报告了他们孩子的优势和积极影响,并完成了有关家庭功能的测量。通过转换混合设计,我们描述了长处和影响的主题、照顾者的积极程度以及与儿童和家庭功能的关系。照顾者报告了广泛的优势和积极影响。适应性优势的频率与家庭功能的测量结果相关,但主题性优势和积极影响却不相关。优势和积极影响有别于对功能的衡量,在以缺陷为重点的研究中没有得到很好的体现。
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引用次数: 0
Block Design Performance in Williams Syndrome: Visuospatial Abilities or Task Approach Skills? 威廉姆斯综合征的块设计表现:视觉空间能力还是任务方法技能?
IF 2.1 4区 医学 Q1 EDUCATION, SPECIAL Pub Date : 2022-09-01 DOI: 10.1352/1944-7558-127.5.390
Elisa Back, Emily K Farran, Jo Van Herwegen

The block design task (BDT) is a visuospatial measure that individuals with Williams syndrome (WS) perform poorly on. However, it is unclear what underlies their impaired performance. This study investigated whether poorer performance is a result of visuospatial difficulties, executive function (EF) difficulties, atypical looking strategies, or a combination of these. Eleven individuals with WS participated alongside mental age (MA)- and chronological age (CA)-matched control groups. Eye movements were recorded while they took the BDT. Dwell times and visits to areas of interest in WS differed from CA, but not MA, groups. Findings suggest that BDT abilities of individuals with WS are delayed, but not atypical. Delays result from visuospatial and attention-switching difficulties rather than atypical looking strategies.

块设计任务(block design task, BDT)是一项视觉空间测试,威廉姆斯综合征患者在该测试中表现不佳。然而,目前还不清楚是什么导致了他们的表现受损。这项研究调查了较差的表现是由视觉空间困难、执行功能困难、非典型的观察策略还是这些因素的结合造成的。11名WS患者与心理年龄(MA)和实足年龄(CA)匹配的对照组一起参加了研究。他们在服用BDT时记录眼球运动。停留时间和对感兴趣区域的访问与CA组不同,但与MA组不同。研究结果表明,WS患者的BDT能力是延迟的,但不是不典型的。延迟是由视觉空间和注意力转换困难造成的,而不是非典型的观看策略。
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引用次数: 0
Improving Social and Play Outcomes for Students With Significant Disabilities During Recess. 改善严重残疾学生在课间休息时的社交和游戏结果。
IF 2.1 4区 医学 Q1 EDUCATION, SPECIAL Pub Date : 2022-09-01 DOI: 10.1352/1944-7558-127.5.400
Chelsea J Amadi, Matthew E Brock, Mary A Barczak, Eric J Anderson
For students with autism, recess is often a missed opportunity to develop social competence and relationships. Although interventions have been developed to promote interactions and social skills for students with average or above-average intellectual functioning, there has been less focus on students with autism who have below-average intellectual functioning or who meet the criteria for intellectual disability. In this single-case design study, we tested the efficacy of a combined peer-mediated and social skills instruction intervention on the interactions, play, and social skills of three students with autism who met their state's criteria for alternate assessment for students with significant cognitive disabilities. Social skills instruction featured video models that portrayed same-aged peers demonstrating individualized social skills on the playground. For all three students, there were substantial increases in interactions, play and social skills, and students and their peers provided positive feedback about the intervention.
对于自闭症学生来说,课间休息往往是一个错失发展社交能力和人际关系的机会。虽然已经开发了干预措施,以促进智力正常或高于平均水平的学生的互动和社交技能,但对智力功能低于平均水平或符合智力残疾标准的自闭症学生的关注较少。在这项单例设计研究中,我们测试了同伴介导和社交技能指导相结合的干预对三名自闭症学生的互动、游戏和社交技能的效果,这些学生符合他们所在州对严重认知障碍学生的替代评估标准。社交技能教学的特色是视频模型,描绘了同龄的同龄人在操场上展示个性化的社交技能。对于这三个学生来说,互动、玩耍和社交技能都有了实质性的提高,学生和他们的同龄人对干预提供了积极的反馈。
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引用次数: 3
Unique Pain Responses in Different Etiological Subgroups of Intellectual and Developmental Disabilities. 智力和发育障碍不同病因亚群的独特疼痛反应。
IF 2.1 4区 医学 Q1 EDUCATION, SPECIAL Pub Date : 2022-09-01 DOI: 10.1352/1944-7558-127.5.417
Ruth Defrin, Tali Benromano, Chaim G Pick

We studied whether there exist variations in pain responses between different intellectual and developmental disability (IDD) etiologies. Self-reports and facial expressions (Facial Action Coding System = FACS) were recorded during experimental pressure stimuli and compared among 31 individuals with IDD-13 with cerebral palsy (CP), nine with Down syndrome (DS), nine with unspecified origin (UIDD)-and among 15 typically developing controls (TDCs). The CP and DS groups had higher pain ratings and FACS scores compared to the UIDD and TDC groups, and steeper stimulus-response functions. The DS group exhibited the most diverse facial expressions. There were variations in the foci of facial expressions between groups. It appears that different IDD etiologies display distinct pain responses.

我们研究了不同的智力和发育障碍(IDD)病因是否存在疼痛反应的差异。在实验压力刺激时记录自我报告和面部表情(面部动作编码系统= FACS),并比较31名IDD-13脑瘫(CP)患者、9名唐氏综合征(DS)患者、9名不明原因(UIDD)患者和15名典型发展对照(tdc)患者。与UIDD和TDC组相比,CP组和DS组的疼痛评分和FACS评分更高,刺激反应函数更陡。DS组表现出最多样化的面部表情。两组之间的面部表情焦点存在差异。似乎不同的IDD病因表现出不同的疼痛反应。
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引用次数: 0
On Terms: What's in a Name? Intellectual Disability and "Condition," "Disorder," "Syndrome," "Disease," and "Disability". 术语:名字里有什么?智力残疾和“状况”、“紊乱”、“综合症”、“疾病”和“残疾”。
IF 2.1 4区 医学 Q1 EDUCATION, SPECIAL Pub Date : 2022-09-01 DOI: 10.1352/1944-7558-127.5.349
Audrey Thurm, Siddharth Srivastava

Individuals living with intellectual disability can have multiple co-occurring medical conditions including associated genetic diagnoses. The number of genetic etiologies associated with ID is growing, with some quite new and rare, and others more common and associated with what is often considered a syndrome. In the context of genetic etiologies and associated medical comorbidities, appropriate use of descriptive terminology warrants clarification. Using accurate nomenclature is essential for descriptions, especially as terms are used across both research and clinical reports. Here we discuss several terms that may be confused with each other, including "condition," "disorder," "syndrome," "disease," and "disability." Our goal is to shed light on the meanings of the five descriptors and their appropriate use in the ID population, especially in relation to those who have a genetic diagnosis.

患有智力残疾的人可能同时患有多种疾病,包括相关的遗传诊断。与ID相关的遗传病因的数量正在增长,其中一些是相当新的和罕见的,而另一些则更常见,通常被认为是一种综合症。在遗传病因和相关的医学合并症的背景下,适当使用描述性术语值得澄清。使用准确的命名法对描述至关重要,特别是当术语在研究和临床报告中使用时。这里我们讨论几个可能相互混淆的术语,包括“条件”、“紊乱”、“综合症”、“疾病”和“残疾”。我们的目标是阐明五个描述符的含义及其在ID人群中的适当使用,特别是与那些有遗传诊断的人有关。
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引用次数: 3
Resúmenes al Español. 英文摘要。
IF 2.1 4区 医学 Q1 EDUCATION, SPECIAL Pub Date : 2022-08-26 DOI: 10.1352/1944-7558-127.5.433
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引用次数: 0
Résumés en Français. 英文摘要。
IF 2.1 4区 医学 Q1 EDUCATION, SPECIAL Pub Date : 2022-08-26 DOI: 10.1352/1944-7558-127.5.431
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引用次数: 0
Evaluating Verbal Fluency Outcome Measures in Children With Down Syndrome. 评估唐氏综合症儿童语言流畅性结果测量。
IF 2.1 4区 医学 Q1 EDUCATION, SPECIAL Pub Date : 2022-07-01 DOI: 10.1352/1944-7558-127.4.328
Catelyn N Smeyne, Anna J Esbensen, Emily K Schworer, Shequanna Belizaire, Emily K Hoffman, Dean W Beebe, Susan Wiley

This study evaluates the psychometric properties of a verbal fluency task for potential use as an outcome measure in future clinical trials involving children with Down syndrome. Eighty-five participants attempted a modified version of the Neuropsychological Assessment of Children, Second Edition Word Generation Task at two time points. In the full sample, the measure fell below a priori reliability and feasibility criteria, though feasibility of the semantic trials were higher than feasibility of the phonemic trials. Performance on the measure correlated with chronological age and IQ scores, and no sex-related effects were found. Additional analyses suggested that the semantic verbal fluency trials might be appropriate for children with Down syndrome 10 years of age and older.

本研究评估了语言流畅性任务的心理测量特性,以便在未来涉及唐氏综合症儿童的临床试验中作为一种结果测量方法。85名参与者在两个时间点尝试了修改版的儿童神经心理评估,第二版单词生成任务。在整个样本中,该测量低于先验的可靠性和可行性标准,尽管语义试验的可行性高于音素试验的可行性。测试中的表现与实际年龄和智商分数相关,没有发现与性别有关的影响。进一步的分析表明,语义语言流畅性试验可能适用于10岁及以上的唐氏综合症儿童。
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引用次数: 5
期刊
Ajidd-American Journal on Intellectual and Developmental Disabilities
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