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首页> 美国卫生研究院文献>JIMD Reports >Assessing Psychological Functioning in Metabolic Disorders: Validation of the Adaptive Behavior Assessment System Second Edition (ABAS-II) and the Behavior Rating Inventory of Executive Function (BRIEF) for Identification of Individuals at Risk
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Assessing Psychological Functioning in Metabolic Disorders: Validation of the Adaptive Behavior Assessment System Second Edition (ABAS-II) and the Behavior Rating Inventory of Executive Function (BRIEF) for Identification of Individuals at Risk

机译：评估代谢性疾病的心理功能：适应性行为评估系统第二版（ABAS-II）的验证以及执行功能的行为评级清单（BRIEF）用于识别有风险的个体

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Long-term follow-up of neuropsychological functioning in metabolic disorders remains difficult due to limited opportunities for comprehensive neuropsychological evaluations. This study examined the validity of using the Adaptive Behavior Assessment System, Second Edition (ABAS-II), and the Behavior Rating Inventory of Executive Function (BRIEF) for assessing developmental status in metabolic disorders and for identifying individuals at risk for cognitive deficits. Results from individuals with urea cycle disorders, phenylketonuria, galactosemia, and fatty acid oxidation disorders were obtained on the ABAS-II and BRIEF and were compared to results obtained from neuropsychological testing performed on the same day. Correlations between scores on the ABAS-II and developmental or IQ tests for individuals with urea cycle disorders ranged from 0.48 to 0.72 and concordance rates for scores greater than a standard deviation below the normative mean ranged from 69 to 89%. Correlations ranged from 0.20 to 0.68 with concordance ranging from 73 to 90% in the other metabolic disorders. For the BRIEF, correlations with other tests of executive functioning were significant for urea cycle disorders, with concordance ranging from 52 to 80%. For the other metabolic disorders, correlations ranged from −0.09 to −0.55. Concordance rates for at-risk status on the BRIEF and executive functioning tests ranged from 55% in adults to 80% in children with other metabolic disorders. These results indicate that the ABAS-II and BRIEF together can confidently be used as an adjunct or supplementary method for clinical follow-up and for research on functional status involving infants, children, and adults with metabolic disorders.
机译：由于进行全面的神经心理学评估的机会有限，因此对代谢紊乱中神经心理学功能的长期随访仍然很困难。这项研究检查了使用适应性行为评估系统第二版（ABAS-II）和执行功能行为评估量表（BRIEF）评估代谢性疾病的发展状况以及识别有认知缺陷风险的个体的有效性。在ABAS-II和Brief上获得了患有尿素循环异常，苯丙酮尿症，半乳糖血症和脂肪酸氧化异常的个体的结果，并将其与同一天进行的神经心理学测试的结果进行了比较。对于患有尿素循环障碍的个体，ABAS-II评分与发育或智商测试的得分之间的相关性在0.48至0.72之间，并且得分低于标准均值的标准偏差的一致性比率在69％至89％之间。在其他代谢性疾病中，相关性在0.20至0.68之间，一致性在73％至90％之间。对于简而言之，与其他执行功能测试的相关性对于尿素循环障碍很重要，一致性范围为52％至80％。对于其他代谢性疾病，相关性介于-0.09至-0.55之间。在简报和执行功能测试中，处于危险状态的一致性比率范围从成人的55％到患有其他代谢性疾病的儿童的80％不等。这些结果表明，ABAS-II和Brief可以一起可靠地用作临床随访以及涉及代谢异常的婴儿，儿童和成人的功能状态研究的辅助或补充方法。

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