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早期持续接受治疗的苯丙酮尿症患儿的信息处理缺陷?

Information processing deficits in children with early and continuously treated phenylketonuria?

作者信息

Stemerdink B A, van der Molen M W, Kalverboer A F, van der Meere J J, Hendrikx M M, Huisman J, van der Schot L W, Slijper F M

机构信息

Department of Developmental and Experimental Clinical Psychology, State University Groningen, The Netherlands.

出版信息

Acta Paediatr Suppl. 1994 Dec;407:106-7. doi: 10.1111/j.1651-2227.1994.tb13466.x.

Abstract

Thirty-three patients with early and continuously treated classical phenylketonuria (PKU) and 33 controls matched for age, gender, and educational level of both parents, participated in a chronometric study exploring elementary mechanisms of information processing. Subjects performed speeded performance tasks designed to systematically vary the load on perceptual, central, and output-related mechanisms of information processing. A preliminary analysis of the data indicated that the overall performance of patients with early and continuously treated PKU practically matched that of the controls on all three tasks. Although this finding must be interpreted with caution as it is based on only a preliminary analysis of the data, it suggests that PKU patients who are continuously maintained on a well-controlled phenylalanine-restricted diet are not deficient in the elementary mechanisms of processing. Given the more recent findings indicating that young children with early-treated classical PKU have specific cognitive deficits in the executive function skills, despite relatively strict dietary control, the authors suggest that future studies should focus on these higher-order cognitive processes.

摘要

33例早期持续接受治疗的经典型苯丙酮尿症(PKU)患者以及33名在年龄、性别和父母教育水平方面与之匹配的对照者参与了一项计时研究,该研究旨在探索信息处理的基本机制。受试者完成了旨在系统改变信息处理的感知、中枢和输出相关机制负荷的快速执行任务。对数据的初步分析表明,早期持续接受治疗的PKU患者在所有三项任务中的总体表现实际上与对照组相当。尽管这一发现基于对数据的初步分析,必须谨慎解读,但它表明持续维持严格控制苯丙氨酸饮食的PKU患者在基本处理机制方面并无缺陷。鉴于最近的研究结果表明,尽管饮食控制相对严格,但早期接受治疗的经典型PKU幼儿在执行功能技能方面存在特定的认知缺陷,作者建议未来的研究应关注这些高阶认知过程。

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