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苯丙酮尿症青少年的学业表现与智力成果

School performance and intellectual outcome in adolescents with phenylketonuria.

作者信息

Weglage J, Fünders B, Wilken B, Schubert D, Ullrich K

机构信息

Department of Paediatrics, University of Münster, Germany.

出版信息

Acta Paediatr. 1993 Jun-Jul;82(6-7):582-6. doi: 10.1111/j.1651-2227.1993.tb12759.x.

Abstract

In a retrospective study, 34 treated adolescents with phenylketonuria and their relatives were tested with scale 2 of the Culture Fair Intelligence Test (CFT20) and self-developed questionnaire concerning their development in school. The patients also filled in the Anxiety Questionnaire for Children. With an IQ of 93.6, the patients reached a normal mean intellectual performance but this was significantly below the mean IQ of the general population (100, p < 0.01), the IQ of their mothers (98.2, p < 0.05), their fathers (105.4, p < 0.05) and their siblings (110.3, p < 0.05). The patients' IQs correlated significantly with the IQs of their mothers, the socio-economic status of their families, the quality of dietary control since birth and the serum phenylalanine concentration at the moment of testing. With respect to age at which the patients started school, type of school attended and number of school years which had to be repeated, there were no significant differences between the patients and their siblings. The patients did not display a markedly higher degree of general anxiety, nervousness at examination time or a greater reluctance to attend school.

摘要

在一项回顾性研究中,对34名接受治疗的苯丙酮尿症青少年及其亲属进行了文化公平智力测验第二版(CFT20)以及一份关于他们在学校发展情况的自编问卷测试。患者还填写了儿童焦虑问卷。患者的智商为93.6,平均智力表现达到正常水平,但显著低于一般人群的平均智商(100,p<0.01)、其母亲的智商(98.2,p<0.05)、其父亲的智商(105.4,p<0.05)以及其兄弟姐妹的智商(110.3,p<0.05)。患者的智商与他们母亲的智商、家庭的社会经济地位、自出生以来的饮食控制质量以及测试时的血清苯丙氨酸浓度显著相关。在患者开始上学的年龄、所就读学校的类型以及必须重读的学年数方面,患者与其兄弟姐妹之间没有显著差异。患者并未表现出明显更高程度的总体焦虑、考试时的紧张情绪或更不愿意上学的情况。

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