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[苯丙酮尿症患儿的心理调适]

[Psychological adjustment of children with phenylketonuria].

作者信息

Jusiene Roma, Cimbalistiene Loreta, Bieliauskaite Rasa

机构信息

Vilniaus universiteto Klinikines ir socialines psichologijos katedra, Didlaukio 47, 2057 Vilnius.

出版信息

Medicina (Kaunas). 2002;38(4):424-30.

Abstract

INTRODUCTION

Phenylketonuria is an inherited disorder, which prevents the normal use of protein food and causes changes in body chemistry, which, if left uncontrolled can lead to severe learning disabilities. By following a strict low protein diet early from birth phenylketonuria children can avoid mental retardation, other somatic as well as psychiatric disorders. However, early treated children with phenylketonuria are found to have more emotional and behavioral problems. Because of the lack of correlation between elevated phenylalanine (Phe) levels and psychological adjustment recent studies strongly indicate a psychological perspective for the development of emotional and behavioral problems in phenylketonuria patients. THE AIM OF PRESENT STUDY: To evaluate psychological adjustment of Lithuanian phenylketonuria children and to find out possible reasons for psychological problems of phenylketonuria children.

METHODS

Parents of 45 phenylketonuria children and 45 normal controls aged 2 to 14 years old were asked to fill in Lithuanian version of Child Behavior Checklist (CBCL, Achenbach, 1991) and a questionnaire on reactions to child's disease. The mean Phe level and IQ of some phenylketonuria children were considered as well.

RESULTS AND CONCLUSIONS

Older phenylketonuria children have significantly more psychological problems than healthy peers. There were no such considerable differences found in younger groups. Parental maladjustment to child's chronic illness is related to higher levels of internalizing and other problems of phenylketonuria children.

摘要

引言

苯丙酮尿症是一种遗传性疾病,它会阻碍蛋白质食物的正常利用,并导致身体化学变化,如果不加以控制,可能会导致严重的学习障碍。通过从出生起就严格遵循低蛋白饮食,苯丙酮尿症患儿可以避免智力迟钝、其他躯体和精神疾病。然而,人们发现早期接受治疗的苯丙酮尿症患儿有更多的情绪和行为问题。由于苯丙氨酸(Phe)水平升高与心理调适之间缺乏相关性,最近的研究强烈表明,苯丙酮尿症患者情绪和行为问题的发展具有心理学方面的原因。本研究的目的:评估立陶宛苯丙酮尿症患儿的心理调适情况,并找出苯丙酮尿症患儿心理问题的可能原因。

方法

要求45名年龄在2至14岁之间的苯丙酮尿症患儿的父母和45名正常对照填写立陶宛语版的儿童行为检查表(CBCL,阿肯巴克,1991年)以及一份关于对孩子疾病反应的问卷。还考虑了一些苯丙酮尿症患儿的平均苯丙氨酸水平和智商。

结果与结论

年龄较大的苯丙酮尿症患儿比健康同龄人有明显更多的心理问题。在较年幼的群体中未发现如此显著的差异。父母对孩子慢性病的调适不良与苯丙酮尿症患儿更高水平的内化及其他问题有关。

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