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[苯丙酮尿症——治疗策略的改变。苯丙酮尿症患儿智力发育及饮食依从性研究]

[Phenylketonuria--change in therapeutic strategies. Study of intellectual development and dietary compliance of young phenylketonuria patients].

作者信息

Weglage J, Fünders B, von Teeffelen-Heithoff A, Ullrich K

机构信息

Klinik und Poliklinik für Kinderheilkunde-Allgemeine Kinderheilkunde, Universität Münster/Westf.

出版信息

Fortschr Med. 1993 Nov 10;111(31):485-8.

PMID:8276335
Abstract

Recent results of research appear to show that over the past few years, clearly more restrictive dietary recommendations have become accepted in the treatment of phenylketonuria (PKU). A lifelong diet low in phenylalanine is the goal, since poor dietary discipline can lead to an appreciable decrease in the level of intelligence and, in rare cases, to severe neurological deficits. Furthermore, neuropsychological symptoms as sequelae of current high serum phenylalanine levels also occur. In the present study, the question as to the development of intelligence and compliance with diet was investigated. The results underscore the need for continuing adherence to a diet on the one hand, and for continuous psychosocial support on the other.

摘要

最近的研究结果似乎表明,在过去几年中,在苯丙酮尿症(PKU)的治疗中,明显更为严格的饮食建议已被接受。目标是终身食用低苯丙氨酸饮食,因为饮食控制不佳会导致智力水平明显下降,在极少数情况下还会导致严重的神经功能缺损。此外,当前高血清苯丙氨酸水平的后遗症还会出现神经心理症状。在本研究中,对智力发育和饮食依从性问题进行了调查。结果强调一方面需要持续坚持饮食,另一方面需要持续的社会心理支持。

相似文献

1
[Phenylketonuria--change in therapeutic strategies. Study of intellectual development and dietary compliance of young phenylketonuria patients].[苯丙酮尿症——治疗策略的改变。苯丙酮尿症患儿智力发育及饮食依从性研究]
Fortschr Med. 1993 Nov 10;111(31):485-8.
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[Phenylketonuria: illness experience and coping mechanisms].
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[Effect of dietary non-compliance on development in phenylketonuria--studies of 14-year-old patients].
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Predictors of intelligence quotient and intelligence quotient change in persons treated for phenylketonuria early in life.早年接受苯丙酮尿症治疗者智商及智商变化的预测因素。
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[An adult patient with phenylketonuria before and one year after reinstitution of diet therapy].[一名成年苯丙酮尿症患者在重新开始饮食治疗前及治疗一年后]
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Blood phenylalanine control in phenylketonuria: a survey of 10 European centres.苯丙酮尿症患者的血苯丙氨酸控制:10 个欧洲中心的调查。
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Loss of intellectual function in children with phenylketonuria after relaxation of dietary phenylalanine restriction.苯丙酮尿症患儿放松饮食苯丙氨酸限制后智力功能丧失。
Pediatrics. 1985 Feb;75(2):226-32.
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[Intellectual development of phenylketonuria children treated early--a longitudinal study. 10 years' concomitant psychological study in The Netherlands].早期治疗的苯丙酮尿症儿童的智力发育——一项纵向研究。荷兰为期10年的同步心理学研究
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Social quotients of children with phenylketonuria before and after discontinuation of dietary therapy.苯丙酮尿症患儿停止饮食治疗前后的社交商数。
Am J Ment Defic. 1986 Jul;91(1):92-4.

引用本文的文献

1
Intellectual and school performance in adolescents with phenylketonuria according to their dietary compliance. The Czech-Slovak Collaborative Study.
Eur J Pediatr. 1996 Jul;155 Suppl 1:S56-8. doi: 10.1007/pl00014251.