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酪氨酸补充剂对苯丙酮尿症神经心理表现影响的随机对照试验。

Randomised controlled trial of tyrosine supplementation on neuropsychological performance in phenylketonuria.

作者信息

Smith M L, Hanley W B, Clarke J T, Klim P, Schoonheyt W, Austin V, Lehotay D C

机构信息

Department of Psychology, University of Toronto, Mississauga, Ontario, Canada.

出版信息

Arch Dis Child. 1998 Feb;78(2):116-21. doi: 10.1136/adc.78.2.116.

Abstract

OBJECTIVE

To test the efficacy of tyrosine supplementation, as an adjunct to dietary treatment, on neuropsychological test performance in individuals with phenylketonuria.

DESIGN

A randomised controlled trial of tyrosine supplementation using a double blind crossover procedure with three four week phases.

SETTING

The Hospital for Sick Children, Toronto.

PARTICIPANTS

21 individuals with phenylketonuria (ages 6 to 28 years, mean 11.3).

INTERVENTION

Participants were given 100 mg/kg body weight/d of L-tyrosine or L-alanine (placebo).

RESULTS

At baseline, performance on several of the neuropsychological test measures was correlated with tyrosine levels. Dietary supplements of tyrosine increased plasma tyrosine concentrations; however, no change in test performance was found across the tyrosine and placebo phases of the study.

CONCLUSIONS

Tyrosine supplementation of this type does not appear to alter neuropsychological performance in individuals with phenylketonuria.

摘要

目的

测试补充酪氨酸作为饮食治疗的辅助手段,对苯丙酮尿症患者神经心理测试表现的疗效。

设计

采用双盲交叉程序进行的酪氨酸补充随机对照试验,分为三个为期四周的阶段。

地点

多伦多病童医院。

参与者

21名苯丙酮尿症患者(年龄6至28岁,平均11.3岁)。

干预措施

给予参与者100毫克/千克体重/天的L-酪氨酸或L-丙氨酸(安慰剂)。

结果

在基线时,几项神经心理测试指标的表现与酪氨酸水平相关。补充酪氨酸的饮食增加了血浆酪氨酸浓度;然而,在研究的酪氨酸阶段和安慰剂阶段,测试表现没有变化。

结论

这种类型的酪氨酸补充似乎不会改变苯丙酮尿症患者的神经心理表现。

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J Pediatr. 1995 Dec;127(6):936-43. doi: 10.1016/s0022-3476(95)70031-5.
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An animal model of early-treated PKU.早期治疗的苯丙酮尿症动物模型。
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