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苯丙氨酸代谢产物作为苯丙酮尿症患儿饮食依从性的指标。

Phenylalanine metabolites as indicators of dietary compliance in children with phenylketonuria.

作者信息

Michals K, Lopus M, Matalon R

机构信息

Department of Nutrition, University of Illinois, Chicago 60612.

出版信息

Biochem Med Metab Biol. 1988 Feb;39(1):18-23. doi: 10.1016/0885-4505(88)90053-9.

DOI:10.1016/0885-4505(88)90053-9
PMID:3355711
Abstract

The data from this study showed that the excretion of three major metabolites of phenylalanine in patients with PKU approach normal values at blood phenylalanine levels less than 5.0 mg/dl. The MANOVA showed statistically significant differences in phenyllactate excretion when blood phenylalanine was greater than 10.0 mg/dl. The PL and total metabolite excretion were significantly correlated to blood phenylalanine in multiple samples taken from two individual subjects. Using data obtained from single patient observations may serve as a means for individualizing the PKU diet to insure low levels of phenylalanine metabolites and thus insure optimal development for patients with PKU.

摘要

该研究数据表明,苯丙酮尿症(PKU)患者体内苯丙氨酸的三种主要代谢产物在血液苯丙氨酸水平低于5.0mg/dl时,其排泄量接近正常值。多变量方差分析(MANOVA)显示,当血液苯丙氨酸水平高于10.0mg/dl时,苯乳酸排泄存在统计学显著差异。在从两名个体受试者采集的多个样本中,苯乳酸(PL)和总代谢产物排泄与血液苯丙氨酸显著相关。利用从单个患者观察中获得的数据,可作为使PKU饮食个体化的一种方法,以确保苯丙氨酸代谢产物处于低水平,从而确保PKU患者的最佳发育。

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引用本文的文献

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Prevalence of classical phenylketonuria in mentally retarded individuals in Iran.伊朗智障人群中经典苯丙酮尿症的流行率。
J Inherit Metab Dis. 2009 Dec;32 Suppl 1:S283-7. doi: 10.1007/s10545-009-1222-9. Epub 2009 Sep 19.
2
Carcinogenic effects in a phenylketonuria mouse model.苯丙酮尿症小鼠模型中的致癌作用。
PLoS One. 2009;4(1):e4292. doi: 10.1371/journal.pone.0004292. Epub 2009 Jan 27.
3
A synopsis of the unconjugated acidic transamination metabolites of phenylalanine in phenylketonuria.苯丙酮尿症中苯丙氨酸非共轭酸性转氨代谢产物的综述。
J Inherit Metab Dis. 1992;15(1):136-44. doi: 10.1007/BF01800355.