[Vitamin B 6 metabolism in phenylketonuria].

Voprosy meditsinskoi khimii Pub Date : 2000-01-01
L A Kharitonchik, V M Kodentsova, O A Vrzhesinskaia, S N Denisova, V B Spirichev
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Abstract

Based on the analysis of the dependence of 4-pyridoxic acid urinary excretion from pyridoxal-5'-phosphate blood plasma level and its statistic distribution it has been shown that vitamin B-6 metabolism in children suffering from phenylketonuria and therefore the criteria of the body saturation with this vitamin differ from those for healthy people. Increased pyridoxal-5'-phosphate blood plasma level has been demonstrated for PKU children. The concentration of 11 ng/ml should be considered as a bottom border of the adequate supply with vitamin B-6. The elevated vitamin B-2 intake approximates vitamin B-6 status indexes of sick children to those usually measured in healthy children. The necessity for the reevaluation of vitamin B-2 and B-6 optimal diet content under this disease and its biochemical validation are discussed.

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[苯丙酮尿症中维生素b6的代谢]。
通过分析4-吡哆酸尿排泄对吡哆醛-5′-磷酸血浆水平的依赖性及其统计分布,表明苯丙酮尿症患儿的维生素B-6代谢与正常人群的维生素B-6饱和标准存在差异。PKU患儿血浆吡哆醛-5′-磷酸水平升高。11 ng/ml的浓度应该被认为是维生素B-6充足供应的下限。升高的维生素B-2摄入量使患病儿童的维生素B-6状态指标接近于通常在健康儿童中测量的指标。讨论了重新评价该疾病下维生素B-2和B-6最佳日粮含量的必要性及其生化验证。
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