Lombeck I, Menzel H, Steiner G, Kasperek K
Klin Padiatr. 1982 Sep-Oct;194(5):303-5. doi: 10.1055/s-2008-1033824.
Plasma glutathione peroxidase activity is markedly reduced in dietetically treated patients with PKU or MSUD in comparison to health children of the same age. This is due to the low selenium content of their diet. During supplementation with yeast rich in selenium (200 micrograms selenium per day) for 3 months 2 healthy adults did not show any significant change of their plasma GSHPx activity. 5 dietetically treated patients with PKU or MSUD and a reduced selenium state showed a rapid increase of the plasma GSHPx activity after selenium supplementation were started with 120 micrograms Se/m2 x d. The values doubled within the first two days and reached a plateau after 1--3 weeks. The patients showed no clinical anomalities before or during the selenium supplementation besides the inherited defect of amino acid metabolism. Plasma GSHPx activity seems to be a good indicator of short-term changes of selenium intake in patients with reduced selenium state.
与同龄健康儿童相比,接受饮食治疗的苯丙酮尿症(PKU)或枫糖尿症(MSUD)患者的血浆谷胱甘肽过氧化物酶活性显著降低。这是由于他们饮食中硒含量低。2名健康成年人在补充富含硒的酵母(每天200微克硒)3个月期间,其血浆谷胱甘肽过氧化物酶(GSHPx)活性未显示任何显著变化。5名接受饮食治疗且硒状态降低的PKU或MSUD患者,在开始以120微克硒/平方米×天补充硒后,血浆GSHPx活性迅速增加。数值在头两天内翻倍,并在1至3周后达到平稳状态。除了氨基酸代谢的遗传性缺陷外,患者在补充硒之前或期间未表现出临床异常。血浆GSHPx活性似乎是硒状态降低患者短期硒摄入量变化的良好指标。