Selenium requirements in patients with inborn errors of amino acid metabolism and selenium deficiency

Abstract

The diets of 5 patients with phenylketonuria of maple-syrup-urine disease were supplemented with yeast which was rich in selenium. For 120 days the patients received 45 micrograms Se/day to increase the Se content of their diets to 10--12 ng Se/Kjoule. Before supplementation the selenium content of serum (5--15 ng/ml) and whole blood (10--27 ng/ml), and the activity of the erythrocyte glutathione peroxidase (0.19--2.69 U37/g Hb), amounted to only 10--20% of normal. The serum selenium content reached normal values within 4 weeks of supplementation, followed by normalisation of the selenium content of whole blood within 4--8 weeks. Restoration of the activity of erythrocyte glutathione peroxidase took 9 to 15 weeks--the red cell life span. There was a significant positive correlation between the selenium content of the erythrocytes and the activity of erythrocyte glutathione peroxidase.