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

✍ Scribed by Ingrid Lombeck; K. Kasperek; Dorothea Bachmann; L. E. Feinendegen; H. J. Bremer

• Publisher: Springer
• Year: 1980
• Tongue: English
• Weight: 314 KB
• Volume: 134
• Category: Article
• ISSN: 0340-6997
• DOI: 10.1007/bf00442405

✦ Synopsis

The diets of 5 patients with phenylketonuria or maple-syrup-urine disease were supplemented with yeast which was rich in selenium. For 120 days the patients received 45 gg Se/day to increase the Se content of their diets to 10-12ng 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.