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.

Assessment of selenium and mercury in biological samples of normal and night blindness children of age groups (3–7) and (8–12) years

“…The concentrations of selenium in blood samples of control children, found in present study, are higher than the findings of Hungary (Cser et al 1996), Belgium Urine (μg/l) 0.45±0.71 (Van Caillle-Bertrand et al 1986), England (Lombeck et al 1980), Germany (Heinzow et al 1989), and Poland (Wasowicz et al 1994) studies (Table 6). The concentrations of mercury in scalp hair samples of our referent children were lower than those reported from Spanish (Montuori et al 2006) and Japanese (Murata et al 2004) studies, while higher than USA (McDowell et al 2004) and German (Pesch et al 2002) studies (Table 6).…”

Trace Element Status of PKU Children Ingesting an Elemental Diet

“…This hormonal environment was associated with retention of N of 3.9g, potassium 29mEq and phosphate of 278mg, a ratio of these elements similar to that found in skeletal muscle (1,5,68). N retention continued the week following GH, but the marked retention of K and P did not.…”

Glutathione Peroxidase Activity, Lipid Peroxides and Selenium Status in Blood in Patients with Down’s Syndrome

“…The number of observations are shown in parenthesis. 4.04 ± 0.89 (9) 0.36 ± 0.04 70.2 ± 12.7 (6) 116.5 ±23.8 (6) 47.8 ±11.9 (6) 24.1 ± 3.2 (6) 129.1 ± 29.0 (6) 3.06 ± 0.78 (6) 0.39 ± 0.04 68.1 ± 14.3 (8) 110.3 ±37.5 (8) 46.4 ±11.4 (8) 19.3 ± 5.1 (8) 156.1 ±34.4 (8) 2.49 ± 0.89 (8) In the control group (aged 17 -27 years) the selenium concentrations in whole blood, erythrocytes and plasma were statistically higher than in the group of healthy children (6-16 years) (p < 0.001). No statistical differences between children (6 -16 years) and adults (17-27 years) were found in the trisomy 21 group with respect to the selenium concentration in whole blood, erythrocytes and plasma.…”

“…._ N . * , Selenium (Se) is necessary for the enzymatic activity Many papers have reported investigations of oxygen of glutathione peroxidase -it is the component of metabolism in trisomy 21 patients (Down's syndrome) the catalytic site of the enzyme (5) and probably and there have been suggestions that some of the stimulates glutathione peroxidase synthesis in humans pathological symptoms of this disease, for example (6) and animals (7). The gene for glutathione peroxpremature ageing, or specific brain lesion, result from idase synthesis is situated on chromosome No.…”