Abstract
The selenium content of serum, whole blood and hair was measured by neutron activation analysis in dietetically treated patients with phenylketonuria (PKU) and maple-syrup-urine disease (MSUD). Follow-up studies showed a decrease of the serum selenium content and the glutathione peroxidase activity of erythrocytes—a selenoenzyme—from normal values at the beginning of the diet to 20% (selenium) and 50% (gluthione peroxidase) of normal within 10–12 weeks of dietary treatment. In 36 patients the serum selenium content was lower at 6.7–28×10-9 g/ml, independent of the age of the patients (0.5 to 10 years). The selenium content of whole blood was reduced: median=98×10-9 g/g dry weight; range 75 to 165×10-9 g/g dry weight (healthy children: median=381×10-9 g/g dry weight; range 245 to 588×10-9 g/g dry weight). The selenium content of hair was markedly lower in the patients (median=62×10-9 g/g; range 13–140×10-9 g/g) than in healthy children (median=429; range 213 to 720×10-9 g/g). The mean glutathione peroxidase activity of erythrocytes was reduced to 4.6±0.64 U37/g Hb, in comparison to normal values (mean=8.8±0.88 U37/gHb).
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Lombeck, I., Kasperek, K., Harbisch, H.D. et al. The selenium state of children. Eur J Pediatr 128, 213–223 (1978). https://doi.org/10.1007/BF00445606
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DOI: https://doi.org/10.1007/BF00445606