Abstract
Selenium concentrations of serum, whole blood, hair and food were measured by instrumental neutron activation analysis in dietetically treated patients with maple-syrup-urine disease (M.S.U.D.) and phenylketonuria (P.K.U.) and in control children with normal food. Concomitantly glutathione peroxidase activity of erythrocytes as a marker enzyme of selenium deficiency was estimated. Follow-up studies showed that within 2 months of dietotherapy the serum selenium concentration fell from normal values at birth to very low values. The activity of the glutathione peroxidase of the erythrocytes also decreased to 50 per cent of the normal values. According to the intake the serum selenium concentrations and the activity of the glutathione peroxidase of the erythrocytes in 18 infants and children with P.K.U. and M.S.U.D. remained significantly lower during dietotherapy than those of normal children. The serum selenium concentration of the control persons showed an age dependency.
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Lombeck, I., Harbisch, H., Kasperek, K. et al. 146: Selenium and reduced glutathione peroxidase activity in dietetically treated patients with metabolic diseases. Pediatr Res 10, 894 (1976). https://doi.org/10.1203/00006450-197610000-00137
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DOI: https://doi.org/10.1203/00006450-197610000-00137