A low Se intake in dietetically treated patients with phenylketonuria (PKU) or maple syrup urine disease (MSUD) leads to a marked reduction of the platelet glutathione peroxidase activity (GSHPx). The mean value amounted to 2.0 U/1011 platelets with t-butyl hydroperoxide (t-BOOH) (2.2 U/1011 with H202) in patients and 5.8 U/10 n with t-BOOH (5.4 U/10 ~1 with H202) in the control children. After Se supplementation with yeast rich in Se (dose: 135 gg Se/m 2) the GSHPx activities rapidly increased. They reached a plateau after 2-3 weeks and remained there during the following 15-20 weeks of supplementation. After the cessation of supplementation there was a slow decrease, the values reached a low plateau after 24 weeks.
In addition platelet glutathione S-transferase (GSHTf) was estimated with 1-chloro-2,4-dinitrobenzene. No significant difference between the values in healthy and dietetically treated patients in a low or normal Se state was observed.GSHTf did not exhibit peroxidase activity and did not show a compensatory increase when Se dependent GSHPx activity was low.
The patients do not reveal clinical signs of disturbed platelet function. GSHPx may act in platelets via lipoxygenase on the prostaglandin pathway. The physiologic consequence of altered arachidonate metabolism, when GSHPx is deficient in platelets, remains to be elucidated.