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[Trace elements balance in treated phenylketonuria children. Consequences of selenium deficiency on lipid
B C Wilke1, M Vidailhet, M J Richard
1Service de Pédiatrie 3, C.H.U. de Nancy.
Insights
Phenylketonuria (PKU) children on low phenylalanine diets show lower selenium levels and impaired Glutathione-Peroxidase (GSH-Px) activity. This suggests selenium supplementation may be crucial for PKU treatment.
Area of Science:
- Biochemistry
- Pediatric Nutrition
- Trace Element Metabolism
Context:
- Phenylketonuria (PKU) is a genetic disorder requiring strict phenylalanine restriction.
- Dietary management of PKU may impact essential trace element status.
- Selenium is a critical component of antioxidant enzymes.
Purpose:
- To investigate the status of calcium, magnesium, and trace elements, specifically selenium, in children with PKU.
- To assess the activity of seleno-dependent Glutathione-Peroxidase (GSH-Px) in PKU patients.
- To evaluate the relationship between selenium status, GSH-Px activity, and lipid peroxidation in PKU.
Summary:
- PKU children on low phenylalanine diets exhibited significantly lower plasma and erythrocyte selenium levels (p < 0.01).
- Reduced activity of Glutathione-Peroxidase (GSH-Px), a selenium-dependent enzyme, was observed in PKU patients.
- Elevated levels of lipid peroxidation products, malondialdehyde (MDA) and organic hydroperoxides (O.H.P.), were associated with defective GSH-Px activity.
Impact:
- Findings highlight a potential deficiency in selenium and impaired antioxidant defense in PKU children on restricted diets.
- Results suggest that selenium supplementation could be beneficial and necessary for improving health outcomes in PKU management.
- This research underscores the importance of monitoring trace element status in metabolic disorder treatments.
Abstract:
We studied Ca and Mg and trace elements (Zn, Cu, Fe, Mn, Se) in 15 P.K.U. children treated with strict Phe-reduced diet and in a control group. P.K.U. children are significantly lower (p < 0.01) plasma and erythrocyte Se as well as significantly lower (p < 0.01) activities of Glutathione-Peroxidase (GSH-Px), a seleno-dependent enzyme in erythrocytes and in plasma. Concomitantly with defective GSH-Px activities we observe significantly (p < 0.01) increased levels of lipid peroxidation products: malonaldialdehyde (MDA) and organic hydroperoxides (O.H.P.). So, Se supplementation appears to be necessary in low phenylalanine products used in P.K.U. treatment.