Lipophilic antioxidants in patients with phenylketonuria
Por:
Colomé C, Artuch-Iriberri R, Vilaseca MA, Sierra-March C, Brandi-Tarrau N, Lambruschini N, Cambra-Lasaosa FJ and Campistol-Plana J
Publicada:
1 ene 2003
Resumen:
Background: Low serum ubiquinone-10 concentrations have been described in phenylketonuric patients fed natural-protein-restricted diets. Such low concentrations may be related to increased free radical damage.
Objective: We evaluated the relation between low serum ubiquinone-10 concentrations and other lipophilic antioxidants (tocopherol and retinol), selenium, glutathione peroxidase activity, and malondialdehyde concentrations as a marker of lipid peroxidation.
Design: This was a cross-sectional study of 58 patients with phenylketonuria (aged 2-36 y; median: 13 y) under dietary treatment, 58 age-matched control subjects, and 30 children with moderate hyperphenylalaninemia fed unrestricted diets (aged 3-17 y; median: 7.5 y). Serum ubiquinone-10 concentrations were analyzed by HPLC with electrochemical detection. Serum retinol, serum tocopherol, and plasma malondialdehyde were analyzed by HPLC with ultraviolet detection.
Results: A significant positive correlation was observed between ubiquinone-10 and tocopherol (r = 0.510, P < 0.001) in the patients with phenylketonuria. After the patients were stratified into 2 groups according to ubiquinone-10 values, significantly lower concentrations of tocopherol were observed in group I (low ubiquinone values) than in group 2 (normal ubiquinone values), the hyperphenylalaninemic children, and the control group. Plasma malondialdehyde concentrations were significantly higher in group I than in the other groups. No significant differences between groups 1 and 2 were observed in daily intakes of selenium, ascorbate, tocopherol, or retinol.
Conclusions: Plasma lipid peroxidation seems to be increased in phenylketonuria. Low concentrations of ubiquinone-10 could be associated with either excessive tocopherol consumption or high malondialdehyde concentrations in patients with phenylketonuria.
Filiaciones:
Univ Barcelona, Serv Bioquim, Hosp St Joan Deu, Barcelona 08950, Spain.
Univ Barcelona, Serv Pediat, Hosp St Joan Deu, Barcelona 08950, Spain.
Univ Barcelona, Serv Gastroenterol, Hosp St Joan Deu, Barcelona 08950, Spain.
Univ Barcelona, Serv Neuropediat, Hosp St Joan Deu, Barcelona 08950, Spain.
Univ Barcelona, Serv Bioquim, Hosp St Joan Deu, Barcelona 08950, Spain
Univ Barcelona, Serv Pediat, Hosp St Joan Deu, Barcelona 08950, Spain
Univ Barcelona, Serv Gastroenterol, Hosp St Joan Deu, Barcelona 08950, Spain
Univ Barcelona, Serv Neuropediat, Hosp St Joan Deu, Barcelona 08950, Spain
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