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American Journal of Clinical Nutrition, Vol. 77, No. 1, 185-188, January 2003
© 2003 American Society for Clinical Nutrition

Original Research Communication

Lipophilic antioxidants in patients with phenylketonuria1,2,3

Catrina Colomé, Rafael Artuch, Maria-Antònia Vilaseca, Cristina Sierra, Núria Brandi, Nilo Lambruschini, Francisco J Cambra and Jaume Campistol

1 From the Serveis de Bioquímica (CC, RA, M-AV, CS, and NB), Pediatria (FJC), Gastroenterologia (NL), i Neuropediatria (JC), Hospital Sant Joan de Déu, Universitat de Barcelona, Spain.

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 1 (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 1 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.

Key Words: Phenylketonuria • tocopherol • ubiquinone-10 • lipid peroxidation • malondialdehyde • oxidative stress • lipophilic antioxidants






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