1. Assessment of antioxidant enzymes, total sialic acid, lipid bound sialic acid, vitamins and selected amino acids in children with phenylketonuria.
- Author
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Ekin S, Dogan M, Gok F, and Karakus Y
- Subjects
- 2-Aminoadipic Acid blood, Case-Control Studies, Catalase blood, Child, Cholecalciferol blood, Erythrocytes cytology, Female, Glutathione Peroxidase blood, Humans, Lipid Peroxidation, Male, Malondialdehyde blood, Oxidative Stress, Phenylalanine blood, Regression Analysis, Superoxide Dismutase blood, Vitamin A blood, Vitamin K 1 blood, alpha-Tocopherol blood, Amino Acids blood, Antioxidants metabolism, Lipids blood, N-Acetylneuraminic Acid blood, Phenylketonurias blood, Vitamins blood
- Abstract
Background: In this study, children with phenylketonuria and healthy control subjects were assessed for glutathione peroxidase (GSH-Px), superoxide dismutase (SOD), catalase (CAT) activity, malondialdehyde (MDA), glutathione (GSH), retinol, cholecalciferol, α-tocopherol, phylloquinone, total sialic acid (TSA), lipid bound sialic acid (LSA), total antioxidant (TAS), total oxidation (TOS), and amino acid levels, and the relationships of these variables with phenylketonuria were evaluated., Methods: The study included 60 children with phenylketonuria and 30 control subjects. Children with phenylketonuria were divided into hyperphenylalaninemia (HPA) and amino acid mixture (AAM) groups., Results: The HPA group had significantly lower levels of GSH-Px, CAT, GSH, TAS, α-aminobutyric acid, and taurine levels (p < 0.01, p < 0.05, p < 0.05, p < 0.001, p < 0.01, p < 0.05, respectively) than the control group. Additionally, the AAM group had significantly lower levels of CAT, TAS, and phylloquinones (p < 0.05, p < 0.05, p < 0.05, respectively) than the control group. It was observed in our study that in the HPA group, a significantly strong positive linear correlation was observed between phenylalanine and α-aminoadipic acid (r = 0.777; p = 0.002)., Conclusions: It was concluded that the levels of α-aminoadipic acid and phylloquinone might be an appropriate choice for the determination of phenylketonuria in parallel with the levels of phenylalanine. α-aminobutyric acid and phylloquinone as a supplement can decrease HPA damage.
- Published
- 2018
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