Assessment of Antioxidant Enzymes, Total Sialic Acid, Lipid Bound Sialic Acid, Vitamins and Selected Amino Acids in Children With Phenylketonuria

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, alpha-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, alpha-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 alpha-aminoadipic acid (r = 0.777; p = 0.002). CONCLUSIONS: It was concluded that the levels of alpha-aminoadipic acid and phylloquinone might be an appropriate choice for the determination of phenylketonuria in parallel with the levels of phenylalanine. alpha-aminobutyric acid and phylloquinone as a supplement can decrease HPA damage.