Increased Oxidative/Nitrosative Stress in Common Metabolic Diseases in Gaziantep Region

The balance between oxidative/nitrosative stress and antioxidant activity has an important role in oxidative stress associated diseases such as phenylketonuria (PKU) and maple syrup urine disease (MSUD). We aimed in this study to evaluate the possible association between oxidative/nitrosative balance and clinical features of PKU and MSUD patients. The study included 22 controls, 40 PKU and 18 MSUP patients. Serum malondialdehyde (MDA), peroxynitrite (ONOO-), 8-OH deoxyguanosine (8-OHdG), oxidative DNA damage marker, levels and some amino acid in plasma of patients were measured. Serum MDA and ONOOlevels were significantly higher in PKU and MSUD patients compared to the control group. Plasma valine leucine, isoleucine and phenylalanine levels were significantly higher in MSUD patients than in the control and PKU groups. Similarly, plasma phenylalanine levels were significantly higher in PKU patients compared to control and MSUP group. There was no difference between 8-OHdG levels when all groups were compared. Consequently, the results of this study clearly show that oxidative / nitrosative stress increases in patients with PKU and MSUD. Antioxidant supplementation may be beneficial in the treatment of these patients.