Daño oxidativo y respuesta antioxidante en pacientes con ataxia–telangiectasia (estudio de casos y controles)

OBJECTIVE: To determine markers of oxidative damage and antioxidant defense in patients with ataxia–telangiectasia (AT). METHODS: We performed an observational case–control study. We included a total of 5 patients of both sexes and 15 healthy children as controls. Were determined plasma levels of malondialdehyde and advanced oxidation protein as markers of oxidative damage and the intraerythrocytic activities of antioxidant enzymes Cu–Zn superoxide dismutase, catalase, glutathione peroxidase, glutathione reductase and levels of free thiol groups, as markers of antioxidant defense. RESULTS: Patients showed a significant increase in plasma levels of malondialdehyde (0.95 µM versus 0.54 µM, p= 0.020) and an increase in the activities of superoxide dismutase (4612.0 U/g hemoglobin (Hb) versus 3142.5 U/g Hb, p= 0.010) and catalase (2346.9 U/g Hb versus 1380.9 U/g Hb, p= 0.020) compared to controls. CONCLUSIONS: These findings are indicative of the presence of a maintained state of oxidative stress in patients with AT. The results from this study could be the support for other studies for the application of therapeutic strategies based on the use of antioxidants.