[P2-166] Antioxidant and oxidative stress markers in patients with ataxia telangiectasia during oral betamethasone treatment
Ataxia telangiectasia (A-T) is a rare autosomal recessive disorder of childhood that results from defective DNA repair mechanisms. To determine the involvement of oxidative stress in the pathogenesis of A-T, we measured the levels of oxidative stress markers 8-hydroxy-2'-deoxyguanosine (8-OHdG) and hexanoyl lysine adduct (HEL), total antioxidant power (TAO), and 6-sufatoxymelatonin (6-SM) in urine samples obtained from patients with A-T during oral betamethasone treatment. Although there were no significant differences of the mean values of 8-OHdG, HEL and TAO between patients with A-T and control subjects during two year treatment, a few individual cases showed increased oxidative stress markers. In contrast, TAO remarkably reduced in 3 patients during the trial and in one patient at 1 year after the end of treatment. Our results suggest that impaired antioxidant mechanism rather than increased oxidative stress was predominant in A-T patients. However, we could not find the evidence that oral betamethasone treatment improved the redox status in A-T patients. A further large-scale study is required in order to verify our preliminary data.