title: Quantitative evaluation of oxidative stress status on peripheral blood in beta-thalassaemic patients by means of electron paramagnetic resonance spectroscopy.
abstract: High oxidative stress status (OSS) is known to be one of the most important factors determining cell injury and consequent organ damage in thalassaemic patients with secondary iron overload. Using an innovative hydroxylamine 'radical probe' capable of efficiently trapping majority of oxygen-radicals including superoxide we measured, by electron paramagnetic resonance (EPR) spectroscopy, OSS in peripheral blood of 38 thalassaemic patients compared with sex-/age-matched healthy controls. Thalassaemic patients showed sixfold higher EPR values of OSS than controls. Significantly higher EPR values of OSS were observed in those with a severe phenotype (thalassaemia major, transfusion-dependent) with respect to mild phenotype (sickle-cell/beta-thalassaemia, not transfusion-dependent) or thalassaemia intermedia. In patients with thalassaemia major, EPR values of OSS were positively correlated with serum ferritin and with alanine aminotransferase levels. In patients with sickle cell/beta-thalassaemia, there was no correlation between EPR value of OSS and all parameters considered. The type of chelating therapy (desferrioxamine or deferiprone) did not have an effect on EPR value of OSS. In conclusion, EPR 'radical probe' seems to be a valid innovative method to determine total OSS in patients affected by thalassaemia and might be used for evaluating new strategies of chelation, new chelators, or the efficacy of antioxidant formula.
authors.affiliation: Department of Paediatrics, 'A. Cardarelli' Hospital, Naples, Italy. aldo.filosa@ospedalcardarelli.it
authors.forename: A
authors.initials: A
authors.lastname: Filosa
authors.forename: L
authors.initials: L
authors.lastname: Valgimigli
authors.forename: G F
authors.initials: GF
authors.lastname: Pedulli
authors.forename: A
authors.initials: A
authors.lastname: Sapone
authors.forename: A
authors.initials: A
authors.lastname: Maggio
authors.forename: D
authors.initials: D
authors.lastname: Renda
authors.forename: C
authors.initials: C
authors.lastname: Scazzone
authors.forename: R
authors.initials: R
authors.lastname: Malizia
authors.forename: L
authors.initials: L
authors.lastname: Pitrolo
authors.forename: C
authors.initials: C
authors.lastname: Lo Pinto
authors.forename: Z
authors.initials: Z
authors.lastname: Borsellino
authors.forename: L
authors.initials: L
authors.lastname: Cuccia
authors.forename: M
authors.initials: M
authors.lastname: Capra
authors.forename: D
authors.initials: D
authors.lastname: Canistro
authors.forename: M
authors.initials: M
authors.lastname: Broccoli
authors.forename: A
authors.initials: A
authors.lastname: Soleti
authors.forename: M
authors.initials: M
authors.lastname: Paolini
mesh_terms: D000328:Adult; D000704:Analysis of Variance; D016022:Case-Control Studies; D002614:Chelating Agents / Q000627:therapeutic use; D004578:Electron Spin Resonance Spectroscopy; D005260:Female; D006801:Humans; D019190:Iron Overload / Q000097:blood; D008297:Male; D018384:Oxidative Stress; D017086:beta-Thalassemia / Q000097:blood* / Q000188:drug therapy
chemical_list: D002614:Chelating Agents