Evaluation of Oxidative Stress by Dynamic Thiol/Disulfide Homeostasis and Ischemia-Modified Albumin Levels in Children with β-Thalassemia Major.

Abstract

β-thalassemia major (β-TM) is a hemoglobinopathy characterized by reduced or absent β-globin production. A balance remains between the production of free radicals and suppression of increased levels of reactive oxygen species by the antioxidant system. This study aimed to examine thiol/disulfide homeostasis (TDH) and serum ischemia-modified albumin (IMA) levels to evaluate the oxidant/antioxidant balance in healthy children and persons with β-TM receiving and not receiving chelation therapy. This prospective study was carried out from January to June 2021 among 46 individuals with β-TM and 35 healthy controls. A spectrophotometric method was used to analyze TDH and IMA concentrations. We found that, compared to controls, native thiol (NT) (P = .048) and total thiol (TT) (P = .027) values were lower in the patient group, whereas disulfide (P < .001), disulfide/native thiol (D/NT) (P = .004), disulfide/total thiol (D/TT) (P = .005), native thiol/total thiol (NT/TT) (P = .004) and IMA (P = .045) values were higher. NT and TT levels were significantly lower in the chelation- group compared to the chelation+ and control groups (P = .002, P = .001). D/NT, D/TT, and NT/TT levels were higher in the chelation+ group than the control group (P = .007), and IMA levels were significantly higher in the chelation+ and chelation- groups compared to the control group (P = .002). The receiver operating characteristic analysis demonstrated that IMA levels were significantly higher in the children with β-TM not taking regular chelation therapy. Thiol/disulfide homeostasis was observed to be weakened in children with β-TM in our study. Our findings show that when children with β-TM do not receive regular chelation therapy, their oxidant imbalance worsens.