title: Antioxidant deficit and enhanced susceptibility to oxidative damage in individuals with different forms of alpha-thalassaemia.
abstract: alpha-Thalassaemia is a common red cell disorder in Taiwan, affecting 6-8% of Taiwanese. Previous studies have shown that reactive oxygen species are generated in increased amounts in thalassaemic red cells. This implies the possible alteration of redox status in thalassaemic patients, which may adversely affect their health. In the present study, the redox status of patients with alpha-thalassaemia trait and haemoglobin H (Hb H) disease was investigated. Lipid peroxidation, as measured by the level of plasma thiobarbituric acid reactive substances (TBARS), was increased in alpha-thalassaemic patients, with the highest level of TBARS in Hb H disease patient. The plasma levels of vitamin A, C, and E were significantly lower in alpha-thalassaemic patients than in controls. The overall antioxidant capacity in plasma was inversely correlated with the severity of alpha-globin gene defect: the more severe the form of alpha-thalassaemia, the lower the overall antioxidant capacity in plasma. Erythrocytes isolated from alpha-thalassaemia patients had lower levels of vitamin E, glutathione, catalase and superoxide dismutase. In addition, these alpha-thalassaemic red cells were more susceptible to hydrogen peroxide-induced lipid peroxidation and decrease in deformability. All these data suggest that the alpha-thalassaemic patients suffer from increased oxidative stress and antioxidant deficit, which may complicate the pathophysiology of alpha-thalassaemia.
authors.affiliation: Graduate Institute of Medical Biotechnology & Department of Medical Biotechnology and Laboratory Science, Chang Gung University, Kwei-san, Tao-yuan, Taiwan.
authors.forename: Mei-Ling
authors.initials: ML
authors.lastname: Cheng
authors.forename: Hung-Yao
authors.initials: HY
authors.lastname: Ho
authors.forename: Hsiu-Chuan
authors.initials: HC
authors.lastname: Tseng
authors.forename: Chien-Hong
authors.initials: CH
authors.lastname: Lee
authors.forename: Lee-Yung
authors.initials: LY
authors.lastname: Shih
authors.forename: Daniel Tsun-Yee
authors.initials: DT
authors.lastname: Chiu
mesh_terms: D000328:Adult; D000704:Analysis of Variance; D000975:Antioxidants / Q000032:analysis*; D001205:Ascorbic Acid / Q000097:blood; D016022:Case-Control Studies; D002374:Catalase / Q000097:blood; D004198:Disease Susceptibility; D004907:Erythrocyte Deformability; D004912:Erythrocytes / Q000737:chemistry*; D005260:Female; D005978:Glutathione / Q000097:blood; D006447:Hemoglobin H; D006801:Humans; D015227:Lipid Peroxidation; D008297:Male; D018384:Oxidative Stress; D013482:Superoxide Dismutase / Q000097:blood; D014801:Vitamin A / Q000097:blood; D014810:Vitamin E / Q000097:blood; D017085:alpha-Thalassemia / Q000097:blood*
chemical_list: D000975:Antioxidants; D014801:Vitamin A; D014810:Vitamin E; D006447:Hemoglobin H; D002374:Catalase; D013482:Superoxide Dismutase; D005978:Glutathione; D001205:Ascorbic Acid