Oxidative stress in sickle cell patients: influence of haplotypes and specific medication

E. Belini Júnior

The main cause of sickle cell disease (SCD) is a single b-globin gene mutation that leads to the unusual hemoglobin (Hb) called Hb S with characteristics that differ from those of normal Hb. SCD is characterized by a multifaceted pathophysiology, which involves multiple changes in sickle erythrocytes, vaso-occlusive episodes, hemolysis, inflammatory mediator activation, oxidative stress, and endothelial cell dysfunction. Therefore, there is a decreased blood flow and microcirculation obstruction leading to anemia, pain crises and multiple organ failure. Since an excess of reactive oxygen species is cytotoxic, modifying various cellular mechanisms and, thus, causing organ damage, the aim of this study was to assess the antioxidant capacity and oxidative stress in SCD, correlating with patient genotype, b-globin cluster haplotypes and specific medication. In a longitudinal study, SCD patients were evaluated at two times: for time one (T1), 69 blood samples and for time two (T2), 55 blood samples. The patients were from the São José do Rio Preto Blood Bank (SJRP) and São Paulo Santa Casa Medical School Blood Center (SP). Electrophoretic, chromatographic and molecular assays were performed for SCD genotyping and b-globin cluster identification, including biochemical measurements for determination of thiobarbituric acid reactive species (TBARS) and Trolox equivalence antioxidant capacity (TEAC). Read More…

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