Association between Oxidative Stress, Genetic Factors, and Clinical Severity in Children with Sickle Cell Anemia

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Renoux, Céline | Joly, Philippe | Faes, Camille | Mury, Pauline | Eglenen, Buse | Turkay, Mine | Yavas, Gokce | Yalcin, Ozlem | Bertrand, Yves | Garnier, Nathalie | Cuzzubbo, Daniela | Gauthier, Alexandra | Romana, Marc | Möckesch, Berenike | Cannas, Giovanna | Antoine-Jonville, Sophie | Pialoux, Vincent | Connes, Philippe

Edité par CCSD ; Elsevier -

International audience. OBJECTIVES:To investigate the associations between several sickle cell disease genetic modifiers (beta-globin haplotypes, alpha-thalassemia, and glucose-6-phosphate dehydrogenase deficiency) and the level of oxidative stress and to evaluate the association between oxidative stress and the rates of vaso-occlusive events.STUDY DESIGN:Steady-state oxidative and nitrosative stress markers, biological variables, genetic modulators, and vaso-occlusive crisis events requiring emergency admissions were measured during a 2-year period in 62 children with sickle cell anemia (58 SS and 4 Sβ0). Twelve ethnic-matched children without sickle cell anemia also participated as healthy controls (AA) for oxidative and nitrosative stress level measurement.RESULTS:Oxidative and nitrosative stress were greater in patients with sickle cell anemia compared with control patients, but the rate of vaso-occlusive crisis events in sickle cell anemia was not associated with the level of oxidative stress. The presence of alpha-thalassemia, but not glucose-6-phosphate dehydrogenase deficiency or beta-globin haplotype, modulated the level of oxidative stress in children with sickle cell anemia.CONCLUSION:Mild hemolysis in children with alpha-thalassemia may limit oxidative stress and could explain the protective role of alpha-thalassemia in hemolysis-related sickle cell complications.

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