Céline Renoux1, Philippe Joly1, Camille Faes2, Pauline Mury2, Buse Eglenen3, Mine Turkay3, Gokce Yavas3, Ozlem Yalcin3, Yves Bertrand4, Nathalie Garnier4, Daniela Cuzzubbo4, Alexandra Gauthier5, Marc Romana6, Berenike Möckesch7, Giovanna Cannas8, Sophie Antoine-Jonville7, Vincent Pialoux9, Philippe Connes10. 1. Inter-University Laboratory of Human Movement Biology (LIBM) EA7424, Team Vascular Biology and Red Blood Cell Team, University Claude Bernard Lyon, Villeurbanne, France; Laboratory of Excellence on Red Blood Cell (Labex GR-Ex), PRES Sorbonne, Paris, France; Department of Biochemistry on Red Blood Cell Disease, Biologie Est Center, Hospices Civils de Lyon, Lyon, France. 2. Inter-University Laboratory of Human Movement Biology (LIBM) EA7424, Team Vascular Biology and Red Blood Cell Team, University Claude Bernard Lyon, Villeurbanne, France; Laboratory of Excellence on Red Blood Cell (Labex GR-Ex), PRES Sorbonne, Paris, France. 3. School of Medicine, Koç University, Istanbul, Turkey. 4. Institute of Pediatric Hematology and Oncology (IHOP), Hospices Civils de Lyon, Lyon, France. 5. Inter-University Laboratory of Human Movement Biology (LIBM) EA7424, Team Vascular Biology and Red Blood Cell Team, University Claude Bernard Lyon, Villeurbanne, France; Laboratory of Excellence on Red Blood Cell (Labex GR-Ex), PRES Sorbonne, Paris, France; Institute of Pediatric Hematology and Oncology (IHOP), Hospices Civils de Lyon, Lyon, France. 6. Laboratory of Excellence on Red Blood Cell (Labex GR-Ex), PRES Sorbonne, Paris, France; UMR Inserm 1134, Ricou Hospital, Academic Hospital of Pointe-à-Pitre, Pointe-à-Pitre, Guadeloupe. 7. Laboratory ACTES EA3596, University of French West Indies, Pointe-à-Pitre, Guadeloupe. 8. Inter-University Laboratory of Human Movement Biology (LIBM) EA7424, Team Vascular Biology and Red Blood Cell Team, University Claude Bernard Lyon, Villeurbanne, France; Laboratory of Excellence on Red Blood Cell (Labex GR-Ex), PRES Sorbonne, Paris, France; Internal Medicine, Hématology, Edouard Herriot Hospital, Lyon, France. 9. Inter-University Laboratory of Human Movement Biology (LIBM) EA7424, Team Vascular Biology and Red Blood Cell Team, University Claude Bernard Lyon, Villeurbanne, France; Laboratory of Excellence on Red Blood Cell (Labex GR-Ex), PRES Sorbonne, Paris, France; French University Institute (IUF), Paris, France. 10. Inter-University Laboratory of Human Movement Biology (LIBM) EA7424, Team Vascular Biology and Red Blood Cell Team, University Claude Bernard Lyon, Villeurbanne, France; Laboratory of Excellence on Red Blood Cell (Labex GR-Ex), PRES Sorbonne, Paris, France; French University Institute (IUF), Paris, France. Electronic address: philippe.connes@univ-lyon1.fr.
Abstract
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.
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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