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

Céline Renoux 1 Philippe Joly Camille Faes 1 Pauline Mury Buse Eglenen Mine Turkay Gokce Yavas Ozlem Yalcin Yves Bertrand Nathalie Garnier Daniela Cuzzubbo Alexandra Gauthier 1 Marc Romana 2 Berenike Möckesch 3 Giovanna Cannas 4 Sophie Antoine-Jonville 5 Vincent Pialoux 1 Philippe Connes 5
1 LIBM - Laboratoire Interuniversitaire de Biologie de la Motricité
2 Protéines de la membrane érythrocytaire et homologues non-érythroides
3 Université des Antilles (Pôle Guadeloupe)
4 Hôpital Edouard Herriot [CHU - HCL]
5 ACTES - Adaptation, Climat Tropical, Exercice et Santé
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.
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Journal articles
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https://hal.univ-antilles.fr/hal-01825587
Contributor : Marc Romana <>
Submitted on : Thursday, June 28, 2018 - 2:37:40 PM
Last modification on : Tuesday, September 1, 2020 - 3:07:05 AM

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Céline Renoux, Philippe Joly, Camille Faes, Pauline Mury, Buse Eglenen, et al.. Association between Oxidative Stress, Genetic Factors, and Clinical Severity in Children with Sickle Cell Anemia. Journal of Pediatrics, Elsevier, 2018, 195, pp.228 - 235. ⟨10.1016/j.jpeds.2017.12.021⟩. ⟨hal-01825587⟩

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