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Journal articles
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
Marc Romana 2
Author IdHAL : marc-romana ORCID : https://orcid.org/0000-0002-8715-9836
Berenike Möckesch 3
Author
Giovanna Cannas 4
Author
Sophie Antoine-Jonville 5
Author IdHAL : sophie-antoine-jonville ORCID : https://orcid.org/0000-0003-0691-2177
Vincent Pialoux 1
Author
Philippe Connes 5
Author IdHAL : philippe-connes ORCID : https://orcid.org/0000-0002-9232-0268
1
LIBM - Laboratoire Interuniversitaire de Biologie de la Motricité (CAMPUS SANTE INNOVATIONS - Bâtiment IRMIS - 10 rue de la Marandière 42270 Saint Priest en Jarez /
Université Savoie Mont Blanc - Campus Scientifique - 73370 Le Bourget du Lac / UFR STAPS - Université Claude Bernard Lyon I - 27/29 Boulevard du 11 novembre 1918 - 69622 Villeurbanne Cedex - France)
- USMB [Université de Savoie] [Université de Chambéry] - Université Savoie Mont Blanc : EA7424 (27, rue Marcoz - 73000 Chambéry - France)
- UCBL - Université Claude Bernard Lyon 1 : EA7424 (43, boulevard du 11 novembre 1918, 69622 Villeurbanne cedex - France)
- Université de Lyon (92 rue Pasteur - CS 30122, 69361 Lyon Cedex 07 - France)
- UJM - Université Jean Monnet [Saint-Étienne] : EA7424 (10 rue Tréfilerie - 42100 Saint-Étienne - France)
2
Protéines de la membrane érythrocytaire et homologues non-érythroides (INTS 6, Rue Alexandre Cabanel 75739 PARIS CEDEX 15 - France)
- UAG - Université des Antilles et de la Guyane (Fouillole - BP 250 - 97157 Pointe-à-Pitre - Guadeloupe)
- INTS - Institut National de la Transfusion Sanguine [Paris] (6 rue Alexandre Cabanel, 75739 Paris cedex 15 - France)
- UPD7 - Université Paris Diderot - Paris 7 (5 rue Thomas-Mann - 75205 Paris cedex 13 - France)
- UR - Université de La Réunion (15, avenue René Cassin - CS92003 -97744 Saint Denis Cedex 9 - Réunion)
- INSERM - Institut National de la Santé et de la Recherche Médicale : U665 (101, rue de Tolbiac, 75013 Paris - France)
3
Université des Antilles (Pôle Guadeloupe) (Fouillole - BP 250 - 97157 Pointe à Pitre, Guadeloupe - Guadeloupe)
- UA - Université des Antilles (Université des Antilles | Fouillole - BP 250 - 97157 Pointe-à-Pitre - France)
4
Hôpital Edouard Herriot [CHU - HCL] (5 Place d'Arsonval
69003 Lyon - France)
- HCL - Hospices Civils de Lyon (3 Quai des Célestins 69002 Lyon - France)
5
ACTES - Adaptation, Climat Tropical, Exercice et Santé (UFR STAPS - Campus de Fouillole - BP 592 - 97157 Pointe-à-Pitre Cedex - France)
- UA - Université des Antilles : EA 3596 (Université des Antilles | Fouillole - BP 250 - 97157 Pointe-à-Pitre - France)
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.
Document type :
Journal articles
Domain :
Complete list of metadatas
https://hal.univ-antilles.fr/hal-01825587
Contributor : Marc Romana <>
Submitted on : Thursday, June 28, 2018 - 2:37:40 PM
Last modification on : Friday, November 6, 2020 - 3:28:21 AM
Contributor : Marc Romana <>
Submitted on : Thursday, June 28, 2018 - 2:37:40 PM
Last modification on : Friday, November 6, 2020 - 3:28:21 AM
Identifiers
- HAL Id : hal-01825587, version 1
- DOI : 10.1016/j.jpeds.2017.12.021
- PUBMED : 29449005
Collections
UNIV-AG | HCL | HEH | UNIV-ST-ETIENNE | UNIV-LYON1 | ACTES | UNIV-SAVOIE | UNIV-PARIS7 | USPC | LIBM | UDL | UNIV-PARIS | AFRIQ | UGA
Citation
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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