Oxidative stress status, clinical outcome, and β-globin gene cluster haplotypes in pediatric patients with sickle cell disease

2010 ◽  
Vol 85 (6) ◽  
pp. 529-537 ◽  
Author(s):  
Iryna Rusanova ◽  
Germaine Escames ◽  
Gladys Cossio ◽  
Rosaura G. De Borace ◽  
Belgica Moreno ◽  
...  
Keyword(s):  
Oxidative Stress ◽  
Sickle Cell Disease ◽  
Clinical Outcome ◽  
Gene Cluster ◽  
Sickle Cell ◽  
Pediatric Patients ◽  
Globin Gene ◽  
Cell Disease ◽  
Globin Gene Cluster ◽  
Oxidative Stress Status

Rapid and reliable β-globin gene cluster haplotyping of sickle cell disease patients by FRET Light Cycler and HRM assays

2011 ◽  
Vol 412 (13-14) ◽  
pp. 1257-1261 ◽  
Author(s):  
Philippe Joly ◽  
Philippe Lacan ◽  
Caroline Garcia ◽  
Angelique Delasaux ◽  
Alain Francina
Keyword(s):  
Sickle Cell Disease ◽  
Gene Cluster ◽  
Sickle Cell ◽  
Globin Gene ◽  
Cell Disease ◽  
Globin Gene Cluster ◽  
Light Cycler

Beta-Globin Gene Cluster Haplotypes in Yemeni Children with Sickle Cell Disease

2010 ◽  
Vol 123 (3) ◽  
pp. 182-185 ◽  
Author(s):  
Abdul-Wahab M. Al-Saqladi ◽  
Bernard J. Brabin ◽  
Hassan A. Bin-Gadeem ◽  
Warsha A. Kanhai ◽  
Marion Phylipsen ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Gene Cluster ◽  
Sickle Cell ◽  
Globin Gene ◽  
Beta Globin ◽  
Cell Disease ◽  
Beta Globin Gene ◽  
Globin Gene Cluster

β-Globin gene cluster haplotypes and HbF levels are not the only modulators of sickle cell disease in Lebanon

2003 ◽  
Vol 70 (2) ◽  
pp. 79-83 ◽  
Author(s):  
A. Inati ◽  
A. Taher ◽  
W. Bou Alawi W ◽  
S. Koussa ◽  
H. Kaspar ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Gene Cluster ◽  
Sickle Cell ◽  
Globin Gene ◽  
Cell Disease ◽  
Globin Gene Cluster

β-Globin gene cluster haplotypes and α-thalassemia in sickle cell disease patients from Trinidad

2008 ◽  
Vol 20 (3) ◽  
pp. 342-344 ◽  
Author(s):  
Altheia Jones-Lecointe ◽  
Erskine Smith ◽  
Marc Romana ◽  
Marie-Georges Gilbert ◽  
Waveney P. Charles ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Gene Cluster ◽  
Sickle Cell ◽  
Globin Gene ◽  
Cell Disease ◽  
Globin Gene Cluster

scholarly journals Sickle Cell Disease: Role of Oxidative Stress and Antioxidant Therapy

Antioxidants ◽  
2021 ◽  
Vol 10 (2) ◽  
pp. 296
Author(s):  
Rosa Vona ◽  
Nadia Maria Sposi ◽  
Lorenza Mattia ◽  
Lucrezia Gambardella ◽  
Elisabetta Straface ◽  
...  
Keyword(s):  
Oxidative Stress ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Globin Gene ◽  
Organ Damage ◽  
Cell Disease ◽  
Vessel Occlusion ◽  
Antioxidant Agents ◽  
Oxidant Status

Sickle cell disease (SCD) is the most common hereditary disorder of hemoglobin (Hb), which affects approximately a million people worldwide. It is characterized by a single nucleotide substitution in the β-globin gene, leading to the production of abnormal sickle hemoglobin (HbS) with multi-system consequences. HbS polymerization is the primary event in SCD. Repeated polymerization and depolymerization of Hb causes oxidative stress that plays a key role in the pathophysiology of hemolysis, vessel occlusion and the following organ damage in sickle cell patients. For this reason, reactive oxidizing species and the (end)-products of their oxidative reactions have been proposed as markers of both tissue pro-oxidant status and disease severity. Although more studies are needed to clarify their role, antioxidant agents have been shown to be effective in reducing pathological consequences of the disease by preventing oxidative damage in SCD, i.e., by decreasing the oxidant formation or repairing the induced damage. An improved understanding of oxidative stress will lead to targeted antioxidant therapies that should prevent or delay the development of organ complications in this patient population.

Sign in / Sign up

Export Citation Format

Share Document