Document Detail


Oxidative stress status, clinical outcome, and β-globin gene cluster haplotypes in pediatric patients with sickle cell disease.
MedLine Citation:
PMID:  20846340     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
OBJECTIVE: To correlate the clinical and hematological features of β-globin gene haplotypes with the oxidative stress status in pediatric patients with sickle cell disease (SCD).
METHODS: A total of 95 patients with SCD and 40 healthy children were studied. The β-globin cluster, plasma lipid peroxidation (LPO) and plasma nitrite plus nitrate (NOx), and erythrocyte content of glutathione (GSH) and glutathione disulfide (GSSG), and glutathione peroxidase (GPx), reductase (GRd), and superoxide dismutase (SOD) activities were measured.
RESULTS: Plasma LPO (P < 0.001) and NOx (P < 0.05) were significantly higher in patients than in controls. In erythrocytes of patients with SCD, the activities of GRd (P < 0.001) and SOD (P < 0.05) were lower, and the GSSG/GSH ratio (P < 0.001) and GPx activity (P < 0.001) were higher than in controls. High LPO levels and low SOD plus GRd activities were associated with increased severity of clinical manifestations, which correspond mainly to patients with Bantu and Benin haplotypes. LPO levels were reduced in patients with high fetal hemoglobin (HbF) levels, whereas the NOx levels and GRd activity tended to increase in this group.
CONCLUSION: Our results detected an important oxidative stress in patients with SCD and suggest that at least three redox markers, i.e., LPO, GRd, and SOD, were related with their clinical outcomes. Moreover, a relationship between high HbF and low LPO, and high HbF and high GRd activity and NOx levels were found.
Authors:
Iryna Rusanova; Germaine Escames; Gladys Cossio; Rosaura G de Borace; Belgica Moreno; Mariam Chahboune; Luís C López; Tomas Díez; Dario Acuña-Castroviejo
Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  European journal of haematology     Volume:  85     ISSN:  1600-0609     ISO Abbreviation:  Eur. J. Haematol.     Publication Date:  2010 Dec 
Date Detail:
Created Date:  2010-11-12     Completed Date:  2010-12-20     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8703985     Medline TA:  Eur J Haematol     Country:  England    
Other Details:
Languages:  eng     Pagination:  529-37     Citation Subset:  IM    
Copyright Information:
© 2010 John Wiley & Sons A/S.
Affiliation:
Departamento de Biomédica, Universidad Especializada de las Américas, Panamá, República de Panamá.
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MeSH Terms
Descriptor/Qualifier:
Adolescent
Anemia, Sickle Cell / blood*,  genetics*,  pathology
Child
Child, Preschool
Female
Fetal Hemoglobin / genetics,  metabolism
Glutathione Disulfide / blood
Haplotypes*
Humans
Infant
Lipid Peroxidation*
Male
Multigene Family*
Nitrates / blood
Nitrites / blood
Oxidative Stress*
Oxidoreductases / blood
Severity of Illness Index
beta-Globins / genetics*,  metabolism
Chemical
Reg. No./Substance:
0/Nitrates; 0/Nitrites; 0/beta-Globins; 27025-41-8/Glutathione Disulfide; 9034-63-3/Fetal Hemoglobin; EC 1.-/Oxidoreductases

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