title: Oxidative stress status, clinical outcome, and β-globin gene cluster haplotypes in pediatric patients with sickle cell disease.
abstract: 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.
  
  
  CONCLUSIONS
  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.affiliation: Departamento de Biomédica, Universidad Especializada de las Américas, Panamá, República de Panamá.
authors.forename: Iryna
authors.initials: I
authors.lastname: Rusanova
authors.forename: Germaine
authors.initials: G
authors.lastname: Escames
authors.forename: Gladys
authors.initials: G
authors.lastname: Cossio
authors.forename: Rosaura G
authors.initials: RG
authors.lastname: de Borace
authors.forename: Belgica
authors.initials: B
authors.lastname: Moreno
authors.forename: Mariam
authors.initials: M
authors.lastname: Chahboune
authors.forename: Luís C
authors.initials: LC
authors.lastname: López
authors.forename: Tomas
authors.initials: T
authors.lastname: Díez
authors.forename: Dario
authors.initials: D
authors.lastname: Acuña-Castroviejo
mesh_terms: D000293:Adolescent; D000755:Anemia, Sickle Cell / Q000097:blood* / Q000235:genetics* / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D005260:Female; D005319:Fetal Hemoglobin / Q000235:genetics / Q000378:metabolism; D019803:Glutathione Disulfide / Q000097:blood; D006239:Haplotypes; D006801:Humans; D007223:Infant; D015227:Lipid Peroxidation; D008297:Male; D005810:Multigene Family; D009566:Nitrates / Q000097:blood; D009573:Nitrites / Q000097:blood; D018384:Oxidative Stress; D010088:Oxidoreductases / Q000097:blood; D012720:Severity of Illness Index; D055544:beta-Globins / Q000235:genetics* / Q000378:metabolism
chemical_list: D009566:Nitrates; D009573:Nitrites; D055544:beta-Globins; D005319:Fetal Hemoglobin; D010088:Oxidoreductases; D019803:Glutathione Disulfide