RGD Reference Report - Oxidative stress status, clinical outcome, and beta-globin gene cluster haplotypes in pediatric patients with sickle cell disease. - Rat Genome Database

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

Authors: Rusanova, I  Escames, G  Cossio, G  De Borace, RG  Moreno, B  Chahboune, M  Lopez, LC  Diez, T  Acuna-Castroviejo, D 
Citation: Rusanova I, etal., Eur J Haematol. 2010 Dec;85(6):529-37. doi: 10.1111/j.1600-0609.2010.01528.x.
RGD ID: 11352775
Pubmed: PMID:20846340   (View Abstract at PubMed)
DOI: DOI:10.1111/j.1600-0609.2010.01528.x   (Journal Full-text)

OBJECTIVE: To correlate the clinical and hematological features of beta-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 beta-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.



RGD Manual Disease Annotations    Click to see Annotation Detail View

  
Object SymbolSpeciesTermQualifierEvidenceWithNotesSourceOriginal Reference(s)
GPX1Humansickle cell anemia  IDA  RGD 
Gpx1Ratsickle cell anemia  ISOGPX1 (Homo sapiens) RGD 
Gpx1Mousesickle cell anemia  ISOGPX1 (Homo sapiens) RGD 

Objects Annotated

Genes (Rattus norvegicus)
Gpx1  (glutathione peroxidase 1)

Genes (Mus musculus)
Gpx1  (glutathione peroxidase 1)

Genes (Homo sapiens)
GPX1  (glutathione peroxidase 1)


Additional Information