| Oxidative stress status, clinical outcome, and β-globin gene cluster haplotypes in pediatric patients with sickle cell disease. | |
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MedLine Citation:
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PMID: 20846340 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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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. |
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Authors:
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Iryna Rusanova; Germaine Escames; Gladys Cossio; Rosaura G de Borace; Belgica Moreno; Mariam Chahboune; Luís C López; Tomas Díez; Dario Acuña-Castroviejo |
Publication Detail:
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Type: Journal Article; Research Support, Non-U.S. Gov't |
Journal Detail:
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Title: European journal of haematology Volume: 85 ISSN: 1600-0609 ISO Abbreviation: Eur. J. Haematol. Publication Date: 2010 Dec |
Date Detail:
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Created Date: 2010-11-12 Completed Date: 2010-12-20 Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 8703985 Medline TA: Eur J Haematol Country: England |
Other Details:
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Languages: eng Pagination: 529-37 Citation Subset: IM |
Copyright Information:
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© 2010 John Wiley & Sons A/S. |
Affiliation:
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Departamento de Biomédica, Universidad Especializada de las Américas, Panamá, República de Panamá. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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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:
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0/Nitrates; 0/Nitrites; 0/beta-Globins; 27025-41-8/Glutathione Disulfide; 9034-63-3/Fetal Hemoglobin; EC 1.-/Oxidoreductases |
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