ArticleBMC medical genetics2013
Genetic and biochemical markers of hydroxyurea therapeutic response in sickle cell anemia.
Article in BMC medical genetics, 2013. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 14 papers, 1 of them a synthesis that pooled it.
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Who cites it
14 citing papers in PubMed, 1 synthesis or guideline pooled it, 23 citations in OpenAlex.
- Genetic Variation and Sickle Cell Disease Severity: A Systematic Review and Meta-Analysis.JAMA network open · 2023Pooled it
- Evaluation of The Use of Hydroxyurea in Treating Children With Sickle Cell Anemia in Central Africa's Rural Area.EJHaem · 2026Article
- Role ofLife (Basel, Switzerland) · 2025Article
- Advancing life: innovative approaches to enhance survival in sickle cell anemia patients.Annals of medicine and surgery (2012) · 2024Review
- Ferroptosis as an emerging target in sickle cell disease.Current research in toxicology · 2024Article
- Evaluation of Antioxidants Using Electrochemical Sensors: A Bibliometric Analysis.Sensors (Basel, Switzerland) · 2022Review
- Impact of Superoxide Dismutase Genetic Polymorphism (SOD2 Val16Ala) and Superoxide Dismutase Level on Disease Severity in a Cohort of Egyptian Sickle Cell Disease Patients.Mediterranean journal of hematology and infectious diseases · 2022Article
- Toward a Conversational Agent to Support the Self-Management of Adults and Young Adults With Sickle Cell Disease: Usability and Usefulness Study.Frontiers in digital health · 2021Article
- Inheritance of the Bantu/Benin haplotype causes less severe hemolytic and oxidative stress in sickle cell anemia patients treated with hydroxycarbamide.Journal of human genetics · 2016Article
- Minireview: Prognostic factors and the response to hydroxurea treatment in sickle cell disease.Experimental biology and medicine (Maywood, N.J.) · 2016Review
- Inflammation in Sickle Cell Disease: Differential and Down-Expressed Plasma Levels of Annexin A1 Protein.PloS one · 2016Article
- Oxidative profile of sickle cell patients in a Cameroonian urban hospital.BMC clinical pathology · 2016Article
- Frequency of cholelithiasis in patients with Beta-thalassemia intermedia with and without hydroxyurea.Iranian Red Crescent medical journal · 2014Article
- [Not Available].Revista brasileira de hematologia e hemoterapiaArticle
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Authors and funding
8 authors at 3 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundSickle cell anemia (SCA) presents a complex pathophysiology which can be affected by a number of modifying factors, including genetic and biochemical ones. In Brazil, there have been no studies verifying βS-haplotypes effect on oxidative stress parameters. This study evaluated βS-haplotypes and Hb F levels effects on oxidative stress markers and their relationship with hydroxyurea (HU) treatment in SCA patients.
methodsThe studied group was composed by 28 SCA patients. Thirteen of these patients were treated with HU and 15 of them were not. We used molecular methodology (PCR-RFLP) for hemoglobin S genotype confirmation and haplotypes identification. Biochemical parameters were measured using spectrophotometric methods (Thiobarbituric-acid-reactive substances and Trolox equivalent antioxidant capacity levels, catalase and GST activities) and plasma glutathione levels by High-performance liquid chromatography coupled to electrochemical detection.
resultsWe found the highest frequency of Bantu haplotype (48.2%) which was followed by Benin (32.1%). We observed also the presence of Cameroon haplotype, rare in Brazilian population and 19.7% of atypical haplotypes. The protective Hb F effect was confirmed in SCA patients because these patients showed an increase in Hb F levels that resulted in a 41.3% decrease on the lipid peroxidation levels (r =-0.74, p=0.01). Other biochemical parameters have not shown differential expression according to patient's haplotypes. Bantu haplotype presence was related to the highest lipid peroxidation levels in patients (p < 0,01), but it also conferred a differential response to HU treatment, raising Hb F levels in 52.6% (p = 0.03) when compared with the group with the same molecular profile without HU usage.
conclusionsSCA patients with Bantu haplotype showed the worst oxidative status. However these patients also demonstrated a better response to the treatment with HU. Such treatment seems to have presented a "haplotype-dependent" pharmacological effect.
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