Avaliação dos produtos da degradação oxidativa da Hb S nos genótipos SS, SF (S/beta0 talassemia) e AS, em comparação com hemoglobinas normais

Naoum, Paulo Cesar; Souza, Patrícia Caetano de

doi:10.1590/s1676-24442004000400007

Cited by 3 publications

(3 citation statements)

References 9 publications

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“…It has been shown that hemoglobin has an increased susceptibility to undergo oxidation, and when it is subjected to low oxygen tensions, transforming discoid erythrocytes into sickle erythrocytes, increasing the production of MetHb, hemicromos, or products of heme; and causing globin S precipitation in oxidized form of Heinz bodies (13,15,19) . Naoum e Souza (15) , Allen et al (1) and Silva Filho (19) also observed an increase of MetHb in patients with hemoglobinopathies.…”

Section: Discussionmentioning

confidence: 99%

Methemoglobin measure in adult patients with sickle-cell anemia: influence of hydroxyurea therapy

Laurentino¹,

Carvalho²,

Santos

et al. 2014

Jornal Brasileiro De Patologia E Medicina Laboratorial

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Introduction: Hemoglobin S (HbS) is unstable hemoglobin that easily oxidizes, causing methemoglobin (MetHb) increased production in patients with sickle-cell anemia (SCA). Objectives: To determine MetHb levels and the influence of hydroxyurea (HU) therapy on this marker in patients with SCA. Materials and methods: Blood samples from 53 patients with SCA at the steady-state, with and without HU therapy, and 30 healthy individuals were collected to evaluate MetHb levels. The MetHb measurement was performed by spectrophotometry. Complete blood count, HU measurements, and fetal hemoglobin (HbF) and HbS concentrations were taken from medical records. Results: MetHb levels were statically higher in patients with SCA when compared to control group (p < 0.001). There was no statistical difference in MetHb level between SCA patients, either using or not HU. We obtained a positive correlation between MetHb measurements and HbS concentration (r = 0.2557; p = 0.0323). Conclusion: HbS presence favored hemoglobin breaking down, and consequently increased MetHb production. Treatment with HU, however, did not influence the levels of this marker.

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Section: Discussionmentioning

confidence: 99%

Methemoglobin measure in adult patients with sickle-cell anemia: influence of hydroxyurea therapy

Laurentino¹,

Carvalho²,

Santos

et al. 2014

Jornal Brasileiro De Patologia E Medicina Laboratorial

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“…The plasma iron content was determined using an atomic absorption spectrophotometer and an air-acetylene flame. ( 15 )…”

Section: Methodsmentioning

confidence: 99%

Analysis of oxidative status and biochemical parameters in adult patients with sickle cell anemia treated with hydroxyurea, Ceará, Brazil

Neto¹,

Gonçalves²,

Elias³

et al. 2011

Rev. Bras. Hematol. Hemoter.

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BackgroundSickle cell anemia is a hemoglobinopathy caused by a mutation that results in the production of an abnormal hemoglobin molecule, hemoglobin S (Hb S). This is responsible for profound physiological changes, such as the sickling of red blood cells. Several studies have shown that hydroxyurea protects against vaso-occlusive crises.ObjectiveThe aim of this study was to evaluate the oxidative stress associated with biochemical parameters in patients with sickle cell anemia treated with hydroxyurea.MethodsThe study was conducted with 20 male and 25 female patients at the Hospital Universitário Walter Cantídio. The patients were divided into two groups: a study group (n = 12), patients with sickle cell anemia who were receiving hydroxyurea and a control group (n = 33) of sickle cell anemia patients not submitted to hydroxyurea treatment. The biochemical parameters analyzed were ferritin, transferrin, and serum iron. Glutathione was measured in its reduced form to analyze the oxidative state.ResultsThe results showed insignificant increases in the levels of serum iron, transferrin and ferritin in patients treated with hydroxyurea when compared with those who did not take the medication. However, the glutathione levels were significantly higher in patients taking hydroxyurea than in controls.ConclusionsThese results indicate that hydroxyurea possibly acts as an antioxidant by increasing glutathione levels.

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“…Sickle cell erythrocyte damage is directly intensified by the concentration of intra-erythrocyte Hb S (Beutler et al, 1995;Frenette and Atweh, 2007). Any pathology that increases radical generation, whether by an increase in oxidative stress or by an imbalance of antioxidant defenses, will increase the production of activated oxygen species, and will trigger oxidative processes with free radical generation (Naoum and Souza, 2004). A similar effect was found in cases of iron overload, as a consequence of hereditary hemochromatosis.…”

Section: Introductionmentioning

confidence: 96%

Comparison of oxidative stress and the frequency of polymorphisms in the HFE gene between hemoglobin S trait blood donors and sickle cell disease patients

Viana-Baracioli¹,

Tukamoto²,

Júnior³

et al. 2011

Genet. Mol. Res.

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ABSTRACT. It is well documented that Hb S and iron affect blood cells, and trigger oxidative processes and generation of free radicals with potential for lipid peroxidation. We evaluated the frequency of polymorphisms in the HFE gene in Hb AS blood donors and how these polymorphisms influenced lipid peroxidation and antioxidant capacity. Blood samples were collected from 211 Hb AS blood donors, 119 Hb AA blood donors as a control group, and 28 sickle cell disease patients (Hb SS). The H63D allele was found at a frequency of 10.5% in the Hb ©FUNPEC-RP www.funpecrp.com.br Genetics and Molecular Research 10 (4): 3446-3454 (2011) Oxidative stress, gene HFE mutation, and HbS 3447AS samples, and the C282Y allele frequency was 0.7%. In the control group, the frequencies of the H63D and C282Y alleles were 13.4 and 2.1%, respectively. In the sickle-cell disease patients, the H63D and the C282Y allele frequencies were 10.7 and 3.5%, respectively. The frequencies of the C282Y and H63D polymorphisms in Hb AS blood donors are similar to those reported for the Brazilian population. Serum malondialdehyde values, indicative of lipid peroxidation, were highest in sickle cell patients, independent of the polymorphisms in the HFE gene, with significant differences, showing the influence of Hb S allele in the levels of lipid peroxidation. However, the trolox equivalent antioxidant capacity average levels, indicative of the antioxidant capacity, were reduced with significant differences, indicating that in spite of a lipid peroxidation raise, this is not followed by the increased of the antioxidant capacity, leading to oxidative stress.

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Avaliação dos produtos da degradação oxidativa da Hb S nos genótipos SS, SF (S/beta0 talassemia) e AS, em comparação com hemoglobinas normais

Cited by 3 publications

References 9 publications

Methemoglobin measure in adult patients with sickle-cell anemia: influence of hydroxyurea therapy

Methemoglobin measure in adult patients with sickle-cell anemia: influence of hydroxyurea therapy

Analysis of oxidative status and biochemical parameters in adult patients with sickle cell anemia treated with hydroxyurea, Ceará, Brazil

Comparison of oxidative stress and the frequency of polymorphisms in the HFE gene between hemoglobin S trait blood donors and sickle cell disease patients

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