A associação anemia falciforme e hemoglobina fetal

Gualandro, Sandra Fátima Menosi

doi:10.1590/s1516-84842009000600003

Cited by 3 publications

(6 citation statements)

References 9 publications

(18 reference statements)

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“…Several studies show an association of the clinical heterogeneity ofSCD with Hb F levels and the intensity of the hemolytic process (3,18,19) . …”

Section: Discussionmentioning

confidence: 99%

Correlation of low levels of nitrite and high levels of fetal hemoglobin in patients with sickle cell disease at baseline

Elias¹,

Rocha²,

Cavalcante³

et al. 2012

Revista Brasileira De Hematologia E Hemoterapia

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Background Sickle cell disease is a hemoglobinopathy characterized by hemolytic anemia, increased susceptibility to infections and recurrent vaso-occlusive crises that reduces the quality of life of sufferers. Objective To evaluate the correlation of the levels of lactate dehydrogenase, malonaldehyde and nitrite to fetal hemoglobin in patients with sickle cell disease not under treatment with hydroxyurea in outpatients at a university hospital in Fortaleza, Ceará, Brazil. Methods Forty-four patients diagnosed with sickle cell disease were enrolled at baseline. Diagnosis was confirmed by evaluating the beta globin gene using polymerase chain reaction-restriction fragment length polymorphism. The concentration of fetal hemoglobin was obtained by high-performance liquid chromatography. Serum levels of nitrite, malonaldehyde and lactate dehydrogenase were measured by biochemical methods. Results Significantly higher levels of lactate dehydrogenase, nitrite and malonaldehyde were observed in patients with sickle cell disease compared to a control group. The study of the correlation between fetal hemoglobin levels and these variables showed a negative correlation with nitrite levels. No correlation was found between fetal hemoglobin and malonaldehyde or lactate dehydrogenase. When the study population was stratified according to fetal hemoglobin levels, a decrease in the levels of nitrite was observed with higher levels of fetal hemoglobin (p-value = 0.0415). Conclusion The results show that, similar to fetal hemoglobin levels, the concentration of nitrite can predict the clinical course of the disease, but should not be used alone as a modulator of prognosis in patients with sickle cell disease.

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“…Several studies show an association of the clinical heterogeneity ofSCD with Hb F levels and the intensity of the hemolytic process (3,18,19) . …”

Section: Discussionmentioning

confidence: 99%

Correlation of low levels of nitrite and high levels of fetal hemoglobin in patients with sickle cell disease at baseline

Elias¹,

Rocha²,

Cavalcante³

et al. 2012

Revista Brasileira De Hematologia E Hemoterapia

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show abstract

“…Sickle cell anemia (SCA) is a genetic disorder characterized by a point mutation in the beta globin gene generating, when homozygous, an abnormal hemoglobin known as hemoglobin S (Hb S) 1., 2.. Clinical manifestations result from the tendency of Hb S to polymerize in the deoxygenated state, causing vascular obstructions and ischemia, and consequent painful crises associated with episodes of chronic hemolysis 1., 3.…”

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Influence of βS-globin haplotypes hydroxyurea on tumor necrosis factor-alpha levels in sickle cell anemia

Laurentino

Filho

Barbosa

et al. 2014

Revista Brasileira de Hematologia e Hemoterapia

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BackgroundSickle cell anemia is a chronic inflammatory disease characterized by an increased production of proinflammatory cytokines including tumor necrosis factor-alpha. Hydroxyurea, by decreasing the polymerization of hemoglobin, reduces inflammatory states. The effect of the genetic polymorphisms of sickle cell patients on tumor necrosis factor-alpha levels remains unknown.ObjectiveThe aim of this study was to investigate the association of tumor necrosis factor-alpha levels with β-globin haplotypes and the use of hydroxyurea.MethodsA cross-sectional study was performed of 67 patients with sickle cell anemia diagnosed at steady-state in a referral hospital in Fortaleza, Ceará, Brazil. A group of 26 healthy individuals was used as control. βS haplotype analysis was performed by restriction fragment length polymorphism-polymerase chain reaction. The tumor necrosis factor- alpha levels were measured by the enzyme-linked immunosorbent assay test. Laboratory data (complete blood count and fetal hemoglobin) and information regarding the use of hydroxyurea were obtained from medical records. Statistical analysis was performed using R software with the Kruskal-Wallis and Mann–Whitney tests. Statistical significance was established for p-values < 0.05 for all analyses.ResultsThe mean age of the participants was 35.48 years. Patients with sickle cell anemia had significantly higher tumor necrosis factor-alpha levels than controls (p-values < 0.0001). Tumor necrosis factor-alpha levels were lower in sickle cell anemia patients who were receiving hydroxyurea treatment than those who were not (p-value = 0.1249). Sickle cell anemia patients with Bantu/n genotype had significantly higher levels than patients with the Bantu/Benin genotype (p-value = 0.0021).ConclusionIn summary, βS-globin haplotypes, but not hydroxyurea therapy, have a role in modulating tumor necrosis factor-alpha levels in sickle cell anemia adults at steady-state. Many previous studies have investigated prognosis and inflammatory states in sickle cell anemia patients, but the discovery that tumor necrosis factor-alpha levels vary according to the genetic polymorphism of the patient is a new finding. © 2014 Associação Brasileira de Hematologia, Hemoterapia e Terapia Celular. All rights reserved.

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“…A falcização da hemácia deu-se na troca do ácido glutâmico pela valina na posição 6 da cadeia β da globina, em sua forma não oxigenada leva a falcização da hemácia (GUALANDRO, 2009;BRUNETTA et al, 2010). Esta hemácia se torna mais rígida e em formato de foice o que caracteriza o nome de falciforme, por sua rigidez e formato e gera vários efeitos no organismo.…”

Section: Introductionunclassified

Estudo De Caso Sobre a Herança Genética Para Os Portadores De Traço Ou Anemia Falciforme

Broto

2019

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No Brasil observamos uma crescente quantidade de Hemoglobinopatias (HBs), com estimativa de 3.500 recém-nascidos vivos portadores de novos casos de doença falciforme ao ano, tendo com maior número o Traço Falciforme (TF), com estimativa de 7.200.000 portadores. Se tornando a doença hereditária com maior prevalência no Brasil, a qual se originou na África e disseminou-se de maneira agressiva por vários países. Nesse estudo será abordado como tema principal a relação entre o traço e a anemia falciforme que possui grandes impactos na saúde física e mental. Vale ressaltar que os portadores da anemia e traço falciforme necessitam de orientação sobre a doença, primordialmente a respeito da sua hereditariedade, proporcionando assim melhorias na qualidade de vida. Tendo como finalidade principal a descrição de um estudo de caso, com objetivo de avaliar através de exame de eletroforese a incidência de traço falciforme em uma família, o qual foi submetido e aprovado pelo comitê de ética. Os resultados confirmam a presença de TF nos filhos do casal, o que revela a importância da orientação genética para o grupo estudado, em relação à reprodução consciente, a qual é realizada através do exame de eletroforese de hemoglobina.

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A associação anemia falciforme e hemoglobina fetal

Cited by 3 publications

References 9 publications

Correlation of low levels of nitrite and high levels of fetal hemoglobin in patients with sickle cell disease at baseline

Correlation of low levels of nitrite and high levels of fetal hemoglobin in patients with sickle cell disease at baseline

Influence of βS-globin haplotypes hydroxyurea on tumor necrosis factor-alpha levels in sickle cell anemia

Estudo De Caso Sobre a Herança Genética Para Os Portadores De Traço Ou Anemia Falciforme

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