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| Medeiros,Fernanda Silva; Mendonça,Taciana Furtado de; Lopes,Katiuscia Araújo de Miranda; França,Laís Medeiros da Câmara; Silva,Andreia Soares da; Vasconcelos,Luydson Richardson Silva; Oliveira,Maria do Carmo Valgueiro Costa de; Anjos,Ana Cláudia Mendonça dos; Hatzlhofer,Betânia Lucena Domingues; Bezerra,Marcos André Cavalcanti; Araújo,Aderson da Silva; Moura,Patrícia; Cavalcanti,Maria do Socorro de Mendonça. |
| Abstract Sickle cell anemia (SCA) presents heterogenous clinical manifestations that cannot be explained solely by alterations to hemoglobin (Hb); other components such as endothelial adhesion, thrombosis and inflammation may be involved. The mannose-binding lectin (MBL) has an important role in innate immunity and inflammatory diseases. In this report, we describe an association between MBL2 polymorphism related to low production of serum MBL and the frequency of vasoocclusive events (FVOE) in children ≤ 5 years old with SCA (p = 0.0229; OR 5.55; CI 1.11-27.66). Further studies are needed to explore the role of low MBL2 in the pathophysiology of vasoocclusive events in SCA. |
| Tipo: Info:eu-repo/semantics/article |
Palavras-chave: MBL2; Polymorphism; Sickle cell anemia; Vaso-occlusive events. |
| Ano: 2017 |
URL: http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1415-47572017000400600 |
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| Furman,Aline Emmer Ferreira; Henneberg,Railson; Hermann,Priscila Bacarin; Leonart,Maria Suely Soares; Nascimento,Aguinaldo José do. |
| Sickle cell disease promotes hemolytic anemia and occlusion of small blood vessels due to the presence of high concentrations of hemoglobin S, resulting in increased production of reactive oxygen species and decreased antioxidant defense capacity. The aim of this study was to evaluate the protective action of a standardized extract of Ginkgo biloba (EGb 761), selected due to its high content of flavonoids and terpenoids, in erythrocytes of patients with sickle cell anemia (HbSS, SS erythrocytes) subjected to oxidative stress using tert-butylhydroperoxide or 2,2-azobis-(amidinepropane)-dihydrochloride, in vitro. Hemolysis indexes, reduced glutathione, methemoglobin concentrations, lipid peroxidation, and intracellular reactive oxygen species were... |
| Tipo: Info:eu-repo/semantics/article |
Palavras-chave: Ginkgo biloba; Oxidative stress; Erythrocytes; Azo compounds; Sickle cell anemia. |
| Ano: 2012 |
URL: http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1984-82502012000400009 |
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| Chies,J.A.B.; Hutz,M.H.. |
| Homozygous sickle cell disease (SCD) has a wide spectrum of clinical manifestations. In Brazil, the main cause of death of individuals with SCD is recurrent infection. The CCR5delta32 allele, which confers relative resistance to macrophage-tropic HIV virus infection, probably has reached its frequency and world distribution due to other pathogens that target macrophage in European populations. In the present investigation a relatively higher prevalence (5.1%) of the CCR5delta32 allele was identified, by PCR amplification using specific primers, in 79 SCD patients when compared to healthy controls (1.3%) with the same ethnic background (Afro-Brazilians). Based on a hypothesis that considers SCD as a chronic inflammatory condition, and since the CCR5... |
| Tipo: Info:eu-repo/semantics/other |
Palavras-chave: CCR5delta32; Sickle cell anemia; Inflammation. |
| Ano: 2003 |
URL: http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0100-879X2003000100010 |
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| Barbosa,C.G.; Goncalves-Santos,N.J.; Souza-Ribeiro,S.B.; Moura-Neto,J.P.; Takahashi,D.; Silva,D.O.; Hurtado-Guerrero,A.F.; Reis,M.G.; Goncalves,M.S.. |
| Fetal hemoglobin (HbF), encoded by the HBG2 and HBG1 genes, is the best-known genetic modulator of sickle cell anemia, varying dramatically in concentration in the blood of these patients. This variation is partially associated with polymorphisms located in the promoter region of the HBG2 and HBG1 genes. In order to explore known and unknown polymorphisms in these genes, the sequences of their promoter regions were screened in sickle cell anemia patients and correlated with both their HbF levels and their βS-globin haplotypes. Additionally, the sequences were compared with genes from 2 healthy groups, a reference one (N = 104) and an Afro-descendant one (N = 98), to identify polymorphisms linked to the ethnic background.The reference group was composed by... |
| Tipo: Info:eu-repo/semantics/article |
Palavras-chave: Fetal hemoglobin; Sickle cell anemia; HBG1 gene; HBG2 gene. |
| Ano: 2010 |
URL: http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0100-879X2010000800002 |
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| Lopes,A.J.; Marinho,C.L.; Alves,U.D.; Gonçalves,C.E.A.; Silva,P.O.; Botelho,E.C.; Bedirian,R.; Soares,A.R.; Maioli,M.C.P.. |
| Sickle cell anemia (SCA) causes dysfunction of multiple organs, with pulmonary involvement as a major cause of mortality. Recently, there has been growing interest in the nitrogen single-breath washout (N2SBW) test, which is able to detect ventilation heterogeneity and small airway disease when the results of other pulmonary function tests (PFTs) are still normal. Thus, the objectives of the present study were to assess the heterogeneity in the ventilation distribution in adults with SCA and to determine the association between the ventilation distribution and the clinical, cardiovascular, and radiological findings. This cross-sectional study included 38 adults with SCA who underwent PFTs, echocardiography, computed tomography (CT), and 6-min walk test. To... |
| Tipo: Info:eu-repo/semantics/article |
Palavras-chave: Sickle cell anemia; Respiratory mechanics; Respiratory function tests; Ventilation; Exercise. |
| Ano: 2017 |
URL: http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0100-879X2017000800607 |
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| Adorno,E.V.; Moura-Neto,J.P.; Lyra,I.; Zanette,A.; Santos,L.F.O.; Seixas,M.O.; Reis,M.G.; Goncalves,M.S.. |
| The fetal hemoglobin (HbF) levels and ßS-globin gene haplotypes of 125 sickle cell anemia patients from Brazil were investigated. We sequenced the Gg- and Ag-globin gene promoters and the DNase I-2 hypersensitive sites in the locus control regions (HS2-LCR) of patients with HbF level disparities as compared to their ßS haplotypes. Sixty-four (51.2%) patients had CAR/Ben genotype; 36 (28.8%) Ben/Ben; 18 (14.4%) CAR/CAR; 2 (1.6%) CAR/Atypical; 2 (1.6%) Ben/Cam; 1 (0.8%) CAR/Cam; 1 (0.8%) CAR/Arab-Indian, and 1 (0.8%) Sen/Atypical. The HS2-LCR sequence analyses demonstrated a c.-10.677G>A change in patients with the Ben haplotype and high HbF levels. The Gg gene promoter sequence analyses showed a c.-157T>C substitution shared by all patients, and a... |
| Tipo: Info:eu-repo/semantics/other |
Palavras-chave: Fetal hemoglobin; Sickle cell anemia; SSS-globin gene haplotypes; Locus control region; G-globin promoter. |
| Ano: 2008 |
URL: http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0100-879X2008000200003 |
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| Gonçalves,M.S.; Bomfim,G.C.; Maciel,E.; Cerqueira,I.; Lyra,I.; Zanette,A.; Bomfim,G.; Adorno,E.V.; Albuquerque,A.L.; Pontes,A.; Dupuit,M.F.; Fernandes,G.B.; Reis,M.G. dos. |
| ßS-Globin haplotypes were studied in 80 (160 ßS chromosomes) sickle cell disease patients from Salvador, Brazil, a city with a large population of African origin resulting from the slave trade from Western Africa, mainly from the Bay of Benin. Hematological and hemoglobin analyses were carried out by standard methods. The ßS-haplotypes were determined by PCR and dot-blot techniques. A total of 77 (48.1%) chromosomes were characterized as Central African Republic (CAR) haplotype, 73 (45.6%) as Benin (BEN), 1 (0.63%) as Senegal (SEN), and 9 (5.63%) as atypical (Atp). Genotype was CAR/CAR in 17 (21.3%) patients, BEN/BEN in 17 (21.3%), CAR/BEN in 37 (46.3%), BEN/SEN in 1 (1.25%), BEN/Atp in 1 (1.25%), CAR/Atp in 6 (7.5%), and Atp/Atp in 1 (1.25%). Hemoglobin... |
| Tipo: Info:eu-repo/semantics/article |
Palavras-chave: Beta(S)-haplotypes; Fetal hemoglobin; Sickle cell anemia; S hemoglobin; Brazilian population. |
| Ano: 2003 |
URL: http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0100-879X2003001000001 |
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