| Author | Gonçalves, Marilda de Souza | |
| Author | Arruda, Maria da Gloria Bomfim | |
| Author | Maciel, Elves Anderson Pires | |
| Author | Cerqueira, I. | |
| Author | Lyra, Isa Menezes | |
| Author | Zanette, Angela Maria Dias | |
| Author | Arruda, Maria da Gloria Bomfim | |
| Author | Adorno, Elisângela Vitória | |
| Author | Albuquerque, Alessandra B. Lima | |
| Author | Pontes, A. | |
| Author | Dupuit, Marie France | |
| Author | Fernandes, G. B. | |
| Author | Reis, Mitermayer Galvão dos | |
| Access date | 2012-12-13T17:11:00Z | |
| Available date | 2012-12-13T17:11:00Z | |
| Document date | 2003 | |
| Citation | GONÇALVES, M. S. et al. ßS-Haplotypes in sickle cell anemia patients from Salvador, Bahia, Northeastern Brazil. Brazilian Journal of Medical Biological Research, v. 36, n. 10, p. 1283-1288, Oct. 2003. | pt_BR |
| ISSN | 0100-879X | |
| URI | https://www.arca.fiocruz.br/handle/icict/5985 | |
| Language | eng | pt_BR |
| Rights | open access | pt_BR |
| Title | ßS-Haplotypes in sickle cell anemia patients from Salvador, Bahia, Northeastern Brazil | pt_BR |
| Type | Article | pt_BR |
| Abstract | 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 concentrations
and hematocrit values did not differ among genotype groups but were
significantly higher in 25 patients presenting percent fetal hemoglobin
(%HbF) ≥10% (P = 0.002 and 0.003, respectively). The median HbF
concentration was 7.54 ± 4.342% for the CAR/CAR genotype, 9.88 ±
3.558% for the BEN/BEN genotype, 8.146 ± 4.631% for the CAR/
BEN genotype, and 4.180 ± 2.250% for the CAR/Atp genotype
(P = 0.02), although 1 CAR/CAR individual presented an HbF concentration
as high as 15%. In view of the ethnic and geographical
origin of this population, we did not expect a Hardy-Weinberg equilibrium
for CAR/CAR and BEN/BEN homozygous haplotypes and a
high proportion of heterozygous CAR/BEN haplotypes since the State
of Bahia historically received more slaves from Western Africa than
from Central Africa. | pt_BR |
| Affilliation | Fundação Oswaldo Cruz. Centro de Pesquisas Gonçalo Moniz. Salvador, BA, Brasil. | pt_BR |
| Affilliation | Fundação Oswaldo Cruz. Centro de Pesquisas Gonçalo Moniz. Salvador, BA, Brasil. | pt_BR |
| Affilliation | Fundação Oswaldo Cruz. Centro de Pesquisas Gonçalo Moniz. Salvador, BA, Brasil. | pt_BR |
| Affilliation | Fundação Oswaldo Cruz. Centro de Pesquisas Gonçalo Moniz. Salvador, BA, Brasil. | pt_BR |
| Affilliation | Secretaria de Saúde do Estado da Bahia. Fundação Hemocentro da Bahia. Salvador, BA, Brasil. | pt_BR |
| Affilliation | Secretaria de Saúde do Estado da Bahia. Fundação Hemocentro da Bahia. Salvador, BA, Brasil. | pt_BR |
| Affilliation | Hospital Professor Edgar Santos. Salvador, BA, Brasil. | pt_BR |
| Affilliation | Fundação Oswaldo Cruz. Centro de Pesquisas Gonçalo Moniz. Salvador, BA, Brasil. | pt_BR |
| Affilliation | Fundação Oswaldo Cruz. Centro de Pesquisas Gonçalo Moniz. Salvador, BA, Brasil. | pt_BR |
| Affilliation | Faculdade de Farmácia. Laboratório de Biologia Molecular. Salvador, BA, Brasil. | pt_BR |
| Affilliation | Faculdade de Farmácia. Laboratório de Biologia Molecular. Salvador, BA, Brasil. | pt_BR |
| Affilliation | Universidade Federal da Bahia. Instituto de Matemática. Salvador, BA, Brasil. | pt_BR |
| Affilliation | Fundação Oswaldo Cruz. Centro de Pesquisas Gonçalo Moniz. Salvador, BA, Brasil. | pt_BR |
| Subject | Beta(S)-haplotypes | pt_BR |
| Subject | Fetal hemoglobin | pt_BR |
| Subject | Sickle cell anemia | pt_BR |
| Subject | S hemoglobin | pt_BR |
| Subject | Brazilian population | pt_BR |
| DeCS | Anemia Falciforme/genética | pt_BR |
| DeCS | Hemoglobina Fetal/análise | pt_BR |
| DeCS | Globinas/genética | pt_BR |
| DeCS | Haplotipos/genética | pt_BR |
| DeCS | Anemia Falciforme/sangue | pt_BR |
| DeCS | Anemia Falciforme/etnologia | pt_BR |
| DeCS | Brasil | pt_BR |
| DeCS | República Centro-Africana/etnologia | pt_BR |
| DeCS | Benin/etnologia | pt_BR |
| DeCS | Hemoglobina Fetal/genética | pt_BR |
| DeCS | Genótipo | pt_BR |
| DeCS | Immunoblotting | pt_BR |
| DeCS | Reação em Cadeia da Polimerase | pt_BR |
| DeCS | Senegal/etnologia | pt_BR |
| DeCS | Humanos | pt_BR |
| DeCS | Masculino | pt_BR |
| DeCS | Feminino | pt_BR |
