Author | Gonçalves, Marilda de Souza | |
Author | Bomfim, Gilberto Cafezeiro | |
Author | Maciel, Elves Anderson Pires | |
Author | Cerqueira, I | |
Author | Lyra, Isa Menezes | |
Author | Zanette, Angela Maria Dias | |
Author | Bomfim, Maria da Glória | |
Author | Adorno, Elisângela Vitória | |
Author | Albuquerque, Arlete Barreto Lins de | |
Author | Pontes, A | |
Author | Dupuit, M. F | |
Author | Fernandes, Gilenio Borges | |
Author | Reis, Mitermayer Galvão dos | |
Access date | 2017-07-27T17:09:19Z | |
Available date | 2017-07-27T17:09:19Z | |
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 and Biological Research, v. 36, n. 10, p. 1283-1288, 2003. | pt_BR |
ISSN | 0100-879X | pt_BR |
URI | https://www.arca.fiocruz.br/handle/icict/20425 | |
Sponsorship | PAPESFIOCRUZ (No. 0250250304) and CNPq (No. 521201/96-1). | pt_BR |
Language | eng | pt_BR |
Publisher | Faculdade de Medicina de Ribeirao Preto. Associação Brasileira de Divulgação | pt_BR |
Rights | open access | pt_BR |
Subject in Portuguese | Beta (S) plantastipos | pt_BR |
Subject in Portuguese | Hemoglobina fetal | pt_BR |
Subject in Portuguese | Anemia falciforme | pt_BR |
Subject in Portuguese | Hemoglobina S | pt_BR |
Subject in Portuguese | População brasileira | 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. Laboratório de Patologia e Biologia Molecular. Salvador, BA, Brasil / Faculdade de Farmácia. Laboratório de Biologia Molecular. Departamento de Toxicologia e Análises Clínicas. Salvador, BA, Brasil | pt_BR |
Affilliation | Fundação Oswaldo Cruz. Centro de Pesquisas Gonçalo Moniz. Laboratório de Patologia e Biologia Molecular. Salvador, BA, Brasil | pt_BR |
Affilliation | Fundação Oswaldo Cruz. Centro de Pesquisas Gonçalo Moniz. Laboratório de Patologia e Biologia Molecular. Salvador, BA, Brasil | pt_BR |
Affilliation | Fundação Oswaldo Cruz. Centro de Pesquisas Gonçalo Moniz. Laboratório de Patologia e Biologia Molecular. Salvador, BA, Brasil | pt_BR |
Affilliation | Secretaria de Saúde da Bahia. Fundação Hemocentro da Bahia. Salvador, BA, Brasil | pt_BR |
Affilliation | Secretaria de Saúde da Bahia. Fundação Hemocentro da Bahia. Salvador, BA, Brasil | pt_BR |
Affilliation | Hospitalar Professor Edgard Santos. Salvador, BA, Brasil | pt_BR |
Affilliation | Fundação Oswaldo Cruz. Centro de Pesquisas Gonçalo Moniz. Laboratório de Patologia e Biologia Molecular. Salvador, BA, Brasil | pt_BR |
Affilliation | Fundação Oswaldo Cruz. Centro de Pesquisas Gonçalo Moniz. Laboratório de Patologia e Biologia Molecular. Salvador, BA, Brasil | pt_BR |
Affilliation | Faculdade de Farmácia. Laboratório de Biologia Molecular. Departamento de Toxicologia e Análises Clínicas. Salvador, BA, Brasil | pt_BR |
Affilliation | Faculdade de Farmácia. Laboratório de Biologia Molecular. Departamento de Toxicologia e Análises Clínicas. 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. Laboratório de Patologia e Biologia Molecular. 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 |