Effect of alpha-thalassemia and beta-globin gene cluster haplotypes on the hematological and clinical features of sickle-cell anemia in Brazil

dc.creatorFigueiredo, MS
dc.creatorKerbauy, J
dc.creatorGoncalves, MS
dc.creatorArruda, VR
dc.creatorSaad, STO
dc.creatorSonati, MF
dc.creatorStoming, T
dc.creatorCosta, FF
dc.date1996
dc.dateOCT
dc.date2014-12-16T11:36:22Z
dc.date2015-11-26T16:22:10Z
dc.date2014-12-16T11:36:22Z
dc.date2015-11-26T16:22:10Z
dc.date.accessioned2018-03-28T23:03:52Z
dc.date.available2018-03-28T23:03:52Z
dc.identifierAmerican Journal Of Hematology. Wiley-liss, v. 53, n. 2, n. 72, n. 76, 1996.
dc.identifier0361-8609
dc.identifierWOS:A1996VQ09900003
dc.identifier10.1002/(SICI)1096-8652(199610)53:2<72
dc.identifierhttp://www.repositorio.unicamp.br/jspui/handle/REPOSIP/63737
dc.identifierhttp://www.repositorio.unicamp.br/handle/REPOSIP/63737
dc.identifierhttp://repositorio.unicamp.br/jspui/handle/REPOSIP/63737
dc.identifier.urihttp://repositorioslatinoamericanos.uchile.cl/handle/2250/1268057
dc.descriptionTo compare the features of sickle-cell anemia in Brazil with those in other locales, we studied the effects of the beta-globin-like gene cluster haplotype and alpha-thalassemia upon the clinical and hematological features in 85 patients, The distribution of haplotypes differed from that in the United States and Jamaica, The Central African Republic (CAR) haplotype predominated; 34% of patients were CAR haplotype homozygotes, 45% CAR/Benin homozygotes, and 71% Benin homozygotes. No Senegal haplotype chromosomes were observed, alpha-thalassemia was present in 17.5% of patients, HbF levels were higher in Benin homozygotes, compared with the other two groups (P < 0.05). Nearly half the patients with a CAR haplotype had leg ulcers, compared to 12.5% of the Benin homozygote group; stroke did not occur in alpha-thalassemia carriers, but neither result was statistically significant. As in other studies, our results indicate that the CAR haplotype may be associated with more severe disease. (C) 1996 Wiley-Liss, Inc.
dc.description53
dc.description2
dc.description72
dc.description76
dc.languageen
dc.publisherWiley-liss
dc.publisherNew York
dc.relationAmerican Journal Of Hematology
dc.relationAm. J. Hematol.
dc.rightsfechado
dc.rightshttp://olabout.wiley.com/WileyCDA/Section/id-406071.html
dc.sourceWeb of Science
dc.subjectsickle-cell disease
dc.subjectclinical features
dc.subjecthemoglobin S
dc.subjecthaplotypes
dc.subjectalpha-thalassemia
dc.subjectFetal Hemoglobin
dc.subjectS-haplotypes
dc.subjectDisease
dc.subjectAlpha-thalassemia-2
dc.subjectPopulations
dc.subjectPrevalence
dc.subjectChildren
dc.titleEffect of alpha-thalassemia and beta-globin gene cluster haplotypes on the hematological and clinical features of sickle-cell anemia in Brazil
dc.typeArtículos de revistas


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