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beta S haplotypes, alpha-globin gene status, and hematological data of sickle cell disease patients in Guadeloupe (F.W.I.).
Kéclard, L; Ollendorf, V; Berchel, C; Loret, H; Mérault, G.
Afiliación
  • Kéclard L; INSERM U.359, CHRU Pointe-à-Pitre, Guadeloupe, France.
Hemoglobin ; 20(1): 63-74, 1996 Feb.
Article en En | MEDLINE | ID: mdl-8745433
The beta gene cluster haplotypes, alpha gene status, Hb F level and hematological parameters have been characterized in 154 unrelated Guadeloupe patients with sickle cell disease: 112 with sickle cell anemia, 26 with SC disease, 15 with Hb S-beta-thalassemia, and one patient with Hb S in association with the hereditary persistence of fetal hemoglobin. Fourteen haplotypes in 16 combinations were found, the three major African haplotypes were present on 92% of all chromosomes: 73% Benin, 11% Bantu, 8% Senegal. Among SS patients, 57% were Benin homozygotes, one patient was a Senegal homozygote, one patient was a Bantu homozygote, and all the others were heterozygous. The A gamma T chain was observed on seven chromosomes and about 5% of the analyzed beta S chromosomes exhibited atypical haplotypes. The common haplotype beta C was found in all patients with SC disease. An interesting feature was the high frequency (44%) of deletional alpha-thalassemia among SS patients. Two patients have an alpha-gene globin triplication. The DNA haplotypes and alpha-gene status have been correlated with hematological parameters in these patients. The anthropological aspect of these data is interesting as the haplotypes of the beta-globin gene throw light on the slave trade from the various parts of Africa to the Caribbean Islands in particular, and North America in general.
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Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Hemoglobina Fetal / Globinas / Anemia de Células Falciformes Límite: Adolescent / Child / Female / Humans / Male País/Región como asunto: Caribe / Guadalupe Idioma: En Revista: Hemoglobin Año: 1996 Tipo del documento: Article País de afiliación: Francia Pais de publicación: Reino Unido
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Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Hemoglobina Fetal / Globinas / Anemia de Células Falciformes Límite: Adolescent / Child / Female / Humans / Male País/Región como asunto: Caribe / Guadalupe Idioma: En Revista: Hemoglobin Año: 1996 Tipo del documento: Article País de afiliación: Francia Pais de publicación: Reino Unido