{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,28]],"date-time":"2026-02-28T10:49:27Z","timestamp":1772275767701,"version":"3.50.1"},"reference-count":31,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2011,3,8]],"date-time":"2011-03-08T00:00:00Z","timestamp":1299542400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["American J Hum Biol"],"published-print":{"date-parts":[[2011,5]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p><jats:bold>Objective:<\/jats:bold> To analyze the frequency of the haplotypes of \u03b2\u2010globin gene cluster in randomly selected patients withsickle cell disease (SCD), attended in the Children's Hospital of Panama.<\/jats:p><jats:p><jats:bold>Methods:<\/jats:bold> Five polymorphic sites in the \u03b2\u2010globin gene cluster were analyzed by polymerase chain reaction (PCR) followed by restriction digestion and agarose gel electrophoresis in a total of 100 patients, including 95 homozygous for HbS (sickle cell anemia) and 5 compound heterozygotes for HbS and HbC genes (HbSC disease).<\/jats:p><jats:p><jats:bold>Results:<\/jats:bold> The Bantu haplotype was predominant with a frequency of 51%, followed by the Benin (30%), Senegal (8.5%), and Cameroon (4%); other haplotypes were also identified. Genotype was CAR\/CAR in 39 patients, BEN\/BEN in 22, SEN\/SEN in 6, CAM\/CAM in 4, ARB\/ARB in 1, CAR\/BEN in 15, CAR\/SEN in 5, CAR\/Hp5 in 3, CAR\/Hp1 in 1, BEN\/Hp11 in 1, Atp Hp1\/Hp1 in 2, and Atp Hp5\/Hp5 in 1 individual. Hemoglobin concentrations, hematocrit, and mean corpuscular hemoglobin concentration values did not differ among homozygous forms of haplotypes. The mean HbF in all patients was 15.39 \u00b1 1.21, whereas SEN\/SEN patients had higher HbF than BEN\/BEN patients (24.26 \u00b1 4.18 vs. 13.17 \u00b1 2.39, respectively, <jats:italic>P<\/jats:italic> &lt; 0.05). The percentage of reticulocytes was highest in BEN\/BEN and CAR\/CAR, and it was associated with worst prognosis.<\/jats:p><jats:p><jats:bold>Conclusion:<\/jats:bold> The results show the presence of common \u03b2<jats:sup>S<\/jats:sup> haplotypes in Panama; the prevalence of African origin, and the similarity in the Panamanian and Colombian distribution of haplotypes. Am. J. Hum. Biol. 2011. \u00a9 2011 Wiley\u2010Liss, Inc.<\/jats:p>","DOI":"10.1002\/ajhb.21148","type":"journal-article","created":{"date-parts":[[2011,3,8]],"date-time":"2011-03-08T18:22:54Z","timestamp":1299608574000},"page":"377-380","source":"Crossref","is-referenced-by-count":12,"title":["\u03b2\u2010globin gene cluster haplotypes in sickle cell patients from Panam\u00e1"],"prefix":"10.1002","volume":"23","author":[{"given":"Iryna","family":"Rusanova","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gladys","family":"Cossio","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"B\u00e9lgica","family":"Moreno","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"F.","family":"Javier Perea","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Rosaura G.","family":"De Borace","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mauricio","family":"Perea","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Germaine","family":"Escames","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Dar\u00edo","family":"Acu\u00f1a\u2010Castroviejo","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2011,3,8]]},"reference":[{"key":"e_1_2_6_2_1","first-page":"1","article-title":"Factors associated with the distribution and severity of sickle cell disease in Africa","volume":"17","author":"Adekile AD","year":"1994","journal-title":"Sphere"},{"key":"e_1_2_6_3_1","doi-asserted-by":"publisher","DOI":"10.1038\/1841877a0"},{"key":"e_1_2_6_4_1","volume-title":"Historia de Panam\u00e1 y sus protagonistas: una visi\u00f3n de la historia nacional desde el per\u00edodo precolombino hasta finales del siglo XX","author":"Conte\u2010Porras J","year":"1998"},{"key":"e_1_2_6_5_1","doi-asserted-by":"publisher","DOI":"10.3109\/03630260008997529"},{"key":"e_1_2_6_6_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-294X.2006.03067.x"},{"key":"e_1_2_6_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/0006-3002(59)90183-0"},{"key":"e_1_2_6_8_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajhb.20732"},{"key":"e_1_2_6_9_1","doi-asserted-by":"crossref","first-page":"1742","DOI":"10.1182\/blood.V69.6.1742.1742","article-title":"Fetal hemoglobin levels and beta (s) globin haplotypes in an Indian population with sickle cell disease","volume":"69","author":"Kulozik AE","year":"1987","journal-title":"Blood"},{"key":"e_1_2_6_10_1","first-page":"567","volume-title":"Man's haemoglobins","author":"Lehmann H","year":"1974"},{"key":"e_1_2_6_11_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajhb.20467"},{"key":"e_1_2_6_12_1","doi-asserted-by":"crossref","first-page":"1604","DOI":"10.1182\/blood.V87.4.1604.bloodjournal8741604","article-title":"Fetal hemoglobin in sickle cell anemia: relation to regulatory sequences cis to the beta\u2010globin gene. 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