title: Screening for Structural Hemoglobin Variants in Bahia, Brazil.
abstract: Brazil was the country that received the largest number of Africans during the time of colonization, and Bahia was the Brazilian state that received the largest number of slaves from Africa. The purpose of this study was to evaluate the coverage of the newborn screening program for sickle cell disease in the Recôncavo Baiano region of the state of Bahia, and to show the frequency of the subjects with hemoglobin variants in the 2006-2009 period. Blood samples from neonates in twelve cities in the Recôncavo Baiano region were analyzed by High Performance Liquid Chromatography. A total of 16,402 children were born in this period, 14,773 of which underwent newborn screening. In this period 1416 children were born carrying hemoglobin variants HbS and HbC. Forty-seven patients--20 HbSS genotype and 27 HbSC genotype--were diagnosed in eleven of the twelve cities surveyed. The proportion of children born with sickle cell disease in the Recôncavo Baiano region was 1/314, which was higher than the 1/650 rate for the state of Bahia. The data presented in this study confirm the high frequency of sickle cell disease in Recôncavo Baiano, demonstrating the need to create a referral center for the care of patients with sickle cell diseases in the region.
authors.affiliation: Bahia Adventist College, 44300-000 Cachoeira, Bahia, Brazil. profwellington@hotmail.com.|Functional Electrostimulation Laboratory, Biomorphology Department, Health Sciences Instituto, Federal University of Bahia, Av. Reitor Miguel Calmon, s/n Vale do Canela, 40110-902 Salvador, Bahia, Brazil. profwellington@hotmail.com.|Graduate Program on Medicine and Health, Faculty of Medicine, Federal University of Bahia, Rua Augusto Viana s/n Canela-Hospital Universitário Prof. Edgard Santos, 5o andar, 40110-060 Salvador, Bahia, Brazil. profwellington@hotmail.com.
authors.forename: Wellington Santos
authors.initials: WS
authors.lastname: Silva
authors.affiliation: Bahia Adventist College, 44300-000 Cachoeira, Bahia, Brazil. prof.robertoferreira@icloud.com.
authors.forename: Roberto Ferreira
authors.initials: RF
authors.lastname: de Oliveira
authors.affiliation: Bahia Adventist College, 44300-000 Cachoeira, Bahia, Brazil. sanziar@gmail.com.
authors.forename: Sanzia Bezerra
authors.initials: SB
authors.lastname: Ribeiro
authors.affiliation: Bahia Adventist College, 44300-000 Cachoeira, Bahia, Brazil. isabelambrosio-enf@hotmail.com.
authors.forename: Isabel Batista
authors.initials: IB
authors.lastname: da Silva
authors.affiliation: Feira de Santana State University, Health Department, 44031-460 Feira de Santana, Bahia, Brazil. ednakam@gmail.com.
authors.forename: Edna Maria
authors.initials: EM
authors.lastname: de Araújo
authors.affiliation: Functional Electrostimulation Laboratory, Biomorphology Department, Health Sciences Instituto, Federal University of Bahia, Av. Reitor Miguel Calmon, s/n Vale do Canela, 40110-902 Salvador, Bahia, Brazil. abrahao.baptista@gmail.com.|Graduate Program on Medicine and Health, Faculty of Medicine, Federal University of Bahia, Rua Augusto Viana s/n Canela-Hospital Universitário Prof. Edgard Santos, 5o andar, 40110-060 Salvador, Bahia, Brazil. abrahao.baptista@gmail.com.
authors.forename: Abrahão Fontes
authors.initials: AF
authors.lastname: Baptista
mesh_terms: D000755:Anemia, Sickle Cell / Q000175:diagnosis / Q000453:epidemiology / Q000235:genetics*; D044383:Black People / Q000235:genetics*; D001797:Blood Protein Electrophoresis; D001938:Brazil / Q000453:epidemiology; D005260:Female; D005838:Genotype; D006403:Hematologic Tests; D006451:Hemoglobin, Sickle / Q000032:analysis*; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D015997:Neonatal Screening; D015397:Program Evaluation
chemical_list: D006451:Hemoglobin, Sickle
keywords: hemoglobin variants; newborn screening; sickle cell disease