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.

Sickle Cell Anaemia Prevalence among Newborns in the Brazilian Amazon-Savanna Transition Region

“…Another factor that may have impacted the reduction in the number of cases is prevention actions, such as genetic counselling, directed at parents and individuals identified as having sickle cell trait [25]. Our findings in Maranhão are similar to those in other Northeast states including Bahia [15,26], Rio Grande do Norte [27], and Rio de Janeiro [28], and Minas Gerais [29] in the Southeast, emphasizing the correlation with the history of Brazilian colonisation and reflecting the influx of immigrants of African descent which occurred mainly in these states.…”

Sickle cell disease

“…The incidence of SCD in newborn babies varies substantially among the states in Brazil, reflecting the ethnic heterogeneity of the Brazilian population. In 2014 the incidence of SCD was ~1 in 650 newborn babies screened in the state of Bahia, 1 in 1,300 in the state of Rio de Janeiro and 1 in 13,500 in the state of Santa Catarina 25 . Nationwide, in 2016, 1,071 newborn babies had SCD and >60,000 were heterozygotes for the β S allele 26 .…”

Optimized Antigen-Matched in Sickle Cell Disease Patients: Chances and Challenges in Molecular Times - the Brazilian Way

“…The incidence of SCD in newborn babies varies among the states in Brazil, reflecting the ethnic heterogeneity of the Brazilian population. SCD is more prevalent in the state of Bahia with 1 in 650 patients affected, followed by Rio de Janeiro with 1 in 1,200 patients affected [1]. The Africans brought to Brazil belonged to two major groups: the West-African and the Bantu people (Angola, Congo, and Mozambique).…”