Revista Brasileira de Hematologia e Hemoterapia Revista Brasileira de Hematologia e Hemoterapia
Rev Bras Hematol Hemoter 2017;39:52-6 DOI: 10.1016/j.bjhh.2016.09.008
Review article
Mortality by sickle cell disease in Brazil
Giovanna Abadia Oliveira Arduini1, Let??cia Pinto Rodrigues1, Alessandra Bernadete Trov?? de Marqui,
Universidade Federal do Tri??ngulo Mineiro (UFTM), Uberaba, MG, Brazil
Received 15 May 2016, Accepted 09 September 2016
Abstract

This work aimed to characterize mortality by sickle cell disease in Brazil. The MEDLINE electronic database was searched using the terms ‘mortality’ and ‘sickle cell disease’ and ‘Brazil’ for articles published in the last five years aiming to provide a current analysis of the subject in question. Eight studies on mortality by sickle cell disease were carried out in the Brazilian states of Maranhão, Bahia, Minas Gerais, Rio de Janeiro and Mato Grosso do Sul. The majority of the deaths occurred in patients with sickle cell anemia, which is the most common genotype and causes the most severe clinical manifestation of the disease. In summary, there are few published studies on mortality related to sickle cell disease in Brazil, and most are from the state of Minas Gerais. This study emphasizes the importance of developing more studies on sickle cell disease mortality, so that it may be possible to profile gene carriers and give health professionals more data to strategize the delivery of more effective assistance to these individuals. Despite the early diagnosis of sickle cell disease by the Neonatal Screening Program and the use of preventive and therapeutic measures (penicillin, immunization and hydroxyurea), mortality by sickle cell disease on the world stage is still significant.

Keywords
Sickle cell anemia, Mortality, Neonatal screening, Infection, Acute chest syndrome
Rev Bras Hematol Hemoter 2017;39:52-6 DOI: 10.1016/j.bjhh.2016.09.008