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SICKLE CELL ANEMIA: DELAYED DIAGNOSIS IN BAHIA, BRAZIL-A LARGELY AFRO-DESCENDANT POPULATION
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Hematology and Hemotherapy Center of Bahia. Salvador, BA, Brazil
Fundação Oswaldo Cruz. Centro de Pesquisas Gonçalo Moniz. Salvador, BA, Brasil
Hematology and Hemotherapy Center of Bahia. Salvador, BA, Brazil
Hematology and Hemotherapy Center of Bahia. Salvador, BA, Brazil
Fundação Oswaldo Cruz. Centro de Pesquisas Gonçalo Moniz. Salvador, BA, Brasil
Fundação Oswaldo Cruz. Centro de Pesquisas Gonçalo Moniz. Salvador, BA, Brasil
Hematology and Hemotherapy Center of Bahia. Salvador, BA, Brazil
Hematology and Hemotherapy Center of Bahia. Salvador, BA, Brazil
Fundação Oswaldo Cruz. Centro de Pesquisas Gonçalo Moniz. Salvador, BA, Brasil
Abstract
Hemoglobinopathies are the most common
genetic disorders in the world and include
sickle cell anemia (SCA), which is a public
health problem in Brazil. Nevertheless, the
disease is highly unknown among health
professionals, and delayed diagnosis constitutes
an important cause of concern for
caretakers of SCA patients. The purpose of
this study was to compare the clinical and
laboratory history of SCA patients whose
diagnosis was established during the first year
of life to those of other SCA patients who had
delayed SCA diagnosis. Demographic, clinical,
and laboratory data were all reviewed from 99
steady-state SCA patients who were followed
in a public hematology and hemotherapy
clinic in Salvador, Brazil. The patients were
aged $12 years and attended the outpatient
unit at least once from November 2008 to
June 2009. The data were analyzed in 2010.
For all patients, the mean age (6 SD) at
diagnosis was 12.7(6 12.1) years, ranging from
0 to 47 years. Mean age was higher in patients
whose SCA diagnosis was established after age
5 (32.9 6 11.9 years, P5.005). Increased
unconjugated bilirubin, stroke and splenic
sequestration were more prevalent in patients
who were diagnosed in the first year of life
(P5.043, .024 and .026 respectively).
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