1 UEZO - Centro Universitário Estadual da Zona Oeste, Rio de Janeiro, RJ, Brazil.
2 Clinical Hematology Division, Instituto de Hematologia Arthur de Siqueira Cavalcanti. HEMORIO, Rio de Janeiro, RJ, Brazil.
3 School of Medicine/UFRJ - Federal University of Rio de Janeiro, RJ, Brazil.
4 Postgraduate School of Engineering – COPPE/UFRJ.
5 Clementino Fraga Filho University Hospital/UFRJ.
6 Cardeza Foundation, Department of Medicine, Jefferson Medical College, Thomas Jefferson University, Philadelphia, PA, USA.
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clinical picture of patients with sickle cell anemia (SCA) is
associated with several complications some of which could be fatal. The
objective of this study is to analyze the causes of death and the
effect of sex and age on survival of Brazilian patients with SCA. Data
of patients with SCA who were seen and followed at HEMORIO for 15 years
were retrospectively collected and analyzed. Statistical modeling was
performed using survival analysis in the presence of competing risks
estimating the covariate effects on a sub-distribution hazard function.
Eight models were implemented, one for each cause of death. The
cause‐specific cumulative incidence function was also estimated. Males
were most vulnerable for death from chronic organ damage (p = 0.0005)
while females were most vulnerable for infection (p=0.03). Age was
significantly associated (p ≤ 0.05) with death due to acute chest
syndrome (ACS), infection, and death during crisis. The lower survival
was related to death from infection, followed by death due to ACS. The
independent variables age and sex were significantly associated with
ACS, infection, chronic organ damage and death during crisis. These
data could help Brazilian authorities strengthen public policies to
protect this vulnerable population.
Material and Methods
|Table 1. Risk of subdistribution regression model.