Abstract
Sickle cell disease (SCD) is a severe inherited and multisystem blood disorder characterized by hemolytic anemia, vasoocclusive crises (VOCs), progressive multiorgan damage, and increased mortality. SCD is the most common inherited disorder worldwide, with well over 300,000 children born with SCD yearly, 75% of whom are born in Africa. Based on the Brazilian neonatal screening program, the annual average of new cases of children diagnosed with SCD was 1,087, with an incidence of 3.78 per 10,000 live births and an estimated number of 60,000 to 100,000 individuals living with SCD in Brazil. Our objectives were to describe the sociodemographic and genotype characteristics and clinical complications of patients with SCD and compare the laboratory profile and the impact of healthcare resource utilization between patients with and without hydroxyurea during five years of observation. Objectives: To describe the sociodemographic and clinical complications, compare the laboratory profile and the impact of healthcare resources utilization between patients with and without hydroxyurea of SCD patients during five years of observation. Method: This was a retrospective cross-sectional descriptive study conducted at one Brazilian reference center on SCD - Santa Casa de Sao Paulo, in Sao Paulo (Southeast region of Brazil) during a 5-year observation period (2016-2020).
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