Factors associated with sickle cell disease mortality among hospitalized Angolan children and adolescents

Background: Sickle cell disease complications are an important mortality cause in children mainly in Africa and India. Notwithstanding the magnitude of the problem on the African continent, studies identifying factors related to the adverse outcomes of sickle cell disease in the pediatric population are still scarce. Objective: To identify prognostic factors associated with mortality in children and adolescent aged under fifteen years with diagnosis of sickle cell disease. Methods: Patients meeting inclusion criteria were listed and randomly selected. Clinical and laboratory data collected at time of admission were collected from medical records through the use of standard forms. The association between mortality and explanatory variables was tested using univariable and multivariable analysis. Results: The overall mortality rate was 64(12.9%), and bacterial infections 26(40.1%) were the most common cause of death. Place of residence out of Luanda, lack of outpatient follow-up, symptoms onset more than three days, disease manifestation before age of eighth months and hemoglobin level of < 7 g/dl were independent risk factors related to death. In the study population, sickle cell related deaths were related to quality of health care and access to care. Conclusion: The creation of regional sickle cell disease. centers to support those afflicted by the disorder and their families would contribute to reduce the burden associared with the disease.