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Published on: March 14, 2017
Factors associated with sickle cell disease mortality among hospitalized Angolan children and adolescents
J C Carlos Van-Dunem1, J G B Alves, Luis Bernardino
1Hospital Pediátrico de Luanda, Universidade Agostinho Neto, Luanda, Angola, Brazil.
Insights
Sickle cell disease (SCD) complications cause child mortality in Africa. Key risk factors for death in children with SCD include lack of follow-up care and low hemoglobin levels, highlighting access to care issues.
Area of Science:
- Pediatric Hematology
- Global Child Health
Background:
- Sickle cell disease (SCD) is a major cause of mortality in children, particularly in Africa and India.
- Research on prognostic factors for adverse SCD outcomes in African children is limited.
Purpose of the Study:
- To identify prognostic factors associated with mortality in children and adolescents under 15 with sickle cell disease.
Main Methods:
- Retrospective study of pediatric patients diagnosed with sickle cell disease.
- Data collected included clinical and laboratory parameters at admission.
- Univariable and multivariable analyses were used to assess the association between variables and mortality.
Main Results:
- The overall mortality rate was 12.9% (64 deaths).
- Bacterial infections accounted for 40.1% of deaths.
- Independent risk factors for mortality included residing outside Luanda, lack of outpatient follow-up, delayed symptom onset (>3 days), early disease manifestation (<8 months), and low hemoglobin (<7 g/dl).
- Sickle cell-related deaths were linked to healthcare quality and access.
Conclusions:
- Establishing regional sickle cell disease centers is crucial.
- These centers can support patients and families, potentially reducing the disease burden.
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 associated with the disease.
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