Survival of sickle cell disease patients diagnosed during newborn screening: systematic review
DOI:
https://doi.org/10.33448/rsd-v10i11.19329Keywords:
Sickle cell disease; Neonatal screening; Survival Analysis; Epidemiology; Evidence-based nursing.Abstract
Objective: To analyze the survival data of patients with sickle cell disease diagnosed during the neonatal screening process. Method: Systematic review, carried out in the electronic databases Current Index to Nursing and Allied Health Literature, Cochrane Library, National Library of Medicine, Scopus, and Web of Science with controlled descriptors indexed in the Medical Subject Headings. No time or language constraints. Results: Seven articles composed the final sample, all published in English and with a cohort design. The assessment of the risk of bias by the Newcastle Ottawa Scale indicated that six articles were considered of high quality. Overall survival rates, stratified by genotypes, acute clinical events, sex, and hemoglobin levels, in addition to event-free survival were studied. Overall survival varied between 100% at six months and 85.6% at 18 years, while the Sβº genotype was estimated at 55.2% at 40 years. Conclusion: However, there was a decrease in survival after 30 years of age, with an annual increase between the periods studied. The development of public policies with the inclusion of the community in the context of its actions can help maintain strategies to improve the survival of this population.
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Copyright (c) 2021 Carolina Mariano Pompeo; Marcos Antonio Ferreira Júnior; Andreia Insabralde de Queiroz Cardoso; Mercy da Costa Souza; Felipe Machado Mota; Maria Lúcia Ivo
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