Real-world evidence of the burden of sickle cell disease: a 5-year longitudinal study at a Brazilian reference center.

Autor:	Barros GDS; Faculdade de Ciências Médicas da Santa Casa de São Paulo, São Paulo, SP, Brazil., Leal CVF; Faculdade de Ciências Médicas da Santa Casa de São Paulo, São Paulo, SP, Brazil., Leite LAC; Faculdade de Ciências Médicas da Santa Casa de São Paulo, São Paulo, SP, Brazil., Fujimoto DE; Faculdade de Ciências Médicas da Santa Casa de São Paulo, São Paulo, SP, Brazil., Cançado RD; Faculdade de Ciências Médicas da Santa Casa de São Paulo, São Paulo, SP, Brazil. Electronic address: rodolfo.cancado@gmail.com.
Jazyk:	angličtina
Zdroj:	Hematology, transfusion and cell therapy [Hematol Transfus Cell Ther] 2024 Apr-Jun; Vol. 46 (2), pp. 161-166. Date of Electronic Publication: 2023 Nov 26.
DOI:	10.1016/j.htct.2023.10.001
Abstrakt:	Introduction: Sickle cell disease (SCD) is an inherited and multisystem blood disorder characterized by hemolytic anemia, vaso-occlusive crises (VOCs), progressive multiorgan damage and increased mortality. In Brazil, it is one of the most common monogenic diseases afflicting 60,000 to 100,000 individuals, however, there are sparse epidemiological data, as well as information on the utilization of public healthcare resources. Method: This was a 5-year (2016 - 2020) retrospective study conducted at one Brazilian reference center on SCD - Santa Casa de Sao Paulo, in Sao Paulo, Brazil. Results: Among a total of 100 eligible adult patients, the median age was 31.0 years old, 84% of the patients were aged between 18 and 45 years old; 59% were women and 91% presented the genotype HbSS. The number of hematologist and non-hematologist visits at the outpatient unit were 2,198 and 1,436, respectively. The number of hospital ER visits was 758, of which 51% required 864 days of hospitalization. The main cause for seeking hospital medical care was the VOCs. The numbers and ratios of VOCs were: 1 to 10 VOCs, 64%; 11 to 20, 15%, and; 21 or more, 1%. There was a statistically significant difference between the number of VOCs and hospitalizations, as well as infection. Conclusion: Results indicate the burden of SCD on Brazilian patients' daily lives, the impact of VOCs on public healthcare resources, the importance of having a national surveillance program to improve resource utilization and clinical outcomes of patients with SCD and the urgent need for the revitalizing of the current national comprehensive SCD care programs. Competing Interests: Conflicts of interest None. (Copyright © 2023 Associação Brasileira de Hematologia, Hemoterapia e Terapia Celular. Published by Elsevier España, S.L.U. All rights reserved.)
Databáze:	MEDLINE
Externí odkaz:	Zobrazit plný text záznamu