Mortality in children, adolescents and adults with sickle cell anemia in Rio de Janeiro, Brazil

Autor: Clarisse Lopes de Castro Lobo, Emilia Matos do Nascimento, Leonardo José Carvalho de Jesus, Thiago Gotelip de Freitas, Jocemir Ronaldo Lugon, Samir K. Ballas
Jazyk: angličtina
Rok vydání: 2018
Předmět:
Zdroj: Hematology, Transfusion and Cell Therapy, Vol 40, Iss 1, Pp 37-42 (2018)
Druh dokumentu: article
ISSN: 2531-1379
DOI: 10.1016/j.bjhh.2017.09.006
Popis: Objective: To determine the mortality rate of children, adolescents and adults with sickle cell anemia in Rio de Janeiro, Brazil. Methods: The number of deaths, the mortality rate and the causes of deaths in patients with sickle cell anemia who were treated and followed up at our institution for 15 years were determined and compared to data available for the Brazilian population. Results: The overall number of deaths was 281 patients with a mortality rate of 16.77%. Survival probability was significantly higher in females. The number of deaths and the mortality rate were age-specific with a significant increase in the 19- to 29-year-old age group. The remaining life expectancy of the patients with sickle cell anemia was less than that of Brazilians at large. The gap between the two was about 20 years for ages between one and five years with this gap decreasing to ten years after the age of 65 years. The most common causes of death were infection, acute chest syndrome, overt stroke, organ damage and sudden death during painful crises. Conclusion: To the best of our knowledge, this is the first Brazilian study in a single institution in Rio de Janeiro; the mortality rate was 18.87% among adult patients with sickle cell anemia. The mortality rates in children and adults are higher than those reported in developed countries of the northern hemisphere. Keywords: Sickle cell anemia, Vaso-occlusive crisis, Mortality, Newborn screening, Survival
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