Analysis of the temporal trend of mortality from sickle cell anemia in Brazil

Mota, Felipe Machado; Júnior, Ferreira; Cardoso, Andréia Insabralde de Queiroz; Pompeo, Carolina Mariano; Frota, Oleci Pereira; Tsuha, Daniel Henrique; Souza, Albert Schiaveto de

doi:10.1590/0034-7167-2021-0640

Cited by 6 publications

(10 citation statements)

References 27 publications

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“…The first Brazilian single institutional study in 2017 showed that the mortality rate was 18.87% among adult SCD patients 23 . Further, combined data between 1997 and 2017, from Brazil showed that mortality was more in the males (50.4%), and patients aged between 25 and 34 years had a higher incidence of deaths 24 . In another study from Brazil between 2000 and 2018, during the entire period the mean ages at death is significantly lower for males, than for females, being 29.4 (± 19.6) versus 33.3 (± 20.3) years respectively 25 .…”

Section: Discussionmentioning

confidence: 94%

Multi-center study on mortality in children, and adults with sickle cell anemia-risk factors and causes of death

Alkindi,

Al-Jadidi,

Al-Adawi

et al. 2024

Sci Rep

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Sickle cell disease (SCD) is a major public health burden worldwide with increasing morbidity and mortality. The study evaluates the risk factors associated with mortality in SCD patients, between the years 2006 and 2020 at three hospitals in Oman. The analysis includes clinical manifestations, haematological, biochemical, and radiological parameters, use of antibiotics, and blood and exchange transfusions. Our cohort included 123 patients (82 males, 41 females), with a median age of 27 (Interquartile Range 21–35 years). SCD related complications included acute chest syndrome (ACS) in 52.8%, splenic sequestration in 21.1%, right upper quadrant syndrome in 19.5%, more than > 6 VOC/year in 17.9%, and stroke in 13.8%. At the terminal admission, patients had cough, reduced O2 saturation, crepitation and fever in 24.4%, 49.6%, 53.6% and 68.3% respectively. Abnormal chest X-ray and chest CT scan were seen in 57.7%, and 76.4% respectively. Laboratory parameters showed a significant drop in hemoglobin (Hb) and platelet counts from baseline, with a significant rise in WBC, LDH and CRP from baseline (p < 0.05, Wilcoxon Signed Ranks test). All patients received antibiotics, whereas, 95.9% and 93.5% received simple blood transfusions, and exchange transfusions respectively, and 66.6% required non-invasive ventilation. Among the causes of death, ACS is seen in 32 (26%), sepsis in 49 (40%), and miscellaneous in 42 (34%). Sudden death was seen in 32 (26%) of patients. Male gender, with low HbF, rapid drop in Hb and platelet, and increased in WBC, LDH, ferritin, and CRP, correlated significantly with mortality in this cohort.

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Section: Discussionmentioning

confidence: 94%

Multi-center study on mortality in children, and adults with sickle cell anemia-risk factors and causes of death

Alkindi,

Al-Jadidi,

Al-Adawi

et al. 2024

Sci Rep

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“…123 patients (82 males, 41 females) who were enrolled in the study had a median age (IQR) of 27 (21)(22)(23)(24)(25)(26)(27)(28)(29)(30)(31)(32)(33)(34)(35) years, with a range between 1.5 to 79 years. Painful VOC episodes > 6/year were seen in almost one-fth of this cohort (17.9%), while most patients had a signi cant past history of SCD-related complications including ACS (52.8%), splenic sequestration (21.1%), right upper quadrant syndrome (19.5%) and stroke (13.8%) (Table 1).…”

Section: Resultsmentioning

confidence: 99%

Multi-center study on mortality in children, and adults with sickle cell anemia-risk factors and causes of death

Alkindi

Al-Jadidi

Al-Adawi

et al. 2023

Preprint

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Sickle cell disease (SCD) is a major public health burden worldwide with increasing morbidity and mortality. We studied the risk factors associated with mortality in SCD patients, between the years 2006 and 2020 at three hospitals in Oman. We analyzed clinical manifestations, haematological, biochemical, and radiological parameters, use of antibiotics, and blood and exchange transfusions. Our cohort included 123 patients (82 males, 41 females), with a median age of 27 (IQ 21–35 years). SCD related complications included acute chest syndrome (ACS) in 52.8%, splenic sequestration in 21.1%, right upper quadrant syndrome in 19.5%, more than > 6 VOC /year in 17.9%, and stroke in 13.8%. At the terminal event, patients had cough, reduced O2 saturation, crepitation and fever in 24.4%, 49.6%, 53.6% and 68.3% respectively. Abnormal chest x-ray and chest CT scan were seen in 57.7%, and 76.4% respectively. Laboratory parameters showed a significant drop in hemoglobin (Hb) and platelet counts from baseline, with a significant rise in WBC, LDH and CRP from baseline (p < 0.05, Wilcoxon Signed Ranks test).. All patients received antibiotics, whereas, 95.9% & 93.5% received simple blood transfusions, and exchange transfusions respectively, and 66.6% required non-invasive ventilation. Among the causes of death, ACS is seen in 32 (26%), sepsis in 49 (40%), and miscellaneous in 42 (34%). Sudden death was seen in 32 (26%) of patients. Male gender, with low HbF, rapid drop in Hb and platelet, and increased in WBC, LDH, ferritin, and CRP, correlated significantly with mortality in this cohort.

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“… 10 Mota et al analyzed data from 1997–2017 and observed a higher incidence of deaths between the ages of 25 and 34 years. 24 …”

Section: Discussionmentioning

confidence: 99%

Mortality trends and sociodemographic factors associated with early death in sickle cell disease patients in the state of São Paulo

Avelino,

Konstantyner,

Areco

et al. 2024

Rev. paul. pediatr.

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Objective: To estimate trends in mortality rate and average age of death, and identify sociodemographic factors associated with early death in patients with sickle cell disease (SCD). Methods: An ecological and cross-sectional study was conducted using data from the Mortality Information System. All deaths of patients residing in the state of São Paulo from 1996 to 2015 with at least one International Disease Code for SCD in any field of the death certificate were included. Simple linear regression was used to estimate trends. The Log-rank test and multiple Cox regression were used to identify factors associated with early death. Results: The age-standardized mortality rate per million inhabitants increased by 0.080 per year (R2=0.761; p<0.001). When the events were stratified by age at death, the increase was 0.108 per year for those occurring at age 20 years or older, (R2=0.789; p<0.001) and 0.023 per year for those occurring before age 20 years old (R2=0.188; p=0.056). The average age at death increased by 0.617 years (7.4 months) per year (R2=0.835; p<0.001). Sociodemographic factors associated with early death identified were male gender (hazard ratio — HR=1.30), white race (HR=1.16), death occurring in the hospital (HR=1.29), and living in the Greater São Paulo (HR=1.13). Conclusions: The mortality rate and the average age of death in patients with SCD have increased over the last two decades. Sociodemographic factors such as gender, race, place of occurrence, and residence were found to be associated with early death.

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Analysis of the temporal trend of mortality from sickle cell anemia in Brazil

Cited by 6 publications

References 27 publications

Multi-center study on mortality in children, and adults with sickle cell anemia-risk factors and causes of death

Multi-center study on mortality in children, and adults with sickle cell anemia-risk factors and causes of death

Multi-center study on mortality in children, and adults with sickle cell anemia-risk factors and causes of death

Mortality trends and sociodemographic factors associated with early death in sickle cell disease patients in the state of São Paulo

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