Sickle cell disease related mortality in Brazil, 2000–2018

Santo, Augusto Hasiak

doi:10.1016/j.htct.2020.09.154

Cited by 20 publications

(22 citation statements)

References 22 publications

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“…For example, a study carried out in Rio de Janeiro, Brazil, found a mortality rate of 10.48% for children under 18 years of age and 16.77% for cases aged 18 years and over. 50 Another study 55 found a mean age of death of 29.4 (± 19.5) years for SCD as the primary cause of death and 36.0 (± 20.6) as a secondary cause according to official death records, age data similar to that found in this research.…”

supporting

confidence: 84%

Clinical-Epidemiological Characteristics and Mortality in Patients with Sickle Cell Anemia: A Retrospective Cohort Study of 1980 at 2018

Pompeo¹,

Júnior²,

Cardoso³

et al. 2022

IJGM

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To analyze the clinical-epidemiological characteristics and mortality in patients with sickle-cell anemia (SCA). Patients and Methods: A cohort study with retrospective data, conducted in two reference hospitals for SCA treatment from January 1980 to December 2018, recorded in two reference services. With a 5% significance level, the Chi-Square and Student's t-tests were employed in the inferential statistical analysis. Results: A total of 128 patients with SCA were studied. Diagnosis up to the fifth day of life was made in 10 patients. There were 19 deaths, of which 12 (63.2%) were female, and the average age at death was 27.05 (± 14.78) years. The leading causes of death were septic shock and cardiogenic shock. The use of invasive medical devices was considered a risk factor for death (RR=2.63; 95% CI=1.16-5.96; p=0.018), and monitoring time up to 20 years presented a 31% reduction in the risk of dying (RR=0.31; 95% CI=0.12-0.82; p=0.011) when compared to the monitoring of more than 20 years. Conclusion: These findings are to be considered in the treatment of patients with SCA, mainly regarding early diagnosis and access to the treatment immediately afterward, since they are fundamental in improving survival and reducing severe complications.

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supporting

confidence: 84%

Clinical-Epidemiological Characteristics and Mortality in Patients with Sickle Cell Anemia: A Retrospective Cohort Study of 1980 at 2018

Pompeo¹,

Júnior²,

Cardoso³

et al. 2022

IJGM

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“…According to the IBGE population projection, this prevalence results in approximately 50,000 patients with the disease in 2018, segmented by age [ 30 ]. A mortality rate of 2.68/1,000,000 inhabitants was assumed, as the weighted average for men and women resulted in 558 patients lost to the disease in the same year [ 31 ]. The estimative is that the SCD mortality rate is underreported because the primary diagnosis is omitted and considered only the immediate death cause, as an acute stroke or acute chest syndrome.…”

Section: Resultsmentioning

confidence: 99%

Economic burden of sickle cell disease in Brazil

et al. 2022

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Background Sickle cell disease (SCD) may cause several impacts to patients and the whole society. About 4% of the population has the sickle cell trait in Brazil, and 60,000 to 100,000 have SCD. However, despite recognizing the significant burden of disease, little is known about SCD costs. Objective To estimate SCD societal costs based on disease burden modelling, under Brazilian societal perspective. Methods A disease burden model was built considering the societal perspective and a one-year time horizon, including direct medical and indirect costs (morbidity and mortality). The sum of life lost and disability years was considered to estimate disability-adjusted life years (DALYs). Data from a public database (DATASUS) and the prevalence obtained from literature or medical experts were used to define complications prevalence and duration. Costs were defined using data from the Brazilian public healthcare system table of procedures and medications (SIGTAP) and the human capital method. Results Annual SCD cost was 413,639,180 USD. Indirect cost accounted for the majority of burden (70.1% of the total; 290,158,365 USD vs 123,480,816 USD). Standard of care and chronic complications were the main source of direct costs among adults, while acute conditions were the main source among children. Vaso-occlusive crisis represented the complication with the highest total cost per year in both populations, 11,400,410 USD among adults and 11,510,960 USD among children. Conclusions SCD management may impose an important economic burden on Brazilian society that may reach more than 400 million USD per year.

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“…Brazil also reports wide variations in SCD and mortality across their five geographic regions. 41 , 42 These variations may simply reflect and correlate with historical agrarian economic activity where descendants of African servitude settled.…”

Section: Discussionmentioning

confidence: 99%

“…The growing prevalence of obesity, which increases with age among childbearing women who increasingly delay childbearing, must be addressed within the care strategies. 41 …”

Section: Discussionmentioning

confidence: 99%

Maternal mortality among women with sickle cell disease in Jamaica over two decades (1998–2017)

McCaw-Binns

Campbell²,

Harris

et al. 2022

eClinicalMedicine

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Summary Background Sickle cell disease (SCD) affects 2.8% of Jamaican antenatal women. Between 1998–2007 their maternal mortality ratio was 7–11 times higher than women without these disorders. We aim to determine if outcomes improved between 2008 and 17 amid declining fertility and changes in referral obstetric care. Methods Maternal deaths in Jamaica's maternal mortality surveillance database (assembled since 1998) with SCD reported as underlying or associated cause of death were compared to those without known SCD, over two decades from 1998 to 2017. Social, demographic and health service variables were analysed using SPSS and EpiInfo Open. Findings Over the two decades from 1998 to 2017, 806 (74%) of the 1082 pregnancy-associated deaths documented by the Jamaican Ministry of Health and Wellness were maternal deaths. The maternal mortality ratio (MMR) did not statistically change over the two periods for women with ( p = 0.502) and without SCD ( p = 0.629). The MMR among women with and without SCD in 2008–17 was 378.1 ( n = 41) and 89.2/100,000 live births ( n = 336) respectively, an odds ratio of 4.24 (95% CI: 3.07–5.87). When deaths due to their blood disorders were excluded, risk remained elevated at 2.17 (95% CI: 1.36–3.32). There was an upward trend in direct deaths over the two decades (p [trend]=0.051). Interpretation MMRs were unchanged over two decades for Jamaicans with SCD. The high contribution to maternal mortality by women with SCD may explain some of the persistently higher mortality experience of women in the African diaspora. Multi-disciplinary evidence-based strategies need to be developed and tested which improve survival for women with SCD who want to have children. Funding No external funding was provided.

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Sickle cell disease related mortality in Brazil, 2000–2018

Cited by 20 publications

References 22 publications

Clinical-Epidemiological Characteristics and Mortality in Patients with Sickle Cell Anemia: A Retrospective Cohort Study of 1980 at 2018

Clinical-Epidemiological Characteristics and Mortality in Patients with Sickle Cell Anemia: A Retrospective Cohort Study of 1980 at 2018

Economic burden of sickle cell disease in Brazil

Maternal mortality among women with sickle cell disease in Jamaica over two decades (1998–2017)

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