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      Analysis of the temporal trend of mortality from sickle cell anemia in Brazil Translated title: Análise da tendência temporal da mortalidade por anemia falciforme no Brasil Translated title: Análisis de la tendencia temporal de la mortalidad por anemia de células falciformes en Brasil

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          Abstract

          ABSTRACT Objectives: To analyze the temporal trend of mortality from sickle cell anemia in Brazil, by region, in the period 1997-2017. Methods: epidemiological study, with an ecological design, with a temporal trend, carried out with data from the Mortality Information System. For descriptive analysis, absolute and relative frequencies were used. In the correlation analysis, the ANOVA test was used, followed by Tukey’s post-test. The temporal trend was obtained using the cubic polynomial regression test. Results: 6,813 deaths from sickle cell anemia were registered. Brown individuals (50.87%) were more frequent, with a predominance of males (50.4%), aged between 25 and 34 years and a higher incidence of deaths in the Midwest (0.25/100 thousand inhabitants). The time curve showed an increasing trend of deaths in the country between 1997 and 2015 (R2 = 0.98). Conclusions: sickle cell anemia showed increasing mortality in the 21 years analyzed and alerts health professionals and managers.

          Translated abstract

          RESUMO Objetivos: analisar a tendência temporal da mortalidade por anemia falciforme no Brasil, por regiões, no período compreendido entre 1997 e 2017. Métodos: estudo epidemiológico, de delineamento ecológico, de tendência temporal, realizado com dados do Sistema de Informações sobre Mortalidade. Para análise descritiva, utilizaram-se frequências absolutas e relativas. Na análise de correlação, utilizou-se o teste ANOVA seguido pelo pós-teste de Tukey. A tendência temporal foi obtida mediante o teste de regressão polinomial cúbico. Resultados: foram registrados 6.813 óbitos por anemia falciforme. Indivíduos pardos (50,87%) foram mais frequentes, com predomínio do sexo masculino (50,4%), com faixa etária de 25 a 34 anos e maior incidência de óbitos no Centro-Oeste (0,25/100 mil habitantes). A curva temporal apresentou tendência crescente de óbitos no país entre 1997 a 2015 (R2 = 0,98). Conclusões: a anemia falciforme apresentou mortalidade crescente nos 21 anos analisados e desperta o alerta aos profissionais de saúde e gestores.

          Translated abstract

          RESUMEN Objetivos: analizar tendencia temporal de mortalidad por anemia falciforme en Brasil, por regiones, entre 1997 y 2017. Métodos: estudio epidemiológico, de delineamento ecológico, de tendencia temporal, realizado con datos del Sistema de Informaciones sobre Mortalidad. Utilizado frecuencias absolutas y relativas para análisis descriptivo. Utilizado la prueba ANOVA seguido por la prueba de Tukey en el análisis de correlación. La tendencia temporal fue obtenida mediante la prueba de regresión polinomial cúbico. Resultados: fueron registrados 6.813 óbitos por anemia falciforme. Indivíduos pardos (50,87%) fueron más frecuentes, con predominio del sexo masculino (50,4%), con franja etaria de 25 a 34 años y mayor incidencia de óbitos en Centro-Oeste (0,25/100 mil habitantes). La curva temporal presentó tendencia creciente de óbitos en el país entre 1997 a 2015 (R2 = 0,98). Conclusiones: la anemia falciforme presentó mortalidad creciente en los 21 años analizados y despierta el alerta a profesionales de salud y gestores.

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          Sickle cell disease

          Sickle cell disease (SCD) is a group of inherited disorders caused by mutations in HBB, which encodes haemoglobin subunit β. The incidence is estimated to be between 300,000 and 400,000 neonates globally each year, the majority in sub-Saharan Africa. Haemoglobin molecules that include mutant sickle β-globin subunits can polymerize; erythrocytes that contain mostly haemoglobin polymers assume a sickled form and are prone to haemolysis. Other pathophysiological mechanisms that contribute to the SCD phenotype are vaso-occlusion and activation of the immune system. SCD is characterized by a remarkable phenotypic complexity. Common acute complications are acute pain events, acute chest syndrome and stroke; chronic complications (including chronic kidney disease) can damage all organs. Hydroxycarbamide, blood transfusions and haematopoietic stem cell transplantation can reduce the severity of the disease. Early diagnosis is crucial to improve survival, and universal newborn screening programmes have been implemented in some countries but are challenging in low-income, high-burden settings.
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            Sickle Cell Disease

            New England Journal of Medicine, 376(16), 1561-1573
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              Global epidemiology of sickle haemoglobin in neonates: a contemporary geostatistical model-based map and population estimates

              Summary Background Reliable estimates of populations affected by diseases are necessary to guide efficient allocation of public health resources. Sickle haemoglobin (HbS) is the most common and clinically significant haemoglobin structural variant, but no contemporary estimates exist of the global populations affected. Moreover, the precision of available national estimates of heterozygous (AS) and homozygous (SS) neonates is unknown. We aimed to provide evidence-based estimates at various scales, with uncertainty measures. Methods Using a database of sickle haemoglobin surveys, we created a contemporary global map of HbS allele frequency distribution within a Bayesian geostatistical model. The pairing of this map with demographic data enabled calculation of global, regional, and national estimates of the annual number of AS and SS neonates. Subnational estimates were also calculated in data-rich areas. Findings Our map shows subnational spatial heterogeneities and high allele frequencies across most of sub-Saharan Africa, the Middle East, and India, as well as gene flow following migrations to western Europe and the eastern coast of the Americas. Accounting for local heterogeneities and demographic factors, we estimated that the global number of neonates affected by HbS in 2010 included 5 476 000 (IQR 5 291 000–5 679 000) AS neonates and 312 000 (294 000–330 000) SS neonates. These global estimates are higher than previous conservative estimates. Important differences predicted at the national level are discussed. Interpretation HbS will have an increasing effect on public health systems. Our estimates can help countries and the international community gauge the need for appropriate diagnoses and genetic counselling to reduce the number of neonates affected. Similar mapping and modelling methods could be used for other inherited disorders. Funding The Wellcome Trust.
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                Author and article information

                Journal
                reben
                Revista Brasileira de Enfermagem
                Rev. Bras. Enferm.
                Associação Brasileira de Enfermagem (Brasília, DF, Brazil )
                0034-7167
                1984-0446
                2022
                : 75
                : 4 suppl 3
                : e20210640
                Affiliations
                [1] Campo Grande Mato Grosso do Sul orgnameUniversidade Federal de Mato Grosso do Sul Brazil
                [2] Campo Grande Mato Grosso do Sul orgnameSecretaria de Saúde de Mato Grosso do Sul Brazil
                Article
                S0034-71672022000500170 S0034-7167(22)07500400170
                10.1590/0034-7167-2021-0640
                35442313
                8930641a-44bc-49a6-a7a8-f9b1f0537a03

                This work is licensed under a Creative Commons Attribution 4.0 International License.

                History
                : 02 September 2021
                : 11 December 2021
                Page count
                Figures: 0, Tables: 0, Equations: 0, References: 32, Pages: 0
                Product

                SciELO Brazil


                Anemia Falciforme,Mortalidade,Distribuição Temporal,Epidemiologia,Brasil,Anemia de Células Falciformes,Mortalidad,Distribución Temporal,Epidemiología,SICKLE CELL ANEMIAS,MORTALITY,SPATIO TEMPORAL ANALYSIS,EPIDEMIOLOGY,BRAZIL

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