Prevalência de hemoglobina S em recém-nascidos de Fortaleza: importância da investigação neonatal

Pinheiro, Luciano Silveira; Gonçalves, Raquel; Tomé, Carlos Alberto Sousa; Alcântara, Ana Elisa Evangelista; Marques, Anna Renée Cintra; Silva, Márcio Medeiros da

doi:10.1590/s0100-72032006000200008

“…(3) According to SINASC, there were 40,532 live births in 2001 and 2002 in Fortaleza which gives a coverage of only 0.95% for this study. On considering the state of Ceará, the coverage is approximately 0.26%.…”

Section: Discussionmentioning

confidence: 94%

“…It is estimated that approximately 2500 children are born every year with sickle cell disease in Brazil. (3,4) …”

Section: Introductionmentioning

confidence: 99%

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Prevalence of sickle cell disease and sickle cell trait in national neonatal screening studies

Lervolino¹,

Baldin²,

Picado³

et al. 2011

Rev. Bras. Hematol. Hemoter.

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Sickle cell anemia is the best known hereditary blood disorder; there are serious complications associated with the condition. Diagnosis and early intervention reduce morbidity and mortality. These benefits have resulted in the widespread use of newborn screening education programs. In Brazil, the National Neonatal Screening Program established by decree 822/01 included sickle cell disease in the list of diseases tested in the so called "heel prick test". Since then, national studies of the results of this program have been periodically published. To review the literature in order to assess the prevalence of sickle cell trait and sickle cell anemia from data of national neonatal screening studies on hemoglobin S (Hb S). A bibliographic review was carried out using the key words: sickle cell anemia & hemoglobinopathies & neonatal screening & Brazil in the Bireme and SciELO databases. Original Brazilian studies presenting data on prevalence of the sickle cell trait (Hb AS) and sickle cell anemia (Hb SS) based on neonatal screening for Hb S were analysed. Twelve original national studies were identified with prevalences varying from 1.1% to 9.8% for the sickle cell trait and from 0.8 to 60 per 100,000 live births for sickle cell disease in different Brazilian regions. Conclusion: Neonatal screening for Hb S is a very useful method to assess the prevalence of sickle cell trait (Hb AS) and sickle cell anemia (Hb SS) in Brazil. There is a heterogeneous distribution of this disease with the highest prevalence in the northeastern region and the lowest prevalence in the south.

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“…No estado do Ceará, analisaram 389 amostras de sangue de cordão umbilical. Os resultados obtidos mostraram presença de HbAS, em 38,5:1000 nascidos vivos, correspondendo a 3,8% do total, e um com a hipótese diagnóstica de anemia falciforme HbSS (0,2%) (Pinheiro, et al, 2006). No Maranhão no ano de 2008 evidenciou que a cada 1:25,4 nascidos vivos apresentavam HbAS e 1:1.604 com HbSS (Lopes, et al, 2011) Um estudo realizado no estado Piauí coletou dados de triagem neonatos entre os anos .…”

Section: Introductionunclassified

Distribuição geográfica das hemoglobinopatias em recém-nascidos em Teresina, Piauí

Costa

¹

,

Filho

²

,

Neto

³

et al. 2020

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The present study aims to determine the geographical distribution of HbS, HbC, HbD variant hemoglobins, as well as patients diagnosed with sickle cell disease in a public health laboratory in the municipality of Teresina - Piauí, based on the neonatal screening program. A descriptive, cross-sectional, observational and retrospective study with a quantitative approach was conducted, based on secondary data provided by the Central Public Health Laboratory of the State of Piauí (LACEN-PI). The neonatal screening databases were analyzed, and all newborns who underwent the neonatal screening test in the agreed collection network from January 2018 to August 2019 were sampled. About 17,236 samples were analyzed from the municipality of Teresina (25,3% total analyzed) and organized according to the municipal administrative zones. This study allowed us to draw a map in the municipality of teresinense showing the areas with the highest incidence of hemoglobinopathies.

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“…It is estimated that approximately 2500 children are born every year with sickle cell disease in Brazil. (3,4) The non-white population in Brazil was estimated at 44.66% by the 2000 Population Census and from 1% to 6% of them have the Hb S gene, thus favoring the continuation of sickle cell anemia in what is suggested by Brazilian scientific literature as a serious public health problem. (3,4) The disease occurs due to a mutation of the beta globin gene of hemoglobin, causing a substitution of the glutamic amino acid for valine at position 6 of the beta chain, thereby producing an abnormal hemoglobin, called hemoglobin S (Hb S), instead of normal hemoglobin, hemoglobin A (Hb A).…”

Section: Introductionmentioning

confidence: 99%

Prevalence of sickle cell disease and sickle cell trait in national neonatal screening studies

Lervolino¹,

Baldin²,

Picado³

et al. 2011

Rev. Bras. Hematol. Hemoter.

View full text Add to dashboard Cite

Sickle cell anemia is the best known hereditary blood disorder; there are serious complications associated with the condition. Diagnosis and early intervention reduce morbidity and mortality. These benefits have resulted in the widespread use of newborn screening education programs. In Brazil, the National Neonatal Screening Program established by decree 822/01 included sickle cell disease in the list of diseases tested in the so called "heel prick test". Since then, national studies of the results of this program have been periodically published. To review the literature in order to assess the prevalence of sickle cell trait and sickle cell anemia from data of national neonatal screening studies on hemoglobin S (Hb S). A bibliographic review was carried out using the key words: sickle cell anemia & hemoglobinopathies & neonatal screening & Brazil in the Bireme and SciELO databases. Original Brazilian studies presenting data on prevalence of the sickle cell trait (Hb AS) and sickle cell anemia (Hb SS) based on neonatal screening for Hb S were analysed. Twelve original national studies were identified with prevalences varying from 1.1% to 9.8% for the sickle cell trait and from 0.8 to 60 per 100,000 live births for sickle cell disease in different Brazilian regions. Conclusion: Neonatal screening for Hb S is a very useful method to assess the prevalence of sickle cell trait (Hb AS) and sickle cell anemia (Hb SS) in Brazil. There is a heterogeneous distribution of this disease with the highest prevalence in the northeastern region and the lowest prevalence in the south.

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Prevalência de hemoglobina S em recém-nascidos de Fortaleza: importância da investigação neonatal

Cited by 11 publications

References 8 publications

Prevalence of sickle cell disease and sickle cell trait in national neonatal screening studies

Prevalence of sickle cell disease and sickle cell trait in national neonatal screening studies

Distribuição geográfica das hemoglobinopatias em recém-nascidos em Teresina, Piauí

Prevalence of sickle cell disease and sickle cell trait in national neonatal screening studies

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