Survival of children with sickle cell disease in the comprehensive newborn screening programme in Minas Gerais, Brazil

Sabarense, Alessandra Palhoni; Lima, Gabriella Oliveira; Silva, Lívia Maria Lellis; Viana, Marcos Borato

doi:10.1080/20469047.2015.1109235

Cited by 21 publications

(23 citation statements)

References 12 publications

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“…The main causes were infection (45%) and acute splenic sequestration (14%). 257 In another study in the state of Rio de Janeiro, >1.2 million newborn babies were screened between 2000 and 2010, and 912 had SCD. The mortality was 4.2% during the 10-year period and the main causes were acute chest syndrome (36.8%), sepsis (31.6%) and splenic sequestration (21.1%).…”

Section: Box 2 Screening In Ghanamentioning

confidence: 99%

Sickle cell disease

Kato

Piel

Reid

et al. 2018

Nat Rev Dis Primers

1,004

929

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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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Section: Box 2 Screening In Ghanamentioning

confidence: 99%

Sickle cell disease

Kato

Piel

Reid

et al. 2018

Nat Rev Dis Primers

1,004

929

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“…Eight studies on mortality by SCD were carried out in the Brazilian states of Maranhão, 19 Bahia, 20 Minas Gerais,21, 22, 23 Rio de Janeiro24, 25 and Mato Grosso do Sul 26 …”

Section: Resultsmentioning

confidence: 99%

“…The mortality and survival of children with SCD were investigated in Minas Gerais 21, 22. Between 1998 and 2012, 2591 children were diagnosed with SCD (1:1.400).…”

Section: Resultsmentioning

confidence: 99%

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Mortality by sickle cell disease in Brazil

Arduini

Rodrigues

Marqui

2017

Revista Brasileira de Hematologia e Hemoterapia

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This work aimed to characterize mortality by sickle cell disease in Brazil. The MEDLINE electronic database was searched using the terms ‘mortality’ and ‘sickle cell disease’ and ‘Brazil’ for articles published in the last five years aiming to provide a current analysis of the subject in question. Eight studies on mortality by sickle cell disease were carried out in the Brazilian states of Maranhão, Bahia, Minas Gerais, Rio de Janeiro and Mato Grosso do Sul. The majority of the deaths occurred in patients with sickle cell anemia, which is the most common genotype and causes the most severe clinical manifestation of the disease. In summary, there are few published studies on mortality related to sickle cell disease in Brazil, and most are from the state of Minas Gerais. This study emphasizes the importance of developing more studies on sickle cell disease mortality, so that it may be possible to profile gene carriers and give health professionals more data to strategize the delivery of more effective assistance to these individuals. Despite the early diagnosis of sickle cell disease by the Neonatal Screening Program and the use of preventive and therapeutic measures (penicillin, immunization and hydroxyurea), mortality by sickle cell disease on the world stage is still significant.

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“…In Brazil, however, the mortality rate of children with SCD remains high, at 7.4%, despite an effective, ongoing and comprehensive screening program. There are many reasons for this, including the low socio-economic and cultural status of affected families complicating regular clinical monitoring, long trips to health facilities, the short interval between onset of symptoms and death and inexperience of the health staff to recognize and manage SCD acute events [15]. Quinn et al have demonstrated that neonatal screening minimizes morbidity and mortality through antibiotic prophylaxis and parental education [16,17].…”

Section: Discussionmentioning

confidence: 99%

Neonatal Screening for Sickle Cell Disease in Belgium for More than 20 Years: An Experience for Comprehensive Care Improvement

Gulbis

Lê

Ketelslegers

et al. 2018

IJNS

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Our previous results reported that compared to sickle cell patients who were not screened at birth, those who benefited from it had a lower incidence of a first bacteremia and a reduced number and days of hospitalizations. In this context, this article reviews the Belgian experience on neonatal screening for sickle cell disease (SCD). It gives an update on the two regional neonatal screening programs for SCD in Belgium and their impact on initiatives to improve clinical care for sickle cell patients. Neonatal screening in Brussels and Liège Regions began in 1994 and 2002, respectively. Compiled results for the 2009 to 2017 period demonstrated a birth prevalence of sickle cell disorder above 1:2000. In parallel, to improve clinical care, (1) a committee of health care providers dedicated to non-malignant hematological diseases has been created within the Belgian Haematology Society;(2) a clinical registry was implemented in 2008 and has been updated in 2018; (3) a plan of action has been proposed to the Belgian national health authority. To date, neonatal screening is not integrated into the respective Belgian regional neonatal screening programs, the ongoing initiatives in Brussels and Liège Regions are not any further funded and better management of the disease through the implementation of specific actions is not yet perceived as a public health priority in Belgium.

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Survival of children with sickle cell disease in the comprehensive newborn screening programme in Minas Gerais, Brazil

Abstract: Despite an effective ongoing comprehensive screening programme, mortality from SCD in Minas Gerais is still high. To decrease mortality rates, socio-economic development and SCD education programmes for health professionals and families are required.

Cited by 21 publications

References 12 publications

Sickle cell disease

Sickle cell disease

Mortality by sickle cell disease in Brazil

Neonatal Screening for Sickle Cell Disease in Belgium for More than 20 Years: An Experience for Comprehensive Care Improvement

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