Characterization of mortality in children with sickle cell disease diagnosed through the Newborn Screening Program

Sabarense, Alessandra Palhoni; Lima, Gabriella Oliveira; Silva, Lívia M.L.; Viana, Marcos Borato

doi:10.1016/j.jped.2014.08.006

Cited by 54 publications

(39 citation statements)

References 26 publications

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“…The term ‘sickle cell’ was not cited in 46% of death certificates. The mortality rate between 1998 and 2005 was 5.43% vs. 5.12% between 2005 and 2012 21 . Another study showed that the 5-year estimated mortality was lower, albeit not significantly, for children born between 2009 and 2011 ( n = 509) than for those born between 1999 and 2001 ( n = 624) [mean (standard deviation): 5.8% (1.1) vs. 6.2% (1.0), respectively] 22 …”

Section: Resultsmentioning

confidence: 92%

“…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%

“…Of the deaths, 56.5% occurred in children under the age of two and 76.7% in under five-year olds. The main causes of death were infection (45%), indeterminate (28%) and ASS (14%) 21 . The term ‘sickle cell’ was not cited in 46% of death certificates.…”

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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Section: Resultsmentioning

confidence: 92%

“…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%

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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“…% [43]. The importance of integrating neonatal screening in an efective program of care of the patient at a specialized reference center has been more recently conirmed by another recent Brazilian study, which indicated in Minias Gerais State a mortality of .5% patients with SCD in the irst 14 years of life, even though they were undergoing newborn screening, because of a non-efective care program [44].…”

Section: Neonatal Screening For Scd: Major International Experiencesmentioning

confidence: 99%

A Global Perspective on Milestones of Care for Children with Sickle Cell Disease

Sainati¹,

Montanaro²,

Colombatti³

2016

Sickle Cell Disease - Pain and Common Chronic Complications

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Prevalence and incidence of erythrovirus B19 infection in children with sickle cell disease: The impact of viral infection in acute clinical events

Furtado

Viana

Rrios

et al. 2015

Journal of Medical Virology

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B19V infection is common during childhood. It is self-limited in healthy individuals, but is often associated with transient aplastic crisis in children with sickle cell disease. The aim of this study was to estimate the prevalence and incidence of B19V infection in children with sickle cell disease screened by the Newborn Screening Program of Minas Gerais, Brazil, and followed-up at Fundação Hemominas. Serum or plasma samples from 278 patients were tested for anti-B19V IgG and IgM using commercial ELISA and for viral DNA using in-house real-time PCR assays; 127 negative-children were retested about 1 year later. The median age of children at first testing was 5.9 years (0.8-12.3). The estimated prevalence of B19V was 29.5 % (95%CI 24.1-34.9 %). The incidence of B19V in those 127 negative-children was 18.2 cases/100 patient-years. All DNA-positive samples were identified as genotype 1, except one sample, in which both genotypes 1 and 3 were identified. It was observed that the higher the child's age, the higher the probability of B19V infection. The analysis of clinical and hematological data showed a significant association of B19V infection with transient aplastic crisis and acute splenic sequestration, higher frequency of transfusions, and higher rate of hospitalization, but not with acute chest syndrome or stroke. These results emphasize the impact of B19V infection on the course of sickle cell disease. Strategies to prevent and monitor B19V infection in children with sickle cell disease should be considered to diminish its morbidity in this susceptible population.

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Characterization of mortality in children with sickle cell disease diagnosed through the Newborn Screening Program

Cited by 54 publications

References 26 publications

Mortality by sickle cell disease in Brazil

Mortality by sickle cell disease in Brazil

A Global Perspective on Milestones of Care for Children with Sickle Cell Disease

Prevalence and incidence of erythrovirus B19 infection in children with sickle cell disease: The impact of viral infection in acute clinical events

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