Characterization of mortality in children with sickle cell disease diagnosed through the Newborn Screening Program
Journal Title: Jornal de Pediatria - Year 2015, Vol 91, Issue 3
Abstract
Objective To characterize the deaths of 193 children with sickle cell disease screened by a neonatal program from 1998 to 2012 and contrast the initial years with the final years. Methods Deaths were identified by active surveillance of children absent to scheduled appointments in Blood Bank Clinical Centers (Hemominas). Clinical and epidemiological data came from death certificates, neonatal screening database, medical records, and family interviews. Results Between 1998 and 2012, 3,617,919 children were screened and 2,591 had sickle cell disease (1:1,400). There were 193 deaths (7.4%): 153 with SS/Sβ0-talassemia, 34 SC and 6 Sβ+thalassemia; 76.7% were younger than five years; 78% died in the hospital and 21% at home or in transit. The main causes of death were infection (45%), indeterminate (28%), and acute splenic sequestration (14%). In 46% of death certificates, the term “sickle cell” was not recorded. Seven-year death rate for children born between 1998 and 2005 was 5.43% versus 5.12% for those born between 2005 and 2012 (p = 0.72). Medical care was provided to 75% of children; 24% were unassisted. Medical care was provided within 6hours of symptom onset in only half of the interviewed cases. In 40.5% of cases, death occurred within the first 24hours. Low family income was recorded in 90% of cases, and illiteracy in 5%. Conclusions Although comprehensive and effective, neonatal screening for sickle cell disease was not sufficient to significantly reduce mortality in a newborn screening program. Economic and social development and increase of the knowledge on sickle cell disease among health professionals and family are needed to overcome excessive mortality.
Authors and Affiliations
Marcos Viana
Keywords
Related Articles
- EP ID EP558366
- DOI 10.1016/j.jped.2014.08.006
- Views 37
- Downloads 0
Maternal immunity, a way to confer protection against enteropathogenic Escherichia coli
Diarrheal diseases caused by Escherichia coli remain as important causes of morbidity and mortality worldwide; children in the developing world suffer the major burden of diarrheal illnesses, while the burden among child...
Prevalence and risk factors of anemia in children
Objective To identify the prevalence and factors associated with anemia in children attending Municipal Early Childhood Education Day Care Center (Centros Municipais de Educação Infantil [CMEI]) nurseries in Colombo-PR....
Association of eating frequency with anthropometric indices and blood pressure in children and adolescents: the CASPIAN-IV Study
Objectives This study was conducted to explore the association of eating frequency (EF) with anthropometric indices and blood pressure (BP) in children and adolescents. Methods This nationwide cross-sectional study was...
Sweat conductivity and coulometric quantitative test in neonatal cystic fibrosis screening
Objective To compare the results obtained with the sweat test using the conductivity method and coulometric measurement of sweat chloride in newborns (NBs) with suspected cystic fibrosis (CF) in the neonatal screening pr...
The use of high-flow nasal cannula in the pediatric emergency department
Objectives To summarize the current literature describing high-flow nasal cannula use in children, the components and mechanisms of action of a high-flow nasal cannula system, the appropriate clinical applications, and i...