Newborn screening for sickle cell disease: necessary but not sufficient
Journal Title: Jornal de Pediatria - Year 2015, Vol 91, Issue 3
Abstract
Sickle cell disease is a global health problem that affects>300,000 newborns per year, predominantly in Sub-Saharan Africa,1 where mortality is estimated to be>50% by age 5 years for those with homozygous hemoglobin S (HbSS).1 In Brazil, there are estimated to be>30,000 individuals with sickle cell disease and 2,500 newborns per year, making this the most common genetic disorder in the country.2 Advances in the management of sickle cell disease in the United States have led to a decline in mortality from about 26% to approximately 1-2% in the first 18 years of life, with the greatest progress demonstrated by a flattening of the mortality peak in the ages of 1-4 years from 13% to 2%.3,4 Much of this progress resulted from the implementation of universal newborn screening in all 50 states in the U.S. following publication of the results of the prophylactic penicillin study, which demonstrated a dramatic reduction in sepsis and mortality due to S. pneumonia in the first five years of life.5
Authors and Affiliations
Winfred Wang
Keywords
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- EP ID EP558347
- DOI 10.1016/j.jped.2015.01.002
- Views 32
- Downloads 0
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