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A preliminary study was conducted involving 88 three-year-old children with sickle cell disease (SCD) who were followed at the St. Jude Children's Research Hospital Sickle Cell Center.(1)They were offered developmental screening with the Brigance Preschool Screen-II test during their regular clinic visits from January 2006 to August 2008. Data from this work showed that 50% of 3 year old children with SCD had low developmental screening scores. In addition, the low scores were found to be associated with less parental education and with speech deficits. However they were not associated with sickle cell genotype and hemoglobin level.
The primary goal of the BRAIN2 study is to prospectively administer Brigance Preschool Screen -II to 3 year old children with SCD and 3 year old children without SCD who come from similar socioeconomic backgrounds and compare the results between the two groups.
The primary objective of this study is to compare development in 3 year old children with SCD who are not on any treatment to age matched healthy controls using pass/fail rate for the Brigance Preschool Screen II.
1. To compare the raw scores of the Brigance Preschool Screen II between SCD and control groups.
2. To compare the pass/fail rate and the raw scores between SCD patients who are not on any treatment and those who are being treated with hydroxyurea.
3. To compare the pass/fail rate and the raw scores between SCD patients on hydroxyurea treatment and the healthy controls
4. To assess the influence of medical factors on the Brigance Preschool Screen II performance in children with SCD.
5. To assess the influence of socioeconomic factors on the Brigance Preschool II results in children with SCD and controls.
Observational Model: Cohort, Time Perspective: Prospective
Sickle Cell Disease
Brigance Preschool Screen -II, Brigance Preschool Screen -II
St. Jude Children's Research Hospital
St. Jude Children's Research Hospital
Published on BioPortfolio: 2014-07-23T21:09:04-0400
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An abnormal hemoglobin resulting from the substitution of valine for glutamic acid at position 6 of the beta chain of the globin moiety. The heterozygous state results in sickle cell trait, the homozygous in sickle cell anemia.
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