The Brigance Assessment Of Individual Neurodevelopment In Young Children With Sickle Cell Disease- 2

2014-07-23 21:09:04 | BioPortfolio


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.

Secondary objectives:

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.

Study Design

Observational Model: Cohort, Time Perspective: Prospective


Sickle Cell Disease


Brigance Preschool Screen -II, Brigance Preschool Screen -II


St. Jude Children's Research Hospital
United States




St. Jude Children's Research Hospital

Results (where available)

View Results


Published on BioPortfolio: 2014-07-23T21:09:04-0400

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Screens which absorb the energy in the x-ray beam that has penetrated the patient and convert this energy into a light pattern which has as nearly as possible the same information as the original x-ray beam. The more light a screen produces for a given input of x-radiation, the less x-ray exposure and thus shorter exposure time are needed to expose the film. In most film-screen systems, the film is sandwiched between two screens in a cassette so that the emulsion on each side is exposed to the light from its contiguous screen.

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A rapid, low-dose, digital imaging system using a small intraoral sensor instead of radiographic film, an intensifying screen, and a charge-coupled device. It presents the possibility of reduced patient exposure and minimal distortion, although resolution and latitude are inferior to standard dental radiography. A receiver is placed in the mouth, routing signals to a computer which images the signals on a screen or in print. It includes digitizing from x-ray film or any other detector. (From MEDLINE abstracts; personal communication from Dr. Charles Berthold, NIDR)

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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