Fetal hemoglobin levels in African American and Hispanic children with sickle cell disease at baseline and in response to hydroxyurea.

ABSTRACT: The degree of fetal hemoglobin (HbF) expression is a major determinant of phenotypic severity of sickle cell disease (SCD). Genetic regulation of HbF production is complex and can vary among ethnic groups. The pediatric sickle cell population at our institution is approximately half Hispanic, nearly all from the Dominican Republic. Hydroxyurea (HU) is the only Food and Drug Administration (FDA)-approved drug to ameliorate symptoms of SCD. We retrospectively compared baseline and HU-induced percent HbF (%HbF) in African American (AA) and Hispanic (H) patients aged 4 to 21 years with homozygous Sickle hemoglobin or HbSβ(0)Thalassemia. No significant differences were detected in average baseline %HbF between AA (N=48) and H (N=58) patients (P=0.63). In the subset of children taking HU who rea

SUBMITTER: Ender KL 

PROVIDER: S-EPMC3179608 | biostudies-literature | 2011 Oct

REPOSITORIES: biostudies-literature