FETAL HEMOGLOBIN SYNTHESIS IN CHILDREN WITH SICKLE CELL ANEMIA

$0M01FY2000RRNIH

Johns Hopkins University, Baltimore MD

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Abstract

This research proposal is designed to test whether sodium phenylbutyrate (SPB) will increase fetal hemoglobin (HbF) in children with sickle cell anemia and thereby ameliorate complications from their disease. Increases in Hbf lessens the severity of disease and enhances survival. Several fatty acids, including butyrate analogues, have been shown to stimulate HbF production, suggesting that these agents may be beneficial in treating patients with sickle cell anemia. The purpose of this study is to determine if oral SPB will induce HbF synteiss in children with sickle cell anemia.

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