Article
Oral sodium phenylbutyrate therapy in homozygous beta thalassemia: a clinical trial.
Blood - 1 Jan 1995
Collins A F, Pearson H A, Giardina P, McDonagh K T, Brusilow S W, Dover G J
Abstract excerpt
Butyrate analogues have been shown to increase fetal hemoglobin (HbF) production in vitro and in vivo. Sodium phenylbutyrate (SPB), an oral agent used to treat individuals with urea-cycle disorders, has been shown to increase HbF in nonanemic individuals and in individuals with sickle cell diseas...
Topics
- Adult
- Anemia, Sickle Cell
- Blood Transfusion
- Erythropoietin
- Female
- Fetal Hemoglobin
- Globins
- Hemoglobins
- Hemolysis
- Homozygote
- Humans
- Male
- Mutation
- Patient Compliance
- Phenylbutyrates
- Reticulocytes
- beta-Thalassemia