Perspectives Fetal hemoglobin in sickle cell anemia: a glass half full?
نویسندگان
چکیده
Center of Excellence in Sickle Cell Disease, Department of Medicine, Boston University School of Medicine, Boston, MA; Department of Pediatrics, Johns Hopkins University School of Medicine, Baltimore, MD; Department of Biostatistics, Boston University School of Public Health, Boston, MA; and Sickle Cell Disease Research Center and Department of Pediatrics, College of Medicine, King Saud University, Riyadh, Saudi Arabia
منابع مشابه
The Influence of Fetal Hemoglobin on Clinical and Hematological Variables of Children and Adolescents with Sickle Cell Anemia in Basra, Southern Iraq
Background: There are many parameters that modulate the severity of sickle cell anemia. Fetal hemoglobin (Hb F) is one of these major variables. However, its effect is clinically inconsistent. We conducted a descriptive study to assess the influence of Hb F on clinical events and hematological variables in patients with sickle cell anemia. Methods: 151 patients with sickle cell anemia with a st...
متن کاملBeta-Globin Gene Cluster Haplotypes in Iranian Sickle Cell Patients: Relation to Some Hematologic
Background: Sickle cell anemia is relatively common in Khuzestan province located in Southwest Iran. The characteristics of sickle cell disease in Iran are apparently different from other regions some of these characteristics might be related to β-chain haplotypes. The purpose of this study was to determine the frequency of β-chain haplotypes in 50 patients with homozygous sickle cell anemia in...
متن کاملSide Effects of Hydroxyurea in Patients with Sickle Cell Anemia
Background: Hemoglobin S arises is the result of a point mutation (A-T) in the sixth codon on the -globin gene on chromosome 11 causing sickle cell anemia. The presence of fetal hemoglobin in infancy plays a relatively protective role for vaso-occlusive symptoms that are the major contributor for the morbidity and mortality among patients with sickle cell anemia. hydroxyurea, an s-phase-specif...
متن کاملFetal hemoglobin in sickle cell anemia: a glass half full?
Fetal hemoglobin (HbF) modulates the phenotype of sickle cell anemia by inhibiting deoxy sickle hemoglobin (HbS) polymerization. The blood concentration of HbF, or the number of cells with detectable HbF (F-cells), does not measure the amount of HbF/F-cell. Even patients with high HbF can have severe disease because HbF is unevenly distributed among F-cells, and some cells might have insufficie...
متن کاملSide effects of hydroxyurea in patients with Thalassemia major and thalassemia intermedia and sickle cell anemia
Background Sickle hemoglobin is the most common abnormal hemoglobin in the United States. Hemoglobin S arises as a result of a single amino acid substitution (glutamic acid to valin at position 6 of the β-globine chain). The presence of fetal hemoglobin (HbF) plays a relatively protective role since a significant amount of HbF interferes with HbS polymerization, the pathogenesis mechanism of ...
متن کامل