Hydroxyurea treatment of sickle cell anemia in hospital-based practices

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Side 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...

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Hydroxyurea in the treatment of sickle-cell anemia.

Sickle-cell anemia is a congenital hemolytic anemia characterized by sickle-shaped RBCs. The deformed RBCs become distorted and rigid and may occlude small arterioles and capillaries leading to tissue ischemia and infarction. Sickled RBCs are too fragile to withstand the trauma of circulation, and hemolysis occurs after they enter the circulation. RBCs with a high level of Hb F are resistant to...

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Hydroxyurea for Treatment of Severe Sickle Cell Anemia:

Hydroxyurea (HU) enhances the synthesis of fetal hemoglobin (HbF) and can improve the clinical course of some adult patients with sickle cell anemia (SCA). In a randomized trial, we studied the biologic effects and the clinical benefit of HU in children and young adults with severe SCA. Twenty-five patients (median age, 9 years) were randomized to receive either HU (at the initial dosage of 20 ...

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Hydroxyurea for the treatment of sickle cell anemia.

There are about 50,000 people in the United States who are homozygous for the sickle hemoglobin gene and thus have sickle cell anemia. Sickle cell anemia is primarily seen in persons of African heritage, about 1 in 14 of whom is an asymptomatic carrier (a heterozygote). One in 700 newborns of African heritage is affected.1 Although it is the most severe of the common sickle cell diseases (which...

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Side 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 ...

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ژورنال

عنوان ژورنال: American Journal of Hematology

سال: 2002

ISSN: 0361-8609,1096-8652

DOI: 10.1002/ajh.10184