Cellular normoxic biophysical markers of hydroxyurea treatment in sickle cell disease
نویسندگان
چکیده
منابع مشابه
Cellular normoxic biophysical markers of hydroxyurea treatment in sickle cell disease.
Hydroxyurea (HU) has been used clinically to reduce the frequency of painful crisis and the need for blood transfusion in sickle cell disease (SCD) patients. However, the mechanisms underlying such beneficial effects of HU treatment are still not fully understood. Studies have indicated a weak correlation between clinical outcome and molecular markers, and the scientific quest to develop compan...
متن کاملHydroxyurea treatment for sickle cell disease.
High fetal hemoglobin (HbF) levels inhibit the polymerization of sickle hemoglobin (HbS) and reduce the complications of sickle cell disease. Pharmacologic agents that can reverse the switch from gamma- to beta-chain synthesis--gama-globin chains characterize HbF, and sickle beta-globin chains are present in HbS--or selectively increase the proportion of adult erythroid precursors that maintain...
متن کاملSickle Cell Disease: Is Hydroxyurea
We read with interest the recent article on efficacy of hydroxyurea (HU) in sickle cell disease (SCD) [1]. We share our experience in managing nine such patients (4 males) aged between 3 and 19 years. Seven patients were on regular HU therapy (15-20 mg/kg/d) and penicillin prophylaxis for median period of 2.8 years. All had history of repeated fever, chest infections and vasoocclusive episodes ...
متن کاملHydroxyurea for the treatment of sickle cell disease.
In this study 21 adults with severe form of sickle cell disease (SCD; sickle cell anaemia, n = 15; Hb S/beta degree-thal, n = 6) were treated with hydroxyurea (HU) to assess the effectiveness of the drug in managing SCD. The individual dose was selected for each patient. The dose selection was based on the HU clearance study. Thereafter, the patients received daily doses of 15-20 mg/kg body wei...
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...
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ژورنال
عنوان ژورنال: Proceedings of the National Academy of Sciences
سال: 2016
ISSN: 0027-8424,1091-6490
DOI: 10.1073/pnas.1610435113