Hydroxyurea 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 γ- to β-chain synthesis — γ-globin chains characterize HbF, and sickle β-globin chains are present in Hb...
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| Format: | Article |
| Language: | English |
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Wiley
2002-01-01
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| Series: | The Scientific World Journal |
| Online Access: | http://dx.doi.org/10.1100/tsw.2002.295 |
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| author | Martin H. Steinberg |
| author_facet | Martin H. Steinberg |
| author_sort | Martin H. Steinberg |
| collection | DOAJ |
| description | 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 γ- to β-chain synthesis — γ-globin chains characterize HbF, and sickle β-globin chains are present in HbS — or selectively increase the proportion of adult erythroid precursors that maintain the ability to produce HbF are therapeutically useful. Hydroxyurea promotes HbF production by perturbing the maturation of erythroid precursors. This treatment increases the total hemoglobin concentration, reduces the vaso-occlusive complications of pain and acute chest syndrome, and attenuates mortality in adults. It is a promising beginning for pharmacologic therapy of sickle cell disease. Still, its effects are inconsistent, trials in infants and children are ongoing, and its ultimate value — and peril — when started early in life are still unknown. |
| format | Article |
| id | doaj-art-ca04fa168d9649f6bb2b9add967dff61 |
| institution | Kabale University |
| issn | 1537-744X |
| language | English |
| publishDate | 2002-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | The Scientific World Journal |
| spelling | doaj-art-ca04fa168d9649f6bb2b9add967dff612025-02-03T01:28:05ZengWileyThe Scientific World Journal1537-744X2002-01-0121706172810.1100/tsw.2002.295Hydroxyurea Treatment for Sickle Cell DiseaseMartin H. Steinberg0Boston University School of Medicine, 88 E. Newton St., Boston, MA 02118, USAHigh 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 γ- to β-chain synthesis — γ-globin chains characterize HbF, and sickle β-globin chains are present in HbS — or selectively increase the proportion of adult erythroid precursors that maintain the ability to produce HbF are therapeutically useful. Hydroxyurea promotes HbF production by perturbing the maturation of erythroid precursors. This treatment increases the total hemoglobin concentration, reduces the vaso-occlusive complications of pain and acute chest syndrome, and attenuates mortality in adults. It is a promising beginning for pharmacologic therapy of sickle cell disease. Still, its effects are inconsistent, trials in infants and children are ongoing, and its ultimate value — and peril — when started early in life are still unknown.http://dx.doi.org/10.1100/tsw.2002.295 |
| spellingShingle | Martin H. Steinberg Hydroxyurea Treatment for Sickle Cell Disease The Scientific World Journal |
| title | Hydroxyurea Treatment for Sickle Cell Disease |
| title_full | Hydroxyurea Treatment for Sickle Cell Disease |
| title_fullStr | Hydroxyurea Treatment for Sickle Cell Disease |
| title_full_unstemmed | Hydroxyurea Treatment for Sickle Cell Disease |
| title_short | Hydroxyurea Treatment for Sickle Cell Disease |
| title_sort | hydroxyurea treatment for sickle cell disease |
| url | http://dx.doi.org/10.1100/tsw.2002.295 |
| work_keys_str_mv | AT martinhsteinberg hydroxyureatreatmentforsicklecelldisease |