%0 Journal Article
%T Role of hydroxycarbamide in prevention of complications in patients with sickle cell disease
%A NM Wiles
%A J Howard
%J Therapeutics and Clinical Risk Management
%D 2009
%I 
%R http://dx.doi.org/10.2147/TCRM.S4769
%X le of hydroxycarbamide in prevention of complications in patients with sickle cell disease Review (3970) Total Article Views Authors: NM Wiles, J Howard Published Date September 2009 Volume 2009:5 Pages 745 - 755 DOI: http://dx.doi.org/10.2147/TCRM.S4769 NM Wiles, J Howard Department of Haematology, St Thomas¡¯ Hospital, Westminster, Bridge Road, London, SE1 7EH, UK Abstract: Sickle cell disease (SCD) is a genetically inherited condition caused by a point mutation in the beta globin gene. This results in the production of the abnormal hemoglobin, sickle hemoglobin (HbS). Hydroxycarbamide, is an antimetabolite/cytotoxic which works by inhibiting ribonucleotide reductase, blocking the synthesis of DNA and arresting cells in the S phase. In sickle cell anemia, it promotes fetal hemoglobin (HbF) synthesis, improves red cell hydration, decreases neutrophil and platelet count, modifies red cell endothelial cell interactions and acts as a nitric oxide donor. Trials have shown the clinical benefit of hydroxycarbamide in a subpopulation of adult patients with SCD, with a 44% reduction in the median annual rate of painful crises, a decrease in the incidence of acute chest syndrome and an estimated 40% reduction in overall mortality over a 9-year observational period. Its use in pediatrics has also been well established; trials have shown it is well tolerated and does not impair growth or development. In addition it decreases the number and duration of hospital attendences. A number of emerging uses of hydroxycarbamide currently are being investigated, such as stroke prevention.
%K sickle cell anemia
%K hydroxycarbamide
%K hydroxyurea
%K maximum tolerated dose
%K vaso-occlusive crisis
%U https://www.dovepress.com/role-of-hydroxycarbamide-in-prevention-of-complications-in-patients-wi-peer-reviewed-article-TCRM