Hydroxyurea treatment and young children with sickle cell. Hydroxyurea, which has long been used to treat myeloproliferative diseases, reduces the rates of vasoocclusive crisis, acute chest syndrome, and transfusion requirement in patients with sicklecell disease 1, 2. Combination erythropoietinhydroxyurea therapy in sickle cell disease. Combination erythropoietin hydroxyurea therapy in sickle cell disease. Effect on quality of life article pdf available in health and quality of life outcomes 41. Hydroxyurea treatment in children with sickle cell disease in central america and the caribbean countries. Survival and mortality among users and nonusers of hydroxyurea. Treatment with moderate doses of hydroxyurea in drepanocytosis. Hydroxyurea and cysno also increased the level of several minor globin peaks between hbf and adult hemoglobin hba. Sickle cell anemia red blood cells with normal hemoglobin red blood cells with sickle cell hemoglobin. Program for comprehensive sickle cell disease care in cuba. The most common indication for splenectomy in sickle cell disease is ssc. The current data on the risks of both short and longterm harms of hydroxyurea therapy are reassuring, and the risks of hydroxyurea use in adults are acceptable compared with the risks of untreated sickle cell disease. Sickle cell disease is a multisystemic condition, and splenic complications are among its most prominent manifestations.
Voet and voet biochemistry, klatt webpath, stryer biochemistry, goldman cecil textbook of medicine charged objects like to be surrounded by water. Hydroxyurea dermopathy is caused by chronic use of hydroxyurea for chronic myelogenous leukemia, thrombocytosis, or psoriasis, and presents with skin lesions characteristic of dermatomyositis. Department of health and human services 540 gaither road rockville, md 20850. Hydroxyurea is used for sicklecell disease patients in order to increase fetal hemoglobin synthesis and consequently decrease the severity of pain episodes. Voet and voet biochemistry, klatt webpath, stryer biochemistry, goldman cecil textbook of medicine. An orphan drug based on hydroxycarbamide hydroxyurea has obtained european marketing authorization for the severe forms of the disease. Skin cancer has also been reported in patients receiving longterm hydroxyurea. Hydroxyurea for the treatment of sickle cell disease. Listing a study does not mean it has been evaluated by.
Hydroxyurea for the treatment of sickle cell disease prepared for. Hydroxyurea induces fetal hemoglobin by the nitric oxide. Benefits of hidroxyurea therapy in children with sickle cell disease. The most common type is known as sickle cell anaemia sca. Nih stateofthescience conference statement on hydroxyurea.
Hydroxyurea dermopathy is caused by chronic use of hydroxyurea for chronic myelogenous leukemia, thrombocytosis, or psoriasis, and presents with skin lesions characteristic of dermatomyositis 5 see also. Problems in sickle cell disease typically begin around 5 to. Hydroxyurea in the treatment of children and adolescents with sickle cell. This leads to a rigid, sicklelike shape under certain circumstances.
Predictors of benefit from hydroxyurea in studies of sickle cell disease41 table 4. However, splenomegaly persists in some patients to later ages, and other patients require. Outcomes of hydroxyurea use in sickle cell disease reported by genotype42 table 5. Hu en pacientes con drepanocitosis y complicaciones clinicas severas. Morbiletalidad en pacientes adultos con drepanocitosis. Fetal hemoglobin, which is formed by g globin chains a and g, is present in a constant composition throughout fetal development. Moderate dose of hydroxyurea in children with sickle cell. Hydroxyurea versus aspirin and hydroxyurea in essential. The mechanism of the salutary effect of hydroxyurea is largely attributable to its ability to increase fetal hemoglobin hbf levels. Hydroxyurea versus aspirin and hydroxyurea in essential thrombocythemia fast the safety and scientific validity of this study is the responsibility of the study sponsor and investigators. Jan 15, 2003 hydroxyurea and cysno both caused a monotonic increase in hbf, as compared with control cells, during subsequent growth in culture, with about a 1.
Strouse jj1, lanzkron s, beach mc, haywood c, park h, witkop c, wilson rf, bass eb, segal jb. Sickle cell anemia sca is a disease caused by production of abnormal hemoglobin, which binds with other abnormal hemoglobin molecules within the red blood cell to. Martinez r, svarch e, menendez a, machado t, alvarez ma. Sintomas y tratamientos anemia drepanocitica ncbddd cdc. Highlights of prescribing information these highlights do.
Pdf effect of hydroxyurea on hemoglobin s researchgate. A previous scientific article external icon from the baby hug study found that 1 year old children with sickle cell anemia randomly selected to receive hu had fewer hospital stays. This includes folic acid administration throughout life, oral prophylactic penicillin from 3 months through 5 years of age, and a daily dose of hydroxyurea 15 mgkg. Hospitalization, hydroxyurea, sickle cell anemia, toxicity, transfusion.
To assess the toxicity and benefits of hydroxyurea hu in the management of children with sickle cell. Average medicaid reimbursements for hospital stays for us children with sickle cell anemia in 2009 were used to calculate the costs of hospital care. The journal pediatrics has published the first study to look at the costs and health outcomes costeffectiveness of hydroxyurea hu treatment in young children with sickle cell disease scd. In patients receiving longterm hydroxyurea for myeloproliferative disorders, secondary leukemia has been reported. Pdf previous studies have determined the laboratory alterations, clinical.
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