Hydroxyurea in the treatment of children and adolescents with sickle cell. 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. Martinez r, svarch e, menendez a, machado t, alvarez ma. Sickle cell disease scd is a group of blood disorders typically inherited from a persons parents. 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.
The most common indication for splenectomy in sickle cell disease is ssc. Moderate dose of hydroxyurea in children with sickle cell disease and stroke. Outcomes of hydroxyurea use in sickle cell disease reported by genotype42 table 5. Objective to present the casuistics of a pediatric hospital. However, splenomegaly persists in some patients to later ages, and other patients require. Skin cancer has also been reported in patients receiving longterm hydroxyurea. This leads to a rigid, sicklelike shape under certain circumstances.
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. Hydroxyurea dermopathy is caused by chronic use of hydroxyurea for chronic myelogenous leukemia, thrombocytosis, or psoriasis, and presents with skin lesions characteristic of dermatomyositis. Problems in sickle cell disease typically begin around 5 to. 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. Previous studies have determined the laboratory alterations, clinical efficacy and toxicity profile associated with hydroxyurea hu therapy in patients with severe sickle cell anemia. Hydrea hydroxyurea is an antineoplastic anticancer agent used to treat melanoma, resistant chronic myelocytic leukemia, and recurrent, metastatic, or inoperable carcinoma of the ovary and primary squamous cell epidermoid carcinomas of the head and neck. Program for comprehensive sickle cell disease care in cuba. Department of health and human services 540 gaither road rockville, md 20850. Effect on quality of life article pdf available in health and quality of life outcomes 41. 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. It results in an abnormality in the oxygencarrying protein haemoglobin found in red blood cells.
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. Voet and voet biochemistry, klatt webpath, stryer biochemistry, goldman cecil textbook of medicine charged objects like to be surrounded by water. Survival and mortality among users and nonusers of hydroxyurea. Hydroxyurea treatment and young children with sickle cell. Fetal hemoglobin, which is formed by g globin chains a and g, is present in a constant composition throughout fetal development. Hydroxyurea treatment in children with sickle cell disease in central america and the caribbean countries. Hu en pacientes con drepanocitosis y complicaciones clinicas severas. Combination erythropoietinhydroxyurea therapy in sickle cell disease. Hydroxyurea for the treatment of sickle cell disease. Average medicaid reimbursements for hospital stays for us children with sickle cell anemia in 2009 were used to calculate the costs of hospital care. Hydroxyurea is used for sicklecell disease patients in order to increase fetal hemoglobin synthesis and consequently decrease the severity of pain episodes.
An orphan drug based on hydroxycarbamide hydroxyurea has obtained european marketing authorization for the severe forms of the disease. Strouse jj, lanzkron s, beach mc, haywood c, park h, witkop c, et al. 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. The most common type is known as sickle cell anaemia sca. Moderate dose of hydroxyurea in children with sickle cell. Hydroxyurea for the treatment of sickle cell disease prepared for.
Apr 25, 2019 hydrea hydroxyurea is an antineoplastic anticancer agent used to treat melanoma, resistant chronic myelocytic leukemia, and recurrent, metastatic, or inoperable carcinoma of the ovary and primary squamous cell epidermoid carcinomas of the head and neck. Pdf effect of hydroxyurea on hemoglobin s researchgate. Hydroxyurea induces fetal hemoglobin by the nitric oxide. Pdf previous studies have determined the laboratory alterations, clinical. Sickle cell anemia red blood cells with normal hemoglobin red blood cells with sickle cell hemoglobin. Jan 07, 2019 morbiletalidad en pacientes adultos con drepanocitosis. Sintomas y tratamientos anemia drepanocitica ncbddd cdc. Morbiletalidad en pacientes adultos con drepanocitosis. 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.
Advise protection from sun exposure and monitor for the development of secondary malignancies. Nih stateofthescience conference statement on hydroxyurea. Effect of hydroxyurea on g gamma chain fetal hemoglobin. Strouse jj1, lanzkron s, beach mc, haywood c, park h, witkop c, wilson rf, bass eb, segal jb. Combination erythropoietin hydroxyurea therapy in sickle cell disease.
To assess the toxicity and benefits of hydroxyurea hu in the management of children with sickle cell. In patients receiving longterm hydroxyurea for myeloproliferative disorders, secondary leukemia has been reported. 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. Predictors of benefit from hydroxyurea in studies of sickle cell disease41 table 4. Hydroxyurea versus aspirin and hydroxyurea in essential. 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. Listing a study does not mean it has been evaluated by. Benefits of hidroxyurea therapy in children with sickle cell disease. Voet and voet biochemistry, klatt webpath, stryer biochemistry, goldman cecil textbook of medicine. The mechanism of the salutary effect of hydroxyurea is largely attributable to its ability to increase fetal hemoglobin hbf levels.
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