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ORIGINAL ARTICLE
Year : 2012  |  Volume : 1  |  Issue : 4  |  Page : 227-232

To assess the efficacy of hydroxyurea, in children with homozygous sickle cell disease, in the age group of 1 year to 18 years, at tertiary care hospital


Department of Pediatrics, Maharajah's Institute of Medical Sciences (MIMS), Nellimarla, Vizianagaram, Andhra Pradesh, India

Correspondence Address:
Sunil K Pondugala
54-3-15/6/16, SF-5, TSR Sadvilas Apts, Isukathota, Visakhapatnam, Andhra Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2277-8632.105107

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Aims: To evaluate the increase in fetal hemoglobin percentage and MCV in children of sickle cell disease treated with hydroxyurea. To observe the safety of hydroxyurea in treated children. To arrive at the therapeutic dose of the drug to produce the beneficial effects. Settings and Design: A single blind randomized controlled trial of 12 months duration was designed to study the effect of hydroxyurea on sickle cell patients which aims to study the clinical and laboratory effects, in tertiary care hospital. Materials and Methods: Sixty children with severe sickle cell disease in the age group of 1-18 years were studied. Outcomes were measured in terms of decrease in number of vaso-occlusive crises, necessity of blood transfusions, laboratory parameters for different organ functions, blood cell counts and toxicity of hydroxyurea. Results: In patients of sickle, cell disease treated with hydroxyurea there was significant reduction in number and severity of vaso- occlusive crises and necessity of blood transfusions. Total hemoglobin, MCV and fetal hemoglobin percentage were raised significantly. Bone marrow suppression as reflected by the decrease in white blood cell and reticulocyte counts occurred but it was not below the defined toxicity levels. Serum bilirubin was decreased significantly. Conclusions: In the present study, we concluded that, hydroxyurea decreases number of vaso-occlusive crises and need for blood transfusions in children with severe sickle cell disease. It increases total, fetal hemoglobin concentration and MCV. It causes bone marrow suppression thereby decreasing white blood cell and reticulocyte count. However, this fall was never below defined toxicity levels. It causes no hematological, hepatic, renal or general toxicities when given on short-term basis. Overall, hydroxyurea has proved to be safe and efficacious in children between the age groups of 1-18 years.


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