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1 Tishreen University Journal for Research and Scientific Studies - Medical Sciences Series Vol. (0) No. ( ) HB ٩٩

2 Tishreen University Journal for Research and Scientific Studies - Medical Sciences Series Vol. (0) No. ( ) 008 Hydroxyurea in the Treatment of Sickle Cell Disease (Received 7 / / 008. Accepted 4/8/008) ABSTRACT Dr. Michael Georgeos * Dr. Ryad Galek** Youla Ahmad*** This study aims to assess the laboratory and clinical benefits of hydroxyurea in the treatment of sickle cell disease. This study includes (0 ) patients of SCD who had reported at least three painful crises in the previous year, or had reported at least three transfusions or hospitalization in a year, or who had a history of stroke, acute chest syndrome or splenic sequestration, or who had systemic sever anemia. Hydroxyurea was gradually increased from an initial dose of mg / kg / d to 0 mg / kg / d. These patients were monitored by -month visits for one year to assess laboratory changes and clinical efficacy. Significant hematologic changes included increase in (HB - - ) and decrease in. And there was decrease in the number of transfusions, and painful crises. However, there were five patients who did not show any response to the treatment and the concentration did not increase more than % after a treatment for one year. Key words: Hydroxyurea, sickle cell disease, painful crises, recurent hospitalaization, fetal hemoglobin. * prof, Medical Department, Faculty of medicine, Tishreen University, Lattakia, Syria. **Associate prof, Department of Internal Disease, Faculty of Medicine, Tishreen University, Lattakia, Syria. ***postgraduate student, Department of Internal Disease, Faculty of Medicine, Tishreen University, Lattakia, Syria. ٠

3 Tishreen University Journal. Med. Sciences Series alpha, beta S S β S [] γ, α [] S γ α β [] HbF [] HbF SCD [4] SCD [] ] ١

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5 Tishreen University Journal. Med. Sciences Series X X i P i 00 HB p i ( ( ٣

6 بدء الدراس ة بعد ٦ أشھر بعد عام مریض ١ مریض ٢ مریض ٣ مریض ٤ مریض ٥ مریض ٦ مریض ٧ مریض ٨ مریض ٩ مریض ١٠ مریض ١١ مریض ١٢ مریض ١٣ مریض ١٤ مریض ١٥ مریض ١٦ مریض ١٧ مریض ١٨ مریض ١٩ مریض ٢٠ مریض ٢١ مریض ٢٢ مریض ٢٣ مریض ٢٤ مریض ٢٥ ٤

7 Tishreen University Journal. Med. Sciences Series بعد عام بعد ٦ أشھر بدء الدراسة مریض ١ مریض ٢ مریض ٣ مریض ٤ مریض ٥ مریض ٦ مریض ٧ مریض ٨ مریض ٩ مریض ١٠ مریض ١١ مریض ١٢ مریض ١٣ مریض ١٤ مریض ١٥ مریض ١٦ مریض ١٧ مریض ١٨ مریض ١٩ مریض ٢٠ مریض ٢١ مریض ٢٢ مریض ٢٣ مریض ٢٤ مریض ٢٥ ٥

8 p i ( 4 4 ٦

9 Tishreen University Journal. Med. Sciences Series بدء الدراسة بعد ٦ أشھر بعد عام مریض ١ مریض ٢ مریض ٣ مریض ٤ مریض ٥ مریض ٦ مریض ٧ مریض ٨ مریض ٩ مریض ١٠ مریض ١١ مریض ١٢ مریض ١٣ مریض ١٤ مریض ١٥ مریض ١٦ مریض ١٧ مریض ١٨ مریض ١٩ مریض ٢٠ مریض ٢١ مریض ٢٢ مریض ٢٣ مریض ٢٤ مریض ٢٥ ٧

10 بدء الدراسة بعد ٦ أشھر بعد عام مریض ١ مریض ٢ مریض ٣ مریض ٤ مریض ٥ مریض ٦ مریض ٧ مریض ٨ مریض ٩ مریض ١٠ مریض ١١ مریض ١٢ مریض ١٣ مریض ١٤ مریض ١٥ مریض ١٦ مریض ١٧ مریض ١٨ مریض ١٩ مریض ٢٠ مریض ٢١ مریض ٢٢ مریض ٢٣ مریض ٢٤ مریض ٢٥ ٨

11 Tishreen University Journal. Med. Sciences Series 00 HB ).±.±..+_.) +_.) 44 4 (. 7 ٩

12 7.+_0.8) HB (8) ٠

13 Tishreen University Journal. Med. Sciences Series متوسط قیم HB 0 بعد عام بعد ٦ أشھر عند بدء الدراسة بدء الدراسة بعد ٦ أشھر بعد عام متوسط قیم ١

14 بدء الدراسة متوسط بعد عام بعد ٦ أشھر ) Fetal hemaglobin in sickle cell anemia : Determinants of Respanse to Hydroxyurea(7) ) Five years of experience with hydroxyurea in children and young adults with sickle cell disease (8) ) Fetal hemoglobin f-cell Respanses to long term hydroxyurea Treatment in Young Sickle cell patients.(9) ٢

15 Tishreen University Journal. Med. Sciences Series 0 /B-Thal /B -Thal S! safty of hydroxyurea in children with sickle cell Anemia (0) HB F ٣

16 HB ٤

17 Tishreen University Journal. Med. Sciences Series - BUNN,H.F. pathogenesis and treatment of sickle cell disease. N Engl J Med, 997, 7:7. - VICHINSKY, E. New therapies in sickle cell disease. Lancet, 00, 0:9. - CHARACHE, S; TERRIN, M; Moore, R. Effect of hydroxyurea on the frequency of painful crisis in sickle cell anemia. N Engl J Med, 99, : CHARACHE, S; DOVER, G; MOYER, M. Hydroxyurea - induced augmentation of fetal hemoglobin production in patients with sickle cell anemia. Blood, 987, 9: MARTIN, H. Current use of hydroxy urea in sickle cell diseas. Hematol, : MARTIN, H, FRANCA, B, MICHAEL, L fetal hemoglobine in sickle cell anemia;determinants of response to hydroxyurea. BLOOD vol. 89no. (february)997; ALINA, F, CHRISTIANE, V, KATHLEEN, H five years of experience with hydroxyurea in children and young adults with sickl e cell disease. BLOOD vol.79no.(june),00; MICHELINE, M. ANTOINE, ROBERT,G. fetal hemoglobin and f-cell responses to long -term hydroxyurea treatment in young sickle cell patients. BLOOD, vol. 9no.(juneis),998; THOMAS, R, RONALD,w, HELMS, E.saftey of hydroxyurea in children with sickle cell anemia BLOOD,vol94no. (septemper),999; 0-4 ٥

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