title: [Effect of hydroxyurea on hemoglobin S].
abstract: Previous studies have determined the laboratory alterations, clinical efficacy and toxicity profile associated with hydroxyurea (HU) therapy in patients with severe sickle cell anemia. We report the efficacy of HU treatment in the prevention of vaso-occlusive crises in an 11-years-old boy with severe sickle cell disease. The number of vaso-occlusive crises, hospital days and blood transfusions in the year before HU treatment were compared with the same parameters at 6, 12, 24, 36 and 72 months of treatment. A decrease in the frequency of vaso-occlusive crises, blood transfusions and days spent in hospital were demonstrated during the HU treatment period compared to the same period before hand. The clinical and laboratory response to HU was dramatic in this severely affected patient, allowing him a normal schooling and social life. The adverse effects observed were not serious and reversed after transient discontinuation of HU. We conclude that long-term chronic treatment with HU for seriously ill sickle cell patients appears feasible, and devoid of any major toxicity.
authors.affiliation: Servicio de Hematología-Oncología, Hospital de Pediatría Juan P. Garrahan, Combate de los Pozos 1881, 1245 Buenos Aires, Argentina. afeliu@garrahan.gov.ar
authors.forename: Aurora Felíu
authors.initials: AF
authors.lastname: Torres
authors.forename: Silvia Eandi
authors.initials: SE
authors.lastname: Eberle
authors.forename: Gabriela
authors.initials: G
authors.lastname: Sciuccati
authors.forename: Mariana
authors.initials: M
authors.lastname: Bonduel
mesh_terms: D000755:Anemia, Sickle Cell / Q000150:complications / Q000188:drug therapy*; D000986:Antisickling Agents / Q000627:therapeutic use*; D001157:Arterial Occlusive Diseases / Q000188:drug therapy / Q000209:etiology / Q000517:prevention & control*; D001803:Blood Transfusion; D002648:Child; D006451:Hemoglobin, Sickle / Q000187:drug effects*; D006801:Humans; D006918:Hydroxyurea / Q000627:therapeutic use*; D008297:Male
chemical_list: D000986:Antisickling Agents; D006451:Hemoglobin, Sickle; D006918:Hydroxyurea