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	Provedor de dados BJMBR (2) Autor Arruda,V.R. (2) Costa,F.F. (2) Saad,S.T.O. (2) Franco de Carvalho,R. (1) Lima,C.S.P. (1) Palavra-chave Complement PIG-A (1) Hemoglobinopathy (1) Hydroxyurea (1) Intravascular hemolysis (1) Mutation (1) Paroxysmal nocturnal hemoglobinuria (1) Sickle cell disease (1) Mais... Tipo do documento Info:eu-repo/semantics/article (2) Ano 2001 (1) 1997 (1) País Brazil (2) Idioma Inglês (2)		Ordenar por:  Relevância Autor Título Ano Registros recuperados: 2 Primeira ... 1 ... Última Detection of somatic mutations of the PIG-A gene in Brazilian patients with paroxysmal nocturnal hemoglobinuria BJMBR Franco de Carvalho,R.; Arruda,V.R.; Saad,S.T.O.; Costa,F.F.. Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired clonal syndrome characterized by intravascular hemolysis mediated by complement, thrombotic events and alterations in hematopoiesis. Basically, the molecular events which underlie the complexity of the syndrome consist of the absence of the glycosylphosphatidylinositol (GPI) anchor as a consequence of somatic mutations in the PIG-A gene, located on the X chromosome. The GPI group is responsible for the attachment of many proteins to the cytoplasmic membrane. Two of them, CD55 and CD59, have a major role in the inhibition of the action of complement on the cellular membrane of blood cells. The absence of GPI biosynthesis can lead to PNH. Since mutations in the PIG-A gene are always present in patients... Tipo: Info:eu-repo/semantics/article Palavras-chave: Paroxysmal nocturnal hemoglobinuria; Intravascular hemolysis; Complement PIG-A; Mutation. Ano: 2001 URL: http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0100-879X2001000600010 Minimal doses of hydroxyurea for sickle cell disease BJMBR Lima,C.S.P.; Arruda,V.R.; Costa,F.F.; Saad,S.T.O.. The use of hydroxyurea (HU) can improve the clinical course of sickle cell disease. However, several features of HU treatment remain unclear, including the predictability of drug response and determination of adequate doses, considering positive responses and minimal side effects. In order to identify adequate doses of HU for treatment of sickle cell disease, 10 patients, 8 with sickle cell anemia and 2 with Sß thalassemia (8SS, 2Sß), were studied for a period of 6 to 19 months in an open label dose escalation trial (10 to 20 mg kg-1 day-1). Hemoglobin (Hb), fetal hemoglobin (Hb F) and mean corpuscular volume (MCV) values and reticulocyte, neutrophil and platelet counts were performed every two weeks during the increase of the HU dose and every 4 weeks... Tipo: Info:eu-repo/semantics/article Palavras-chave: Sickle cell disease; Hydroxyurea; Hemoglobinopathy. Ano: 1997 URL: http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0100-879X1997000800004 Registros recuperados: 2 Primeira ... 1 ... Última

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