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Br J Haematol ; 142(2): 250-6, 2008 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-18492116

RESUMO

Hemophilia is traditionally classified according to the levels of the deficient coagulation factor as Severe (<1%), Moderate (1-5%) or Mild (>5%). However, it is well known that the factor activity does not necessarily correspond to the clinical bleeding manifestations. As prophylactic therapy is the best method of prevention of serious complications such as hemophilic arthropathy, a test that may predict the bleeding pattern would be extremely beneficial. Thromboelastography (TEG) uses whole blood to determine clot formation characteristics, such as initiation, propagation as well as strength of the clot, and is now being extensively studied in bleeding and thrombophilia. This study attempted to determine the TEG characteristics in 47 children with moderate hemophilia (MH) and severe hemophilia with (SHI) and without inhibitors (SH) and tried to retrospectively correlate them to the clinical bleeding patterns. TEG showed evidence of faster and better clot formation, as evidenced by a higher maximum thrombin/fibrin generation, in those with mild bleeding manifestations compared to those with severe bleeding tendency, in addition to the expected prolongation in time to formation of clot related to factor deficiency. This may be a potentially useful tool to evaluate the bleeding tendency and determine need for prophylaxis in children with hemophilia.


Assuntos
Fatores de Coagulação Sanguínea/metabolismo , Coagulação Sanguínea/efeitos dos fármacos , Hemofilia A/sangue , Tromboelastografia , Trombina/metabolismo , Adolescente , Biomarcadores/sangue , Testes de Coagulação Sanguínea/métodos , Criança , Pré-Escolar , Fator V/metabolismo , Fator VIII/metabolismo , Feminino , Fibrinogênio/metabolismo , Hemofilia A/genética , Homocistina/metabolismo , Humanos , Lactente , Masculino , Mutação , Protrombina/genética , Protrombina/metabolismo , Tromboelastografia/métodos , Adulto Jovem
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