Hemophilia.

Hemophilia is a genetic disease in which the clinical manifestation is mainly the presence of hemorrhage. There are two known types of hemophilia: hemophilia A and B, which have a deficiency of factor VIII or IX clotting, respectively. The intensity of bleeding in hemophilia depends on the plasma le...

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Veröffentlicht in:Revista médica (Mexico : 1983) 2013-11, Vol.51 (6), p.638
Hauptverfasser: Amador-Medina, Lauro Fabián, Vargas-Ruiz, Angel Gabriel
Format: Artikel
Sprache:spa
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Zusammenfassung:Hemophilia is a genetic disease in which the clinical manifestation is mainly the presence of hemorrhage. There are two known types of hemophilia: hemophilia A and B, which have a deficiency of factor VIII or IX clotting, respectively. The intensity of bleeding in hemophilia depends on the plasma levels of factor VIII or IX and has traditionally been classified as mild (> 5 % activity), moderate (1-5 % activity) and severe (< 1 % activity). In laboratory tests, isolated prolongation of activated partial thromboplastin time (aPTT) can be found, but it is necessary to determine the plasma levels of factor VIII or IX to establish the diagnosis of hemophilia A or B. The treating of this disease involves replacing exogenous factor VIII or IX concentrates. Gene therapy could be an option in the future to achieve the cure of the disease. Complications of hemophilia are the risk of transfusion-associated infections, pseudotumor hemophilic, hemophilic arthropathy and the presence of serum inhibitors.
ISSN:0443-5117