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Uso del FVII activado recombinante en la enfermedad de Rendú-Osler-Weber o telangiectasia hemorrágica hereditaria y sangramiento digestivo: Primer caso comunicado en Cuba

Keywords: rfviia, non-hemophilic patients, liver transplant, rendú osler weber's disease.

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Abstract:

the recombinat activated factor vii (rfviia novoseven, novonordisk, denmark) has been successfully used in the treatment of hemorrhages in hemophilic patients with inhibitors, in the congenital deficiency of fvii and in glanzmann's thromboasthenia. besides, its use has been recommended in non-hemoplilic patients with acquired antibodies against fviii (acquired hemophilia) and in other disorders of the platelet function as bernard soulier syndrome (sbs). when it is administered at pharmacological doses, it increases the generation of thrombin on the activated platelet, and it may be benefitial in other disorders characterized by profuse bleeding and inadequate generation of thrombin, such as the thrombocytopenias. it has been used in hemorrhages secondary to alterations of the liver function and in severe trauma. its administration to patients with rendú osler weber's disease and severe bleedings is a new indication, taking into account the activation of coagulation in the specific site of the lesion. it is reported the favorable response of a patient with severe digestive bleeding endangering his life that received rfvii at doses of 90 μg/kg until completing 3 doses in 24 hours. the hemorrhage stopped and its potent hemostatic character in uncontrollable bleedings was confirmed

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