S PONTANEOUS BLEEDING episodes and hemorrhage following surgery or trauma in hemophilia can be treated readily with the potent Factor VIII concentrates now available.1#{176} However, requirements for long-term prophylactic therapy to prevent hemarthroses and other spontaneous hemorrhages have not been clearly defined. Three reports of prophylactic therapy in hemophilia employing widely differin...

a group of iranian patients suffering from factor viii deficiency (hemophilia a) and treated with contaminated coagulation factor (imported), became seropositive as determined by elisa method. sixty of these individuals, which were available, were studied for abo distribution. the b blood group in anti hiv pos. individuals (13.33%) shows a significant decrease in comparison with the total (1504...

Hemophilia A is an X-linked recessive disorder caused by factor VIII deficiency, which is an important factor in the coagulation system. Here, we describe a 1-year-old boy with hemophilia A who developed West syndrome (WS). Recombinant factor VIII was administered during adrenocorticotropic hormone (ACTH) therapy to prevent intracranial hemorrhage. Infusion of factor VIII at fixed intervals is ...

Previously we created two strains of factor VIII-deficient mice by insertion of a neo gene into (1) the 3' end of exon 16 and (2) exon 17 of the factor VIII gene. Affected mice of both strains have no plasma factor VIII activity, yet are healthy with no spontaneous bleeding. Factor VIII-deficient females bred with affected males survive pregnancy and delivery. We used reverse transcriptase-poly...

BACKGROUND Pseudoaneurysm formation is a rare complication of arterial puncture. CASE CHARACTERISTICS 3-week-old male developed an enlarging mass over the anterior aspect of left wrist following radial arterial puncture. OBSERVATION Doppler ultrasonography revealed mass to be left radial arterial pseudoaneurysm. Subsequent presentation of ecchymoses and investigations confirmed factor VIII ...

Hemophilia A is an inherited bleeding disorder characterized by factor VIII deficiency. The basis for insufficient hemostasis lies within inadequate amplification of factor Xa production with the undersupplied factor VIII. We report on a young patient with critical aortic stenosis bearing all the clinical stigmata of severe hemophilia, in whom aortic valve replacement was performed with a tissu...

Combined factor V and factor VIII deficiency (F5F8D) is a rare, autosomal recessive congenital bleeding disorder with a prevalence of 1:1,000,000. Individuals present with a variable clinical bleeding phenotype, elevated prothrombin time (PT) and partial thromboplastin time (PTT), and plasma levels of factor V and VIII of 5–30%. Mutations in 2 proteins, LMAN1 and MCFD, required for concurrent t...

There are three major types of VWD disease. Type 1, the most frequent form, is characterized by a partial quantitative deficiency in von Willebrand factor (VWF). Type 2 is a qualitative deficiency, and Type 3 is a virtually complete deficiency. Type 2 VWD is divided into four subtypes. Type 2A includes variants with decreased platelet adhesion caused by a selective deficiency in high-molecular ...

There are three major types of VWD disease. Type 1, the most frequent form, is characterized by a partial quantitative deficiency in von Willebrand factor (VWF). Type 2 is a qualitative deficiency, and Type 3 is a virtually complete deficiency. Type 2 VWD is divided into four subtypes. Type 2A includes variants with decreased platelet adhesion caused by a selective deficiency in high-molecular ...