Identification of five novel mutations in the factor XI gene (F11) of patients with factor XI deficiency.

Factor XI (FXI) deficiency is an inherited autosomal recessive disorder associated with bleeding of variable severity. However, many cases of dominant disease transmission have been recently described. This disorder is rare in the general population, whereas it is commonly found in individuals of Ashkenazi Jewish ancestry.

Study of human von Willebrand factor immunogenicity in pigs with severe von Willebrand disease.

Replacement therapy is the treatment of choice for patients with von Willebrand disease who are unresponsive to desmopressin. In order to prevent transmission of non-enveloped viruses, a solvent/detergent-treated plasma-derived von Willebrand factor available in France since 1989 has been subjected to additional removal/inactivation steps by 35 nm filtration and dry heating for 72 h at 80 degrees C. This preclinical study evaluates the potential immunogenicity of this new product by comparing the antibodies raised in pigs affected with von Willebrand disease after intravenous injection of either a solvent/detergent-treated product or a triple-secured product.