Oral Communication

Inherited factor VII (FVII) deficiency is the most common of the rare autosomal recessive bleeding disorders (RBD), with an estimated prevalence of 1 per 300,000 in European countries.1,2 It is likely that the prevalence is higher in those countries where consanguineous marriages are frequent.We here report STER study results on 112 evaluable enrolled cases

Bleeding symptoms at disease presentation and prediction of ensuing bleeding in inherited FVII deficiency.

SummaryIndividuals with inherited factor VII (FVII) deficiency display bleeding phenotypes ranging from mild to severe, with 30% of patients having always been asymptomatic (non-bleeding). In 626 FVII-deficient individuals, by analysing data from the International Factor VII (IF7) Registry and the Seven Treatment Evaluation Registry (STER), we determined whether bleeding type at disease presentation and FVII coagulant activity (FVIIc) predict ensuing bleeds. At disease presentation/diagnosis, 272 (43.5%) individuals were non-bleeding, 277 (44.2%) had minor bleeds, and 77 (12.3%) had major bleeds. During a median nine-year index period (IP) observation, 87.9% of non-bleeding individuals at p…

Recombinant, activated factor VII for surgery in factor VII deficiency: a prospective evaluation - the surgical STER

Pharmacokinetic properties of recombinant FVIIa in inherited FVII deficiency account for a large volume of distribution at steady state and a prolonged pharmacodynamic effect.

Pharmacokinetic properties of recombinant FVIIa in inherited FVII deficiency account for a large volume of distribution at steady state and a prolonged pharmacodynamic effect -

Thrombosis in inherited factor VII deficiency

Thrombosis in congenital factor (F) VII deficiency was investigated through extensive phenotypic and molecular-genetic studies. Patients with a history of thrombosis among 514 entries in the FVII Deficiency Study Group database were evaluated. Thrombotic events were arterial in one case, disseminated intravascular coagulation in another and venous in seven. Gene mutations were characterized in eight patients: three were homozygous, three compound heterozygous and two heterozygous. FXa and IIa generation assays were consistent with the genetic lesions. One patient was heterozygous for the FV Leiden and one for the FIIG20210A mutation. In seven patients, surgical interventions and/or replacem…

Invasive procedures and minor surgery in factor VII deficiency

Factor VII deficiency: evidence that in minor surgery one-day replacement therapy is sufficient