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…

Coagulation and clinical features associated with the Arg304Gln mutation (Factor VII Padua) in the IRF7 Study Group

Inhibitors to factor VII in congenital factor VII deficiency.

Ruolo delle caratteristiche di personalità nel rapporto tra aspetti sintomatologici, qualità della vita ed andamento della patologia nelle pazienti con neoplasie ginecologiche in trattamento e in follow up

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

The effects of different levels of exposure on persistence of stress disorders in rescue volunteers: the case of the ATR 72 air disaster in Palermo (Gli effetti di differenti livelli espositivi sulla persistenza dei disturbi da stress nei soccorritori volontari: il caso del disastro aereo dell’ATR 72 a Palermo), Giorn Ital Psicopat 2011.

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 -

Proposal and validation of a novel quantitative approach (QUEM) for the evaluation of menstrual blood losses. Preliminary implementation for the management of menorrhagia in congenital bleeding disorders.

Menorrhagiaisaveryprevalentbleedingsymptomincongenitalbleedingdisorders(from60to100%)and in patients on OralAnticoagulants(OA),but no prospective studies are available. We here describe the validation of a new and simple method for determination of menstrual blòood losses

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

Pharmacokinetic of Factor VII