Actualités

Consultez les derniers articles autour de la maladie de Willebrand.

Dernière mise à jour : Vendredi 20 mai 2022

Quantification of the relationship between desmopressin concentration and Von Willebrand factor in Von Willebrand disease type 1: A pharmacodynamic study

Von Willebrand disease (VWD) is the most common inherited bleeding disorder and is caused by a deficiency or qualitative defect of von Willebrand factor (VWF). VWF is a plasma glycoprotein which plays...
Lire la suite

Haemophilia 2022; AOP: 10.1111/hae.14582
Jessica M. Heijdra et al.

Efficacy and safety of von Willebrand factor concentrate almost devoid of factor VIII (Wilfactin®) in paediatric patients under 6 years of age with severe von Willebrand disease.

Plasma-derived von Willebrand factor (VWF) (Wilfactin®, LFB, France) was developed for prophylaxis and treatment of haemorrhages in both adults and adolescents with von Willebrand disease (VWD). Replacement...
Lire la suite

Blood transfusion = Trasfusione del sangue 2022 Apr
Gouider Emna et al.

Desmopressin response depends on the presence and type of genetic variants in patients with type 1 and type 2 von Willebrand disease.

Patients with type 1 and type 2 von Willebrand disease (VWD) can be treated with desmopressin. Although a previous study has shown that the location of the causative VWF gene variant is associated with...
Lire la suite

Blood advances 2022 Apr
Atiq Ferdows et al.

Phenotypic and genetic characterization of the Milan cohort of von Willebrand disease type 2.

von Willebrand disease (VWD) type 2 is caused by qualitative abnormalities of von Willebrand factor (VWF). This study aimed to determine the genotype and phenotype characterization of a large VWD type 2 cohort from Milan. We included 321 patients (54% females) within 148 unrelated families from 1995-2021. Patients were fully characterized using laboratory phenotype tests and the genotype diagnosis was confirmed by target genetic analysis using Sanger...
Lire la suite

Blood advances 2022 Apr
Seidizadeh Omid et al.

Découvrez WillebrandXPERTet ses fonctionnalités en quelques instants