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...
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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...
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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...
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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...
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Blood advances 2022 Apr
Seidizadeh Omid et al.

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