Abnormal von Willebrand factor secretion, factor VIII stabilization and thrombus dynamics in type 2N von Willebrand disease mice.

Unlabelled: Essentials Type 2N von Willebrand disease involves impaired von Willebrand factor to factor VIII binding. Type 2N von Willebrand disease mutations exhibit qualitative and mild quantitative deficiencies. Type 2N von Willebrand disease mice exhibit unstable venous hemostatic thrombi.

Relative contributions of bleeding scores and iron status on health-related quality of life in von Willebrand disease: a cross-sectional study.

Introduction: von Willebrand disease (VWD) is the most common inherited bleeding disorder known in humans. Currently, studies investigating the health-related quality of life (HR-QoL) in VWD using standardized tools are limited, particularly among patients with mild decreases in von Willebrand factor or activity.

Aim: To determine HR-QoL and its predictors among patients with mild, moderate and severe forms of VWD.

Generation and optimization of the self-administered bleeding assessment tool and its validation as a screening test for von Willebrand disease.

Introduction/aim: Our aim was to generate, optimize and validate a self-administered bleeding assessment tool (self-BAT) for von Willebrand disease (VWD).

Methods: In Phase 1, medical terminology in the expert-administered International Society on Thrombosis and Haemostasis (ISTH)-BAT was converted into a Grade 4 reading level to produce the first version of the Self-BAT which was then optimized to ensure agreement with the ISTH-BAT. In Phase 2, the normal range of bleeding scores (BSs) was determined and test-retest reliability analysed.

Normal range of bleeding scores for the ISTH-BAT: adult and pediatric data from the merging project.

Bleeding Assessment Tools (BATs) have been developed to aid in the standardized evaluation of bleeding symptoms. The Vicenza Bleeding Questionnaire (BQ), published in 2005, established a common framework and scoring key that has undergone subsequent modification over the years, culminating in the publication of the ISTH-BAT in 2010. Understanding the normal range of bleeding scores is critical when assessing the utility of a BAT.

Ectopic fat in youth: the contribution of hepatic and pancreatic fat to metabolic disturbances.

Objective: To study the relationships between parameters of glucose and insulin metabolism and visceral and abdominal ectopic fat in youth.

Methods: A cross sectional study of 50 children (24 females), 8-18 years old. Anthropometrics, body composition, blood-work and visceral and ectopic fat by magnetic resonance imaging were assessed.