Publications by authors named "K P M van Galen"
Article Synopsis
- The study investigates how patients with Von Willebrand disease (VWD) perceive the severity of their condition, highlighting a gap in understanding patient experiences alongside traditional lab measures.
- A nationwide survey in the Netherlands included 736 VWD patients, who reported their disease severity, bleeding scores, and quality of life through a questionnaire.
- Results show that self-reported severity aligns well with clinical classifications, and factors like type of VWD, bleeding severity, gender, treatment history, and specific lab values significantly influence patients' perception of their disease severity.
Article Synopsis
- Type 2B Von Willebrand disease (VWD) is a bleeding disorder linked to specific genetic variations in the VWF gene, and the study aimed to explore how these genetic differences affect clinical symptoms over a 16-year period in a cohort of 64 patients.
- The research found that 67.2% of patients experienced thrombocytopenia (low platelet counts), which was most significantly associated with the p.Arg1306Trp genetic variant, showing considerably lower platelet counts compared to another variant, p.Arg1308Cys.
- Additionally, while some patient pregnancies led to decreased platelet counts, postpartum hemorrhages occurred despite preventative treatment, highlighting the complex relationship between genetic factors and bleeding events in affected individuals.
Background: Between 2002 and 2011, the incidence of severe primary postpartum hemorrhage (PPH) in Dutch women with von Willebrand disease (VWD) and hemophilia carriers (HCs) was 8% vs 4.5% in the general population.
Objectives: To determine the contemporary incidence of severe primary PPH in women with VWD and HCs.
Article Synopsis
- Patients with von Willebrand disease (VWD) need von Willebrand factor (VWF) concentrates during surgery, and this study looked at how FVIII, a related clotting factor, behaves with repeated infusions of a 1:1 ratio VWF/FVIII concentrate.
- The study involved 125 patients undergoing various surgical procedures and measured FVIII and VWF levels at different times after CFC infusions, finding consistent recovery rates without significant accumulation of FVIII levels even after multiple doses in some patients.
- Results showed that administering the concentrate led to stable FVIII and VWF activity levels, highlighting the product's effectiveness, particularly that most major surgeries only required three CFC infusions for optimal results.
Introduction: In patients with an increased bleeding tendency, extensive diagnostic blood testing is often performed. When results of tier 1 assays of primary haemostasis are normal, protocols recommend additional testing to rule out rare disorders including coagulation factor XIII (FXIII) and α2-antiplasmin (α2AP) deficiency.
Aim: To evaluate the added diagnostic value of FXIII and α2AP levels in patients with a bleeding disorder of unknown cause (BDUC).