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Investigating von Willebrand Factor Pathophysiology Using a Flow Chamber Model of von Willebrand Factor-platelet String Formation
Published on: August 14, 2017
Von Willebrand disease
Jeremy Robertson1, David Lillicrap, Paula D James
1Division of Hematology/Oncology, Hospital for Sick Children, 555 University Avenue, Toronto, ON M5G 1X8, Canada.
Insights
von Willebrand disease, a common inherited bleeding disorder, often diagnosed in childhood, significantly impacts quality of life. Early recognition and diagnosis are crucial for managing this condition in pediatric patients.
Area of Science:
- Hematology
- Pediatric Medicine
- Genetics
Background:
- von Willebrand disease is a prevalent inherited bleeding disorder.
- Diagnosis frequently occurs during childhood, affecting quality of life.
- Timely recognition and diagnosis are essential for patient management.
Purpose of the Study:
- To review the pathophysiology of von Willebrand disease.
- To discuss the current classification system for the disorder.
- To outline available treatments, focusing on pediatric considerations.
Main Methods:
- Literature review of pathophysiology.
- Analysis of current classification schemes.
- Summary of treatment options for pediatric populations.
Main Results:
- Detailed review of von Willebrand disease mechanisms.
- Overview of established diagnostic criteria and classifications.
- Discussion of therapeutic strategies tailored for children.
Conclusions:
- Understanding pathophysiology aids diagnosis and treatment.
- Current classification provides a framework for management.
- Effective treatment strategies are available for pediatric von Willebrand disease, improving quality of life.
Abstract:
von Willebrand disease is a common inherited bleeding disorder and many cases are diagnosed in childhood. It has a negative impact on the quality of life of affected individuals; therefore, it is important that the condition be recognized and diagnosed. This article reviews the pathophysiology of the condition, the current classification scheme, and the available treatments, highlighting issues specific to the pediatric population.
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