الفهرس 
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Abstract
von Willebrand disease (VWD), the most common inherited bleeding disorder, caused by a deficiency or dysfunction of the von Willebrand factor (VWF). This study details the inheritance patterns, genetic characteristics, and classification of the disease, including its major types: Type 1, 2, and 3, each with subtypes and specific functional defects. The clinical presentation of VWD is variable, with symptoms including excessive mucocutaneous bleeding, and more severe musculoskeletal bleeding in certain cases. The severity and type of symptoms depend on factors such as disease subtype, level of von Willebrand factor, and the patient’s age and sex. The approach for diagnosing VWD includes assessing the patient’s personal and family bleeding history and performing requisite laboratory studies.
Bleeding Assessment Tools (BATs) is important in diagnosing bleeding disorders. It emphasizes the necessity of standardized and accurate bleeding history assessments to avoid unnecessary laboratory tests and wastage of health resources. The review traces the development of BATs from the 1995 bleeding questionnaire by Sramek and colleagues to the ISTH/SSC-BAT jointly developed by the International Society on Thrombosis and Haemostasis (ISTH) and the Scientific and Standardization Committee (SSC). These tools, including the Vicenza-based BATs, aim to distinguish between affected and unaffected individuals, predict the risk of bleeding, describe symptom severity, and inform treatment. The document also discusses the limitations of earlier BATs and efforts to rectify them, such as the inclusion of symptom frequency and severity, and the creation of a web-based freely accessible version. Lastly, it provides an overview of the ISTH/SSC-BAT, which includes pediatric-specific symptoms and considerations for symptom frequency and clinical importance beyond just severity.