Diagnosis of Von Willebrand Disease in Children

Nupur      Mittal; Paula      James; Leonard   A.   Valentino

Abstract

Von Willebrand disease (VWD) is an inherited bleeding disorder that is caused by deficiency or dysfunction of Von Willebrand factor, a plasma protein that mediates the initial adhesion of platelets at sites of vascular injury and also binds and stabilizes blood clotting factor VIII in the circulation. VWD prevalence has been estimated in several countries on the basis of the number of symptomatic patients seen at haemostasis centers, and the values range from roughly 23 to 110 per million population. VWD is characterized by three key features: a personal history of excessive mucocutaneous bleeding, abnormal VWF laboratory studies, and evidence of a family history of the condition. Although bruising and epistaxis are common among children who have VWD, these symptoms also are reported in normal children. The accurate assessment of hemorrhagic symptoms is a key component in diagnosing VWD but this can present a significant challenge, particularly in the pediatric population. The challenges involved in making the diagnosis of Von Willebrand disease - focusing on personal bleeding history and the utility of bleeding questionnaires, along with family bleeding history and laboratory diagnosis in the pediatric population - will be reviewed.

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