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. 2002 Apr;7(4):245-9.
doi: 10.1093/pch/7.4.245.

The diagnosis and treatment of von Willebrand disease in children

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The diagnosis and treatment of von Willebrand disease in children

Robert J Klaassen et al. Paediatr Child Health. 2002 Apr.

Abstract

von Willebrand disease is the most common bleeding disorder seen in children and it affects approximately 1% of the population. Because the bleeding symptoms in von Willebrand disease are generally mild, the diagnosis is often delayed. Prompt diagnosis and management can help to avoid potentially life-threatening bleeding events and unnecessary exposure to blood products. In this review, the various types of von Willebrand disease are outlined, the difficulties in diagnosis are discussed and the therapeutic approach to this common disorder is described.

La maladie de von Willebrand est le trouble hémorragique le plus courant chez les enfants. Elle touche environ 1 % de la population. Puisque les symptômes hémorragiques de cette maladie sont généralement bénins, le diagnostic est souvent tardif. Un diagnostic et une prise en charge rapides peuvent toutefois contribuer à éviter des hémorragies pouvant mettre la vie en danger et une exposition inutile aux produits sanguins. Dans la présente analyse bibliographique, les divers types de maladie de von Willebrand sont soulignés, les difficultés reliées au diagnostic sont abordées, et la méthode thérapeutique de ce trouble courant est décrite.

Keywords: Child; Factor VIII; von Willebrand disease; von Willebrand factor.

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Figures

Figure 1)
Figure 1)
Top Normal von Willebrand factor (VWF) function in adhesion of platelets and binding of Factor VIII (FVIII). Bottom Vessel damage as a result of Von Willebrand’s disease (adapted with permission from reference 21)

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