[Diagnostic approach to von Willebrand's disease in childhood].

Von Willebrand's disease seems to be the most common hereditary bleeding disorder in children. Every form of this disease is based on quantitative or qualitative disorder of von Willebrand's factor influencing adhesion of thrombocytes (primary hemostasis) and stabilization FVIII:C in circulation (secondary hemostasis). We present contemporary knowledge on epidemiologic investigations, molecular biology, classification and rational diagnostic approach to von Willebrand's disease in children. We also report on evaluating laboratory tests' reliability in 51 examined children with von Willebrand's disease. It can be concluded that most laboratory tests are highly specific and significant for diagnosis. Determination of vWF activity has optimal value for establishing laboratory diagnosis of von Willebrand's disease; RIPA and multimer analysis are valuable for subtype classification.