SOVREMENNAYA PEDIATRIYA.2017.2(82):122-129; doi 10.15574/SP.2017.82.122

Dubey L.Ya., Dubey N. V., Markin A. I., Tsymbaluk-Voloshyn I. P., Dorosh O. I., Troyanovska O. O., Kozlova O. I., Stepanuk O. I., Vorobel O. I., Sapuzhak M. V., Dubey J. L., Shorobura N. I.
Danylo Halytsky National Medical University, Lviv, Ukraine
Western Ukrainian Specialized Children's Medical Centre, Lviv, Ukraine

Von Willebrand disease (VWD) is a genetic disorder of blood clotting, which is transmitted autosomal genetic factors and is associated with both quantitative and qualitative defects of VW factor (VWF). There are many different types of VWD, classified, however, solely according to the phenotypic characteristics of the protein. VWD is a heterogeneous disease with different clinical and laboratory manifestations that are the basis of pathogenetic mechanisms of its development. The conducted genetic analysis made it possible to identify additional heterogeneity among different types of disease with similar phenotypic characteristics. Despite the fact that the VWD classification is constantly amended and augmented, the ambiguity of its classification criteria still remains unsolved and requires continuing research efforts.

Key words: von Willebrand disease, classification.

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