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199406, Ñàíêò-Ïåòåðáóðã, óë.Ãàâàíñêàÿ, ä. 49, êîðï.2

ISSN 1999-6314

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«
Vol. 14, Art. 10 (pp. 113-134)    |    2013       
»

Von Willebrand disease in women
Koloskov A.V.1,2, Stolitsa A.A.2, Philippova O.I.1,3

1City Hospital ¹ 26, St.Petersburg, Russia
2Almazov federal heart, blood and endocrinology centre
Federal specialized perinatal center, St.Petersburg, Russia
3Russian Research Institute of Hematology and Transfusiology, Russian Federal Medicobiological Agency, Saint-Petersburg



Brief summary

Women with inherited bleeding disorders are particularly at risk of bleeding complications from regular haemostatic challenges during menstruation and childbirth. In the last decade, there has been an international research interest in women with inherited bleeding disorders. This has led to considerable progress in the identification of obstetric and gynaecological problems in these women and raising clinical awareness amongst their care providers. Menorrhagia is the commonest bleeding symptoms in women with inherited bleeding disorders and could be the first or only presenting symptom. Childbirth also presents an intrinsic haemostatic challenge to these women. They require specialized and individualized care during pregnancy. von Willebrand’s disease is the commonest inherited bleeding disorder with a prevalence of approximately 1 % - 2% in the general population. Disease is 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. Von Willebrand disease is not a typical example of the dysfunction of the plasma level of coagulation as a defect of von Willebrand factor causes, above all, the violation of the aggregation of platelets.


Key words

von Willebrand disease, menorrhagia, pregnancy, miscarriage, childbirth





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