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Bernard–Soulier syndrome (BSS), also called hemorrhagiparous thrombocytic dystrophy, is a rare autosomal recessive coagulopathy (bleeding disorder) that causes a deficiency of ''glycoprotein Ib'' (GpIb), the receptor for von Willebrand factor, an important glycoprotein involved in hemostasis. The incidence of BSS is estimated to be less than 1 case per million persons, based on cases reported from Europe, North America, and Japan.〔(Anesthetic and perioperative management of a patient with Bernard-Soulier syndrome ) Georgia Kostopanagiotou MD, Ioanna Siafaka MDa, Constantinos Sikiotis MDa, and Vassilios Smyrniotis MDa. Received 23 July 2003; Revised 21 October 2003〕 BSS is a giant platelet disorder, meaning that it is characterized by abnormally large platelets. ==Signs and symptoms== As with other congenital platelet function defects, BSS often presents as a bleeding disorder with symptoms of: *Perioperative and postoperative bleeding *Bleeding gums *Easy bruising *Heavy menstrual periods *Epistaxis (nosebleeds) *Abnormally prolonged bleeding from small injuries 抄文引用元・出典: フリー百科事典『 ウィキペディア（Wikipedia）』 ■ウィキペディアで「Bernard–Soulier syndrome」の詳細全文を読む スポンサード リンク
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