血管性假血友病综合征,是一组常染色体显性遗传性疾病。它的特征是以出血时间延长,血浆第Ⅷ因子降低/血小板对玻璃柱的粘附性降低以及输鲜血或血浆后第Ⅷ因子过度反应性增高。在国外本病是较常见的遗传性出血疾病之一,近来国内报告也有所增多,值得引起我国临床工作者的重视。现将我院建院以来曾见到的一例报告如下: 患儿董某(住院号19312)女性,14岁,山东人汉族1979年5月2日因月经初潮期流血不止入院诊治。患儿自出生后经常间隔2～20天鼻衄一次,但出血量不多,伴有全身皮肤散在出血点及瘀斑,每次 Von Willebrand syndrome, is a group of autosomal dominant genetic diseases. It is characterized by prolonged bleeding, reduced plasma factor â ... ¡/ platelet adhesion to the glass column decreased and the infusion of blood or plasma â ... § factor hyperresponsiveness increased. In foreign countries the disease is one of the more common hereditary bleeding disease, the recent domestic reports have also increased, it is worthy of our attention of clinical workers. Now I have seen the hospital since the hospital has seen a case of the report is as follows: Children Dong (hospital number 19312) Female, 14 years old, Shandong Han Chinese May 2, 1979 because of menstrual flow more than bloodshed admission treatment. Children from birth often 2 to 20 days after the epistaxis once, but less bleeding, accompanied by systemic bleeding scattered spots and ecchymosis, each time