Scharrer I
Behring Inst Mitt. 1986 Feb(79):12-23.
The von Willebrand syndrome is probably the most frequent heritable bleeding disorder. The pathophysiological key-role plays the impaired platelet adhesion at sites of vascular injury. It is caused by the quantitative or qualitative abnormal von Willebrand factor. Experimental work of previous years led to a better understanding of the structure and function of the factor VIII-von Willebrand factor complex. This knowledge was important for a new classification of the von Willebrand disease.
血管性血友病可能是最常见的遗传性出血性疾病。其病理生理的关键作用在于血管损伤部位血小板黏附功能受损。这是由血管性血友病因子的数量或质量异常所引起的。过去几年的实验工作使人们对因子VIII - 血管性血友病因子复合物的结构和功能有了更好的理解。这一知识对于血管性血友病的新分类很重要。
