Nilsson I M, Bergentz S E, Larsson S A
Ann Surg. 1979 Dec;190(6):746-52. doi: 10.1097/00000658-197912000-00014.
Fifty-eight major surgical procedures were performed in 38 patients with von Willebrand's disease (VWD), one of the most common of the inheritable hemorrhagic disorders. Specific treatment with fraction I-0, (AHF-Kabi) in addition to a fibrinolytic inhibitor, was given to all patients. The effect of the treatment was checked by measuring the Duke bleeding time and factor VIII:C level. A marked difference between hemophilia and VWD from a surgical point of view is demonstrated. While most of the surgery in hemophiliacs is performed for severe joint deformities, contractures and blood cysts, surgery in VWD is mostly general surgery, often necessitated by massive hemorrhages from mucous membranes.
对38例血管性血友病(VWD,最常见的遗传性出血性疾病之一)患者实施了58项大手术。所有患者除接受纤维蛋白溶解抑制剂治疗外,还接受了I-0因子(抗血友病因子-卡比)特异性治疗。通过测量杜克出血时间和因子VIII:C水平来检查治疗效果。从手术角度来看,血友病和血管性血友病存在显著差异。血友病患者的大多数手术是针对严重的关节畸形、挛缩和血囊肿进行的,而血管性血友病患者的手术大多是普通外科手术,通常是由黏膜大量出血引起的。