Hrubisková K
Folia Haematol Int Mag Klin Morphol Blutforsch. 1980;107(4):672-82.
On the basis of the indirectly established statement that activated forms of the coagulation factor are also present in PPSB fractions of own production, the activated concentrate of factor IX and of the prothrombin complex were applied in haemophilia-A patients with antibodies against factor VIII. The fraction was administered to 4 patients during 14 bleeding times, mostly during bleedings of joints an soft tissues, twice during haematuria in a dose of 40-200 E-factor IX per kg of body weight and per day. The total dose was mainly administered in a fractionized way at an interval of 8 or 12 hours. This treatment lasted for 2 to 7 days. With regard to haemostasis no fundamental improvement of global blood coagulation tests (which have pathological results in haemophilia-A patients) could be identified in the course of the treatment. However, the increase of factor II, VII, and X in the patient's plasma was striking. Contrary to exceptations, there was a less distinct increase of factor IX activity. Concluding from these findings it may be assumed that the "strengthening" of the "extrinsic system" is decisive for the haemostatic effect in the treatment mentioned.
基于间接确立的观点,即自身制备的PPSB组分中也存在凝血因子的活化形式,将活化的凝血因子IX浓缩物和凝血酶原复合物应用于患有抗凝血因子VIII抗体的血友病A患者。在14次出血期间,该组分被给予4名患者,主要是在关节和软组织出血期间,血尿期间给药两次,剂量为每千克体重每天40 - 200 E-凝血因子IX。总剂量主要以分次方式给药,间隔8或12小时。这种治疗持续2至7天。关于止血,在治疗过程中未发现整体凝血试验(血友病A患者的结果为病理性)有根本性改善。然而,患者血浆中凝血因子II、VII和X的增加是显著的。与预期相反,凝血因子IX活性的增加不太明显。从这些发现可以推断,在上述治疗中,“外源性系统”的“强化”对止血效果起决定性作用。