Price D A, d'Souza S, Ekert H
Aust N Z J Med. 1977 Jun;7(3):286-90. doi: 10.1111/j.1445-5994.1977.tb03688.x.
Three children with haemophilia and antibodies to factor VIII were treated with a non-activated prothrombin concentrate (Prothrombinex) for 12 bleeding episodes. There was clear clinical response and joint aspirations were performed after infusions of phothrombinex in a dose of 30--50 factor IX units/kg body weight and there was no clinical evidence of thrombosis or febrile reactions. There was a significant shortening of the activated partial thromboplastin time (PTT) at one and four hours after the initial infusion with a return to pre-infusion levels 9--24 hours after infusion. The shortening in the PTT was less marked in subsequent infusions. There were no changes in the level of factor VIII procoagulant activity, factor VIII related antigen or factor VIII antibodies after the infusion. In two patients platelet function studies were unaltered by the infusion and in one patient procoagulant levels of factor II, IX and X were no greater than expected from the infusion. We conclude that infusions of non-activated prothrombin concentrates are clinically effective in the treatment of children with haemophilia and factor VIII antibodies but the mechanism of action is unknown.
三名患有血友病且体内有抗凝血因子 VIII 抗体的儿童因 12 次出血发作接受了非活化凝血酶原复合物（凝血酶原复合物）治疗。临床反应明显，在以 30 - 50 单位/千克体重的剂量输注凝血酶原复合物后进行了关节穿刺，且无血栓形成或发热反应的临床证据。首次输注后 1 小时和 4 小时活化部分凝血活酶时间（PTT）显著缩短，输注后 9 - 24 小时恢复至输注前水平。后续输注时 PTT 的缩短不太明显。输注后凝血因子 VIII 促凝活性、凝血因子 VIII 相关抗原或凝血因子 VIII 抗体水平无变化。两名患者的血小板功能研究未因输注而改变，一名患者的凝血因子 II、IX 和 X 的促凝水平不高于输注预期值。我们得出结论，非活化凝血酶原复合物输注在治疗患有血友病和凝血因子 VIII 抗体的儿童中具有临床疗效，但作用机制尚不清楚。
