Mannucci P M, Ruggeri Z M, Pareti F I, Capitanio A
Lancet. 1977 Apr 23;1(8017):869-72. doi: 10.1016/s0140-6736(77)91197-7.
1-Deamino-8-d-arginine vasopressin (D.D.A.V.P.) infusion causes a marked increase in factor-VIII (antihaemophilic-factor)-related properties in patients with moderate and mild haemophilia and von Willebrand's disease (vWd). The possibility was therefore evaluated that such an autologous factor-VII response might be haemostatically effective, allowing patients to undergo surgery without plasma concentrates. 0.3 microng/kg of D.D.A.V.P. given before dental surgery and repeated in the early postoperative period was followed by a two to three fold rise in factor-VIII coagulant activity (VII C.A.) in four patients with moderate and mild haemophilia. In two, there was no abnormal bleeding after dental extraction, whereas plasma concentrates were necessary to control oozing from the sockets in the remaining two patients. A higher D.D.A.V.P. dosage (0.4-0.5 microng/kg) in patients with higherstarting VII C.A. (9% or more) was followed by a more marked response (four to six fold). VII C.A. levels up to 100% of average normal were achieved and dental extraction and major surgery (such as cholecystectomy, thoracotomy, and two tonsillectomies) were carried out successfullly in six patients with mild haemophilis and in two with vWd. The mean half-life of autologous VII C.A. was 9.4 h (range 7.5-11.6). Plasma and urine osmolality showed no consistent variation after drug administration. Thus D.D.A.V.P. appears a promision pharmacological alternative to plasma concentrates in the management of some patients with haemophilis and vWd.
1-去氨基-8-D-精氨酸加压素(DDAVP)输注可使中度和轻度血友病及血管性血友病(vWd)患者的因子VIII(抗血友病因子)相关特性显著增加。因此,研究了这种自体因子VIII反应在止血方面可能有效的可能性,使患者无需血浆浓缩物即可接受手术。在4例中度和轻度血友病患者的牙科手术前给予0.3μg/kg的DDAVP,并在术后早期重复给药,随后因子VIII凝血活性(VIII C.A.)升高了2至3倍。其中2例患者拔牙后无异常出血,而其余2例患者则需要血浆浓缩物来控制牙槽窝渗血。起始VIII C.A.较高(9%或更高)的患者给予更高剂量的DDAVP(0.4 - 0.5μg/kg)后,反应更为显著(4至6倍)。6例轻度血友病患者和2例vWd患者的VIII C.A.水平达到平均正常水平的100%,并成功进行了拔牙和大手术(如胆囊切除术、开胸手术和2例扁桃体切除术)。自体VIII C.A.的平均半衰期为9.4小时(范围7.5 - 11.6小时)。给药后血浆和尿渗透压没有一致的变化。因此,在一些血友病和vWd患者的治疗中,DDAVP似乎是血浆浓缩物的一种有前景的药理学替代物。