Köhler M, Hellstern P, Reiter B, von Blohn G, Wenzel E
Klin Wochenschr. 1984 Jun 1;62(11):543-8. doi: 10.1007/BF01727749.
Twenty-one patients suffering from mild von Willebrand's disease (vWd) and patients suffering from mild or moderate hemophilia A received 1-desamino-8-D-arginine vasopressin (DDAVP) (Minirin, Ferring AG) s.c. at a dose of 0.4 microgram/kg body weight. Additionally, two hemophiliacs and 22 patients with vWd received DDAVP i.v. Within the observation period of 3 h Factor (F) VIII:C levels increased 2.4 X baseline levels in hemophiliacs, and the maximal effect was observed 3 h post DDAVP s.c. In patients with vWd post DDAVP s.c. (i.v.) a 2.7 (3.4), 2.1 (1.9) and 2.2 (2.8) fold increase for F VIII: C, F VIIIR:Ag and F VIII:Rcof was observed. In eight patients suffering from vWd with additional F XII deficiency a small and transitory but significant increase of F XII levels was detected post DDAVP s.c. No local or systemic side effects were observed. In five patients with vWd tooth extractions were performed without bleeding complications under DDAVP s.c. treatment. Two patients practiced self-treatment by injecting the drug s.c. at home. We thus conclude that s.c. DDAVP is an effective, reliable, and cost-reducing form of treatment that does not bring with it the risk of transmitting infectious diseases in patients with vWd and hemophilia and that can be administered at home.
21例患有轻度血管性血友病(vWd)的患者以及患有轻度或中度甲型血友病的患者接受了剂量为0.4微克/千克体重的1-去氨基-8-D-精氨酸加压素(DDAVP,商品名Minirin,辉凌制药公司生产)皮下注射。此外,2例血友病患者和22例vWd患者接受了DDAVP静脉注射。在3小时的观察期内,血友病患者的因子(F)VIII:C水平升高至基线水平的2.4倍,皮下注射DDAVP后3小时观察到最大效应。皮下注射(静脉注射)DDAVP后,vWd患者的F VIII:C、F VIIIR:Ag和F VIII:Rcof分别升高了2.7(3.4)倍、2.1(1.9)倍和2.2(2.8)倍。在8例合并F XII缺乏的vWd患者中,皮下注射DDAVP后检测到F XII水平有轻微、短暂但显著的升高。未观察到局部或全身副作用。5例vWd患者在皮下注射DDAVP治疗期间拔牙,未出现出血并发症。2例患者在家自行皮下注射该药物进行自我治疗。因此,我们得出结论,皮下注射DDAVP是一种有效、可靠且成本降低的治疗方式,不会给vWd和血友病患者带来传染病传播风险,且可在家中给药。