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1-去氨基-8-D-精氨酸加压素——治疗轻度甲型血友病和血管性血友病的一种替代药物。

1-Deamino-8-D-arginine-vasopressin--an alternative in the management of mild haemophilia A and von Willebrand's disease.

作者信息

Niessner H, Korninger C

出版信息

Wien Klin Wochenschr. 1983 Nov 11;95(21):753-7.

PMID:6424339
Abstract

Intravenous administration of 0.4 micrograms DDAVP/kg body weight in 16 normal controls, 34 patients with haemophilia A and 30 patients with von Willebrand's disease (vWd) was followed by an increase in FVIII: C from 230 to 410%, in FVIIIR:Ag from 160 to 260% and FVIIIR:RC of from 160 to 320%. Additionally, in the patients with vWd, a shortening of the bleeding time and improvement in platelet retention was observed. In 7 haemophiliacs with pretreatment levels of FVIII: C ranging from between 11 and 43% dental extractions were performed successfully after DDAVP whereas in 2 patients with FVIII: C levels of 5 and 6%, respectively, severe bleeding necessitated administration of factor VIII concentrates. In 8 haemophiliacs (FVIII: C between 6.5 and 50%) and 2 patients with vWd (FVIII: C 18 and 36%, respectively) DDAVP enabled minor surgery and successful therapy of spontaneous or traumatic bleeding complications. However, severe postoperative bleeding after stomach surgery in 2 haemophiliacs (FVIII: C 23 and 40%, respectively) and severe menstrual bleeding in one patient with vWd (FVIII: C 15%) required administration of factor VIII concentrates. At present DDAVP therapy should be restricted to minor surgery and non-life-threatening, spontaneous or traumatic bleeding complications in patients with pretreatment FVIII: C levels higher than 10%.

摘要

对16名正常对照者、34名甲型血友病患者和30名血管性血友病(vWd)患者静脉注射0.4微克去氨加压素/千克体重后,VIII因子:C从230%升至410%,VIII因子相关抗原(FVIIIR:Ag)从160%升至260%,VIII因子瑞斯托霉素辅因子(FVIIIR:RC)从160%升至320%。此外,在血管性血友病患者中,观察到出血时间缩短和血小板滞留改善。在7名VIII因子:C预处理水平在11%至43%之间的血友病患者中,去氨加压素治疗后成功进行了拔牙;而在2名VIII因子:C水平分别为5%和6%的患者中,严重出血需要输注凝血因子VIII浓缩物。在8名血友病患者(VIII因子:C在6.5%至50%之间)和2名血管性血友病患者(VIII因子:C分别为18%和36%)中,去氨加压素使小型手术得以进行,并成功治疗了自发性或创伤性出血并发症。然而,2名血友病患者(VIII因子:C分别为23%和40%)胃手术后严重出血,以及1名血管性血友病患者(VIII因子:C为15%)严重月经过多,需要输注凝血因子VIII浓缩物。目前,去氨加压素治疗应仅限于预处理VIII因子:C水平高于10%的患者进行小型手术以及治疗非危及生命的自发性或创伤性出血并发症。

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