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评估去氨加压素(DDAVP)在血友病和血管性血友病手术及出血发作中的有效性。一项针对43例患者的研究。

Evaluation of the effectiveness of DDAVP in surgery and in bleeding episodes in haemophilia and von Willebrand's disease. A study on 43 patients.

作者信息

Mariana G, Ciavarella N, Mazzucconi M G, Antoncecchi S, Solinas S, Ranieri P, Pettini P, Agrestini F, Mandelli F

出版信息

Clin Lab Haematol. 1984;6(3):229-38. doi: 10.1111/j.1365-2257.1984.tb00548.x.

DOI:10.1111/j.1365-2257.1984.tb00548.x
PMID:6440729
Abstract

1-deamino-8-D-arginine-vasopressin (DDAVP), was used in a wide spectrum of clinical situations employing two different dosages (0.3 and 0.4 microgram/kg b.w.) for the management of 43 patients with factor VIII deficiencies--mild and moderate haemophilia A and von Willebrand's disease (vWD). In most instances, the drug was given in association with antifibrinolytics. Twenty-five dental extractions were carried out with two different protocols: one based upon a single infusion and the other based upon three infusions. Bleeding occurred in three patients regardless of the protocol used. The vasopressin analogue promptly stopped bleeding in 12 'spontaneous' open bleeds (haematuria, epistaxis, menometrorrhagia, gum bleeding) and it appears to be also effective in closed bleeds. DDAVP was used to minimize blood loss during surgical interventions and to avoid haemorrhage in the postoperative period. Nine surgical procedures were carried out in six vWD patients and three haemophiliacs. Bleeding occurred late in the postoperative period on one occasion only. No difference was demonstrated between the two doses of the drug either in terms of clinical benefit or rise of factor VIII coagulant activity. The efficacy of DDAVP and the absence of side-effects make this vasopressin analogue worthy of consideration as a reliable alternative to factor VIII concentrates in a wide variety of clinical situations.

摘要

1-去氨基-8-D-精氨酸加压素(DDAVP),以两种不同剂量(0.3和0.4微克/千克体重)用于广泛的临床情况,以治疗43例VIII因子缺乏症患者——轻度和中度甲型血友病以及血管性血友病(vWD)。在大多数情况下,该药物与抗纤溶药物联合使用。采用两种不同方案进行了25次拔牙:一种基于单次输注,另一种基于三次输注。无论使用何种方案,均有3例患者发生出血。加压素类似物迅速止住了12例“自发性”开放性出血(血尿、鼻出血、月经过多、牙龈出血),并且似乎对闭合性出血也有效。DDAVP用于尽量减少手术干预期间的失血,并避免术后出血。对6例vWD患者和3例血友病患者进行了9次外科手术。仅1例在术后晚期发生出血。就临床益处或VIII因子凝血活性升高而言,两种剂量的药物均未显示出差异。DDAVP的疗效以及无副作用使得这种加压素类似物在多种临床情况下作为VIII因子浓缩物的可靠替代品值得考虑。

相似文献

1
Evaluation of the effectiveness of DDAVP in surgery and in bleeding episodes in haemophilia and von Willebrand's disease. A study on 43 patients.评估去氨加压素(DDAVP)在血友病和血管性血友病手术及出血发作中的有效性。一项针对43例患者的研究。
Clin Lab Haematol. 1984;6(3):229-38. doi: 10.1111/j.1365-2257.1984.tb00548.x.
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1-Deamino-8-D-arginine-vasopressin--an alternative in the management of mild haemophilia A and von Willebrand's disease.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。
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DDAVP in haemophilia and von Willebrand's disease.去氨加压素在血友病和血管性血友病中的应用
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DDAVP: a useful alternative to blood components in moderate hemophilia A and von Willebrand disease.去氨加压素:中度甲型血友病和血管性血友病中血液成分的有效替代物。
J Pediatr. 1983 Feb;102(2):228-33. doi: 10.1016/s0022-3476(83)80526-5.
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Response of factor VIII/von Willebrand factor to DDAVP in healthy subjects and patients with haemophilia A and von Willebrand's disease.健康受试者、甲型血友病患者及血管性血友病患者中因子VIII/血管性血友病因子对去氨加压素的反应。
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Hyper-responsiveness to DDAVP for patients with type I von Willebrand's disease and normal intra-platelet von Willebrand factor.I型血管性血友病且血小板内血管性血友病因子正常的患者对去氨加压素反应过度。
Eur J Haematol. 1988 Feb;40(2):163-7. doi: 10.1111/j.1600-0609.1988.tb00815.x.
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Factor VII and fibrinolytic response to deamino-8-D-argenine vasopressin in normal subjects and dissociate response in some patients with haemophilia and von Willebrand's disease.正常受试者中因子 VII 及对去氨基-8-D-精氨酸加压素的纤溶反应,以及血友病和血管性血友病患者中的分离反应。
Br J Haematol. 1980 Jul;45(3):499-511. doi: 10.1111/j.1365-2141.1980.tb07169.x.
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Multimeric composition of factor VIII/von Willebrand factor following administration of DDAVP: implications for pathophysiology and therapy of von Willebrand's disease subtypes.去氨加压素给药后因子VIII/血管性血友病因子的多聚体组成:对血管性血友病各亚型病理生理学和治疗的意义
Blood. 1982 Jun;59(6):1272-8.
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Response of patients with mild and moderate hemophilia A and von Willebrand's disease to treatment with desmopressin.轻度和中度甲型血友病及血管性血友病患者对去氨加压素治疗的反应。
Ann Intern Med. 1985 Jul;103(1):6-14. doi: 10.7326/0003-4819-103-1-6.

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DDAVP response and its determinants in bleeding disorders: a systematic review and meta-analysis.去氨加压素反应及其在出血性疾病中的决定因素:一项系统评价和荟萃分析。
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Use of desmopressin in the treatment of hemophilia A: towards a golden jubilee.去氨加压素在甲型血友病治疗中的应用:迈向五十周年纪念
Haematologica. 2018 Mar;103(3):379-381. doi: 10.3324/haematol.2018.187567.
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Desmopressin in moderate hemophilia A patients: a treatment worth considering.中重度血友病 A 患者使用去氨加压素治疗:一种值得考虑的治疗方法。
Haematologica. 2018 Mar;103(3):550-557. doi: 10.3324/haematol.2017.180059. Epub 2018 Jan 5.
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LIM kinase/cofilin dysregulation promotes macrothrombocytopenia in severe von Willebrand disease-type 2B.LIM 激酶/丝切蛋白失调促进重型血管性血友病 2B 型的巨血小板减少症。
JCI Insight. 2016 Oct 6;1(16):e88643. doi: 10.1172/jci.insight.88643.
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The use of desmopressin in acquired haemophilia A: a systematic review.去氨加压素在获得性血友病A中的应用:一项系统评价。
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