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去氨加压素在接受侵入性操作的血管性血友病患者中应用的实践情况:一项欧洲调查

Practical aspects of DDAVP use in patients with von Willebrand Disease undergoing invasive procedures: a European survey.

作者信息

Windyga J, Dolan G, Altisent C, Katsarou O, López Fernández M-F, Zülfikar B

机构信息

Department of Disorders of Hemostasis and Internal Medicine, Institute of Hematology and Transfusion Medicine, Warsaw, Poland.

Queen's Medical Centre, University Hospital, Nottingham, UK.

出版信息

Haemophilia. 2016 Jan;22(1):110-20. doi: 10.1111/hae.12763. Epub 2015 Jul 24.

Abstract

INTRODUCTION

Desamino D-arginine vasopressin (DDAVP or desmopressin) is a useful and effective haemostatic treatment for patients with von Willebrand Disease (VWD). However, there are still issues regarding in which subtypes of VWD DDAVP is appropriate and little consensus on its use in different surgical settings. We also lack information concerning the appropriate laboratory parameters that should be monitored.

AIM

The European Haemophilia Therapy Strategy Board (EHTSB) wished to investigate published information and clinical use of DDAVP in VWD patients.

METHODS

We conducted a literature survey on management of VWD during surgical interventions and undertook a survey of specialist haematologist centres across Europe to assess current management of VWD patients.

RESULTS

DDAVP is ineffective in type 3 VWD and its use in type 2B remains controversial due to the possibility of thrombocytopenia. It can, however, be used effectively to cover minor surgery and dental procedures in most other VWD patients. For major surgery there is wider use of factor concentrate in preference to DDAVP depending on the subtype of VWD. We give consensus recommendations on the use of DDAVP for surgical interventions in VWD including laboratory parameters that denote an adequate response and contraindications to its use.

CONCLUSIONS

DDAVP can be recommended to cover invasive procedure in selected patients with VWD, however, we need more information and systematic recording of adverse events associated with DDAVP use in VWD. A companion paper will be published covering the use of factor concentrates in VWD patients.

摘要

引言

去氨基 D - 精氨酸加压素(DDAVP 或去氨加压素)是治疗血管性血友病(VWD)患者的一种有效止血疗法。然而,关于 DDAVP 适用于哪些 VWD 亚型以及在不同手术场景中的使用仍存在问题,并且对于应监测哪些合适的实验室参数也缺乏相关信息。

目的

欧洲血友病治疗策略委员会(EHTSB)希望调查 DDAVP 在 VWD 患者中的已发表信息及临床应用情况。

方法

我们对手术干预期间 VWD 的管理进行了文献调查,并对欧洲各地的血液学专家中心进行了调查,以评估 VWD 患者的当前管理情况。

结果

DDAVP 对 3 型 VWD 无效,由于存在血小板减少的可能性,其在 2B 型中的使用仍存在争议。然而,在大多数其他 VWD 患者中,它可有效用于小型手术和牙科手术。对于大型手术,根据 VWD 的亚型,更广泛地使用凝血因子浓缩物而非 DDAVP。我们针对 VWD 手术干预中 DDAVP 的使用给出了共识性建议,包括表示充分反应的实验室参数及其使用的禁忌证。

结论

对于特定的 VWD 患者,可推荐使用 DDAVP 来应对侵入性操作,然而,我们需要更多关于 VWD 患者使用 DDAVP 相关不良事件的信息及系统记录。一篇关于 VWD 患者使用凝血因子浓缩物的配套论文将发表。

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