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遗传性凝血障碍综述。

Review of Inherited Coagulation Disorders.

出版信息

Anesth Prog. 2024 Jul 8;71(2):87-95. doi: 10.2344/anpr-71-2_continuing_edu.

Abstract

Most invasive dental procedures elicit some degree of bleeding which ultimately leads to clotting and eventual hemostasis. However, patients with inherited coagulation disorders may exhibit prolonged or, in some cases, excessive bleeding requiring multiple perioperative interventions. Von Willebrand disease is the most common inherited coagulopathy and often manifests via easy bruising, epistaxis, or prolonged bleeding. Hemophilia A (factor VII) and B (factor IX) are factor deficiencies that are clinically indistinguishable and managed according to severity and the required dental treatment. Other coagulopathies are rare (ie, inheritance is autosomal recessive) and may only become evident in homozygotes or compound heterozygotes. Current lab values and medical consultation with the patient's hematologist are imperative prior to rendering invasive dental treatment. There are a myriad of sedation and general anesthesia considerations, including risks for epistaxis with nasal instrumentation and bruising with improper patient positioning. Preoperative treatment with desmopressin or factor replacement may be required and generally should facilitate normal hemostasis. Additional therapies should be considered to help ensure adequate postoperative hemostasis, including pressure dressings, resorbable clotting materials, laser therapy, and oral rinses.

摘要

大多数有创性牙科手术都会引起一定程度的出血,最终导致凝血和止血。然而,患有遗传性凝血障碍的患者可能会出现延长或在某些情况下过度出血,需要多次围手术期干预。血管性血友病是最常见的遗传性凝血障碍,常表现为容易瘀伤、鼻出血或出血时间延长。血友病 A(因子 VII)和 B(因子 IX)是因子缺乏症,临床上无法区分,根据严重程度和所需的牙科治疗进行管理。其他凝血障碍很少见(即遗传是常染色体隐性遗传),可能仅在纯合子或复合杂合子中表现出来。在进行有创性牙科治疗之前,必须进行当前的实验室检查值和与患者血液科医生的医疗咨询。有许多镇静和全身麻醉的注意事项,包括鼻器械引起的鼻出血风险和不当患者体位引起的瘀伤风险。可能需要术前使用去氨加压素或因子替代治疗,通常应促进正常止血。应考虑其他治疗方法以帮助确保术后充分止血,包括加压敷料、可吸收的止血材料、激光治疗和口腔冲洗。

相似文献

1
Review of Inherited Coagulation Disorders.遗传性凝血障碍综述。
Anesth Prog. 2024 Jul 8;71(2):87-95. doi: 10.2344/anpr-71-2_continuing_edu.

本文引用的文献

1
Haemophilia.血友病。
Nat Rev Dis Primers. 2021 Jun 24;7(1):45. doi: 10.1038/s41572-021-00278-x.
2
Platelet secretion: From haemostasis to wound healing and beyond.血小板分泌:从止血到伤口愈合及其他方面。
Blood Rev. 2015 May;29(3):153-62. doi: 10.1016/j.blre.2014.10.003. Epub 2014 Oct 31.
3
Von Willebrand disease: an overview.血管性血友病:概述
Indian J Pharm Sci. 2011 Jan;73(1):7-16. doi: 10.4103/0250-474X.89751.

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