Review of Inherited Coagulation Disorders.

Tiffany Hoang, Regina A E Dowdy
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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.

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遗传性凝血障碍综述。
大多数侵入性牙科手术都会引起一定程度的出血,最终导致凝血并最终止血。然而,患有遗传性凝血功能障碍的患者可能会表现出长时间出血,有时甚至是过度出血,需要进行多次围手术期干预。Von Willebrand 病是最常见的遗传性凝血疾病,通常表现为易瘀伤、鼻衄或长时间出血。血友病 A(因子 VII)和血友病 B(因子 IX)是因子缺乏症,临床上难以区分,根据严重程度和所需的牙科治疗进行处理。其他凝血病很少见(即常染色体隐性遗传),只有在同卵或复合杂合子中才会显现。在进行侵入性牙科治疗之前,必须提供最新的化验值,并向患者的血液科医生咨询。镇静和全身麻醉有许多注意事项,包括鼻腔器械操作时的鼻衄风险和患者体位不当造成的瘀伤风险。术前可能需要使用去氨加压素或因子替代治疗,一般应有助于正常止血。为确保术后充分止血,还应考虑其他疗法,包括加压敷料、可吸收凝血材料、激光疗法和口腔冲洗。
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