Eastman J R, Nowakowski A R, Triplett D A
Oral Surg Oral Med Oral Pathol. 1983 Sep;56(3):246-51. doi: 10.1016/0030-4220(83)90004-x.
Classic hemophilia or factor VIII deficiency is a recessive, sex-linked bleeding diathesis. The primary clinical problem is hemorrhage, which can be severe and often life threatening, even in the presence of only minor trauma. In the past this inadequate hemostasis has been treated with transfusions of cryoprecipitate, fresh frozen plasma, or commercially prepared factor VIII concentrate. Unfortunately, such treatment carries with it a number of risks, including the development of hepatitis B or hemolytic anemia and the formation of anti-factor VIII antibodies. Because of hemorrhage severity and the risks of conventional treatment, elective surgery in general and oral surgery in particular have often been neglected in patients with hemophilia. This article reviews a drug, 1-desamino-8-d-arginine (DDAVP), heretofore not discussed in the dental literature, and reports on its use in conjunction with epsilon-aminocaproic acid (EACA), a synthetic antifibrinolytic agent, in the surgical dental treatment of a patient with hemophilia A. The results suggest that certain dental surgical procedures can be performed in the presence of subclinical and mild hemophilia without conventional factor VIII replacement therapy with its associated costs and risks.
典型血友病或因子VIII缺乏症是一种隐性的、性连锁的出血素质。主要的临床问题是出血,即使仅有轻微创伤,出血也可能很严重,且常常危及生命。过去,这种止血功能不足的情况通过输注冷沉淀、新鲜冰冻血浆或商业制备的因子VIII浓缩物来治疗。不幸的是,这种治疗伴随着许多风险,包括乙型肝炎的发生、溶血性贫血以及抗因子VIII抗体的形成。由于出血严重程度以及传统治疗的风险,血友病患者通常忽视择期手术,尤其是口腔手术。本文回顾了一种药物,即1-去氨基-8-D-精氨酸血管加压素(DDAVP),此前牙科文献中未对其进行过讨论,并报告了其与合成抗纤溶药物ε-氨基己酸(EACA)联合用于一名甲型血友病患者口腔外科治疗的情况。结果表明,在亚临床和轻度血友病患者中,在不进行伴有相关费用和风险的传统因子VIII替代治疗的情况下,某些口腔外科手术也可以进行。