Stine K C, Becton D L
Department of Pediatrics, University of Arkansas for Medical Sciences, Little Rock 72202, USA.
J Pediatr Hematol Oncol. 1997 Mar-Apr;19(2):156-8. doi: 10.1097/00043426-199703000-00012.
Patients with Ehlers-Danlos syndrome (EDS), especially types IV, VI, and VIII, are at increased risk of bleeding, and most do not have specific hemostatic deficiencies that would be amenable to replacement therapy. We have investigated the ability of DDAVP (desmopressin acetate) to control bleeding in EDS.
Two children with EDS, types VIII and VI, presented with hemorrhagic symptoms and scheduled surgical procedures. Ivy bleeding times (BTs) were measured before and after intravenous (i.v.) DDAVP challenge, and i.v. DDAVP was used prophylactically for their procedures. Laboratory testing was performed to rule out other hemostatic disorders.
Both patients had prolonged BTs that corrected following i.v. DDAVP therapy; all other laboratory values were normal. Both patients had excellent clinical hemostasis with surgery, and one has continued to use intranasal DDAVP to control epistaxis and gingival bleeding.
The bleeding time in both patients was corrected with DDAVP, and the patients did not have any postoperative bleeding. DDAVP should be considered in other patients who have EDS with bleeding tendencies.
患有埃勒斯-当洛综合征（EDS）的患者，尤其是IV型、VI型和VIII型患者，出血风险增加，且大多数患者没有适合替代疗法的特定止血缺陷。我们研究了去氨加压素（醋酸去氨加压素）控制EDS患者出血的能力。
两名分别患有VIII型和VI型EDS的儿童出现出血症状并计划进行外科手术。在静脉注射去氨加压素激发前后测量伊维出血时间（BTs），并在手术中预防性使用静脉注射去氨加压素。进行实验室检测以排除其他止血障碍。
两名患者的BTs均延长，但在静脉注射去氨加压素治疗后恢复正常；所有其他实验室值均正常。两名患者手术时临床止血效果良好，其中一名患者继续使用鼻内去氨加压素控制鼻出血和牙龈出血。
两名患者的出血时间均通过去氨加压素得到纠正，且患者术后均未出现出血情况。对于其他有出血倾向的EDS患者，应考虑使用去氨加压素。
