Quitt M, Froom P, Veisler A, Falber V, Sova J, Aghai E
Hematology Institute, Lady Davis Carmel Hospital, Haifa, Israel.
Arch Intern Med. 1990 Aug;150(8):1744-6.
A patient with hereditary hemorrhagic telangiectasia and reduced levels of von Willebrand factor had severe recurrent gastrointestinal bleeding. Treatment with either desmopressin acetate or cryoprecipitate normalized both bleeding time and kaolin-activated partial thromboplastin time. Hematochezia (five episodes) continued, however, for a week despite daily treatment with cryoprecipitate, whereas treatment with desmopressin at the onset of three later episodes of hematochezia resulted in the prompt cessation of bleeding. After an episode of life-threatening gastrointestinal bleeding, desmopressin administration allowed a total colectomy without excessive bleeding. During the 3 years after colectomy, no further gastrointestinal bleeding occurred. In the past year, however, three episodes of severe epistaxis responded promptly to intravenous infusion of desmopressin. We conclude that desmopressin may be useful in the management of bleeding in patients with hereditary hemorrhagic telangiectasia with or without von Willebrand factor deficiency.
一名患有遗传性出血性毛细血管扩张症且血管性血友病因子水平降低的患者出现严重复发性胃肠道出血。使用醋酸去氨加压素或冷沉淀治疗后，出血时间和高岭土激活的部分凝血活酶时间均恢复正常。然而，尽管每天使用冷沉淀治疗，便血（共5次发作）仍持续了一周，而在随后3次便血发作开始时使用去氨加压素治疗则导致出血迅速停止。在一次危及生命的胃肠道出血发作后，使用去氨加压素使得患者能够进行全结肠切除术且未出现过多出血。在结肠切除术后的3年里，未再发生胃肠道出血。然而，在过去一年中，3次严重鼻出血发作经静脉输注去氨加压素后迅速得到缓解。我们得出结论，去氨加压素在治疗伴有或不伴有血管性血友病因子缺乏的遗传性出血性毛细血管扩张症患者的出血方面可能有效。
