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蛋白S缺乏症所致的大面积急性肺栓塞——病例报告

Massive acute pulmonary embolism in protein S deficiency--a case report.

作者信息

Kam R M, Tan A T, Chee T S, Wong J

机构信息

Department of Cardiology, Singapore General Hospital.

出版信息

Ann Acad Med Singap. 1994 May;23(3):396-9.

PMID:7944258
Abstract

A young man with a history of deep vein thrombosis and pulmonary embolism 11 years ago presented again with acute pulmonary embolism and was treated initially with intravenous heparin at our institution. Five days later he had another massive bout of pulmonary embolism causing hypotension. Pulmonary angiography confirmed the presence of thrombi in both pulmonary arteries, with complete obstruction of the left pulmonary artery. He was treated successfully by emergency pulmonary embolectomy. Blood investigations later confirmed the diagnosis of protein S deficiency and he was started on warfarin therapy for life. Massive pulmonary embolism should be treated aggressively. Thrombolytic therapy accelerates clot lysis, reduces pulmonary pressures, restores pulmonary capillary volume and reverses right heart failure faster than heparin alone. There is also a trend towards decreased mortality with thrombolysis. In the presence of shock, the patient should be resuscitated and if facilities for emergency embolectomy are available, surgery is a viable alternative to thrombolysis, especially if the clot burden is massive. In young patients with recurrent venous thromboembolism in the absence of obvious predisposing factors, it is important to exclude inherited plasma protein deficiencies of protein S, protein C, antithrombin III, plasminogen and fibrinogen.

摘要

一名11年前有深静脉血栓形成和肺栓塞病史的年轻男子再次出现急性肺栓塞,最初在我们机构接受静脉注射肝素治疗。五天后,他又发生了一次严重的肺栓塞,导致低血压。肺血管造影证实双侧肺动脉存在血栓,左肺动脉完全阻塞。他通过急诊肺动脉血栓切除术成功治愈。血液检查后来确诊为蛋白S缺乏症,他开始终身服用华法林治疗。大面积肺栓塞应积极治疗。溶栓治疗比单独使用肝素能更快地加速血栓溶解、降低肺动脉压力、恢复肺毛细血管容量并逆转右心衰竭。溶栓治疗还有降低死亡率的趋势。在出现休克的情况下,应进行复苏,如果有急诊血栓切除术的设备,手术是溶栓治疗的可行替代方案,特别是在血栓负荷较大的情况下。在没有明显易感因素的复发性静脉血栓栓塞的年轻患者中,排除蛋白S、蛋白C、抗凝血酶III、纤溶酶原和纤维蛋白原的遗传性血浆蛋白缺乏症很重要。

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引用本文的文献

1
Massive pulmonary embolism and thrombophilia.大面积肺栓塞与血栓形成倾向
BMJ Case Rep. 2013 Jan 22;2013:bcr2012008197. doi: 10.1136/bcr-2012-008197.