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肠系膜缺血与蛋白S缺乏症:一例罕见病例报告

Mesenteric ischemia and protein S deficiency: a rare case report.

作者信息

Llovera Ingrid, Roit Zhanna, Kiriaki Shadi, Sama Andrew, Loscalzo John, Goyal Nikhil

机构信息

Department of Emergency Medicine and Radiology, North Shore University Hospital, Manhasset, New York 11030, USA.

出版信息

J Emerg Med. 2010 Nov;39(5):579-82. doi: 10.1016/j.jemermed.2007.05.056. Epub 2008 Jan 4.

Abstract

We present an unusual case of a 31-year-old nulliparous woman who was in her normal state of health until 3 weeks before her Emergency Department visit, when she began to have generalized abdominal pain that got acutely worse over a few days. She had a soft abdomen, but complained of excruciating pain. Her computed tomography (CT) scan revealed thrombosis in the superior mesenteric, splenic, and portal veins. Her hematological work-up detected a protein S deficiency, which is associated with recurrent venous thrombosis. The finding of mesenteric venous thrombosis associated with protein S deficiency is rare. The most important factor in survival is early diagnosis and prompt treatment with anticoagulants. Properly treated, patients with mesenteric venous thrombosis should have a good long-term prognosis. Past medical or family history of thrombosis in combination with abdominal symptoms should increase the suspicion for the disease.

摘要

我们介绍了一例不同寻常的病例,患者为一名31岁未生育女性,在就诊于急诊科前3周一直健康,之后开始出现全腹疼痛,并在数天内急剧加重。她腹部柔软,但主诉疼痛难忍。她的计算机断层扫描(CT)显示肠系膜上静脉、脾静脉和门静脉血栓形成。血液学检查发现蛋白S缺乏,这与复发性静脉血栓形成有关。与蛋白S缺乏相关的肠系膜静脉血栓形成的发现很罕见。生存的最重要因素是早期诊断和及时使用抗凝剂治疗。经过适当治疗,肠系膜静脉血栓形成患者应具有良好的长期预后。有血栓形成的既往病史或家族史并伴有腹部症状时,应增加对该病的怀疑。

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