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孤立性胃静脉曲张与抗磷脂综合征和蛋白 S 缺乏相关:病例报告及文献复习。

Isolated gastric varices associated with antiphospholipid syndrome and protein S deficiency: a case report and review of the literature.

机构信息

Department of Gastroenterology, Union Hospital of Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei Province, China.

出版信息

J Int Med Res. 2024 Apr;52(4):3000605241240579. doi: 10.1177/03000605241240579.

DOI:10.1177/03000605241240579
PMID:38603605
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11010754/
Abstract

The mortality rate of gastric varices bleeding can reach 20% within 6 weeks. Isolated gastric varices (IGVs) refer to gastric varices without esophageal varices and typically arise as a common complication of left portal hypertension. Although IGVs commonly form in the setting of splenic vein occlusion, the combination of antiphospholipid syndrome and protein S deficiency leading to splenic vein occlusion is rare. We herein present a case of a 28-year-old woman with intermittent epigastric pain and melena. She was diagnosed with antiphospholipid syndrome based on the triad of pregnancy morbidity, unexplained venous occlusion, and positive lupus anticoagulant. Laparoscopic splenectomy and pericardial devascularization were performed for the treatment of IGVs. During the 6-month postoperative follow-up, repeated endoscopy and contrast-enhanced computed tomography revealed disappearance of the IGVs. This is the first description of splenic vein occlusion associated with both antiphospholipid syndrome and protein S deficiency. We also provide a review of the etiology, clinical manifestations, diagnosis, and treatment methods of IGVs.

摘要

胃静脉曲张出血的死亡率在 6 周内可达 20%。孤立性胃静脉曲张(IGVs)是指无食管静脉曲张的胃静脉曲张,通常作为左门静脉高压的常见并发症出现。尽管 IGVs 通常在脾静脉闭塞的情况下形成,但抗磷脂综合征和蛋白 S 缺乏导致脾静脉闭塞的情况很少见。本文报告了一例 28 岁女性间歇性上腹痛和黑便的病例。根据妊娠发病率、不明原因静脉闭塞和狼疮抗凝剂阳性三联征,她被诊断为抗磷脂综合征。为治疗 IGVs,进行了腹腔镜脾切除术和贲门血管离断术。在术后 6 个月的随访中,重复内镜和增强 CT 显示 IGVs 消失。这是首例描述抗磷脂综合征和蛋白 S 缺乏症同时伴发脾静脉闭塞的病例。我们还对 IGVs 的病因、临床表现、诊断和治疗方法进行了综述。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b3a0/11010754/595814c66b28/10.1177_03000605241240579-fig2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b3a0/11010754/431a69e3e5f4/10.1177_03000605241240579-fig1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b3a0/11010754/595814c66b28/10.1177_03000605241240579-fig2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b3a0/11010754/431a69e3e5f4/10.1177_03000605241240579-fig1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b3a0/11010754/595814c66b28/10.1177_03000605241240579-fig2.jpg

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Epidemiology of Antiphospholipid Syndrome in the General Population.抗磷脂综合征在普通人群中的流行病学。
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Endoscopic ultrasound-guided coil deployment with sclerotherapy for isolated gastric varices: Case series of feasibility, safety, and long-term follow-up.
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