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休斯-斯托文综合征与复发性肺动脉瘤的急性处理

Hughes-Stovin Syndrome and the Acute Management of Recurrent Pulmonary Aneurysms.

作者信息

Cole Adam, Mandava Anupa

机构信息

Internal Medicine, SUNY Upstate Medical University, Syracuse, USA.

出版信息

Cureus. 2022 Sep 1;14(9):e28672. doi: 10.7759/cureus.28672. eCollection 2022 Sep.

DOI:10.7759/cureus.28672
PMID:36199648
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC9526475/
Abstract

Hughes-Stovin syndrome (HSS) is a rare autoimmune disease that is considered to be a variant of Behçet's disease. It typically presents with multiple venous thrombi, thrombophlebitis, as well as pulmonary and bronchial aneurysms. The disease is typically associated with high morbidity and mortality in those diagnosed. This case discusses a 28-year-old male with HSS complicated by venous cardiac emboli and a mechanical valve on chronic anticoagulation. Despite medical and surgical management, the patient experienced continued progression of his pulmonary aneurysms and excessive bleeding, eventually requiring lung transplantation.

摘要

休斯-斯托文综合征(HSS)是一种罕见的自身免疫性疾病,被认为是白塞病的一种变体。它通常表现为多发性静脉血栓、血栓性静脉炎以及肺和支气管动脉瘤。该疾病在确诊患者中通常具有较高的发病率和死亡率。本病例讨论了一名28岁患有HSS的男性,并发静脉心脏栓塞,且因慢性抗凝而植入了机械瓣膜。尽管进行了药物和手术治疗,患者的肺动脉瘤仍持续进展,且出血过多,最终需要进行肺移植。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ec4e/9526475/2109dbcdced8/cureus-0014-00000028672-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ec4e/9526475/7968806ef377/cureus-0014-00000028672-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ec4e/9526475/909cd44475e7/cureus-0014-00000028672-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ec4e/9526475/2109dbcdced8/cureus-0014-00000028672-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ec4e/9526475/7968806ef377/cureus-0014-00000028672-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ec4e/9526475/909cd44475e7/cureus-0014-00000028672-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ec4e/9526475/2109dbcdced8/cureus-0014-00000028672-i03.jpg

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引用本文的文献

1
Behçet's Disease: A Radiological Review of Vascular and Parenchymal Pulmonary Involvement.白塞病:肺部血管及实质受累的影像学综述
Diagnostics (Basel). 2022 Nov 19;12(11):2868. doi: 10.3390/diagnostics12112868.

本文引用的文献

1
A critical analysis of 57 cases of Hughes-Stovin syndrome (HSS). A report by the HSS International Study Group (HSSISG).对 57 例 Hughes-Stovin 综合征(HSS)的批判性分析。HSS 国际研究组(HSSISG)的报告。
Int J Cardiol. 2021 May 15;331:221-229. doi: 10.1016/j.ijcard.2021.01.056. Epub 2021 Jan 30.
2
Hughes-Stovin syndrome.休斯-斯托文综合征。
Orphanet J Rare Dis. 2011 Apr 13;6:15. doi: 10.1186/1750-1172-6-15.
3
Hughes-Stovin syndrome: a case report and review of the literature.休斯-斯托文综合征:一例病例报告及文献综述
Cases J. 2009 Jan 29;2:98. doi: 10.1186/1757-1626-2-98.
4
Successful cyclophosphamide therapy with complete resolution of pulmonary artery aneurysm in Hughes-Stovin syndrome patient.环磷酰胺治疗成功,休斯-斯托文综合征患者肺动脉瘤完全消退。
Clin Rheumatol. 2008 Nov;27(11):1455-8. doi: 10.1007/s10067-008-0951-8. Epub 2008 Jul 1.