脾动脉瘤破裂后发现血管性埃勒-丹洛斯综合征1例。

Q4 Medicine
Takuya Mizugami, Hiroyuki Yuasa, Takao Omori, Yu Nobuoka, Makoto Shimomura
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引用次数: 0

摘要

我们报告一例血管性埃勒-丹洛斯综合征在一个30岁的男性患者。他于XXXX年7月3日下午5点左右因突发心外膜疼痛就诊于当地医生。同日因腹部CT疑似大面积腹腔内出血转至我院就诊。在紧急转运过程中,患者腹部平坦、柔软、心包区有压痛感,血液生化检查显示血红蛋白下降10.2g/dL。他住进了我们医院,并开始了保守治疗。五年前,他在另一家医院因乙状结肠特发性穿孔接受了哈特曼手术。出院1个月后,因结肠造口口侧结肠穿孔,再次在我院急诊行结肠切除术及结肠造口重建手术。他报告了结肠造口术口侧结肠穿孔的病史。6个月后,他做了结肠造口手术,被诊断出患有绝症。根据其母亲侧的特发性结直肠穿孔家族史和破裂的动脉瘤,我们怀疑是血管型埃勒斯-丹洛斯综合征。入院后,他的心包疼痛症状有所改善,但第16天的CT扫描显示肝和脾动脉瘤肿大。7月24日,他被转移到当地医院接受脾动脉瘤治疗,并于第二天进行了盘绕手术。术后病程良好,于7月26日转回我院。病情持续好转,于8月1日出院。出院后到门诊遗传诊所进一步检查。然而,同年10月9日,他患上了主动脉夹层,去世了。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
[Vascular Ehlers-Danlos syndrome discovered after splenic aneurysm rupture: a case report].

We report a case of vascular Ehlers-Danlos syndrome in a 30-year-old male patient. He presented to his local doctor with sudden onset of epicardial pain at around 5:00 p.m. on July 3, XXXX. On the same day, he was transferred to our hospital for extensive intra-abdominal hemorrhage suspected on abdominal computed tomography (CT). During emergency transport, his abdomen was flat, soft, and tender in the pericardial area, and blood biochemistry tests revealed a decreased hemoglobin of 10.2g/dL. He was admitted to our hospital, and conservative treatment was initiated. Five years ago, he underwent Hartmann procedure at another hospital for idiopathic perforation of the sigmoid colon. A month after discharge from the hospital, he had to undergo emergency surgery again at our hospital for colon resection and colostomy reconstruction due to colon perforation at the mouth side of the colostomy. He reported a history of colorectal perforation on the mouth side of the colostomy. After 6 months, a colostomy was performed and he was diagnosed with a terminal illness. Based on a family history of idiopathic colorectal perforation on his mother's side and the ruptured aneurysm, vascular-type Ehlers-Danlos syndrome was suspected. After admission, his symptoms of pericardial pain improved, but a CT scan on the 16th day revealed enlarged hepatic and splenic aneurysms. On July 24, he was transferred to a local hospital for splenic artery aneurysm treatment and underwent coiling the next day. His postoperative course was good, and he was transferred back to our hospital on July 26. He continued to show good progress and was discharged from the hospital on August 1. After discharge, he visited the outpatient genetic clinic for further examination. However, on October 9 of the same year, he developed aortic dissection and passed away.

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来源期刊
Japanese Journal of Gastroenterology
Japanese Journal of Gastroenterology Medicine-Gastroenterology
CiteScore
0.20
自引率
0.00%
发文量
0
期刊介绍: The Journal of Gastroenterology, which is the official publication of the Japanese Society of Gastroenterology, publishes Original Articles (Alimentary Tract/Liver, Pancreas, and Biliary Tract), Review Articles, Letters to the Editors and other articles on all aspects of the field of gastroenterology. Significant contributions relating to basic research, theory, and practice are welcomed. These publications are designed to disseminate knowledge in this field to a worldwide audience, and accordingly, its editorial board has an international membership.
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