Suppr超能文献

[遗传性出血性毛细血管扩张症的全身器官受累:多学科诊断与介入治疗]

[Universal organ involvement in Rendu-Osler-Weber disease: interdisciplinary diagnosis and interventional therapy].

作者信息

Kirchner J, Zipf A, Dietrich C F, Hohmann A, Heyd R, Berkefeld J

机构信息

Institut für Allgemeine Röntgendiagnostik, Johann Wolfgang Goethe-Universität, Frankfurt am Main.

出版信息

Z Gastroenterol. 1996 Nov;34(11):747-52.

PMID:9045536
Abstract

A case of hereditary hemorrhagic teleangiectasia (HHT, Morbus Rendu-Osler-Weber) in a 63-years-old patient presenting with recurrent gastrointestinal and nasopharyngeal bleeding who required transfusion of 100-200 units of red blood cell concentrate a year is reported. Endoscopically, one source of bleeding could be located in the upper part of the jejunum. Multiple Osler spots were found in the antrum, the bulbal and descending part of the duodenum without signs of bleeding. Colonoscopy revealed no further lesions. Selective mesentericography visualized dilatation of the arterial arcardes in the duodenal region with early venous filling. During coeliacography, parenchymal deposits of contrast medium appeared in the liver. Numerous angiodysplastic arterial alterations from the lower ethmoidal arteria were shown to be another source of bleeding. They were embolized using microspheres and thereby terminating epistaxis. Termination of epistaxis was the crucial prerogative for the consecutive aortal valve replacement. At abdominal ultrasound, the liver harbored multiple anechoic lesions and a bigger cystic formation with portal inflow and early venous distribution. This teleangiectatic portovenous-malformation was confirmed on abdominal computertomography and on T1-enhanced images of MRT, too. This case examplifies the universal character of organ distribution in HHT and underlines the value of complementary diagnostic and therapeutic modalities.

摘要

报告了一例63岁遗传性出血性毛细血管扩张症(HHT,即Rendu-Osler-Weber病)患者,该患者反复出现胃肠道和鼻咽部出血,每年需要输注100 - 200单位红细胞浓缩液。内镜检查发现一处出血源位于空肠上段。在胃窦、十二指肠球部和降部发现多个奥斯勒斑,但无出血迹象。结肠镜检查未发现其他病变。选择性肠系膜血管造影显示十二指肠区域动脉弓扩张,静脉早期充盈。腹腔动脉造影时,肝脏出现造影剂实质沉积。来自筛骨下动脉的大量血管发育异常的动脉改变被证明是另一个出血源。使用微球对其进行栓塞,从而止住鼻出血。止住鼻出血是后续进行主动脉瓣置换的关键前提。腹部超声检查发现肝脏有多个无回声病变以及一个较大的囊性结构,有门静脉血流和早期静脉分布。腹部计算机断层扫描及磁共振成像T1增强图像也证实了这种毛细血管扩张性门静脉畸形。该病例体现了HHT器官分布的普遍性,并强调了辅助诊断和治疗方式的价值。

文献检索

告别复杂PubMed语法,用中文像聊天一样搜索,搜遍4000万医学文献。AI智能推荐,让科研检索更轻松。

立即免费搜索

文件翻译

保留排版,准确专业,支持PDF/Word/PPT等文件格式,支持 12+语言互译。

免费翻译文档

深度研究

AI帮你快速写综述,25分钟生成高质量综述,智能提取关键信息,辅助科研写作。

立即免费体验