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遗传性出血性毛细血管扩张症的胃肠道出血管理

Management of Gastrointestinal Bleeding in Rendu-Osler Disease.

作者信息

Tortora Annalisa, Marmo Clelia, Gasbarrini Antonio, Costamagna Guido, Riccioni Maria Elena

机构信息

Department of Gastroenterological, Fondazione Policlinico Gemelli IRCCS, Endocrino-Metabolic, and Nefro-urological Science, Rome, Italy.

出版信息

Rev Recent Clin Trials. 2020;15(4):321-327. doi: 10.2174/1574887115666200603160033.

DOI:10.2174/1574887115666200603160033
PMID:32493201
Abstract

BACKGROUND

Hereditary hemorrhagic telangiectasia (HHT, or Rendu-Osler-Weber disease) is a rare inherited syndrome, characterized by artero-venous malformations (AVMs or telangiectasia) with autosomal dominant transmission. AVMs can occur in any organ of the body but most commonly it occurs in the nose, pulmonary, hepatic and cerebral circulations. In patients with HHT, we report teleangectasia of mucosa of the gastrointestinal tract.

METHODS

Research and online content related to HHT online activity is reviewed, and DOC writing excerpts are used to illustrate key themes.

RESULTS

Patients with HHT have a high rate of complications related to bleeding; of them gastrointestinal bleeding accounts for 10.8%. Several therapies, both medical and endoscopic, were utilized to reduce the need for transfusions and hospitalization.

CONCLUSION

A combination of medical and endoscopic therapy is probably the best option.

摘要

背景

遗传性出血性毛细血管扩张症(HHT,或朗杜-奥斯勒-韦伯病)是一种罕见的遗传性综合征,其特征为常染色体显性遗传的动静脉畸形(AVM或毛细血管扩张)。AVM可发生于身体的任何器官,但最常见于鼻、肺、肝和脑循环系统。在此,我们报告1例HHT患者发生的胃肠道黏膜毛细血管扩张症。

方法

回顾与HHT在线活动相关的研究及在线内容,并采用文献撰写摘录来说明关键主题。

结果

HHT患者发生与出血相关并发症的几率较高;其中,胃肠道出血占10.8%。为减少输血需求和住院率,采用了多种药物和内镜治疗方法。

结论

药物治疗与内镜治疗相结合可能是最佳选择。

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