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沙利度胺成功治疗遗传性出血性毛细血管扩张症合并的胃肠道血管发育异常出血

Successful treatment of bleeding gastro-intestinal angiodysplasia in hereditary haemorrhagic telangiectasia with thalidomide.

作者信息

Alam Mohamed Aftab, Sami Sarmad, Babu Sathish

机构信息

Department of Gastroenterology/General Medicine, Scarborough General Hospital, Scarborough, North Yorkshire, UK.

出版信息

BMJ Case Rep. 2011 Nov 8;2011:bcr0820114585. doi: 10.1136/bcr.08.2011.4585.

Abstract

Hereditary haemorrhagic telangiectasia (HHT) is an autosomal dominant disorder characterised by epistaxis, cutaneous telangiectasia and visceral arterio-venous malformations (AVMs). It affects approximately one in 5000 people. Control of sustained and repeated haemorrhages from telangiectasias in the nose and gut in patients who may be transfusion dependent is clinically challenging. After repeated endoscopic coagulations, multiple lesions often recur at other sites of gastro-intestinal tract, where endoscopic therapy or surgical resection is not possible. Hormonal therapy has been employed for more than 50 years but has recently been shown to be ineffective. Thalidomide, with its antiangiogenic mechanism of action, seems to be promising drug as a treatment option where other modalities have been unsuccessful. In this article, the authors discuss a novel treatment of bleeding gastro-intestinal angiodysplasia.

摘要

遗传性出血性毛细血管扩张症(HHT)是一种常染色体显性疾病,其特征为鼻出血、皮肤毛细血管扩张和内脏动静脉畸形(AVM)。该病影响约五千分之一的人群。对于可能依赖输血的患者,控制鼻部和肠道毛细血管扩张引起的持续性反复出血在临床上具有挑战性。经过反复内镜下凝血治疗后,多个病变常于胃肠道其他无法进行内镜治疗或手术切除的部位复发。激素治疗已应用50多年,但最近已证明无效。沙利度胺具有抗血管生成作用机制,在其他治疗方式均未成功的情况下,似乎是一种有前景的治疗选择药物。在本文中,作者讨论了一种治疗出血性胃肠道血管发育异常的新方法。

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