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易混淆的非典型肠道白塞病:伴有严重下消化道出血的跳跃性溃疡

Confusing untypical intestinal Behcet's disease: Skip ulcers with severe lower gastrointestinal hemorrhage.

作者信息

Wang Zhen-Kai, Shi Hui, Wang Shao-Dong, Liu Jiong, Zhu Wei-Ming, Yang Miao-Fang, Liu Chan, Lu Heng, Wang Fang-Yu

机构信息

Zhen-Kai Wang, Hui Shi, Shao-Dong Wang, Jiong Liu, Miao-Fang Yang, Chan Liu, Heng Lu, Fang-Yu Wang, Department of Gastroenterology and Hepatology, Jinling Hospital, Nanjing 210002, Jiangsu Province, China.

出版信息

World J Gastrointest Endosc. 2014 Jan 16;6(1):27-31. doi: 10.4253/wjge.v6.i1.27.

DOI:10.4253/wjge.v6.i1.27
PMID:24527178
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC3921443/
Abstract

Behcet's disease (BD) is a rare and life-long disorder characterized by inflammation of blood vessels throughout the body. BD was originally described in 1937 as a syndrome involving oral and genital ulceration in addition to ocular inflammation. Intestinal BD refers to colonic ulcerative lesions documented by objective measures in patients with BD. Many studies have shown that over 40% of BD patients have gastrointestinal complaints. Symptoms include abdominal pain, diarrhea, nausea, anorexia and abdominal distension. Although gastrointestinal symptoms are common, the demonstration of gastrointestinal ulcers is rare. This so-called intestinal BD accounts for approximately 1% of cases. There is no specific test for BD, and the diagnosis is based on clinical criteria. The manifestations of intestinal BD are similar to those of other colitis conditions such as Crohn's disease or intestinal tuberculosis, thus, it is challenging for gastroenterologists to accurately diagnose intestinal BD in patients with ileo-colonic ulcers. However, giant ulcers distributed in the esophagus and ileocecal junction with gastrointestinal hemorrhage are rare in intestinal BD. Here, we present a case of untypical intestinal BD. The patient had recurrent aphthous ulceration of the oral mucosa, and esophageal and ileo-colonic ulceration, but no typical extra-intestinal symptoms. During examination, the patient had massive acute lower gastrointestinal bleeding. The patient underwent ileostomy after an emergency right hemicolectomy and partial ileectomy, and was subsequently diagnosed with incomplete-type intestinal BD by pathology. The literature on the evaluation and management of this condition is reviewed.

摘要

白塞病(BD)是一种罕见的终身性疾病,其特征是全身血管炎症。BD最初于1937年被描述为一种除眼部炎症外还涉及口腔和生殖器溃疡的综合征。肠道白塞病是指白塞病患者经客观检查证实的结肠溃疡性病变。许多研究表明,超过40%的白塞病患者有胃肠道症状。症状包括腹痛、腹泻、恶心、厌食和腹胀。虽然胃肠道症状很常见,但胃肠道溃疡的表现却很少见。这种所谓的肠道白塞病约占病例的1%。白塞病没有特异性检查,诊断基于临床标准。肠道白塞病的表现与其他结肠炎疾病如克罗恩病或肠结核相似,因此,胃肠病学家准确诊断回结肠溃疡患者的肠道白塞病具有挑战性。然而,在肠道白塞病中,分布于食管和回盲部并伴有胃肠道出血的巨大溃疡很少见。在此,我们报告一例非典型肠道白塞病病例。该患者有复发性口腔黏膜阿弗他溃疡、食管和回结肠溃疡,但无典型的肠外症状。检查期间,患者发生大量急性下消化道出血。患者在急诊右半结肠切除术和部分回肠切除术后行回肠造口术,随后经病理诊断为不完全型肠道白塞病。本文对该疾病的评估和管理相关文献进行了综述。

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本文引用的文献

1
Recurrent aortic aneurysm due to Behcet's disease: a case report from China.白塞病所致复发性主动脉瘤:来自中国的一例病例报告
Ann Thorac Cardiovasc Surg. 2013;19(2):173-5. doi: 10.5761/atcs.cr.12.01923. Epub 2012 Aug 20.
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How to deal with Behcet's disease in daily practice.如何在日常实践中处理白塞病。
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A case of Behçet's disease presenting with massive lower gastrointestinal bleeding.1例以大量下消化道出血为表现的白塞病。
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Clinical Studies on Behçet's Syndrome.白塞氏综合征的临床研究
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Behçet's disease: ocular effects and treatment.白塞病:眼部影响与治疗
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Thalidomide has both anti-inflammatory and regulatory effects in Behcet's disease.沙利度胺对白塞病具有抗炎和调节作用。
Clin Rheumatol. 2008 Mar;27(3):373-5. doi: 10.1007/s10067-007-0786-8. Epub 2007 Nov 23.
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Treatment of recurrent perforating intestinal ulcers with thalidomide in Behçet's disease.沙利度胺治疗白塞病复发性肠道穿孔性溃疡
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A reassessment of the International Study Group criteria for the diagnosis (classification) of Behçet's syndrome.白塞病诊断(分类)国际研究组标准的重新评估
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