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小肠内镜检查与 Peutz-Jeghers 综合征。

Small bowel endoscopy and Peutz-Jeghers syndrome.

机构信息

Department of Gastroenterology and Hepatology, Erasmus University Medical Centre, Rotterdam, The Netherlands.

出版信息

Best Pract Res Clin Gastroenterol. 2012 Jun;26(3):263-78. doi: 10.1016/j.bpg.2012.03.009.

Abstract

Peutz-Jeghers syndrome (PJS) is a rare, autosomal dominant inherited disease. It is clinically characterized by the development of gastrointestinal hamartomas, mainly located in the small bowel. These hamartomas are prone to complications such as intussusceptions, abdominal complaints and anaemia. Furthermore, patients are at increased risk for developing small bowel cancer. Therefore, regular surveillance of the small bowel is indicated. However, the optimal strategy for surveillance has not been determined yet. This review gives an overview of the different techniques that have been described to examine the small bowel of PJS patients. First, a number of radiologic and endoscopic imaging modalities with diagnostic value are discussed. Secondly, recently developed advanced endoscopy techniques are described that can serve both as a diagnostic and therapeutic tool in the surveillance of the small bowel. Finally, a recommendation is given how to apply these individual techniques for small bowel surveillance in a step-up approach.

摘要

佩-杰综合征(PJS)是一种罕见的常染色体显性遗传性疾病。其临床特征为胃肠道错构瘤的发展,主要位于小肠。这些错构瘤容易发生并发症,如肠套叠、腹部不适和贫血。此外,患者发生小肠癌的风险增加。因此,建议对小肠进行定期监测。然而,尚未确定最佳的监测策略。这篇综述概述了已描述的用于检查 PJS 患者小肠的不同技术。首先,讨论了一些具有诊断价值的放射学和内镜成像方式。其次,描述了最近开发的先进内镜技术,这些技术可作为小肠监测的诊断和治疗工具。最后,给出了如何逐步应用这些技术进行小肠监测的建议。

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