中国小肠镜诊治Peutz‑Jeghers综合征的专家共识意见(2022年)
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1.空军军医大学特色医学中心消化内科;2.海军军医大学附属长海医院消化内科;3.上海交通大学医学院附属仁济医院消化科

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Expert consensus on enteroscopic diagnosis and treatment for Peutz‑Jeghers syndrome in China (2022)
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    摘要:

    黑斑-息肉综合征(Peutz‑Jeghers syndrome,PJS)是一种常染色体显性遗传疾病,以皮肤黏膜色素沉着、胃肠道多发错构瘤性息肉和肿瘤易感性为主要特征。小肠是PJS息肉好发部位之一,易伴发肠套叠、肠梗阻、消化道出血、癌变等并发症。随着小肠内镜技术的迅速发展,小肠镜已经成为PJS诊治的关键技术,在小肠息肉诊断及治疗中发挥重要作用。近年来,随着对PJS临床实践和认识的不断深入,我国消化内镜医师对PJS诊治有了较为丰富的经验积累。为进一步促进小肠镜技术在该领域的推广应用,有必要制定中国小肠镜诊治PJS的专家共识意见。本共识基于循证依据及专家经验对PJS的流行病学、遗传特征、临床表现、诊断标准、内镜治疗、外科手术、息肉监测及随访等方面进行梳理,以便于指导专科医师、内镜医师加强对PJS患者全生命周期的规范诊治和管理,从而更好地发挥小肠镜在PJS患者小肠息肉诊治中的作用。

    Abstract:

    Peutz‑Jeghers syndrome (PJS) is an autosomal dominant genetic disease, characterized by pigmentation of skin and mucosa, multiple hamartoma polyps of gastrointestinal tract and susceptibility to tumor. The small intestine is one of the predilection sites of polyps in PJS, which is usually complicated with intussusception, intestinal obstruction, bleeding or cancerization. With the rapid development of endoscopic technology, enteroscopy has become the key technology for the diagnosis and treatment of PJS. It plays an important role in the diagnosis and treatment of small bowel polys. In recent years, Chinese gastroenterologists have accumulated rich experience in endoscopic diagnosis and treatment of PJS with the advancement of clinical practice and acknowledgement of PJS. And it is necessary to launch a consensus on the diagnosis and treatment of PJS by enteroscopy so as to promote the application of enteroscopic technology in this field. This consensus included the epidemiology, genetic characteristics, clinical manifestations, diagnostic standard, endoscopic treatment, surgery, monitoring and follow-up of PJS based on evidence and experience of experts. This consensus may better guide the specialists and endoscopists to strengthen the standard diagnosis, treatment and management of PJS patients of full life circle in order to play a better role in the enteroscopic diagnosis and treatment for small bowel polyps in PJS patients.

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宁守斌,杜奕奇,戈之铮.中国小肠镜诊治Peutz‑Jeghers综合征的专家共识意见(2022年)[J].中华消化内镜杂志,2022,39(7):505-515.

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  • 收稿日期:2022-02-24
  • 最后修改日期:2022-05-23
  • 录用日期:2022-03-09
  • 在线发布日期: 2022-05-24
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