To improve the the diagnosis and treatment of Peutz-Jeghers syndrome. Methods:The clinical data of 9 patients with Peutz-Jeghers syndrome hospitalized in Zhongshan Hospital, Fudan University from 2003 to 2011 was retrospectively analyzed. Results: The age of onset ranged from 1 to 49 years old. Among them, 5 cases (56%) had family histories. Six cases underwent endoscopic mucosal resection of polyps once or repeatedly, and one of them underwent small intestinal resection because of ischemic necrosis of small intestine caused by intussusception. Two cases who had to undergo surgical operation for the huge polyps denied treatment. One case developed polyp carcinoma. Conclusion: Patients with Peutz-Jeghers syndrome are more susceptible for tumors. Endoscopic therapy and regular follow-up are sound ways to reduce the incidence of complications of Peutz-Jeghers syndrome, as well as to reduce the risk of malignant tumor.%目的:提高Peutz-Jeghers综合征(Peutz-Jeghers syndrome,PJS)的诊治水平.方法:通过分析2003年-2011年复旦大学附属中山医院收治的9例PJS患者的临床资料,结合文献复习,探讨PJS的发病机制、临床表现、诊断、治疗及预后.结果:9例患者发病年龄1～49岁,有家族史者5例(56％).6例1次或多次行内镜下胃肠道息肉切除术,术后其症状明显好转,其中1例并发肠套叠导致小肠部分缺血坏死而行小肠部分切除术；2例因息肉巨大而无法在内镜下将其切除,患者拒绝外科手术治疗自动出院；1例就诊时结肠息肉已癌变并全身多发转移,患者放弃治疗.结论:PJS患者有肿瘤易感性,内镜下行胃肠道息肉切除术和定期随访可降低息肉引起的并发症的发生率,并降低患者发生恶性肿瘤的风险.
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