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目的分析Peutz-Jeghers综合征(PJS)的临床特征及诊断、治疗。方法观察一家系三代PJS共5例,并重点分析在仁济医院接受积极内、外科治疗的2例。结果多发性息肉在内镜下分批行电凝、电切术切除,1例并发肠套叠、肠梗阻转外科手术,2例发生息肉恶变并死亡。结论内镜治疗对PJS疗效满意,出现并发症时应及时外科干预,由PJS所引起的肿瘤的早期发现与治疗则有赖于长期监测。
Objective To analyze the clinical features, diagnosis and treatment of Peutz-Jeghers syndrome (PJS). Methods A total of 5 cases of three generations of PJS in a pedigree were observed. Two cases of positive internal and surgical treatment in Renji Hospital were analyzed. Results Multiple polyps underwent endoscopic electrocoagulation, resection by electrotomy, one case complicated by intussusception and intussusception and two cases with malignant polyps. Conclusions Endoscopic treatment is satisfactory for PJS. When complications occur, surgical intervention should be timely. The early detection and treatment of tumors caused by PJS depend on long-term monitoring.