一家系三代Peutz-Jeghers综合征临床分析并文献复习  

Clinical Analysis and Review of Literature of Peutz-Jeghers in 3 Generations of A Family

作　　者：许今[1] 王坚[1] 劳立峰[1] 马辰凯[1] 马源[1] 陈涛[1] 

机构地区：[1]上海交通大学医学院附属仁济医院普外科,200127

出　　处：《中国误诊学杂志》2010年第24期5806-5809,共4页Chinese Journal of Misdiagnostics

摘　　要：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 review the characteristics,diagnosis and treatment of Peutz-Jeghers syndrome（PJS）.Method Focusing on the 2 cases who received medical and surgical treatment in Renji hospital from 3 generations of a family in which 5 cases of PJS occured.Result Polyposis was expected to be electric coagulated or electric removed in groups.Intussusception,volvulus and intestinalobstructionileus occured in 1 case and looked for surgical treatmetnt while 2 patients died of malignant change in polyposis.Conclusion Endoscopic treatment is effective to PJS while in-time surgical intervention is needed when complication happened.Long-term supervision is a must for early detection and treatment of tumor induced by PJS.

关 键 词：色素沉着过多 腺瘤息肉病 结肠 综合征 病例报告 人类 

分 类 号：R574.62[医药卫生—消化系统]