Peutz-Jeghers综合征临床综合治疗模式初探(附71例报道)  

Clinical Comprehensive Therapy of Peutz-Jeghers Syndrome(Report of 71 Cases)

作　　者：王石林[1] 顾国利[1] 魏学明[1] 毛高平[2] 宁守斌[2] 

机构地区：[1]空军总医院普通外科,北京100142 [2]空军总医院消化内科,北京100142

出　　处：《中国普外基础与临床杂志》2012年第5期502-506,共5页Chinese Journal of Bases and Clinics In General Surgery

基　　金：空军总医院临床攻关课题(项目编号:KZG2009006)~~

摘　　要：目的探索将临床综合治疗模式应用于Peutz-Jeghers综合征(PJS)的临床治疗效果。方法 2000年1月至2010年12月期间我院收治的71例PJS患者先接受内镜下息肉灼除或圈套切除,对无法镜下切除或出现严重并发症者进行外科解救治疗;在内镜或手术治疗后对患者进行宣教,签署知情同意书者口服塞来昔布6～9个月进行预防性治疗。定期随访,对其治疗结果进行分析。结果 71例(男41例,女30例)中有家族史者29例(40.8%)。62例共行94次手术,肠套叠为其最主要的手术原因(72.3%,68/94)。65例行169次双气囊电子小肠镜下治疗,共摘除或灼除胃肠道息肉1 714枚,最大者直径8 cm;3例发生小肠穿孔。口服塞来昔布者共8例,完成6个月疗程者共3例,息肉的数量减少、大小缩小。结论包括局部治疗(内镜)、解救治疗(手术)、预防治疗(药物干预)的综合治疗模式用于PJS胃肠道息肉的临床治疗是积极、有效的。Objective To explore the clinical comprehensive therapy of Peutz-Jeghers syndrome.Methods From January 2000 to December 2010,71 cases of Peutz-Jeghers syndrome underwent endoscopic polyp resection firstly,and those with unresectable lesions or with severe complications underwent rescue laparotomy.After endoscopic or surgical treatment,the patients took Celecoxib capsules voluntarily for 6 to 9 months under informed consents.All cases were followed up from 6 months to 8 years.Results Twenty-nine patients had familial history of Peutz-Jeghers syndrome among the 71 patients（41 males and 30 females）.Sixty-two cases underwent 94 surgeries and intussusception was the most common cause of laparotomy.Sixty-five patients underwent 169 double-balloon endoscopy（DBE）therapies,and a total of 1 714 polyps were resected by DBE polypectomy.The largest major axis of small-bowel polyp was 8 cm.No severe complications occurred after DBE polypectomy except for 3 cases of intestinal perforation.Eight patients took Celecoxib capsule,3 of them were treated more than 6 months,and DBE examination showed the gastrointestinal polyps reduced in number and size.Conclusion The comprehensive treatment（including of endoscopic therapy,operation,and drug intervention）is a safe and effective clinical model to treat Peutz-Jeghers syndrome.

关 键 词：PEUTZ-JEGHERS综合征 胃肠道肿瘤 双气囊小肠镜 手术 药物干预 

分 类 号：R656[医药卫生—外科学] R735.3[医药卫生—临床医学]