摘要
目的:探讨Peuti-jeghers(PJS)综合征临床特点及诊治方法。方法:回顾分析皖东地区3家医院1983年1月~2009年12月共收治的32例PJS综合征患者的临床资料。结果:32例患者全部有胃肠息肉及皮肤黏膜皮肤色素斑,22例有家族史(占68.7%)。临床表现:以腹痛、便血为主,可并发肠梗阻。行手术治疗18例,内镜切除7例,保守治疗7例,病理结果示32例均为错构瘤。结论:PJS综合征是常染色体显性遗传性错构瘤综合征易合并肠梗阻、肠套叠、消化道出血等,可采取内镜与手术相结合治疗,本病易癌变,须定期随访。
Objective:To investigate the Peuti-jeghers(PJS) syndrome clinical features, diagnosis and treatment.Methods:Retrospective analysis of east Anhui three hospitals from January 1983 to December 2009 32 cases were treated syndrome in PJS.Results:All 32 patients had gastrointestinal polyps and mucocutaneous pigmented skin, 22 patients with family history (68.7%).Clinical manifestations:abdominal pain, blood in the stool-based, can be complicated by intestinal ob-struction.Surgical treatment of 18 cases, endoscopic resection in 7 cases, conservative treatment in 7 cases, pathological findings:32 patients were hamartoma.Conclusion:PJS is autosomal dominant hamartoma syndrome is easily complicated by intestinal obstruction, intussusception, gastrointestinal bleeding, can take the combination of endoscopic and surgical treatment of carcinoma of the disease to be easily followed up.
出处
《中国当代医药》
2010年第28期32-33,共2页
China Modern Medicine
关键词
黑斑息肉综合征
息肉
诊治
Peuti-jeghers syndrome
Polyps
Diagnosis and treatment