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題 名 | Peutz-Jeghers Syndrome--25 Years Experience=皮捷氏症候群(Peutz-Jeghers Syndrome)--二十五年之經驗 |
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作 者 | 謝榮鴻; 陳維熊; 姜正愷; 林資琛; 林楨國; 楊靜芬; | 書刊名 | 中華民國大腸直腸外科醫學會雜誌 |
卷 期 | 8:2 1997.06[民86.06] |
頁 次 | 頁35-40 |
分類號 | 416.245 |
關鍵詞 | 皮捷氏症候群; Hamartomatous polyp; Neoplasm; Peutz-jeghers syndrome; |
語 文 | 英文(English) |
中文摘要 | 皮捷氏症候群(Peutz-Jeghers Syndrome)是一種顯性遺傳疾病,並具有胃腸道息 肉症及皮膚黏膜色素沉積的特質。由於皮捷氏息肉惡性變化可能性和胃腸道非胃腸道惡性腫 瘤之相關性並不是十分了解,故本文之主要目的就是要研究本病和多種腫瘤之相關性、探討 其治療的方法及強調追蹤治療的重要性。從 1972 年到 1996 年,共有十二位病人在本院被 診斷及接受治療;其臨床症狀、治療及預後都經詳細地分析。腹痛是最常見的症狀。小腸攝 影、大腸鋇劑權腸攝影、上消化道內視鏡檢、及大腸鏡檢都是有效的診斷工具。在這十二位 病人當中,有兩位死於惡性腫瘤;一位死於息肉的惡性病變,另一位則死於非胃腸道的癌症 。共有八位病人接受手術,其中有四位復發。皮捷氏症候群有惡性變化的危險,並和其他腫 瘤的發病有相關。定期的追蹤檢查是必要的,同時臨床醫師也必須保持高度的警覺,以便早 期診斷其他腫瘤,早期治療。 arefully. The mean follow-up period was 93.3 months. Abdominal pain was the most common presenting symptom. Small intestine follw-through examination, barium enema, upper endoscopy, and colonoscopy were effective diagnostic methods. Two of the 12 patients (16.7%) died of malignancy either directly associated with this disease or not directly related to it. One patient died of the disease with malignant change of polyps and intraabdominal carcinomatosis. One patients died of nongastrointestinal malignancies. PJS has an increased risk of malignant potential and association with multiple neoplasms. The postoperative recurrence rate is high in patients with PJS. The patients with PJS usually acquire and die of cancer at a relatively young age. We suggest combined surgical-endoscopical approach for symtpomatic patients and those with polyps greater than 1.5 cm. Rectum-sparing colectomy may be considered for those with advanced cancer or multiple polyposis in the colon to decrease the recurrence rate in the colon and to facilitate subsequent colonoscopic follow-up. For asymptomatic patients, biennial small intestine follow-through exmination and enteroscopy are necessary. The physician should keep alert to early detect the development of multiple neoplastic lesions in patients with PJS. |
本系統中英文摘要資訊取自各篇刊載內容。