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对1979年6月至1991年6月间收治的20例家族性腺瘤性息肉病(FAP)进行临床分析。有家族史者10例,占50%。16例癌变,占80%。术前均经肠镜检查确诊。3例行预防性结、直肠次全切除或全切除,已分别存活6、7、13年;7例行结肠或直肠癌根治加结、直肠次全切除或全切除,3年、5年生存率为85.7%(6/7)与57.2%(4/7);姑息性结肠或直肠癌切除者均于1年内死亡。共有9例结、直肠次全切除或全切除后行回肠贮袋成形术,无术中死亡及严重并发症,术后大便功能恢复较好。结果提示,FAP的大肠腺瘤易癌变,且较早。早期诊断并行预防性结、直肠次全切除或全切除可防治癌变,回肠贮袋成形术可提高病人生活质量
A clinical analysis of 20 cases of familial adenomatous polyposis (FAP) was performed between June 1979 and June 1991. There are 10 cases of family history, accounting for 50%. 16 cases of cancer, accounting for 80%. Preoperative colonoscopy confirmed. 3 routine prophylactic, subresection of the rectum or total resection, have survived for 6, 7 and 13 years, respectively; 7 patients underwent radical resection and resection of colon or rectal cancer, subtotal or total resection, and 3 and 5 years of survival. The rates were 85.7% (6/7) and 57.2% (4/7); palliative colon or rectal cancer resection all died within 1 year. A total of 9 cases with subtotal resection of the rectum or total excision had undergone an ileal pouch reconstructive surgery with no intraoperative death and serious complications. Postoperative stool function recovered well. The results suggest that colorectal adenomas of FAP are prone to canceration and earlier. Early diagnosis of concurrent prophylactic knots, subtotal rectal resection, or total resection can prevent canceration, and ileal pouches can improve patient quality of life