Causes of death and postsurgical survival in familial adenomatous polyposis: results from the Italian Registry. Italian Registry of Familial Polyposis Writing Committee.
Causes of death and postsurgical survival in familial adenomatous polyposis: results from the Italian Registry. Italian Registry of Familial Polyposis Writing Committee.
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家族性腺瘤性息肉病的死亡原因和术后生存：来自意大利登记处的结果。
DOI：
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发表时间：
1994
期刊：
影响因子：
--
通讯作者：
M. Pietroiusti
中科院分区：
文献类型：
--
作者：
L. Bertario;S. Presciuttini;P. Sala;C. Rossetti;M. Pietroiusti
在意大利息肉病登记处记录的350例家族性腺瘤性息肉病（FAP）死亡患者中评估了死亡原因：78.1%是由于结直肠癌，9.5%是结肠外癌（超过一半的上消化道），3.6%是硬纤维瘤，8.8%是其他原因。研究了604例患者诊断时的年龄与临床症状的存在和手术时结肠癌的存在的关系。在30岁以下的无症状患者中，结肠癌的风险为3.3%，在40岁以上的有症状患者中，结肠癌的风险为80%。一项生命表分析显示，结肠切除术时未患癌症的患者30年后的术后生存率为68%，而癌症患者10年后的生存率为41%。在术后生存率方面，比较了全结肠切除加回肠直肠吻合术与全直肠结肠切除术（伊拉vs. IAA）的替代预防性治疗。两种治疗均显示10年后的存活率为83%。伊拉术后15年和25年直肠残端癌症的风险分别为14.5%和25.2%;相应的死亡风险分别为4.3%和9.3%。
Causes of death were evaluated among 350 deceased patients with familial adenomatous polyposis (FAP) recorded in the Italian Polyposis Registry: 78.1% were due to colorectal cancer, 9.5% to extracolonic cancer (more than half of the upper gastrointestinal tract), 3.6% to desmoid tumors, and 8.8% to other causes. The age at diagnosis among 604 patients was studied in relation to presence of symptoms at presentation and presence of colonic cancer at surgery. In asymptomatic patients younger than 30 years the risk of colonic cancer was 3.3% and in symptomatic patients older than 40 it was 80%. A life-table analysis showed that postsurgical survival among patients without cancer at colectomy was 68% after 30 years, whereas that of patients with cancer was 41% after 10 years. The alternative prophylactic treatments of total colectomy with ileorectal anastomosis versus total proctocolectomy (IRA vs. IAA) were compared in terms of postsurgical survival. Both treatments showed a survival of 83% after 10 years. The risk of cancer in the rectal stump after IRA was 14.5% after 15 years and 25.2% after 25 years; the corresponding risks of dying from it were 4.3% and 9.3%, respectively.
DOI：
10.1002/ajmg.1320280217
发表时间：
1987-10
期刊：
American journal of medical genetics
影响因子：
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作者：
Sarah Klemmer;Leigh Pascoe;J. Decosse;J. M. Opitz;J. Reynolds
通讯作者：
Sarah Klemmer;Leigh Pascoe;J. Decosse;J. M. Opitz;J. Reynolds
影响因子：
10.3
作者：
DECOSSE, JJ;MILLER, HH;LESSER, ML
通讯作者：
LESSER, ML
影响因子：
29.4
作者：
PETERSEN, GM;SLACK, J;NAKAMURA, Y
通讯作者：
NAKAMURA, Y