Brosens Lodewijk A A, van Hattem Arnout, Hylind Linda M, Iacobuzio-Donahue Christine, Romans Katharine E, Axilbund Jennifer, Cruz-Correa Marcia, Tersmette Anne C, Offerhaus G Johan A, Giardiello Francis M
Department of Medicine, The Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
Gut. 2007 Jul;56(7):965-7. doi: 10.1136/gut.2006.116913. Epub 2007 Feb 15.
Juvenile polyposis (JP) is an autosomal-dominant syndrome characterised by the development of hamartomatous gastrointestinal polyps and is associated with colorectal cancer. However, the relative and absolute risk of colorectal malignancy in these patients is not known.
The incidence rates of colorectal cancer in patients with JP were compared with that of the general population through person-year analysis with adjustment for demographics.
In patients with JP, the RR (95% CI) of colorectal cancer was 34.0 (14.4 to 65.7). Similar risks were noted in both males (30.0, 9.6 to 68.6) and females (43.7, 8.8 to 125). The cumulative life-time risk for colorectal cancer was 38.7%. The mean (SD) age of diagnosis of colorectal cancer was 43.9 (10.4) years. Other gastrointestinal malignancies were not noted in this cohort.
Patients with JP have a markedly increased RR and absolute risk for colorectal cancer and require vigilant colorectal surveillance starting at young age. A low threshold for recommending surgery with consideration for removal of the entire colorectum seems warranted.
幼年性息肉病（JP）是一种常染色体显性综合征，其特征为错构瘤性胃肠道息肉的发生，并与结直肠癌相关。然而，这些患者发生结直肠恶性肿瘤的相对风险和绝对风险尚不清楚。
通过人年分析并对人口统计学因素进行校正，将JP患者的结直肠癌发病率与普通人群进行比较。
在JP患者中，结直肠癌的相对危险度（RR，95%可信区间）为34.0（14.4至65.7）。男性（30.0，9.6至68.6）和女性（43.7，8.8至125）的风险相似。结直肠癌的累积终生风险为38.7%。结直肠癌诊断的平均（标准差）年龄为43.9（10.4）岁。该队列中未发现其他胃肠道恶性肿瘤。
JP患者患结直肠癌的RR和绝对风险显著增加，需要从年轻时就开始进行严密的结直肠监测。考虑到切除整个结肠直肠，推荐手术的阈值似乎较低是合理的。
