Occurrence of desmoids in patients with familial adenomatous polyposis of the colon.
Occurrence of desmoids in patients with familial adenomatous polyposis of the colon.
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DOI：
10.1002/ajmg.1320280217
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发表时间：
1987-10
期刊：
影响因子：
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通讯作者：
Sarah Klemmer;Leigh Pascoe;J. Decosse;J. M. Opitz;J. Reynolds
中科院分区：
文献类型：
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作者：
Sarah Klemmer;Leigh Pascoe;J. Decosse;J. M. Opitz;J. Reynolds
与家族性腺瘤性息肉病相关的硬纤维瘤的发生率比与任何其他疾病相关的硬纤维瘤的发生率高得多。然而，公布的风险估计有很大的差异。在这项研究中，我们检查了息肉病患者人群中硬纤维瘤的发生率，以确定硬纤维瘤疾病风险的准确估计。我们人群中硬纤维瘤的粗频率约为6%，但风险取决于风险个体的年龄和性别。一生中发生硬纤维瘤的风险男性为8%，女性为13%。硬纤维瘤的发生率随年龄的分布在每种性别中呈单峰分布，而在非息肉病患者中观察到的硬纤维瘤的发生率呈多峰分布。女性比男性更容易受到影响，而且年龄更小。我们还检测到息肉病基因分离的家族之间的风险异质性，硬纤维瘤病倾向于聚集在其中一些家族中。因此，应谨慎使用粗略的风险估计。
Desmoid tumors occur at a much higher frequency in association with familial adenomatous polyposis than with any other disease. However, published estimates of risk have varied widely. In this study we have examined the occurrence of desmoids in a population of polyposis patients in order to determine an accurate estimate of the risk of desmoid disease. The crude frequency of desmoids in our population was about 6%, but the risk was dependent on the age and sex of the individuals at risk. The lifetime risk of incurring a desmoid was 8% for males and 13% for females. The distribution of occurrence of desmoid disease with regard to age was unimodal in each sex in contrast to the multimodal distribution observed for desmoids in nonpolyposis patients. Females were more likely to be affected than males and at an earlier age. We also detected heterogeneity of risk between families segregating for the polyposis gene, with the desmoid disease tending to cluster in some of those families. Consequently crude estimates of risk should be used with caution.