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家族性腺瘤性息肉病和遗传性非息肉病性结直肠癌中的隐匿性不透射线颌骨病变。

Occult radiopaque jaw lesions in familial adenomatous polyposis coli and hereditary nonpolyposis colorectal cancer.

作者信息

Offerhaus G J, Levin L S, Giardiello F M, Krush A J, Welsh S B, Booker S V, Hasler J F, McKusick V A, Yardley J H, Hamilton S R

出版信息

Gastroenterology. 1987 Sep;93(3):490-7. doi: 10.1016/0016-5085(87)90910-3.

Abstract

The purposes of this study were to determine the association, in 10 pedigrees, between adenomatous polyposis coli, hereditary nonpolyposis colorectal cancer, and occult radiopaque jaw lesions, and to assess whether these radiodensities are predictors for adenomatous polyposis. In seven kindreds with adenomatous polyposis, all patients with polyps had jaw lesions; in one kindred, no jaw lesions were found. In one of two kindreds with hereditary nonpolyposis colorectal cancer, no affected individuals had jaw lesions. In the other, the 1 affected patient with dental radiographs had generalized jaw lesions. Twelve children less than 16 yr old at risk for adenomatous polyposis were observed. Seven children with jaw lesions developed polyps after a mean interval of 4 yr. Five children without jaw lesions were polyp-free during a 5-10-yr follow-up. Thus, occult jaw lesions are consistently found only in some families with adenomatous polyposis coli, providing support for heterogeneity in polyposis syndromes. Jaw lesions are good predictors for polyp development in kindreds with adenomatous polyposis coli and jaw lesions. Their role as markers in hereditary nonpolyposis colorectal cancer needs exploration.

摘要

本研究的目的是确定在10个家系中,腺瘤性息肉病(adenomatous polyposis coli)、遗传性非息肉病性结直肠癌(hereditary nonpolyposis colorectal cancer)与隐匿性不透射线颌骨病变之间的关联,并评估这些放射密度是否为腺瘤性息肉病的预测指标。在7个患有腺瘤性息肉病的家系中,所有息肉患者均有颌骨病变;在1个家系中,未发现颌骨病变。在2个患有遗传性非息肉病性结直肠癌的家系中,其中1个家系中没有受累个体有颌骨病变。在另一个家系中,1名接受牙科X光检查的受累患者有广泛性颌骨病变。观察了12名年龄小于16岁且有腺瘤性息肉病风险的儿童。7名有颌骨病变的儿童在平均4年后出现了息肉。5名无颌骨病变的儿童在5至10年的随访期间未出现息肉。因此,隐匿性颌骨病变仅在一些患有腺瘤性息肉病的家系中持续出现,这为息肉病综合征的异质性提供了支持。颌骨病变是腺瘤性息肉病家系中息肉发生的良好预测指标。它们作为遗传性非息肉病性结直肠癌标志物的作用有待探索。

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