Upper Gastrointestinal Polyps in Gardner's Syndrome

Upper Gastrointestinal Polyps in Gardner's Syndrome
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加德纳综合征中的上胃肠道息肉

DOI:
10.1016/0016-5085(84)90414-1
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发表时间:
1984
期刊:
影响因子:
29.4
通讯作者:
E. Gardner
E. Gardner
中科院分区:
医学1区
文献类型:
--
作者:
R. Burt;M. Berenson;Randall G. Lee;K. Tolman;J. Freston;E. Gardner

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上消化道息肉在加德纳综合征和家族性结肠息肉病患者中并不常见。然而,来自日本的研究人员最近报告说,在患有这些疾病的日本患者中,胃和十二指肠息肉的比例很高。我们对11名最初描述的加德纳综合征家族的受影响成员进行了内镜检查,以确定居住在美国的加德纳综合征患者是否也发生上消化道息肉。其中6例发现胃底和胃体有大量小息肉。6例患者中5例息肉组织学与胃底腺增生一致。其余患者的活检标本仅显示正常粘膜。另一个没有胃底息肉的病人有一个单一的胃窦息肉,是一个腺瘤。8例患者表现为十二指肠小息肉。8例中7例获得活检标本。所有息肉活检均为腺瘤。在11名研究患者中,9名患者通过内窥镜检查了回肠。9例均为回肠息肉,其中6例为腺瘤,3例为淋巴样聚集体。结果表明,上消化道息肉是一种常见的多效性表现的遗传缺陷负责加德纳氏综合征。
Upper gastrointestinal polyps have been considered an uncommon finding in patients with Gardner's syndrome and familial polyposis coli. Investigators from Japan, however, have recently reported finding gastric and duodenal polyps in a high percentage of Japanese patients with these conditions. We endoscopically examined 11 affected members of the originally described Gardner's syndrome kindred to determine if upper gastrointestinal polyps also occurred in patients with Gardner's syndrome living in the United States. Six of the patients were found to have numerous small polyps of the gastric fundus and body. Polyp histology in 5 of the 6 patients was consistent with fundic gland hyperplasia. Biopsy specimens from the remaining patient demonstrated normal mucosa only. Another patient with no fundic polyps had a single antral polyp that was an adenoma. Eight patients exhibited small polyps of the duodenum. Biopsy specimens were obtained in 7 of the 8. All polyps biopsied were adenomas. The lerminal ileum was examined by endoscopy in 9 of the 11 study patients. All 9 had ileal polyps, but the polyps were adenomas in 6 patients and lymphoid aggregates in 3 patients. The results indicate that upper gastrointestinal polyps are a common pleiotropic manifestation of the genetic defect responsible for Gardner's syndrome.
加德纳综合征和壶腹周围恶性肿瘤。
DOI: 10.1002/ajmg.1320060305
发表时间: 1980
期刊: American journal of medical genetics
影响因子: --
作者:
Pauli,RM;Pauli,ME;Hall,JG
通讯作者: Hall,JG