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加德纳综合征中的自发性肠系膜纤维瘤病

Spontaneous mesenteric fibromatosis in Gardner's syndrome.

作者信息

Richards R C, Rogers S W, Gardner E J

出版信息

Cancer. 1981 Feb 1;47(3):597-601. doi: 10.1002/1097-0142(19810201)47:3<597::aid-cncr2820470329>3.0.co;2-z.

Abstract

Mesenteric fibromatosis has been reported in frequent association with familial polyposis and with Gardner's syndrome. This vague "benign" process has been characterized as a postsurgical phenomenon with low morbidity. Two cases of spontaneous mesenteric fibromatosis, noted at the time of original laparotomy for colectomy in Gardner's syndrome patients, are reported. No history of abdominal trauma was present, and both have well-documented Gardner's findings. A 32% incidence of desmoid reaction is reported among affected members of the original Gardner's syndrome Kindred 109; five or 55% of these patients had the mesenteric form of the process. The potential fatal course of the mesenteric disease is emphasized.

摘要

肠系膜纤维瘤病常与家族性息肉病和加德纳综合征相关。这种模糊的“良性”病变被认为是一种术后现象,发病率较低。本文报告了两例在加德纳综合征患者初次行结肠切除术时发现的自发性肠系膜纤维瘤病。患者均无腹部外伤史,且均有充分记录的加德纳综合征表现。据报道,在最初的加德纳综合征家族109的受累成员中,硬纤维瘤反应的发生率为32%;其中5例(占55%)患者为肠系膜型病变。文中强调了肠系膜疾病可能的致命病程。

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