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加德纳综合征的诊断与治疗。一家系报告。

Gardner's syndrome, diagnosis and treatment. Report on a family.

作者信息

Svanes K, Bjerkeseth T, Ellekjaer E, Gilhuus-Moe O

出版信息

Acta Chir Scand. 1979;145(4):267-72.

PMID:494975
Abstract

We have examined 15 members of a family with Gardner's syndrome. In the first and second generations, 4 (possibly 5) of 6 members died of cancer of the colon/rectum. Two of them were known to have plyposis coli. Three members of the third generation had the complete triad of Gardner's syndrome with polyposis coli, soft tissue and hard tissue tumours. Three of 5 children in the fourth generation, aged between 7 and 11 years, probably have polyposis coli, and 2 of them have stigmata of Gardner's syndrome. All members of any family with Gardner's syndrome should be examined regularly at 2--3 year intervals from an age of about 10 years until the diagnosis is established, or if polyposis does not develop, up to the age of 40 years. Proctocolectomy is recommended at the age of 18--20 years or later as soon as the diagnosis is determined.

摘要

我们对一个患有加德纳综合征的家族中的15名成员进行了检查。在第一代和第二代中,6名成员中有4名(可能是5名)死于结肠/直肠癌。其中两人已知患有结肠息肉病。第三代的三名成员患有加德纳综合征的完整三联征,即结肠息肉病、软组织和硬组织肿瘤。第四代的5名儿童中,年龄在7至11岁之间,有3名可能患有结肠息肉病,其中2名有加德纳综合征的体征。任何患有加德纳综合征的家族成员都应从大约10岁开始,每隔2至3年定期进行检查,直到确诊,或者如果没有发生息肉病,则检查至40岁。一旦确诊,建议在18至20岁或更晚进行直肠结肠切除术。

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