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Gardner’s Syndrome으로 진단된 가족성 대장 용종증 1예 보고

Gardner’s Syndrome A case report

대한대장항문학회지 1997년 13권 2호 p.291 ~ 299
정동원 ( Jung Dong-Won ) - 전남대학교 의과대학 외과학교실

김동의 ( Kim Dong-Yi ) - 전남대학교 의과대학 외과학교실
김형록 ( kim Hyeong-Rok ) - 전남대학교 의과대학 외과학교실
김영진 ( Kim Young-Jin ) - 전남대학교 의과대학 외과학교실

Abstract


Familial adenomatous polyposis(FAP) is a hereditary disorder characterized by the development in adolescence or early adult life of multiple adenomatous polyps throughout the colon and rectum. The risk of malignant transformation is so high as to virtually 100 per cent if the patient lives long enough. Gardner’s syndrome, a variant of familial adenomatous polyposis, is characterized by colorectal adenomas, multiple osteomas and variety of soft tissue lesions, including sebaceous cysts, fibromas, lipomas, and desmoid tumors. The recent study show that this group of diseases are caused by different mutations of the same gene located on the long arm of chromosome 5. All family members at risk of inheriting this gene need frequent surveillance of the colon. This should start between the ages of 8 and 12 years and should be continued at regular intervals. Prompt colectomy is indicated at the time the diagnosis is made. We experienced a case of Gardner’s syndrome, which had typical extracolonic manifestations. Herein, we report this case with a review of the literature.

키워드

Gardner’s Syndrome;Familial Adenomatous Polyposis(FAP)
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