Article
An unusually severe phenotype for familial adenomatous polyposis.
Archives of disease in childhood - 1 Nov 1997
Eccles D M, Lunt P W, Wallis Y, Griffiths M, Sandhu B, McKay S, Morton D, Shea-Simonds J, Macdonald F
Abstract excerpt
Familial adenomatous polyposis (FAP) is a dominantly inherited predisposition to the development of many hundreds to thousands of adenomatous polyps of the colon. The mean age of onset is around 15 years, symptoms may arise in the third decade, and the median age for the development of colonic cancer is 35-40 years. Prophylactic colectomy reduces the risk of death from colorectal cancer to such an extent that...
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