| Literature DB >> 12728797 |
P Bronzino1, P C Rassu, G Cassinelli, T Stanizzi, M Casaccia.
Abstract
The Authors describe three cases of Familial Adenomatous Polyposis, (FAP), in patients of the same family, mother and two daughters, with different stages of the disease. Familial adenomatous polyposis is a mendelian dominant inherited syndrome with an incidence of 1:11,000, caused by an alteration of APC gene, which causes multiple disorders of the development ecto-, endo- and mesoderma. The syndrome is characterized by the presence of adenomatous polyps in the gastroenteric tract, mostly in colon-rectum and duodenum with demonstrated adenoma-carcinoma sequence. In the family here reported a case of familial adenomatous polyposis at the adenomatous stage and two of cancer of colon-rectum are registered. In the first case surgery had a preventive aim, and ileo-rectal anastomosis was performed; in the other two cases the treatment was Miles operation with radical intention.Entities:
Mesh:
Year: 2003 PMID: 12728797
Source DB: PubMed Journal: G Chir ISSN: 0391-9005