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Tunisie Medicale [La]. 2007; 85 (7): 563-568
en Francés | IMEMR | ID: emr-139300

RESUMEN

Three main polyposis syndromes are transmitted as an autosomal dominant disorder: familial adenomatous polyposis [FAP], juvenile polyposis syndrome [JPS] and Peutz-Jeghers syndrome. Evaluate this management of digestive polyposis. Our study included 20 patients which were collected in the departements of pathology surgery and garstroenterology of MT Maarmouri's Hospital, Nabeul city. We reported 15 cases of adenomatous polyposis with 2 family groups. We identified a family group of JPS with 3 members and 2 cases of Peutz-Jeghers syndrome. We found 11 cases of colonic adenocarcinoma out of the 15 patients affected by adenomatous polyposes. FAP is a generalized disorder involving the entire colorectum segment with numerous extra-colonic manifestations. The risk to develop colonic cancer is 100%. JPS is characterised by the development of numerous gastrointestinal juvenile polyps and occurs usually before 20 years old, the progression to cancer is rarely observed. Peutz-Jeghers syndrome consists in hamartomatous polyps associated to a characteristic mucosal pigmentation. The patients are usually young adults and have an increased incidence of cancer in extradigestive sites

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