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A review indicates that individuals with Gardner syndrome may have a 100\u2010 to 200\u2010fold increased risk of developing periampullary carcinoma when compared to the general population. While certain families and certain individuals (those with other duodenal involvement, males and those with all of the characteristics of Gardner syndrome expressed) may be most susceptible, all patients with Gardner syndrome should be periodically endoscopically evaluated for the presence of upper gastrointestinal tract disease.<\/jats:p>","DOI":"10.1002\/ajmg.1320060305","type":"journal-article","created":{"date-parts":[[2005,6,11]],"date-time":"2005-06-11T17:35:55Z","timestamp":1118511355000},"page":"205-219","source":"Crossref","is-referenced-by-count":64,"title":["Gardner syndrome and periampullary malignancy"],"prefix":"10.1002","volume":"6","author":[{"given":"Richard M.","family":"Pauli","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mary E.","family":"Pauli","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Judith G.","family":"Hall","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"John M.","family":"Opitz","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2005,6,2]]},"reference":[{"key":"e_1_2_1_2_2","first-page":"165","article-title":"Two cases of disseminated polypus of the rectum","volume":"33","author":"Cripps WH","year":"1882","journal-title":"Trans Pathol Soc London"},{"key":"e_1_2_1_3_2","first-page":"278","article-title":"Devic, Bussy: Un cas de polypose ad\u00e9nomateuse g\u00e9n\u00e9ralis\u00e9e \u00e0 tout l'intestin","volume":"6","year":"1912","journal-title":"Arch Fr Mal App Dig"},{"key":"e_1_2_1_4_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM193502072120606"},{"key":"e_1_2_1_5_2","first-page":"167","article-title":"A genetic and clinical study of intestinal polyposis, a predisposing factor for carcinoma of the colon and rectum","volume":"3","author":"Gardner EJ","year":"1951","journal-title":"Am J Hum Genet"},{"key":"e_1_2_1_6_2","first-page":"31","article-title":"Hereditary pattern for multiple osteomas in a family group","volume":"4","author":"Gardner EJ","year":"1952","journal-title":"Am J Hum Genet"},{"key":"e_1_2_1_7_2","first-page":"139","article-title":"Multiple cutaneous and subcutaneous lesions occurring simultaneously with hereditary polyposis and osteomatosis","volume":"5","author":"Gardner EJ","year":"1953","journal-title":"Am J Hum Genet"},{"key":"e_1_2_1_8_2","unstructured":"GardnerEJ:Mendelian pattern of dominant inheritance for a syndrome including intestinal polyposis osteomas fibromas and sebaceous cysts in a human family group. 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