Clinics in Colon and Rectal Surgery · 2008 · 47 citations · 18 references
Children with polyps usually present with bleeding or pain. Most pediatric intestinal polyps are sporadic and are not associated with malignancy. Polyposis syndromes are also well described in children. Peutz-Jeghers syndrome is the most common hamartomatous polyposis condition. Although the polyps are not thought to be premalignant in most patients, there is an increased risk of other cancers. Familial adenomatous polyposis is also seen in childhood and is associated with a very high risk of malignant transformation as well as extracolonic adenomas and malignancy. The diagnosis and management of sporadic juvenile polyps, Peutz-Jeghers syndrome, and familial adenomatous polyposis, as well as rarer conditions associated with intestinal polyps are reviewed in this article.
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Peutz-Jeghers syndrome is caused by mutations in a novel serine threoninekinase
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Increased Risk of Cancer in the Peutz–Jeghers Syndrome
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Xiaoping Zhou, K Woodford-Richens, Rainer Lehtonen et al. · The American Journal of Human Genetics · 2001 · 257 citations · Full text