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KMID : 0360120120340050363
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Journal of the Korean Society of Maxillofacial Plastic Reconstructive Surgeons
2012 Volume.34 No. 5 p.363 ~ p.366
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Peutz-Jeghers Syndrome: A Case Report and Review of Literature
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Kim Hyun-Soo
Kim Soung-Min
Choi Jin-Young
Myoung Hoon
Lee Jong-Ho
Lee Suk-Keun
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Abstract
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Peutz-Jeghers syndrome is a rare syndrome with characteristic features of multiple hamartomatous polyps and mucocutaneous pigmentation. This syndrome is an autosomal dominant disease, and has complications related with polyps of the gastrointestinal tract, such as small bowel obstruction, iron deficiency anemia associated with bleeding, and intussusceptions. Many studies have reported about higher cancer risk of patients with this syndrome than those with no syndrome in the gastrointestinal tract, including gastric, duodenal, jejunal and the extragastrointestinal organs, such as gallbladder, breast and reproductive system. There are guidelines for periodic test for early detection and treatment for higher risk organs. We report a case of Peutz-Jeghers syndrome patient in the emphasis of Oral and Maxillofacial surgeon’s role with review of the literature.
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KEYWORD
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Peutz-Jeghers syndrome, Pigmentation, Intestinal polyposis
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