KMID : 0882420110810050630
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Korean Journal of Medicine 2011 Volume.81 No. 5 p.630 ~ p.635
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A Case of Peutz-Jeghers Syndrome with Nasal Polyposis
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Lim Sung-Ryoun
Lee Kyoung-Rok Jeong Hye-Kyong Kim Hyung-Il Cho Sung-Bum Lee Wan-Sik Joo Young-Eun
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Abstract
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Peutz-Jeghers syndrome (PJS) is a rare autosomal dominant disease characterized by typical mucocutaneous pigmentation and multiple hamartomatous polyps in the gastrointestinal tract. The clinical manifestations of PJS include polyp-induced bowel obstruction, abdominal pain, and gastrointestinal bleeding. The risk of gastrointestinal and extragastrointestinal cancers is also significantly increased in patients with PJS. Nasal polyposis associated with PJS has been described, and is considered an extragastrointestinal manifestation of PJS. We present an unusual case of a patient with PJS and nasal polyposis causing complete unilateral nasal obstructio.
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KEYWORD
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Peutz-Jeghers syndrome, Nasal polyps
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