The Korean Journal of Medicine

  • 제81권5호
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  • Pages.630-635
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  • 2011
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  • 1738-9364(pISSN)
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  • 2289-0769(eISSN)
대한내과학회 (The Korean Association of Internal Medicine)
권호 표지

비용종증을 동반한 Peutz-Jeghers 증후군 1예

A Case of Peutz-Jeghers Syndrome with Nasal Polyposis

  • 임성륜 (전남대학교 의과대학 내과학교실) ;
  • 이경록 (전남대학교 의과대학 내과학교실) ;
  • 정혜경 (전남대학교 의과대학 내과학교실) ;
  • 김형일 (전남대학교 의과대학 내과학교실) ;
  • 조성범 (전남대학교 의과대학 내과학교실) ;
  • 이완식 (전남대학교 의과대학 내과학교실) ;
  • 주영은 (전남대학교 의과대학 내과학교실)
  • Lim, Sung-Ryoun (Department of Internal Medicine, Chonnam National University Medical School) ;
  • Lee, Kyoung-Rok (Department of Internal Medicine, Chonnam National University Medical School) ;
  • Jeong, Hye-Kyong (Department of Internal Medicine, Chonnam National University Medical School) ;
  • Kim, Hyung-Il (Department of Internal Medicine, Chonnam National University Medical School) ;
  • Cho, Sung-Bum (Department of Internal Medicine, Chonnam National University Medical School) ;
  • Lee, Wan-Sik (Department of Internal Medicine, Chonnam National University Medical School) ;
  • Joo, Young-Eun (Department of Internal Medicine, Chonnam National University Medical School)
  • 발행 : 2011.11.01
⟨ 이전 논문 다음 논문 ⟩

초록

PJS는 위장관의 다발성 과오종성 용종과 피부 점막의 색소 침착을 특징으로 하는 상염색체 우성 유전 질환으로 120,000명당 1명 정도로 발생하는 드문 질환이다. 위장관 용종으로 인한 복통, 장폐색, 장출혈과 빈혈 등이 주된 임상 소견으로 나타나며, 위장관과 다른 장기에서의 악성 종양 빈도가 높은 것으로 보고되고 있다. 1921년 Peutz가 PJS에서 처음으로 비용종증 동반을 보고한 이후, PJS 관련 비용종증에 대한 외국 문헌 보고들이 있어 왔다. 이는 비용종증이 PJS의 하나의 장외 증상임을 시사한다. 그러나 현재까지 PJS 관련 비용종증에 대한 국내 문헌 보고는 없는 실정이다. 이에 저자들은 일측 비강의 완전 폐쇄를 주증상으로 내원한 PJS 환자에서 비용종증을 진단하고, 수술적 치료를 시행한 1예를 경험하였기에 문헌고찰과 함께 보고하는 바이다.

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.

키워드

참고문헌

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