Peutz-Jeghers Syndrome: A Case Report and Review of Literature

Peutz-Jeghers 증후군: 증례보고와 문헌고찰

  • Kim, Hyun Soo (Department of Oral and Maxillofacial Surgery, Seoul National University School of Dentistry) ;
  • Kim, Soung Min (Department of Oral and Maxillofacial Surgery, Seoul National University School of Dentistry) ;
  • Choi, Jin Young (Department of Oral and Maxillofacial Surgery, Seoul National University School of Dentistry) ;
  • Myoung, Hoon (Department of Oral and Maxillofacial Surgery, Seoul National University School of Dentistry) ;
  • Lee, Suk Keun (Department of Oral Pathology, Gangnung-Wonju National University College of Dentistry) ;
  • Lee, Jong Ho (Department of Oral and Maxillofacial Surgery, Seoul National University School of Dentistry)
  • 김현수 (서울대학교 치의학대학원 구강악안면외과학교실) ;
  • 김성민 (서울대학교 치의학대학원 구강악안면외과학교실) ;
  • 최진영 (서울대학교 치의학대학원 구강악안면외과학교실) ;
  • 명훈 (서울대학교 치의학대학원 구강악안면외과학교실) ;
  • 이석근 (강릉원주대학교 치과대학 구강병리학교실) ;
  • 이종호 (서울대학교 치의학대학원 구강악안면외과학교실)
  • Received : 2012.06.12
  • Accepted : 2012.08.27
  • Published : 2012.09.30

Abstract

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.

Keywords

References

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