Peutz-Jeghers Syndrome with Intussusception and Anemia.
- Author:
Jeong Kye HWANG
1
;
Won Kyung KANG
;
Sang Chul LEE
;
Yoon Suk LEE
;
Seung Man PARK
;
Sung Eun RHA
;
Seong Taek OH
Author Information
1. Departments of Surgery, College of Medicine, The Catholic University of Korea, Seoul, Korea. stoh@catholic.ac.kr
- Publication Type:Case Report
- Keywords:
Peutz-Jeghers syndrome;
Anemia;
Intussusception;
Hamartoma
- MeSH:
Abdominal Pain;
Anemia*;
Arm;
Breast;
Chromosomes, Human, Pair 19;
Female;
Gastrointestinal Tract;
Hamartoma;
Hemorrhage;
Humans;
Intussusception*;
Pancreas;
Peutz-Jeghers Syndrome*;
Pigmentation;
Testis;
Wills;
Young Adult
- From:Journal of the Korean Society of Coloproctology
2004;20(4):231-235
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
Peutz-Jeghers syndrome is a disease entity consisting of gastrointestinal hamartoma, mucocutaneous pigmentation, and autosomal dominant inheritance. From a surgical standpoint, the syndrome is of significance because the hamartoma may lead to intussusception, gastrointestinal bleeding, abdominal pain, and a fifteen-times-increased risk of malignancy in the GI tract, pancreas, breast, ovarles, and testes. Recent reports indicate the STK11 (LKB1) gene, located on region 13.3 of the short arm of chromosome 19, as being the locus of autosomal dominant indentify. The case of a 21-year-old female who required a partial jejunal resection due to intussusception and bleeding from a jejunal hamartoma twice in five years is presented.