J Korean Soc Coloproctol.  2004 Aug;20(4):231-235.

Peutz-Jeghers Syndrome with Intussusception and Anemia

• Hwang JK
• Kang WK
• Lee SC
• Lee YS
• Park SM
• Rha SE
• Oh ST

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.

Keyword

Peutz-Jeghers syndrome; Anemia; Intussusception; Hamartoma

MeSH Terms

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