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KMID : 0356720040200040231
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Journal of the Korean Society of Coloproctology
2004 Volume.20 No. 4 p.231 ~ p.235
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Peutz-Jeghers Syndrome with Intussusception and Anemia
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Hwang Jeong-Kye
Kang Won-Kyung
Lee Sang-Chul
Lee Yoon-Suk
Park Seung-Man
Rha Sung-Eun
Oh Seung-Tack
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Abstract
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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. J Korean Soc Coloproctol 2004;20:231-235
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KEYWORD
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Peutz-Jeghers syndrome, Anemia, Intussusception, Hamartoma
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