All issues > Volume 28(6); 1985

Case Report

J Korean Pediatr Soc. 1985;28(6):622-626. Published online June 30, 1985.

A Case of Peutz-Jeghers Syndrome.

Sang Kie Kim, Choon Ho Park, Jin Heon Kim, Keun Chul Myung, Chang Soo Ra

¹Department of Pediatrics, Chosun University College of Medicine, Kwang Ju, Korea

Abstract

Peutz-Jeghers syndrome, also known as intestinal polyposis II, is a familial condition characterized by the triad of (1) mucocutaneous pigmentation, (2) benign polyps occuring in any part of the intestinal tract but mainly in the jejunum, and (3) autosomal dominant inheritance. This syndrome was first reported by Peutz in 1929. Following reemphasis by Jegers in 1949, it became a definite clinical disease entity known as Peutz-Jeghers syndrome, whith is a rare disease with about 15 cases reported in Korea. This was a report of our experience with a case of Peutz-Jeghers syndrome, whith was diagnosed in a 10 year and 2 month old boy, who had mucocutaneous pigmentation and mu- ltiple polypos is of gastrointestinal tract, and an uncle of the patient was found with partial manifestation of the syndrome.

Keywords :Peutz-Jeghers syndrome; Intestinal polyps