All issues > Volume 30(8); 1987

Original Article

J Korean Pediatr Soc. 1987;30(8):907-915. Published online August 31, 1987.

Two Cases of Peutz-Jeghers Syndrome.

Joo Saeng Moon¹, Sung Ki Jin¹, Doo Sung Moon¹, Kyung Sook Cho¹, Jong Dase Cho¹

¹Department of Pediatrics, Maryknoll Hospital, Pusan, Korea

Abstract

Peutz-Jeghers syndrome is characterized by hamartomtous polyps of the gastrointestinal tract (stomach, small bowel, and colon) and mucocutaneous pigmentation (lips, oral mucosa, fingers, forearms, toes and umbilical area). About 50% of the reported cases have a positive family history of autosomal dominant mode of inheritacnce, whereas the remaining 50% are sporadic. We have experienced two case of Peutz-Jeghers syndrome with no definite family history. One was asymptomatic and the other had intussusception of colocolic type. This prompted a review of the literature on the subject

Keywords :Peutz-Jeghers syndrome; Sporadic cases.