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Two Cases of Peutz-Jeghers Syndrome.

Journal of the Korean Pediatric Society 1987;30(8):907-915.
Published online August 31, 1987.
Two Cases of Peutz-Jeghers Syndrome.
Joo Saeng Moon, Sung Ki Jin, Doo Sung Moon, Kyung Sook Cho, Jong Dase Cho
Department of Pediatrics, Maryknoll Hospital, Pusan, Korea
Peutz-Jeghers 증후군 2례
문주생, 진성기, 문두성, 조경숙, 조종대
부산메리놀병원 소아과
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
Key Words: Peutz-Jeghers syndrome, Sporadic cases.


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