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 |
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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
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Key Words:
Peutz-Jeghers syndrome, Sporadic cases.
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