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Clinical characteristics of Peutz-Jeghers syndrome in Korean polyposis patients

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Affiliated Author(s)
박의준배옥석
Alternative Author(s)
Park, Ui JunBae, Ok Suk
Journal Title
International Journal of Colorectal Disease
ISSN
0179-1958
Issued Date
2000
Abstract
Peutz-Jeghers syndrome is
an autosomal dominant inherited disorder
characterized by hamartomatous
polyps in the small bowel and mucocutaneous
pigmentation. Patients with
Peutz-Jeghers syndrome often present
as surgical emergencies with complications
of the polyps, such as intussusception,
bowel obstruction, and
bleeding. Recently an increased risk
of malignancies has also been reported.
This study was initiated to determine
the clinical features of Peutz-
Jeghers syndrome in Korean patients,
with special attention to the development
of malignancies. Thirty patients
with Peutz-Jeghers syndrome were investigated;
their median age was
23.5 years, and symptoms appeared at
a median age of 12.5 years. Family
history was positive in one-half of
cases, and mucocutaneous pigmentation
was observed in almost all patients
(93%). The jejunoileum was the
most frequent site of the polyps, and
there were generally 10–100 polyps.
Multiple laparotomies were performed
in a substantial portion of the patients,
due mainly to polyp-induced bowel
obstruction, and the surgical interventions
were begun at a relatively young
age (average 21.4 years). Four cases
of small-bowel cancer and one case of
breast cancer were detected in probands,
at a relatively young age (mean
36 years). Cancers of the small bowel,
stomach, colon, breast and cervix
were diagnosed in the first relatives of
the probands. Close follow-up from
an early age should thus be performed
in patients with Peutz-Jeghers syndrome
as they are at high risk of surgical
emergency and development of
malignancy.
Keywords Peutz-Jeghers syndrome ·
Hamartomatous polyps ·
Intussusception · Cancer
Department
Dept. of Surgery (외과학)
Publisher
School of Medicine
Citation
H.S. Choi et al. (2000). Clinical characteristics of Peutz-Jeghers
syndrome in Korean polyposis patients. International Journal of Colorectal Disease, 15(1), 35–38.
Type
Article
ISSN
0179-1958
URI
https://kumel.medlib.dsmc.or.kr/handle/2015.oak/35960
Appears in Collections:
1. School of Medicine (의과대학) > Dept. of Surgery (외과학)
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