Peutz-Jeghers Syndrome Flashcards
1
Q
What sort of inheritance is Peutz-Jeghers syndrome?
A
It is an autosomal dominant inheritance.
2
Q
What are the features of Peutz-Jeghers syndrome?
A
- Hamartomatous polyps in GI tract (mainly small bowel)
- Pigmented lesions on lips, oral mucosa, face, palms and soles
- Intestinal obstruction e.g. intussusception
- GI bleeding.
3
Q
What is the management of Peutz-Jeghers Syndrome?
A
Conservative unless complications develop
4
Q
A
5
Q
A
6
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7
Q
A
8
Q
What % of patients will die from GI tract cancer by the age of 60 years?
A
50%
