Background
Peutz-Jeghers syndrome (PJS) is characterized by multiple hamartomatous polyps of the gastrointestinal tract and mucocutaneous pigmentation occurring around the body orifices [1–2]. This autosomal dominant disease is caused by a germline mutation in the STK11/LKB1 gene and carries an increased risk of several types of cancer, such as gastrointestinal tumors, pancreatic cancer, genital tract malignancies and, especially, breast cancer [1–2]. Approximately 50% of patients develop intussusception by the age of 15, because of jejunal and/or ileal polyps [3]. Previously, this complication was usually treated by surgery. Recently, video-capsule-endoscopy (VCE) and enteroscopy have changed both the diagnostic and the therapeutic management of small bowel hamartomas [4–8].
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Multiple Choice Questionnaire
-
1)
Peutz-Jeghers syndrome (PJS) is
-
a.
characterized by multiple hamartomatous polyps of the gastrointestinal tract and mucocutaneous pigmentation occurring around the body orifices
-
b.
associated with an increased risk of cancer
-
c.
an autosomal dominant disease
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d.
all of the above
-
e.
none of the above
-
a.
-
2)
The most common cancer(s) associated with PJS is/are
-
a.
gastrointestinal tumors
-
b.
pancreatic cancer
-
c.
genital tract malignancies
-
d.
lung cancer
-
e.
breast cancer
-
a.
-
3)
Gastrointestinal polyps may cause
-
a.
gastrointestinal bleeding and anemia
-
b.
abdominal pain due to intussusception
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c.
gastrointestinal obstruction or infarction
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d.
all of the above
-
e.
none of the above
-
a.
-
4)
Which of these statements is false?
-
a.
VCE detects polyps less than 10 mm in diameter
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b.
VCE reduces radiation exposure and is well accepted by patients
-
c.
balloon enteroscopy allows standard snare polypectomy of small bowel polyps
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d.
therapeutic balloon enteroscopy has reduced the need for surgical resection of small bowel polyps
-
e.
VCE and balloon enteroscopy has not changed the current management of patients with PJS
-
a.
1.d — 2.e — 3.d — 4.e
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Rossi, G.B., Di Nardo, G., de Bellis, M., Oliva, S., Di Girolamo, E. (2013). Videocapsule Endoscopy and Therapeutic Enteroscopy for the Management of Small Bowel Polyps in a Patient with Peutz-Jeghers Syndrome. In: Trecca, A. (eds) Atlas of Ileoscopy. Springer, Milano. https://doi.org/10.1007/978-88-470-5205-5_21
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DOI: https://doi.org/10.1007/978-88-470-5205-5_21
Publisher Name: Springer, Milano
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