All::Gastroenterology::Diseases::Peutz-Jeghers syndrome
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What is Peutz-Jeghers syndrome?
hereditary condition characterised by numerous hamartomatous polyps in the gastrointestinal tract (mainly small bowel)
Peutz-Jeghers syndromeWhat is the inheritance pattern of Peutz-Jeghers syndrome?
autosomal dominant
Peutz-Jeghers syndromeWhat are the main clinical features of Peutz-Jeghers syndrome?
- numerous hamartomatous polyps in the gastrointestinal tract (mainly small bowel)
- pigmented lesions on lips, oral mucosa, face, palms and soles
What is the management of Peutz-Jeghers syndrome?
conservative
Peutz-Jeghers syndromeWhat are the main causes of Peutz-Jeghers syndrome?
responsible gene encodes serine threonine kinase LKB1 or STK11
Peutz-Jeghers syndromeWhat are the common complications of Peutz-Jeghers syndrome?
- small bowel obstruction is a common presenting complaint, often due to intussusception
- gastrointestinal bleeding
- increased life time risk of GI cancers