A Peutz – Jeghers syndrome case with iron deficiency anaemia and small bowel intussusseption
Peutz-Jeghers syndrome (PJS) is an autosomal dominant inherited syndrome characterized by mucocutaneous pigmentations, with intestinal and extraintestinal polyps. It is a precancerous syndrome. The polyps can cause anaemia, intestinal obstruction and intussusception. We present a case who first p...
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Format: | Article |
Language: | English |
Published: |
Malaysian Medical Association
2005
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Online Access: | http://irep.iium.edu.my/52435/ http://irep.iium.edu.my/52435/ http://irep.iium.edu.my/52435/1/52435.pdf |
Summary: | Peutz-Jeghers syndrome (PJS) is an autosomal dominant inherited syndrome characterized by
mucocutaneous pigmentations, with intestinal and extraintestinal polyps. It is a precancerous syndrome.
The polyps can cause anaemia, intestinal obstruction and intussusception. We present a case who first
presented to us with anaemia and history of intussusseption two years ago. Subsequent Small Bowel
Enteroscopy revealed multiple polyps located in the jejunum which was removed by snare polypectomy.
She also had multiple pigmentations on the lips. Histopathological examination of the polyps showed
hamartomatous change consistent with this syndrome. Finally, we present some surveillance
recommendations for people with PJS and for those at risk for PJS. This case also illustrated the
advantage of using Double Balloon Enteroscopy Method which allowed us to examine to the ileum for
polyps surveillance. |
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