Gastrointestinal aspects of Peutz-Jeghers syndrome

There are two main problems in the clinical management of the gastrointestinal (GI) tract in patients with Peutz-Jeghers syndrome (PJS), namely long-term cancer risk and managing polyp related complications (of which the most important clinically is intussusception). Given the rarity of this conditi...

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Published inBaillière's best practice & research. Clinical gastroenterology Vol. 58-59; p. 101789
Main Authors Latchford, A.R., Clark, S.K.
Format Journal Article
LanguageEnglish
Published Kidlington Elsevier Ltd 01.06.2022
Elsevier Limited
Subjects
Age
Bias
Cancer
Endoscopy
Family medical history
Histology
Intestinal obstruction
Intussusception
Kinases
Laparotomy
Meta-analysis
Peutz-Jeghers
Polyp
Polyps
Smooth muscle
Surveillance
United Kingdom > UK
Peutz-Jeghers
Endoscopy
Surveillance
Intussusception
Polyp
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Summary:There are two main problems in the clinical management of the gastrointestinal (GI) tract in patients with Peutz-Jeghers syndrome (PJS), namely long-term cancer risk and managing polyp related complications (of which the most important clinically is intussusception). Given the rarity of this condition, the evidence base upon which to make recommendations is small. Furthermore, controversies persist regarding the relationship between PJ polyps, cancer development and cancer risk. In this article we will explore some of these controversies, to put into context the recommendations for clinical management of these patients. We will provide an overview, particularly focusing on clinical data, and on the recommendations for clinical management and surveillance of the GI tract in PJS. We highlight knowledge gaps which need to be addressed by further research.
Bibliography:ObjectType-Article-2
SourceType-Scholarly Journals-1
ObjectType-Feature-3
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ObjectType-Review-1
ISSN:1521-6918
1532-1916
DOI:10.1016/j.bpg.2022.101789