Management of familial adenomatous polyposis in children and adolescents: Position paper from the espghan polyposis working group

Warren Hyer, Shlomi Cohen, Thomas Attard, Victor Vila-Miravet, Corina Pienar, Marcus Auth, Seth Septer, Jackie Hawkins, Carol Durno, Andrew Latchford

Research output: Contribution to journalArticlepeer-review

Abstract

Familial adenomatous polyposis (FAP) is a well-described inherited syndrome, characterized by the development of hundreds to thousands of adenomas in the colorectum, with implications in children and adolescents. Almost all adult patients will develop colorectal cancer if they are not identified and treated early enough. Identifying and screening for FAP commences in adolescence. The syndrome is inherited as an autosomal dominant trait and caused by mutations in the adenomatous polyposis (APC) gene. This European Society for Paediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN) position paper provides a guide for diagnosis, assessment, and management of FAP in children and adolescents.This is the first position paper regarding FAP published by ESPGHAN. Literature from PubMed, Medline, and Embase was reviewed and in the absence of evidence, recommendations reflect the opinion of paediatric and adult experts involved in the care of polyposis syndromes. Because many of the studies that form the basis for the recommendations were descriptive and/or retrospective in nature, these of the recommendations are supported on expert opinion. This position paper will instruct on the appropriate management and timing of procedures in children and adolescents with FAP.

Original languageEnglish
Pages (from-to)428-441
Number of pages14
JournalJournal of Pediatric Gastroenterology and Nutrition
Volume68
Issue number3
DOIs
StatePublished - 1 Mar 2019
Externally publishedYes

Keywords

  • adolescent
  • child
  • colonoscopy
  • colorectal cancer
  • familial adenomatous polyposis
  • polyposis

Fingerprint

Dive into the research topics of 'Management of familial adenomatous polyposis in children and adolescents: Position paper from the espghan polyposis working group'. Together they form a unique fingerprint.

Cite this