| Gastric adenocarcinoma and proximal polyposis of the stomach (GAPPS): a new autosomal dominant syndrome. | |
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MedLine Citation:
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PMID: 21813476 Owner: NLM Status: Publisher |
Abstract/OtherAbstract:
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Objective The purpose of this study was the clinical and pathological characterisation of a new autosomal dominant gastric polyposis syndrome, gastric adenocarcinoma and proximal polyposis of the stomach (GAPPS). Methods Case series were examined, documenting GAPPS in three families from Australia, the USA and Canada. The affected families were identified through referral to centralised clinical genetics centres. Results The report identifies the clinical and pathological features of this syndrome, including the predominant dysplastic fundic gland polyp histology, the exclusive involvement of the gastric body and fundus, the apparent inverse association with current Helicobacter pylori infection and the autosomal dominant mode of inheritance. Conclusions GAPPS is a unique gastric polyposis syndrome with a significant risk of gastric adenocarcinoma. It is characterised by the autosomal dominant transmission of fundic gland polyposis, including areas of dysplasia or intestinal-type gastric adenocarcinoma, restricted to the proximal stomach, and with no evidence of colorectal or duodenal polyposis or other heritable gastrointestinal cancer syndromes. |
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Authors:
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D L Worthley; K D Phillips; N Wayte; K A Schrader; S Healey; P Kaurah; A Shulkes; F Grimpen; A Clouston; D Moore; D Cullen; D Ormonde; D Mounkley; X Wen; N Lindor; F Carneiro; D G Huntsman; G Chenevix-Trench; G K Suthers |
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Publication Detail:
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Type: JOURNAL ARTICLE Date: 2011-8-3 |
Journal Detail:
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Title: Gut Volume: - ISSN: 1468-3288 ISO Abbreviation: - Publication Date: 2011 Aug |
Date Detail:
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Created Date: 2011-8-4 Completed Date: - Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 2985108R Medline TA: Gut Country: - |
Other Details:
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Languages: ENG Pagination: - Citation Subset: - |
Affiliation:
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Columbia University, New York, New York, USA. |
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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