Gastric adenocarcinoma and proximal polyposis of the stomach (GAPPS): A new autosomal dominant syndrome

Worthley, D.L., Phillips, K.D., Wayte, N., Schrader, K.A., Healey, S., Kaurah, P., Shulkes, A., Grimpen, F., Clouston, A., Moore, D., Cullen, D., Ormonde, D., Mounkley, D., Wen, X., Lindor, N., Carneiro, F., Huntsman, D.G., Chenevix-Trench, G. and Suthers, G.K. (2011) Gastric adenocarcinoma and proximal polyposis of the stomach (GAPPS): A new autosomal dominant syndrome. Gut, 61 5: 1-6. doi:10.1136/gutjnl-2011-300348

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Author Worthley, D.L.
Phillips, K.D.
Wayte, N.
Schrader, K.A.
Healey, S.
Kaurah, P.
Shulkes, A.
Grimpen, F.
Clouston, A.
Moore, D.
Cullen, D.
Ormonde, D.
Mounkley, D.
Wen, X.
Lindor, N.
Carneiro, F.
Huntsman, D.G.
Chenevix-Trench, G.
Suthers, G.K.
Title Gastric adenocarcinoma and proximal polyposis of the stomach (GAPPS): A new autosomal dominant syndrome
Journal name Gut   Check publisher's open access policy
ISSN 0017-5749
Publication date 2011-08
Sub-type Article (original research)
DOI 10.1136/gutjnl-2011-300348
Volume 61
Issue 5
Start page 1
End page 6
Total pages 6
Place of publication London, England,U.K.
Publisher B M J Group
Collection year 2012
Language eng
Formatted abstract
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).

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.

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.

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.
Q-Index Code C1
Q-Index Status Confirmed Code
Institutional Status UQ
Additional Notes Published online 3 August 2011.

Document type: Journal Article
Sub-type: Article (original research)
Collections: Official 2012 Collection
School of Medicine Publications
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Citation counts: TR Web of Science Citation Count  Cited 27 times in Thomson Reuters Web of Science Article | Citations
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Created: Thu, 22 Sep 2011, 08:36:51 EST by Matthew Lamb on behalf of School of Medicine