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Ménétrier’s Disease and Its Atypical Presentation in Four Siblings

Ménétrier’s disease is a rare, acquired, premalignant disorder of the stomach distinguished by hypertrophic giant mucosal folds in the proximal part of the stomach (body and fundus), diminished acid secretion and excessive mucous production, resulting in hypoalbuminemia. Menetrier’s is also known as...

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Autores principales: Hassan, Imran H, Soliman, Mina, Shirazi-Nejad, Ahmad R
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Cureus 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9611642/
https://www.ncbi.nlm.nih.gov/pubmed/36320788
http://dx.doi.org/10.7759/cureus.30759
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author Hassan, Imran H
Soliman, Mina
Shirazi-Nejad, Ahmad R
author_facet Hassan, Imran H
Soliman, Mina
Shirazi-Nejad, Ahmad R
author_sort Hassan, Imran H
collection PubMed
description Ménétrier’s disease is a rare, acquired, premalignant disorder of the stomach distinguished by hypertrophic giant mucosal folds in the proximal part of the stomach (body and fundus), diminished acid secretion and excessive mucous production, resulting in hypoalbuminemia. Menetrier’s is also known as hypoproteinaemic hypertrophic gastropathy and giant hypertrophic gastritis. We present a case of four siblings who presented between 2010 and 2017 with symptomatic iron deficiency anaemia or upper gastrointestinal symptoms with confirmed Ménétrier’s disease. The etiology of Ménétrier’s disease is unknown but it has been linked to raised TGF-α (transforming growth factor alpha protein) in the gastric mucosa causing increased signalling of epidermal growth factor. It is also associated with infections such as cytomegalovirus and Helicobacter pylori. Despite these associations, there have been cases without any of these viruses detected.  Diagnosis is usually made by barium swallow or endoscopic evaluation, which characteristically shows thickened gastric folds, while the biopsies' hallmark feature is massive foveolar hyperplasia. We present a case series of four siblings who presented between 2010 and 2017 with symptomatic iron deficiency anaemia or upper gastrointestinal symptoms with confirmed Ménétrier’s disease.
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spelling pubmed-96116422022-10-31 Ménétrier’s Disease and Its Atypical Presentation in Four Siblings Hassan, Imran H Soliman, Mina Shirazi-Nejad, Ahmad R Cureus Gastroenterology Ménétrier’s disease is a rare, acquired, premalignant disorder of the stomach distinguished by hypertrophic giant mucosal folds in the proximal part of the stomach (body and fundus), diminished acid secretion and excessive mucous production, resulting in hypoalbuminemia. Menetrier’s is also known as hypoproteinaemic hypertrophic gastropathy and giant hypertrophic gastritis. We present a case of four siblings who presented between 2010 and 2017 with symptomatic iron deficiency anaemia or upper gastrointestinal symptoms with confirmed Ménétrier’s disease. The etiology of Ménétrier’s disease is unknown but it has been linked to raised TGF-α (transforming growth factor alpha protein) in the gastric mucosa causing increased signalling of epidermal growth factor. It is also associated with infections such as cytomegalovirus and Helicobacter pylori. Despite these associations, there have been cases without any of these viruses detected.  Diagnosis is usually made by barium swallow or endoscopic evaluation, which characteristically shows thickened gastric folds, while the biopsies' hallmark feature is massive foveolar hyperplasia. We present a case series of four siblings who presented between 2010 and 2017 with symptomatic iron deficiency anaemia or upper gastrointestinal symptoms with confirmed Ménétrier’s disease. Cureus 2022-10-27 /pmc/articles/PMC9611642/ /pubmed/36320788 http://dx.doi.org/10.7759/cureus.30759 Text en Copyright © 2022, Hassan et al. https://creativecommons.org/licenses/by/3.0/This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
spellingShingle Gastroenterology
Hassan, Imran H
Soliman, Mina
Shirazi-Nejad, Ahmad R
Ménétrier’s Disease and Its Atypical Presentation in Four Siblings
title Ménétrier’s Disease and Its Atypical Presentation in Four Siblings
title_full Ménétrier’s Disease and Its Atypical Presentation in Four Siblings
title_fullStr Ménétrier’s Disease and Its Atypical Presentation in Four Siblings
title_full_unstemmed Ménétrier’s Disease and Its Atypical Presentation in Four Siblings
title_short Ménétrier’s Disease and Its Atypical Presentation in Four Siblings
title_sort ménétrier’s disease and its atypical presentation in four siblings
topic Gastroenterology
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9611642/
https://www.ncbi.nlm.nih.gov/pubmed/36320788
http://dx.doi.org/10.7759/cureus.30759
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