Ménétrier’s Disease and Its Atypical Presentation in Four Siblings

Abstract

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.

Comorbidity of Menetrier’s disease and diabetes mellitus. A clinical case

“…Menetrier's disease is a rare gastric disorder that causes enlargement of gastric mucus-secreting cells with subsequent atrophy of the acid-secreting glands. It is not well-documented, with less than 1,000 cases currently reported in the literature [1]. The disease tends to affect males slightly more often than females and typically presents between 30 and 60 years with an average age of 55 [2].…”

“…Menetrier's disease is a rare acquired disorder of the stomach associated with giant rugal folds involving the body and fundus, hypochlorhydria or achlorhydria, excess gastric mucus production, and hypoalbuminemia resulting from gastric mucosal protein loss [1,2]. Patients with Menetrier's disease generally are men, diagnosed at a mean age of 55 years with progressive and insidious symptoms of abdominal pain, nausea, and vomiting, often with concomitant peripheral edema resulting from severe protein-losing enteropathy [3,4].…”

“…Since this time, very few publications have addressed the etiology or outcomes of this disease, with a lack of data about the demographic characteristics or long-term follow-up evaluation of patients with Menetrier's disease. Currently, there have been less than 1,000 reported cases of this disease [1].…”