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Ménétrier's disease

Ménétrier disease (also known as hyperplastic hypersecretory gastropathy, and named after a French physician Pierre Eugène Ménétrier, 1859-1935), is a disorder in which the gastric mucosal folds (rugae) are enlarged^[1] (and the total weight of the stomach is increased), making the surface of the stomach look a bit like the brain gyri. The altered gastric mucosa secretes massive amounts of mucus, resulting in low plasma protein levels. The tissue may be inflamed and may contain ulcers. The disease also causes glands in the stomach to waste away and causes the body to lose fluid containing a protein called albumin.

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There are two forms of the disease: a childhood form and an adult form. The childhood form has a better prognosis. It affects boys and girls equally, most often after they have a viral illness caused by cytomegalovirus (CMV) or a bacterial infection caused by Helicobacter pylori. Children are not born with this disease, and it is not passed from parents to their children.^[2]. The adult form linked with overexpression of transforming growth factor alpha (TGF-α). The adult variety is four times more common in men, primarily affecting men between ages 30 and 60.

The presenting symptoms are

pain after the meal (=postprandial), relieved by antacids, is very usual
weight loss, cachexia
peripheral edema, ascites
anemia symptoms secondary to blood loss

Ménétrier disease increases a person's risk of stomach cancer.^[3]

Microscopically, the disease is characterized by hyperplasia of the crypts, which are elongated and may appear cystic or corkscrew-like. Since it predisposes to stomach cancer, periodic endoscopic surveillance is mandated. CMV-related Ménétrier is often self-limited.

The disease must be diagnosed by x-ray (rare) or endoscopy and biopsy of the stomach. In adults, thre is no treatment other thangastrectomy. and a high-protein diet. Part or all of the stomach may need to be removed if the disease is severe. Pediatric cases are normally treated for symptoms with the disease clearing up in weeks to months.

Other forms of hyperplastic gastropathy include Zollinger-Ellison syndrome.

References

Rubin's Pathology, Clinicopathological Foundations of Medicine, 4th edition, Rubin, Gorstein, Rubin, Schwarting, Strayer. Lippincott Williams & Wilkins. ISBN 0-7817-4733-3