Ménétrier's disease
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Ménétrier's disease (also known as hyperplastic hypersecretory gastropathy, and named after a French physician P. Ménétrier, 1859-1935), is a disorder in which the gastric mucosal folds (rugae) are enlarged (and the total weight of the stomach is increased), making the surface of the stomach look a bit like the brain gyri. The alter gastric mucosa secretes massive amounts of mucus, resulting in low plasma protein levels.
There are 2 forms; a childhood form attributed to cytomegalovirus-infections (CMV), and an adult form linked with overexpression of transforming growth factor alpha (TGF-α). It is 4 times more common in men. The presenting symptoms are
- pain after the meal (=postprandial), relieved by antacids, is very usual
- weight loss, cachexia
- peripheral edema, ascites
So, there is clearly a differential diagnosis with cancer (cachexia, ascites).
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.
Other forms of hyperplastic gastropathy include Zollinger-Ellison syndrome.
[edit] References
Rubin's Pathology, Clinicopathological Foundations of Medicine, 4th edition, Rubin, Gorstein, Rubin, Schwarting, Strayer. Lippincott Williams & Wilkins. ISBN 0-7817-4733-3