Ménétrier's disease
| Ménétrier disease | |
|---|---|
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| Gross specimen of biopsy of stomach in Ménétrier disease. In this case, the substantial pit hyperplasia makes the large rugal folds appear to be covered by myriad polyps resembling hyperplastic polyps. The muscularis propria is the folded structure at the bottom center. | |
| Classification and external resources | |
| Specialty | gastroenterology |
| ICD-10 | K29.6 |
| ICD-9-CM | 535.2 |
| OMIM | 137280 |
| DiseasesDB | 8001 |
| MeSH | D005758 |
| Orphanet | 2494 |
Ménétrier disease (also known as hypoproteinemic hypertrophic gastropathy; named after a French physician Pierre Eugène Ménétrier, 1859–1935), is a rare, acquired, premalignant disease of the stomach characterized by massive gastric folds, excessive mucous production with resultant protein loss, and little or no acid production. The disorder is associated with excessive secretion of transforming growth factor alpha (TGF-α).
Individuals with the disease present with upper abdominal pain (epigastric), at times accompanied by nausea, vomiting, loss of appetite, edema, and weight loss. A small amount of gastrointestinal bleeding may occur, which is typically due to superficial mucosal erosions; large volume bleeding is rare. 20% to 100% of patients, depending on time of presentation, develop a protein-losing gastropathy accompanied by low blood albumin and edema.
Symptoms and pathological features of Ménétrier disease in children are similar to those in adults, but disease in children is usually self-limited and often follows respiratory infection.
The cause of Ménétrier disease is unknown, but it has been associated with HCMV infection in children and H. pylori infections in adults. Additionally, increased TGF-α has been noted in the gastric mucosa of patients with the disease.
With Ménétrier disease, the stomach is characterized by large, tortuous gastric folds in the fundus and body of the stomach, with antrum generally spared, giving the mucosa a cobblestone or cerebriform (brain-like) appearance. Histologically, the most characteristic feature is massive foveolar hyperplasia (hyperplasia of surface and glandular mucous cells). The glands are elongated with a corkscrew-like appearance and cystic dilation is common. Inflammation is usually only modest, although some cases show marked intraepithelial lymphocytosis. Diffuse or patchy glandular atrophy, evident as hypoplasia of parietal and chief cells, is typical.
Although ICD-10 classifies it under "Other gastritis" (K29.6), and the lamina propria may contain mild chronic inflammatory infiltrate, Ménétrier disease is not considered a form of gastritis. It is rather considered as one of the two most well understood hypertrophic gastropathies; the other being Zollinger–Ellison syndrome.
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