Menetrier disease

What causes Menetrier disease?

The exact cause of Ménétrier disease is unknown. However, it has been associated with cytomegalovirus (CMV) infection in children and Helicobacter pylori( H. pylori ) infection in adults. In addition, some have suggested that overexpression of a type of growth factor called the transforming growth factor-α, which is found in a specific part of the stomach, called the superficial gastric epithelium, might play a role.

Last updated on 05-01-20

What is Menetrier disease?

Ménétrier disease is a gastrointestinal condition characterized by overgrowth of the mucous cells (foveola) in the mucous membrane lining the stomach, causing enlarged gastric folds. This leads to excessive mucus production, causing protein loss from the stomach and low or absent levels of stomach acid. Symptoms may include pain in the upper middle region of the stomach, nausea, vomiting, and diarrhea. The cause of Ménétrier disease is largely not known, although some cases are associated with infection, particularly pediatric cases, which can be caused by CMV infections. Treatment may include medications such as cetuximab and gastrectomy in severe cases.

Last updated on 05-01-20

Is Menetrier disease genetic?

Although the cause of Ménétrier disease is unknown, several cases of the condition have been reported in a familial setting. In a 2003 journal article titled "An unusual expression of hyperplastic gastropathy (Menetrier type) in twins," Ibarrola et al. described the first documented example of an occurrence in twins. They suggest that these two cases suggest the possibility of a genetic predisposition for this condition. There have been several other reports of familial occurrences. You can read more about these cases by clicking here to access the information page on Ménétrier disease from Online Mendelian Inheritance in Man (OMIM).

If you are concerned about a family history of Ménétrier disease, we would recommend consulting with a genetics professional.

Last updated on 05-01-20

What is the long-term outlook for Menetrier disease?

The prognosis of Ménétrier disease varies from person to person. Resolution of symptoms may occur in adults with an underlying Helicobacter Pylori (H. Pylori) infection once treatment of the infection occurs. Cases in children may resolve spontaneously or with treatment of the underlying CMV infection.

Although it has been suggested that patients with Ménétrier disease may be at an increased risk of developing stomach cancer; the risk remains uncertain. Studies estimate that between 2% and 15% of individuals with Ménétrier disease may develop stomach cancer in their lifetime. Given this potential, some authors suggest surveillance with annual or biannual endoscopy.

Last updated on 05-01-20

How might Menetrier disease be treated?

No one treatment has proven effective for all patients with Ménétrier disease. In milder cases, treatment may be supportive with a recommended high protein diet to offset loss of protein. Other treatment options for moderate or more severe cases may include: Treatment of underlying CMV/ _Helicobacter pylori (H. Pylori) _infection; medications such as Gastric antisecretory agents (protein pump inhibitors), octreotide, and cetuximab; and a gastrectomy.

Last updated on 05-01-20

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The RareGuru disease database is regularly updated using data generously provided by GARD, the United States Genetic and Rare Disease Information Center.

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