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Diseases

Genetic and Rare Diseases Information Center (GARD)

Menetrier disease


Other Names for this Disease
  • Giant hypertrophic gastritis
  • Giant hypertrophic gastropathy
  • Gastroenteropathy, protein losing
  • Giant hypertrophy of the gastric mucosa
  • Hypertrophic gastropathy
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.

Your Question

I am an adult woman who has been recently diagnosed with Ménétrier disease.  My symptoms seem to be worsening.  I am seeking information about the symptoms, cause(s), prognosis, genetic association, and treatment.  I am eager to hear from you and appreciate your assistance.

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

What is Ménétrier 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.[1] This leads to excessive mucus production, causing protein loss from the stomach and low or absent levels of stomach acid.[2] Symptoms may include pain in the upper middle region of the stomach, nausea, vomiting, and diarrhea.[1] 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.[1][2]
Last updated: 6/1/2016

What are the signs and symptoms of Ménétrier disease?

The symptoms of Ménétrier disease may vary from individual to individual. While some individuals may not experience any symptoms, the most common symptom is pain in the upper middle region of the stomach (epigastric pain). Other symptoms may include:[1][3]
  • Weight loss
  • Nausea and vomiting
  • Gastrointestinal bleeding
  • Diarrhea
  • Edema


Last updated: 6/1/2016

What causes Ménétrier 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.[4][2]
Last updated: 6/1/2016

Is Ménétrier 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. [5] 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: 6/1/2016

How can I find a genetics professional in my area?

Genetics clinics are a source of information for individuals and families regarding genetic conditions, treatment, inheritance, and genetic risks to other family members. More information about genetic consultations is available from Genetics Home Reference. To find a genetics clinic, we recommend that you contact your primary healthcare provider for a referral.

The following online resources can help you find a genetics professional in your community:
  • GeneTests has a searchable directory of US and international genetics and prenatal diagnosis clinics.
  • The National Society of Genetic Counselors provides a searchable directory of US and international genetic counseling services.
  • The American College of Medical Genetics has a searchable database of US genetics clinics.
  • The American Society of Human Genetics maintains a database of its members, which includes individuals who live outside of the United States. Visit the link to obtain a list of the geneticists in your country, some of whom may be researchers that do not provide medical care.
Last updated: 6/1/2016

How might Ménétrier 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.[3]
Last updated: 6/1/2016

What is the long-term outlook for Ménétrier 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.[3]
Last updated: 6/1/2016

References
Other Names for this Disease
  • Giant hypertrophic gastritis
  • Giant hypertrophic gastropathy
  • Gastroenteropathy, protein losing
  • Giant hypertrophy of the gastric mucosa
  • Hypertrophic gastropathy
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.