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Menetrier Disease

NORD is very grateful to Nicholas J. Talley, MD, PhD, Professsor and pro Vice-Chancellor, University of Newcastle, Adjunct Professor of Medicine, Mayo Clinic and John Y. Kao, MD, Director, Michigan Gut Peptide Center Visiting Professor Series, Assistant Director of GI Fellowship Program, Assistant Professor of Medicine, Division of Gastroenterology and Hepatology, University of Michigan Medical School, Ann Arbor, Michigan, for assistance in the preparation of this report.

Synonyms of Menetrier Disease

  • No synonyms found.

Disorder Subdivisions

  • No subdivisions found.

General Discussion

Menetrier disease is a rare disorder characterized by massive overgrowth of mucous cells (foveola) in the mucous membrane lining the stomach, resulting in large gastric folds. The main symptom associated with Menetrier disease is pain in the upper middle region of the stomach (epigastric pain). The cause of Menetrier disease is unknown.

There is considerable confusion and contradiction in the medical literature regarding disorders involving large gastric folds. The name Menetrier disease is often erroneously used to describe any condition with large gastric folds or as a synonym for giant hypertrophic gastritis (GHG). However, Menetrier disease is not a true form of gastritis. A diagnosis of Menetrier disease should be reserved for individuals with large gastric folds due to overgrowth of mucous cells. There is minimal or no stomach inflammation in Menetrier disease. Because inflammation is minimal or not present, Menetrier disease is classified as a form of hyperplastic gastropathy and not as a form of gastritis. Some researchers believe that Menetrier disease and GHG may be variants of the same disorder or different parts of one disease spectrum.

Symptoms

The symptoms of Menetrier disease may vary from case to case. Some individuals may not exhibit any symptoms (asymptomatic). The most common symptom is pain in the upper middle region of the stomach (epigastric pain). Less frequent findings include nausea, vomiting, and diarrhea. In some cases, weight loss and profound loss of appetite (anorexia) may also occur.

An additional variable finding sometimes associated with Menetrier disease is the loss of the protein from the gastrointestinal tract (protein-losing gastropathy) such as the loss of the protein albumin (hypoalbuminemia). Protein loss may be severe in some cases. Protein loss may result in fluid accumulation (edema) in the stomach or the legs.

Gastrointestinal bleeding has also been reported in some cases of Menetrier disease usually as a result of erosions (ulcers) in the mucosal wall. Acid secretion within the stomach is often markedly decreased (hypochlorhydria) or absent. Excess mucous secretion in the stomach may also occur.

Some researchers believe that individuals with Menetrier disease have a greater risk of developing gastric cancer than the general population has. However, other researchers do not believe this to be the case. Sepsis (severe illness caused by infection of the bloodstream) and vascular thromboembolic complications are probably a greater threat to affected individuals.

Causes

The exact cause of Menetrier disease is unknown. In children, some cases of Menetrier disease may be associated with infection with cytomegalovirus (CMV). Helicobacter pylori has been implicated in adults with Menetrier disease. The exact role, if any, that these infections play in the development of Menetrier disease is unknown.

Some researchers believe that the large gastric folds that characterize Menetrier disease may result from increased activation of the epidermal growth factor receptor in the stomach.

In extremely rare cases, siblings have developed Menetrier disease as children suggesting a genetic link in these cases. Researchers believe that, in these cases, Menetrier disease may be inherited as an autosomal dominant trait. Genetic diseases are determined by two genes, one received from the father and one from the mother.

Dominant genetic disorders occur when only a single copy of an abnormal gene is necessary for the appearance of the disease. The abnormal gene can be inherited from either parent, or can be the result of a new mutation (gene change) in the affected individual. The risk of passing the abnormal gene from affected parent to offspring is 50% for each pregnancy regardless of the sex of the resulting child.

Affected Populations

Menetrier disease affects males slightly more often than females. It most often affects adults in their 50s or older. However, a childhood form of the disorder exists. Because of the confusion in the literature regarding the term Menetrier disease, it is difficult to determine its true frequency in the general population.

Related Disorders

Symptoms of the following disorders can be similar to those of Menetrier disease. Comparisons may be useful for a differential diagnosis:

Zollinger-Ellison syndrome (ZES) is a rare, digestive disorder characterized by the development of a tumor (gastrinoma) or tumors that secrete excessive levels of gastrin, a hormone that stimulates production of acid by the stomach. Many affected individuals develop multiple gastrinomas, approximately half to two-thirds of which may be cancerous (malignant). In most cases, the tumors arise within the pancreas and/or the upper region of the small intestine (duodenum). Due to excessive acid production (gastric acid hypersecretion), individuals with ZES may develop peptic ulcers of the stomach, the duodenum, and/or other regions of the digestive tract. Peptic ulcers are sores or raw areas within the digestive tract where the lining has been eroded by stomach acid and digestive juices. Symptoms and findings associated with ZES may include mild to severe abdominal pain; diarrhea; increased amounts of fat in the stools (steatorrhea); and/or other abnormalities. Individuals with Zollinger-Ellison syndrome may have giant gastric folds. (For more information on this disorder, choose "Zollinger-Ellison" as your search term in the Rare Disease Database.)

Lymphocytic gastritis is a rare disorder characterized by inflammation of the stomach due to the accumulation of inflammatory cells in the inner wall (mucosa) of the stomach resulting in abnormally large, coiled ridges or folds that resemble polyps in the inner wall of the stomach (large gastric folds). Many individuals with lymphocytic gastritis do not have symptoms (asymptomatic). Symptoms that may occur include nausea, vomiting, abdominal pain, diarrhea, and weight loss. The exact cause of lymphocytic gastritis is unknown although the disorder has been linked to celiac sprue and Helicobacter pylori infection. (For more information on this disorder, choose "lymphocytic gastritis" as your search term in the Rare Disease Database.)

Additional causes of large gastric folds include Helicobacter pylori-associated gastritis, certain infections including syphilis, and various forms of cancer include certain forms of carcinoma or lymphoma. Other rare disorders such as sarcoidosis or Cronkite-Canada disease may also be associated with large gastric folds. (For more information on these disorders, choose the specific disorder name as your search term in the Rare Disease Database.)

Standard Therapies

Diagnosis
Menetrier disease may be suspected in individuals with large gastric folds. Large gastric folds may be diagnosed by an endoscopic exam, a procedure in which a thin, flexible tube (endoscope) is inserted through the mouth and used to examine the interior of the stomach and obtain tissue samples for microscopic study (biopsy). Histopathologic study of affected stomach tissue obtained by biopsy can confirm a diagnosis of Menetrier disease. Histopathology is the study of microscopic anatomical changes in diseased tissue.

Treatment
Menetrier disease has been treated with anticholergic drugs, acid suppression therapy, and antibiotic therapy directed against H. pylori infection. These therapies have produced inconsistent results. In some cases, a high-protein diet may be recommended to treat protein loss. Albumin transfusions may also be used to combat protein loss.

In severe cases such as those with significant protein loss or a high probability of progression to gastric cases, partial or total gastrectomy may be necessary. Gastrectomy is the surgical removal of part or all of the stomach.

In children with Menetrier disease linked to cytomegalovirus infection, treatment directed against the virus may lead to improvement of symptoms.

Other treatment is symptomatic and supportive.

Investigational Therapies

Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government website.

For information about clinical trials being conducted at the National Institutes of Health (NIH) Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:

Tollfree: (800) 411-1222
TTY: (866) 411-1010
Email: prpl@cc.nih.gov

For information about clinical trials sponsored by private sources, contact:
www.centerwatch.com

Contact for additional information about Menetrier disease:

John Y. Kao, MD
Director, Michigan Gut Peptide Center Visiting Professor Series
Assistant Director of GI Fellowship Program
Assistant Professor of Medicine
Division of Gastroenterology and Hepatology
University of Michigan Medical School
6520A MSRB-I, SPC 5682
1150 W. Medical Center Drive
Ann Arbor, MI 48109-5682
Office: 734-647-2964
Lab: 734-647-2962
Fax: 734-763-2535
email: jykao@umich.edu
Website: http://sitemaker.umich.edu/kao.lab

Nicholas J. Talley, MD, PhD
Professor and pro Vice-Chancellor, University of Newcastle
Adjunct Professor of Medicine, Mayo Clinic
200 First St. S.W.
Rochester, MN 55905
507-284-2511

Organizations related to Menetrier Disease

Please note that some of these organizations may provide information concerning certain conditions potentially associated with this disorder.

References

TEXTBOOKS
Merchant JL. Giant Hypertrophic Gastritis. In: NORD Guide to Rare Disorders. Lippincott Williams & Wilkins. Philadelphia, PA. 2003:341.

Fauci AS, et al., eds. Harrison's Principles of Internal Medicine, 14th Ed. New York, NY: McGraw-Hill, Inc; 1998:1610-3.

Bennett JC, Plum F., eds. Cecil Textbook of Medicine. 20th ed. Philadelphia, PA: W.B. Saunders Co; 1996:661.

Yamada T, et al., eds. Textbook of Gastroenterology. 2nd ed. Philadelphia, PA: J.B. Lippincott Company; 1995:1456-82.

JOURNAL ARTICLES
Hoffer V, et al., Ganciclovir treatment in Menetrier's disease. Acta Paediatr. 2003;92:983-5.

Dykes CM. Menetrier's disease: case study in the quality of life. Gastroenterol Nurs. 2003;26:3-6.

Di Vita G, et al., Resolution of Menetrier's disease after Helicobacter pylori eradicating therapy. Dig Dis. 2001;19:179-83.

Rimar Y, Jaffe M, Shaoul R. Harefuah. 2001;140:586-7, 679.

Burdick JS, et al., Treatment of Menetrier's disease with a monoclonal antibody against the epidermal growth factor receptor. N Engl J Med. 2000;343:1697-701.

Chang KW, et al., Menetrier's disease associated with cytomegalovirus infection in a child. Acta Paediatr Taiwan. 2000;41:339-40.

Raderer M, et al., Successful symptomatic management of a patient with Menetrier's disease with long-term antibiotic treatment. Digestion. 1999;60:358-62.

Kindermann A, Koletzko S. Protein-losing giant fold gastritis in childhood ? a case report and differentiation from Menetrier disease of adulthood. Z Gastroenterol. 1998;36:165-71.

Wolfsen C, Carpenter HA, Talley NJ. Menetrier's disease: a form of hypertrophic gastropathy or gastritis? Gastroenterology. 1993;104:1310-9.

Sachs M, Encke A. Menetrier disease? A Rare Disease of the Stomach. Zentralbl Chir. 1993;118:160-5.

Meuwissen SG, et al., Hypertrophic protein-losing gastropathy. A retrospective analysis of 40 cases in the Netherlands. The Dutch Menetrier Study Group. Scand J Gastroenterol Suppl. 1992;194:1-7.

Mosnier JF, et al., Hypertrophic gastropathy with gastric adenocarcinoma: Menetrier's disease and lymphocytic gastritis? Gut. 1991;32:1565-7.

FROM THE INTERNET
McKusick VA., ed. Online Mendelian Inheritance in Man (OMIM). Baltimore. MD: The Johns Hopkins University; Entry No:137280; Last Update: 3/30/2007. Available at: http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=137280 Accessed: 10/12/2010.

The information in NORD’s Rare Disease Database is for educational purposes only. It should never be used for diagnostic or treatment purposes. If you have questions regarding a medical condition, always seek the advice of your physician or other qualified health professional. NORD’s reports provide a brief overview of rare diseases. For more specific information, we encourage you to contact your personal physician or the agencies listed as “Resources” on this report.

Report last updated: 2010/10/13 00:00:00 GMT+0

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