NORD gratefully acknowledges 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.
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.
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.
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.
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.
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.
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.
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
For information about clinical trials sponsored by private sources, contact:
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
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
Please note that some of these organizations may provide information concerning certain conditions potentially associated with this disorder.
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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.
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