Menetrier Disease

Menetrier Disease

National Organization for Rare Disorders, Inc.

Important

It is possible that the main title of the report Menetrier Disease is not the name you expected.

Disorder Subdivisions

  • None

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.

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

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.

Resources

NIH/National Institute of Diabetes, Digestive & Kidney Diseases

Office of Communications & Public Liaison

Bldg 31, Rm 9A06

31 Center Drive, MSC 2560

Bethesda, MD 20892-2560

Tel: (301)496-3583

Email: NDDIC@info.niddk.nih.gov

Internet: http://www2.niddk.nih.gov/



Madisons Foundation

PO Box 241956

Los Angeles, CA 90024

Tel: (310)264-0826

Fax: (310)264-4766

Email: getinfo@madisonsfoundation.org

Internet: http://www.madisonsfoundation.org



For a Complete Report

This is an abstract of a report from the National Organization for Rare Disorders, Inc.® (NORD). Cigna members can access the complete report by logging into myCigna.com. For non-Cigna members, a copy of the complete report can be obtained for a small fee by visiting the NORD website. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational treatments (if available), and references from medical literature. For a full-text version of this topic, see http://www.rarediseases.org/search/rdblist.html.

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