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What

Ménétrier's disease is a disorder of the stomach, causing giant folds of tissue to grow in the wall of the stomach. This tissue may be inflamed and may contain ulcers. The disease also causes glands in the stomach to waste away and the body to lose fluid containing protein, causing abdominal pain, vomiting, and causing a child's body to swell.

Who

Ménétrier's disease is a rare disease most commonly affects adults over the age of 50 years. However, a childhood form also exists that is not as serious and has a better prognosis. It affects boys and girls equally, most often after a viral illness. There is no racial predisposition for this disease. Children are not born with this disease, and it is not passed from parents to their children.

Signs and Symptoms

Children with Ménétrier's disease are most often diagnosed after they become swollen around their eyes or around their ankles or bellies. The following symptoms can also be present as the disease progresses:

1. Swelling around the eyes, belly or scrotum (edema)

2. Vomiting either food or blood or both

3. Nausea and loss of appetite

4. Abdominal pain in the top middle part of the abdomen

5. Blood in the urine (hematuria)

6. Protein in the urine (proteinuria)

7. Fatigue due to blood loss

8. Weight loss

9. Diarrhea

Possible Causes

The cause of Ménétrier's disease in children is thought to be triggered by an infection, either by a virus called cytomegalovirus or a bacterium called Helicobacter pylori. The infection triggers the development of large folds in the lining of the stomach, which causes a loss of protein into the urine and stool. The loss of protein is what leads to the swelling of the body.

Diagnosis

Diagnosis of Ménétrier's disease is made by a special procedure called endoscopy, in which a small camera attached to a flexible tube is inserted into the mouth and used to view the inside of a child's throat, esophagus and stomach. A small piece of stomach tissue will be removed for examination under a microscope to look for signs of disease. This is called a biopsy. Other tests may be performed to make sure that there are no other causes of your child's medical condition: a urine test may be performed and blood work may be done to assess how well your child's kidneys and liver are working.

Treatment

Treatment for Ménétrier's disease focuses on relieving the discomfort of swelling and stomach pain. Medicines that help treat ulcers can also be helpful with this disease. Children with Ménétrier's disease should be instructed to eat a high protein diet.

Prognosis

The symptoms of Ménétrier's disease in children will get better by itself and over time, usually within weeks or months. Adults who acquire the disease have more serious complications and often require surgery, but this is not necessary in children.

Connect with other parents

In the spirit of community and support, Madisons Foundation offers the unique service of connecting parents of children with rare diseases. If you would like to be connected to other parents of children with this disease, please fill out this brief form.

Weblinks

eMedicine

http://author.emedicine.com/med/topic1926.htm

Web link takes you to "Protein losing enteropathy" which is medical terminology for this disease. eMedicine is a private, U.S.-based company with extensive articles written by physicians for other health care providers. This site provides detailed and technical descriptions of specific illnesses.

National Digestive Diseases Information Clearinghouse Website

http://digestive.niddk.nih.gov/ddiseases/pubs/menetrierM%C3%A9n%C3%A9trier's/index.htm

A Maryland-based organization run by the National Institutes of Health, part of the United States government, responsible for conducting and supporting research.

National Organization for Rare Disorders Inc. (NORD)

http://www.rarediseases.org/search/rdbdetail_abstract.html?disname=Menetrier%20Disease

The National Organization for Rare Disorders, is a unique federation of voluntary health organizations dedicated to helping people with rare diseases and assisting the organizations that serve them. NORD is committed to the identification, treatment, and cure of rare disorders through programs of education, advocacy, research, and service.

Google Search for Menetrier's Disease

References and Sources

• 1. Coffey RJ, et al. "Menetrier disease and gastrointestinal stromal tumors: hyperproliferative disorders of the stomach". The Journal of Clinical Investigation. 117:70-80 (2007).
• 2. Hassall E (1996). Chapter 15.19.1. Gastritis. In: Rudolph AM, Hoffman JIE, Rudolph CD (eds.) Rudolph's Pediatrics, 20th ed., Appleton & Lange, p. 1090. 2. Hochman JA, Witte DP, Cohen MB (1996). Diagnosis of cytomegalovirus infection in pediatric Ménétrier's disease by in situ hybridization. Journal of Clinical Microbiology, October, 1996, p. 2588-2589.
• 3. Yardley, JH et al. "Hyperplastic gastropathies and other causes of enlarged gastric folds" www.UpToDate.com. 2008.