Description, Causes and Risk Factors:
Ménétrier syndrome is a rare disorder caused by excessive secretion of transforming growth factor alpha (TGF-?). The disease is characterized by diffuse hyperplasia of the foveolar epithelium of the body and fundus and hypoproteinemia due to protein losing enteropathy. Secondary symptoms such as weight loss, diarrhea, and peripheral edema are commonly present. Symptoms and pathologic features of Ménétrier syndrome in children are similar to those in adults, but pediatric disease is usually self-limited and often follows respiratory infection.
Ménétrier syndrome can be broken down into 2 types childhood and adult. Both types tend to happen after the person has been infected with a specific virus called cytomegalovirus (CMV) or bacteria call H. pylori. The childhood type has a good prognosis and usually clears up on its own. The adult type requires treatment. Kids are not born with this disease and it does not get passed from parents to child which makes it unlikely that your pyloric stenosis is related to Ménétrier syndrome.
The condition is more common in females frequently middle aged and with an autoimmune or connective tissue condition. In up to one-third of patients, there is an association with cirrhosis, and it can be a manifestation of portal hypertensive gastropathy and reduced serum gastrin levels.
The lesions are dilated ectatic vessels surrounded by fibrosis. In cirrhosis, they may develop from intramural vascular shunts caused by portal hypertension.
Symptoms include discomfort and tenderness in the top middle part of the abdomen, loss of appetite, nausea, diarrhea, blood vomiting, and ulcer-like pain after eating.
The diagnosis is usually made at endoscopy. Typically, red/blue lesions are clustered on the crests of longitudinal rugal folds that converge on the pylorus like stripes on a watermelon. Scattered rounded lesions are also seen. Portal hypertension gastropathy my present with a similar appearance, but tends to be more diffuse and less antrum predominant.
Treatment of Ménétrier syndrome is supportive with intravenous albumin and parenteral nutritional supplementation. In severe cases gastrectomy may be performed. More recently, agents that block TGF-? mediated activation of the epidermal growth factor receptor have shown promise.
NOTE: The above information is educational purpose. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition.
DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.
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