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Menetrier disease

Orpha number ORPHA2494
Synonym(s) Giant hypertrophic gastritis
Hypoproteinemic hypertrophic gastropathy
Prevalence Unknown
Inheritance
  • Unknown
Age of onset Variable
ICD-10
  • K29.6
OMIM
UMLS -
MeSH -
MedDRA -
SNOMED CT
  • 413219009
  • 60002000

Summary

Menetrier's disease or hypoproteinemic hypertrophic gastropathy is a rare, acquired, premalignant disorder of the stomach. Prevalence is unknown. Symptoms include epigastric pain, nausea, vomiting, edema, anorexia and weight loss. Menetrier's disease is characterised by giant hypertrophic folds that most often involve the fundus with histological features of marked foveolar hyperplasia, atrophy of glands and an increase in mucosal thickness. Additional findings include loss of enteric proteins and hypoalbuminemia, excess mucus secretion, decreased gastric acid secretion and normal or slightly elevated fasting serum gastrin levels. Most reports quote a 10 to 15% association with gastric carcinoma. The cause of the disease is unknown, although infections with Helicobacter pylori in adults and cytomegalovirus in children have been implicated. A few cases have been reported to be inherited as a dominant trait. The differential diagnosis of Menetrier's disease includes other forms of hypertrophic gastropathy such as Zollinger-Ellison syndrome, hypertrophic hypersecretory gastropathy, lymphocytic gastritis, or an infiltrating neoplasm. Spontaneous remission of the disease is rare. There is no established treatment. Reported treatments include anticholinergics, H2 receptor antagonists, tranexamic acid, corticosteroids and H. pylori eradication. Partial or total gastrectomy is generally reserved for patients with debilitating disease and for cases at risk of developing gastric cancer.


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