Menetrier Disease




© Springer International Publishing Switzerland 2016
Stefano Guandalini, Anil Dhawan and David Branski (eds.)Textbook of Pediatric Gastroenterology, Hepatology and Nutrition10.1007/978-3-319-17169-2_13


13. Menetrier Disease



Yvan Vandenplas 


(1)
Department of Pediatrics, UZ Brussel, Laarbeeklaan 101, 1090 Brussels, Belgium

 



 

Yvan Vandenplas



Keywords
Ménétrier diseaseHypoalbuminemiaHypoproteinemiaHypertrophy mucosal folds


In 1888, Pierre Ménétrier first described the disease that bears his name. Menetrier disease (MD) is a rare condition of unknown etiology characterized by giant hypertrophy of the mucosal folds in the stomach. Vomiting, abdominal pain, anorexia, and edema secondary to the protein loss from the stomach are the most common presenting features. In MD, gastric acid secretion is decreased. Peripheral edema, ascites, and pleural effusion are typical manifestations of the severe hypoalbuminemia found in MD. Hypoalbuminemia occurs due to protein loss from hypertrophic gastric folds. Fever, anorexia, diarrhea, and weight loss are as well reported. MD may develop after a prodromal viral infection, including epigastric pain, anorexia, vomiting, but also raised immunoglobulin (Ig)E levels. In adults, MD is a chronic disease requiring specific therapy, while pediatric MD is typically of acute onset, transient, and self-resolving. The mean age of affected children is 4.5–5.0 years, although exceptionally MD has also been reported in the neonatal period. A young infant who developed gastric outlet obstruction by 3 months of age was also eventually diagnosed with MD, suggesting the possibility that infantile MD may be an entity distinct from the childhood and adult forms [1]. In adults, MD is an acquired premalignant disorder. Pediatric MD usually has an insidious onset and progressive, chronic clinical course, and it spontaneously resolves in weeks or months. Pediatric MD is considered uncommon although the precise incidence is unknown. Cytomegalovirus (CMV) has been implicated in several children, but also Helicobacter pylori (Hpyl) [2, 3], Herpes simplex, and Mycoplasma have been thought to be possibly responsible. As for Hpyl infection, given its high prevalence and the low incidence of MD, the association of Hpyl and MD may only be a coincidence. Given the transient nature of MD in children, even if eradication of Hpyl results in the cure of MD, the causative role of Hpyl would remain unproven. In case of CMV-MD, however, antiviral agents are related to a better cure. In children, lymphoma can also present with hypertrophic gastric folds. Anisakiasis (a parasitic disease caused by anisakid nematodes), granulomatous gastritis, eosinophilic and allergic gastritis, and other rare conditions such as plasmacytoma and systemic lupus erythematosus may all present with hypertrophic gastric folds. MD-like disease occurs also in animals [4].
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Jul 12, 2016 | Posted by in HEPATOPANCREATOBILIARY | Comments Off on Menetrier Disease

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