Ménétrier Disease

Overview


Plain-Language Overview

Ménétrier Disease is a rare condition that affects the stomach lining, causing it to become abnormally thick and enlarged. This leads to the formation of large folds in the stomach wall, which can interfere with normal digestion and absorption of nutrients. People with this disease often experience symptoms like abdominal pain, nausea, and swelling due to low protein levels in the blood. The disease primarily impacts the digestive system and can cause significant discomfort and nutritional problems. It is important to understand that this condition involves changes in the stomach's structure that affect its ability to function properly.

Clinical Definition

Ménétrier Disease is a rare hypertrophic gastropathy characterized by giant rugal folds in the gastric body and fundus, resulting from hyperplasia of the mucous cells and glandular atrophy. The pathogenesis involves overexpression of transforming growth factor-alpha (TGF-α) leading to excessive activation of the epidermal growth factor receptor (EGFR) pathway, causing mucous cell proliferation and decreased acid secretion. This results in protein-losing gastropathy with hypoalbuminemia and edema. Clinically, it presents with epigastric pain, nausea, vomiting, and peripheral edema due to protein loss. The disease is significant because it can mimic gastric cancer and carries a risk of malignant transformation. It is most commonly seen in middle-aged adults and may be associated with cytomegalovirus (CMV) infection in children.

Inciting Event

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Clinical Presentation


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Diagnostic Workup


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Pathophysiology


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