Ménétrier Syndrome
Ménétrier Syndrome
A rare hyperproliferative gastropathy characterized by massive folds in the stomach wall, resulting in protein loss and hypochlorhydria.
Key Points
- Also known as Giant Hypertrophic Gastritis
- Rare disease affecting both adults and children
- Associated with increased risk of gastric cancer
- Different clinical course in children vs adults
- Linked to excessive TGF-α production
Clinical Manifestations
Common Symptoms
- Gastrointestinal:
- Epigastric pain
- Nausea and vomiting
- Early satiety
- Anorexia
- Weight loss
- Systemic manifestations:
- Peripheral edema
- Fatigue
- Muscle weakness
- Growth delay in children
Age-Specific Features
- Pediatric presentation:
- Usually acute onset
- Often self-limiting
- Associated with CMV infection
- Better prognosis
- Adult presentation:
- Chronic progressive course
- Higher cancer risk
- More severe protein loss
- Often requires surgical intervention
Diagnostic Approach
Laboratory Studies
- Serum studies:
- Serum albumin (typically decreased)
- Total protein
- Complete blood count
- Electrolytes
- Gastrin levels
- Other tests:
- 24-hour urinary protein
- Stool alpha-1 antitrypsin
- CMV testing in children
- H. pylori testing
Imaging Studies
- Endoscopic findings:
- Enlarged gastric folds
- Typically sparing antrum
- Eroded or polypoid surface
- Thick mucous coating
- Radiologic studies:
- CT scan
- Upper GI series
- Endoscopic ultrasound
Histopathology
- Key features:
- Foveolar hyperplasia
- Glandular atrophy
- Reduced parietal cells
- Increased mucous cells
- Minimal inflammation
- Required sampling:
- Multiple deep biopsies
- Full-thickness specimens
Management Strategies
Medical Management
- Protein replacement:
- Albumin infusions
- High-protein diet
- Nutritional support
- Targeted therapy:
- Cetuximab (EGFR inhibitor)
- Octreotide
- H2 blockers
- Proton pump inhibitors
Surgical Management
- Indications:
- Severe symptoms
- Refractory cases
- Cancer risk
- Complications
- Procedures:
- Total gastrectomy
- Partial gastrectomy
- Reconstructive options
Supportive Care
- Nutritional support
- Pain management
- Regular monitoring
- Psychological support
Pathophysiology & Recent Research
Molecular Mechanisms
- Growth factor involvement:
- Increased TGF-α expression
- EGFR pathway activation
- Altered cell proliferation
- Associated factors:
- CMV infection
- H. pylori infection
- Genetic predisposition
Disease Progression
- Natural history:
- Progressive in adults
- Self-limiting in children
- Cancer risk (10-15% in adults)
- Monitoring requirements:
- Regular endoscopy
- Protein level monitoring
- Cancer surveillance
Current Research
- Therapeutic targets:
- Novel EGFR inhibitors
- Growth factor pathways
- Immunological factors
- Clinical trials:
- Targeted therapies
- Biomarker studies
- Prognostic indicators
