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  • • Giant hypertrophy of the gastric rugae with excessive loss of protein from the thickened mucosa into the gut, with resulting hypoproteinemia

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Epidemiology

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  • • High, normal, or low acid secretion

    • Excessive loss of protein from the thickened mucosa into the gut, with resulting hypoproteinemia

    • In children, the disease characteristically is self-limited and benign

    • There is an increased risk of adenocarcinoma of the stomach in adults with Ménétrier disease

    • Associated with Helicobacter pylori infection

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Symptoms and Signs

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  • • Diarrhea

    • Indigestion

    • Anorexia

    • Weight loss

    • Skin rash

    • Edema from hypoproteinemia

    • Symptomatic anemia

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Laboratory Findings

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  • • Hypoproteinemia

    • Anemia

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Imaging Findings

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  • • The hypertrophic rugae present as enormous filling defects on upper GI contrast studies and are frequently misinterpreted as carcinoma

    • Hypertrophic rugae apparent on upper GI endoscopy

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  • • Radiographic or endoscopic evidence of hypertrophic gastric rugae with hypoproteinemia is strongly suggestive of diagnosis

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Rule Out

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  • • Adenocarcinoma

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  • • Upper GI contrast radiographic study

    • Upper GI endoscopy

    • Serum protein, Hct

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  • • Goal is to reduce protein loss

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Surgery

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  • • Total gastrectomy

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Indications

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  • • Rarely indicated for severe intractable hypoproteinemia, anemia, or inability to exclude cancer

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Medications

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  • • Protein leak may respond to atropine, hexamethonium bromide, eradication of H pylori, H2 blocking agents, or omeprazole

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Treatment Monitoring

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  • • Endoscopic surveillance for development of adenocarcinoma

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Complications

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  • • Hypoproteinemia

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Prognosis

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  • • Despite medical management, gastric abnormalities and hypoproteinemia may persist

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References

Madsen LG et al. Ménétrier's disease and Helicobacter pylori: normalization of gastrointestinal protein loss after eradication therapy. Dig Dis Sci. 1999;44:2307.  [PubMed: 10573379]

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