Ménétrier Disease

Figure

Figure

A previously healthy 16-month-old boy was hospitalized because of vomiting of 10 days' duration, fever of 4 days' duration (temperature up to 38.6°C [101.4°F]), and watery diarrhea. He also had had a maculopapular rash, which resolved the day before presentation. Family history was unremarkable.

Physical examination, including abdominal examination, was normal. No rash was noted. Weight was 12.5 kg (90th percentile), and development was appropriate for age. On the third hospital day, significant pitting edema (mainly on the trunk) and periorbital swelling developed; the albumin level was 1.8 g/dL, and total protein level was 3.5 g/dL.

Urinalysis results, liver enzyme levels, prothrombin time, partial thromboplastin time, urea nitrogen level, and creatinine level were normal. A protein-losing enteropathy/gastropathy was suspected. Gastroscopy revealed hypertrophic gastric folds and severe gastritis (A). Histopathological examination of the gastric mucosa showed foveolar hyperplasia and inflammatory changes (B), consistent with Mntrier disease, and Cytomegalovirus (CMV) inclusion bodies (C, arrows).

Mntrier disease in children is a rare, self-limited disorder that is characterized by marked protein-losing gastropathy associated with enlarged and thickened gastric folds.^1-3 Abnormal regulation of gastric epithelial growth, probably triggered by an infectious agent (CMV or Helicobacter pylori), may be a cause of this disorder.^4-9 In this case, enzyme immunoassay testing was positive for CMV-specific IgM antibody and urine culture was positive for CMV antigen.

The clinical, radiological, and histological features of Mntrier disease in children resemble those of the adult form. However, there are important differences. Generalized edema occurs in fewer than 25% of adult cases but is the most common finding in children. In addition, the clinical course is usually unfavorable in adults; many require partial or total gastrectomy for persistent symptoms. The prognosis for children, on the other hand, is excellent.

This patient was treated with omeprazole; within 3 weeks, his symptoms had resolved and the serum albumin level had normalized.

References:

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2.

Oliver MR, Trevenen C, Scott RB. Childhood Ménétrier's disease: a rare cause of exudative enteral protein loss.

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Proujansky R. Protein-losing enteropathy. In: Walker WA, Durie PR, Hamilton JR, et al, eds.

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4.

Dempsey PJ, Goldenring JR, Soroka CJ, et al. Possible role of transforming growth factor alpha in the pathogenesis of Ménétrier's disease: supportive evidence from humans and transgenic mice.

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Badov D, Lambert JR, Finlay M, Balazs ND. Helicobacter pylori as a pathogenic factor in Ménétrier's disease.

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Hochman JA, Witte DP, Cohen MB. Diagnosis of cytomegalovirus infection in pediatric Ménétrier's disease by in situ hybridization.

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Occena RO, Taylor SF, Robinson CC, Sokol RJ. Association of cytomegalovirus with Ménétrier's disease in childhood: report of two new cases with a review of literature.

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Sferra TJ, Pawel BR, Qualman SJ, Li BU. Ménétrier disease of childhood: role of cytomegalovirus and transforming growth factor alpha.

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