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Menetrier Disease of Early Infancy: A Separate Entity?

Menetrier disease (MD), a rare hypertrophic gastropathy, has been described in adult and pediatric patients. The histologic features, similar in the adult and pediatric forms of the disease, include foveolar hyperplasia of the gastric mucosa accompanied by mucus hypersecretion and glandular atrophy of the gastric body (1,2). Clinical findings include hypochlorhydria, hypoproteinemia with hypoalbuminemia, and edema accompanied by vomiting, nausea, and abdominal pain. A pattern of transforming growth factor(TGF) immunohistochemical overexpression has been described in the gastric mucosa of adults and children with MD (3,4). Despite these similarities, pediatric MD is a transient condition, usually resolving within 5 months, whereas adult MD is more chronic and may require gastrectomy for contol of symptoms (1,2). In approximately one third of children with MD, there is histologic evidence of gastric cytomegalovirus (CMV) infection (5–7) In adult MD, such infection has only rarely been demonstrated (8). Infection with H. pylori (HP) has been implicated in the pathogenesis of MD in adults, and eradication of the bacterium occasionally leads to resolution of the disease (9,10). HP infection has only rarely been associated with MD of childhood, but here too, HP eradication has been reported to result in the resolution of MD (11,12). A small number of MD cases have been reported in neonates (13–16). We report two additional cases MD that we encountered within a 7-year period, which were diagnosed at autopsy. Their clinical and pathologic features are compared with those of the cases previously published. TGFimmunohistochemical expression was assessed for the first time on the gastric mucosa of the patients and control subjects. CASE 1