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Protein-losing gastropathy is an uncommon disease of uncertain etiology, known also as Menetrier's disease. In medical literature only 50 pediatric cases have been described. These childhood forms, in contrast to classic adult Menetrier's disease, have a typical benign and transient course, and require only supportive therapy. The role of Cytomegalovirus (CMV) in the pathogenesis has been demonstrated by gastric biopsy in one third of the cases. Also other infectious, allergic and immunological factors have been hypothesized. We describe a case of hypertrophic gastropathy with important protein-loss, admitted to our Pediatric Department for evaluation because of vomit, weight loss, abdominal pain and hypoalbuminemia. Gastric mucosal biopsy revealed a morphological evidence of CMV infection.

We report a patient with Ménétrier's disease presenting with extensive subcutaneous oedema, ascites and pleural effusion due to hypoalbuminaemia. Gastric secretory studies showed no free basal and stimulated acid secretion. The gastric juice contained significant amounts of albumin (0.2 g/dl) and immunoglobulin G (IgG) (1.11 mg/dl), corresponding to an estimated daily loss of 9.7 g and 45 mg, respectively. Protein-losing gastropathy was initially unsuccessfully treated with famotidine (80 mg/day) for 17 months, but a long-term (25 months) clinical remission was subsequently achieved with omeprazole (20 mg/day). We suggest that excellent clinical remission of Ménétrier's disease and the associated protein-losing gastropathy may be obtained with long-term omeprazole maintenance treatment, possibly due to Helicobacter pylori suppression.

Unlabelled: There is as yet no consensus about the need for treatment of Helicobacter pylori infections, except for H. pylori-associated peptic ulcer. We present a 3-year-old boy with a 3-month history of oedema and hypoproteinaemia associated with hypertrophic gastropathy. Abdominal scintigram using intravenous 99mTc-labelled human serum albumin scintigraphy and direct measurement of protein in gastric juice proved that serum proteins were massively secreted in the stomach. Histological findings of mucosal inflammation were observed and H. pylori was isolated from the gastric mucosa. The protein loss promptly stopped within 2 weeks of the beginning of eradication therapy., Conclusion: Protein-losing hypertrophic gastropathy may be induced by Helicobacter pylori-associated gastritis and should therefore be carefully evaluated by histological and bacteriological examination to provide a basis for eradication of H. pylori.

Hypertrophic gastropathy--that is, Ménétrier's disease--was found, in a retrospective analysis, to be associated with Helicobacter pylori in more than 90% of patients. It is proposed that hypertrophic gastropathy represents a special form of H pylori gastritis in these patients. A case is described of a 28 year old woman with Ménétrier's disease associated with proved protein loss from the stomach. Treatment with cimetidine for more than three years had little benefit when colonisation by H pylori was detected. Density of H pylori colonisation and activity of gastritis, which was also present in the first biopsy specimens taken five years ago, were more pronounced in the body than in the antrum, which is in agreement with the characteristics of H pylori gastritis found in other cases with Ménétrier's disease. A 14 day antibacterial treatment course with 750 mg amoxicillin three times a day combined with 40 mg omeprazole three times a day was started in April 1991. This resulted in eradication of H pylori and the return to normal of giant folds and the mucosal histology. Serum protein concentrations returned to normal within six weeks and remained normal at two endoscopies during a two year follow up. This case report suggests that a subgroup of the patients with Ménétrier's disease may be healed by the eradication of H pylori.

A 47-yr-old man with Ménétrier's disease presented with profound enteral protein losses documented by 51Cr-albumin stool studies. There was no response to anticholinergics, H2-receptor antagonists, or proton pump inhibition. Subcutaneous octreotide acetate, 100 micrograms bid, resulted in clinical improvement with rapid resolution of enteral protein losses. Octreotide was continued for 12 months. During an additional 5-month follow-up period without therapy, the patient remained improved clinically with low to normal serum proteins, despite the persistence of gastric rugal hypertrophy. The evidence that octreotide resolved enteral protein losses and eliminated the need for urgent gastrectomy in our patient warrants a trial of this drug for other patients with Ménétrier's disease.

Hypoproteinaemia (total proteins 5.2 g/dl) and hypoalbuminaemia (2.7 g/dl) were demonstrated in a 44-year-old woman with leg oedema during the previous four months and 10-kg weight gain during the previous year. A protein-losing enteropathy was diagnosed, all other causes having been excluded. Gastroscopy revealed giant hypertrophic gastritis. A biopsy showed foveolar hyperplasia with some inflammatory infiltrates, typical of Ménétrier's disease. Endosonography excluded any additional infiltrative process of the bowel wall. Long-term treatment with ranitidine, 150 g twice daily, achieved normalization of total protein and albumin concentrations. But the macroscopic changes in the gastric wall were still recognizable on repeat gastroscopy. As there is a danger of malignant degeneration gastroscopy and, if indicated, endosonography should be repeated annually.

A 3-year-old boy with transient protein loss due to hypertrophic gastropathy is described. Serologic evidence of concomitant Mycoplasma pneumoniae infection was present. Although sometimes associated with infections, protein-losing hypertrophic gastropathy has not been reported previously in association with Mycoplasma pneumoniae infection.

Seven patients with giant hypertrophic gastropathy participated in a gastric intubation perfusion study to investigate the route and mechanism of protein leakage associated with this disease. All patients had gastric tight junctions wider than those in healthy controls. Acute administration of propantheline bromide reduced gastric albumin leakage (-50.7%, p less than 0.05) and concurrently decreased width of tight junctions (p less than 0.05) in all patients. Another antisecretory agent, cimetidine, had no consistent effect on protein leakage or on the width of tight junctions. Pentagastrin and bethanechol chloride increased protein loss but had no effect on the width of tight junctions. These results are consistent with the hypothesis that proteins may take a paracellular route via the tight junctions as they traverse the gastric mucosa and that this may have a cholinergic mechanism.