Surgical management of Menetrier's disease with protein-losing gastropathy.
Three patients with Menetrier's disease and protein-losing gastropathy who were studied during a 12 year period have been presented. The characteristic findings which differentiate them from patients with hypertrophic hypersecretory gastropathy, including the Zollinger-Ellison syndrome, are: 1) hypertrophy of gastric mucosa with giant rugal folds involving the fundus, cardia and body of the stomach but sparing the antrum; 2) muscosal hypertrophy consisting of gastric mjcus-secreting cells while parietal cells and chief cells are diminished in number and may be absent from many microscopic sections; 3) gastric secretion of large volume containing excess mucus, low to absent hydrochloric acid and protein concentration 5 or 6 times normal (1.7 mg/ml); 4) hypoalbuminemia and hypoglobulinemia due to loss of serum proteins fron gastric mucosa into the gastric lumen; 5) rare association with gastric ulcer. Unlike the Zollinger-Ellison syndrome none of our patients had duodenal ucler or multiple endocrine adenomatosis or a family history of these conditions. We have found no authenticated reports in the literature which document a relationship of Menetrier's disease ( as defined above) with multiple endocrine adenomatosis. Menetrier's disease with protein-losing gastropathy is a potentially lethal disorder of unknown cause with no specific treatment. Resection of the site of gastric protein losses as first done by Waugh is logical and effective. One of our three patients died in hospital before gastrectomy was done. Two others have done well for 11 months and 12 years, respectively, after total gastrectomy with Roux-en-Y esophagojejunostomy and Hunt-Lawrence jejunal pouch.
D000328:Adult; D001344:Autopsy; D001797:Blood Protein Electrophoresis; D004947:Esophagus / Q000000981:diagnostic imaging / Q000601:surgery; D005260:Female; D005743:Gastrectomy; D005753:Gastric Mucosa / Q000473:pathology; D005756:Gastritis / Q000150:complications / Q000000981:diagnostic imaging / Q000601:surgery*; D006801:Humans; D006984:Hypertrophy; D007583:Jejunum / Q000000981:diagnostic imaging / Q000601:surgery; D008297:Male; D011504:Protein-Losing Enteropathies / Q000150:complications / Q000000981:diagnostic imaging / Q000601:surgery*; D011859:Radiography; D013270:Stomach / Q000473:pathology; D013577:Syndrome