Abstract

Objective: Menetrier’s disease, also called hypoproteinemic hypertrophic gastropathy, is a rare, acquired, premalignant disorder of the stomach. It is generally characterized by giant hypertrophic folds that most often involve the fundus, excessive mucus secretion, decreased acid secretion (hypochlorhydria), and hypoproteinemia due to selective loss of serum proteins across the gastric mucosa. Aims and methods: A four year old boy was referred to our Pediatric Gastroenterology and Liver Unit because of iron deficiency anemia. The child had been healthy since his birth; no illness during the delivery or the neonatal period were accounted. He had been breastfed till 12 months of age, his physical and psychological development was unremarkable and his growth curves were within the normal ranges. At medical history, it was reported that his grandfather suffered from Menetrier’s disease. Therefore, on suspicion of chronic obscure gastrointestinal bleeding, we performed upper gastrointestinal tract (GI) endoscopy which disclosed markedly thickened gastric folds with nodularity, erythema, and exudate involving the proximal stomach (corpus and fundus); the antrum and pylorus did not show pathological features. Histological specimens of the stomach obtained by multiple superficial cold forceps biopsies showed only foveolar hyperplasia and dilatation of some glands. Helicobacter pylori was not found. Cytomegalovirus (CMV) PCR on gastric aspiration, serum and urine did not show the presence of viral load. Results: After one month, we re-performed upper GI endoscopy in order to obtain a wider sample for histological examination by jumbo forceps. At this time, the pathologist identified marked foveolar hyperplasia and elongation of gastric pits, cystic dilatation of gastric gland in the deeper part of the mucosa, moderate atrophy of fundic glands; in the lamina propria there was no inflammation, but marked dilatation and congestion of small vessels, and diffuse edema has been observed. These findings suggested the diagnosis of Menetrier’s disease, and the child was thus treated with Octreotide longacting release (LAR) which brought to clinical amelioration of the anemia. Conclusion: The cause of Menetrier’s disease is unknown, although in children infection by CMV and by Helicobacter Pylori have been implicated. Classical symptoms include epigastric pain, vomiting, edema, anorexia, and weight loss. In this case of pediatric Menetrier’s disease, the only clinical feature was iron deficiency anemia, probably due to congested small vessel leakage. We might speculate that this child had not expressed yet all the other features of the disease, such as the protein-loss syndrome, because of his early diagnosis. The medical treatment of Menetrier’s disease consists of Octreotide administration, which is a somatostatin analogue, and in our case successfully controlled hemorrhage from gastric lesions.

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