Abstract

Ménétrier's disease is an uncommon condition of unknown aetiology. It is characterized with giant hypertrophic mucosal folds of the stomach and associated with loss of protein from the mucosa. There is no enough data about genetic predisposition of Ménétrier disease. However, some paper regarding to familial occurence have been reported. We reported a case of Ménétrier disease with malnutrition and severe hypoproteinemia despite of treatment, its surgical treatment, and his sibling suggesting the possibility of genetic occurence for this condition. A male with a diagnosis of Ménétrier disease with severe hypoproteinemia was operated. Patient overcame some complications such as nasocomial pneumonia, hypoxic ischemic encephalopathy resulted in left monoparesis. He discharged from hospital after thirty days postoperatively. His son complaining some dispeptic symptoms performed gastroscopy and biopsises was diagnosed of mild hypertrophic gastritis, and is following for possibility of familial Ménétrier disease by periodical gastroscopy and biopsies. The risk of malignancy in Ménétrier disease is still controversial. However, total gastrectomy is still the most definitive treatment for Ménétrier's disease, especially for patients with uncontrollable severe protein loss.