Menetrier's disease. A diagnostic and therapeutic challenge (original) (raw)
Related papers
Ménétrier’s Disease and Its Atypical Presentation in Four Siblings
Cureus
Ménétrier's disease is a rare, acquired, premalignant disorder of the stomach distinguished by hypertrophic giant mucosal folds in the proximal part of the stomach (body and fundus), diminished acid secretion and excessive mucous production, resulting in hypoalbuminemia. Menetrier's is also known as hypoproteinaemic hypertrophic gastropathy and giant hypertrophic gastritis. We present a case of four siblings who presented between 2010 and 2017 with symptomatic iron deficiency anaemia or upper gastrointestinal symptoms with confirmed Ménétrier's disease. The etiology of Ménétrier's disease is unknown but it has been linked to raised TGF-α (transforming growth factor alpha protein) in the gastric mucosa causing increased signalling of epidermal growth factor. It is also associated with infections such as cytomegalovirus and Helicobacter pylori. Despite these associations, there have been cases without any of these viruses detected. Diagnosis is usually made by barium swallow or endoscopic evaluation, which characteristically shows thickened gastric folds, while the biopsies' hallmark feature is massive foveolar hyperplasia. We present a case series of four siblings who presented between 2010 and 2017 with symptomatic iron deficiency anaemia or upper gastrointestinal symptoms with confirmed Ménétrier's disease.
A Rare Etiology of Abnormally Large Gastric Folds: Menetrier’s Disease
Case Reports in Gastrointestinal Medicine, 2019
Menetrier’s disease (MD) is described as hypertrophied giant gastric folds causing hypoproteinemia due to protein losing gastropathy and is associated with higher risk of gastric adenocarcinoma. We present a case of a 58-year-old male who presented to our clinic with Melena and endoscopic work up showed enlarged gastric folds and erythematous mucosa in the antrum and three nonbleeding angioectasias in the duodenum. Mucosa biopsies were negative for H. pylori infection. He underwent polypectomy which showed fundic gland polyps. After 1½ years, EGD was repeated for abnormal computerized tomography of abdomen which showed enlarged gastric folds and biopsy revealed gastric fundic mucosa with foveolar hyperplasia, dilated fundic glands, and chronic gastritis. Stomach biopsy results were consistent with MD. Our patient had progressive disease in one and half years. It is important to follow patient with large gastric folds regular as they can develop MD over time which has increased risk ...
An Unusual Presentation of Ménétrier's Disease
Ménétrier's Disease (MD) is a rare acquired hypertrophic gastropathy characterized by giant hypertrophic rugal folds, hypochlorhydria, and hypoproteinemia. The definitive etiology of MD is controversial, although infection with Helicobacter pylori (H. pylori) has been implicated in adults. It presents as a constellation of symptoms including epigastric pain, fatigue, vomiting, weight loss, anorexia, and edema. None of these signs and symptoms is specific for the disease. The gastrointestinal symptoms and the degree of hypoalbuminemia can be profound, the latter resulting from the leakage of protein from the gastric lining. The disease is more common in males. Herein, we report a case of a young woman presenting with the chief complaint of peripheral edema with minimal gastrointestinal symptoms, which was diagnosed as MD on endoscopic evaluation and histopathological examination of gastric biopsy. A high index of suspicion is needed to correctly diagnose this condition for its optimal management.
Menetrier’s disease: A rare diagnosis established by ultrasonography and computed tomography
International journal of radiology and diagnostic imaging, 2022
Menetrier's disease is a rare condition of the stomach characterized by giant mucosal folds in the gastric fundus and body, diminished acid secretory capacity, and a protein-losing state with hypoalbuminemia. It is also called protein-losing hypertrophic gastropathy. It was first described by the French pathologist Pierre Menetrier in 1888. Menetrier's disease has also a recognized premalignant potential although the precise risk of progression to gastric cancer is not known. Several studies reported regression of disease after treatment with the monoclonal antibody against the EGFR receptor. But the only satisfactory treatment has historically been and remains the surgical intervention with total or partial gastrectomy. A case report of A 10-year male presented to the emergency room with a history of progressively increasing abdominal pain on and off for 1 month with epigastric region fullness, nausea, and vomiting. Generalized edema was noted on examination. An abdominal ultrasound examination was performed, and the patient was found to have diffuse mucosal thickening of the gastric wall with prominent rugal folds and echogenic mucosal lining with generalized subcutaneous edema and mild ascites. The patient underwent surgery and the diagnosis was confirmed by the histopathological report.
Menetrier’s Disease: a Surgical and Pathological Challenge
SAS Journal of Surgery, 2022
Menetrier’s disease (MD) is a rare hypertrophic gastropathy clinically characterized by digestive signs as epigastric pain, nausea, vomiting and diarrhea, with hypoalbuminemia and anemia, endoscopically by giant rugal folds and histologically by tortuous foveolar hyperplasia with dilated glands and hypertrophic muscularis mucosae. The current study presents a case of MD in 44 year-old-man admitted to the hospital of Hassan II in Fez, Morocco with epigastralgia, vomiting and weight loss. Physical examination fond a pale face and anemia in laboratory studies. A total gastrectomy was performed given the resistance to treatment and the persistence of symptoms, as well as the suspect endoscopic aspect. Macroscopic examination showed hypertrophic and polyploid gastric mucosa resembles cerebral convolutions. Histological examination showed a foveolar hyperplasia, tortuosity and dilatation of the glands. Hypertrophic muscularis mucosae with smooth muscle bundles extending into the lamina pr...
Journal of Clinical Investigation, 1992
Transforming growth factor a (TGFa) is thought to participate in the normal and pathologic processes of numerous tissues, including the gastric mucosa. To explore its role in vivo, transgenic mice were generated overexpressing TGFa in the stomach. TGFa induced dramatic structural and functional lesions of the glandular stomach that were similar to Menetrier's disease in humans. Transgenic mice developed severe adenomatous hyperplasia that resulted in a striking nodular thickening or hypertrophy of the gastric mucosa. Secretions obtained from affected stomachs contained no detectable gastric acid, suggesting that parietal cell function had been greatly impaired. These findings demonstrate that overproduction of TGFa can stimulate cellular proliferation, suppress acid secretion, and perturb organogenesis ofthe stomach oftransgenic mice. Moreover, TGFa may contribute to the pathogenesis of related human hypertrophic gastropathies, such as Menetrier's disease.
Gastroenterology, 2005
BACKGROUND & AIMS: Increase of intramucosal transforming growth factor alpha (TGFalpha) levels in the gastric fundus leads to oxyntic atrophy and massive foveolar hyperplasia in both metallothionein (MT)-TGFalpha mice and patients with Ménétrier's disease. We have evaluated the hypothesis that increased levels of TGFalpha in the fundus induces an antral pattern of cell differentiation in fundic glands by studying Pdx1, a transcription factor whose expression normally is confined to the gastric antrum.METHODS: Induction of Pdx1 expression was evaluated in Pdx1(lacZ/+)/MT-TGFalpha bigenic mice treated with zinc. The distribution of Pdx1 in MT-TGFalpha mice and Ménétrier's disease patients was evaluated with anti-Pdx1 antibodies. Transcript levels were evaluated by quantitative polymerase chain reaction in mouse and human tissues and AGS cells.RESULTS: In Pdx1(lacZ/+) mice, Pdx1 was expressed in antral mucosal cells including gastrin cells and TFF2-expressing deep glandular mucous cells. Zinc treatment for 2 to 8 weeks in Pdx1(lacZ/+)/MT-TGFalpha transgenic mice resulted in expression of Pdx1 throughout the fundus. No ectopic fundic Pdx1 expression was observed in either H. felis-infected or DMP777-treated mice. In MT-TGFalpha mice, 8 weeks of zinc treatment elicited nuclear Pdx1 staining throughout the fundic mucosa. TGFalpha treatment in AGS cells led to increases in Pdx1 and gastrin messenger RNA expression. Fundic sections from Ménétrier's disease patients showed nuclear Pdx1 staining throughout the fundic glands. Treatment of a Ménétrier's disease patient with an anti-epidermal growth factor receptor monoclonal antibody reduced fundic expression of both Pdx1 and gastrin.CONCLUSIONS: Overexpression of TGFalpha in MT-TGFalpha mice and Ménétrier's disease patients elicits ectopic expression in the fundus of Pdx1, consistent with the phenotype of antralization.