[Menetrier's disease and its association with gastric hyperplastic polyps]. / Enfermedad de Menetrier y su asociación a pólipos gástricos hiperplásicos.

Menetrier's disease represents a low prevalence clinical entity, characterized by complexity in its diagnosis, particularly due to the need to exclude its potential association with gastric cancer. In this context, we present the clinical case of a 54-year-old male with nonspecific gastrointestinal symptoms and hypoalbuminemia. During the upper endoscopy procedure, a noticeable thickening of gastric folds was observed, associated with multiple polypoid lesions in the stomach, predominantly in the fundus and body. Since the patient did not show improvement in symptoms and given the inability to rule out gastric cancer, total gastrectomy was chosen as the treatment. Surgical specimen and histology confirmed the presence of Menetrier's disease.

Survival Times of Patients With Menetrier's Disease and Risk of Gastric Cancer.

BACKGROUND & AIMS: Menetrier's disease is a rare acquired disorder associated with giant gastric folds along with protein-losing enteropathy, low stomach acid, or achlorhydria, and histologic features of massive foveolar hyperplasia. Little is known about the etiology, clinical features, or epidemiology of this disorder, including risk of gastric cancer. We investigated the outcomes and characteristics of patients with Menetrier's disease, including development of gastric cancer and survival times. METHODS: We performed a case-control study of all Menetrier's disease cases (n = 76; mean age, 56 ± 45 y; 59% male; mean body mass index, 24) diagnosed at Mayo Clinic, Rochester, MN, from January 1975 through 2005. Diagnosis of Menetrier's disease was based on a combination of clinical, endoscopic, radiologic, and histologic features. Patients with dyspepsia who underwent gastric biopsy analysis were included as controls. We obtained demographic, clinical history, laboratory, imaging, histopathology, and follow-up data from medical records. Clinical characteristics of Menetrier's disease were analyzed using descriptive statistics. The Kaplan-Meier method was used to estimate overall survival in cases. RESULTS: Clinical features found in a significantly higher proportion of patients with Menetrier's disease than controls included vomiting, abdominal pain, postprandial fullness, and weight loss of 10 lb or more. Smoking was associated with Menetrier's disease (P = .002 vs controls), but not alcohol use. Infection with Helicobacter pylori was not associated with Menetrier's disease (2.6% of patients vs 4.0% of controls; P = 1.00). There was no significant difference between patients with Menetrier's disease vs controls in proportions with inflammatory bowel disease. Gastric cancer developed in 8.9% of patients with Menetrier's disease by 10 years after the Menetrier's disease diagnosis vs 3.7% of controls over the same time period (P = .09). Of patients with Menetrier's disease, 72.7% and 65.0% survived for 5 and 10 years, respectively, compared with 100% of controls (P < .0001 for both time periods). CONCLUSIONS: In a case-control study of 76 patients with Menetrier's disease, we found this rare disorder to be associated with increased mortality. Patients with Menetrier's disease therefore should be followed up with surveillance endoscopy.

Menetrier disease and Cytomegalovirus infection in paediatric age: report of three cases and a review of the literature.

Paediatric Menetrier disease (PMD) is a protein-losing gastropathy, presenting with generalized oedema and abdominal symptoms. PMD commonly has an acute course and may be associated with Cytomegalovirus (CMV) infection. The aim of this retrospective study is to assess the epidemiological and clinical data, diagnostic procedures, treatment and outcome of CMV-associated PMD. The medical charts of the patients with PMD and CMV infection diagnosed at our hospital have been reviewed. Then, a systematic literature's review of all the cases of PMD and a selection of those associated with CMV infection have been performed. Three previously healthy boys were admitted for vomiting and oedema. Endoscopy showed hypertrophic gastric folds and CMV infection was diagnosed. Albumin was administered in all cases, with clinical resolution within few weeks. In literature, PMD has been described in 150 children and the association between CMV and PMD was found in 89 cases. Clinical and laboratory data, radiological and histological exams, therapy and outcome were reviewed.Conclusions: Basing on the present experience and on the current knowledge, PMD has a benign course without long-term sequelae. Although PMD is rare in children, we recommend paediatricians to consider CMV-related PMD when facing children with vomiting and diffuse oedema. What is Known: • Paediatricians should consider Menetrier disease (MD) when facing oedematous child complaining of abdominal symptoms with hypoalbuminemia, without proteinuria and liver dysfunction. • Typical ultrasound features (hypertrophic gastric folds) suggest such condition which requires endoscopy and biopsy for definitive diagnosis. What is New: • A familial susceptibility to CMV gastric infection has been recently suggested; thus when suspecting MD, the family history of gastric diseases should be investigated. • Menetrier disease has been found associated with other unusual conditions either benign (such as gastric bezoar) or malign as neoplasms (acute lymphatic leukaemia and adenocarcinoma) even in children.

Ménétrier's disease in childhood: a case report from China.

BACKGROUND: Ménétrier's disease (MD) is a protein-losing gastropathy characterized by gastric hypertrophy, foveolar hyperplasia and hypoalbuminemia. MD is uncommon in childhood with nonspecific clinical symptoms, and the exact cause of pediatric MD is still unclear. CASE PRESENTATION: Here, we reported a 4 year and 10-month boy presenting with MD from China. The patient was suffered with vomiting, abdominal pain, hypoproteinemia and edema. Laboratory tests showed that the boy was infected with Clostridium difficile (CD). Gastrointestinal endoscopy revealed giant gastric folds, and histological gastric biopsies showed foveolar hyperplasia with glandular atrophy, infiltration of eosinophils in the lamina propria of the patient. Finally, the boy was recovered after supportive therapy with intravenous albumin and CD eradication. CONCLUSION: For the nonspecific clinical symptoms of MD, gastrointestinal endoscopic evaluations with gastric tissue biopsies are required to establish the diagnosis of MD in children with unexplained hypoalbuminemia.

[An infrequent cause of iron-deficiency anemia: Ménétrier disease]. / Una causa infrecuente de anemia ferropénica: Enfermedad de Ménétrier.

Ménétrier disease is a rare condition of an unknown origin, with a wide spectrum of clinical signs and symptoms at presentation. Diagnosis is made through the combination of clinical, analytical, endoscopic and histological data. Surgery is the classical treatment. However, new medical approaches have emerged.

[Digestive insufficiency of the stomach in children from the positions of clinical morphology and its role in the assessment of the indications for nutritional support ].

The structure of gastric mucosa (GM) of the stomach fundus (SF) was studied in children with various gastrointestinal diseases. In children, the main structural parameters of the SF (GM thickness, depth of glands and thickness of the mucosal epithelium area) varied widely (3.5-5.3 times). The following ranges were allocated: hypotrophy ("atrophy"), eutrophy (area of mean values) and hypertrophy of SF GM thickness, depth of the glands and thickness of the mucosal epithelium area. Hypotrophy ("atrophy") of the SF glands was found in approximately one third of the children of different age which could lead to decrease in the digestive function of the stomach and cause specific clinical symptoms of dyspepsia. Atrophic changes of SF GM were observed in children of all age groups. Most often (49%), fundic glands hypotrophy was observed in children of early age. With age, the incidence of atrophic changes of SF GM decreased. Atrophic changes in the GM can be detected during microanatomical or histopathological (using morphometry) examination of the SF.

Menetrier's disease associated with Helicobacter pylori: three cases with sonographic findings and a literature review.

Menetrier's disease is a rare cause of protein-losing gastropathy characterised by hypertrophy of the gastric folds. Three cases are reported. Helicobacter pylori was detected by culture and CLO test in two cases and on follow-up gastroscopy in the other. The first two patients were given eradication therapy and hypertrophic gastropathy resolved whereas in the third the disease remitted prior to the eradication treatment. Typical sonographic features were thickened gastric wall with preservation of the wall stratification. A review of the literature found eight cases of Menetrier's disease associated with H. pylori in children and these cases are briefly reviewed. H. pylori infection should be considered in all children with Menetrier's disease and, if isolated, eradication treatment should be administered.

[Diffuse gastric cancer associated with Ménétrier's disease]. / Adenocarcinoma gástrico difuso asociado a enfermedad de Ménétrier.

BACKGROUND: Ménétrier's disease is a rare disease of the stomach generally described as hypertrophic gastropathy. Its etiology is unknown and its malignant potential is controversial. Only a few reports supporting its association with gastric cancer have been found. We present a case of gastric cancer associated with Ménétrier's disease. CASE REPORT: We present a 72 year-old-male with epigastric pain and early satiety during the last 5 months. He had been treated with proton pump inhibitors with unfavorable response and began with loss of weight and asthenia. An upper digestive endoscopy showed an erythematous nodular gastric mucosa, with enlarged folds. An abdominal CT scan demonstrated a circumferential parietal thickening of the gastric wall and adenopathies. A gastric macrobiopsy done by endoscopic mucosal resection evidenced a mucin infiltrating adenocarcinoma with invasion of the lamina propria. Subtotal gastrectomy was done. The histology showed a stomach with changes compatible with Ménétrier's disease and diffuse infiltration by a highly undifferentiated adenocarcinoma (plastic linitis). CONCLUSION: A patient with diffuse gastric adenocarcinoma and Ménétrier's disease is reported.

Distinguishing Ménétrier's disease from its mimics.

OBJECTIVE: Ménétrier's disease (MD) is a rare hypertrophic gastropathy characterised by giant rugal folds, hypochlorhydria, protein loss and a classic constellation of symptoms (nausea, vomiting, abdominal pain and peripheral oedema). It is considered a clinical diagnosis that may at times be difficult to establish. Firm diagnostic criteria for MD are proposed by delineating the clinicopathological features that best differentiate MD from its mimics. METHOD: 48 patients referred to Vanderbilt University Medical Center for consideration of enrolment in a clinical trial of treatment of patients with MD with cetuximab were evaluated for a definitive diagnosis by assessing the clinical presentation, pertinent laboratory values and histopathological features. RESULTS: MD was confirmed in 25 of the 48 patients (52%). The remaining 23 patients were considered to be mimics of MD, the most common diagnoses being gastric polyps or polyposis syndromes (13/23, 57%). Gastric slides were available from 40 of the 48 patients for detailed histological analysis (22/25 MD and 18/23 non-MD). Foveolar hyperplasia, glandular tortuosity and dilation, and a marked reduction in parietal cell number were present in all 22 cases of MD. Lamina propria smooth muscle hyperplasia and oedema characterised most cases (18/22 and 19/22, respectively). More than half had prominent eosinophils (11/22) and/or plasma cells (12/22) in the lamina propria. The clinical presentation of patients with MD was characterised by significantly younger age of onset, male predominance and increased vomiting compared with non-MD patients, and a lower prevalence of anaemia compared with MD patients with polyps. There was a trend towards increased frequency of peripheral oedema in patients with MD compared with non-MD patients. CONCLUSIONS: MD is most accurately diagnosed by clinicohistopathological analysis including oesophagogastroduodenoscopy with gastric pH, appropriate laboratory tests (complete blood count, serum albumin, serum gastrin, Helicobacter pylori and cytomegalovirus serology) and full-thickness mucosal biopsy of the involved gastric mucosa.

Ménétrier's disease in a patient with refractory ulcerative colitis: a clinical challenge and review of the literature.

Ménétrier's disease (MD) is a rare disease of the stomach, characterised by hypertrophic gastric folds leading to protein loss. The association with ulcerative colitis (UC) is rare but has been reported in the literature. We report a case of a 29-year-old male affected by UC with an additional diagnosis of MD 3 years after UC diagnosis. UC was refractory to several treatment lines (thiopurines, infliximab, vedolizumab and ustekinumab), and the patient underwent colectomy. Octreotide was administered for MD normalising blood biochemistry, but it was not effective in inducing endoscopic remission of the stomach. Treatment options in patients with MD and UC are discussed.

Menetrier's disease exacerbating ulcerative colitis and relieved by gastrectomy.

Ménétrier's disease (MD) is a rare gastropathy characterised by giant rugal folds which can present with nausea, vomiting, abdominal pain and protein losing gastropathy. We report a 21-year-old woman with comorbid MD and ulcerative colitis (UC). Management was complicated by limited treatment options for MD, significant symptom burden, worsening nutrition and difficulty determining which disease was the predominant cause of symptoms. Since age 18 the patient experienced recurrent UC flares characterised by diarrhoea, persistent vomiting and corticosteroid dependence. Endoscopic assessment demonstrated concurrent MD and active UC. Octreotide and cetuximab were trialled given persistent hypoalbuminaemia and suspicion for MD associated protein-losing gastropathy. UC management comprised dose-optimised infliximab and methotrexate. Repeat endoscopic assessment demonstrated improvement in UC without corresponding improvement in symptoms or hypoalbuminaemia. Nasojejunal feeding and parenteral nutrition failed to significantly improve nutritional status and accordingly the patient proceeded to radical total gastrectomy. Postoperatively, MD-associated symptoms and hypoalbuminemia resolved completely.

Ménétrier disease in an acquired immunodeficiency syndrome patient.

Ménétrier disease is a rare disorder of unknown etiology. An overexpression of TGF-alpha has been proposed to play a role in the pathophysiology. HIV-1 tat gene product has been shown to stimulate TGF-alpha production leading to a positive feedback autocrine loop. The case of a 41-year-old male with AIDS who presented with weight loss, abdominal pain, ascites, edema, nausea, vomiting, and diarrhea is discussed. A computed tomography (CT) scan of the abdomen showed avid enhancement of the stomach mucosa. Magnetic resonance angiography revealed gastric and small bowel distention with diffuse wall thickening. Biopsies of the stomach showed marked foveolar hyperplasia with active inflammation and gland changes consistent with Ménétrier disease.

[Clinical case: Ménétrier's disease of childhood]. / Le cas clinique du mois. Maladie de Ménétrier chez l'enfant.

We report the case of 3 years and 2 months old boy who presented vomiting associated with eyelid edema of recent onset. The paraclinical exploration has allowed us to establish the diagnosis of benign hypertrophic gastritis or Ménétrier's disease. The interest in this case resides in his rare appearance, benignity and transience, during the childhood, contrasted with the adult where chronic form and a risk of cancer predominates.

[A case of juvenile polyposis limited to the stomach accompanied by double gastric cancers and Ménétrier's disease].

A 48-year-old woman who had innumerable polyps in her stomach for 4 years visited our hospital to receive further examinations. Her younger brother had undergone gastrectomy because of cancer with gastric polyposis. Gastrography and gastroscopy showed numerous reddish polyps finger-like in form with much mucin in the antrum. On the other hand, polyposis in the corpus and fornix was nodular which formed giant folds. There was no polyps in other organs, therefore, we diagnosed juvenile polyposis limited to the stomach accompanied with Ménétrier disease. Double carcinoma were proved histologically after total gastrectomy. 55.8% of juvenile poplyposis limited to the stomach are accompanied with neoplasms, however, the rate of preoperatively correctly diagnosed neoplasms was 55%, so total gastrectomy was recommended.

Ménétrier's disease presenting as recurrent unprovoked venous thrombosis: a case report.

BACKGROUND: Acquired thrombophilia is a potential sequela of malignancy, chronic inflammation, and conditions characterized by severe protein deficiency (for example, nephrotic syndrome, protein-losing enteropathy). As such, venous thrombosis is often a feature, and occasionally a presenting sign, of systemic disease. Ménétrier's disease is a rare hyperplastic gastropathy that may lead to gastrointestinal protein loss and hypoalbuminemia. To date, reports of venous thrombosis associated with Ménétrier's disease are exceedingly scarce. CASE PRESENTATION: We report the case of a 40-year-old white man who presented with unprovoked deep venous thrombosis, pulmonary embolism, and renal vein thrombosis. Upon receiving therapeutic anticoagulation, he developed severe gastrointestinal bleeding, and endoscopic evaluation led to a diagnosis of Ménétrier's disease. A laboratory workup revealed deficiency of protein C, protein S, and antithrombin III, as well as markedly elevated levels of factor VIII. He was determined to have an acquired thrombophilia as a direct result of Ménétrier's disease. CONCLUSIONS: This case describes an acquired thrombophilic state in a patient with Ménétrier's disease and profound hypoalbuminemia. Although this association is rarely described, we discuss the probable mechanisms leading to our patient's thrombosis. Specifically, we posit that his gastrointestinal protein loss led to a deficiency of several anticoagulant proteins and a compensatory elevation in factor VIII, as occurs in nephrotic syndrome and inflammatory bowel disease. Of note, this patient's recurrent venous thrombosis was the initial clinical sign of his gastrointestinal pathology.