[MENETRIER DISEASE].

Menetrier disease (MD) is a very rare stomach pathology of unknown etiology characterized by manifest hypertrophy of gastric mucosa. The main causes of MD are believed to be Helicobacter pylori and cytomegalovirus infections. The most frequent symptom is epigastric pain. Also common are peripheral oedema due to hypoalbuminemia and increased permeability of gastric mucosa. The main diagnostic signs of MD include diffusive enhancement of mucosal folds, foveolar hyperplasia and glandular atrophy with a decrease in the number of main and parietal cells, hypoalbuminemia and peripheral oedema. MD being a very rare condition, the optimal methodfor its treatment is unknown.

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.

Ménétrier's disease-like hypertrophic gastritis in two red-capped mangabeys (Cercocebus torquatus).

Chronic lymphoplasmacytic gastritis in two red-capped mangabeys (Cercocebus torquatus) at a zoological facility progressed to severe hypertrophic gastropathy similar to Ménétrier's disease that affects humans. Clinical signs included emesis, diarrhea, hunched posture consistent with abdominal pain, anemia, and hypoproteinemia. Large gastric masses were present and in one case created a gastric outflow obstruction. Both cases were positive for simian immunodeficiency virus and Helicobacter spp. were variably isolated, although the association with the hypertrophic gastropathy is unclear. Medical treatment had varying success and included sucralfate, H2 receptor antagonists, proton pump inhibitors, diet manipulations, and antibiotic therapies targeting Helicobacter spp. Surgical resection of a large portion of the stomach resulted in some palliative improvement in one case. Overall, this disease presented many challenges regarding identification, confirmation of diagnosis, and clinical management. Both aggressive medical and surgical treatments were unrewarding for long-term management of hypertrophic gastropathy in this pair of red-capped mangabeys and resulted in a poor prognosis in these cases.

[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.

Large gastric folds arising in polyposis syndromes / Grandes pliegues gástricos en síndromes de poliposis

Large gastric folds (LGF) can be caused by benign conditions as well as malignancies. Unfortunately, endoscopic features and biopsy results are often equivocal, making the diagnosis and management of large gastric folds difficult. Polyposis syndromes encompass a group of conditions in which multiple gastrointestinal polyps occur in the lumen of the gut. Large gastric folds are extremely rare in these syndromes. We present the case of a patient with polyposis who was found to have large gastric folds in the entire gastric fundus and body, mimicking malignancy. The patient’s medical history and endoscopic ultrasonography (EUS) with mucosal resection confirmed the diagnosis of a pre-malignant disease. The lesion was monitored by serial endoscopic ultrasonography and biopsy, abdominal computed tomography (CT), and positron emission and computed tomography (PET-CT) for 6 years. The lesion remained stable, with the exception of abnormal fluorodeoxyglucose uptake on PET-CT in the gastric folds, which was determined to be a false-positive sign. To date, the patient remains healthy. We further discuss the mechanisms underlying the formation of large gastric folds caused by polyposis syndromes. Helicobacter pylori (H. pylori) or cytomegalovirus (CMV) is unnecessary for this progression. Immunohistochemistry (IHC) staining suggested that overexpression of transforming growth factor alpha (TGF-Alpha) and down-regulation of myocyte enhancerbinding factor 2 (MEF2) may be involved in this case (AU)

No disponible

A case of Ménétrier's disease without Helicobacter pylori infection.

Ménétrier's disease (MD) is a rare, acquired, premalignant disorder of the stomach characterized by enlarged gastric folds with foveolar hyperplasia, the phenotype of antralization of gastric glands, hypochlorhydria and hypoproteinemia. The etiology of MD is unknown, but both increased signaling by transforming growth factor-α and infection with Helicobacter pylori (H. pylori) have been implicated. Here, a case involving 70-year-old man who lost weight after developing anorexia and diarrhea is reported. He was diagnosed as MD without H. pylori infection, and in spite of intensive care, he died 40 days after admission. An autopsy confirmed MD. Immunohistochemistry revealed overexpression of transforming growth factor-α in the foveolar region of the gastric mucosa. The autopsy also distinguished this H. pylori-negative MD from hyperplastic polyp of the stomach, which is important in clarifying the entity of H. pylori-negative MD.

A case of Ménétrier disease in a child.

Ménétrier disease is a protein-losing gastroenteropathy often misdiagnosed in the pediatric population. The disease is characterized by hypoalbuminemia secondary to protein loss through the gastrointestinal mucosa and resultant peripheral edema. It is important for emergency department practitioners to consider this diagnosis in the differential diagnosis for edema and low albumin levels in pediatric patients. We present a case report of Ménétrier disease in an edematous child and a brief review.

Ménétrier's disease of the stomach: a clinical challenge.

Ménétrier's disease is a rare hyperproliferative protein-losing gastropathy of the gastric foveolar epithelium. Most common symptoms include epigastric pain with fullness and vomiting, and generalized peripheral edema with hypoalbuminemia. Radiologically, the wall of the gastric body and fundus is diffusely thickened, often with antral sparing. Giant rugal edematous folds are seen on gastroscopy, and histology of biopsy material shows diffuse foveolar hyperplasia with cystic dilatation of the glandular portion of the gastric mucosa in the absence of significant inflammatory infiltrate. The recent discovery of transforming growth factor α overexpression opens the way of epidermal growth factor receptor blockade with cetuximab as first-line treatment modality in severe cases of Ménétrier's disease.

Menetrier's disease therapy: rebooting mucosal signaling.

Ménétrier's disease, a rare hyperproliferative disorder of the stomach, is associated with chronic abdominal pain, vomiting, weight loss, and edema, as well as an increased risk of gastric cancer. Therapy, other than surgical resection of the stomach, is limited to supportive measures and reflects the limited understanding of Ménétrier's disease pathogenesis. Data reported in this issue describe a promising targeted therapeutic approach and provide new insight into the causes of Ménétrier's disease.

Enfermedad de Ménétrier y edemas bilaterales en un paciente joven / Menetrier’s disease and bilateral oedemas in a young patient

Introducción. La enfermedad de Ménétrier es una gastropatía hiperplásica de aparición excepcional, que se caracteriza por un engrosamiento marcado de los pliegues gástricos, a expensas fundamentalmente de una hiperplasia foveolar. La presentación clínica más frecuente es dolor epigástrico, náuseas o vómitos. Se ha descrito una elevada prevalencia de infección por Helicobacter pylori (90%), y se ha comprobado una mejoría clínica, analítica e histológica tras la erradicación de éste. Caso clínico. Varón de 45 años que consultó por edemas de dos meses de evolución en los miembros inferiores. La ecografía Doppler de los miembros inferiores y la linfogammagrafía isotópica fueron normales. En la analítica se observó una marcada hipoproteinemia, y entre los estudios adicionales destacaba una serología positiva para H. pylori. Se le realizó un tránsito esofagogastroduodenal donde se observó un engrosamiento marcado de los pliegues gástricos, que se confirmó con gastroscopia y biopsia. El examen anatomopatológico objetivó la existencia de una gastritis crónica mixta con hiperplasia foveolar focal, junto con la presencia de H. pylori. Se instauró un tratamiento erradicador, y el paciente mostró una mejoría significativa de los edemas y una normalización de la cifra de proteínas. Conclusión. Presentamos un caso de enfermedad de Ménétrier, cuya principal manifestación clínica –a diferencia de lo habitual– son los edemas periféricos. Cabe reseñar la importancia de incluir las gastroenteropatías pierdeproteínas dentro del diagnóstico diferencial de edemas periféricos sin causa vascular

Introduction. Ménétrier’s disease is rare hyperplastic gastropathy that is characterised by a notable thickening of the gastric folds, mainly due to foveolar hyperplasia. The most frequent clinical presentation is epigastric pain, nausea or vomiting. A high rate of prevalence of infection by Helicobacter pylori (90%) has been reported and clinical, analytical and histological improvement is observed once this has been eradicated. Case report. A 45-year-old male who visited due to a two-month history of oedemas in the lower limbs. Results of Doppler ultrasound scans of the lower limbs and isotopic lymphoscintigraphy imaging were normal. Lab tests revealed a notable hypoproteinemia and one of the most significant findings in the additional studies was positive serology for H. pylori. The upper gastrointestinal series that was performed revealed a notable thickening of the gastric folds, which was confirmed by means of gastroscopic and biopsy tests. A pathological examination revealed the existence of a chronic non-specific gastritis with focal foveolar hyperplasia, together with the presence of H. pylori. Treatment was established to eradicate the infection and the patient showed significant improvement with regard to the oedemas and normalisation of the protein count. Conclusion. We report a case of Ménétrier’s disease in which, uncharacteristically, the main clinical feature is the presence of peripheral oedemas. It is important to include protein-losing gastroenteropathies within the differential diagnosis of peripheral oedemas that have no vascular causation

Enfermedad de menetrier: presentación casos clínicos / Menetriers disease: presentation clinical cases

Enfermedad de Ménétrier en niños es poco frecuente y se caracteriza por la presencia de una gastropatía perdedora de proteínas asociada a un aumento y engrosamiento de los pliegues gástricos. Se ha sugerido como posible etiología para ésta enfermedad una alteración en la regulación normal del crecimiento del epitelio gástrico, probablemente disparado por un agente infeccioso. Se reporta el caso de un niño de 7 años de edad con historia de vómitos, epigastralgia, edema periorbitario, de miembros inferiores e hiporexia de 3 semanas de evolución. Las pruebas de laboratorio revelaron hipoproteinemia. En la endoscopia digestiva superior se observó engrosamiento marcado de los pliegues de la mucosa gástrica de fundus y cuerpo, el estudio histopatológico demostró hiperplasia foveolar marcada con incremento de las células productoras de moco. Se descartó etilogía infecciosa. Su condición clínica mejoró progresivamente posterior al tratamiento con omeprazol

High-resolution real-time compound ultrasound imaging of transient protein-losing gastropathy of childhood.

We report the case of a 1.5-year-old girl with transient protein-losing gastropathy with hypertrophic gastric folds (PLGH). The diagnosis of PLGH was made by abdominal ultrasound (US) and not by an upper gastrointestinal (UGI) study as reported in many previous publications. Real-time compound ultrasound imaging showed in high detail the echogenic thickening of the mucosal gastric layer and associated hyperaemia on colour Doppler US. These ultrasonic findings highly correlated with the endoscopic US findings and microscopic changes of the gastric wall in PLGH. An awareness of the high-resolution abdominal ultrasound appearances of PLGH may facilitate earlier diagnosis and obviate the need for an upper GI contrast series.

[Ménétriér's disease in children].

Ménétriér's disease in children is a rare disorder that is characterized by the presence of marked protein losing gastropathy associated with enlarged and thickened gastric folds. Abnormal regulation of gastric epithelial growth, probably triggered by an infectious agent, has been suggested as an etiology for this disorder. We describe a case of Ménétriér's disease in a young child and review the current literature encompassing the different aspects of the disease.

Menetrier's disease.

An 8-year-old girl presented with persistent vomiting, pain abdomen and generalized edema. Barium studies and gastroscopy suggested hypertrophic gastropathy. Histopathological examination pointed the diagnosis towards Menetrier's Disease. There was spontaneous remission and the child required only supportive therapy.