[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.

Metastatic gastric adenocarcinoma associated with hypertrophic gastropathy resembling Ménétriers disease in an adult Labrador Retriever dog

We describe a case of hypertrophic gastropathy (Ménétriers like disease) with metastatic gastric adenocarcinoma in a seven-year-old intact female Labrador Retriever dog. The animal suddenly presented with emesis and died. Gross lesions included a marked diffuse thickening of the gastric mucosa and an ulcerated transmural neoplastic mass in the gastric body. Gastric body and fundus were affected by foveolar hyperplasia with loss of chief and parietal cells replaced by mucous cells and marked dilatation of gastric glands. An area of gastric adenocarcinoma with submucosal lymphatic vessels invasion was also present and metastases were observed in the gastric lymph nodes, small intestine, pancreas, lung and liver. Due to its similarity with other gastric proliferative disorders, including this condition in the list of differentials is a necessary step in the diagnostic investigation of canine gastropathies.(AU)

Metastatic gastric adenocarcinoma associated with hypertrophic gastropathy resembling Ménétrier’s disease in an adult Labrador Retriever dog

We describe a case of hypertrophic gastropathy (Ménétrier’s like disease) with metastatic gastric adenocarcinoma in a seven-year-old intact female Labrador Retriever dog. The animal suddenly presented with emesis and died. Gross lesions included a marked diffuse thickening of the gastric mucosa and an ulcerated transmural neoplastic mass in the gastric body. Gastric body and fundus were affected by foveolar hyperplasia with loss of chief and parietal cells replaced by mucous cells and marked dilatation of gastric glands. An area of gastric adenocarcinoma with submucosal lymphatic vessels invasion was also present and metastases were observed in the gastric lymph nodes, small intestine, pancreas, lung and liver. Due to its similarity with other gastric proliferative disorders, including this condition in the list of differentials is a necessary step in the diagnostic investigation of canine gastropathies.

Menetrier's disease. A diagnostic and therapeutic challenge.

We present a rare case of hypertrophic gastropathy associated with protein loss. A 35-year-old man was hospitalized for bowel habit changes, abdominal pain, generalized edema and symptomatic anemia. Pertinent laboratory findings included iron deficiency anemia (Hb 6.7g/dl, ferritin 5 ng/ml) and marked hypoalbuminemia (albumin 2.5 g/dl). Endoscopic biopsy samples of giant gastric folds observed along the greater gastric curvature revealed foveolar hyperplasia and significant parietal cell loss. Endoscopic ultrasonography showed gastric parietal thickening with preserved architecture and normal gastric wall layers. Menetrier disease was diagnosed and the patient treated with cetuximab, a monoclonal antibody that inhibits ligand binding of transforming growth factor alpha (TGFa), preventing gastric mucosa cell proliferation. After twelve months of treatment, the patient referred symptoms improvement, and gastric biopsy levels of the proliferation marker protein Ki-67 had decreased.

Presentamos un caso infrecuente de gastropatía hipertrófica asociada a pérdida de proteínas. Un hombre de 35 años fue hospitalizado por cambios en los hábitos intestinales, dolor abdominal, edema generalizado y anemia sintomática. Los hallazgos de laboratorio pertinentes incluyeron anemia ferropénica (Hb 6.7 g/dl, ferritina 5 ng/ml) e hipoalbuminemia marcada (albúmina 2.5 g/dl). Las muestras de biopsia endoscópica de pliegues gástricos gigantes observados a lo largo de la curvatura mayor gástrica revelaron hiperplasia foveolar y pérdida significativa de células parietales. La ecografía endoscópica mostró engrosamiento parietal gástrico con arquitectura conservada y capas de pared gástrica normales. Se diagnosticó enfermedad de Menetrier y se trató al paciente con cetuximab, un anticuerpo monoclonal que inhibe la unión del ligando del factor de crecimiento transformante alfa (TGFa), evitando la proliferación de células de la mucosa gástrica. Después de doce meses de tratamiento, el paciente refirió mejoría de los síntomas y los niveles de la proteína marcadora de proliferación Ki-67 en biopsia gástrica habían disminuido.

Menetrier's disease. A diagnostic and therapeutic challenge / Enfermedad de Menetrier. Un desafío diagnóstico y terapéutico

Abstract We present a rare case of hypertrophic gastropathy associated with protein loss. A 35-year-old man was hospitalized for bowel habit changes, abdominal pain, generalized edema and symptomatic anemia. Pertinent laboratory findings included iron deficiency anemia (Hb 6.7g/dl, ferritin 5 ng/ml) and marked hypoalbuminemia (albumin 2.5 g/dl). Endoscopic biopsy samples of giant gastric folds observed along the greater gastric curvature revealed foveolar hyperplasia and significant parietal cell loss. Endoscopic ultrasonography showed gastric parietal thickening with preserved architecture and normal gastric wall layers. Menetrier disease was diagnosed and the patient treated with cetuximab, a monoclonal antibody that inhibits ligand binding of trans forming growth factor alpha (TGFa), preventing gastric mucosa cell proliferation. After twelve months of treatment, the patient referred symptoms improvement, and gastric biopsy levels of the proliferation marker protein Ki-67 had decreased.

Resumen Presentamos un caso infrecuente de gastropatía hipertrófica asociada a pérdida de proteínas. Un hombre de 35 años fue hos pitalizado por cambios en los hábitos intestinales, dolor abdominal, edema generalizado y anemia sintomática. Los hallazgos de laboratorio pertinentes incluyeron anemia ferropénica (Hb 6.7 g/dl, ferritina 5 ng/ml) e hipoal buminemia marcada (albúmina 2.5 g/dl). Las muestras de biopsia endoscópica de pliegues gástricos gigantes observados a lo largo de la curvatura mayor gástrica revelaron hiperplasia foveolar y pérdida significativa de células parietales. La ecografía endoscópica mostró engrosamiento parietal gástrico con arquitectura conservada y capas de pared gástrica normales. Se diagnosticó enfermedad de Menetrier y se trató al paciente con cetuximab, un anticuerpo monoclonal que inhibe la unión del ligando del factor de crecimiento transformante alfa (TGFa), evitando la proliferación de células de la mucosa gástrica. Después de doce meses de tratamiento, el paciente refirió mejoría de los síntomas y los niveles de la proteína marcadora de proliferación Ki-67 en biopsia gástrica habían disminuido.

Infección por citomegalovirus y Helicobacter pylori, en un niño con enfermedad de Ménétrier / Citomegalovirus and Helicobacter pylori infection in a child with Ménétrier´s disease

Resumen La enfermedad de Ménétrier es una entidad clínica rara, de etiología no clara, caracterizada por hiperplasia foveolar gástrica asociada con pérdida secundaria de proteínas. En niños se presenta con edema, que puede ser generalizado, sin compromiso renal ni hepático y difiere de la forma adulta por la persistencia del edema y la remisión espontánea. En la mayoría de casos publicados, se relaciona con infecciones, usualmente por cytomegalovirus y Helicobacter pylori. Aquí se presenta el caso de un niño de 9 años, que consulta por un mes de evolución de edema y dolor abdominal. Al examen físico se documenta anasarca, y estudios de laboratorio revelaron hipoalbuminemia sin proteinuria.

Abstract Ménétrier disease is a rare disorder characterized by gastric foveolar hyperplasia associated with secondary protein loss. In children, this condition presents as an edematous syndrome without renal or hepatic impairment and differs from the adult form by the constant presence of edema and spontaneous remission. It has been related to infections in most published cases, especially to cytomegalovirus and Helicobacter pylori. Here we analyze the case of a 9 year old boy, complaining of one month of edema and abdominal pain. Physical exam revealed a patient with anasarca and laboratory results documented hypoalbumenemia without proteinuria.

Enfermedad de Ménétrier. Presentación de un caso y revisión de la literatura / Ménétrier's disease. Case report and literature review / Doença de Ménétrier. Apresentação de caso e revisão de literatura

RESUMEN Se presentó el caso clínico de un adulto de 56 años de edad, atendido en el Servicio de Cirugía del Hospital Clínico Quirúrgico Docente "Dr. Ambrosio Grillo", provincia Santiago de Cuba, que fue operado de urgencia con el diagnóstico preoperatorio de cáncer gástrico complicado. El estudio anatomopatológico no confirmó dicho diagnóstico y sí evidenció enfermedad de Ménétrier. El paciente no presentó complicaciones en su evolución posoperatoria, por lo que se egresó 7 días después.

ABSTRACT A clinical case of a 56-year-old adult, was treated at the Surgery Service of the Teaching Surgical Clinical Hospital "Dr. Ambrosio Grillo", Santiago de Cuba province, which was operated urgently with the preoperative diagnosis of complicated gastric cancer. The pathological study did not confirm this diagnosis and did show Ménétrier disease. The patient did not present complications in his postoperative evolution, so he discharged the hospital 7 days later.

RESUMO O caso clínico de um adulto de 56 anos, atendido no Serviço de Cirurgia do Hospital Clínico Cirúrgico de Ensino "Dr. Ambrosio Grillo", província de Santiago de Cuba, operada com urgência com o diagnóstico pré-operatório de câncer gástrico complicado. O estudo patológico não confirmou esse diagnóstico e mostrou a doença de Ménétrier. O paciente não apresentou complicações em sua evolução pós-operatória e, assim, aos 7 dias ele saui do hospital.

Enfermedad de Ménétrier, reporte de caso (con video) / Menetrier disease: Case report with video

Resumen La enfermedad de Ménétrier, también conocida como gastritis hipertrófica gigante o gastropatía hipertrófica hipoproteinémica, es una entidad poco frecuente, caracterizada por una gastroenteropatía perdedora de proteínas, hipoclorhidria y engrosamiento de los pliegues mucosos del fondo y el cuerpo gástrico; es causante de un grupo clásico de síntomas que incluyen náuseas, vómitos, dolor abdominal y edema periférico; se asocia con un mayor riesgo de cáncer gástrico, sin embargo, su fisiopatología aún no está del todo esclarecida y su diagnóstico, clínico y endoscópico, puede llegar a ser difícil de establecer, por lo que se describe un caso clínico y se presenta una revisión sucinta de la literatura.

Abstract Menetrier disease (also known as giant hypertrophic gastritis or hypoproteinemic hypertrophic gastropathy) is a rare entity characterized by protein losing enteropathy, hypochlorhydria and thickening of the mucosal folds of the fundus and the gastric corpus. Its constellation of classic symptoms includes nausea, vomiting, abdominal pain and peripheral edema, and it is associated with increased risk of gastric cancer. Nevertheless, its pathophysiology is not yet fully understood and clinical and endoscopic diagnosis can be difficult to establish. This article describes a clinical case and provides a brief review of the literature.

Enfermedad de Ménétrier en pacientes pediátricos secundaria a una infección por citomegalovirus: Presentación de dos casos clínicos en un centro de alta complejidad / Ménétrier's disease in pediatric patients secondary to cytomegalovirus infection: Presentation of two clinical cases in a high complexity center

La enfermedad de Ménétrier es una gastroenteropatía perdedora de proteínas. Definida como una entidad rara y de causa desconocida, la mayoría de los casos reportados la han asociado a infecciones virales. En los pacientes pediátricos, presenta un comienzo agudo con un curso benigno y autolimitado. Se caracteriza por tener pliegues gástricos engrosados que, generalmente, involucran el cuerpo y el fundus gástrico, asociados a hipoalbuminemia, debido a la pérdida de proteína sérica a través de la mucosa. A continuación, se exponen dos casos clínicos de síndrome de Ménétrier infantil asociado a infección por citomegalovirus.

Ménétrier's disease is a protein losing gastroenteropathy. Defined as a rare entity with an unknown cause, most of the reported cases have been associated with viral infections. In pediatric patients, it is characterized by an acute onset with a benign and self-limiting course. It is characterized by thickened gastric folds that generally involve the body and the gastric fundus, associated with hypoalbuminemia due to the loss of serum protein through the mucosa. The following are two clinical cases of infant Ménétrier syndrome associated with cytomegalovirus infection.

[Ménétrier's disease in pediatric patients secondary to cytomegalovirus infection: Presentation of two clinical cases in a high complexity center]. / Enfermedad de Ménétrier en pacientes pediátricos secundaria a una infección por citomegalovirus: Presentación de dos casos clínicos en un centro de alta complejidad.

Ménétrier's disease is a protein losing gastroenteropathy. Defined as a rare entity with an unknown cause, most of the reported cases have been associated with viral infections. In pediatric patients, it is characterized by an acute onset with a benign and self-limiting course. It is characterized by thickened gastric folds that generally involve the body and the gastric fundus, associated with hypoalbuminemia due to the loss of serum protein through the mucosa. The following are two clinical cases of infant Ménétrier syndrome associated with cytomegalovirus infection.

La enfermedad de Ménétrier es una gastroenteropatía perdedora de proteínas. Definida como una entidad rara y de causa desconocida, la mayoría de los casos reportados la han asociado a infecciones virales. En los pacientes pediátricos, presenta un comienzo agudo con un curso benigno y autolimitado. Se caracteriza por tener pliegues gástricos engrosados que, generalmente, involucran el cuerpo y el fundus gástrico, asociados a hipoalbuminemia, debido a la pérdida de proteína sérica a través de la mucosa. A continuación, se exponen dos casos clínicos de síndrome de Ménétrier infantil asociado a infección por citomegalovirus.

Gastropatía hipertrófica por IgG4: reporte del primer caso en Chile / IgG4 related hypertrophic gastropathy: report of one case

IgG4 related disease is a systemic autoimmune disease, which can affect different organs. The most common digestive manifestation is autoimmune pancreatitis (AIP), followed by involvement of bile ducts and the major papilla. The stomach is only rarely involved. We report a 71-year-old diabetic woman consulting for jaundice and weight loss. Abdominal CAT scan, cholangio resonance imaging, endosonography and a serum IgG4 of five times the normal value, lead to the diagnosis of an autoimmune pancreatitis. An upper gastrointestinal endoscopy showed a diffuse thickening of gastric folds. The pathological study confirmed the presence of IgG4 positive plasma cells. The patient was successfully treated with steroids.

Enfermedad de Ménétrier en niños: reporte de dos casos / Two cases of Menétrier's disease in children

Resumen Se presentan 2 casos de enfermedad de Ménétrier (EM) remitidos a nuestra institución por síndrome edematoso. Esta enfermedad de poca prevalencia es una gastropatía hipertrófica perdedora de proteínas que en la mayoría de los casos es de causa desconocida, aunque se ha asociado con procesos infecciosos. Se caracteriza por edema, hipoproteinemia, hipoalbuminemia y, en la infancia, es de carácter benigno y autolimitado.

Abstract We present two cases of Menétrier's Disease (MS) referred to our institution due to edema. The prevalence of this disease is low. It is a hypertrophic gastropathy which causes loss of proteins and which has unknown causes in the majority of cases although it has been associated with infectious processes. It is characterized by edema, hypoproteinemia, hypoalbuminemia. In childhood it is benign and self-limited.

Enfermedad de Menetrier como entidad poco común / Ménétrier's disease as a rare entity

La enfermedad de Menetrier se define como la poliposis gástrica múltiple. Es una entidad infrecuente y se caracteriza por pólipos hiperplásicos de menos de 2 centímetros de diámetro, que rara vez se malignizan. Se presenta una paciente con enfermedad de Menetrier, que llevó seguimiento endoscópico durante tres años y debido al aumento progresivo del número de pólipos y la sintomatología se realizó una gastrectomía subtotal sin complicaciones intra ni posoperatorias(AU)

The Ménétrier's disease is defined as multiple gastric polyposis. It is an infrequent entity characterized by hyperplastic polyps less than 2 centimeters in diameter, which rarely become malignant. We present a patient with Ménétrier's disease, who underwent endoscopic follow-up for three years and, due to the progressive increase in the number of polyps and the symptomatology, was performed a subtotal gastrectomy without intraoperative or postoperative complications(AU)

Severe hypoproteinemia as a harbinger of Ménétrier's disease in autoimmune pancreatitis.

Ménétrier's disease is an extremely rare disease of unknown etiology causing gastric mucosal hypertrophy and protein-losing gastropathy. Rare cases of this condition have been reported in patients with autoimmune diseases. However, to the best of our knowledge, Ménétrier's disease associated with autoimmune pancreatitis (AIP) has never been reported. We described a case of severe hypoproteinemia as a harbinger of Ménétrier's disease associated with AIP. The patient was successfully treated with octreotide and high-protein diet, which led to symptomatic remission and significant improvement in serum levels of albumin and recovery of the nutritional status. Thus, in AIP patients presenting with severe and persistent hypoproteinemia without apparent cause, clinicians need to consider Ménétrier's disease in the differential diagnosis. In this setting, endoscopic evaluation with histological examination of gastric biopsy material, including a full-thickness mucosal biopsy of involved mucosa, may be helpful in promptly establishing the diagnosis and allowing appropriate and timely therapy.

Severe hypoproteinemia as a harbinger of Ménétrier's disease in autoimmune pancreatitis / Hipoproteinemia grave como indicador de doença de Ménétrier na pancreatite autoimune

Summary Ménétrier's disease is an extremely rare disease of unknown etiology causing gastric mucosal hypertrophy and protein-losing gastropathy. Rare cases of this condition have been reported in patients with autoimmune diseases. However, to the best of our knowledge, Ménétrier's disease associated with autoimmune pancreatitis (AIP) has never been reported. We described a case of severe hypoproteinemia as a harbinger of Ménétrier's disease associated with AIP. The patient was successfully treated with octreotide and high-protein diet, which led to symptomatic remission and significant improvement in serum levels of albumin and recovery of the nutritional status. Thus, in AIP patients presenting with severe and persistent hypoproteinemia without apparent cause, clinicians need to consider Ménétrier's disease in the differential diagnosis. In this setting, endoscopic evaluation with histological examination of gastric biopsy material, including a full-thickness mucosal biopsy of involved mucosa, may be helpful in promptly establishing the diagnosis and allowing appropriate and timely therapy.

Resumo A doença de Ménétrier é uma condição extremamente rara, de etiologia desconhecida, caracterizada por hipertrofia da mucosa gástrica e gastropatia perdedora de proteína. Casos raros dessa patologia têm sido relatados em pacientes com doenças autoimunes. Até o momento, desconhecemos qualquer relato dessa doença associada à pancreatite autoimune (PAI). Descrevemos um caso de hipoproteinemia grave como indicador de doença de Ménétrier associada à PAI. O paciente foi tratado de forma satisfatória com octreotide e dieta hiperproteica, alcançando remissão sintomática, melhora significativa das concentrações de albumina e recuperação do estado nutricional. Portanto, em pacientes com PAI e hipoproteinemia grave e persistente, deve-se considerar a doença de Ménétrier como um diagnóstico diferencial. Nesses casos, a avaliação endoscópica com biópsia gástrica, incluindo biópsia de toda a espessura da mucosa, pode ser útil no estabelecimento do diagnóstico e do pronto início da terapêutica.