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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.

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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.

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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.

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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.

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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.

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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.

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La enfermedad de Ménétrier se caracteriza por un crecimiento glar dular con engrosamiento de pliegues en las paredes del estómago, constituyendo parte de un grupo de gastropatías hiperplásicas perdedoras de proteínas de etiología aún no precisada. Es una dolencia que ocurre usualmente en personas con edades entre 30 a 60 años, siendo muy rara en la edad pediátrica. Clinicamente se presenta con diarrea recurrente, vómitos, dolor abdominal y pérdida de peso. No existe un tratamiento específico, requiriendo solo un manejo sintomático de soporte. Los autores informan el caso de un niño de 7 años, con esta enfermedad, la cual se presentó con síntomas poco habituales (edemas, asciti derrame pleural); en los estudios de gabinete se confirmó PC trasonido, ascitis, derrame pleural y una hiperecogenecidad del estomago por la cual se procedió a realizar un tránsito gastrointestinal, cortando irregularidades en el llenado gástrico. El estadio endoscópico mostró un fundus gástrico de forma irregular, engrosando, con secreción espesa, viscosa y transparente en todo el estómago. El tratamiento intaurado consistió en la administración de albúmina, con la cual do obtener la desaparición de su sintomatología (edemas, ascitis ame pleural); posteriormente se continuó con la administració tomática de protectores gástricos y dieta hiperproteica. Fue dado d tres semanas despúes sin molestias significativas

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We report the postmortem findings of three patients with multiple pale polyps in the small bowell, which show the typical histological features of lymphangiectatic cysts.

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Background: The traditional methods to distinguish Chronic Follicular Gastritis and Primary Gastric Lymphoma do not allow an adequate definitive diagnosis in a significant number of cases. The Molecular Biology diagnostic methods are based on the rearrangement of immunoglobulin genes. The polymerase chain reaction (PCR) specifically amplifies this rearrangement and allows molecular analysis of minimal tissue samples obtained with endoscopical biopsies. Aim: To test the usefulness of this PCR method in the differential diagnosis between Chronic Follicular Gastritis and Primary Gastric Lymphoma. Material and methods: We analyzed the endoscopical biopsies of six Chronic Follicular Gastritis cases and eight surgically treated Primary Gastric Lymphoma cases, six with the correct diagnosis in the endoscopical biopsies and two with a diagnosis of Chronic Follicular Gastritis. Results: A policlonal immunoglobulin rearrangement was found in the six cases with Chronic Follicular Gastritis. A monoclonal arrangement was found in 5 of 6 biopsies with the diagnosis of Primary Gastric Lymphoma. The same monoclonal rearrangement was observed in the two biopsies incorrectly diagnosed as Chronic Follicular Gastritis. Conclusions: PCR analysis of immunoglobulin rearrangement is a useful method in the differential diagnosis between Chronic Follicular Gastritis and Primary Gastric Lymphoma

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Las hipoproteinemia y los síndromes edematosos consecutivos representan un problema frecuente en clínica médica

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Se presenta un niño de 18 meses con gastropatía hipertrófica transitoria de la infancia (GHTI) (enfermedad de Ménétrier infantil). Si bien la biopsia gástrica permitió completar el diagnóstico, no demostró la presencia de células con inclusiones tipo citomegalovirus. La presencia de las secuencias génicas de ese virus fue comprobada por la reacción en cadena de polimerasa, en el material de la biopsia. Este caso favorece la existencia de una relación etiopatogénica entre el citomegalovirus y la GHTI (AU)

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Se presenta un niño de 18 meses con gastropatía hipertrófica transitoria de la infancia (GHTI) (enfermedad de Ménétrier infantil). Si bien la biopsia gástrica permitió completar el diagnóstico, no demostró la presencia de células con inclusiones tipo citomegalovirus. La presencia de las secuencias génicas de ese virus fue comprobada por la reacción en cadena de polimerasa, en el material de la biopsia. Este caso favorece la existencia de una relación etiopatogénica entre el citomegalovirus y la GHTI

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Ménétrier's disease (MD) is a rare form of hypertrophic or hyperplastic gastropathy characterized by conspicuous thickening of the gastric mucosal folds and foveolar (crypt) hyperplasia. We examined the presence of cytomegalovirus (CMV) in 2 cases of MD in adults, one associated with gastric carcinoma, using the polymerase chain reaction (PCR). None of the cases showed intranuclear inclusions consistent with CMV infection. Both revealed, besides the features of MD, a peculiar pattern of "chronic active plasmacellular gastritis". Although the samples had been stored in formalin for more than 10 years CMV-DNA was recovered with good yield from both samples. The demonstration of CMV in MD in adults may helps to explain present knowledge of the complex relationships between this virus and gastric mucosa, and strongly suggests a pathogenetic role of the virus with variable phenotypic expression along the years.

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Case presentation of a 61 year old male who presented with a 3 months' history of anorexia, weight loss, prolonged febrile syndrome, myalgias, peripheral polyneuropathy, abdominal pain, anemia, renal insufficiency, sonographically detected splenomegaly, elevated alkaline phosphatase, hypoproteinemia, sudden drops of the hematocrit, gastric ulcer with negative biopsies for malignancy and where the histological study of the surgical specimen confirmed the diagnosis of Ménétrier's disease or hypertrophic gastritis associated with nodose polyarteritis. The first explains the hypoproteinemia, edema; whereas the second explains the renal insufficiency, peripheral polyneuropathy, fever, skeletal muscle, spleen and liver involvements. The gastric ulcer, blood loss, anorexia, weight loss and abdominal pain can be justified by both diseases. We have been unable to find in the literature any other case of a similar association.

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Se presenta un caso de sexo masculino de 61 años de edad cuya sintomatología comienza aparentemente 3 meses antes: anorexia, pérdida de peso, síndrome febril prolongado, mialgias, polineuropatía periférica, dolor abdominal, anemia, síndrome de insuficiencia renal, esplenomegalia detectado ecográficamente, ascitis, fosfatasas aldalinas elevadas, hipoprotidemia, caídas súbitas del hematocrito, úlcera gástrica con biopsias negativas para malignidad y donde el estudio histopatológico de la pieza operatoria (gastrectomía parcial), confirmó los diagnósticos de enfermedad de Ménétrier o gastropatía hipertrófica, asociada a poliarteritis nodosa. La primera explica la hipoprotidemia, los edemas, la ascitis. La segunda, la insuficiencia renal, la polineuropatía periférica, el compromiso muscular esplénico, hepático y la fiebre; mientras que la úlcera gástrica, las pérdidas de sangre, la anorexia, la pérdida de peso y el dolor abdominal pueden ser explicados por ambas enfermedades. En la bibliografía consultada no se detectó ningún caso con esta misma asociación (AU)

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Se presenta un caso de sexo masculino de 61 años de edad cuya sintomatología comienza aparentemente 3 meses antes: anorexia, pérdida de peso, síndrome febril prolongado, mialgias, polineuropatía periférica, dolor abdominal, anemia, síndrome de insuficiencia renal, esplenomegalia detectado ecográficamente, ascitis, fosfatasas aldalinas elevadas, hipoprotidemia, caídas súbitas del hematocrito, úlcera gástrica con biopsias negativas para malignidad y donde el estudio histopatológico de la pieza operatoria (gastrectomía parcial), confirmó los diagnósticos de enfermedad de Ménétrier o gastropatía hipertrófica, asociada a poliarteritis nodosa. La primera explica la hipoprotidemia, los edemas, la ascitis. La segunda, la insuficiencia renal, la polineuropatía periférica, el compromiso muscular esplénico, hepático y la fiebre; mientras que la úlcera gástrica, las pérdidas de sangre, la anorexia, la pérdida de peso y el dolor abdominal pueden ser explicados por ambas enfermedades. En la bibliografía consultada no se detectó ningún caso con esta misma asociación

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No presente trabalho relata-se um caso de carcinoma de células parietais, um novo tipo histológico de câncer gástrico, associado com doença de Ménétrier. O tumor apresentou-se macroscopicamente como lesäo ulcerada de 6 x 6 cm envolvendo a regiäo da cárdia. Identificaram-se metástases nos linfonodos da grande curvatura e epiplóicos. A mucosa do corpo e do fundo gástrico mostrou pregas espessas e tortuosas e o padräo histológico da doença de Ménitrier

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