Gastropatia hiperproliferativa pré-maligna rara caracterizada por crescimento excessivo de células foveolares no revestimento gástrico, resultando em grandes dobras gástricas e manifestando-se com dor epigástrica, náuseas, vômitos, edema periférico e, menos comumente, anorexia e perda de peso.
Introdução
O que você precisa saber de cara
Gastropatia hiperproliferativa pré-maligna rara caracterizada por crescimento excessivo de células foveolares no revestimento gástrico, resultando em grandes dobras gástricas e manifestando-se com dor epigástrica, náuseas, vômitos, edema periférico e, menos comumente, anorexia e perda de peso.
Escala de raridade
<1/50kMuito rara
1/20kRara
1/10kPouco freq.
1/5kIncomum
1/2k
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Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Partes do corpo afetadas
+ 11 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 23 características clínicas mais associadas, ordenadas por frequência.
Linha do tempo da pesquisa
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Genética e causas
O que está alterado no DNA e como passa nas famílias
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Diagnóstico
Os sinais que médicos procuram e os exames que confirmam
Tratamento e manejo
Remédios, cuidados de apoio e o que precisa acompanhar
Onde tratar no SUS
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🇧🇷 Atendimento SUS — Doença de Menetrier
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Publicações mais relevantes
Ménétrier Disease.
Ménétrier disease is a rare disorder characterized by giant mucosal folds in the proximal stomach, reduced acid secretion, and protein-losing enteropathy with resulting hypoalbuminemia. The distal stomach is typically spared. Alternative terms include "hypoproteinemic hypertrophic gastropathy" and "giant hypertrophic gastritis." The condition predominantly affects men but also occurs in women and children. In adults, Ménétrier disease frequently follows a progressive course and is considered premalignant. The etiopathogenesis is incompletely understood.
Ménétrier disease: A clinical review.
Ménétrier disease (MD) is a rare gastric disorder characterized by hypertrophy of the gastric mucosa, resulting in giant gastric folds, excessive mucus secretion, and significant protein loss. It is most common in middle-aged males, although cases have also been reported in children. This condition, also known as hyperplastic hypersecretory gastropathy, primarily affects the fundus and body of the stomach, leading to protein-losing gastropathy due to increased mucosal permeability. The exact pathogenesis of MD remains unclear; however, it has been linked to excessive transforming growth factor-alpha signaling via the epidermal growth factor receptor (EGFR), which promotes mucosal hyperplasia. Clinical manifestations include epigastric pain, nausea, vomiting, anorexia, weight loss, and peripheral edema due to protein-losing enteropathy. Diagnosis is based on clinical presentation, endoscopic findings, and histopathology revealing foveolar hyperplasia and glandular atrophy. Treatment options vary from symptomatic management with proton pump inhibitors and nutritional support to monoclonal antibodies targeting EGFR (e.g., cetuximab) in severe cases. In refractory situations, gastrectomy may be required. This review highlights the current understanding, diagnostic approaches, and therapeutic strategies for MD.
Ménétrier Disease: Rare Manifestations and Mechanistic Insights From 2 Adult Cases.
BACKGROUND Menetrier disease is a rare gastric disorder characterized by hypertrophic hyperplastic changes in the stomach mucosa, hypoproteinemia, and symptoms like abdominal pain and diarrhea. It predominantly affects men aged 40-60 years and involves protein loss from the stomach. There have been few reports of cases complicated with severe anemia and secondary gastric outlet obstruction. The pathogenesis in adults remains unclear, although pediatric cases often are associated with cytomegalovirus (CMV) infection. CASE REPORT We present 2 adult cases. Case 1 was a 35-year-old man with a 2-month history of epigastric pain, vomiting, melena, and severe anemia. Lab tests revealed hypoproteinemia and low hemoglobin. Imaging and endoscopy showed gastric wall thickening, a gastric mass, and mucosal abnormalities. Histopathology confirmed Menetrier disease. Case 2 was a 44-year-old man with 6 years of epigastric fullness and belching. He had hypoproteinemia and multiple gastric nodules. Endoscopy revealed enlarged mucosal folds and polyps. Biopsies showed chronic inflammation and foveolar hyperplasia, consistent with Menetrier disease. CONCLUSIONS These cases highlight the diagnostic challenges of Menetrier disease, emphasizing the need for integrated clinical, endoscopic, and histopathological evaluation. Further studies are needed to explore the associations between Menetrier disease, Helicobacter pylori (H. pylori) infection, and gastric cancer.
Unusual Suspect: Ménétrier Disease Presents as Gastric Outlet Obstruction in a Toddler.
Ménétrier disease (MD) is a rare condition characterized by hypertrophic gastropathy, leading to enlarged gastric folds, excessive mucus production, and protein loss. Among the approximately 150 cases of reported pediatric MD, the usual presenting features are peripheral oedema, anasarca, and gastrointestinal symptoms. This case report describes a 2-year-old child presenting with gastric outlet obstruction, an unusual manifestation of MD, which has not been described previously in the pediatric age group.
Ménétrier disease: a rare cause of severe chronic diarrhea difficult to diagnose with response to treatment with octreotide.
We present the case of a 71-year-old male with difficult to manage severe chronic diarrhea, who after multiple tests was finally diagnosed with Ménétrier's disease and had clinical improvement with treatment with intramuscular octreotide. A high degree of clinical suspicion is essential to diagnose this entity, exclude similar entities and the correlation of clinical, analytical, endoscopic, radiological and histological data.
Publicações recentes
Gastric Adenocarcinoma Arising From the Ménétrier Disease.
Ménétrier disease: A clinical review.
Ménétrier Disease: Rare Manifestations and Mechanistic Insights From 2 Adult Cases.
📚 EuropePMC83 artigos no totalmostrando 26
Ménétrier disease: A clinical review.
World journal of gastroenterologyMénétrier Disease: Rare Manifestations and Mechanistic Insights From 2 Adult Cases.
The American journal of case reportsUnusual Suspect: Ménétrier Disease Presents as Gastric Outlet Obstruction in a Toddler.
ACG case reports journalMenetrier Disease - A Rare Presentation of Gastrointestinal Bleeding.
Journal of community hospital internal medicine perspectivesMénétrier disease: a rare cause of severe chronic diarrhea difficult to diagnose with response to treatment with octreotide.
Revista espanola de enfermedades digestivasMénétrier disease: A rare cause of hypertrophic gastropathy.
Revista de gastroenterologia de Mexico (English)Chronic Ménétrier disease leading to gastric cancer in youth.
Clinical endoscopyMénétrier Disease: A Rare Cause of Iron Deficiency Anemia.
Clinical gastroenterology and hepatology : the official clinical practice journal of the American Gastroenterological AssociationThe Use of Deep Snare Biopsies to Diagnose Cronkhite-Canada Syndrome.
ACG case reports journalMenetrier Disease in a Pediatric Patient With Ulcerative Colitis and Primary Sclerosing Cholangitis.
Journal of pediatric gastroenterology and nutritionAbnormal Levels of Pepsinogen I and Gastrin 17 in a case of Ménétrier Disease.
Acta bio-medica : Atenei ParmensisMenetrier's disease. A diagnostic and therapeutic challenge.
MedicinaDiagnosis and Management of Ménétrier Disease in Children: A Case Series Review.
Pediatric gastroenterology, hepatology & nutritionMenetrier disease and Cytomegalovirus infection in paediatric age: report of three cases and a review of the literature.
European journal of pediatricsUnusual case of adult familial Menetrier disease in siblings.
BMJ case reportsHyperplastic and fibrosing gastropathy resembling Ménétrier disease in a cat.
JFMS open reports[Ménétrier's disease in pediatric patients secondary to cytomegalovirus infection: Presentation of two clinical cases in a high complexity center].
Archivos argentinos de pediatria[An infrequent cause of iron-deficiency anemia: Ménétrier disease].
Acta gastroenterologica LatinoamericanaMIB2 variants altering NOTCH signalling result in left ventricle hypertrabeculation/non-compaction and are associated with Ménétrier-like gastropathy.
Human molecular geneticsMassive gastric juvenile-type polyposis: a clinicopathological analysis of 22 cases.
HistopathologyMénétrier disease manifested by polyposis and involved in both the small bowel and entire colon: A Case Report.
MedicineCytomegalovirus-Associated Menetrier Disease in Childhood.
Clinical pediatricsFDG PET/CT Findings of Ménétrier Disease.
Clinical nuclear medicineGeneration of stomach tissue from mouse embryonic stem cells.
Nature cell biologyMassive gastric polyposis associated with a germline SMAD4 gene mutation.
Familial cancerMénétrier Disease and Ulcerative Colitis.
Journal of clinical gastroenterologyAssociações
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Referências e fontes
Bases de dados externas citadas neste artigo
Publicações científicas
Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.
- Ménétrier Disease.
- Ménétrier disease: A clinical review.
- Ménétrier Disease: Rare Manifestations and Mechanistic Insights From 2 Adult Cases.
- Unusual Suspect: Ménétrier Disease Presents as Gastric Outlet Obstruction in a Toddler.
- Ménétrier disease: a rare cause of severe chronic diarrhea difficult to diagnose with response to treatment with octreotide.
- Gastric Adenocarcinoma Arising From the Ménétrier Disease.
- Ménétrier Disease.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:2494(Orphanet)
- OMIM OMIM:137280(OMIM)
- MONDO:0007652(MONDO)
- GARD:2436(GARD (NIH))
- Busca completa no PubMed(PubMed)
- Q975705(Wikidata)
Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.
Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar
