Dec 18, 2015 menetriers disease md, hypoproteinemic hypertrophic gastropathy, is a rare acquired disorder characterized by giant gastric rugal folds in the body and fundus, often with antral sparing, decreased acid secretion, increased gastric mucus production, and hypoalbuminemia secondary to protein loss in the gastric mucosa. Talley, division of gastroenterology and internal medicine, department of health sciences research, and department of laboratory medicine and. Menetriers disease md is included in the group of hypertrophic. Unusual coexistence of menetrier disease with adenocarcinoma. Jun 01, 2016 although the cause of menetrier disease is unknown, several cases of the condition have been reported in a familial setting. It is a rare condition characterised by gross hypertrophy of the gastric mucosa, resulting in an increased production of mucus, reduced acid secretion and, due to selective serum protein loss across the gastric mucosa, severe hypoproteinaemia. Menetriers disease is a rare disease that can result in diffusely thickened or giant gastric folds. Article pdf available in journal of the college of physicians and surgeonspakistan. The disorder is associated with excessive secretion of transforming growth factor alpha tgf. Menetrier disease md, also known as giant hypertrophic gastritis or hypoproteinemic hypertrophic gastropathy, is a form of rare idiopathic hypertrophic gastropathy. Menetrier disease is listed by the office of rare diseases of the national institutes of health as a rare disease, a designation that means it has a prevalence of less than 1 in 200,000 people. Disease most commonly involves the antrum andor antrumcorpus junction, but may be seen anywhere in the stomach histopathology is characterized by.
Menetriers disease is a rare acquired disorder of the fundus and body of the stomach ie, oxyntic mucosa characterized by giant. Histologically, it can be mistaken for other disorders showing hypertrophic gastropathy. Rare localized hyperplastic gastropathy, associated with stomach adenocarcinoma am j surg pathol 1997. Menetrier s disease generally presents with a clinical syndrome of weight loss, edema, and diarrhea secondary to protein losing enteropathy. Rare disease with incidence menetriers disease is a premalignant condition and though almost 200 cases of the disease have been reported in the literature, only few cases have been associated with gastric adenocarcinoma. Menetriers disease is a premalignant condition and though almost 200 cases of the disease have been reported in the literature, only few cases have been associated with gastric adenocarcinoma. B histopathological analysis of endoscopic mucosal resection. Menetriers disease is a rare hyperproliferative proteinlosing gastropathy of the gastric foveolar epithelium. Atypical clinical and diagnostic features in menetriers disease in a. In adults, this disease can be devastating with significant morbidity and mortality. In underdeveloped areas such as the columbian andes, hpinfected gastritis is even more common than in the western world, but its histopathology is different. Dec 21, 2018 home medterms medical dictionary az list menetrier s disease definition medical definition of menetrier s disease medical author.
Menetrier disease is a rare disorder characterized by massive overgrowth of mucous cells foveola in the mucous membrane lining the stomach, resulting in large gastric folds. An unusual presentation of menetriers disease farina mohammed hanif1, nasir hassan luck 1, zaigham abbas, syed mujahid hassan, sabhita shabir1 and muhammad mubarak2 abstract menetriers disease md is a rare acquired hypertrophic gastropathy characterized by giant hypertrophic rugal folds, hypochlorhydria, and hypoproteinemia. Menetriers di sease is a rare, acquired hypertrophic gastropathy of unknown etiology 1, in several situations associated with helicobacter pylori hp or cytomegalovirus cmv infection 2, 3. Menetrier s disease is a rare disease that can result in diffusely thickened or giant gastric folds. Menetriers disease or hypertrophic gastritis is a premalignant rare disease that often presents with hypertrophy. Menetrier s disease is characterized by giant gastric folds and foveolar hyperplasia. Menetriers disease md, hypoproteinemic hypertrophic gastropathy, is a rare acquired disorder characterized by giant gastric rugal folds in the body and fundus, often with antral sparing, decreased acid secretion, increased gastric mucus production, and hypoalbuminemia secondary to protein loss in the gastric mucosa.
There are also patients in whom neither hp nor cmv were detected. Menetriers disease generally presents with a clinical syndrome of weight loss, edema, and diarrhea secondary to protein losing enteropathy. It affects men more frequently than women, and the typical age at. Menetriers disease is a rare hyperplastic gastropathy that may lead to. Jan 11, 2019 researchers are still not quite sure about the exact cause of menetriers disease, although they are of the opinion that this disease is an acquired one rather than an inherited one, although there have been extremely rare cases in which siblings have gone on to develop this condition suggesting that there may be a genetic link to menetriers disease. A read is counted each time someone views a publication summary such as the title, abstract, and list of authors, clicks on a figure, or views or downloads the fulltext.
Menetriers disease md is a rare type of hypertrophic gastropathy involving the body of the stomach, which was initially described in 1888. In menetriers disease gastric rugae are typically wider than 25mm and sometimes polypoid in appearancethe diameter of normal folds does not exceed 5mm. Menetriers disease md is a rare disease characterized by. Menetrier disease an overview sciencedirect topics. All structured data from the file and property namespaces is available under the creative commons cc0 license. This means that menetriers disease, or a subtype of menetriers disease, affects less than 200,000 people in the us population. Successful use of octreotide to treat menetriers disease. The niddk translates and disseminates research findings to increase knowledge and understanding about health and disease among patients, health professionals, and the public.
But full recovery is usually observed within 56 months. In a few, disease course might be stormy like the present child. Eisenstat dd, griffiths am, cutz e, petric m, drumm b. It is named after a french physician pierre eugene menetrier, 18591935. Jul 22, 2014 this much misspelled disease was named for pierre eugene menetrier, a french physician 18591935 who first described it in 1888. Jan 20, 2011 this study reports a case of menetriers disease md in an adult who presented with egigastric pain and peripheric edema. May 16, 2018 the stomach tissue may be inflamed and may contain ulcers.
Menetriers disease, a premalignant condition, with. The aim of this study was to test the hypothesis that patients previously diagnosed with menetrier s disease actually represent at least two diseases. Menetriers disease presenting with ulcerative colitis who emro. Lymphocytic cholecystitischolangitis american journal of. Menetriers disease is a rare condition characterized by the presence of gastric hypertrophy due to massive gastric folds, with foveolar infiltration replacing the normal glandular architecture. Menetriers disease should be suspected in patients with upper gastrointestinal symptoms, hypoalbuminemia and hypertrophied gastric mucosa even without helicobacter pylori infection. It is considered a clinical diagnosis that may at times be difficult to establish. Menetriers disease without helicobacter pylori mimicking. Menetriers disease in children is a rare but mostly a benign, self limiting condition. Jan 17, 2019 acquired thrombophilia is a potential sequela of malignancy, chronic inflammation, and conditions characterized by severe protein deficiency for example, nephrotic syndrome, proteinlosing enteropathy.
Department ofpaediatric pathology, mother and child health institute, 8 radoja dakica. Menetriers disease also causes glands in the stomach to waste away and causes the body to lose fluid containing a protein called albumin. In a 2003 journal article titled an unusual expression of hyperplastic gastropathy menetrier type in twins, ibarrola et al. Computed tomography ct enteroclysis is a new imaging technique which combines enteroclysis and spiral multislice ct.
Menieres disease md is a disorder of the inner ear that is characterized by episodes of feeling like the world is spinning, ringing in the ears, hearing loss, and a fullness in the ear. Menetrier disease is a diffuse foveolar hyperplasia menetrier disease, is a rare, acquired, premalignant disease of the stomach characterized by massive gastric folds, excessive mucous production with resultant protein loss, and little or no acid production. Firm diagnostic criteria for md are proposed by delineating the. As such, venous thrombosis is often a feature, and occasionally a presenting sign, of systemic disease. Histologically, it can be mistaken for other disorders showing hypertrophic. Source national institutes of health nih ophanet, a consortium of european partners, currently defines a condition rare when it affects 1 person per 2,000. Jan 01, 2015 since the advent of laparoscopic surgery, there has been a dramatic increase in the number of cholecystectomies, the vast majority of which are performed for symptomatic gallstone disease and its complication. The most common symptom associated with menetrier disease is pain in the upper middle region of the stomach epigastric pain. Menetrier disease is a rare, acquired, premalignant disease of the stomach characterized by massive gastric folds, excessive mucous production with resultant protein loss, and little or no acid production. In childhood, it is a selflimiting, transient and benign. Menetriers disease presenting as recurrent unprovoked venous. Menetrier disease genetic and rare diseases information.
Menetrier disease in a pediatric patient with particular. We present the case of a 46yearold male patient who was diagnosed with adenocarcinoma of the esophagogastric junction siewert type ii, ut3 un0. The hallmark of the disease is gastric mucosal hypertrophy, which may cause the rugae to resemble convolutions of the brain, 2. It is characterized clinically by nonspecific gastrointestinal symptoms and edema, biochemically by hypoalbuminemia, and pathologically by enlarged gastric folds. Helicobacter pylori as a pathogenic factor in irondeficiency anaemia. Factors such as hypochlorhydria and antral atrophy may predispose to gastric cancer as a consequence of bacterial overgrowth and a concomitant. The extent of mucosal inflammation and glandular atrophy, however, is controversial. Menetriers disease symptoms, diagnosis, treatments and. Chronic inflammation gland atrophy intestinal metaplasia. Printable menetrier disease surgical pathology criteria. Histopathology of menetriers disease is similar in adults and children with hypertrophy of gastric glands, cystic dilatation of glands deep in the mucosa. This content is provided as a service of the national institute of diabetes and digestive and kidney diseases niddk, part of the national institutes of health. People who have it are usually men between ages 30 and 60. Objective menetriers 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.
Jun 01, 2016 menetrier disease is a gastrointestinal condition characterized by overgrowth of the mucous cells foveola in the mucous membrane lining the stomach, causing enlarged gastric folds. Histopathology showed data compatible with menetrier disease. Serial morphological, secretory, and serological observations of a patient with mimetriers disease disclosed hypergastrinemia, antibodies to parietal cells and dietary substances, and an acute reduction of gastrointestinal protein loss after atropine administration. Acute cytomegalovirus infection in a child with menetriers disease gastroenterology. Preoperative diagnosis could not be confirmed unless adequate deep gastric biopsy is performed.
A case report of menetriers disease pacific group of e. Distinguishing menetriers disease from its mimics gut. Menetrier disease in a five year old girl a case report and. Files are available under licenses specified on their description page. Atypical clinical and diagnostic features in menetriers. Media in category histopathology of menetriers disease the following 5 files are in this category, out of 5 total. Menetriers disease is a rare proteinlosing hypertrophic gastropathy. Remission of severe anaemia persisting for over 20 years after eradication of helicobacter pylori in cases of menetriers disease and atrophic gastritis. Menetriers disease is characterized by giant gastric folds and foveolar hyperplasia. This leads to excessive mucus production, causing protein loss from the stomach and low or absent levels of stomach acid. In paediatric form of the disease, association of the allergy is common along with the cmv infection which resolves spontaneously, but should be distinguished from cmv gastritis. Menetrier disease nord national organization for rare.
Menetriers disease is an idiopathic condition characterized by excessive mucus production, giant mucosal hypertrophy, hypoproteinemia, and hypochlorhydria due to the loss of parietal cells. It is characterized by thickening of the gastric mucosa in the form of giant rugal folds, hypochlorhydria and protein loss. We focused in particular on the imaging and diagnostic aspects of the presenting case as well as clinical, histologic, and therapeutic aspects. Chouraqui jp, roy cc, brochu p, gregoire h, morin cl, et al.
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