Ménétrier's disease
5197404
223652321
2008-07-05T02:34:15Z
Arcadian
104523
infobox/nav
{{cleanup|date=June 2008}}
{{Infobox_Disease |
Name = {{PAGENAME}} |
Image = |
Caption = |
DiseasesDB = 8001 |
ICD10 = |
ICD9 = {{ICD9|535.2}} |
ICDO = |
OMIM = 137280 |
MedlinePlus = |
eMedicineSubj = |
eMedicineTopic = |
MeshID = D005758 |
}}
'''Ménétrier disease''' (also known as ''hyperplastic hypersecretory gastropathy'', and named after a French physician [[Pierre Eugène Ménétrier]], 1859-1935), is a disorder in which the [[stomach|gastric]] [[mucosa]]l folds ([[rugae]]) are [[Organ hypertrophy|enlarged]]<ref>http://www.gpnotebook.co.uk/cache/-2026569705.htm</ref> (and the total weight of the stomach is increased), making the surface of the stomach look a bit like the [[brain]] [[gyrus|gyri]].
==Presentation==
The altered gastric mucosa [[secretion|secretes]] massive amounts of [[mucus]], resulting in low [[plasma protein]] levels. The tissue may be inflamed and may contain ulcers. The disease also causes glands in the stomach to waste away and causes the body to lose fluid containing a protein called [[human serum albumin|albumin]].There are two forms of the disease: a childhood form and an adult form.� The childhood form has a better prognosis. It affects boys and girls equally, most often after they have a viral illness caused by [[cytomegalovirus]] (CMV) or a bacterial infection caused by ''[[Helicobacter pylori]]''. Children are not born with this disease, and it is not passed from parents to their children.<ref>http://www.madisonsfoundation.org/content/3/1/display.asp?did=413</ref> The adult form linked with overexpression of [[transforming growth factor alpha]] (TGF-α). The adult variety is four times more common in men, primarily affecting men between ages 30 and 60.
==Symptoms==
The presenting symptoms are
* pain after the meal ([[postprandial]]), relieved by [[antacid]]s, is very usual
* [[weight loss]], [[cachexia]]
* peripheral [[edema]], [[ascites]]
* [[anemia]] symptoms secondary to blood loss
Ménétrier disease increases a person's risk of [[stomach cancer]].<ref>[http://www.mamashealth.com/stomach/menetrier.asp Menetrier's Disease: symptoms, cause, treatment, prevention, long-term outlook, complication, risks<!-- Bot generated title -->]</ref> [[Histology|Microscopically]], the disease is characterized by [[hyperplasia]] of the [[Stomach#Histology of the human stomach|crypts]], which are elongated and may appear cystic or corkscrew-like.
Since it predisposes to [[stomach cancer]], periodic [[endoscopy|endoscopic]] surveillance is mandated. CMV-related Ménétrier is often self-limited.
==Diagnosis==
The disease must be diagnosed by x-ray (rare) or endoscopy and biopsy of the stomach.
==Treatment==
In adults, there is no treatment other than gastrectomy and a high-protein diet. Part or all of the stomach may need to be removed if the disease is severe.
Pediatric cases are normally treated for symptoms with the disease clearing up in weeks to months.
==Related conditions==
Other forms of hyperplastic gastropathy include [[Zollinger-Ellison syndrome]].
==References==
''Rubin's Pathology, Clinicopathological Foundations of Medicine'', 4th edition, Rubin, Gorstein, Rubin, Schwarting, Strayer. Lippincott Williams & Wilkins. ISBN 0-7817-4733-3
{{reflist}}
[[Category:Digestive diseases|Menetrier disease]]
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{{Digestive system diseases}}
[[de:Ménétrier-Syndrom]]
[[es:Enfermedad de Menetrier]]
[[pl:Zapalenie żołądka przerostowe, łagodne]]
[[ru:Болезнь Менетрие]]