Ménétrier's disease
Encyclopedia
Ménétrier disease a disorder in which the gastric
mucosal folds (rugae
) are enlarged (and the total weight of the stomach is increased), making the surface of the stomach look a bit like the brain
gyri
. This leads to protein loss, parietal cell atrophy, and an increase in mucous cells. It results from profound hyperplasia of the surface mucous cells with accompanying glandular atrophy.
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 the protein albumin
.There are two forms of the disease: a childhood form and an adult form. The childhood form has a better prognosis. It affects males three times more than females, 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. The adult form is 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.
Ménétrier disease increases a person's risk of stomach cancer
. Microscopically
, the disease is characterized by hyperplasia
of the crypts, which are elongated and may appear cystic or corkscrew-like.
Since it predisposes to stomach cancer
, periodic endoscopic
surveillance is mandated. CMV-related Ménétrier is often self-limited.
and biopsy of the stomach.
Pediatric cases are normally treated for symptoms with the disease clearing up in weeks to months.
.
Stomach
The stomach is a muscular, hollow, dilated part of the alimentary canal which functions as an important organ of the digestive tract in some animals, including vertebrates, echinoderms, insects , and molluscs. It is involved in the second phase of digestion, following mastication .The stomach is...
mucosal folds (rugae
Rugae
Rugae is a term used in anatomy that refers to a series of ridges produced by folding of the wall of an organ. Most commonly the term is applied to the internal surface of the stomach...
) are enlarged (and the total weight of the stomach is increased), making the surface of the stomach look a bit like the brain
Brain
The brain is the center of the nervous system in all vertebrate and most invertebrate animals—only a few primitive invertebrates such as sponges, jellyfish, sea squirts and starfishes do not have one. It is located in the head, usually close to primary sensory apparatus such as vision, hearing,...
gyri
Gyrus
A gyrus is a ridge on the cerebral cortex. It is generally surrounded by one or more sulci .-Notable gyri:* Superior frontal gyrus, lat. gyrus frontalis superior* Middle frontal gyrus, lat. gyrus frontalis medius...
. This leads to protein loss, parietal cell atrophy, and an increase in mucous cells. It results from profound hyperplasia of the surface mucous cells with accompanying glandular atrophy.
Presentation
The altered gastric mucosa secretesSecretion
Secretion is the process of elaborating, releasing, and oozing chemicals, or a secreted chemical substance from a cell or gland. In contrast to excretion, the substance may have a certain function, rather than being a waste product...
massive amounts of mucus
Mucus
In vertebrates, mucus is a slippery secretion produced by, and covering, mucous membranes. Mucous fluid is typically produced from mucous cells found in mucous glands. Mucous cells secrete products that are rich in glycoproteins and water. Mucous fluid may also originate from mixed glands, which...
, 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 the protein albumin
Human serum albumin
Human serum albumin is the most abundant protein in human blood plasma. It is produced in the liver. Albumin constitutes about half of the blood serum protein...
.There are two forms of the disease: a childhood form and an adult form. The childhood form has a better prognosis. It affects males three times more than females, most often after they have a viral illness caused by cytomegalovirus
Cytomegalovirus
Cytomegalovirus is a viral genus of the viral group known as Herpesviridae or herpesviruses. It is typically abbreviated as CMV: The species that infects humans is commonly known as human CMV or human herpesvirus-5 , and is the most studied of all cytomegaloviruses...
(CMV) or a bacterial infection caused by Helicobacter pylori
Helicobacter pylori
Helicobacter pylori , previously named Campylobacter pyloridis, is a Gram-negative, microaerophilic bacterium found in the stomach. It was identified in 1982 by Barry Marshall and Robin Warren, who found that it was present in patients with chronic gastritis and gastric ulcers, conditions that were...
. Children are not born with this disease, and it is not passed from parents to their children. The adult form is 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:- Epigastric Pain - pain after the meal (postprandialPostprandialPostprandial means after eating a meal while preprandial is before a meal.-Usages of the term:This term is used in many contexts but also in relation to blood sugar levels, which are normally measured 2 hours after and before eating in a postprandial glucose test...
), relieved by antacidAntacidAn antacid is a substance which neutralizes stomach acidity.-Mechanism of action:Antacids perform a neutralization reaction, increasing the pH to reduce acidity in the stomach. When gastric hydrochloric acid reaches the nerves in the gastrointestinal mucosa, they signal pain to the central nervous...
s, is very usual - weight lossWeight lossWeight loss, in the context of medicine, health or physical fitness, is a reduction of the total body mass, due to a mean loss of fluid, body fat or adipose tissue and/or lean mass, namely bone mineral deposits, muscle, tendon and other connective tissue...
, cachexiaCachexiaCachexia or wasting syndrome is loss of weight, muscle atrophy, fatigue, weakness, and significant loss of appetite in someone who is not actively trying to lose weight... - peripheral edemaEdemaEdema or oedema ; both words from the Greek , oídēma "swelling"), formerly known as dropsy or hydropsy, is an abnormal accumulation of fluid beneath the skin or in one or more cavities of the body that produces swelling...
, ascitesAscitesAscites is a gastroenterological term for an accumulation of fluid in the peritoneal cavity.The medical condition is also known as peritoneal cavity fluid, peritoneal fluid excess, hydroperitoneum or more archaically as abdominal dropsy. Although most commonly due to cirrhosis and severe liver... - anemiaAnemiaAnemia is a decrease in number of red blood cells or less than the normal quantity of hemoglobin in the blood. However, it can include decreased oxygen-binding ability of each hemoglobin molecule due to deformity or lack in numerical development as in some other types of hemoglobin...
symptoms secondary to blood loss
Ménétrier disease increases a person's risk of stomach cancer
Stomach cancer
Gastric cancer, commonly referred to as stomach cancer, can develop in any part of the stomach and may spread throughout the stomach and to other organs; particularly the esophagus, lungs, lymph nodes, and the liver...
. Microscopically
Histology
Histology is the study of the microscopic anatomy of cells and tissues of plants and animals. It is performed by examining cells and tissues commonly by sectioning and staining; followed by examination under a light microscope or electron microscope...
, the disease is characterized by hyperplasia
Hyperplasia
Hyperplasia means increase in number of cells/proliferation of cells. It may result in the gross enlargement of an organ and the term is sometimes mixed with benign neoplasia/ benign tumor....
of the crypts, which are elongated and may appear cystic or corkscrew-like.
Since it predisposes to stomach cancer
Stomach cancer
Gastric cancer, commonly referred to as stomach cancer, can develop in any part of the stomach and may spread throughout the stomach and to other organs; particularly the esophagus, lungs, lymph nodes, and the liver...
, periodic endoscopic
Endoscopy
Endoscopy means looking inside and typically refers to looking inside the body for medical reasons using an endoscope , an instrument used to examine the interior of a hollow organ or cavity of the body. Unlike most other medical imaging devices, endoscopes are inserted directly into the organ...
surveillance is mandated. CMV-related Ménétrier is often self-limited.
Diagnosis
The disease maybe diagnosed by x-ray or endoscopyEndoscopy
Endoscopy means looking inside and typically refers to looking inside the body for medical reasons using an endoscope , an instrument used to examine the interior of a hollow organ or cavity of the body. Unlike most other medical imaging devices, endoscopes are inserted directly into the organ...
and biopsy of the stomach.
Treatment
In adults, there is no FDA approved 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. However, the monoclonal antibody cetuximab has been used in compassionate use for treatment of Ménétrier's disease .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 syndromeZollinger-Ellison syndrome
Zollinger–Ellison syndrome is a triad of gastric acid hypersecretion, severe peptic ulceration, and non-beta cell islet tumor of pancreas . In this syndrome increased levels of the hormone gastrin are produced, causing the stomach to produce excess hydrochloric acid. Often the cause is a tumor of...
.