Menetrie's disease: what is it, symptoms, causes, treatment, prognosis

What is Menetrie's disease?

Menetrie's disease Is a rare disease characterized by massive proliferation of mucous cells (foveola) in the lining of the stomach, resulting in large gastric folds. The most common symptom associated with Menetriere's disease is pain in the upper middle region of the stomach (epigastric pain). The cause of Menetriere's disease is unknown.

There is considerable confusion and controversy in the medical literature regarding disorders associated with large gastric folds. The name Menetrie's disease is often mistakenly used to describe any condition with large gastric folds or synonymous with giant hypertrophic gastritis. However, Menetrie's disease is not the true form. gastritis. Menetrie's disease should only be diagnosed in individuals with large gastric folds caused by overgrowth of mucous cells. In Menetrie's disease, inflammation of the stomach is minimal or absent. Because inflammation is minimal or absent, Menetrier's disease is classified as a form of hyperplastic gastropathy and not as a form of gastritis.

Some scientists believe that Menetriere's disease and giant hypertrophic gastritis may be variants of the same disorder or different parts of the same spectrum of diseases.

Signs and symptoms

The symptoms of Menetriere's disease can vary from person to person. Some people may be asymptomatic (i.e. asymptomatic). The most common symptom is pain in the upper middle region of the stomach (epigastric pain). Less common symptoms include nausea, vomiting, and diarrhea. In some cases, weight loss and profound loss of appetite (anorexia).

An additional variable sometimes associated with Menetriere's disease is protein loss from the circulation in the gastrointestinal tract (protein loss in gastropathy), albumin (hypoalbuminemia). Protein loss can be serious in some cases. Protein loss can lead to fluid buildup (swelling), especially in the legs.

Gastrointestinal bleeding has also been reported in some cases of Menetriere's disease, usually as a result of erosion or ulcers in the stomach lining. Excretion of acid by the stomach is often markedly reduced (hypochlorhydria) or absent. Excessive secretion of mucus in the stomach may also occur.

Some scientists believe that people with Menetrie's disease have a greater risk of developing stomach cancer than the general population. However, other scholars do not believe this has been proven with certainty. Several series of studies have found a link between Menetrie's disease and ulcerative colitis.

What to do if symptoms of Mentrier's disease appear?

If you have the symptoms described above, please contact your general practitioner. Attempts to self-medicate will not bring results, but can only harm.

Causes

The exact cause of Menetriere's disease is unknown. It can have several reasons for its occurrence. In children, some cases of the disease may be associated with cytomegalovirus infections (CMV). In adults, bacteria are involved Helicobacter pylori. The exact role these microorganisms play in the development of Menetriere's disease is unknown.

Some scientists believe that the large gastric folds that characterize Menetrie's disease may result from increased activation of the epidermal growth factor receptor in the stomach by a protein called transforming growth factor alpha (TGF-alpha).

In extremely rare cases, the brothers and sisters of patients developed Menethier's disease in childhood, which indicates the presence of a genetic link in these cases. Scientists believe that in these cases, the disease can be inherited as an autosomal dominant trait. Genetic diseases are defined by two sets of genes, one from the father and the other from the mother. Dominant genetic disorders occur when only one copy of a defective gene is needed for a disease to appear. The defective gene can be inherited from either parent or be the result of a new mutation (gene change) in the affected person. The risk of passing the abnormal gene from a sick parent to offspring is 50% with every pregnancy, regardless of the sex of the child.

Affected populations

Menetrie's disease affects men more often than women. The disease most often occurs in adults aged 50 and over. However, there is also a childhood form of the disorder. Due to confusion in the literature regarding the term Menetrie's disease, it is difficult to determine its true frequency in the general population.

Related disorders

Symptoms of the following disorders may be similar to those of Menetrie's disease. Comparisons can be useful for differential diagnosis:

• Zollinger-Ellison syndrome (ulcerogenic adenoma of the pancreas, gastrinoma) is a rare digestive disorder characterized by the development of tumors (gastrinomas) or tumors that produce excessive levels of gastrin, a hormone that stimulates acid production stomach. Many affected people develop multiple gastrinomas, about half to two-thirds of which may be malignant (cancerous). In most cases, tumors arise in pancreas and / or in the upper region of the small intestine (duodenum). Due to excessive acid production (hypersecretion of stomach acid) in people with the syndrome Zollinger-Ellison may develop peptic ulcers of the stomach, duodenum, and / or other areas digestive tract. Peptic ulcers are ulcers or moist areas in the digestive tract where the mucous membrane has been destroyed by stomach acid and digestive juices. Symptoms and signs associated with Zollinger-Ellison syndrome may include mild to severe abdominal pain; diarrhea; gastrointestinal bleeding; increased amount of fat in the stool (steatorrhea); and / or other violations. People with Zollinger-Ellison syndrome may have giant gastric folds.
• Lymphocytic gastritis - a rare disease characterized by inflammation of the stomach due to the accumulation of inflammatory cells in the inner wall (mucous membrane) of the stomach, resulting in abnormally large spiral ridges or folds that resemble polyps on the inner wall of the stomach (large stomach folds). Many people with lymphocytic gastritis have no symptoms. Symptoms that may occur include nausea, vomiting, abdominal pain, diarrhea, and weight loss. The exact cause of lymphocytic gastritis is unknown, although the disorder is associated with celiac diseaseas well as bacteria Helicobacter pylori.

Additional causes of large gastric folds include gastritis associated with Helicobacter pylorisome infections, including syphilis, and various forms of cancer include certain forms of carcinoma or lymphoma. Other rare disorders, such as sarcoidosis or Cronkit-Canada disease, can also be associated with large gastric folds.

Diagnostics

The disease can be suspected in people with large gastric folds. Large gastric folds can be diagnosed by x-rays after the patient has drunk a barium solution, or by endoscopy, in which a thin a flexible tube (endoscope) is inserted through the mouth and is used to examine the inside of the stomach and, if necessary, to obtain tissue samples for microscopic examination (biopsy). Histopathological examination of the affected stomach tissue obtained by biopsy may confirm the diagnosis of Menetrie's disease. Histopathology is the examination of microscopic anatomical changes in the affected tissue.

Standard treatments

Menetrie's disease was treated with anticholergic drugs, acid suppressive therapy, and antibiotic therapy against infection H. pylori. These treatments have produced conflicting results. In some cases, a high protein diet may be recommended to combat protein loss. Albumin transfusion can also be used to compensate for the loss of albumin.

Infusions of cetuximab (erbitux) block the epidermal growth factor receptor and have been used successfully to treat some patients with Menetrie's disease. Treatment with cetuximab can improve quality of life, restore stomach acid production, and possibly even reduce the thickness of the stomach wall. In some cases, after the cetuximab infusion is stopped, the patient remains healthy, at least temporarily. Erbitux can cause cardiac arrest, acne and low levels of magnesium, and thus patients should be closely monitored while being treated with this drug.

In severe cases, such as those with significant protein loss or a high likelihood of progression of gastric cancer, partial or complete gastrectomy may be required. A gastrectomy is the surgical removal of part or all of the stomach.

In children with CMV-associated Menetriere's disease, antiviral treatment against CMV may improve symptoms.

Other treatments are symptomatic and supportive.

Forecast

The prognosis of the disease varies from person to person. Resolution of symptoms can occur in adults with an underlying infection Helicobacter Pylori (H. Pylori) after treating the infection. Cases in children may resolve spontaneously or with treatment for an underlying CMV infection.

Although it has been suggested that patients with the disease may have an increased risk of developing stomach cancer; the risk remains uncertain. Research shows that between 2% and 15% of people with Menetriere's disease can develop stomach cancer in their lifetime. Given this potential, some authors propose observation with annual or semi-annual endoscopy.