Gastritis, familial giant hypertrophic

Familial Giant Hypertrophic Gastritis (FGHG), also known as Ménétrier's disease when occurring in adults, is a rare gastrointestinal disorder characterized by the massive enlargement of the gastric mucosa (stomach lining) which leads to the formation of large, coiled folds in the stomach. This condition is hereditary, suggesting a genetic predisposition. It is important to differentiate FGHG from similar conditions that cause gastric hypertrophy, as its management and prognosis can significantly differ.

Symptoms and Signs

Patients with Familial Giant Hypertrophic Gastritis may exhibit a range of symptoms, which can vary significantly in severity. Common symptoms include:

• Nausea and vomiting
• Abdominal pain
• Gastrointestinal bleeding
• Protein-losing enteropathy leading to edema due to loss of protein through the gastrointestinal tract
• Anorexia (loss of appetite)
• Weight loss

Causes

The exact cause of FGHG is not fully understood, but it is known to be hereditary, indicating a genetic component to the disease. It is thought to involve abnormal growth signals to the stomach lining, leading to the hypertrophy of the mucosa.

Diagnosis

Diagnosis of Familial Giant Hypertrophic Gastritis involves a combination of clinical evaluation, endoscopy, and histological examination of biopsy samples. During an endoscopy, the characteristic large, coiled folds of the stomach lining can be observed, and biopsy samples can be taken for further analysis.

Treatment

Treatment options for FGHG are limited and mainly focus on managing symptoms. They may include:

• Proton pump inhibitors (PPIs) to reduce stomach acid production
• Nutritional support, including protein supplements if protein-losing enteropathy is present
• In severe cases, partial gastrectomy (surgical removal of part of the stomach) may be necessary

Prognosis

The prognosis for patients with Familial Giant Hypertrophic Gastritis varies. While some patients may experience only mild symptoms that can be managed with medication, others may suffer from severe complications requiring surgical intervention. The condition can significantly impact the quality of life and nutritional status of affected individuals.

Epidemiology

Familial Giant Hypertrophic Gastritis is extremely rare, with only a few documented cases in the medical literature. It can affect individuals of any age but is typically diagnosed in childhood or early adulthood.

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