What is Zollinger ellison syndrome?

By | 14 May 2021

In healthy individuals, a sufficient amount of stomach acid is produced by the stomach to digest and break down the food that reaches the stomach after eating. The secretion of stomach acid is governed by gastrin, a type of hormone produced by the stomach. The acid produced is only in the amount to be used in the digestion of food and is not at a level that will damage the stomach wall or any organ in the further parts of the digestive tract. However, in some cases, the secretion pattern of gastrin hormone changes, and diseases related to excessive acid production may occur. One of them is Zollinger Ellison Syndrome.

What is Zollinger Ellison syndrome?

Zollinger-Ellison Syndrome is an extremely rare type of disease in which multiple tumors occur in the pancreas or small intestines. These tumors, called gastrinomas, secrete gastrin hormone that causes the stomach to produce excessive amounts of acid. The high amount of acid produced in the stomach causes ulcers in the advanced parts of the intestines, especially the duodenum, by damaging the digestive tract, just as the stomach is damaged in peptic ulcers (stomach ulcer). In Zollinger-Ellison Syndrome, tumors in the pancreas or small intestines can be benign or malignant. If the tumors are malignant, they can be classified as a type of cancer and have the risk of spreading to different tissues and organs of the body. Zollinger Ellison Syndrome, a rare type of disease, occurs in only 1 out of every 1 million people. Although the disease can be seen in both men and women and in all age groups, it has been found that the rate of getting this disease is higher in male individuals between the ages of 30-50. This disease, which is thought to be influenced by genetic factors, requires early diagnosis and treatment as it can cause major damage to the digestive tract.

What are the symptoms of Zollinger Ellison syndrome?

The symptoms seen in Zollinger-Ellison Syndrome patients are very similar to peptic ulcer disease. The most common and basic symptom of the disease is abdominal pain. This pain is a dull or burning pain that can be felt anywhere between the navel area and the upper part of the abdomen that corresponds to the stomach. This pain is also the main symptom of peptic ulcer disease. Abdominal pains felt by the patients are generally aggravated during hunger times, between meals, or at night when the stomach is empty. Although the pain eases a little with eating, it usually does not completely disappear. The pain sometimes continues for minutes and sometimes for hours, making daily life very difficult. Apart from this, other symptoms seen in Zollinger-Ellison Syndrome are:

  • Diarrhea
  • The gas outlet from the mouth (burping)
  • Nausea and vomiting
  • Swelling
  • Loss of appetite and weight

In addition to the above symptoms, gastroesophageal reflux problems may also develop in some patients. Gastroesophageal reflux, also known as reflux disease among people, is the problem of stomach contents leaking into the esophagus (esophagus). In patients with this problem, other than the above symptoms, a burning sensation in the chest area and acidic gastric fluid may come into the mouth. In some patients with Zollinger-Ellison Syndrome, the only symptom can be diarrhea.

What causes Zollinger Ellison syndrome?

Although the exact cause of Zollinger-Ellison syndrome is not yet known, scientific research on this issue continues. Approximately 25-30% of the pancreas or small intestine tumors, also called gastrinomas, are due to a genetic disorder called multiple endocrine neoplasias (MEN1). This is important evidence that genetic factors play an important role in the development of the disease. Individuals with a family history of Zollinger-Ellison Syndrome are more likely to develop this disease than other individuals. The genetic disorder in question is also associated with diabetes (diabetes) disease, kidney stones, weakness and fracture of bones, muscle weakness, and hormonal disorders.

How is Zollinger Ellison syndrome diagnosed?

Zollinger-Ellison Syndrome patients refer to healthcare institutions with the above symptoms, especially diarrhea and stomach pain. After a detailed medical history is taken in these patients, the physician can perform a physical examination. During the physical examination, the external appearance of the body is examined, pressure is applied to the areas where the pain is felt to check whether there is any change in pain, palpable swelling is checked and the sounds coming from the body are listened to with the help of a stethoscope. Because of symptoms pointing to the digestive tract, patients who present with such complaints are usually referred for endoscopy. In addition, some blood tests, stomach acid measurements, and medical imaging tests can be used. The presence of gastrinoma tumors in the pancreas or small intestine can be easily detected as a result of endoscopy applications and medical imaging examinations that enable the visualization of the upper gastrointestinal tract, and thus the diagnosis of the disease can be made. At this time, a biopsy is taken from the tumors seen at this time and sent to pathology laboratories for examination. Whether the tumor is a gastrinoma tumor, benign (benign) or malignant (malignant) can be clearly learned as a result of this examination. At the end of all these examinations, the treatment process is planned and started as soon as possible in patients with Zollinger-Ellison syndrome. In the case of malignant tumors, more detailed investigations should be done in case of spreading to surrounding tissues and organs.

How is Zollinger Ellison syndrome treated?

In patients diagnosed with Zollinger-Ellison Syndrome, treatment should be started as soon as possible and permanent damage to the organs of the gastrointestinal system should be prevented. The treatment plan for these patients usually includes medications, surgical operations, chemotherapy, and medical nutrition therapy. First of all, drug therapy is recommended to reduce the acid secretion of the stomach to heal ulcers in the digestive system and prevent new damage. These drugs belong to a group of drugs called proton pump inhibitors and contain active ingredients such as esomeprazole, lansoprazole, pantoprazole, omeprazole, and dexlansoprazole. Since long-term use of these drugs may affect calcium absorption, supportive supplements may be recommended by the physician to prevent bone and spine diseases. Then, the gastrinomas are removed with surgical operations, which are the only treatment options in Zollinger-Ellison Syndrome. These tumors are often malignant, and because they are so small, it is difficult to detect and remove gastrinomas before they spread to different parts of the body. Gastrinomas most often spread to the liver and bones. If such a spread is detected, chemotherapy applications should be performed in addition to the surgical operation. Chemotherapy can also be used for gastrinomas that are not suitable for surgical removal. As a result of the researches, it has been observed that the diet and style have no effect on reducing the risk of getting Zollinger-Ellison Syndrome or preventing the disease. However, with the recommendation of a nutritional program such as the diet applied in diseases such as gastritis and stomach ulcer, the symptoms such as diarrhea, stomach pain, and reflux can be significantly reduced. As a result of all these applications, the success of treatment is quite high, especially in patients diagnosed at an early stage.

If you have been diagnosed with Zollinger-Ellison Syndrome or are experiencing symptoms related to this syndrome, you should apply to a healthcare institution immediately and have the necessary examinations under your doctor’s direction. In this way, your disease can be treated before it progresses, so you can live a healthy and long life.

 

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