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Zollinger-Ellison syndrome (Z-E syndrome) is a symptom complex that is the clinical manifestation of excessive gastrin secretion. Zollinger-Ellison syndrome is usually diagnosed when a patient has complaints about the symptoms of peptic ulcer disease, but treatment is not having the desired effect.

Zollinger-Ellison syndromeis a very rare disease syndrome and consists of an inadequate amount of gastrin secreted, which stimulates the production of hydrochloric acid in the stomach. A tumor in the gastrointestinal tract is responsible for the production of this hormone. Usually it is the duodenum, but involvement of other structures, such as the pancreas or upper gastrointestinal lymph nodes, is observed. Symptoms of the Z-E syndrome are very often clinically similar to peptic ulcer disease, therefore the suspicion of the discussed pathology may appear only when standard methods of peptic ulcer disease treatment turn out to be ineffective. The therapeutic management of the Z-E syndrome is a combination of pharmacotherapy - proton pump inhibitors and surgical removal of the tumor. Even in a quarter of patients with. Z-E is part of the type 1 endocrine neoplasia syndrome, abbreviated MEN. It is a genetically determined tendency to create nodular changes within the parathyroid glands, pancreatic islet cells and the anterior pituitary gland.

Zollinger-Ellison syndrome: causes

The causes of the Z-E syndrome are believed to be a neoplastic tumor, medical term gastrinoma, which can be observed in the duodenum, pancreas or lymph nodes. Unfortunately, the pathological formation tends to grow and often metastasizes to nearby organs, e.g. the liver. High levels of hydrochloric acid are responsible for the ulcer formation which can be distinguished from typical gastric and duodenal ulcers by the lack of treatment effectiveness. The diseases mainly concern men, usually after the age of 40.

What are the symptoms of Zollinger-Ellison syndrome?

It should be noted that the clinical manifestation is similar to that of peptic ulcer disease. Common symptoms of the syndrome include:

  • peptic ulcers not responding to treatment, despite the use of various forms of therapy, with a tendency to relapse
  • diarrhea
  • pain complaints - the pain is located primarily inupper abdomen, with a characteristic time of onset. Well, patients report a feeling of discomfort 1-3 hours after eating a meal, usually during the night or immediately after waking up.
  • nausea
  • vomiting
  • in patients with Z-E syndrome as a component of MEN 1 syndrome, the presence of pancreatic islet, pituitary tumor or hyperparathyroidism is synchronously observed.

How to make a correct diagnosis?

In order to make a diagnosis, it is necessary, first of all, to conduct a reliable interview with the patient. The doctor asks carefully about the nature of the reported symptoms and the circumstances of their occurrence. The gastrinoma tumor itself requires imaging diagnostics. You can additionally confirm the disease by conducting laboratory tests - in most cases the concentration of gastrin exceeds the upper limit of normal even ten times and the pH of gastric juice is strongly acidic.

Imaging tests are very useful and verify the doctor's doubts. The most popular method is endosonography. It consists in inserting the probe into the gastrointestinal tract through the oral cavity. This is a specific type of ultrasound in which the doctor assesses the condition of the abdominal organs. It can assess whether a given image should raise anxiety and should be considered in the context of the neoplastic process. Endoscopy has one more advantage - it allows you to collect material for histopathological examination.

Scintigraphy is a tracer test. It is programmed to detect the sites / clusters of cells involved in the overproduction of gastrin in this case. Of course, if the above-mentioned diagnostic methods fail, alternative computed tomography or magnetic resonance imaging remains.

Treatment of the Z-E syndrome

The main goal of the therapy is to reduce ailments that reduce the quality of life. Doctors strive to heal the ulcer as quickly as possible. Effectiveness depends on the possibility of radical tumor removal. Unfortunately, the diagnosis of metastases is a bad prognostic factor and in such a case complete recovery is impossible. Pharmacotherapy in the Z-E syndrome consists mainly of drugs that inhibit the excessive secretion of hydrochloric acid. Dosage of drugs is based on regular intake of preparations on an empty stomach, about 30 minutes before a meal.

In addition to the therapy, all activities aimed at proper wound healing are important. Smoking and the frequent use of NSAIDs (non-steroidal anti-inflammatory drugs) are not recommended. Acetylsalicylic acid, a component of many common preparations, also has a negative effect. Moreover, it is necessary to be carefulobservation of the diet and its possible modification. Perhaps the kitchen uses products that aggravate ailments that should be eliminated.

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