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Zollinger-Ellison syndrome (ZES) is a rare digestive condition that causes the body to produce excess stomach acid, leading to several symptoms. ZES can affect people of any age, although it is most common in men aged between 30 and 50. According to the National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK), around one in every one million people suffer from Zollinger-Ellison syndrome. 
Zollinger-Ellison syndrome causes tumors to form in the patient’s pancreas, small intestine, or lymph nodes located nearby. These tumors (known as gastrinomas) produce excess amounts of the hormone gastrin. Unnecessarily high amounts of this hormone result in the stomach producing higher amounts of gastric acid. Stomach acid is needed in order to break down the foods that we eat. However, excessive stomach acid can lead to painful stomach ulcers and diarrhea. Around 90 percent of ZES patients develop ulcers in the stomach or small intestine.  
Keep reading to learn more about Zollinger-Ellison syndrome, including the symptoms, causes, and how it can be treated using medications such as pantoprazole (Protonix).
Symptoms of Zollinger-Ellison Syndrome
There are several common symptoms of Zollinger-Ellison syndrome. However, not all patients display signs, which can make the condition difficult to diagnose. Symptoms are similar to those of other more common digestive conditions, including peptic ulcer disease and gastroesophageal reflux disease (GERD).
The most common symptoms of this condition include abdominal pain and diarrhea. If you have these symptoms or any of the following, then speak to your doctor:
- Vomiting, which may contain blood
- Unexplained weight loss
- Severe heartburn
- Loss of appetite
- Pain or a burning feeling in the abdomen
- Weakness or fatigue
- Signs of intestinal bleeding such as black, bloody, or tarry stools  
Along with the unpleasant symptoms of Zollinger-Ellison syndrome, complications may also occur. Around half of ZES tumors are cancerous (malignant).  These gastrinomas can spread to other areas of the body if they are not treated. Cancerous tumors can spread to the liver, spleen, skin, bones, and lymph nodes. Gastrinomas usually grow and spread slowly. If they have not spread to the liver, then the five-year survival rate can be as high as 90 percent. 
What Causes Zollinger-Ellison Syndrome?
The majority of the time, the exact cause of Zollinger-Ellison syndrome is unknown. It is currently thought of as a sporadic condition that occurs spontaneously.  Around 25 percent of the time, Zollinger-Ellison syndrome is a result of endocrine neoplasia type 1 (MEN1). As well as ZES, MEN1 can also cause several types of endocrine cancers. 
Zollinger-Ellison syndrome is thought to be partly genetic because MEN1 is a hereditary condition. People that have a parent with ZES have around a 50 percent chance of developing the condition themselves. 
Treating Zollinger-Ellison Syndrome with Pantoprazole
The aim of ZES treatment is to remove tumors that cause the condition and control the production of stomach acid. Once ZES has been treated, patients should still visit their doctor regularly to check for possible re-occurrences. 
There are several tests that may be used to help diagnose Zollinger-Ellison syndrome. ZES is a rare condition that shares symptoms with other more common conditions, so it is important that it is diagnosed correctly.
Blood tests can identify if you have high levels of the hormone gastrin. However, elevated gastrin levels do not necessarily indicate ZES because other conditions and medications can increase gastrin levels.
Doctors may also use imaging tests and endoscopic ultrasounds to locate tumors in the stomach, pancreas, and small intestine. Another method of diagnosis is an upper gastrointestinal endoscopy, which involves a video camera inserted down the throat to look for ulcers. Doctors may also remove a sample of tissue during this procedure. 
As well as removing tumors, it is important that the production of stomach acid is controlled. This is typically achieved by using a proton pump inhibitor (PPI) drugs. A common PPI medication is pantoprazole (Protonix). Pantoprazole works by hindering small ‘pumps’ that are found inside cells that produce stomach acid. Once the levels of stomach acid are lowered, then peptic ulcers have a better chance to heal. This can reduce the symptoms of Zollinger-Ellison syndrome.
c. Removing Tumors
Tumors that are caused by Zollinger-Ellison syndrome are often very small and hard to find. If your doctor believes that there is only one tumor, then surgery may be the best option to remove it. Surgery may still be used to remove large tumors even if you have several other smaller ones. Other methods to remove tumors can include chemotherapy, cutting off the blood supply, injecting drugs to relieve cancer symptoms, or by destroying cancerous cells with heat. 
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