An Overview of Zollinger-Ellison syndrome(ZES)

 

Zollinger-Ellison syndrome is one of the rare conditions in which more than one tumor grows in the pancreas or in the upper part of the small intestines. Usually, tumor are generally called gastrinomas. These gastrinomas typically produce a very huge amount of  hormones called gastrin. Gastrin usually causes the stomach to produce a huge amount of acid which generally leads to peptic ulcers. These high gastrin levels also cause diseases of belly pain and other symptoms.

Zollinger-Ellison syndrome disease might occur at any period in life. But people typically identify this disease sometimes in between the ages 20 to 60. The usual treatment of Zollinger-Ellison syndrome is medicines to cut down stomach acid levels and also medicines to heal the ulcers. Mostly people need surgery to remove the tumors.

Symptoms

In the endocrine system, people mostly witn Zollinger-Ellison syndrome have multiple tumors, as well as people have tumors in the pancreas.

Some common signs are similar to the symptoms of peptic ulcer which includes:

• discomfort or pain in the upper abdomen

• burning sensation and aching sensation in the upper abdomen

• diarrhea

• bleeding in the digestive tract

• general weakness

• black, “tarry” feces, which is result from bleeding in the digestive tract

• nausea

• unintentional weight loss

• low appetite or feeling full in stomach too quickly

• vomiting

Most people experience heartburn or gastroesophageal reflux, when the gastric acid and foods you eat from the stomach which backs up into the esophagus, that might be more severe.

Causes

Until now, Exact cause of Zollinger-Ellison syndrome is not known. But the sequence of symptoms which occurs in Zollinger-Ellison syndrome generally follows the same sequence. Typically, Zollinger-Ellison syndrome starts when one or more tumors form in the pancreas or in a part of your small intestine called the duodenum. Generally, duodenum is the section that can be connected to your stomach. In some cases, tumors forms at other parts of the body, such as lymph nodes which are next to your pancreas.

Generally, this pancreas sits behind and below your stomach. Pancreas produce enzymes which are needed for digesting foods you eat. These pancreas also produce many hormones which include insulin. Insulin is one of the hormones which helps to control your blood sugar and is also called glucose.

Juice from the digestive system like the pancreas, liver and gallbladder mix in the duodenum. Duodenum is the place where the most of your digestion happens.

Generally, tumors which occur with Zollinger-Ellison syndrome are generally made up of cells which usually release a very huge amount of the hormone called gastrin. Because of this, tumors may sometimes be called gastrinomas. That increased gastrin level causes the stomach to produce a high level of acid. That excess acid then leads to peptic ulcers followed by diarrhea.

Other than the excess acid production, the tumors are mostly cancerous. And also tumors grow slowly, but the cancer can spread elsewhere but it can be on nearby lymph nodes or your liver.

Diagnosis

Your healthcare physician will start by asking about your health history and symptoms. If your history and symptoms lead to Zollinger-Ellison syndrome then they will order some lab tests to check your gastrin levels or your stomach acid levels. If that lab test results are high then the physicians may suggest one or more imaging tests to identify gastrinomas in your digestive system. Your physicians want to know how many tumors you have, where the tumors are and how high the tumors are. But  the physicians want to sample one of the tumors to check it for cancer.

Some of the tests are:

 

Blood test. For this test, your physician will take a small sample of blood from your veins and test it in a lab. This test will help you to show high levels of gastrin in your blood circulation.

Stomach acid test.  Your physician wants to directly test your stomach acid levels. Physicians do this by taking fluid samples from your stomach through a nasogastric tube.This test can be called a gastric acid secretion test.

Radiology. Your physician will try to identify gastrinomas with different types of imaging tests like ultrasound, a CT scan or an MRI. These imaging tests are noninvasive because this test doesn’t go inside your body.

Endoscopy. Your physician will provide a closer look inside your body by the help of an endoscopic procedure.

Your healthcare provider wants to look inside your body by using an endoscopic procedure. This procedure involves passing an endoscope down to your throat. Your physician will use an endoscopic ultrasound to guide a needle to reach your tumor and then take a sample of the tumor.

Treatment

Your physicians may suggest a variety of treatments.

Proton pump inhibitors

Your healthcare provider will prescribe you drugs called proton pump inhibitors. These drugs are used to help reduce the gastric acids that are produced by this syndrome. And also, it helps to heal the ulcer.

Generally, these drugs are given in higher doses to people with ZES than to people who have ulcers without gastrinomas.

People who are taking these drugs will be carefully monitored. Long term use of proton pump inhibitors drugs will increase the risk of hip, wrist, and spine fractures, particularly after the age of 50.

Surgery

In some rare cases, you might need to remove the tumors. But surgeries are difficult because many gastrinomas are very small and tough to locate and it can be present in multiples.

Chemotherapy

Your physicians might suggest treatments like chemotherapy which helps to shrink the tumors or radiofrequency ablation which is to kill the cancer cells and also it stops the growth of the tumors.

Possible Complications

Some common complications are:

• During surgery, failure to locate the tumor

• intestinal bleeding and hole from ulcers in the stomach and duodenum part

• Severe diarrhea and sudden weightloss

• Tumor spreads to other organs

Preventing tumor growth

Some common treatment options to stops the growth of tumor which include:

• chemotherapy, which helps to slow the growth of tumors

• You should remove gastrinomas from the liver and it can prevent other tumors developing in the liver

• Other method called embolization, or cutting off the blood supply to the tumor(cancer cells)

• Or else directly inject drugs into the tumor

Frequently Asked Questions

Q1. What causes Zollinger-Ellison syndrome?

Ans: Zollinger-Ellison syndrome is caused by tumors. These growths are most often found in the head of the pancreas and the upper small intestine.

Q2. What is the treatment for Zollinger syndrome?

Ans: Medicines known as proton pump inhibitors are the first line of treatment. 

Q3. What gastric pH indicates Zollinger-Ellison?

Ans: Serum gastrin levels higher than 100 pg/mL are highly suggestive of Zollinger-Ellison syndrome (ZES). If the gastric pH level is less than 2, a gastrin level greater than 1000 pg/mL is diagnostic of this disease.

Q4. What is a normal gastrin level?

Ans: Normal values are generally less than 100 pg/mL (48.1 pmol/L). Normal value ranges may vary slightly among different laboratories.

Q5. What is the blood test for gastric acid?

Ans: The gastrin test is primarily used to help detect excess production of gastrin and gastric acid.