Pancreatitis and WAGR Syndrome

What Is Pancreatitis?

The pancreas is an important organ. It releases insulin and glucagon into the bloodstream. These are hormones that help the body use the glucose (sugar) it takes from food for energy.

The pancreas also secretes digestive enzymes into the small intestines through a small tube called the pancreatic duct. These enzymes help digest fats, proteins, and carbohydrates in food. Digestive enzymes usually do not become active until they reach the small intestine, where they begin digesting food. If these enzymes become active while still inside the pancreas, they start "digesting" the pancreas itself.

There are two types of pancreatitis: “Acute,” and “Chronic.” Acute pancreatitis occurs suddenly and lasts for a short period of time. Chronic pancreatitis does not resolve and can result in a slow destruction of the pancreas. Either type of pancreatitis can cause serious complications including bleeding, tissue damage and infection. In extreme cases there can also be damage to other organs.

What causes Pancreatitis?

There are two reasons why people with WAGR syndrome may be susceptible to pancreatitis. First, the PAX6 gene is deleted in most people with WAGR syndrome. This gene is involved in the development of the pancreas, and it is possible that deletions of this gene may result in defects in the anatomy or function of the pancreas.

Another reason some people with WAGR syndrome may develop pancreatitis is a condition called “hyperlipidemia” or “hypertriglyceridemia.” These conditions occur

when there are high levels of fatty molecules in the blood, called lipids. It is thought that having high levels of fats in the blood increases the risk of pancreatitis.

Certain drugs can also cause pancreatitis. These include a wide variety of common medications and anesthetic agents.

Diagnosis

Signs of acute pancreatitis can include:

·       Nausea

·       Vomiting with or without diarrhea

·       Upper stomach pain that may or may not penetrate to the back

·     Fever

·     Rapid pulse

·     Anxiety

·     Restlessness or irritability

·     Tender abdomen upon examination

·     Discoloration of bowel movements (yellow and/or gray)

During an acute attack, the blood contains high levels of amylase and lipase. Amylase and lipase are digestive enzymes formed in the pancreas. A doctor can order a blood test to look for higher than normal levels of these enzymes, which may indicate that pancreatitis is present. The doctor may also order imaging tests, such as x-rays or CT or MRI scans.

In chronic pancreatitis, patients usually have abdominal pain. The pain may get worse when eating or drinking. The pain may spread to the back or become constant and disabling. Other symptoms may include nausea, vomiting, weight loss, oily stools, frequent bowel movements with passage of undigested food.

One important consideration for the diagnosis of pancreatitis in people with WAGR syndrome is the possibility of altered pain perception. Some people with WAGR syndrome appear to have an abnormally high tolerance for pain. In these cases, the person may not exhibit signs of pain or discomfort, even when acute pancreatitis is present.

In addition, individuals with WAGR syndrome who are nonverbal may have difficulty communicating their pain. These individuals may express pain as a change in behavior. For example, they may become withdrawn, clingy, or aggressive.

TREATMENT

Treatment for pancreatitis might include medication for pain relief, placing a patient on an IV or an all liquid diet following an acute attack, and then gradually moving to a diet high in carbohydrates and low in fat. Antibiotics may be prescribed. A doctor may also prescribe pancreatic enzymes to be taken with meals if it is determined that the pancreas is not secreting enough enzymes on its own.

PREVENTION

It is not known why some people with WAGR syndrome develop pancreatitis. It is possible that the risk may be reduced somewhat by eating a diet low in fat, and managing hyperlipidemia with medication when necessary. Patients and caregivers should be aware that some foods and medications contain alcohol, which can cause or worsen an episode of pancreatitis.

Physicians should be aware that many drugs are associated with pancreatitis. Since these drugs include a wide variety of commonly prescribed medications and anesthetic agents (for list, see references below) risk versus benefit of the use of these drugs should be discussed by caregivers.

For more information about pancreatitis:

http://digestive.niddk.nih.gov/ddiseases/pubs/pancreatitis

http://www.emedicinehealth.com/pancreatitis/article_em.htm

References:

See: National Institutes of Health Study of WAGR syndrome, 11p Deletions, and Aniridia 2006 - 2014 Summary of Results (Unpublished findings).

Drug-induced acute pancreatitis. Kaurich T. Proc (Bayl Univ Med Cent). 2008 Jan;21(1):77-81.

Drug induced acute pancreatitis: an evidence-based review. Badalov N, Baradarian R, Iswara K, Li J, Steinberg W, Tenner S. Clin Gastroenterol Hepatol 2007;5(6):648661.

Drug-induced pancreatitis: an update. Trivedi CD, Pitchumoni CS. J Clin Gastroenterol. 2005 Sep;39(8):709-16.

 

Last Updated: May, 2015

Kelly Trout, BSN, RN

Health Consultant

International WAGR Syndrome Association