Pancreatic Insufficiency

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  • Also Known As:
  • Exocrine pancreatic insufficiency
  • EPI
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What is pancreatic insufficiency?

Pancreatic insufficiency is a condition in which the pancreas is not able to produce and/or transport enough digestive enzymes to break down food in the intestine. This condition is also known as exocrine pancreatic insufficiency or EPI.

The pancreas is a narrow, flat gland about six inches long located deep in your abdominal cavity, behind the stomach and below the liver. It has head, middle, and tail sections. Its head section connects to the first part of the small intestine (duodenum).

  • Inside the pancreas, small ducts (tubes) feed digestive enzymes and bicarbonate produced by the pancreas into the pancreatic duct. This large duct carries the digestive enzymes and bicarbonate down the length of the pancreas, from the tail to the head section, and into the duodenum.
  • The common bile duct also runs through the head section of the pancreas, carrying bile from the liver and gallbladder into the small intestine.
  • The bile duct and pancreatic duct usually join just before entering the duodenum and share a common opening into the small intestine.

The pancreas consists of two kinds of tissues that perform different functions:

  • The exocrine pancreas makes, stores and releases powerful enzymes to digest fats, proteins, and carbohydrates in the small intestine. The enzymes normally are produced and carried in an inactive form to the small intestine, where the enzymes are activated as needed. Exocrine tissues also make and release bicarbonate that neutralizes stomach acids and allows for the activation of pancreatic enzymes.
  • The endocrine pancreas produces hormones, including insulin and glucagon, and releases them into the blood. These hormones regulate sugar (glucose) transport into the body’s cells, where it is used for energy and to help maintain normal blood sugar levels.

In adults, pancreatic insufficiency typically occurs as a result of ongoing (progressive) pancreatic damage that may be caused by repeated bouts of acute pancreatitis or by chronic pancreatitis due to a variety of conditions.

In children, it is most frequently associated with cystic fibrosis (CF) or Shwachman-Diamond Syndrome (SDS). SDS is the second most common cause of inherited pancreatic insufficiency, after CF. All those with SDS have some degree of pancreatic insufficiency that begins when they are infants.

People with the following conditions may also develop EPI:

Zollinger-Ellison syndrome is a rare condition caused by a tumor of the pancreas that stimulates the cells of the stomach to release excess acid. This excess acid may cause temporary pancreatic insufficiency. Once the excess acid is treated, the pancreatic insufficiency usually goes away.

Some surgical procedures that affect the function of the digestive tract or the pancreas can lead to EPI.

 

About Pancreatic Insufficiency

Signs and Symptoms

The most common signs and symptoms of exocrine pancreatic insufficiency include:

  • Frequent gas and/or abdominal bloating
  • Abdominal pain, cramps (the pain is sometimes referred to as “epigastric” because it occurs in the upper middle part of the abdomen)
  • Unexplained weight loss (inability to gain weight in children)
  • Fatigue (tiredness)
  • Frequent diarrhea
  • Greasy, foul-smelling stools that sometimes float

People with EPI may experience malabsorption, malnutrition and vitamin deficiencies. Laboratory evaluation is often necessary to diagnose pancreatic insufficiency because symptoms may not be evident or may be similar to those of other digestive tract disorders.

Tests

Laboratory tests that may be used to detect pancreatic insufficiency include:

  • Stool elastase—this test measures the level of elastase (an enzyme made by the pancreas) in a sample of your stool. The amount of this enzyme is reduced in pancreatic insufficiency. This test is more effective at detecting moderate to severe EPI than milder cases.
  • Chymotrypsin—this test measures chymotrypsin (a pancreatic enzyme that breaks down proteins) in your stool. With EPI, chymotrypsin is absent in the stool.
  • Immunoreactive trypsinogen (IRT)—this is a blood test that measures trypsinogen, an inactive precursor produced by the pancreas that is converted to the enzyme trypsin. Trypsin is the pancreatic enzyme that digests proteins. IRT may be elevated with pancreatitis, which may cause EPI.
  • Fecal fat (fat in the stool)—this test detects excess fat in your stool, often the first sign of EPI. Because the stool elastase test (see above) is easier to perform, it has largely replaced this test.
  • 13C-mixed triglyceride breath test—this is a newer method of diagnosing moderate pancreatic insufficiency, but it is not widely used and not commonly available. The test may also be used to evaluate the effectiveness of enzyme therapy on fat digestion in people with EPI.

Non-laboratory tests used in diagnosing pancreatic insufficiency may include:

  • Computed tomography (CT) scan: can be used to detect inflammation or scarring of the pancreas
  • Endoscopic retrograde cholangiopancreatography (ERCP): an endoscope (a hollow tube) is guided through your mouth, down your throat and stomach to your small intestine. A smaller tube (catheter) is inserted through the endoscope to inject contrast dye into the pancreatic and bile ducts. Abdominal x-rays are taken to examine the pancreas, gallbladder, and the ducts that carry digestive enzymes from those organs to the small intestine. ERCP can be used look for blockages or damage to the ducts.
  • Magnetic resonance cholangiopancreatography (MRCP): a specific type of magnetic resonance imaging (MRI) that uses powerful magnets and radio waves to create images of the pancreas, pancreatic duct, and bile ducts. It is often used before or instead of ERCP to look for abnormalities in the pancreas because it is faster and non-invasive. It is also useful in distinguishing pancreatitis from pancreatic cancer.
  • Ultrasound: may be used to create and send images of your pancreas to a video monitor. An endoscopic ultra sound is an exam that involves a thin, flexible tube that is used to view inside the digestive tract and is more accurate than an abdominal ultrasound.

For more on imaging studies, see RadiologyInfo.org.

Treatment

Management of pancreatic insufficiency may include:

  • Taking enzyme supplements (pancreatic enzyme replacement therapy, PERT) to replace the enzymes your pancreas isn’t making any more
  • Eating a healthy, balanced diet; you might need guidance from a nutritionist to create a diet that meets your needs.
  • Taking vitamin supplements (especially vitamins A, D, E, and K) if needed
  • Making lifestyle changes, such not drinking alcohol and not smoking

Treatment of EPI also involves managing or resolving the underlying cause when possible.

In Shwachman-Diamond Syndrome (SDS), unlike in cystic fibrosis, production of pancreatic lipase (an enzyme that digests fats) often increases with age. The reasons for this are not yet known, but about 50% of children with SDS will experience improvements in pancreatic function and fat absorption as they get older and will no longer require enzyme replacement therapy.

For more information on other, less common conditions involving the pancreas, see Genetic and Rare Diseases Information Center.

Related Images

  • Location of the pancreas. Image credit: Don Bliss, National Cancer Institute
  • The pancreas and nearby organs and structures. Image credit: Don Bliss, National Cancer Institute

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Gregory C. Sephel PhD FACB MT(ASCP). Lab Tests Online adjunct board member. Director Clinical Pathology, VA TN Valley Healthcare System; Associate Professor Pathology, Microbiology, Immunology, Vanderbilt University School of Medicine.