Pancreatic Insufficiency

Pancreatic insufficiency usually presents with signs and symptoms of malabsorption, malnutrition, vitamin deficiencies, abdominal cramps, and weight loss (or, in children, an inability to gain weight) and is often associated with steatorrhea (loose, fatty, foul-smelling stools). Diabetes may also be present in adults with pancreatic insufficiency.

Laboratory evaluation is often necessary to diagnose pancreatic insufficiency because symptoms may not be evident or may be similar to those of other gastrointestinal disorders.

Laboratory tests used to detect pancreatic insufficiency

• Elastase is a protein-cleaving enzyme produced and secreted by the pancreas. It is resistant to degradation by other enzymes and so is excreted and can be measured in the stool. The amount of this enzyme is reduced in pancreatic insufficiency. This test, also known as the fecal elastase-1 test, is often chosen to aid in the diagnosis of pancreatic insufficiency in children and adults; however, it is more effective at detecting severe exocrine pancreatic insufficiency and not as effective for mild to moderate forms.
• Chymotrypsin is a pancreatic enzyme that cleaves proteins. Unlike elastase, chymotrypsin is digested while passing through the intestine; however, there remains sufficient residual activity for measurement.
• Fecal fat (fat in the stool) is often the first sign of insufficiency. The simplest test for fecal fat is to place a suspension of stool onto a glass slide, add a fat stain, and observe the number and size of fat globules that are present. A more accurate assessment of fecal fat requires a timed stool collection, typically spanning 72 hours, and a dietary sheet to help calculate the total fat intake during the collection period. This test is generally thought of as unreliable because it is difficult to achieve proper control of the diet, challenging to ensure complete sample collection, and there is significant test variation.
• ¹³C-mixed triglyceride breath test is a novel test that is a noninvasive, reliable 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 efficacy of enzyme therapy on fat digestion in people with insufficiency.

Non-laboratory tests used in diagnosing pancreatic insufficiency

• Endoscopic retrograde cholangiopancreatography (ERCP): a test that uses a flexible scope inserted through the mouth and threaded through the esophagus to see and document damage to the pancreas and/or bile ducts.
• Magnetic resonance cholangiopancreatography (MRCP): a type of magnetic resonance imaging (MRI) used to examine the pancreas and bile ducts.
• Secretin test (not widely available): a tube is positioned in the duodenum to collect pancreatic secretions stimulated by intravenous (IV) secretin. Secretin is a hormone that causes the pancreas to release fluid containing digestive enzymes. The amount of certain enzymes and bicarbonate in this fluid is measured and compared to normal values.

For more on imaging studies, see RadiologyInfo.org.

Treatment of pancreatic insufficiency involves resolving the underlying condition, where possible, to prevent or minimize further pancreatic damage and manage the symptoms.

Those affected may be given oral preparations of pancreatic enzymes as a supplement, a treatment known as pancreatic enzyme replacement therapy or PERT, to make up for decreased enzyme production and to aid digestion. The primary purpose of this therapy is to ensure proper nutritional status. The therapy involves taking porcine (pig)-derived pancreatic enzyme products (PEPs), which are similar to human pancreatic enzymes, to supplement normal pancreatic function. The enzymes lipase, amylase, and protease (important pancreatic enzymes) are contained in the PEP mixture and aid in digesting fats, carbohydrates and proteins.

People with pancreatic insufficiency may also be given vitamin supplements, especially the fat-soluble vitamins A, D, E, and K, since absorption of fats (and therefore absorption of the aforementioned vitamins) is frequently impaired. They may be put on a diet low in fat and high in protein and calories to help gain and maintain weight. Limiting of alcohol intake may also be required because alcohol can cause further pancreatic damage.

In Shwachman-Diamond Syndrome (SDS), unlike in cystic fibrosis, secretion 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 Pancreas.org: Rare Syndromes.