Exocrine pancreatic insufficiency (EPI) is a condition in which people are unable to properly digest food due to a deficiency of the exocrine pancreatic enzyme.
Symptoms of exocrine pancreatic insufficiency include:
- Fat in stools
- Bulky stools that float in the water and are difficult to flush
- Stools may appear pale and oily
- Stools may be foul-smelling
- Weight loss
- Diarrhea (may be watery)
- Fatigue
- Gas (flatulence), which may cause abdominal cramping
- Abdominal distention
- Fluid retention/swelling (edema)
- Swelling of extremities
- Buildup of fluid in the lower abdomen (ascites)
- Anemia due to malabsorption of iron or vitamin B-12
- Bleeding disorders due to vitamin K malabsorption
- Metabolic bone disease due to vitamin D deficiency, which can result in osteopenia or osteomalacia, and in severe cases, bone pain and fractures
- Neurologic manifestations due to electrolyte disturbances (e.g., low calcium and low magnesium)
- Generalized motor weakness due to malabsorption of pantothenic acid and vitamin D
- Numbness and tingling of extremities due to malabsorption of thiamine
- Loss of a sense of vibration and position due to malabsorption of cobalamin
- Night blindness due to malabsorption of vitamin A
- Seizures due to malabsorption of biotin
What Causes Exocrine Pancreatic Insufficiency?
Exocrine pancreatic insufficiency has both pancreatic and non-pancreatic causes.
Pancreatic causes of exocrine pancreatic insufficiency include:
- Chronic pancreatitis (the most common cause of EPI)
- Cystic fibrosis
- Obstructions of the pancreatic duct (e.g., from pancreatic cancer or ampullary tumors)
- Shwachman-Diamond syndrome (SDS), a rare genetic disorder
- Diabetes
Non-pancreatic causes of exocrine pancreatic insufficiency include:
- Celiac disease
- Crohn’s disease
- Autoimmune pancreatitis
- Zollinger-Ellison syndrome
- Gastrointestinal and pancreatic surgical procedures
How Is Exocrine Pancreatic Insufficiency Diagnosed?
In addition to a patient history and physical examination, tests used to diagnose exocrine pancreatic insufficiency include:
- Blood tests
- Complete blood count (CBC)
- Serum iron, vitamin B-12, and folate concentrations
- Serum nutritional markers (e.g., magnesium, albumin, prealbumin)
- Electrolytes
- Erythrocyte sedimentation rate (ESR)
- Antigliadin and antiendomysial antibody levels
- Immunoglobulin A (IgA) levels
- Stool tests
- Fecal elastase and chymotrypsin (2 proteases produced by the pancreas)
- Tests of malabsorption
- Fat absorption tests
- D-xylose test
- Carbohydrate absorption test
- Bile salt absorption test
- Schilling test (for vitamin B-12)
- C-D-xylose breath test
- Pancreatic function tests
- Direct testing
- Endoscopy
- Dreiling tube method after stimulation with secretin or cholecystokinin (CCK)
- Secretin test
- CCK test
- Secretin-CCK test
- Indirect testing
- Fecal fat analysis
- Measurement of fecal elastase and fecal chymotrypsin levels
- Direct testing
- Abdominal imaging tests
What Is the Treatment for Exocrine Pancreatic Insufficiency?
Treatment for exocrine pancreatic insufficiency involves correcting nutritional deficiencies and treating any underlying diseases that may be causing the condition. Treatment for exocrine pancreatic insufficiency includes:
- Lifestyle modifications
- Vitamin supplementation
- Primarily with fat-soluble vitamins A, D, E, and K
- Pancreatic enzyme replacement therapy (PERT)
- This is the mainstay of treatment
- They should be taken with meals and snacks because they are intended to act on ingested foods so gastric emptying of nutrients occurs at the same time as the pancreatic enzymes reach the duodenum
- Pancreatic enzyme products (PEPs): pancrelipase (Creon, Pancreaze, Pertzye, Ultresa, Viokace, Zenpep)
- PEPs are not interchangeable
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References
https://emedicine.medscape.com/article/2121028-overview