Exocrine pancreatic insufficiency

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Exocrine pancreatic insufficiency


Q: How is exocrine pancreatic insufficiency (EPI) tested?
A: EPI can be tested using various methods, including the 72-hour fecal fat test and the fecal elastase test.

Q: What is the myth regarding fecal elastase testing for EPI in patients with chronic diarrhea?
A: The myth is that routinely checking fecal elastase in these patients is a useful and effective diagnostic approach, with values of 100–200 μg/g of stool indicating mild to moderate pancreatic insufficiency, and values below 100 μg/g indicating severe EPI.

Q: What is the expert opinion on using fecal elastase testing for EPI in patients with chronic diarrhea?
A: The expert opinion is that fecal elastase testing is unreliable for diagnosing EPI in the absence of testing formed stool.

Q: What are the limitations of other tests for EPI?
A: Many other tests, like serum trypsin levels and qualitative stool fat tests, have poor sensitivity or specificity and may only be available at limited centers (e.g., 13C mixed triglyceride breath test).

Q: What is the gold standard test for EPI?
A: The 72-hour fecal fat test is considered the gold standard. It involves collecting stool over 72 hours while consuming a high-fat diet, diagnosing fat malabsorption based on fat content in stool. However, this test is time-consuming and can cause discomfort, and errors may occur in stool collection and fat intake recording.

Q: How does the fecal elastase test work?
A: The fecal elastase test measures pancreatic elastase 1, a stable enzyme in stool. This test has high sensitivity in moderate to severe EPI when using formed stools, does not require a special diet, and has a high negative predictive value.

Q: What are the reference ranges for fecal elastase values?
A: Values less than 200 μg/g of stool are used to diagnose EPI, with values below 100 μg/g considered diagnostic of severe EPI. Values between 100 and 200 μg/g are often indeterminate.

Q: What are the limitations of fecal elastase testing?
A: In cases of mild to moderate EPI, fecal elastase testing may have lower sensitivity and specificity. Additionally, watery stools can result in falsely low elastase values. Therefore, it is important to test formed stool for accurate results.

Q: How does fecal elastase testing compare to the 72-hour fecal fat test?
A: Fecal elastase testing has largely replaced the 72-hour fecal fat test due to its non-invasive nature and ease of use, although both tests are used to assess the effectiveness of pancreatic enzyme replacement therapy (PERT).


Q: What is the myth about starting pancreatic enzyme replacement therapy (PERT)?
A: The myth is that PERT should be started at the lowest dose available and taken any time before a meal and at bedtime.

Q: What is the expert opinion on the administration of PERT?
A: PERT should be titrated to the degree of insufficiency and administered with the first bite of a meal. Additional enzymes may be added during or towards the end of the meal.

Q: What role does dietary management play in treating exocrine pancreatic insufficiency (EPI)?
A: Dietary management aims to address fat malabsorption, which causes symptoms like weight loss and deficiencies in fat-soluble vitamins A, D, E, and K. A low-fat diet is recommended for chronic pancreatitis, while a high-fat diet is used in cystic fibrosis (CF) with increased PERT to improve lung disease. Fat-soluble vitamins should be supplemented and taken with PERT. Consulting a dietitian is helpful, and smoking and alcohol cessation are recommended due to their negative impact on pancreatic function.

Q: What is the goal of pancreatic enzyme replacement therapy (PERT)?
A: The goal of PERT is to eliminate malabsorption, reduce symptoms of maldigestion, and prevent malnutrition-related complications.

Q: How has the regulation of PERTs changed over time?
A: Before 2010, pancreatic enzymes were not FDA regulated and had variable consistency. Since 2010, the FDA mandates approval for all prescribed PERT formulations to ensure consistency.

Q: What types of PERT preparations are available, and what are their characteristics?
A: PERTs are extracts from porcine pancreas and come in forms like mini-microspheres or microtablets with varying particle sizes and pH-related release kinetics. Enteric-coated microspheres protect lipase from gastric acid but may be less effective in CF due to lower duodenal pH.

Q: What are some PERT brands and their lipase unit options?
A: FDA-approved PERT brands and their lipase units include:

  • Creon: 3000; 6000; 12,000; 24,000; 36,000
  • Zenpep: 3000; 5000; 10,000; 15,000; 20,000; 25,000
  • Pancreaze: 4200; 10,500; 16,800; 21,000
  • Ultresa: 13,800; 20,700; 23,000
  • Viokase: 10,440; 20,880 (requires acid suppression)
  • Pertzye: 8000; 16,000

Q: How should PERT dosing and frequency be adjusted?
A: PERT should be taken with the first bite of a meal, with additional doses during or towards the end of the meal based on meal duration. For meals less than 15 minutes, all enzymes can be taken at the beginning. For longer meals, split doses accordingly. There is no universal consensus on dosing, with various recommendations suggesting different administration schedules.

Q: What should be considered if there is a poor response to PERT?
A: Consider increasing the dosage, checking patient compliance, adding acid inhibitors, and evaluating the timing of enzyme administration. It may also be necessary to look for concurrent gastrointestinal disorders or issues with diet.

Q: What are the suggested PERT dosing guidelines for different age groups?
A:

  • Infants: 2000–4000 units per 120 mL of formula or breastmilk
  • Children < 4 years: 1000 units/kg per meal; 500 units/kg per snack
  • Children ≥ 4 years: 500 units/kg per meal; 250 units/kg per snack
  • Adults: Starting dose of 50,000 units per meal; 25,000 units per snack. Maximum dose of 150,000 units per meal; 70,000 units per snack.


Q: What changes have occurred in the treatment of exocrine pancreatic insufficiency (EPI) since the FDA approval of pancreatic enzyme replacement therapy (PERT) in 2010?
A: Since the FDA approval in 2010, reliable PERT medications have improved symptoms of EPI such as steatorrhea, abdominal discomfort, weight loss, and malnutrition. PERT has been lifesaving for patients with cystic fibrosis (CF), who previously faced high mortality from malnutrition within the first year of life.

Q: What challenges remain in the treatment of EPI despite the availability of PERT?
A: There is still confusion among medical practitioners regarding the best diagnostic methods, dosing, and administration of PERT. Different countries have developed varying guidelines, highlighting the need for a consensus and practical guide for diagnosing and treating EPI.

Q: What changes have occurred in diagnostic approaches for EPI?
A: Diagnostic approaches have shifted from unreliable qualitative stool tests and the cumbersome 72-hour fecal fat collection to the more sensitive, though less specific, fecal elastase test. This is particularly important for diagnosing mild to moderate EPI, though there are challenges with analyzing formed stools in cases of chronic diarrhea.

Q: What areas need emphasis in the management of EPI?
A: Two key areas needing emphasis are:

  1. Standardizing diagnostic approaches and methods for EPI.
  2. Optimizing the use of available therapies, including determining the best dosing and administration schedules for PERT.

Q: What are the current recommendations for administering PERT?
A: Recommendations for PERT include:

  1. Titrating the dose to the degree of pancreatic insufficiency.
  2. Administering PERT with the first bite of a meal and considering additional doses during or towards the end of the meal.
  3. Using microspheres and possibly adding rapid-release enzyme preparations and/or acid-blockade.
  4. Adjusting the dose based on the fat content of the meal.

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