Guidelines Summary

Guidelines concerning the diagnosis, etiology, and management of pancreatic exocrine insufficiency (PEI) were voted on by pancreatic specialists at the 2019 Annual Meeting of the Pancreatic Society of Great Britain and Ireland and published in 2021. Some, but not all, of the recommendations are listed below^[46]:

The FE-1 test is recommended as a suitable first-line PEI exam, despite the fact that the gold-standard diagnostic test for PEI is considered to be the coefficient of fat absorption
If a diagnosis of PEI is unclear, it can be supported by employing positive markers of malnutrition, including clinical history, anthropometric measurements, or serum micronutrient levels (such as magnesium, vitamin E, and retinol-binding protein/vitamin A); when diagnosing PEI, however, do not consider these markers in isolation
It is recommended that PERT commence at a dose of at least 50,000 units lipase with meals and 25,000 units lipase with snacks; if this is ineffective, dose adjustment by the patient should be encouraged
Patients should spread pancreatic enzyme consumption throughout a meal
Patients should avoid diets that are very high in fiber, but restriction of dietary fat is not routinely recommended
No significant complications are associated with PERT
PERT’s efficacy is impacted by acid and temperature; if PERT is ineffective, a proton pump inhibitor can boost its usefulness, but if, after addition of the inhibitor, one of the PERT agents is still not effective, it is recommended that a trial be conducted of an alternative PERT preparation
With limited evidence suggesting an association between long-term use of very–high-dose PERT and fibrosing colonopathy in children with cystic fibrosis, doses in this population should be restricted to 10,000 units lipase/kg/day

Medication

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Media Gallery

Complete replacement of the pancreas with cystic disease and intrahepatic biliary dilation caused by extrinsic compression of the common bile duct. Note also the renal cysts and masses. This patient had exocrine pancreatic insufficiency. Image courtesy of Wikimedia Commons.
Residual islets in dense fibrous stroma secondary to loss of exocrine pancreatic tissue in chronic pancreatitis (hematoxylin-eosin stain, medium magnification). Image courtesy of Dr. Rose Anton.
Pancreas anatomy.
The duodenum and pancreas.
The pancreas and duodenum, posterior view.
Factors controlling release of pancreatic secretions. Image courtesy of Wikimedia Commons.
Defective protein transmembrane conductance regulator (CFTR) in cystic fibrosis.

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Sections Exocrine Pancreatic Insufficiency
Overview
Presentation
DDx
Workup
Treatment
Guidelines
Medication
Questions & Answers
Media Gallery
Tables
References

Pancreatic Enzyme Product	Lipase Content, units
Creon 1203	3000
Creon 1206	6000
Creon 1212	12,000
Creon 1224	24,000
Zenpep EURAND 3	3000
Zenpep EURAND 5	5000
Zenpep EURAND 10	10,000
Zenpep EURAND 15	15,000
Zenpep EURAND 20	20,000
Zenpep EURAND 25	25,000
Pancreaze MT 4	4200
Pancreaze MT 10	10,500
Pancreaze MT 16	16,800
Pancrease MT 24	21,000
Ultresa 13800UL	13,800
Ultresa 20700UL	20,700
Ultresa 23000UL	23,000
Viokace 9111	10,440
Viokace 9116	20,880
Pertzye 8	8000
Pertzye 16	16,000

Exocrine Pancreatic Insufficiency Guidelines

Guidelines Summary

References

Tables

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