Abstract

Exocrine pancreatic insufficiency (EPI), an important cause of maldigestion and malnutrition, results from primary pancreatic disease or is secondary to impaired exocrine pancreatic function. Although chronic pancreatitis is the most common cause of EPI, several additional causes exist. These include pancreatic tumors, pancreatic resection procedures, and cystic fibrosis. Other diseases and conditions, such as diabetes mellitus, celiac disease, inflammatory bowel disease, and advanced patient age, have also been shown to be associated with EPI, but the exact etiology of EPI has not been clearly elucidated in these cases. The causes of EPI can be divided into loss of pancreatic parenchyma, inhibition or inactivation of pancreatic secretion, and postcibal pancreatic asynchrony. Pancreatic enzyme replacement therapy (PERT) is indicated for the conditions described above presenting with clinically clear steatorrhea, weight loss, or symptoms related to maldigestion and malabsorption. This review summarizes the current literature concerning those etiologies of EPI less common than chronic pancreatitis, the pathophysiology of the mechanisms of EPI associated with each diagnosis, and treatment recommendations.

Highlights

  • Exocrine pancreatic insufficiency (EPI), which is an important cause of maldigestion, malabsorption, and subsequently malnutrition, arises from primary pancreatic diseases or is secondary to impaired exocrine pancreatic function [1]

  • The etiology of EPI can be divided into diseases having obvious association with EPI (A) and diseases and conditions where the exact mechanism2sorfe2s2ponsible for EPI have not been elucidated (B) (Table 1, adapted from [1,3])

  • Screening for and treatment of Small Intestinal Bacterial Overgrowth (SIBO) can be recommended for patients with EPI

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Summary

Introduction

Exocrine pancreatic insufficiency (EPI), which is an important cause of maldigestion, malabsorption, and subsequently malnutrition, arises from primary pancreatic diseases or is secondary to impaired exocrine pancreatic function (either inhibition or inactivation of pancreatic secretion or postictal pancreatic asynchrony) [1]. Pancr3eoafti2c2 enzyme replacement therapy (PERT) is indicated for all conditions with clinically clear steatorrhea, weight loss, or symptoms related to maldigestion and malabsorption [4,5,6]. Findings by Bruno et al indicate that patients with unresectable pancreatic cancer could benefit from PERT to decelerate weight loss [20]. In a double-blind, placebo-controlled study in patients with unresectable pancreatic cancer (43% had severe EPI), mean weight loss after 8 weeks in patients receiving PERT (−1.49%) was not significantly different from that in the placebo group (−2.99%). The value of PERT in PDAC patients is generally underappreciated It leads to increased median survival in patients with inoperable PDAC when compared with standard palliative therapy alone

Pancreatic Resections
Obstruction of the Pancreatic Duct
Exocrine Pancreatic Insufficiency in Children
Shwachman–Diamond Syndrome
Chronic Pancreatitis
Johanson–Blizzard Syndrome
Pearson Syndrome
Other Causes
Celiac Disease
Inflammatory Bowel Diseases
Gastrointestinal Surgeries Other Than Pancreatic Resections
Zollinger–Ellison Syndrome
Non-Alcoholic Fatty Pancreas Disease
Findings
Conclusions
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