Abstract

Exocrine pancreatic insufficiency (EPI) is characterized by inadequate pancreatic enzyme delivery to the small intestine Exocrine pancreatic insufficiency (EPI) is characterized by inadequate pancreatic enzyme delivery to the small intestine, resulting in malabsorption. Clinical manifestations of EPI are often nonspecific and can lead to lack of timely recognition and diagnosis. Central to this clinical dilemma is the lack of highly accurate or specific testing which leads to misdiagnosis and suboptimal treatment. Identification of high-risk patients is key in the diagnosis of EPI and this includes patients with pancreatic parenchyma disorders such as chronic pancreatitis, pancreatic malignancy, cystic fibrosis, and those undergoing pancreatic resection for benign and malignant disease. Less recognized are the number of additional conditions which may also have EPI as a consequence. Owing to an increase in morbidity and impaired quality of life associated with this condition, goals of treatment have been aimed at repleting exocrine enzyme deficiency by oral pancreatic enzyme replacement therapy (PERT). The basis of PERT is to provide activated digestive enzymes to the small bowel during the prandial period, mainly, leading to sufficient absorption of fat and fat-soluble vitamins. The benefits of PERT have been shown to go beyond the improvement in signs and symptoms associated with EPI and include decreasing prevalence of osteopathy and improving survival outcomes in subsets of patients with this condition. However, despite the overall benefits in treatment, the diagnosis and management of EPI are suboptimal. Current literature suggests patients at high risk of developing EPI are not tested and those who are diagnosed are not treated with adequate dosages. In this review, we highlight patients who are at high risk for the development of EPI, analyze consequences and treatment of this disorder, review rationale for enzyme replacement therapy, and examine current evidence for treatment optimization.

Highlights

  • Exocrine pancreatic insufficiency (EPI) is characterized by the maldigestion of macronutrients and micronutrients as a result of inadequate intraduodenal pancreatic exocrine enzyme delivery

  • The symptoms and manifestations of EPI are not specific and are shared with other common gastrointestinal conditions. This can lead to a lack of recognition and a lack of diagnostic testing in many patients. Even in those who are correctly diagnosed, many are not treated with appropriate dosages of pancreatic enzyme replacement therapy (PERT)

  • EPI is frequently an unrecognized consequence of benign and malignant pancreatic disease, leading to various consequences such as abdominal discomfort, steatorrhea, malnutrition, sarcopenia, osteoporosis, weight loss, reduced quality of life, and diminished survival. Despite these rather severe outcomes, the diagnosis and management of EPI is suboptimal, which underscores the importance of awareness in high-risk patients, appropriate use of diagnostic testing, and understanding of treatment goals and strategies

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Summary

Introduction

Exocrine pancreatic insufficiency (EPI) is characterized by the maldigestion of macronutrients and micronutrients as a result of inadequate intraduodenal pancreatic exocrine enzyme delivery. The symptoms and manifestations of EPI are not specific and are shared with other common gastrointestinal conditions. This can lead to a lack of recognition and a lack of diagnostic testing in many patients. Levels above 200 mcg/g stool are normal, and levels between 100 and 200 are indeterminate[11] Many shortcomings of this test exist and include high false positive rates dependent on cut-off levels chosen, stool consistency at the time of sampling (may be falsely low in watery stool because of dilution), and low fecal elastase in advanced age and other medical conditions such as chronic kidney disease or diabetes mellitus (DM). The diagnosis of EPI is often heavily reliant on the identification of patients who are at increased risk and who present with suggestive clinical symptoms

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