Copyright ©The Author(s) 2019. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastroenterol. May 28, 2019; 25(20): 2430-2441
Published online May 28, 2019. doi: 10.3748/wjg.v25.i20.2430
Contribution of pancreatic enzyme replacement therapy to survival and quality of life in patients with pancreatic exocrine insufficiency
Peter Layer, Nataliya Kashirskaya, Natalya Gubergrits
Peter Layer, Department of Internal Medicine, Israelitic Hospital, Hamburg 22297, Germany
Nataliya Kashirskaya, Department of Genetic Epidemiology (Cystic Fibrosis group), Federal State Budgetary Institution “Research Centre for Medical Genetics”, Moscow 115522, Russia
Natalya Gubergrits, Department of Internal Medicine, Donetsk National Medical University, Lyman 83001, Ukraine
Author contributions: All authors contributed to the interpretation of the data, drafting of the manuscript, approval of the final version, and agree to be accountable for all aspects of the work.
Conflict-of-interest statement: Layer P has participated in consulting and speaking activities for Abbott Laboratories Ltd, Allergan plc, Almirall, Dr. Falk Pharma GmbH, Nordmark Arzneimittel GmbH & Co. KG and Shire. Kashirskaya N has participated in consulting and speaking activities for Abbott Laboratories Ltd, Chiesi Pharmaceuticals B.V., Pharmaxis Ltd, PRO.MED.CS Praha a.s., Nordmark Arzneimittel GmbH & Co. KG. Gubergrits N has no relevant conflicts of interest to be declared.
Open-Access: This is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See:
Corresponding author: Peter Layer, AGAF, FACG, MD, PhD, Professor, Department of Internal Medicine, Israelitic Hospital, Orchideenstieg 14, Hamburg 22297, Germany.
Telephone: +49-40-511255001 Fax: +49-40-511255009
Received: January 22, 2019
Peer-review started: January 22, 2019
First decision: February 21, 2019
Revised: April 2, 2019
Accepted: April 19, 2019
Article in press: April 20, 2019
Published online: May 28, 2019

The objective of this study was to analyze the current evidence for the use of pancreatic enzyme replacement therapy (PERT) in affecting survival and quality of life in patients with pancreatic exocrine insufficiency (PEI). Systematic searches of the literature were performed using the PubMed database. Articles were selected for inclusion if they reported findings from trials assessing the effects of PERT on quality of life, survival, malabsorption, growth parameters (such as height, body weight and body mass index), or gastrointestinal symptoms (such as abdominal pain, stool consistency and flatulence). PERT improved PEI-related malabsorption and weight maintenance in patients with cystic fibrosis, chronic pancreatitis, pancreatic cancer, and post-surgical states. In patients with chronic pancreatitis, PERT improved PEI-related symptoms and quality of life measures. Several small retrospective studies have also suggested that PERT may have a positive impact on survival, but long-term studies assessing this effect were not identified. PERT is effective for treating malnutrition and supporting weight maintenance, and it is associated with improved quality of life and possibly with enhanced survival in patients with PEI. However, there is evidence that not all patients with PEI receive adequate PERT. Future work should aim to assess the long-term effects of PERT on the survival of patients with PEI.

Keywords: Pancreatic exocrine insufficiency, Pancreatic enzyme replacement therapy, Survival, Quality of life, Malabsorption, Cystic fibrosis, Chronic pancreatitis, Pancreatic cancer, Post-surgical states

Core tip: Malnutrition associated with pancreatic exocrine insufficiency (PEI) can have serious consequences, leading to increased morbidity and reduced survival. Awareness and education may be needed to ensure that patients with PEI are managed appropriately in order to increase their survival and improve long-term quality of life. There is currently a lack of evidence for the long-term effects of pancreatic enzyme replacement therapy (PERT) on survival in patients with PEI; however, there is substantial evidence supporting its efficacy in improving PEI-related malnutrition. Furthermore, the available evidence indicates that PERT improves PEI-related symptoms and quality of life measures in patients with PEI.