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Abstract
Pancreatic exocrine insufficiency (PEI) is defined as a reduction in pancreatic exocrine secretion below a level that allows normal digestion of nutrients. Pancreatic disease and pancreatic surgery are the main causes of PEI, but other conditions and upper gastrointestinal surgery can affect the digestive function of the pancreas. PEI can cause symptoms of nutrient malabsorption and nutritional deficiencies, which affect quality of life and increase morbidity and mortality. There is no test allowing for the diagnosis of PEI. Diagnosis should therefore be based on a global assessment of symptoms, nutritional status and a pancreatic secretion test (e.g. faecal elastase). Pancreatic enzyme replacement therapy, together with dietary advice and support, are the cornerstones of PEI therapy. The diagnosis and treatment of PEI varies widely between countries and centres, and there is considerable discrepancies as to whom and how to diagnose and treat. Following the UEG framework for the development of high-quality clinical guidelines, the European multidisciplinary guideline on the diagnosis and treatment of PEI has been developed. This evidence-based guideline summarises the prevalence, clinical impact and general diagnostic and therapeutic approach to PEI, as well as the specifics of PEI in different clinical conditions. Finally, unmet needs for future research are discussed.
References
DiMagno EP, Go VL, Summerskill WH. Relations between pancreatic enzyme ouputs and malabsorption in severe pancreatic insufficiency. N Eng J Med. 1973;288(16):813-5.
Hoffmeister A, Mayerle J, Beglinger C, Büchler M, Bufler P, Dathe K, et al. S3-Leitlinie Chronische Pankreatitis: Definition, Ätiologie, Diagnostik, konservative, interventionell endoskopische und operative Therapie der chronischen Pankreatitis. Leitlinie der Deutschen Gesellschaft für Verdauungs- und Stoffwechselkrankheiten (DGVS). Z Gastroenterol. 2012;50(11):1176-224.
Löhr JM. UEG LINK Award from the National Societies to HaPanEU: Harmonising the diagnosis and treatment of Pancreatitis across EUrope. United European Gastroenterol J. 2015;3(5):483.
Löhr JM, Dominguez-Munoz E, Rosendahl J, Besselink M, Mayerle J, Lerch MM, et al. United European Gastroenterology evidence-based guidelines for the diagnosis and therapy of chronic pancreatitis (HaPanEU). United European Gastroenterol J. 2017;5(2):153-99.
Lewis D. An Updated Review of Exocrine Pancreatic Insufficiency Prevalence finds EPI to be More Common in General Population than Rates of Co-Conditions. J Gastrointestin Liver Dis. 2024.
Chen G, Wang K, Sha Y, Wang D. Current status and prospect of pancreatic exocrine insufficiency (PEI): A bibliometric and visualized study. Asian J Surg. 2024.
Keller J, Layer P. Human pancreatic exocrine response to nutrients in health and disease. Gut. 2005;54 Suppl 6:vi1-28.
Boltin D, Lambregts DM, Jones F, Siterman M, Bonovas S, Cornberg M, et al. UEG framework for the development of high-quality clinical guidelines. United European Gastroenterol J. 2020;8(8):851-64.
Löhr JM, Beuers U, Vujasinovic M, Alvaro D, Frøkjær JB, Buttgereit B, et al. European Guideline on IgG4-related digestive disease — UEG and SGF evidence-based recommendations. UEG Journal. 2020;8(6):637-66.
Hoffmeister A, Mayerle J, Beglinger C, Buchler MW, Bufler P, Dathe K, et al. Englischsprachige Version der S3-Leitlinie Chronische Pankreatitis. Z Gastroenterol. 2015;53(12):1447-95.
Khan M, Rutkowski W, Vujasinovic M, Löhr JM. Adherence to European guidelines for treatment and management of pancreatic exocrine insufficiency in chronic pancreatitis patients. J Clin Med. 2021;10:2737.
Speckman RA, Friedly JL. Asking Structured, Answerable Clinical Questions Using the Population, Intervention/Comparator, Outcome (PICO) Framework. Pm r. 2019;11(5):548-53.
Howick J, Chalmers I, Glasziou P, Greenhalgh T, Heneghan C, Liberati A, et al. Oxford centre for evidence-based medicine 2011 levels of evidence. Centre for Evidence-Based Medicine Retrieved July from https://www cebm net/wp-content/uploads/2014/06/CEBM-Levels-of-Evidence-21 pdf. 2011.
Guyatt GH, Oxman AD, Vist GE, Kunz R, Falck-Ytter Y, Alonso-Coello P, Schünemann HJ. GRADE: an emerging consensus on rating quality of evidence and strength of recommendations. Bmj. 2008;336(7650):924-6.
Phillips ME, Hopper AD, Leeds JS, Roberts KJ, McGeeney L, Duggan SN, Kumar R. Consensus for the management of pancreatic exocrine insufficiency: UK practical guidelines. BMJ Open Gastroenterol. 2021;8(1).
Layer P, Keller J. Pancreatic enzymes: secretion and luminal nutrient digestion in health and disease. J Clin Gastroenterol. 1999;28(1):3-10.
de la Iglesia-García D, Huang W, Szatmary P, Baston-Rey I, Gonzalez-Lopez J, Prada-Ramallal G, et al. Efficacy of pancreatic enzyme replacement therapy in chronic pancreatitis: systematic review and meta-analysis. Gut. 2017;66(8):1354-5.
Huang W, de la Iglesia-Garcia D, Baston-Rey I, Calvino-Suarez C, Larino-Noia J, Iglesias-Garcia J, et al. Exocrine Pancreatic Insufficiency Following Acute Pancreatitis: Systematic Review and Meta-Analysis. Dig Dis Sci. 2019;64(7):1985-2005.
Iglesia D, Avci B, Kiriukova M, Panic N, Bozhychko M, Sandru V, et al. Pancreatic exocrine insufficiency and pancreatic enzyme replacement therapy in patients with advanced pancreatic cancer: A systematic review and meta-analysis. United European Gastroenterol J. 2020;8(9):1115-25.
Scholten L, Stoop TF, Del Chiaro M, Busch OR, van Eijck C, Molenaar IQ, et al. Systematic review of functional outcome and quality of life after total pancreatectomy. Br J Surg. 2019;106(13):1735-46.
Chaudhary A, Domínguez-Muñoz JE, Layer P, Lerch MM. Pancreatic Exocrine Insufficiency as a Complication of Gastrointestinal Surgery and the Impact of Pancreatic Enzyme Replacement Therapy. Dig Dis. 2020;38(1):53-68.
Beger HG, Mayer B, Poch B. Resection of the duodenum causes long-term endocrine and exocrine dysfunction after Whipple procedure for benign tumors — Results of a systematic review and meta-analysis. HPB (Oxford). 2020;22(6):809-20.
Straatman J, Wiegel J, van der Wielen N, Jansma EP, Cuesta MA, van der Peet DL. Systematic Review of Exocrine Pancreatic Insufficiency after Gastrectomy for Cancer. Dig Surg. 2017;34(5):364-70.
Uribarri-Gonzalez L, Nieto-Garcia L, Martis-Sueiro A, Dominguez-Munoz JE. Exocrine pancreatic function and dynamic of digestion after restrictive and malabsorptive bariatric surgery: a prospective, cross-sectional, and comparative study. Surg Obes Relat Dis. 2021;17(10):1766-72.
Lindkvist B, Phillips ME, Dominguez-Munoz JE. Clinical, anthropometric and laboratory nutritional markers of pancreatic exocrine insufficiency: Prevalence and diagnostic use. Pancreatology. 2015;15(6):589-97.
Kuan LL, Dennison AR, Garcea G. Prevalence and Impact of Sarcopenia in Chronic Pancreatitis: A Review of the Literature. World J Surg. 2021;45(2):590-7.
Martinez-Moneo E, Stigliano S, Hedström A, Kaczka A, Malvik M, Waldthaler A, et al. Deficiency of fat-soluble vitamins in chronic pancreatitis: A systematic review and meta-analysis. Pancreatology. 2016;16(6):988-94.
Capurso G, Signoretti M, Archibugi L, Stigliano S, Delle Fave G. Systematic review and meta-analysis: Small intestinal bacterial overgrowth in chronic pancreatitis. United European Gastroenterol J. 2016;4(5):697-705.
El Kurdi B, Babar S, El Iskandarani M, Bataineh A, Lerch MM, Young M, Singh VP. Factors That Affect Prevalence of Small Intestinal Bacterial Overgrowth in Chronic Pancreatitis: A Systematic Review, Meta-Analysis, and Meta-Regression. Clin Transl Gastroenterol. 2019;10(9):e00072.
Frost F, Kacprowski T, Ruhlemann M, Pietzner M, Bang C, Franke A, et al. Long-term instability of the intestinal microbiome is associated with metabolic liver disease, low microbiota diversity, diabetes mellitus and impaired exocrine pancreatic function. Gut. 2021;70(3):522-30.
Shandro BM, Ritehnia J, Chen J, Nagarajah R, Poullis A. The investigation and management of pancreatic exocrine insufficiency: A retrospective cohort study. Clin Med (Lond). 2020;20(6):535-40.
Lindkvist B. Diagnosis and treatment of pancreatic exocrine insufficiency. World J Gastroenterol. 2013;19(42):7258-66.
Nikfarjam M, Wilson JS, Smith RC, Australasian Pancreatic Club Pancreatic Enzyme Replacement Therapy Guidelines Working G. Diagnosis and management of pancreatic exocrine insufficiency. Med J Aust. 2017;207(4):161-5.
Dominguez-Munoz JE. Diagnosis and treatment of pancreatic exocrine insufficiency. Curr Opin Gastroenterol. 2018;34(5):349-54.
Arasaradnam RP, Brown S, Forbes A, Fox MR, Hungin P, Kelman L, et al. Guidelines for the investigation of chronic diarrhoea in adults: British Society of Gastroenterology, 3rd edition. Gut. 2018;67(8):1380-99.
Vanga RR, Tansel A, Sidiq S, El-Serag HB, Othman MO. Diagnostic Performance of Measurement of Fecal Elastase-1 in Detection of Exocrine Pancreatic Insufficiency: Systematic Review and Meta-analysis. Clin Gastroenterol Hepatol. 2018;16(8):1220-8 e4.
Dominguez-Munoz JE, P DH, Lerch MM, Löhr MJ. Potential for Screening for Pancreatic Exocrine Insufficiency Using the Fecal Elastase-1 Test. Dig Dis Sci. 2017;62(5):1119-30.
Tseng DS, Molenaar IQ, Besselink MG, van Eijck CH, Borel Rinkes IH, van Santvoort HC. Pancreatic Exocrine Insufficiency in Patients With Pancreatic or Periampullary Cancer: A Systematic Review. Pancreas. 2016;45(3):325-30.
Kroon VJ, Daamen LA, Tseng DSJ, de Vreugd AR, Brada LJH, Busch OR, et al. Pancreatic exocrine insufficiency following pancreatoduodenectomy: A prospective bi-center study. Pancreatology. 2022;22(7):1020-7.
Pezzilli R, Capurso G, Falconi M, Frulloni L, Macarri G, Costamagna G, et al. The Applicability of a Checklist for the Diagnosis and Treatment of Exocrine Pancreatic Insufficiency: Results of the Italian Exocrine Pancreatic Insufficiency Registry. Pancreas. 2020;49(6):793-8.
Jalal M, Campbell JA, Tesfaye S, Al-Mukhtar A, Hopper AD. Yield of testing for micronutrient deficiencies associated with pancreatic exocrine insufficiency in a clinical setting: An observational study. World journal of clinical cases. 2021;9(36):11320-9.
Kolodziejczyk E, Wejnarska K, Dadalski M, Kierkus J, Ryzko J, Oracz G. The nutritional status and factors contributing to malnutrition in children with chronic pancreatitis. Pancreatology. 2014;14(4):275-9.
Lindkvist B, Dominguez-Munoz JE, Luaces-Regueira M, Castineiras-Alvarino M, Nieto-Garcia L, Iglesias-Garcia J. Serum nutritional markers for prediction of pancreatic exocrine insufficiency in chronic pancreatitis. Pancreatology. 2012;12(4):305-10.
Mascarenhas MR, Mondick J, Barrett JS, Wilson M, Stallings VA, Schall JI. Malabsorption blood test: Assessing fat absorption in patients with cystic fibrosis and pancreatic insufficiency. Journal of clinical pharmacology. 2015;55(8):854-65.
Muniz CK, dos Santos JS, Pfrimer K, Ferrioli E, Kemp R, Marchini JS, Cunha SF. Nutritional status, fecal elastase-1, and 13C-labeled mixed triglyceride breath test in the long-term after pancreaticoduodenectomy. Pancreas. 2014;43(3):445-50.
Rana M, Wong-See D, Katz T, Gaskin K, Whitehead B, Jaffe A, et al. Fat-soluble vitamin deficiency in children and adolescents with cystic fibrosis. J Clin Pathol. 2014;67(7):605-8.
Shintakuya R, Uemura K, Murakami Y, Kondo N, Nakagawa N, Urabe K, et al. Sarcopenia is closely associated with pancreatic exocrine insufficiency in patients with pancreatic disease. Pancreatology. 2017;17(1):70-5.
Vanacor R, Raimundo FV, Marcondes NA, Corte BP, Ascoli AM, Azambuja AZ, et al. Prevalence of low bone mineral density in adolescents and adults with cystic fibrosis. Rev Assoc Med Bras (1992). 2014;60(1):53-8.
Vujasinovic M, Tepes B, Makuc J, Rudolf S, Zaletel J, Vidmar T, et al. Pancreatic exocrine insufficiency, diabetes mellitus and serum nutritional markers after acute pancreatitis. World J Gastroenterol. 2014;20(48):18432-8.
Vujasinovic M, Hedström A, Maisonneuve P, Valente R, von Horn H, Löhr JM, Haas SL. Zinc deficiency in patients with chronic pancreatitis. World J Gastroenterol. 2019;25(5):600-7.
Vujasinovic M, Nezirevic Dobrijevic L, Asplund E, Rutkowski W, Dugic A, Kahn M, et al. Low Bone Mineral Density and Risk for Osteoporotic Fractures in Patients with Chronic Pancreatitis. Nutrients. 2021;13(7).
Mann ST, Mann V, Stracke H, Lange U, Klor HU, Hardt P, Teichmann J. Fecal elastase 1 and vitamin D3 in patients with osteoporotic bone fractures. Eur J Med Res. 2008;13(2):68-72.
Del Rosario MA, Fitzgerald JF, Gupta SK, Croffie JM. Direct measurement of pancreatic enzymes after stimulation with secretin versus secretin plus cholecystokinin. J Pediatr Gastroenterol Nutr. 2000;31(1):28-32.
Stevens T, Conwell DL, Zuccaro G, Jr., Lewis SA, Love TE. The efficiency of endoscopic pancreatic function testing is optimized using duodenal aspirates at 30 and 45 minutes after intravenous secretin. Am J Gastroenterol. 2007;102(2):297-301.
Brydon WG, Kingstone K, Ghosh S. Limitations of faecal elastase-1 and chymotrypsin as tests of exocrine pancreatic disease in adults. Ann Clin Biochem. 2004;41(Pt 1):78-81.
Vantrappen GR, Rutgeerts PJ, Ghoos YF, Hiele MI. Mixed triglyceride breath test: a noninvasive test of pancreatic lipase activity in the duodenum. Gastroenterology. 1989;96(4):1126-34.
Keller J, Hammer HF, Afolabi PR, Benninga M, Borrelli O, Dominguez-Munoz E, et al. European guideline on indications, performance and clinical impact of (13) C-breath tests in adult and pediatric patients: An EAGEN, ESNM, and ESPGHAN consensus, supported by EPC. United European Gastroenterol J. 2021;9(5):598-625.
Dominguez-Munoz JE, Iglesias-Garcia J, Vilarino-Insua M, Iglesias-Rey M. 13C-mixed triglyceride breath test to assess oral enzyme substitution therapy in patients with chronic pancreatitis. Clin Gastroenterol Hepatol. 2007;5 SRC — GoogleScholar:484-8.
Bali MA, Sztantics A, Metens T, Arvanitakis M, Delhaye M, Deviere J, Matos C. Quantification of pancreatic exocrine function with secretin-enhanced magnetic resonance cholangiopancreatography: normal values and short-term effects of pancreatic duct drainage procedures in chronic pancreatitis. Initial results. Eur Radiol. 2005;15(10):2110-21.
Manfredi R, Brizi MG, Tancioni V, Vecchioli A, Marano P. Magnetic resonance pancreatography (MRP): morphology and function. Rays. 2001;26(2):127-33.
Dominguez-Munoz JE, Drewes AM, Lindkvist B, Ewald N, Czako L, Rosendahl J, et al. Recommendations from the United European Gastroenterology evidence-based guidelines for the diagnosis and therapy of chronic pancreatitis. Pancreatology. 2018;18(8):847-54.
Forsmark CE. Management of chronic pancreatitis. Gastroenterology. 2013;144(6):1282-91 e3.
Duggan SN. Negotiating the complexities of exocrine and endocrine dysfunction in chronic pancreatitis. Proc Nutr Soc. 2017;76(4):484-94.
Duggan S, O'Sullivan M, Feehan S, Ridgway P, Conlon K. Nutrition treatment of deficiency and malnutrition in chronic pancreatitis: a review. Nutr Clin Pract. 2010;25(4):362-70.
Gan C, Chen YH, Liu L, Gao JH, Tong H, Tang CW, Liu R. Efficacy and safety of pancreatic enzyme replacement therapy on exocrine pancreatic insufficiency: a meta-analysis. Oncotarget. 2017;8(55):94920-31.
Kahl S, Schutte K, Glasbrenner B, Mayerle J, Simon P, Henniges F, et al. The effect of oral pancreatic enzyme supplementation on the course and outcome of acute pancreatitis: a randomized, double-blind parallel-group study. JOP. 2014;15(2):165-74.
Gubergrits N, Malecka-Panas E, Lehman GA, Vasileva G, Shen Y, Sander-Struckmeier S, et al. A 6-month, open-label clinical trial of pancrelipase delayed-release capsules (Creon) in patients with exocrine pancreatic insufficiency due to chronic pancreatitis or pancreatic surgery. Aliment Pharmacol Ther. 2011;33(10):1152-61.
D'Haese JG, Ceyhan GO, Demir IE, Layer P, Uhl W, Löhr M, et al. Pancreatic enzyme replacement therapy in patients with exocrine pancreatic insufficiency due to chronic pancreatitis: a 1-year disease management study on symptom control and quality of life. Pancreas. 2014;43(6):834-41.
Ramesh H, Reddy N, Bhatia S, Rajkumar JS, Bapaye A, Kini D, et al. A 51-week, open-label clinical trial in India to assess the efficacy and safety of pancreatin 40000 enteric-coated minimicrospheres in patients with pancreatic exocrine insufficiency due to chronic pancreatitis. Pancreatology. 2013;13(2):133-9.
de la Iglesia-Garcia D, Vallejo-Senra N, Iglesias-Garcia J, Lopez-Lopez A, Nieto L, Dominguez-Munoz JE. Increased Risk of Mortality Associated With Pancreatic Exocrine Insufficiency in Patients With Chronic Pancreatitis. J Clin Gastroenterol. 2018;52(8):e63-e72.
Roberts KJ, Bannister CA, Schrem H. Enzyme replacement improves survival among patients with pancreatic cancer: Results of a population based study. Pancreatology. 2019;19(1):114-21.
Roberts KJ, Schrem H, Hodson J, Angelico R, Dasari BVM, Coldham CA, et al. Pancreas exocrine replacement therapy is associated with increased survival following pancreatoduodenectomy for periampullary malignancy. HPB (Oxford). 2017;19(10):859-67.
Löhr JM, Hummel FM, Pirilis KT, Steinkamp G, Korner A, Henniges F. Properties of different pancreatin preparations used in pancreatic exocrine insufficiency. Eur J Gastroenterol Hepatol. 2009;21(9):1024-31.
Maev IV, Kucheryavyy YA, Gubergrits NB, Bonnacker I, Shelest EA, Janssen-van Solingen GP, Dominguez-Munoz JE. Differences in In Vitro Properties of Pancreatin Preparations for Pancreatic Exocrine Insufficiency as Marketed in Russia and CIS. Drugs R D. 2020;20(4):369-76.
Shrikhande SV, Prasad VGM, Dominguez-Munoz JE, Weigl KE, Sarda KD. In vitro Comparison of Pancreatic Enzyme Preparations Available in the Indian Market. Drug Des Devel Ther. 2021;15:3835-43.
Layer P, Holtmann G. Pancreatic enzymes in chronic pancreatitis. Int J Pancreatol. 1994;15(1):1-11.
Shandro BM, Nagarajah R, Poullis A. Challenges in the management of pancreatic exocrine insufficiency. World J Gastrointest Pharmacol Ther. 2018;9(5):39-46.
Kühnelt P, Mundlos S, Adler G. Einfluß der Pelletgröße eines Pankreasenzympräparates auf die duodenale lipolytische Aktivität. Z Gastroenterol. 1991;29:417-21.
Gan KH, Geus WP, Bakker W, Lamers CB, Heijerman HG. In vitro dissolution profiles of enteric-coated microsphere/microtablet pancreatin preparations at different pH values. Aliment Pharmacol Ther. 1996;10(5):771-5.
Meyer JH, Elashoff J, Porter-Fink V, Dressman J, Amidon GL. Human postprandial gastric emptying of 1-3-millimeter spheres. Gastroenterology. 1988;94(6):1315-25.
Stead RJ, Skypala I, Hodson ME, Batten JC. Enteric coated microspheres of pancreatin in the treatment of cystic fibrosis: comparison with a standard enteric coated preparation. Thorax. 1987;42(7):533-7.
Norregaard P, Lysgaard Madsen J, Larsen S, Worning H. Gastric emptying of pancreatin granules and dietary lipids in pancreatic insufficiency. Aliment Pharmacol Ther. 1996;10(3):427-32.
Mundlos S, Kuhnelt P, Adler G. Monitoring enzyme replacement treatment in exocrine pancreatic insufficiency using the cholesteryl octanoate breath test. Gut. 1990;31(11):1324-8.
Dutta SK, Rubin J, Harvey J. Comparative evaluation of the therapeutic efficacy of a pH-sensitive enteric coated pancreatic enzyme preparation with conventional pancreatic enzyme therapy in the treatment of exocrine pancreatic insufficiency. Gastroenterology. 1983;84(3):476-82.
Konstan M, Wagener J, Wilschanski M, Laki I, Boas S, Sands D, et al. A comparison of liprotamase, a non-porcine pancreatic enzyme replacement therapy, to porcine extracted pancrelipase in a noninferiority randomized clinical trial in patients with cystic fibrosis. Clin Investig. 2018;8:147-54.
de-Madaria E, Abad-Gonzalez A, Aparicio JR, Aparisi L, Boadas J, Boix E, et al. The Spanish Pancreatic Club's recommendations for the diagnosis and treatment of chronic pancreatitis: part 2 (treatment). Pancreatology. 2013;13(1):18-28.
Working Party of the Australasian Pancreatic C, Smith RC, Smith SF, Wilson J, Pearce C, Wray N, et al. Summary and recommendations from the Australasian guidelines for the management of pancreatic exocrine insufficiency. Pancreatology. 2016;16(2):164-80.
Gheorghe C, Seicean A, Saftoiu A, Tantau M, Dumitru E, Jinga M, et al. Romanian guidelines on the diagnosis and treatment of exocrine pancreatic insufficiency. J Gastrointestin Liver Dis. 2015;24(1):117-23.
Turck D, Braegger CP, Colombo C, Declercq D, Morton A, Pancheva R, et al. ESPEN-ESPGHAN-ECFS guidelines on nutrition care for infants, children, and adults with cystic fibrosis. Clinical nutrition. 2016;35(3):557-77.
Borowitz D, Gelfond D, Maguiness K, Heubi JE, Ramsey B. Maximal daily dose of pancreatic enzyme replacement therapy in infants with cystic fibrosis: a reconsideration. Journal of Cystic Fibrosis. 2013;12(6):784-5.
Ng C, Major G, Smyth AR. Timing of pancreatic enzyme replacement therapy (PERT) in cystic fibrosis. Cochrane Database of Systematic Reviews. 2021(8).
Dominguez‐Munoz J, Iglesias‐Garcia J, Iglesias‐Rey M, Figueiras A, Vilariño‐Insua M. Effect of the administration schedule on the therapeutic efficacy of oral pancreatic enzyme supplements in patients with exocrine pancreatic insufficiency: a randomized, three‐way crossover study. Alimentary pharmacology & therapeutics. 2005;21(8):993-1000.
Arvanitakis M, Ockenga J, Bezmarevic M, Gianotti L, Krznarić Ž, Lobo DN, et al. ESPEN guideline on clinical nutrition in acute and chronic pancreatitis. Clinical Nutrition. 2020;39(3):612-31.
Somaraju URR, Solis-Moya A. Pancreatic enzyme replacement therapy for people with cystic fibrosis. Cochrane Database of systematic reviews. 2020(8).
Layer P, Kashirskaya N, Gubergrits N. Contribution of pancreatic enzyme replacement therapy to survival and quality of life in patients with pancreatic exocrine insufficiency. World Journal of Gastroenterology. 2019;25(20):2430.
Gooden H, White K. Pancreatic cancer and supportive care—pancreatic exocrine insufficiency negatively impacts on quality of life. Supportive Care in Cancer. 2013;21:1835-41.
Dunleavy L, Al-Mukhtar A, Halliday V. Pancreatic enzyme replacement therapy following surgery for pancreatic cancer: an exploration of patient self-management. Clinical nutrition ESPEN. 2018;26:97-103.
Landers A, Donaldson F, Brown H, McKenzie C, Pendharkar S. The impact of pancreatic enzyme replacement therapy on patients with advanced pancreatic adenocarcinoma: a systematic review and narrative synthesis. Pancreas. 2022;51(5):405-14.
Bruno MJ, Haverkort EB, Tijssen GP, Tytgat GN, van Leeuwen DJ. Placebo controlled trial of enteric coated pancreatin microsphere treatment in patients with unresectable cancer of the pancreatic head region. Gut. 1998;42(1):92-6.
Halm U, Loser C, Löhr M, Katschinski M, Mössner J. A double-blind, randomized, multicentre, crossover study to prove equivalence of pancreatin minimicrospheres versus microspheres in exocrine pancreatic insufficiency. Aliment Pharmacol Ther. 1999;13(7):951-7.
Neoptolemos J, Ghaneh P, Andrén-Sandberg Å, Bramhall S, Patankar R, Kleibeuker J, Johnson C. Treatment of pancreatic exocrine insufficiency after pancreatic resection: Results of a randomized, double-blind, placebo-controlled, crossover study of high vs standard dose pancreatin. International journal of pancreatology. 1999;25:171-80.
Vecht J, Symersky T, Lamers CB, Masclee AA. Efficacy of lower than standard doses of pancreatic enzyme supplementation therapy during acid inhibition in patients with pancreatic exocrine insufficiency. Journal of clinical gastroenterology. 2006;40(8):721-5.
Heijerman HG, Lamers CB, Bakker W, Dijkman JH. Improvement of fecal fat excretion after addition of omeprazole to pancrease in cystic fibrosis is related to residual exocrine function of the pancreas. Digestive diseases and sciences. 1993;38:1-6.
Nakamura T, Takebe K, Kudoh K, Ishii M, Imamura K, Kikuchi H, et al. Effects of pancreatic digestive enzymes, sodium bicarbonate, and a proton pump inhibitor on steatorrhoea caused by pancreatic diseases. Journal of international medical research. 1995;23(1):37-47.
Dominguez-Munoz JE, Iglesias-Garcia J, Iglesias-Rey M, Vilarino-Insua M. Optimising the therapy of exocrine pancreatic insufficiency by the association of a proton pump inhibitor to enteric coated pancreatic extracts. Gut. 2006;55(7):1056-7.
Chonchubhair HMN, Bashir Y, Dobson M, Ryan BM, Duggan SN, Conlon KC. The prevalence of small intestinal bacterial overgrowth in non-surgical patients with chronic pancreatitis and pancreatic exocrine insufficiency (PEI). Pancreatology. 2018;18(4):379-85.
Muniz CK, dos Santos JS, Pfrimer K, Ferrioli E, Kemp R, Marchini JS, Cunha SF. Nutritional status, fecal elastase-1, and 13C-labeled mixed triglyceride breath test in the long-term after pancreaticoduodenectomy. Pancreas. 2014;43(3):445-50.
Ferrone M, Raimondo M, Scolapio JS. Pancreatic enzyme pharmacotherapy. Pharmacotherapy: The Journal of Human Pharmacology and Drug Therapy. 2007;27(6):910-20.
Bergner A, Bergner RK. Pulmonary hypersensitivity associated with pancreatin powder exposure. Pediatrics. 1975;55(6):814-7.
Thornton CS, Waddell BJ, Congly SE, Svishchuk J, Somayaji R, Fatovich L, et al. Porcine-derived pancreatic enzyme replacement therapy may be linked to chronic hepatitis E virus infection in cystic fibrosis lung transplant recipients. Gut. 2024.
Whitcomb DC, Lehman GA, Vasileva G, Malecka-Panas E, Gubergrits N, Shen Y, et al. Pancrelipase delayed-release capsules (CREON) for exocrine pancreatic insufficiency due to chronic pancreatitis or pancreatic surgery: A double-blind randomized trial. Am J Gastroenterol. 2010;105(10):2276-86.
Taylor C, Thieroff-Ekerdt R, Shiff S, Magnus L, Fleming R, Gommoll C. Comparison of two pancreatic enzyme products for exocrine insufficiency in patients with cystic fibrosis. Journal of Cystic Fibrosis. 2016;15(5):675-80.
Giuliano CA, Dehoorne-Smith ML, Kale-Pradhan PB. Pancreatic enzyme products: digesting the changes. Annals of Pharmacotherapy. 2011;45(5):658-66.
FitzSimmons SC, Burkhardt GA, Borowitz D, Grand RJ, Hammerstrom T, Durie PR, Lloyd-Stil JD. High-dose pancreatic-enzyme supplements and fibrosing colonopathy in children with cystic fibrosis. N Eng J Med. 1997;336:1283-9.
Serban DE, Florescu P, Miu N. Fibrosing colonopathy revealing cystic fibrosis in a neonate before any pancreatic enzyme supplementation. Journal of pediatric gastroenterology and nutrition. 2002;35(3):356-9.
Spasova V, Koleva L, Toncheva D, Karamisheva V. Case report of a successful pregnancy in a cystic fibrosis patient with the c. 1521_1523delCTT/c. 3718-2477C> t genotypes. Balkan Journal of Medical Genetics. 2020;23(2):103-6.
Cordell V, Osoba L. Pregnancy in a patient with Schwachman-Diamond syndrome. Case Reports. 2015;2015:bcr2015209644.
Horne GA, Chevassut T. Pregnancy in Shwachman-Diamond syndrome: a novel genetic mutation with minimal consequence. Case Reports. 2012;2012:bcr2012007305.
Powers HJ. Approaches to setting dietary reference values for micronutrients, and translation into recommendations. Proceedings of the Nutrition Society. 2021;80(3):365-72.
Ferrie S, Graham C, Hoyle M. Pancreatic enzyme supplementation for patients receiving enteral feeds. Nutrition in clinical practice. 2011;26(3):349-51.
Berry A, Gettle LS, Phillips ME. Pancreatic exocrine insufficiency and enteral feeding: a practical guide with case studies. PRACTICAL GASTROENTEROLOGY. 2018:63.
Lieb II JG, Patel D, Karnik N, Toskes PP. Study of the gastrointestinal bioavailability of a pancreatic extract product (Zenpep) in chronic pancreatitis patients with exocrine pancreatic insufficiency. Pancreatology. 2020;20(6):1092-102.
Hauenschild A, Ewald N, Klauke T, Liebchen A, Bretzel RG, Kloer HU, Hardt PD. Effect of liquid pancreatic enzymes on the assimilation of fat in different liquid formula diets. JPEN J Parenter Enteral Nutr. 2008;32(1):98-100.
Borowitz D, Stevens C, Brettman LR, Campion M, Wilschanski M, Thompson H, Group LS. Liprotamase long-term safety and support of nutritional status in pancreatic-insufficient cystic fibrosis. Journal of pediatric gastroenterology and nutrition. 2012;54(2):248-57.
Samarasekera E, Mahammed S, Carlisle S, Charnley R. Pancreatitis: summary of NICE guidance. BMJ. 2018;362.
Sikkens EC, Cahen DL, van Eijck C, Kuipers EJ, Bruno MJ. Patients with exocrine insufficiency due to chronic pancreatitis are undertreated: a Dutch national survey. Pancreatology. 2012;12(1):71-3.
Singh S, Midha S, Singh N, Joshi YK, Garg PK. Dietary counseling versus dietary supplements for malnutrition in chronic pancreatitis: a randomized controlled trial. Clinical Gastroenterology and Hepatology. 2008;6(3):353-9.
Harvey P, McKay S, Wilkin R, Layton G, Powell-Brett S, Okoth K, et al. Pancreatic enzyme replacement therapy in patients with pancreatic cancer: A national prospective study. Pancreatology. 2021;21(6):1127-34.
Phillips ME, McGeeney LM, Griffin O, Freeman K, Dann S, Duggan SN. Training 1,200 dietitians: An evaluation of a training course for non-specialist dietitians on the management of pancreatic exocrine insufficiency. Clinical Nutrition Open Science. 2022;44:155-62.
Tiengou LE, Gloro R, Pouzoulet J, Bouhier K, Read MH, Arnaud‐Battandier F, et al. Semi‐elemental formula or polymeric formula: is there a better choice for enteral nutrition in acute pancreatitis? Randomized comparative study. Journal of Parenteral and Enteral Nutrition. 2006;30(1):1-5.
Layer P, Yamamoto H, Kalthoff L, Clain JE, Bakken LJ, DiMagno EP. The different courses of early- and late-onset idiopathic and alcoholic chronic pancreatitis. Gastroenterology. 1994;107(5):1481-7.
Ammann RW, Buehler H, Muench R, Freiburghaus AW, Siegenthaler W. Differences in the natural history of idiopathic (nonalcoholic) and alcoholic chronic pancreatitis. A comparative long-term study of 287 patients. Pancreas. 1987;2(4):368-77.
Olesen SS, Poulsen JL, Drewes AM, Frøkjær JB, Laukkarinen J, Parhiala M, et al. The Scandinavian baltic pancreatic club (SBPC) database: design, rationale and characterisation of the study cohort. Scand J Gastroenterol. 2017;52(8):909-15.
Machicado JD, Chari ST, Timmons L, Tang G, Yadav D. A population-based evaluation of the natural history of chronic pancreatitis. Pancreatology. 2018;18(1):39-45.
Kempeneers MA, Ahmed Ali U, Issa Y, van Goor H, Drenth JPH, van Dullemen HM, et al. Natural Course and Treatment of Pancreatic Exocrine Insufficiency in a Nationwide Cohort of Chronic Pancreatitis. Pancreas. 2020;49(2):242-8.
Lanzillotta M, Tacelli M, Falconi M, Arcidiacono PG, Capurso G, Della-Torre E. Incidence of endocrine and exocrine insufficiency in patients with autoimmune pancreatitis at diagnosis and after treatment: a systematic review and meta-analysis. Eur J Intern Med. 2022;100:83-93.
Nikolic S, Maisonneuve P, Dahlman I, Löhr JM, Vujasinovic M. Exocrine and Endocrine Insufficiency in Autoimmune Pancreatitis: A Matter of Treatment or Time? J Clin Med. 2022;11(13).
DiMagno EP, Go VL, Summerskill WH. Relations between pancreatic enzyme outputs and malabsorption in severe pancreatic insufficiency. N Engl J Med. 1973;288(16):813-5.
Frulloni L, Falconi M, Gabbrielli A, Gaia E, Graziani R, Pezzilli R, et al. Italian consensus guidelines for chronic pancreatitis. Dig Liver Dis. 2010;42 Suppl 6:S381-406.
Pezzilli R, Andriulli A, Bassi C, Balzano G, Cantore M, Delle Fave G, Falconi M. Exocrine pancreatic insufficiency in adults: a shared position statement of the Italian Association for the Study of the Pancreas. World J Gastroenterol. 2013;19(44):7930-46.
Johnson CD, Williamson N, Janssen-van Solingen G, Arbuckle R, Johnson C, Simpson S, et al. Psychometric evaluation of a patient-reported outcome measure in pancreatic exocrine insufficiency (PEI). Pancreatology. 2019;19(1):182-90.
Nikfarjam M, Wilson JS, Smith RC. Diagnosis and management of pancreatic exocrine insufficiency. Med J Aust. 2017;207(4):161-5.
Keller J, Layer P. Human pancreatic exocrine response to nutrients in health and disease. Gut. 2005;54 Suppl 6(Suppl 6):vi1-28.
Duggan SN, Smyth ND, Murphy A, Macnaughton D, O'Keefe SJ, Conlon KC. High prevalence of osteoporosis in patients with chronic pancreatitis: a systematic review and meta-analysis. Clin Gastroenterol Hepatol. 2014;12(2):219-28.
Winny M, Paroglou V, Bektas H, Kaltenborn A, Reichert B, Zachau L, et al. Insulin dependence and pancreatic enzyme replacement therapy are independent prognostic factors for long-term survival after operation for chronic pancreatitis. Surgery. 2014;155(2):271-9.
Thorat V, Reddy N, Bhatia S, Bapaye A, Rajkumar JS, Kini DD, et al. Randomised clinical trial: the efficacy and safety of pancreatin enteric-coated minimicrospheres (Creon 40000 MMS) in patients with pancreatic exocrine insufficiency due to chronic pancreatitis--a double-blind, placebo-controlled study. Aliment Pharmacol Ther. 2012;36(5):426-36.
Safdi M, Bekal PK, Martin S, Saeed ZA, Burton F, Toskes PP. The effects of oral pancreatic enzymes (Creon 10 capsule) on steatorrhea: a multicenter, placebo-controlled, parallel group trial in subjects with chronic pancreatitis. Pancreas. 2006;33(2):156-62.
Paris JC. A Multicentre Double-Blind Placebo-Controlled Study of the Effect of a Pancreatic Enzyme Formulation (Panzytrat® 25 000) on Impaired Lipid Digestion in Adults with Chronic Pancreatitis. Drug Investigation. 1993;5(4):229-37.
O'Keefe SJ, Cariem AK, Levy M. The exacerbation of pancreatic endocrine dysfunction by potent pancreatic exocrine supplements in patients with chronic pancreatitis. J Clin Gastroenterol. 2001;32(4):319-23.
FitzSimmons SC, Burkhart GA, Borowitz D, Grand RJ, Hammerstrom T, Durie PR, et al. High-dose pancreatic-enzyme supplements and fibrosing colonopathy in children with cystic fibrosis. N Engl J Med. 1997;336(18):1283-9.
Singh VK, Yadav D, Garg PK. Diagnosis and Management of Chronic Pancreatitis: A Review. Jama. 2019;322(24):2422-34.
de Rijk FEM, van Veldhuisen CL, Besselink MG, van Hooft JE, van Santvoort HC, van Geenen EJM, et al. Diagnosis and treatment of exocrine pancreatic insufficiency in chronic pancreatitis: An international expert survey and case vignette study. Pancreatology. 2022;22(4):457-65.
Arvanitakis M, Ockenga J, Bezmarevic M, Gianotti L, Krznarić Ž, Lobo DN, et al. ESPEN guideline on clinical nutrition in acute and chronic pancreatitis. Clin Nutr. 2020;39(3):612-31.
Beyer G, Hoffmeister A, Michl P, Gress TM, Huber W, Algül H, et al. [Not Available]. Z Gastroenterol. 2022;60(3):419-521.
Löhr JM, Dominguez-Munoz E, Rosendahl J, Besselink M, Mayerle J, Lerch MM, et al. United European Gastroenterology evidence-based guidelines for the diagnosis and therapy of chronic pancreatitis (HaPanEU). United European Gastroenterol J. 2017;5(2):153-99.
Al-Najjar Y, Clark AL. Predicting outcome in patients with left ventricular systolic chronic heart failure using a nutritional risk index. Am J Cardiol. 2012;109(9):1315-20.
Overview | Nutrition support in adults | Quality standards | NICE.
Hollemans RA, Hallensleben NDL, Mager DJ, Kelder JC, Besselink MG, Bruno MJ, et al. Pancreatic exocrine insufficiency following acute pancreatitis: Systematic review and study level meta-analysis. Pancreatology. 2018;18(3):253-62.
Mattar R, Lima GA, da Costa MZ, Silva-Etto JM, Guarita D, Carrilho FJ. Comparison of fecal elastase 1 for exocrine pancreatic insufficiency evaluation between ex-alcoholics and chronic pancreatitis patients. Arq Gastroenterol. 2014;51(4):297-301.
Vanga RR, Tansel A, Sidiq S, El-Serag HB, Othman MO. Diagnostic Performance of Measurement of Fecal Elastase-1 in Detection of Exocrine Pancreatic Insufficiency: Systematic Review and Meta-analysis. Clin Gastroenterol Hepatol. 2018;16(8):1220-8.e4.
Garip G, Sarandöl E, Kaya E. Effects of disease severity and necrosis on pancreatic dysfunction after acute pancreatitis. World J Gastroenterol. 2013;19(44):8065-70.
Koziel D, Suliga E, Grabowska U, Gluszek S. Morphological and functional consequences and quality of life following severe acute pancreatitis. Ann Ital Chir. 2017;6:403-11.
Boreham B, Ammori BJ. A prospective evaluation of pancreatic exocrine function in patients with acute pancreatitis: correlation with extent of necrosis and pancreatic endocrine insufficiency. Pancreatology. 2003;3(4):303-8.
Migliori M, Pezzilli R, Tomassetti P, Gullo L. Exocrine pancreatic function after alcoholic or biliary acute pancreatitis. Pancreas. 2004;28(4):359-63.
Ho TW, Wu JM, Kuo TC, Yang CY, Lai HS, Hsieh SH, et al. Change of Both Endocrine and Exocrine Insufficiencies After Acute Pancreatitis in Non-Diabetic Patients: A Nationwide Population-Based Study. Medicine (Baltimore). 2015;94(27):e1123.
Chandrasekaran P, Gupta R, Shenvi S, Kang M, Rana SS, Singh R, Bhasin DK. Prospective comparison of long term outcomes in patients with severe acute pancreatitis managed by operative and non operative measures. Pancreatology. 2015;15(5):478-84.
Sabater L, Pareja E, Aparisi L, Calvete J, Camps B, Sastre J, et al. Pancreatic function after severe acute biliary pancreatitis: the role of necrosectomy. Pancreas. 2004;28(1):65-8.
Gupta R, Wig JD, Bhasin DK, Singh P, Suri S, Kang M, et al. Severe acute pancreatitis: the life after. J Gastrointest Surg. 2009;13(7):1328-36.
Büchler M, Malfertheiner P, Block S, Maier W, Beger HG. [Morphologic and functional changes in the pancreas following acute necrotizing pancreatitis]. Z Gastroenterol. 1985;23(2):79-83.
Seidensticker F, Otto J, Lankisch PG. Recovery of the pancreas after acute pancreatitis is not necessarily complete. Int J Pancreatol. 1995;17(3):225-9.
Domínguez-Muñoz JE. Pancreatic exocrine insufficiency: diagnosis and treatment. J Gastroenterol Hepatol. 2011;26 Suppl 2:12-6.
Patankar RV, Chand R, Johnson CD. Pancreatic enzyme supplementation in acute pancreatitis. HPB Surg. 1995;8(3):159-62.
Domínguez-Muñoz JE, de la Iglesia-García D, Nieto-García L, Álvarez-Castro A, San Bruno-Ruz A, Monteserín-Ron L, et al. Endoscopic Pancreatic Drainage Improves Exocrine Pancreatic Function in Patients With Unresectable Pancreatic Cancer: A Double-Blind, Prospective, Randomized, Single-Center, Interventional Study. Pancreas. 2021;50(5):679-84.
Sikkens EC, Cahen DL, de Wit J, Looman CW, van Eijck C, Bruno MJ. A prospective assessment of the natural course of the exocrine pancreatic function in patients with a pancreatic head tumor. J Clin Gastroenterol. 2014;48(5):e43-6.
Dominguez-Munoz JE, Nieto-Garcia L, Lopez-Diaz J, Larino-Noia J, Abdulkader I, Iglesias-Garcia J. Impact of the treatment of pancreatic exocrine insufficiency on survival of patients with unresectable pancreatic cancer: a retrospective analysis. BMC Cancer. 2018;18(1):534.
Powell-Brett S, de Liguori Carino N, Roberts K. Understanding pancreatic exocrine insufficiency and replacement therapy in pancreatic cancer. Eur J Surg Oncol. 2021;47(3 Pt A):539-44.
Roeyen G, Berrevoet F, Borbath I, Geboes K, Peeters M, Topal B, et al. Expert opinion on management of pancreatic exocrine insufficiency in pancreatic cancer. ESMO Open. 2022;7(1):100386.
Partelli S, Frulloni L, Minniti C, Bassi C, Barugola G, D'Onofrio M, et al. Faecal elastase-1 is an independent predictor of survival in advanced pancreatic cancer. Dig Liver Dis. 2012;44(11):945-51.
Kim E, Kang JS, Han Y, Kim H, Kwon W, Kim JR, et al. Influence of preoperative nutritional status on clinical outcomes after pancreatoduodenectomy. HPB (Oxford). 2018;20(11):1051-61.
Danai LV, Babic A, Rosenthal MH, Dennstedt EA, Muir A, Lien EC, et al. Altered exocrine function can drive adipose wasting in early pancreatic cancer. Nature. 2018;558(7711):600-4.
Choi Y, Oh DY, Kim TY, Lee KH, Han SW, Im SA, et al. Skeletal Muscle Depletion Predicts the Prognosis of Patients with Advanced Pancreatic Cancer Undergoing Palliative Chemotherapy, Independent of Body Mass Index. PLoS One. 2015;10(10):e0139749.
Peng P, Hyder O, Firoozmand A, Kneuertz P, Schulick RD, Huang D, et al. Impact of sarcopenia on outcomes following resection of pancreatic adenocarcinoma. J Gastrointest Surg. 2012;16(8):1478-86.
Gooden HM, White KJ. Pancreatic cancer and supportive care--pancreatic exocrine insufficiency negatively impacts on quality of life. Support Care Cancer. 2013;21(7):1835-41.
Landers A, Brown H, Strother M. The effectiveness of pancreatic enzyme replacement therapy for malabsorption in advanced pancreatic cancer, a pilot study. Palliat Care. 2019;12:1178224218825270.
Trestini I, Carbognin L, Peretti U, Sperduti I, Caldart A, Tregnago D, et al. Pancreatic Enzyme Replacement Therapy in Patients Undergoing First-Line Gemcitabine Plus nab-paclitaxel for Advanced Pancreatic Adenocarcinoma. Front Oncol. 2021;11:688889.
Saito T, Nakai Y, Isayama H, Hirano K, Ishigaki K, Hakuta R, et al. A Multicenter Open-Label Randomized Controlled Trial of Pancreatic Enzyme Replacement Therapy in Unresectable Pancreatic Cancer. Pancreas. 2018;47(7):800-6.
Ammar K, Leeds JS, Ratnayake CB, Sen G, French JJ, Nayar M, et al. Impact of pancreatic enzyme replacement therapy on short- and long-term outcomes in advanced pancreatic cancer: meta-analysis of randomized controlled trials. Expert Rev Gastroenterol Hepatol. 2021;15(8):941-8.
Dunleavy L, Al-Mukhtar A, Halliday V. Pancreatic enzyme replacement therapy following surgery for pancreatic cancer: An exploration of patient self-management. Clin Nutr ESPEN. 2018;26:97-103.
Turck D, Braegger CP, Colombo C, Declercq D, Morton A, Pancheva R, et al. ESPEN-ESPGHAN-ECFS guidelines on nutrition care for infants, children, and adults with cystic fibrosis. Clin Nutr. 2016;35(3):557-77.
Capurso G, Traini M, Piciucchi M, Signoretti M, Arcidiacono PG. Exocrine pancreatic insufficiency: prevalence, diagnosis, and management. Clin Exp Gastroenterol. 2019;12:129-39.
Augarten A, Ben Tov A, Madgar I, Barak A, Akons H, Laufer J, et al. The changing face of the exocrine pancreas in cystic fibrosis: the correlation between pancreatic status, pancreatitis and cystic fibrosis genotype. European journal of gastroenterology & hepatology. 2008;20(3):164-8.
Singh VK, Schwarzenberg SJ. Pancreatic insufficiency in Cystic Fibrosis. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society. 2017;16 Suppl 2:S70-S8.
Kerem E, Corey M, Kerem BS, Rommens J, Markiewicz D, Levison H, et al. The relation between genotype and phenotype in cystic fibrosis--analysis of the most common mutation (delta F508). The New England journal of medicine. 1990;323(22):1517-22.
Kristidis P, Bozon D, Corey M, Markiewicz D, Rommens J, Tsui LC, Durie P. Genetic determination of exocrine pancreatic function in cystic fibrosis. Am J Hum Genet. 1992;50(6):1178-84.
Wilschanski M, Durie PR. Pathology of pancreatic and intestinal disorders in cystic fibrosis. Journal of the Royal Society of Medicine. 1998;91 Suppl 34:40-9.
Wilschanski M, Novak I. The cystic fibrosis of exocrine pancreas. Cold Spring Harb Perspect Med. 2013;3(5):a009746.
Ooi CY, Dorfman R, Cipolli M, Gonska T, Castellani C, Keenan K, et al. Type of CFTR mutation determines risk of pancreatitis in patients with cystic fibrosis. Gastroenterology. 2011;140(1):153-61.
Dequeker E, Stuhrmann M, Morris MA, Casals T, Castellani C, Claustres M, et al. Best practice guidelines for molecular genetic diagnosis of cystic fibrosis and CFTR-related disorders--updated European recommendations. Eur J Hum Genet. 2009;17(1):51-65.
Farrell PM, White TB, Ren CL, Hempstead SE, Accurso F, Derichs N, et al. Diagnosis of Cystic Fibrosis: Consensus Guidelines from the Cystic Fibrosis Foundation. The Journal of pediatrics. 2017;181S:S4-S15 e1.
Levy P, Dominguez-Munoz E, Imrie C, Löhr M, Maisonneuve P. Epidemiology of chronic pancreatitis: burden of the disease and consequences. United European Gastroenterol J. 2014;2(5):345-54.
Scheers I. Inherited pancreatic exocrine insufficiency and pancreatitis: When children transition to adult care. Best Pract Res Clin Gastroenterol. 2022;56-57:101782.
Kunovsky L, Dite P, Jabandziev P, Eid M, Poredska K, Vaculova J, et al. Causes of Exocrine Pancreatic Insufficiency Other Than Chronic Pancreatitis. J Clin Med. 2021;10(24).
Castellani C, De Boeck K, De Wachter E, Sermet-Gaudelus I, Simmonds NJ, Southern KW, Group EDNW. ECFS standards of care on CFTR-related disorders: Updated diagnostic criteria. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society. 2022;21(6):908-21.
Baldwin C, Zerofsky M, Sathe M, Troendle DM, Perito ER. Acute Recurrent and Chronic Pancreatitis as Initial Manifestations of Cystic Fibrosis and Cystic Fibrosis Transmembrane Conductance Regulator-Related Disorders. Pancreas. 2019;48(7):888-93.
Borowitz D. Update on the evaluation of pancreatic exocrine status in cystic fibrosis. Curr Opin Pulm Med. 2005;11(6):524-7.
Bronstein MN, Sokol RJ, Abman SH, Chatfield BA, Hammond KB, Hambidge KM, et al. Pancreatic insufficiency, growth, and nutrition in infants identified by newborn screening as having cystic fibrosis. J Pediatr. 1992;120(4 Pt 1):533-40.
Castellani C, Duff AJA, Bell SC, Heijerman HGM, Munck A, Ratjen F, et al. ECFS best practice guidelines: the 2018 revision. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society. 2018;17(2):153-78.
Gaskin K, Waters D, Dorney S, Gruca M, O'Halloran M, Wilcken B. Assessment of pancreatic function in screened infants with cystic fibrosis. Pediatr Pulmonol Suppl. 1991;7:69-71.
O'Sullivan BP, Baker D, Leung KG, Reed G, Baker SS, Borowitz D. Evolution of pancreatic function during the first year in infants with cystic fibrosis. J Pediatr. 2013;162(4):808-12.e1.
Couper RT, Corey M, Moore DJ, Fisher LJ, Forstner GG, Durie PR. Decline of exocrine pancreatic function in cystic fibrosis patients with pancreatic sufficiency. Pediatr Res. 1992;32(2):179-82.
Walkowiak J, Herzig KH, Witt M, Pogorzelski A, Piotrowski R, Barra E, et al. Analysis of exocrine pancreatic function in cystic fibrosis: one mild CFTR mutation does not exclude pancreatic insufficiency. Eur J Clin Invest. 2001;31(9):796-801.
Walkowiak J, Sands D, Nowakowska A, Piotrowski R, Zybert K, Herzig KH, Milanowski A. Early decline of pancreatic function in cystic fibrosis patients with class 1 or 2 CFTR mutations. J Pediatr Gastroenterol Nutr. 2005;40(2):199-201.
Dorlöchter L, Aksnes L, Fluge G. Faecal elastase-1 and fat-soluble vitamin profiles in patients with cystic fibrosis in Western Norway. Eur J Nutr. 2002;41(4):148-52.
Borowitz D, Robinson KA, Rosenfeld M, Davis SD, Sabadosa KA, Spear SL, et al. Cystic Fibrosis Foundation evidence-based guidelines for management of infants with cystic fibrosis. J Pediatr. 2009;155(6 Suppl):S73-93.
De Boeck K, Weren M, Proesmans M, Kerem E. Pancreatitis among patients with cystic fibrosis: correlation with pancreatic status and genotype. Pediatrics. 2005;115(4):e463-9.
Peterson ML, Jacobs DR, Jr., Milla CE. Longitudinal changes in growth parameters are correlated with changes in pulmonary function in children with cystic fibrosis. Pediatrics. 2003;112(3 Pt 1):588-92.
Konstan MW, Butler SM, Wohl ME, Stoddard M, Matousek R, Wagener JS, et al. Growth and nutritional indexes in early life predict pulmonary function in cystic fibrosis. The Journal of pediatrics. 2003;142(6):624-30.
Rosenfeld M, Wainwright CE, Higgins M, Wang LT, McKee C, Campbell D, et al. Ivacaftor treatment of cystic fibrosis in children aged 12 to <24 months and with a CFTR gating mutation (ARRIVAL): a phase 3 single-arm study. Lancet Respir Med. 2018;6(7):545-53.
Davies JC, Cunningham S, Harris WT, Lapey A, Regelmann WE, Sawicki GS, et al. Safety, pharmacokinetics, and pharmacodynamics of ivacaftor in patients aged 2-5 years with cystic fibrosis and a CFTR gating mutation (KIWI): an open-label, single-arm study. Lancet Respir Med. 2016;4(2):107-15.
Rosenfeld M, Cunningham S, Harris WT, Lapey A, Regelmann WE, Sawicki GS, et al. An open-label extension study of ivacaftor in children with CF and a CFTR gating mutation initiating treatment at age 2-5years (KLIMB). Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society. 2019;18(6):838-43.
Davies JC, Wainwright CE, Sawicki GS, Higgins MN, Campbell D, Harris C, et al. Ivacaftor in Infants Aged 4 to <12 Months with Cystic Fibrosis and a Gating Mutation. Results of a Two-Part Phase 3 Clinical Trial. American journal of respiratory and critical care medicine. 2021;203(5):585-93.
McNamara JJ, McColley SA, Marigowda G, Liu F, Tian S, Owen CA, et al. Safety, pharmacokinetics, and pharmacodynamics of lumacaftor and ivacaftor combination therapy in children aged 2-5 years with cystic fibrosis homozygous for F508del-CFTR: an open-label phase 3 study. Lancet Respir Med. 2019;7(4):325-35.
Ramsey ML, Gokun Y, Sobotka LA, Wellner MR, Porter K, Kirkby SE, et al. Cystic Fibrosis Transmembrane Conductance Regulator Modulator Use Is Associated With Reduced Pancreatitis Hospitalizations in Patients With Cystic Fibrosis. The American journal of gastroenterology. 2021;116(12):2446-54.
Akshintala VS, Kamal A, Faghih M, Cutting GR, Cebotaru L, West NE, et al. Cystic fibrosis transmembrane conductance regulator modulators reduce the risk of recurrent acute pancreatitis among adult patients with pancreas sufficient cystic fibrosis. Pancreatology : official journal of the International Association of Pancreatology. 2019;19(8):1023-6.
Kounis I, Levy P, Rebours V. Ivacaftor CFTR Potentiator Therapy is Efficient for Pancreatic Manifestations in Cystic Fibrosis. The American journal of gastroenterology. 2018;113(7):1058-9.
Johns JD, Rowe SM. The effect of CFTR modulators on a cystic fibrosis patient presenting with recurrent pancreatitis in the absence of respiratory symptoms: a case report. BMC Gastroenterol. 2019;19(1):123.
Del Chiaro M, Rangelova E, Segersvard R, Arnelo U. Are there still indications for total pancreatectomy? Updates Surg. 2016;68(3):257-63.
Moore JV, Tom S, Scoggins CR, Philips P, Egger ME, Martin RCG, 2nd. Exocrine Pancreatic Insufficiency After Pancreatectomy for Malignancy: Systematic Review and Optimal Management Recommendations. J Gastrointest Surg. 2021;25(9):2317-27.
Beger HG, Nakao A, Mayer B, Poch B. Duodenum-preserving total and partial pancreatic head resection for benign tumors--systematic review and meta-analysis. Pancreatology. 2015;15(2):167-78.
Erchinger F, Engjom T, Dimcevski G, Drewes AM, Olesen SS, Vujasinovic M, et al. Exocrine pancreas insufficiency in chronic pancreatitis — Risk factors and associations with complications. A multicentre study of 1869 patients. Pancreatology. 2022;22(3):374-80.
Beger HG, Poch B, Mayer B, Siech M. New Onset of Diabetes and Pancreatic Exocrine Insufficiency After Pancreaticoduodenectomy for Benign and Malignant Tumors: A Systematic Review and Meta-analysis of Long-term Results. Ann Surg. 2018;267(2):259-70.
Aloulou A, Puccinelli D, Sarles J, Laugier R, Leblond Y, Carriere F. In vitro comparative study of three pancreatic enzyme preparations: dissolution profiles, active enzyme release and acid stability. Aliment Pharmacol Ther. 2008;27(3):283-92.
Liddle RA, Goldfine ID, Rosen MS, Taplitz RA, Williams JA. Cholecystokinin bioactivity in human plasma. Molecular forms, responses to feeding, and relationship to gallbladder contraction. J Clin Invest. 1985;75(4):1144-52.
Love JA, Yi E, Smith TG. Autonomic pathways regulating pancreatic exocrine secretion. Auton Neurosci. 2007;133(1):19-34.
Okano K, Murakami Y, Nakagawa N, Uemura K, Sudo T, Hashimoto Y, et al. Remnant pancreatic parenchymal volume predicts postoperative pancreatic exocrine insufficiency after pancreatectomy. Surgery. 2016;159(3):885-92.
Kusakabe J, Anderson B, Liu J, Williams GA, Chapman WC, Doyle MMB, et al. Long-Term Endocrine and Exocrine Insufficiency After Pancreatectomy. J Gastrointest Surg. 2019;23(8):1604-13.
Miyamoto R, Inagaki Y, Ikeda N, Oda T. Three-dimensional remnant pancreatic volume ratio indicates postoperative pancreatic exocrine insufficiency in pancreatic cancer patients after distal pancreatectomy. Pancreatology. 2020;20(5):867-74.
Thomas AS, Huang Y, Kwon W, Schrope BA, Sugahara K, Chabot JA, et al. Prevalence and Risk Factors for Pancreatic Insufficiency After Partial Pancreatectomy. J Gastrointest Surg. 2022;26(7):1425-35.
Maignan A, Ouaissi M, Turrini O, Regenet N, Loundou A, Louis G, et al. Risk factors of exocrine and endocrine pancreatic insufficiency after pancreatic resection: A multi-center prospective study. J Visc Surg. 2018;155(3):173-81.
Pathanki AM, Attard JA, Bradley E, Powell-Brett S, Dasari BVM, Isaac JR, et al. Pancreatic exocrine insufficiency after pancreaticoduodenectomy: Current evidence and management. World J Gastrointest Pathophysiol. 2020;11(2):20-31.
Takada T, Yasuda H, Uchiyama K, Hasegawa H, Misu Y, Iwagaki T. Pancreatic enzyme activity after a pylorus-preserving pancreaticoduodenectomy reconstructed with pancreaticogastrostomy. Pancreas. 1995;11(3):276-82.
Gianotti L, Besselink MG, Sandini M, Hackert T, Conlon K, Gerritsen A, et al. Nutritional support and therapy in pancreatic surgery: A position paper of the International Study Group on Pancreatic Surgery (ISGPS). Surgery. 2018;164(5):1035-48.
Sabater L, Ausania F, Bakker OJ, Boadas J, Dominguez-Munoz JE, Falconi M, et al. Evidence-based Guidelines for the Management of Exocrine Pancreatic Insufficiency After Pancreatic Surgery. Ann Surg. 2016;264(6):949-58.
Lohr JM, Dominguez-Munoz E, Rosendahl J, Besselink M, Mayerle J, Lerch MM, et al. United European Gastroenterology evidence-based guidelines for the diagnosis and therapy of chronic pancreatitis (HaPanEU). United European Gastroenterol J. 2017;5(2):153-99.
Heneghan HM, Zaborowski A, Fanning M, McHugh A, Doyle S, Moore J, et al. Prospective Study of Malabsorption and Malnutrition After Esophageal and Gastric Cancer Surgery. Ann Surg. 2015;262(5):803-7; discussion 7-8.
Huddy JR, Macharg FM, Lawn AM, Preston SR. Exocrine pancreatic insufficiency following esophagectomy. Dis Esophagus. 2013;26(6):594-7.
Shils ME, Gilat T. The effect of esophagectomy on absorption in man: clinical and metabolic observations. Gastroenterology. 1966;50(3):347-57.
Blonk L, Wierdsma NJ, Jansma EP, Kazemier G, van der Peet DL, Straatman J. Exocrine pancreatic insufficiency after esophagectomy: a systematic review of literature. Dis Esophagus. 2021;34(12).
Friess H, Bohm J, Muller MW, Glasbrenner B, Riepl RL, Malfertheiner P, Büchler MW. Maldigestion after total gastrectomy is associated with pancreatic insufficiency. Am J Gastroenterol. 1996;91(2):341-7.
Nakamura H, Murakami Y, Morifuji M, Uemura K, Hayashidani Y, Sudo T, et al. Analysis of fat digestive and absorptive function after subtotal gastrectomy by a 13C-labeled mixed triglyceride breath test. Digestion. 2009;80(2):98-103.
Gullo L, Costa PL, Ventrucci M, Mattioli S, Viti G, Labò G. Exocrine pancreatic function after total gastrectomy. Scand J Gastroenterol. 1979;14(4):401-7.
Borbély Y, Plebani A, Kröll D, Ghisla S, Nett PC. Exocrine Pancreatic Insufficiency after Roux-en-Y gastric bypass. Surg Obes Relat Dis. 2016;12(4):790-4.
Kwon JY, Nelson A, Salih A, Valery J, Harris DM, Stancampiano F, Bi Y. Exocrine pancreatic insufficiency after bariatric surgery. Pancreatology. 2022.
Vujasinovic M, Kunst G, Breznikar B, Rozej B, Tepes B, Rudolf S, Kuster A. Is pancreatic exocrine insufficiency a cause of malabsorption in patients after bariatric surgery? . JOP J Pancreas. 2016;17:241-4.
Catarci M, Berlanda M, Grassi GB, Masedu F, Guadagni S. Pancreatic enzyme supplementation after gastrectomy for gastric cancer: a randomized controlled trial. Gastric Cancer. 2018;21(3):542-51.
Vujasinovic M, Valente R, Thorell A, Rutkowski W, Haas SL, Arnelo U, et al. Pancreatic Exocrine Insufficiency after Bariatric Surgery. Nutrients. 2017;9(11).
Foster TP, Bruggeman B, Campbell-Thompson M, Atkinson MA, Haller MJ, Schatz DA. Exocrine Pancreas Dysfunction in Type 1 Diabetes. Endocr Pract. 2020;26(12):1505-13.
Radlinger B, Ramoser G, Kaser S. Exocrine Pancreatic Insufficiency in Type 1 and Type 2 Diabetes. Current diabetes reports. 2020;20(6):18.
Zhang J, Hou J, Liu D, Lv Y, Zhang C, Su X, Li L. The Prevalence and Characteristics of Exocrine Pancreatic Insufficiency in Patients with Type 2 Diabetes: A Systematic Review and Meta-Analysis. Int J Endocrinol. 2022;2022:7764963.
Vujasinovic M, Zaletel J, Tepes B, Popic B, Makuc J, Epsek Lenart M, et al. Low prevalence of exocrine pancreatic insufficiency in patients with diabetes mellitus. Pancreatology. 2013;13(4):343-6.
Søfteland E, Poulsen JL, Starup-Linde J, Christensen TT, Olesen SS, Singh S, et al. Pancreatic exocrine insufficiency in diabetes mellitus — prevalence and characteristics. Eur J Intern Med. 2019;68:18-22.
Hardt PD, Krauss A, Bretz L, Porsch-Ozcürümez M, Schnell-Kretschmer H, Mäser E, et al. Pancreatic exocrine function in patients with type 1 and type 2 diabetes mellitus. Acta Diabetol. 2000;37(3):105-10.
Mohapatra S, Majumder S, Smyrk TC, Zhang L, Matveyenko A, Kudva YC, Chari ST. Diabetes Mellitus Is Associated With an Exocrine Pancreatopathy: Conclusions From a Review of Literature. Pancreas. 2016;45(8):1104-10.
Zsóri G, Illés D, Terzin V, Ivány E, Czakó L. Exocrine pancreatic insufficiency in type 1 and type 2 diabetes mellitus: do we need to treat it? A systematic review. Pancreatology. 2018;18(5):559-65.
Anoop S, Dasgupta R, Jebasingh FK, Ramachandran R, Kurian ME, Rebekah G, et al. Exocrine pancreatic insufficiency related fat malabsorption and its association with autonomic neuropathy in Asian Indians with type 2 diabetes mellitus. Diabetes Metab Syndr. 2021;15(5):102273.
de la Iglesia D, Vallejo-Senra N, López-López A, Iglesias-Garcia J, Lariño-Noia J, Nieto-García L, Domínguez-Muñoz JE. Pancreatic exocrine insufficiency and cardiovascular risk in patients with chronic pancreatitis: A prospective, longitudinal cohort study. J Gastroenterol Hepatol. 2019;34(1):277-83.
Sikkens EC, Cahen DL, Koch AD, Braat H, Poley JW, Kuipers EJ, Bruno MJ. The prevalence of fat-soluble vitamin deficiencies and a decreased bone mass in patients with chronic pancreatitis. Pancreatology. 2013;13(3):238-42.
Hart PA, Bellin MD, Andersen DK, Bradley D, Cruz-Monserrate Z, Forsmark CE, et al. Type 3c (pancreatogenic) diabetes mellitus secondary to chronic pancreatitis and pancreatic cancer. Lancet Gastroenterol Hepatol. 2016;1(3):226-37.
Löhr JM, Panic N, Vujasinovic M, Verbeke CS. The ageing pancreas: a systematic review of the evidence and analysis of the consequences. J Intern Med. 2018;283(5):446-60.
Glaser J, Stienecker K. Does aging influence pancreatic response in the ultrasound secretin test by impairing hydrokinetic exocrine function or sphincter of Oddi motor function? Dig Liver Dis. 2000;32(1):25-8.
Gullo L, Simoni P, Migliori M, Lucrezio L, Bassi M, Frau F, et al. A study of pancreatic function among subjects over ninety years of age. Pancreatology. 2009;9(3):240-4.
Gullo L, Ventrucci M, Naldoni P, Pezzilli R. Aging and exocrine pancreatic function. J Am Geriatr Soc. 1986;34(11):790-2.
Vellas B, Balas D, Moreau J, Bouisson M, Senegas-Balas F, Guidet M, Ribet A. Exocrine pancreatic secretion in the elderly. Int J Pancreatol. 1988;3(6):497-502.
Fikry ME. Exocrine pancreatic functions in the aged. J Am Geriatr Soc. 1968;16(4):463-7.
Tiscornia OM, Cresta MA, de Lehmann ES, Celener D, Dreiling DA. Effects of sex and age on pancreatic secretion. Int J Pancreatol. 1986;1(2):95-118.
Ishibashi T, Matsumoto S, Harada H, Ochi K, Tanaka J, Seno T, et al. [Aging and exocrine pancreatic function evaluated by the recently standardized secretin test]. Nihon Ronen Igakkai Zasshi. 1991;28(5):599-605.
Laugier R, Bernard JP, Berthezene P, Dupuy P. Changes in pancreatic exocrine secretion with age: pancreatic exocrine secretion does decrease in the elderly. Digestion. 1991;50(3-4):202-11.
Mössner J, Pusch HJ, Koch W. [Exocrine pancreas function — does it change with age? (author's transl)]. Aktuelle Gerontol. 1982;12(2):40-3.
Torigoe T, Ito K, Yamamoto A, Kanki A, Yasokawa K, Tamada T, Yoshida K. Age-related change of the secretory flow of pancreatic juice in the main pancreatic duct: evaluation with cine-dynamic MRCP using spatially selective inversion recovery pulse. AJR Am J Roentgenol. 2014;202(5):1022-6.
Rothenbacher D, Low M, Hardt PD, Klor HU, Ziegler H, Brenner H. Prevalence and determinants of exocrine pancreatic insufficiency among older adults: results of a population-based study. Scand J Gastroenterol. 2005;40(6):697-704.
Herzig KH, Purhonen AK, Rasanen KM, Idziak J, Juvonen P, Phillps R, Walkowiak J. Fecal pancreatic elastase-1 levels in older individuals without known gastrointestinal diseases or diabetes mellitus. BMC Geriatr. 2011;11:4.
Maetzel H, Rutkowski W, Panic N, Mari A, Hedström A, Kulinski P, et al. Non-alcoholic fatty pancreas disease and pancreatic exocrine insufficiency: pilot study and systematic review. Scand J Gastroenterol. 2023:1-8.
Tahtacı M, Algın O, Karakan T, Yürekli Ö T, Alışık M, Köseoğlu H, et al. Can pancreatic steatosis affect exocrine functions of pancreas? Turk J Gastroenterol. 2018;29(5):588-94.
Miyake H, Sakagami J, Yasuda H, Sogame Y, Kato R, Suwa K, et al. Association of fatty pancreas with pancreatic endocrine and exocrine function. PLoS One. 2018;13(12):e0209448.
Kromrey ML, Friedrich N, Hoffmann RT, Bülow R, Völzke H, Weiss FU, et al. Pancreatic Steatosis Is Associated With Impaired Exocrine Pancreatic Function. Invest Radiol. 2019;54(7):403-8.
Althausen TL, Doig RK, Weiden S, Motteram R, Turner CN, Moore A. Hemochromatosis; investigation of twenty-three cases, with special reference to etiology, nutrition, iron metabolism, and studies of hepatic and pancreatic function. AMA Arch Intern Med. 1951;88(5):553-70.
Simon M, Gosselin M, Kerbaol M, Delanoe G, Trebaul L, Bourel M. Functional study of exocrine pancreas in idiopathic hemochromatosis, untreated and treated by venesections. Digestion. 1973;8(6):485-96.
Vobugari N, Kim J, Gandhi KD, Lee ZE, Smith HP. Iron-Storage Disorder Presenting as Chronic Diarrhea. Cureus. 2021;13(10):e18864.
Jansen PL, Thien T, Lamers CB, Yap SH, Reekers P, Strijk S. Exocrine pancreatic insufficiency and idiopathic haemochromatosis. Postgrad Med J. 1984;60(708):675-8.
Hussain M, Dandona P, Fedail SS, Ramdial L, Flynn D, Hoffbrand AV. Serum immunoreactive trypsin in beta-thalassaemia major. J Clin Pathol. 1981;34(9):970-1.
Gullo L, Corcioni E, Brancati C, Bria M, Pezzilli R, Sprovieri G. Morphologic and functional evaluation of the exocrine pancreas in beta-thalassemia major. Pancreas. 1993;8(2):176-80.
Montalto G, D'Angelo P, Lo Casto A, Carroccio A, Soresi M, Midiri M, et al. Serum and fecal pancreatic enzymes in beta-thalassemia major. Int J Pancreatol. 1997;22(2):131-5.
Nousia-Arvanitakis S, Karagiozoglou-Lamboudes T, Aggouridaki C, Malaka-Lambrellis E, Galli-Tsinopoulou A, Xefteri M. Influence of jejunal morphology changes on exocrine pancreatic function in celiac disease. J Pediatr Gastroenterol Nutr. 1999;29(1):81-5.
Nousia-Arvanitakis S, Fotoulaki M, Tendzidou K, Vassilaki C, Agguridaki C, Karamouzis M. Subclinical exocrine pancreatic dysfunction resulting from decreased cholecystokinin secretion in the presence of intestinal villous atrophy. J Pediatr Gastroenterol Nutr. 2006;43(3):307-12.
DiMagno EP, Go WL, Summerskill WH. Impaired cholecystokinin-pancreozymin secretion, intraluminal dilution, and maldigestion of fat in sprue. Gastroenterology. 1972;63(1):25-32.
Jiang C, Barkin JA, Barkin JS. Exocrine Pancreatic Insufficiency Is Common in Celiac Disease: A Systematic Review and Meta-Analysis. Dig Dis Sci. 2023;68(8):3421-7.
Sadr-Azodi O, Sanders DS, Murray JA, Ludvigsson JF. Patients with celiac disease have an increased risk for pancreatitis. Clin Gastroenterol Hepatol. 2012;10(10):1136-42.e3.
Carroccio A, Iacono G, Montalto G, Cavataio F, Lorello D, Greco L, et al. Pancreatic enzyme therapy in childhood celiac disease. A double-blind prospective randomized study. Dig Dis Sci. 1995;40(12):2555-60.
Ludvigsson JF, Bai JC, Biagi F, Card TR, Ciacci C, Ciclitira PJ, et al. Diagnosis and management of adult coeliac disease: guidelines from the British Society of Gastroenterology. Gut. 2014;63(8):1210-28.
Al-Toma A, Volta U, Auricchio R, Castillejo G, Sanders DS, Cellier C, et al. European Society for the Study of Coeliac Disease (ESsCD) guideline for coeliac disease and other gluten-related disorders. United European Gastroenterol J. 2019;7(5):583-613.
Angelini G, Cavallini G, Bovo P, Brocco G, Castagnini A, Lavarini E, et al. Pancreatic function in chronic inflammatory bowel disease. Int J Pancreatol. 1988;3(2-3):185-93.
Maconi G, Dominici R, Molteni M, Ardizzone S, Bosani M, Ferrara E, et al. Prevalence of pancreatic insufficiency in inflammatory bowel diseases. Assessment by fecal elastase-1. Dig Dis Sci. 2008;53(1):262-70.
Massironi S, Fanetti I, Viganò C, Pirola L, Fichera M, Cristoferi L, et al. Systematic review-pancreatic involvement in inflammatory bowel disease. Aliment Pharmacol Ther. 2022;55(12):1478-91.
Hedström A, Steiner C, Valente R, Haas SL, Löhr JM, Vujasinovic M. Pancreatic exocrine insufficiency and Crohn's disease. Minerva Gastroenterol Dietol. 2020;66(1):17-22.
Lorenzo D, Maire F, Stefanescu C, Gornet JM, Seksik P, Serrero M, et al. Features of Autoimmune Pancreatitis Associated With Inflammatory Bowel Diseases. Clin Gastroenterol Hepatol. 2018;16(1):59-67.
Nikolic S, Lanzillotta M, Panic N, Brismar TB, Moro CF, Capurso G, et al. Unraveling the relationship between autoimmune pancreatitis type 2 and inflammatory bowel disease: Results from two centers and systematic review of the literature. United European Gastroenterol J. 2022;10(5):496-506.
Leeds JS, Hopper AD, Sidhu R, Simmonette A, Azadbakht N, Hoggard N, et al. Some patients with irritable bowel syndrome may have exocrine pancreatic insufficiency. Clin Gastroenterol Hepatol. 2010;8(5):433-8.
Goepp J, Fowler E, McBride T, Landis D. Frequency of abnormal fecal biomarkers in irritable bowel syndrome. Glob Adv Health Med. 2014;3(3):9-15.
Emmanuel A, Landis D, Peucker M, Hungin AP. Faecal biomarker patterns in patients with symptoms of irritable bowel syndrome. Frontline Gastroenterol. 2016;7(4):275-82.
Olmos JI, Piskorz MM, Litwin N, Schaab S, Tevez A, Bravo-Velez G, et al. Exocrine Pancreatic Insufficiency is Undiagnosed in Some Patients with Diarrhea-Predominant Irritable Bowel Syndrome Using the Rome IV Criteria. Dig Dis Sci. 2022;67(12):5666-75.
Poon D, Law GR, Major G, Andreyev HJN. A systematic review and meta-analysis on the prevalence of non-malignant, organic gastrointestinal disorders misdiagnosed as irritable bowel syndrome. Sci Rep. 2022;12(1):1949.
Talley NJ, Holtmann G, Nguyen QN, Gibson P, Bampton P, Veysey M, et al. Undiagnosed pancreatic exocrine insufficiency and chronic pancreatitis in functional GI disorder patients with diarrhea or abdominal pain. J Gastroenterol Hepatol. 2017;32(11):1813-7.
Money ME, Walkowiak J, Virgilio C, Talley NJ. Pilot study: a randomised, double blind, placebo controlled trial of pancrealipase for the treatment of postprandial irritable bowel syndrome-diarrhoea. Frontline Gastroenterol. 2011;2(1):48-56.
Graham DY, Ketwaroo GA, Money ME, Opekun AR. Enzyme therapy for functional bowel disease-like post-prandial distress. J Dig Dis. 2018;19(11):650-6.
Money ME, Camilleri M. Review: Management of postprandial diarrhea syndrome. Am J Med. 2012;125(6):538-44.
Eshet Y, Baruch EN, Shapira-Frommer R, Steinberg-Silman Y, Kuznetsov T, Ben-Betzalel G, et al. Clinical Significance of Pancreatic Atrophy Induced by Immune-Checkpoint Inhibitors: A Case-Control Study. Cancer Immunol Res. 2018;6(12):1453-8.
Díaz-González Á, Belmonte E, Sapena V, Sanduzzi-Zamparelli M, Darnell A, Díaz A, et al. Pancreatic Insufficiency in Patients Under Sorafenib Treatment for Hepatocellular Carcinoma. J Clin Gastroenterol. 2021;55(3):263-70.
Mir O, Coriat R, Boudou-Rouquette P, Durand JP, Goldwasser F. Sorafenib-induced diarrhea and hypophosphatemia: mechanisms and therapeutic implications. Ann Oncol. 2012;23(1):280-1.
Shinagare AB, Steele E, Braschi-Amirfarzan M, Tirumani SH, Ramaiya NH. Sunitinib-associated Pancreatic Atrophy in Patients with Gastrointestinal Stromal Tumor: A Toxicity with Prognostic Implications Detected at Imaging. Radiology. 2016;281(1):140-9.
Oshiro Y, Nishida K, Shimazaki J, Shimoda M, Suzuki S. Investigation of morphological and functional changes in the liver and pancreas during bevacizumab treatment. Scand J Gastroenterol. 2020;55(6):712-7.
Boocock GR, Morrison JA, Popovic M, Richards N, Ellis L, Durie PR, Rommens JM. Mutations in SBDS are associated with Shwachman-Diamond syndrome. Nat Genet. 2003;33(1):97-101.
Hill RE, Durie PR, Gaskin KJ, Davidson GP, Forstner GG. Steatorrhea and pancreatic insufficiency in Shwachman syndrome. Gastroenterology. 1982;83(1 Pt 1):22-7.
Sukalo M, Fiedler A, Guzmán C, Spranger S, Addor MC, McHeik JN, et al. Mutations in the human UBR1 gene and the associated phenotypic spectrum. Hum Mutat. 2014;35(5):521-31.
Ellery KM, Erdman SH. Johanson-Blizzard syndrome: expanding the phenotype of exocrine pancreatic insufficiency. Jop. 2014;15(4):388-90.
Ying Y, Liang Y, Luo X, Wei M. Case Report: Clinical and Genetic Characteristics of Pearson Syndrome in a Chinese Boy and 139 Patients. Front Genet. 2022;13:802402.
Shteyer E, Saada A, Shaag A, Al-Hijawi FA, Kidess R, Revel-Vilk S, Elpeleg O. Exocrine pancreatic insufficiency, dyserythropoeitic anemia, and calvarial hyperostosis are caused by a mutation in the COX4I2 gene. Am J Hum Genet. 2009;84(3):412-7.
Allen HL, Flanagan SE, Shaw-Smith C, De Franco E, Akerman I, Caswell R, et al. GATA6 haploinsufficiency causes pancreatic agenesis in humans. Nat Genet. 2011;44(1):20-2.
De Franco E, Shaw-Smith C, Flanagan SE, Shepherd MH, Hattersley AT, Ellard S. GATA6 mutations cause a broad phenotypic spectrum of diabetes from pancreatic agenesis to adult-onset diabetes without exocrine insufficiency. Diabetes. 2013;62(3):993-7.
Bellanné-Chantelot C, Chauveau D, Gautier JF, Dubois-Laforgue D, Clauin S, Beaufils S, et al. Clinical spectrum associated with hepatocyte nuclear factor-1beta mutations. Ann Intern Med. 2004;140(7):510-7.
Tjora E, Wathle G, Erchinger F, Engjom T, Molven A, Aksnes L, et al. Exocrine pancreatic function in hepatocyte nuclear factor 1β-maturity-onset diabetes of the young (HNF1B-MODY) is only moderately reduced: compensatory hypersecretion from a hypoplastic pancreas. Diabet Med. 2013;30(8):946-55.
Durie PR. Inherited and congenital disorders of the exocrine pancreas. Gastroenterologist. 1996;4(3):169-87.
Raeder H, Johansson S, Holm PI, Haldorsen IS, Mas E, Sbarra V, et al. Mutations in the CEL VNTR cause a syndrome of diabetes and pancreatic exocrine dysfunction. Nat Genet. 2006;38(1):54-62.
Yilmaz A, Hagberg L. Exocrine pancreatic insufficiency is common in people living with HIV on effective antiretroviral therapy. Infect Dis (Lond). 2018;50(3):193-9.
Carroccio A, Di Prima L, Di Grigoli C, Soresi M, Farinella E, Di Martino D, et al. Exocrine pancreatic function and fat malabsorption in human immunodeficiency virus-infected patients. Scand J Gastroenterol. 1999;34(7):729-34.
Carroccio A, Guarino A, Zuin G, Verghi F, Berni Canani R, Fontana M, et al. Efficacy of oral pancreatic enzyme therapy for the treatment of fat malabsorption in HIV-infected patients. Aliment Pharmacol Ther. 2001;15(10):1619-25.
Price DA, Schmid ML, Ong EL, Adjukeiwicz KM, Peaston B, Snow MH. Pancreatic exocrine insufficiency in HIV-positive patients. HIV Med. 2005;6(1):33-6.
Rawla P, Bandaru SS, Vellipuram AR. Review of Infectious Etiology of Acute Pancreatitis. Gastroenterology Res. 2017;10(3):153-8.
Panic N, Maetzel H, Bulajic M, Radovanovic M, Löhr JM. Pancreatic tuberculosis: A systematic review of symptoms, diagnosis and treatment. United European Gastroenterol J. 2020;8(4):396-402.
Khuroo MS, Zargar SA, Yattoo GN, Koul P, Khan BA, Dar MY, Alai MS. Ascaris-induced acute pancreatitis. Br J Surg. 1992;79(12):1335-8.
Worning H, Müllertz S, Thaysen EH, Bang HO. pH and concentration of pancreatic enzymes in aspirates from the human duodenum during digestion of a standard meal in patients with biliary or hepatic disorders. Scand J Gastroenterol. 1967;2(2):150-6.
Taneja S, Nagi B, Kochhar R, Bhasin DK, Lal A, Singh K. Intraductal pancreatic calculi in patients with choledochal cyst. Australas Radiol. 2004;48(3):302-5.
Wen WH, Chen HL, Chang MH, Ni YH, Shih HH, Lai HS, Hsu WM. Fecal elastase 1, serum amylase and lipase levels in children with cholestasis. Pancreatology. 2005;5(4-5):432-7.
Lankisch PG, Kaboth U, Koop H. [Involvement of the exocrine pancreas in Wilson's disease? (author's transl)]. Klin Wochenschr. 1978;56(19):969-71.
Bartos V, Melichar J, Erben J. The function of the exocrine pancreas in chronic renal disease. Digestion. 1970;3(1):33-40.
Poll M, Werner J, Huber W, Kempmann G, Willig F. [The exocrine pancreatic function in chronic renal insufficiency (author's transl)]. Z Gastroenterol. 1979;17(3):177-86.
Misumi A, Shiratori K, Lee KY, Barkin JS, Chey WY. Effects of SMS 201-995, a somatostatin analogue, on the exocrine pancreatic secretion and gut hormone release in dogs. Surgery. 1988;103(4):450-5.
Lembcke B, Creutzfeldt W, Schleser S, Ebert R, Shaw C, Koop I. Effect of the somatostatin analogue sandostatin (SMS 201-995) on gastrointestinal, pancreatic and biliary function and hormone release in normal men. Digestion. 1987;36(2):108-24.
Köhler E, Beglinger C, Dettwiler S, Whitehouse I, Gyr K. Effect of a new somatostatin analogue on pancreatic function in healthy volunteers. Pancreas. 1986;1(2):154-9.
Creutzfeldt W, Lankisch PG, Fölsch UR. [Inhibition by somatostatin of pancreatic juice and enzyme secretion and gallbladder contraction in man induced by secretin and cholecystokinin-pancreozymin administration (author's transl)]. Dtsch Med Wochenschr. 1975;100(20):1135-8.
Faiss S, Pape UF, Böhmig M, Dörffel Y, Mansmann U, Golder W, et al. Prospective, randomized, multicenter trial on the antiproliferative effect of lanreotide, interferon alfa, and their combination for therapy of metastatic neuroendocrine gastroenteropancreatic tumors--the International Lanreotide and Interferon Alfa Study Group. J Clin Oncol. 2003;21(14):2689-96.
Rinzivillo M, De Felice I, Magi L, Annibale B, Panzuto F. Occurrence of exocrine pancreatic insufficiency in patients with advanced neuroendocrine tumors treated with somatostatin analogs. Pancreatology. 2020;20(5):875-9.
Lamarca A, McCallum L, Nuttall C, Barriuso J, Backen A, Frizziero M, et al. Somatostatin analogue-induced pancreatic exocrine insufficiency in patients with neuroendocrine tumors: results of a prospective observational study. Expert Rev Gastroenterol Hepatol. 2018;12(7):723-31.
Saif MW, Romano A, Smith MH, Patel R, Relias V. Chronic Use of Long-Acting Somatostatin Analogues (SSAs) and Exocrine Pancreatic Insufficiency (EPI) in Patients with Gastroenteropancreatic Neuroendocrine Tumors (GEP-NETs): An Under-recognized Adverse Effect. Cancer Med J. 2020;3(2):75-84.
McDonald JD, Gupta S, Shindorf ML, Copeland A, Good ML, Sadowski SM, Nilubol N. Pancreatic insufficiency following pancreatectomy: Does underlying tumor syndrome confer a greater risk? Am J Surg. 2021;221(2):465-71.
Qureshi SA, Burch N, Druce M, Hattersley JG, Khan S, Gopalakrishnan K, et al. Screening for malnutrition in patients with gastro-entero-pancreatic neuroendocrine tumours: a cross-sectional study. BMJ Open. 2016;6(5):e010765.
Vujasinovic M, Tretjak M, Tepes B, Marolt A, Slemenik Pusnik C, Kotnik Kerbev M, Rudolf S. Is pancreatic exocrine insufficiency a result of decreased splanchnic circulation in patients with chronic heart failure? JOPJ Pancreas (Online). 2016;2016(17):201-3.
Özcan M, Öztürk GZ, Köse M, Emet S, Aydın Ş, Arslan K, et al. Evaluation of malnutrition with blood ghrelin and fecal elastase levels in acute decompensated heart failure patients. Turk Kardiyol Dern Ars. 2015;43(2):131-7.
Xia T, Chai X, Shen J. Pancreatic exocrine insufficiency in patients with chronic heart failure and its possible association with appetite loss. PLoS One. 2017;12(11):e0187804.
Gobelet C, Gerster JC, Rappoport G, Hiroz CA, Maeder E. A controlled study of the exocrine pancreatic function in Sjögren's syndrome and rheumatoid arthritis. Clin Rheumatol. 1983;2(2):139-43.
Coll J, Navarro S, Tomas R, Elena M, Martinez E. Exocrine pancreatic function in Sjögren's syndrome. Arch Intern Med. 1989;149(4):848-52.
Afzelius P, Fallentin EM, Larsen S, Møller S, Schiødt M. Pancreatic function and morphology in Sjögren's syndrome. Scand J Gastroenterol. 2010;45(6):752-8.
Hedström A, Kvarnström M, Lindberg G, Alsabeah S, Alsabeah H, Ndegwa N, et al. High prevalence of gastrointestinal symptoms in patients with primary Sjogren's syndrome cannot be attributed to pancreatic exocrine insufficiency. Scand J Gastroenterol. 2022;57(10):1250-6.