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Individuals diagnosed with EPI are often labeled as having \u201cpancreas insufficient cystic fibrosis (PI\u2010CF)\u201d while those with normal exocrine function are labeled as \u201cpancreas sufficient CF (PS\u2010CF).\u201d This diagnosis of EPI relies on clinical and laboratory features and management involves consumption of pancreas enzyme replacement therapy. In this review, we discuss the nuances of diagnosis and management of EPI in CF. We also present emerging evidence on the effects of CFTR modulating agents on the management of EPI, and speculate that these medications may lead to greater heterogeneity in management of EPI in CF moving forward.<\/jats:p>","DOI":"10.1002\/ppul.27013","type":"journal-article","created":{"date-parts":[[2024,8,6]],"date-time":"2024-08-06T09:34:59Z","timestamp":1722936899000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["Pancreas and pancreatitis: Exocrine pancreatic insufficiency"],"prefix":"10.1002","volume":"59","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-6430-1924","authenticated-orcid":false,"given":"Mitchell L.","family":"Ramsey","sequence":"first","affiliation":[{"name":"Division of Gastroenterology, Hepatology, and Nutrition The Ohio State University Wexner Medical Center Columbus Ohio USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gary J.","family":"Galante","sequence":"additional","affiliation":[{"name":"Section of Pediatric Gastroenterology, Hepatology, and Nutrition, Department of Pediatrics Alberta Children's Hospital, University of Calgary Calgary Alberta Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2024,8,6]]},"reference":[{"key":"e_1_2_10_2_1","first-page":"224","article-title":"Cystic fibrosis of the pancreas","volume":"45","author":"Jeffrey FW","year":"1941","journal-title":"Can Med Assoc J"},{"key":"e_1_2_10_3_1","doi-asserted-by":"publisher","DOI":"10.1001\/archpedi.1938.01980140114013"},{"key":"e_1_2_10_4_1","first-page":"41","article-title":"The pulmonary aspects of cystic fibrosis of the pancreas","volume":"57","author":"Dickey L.","year":"1942","journal-title":"Cal West Med"},{"key":"e_1_2_10_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/0895-4356(88)90063-7"},{"key":"e_1_2_10_6_1","doi-asserted-by":"publisher","DOI":"10.1172\/JCI115358"},{"key":"e_1_2_10_7_1","doi-asserted-by":"publisher","DOI":"10.1097\/MPA.0000000000000421"},{"key":"e_1_2_10_8_1","doi-asserted-by":"publisher","DOI":"10.1053\/j.gastro.2010.09.046"},{"key":"e_1_2_10_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2014.01.007"},{"key":"e_1_2_10_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpeds.2004.04.049"},{"key":"e_1_2_10_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpeds.2012.10.008"},{"key":"e_1_2_10_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2022.08.008"},{"key":"e_1_2_10_13_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2021.03.018"},{"key":"e_1_2_10_14_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2021.09.010"},{"key":"e_1_2_10_15_1","doi-asserted-by":"publisher","DOI":"10.3748\/wjg.v19.i42.7258"},{"key":"e_1_2_10_16_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.pan.2021.11.005"},{"key":"e_1_2_10_17_1","doi-asserted-by":"publisher","DOI":"10.1097\/MPG.0000000000000830"},{"key":"e_1_2_10_18_1","doi-asserted-by":"publisher","DOI":"10.1542\/pir.31.10.407"},{"key":"e_1_2_10_19_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM197304192881603"},{"key":"e_1_2_10_20_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.pan.2015.07.001"},{"key":"e_1_2_10_21_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2011.03.008"},{"key":"e_1_2_10_22_1","first-page":"55","article-title":"Enzyme replacement therapy for pancreatic insufficiency: present and future","volume":"4","author":"Fieker A","year":"2011","journal-title":"Clin Exp Gastroenterol"},{"key":"e_1_2_10_23_1","doi-asserted-by":"publisher","DOI":"10.1136\/jclinpath-2013-201787"},{"key":"e_1_2_10_24_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(99)70059-4"},{"key":"e_1_2_10_25_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF02002713"},{"key":"e_1_2_10_26_1","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.20041"},{"key":"e_1_2_10_27_1","doi-asserted-by":"publisher","DOI":"10.1093\/ajcn\/70.3.378"},{"key":"e_1_2_10_28_1","first-page":"714","article-title":"Studies in pancreatic function; 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