{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,12]],"date-time":"2026-09-12T19:16:05Z","timestamp":1789240565315,"version":"build-2803163510"},"reference-count":22,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2026,4,8]],"date-time":"2026-04-08T00:00:00Z","timestamp":1775606400000},"content-version":"vor","delay-in-days":7,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2026,4,1]],"date-time":"2026-04-01T00:00:00Z","timestamp":1775001600000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Pulmonology"],"published-print":{"date-parts":[[2026,4]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Due to the dysfunction and destruction of pancreatic cells in individuals with cystic fibrosis (CF), most require lifelong exogenous pancreatic enzyme supplementation. The objective of this study was to determine if elexacaftor\/tezacaftor\/ivacaftor (ETI) treatment improves pancreatic function in individuals with CF.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>\n                      A retrospective study was conducted utilizing CF Foundation Patient Registry data for individuals 0\u201321 years receiving care at the Tulsa CF Center. Data collected included demographic variables, fecal elastase (FE\u20101) values, BMI percentile, and CFTR mutations. Pancreatic sufficiency was defined as \u201csevere\u201d (&lt;\u2009100), \u201cmoderate\u201d (100\u2013200), and \u201csufficient\u201d (&gt;\u2009200). Descriptive statistics, and Spearman Correlation post\u2010hoc analysis (\n                      <jats:italic>p<\/jats:italic>\n                      \u2009&lt;\u20090.05) were performed.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      The majority of individuals with a post\u2010ETI FE\u20101 value (\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u200949) were homozygous for the F508del mutation (\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u200938, 78%). After ETI, most individuals had severe pancreatic insufficiency (\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u200939, 79.6%) followed by 10.2% (\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u20095) with moderate insufficiency and 10.2% (\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u20095) with sufficiency. The median time between ETI initiation and highest post\u2010FE\u20101 ranged from 14.07 months (IQR: 8.78\u201314.07) for the pancreatic sufficient group to 18.87 months (IQR: 10.42\u201033.04) for the severe pancreatic insufficiency group. The five individuals achieving post\u2010ETI sufficiency had a mean time of 10.95 months (SD\u2009=\u20096.18) between start of therapy and conversion to pancreatic sufficiency. Age at ETI start and FE\u20101 value were moderately negatively correlated (\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u20090.0024) with pancreatic sufficient individuals having the youngest median initiation age (5.28 years, IQR: 2.95\u20135.54). All pancreatic sufficient individuals at the post\u2010ETI assessment had homozygous F508del CFTR mutations (\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u20095). Twenty\u2010four individuals had both pre\u2010and\u2010post\u2010ETI FE\u20101 values. Of those 24, two converted (8.3%) to moderate sufficiency, and three (12.5%) to pancreatic sufficient.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>While exploring the impacts of ETI therapy on pancreatic function, this study revealed a potential reversal of pancreatic insufficiency. The results suggest the importance of early initiation of therapy and standardized protocols to monitor FE\u20101 values over time.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/ppul.71616","type":"journal-article","created":{"date-parts":[[2026,4,8]],"date-time":"2026-04-08T07:54:17Z","timestamp":1775634857000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Improving Pancreatic Function With Elexacaftor\/Tezacaftor\/Ivacaftor"],"prefix":"10.1002","volume":"61","author":[{"given":"Madison","family":"Pranske","sequence":"first","affiliation":[{"name":"Oklahoma State University College of Osteopathic Medicine Tulsa Oklahoma USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0002-4480-496X","authenticated-orcid":false,"given":"Alizay","family":"Paracha","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Division of Pulmonary and Sleep Medicine, Children's Hospital of Colorado Aurora Colorado USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carson","family":"Loncarich","sequence":"additional","affiliation":[{"name":"University of Oklahoma School of Community Medicine Tulsa Oklahoma USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9376-1524","authenticated-orcid":false,"given":"Amy D.","family":"Hendrix\u2010Dicken","sequence":"additional","affiliation":[{"name":"Department of Pediatrics University of Oklahoma School of Community Medicine Tulsa Oklahoma USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joseph","family":"Walter","sequence":"additional","affiliation":[{"name":"Pediatric Pulmonology, Saint Francis Children's Physicians Tulsa Oklahoma USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michelle","family":"Condren","sequence":"additional","affiliation":[{"name":"Department of Pediatrics University of 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