{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,24]],"date-time":"2026-09-24T04:04:59Z","timestamp":1790222699453,"version":"4.1.0"},"reference-count":39,"publisher":"The Endocrine Society","issue":"1","license":[{"start":{"date-parts":[[2023,7,28]],"date-time":"2023-07-28T00:00:00Z","timestamp":1690502400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/pages\/standard-publication-reuse-rights"}],"funder":[{"name":"Public Health Service","award":["R01 DK97830"],"award-info":[{"award-number":["R01 DK97830"]}]},{"name":"Public Health Service","award":["K23 DK107937"],"award-info":[{"award-number":["K23 DK107937"]}]},{"name":"Public Health Service","award":["UL1 TR001878"],"award-info":[{"award-number":["UL1 TR001878"]}]},{"name":"University of Pennsylvania Center for Human Phenomic Science","award":["P30 DK19525"],"award-info":[{"award-number":["P30 DK19525"]}]},{"name":"University of Pennsylvania Diabetes Research Center"},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Human Metabolism Resource of the University of Pennsylvania Institute for Diabetes, Obesity & Metabolism"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2023,12,21]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:sec>\n                  <jats:title>Context<\/jats:title>\n                  <jats:p>Elexacaftor\/tezacaftor\/ivacaftor (ETI; Trikafta) enhances aberrant cystic fibrosis transmembrane conductance regulator function and may improve the insulin secretory defects associated with a deterioration in clinical outcomes in pancreatic insufficient cystic fibrosis (PI-CF).<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Objective<\/jats:title>\n                  <jats:p>This longitudinal case-control study assessed changes in \u03b2-cell function and secretory capacity measures over 2 visits in individuals with PI-CF who were initiated on ETI after the baseline visit (2012-2018) and (1) restudied between 2019 and 2021 (ETI group) vs (2) those restudied between 2015 and 2018 and not yet treated with cystic fibrosis transmembrane conductance regulator modulator therapy (controls).<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Methods<\/jats:title>\n                  <jats:p>Nine ETI participants (mean \u00b1 SD age, 25 \u00b1 5 years) and 8 matched controls were followed up after a median (interquartile range) 5 (4-7) and 3 (2-3) years, respectively (P &amp;lt; .01), with ETI initiation a median of 1 year before follow-up. Clinical outcomes, glucose-potentiated arginine, and mixed-meal tolerance test measures were assessed with comparisons of within- and between-group change by nonparametric testing.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Results<\/jats:title>\n                  <jats:p>Glucose-potentiated insulin and C-peptide responses to glucose-potentiated arginine deteriorated in controls but not in the ETI group, with C-peptide changes different between groups (P &amp;lt; .05). Deterioration in basal proinsulin secretory ratio was observed in controls but improved, as did the maximal arginine-induced proinsulin secretory ratio, in the ETI group (P &amp;lt; .05 for all comparisons). During mixed-meal tolerance testing, early insulin secretion improved as evidenced by more rapid insulin secretory rate kinetics.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Conclusion<\/jats:title>\n                  <jats:p>ETI preserves \u03b2-cell function in CF through effects on glucose-dependent insulin secretion, proinsulin processing, and meal-related insulin secretion. Further work should determine whether early intervention with ETI can prevent deterioration of glucose tolerance in PI-CF.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1210\/clinem\/dgad443","type":"journal-article","created":{"date-parts":[[2023,7,28]],"date-time":"2023-07-28T10:28:59Z","timestamp":1690540139000},"page":"151-160","source":"Crossref","is-referenced-by-count":16,"title":["Preservation of \u03b2-cell Function in Pancreatic Insufficient Cystic Fibrosis With Highly Effective CFTR Modulator Therapy"],"prefix":"10.1210","volume":"109","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-4315-5136","authenticated-orcid":false,"given":"Anneliese J","family":"Flatt","sequence":"first","affiliation":[{"name":"Division of Endocrinology, Diabetes & Metabolism, Department of Medicine and Institute for Diabetes, Obesity & Metabolism, University of Pennsylvania Perelman School of Medicine , Philadelphia, PA 19104 , 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