{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,30]],"date-time":"2026-09-30T21:28:56Z","timestamp":1790803736271,"version":"4.1.0"},"reference-count":55,"publisher":"The Endocrine Society","issue":"9","license":[{"start":{"date-parts":[[2024,12,9]],"date-time":"2024-12-09T00:00:00Z","timestamp":1733702400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/pages\/standard-publication-reuse-rights"}],"funder":[{"DOI":"10.13039\/100000897","name":"CF Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100000897","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100006920","name":"University of Pennsylvania","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100006920","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100008116","name":"Diabetes Research Center","doi-asserted-by":"publisher","award":["NCT04038047"],"award-info":[{"award-number":["NCT04038047"]}],"id":[{"id":"10.13039\/100008116","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2025,8,7]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Elexacaftor\/tezacaftor\/ivacaftor (ETI) is a highly effective therapy that improves lung disease in people with cystic fibrosis (pwCF), but its effect on glucose tolerance and insulin secretion is unclear.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>PROMISE is a multicenter prospective, observational study of ETI in pwCF \u226512 years and at least one F508del allele. The PROMISE Endocrine substudy (PROMISE-ENDO) enrolled participants at 10 CF Centers where hemoglobin A1c (HbA1c) was collected and 3-hour oral glucose tolerance tests (OGTT) conducted to examine glucose tolerance, glucose excursions, and insulin secretory rates (deconvolution of C-peptide) and sensitivity (oral minimal model) prior to ETI and 12 to 18 months and 24-30 months following ETI initiation. Longitudinal mixed effects models were used to test within-subject ETI effects.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>At baseline, 79 participants completed OGTTs (39 [49%] male, median [IQR] age 19.6 [14.7, 27.3] years, BMI z-score 0.12 [\u22120.51, 0.65]). At 12-18 months n = 68 and at 24-30 months n = 58 completed OGTTs. At 24-30 months, fasting glucose (mg\/dL) decreased (94 [92, 96] to 90 [88, 93], P = .02) in the subset not on insulin therapy (n = 61), but no differences in 1-hour or 2-hour glucose were found. HbA1c decreased from 5.8% (5.6%, 5.9%) to 5.5% (5.4%, 5.6%), P &amp;lt; .001 by 24-30 months. Although insulin sensitivity (mU\/L\u22121\u2005min\u22121) decreased (8.4 [7.2, 9.5] vs 6.8 [5.8, 7.9], P = .03), no changes in oral disposition index were found, P = .14.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>After 2 years of ETI, fasting glucose and HbA1c showed modest decreases. Glucose tolerance varied, and overall measures of insulin secretion did not deteriorate.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1210\/clinem\/dgae857","type":"journal-article","created":{"date-parts":[[2024,12,6]],"date-time":"2024-12-06T23:25:37Z","timestamp":1733527537000},"page":"2674-2684","source":"Crossref","is-referenced-by-count":30,"title":["Glycemia and Insulin Secretion in Cystic Fibrosis 2 Years After Elexacaftor\/Tezacaftor\/Ivacaftor: PROMISE-ENDO"],"prefix":"10.1210","volume":"110","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3067-7672","authenticated-orcid":false,"given":"Christine L","family":"Chan","sequence":"first","affiliation":[{"name":"Department of Pediatrics, Children\u2019s Hospital Colorado, University of Colorado School of Medicine , Aurora, CO 80045 ,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Meghan","family":"Shirley Bezerra","sequence":"additional","affiliation":[{"name":"Division of Endocrinology 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H","family":"Donaldson","sequence":"additional","affiliation":[{"name":"Department of Medicine, University of North Carolina at Chapel Hill , Chapel Hill, NC 27514 ,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carla A","family":"Frederick","sequence":"additional","affiliation":[{"name":"Department of Medicine, Jacobs School of Medicine and Biomedical Sciences of the University of Buffalo , Buffalo, NY 14203 ,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Steven D","family":"Freedman","sequence":"additional","affiliation":[{"name":"Beth Israel Deaconess Medical Center, Harvard Medical School , Boston, MA 02215 ,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daniel","family":"Gelfond","sequence":"additional","affiliation":[{"name":"Western New York Pediatric Gastroenterology , Batavia, NY 14020 ,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lucas R","family":"Hoffman","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, University of Washington School of Medicine , Seattle, WA 98105 ,","place":["USA"]},{"name":"Department of Microbiology, University of Washington School of Medicine , Seattle, WA 98109 ,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael R","family":"Narkewicz","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Children\u2019s Hospital Colorado, University of Colorado School of Medicine , Aurora, CO 80045 ,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Steven M","family":"Rowe","sequence":"additional","affiliation":[{"name":"Cystic Fibrosis Foundation , Bethesda, MD 20814 ,","place":["USA"]},{"name":"Department of Medicine and the Gregory Fleming James Cystic Fibrosis Research Center, University of Alabama at Birmingham , Birmingham, AL 35233 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