{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,25]],"date-time":"2026-09-25T22:52:36Z","timestamp":1790376756128,"version":"4.1.0"},"reference-count":48,"publisher":"Oxford University Press (OUP)","issue":"3","license":[{"start":{"date-parts":[[2019,2,1]],"date-time":"2019-02-01T00:00:00Z","timestamp":1548979200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/pages\/standard-publication-reuse-rights"}],"funder":[{"name":"Cystic Fibrosis Foundation (A.K. and R.C.R.), Public Health Services","award":["R01 DK97830"],"award-info":[{"award-number":["R01 DK97830"]}]},{"name":"Cystic Fibrosis Foundation (A.K. and R.C.R.), Public Health Services","award":["K23 DK107937"],"award-info":[{"award-number":["K23 DK107937"]}]},{"name":"Cystic Fibrosis Foundation (A.K. and R.C.R.), Public Health Services","award":["UL1 TR001878"],"award-info":[{"award-number":["UL1 TR001878"]}]},{"name":"Cystic Fibrosis Foundation (A.K. and R.C.R.), Public Health Services","award":["P30 DK19525"],"award-info":[{"award-number":["P30 DK19525"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2019,2,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Rationale<\/jats:title>\n                    <jats:p>Diabetes is associated with worse cystic fibrosis (CF) outcomes. The CFTR potentiator ivacaftor is suggested to improve glucose homeostasis in individuals with CF.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Objectives<\/jats:title>\n                    <jats:p>To test the hypothesis that clinically indicated ivacaftor would be associated with improvements in glucose tolerance and insulin and incretin secretion.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>Oral glucose tolerance tests, mixed-meal tolerance tests, and glucose-potentiated arginine tests were compared preivacaftor initiation and 16 weeks postivacaftor initiation in CF participants with at least one CFTR gating or conductance mutation. Meal-related 30-minute (early phase) and 180-minute incremental area under the curves were calculated as responses for glucose, insulin, C-peptide, and incretin hormones; glucagon-like peptide-1; and glucose-dependent insulinotropic polypeptide. First-phase insulin secretion, glucose potentiation of arginine-induced insulin secretion, and disposition index were characterized by glucose-potentiated arginine stimulation tests.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Measurements and Main Results<\/jats:title>\n                    <jats:p>Twelve subjects completed the study: six male\/six female; seven normal\/five abnormal glucose tolerance (oral glucose tolerance test 1-h glucose \u2265155 and 2-h glucose &amp;lt;200 mg\/dl); of median (minimum\u2013maximum) age (13.8 yr [6.0\u201342.0]), body mass index-Z of 0.66 (\u22122.4 to 1.9), and FEV1% predicted of 102 (39\u2013122). Glucose tolerance normalized in one abnormal glucose tolerance subject. Ivacaftor treatment did not alter meal responses except for an increase in early phase C-peptide (P\u2009=\u20090.04). First-phase (P\u2009=\u20090.001) and glucose potentiation of arginine-induced (P\u2009=\u20090.027) insulin secretion assessed by acute C-peptide responses improved after ivacaftor treatment. Consistent with an effect on \u03b2-cell function, the disposition index relating the amount of insulin secreted for insulin sensitivity also improved (P\u2009=\u20090.04).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>Insulin secretion improved following 4 months of clinically indicated ivacaftor therapy in this relatively young group of patients with CF with normal to mildly impaired glucose tolerance, whereas incretin secretion remained unchanged.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1164\/rccm.201806-1018oc","type":"journal-article","created":{"date-parts":[[2018,8,21]],"date-time":"2018-08-21T18:47:08Z","timestamp":1534877228000},"page":"342-351","source":"Crossref","is-referenced-by-count":93,"title":["Islet Hormone and Incretin Secretion in Cystic Fibrosis after Four Months of Ivacaftor 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Pennsylvania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christina","family":"Kubrak","sequence":"additional","affiliation":[{"name":"Division of Pulmonary Medicine and Cystic Fibrosis Center, Department of Pediatrics, Children\u2019s Hospital of Philadelphia, Philadelphia, Pennsylvania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Amy J.","family":"Peleckis","sequence":"additional","affiliation":[{"name":"Division of Endocrinology, Diabetes & Metabolism and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Darko","family":"Stefanovski","sequence":"additional","affiliation":[{"name":"Department of Clinical Studies-NCI, University of Pennsylvania School of Veterinary Medicine, Philadelphia, Pennsylvania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Denis","family":"Hadjiliadis","sequence":"additional","affiliation":[{"name":"Division of Pulmonary & Critical Care Medicine and Cystic Fibrosis Center, Department of 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