{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,11]],"date-time":"2026-09-11T00:15:28Z","timestamp":1789085728567,"version":"build-2803163510"},"reference-count":42,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2025,6,17]],"date-time":"2025-06-17T00:00:00Z","timestamp":1750118400000},"content-version":"vor","delay-in-days":16,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"},{"start":{"date-parts":[[2025,6,1]],"date-time":"2025-06-01T00:00:00Z","timestamp":1748736000000},"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":[[2025,6]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Prior studies have documented improvement in pancreatic status after ivacaftor and lumacaftor\/ivacaftor in a small number of patients with cystic fibrosis (CF). There is paucity of data with similar improvement after initiation of elexacaftor\/tezacaftor\/ivacaftor (ETI).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>In this cross\u2010sectional study, we retrospectively reviewed the change in fecal elastase\u20101 (FE\u20101) at least 6 months after initiation of ETI in children less than 12 years of age. Children who demonstrated a change in FE\u20101 of \u2265\u2009100\u2009\u00b5g\/g from the baseline after ETI therapy (post\u2010ETI FE\u20101\u2009minus pre\u2010ETI FE\u20101) were termed as responders and those with a change in FE\u20101\u2009&lt;\u2009100\u2009\u00b5g\/g post\u2010ETI (or no change in FE\u20101) were termed as nonresponders.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>Thirty\u2010five children had post\u2010ETI FE\u20101 value at least 6 months after its initiation and were included for final review. The mean change (\u00b1SD) in FE\u20101 post\u2010ETI from the baseline in the entire cohort was 47.2\u2009\u00b1\u200989.5. Seven children had a change in FE\u20101 of \u2265\u2009100\u2009\u00b5g\/g or more post\u2010ETI (responders). Two of these seven patients had a change in FE\u20101 of \u2265\u2009200\u2009\u00b5g\/g post\u2010ETI. Twenty\u2010three had no change or change &lt;\u2009100\u2009\u00b5g\/g post\u2010ETI (nonresponders). Another five children who did not have a baseline FE\u20101 and had post\u2010ETI FE\u20101\u2009&lt;\u200910\u2009\u00b5g\/g were also added to the nonresponders cohort increasing the total number to 28. The pre\u2010 and post\u2010ETI body mass index was significantly higher in the responder group compared to the nonresponders.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>In our cohort of 35 children, 7 (20%) were noted to have a positive change in FE\u20101 from the baseline value of \u2265\u2009100\u2009\u00b5g\/g post\u2010ETI. Further prospective multicenter studies will help identify predictive factors associated with improvement in FE\u20101 after initiation of CFTR\u2010directed modulator therapies.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/ppul.71156","type":"journal-article","created":{"date-parts":[[2025,6,17]],"date-time":"2025-06-17T08:23:09Z","timestamp":1750148589000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":13,"title":["Impact of Elexacaftor\/Tezacaftor\/Ivacaftor on Fecal Elastase\u20101 in Children With Cystic Fibrosis"],"prefix":"10.1002","volume":"60","author":[{"given":"Senthilkumar","family":"Sankararaman","sequence":"first","affiliation":[{"name":"Division of Pediatric Gastroenterology, Hepatology, and Nutrition Cleveland Clinic Children's Hospital Cleveland Ohio USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Leesa","family":"Prunty","sequence":"additional","affiliation":[{"name":"Division of Pediatric Pulmonology UH Rainbow Babies &amp; Children's Hospital Cleveland Ohio USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Catherine","family":"Pamer","sequence":"additional","affiliation":[{"name":"Division of Pediatric Pulmonology UH Rainbow Babies &amp; Children's Hospital Cleveland Ohio USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Aravind","family":"Thavamani","sequence":"additional","affiliation":[{"name":"Division of Pediatric Gastroenterology, Hepatology, and Nutrition UH Rainbow Babies &amp; Children's Hospital Cleveland Ohio USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Erica","family":"Roesch","sequence":"additional","affiliation":[{"name":"Division of Pediatric Pulmonology UH Rainbow Babies &amp; Children's Hospital Cleveland Ohio USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Teresa","family":"Schindler","sequence":"additional","affiliation":[{"name":"Division of Pediatric Pulmonology UH Rainbow Babies &amp; Children's Hospital Cleveland Ohio USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2025,6,17]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2022.06.005"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2022.10.003"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2023.09.005"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2006.01.005"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpeds.2004.04.049"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpeds.2009.09.001"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.1067\/mpd.2002.124829"},{"key":"e_1_2_8_9_1","unstructured":"S. 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