{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,9]],"date-time":"2026-09-09T08:19:56Z","timestamp":1788941996857,"version":"build-2803163510"},"reference-count":11,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2025,11,26]],"date-time":"2025-11-26T00:00:00Z","timestamp":1764115200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2025,11,26]],"date-time":"2025-11-26T00:00:00Z","timestamp":1764115200000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"funder":[{"DOI":"10.13039\/100000897","name":"Cystic Fibrosis Foundation","doi-asserted-by":"publisher","award":["NARKEW17AB0"],"award-info":[{"award-number":["NARKEW17AB0"]}],"id":[{"id":"10.13039\/100000897","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J. pediatr. gastroenterol. nutr."],"published-print":{"date-parts":[[2026,2]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Objectives<\/jats:title>\n                    <jats:p>The impact of elexacaftor\/tezacaftor\/ivacaftor (ETI) on cystic fibrosis (CF) hepatobiliary involvement (CFHBI) is uncertain. The goal of this study was to investigate the changes in key liver parameters in PUSH study participants who started ETI compared to those who did not (noETI).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>PUSH was a prospective observational study of persons with CF (pwCF) (3\u201312 yo at PUSH entry). Linear mixed effect model (LMEM) with one knot tested if ETI changed the slope of trajectories of clinical parameters by comparing ETI to noETI. Index time (IT) for ETI was ETI start and time of ETI availability for noETI. Time zero in the LMEM was index time.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      One hundred forty\u2010seven participants (104 ETI, 43 noETI). At IT, the groups were similar, for age, and liver parameters. Mean duration of ETI use was 22 months. The annual rate of change over time after IT in ETI compared to noETI was significantly improved for FEV\n                      <jats:sub>1<\/jats:sub>\n                      % pre (+3.4\/yr\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u20090.02) and wt z score (+0.06\/yr,\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u20090.006). There was improvement for ETI vs noETI for GGT (\u221215%\/yr,\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u20090.01) and ALT (\u221212%\/yr,\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u20090.02). Participants with CFHBI on ETI demonstrated similar trends and also had improvements in GGT and GGT to platelet ratio (GPR), but there were no differences in liver stiffness or US classification changes.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>GGT and GPR improve in pwCF and CFHBI who received ETI compared to noETI. There were no changes in other liver parameters. This suggests an early signal for positive impact on CFHBI, but no early improvement in fibrosis.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/jpn3.70290","type":"journal-article","created":{"date-parts":[[2025,11,26]],"date-time":"2025-11-26T09:32:24Z","timestamp":1764149544000},"page":"350-357","source":"Crossref","is-referenced-by-count":2,"title":["Impact of elexacaftor\/tezacaftor\/ivacaftor on biomarkers of cystic fibrosis hepatobiliary involvement in the PUSH study"],"prefix":"10.1002","volume":"82","author":[{"given":"Wen","family":"Ye","sequence":"first","affiliation":[{"name":"Department of Biostatistics University of Michigan Medical School Ann Arbor Michigan USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"John C.","family":"Magee","sequence":"additional","affiliation":[{"name":"Department of Surgery University of Michigan Medical School Ann Arbor Michigan USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Suiyuan","family":"Huang","sequence":"additional","affiliation":[{"name":"Department of Biostatistics University of Michigan Medical School Ann Arbor Michigan USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daniel H.","family":"Leung","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Baylor College of Medicine Division of Gastroenterology, Hepatology and Nutrition, Texas Children\u2032s Hospital Houston Texas USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Molly","family":"Bozic","sequence":"additional","affiliation":[{"name":"Pediatric Gastroenterology, Hepatology and Nutrition Riley Hospital for Children\/Indiana University School of Medicine Indianapolis Indiana USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Janis","family":"Stoll","sequence":"additional","affiliation":[{"name":"Division of Pediatric Gastroenterology, Hepatology and Nutrition Washington University School of Medicine St Louis Missouri USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Simon","family":"C Ling","sequence":"additional","affiliation":[{"name":"Division of Gastroenterology, Hepatology and Nutrition, The Hospital for Sick Children, Department of Paediatrics University of Toronto Toronto Ontario Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shruti M.","family":"Paranjape","sequence":"additional","affiliation":[{"name":"Division of Pediatric Pulmonology John Hopkins School of Medicine Baltimore Maryland USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wikrom","family":"Karnsakul","sequence":"additional","affiliation":[{"name":"Division of Pediatric Gastroenterology Hepatology and Nutrition, John Hopkins School of Medicine Baltimore Maryland USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A.","family":"Jay Freeman","sequence":"additional","affiliation":[{"name":"Division of Pediatric Gastroenterology Hepatology and Nutrition Nationwide Children\u2032s Hospital, The Ohio State College of Medicine Columbus Ohio USA"},{"name":"Department of Pediatrics The Ohio State College of Medicine Columbus Ohio USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sarah Jane","family":"Schwarzenberg","sequence":"additional","affiliation":[{"name":"Pediatric Gastroenterology, MHealth Fairview Masonic Children's Hospital Minneapolis Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joseph","family":"Palermo","sequence":"additional","affiliation":[{"name":"Division of Pediatric Gastroenterology, Hepatology and Nutrition, Cincinnati Children\u2032s Hospital Medical Center University of Cincinnati College of Medicine Cincinnati Ohio USA"},{"name":"Department of Pediatrics University of Cincinnati College of Medicine Cincinnati Ohio USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Estella","family":"Alonso","sequence":"additional","affiliation":[{"name":"Division of Pediatric Gastroenterology Hepatology and Nutrition, Ann &amp; Robert H. Lurie Children\u2032s Hospital Chicago Illinois USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nicole","family":"Green","sequence":"additional","affiliation":[{"name":"Division of Gastroenterology and Hepatology, Department of Pediatrics University of Washington and Seattle Children\u2032s Hospital Seattle Washington USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Prakash","family":"Masand","sequence":"additional","affiliation":[{"name":"Division of Radiology Texas Children\u2032s Hospital Houston Texas USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Boaz","family":"Karmazyn","sequence":"additional","affiliation":[{"name":"Department of Radiology and Imaging Sciences Riley Hospital for Children at IU Health and Indiana University School of Medicine Indianapolis Indiana USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Roger","family":"Harned","sequence":"additional","affiliation":[{"name":"Division of Pediatric Radiology, Children's Hospital ColoradoChildren's Hospital Colorado University of Colorado School of Medicine Aurora Colorado USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Oscar M.","family":"Navarro","sequence":"additional","affiliation":[{"name":"Department of Medical Imaging University of Toronto Toronto Ontario Canada"},{"name":"Department of Diagnostic and Interventional Radiology The Hospital for Sick Children Toronto Ontario Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marilyn J.","family":"Siegel","sequence":"additional","affiliation":[{"name":"Mallinckrodt Institute of Radiology Washington University School of Medicine St Louis Missouri USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alex","family":"Towbin","sequence":"additional","affiliation":[{"name":"Department of Radiology Cincinnati Children\u2032s Hospital Medical Center, University of Cincinnati College of Medicine Cincinnati Cincinnati Ohio USA"},{"name":"Department of Radiology University of Cincinnati College of Medicine Cincinnati Cincinnati Ohio USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Adina","family":"Alazraki","sequence":"additional","affiliation":[{"name":"Departments of Pediatrics and Radiology Emory University School of Medicine and Children\u2032s Healthcare of Atlanta, Arthur M Blank Campus Atlanta Georgia USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Randolph K.","family":"Otto","sequence":"additional","affiliation":[{"name":"Division of Pediatric Radiology Seattle Children\u2032s Hospital and University of Washington School of Medicine Seattle Washington USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jennifer L.","family":"Nicholas","sequence":"additional","affiliation":[{"name":"Department of Radiology Cincinnati Children\u2032s Hospital Medical Center, University of Cincinnati College of Medicine Cincinnati Cincinnati Ohio USA"},{"name":"Department of Radiology University of Cincinnati College of Medicine Cincinnati Cincinnati Ohio USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0002-7645-4685","authenticated-orcid":false,"given":"Michael R.","family":"Narkewicz","sequence":"additional","affiliation":[{"name":"Section of Pediatric Gastroenterology, Hepatology and Nutrition Department of Pediatrics and Digestive Health Institute University of Colorado School of Medicine and Children's Hospital Colorado Aurora Colorado USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2025,11,26]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.201503-0578OC"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa0909825"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.202108-1986OC"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.1097\/MPG.0000000000003944"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2023.03.019"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpeds.2019.12.033"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpeds.2015.06.062"},{"key":"e_1_2_8_9_1","doi-asserted-by":"publisher","DOI":"10.1002\/hep4.1719"},{"key":"e_1_2_8_10_1","doi-asserted-by":"publisher","DOI":"10.1097\/MPG.0000000000000758"},{"key":"e_1_2_8_11_1","doi-asserted-by":"publisher","DOI":"10.1097\/MPG.0000000000002412"},{"key":"e_1_2_8_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.cgh.2016.03.041"}],"container-title":["Journal 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