{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,1]],"date-time":"2026-09-01T05:19:45Z","timestamp":1788239985372,"version":"build-2803163510"},"reference-count":14,"publisher":"Wiley","issue":"8","license":[{"start":{"date-parts":[[2026,8,6]],"date-time":"2026-08-06T00:00:00Z","timestamp":1785974400000},"content-version":"vor","delay-in-days":5,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2026,8,1]],"date-time":"2026-08-01T00:00:00Z","timestamp":1785542400000},"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":[[2026,8]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Elexacaftor\/tezacaftor\/ivacaftor (ETI) has significantly improved lung function and the clinical course of people with cystic fibrosis (PwCF) but also can cause elevations in liver function tests (LFTs). While the prescribing information (PI) provides some recommendations for holding ETI or hepatically adjusting doses based on certain criteria, clinicians are often faced with clinical scenarios that do not meet those criteria (e.g. PwCF with CF liver disease [CFLD, now termed CF hepatobiliary involvement (CFHBI)] that do not meet Child\u2010Pugh Class B or C criteria). The purpose of this survey was to describe clinical practices regarding LFT monitoring and ETI dose adjustments in relation to hepatotoxicity and LFT abnormalities in PwCF with and without baseline CFLD or elevations in LFTs across adult and pediatric CF Foundation accredited care centers and affiliate programs (CFF\u2010ACCAP) in the United States.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>There were 53 unique respondents with representation from physicians, pharmacists, and advanced practitioners. When managing ETI in response to transaminase elevations that didn't meet PI recommendations for holding ETI in PwCF without CFLD or baseline LFT elevations, 51% of respondents would not change ETI therapy, and 41% would repeat LFTs, usually within a 2\u22124 week time frame. For those who met PI recommendations for pausing ETI, 49% of respondents would repeat LFTs in 1 month. In PwCF with baseline CFLD that doesn't meet Child\u2010Pugh Class B or C criteria or baseline LFT elevations, 74% of respondents initiated ETI at full dose, and 79% of respondents checked LFTs earlier than the PI recommendations.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>Despite ETI's remarkable impact on pulmonary and extrapulmonary CF manifestations, the CF care team faces clinical challenges related to hepatotoxicity as well as appropriate monitoring and dosing of ETI in clinically gray scenarios. The results of this survey demonstrate the need for a multidisciplinary guideline to navigate these complex situations.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/ppul.71778","type":"journal-article","created":{"date-parts":[[2026,8,6]],"date-time":"2026-08-06T07:17:42Z","timestamp":1786000662000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Management Strategies for Hepatotoxicity Associated With Elexacaftor\/Tezacaftor\/Ivacaftor: Results of a Provider Survey"],"prefix":"10.1002","volume":"61","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3419-2613","authenticated-orcid":false,"given":"Charissa W.","family":"Kam","sequence":"first","affiliation":[{"name":"Department of Pharmacy University of North Carolina Medical Center Chapel Hill North Carolina USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wendy","family":"Bullington","sequence":"additional","affiliation":[{"name":"Department of Pharmacy Services Medical University of South Carolina Charleston South Carolina USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Amber","family":"Elliott","sequence":"additional","affiliation":[{"name":"Department of Pharmacotherapy and Pharmacy Services University Health San Antonio Texas USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jenna M.","family":"Faircloth","sequence":"additional","affiliation":[{"name":"Department of Pharmacy Texas Children's Hospital Houston Texas USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jonica","family":"Huntman","sequence":"additional","affiliation":[{"name":"St. Louis Children's Hospital St. Louis Missouri USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"HollyAnn R.","family":"Jacobs","sequence":"additional","affiliation":[{"name":"Department of Pharmacy University of Utah Hospitals and Clinics Salt Lake City Utah USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Margaret O.","family":"Poisson","sequence":"additional","affiliation":[{"name":"Department of Pharmacy Children's Hospital of Georgia Augusta Georgia USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0002-7753-5890","authenticated-orcid":false,"given":"Sabrina","family":"Garcia","sequence":"additional","affiliation":[{"name":"Cystic Fibrosis Center of Idaho, St. Luke's Health System Boise Idaho USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jennifer","family":"Witte","sequence":"additional","affiliation":[{"name":"Department of Pharmacy Driscoll Children's Hospital Corpus Christi Texas USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4920-8067","authenticated-orcid":false,"given":"Cameron J.","family":"McKinzie","sequence":"additional","affiliation":[{"name":"Department of Pharmacy University of North Carolina Medical Center Chapel Hill North Carolina USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2026,8,6]]},"reference":[{"key":"e_1_2_10_2_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1908639"},{"key":"e_1_2_10_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(19)32597-8"},{"key":"e_1_2_10_4_1","volume-title":"Trikafta (Elexacaftor\/Tezacaftor\/Ivacaftor) [Package Insert]","year":"2024"},{"key":"e_1_2_10_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2023.12.002"},{"key":"e_1_2_10_6_1","unstructured":"M. A.Chilvers E.Cho R.Dagenais et al. Canadian Clinical Consensus Guideline for Initiation Monitoring and Discontinuation of CFTR Modulator Therapies for Patients with Cystic Fibrosis (2024 Guideline Update) (2024) https:\/\/www.cysticfibrosis.ca\/uploads\/2024-EN-CFTR-Modulator-Guidelines.pdf."},{"key":"e_1_2_10_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2023.01.007"},{"key":"e_1_2_10_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2024.03.011"},{"key":"e_1_2_10_9_1","doi-asserted-by":"publisher","DOI":"10.1097\/HEP.0000000000000646"},{"key":"e_1_2_10_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.clinthera.2023.11.003"},{"key":"e_1_2_10_11_1","doi-asserted-by":"publisher","DOI":"10.1097\/MOG.0000000000001148"},{"key":"e_1_2_10_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2024.01.001"},{"key":"e_1_2_10_13_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.rmcr.2021.101553"},{"key":"e_1_2_10_14_1","doi-asserted-by":"publisher","DOI":"10.1164\/ajrccm-conference.2021.203.1_MeetingAbstracts.A2113"},{"key":"e_1_2_10_15_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2017.07.002"}],"container-title":["Pediatric Pulmonology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/ppul.71778","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1002\/ppul.71778","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/ppul.71778","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,9,1]],"date-time":"2026-09-01T05:04:20Z","timestamp":1788239060000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/ppul.71778"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,8]]},"references-count":14,"journal-issue":{"issue":"8","published-print":{"date-parts":[[2026,8]]}},"alternative-id":["10.1002\/ppul.71778"],"URL":"https:\/\/doi.org\/10.1002\/ppul.71778","archive":["Portico"],"relation":{},"ISSN":["8755-6863","1099-0496"],"issn-type":[{"value":"8755-6863","type":"print"},{"value":"1099-0496","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,8]]},"assertion":[{"value":"2026-01-20","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2026-07-30","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2026-08-06","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}],"article-number":"e71778"}}