{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,8]],"date-time":"2026-02-08T04:24:00Z","timestamp":1770524640063,"version":"3.49.0"},"reference-count":4,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2025,12,26]],"date-time":"2025-12-26T00:00:00Z","timestamp":1766707200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2025,12,26]],"date-time":"2025-12-26T00:00:00Z","timestamp":1766707200000},"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":[[2026,1]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:p>The management of cystic fibrosis (CF) has significantly improved with the approval of the CF transmembrane conductance regulator (CFTR) modulators. Elexacaftor\/tezacaftor\/ivacaftor (ETI) is approved for treatment in people with CF (pwCF) over the age of 2 years with eligible mutations. We report 2 cases highlighting improved clinical outcomes following exposure to ETI in an off\u2010label use, including enhanced nutrition status, decrease in respiratory support, exacerbation rates, and need for antibiotics in one of the infants and resolution of echogenic bowel that was diagnosed in utero in the second.<\/jats:p>","DOI":"10.1002\/ppul.71444","type":"journal-article","created":{"date-parts":[[2025,12,26]],"date-time":"2025-12-26T12:45:21Z","timestamp":1766753121000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["A Tale of Two Infants: Off\u2010Label Use of Elexacaftor\/Tezacaftor\/Ivacaftor in Early Cystic Fibrosis Treatment"],"prefix":"10.1002","volume":"61","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-5529-8869","authenticated-orcid":false,"given":"Nour K.","family":"Kadouh","sequence":"first","affiliation":[{"name":"College of Pharmacy University of Michigan Ann Arbor Michigan USA"},{"name":"Michigan Medicine, C.S. Mott Children\u2032s Hospital Ann Arbor Michigan USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4124-316X","authenticated-orcid":false,"given":"Samya Z.","family":"Nasr","sequence":"additional","affiliation":[{"name":"Michigan Medicine, C.S. Mott Children\u2032s Hospital Ann Arbor Michigan USA"},{"name":"Medical School University of Michigan Ann Arbor Michigan USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6374-4870","authenticated-orcid":false,"given":"Amy","family":"Filbrun","sequence":"additional","affiliation":[{"name":"Michigan Medicine, C.S. Mott Children\u2032s Hospital Ann Arbor Michigan USA"},{"name":"Medical School University of Michigan Ann Arbor Michigan USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4968-1460","authenticated-orcid":false,"given":"Hanna","family":"Phan","sequence":"additional","affiliation":[{"name":"College of Pharmacy University of Michigan Ann Arbor Michigan USA"},{"name":"Michigan Medicine, C.S. Mott Children\u2032s Hospital Ann Arbor Michigan USA"},{"name":"Susan B. Meister Child Health Evaluation and Research (CHEAR) Center University of Michigan Ann Arbor Michigan USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2025,12,26]]},"reference":[{"key":"e_1_2_12_2_1","unstructured":"Trikafta\u2122[package insert] (Vertex Pharmaceuticals Inc 2021)."},{"key":"e_1_2_12_3_1","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.26809"},{"key":"e_1_2_12_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2025.01.0124"},{"key":"e_1_2_12_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2025.08.006"}],"container-title":["Pediatric Pulmonology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/ppul.71444","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1002\/ppul.71444","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/ppul.71444","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,1,29]],"date-time":"2026-01-29T08:27:23Z","timestamp":1769675243000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/ppul.71444"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,12,26]]},"references-count":4,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2026,1]]}},"alternative-id":["10.1002\/ppul.71444"],"URL":"https:\/\/doi.org\/10.1002\/ppul.71444","archive":["Portico"],"relation":{},"ISSN":["8755-6863","1099-0496"],"issn-type":[{"value":"8755-6863","type":"print"},{"value":"1099-0496","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,12,26]]},"assertion":[{"value":"2025-07-28","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2025-12-19","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2025-12-26","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}],"article-number":"e71444"}}