{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,24]],"date-time":"2026-01-24T01:46:28Z","timestamp":1769219188915,"version":"3.49.0"},"reference-count":26,"publisher":"Wiley","issue":"12","license":[{"start":{"date-parts":[[2023,4,17]],"date-time":"2023-04-17T00:00:00Z","timestamp":1681689600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Pulmonology"],"published-print":{"date-parts":[[2023,12]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Solid organ transplant (SOT) recipients with cystic fibrosis (CF) may benefit from the pulmonary and extrapulmonary benefits associated with CF transmembrane conductance regulator modulators. Nevertheless, evolution of modulator safety and efficacy data prompts consideration.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>The search terms \u201ctransplant\u201d AND \u201civacaftor\"(IVA) OR \"lumacaftor\"(LUM) OR \"tezacaftor\" (TEZ) OR \"elexacaftor\" (ELX) were utilized to conduct a scoping review of English articles from the period of January 1, 2012 to December 31, 2022. Search results from PubMed and Embase databases were reviewed by title and abstract for relevance. Included studies reported efficacy and safety outcomes of modulators in SOT recipients.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>One hundred thirty\u2010six patients from one cohort study (90 lung transplant recipients) and eight case reports and series (29 lung transplant recipients, 16 liver transplant recipients and one lung\/liver transplant patient) were included. Post\u2010modulator initiation, 33 patients did not necessitate tacrolimus dose adjustments, 10 required dose uptitration, and 43 required dose reductions. Moreover, LUM\/IVA use with azole antifungals may lead to subtherapeutic levels but opposing effects sustained tacrolimus levels. Liver transplant recipients were more likely to experience elevations in transaminases requiring pharmacologic or medical interventions. Majority of patients experienced improvements in pulmonary function, fasting blood glucose, hemoglobin, body mass index, and rhinosinusitis symptoms. However, intolerance or lack of benefit prompted discontinuation of ELX\/TEZ\/IVA in over 40% of lung\u2010transplant recipients in one study.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Modulator therapy has been reported to produce pulmonary and extra\u2010pulmonary benefits in the CF population with SOT. Considerations for modulator therapy initiation ought to include modulator pharmacokinetics, concomitant medications, and transplant type due to the complex nature of SOT recipients.<\/jats:p><\/jats:sec>","DOI":"10.1002\/ppul.26396","type":"journal-article","created":{"date-parts":[[2023,4,17]],"date-time":"2023-04-17T13:54:32Z","timestamp":1681739672000},"page":"3393-3402","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":9,"title":["Use of CFTR modulators in special populations, part 3: Solid organ transplant"],"prefix":"10.1002","volume":"58","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-5529-8869","authenticated-orcid":false,"given":"Nour K.","family":"Kadouh","sequence":"first","affiliation":[{"name":"Department of Pharmacy Services Michigan Medicine Ann Arbor Michigan USA"},{"name":"Department of Clinical Pharmacy, College of Pharmacy University of Michigan Ann Arbor Michigan USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3933-473X","authenticated-orcid":false,"given":"Joseph","family":"Elijah","sequence":"additional","affiliation":[{"name":"Department of Pharmacy Services Michigan Medicine Ann Arbor Michigan USA"},{"name":"Department of Clinical Pharmacy, College of Pharmacy University of Michigan Ann Arbor Michigan USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Linda J.","family":"Fitzgerald","sequence":"additional","affiliation":[{"name":"Department of Pharmacy Services Michigan Medicine Ann Arbor Michigan USA"},{"name":"Department of Clinical Pharmacy, College of Pharmacy University of Michigan Ann Arbor Michigan USA"},{"name":"Sanofi Medical Affairs, Bridgewater  New Jersey 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":"Department of Pharmacy Services Michigan Medicine Ann Arbor Michigan USA"},{"name":"Department of Clinical Pharmacy, College of Pharmacy University of Michigan 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":[[2023,4,17]]},"reference":[{"key":"e_1_2_9_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2019.03.002"},{"key":"e_1_2_9_3_1","doi-asserted-by":"publisher","DOI":"10.1097\/00005176-199900001-00001"},{"key":"e_1_2_9_4_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1105185"},{"key":"e_1_2_9_5_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1908639"},{"key":"e_1_2_9_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(19)32597-8"},{"key":"e_1_2_9_7_1","first-page":"S1569","article-title":"Effect of highly effective modulator treatment on sinonasal symptoms in cystic fibrosis","volume":"20","author":"DiMango E","year":"2020","journal-title":"J Cyst Fibros"},{"key":"e_1_2_9_8_1","doi-asserted-by":"publisher","DOI":"10.1111\/pedi.12026"},{"key":"e_1_2_9_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2021.01.007"},{"key":"e_1_2_9_10_1","unstructured":"Kalydeco\u2122.Package Insert.Vertex Pharmaceuticals. 2020."},{"key":"e_1_2_9_11_1","unstructured":"Orkambi\u2122.Package Insert.Vertex Pharmaceuticals. 2018."},{"key":"e_1_2_9_12_1","unstructured":"Symdeko\u2122.Package Insert.Vertex Pharmaceuticals. 2020."},{"key":"e_1_2_9_13_1","unstructured":"Trikafta\u2122.Package Insert.Vertex Pharmaceuticals. 2021."},{"key":"e_1_2_9_14_1","doi-asserted-by":"publisher","DOI":"10.7326\/M18-0850"},{"key":"e_1_2_9_15_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.healun.2021.08.009"},{"key":"e_1_2_9_16_1","doi-asserted-by":"publisher","DOI":"10.1111\/petr.14007"},{"key":"e_1_2_9_17_1","doi-asserted-by":"publisher","DOI":"10.1002\/ccr3.2053"},{"key":"e_1_2_9_18_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2021.07.017"},{"key":"e_1_2_9_19_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2021.05.008"},{"key":"e_1_2_9_20_1","article-title":"The use of elexacaftor\/tezacaftor\/ivacaftor in patients with cystic fibrosis postliver transplant: a case series","author":"Ragan H","year":"2021","journal-title":"Pediatr Pulmonol"},{"key":"e_1_2_9_21_1","doi-asserted-by":"publisher","DOI":"10.1002\/phar.2710"},{"key":"e_1_2_9_22_1","doi-asserted-by":"crossref","unstructured":"\u00d8rumMB R\u00f6nsholtFF JeppesenM et al. Outcome of elexacaftor\/tezacaftor\/ivacaftor therapy in patients with cystic fibrosis and solid organ transplantation.Pediatr Pulmonol. Published online October31 2022.doi:10.1002\/ppul.26217","DOI":"10.1002\/ppul.26217"},{"key":"e_1_2_9_23_1","doi-asserted-by":"crossref","unstructured":"RamosKJ GuimbellotJS ValapourM et al. Use of elexacaftor\/tezacaftor\/ivacaftor among cystic fibrosis lung transplant recipients.J Cyst Fibros. Published online April 23 2022.","DOI":"10.1016\/j.jcf.2022.04.009"},{"key":"e_1_2_9_24_1","doi-asserted-by":"publisher","DOI":"10.1111\/ajt.14232"},{"key":"e_1_2_9_25_1","unstructured":"Prograf\u2122.Package Insert.Astellas Pharma US. 2012."},{"key":"e_1_2_9_26_1","doi-asserted-by":"publisher","DOI":"10.1111\/tid.12924"},{"key":"e_1_2_9_27_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1908639"}],"container-title":["Pediatric Pulmonology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/ppul.26396","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,11,22]],"date-time":"2023-11-22T19:16:23Z","timestamp":1700680583000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/ppul.26396"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,4,17]]},"references-count":26,"journal-issue":{"issue":"12","published-print":{"date-parts":[[2023,12]]}},"alternative-id":["10.1002\/ppul.26396"],"URL":"https:\/\/doi.org\/10.1002\/ppul.26396","archive":["Portico"],"relation":{},"ISSN":["8755-6863","1099-0496"],"issn-type":[{"value":"8755-6863","type":"print"},{"value":"1099-0496","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023,4,17]]},"assertion":[{"value":"2022-11-20","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2023-03-15","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2023-04-17","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}