Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society · 2026
Extrapulmonary benefits and pharmacogenomics in lung transplant recipients with Cystic Fibrosis on Elexacaftor/Tezacaftor/Ivacaftor (ETI) therapy.
СтатьяНазвание и аннотация: Перевод в очереди
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Kennedy John C, Taddesse Aderajew A, Coppolino Antonio, Lee Stella E, Dahlberg Suzanne E, Perkins Ryan C, Cagnina Rebecca E, Sheikh Adil, Cernadas Manuela, Jacob Selvin S, Kear Kathryn, Kubiak Sarah, Zhang Yiyang, Keshk Mohammed A, Hackmann Amy E, Lee Stefi F, Fieg Elizabeth, Algarawi Asma, Ito Ethan K, Kerman Benjamin J, Woolley Ann E, Washko George R, Goldberg Hilary J, Raby Benjamin A, Uluer Ahmet Z
Аннотация
Elexacaftor/Tezacaftor/Ivacaftor (ETI) and other highly effective modulator therapies have transformed outcomes for people with cystic fibrosis (pwCF), yielding significant pulmonary and extrapulmonary benefits. Although lung transplant (LTx) rates have declined dramatically in the post ETI era, a substantial number of transplant recipients with CF remain who may benefit from the extrapulmonary effects of these therapies. Unfortunately, available post market data regarding ETI safety, efficacy, and tolerability following LTx are limited and conflicting, raising concerns for Drug-Drug Interactions (DDI) and long-term graft function. In this single center retrospective cohort analysis, we report sustained improvements in sinonasal disease and weight maintenance with preserved spirometric values over a mean follow up of 35.1 months on ETI compared to those not on therapy. We additionally present pharmacogenomic testing in a subset of our cohort as proof-of-concept for predicting DDIs at time of initiation of ETI.