ELX/TEZ/IVA use in cystic fibrosis liver disease: Is the perspective of improved lung function worth the risk?


      • Use of ELX/TEZ/IVA in chronic cystic fibrosis related liver disease may infrequently result in irreversible decline in liver disease requiring transplantation.
      • Although ELX/TEZ/IVA use may provide extremely beneficial pulmonary outcomes, these may come at the cost of significant side effects
      • The use of Child-Pugh grading when deciding to initiate ELX/TEZ/IVA, may have its limitations


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        • Arnon R
        • Annunziato RA
        • Miloh T
        • Padilla M
        • Sogawa H
        • Batemarco L
        • et al.
        Liver and combined lung and liver transplantation for cystic fibrosis: Analysis of the UNOS database.
        Pediatr Transplant. 2011; 15
        • Middleton PG
        • Mall MA
        • Dřevínek P
        • Lands LC
        • McKone EF
        • Polineni D
        • et al.
        Elexacaftor–Tezacaftor–Ivacaftor for cystic fibrosis with a single Phe508del allele.
        N Engl J Med. 2019 Nov 7; 381: 1809-1819
      1. Vertex Pharmaceuticals. Prescribing information for TRIKAFTA® (elexacaftor, tezacaftor, and ivacaftor tablets; ivacaftor tablets). 2021.

        • Stylemans D
        • François S
        • Vincken S
        • Verbanck S
        • Vanderhelst E.
        A case of self-limited drug induced liver injury under treatment with elexacaftor/tezacaftor/ivacaftor: When it is worth taking the risk.
        J Cyst Fibros. 2021;
        • Salehi M
        • Iqbal M
        • Dube A
        • AlJoudeh A
        • Edenborough F.
        Delayed hepatic necrosis in a cystic fibrosis patient taking Elexacaftor/Tezacaftor/Ivacaftor (Kaftrio).
        Respirat Med Case Rep. 2021; 34
        • Ragan HH
        • Autry E
        • Kormelink L
        • Bomersback T
        • Hewlett J
        • Shanley L
        • et al.
        The use of elexacaftor/tezacaftor/ivacaftor in patients with cystic fibrosis post-liver transplantation.
        Pediatr Pulmonol. 2020; 55