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Normalization of total bilirubin after switch from elexacaftor/tezacaftor/ivacaftor to vanzacaftor/tezacaftor/deutivacaftor in patients with cystic fibrosis and Gilbert’s syndrome
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DOI：10.1016/j.jcf.2026.06.011.png)
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Abstract
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• Treatment with ETI might unmask Gilbert’s syndrome in some patients with cystic fibrosis. • One possible explanation might be an interaction of elexacftor and its metabolite M23elexacaftor with the transport proteins OATP1B1 and OATP1B3. • The new triple modulator VTD seems to have less interactions with OATP1B1 and OATP1B3. • We recognized a normalization of total bilirubin levels in pwCF and Gilbert’s syndrome after the switch from ETI to VTD. • Larger subject numbers have to confirm that VTD might be better in pCF and Gilbert’s syndrome.
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