Heterogeneity of short-term elexacaftor-tezacaftor-ivacaftor response in cystic fibrosis using <sup>129</sup> Xe MRI
Though all outcome measures showed a positive response, response to ETI was heterogeneous between individuals and between <sup>129</sup>XeMRI, MBW and spirometry. Leveraging the rich information provided by multimodal tools will be critical to understanding the nature of CF lung disease and measuring response to future therapies in the era of CFTR modulators.
Authors
- Josie Chrenek (ORCID: https://orcid.org/0000-0003-0947-4918)
- Bradley S. Quon (ORCID: https://orcid.org/0000-0003-1785-3870)
- Rachel L. Eddy (ORCID: https://orcid.org/0000-0002-2541-1645)
- V. Diamond (ORCID: https://orcid.org/0000-0001-8314-5130)
- Amardeep Aulakh
- Girija Bhatnagar (ORCID: https://orcid.org/0009-0005-6479-9346)
- Britney X Ha
- David Sag
- Jonathan H. Rayment (ORCID: https://orcid.org/0000-0003-4955-8876)
- Rana Ghadymimahani
Institutions
- University of British Columbia (CA)
- BC Children's Hospital (CA)
Publication Details
- Journal
- Thorax
- Published
- 2025-12-09
- DOI
- https://doi.org/10.1136/thorax-2025-223687
- Primary Topic
- Cystic Fibrosis Research Advances
- Type
- article
- Field-Weighted Citation Impact
- 0.00