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Article

CFTR modulator drug desensitization: Preserving the hope of long term improvement.

Pediatric pulmonology - 1 Aug 2021

Leonhardt Kelsey, Autry Elizabeth B, Kuhn Robert J, Wurth Mark A

Abstract excerpt

The development of modulator therapy has, for the first time, allowed direct targeting of the underlying cause of cystic fibrosis (CF), the cystic fibrosis transmembrane conductance regulator (CFTR). Patients treated with CFTR modulators have improvement in lung function and decreased rates of pulmonary exacerbations. In 2019, elexacaftor/tezacaftor/ivacaftor was approved for use in the United States, opening...

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Identifiers

PMID: 33913624
DOI: 10.1002/ppul.25437

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 6 Sept 2026. Manifest publication-graph-cohort-2026-09-06.1.

Source receipt: pubmed, retrieved 6 Sept 2026.

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