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Article

Impact of age at ivacaftor initiation on pulmonary outcomes among people with cystic fibrosis.

Thorax - 18 Sept 2024

Merlo Christian A, McGarry Lisa J, Thorat Teja, Nguyen Catherine, DerSarkissian Maral, Muthukumar Aruna, Healy Joe, Brookhart M Alan, Rubin Jaime L

Abstract excerpt

BACKGROUND: Ivacaftor (IVA) improves lung function and other extrapulmonary outcomes in people with cystic fibrosis (CF). However, the effect of initiating IVA at earlier versus later ages has not been studied. METHODS: We conducted an observational cohort study of people in the US CF Foundation Patient Registry aged ≥6 years with ≥1 CF transmembrane conductance regulator-gating mutation to compare the effects of...

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Identifiers

PMID: 38719441
DOI: 10.1136/thorax-2023-220559
PMCID: PMC11503177

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