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Article

Lumacaftor/ivacaftor reduces exacerbations in adults homozygous for Phe508del mutation with severe lung disease.

Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society - 1 May 2020

Tong Koliarne, Barker Daniel, France Megan, Burr Lucy, Greville Hugh, Visser Simone, Middleton Peter, Wainwright Claire, Dorahy Douglas, Wark Peter

Abstract excerpt

BACKGROUND: Lumacaftor/ivacaftor (LUM/IVA) improves outcomes in cystic fibrosis (CF) patients homozygous for Phe508del with ppFEV1 > 40%. There is limited safety or efficacy data in patients with ppFEV1 < 40%. We determined whether LUM/IVA in patients with ppFEV1 < 40 would reduce the rate of pulmonary exacerbations. METHODS: This was a case control study performed on patients > 12 years, homozygous for Phe508del...

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Identifiers

PMID: 31848044
DOI: 10.1016/j.jcf.2019.12.006

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