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

Journal of Cystic Fibrosis, no. 3 (2020): 415-420

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•We utilised a real life, case control study with 105 patients; 72 on LUM/IVA and 33 controls.•LUM/IVA demonstrated a large reduction in exacerbations with an incident rate ratio of 0.455 (95%CI; 0.306 – 0.676), p < 0.001.•prolonged the time to first exacerbation and reduced the rate of decline in ppFEV1 over 12 months.•Adverse events wer...更多

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作者
Koliarne Tong
Koliarne Tong
Daniel Barker
Daniel Barker
Megan France
Megan France
Simone Visser
Simone Visser
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