Update in Cystic Fibrosis 2018

Bonnie W Ramsey^{1

2

3}, Gregory P Downey^{4

5

6

7

8}, Christopher H Goss^{9

3

10}

Affiliations

¹ 1 Department of Pediatrics, University of Washington School of Medicine, Seattle, Washington.
² 2 Center for Clinical and Translational Research and.
³ 3 Division of Pediatric Pulmonology, Department of Pediatrics, and.
⁴ 4 Division of Pulmonary, Critical Care, and Sleep Medicine, Department of Medicine.
⁵ 5 Department of Pediatrics, and.
⁶ 6 Department of Biomedical Research, National Jewish Health, Denver, Colorado; and.
⁷ 7 Division of Pulmonary Sciences and Critical Care Medicine, Department of Medicine, and.
⁸ 8 Department of Microbiology and Immunology, University of Colorado, Aurora, Colorado.
⁹ 10 Cystic Fibrosis Foundation Therapeutics Development Network Coordinating Center, Seattle Children's Research Institute, Seattle, Washington.
¹⁰ 9 Division of Pulmonary and Critical Care Medicine, Department of Medicine, University of Washington, Seattle, Washington.

Review

Bonnie W Ramsey et al. Am J Respir Crit Care Med. 2019.

. 2019 May 15;199(10):1188-1194.

doi: 10.1164/rccm.201902-0310UP.

Bonnie W Ramsey^{1

2

3}, Gregory P Downey^{4

5

6

7

8}, Christopher H Goss^{9

3

10}

¹ 1 Department of Pediatrics, University of Washington School of Medicine, Seattle, Washington.
² 2 Center for Clinical and Translational Research and.
³ 3 Division of Pediatric Pulmonology, Department of Pediatrics, and.
⁴ 4 Division of Pulmonary, Critical Care, and Sleep Medicine, Department of Medicine.
⁵ 5 Department of Pediatrics, and.
⁶ 6 Department of Biomedical Research, National Jewish Health, Denver, Colorado; and.
⁷ 7 Division of Pulmonary Sciences and Critical Care Medicine, Department of Medicine, and.
⁸ 8 Department of Microbiology and Immunology, University of Colorado, Aurora, Colorado.
⁹ 10 Cystic Fibrosis Foundation Therapeutics Development Network Coordinating Center, Seattle Children's Research Institute, Seattle, Washington.
¹⁰ 9 Division of Pulmonary and Critical Care Medicine, Department of Medicine, University of Washington, Seattle, Washington.

No abstract available

References

1. Ramsey BW, Davies J, McElvaney NG, Tullis E, Bell SC, Dřevínek P, et al. VX08-770-102 Study Group. A CFTR potentiator in patients with cystic fibrosis and the G551D mutation. N Engl J Med. 2011;365:1663–1672. - PMC - PubMed
1. Wainwright CE, Elborn JS, Ramsey BW, Marigowda G, Huang X, Cipolli M, et al. TRAFFIC Study Group; TRANSPORT Study Group. Lumacaftor-ivacaftor in patients with cystic fibrosis homozygous for Phe508del CFTR. N Engl J Med. 2015;373:220–231. - PMC - PubMed
1. Donaldson SH, Pilewski JM, Griese M, Cooke J, Viswanathan L, Tullis E, et al. VX11-661-101 Study Group. Tezacaftor/ivacaftor in subjects with cystic fibrosis and F508del/F508del-CFTR or F508del/G551D-CFTR. Am J Respir Crit Care Med. 2018;197:214–224. - PMC - PubMed
1. Rowe SM, Daines C, Ringshausen FC, Kerem E, Wilson J, Tullis E, et al. Tezacaftor-ivacaftor in residual-function heterozygotes with cystic fibrosis. N Engl J Med. 2017;377:2024–2035. - PMC - PubMed
1. Keating D, Marigowda G, Burr L, Daines C, Mall MA, McKone EF, et al. VX16-445-001 Study Group. VX-445-tezacaftor-ivacaftor in patients with cystic fibrosis and one or two Phe508del alleles. N Engl J Med. 2018;379:1612–1620. - PMC - PubMed