Triple Therapy for Cystic Fibrosis Phe508del-Gating and -Residual Function Genotypes

First experience in Switzerland in Phe508del homozygous cystic fibrosis patients with end-stage pulmonary disease enrolled in a lumacaftor-ivacaftor therapy trial - preliminary results

Swiss Med Wkly.

Murer C, Huber LC, Kurowski T, Hirt A, Robinson CA, Bürgi U, Benden C

doi: 10.4414/smw.2018.14593.

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Pulmonary Fibrosis

Variation in lung function and nutritional decline in cystic fibrosis by genotype: An analysis of the Canadian cystic fibrosis registry

J Cyst Fibros.

Leung GJ, Cho TJ, Kovesi T, Hamid JS, Radhakrishnan D

doi: 10.1016/j.jcf.2019.06.007.

Pulmonary Fibrosis

SILDENAFIL IMPROVES VASCULAR ENDOTHELIAL FUNCTION IN PATIENTS WITH CYSTIC FIBROSIS

Am J Physiol Heart Circ Physiol.

Rodriguez Miguelez P, Seigler N, Tucker MA, Csanyi G, McKie KT, Forseen C, Harris RA

doi: 10.1152/ajpheart.00301.2018.

Pulmonary Fibrosis

Tezacaftor-Ivacaftor in Patients with Cystic Fibrosis Homozygous for Phe508del

N Engl J Med.

Taylor-Cousar JL, Munck A, McKone EF, van der Ent CK, Moeller A, Simard C, Wang LT, Ingenito EP, McKee C, Lu Y, Lekstrom-Himes J, Elborn JS

doi: 10.1056/

Pulmonary Fibrosis

Islet Hormone and Incretin Secretion in Cystic Fibrosis after Four Months of Ivacaftor Therapy

Am J Respir Crit Care Med.

Kelly A, De Leon DD, Sheikh S, Camburn D, Kubrak C, Peleckis AJ, Stefanovski D, Hadjiliadis D, Rickels MR, Rubenstein RC

doi: 10.1164/rccm.

Pulmonary Fibrosis

Impact of a cystic fibrosis transmembrane conductance regulator (CFTR) modulator on high-dose ibuprofen therapy in pediatric cystic fibrosis patients

Pediatr Pulmonol.

Bruch BA, Singh SB, Ramsey LJ, Starner TD

doi: 10.1002/ppul.24024.

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Inhaled mannitol for cystic fibrosis

Cochrane Database Syst Rev.

Nevitt SJ, Thornton J, Murray CS, Dwyer T

doi: 10.1002/14651858.

Pulmonary Fibrosis

Human epididymis protein 4 (HE4) levels inversely correlate with lung function improvement (delta FEV1) in cystic fibrosis patients receiving ivacaftor treatment

J Cyst Fibros.

Nagy B Jr, Bene Z, Fejes Z, Heltshe SL, Reid D, Ronan NJ, McCarthy Y, Smith D, Nagy A, Joseloff E, Balla G, Kappelmayer J, Macek M Jr, Bell SC, Plant BJ, Amaral MD, Balogh I

doi: 10.1016/j.jcf.2018.08.013.

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Long-term Triple Therapy De-escalation to Indacaterol/Glycopyrronium in COPD Patients (SUNSET): a Randomized, Double-Blind, Triple-Dummy Clinical Trial

Am J Respir Crit Care Med.

Chapman KR, Hurst JR, Frent SM, Larbig M, Fogel R, Guerin T, Banerji D, Patalano F, Goyal P, Pfister P, Kostikas K, Wedzicha JA

doi: 10.1164/

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First experience in Switzerland in Phe508del homozygous cystic fibrosis patients with end-stage pulmonary disease enrolled in a lumacaftor-ivacaftor therapy trial - preliminary results

Variation in lung function and nutritional decline in cystic fibrosis by genotype: An analysis of the Canadian cystic fibrosis registry

SILDENAFIL IMPROVES VASCULAR ENDOTHELIAL FUNCTION IN PATIENTS WITH CYSTIC FIBROSIS

Tezacaftor-Ivacaftor in Patients with Cystic Fibrosis Homozygous for Phe508del

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Long-term Triple Therapy De-escalation to Indacaterol/Glycopyrronium in COPD Patients (SUNSET): a Randomized, Double-Blind, Triple-Dummy Clinical Trial