Title: Investigating the effects of long-term dornase alfa use on lung function using registry data.
Authors Newsome SJ, Daniel RM, Carr SB, Bilton D, Keogh RH.
References J Cyst Fibros. 2018 Aug 29. pii: S1569-1993(18)30747-1. doi: 10.1016/j.jcf.2018.08.004
Keywords Long-term treatment effect; Patient registry; UK Cystic Fibrosis Registry
Title: Correspondence between symptoms and preference-based health status measures in the STOP study.
Authors Gold LS, Patrick DL, Hansen RN, Beckett V, Goss CH, Kessler L.
References J Cyst Fibros. 2018 Aug 28. pii: S1569-1993(18)30743-4. doi: 10.1016/j.jcf.2018.08.001.
Keywords 5-level EuroQOL-5 dimensions, Cost effectiveness analysis, Cystic Fibrosis Respiratory Symptom Diary - Chronic Respiratory Infection Symptom Score, Lung function, Pulmonary exacerbations
Title: DNA-analysis, Extended CFTR-gene analysis, Immuno-reactive trypsin, Newborn screening, Pancreatitis-associated protein,Validity
Authors Dankert-Roelse JE, Bouva MJ, Jakobs BS, Janssens HM, de Winter-de Groot KM, Schönbeck Y, Gille JJP, Gulmans VAM, Verschoof-Puite RK, Schielen PCJI, Verkerk PH
References J Cyst Fibros. 2018 Aug 23. pii: S1569-1993(18)30705-7. doi: 10.1016/j.jcf.2018.07.008
Keywords DNA-analysis, Extended CFTR-gene analysis, Immuno-reactive trypsin, Newborn screening, Pancreatitis-associated protein, Validity
Title: Lumacaftor/Ivacaftor reduces pulmonary exacerbations in patients irrespective of initial changes in FEV1.
Authors McColley SA, Konstan MW, Ramsey BW, Stuart Elborn J, Boyle MP, Wainwright CE, Waltz D, Vera-Llonch M, Marigowda G, Jiang JG, Rubin JL
References J Cyst Fibros. 2018 Aug 23. pii: S1569-1993(18)30719-7. doi: 10.1016/j.jcf.2018.07.011.
Keywords Ivacaftor, Lumacaftor, Percent predicted forced expiratory volume in 1 s, Pulmonary exacerbations
Title: Rescue of CFTR NBD2 mutants N1303K and S1235R is influenced by the functioning of the autophagosome
Authors Liu Q, Sabirzhanova I, Yanda MK, Bergbower EAS, Boinot C, Guggino WB, Cebotaru L
References J Cyst Fibros. 2018 Jun 20. pii: S1569-1993(18)30621-0. doi: 10.1016/j.jcf.2018.05.016.
Keywords Autophagy, CFTR, Correctors, Degradation, Mutations, Short circuit current, Western blot
Title: Eradication of Persistent Methicillin-resistant Staphylococcus aureus Infection in Cystic Fibrosis
Authors Rebecca Dezube*, Mark T. Jennings*, Mary Rykiel, Marie Diener-West, Michael P. Boyle, James F. Chmiel, Elliott C. Dasenbrook - *Co-First Authors
References J Cyst Fibros. 2018 Aug 18. pii: S1569-1993(18)30702-1. doi: 10.1016/j.jcf.2018.07.005
Keywords Clinical trial, MRSA
Title: Antisense oligonucleotide targeting of mRNAs encoding ENaC subunits α, β, and ɣ improves cystic fibrosis-like disease in mice
Authors Zhao C, Crosby J, Lv T, Bai D, Monia BP, Guo S.
References J Cyst Fibros. 2018 Aug 10. pii: S1569-1993(18)30703-3. doi: 10.1016/j.jcf.2018.07.006
Keywords Antisense oligonucleotide, ENaC, Subunit
Title: Continuous glucose monitoring in youth with cystic fibrosis treated with lumacaftor-ivacaftor
Authors Angel Li, TimVigers, Laura Pyle, Edith Zemanick, Kristen Nadeau, Scott D. Sagel, Christine L. Chan
References J Cyst Fibros. 2018 Aug 10. pii: S1569-1993(18)30718-5. doi: 10.1016/j.jcf.2018.07.010
Keywords continuous glucose monitoring, Cystic fibrosis related diabetes, Cystic fibrosis transmembrane conductance regulator protein modulator