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CFTR bearing variant p.Phe312del exhibits function inconsistent with phenotype and negligible response to ivacaftor.
Raraigh KS, Paul KC, Goralski JL, Worthington EN, Faino AV, Sciortino S, Wang Y, Aksit MA, Ling H, Osorio DL, Onchiri FM, Patel SU, Merlo CA, Montemayor K, Gibson RL, West NE, Thakerar A, Bridges RJ, Sheppard DN, Sharma N, Cutting GR. Raraigh KS, et al. Among authors: gibson rl. JCI Insight. 2022 Mar 22;7(6):e148841. doi: 10.1172/jci.insight.148841. JCI Insight. 2022. PMID: 35315358 Free PMC article.
Standardized Treatment of Pulmonary Exacerbations (STOP) study: Observations at the initiation of intravenous antibiotics for cystic fibrosis pulmonary exacerbations.
Sanders DB, Solomon GM, Beckett VV, West NE, Daines CL, Heltshe SL, VanDevanter DR, Spahr JE, Gibson RL, Nick JA, Marshall BC, Flume PA, Goss CH; STOP Study Group. Sanders DB, et al. Among authors: gibson rl. J Cyst Fibros. 2017 Sep;16(5):592-599. doi: 10.1016/j.jcf.2017.04.005. Epub 2017 Apr 29. J Cyst Fibros. 2017. PMID: 28460885 Free PMC article.
Home Monitoring of Patients with Cystic Fibrosis to Identify and Treat Acute Pulmonary Exacerbations. eICE Study Results.
Lechtzin N, Mayer-Hamblett N, West NE, Allgood S, Wilhelm E, Khan U, Aitken ML, Ramsey BW, Boyle MP, Mogayzel PJ Jr, Gibson RL, Orenstein D, Milla C, Clancy JP, Antony V, Goss CH; eICE Study Team. Lechtzin N, et al. Among authors: gibson rl. Am J Respir Crit Care Med. 2017 Nov 1;196(9):1144-1151. doi: 10.1164/rccm.201610-2172OC. Am J Respir Crit Care Med. 2017. PMID: 28608719 Free PMC article. Clinical Trial.
Prevalence and clinical associations of Staphylococcus aureus small-colony variant respiratory infection in children with cystic fibrosis (SCVSA): a multicentre, observational study.
Wolter DJ, Onchiri FM, Emerson J, Precit MR, Lee M, McNamara S, Nay L, Blackledge M, Uluer A, Orenstein DM, Mann M, Hoover W, Gibson RL, Burns JL, Hoffman LR; SCVSA study group. Wolter DJ, et al. Among authors: gibson rl. Lancet Respir Med. 2019 Dec;7(12):1027-1038. doi: 10.1016/S2213-2600(19)30365-0. Epub 2019 Nov 11. Lancet Respir Med. 2019. PMID: 31727592 Free PMC article.
191 results