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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: west ne. 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: west ne. 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: west ne. 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.
Capitalizing on the heterogeneous effects of CFTR nonsense and frameshift variants to inform therapeutic strategy for cystic fibrosis.
Sharma N, Evans TA, Pellicore MJ, Davis E, Aksit MA, McCague AF, Joynt AT, Lu Z, Han ST, Anzmann AF, Lam AN, Thaxton A, West N, Merlo C, Gottschalk LB, Raraigh KS, Sosnay PR, Cotton CU, Cutting GR. Sharma N, et al. PLoS Genet. 2018 Nov 16;14(11):e1007723. doi: 10.1371/journal.pgen.1007723. eCollection 2018 Nov. PLoS Genet. 2018. PMID: 30444886 Free PMC article.
Cystic fibrosis transmembrane conductance regulator modulators reduce the risk of recurrent acute pancreatitis among adult patients with pancreas sufficient cystic fibrosis.
Akshintala VS, Kamal A, Faghih M, Cutting GR, Cebotaru L, West NE, Jennings MT, Dezube R, Whitcomb DC, Lechtzin N, Merlo CA, Singh VK. Akshintala VS, et al. Among authors: west ne. Pancreatology. 2019 Dec;19(8):1023-1026. doi: 10.1016/j.pan.2019.09.014. Epub 2019 Sep 28. Pancreatology. 2019. PMID: 31611131
Unmasking catamenial hemoptysis in the era of CFTR modulator therapy.
Montemayor K, Claudio AT, Carson S, Lechtzin N, Christianson MS, West NE. Montemayor K, et al. Among authors: west ne. J Cyst Fibros. 2020 Jul;19(4):e25-e27. doi: 10.1016/j.jcf.2020.01.005. Epub 2020 Jan 24. J Cyst Fibros. 2020. PMID: 31987762 Free PMC article.
197 results