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Entering the era of highly effective CFTR modulator therapy.

Zemanick ET, Accurso FJ.

Lancet. 2019 Oct 30. pii: S0140-6736(19)32676-5. doi: 10.1016/S0140-6736(19)32676-5. [Epub ahead of print] No abstract available.


Novel Application of Aptamer Proteomic Analysis in Cystic Fibrosis Bronchoalveolar Lavage Fluid.

DeBoer EM, Wagner BD, Popler J, Harris JK, Zemanick ET, Accurso FJ, Sagel SD, Deterding RR.

Proteomics Clin Appl. 2019 May;13(3):e1800085. doi: 10.1002/prca.201800085. Epub 2019 Jan 3.


Characteristics and outcomes of oral antibiotic treated pulmonary exacerbations in children with cystic fibrosis.

Hoppe JE, Wagner BD, Accurso FJ, Zemanick ET, Sagel SD.

J Cyst Fibros. 2018 Nov;17(6):760-768. doi: 10.1016/j.jcf.2018.05.015. Epub 2018 Jun 18.


Treatment of cystic fibrosis in infants.

Accurso FJ.

Lancet Respir Med. 2018 Jul;6(7):483-484. doi: 10.1016/S2213-2600(18)30203-0. Epub 2018 Jun 7. No abstract available.


KB001-A, a novel anti-inflammatory, found to be safe and well-tolerated in cystic fibrosis patients infected with Pseudomonas aeruginosa.

Jain R, Beckett VV, Konstan MW, Accurso FJ, Burns JL, Mayer-Hamblett N, Milla C, VanDevanter DR, Chmiel JF; KB001-A Study Group.

J Cyst Fibros. 2018 Jul;17(4):484-491. doi: 10.1016/j.jcf.2017.12.006. Epub 2017 Dec 29.


Pulmonary exacerbations and clinical outcomes in a longitudinal cohort of infants and preschool children with cystic fibrosis.

Hoppe JE, Wagner BD, Sagel SD, Accurso FJ, Zemanick ET.

BMC Pulm Med. 2017 Dec 11;17(1):188. doi: 10.1186/s12890-017-0546-8.


Airway microbiota across age and disease spectrum in cystic fibrosis.

Zemanick ET, Wagner BD, Robertson CE, Ahrens RC, Chmiel JF, Clancy JP, Gibson RL, Harris WT, Kurland G, Laguna TA, McColley SA, McCoy K, Retsch-Bogart G, Sobush KT, Zeitlin PL, Stevens MJ, Accurso FJ, Sagel SD, Harris JK.

Eur Respir J. 2017 Nov 16;50(5). pii: 1700832. doi: 10.1183/13993003.00832-2017. Print 2017 Nov.


Early childhood lung function is a stronger predictor of adolescent lung function in cystic fibrosis than early Pseudomonas aeruginosa infection.

Pittman JE, Noah H, Calloway HE, Davis SD, Leigh MW, Drumm M, Sagel SD, Accurso FJ, Knowles MR, Sontag MK.

PLoS One. 2017 May 15;12(5):e0177215. doi: 10.1371/journal.pone.0177215. eCollection 2017.


Proteomic profiling identifies novel circulating markers associated with bronchiectasis in cystic fibrosis.

DeBoer EM, Kroehl ME, Wagner BD, Accurso FJ, Harris JK, Lynch DA, Sagel SD, Deterding RR.

Proteomics Clin Appl. 2017 Sep;11(9-10). doi: 10.1002/prca.201600147. Epub 2017 May 29.


Restoring Cystic Fibrosis Transmembrane Conductance Regulator Function Reduces Airway Bacteria and Inflammation in People with Cystic Fibrosis and Chronic Lung Infections.

Hisert KB, Heltshe SL, Pope C, Jorth P, Wu X, Edwards RM, Radey M, Accurso FJ, Wolter DJ, Cooke G, Adam RJ, Carter S, Grogan B, Launspach JL, Donnelly SC, Gallagher CG, Bruce JE, Stoltz DA, Welsh MJ, Hoffman LR, McKone EF, Singh PK.

Am J Respir Crit Care Med. 2017 Jun 15;195(12):1617-1628. doi: 10.1164/rccm.201609-1954OC.


Diagnosis of Cystic Fibrosis in Screened Populations.

Farrell PM, White TB, Howenstine MS, Munck A, Parad RB, Rosenfeld M, Sommerburg O, Accurso FJ, Davies JC, Rock MJ, Sanders DB, Wilschanski M, Sermet-Gaudelus I, Blau H, Gartner S, McColley SA.

J Pediatr. 2017 Feb;181S:S33-S44.e2. doi: 10.1016/j.jpeds.2016.09.065.


Glucose >200 mg/dL during Continuous Glucose Monitoring Identifies Adult Patients at Risk for Development of Cystic Fibrosis Related Diabetes.

Taylor-Cousar JL, Janssen JS, Wilson A, Clair CG, Pickard KM, Jones MC, Brayshaw SJ, Chacon CS, Barboa CM, Sontag MK, Accurso FJ, Nichols DP, Saavedra MT, Nick JA.

J Diabetes Res. 2016;2016:1527932. doi: 10.1155/2016/1527932. Epub 2016 Nov 24.


Prevalence of elevated liver enzymes in children with cystic fibrosis diagnosed by newborn screen.

Woodruff SA, Sontag MK, Accurso FJ, Sokol RJ, Narkewicz MR.

J Cyst Fibros. 2017 Jan;16(1):139-145. doi: 10.1016/j.jcf.2016.08.002. Epub 2016 Aug 20.


Molecular Identification of Staphylococcus aureus in Airway Samples from Children with Cystic Fibrosis.

Johnson EJ, Zemanick ET, Accurso FJ, Wagner BD, Robertson CE, Harris JK.

PLoS One. 2016 Jan 25;11(1):e0147643. doi: 10.1371/journal.pone.0147643. eCollection 2016.


Recovery of Acquired Cystic Fibrosis Transmembrane Conductance Regulator Dysfunction after Smoking Cessation.

Courville CA, Raju SV, Liu B, Accurso FJ, Dransfield MT, Rowe SM.

Am J Respir Crit Care Med. 2015 Dec 15;192(12):1521-4. doi: 10.1164/rccm.201502-0396LE. No abstract available.


Genome-wide association meta-analysis identifies five modifier loci of lung disease severity in cystic fibrosis.

Corvol H, Blackman SM, Boëlle PY, Gallins PJ, Pace RG, Stonebraker JR, Accurso FJ, Clement A, Collaco JM, Dang H, Dang AT, Franca A, Gong J, Guillot L, Keenan K, Li W, Lin F, Patrone MV, Raraigh KS, Sun L, Zhou YH, O'Neal WK, Sontag MK, Levy H, Durie PR, Rommens JM, Drumm ML, Wright FA, Strug LJ, Cutting GR, Knowles MR.

Nat Commun. 2015 Sep 29;6:8382. doi: 10.1038/ncomms9382.


Cystic fibrosis, ivacaftor, and the Arg117His-CFTR mutation.

Accurso FJ.

Lancet Respir Med. 2015 Jul;3(7):498-9. doi: 10.1016/S2213-2600(15)00246-5. No abstract available.


Considerations for the Conduct of Clinical Trials with Antiinflammatory Agents in Cystic Fibrosis. A Cystic Fibrosis Foundation Workshop Report.

Torphy TJ, Allen J, Cantin AM, Konstan MW, Accurso FJ, Joseloff E, Ratjen FA, Chmiel JF; Antiinflammatory Therapy Working Group.

Ann Am Thorac Soc. 2015 Sep;12(9):1398-406. doi: 10.1513/AnnalsATS.201506-361OT. Review.


Use of ibuprofen to assess inflammatory biomarkers in induced sputum: Implications for clinical trials in cystic fibrosis.

Chmiel JF, Konstan MW, Accurso FJ, Lymp J, Mayer-Hamblett N, VanDevanter DR, Rose LM, Ramsey BW; Assessment of Induced Sputum in Cystic Fibrosis Study Group.

J Cyst Fibros. 2015 Nov;14(6):720-6. doi: 10.1016/j.jcf.2015.03.007. Epub 2015 Apr 11.


Sputum induction improves detection of pathogens in children with cystic fibrosis.

Hoppe JE, Towler E, Wagner BD, Accurso FJ, Sagel SD, Zemanick ET.

Pediatr Pulmonol. 2015 Jul;50(7):638-46. doi: 10.1002/ppul.23150. Epub 2015 Jan 7.


Assessment of airway microbiota and inflammation in cystic fibrosis using multiple sampling methods.

Zemanick ET, Wagner BD, Robertson CE, Stevens MJ, Szefler SJ, Accurso FJ, Sagel SD, Harris JK.

Ann Am Thorac Soc. 2015 Feb;12(2):221-9. doi: 10.1513/AnnalsATS.201407-310OC.


Pharmacokinetics and tolerability of oral sildenafil in adults with cystic fibrosis lung disease.

Taylor-Cousar JL, Wiley C, Felton LA, St Clair C, Jones M, Curran-Everett D, Poch K, Nichols DP, Solomon GM, Saavedra MT, Accurso FJ, Nick JA.

J Cyst Fibros. 2015 Mar;14(2):228-36. doi: 10.1016/j.jcf.2014.10.006. Epub 2014 Nov 13.


Ataluren for the treatment of nonsense-mutation cystic fibrosis: a randomised, double-blind, placebo-controlled phase 3 trial.

Kerem E, Konstan MW, De Boeck K, Accurso FJ, Sermet-Gaudelus I, Wilschanski M, Elborn JS, Melotti P, Bronsveld I, Fajac I, Malfroot A, Rosenbluth DB, Walker PA, McColley SA, Knoop C, Quattrucci S, Rietschel E, Zeitlin PL, Barth J, Elfring GL, Welch EM, Branstrom A, Spiegel RJ, Peltz SW, Ajayi T, Rowe SM; Cystic Fibrosis Ataluren Study Group.

Lancet Respir Med. 2014 Jul;2(7):539-47. doi: 10.1016/S2213-2600(14)70100-6. Epub 2014 May 15.


Cystic fibrosis transmembrane conductance regulator and pseudomonas.

Zemanick ET, Accurso FJ.

Am J Respir Crit Care Med. 2014 Apr 1;189(7):763-5. doi: 10.1164/rccm.201402-0209ED. No abstract available.


Sweat chloride as a biomarker of CFTR activity: proof of concept and ivacaftor clinical trial data.

Accurso FJ, Van Goor F, Zha J, Stone AJ, Dong Q, Ordonez CL, Rowe SM, Clancy JP, Konstan MW, Hoch HE, Heltshe SL, Ramsey BW, Campbell PW, Ashlock MA.

J Cyst Fibros. 2014 Mar;13(2):139-47.


Acquired defects in CFTR-dependent β-adrenergic sweat secretion in chronic obstructive pulmonary disease.

Courville CA, Tidwell S, Liu B, Accurso FJ, Dransfield MT, Rowe SM.

Respir Res. 2014 Feb 25;15:25. doi: 10.1186/1465-9921-15-25.


Evaluation of circulating proteins and hemodynamics towards predicting mortality in children with pulmonary arterial hypertension.

Wagner BD, Takatsuki S, Accurso FJ, Ivy DD.

PLoS One. 2013 Nov 20;8(11):e80235. doi: 10.1371/journal.pone.0080235. eCollection 2013.


Bronchoalveolar lavage fluid cytokine profiles in neuroendocrine cell hyperplasia of infancy and follicular bronchiolitis.

Popler J, Wagner BD, Tarro HL, Accurso FJ, Deterding RR.

Orphanet J Rare Dis. 2013 Nov 11;8:175. doi: 10.1186/1750-1172-8-175.


Cigarette smoke induces systemic defects in cystic fibrosis transmembrane conductance regulator function.

Raju SV, Jackson PL, Courville CA, McNicholas CM, Sloane PA, Sabbatini G, Tidwell S, Tang LP, Liu B, Fortenberry JA, Jones CW, Boydston JA, Clancy JP, Bowen LE, Accurso FJ, Blalock JE, Dransfield MT, Rowe SM.

Am J Respir Crit Care Med. 2013 Dec 1;188(11):1321-30. doi: 10.1164/rccm.201304-0733OC.


Anti-PcrV antibody in cystic fibrosis: a novel approach targeting Pseudomonas aeruginosa airway infection.

Milla CE, Chmiel JF, Accurso FJ, VanDevanter DR, Konstan MW, Yarranton G, Geller DE; KB001 Study Group.

Pediatr Pulmonol. 2014 Jul;49(7):650-8. doi: 10.1002/ppul.22890. Epub 2013 Sep 9.


Optimizing nasal potential difference analysis for CFTR modulator development: assessment of ivacaftor in CF subjects with the G551D-CFTR mutation.

Rowe SM, Liu B, Hill A, Hathorne H, Cohen M, Beamer JR, Accurso FJ, Dong Q, Ordoñez CL, Stone AJ, Olson ER, Clancy JP; VX06-770-101 Study Group.

PLoS One. 2013 Jul 26;8(7):e66955. doi: 10.1371/journal.pone.0066955. Print 2013.


Inflammation and airway microbiota during cystic fibrosis pulmonary exacerbations.

Zemanick ET, Harris JK, Wagner BD, Robertson CE, Sagel SD, Stevens MJ, Accurso FJ, Laguna TA.

PLoS One. 2013 Apr 30;8(4):e62917. doi: 10.1371/journal.pone.0062917. Print 2013.


Circulating cytokines and growth factors in pediatric pulmonary hypertension.

Duncan M, Wagner BD, Murray K, Allen J, Colvin K, Accurso FJ, Ivy DD.

Mediators Inflamm. 2012;2012:143428. doi: 10.1155/2012/143428. Epub 2012 Dec 18.


Abnormal infant pulmonary function in young children with neuroendocrine cell hyperplasia of infancy.

Kerby GS, Wagner BD, Popler J, Hay TC, Kopecky C, Wilcox SL, Quinones RR, Giller RH, Accurso FJ, Deterding RR.

Pediatr Pulmonol. 2013 Oct;48(10):1008-15. doi: 10.1002/ppul.22718. Epub 2012 Nov 20.


Preterm birth and airway inflammation in childhood.

Baker CD, Accurso FJ.

J Pediatr. 2012 Dec;161(6):979-80. doi: 10.1016/j.jpeds.2012.08.002. Epub 2012 Sep 14. No abstract available.


Long term effects of denufosol tetrasodium in patients with cystic fibrosis.

Ratjen F, Durham T, Navratil T, Schaberg A, Accurso FJ, Wainwright C, Barnes M, Moss RB; TIGER-2 Study Investigator Group.

J Cyst Fibros. 2012 Dec;11(6):539-49. doi: 10.1016/j.jcf.2012.05.003. Epub 2012 Jun 8.


Urinary desmosine: a biomarker of structural lung injury during CF pulmonary exacerbation.

Laguna TA, Wagner BD, Starcher B, Luckey Tarro HK, Mann SA, Sagel SD, Accurso FJ.

Pediatr Pulmonol. 2012 Sep;47(9):856-63. doi: 10.1002/ppul.22525. Epub 2012 Mar 19.


Future directions in early cystic fibrosis lung disease research: an NHLBI workshop report.

Ramsey BW, Banks-Schlegel S, Accurso FJ, Boucher RC, Cutting GR, Engelhardt JF, Guggino WB, Karp CL, Knowles MR, Kolls JK, LiPuma JJ, Lynch S, McCray PB Jr, Rubenstein RC, Singh PK, Sorscher E, Welsh M.

Am J Respir Crit Care Med. 2012 Apr 15;185(8):887-92. doi: 10.1164/rccm.201111-2068WS. Epub 2012 Feb 3.


Results of a phase IIa study of VX-809, an investigational CFTR corrector compound, in subjects with cystic fibrosis homozygous for the F508del-CFTR mutation.

Clancy JP, Rowe SM, Accurso FJ, Aitken ML, Amin RS, Ashlock MA, Ballmann M, Boyle MP, Bronsveld I, Campbell PW, De Boeck K, Donaldson SH, Dorkin HL, Dunitz JM, Durie PR, Jain M, Leonard A, McCoy KS, Moss RB, Pilewski JM, Rosenbluth DB, Rubenstein RC, Schechter MS, Botfield M, Ordoñez CL, Spencer-Green GT, Vernillet L, Wisseh S, Yen K, Konstan MW.

Thorax. 2012 Jan;67(1):12-8. doi: 10.1136/thoraxjnl-2011-200393. Epub 2011 Aug 8.


Denufosol tetrasodium in patients with cystic fibrosis and normal to mildly impaired lung function.

Accurso FJ, Moss RB, Wilmott RW, Anbar RD, Schaberg AE, Durham TA, Ramsey BW; TIGER-1 Investigator Study Group.

Am J Respir Crit Care Med. 2011 Mar 1;183(5):627-34. doi: 10.1164/rccm.201008-1267OC. Epub 2010 Dec 17.


Reliability of quantitative real-time PCR for bacterial detection in cystic fibrosis airway specimens.

Zemanick ET, Wagner BD, Sagel SD, Stevens MJ, Accurso FJ, Harris JK.

PLoS One. 2010 Nov 30;5(11):e15101. doi: 10.1371/journal.pone.0015101.


Effect of VX-770 in persons with cystic fibrosis and the G551D-CFTR mutation.

Accurso FJ, Rowe SM, Clancy JP, Boyle MP, Dunitz JM, Durie PR, Sagel SD, Hornick DB, Konstan MW, Donaldson SH, Moss RB, Pilewski JM, Rubenstein RC, Uluer AZ, Aitken ML, Freedman SD, Rose LM, Mayer-Hamblett N, Dong Q, Zha J, Stone AJ, Olson ER, Ordoñez CL, Campbell PW, Ashlock MA, Ramsey BW.

N Engl J Med. 2010 Nov 18;363(21):1991-2003. doi: 10.1056/NEJMoa0909825.


SerpinB1 in cystic fibrosis airway fluids: quantity, molecular form and mechanism of elastase inhibition.

Cooley J, Sontag MK, Accurso FJ, Remold-O'Donnell E.

Eur Respir J. 2011 May;37(5):1083-90. doi: 10.1183/09031936.00073710. Epub 2010 Sep 3.


Pulmonary exacerbations in cystic fibrosis with negative bacterial cultures.

Zemanick ET, Wagner BD, Harris JK, Wagener JS, Accurso FJ, Sagel SD.

Pediatr Pulmonol. 2010 Jun;45(6):569-77. doi: 10.1002/ppul.21221.


Effects of gender and age at diagnosis on disease progression in long-term survivors of cystic fibrosis.

Nick JA, Chacon CS, Brayshaw SJ, Jones MC, Barboa CM, St Clair CG, Young RL, Nichols DP, Janssen JS, Huitt GA, Iseman MD, Daley CL, Taylor-Cousar JL, Accurso FJ, Saavedra MT, Sontag MK.

Am J Respir Crit Care Med. 2010 Sep 1;182(5):614-26. doi: 10.1164/rccm.201001-0092OC. Epub 2010 May 6.


The applicability of urinary creatinine as a method of specimen normalization in the cystic fibrosis population.

Wagner BD, Accurso FJ, Laguna TA.

J Cyst Fibros. 2010 May;9(3):212-6. doi: 10.1016/j.jcf.2010.02.004. Epub 2010 Mar 15.


Lessons learned from a randomized trial of airway secretion clearance techniques in cystic fibrosis.

Sontag MK, Quittner AL, Modi AC, Koenig JM, Giles D, Oermann CM, Konstan MW, Castile R, Accurso FJ; Investigators and Coordinators of the Airway Secretion Clearance Trial.

Pediatr Pulmonol. 2010 Mar;45(3):291-300. doi: 10.1002/ppul.21179.


Cystic Fibrosis Foundation evidence-based guidelines for management of infants with cystic fibrosis.

Cystic Fibrosis Foundation, Borowitz D, Robinson KA, Rosenfeld M, Davis SD, Sabadosa KA, Spear SL, Michel SH, Parad RB, White TB, Farrell PM, Marshall BC, Accurso FJ.

J Pediatr. 2009 Dec;155(6 Suppl):S73-93. doi: 10.1016/j.jpeds.2009.09.001.


Cystic Fibrosis Foundation practice guidelines for the management of infants with cystic fibrosis transmembrane conductance regulator-related metabolic syndrome during the first two years of life and beyond.

Cystic Fibrosis Foundation, Borowitz D, Parad RB, Sharp JK, Sabadosa KA, Robinson KA, Rock MJ, Farrell PM, Sontag MK, Rosenfeld M, Davis SD, Marshall BC, Accurso FJ.

J Pediatr. 2009 Dec;155(6 Suppl):S106-16. doi: 10.1016/j.jpeds.2009.09.003.

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