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Lung function changes before and after pulmonary exacerbation antimicrobial treatment in cystic fibrosis.

Wagener JS, VanDevanter DR, Konstan MW, Pasta DJ, Millar SJ, Morgan WJ.

Pediatr Pulmonol. 2019 Nov 20. doi: 10.1002/ppul.24577. [Epub ahead of print]


Clinical care for cystic fibrosis: preparing for the future now.

Konstan MW, Flume PA.

Lancet Respir Med. 2020 Jan;8(1):10-12. doi: 10.1016/S2213-2600(19)30334-0. Epub 2019 Sep 27. No abstract available.


Amikacin liposome inhalation suspension for chronic Pseudomonas aeruginosa infection in cystic fibrosis.

Bilton D, Pressler T, Fajac I, Clancy JP, Sands D, Minic P, Cipolli M, Galeva I, Solé A, Quittner AL, Liu K, McGinnis JP 2nd, Eagle G, Gupta R, Konstan MW; CLEAR-108 Study Group.

J Cyst Fibros. 2019 Aug 23. pii: S1569-1993(19)30833-1. doi: 10.1016/j.jcf.2019.08.001. [Epub ahead of print]


Disease progression in patients with cystic fibrosis treated with ivacaftor: Data from national US and UK registries.

Volkova N, Moy K, Evans J, Campbell D, Tian S, Simard C, Higgins M, Konstan MW, Sawicki GS, Elbert A, Charman SC, Marshall BC, Bilton D.

J Cyst Fibros. 2019 Jun 10. pii: S1569-1993(19)30767-2. doi: 10.1016/j.jcf.2019.05.015. [Epub ahead of print]


Transition to adulthood and adult health care for patients with sickle cell disease or cystic fibrosis: Current practices and research priorities.

Lanzkron S, Sawicki GS, Hassell KL, Konstan MW, Liem RI, McColley SA.

J Clin Transl Sci. 2018 Oct;2(5):334-342. doi: 10.1017/cts.2018.338. Review.


Modeling long-term health outcomes of patients with cystic fibrosis homozygous for F508del-CFTR treated with lumacaftor/ivacaftor.

Rubin JL, O'Callaghan L, Pelligra C, Konstan MW, Ward A, Ishak JK, Chandler C, Liou TG.

Ther Adv Respir Dis. 2019 Jan-Dec;13:1753466618820186. doi: 10.1177/1753466618820186.


Opportunities for life course research through the integration of data across Clinical and Translational Research Institutes.

Hanson HA, Hay WW Jr, Tobin JN, Barkin SL, Atkins M, Karagas MR, Dozier AM, Wetmore C, Konstan MW, Heubi JE.

J Clin Transl Sci. 2018 Jun;2(3):156-162. doi: 10.1017/cts.2018.29.


Lumacaftor/Ivacaftor reduces pulmonary exacerbations in patients irrespective of initial changes in FEV1.

McColley SA, Konstan MW, Ramsey BW, Stuart Elborn J, Boyle MP, Wainwright CE, Waltz D, Vera-Llonch M, Marigowda G, Jiang JG, Rubin JL.

J Cyst Fibros. 2019 Jan;18(1):94-101. doi: 10.1016/j.jcf.2018.07.011. Epub 2018 Aug 23.


Data from the US and UK cystic fibrosis registries support disease modification by CFTR modulation with ivacaftor.

Bessonova L, Volkova N, Higgins M, Bengtsson L, Tian S, Simard C, Konstan MW, Sawicki GS, Sewall A, Nyangoma S, Elbert A, Marshall BC, Bilton D.

Thorax. 2018 Aug;73(8):731-740. doi: 10.1136/thoraxjnl-2017-210394. Epub 2018 May 10.


Pulmonary exacerbations and acute declines in lung function in patients with cystic fibrosis.

Wagener JS, Williams MJ, Millar SJ, Morgan WJ, Pasta DJ, Konstan MW.

J Cyst Fibros. 2018 Jul;17(4):496-502. doi: 10.1016/j.jcf.2018.02.003. Epub 2018 Apr 21.


Association of High-Dose Ibuprofen Use, Lung Function Decline, and Long-Term Survival in Children with Cystic Fibrosis.

Konstan MW, VanDevanter DR, Sawicki GS, Pasta DJ, Foreman AJ, Neiman EA, Morgan WJ.

Ann Am Thorac Soc. 2018 Apr;15(4):485-493. doi: 10.1513/AnnalsATS.201706-486OC.


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.


Treatment Setting and Outcomes of Cystic Fibrosis Pulmonary Exacerbations.

Schechter MS, VanDevanter DR, Pasta DJ, Short SA, Morgan WJ, Konstan MW; Scientific Advisory Group and the Investigators and Coordinators of the Epidemiologic Study of Cystic Fibrosis.

Ann Am Thorac Soc. 2018 Feb;15(2):225-233. doi: 10.1513/AnnalsATS.201702-111OC.


Lung function decline is delayed but not decreased in patients with cystic fibrosis and the R117H gene mutation.

Wagener JS, Millar SJ, Mayer-Hamblett N, Sawicki GS, McKone EF, Goss CH, Konstan MW, Morgan WJ, Pasta DJ, Moss RB.

J Cyst Fibros. 2018 Jul;17(4):503-510. doi: 10.1016/j.jcf.2017.10.003. Epub 2017 Oct 31.


Cystic fibrosis clinical characteristics associated with dornase alfa treatment regimen change.

VanDevanter DR, Craib ML, Pasta DJ, Millar SJ, Morgan WJ, Konstan MW; Scientific Advisory Group and the Investigators and Coordinators of the Epidemiologic Study of Cystic Fibrosis.

Pediatr Pulmonol. 2018 Jan;53(1):43-49. doi: 10.1002/ppul.23897. Epub 2017 Oct 24.


Comparison of FEV1 reference equations for evaluating a cystic fibrosis therapeutic intervention.

Konstan MW, Wagener JS, VanDevanter DR, Pasta DJ, Millar SJ, Morgan WJ; Scientific Advisory Group and the Investigators and Coordinators of the Epidemiologic Study of Cystic Fibrosis.

Pediatr Pulmonol. 2017 Aug;52(8):1013-1019. doi: 10.1002/ppul.23751. Epub 2017 Jul 3.


Patients with Cystic Fibrosis and a G551D or Homozygous F508del Mutation: Similar Lung Function Decline.

Sawicki GS, McKone EF, Millar SJ, Pasta DJ, Konstan MW, Lubarsky B, Wagener JS.

Am J Respir Crit Care Med. 2017 Jun 15;195(12):1673-1676. doi: 10.1164/rccm.201608-1678LE. No abstract available.


Risk factors for mortality before age 18 years in cystic fibrosis.

McColley SA, Schechter MS, Morgan WJ, Pasta DJ, Craib ML, Konstan MW.

Pediatr Pulmonol. 2017 Jul;52(7):909-915. doi: 10.1002/ppul.23715. Epub 2017 Apr 24.


Relationship of Antibiotic Treatment to Recovery after Acute FEV1 Decline in Children with Cystic Fibrosis.

Morgan WJ, Wagener JS, Pasta DJ, Millar SJ, VanDevanter DR, Konstan MW; Scientific Advisory Group, Investigators, and Coordinators of the Epidemiologic Study of Cystic Fibrosis.

Ann Am Thorac Soc. 2017 Jun;14(6):937-942. doi: 10.1513/AnnalsATS.201608-615OC.


Reply: Combining Clinical Trial and Patient Registry Data in Cystic Fibrosis: Who Should Be Compared?

Sawicki GS, Konstan MW; all the authors.

Am J Respir Crit Care Med. 2017 Feb 1;195(3):405-406. doi: 10.1164/rccm.201609-1762LE. No abstract available.


Assessment of safety and efficacy of long-term treatment with combination lumacaftor and ivacaftor therapy in patients with cystic fibrosis homozygous for the F508del-CFTR mutation (PROGRESS): a phase 3, extension study.

Konstan MW, McKone EF, Moss RB, Marigowda G, Tian S, Waltz D, Huang X, Lubarsky B, Rubin J, Millar SJ, Pasta DJ, Mayer-Hamblett N, Goss CH, Morgan W, Sawicki GS.

Lancet Respir Med. 2017 Feb;5(2):107-118. doi: 10.1016/S2213-2600(16)30427-1. Epub 2016 Dec 21.


BMI fails to identify poor nutritional status in stunted children with CF.

Konstan MW, Pasta DJ, Wagener JS, VanDevanter DR, Morgan WJ.

J Cyst Fibros. 2017 Jan;16(1):158-160. doi: 10.1016/j.jcf.2016.11.005.


Lumacaftor/Ivacaftor Treatment of Patients with Cystic Fibrosis Heterozygous for F508del-CFTR.

Rowe SM, McColley SA, Rietschel E, Li X, Bell SC, Konstan MW, Marigowda G, Waltz D, Boyle MP; VX09-809-102 Study Group.

Ann Am Thorac Soc. 2017 Feb;14(2):213-219. doi: 10.1513/AnnalsATS.201609-689OC.


Efficacy and safety of lumacaftor/ivacaftor combination therapy in patients with cystic fibrosis homozygous for Phe508del CFTR by pulmonary function subgroup: a pooled analysis.

Elborn JS, Ramsey BW, Boyle MP, Konstan MW, Huang X, Marigowda G, Waltz D, Wainwright CE; VX-809 TRAFFIC and TRANSPORT study groups.

Lancet Respir Med. 2016 Aug;4(8):617-626. doi: 10.1016/S2213-2600(16)30121-7. Epub 2016 Jun 10.


Probability of IV antibiotic retreatment within thirty days is associated with duration and location of IV antibiotic treatment for pulmonary exacerbation in cystic fibrosis.

VanDevanter DR, Flume PA, Morris N, Konstan MW.

J Cyst Fibros. 2016 Nov;15(6):783-790. doi: 10.1016/j.jcf.2016.04.005. Epub 2016 Apr 29.



Morgan WJ, Pasta DJ, Konstan MW.

J Pediatr. 2016 May;172:228-9. doi: 10.1016/j.jpeds.2016.01.036. Epub 2016 Feb 9. No abstract available.


Preliminary comparison of normalized T1 and non-contrast perfusion MRI assessments of regional lung disease in cystic fibrosis patients.

Donnola SB, Dasenbrook EC, Weaver D, Lu L, Gupta K, Prabhakaran A, Yu X, Chmiel JF, McBennett K, Konstan MW, Drumm ML, Flask CA.

J Cyst Fibros. 2017 Mar;16(2):283-290. doi: 10.1016/j.jcf.2015.11.009. Epub 2015 Dec 22.


One-year safety and efficacy of tobramycin powder for inhalation in patients with cystic fibrosis.

Konstan MW, Flume PA, Galeva I, Wan R, Debonnett LM, Maykut RJ, Angyalosi G.

Pediatr Pulmonol. 2016 Apr;51(4):372-8. doi: 10.1002/ppul.23358. Epub 2015 Dec 27.


IV-treated pulmonary exacerbations in the prior year: An important independent risk factor for future pulmonary exacerbation in cystic fibrosis.

VanDevanter DR, Morris NJ, Konstan MW.

J Cyst Fibros. 2016 May;15(3):372-9. doi: 10.1016/j.jcf.2015.10.006. Epub 2015 Oct 23.


Forced Expiratory Volume in 1 Second Variability Helps Identify Patients with Cystic Fibrosis at Risk of Greater Loss of Lung Function.

Morgan WJ, VanDevanter DR, Pasta DJ, Foreman AJ, Wagener JS, Konstan MW; Scientific Advisory Group; Investigators and Coordinators of Epidemiologic Study of Cystic Fibrosis.

J Pediatr. 2016 Feb;169:116-21.e2. doi: 10.1016/j.jpeds.2015.08.042. Epub 2015 Sep 19. Erratum in: J Pediatr. 2018 Jun;197:322.


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.


Sustained Benefit from ivacaftor demonstrated by combining clinical trial and cystic fibrosis patient registry data.

Sawicki GS, McKone EF, Pasta DJ, Millar SJ, Wagener JS, Johnson CA, Konstan MW.

Am J Respir Crit Care Med. 2015 Oct 1;192(7):836-42. doi: 10.1164/rccm.201503-0578OC. Erratum in: Am J Respir Crit Care Med. 2016 Jun 1;193(11):1317-20.


Decline in lung function does not predict future decline in lung function in cystic fibrosis patients.

Rosenfeld M, VanDevanter DR, Ren CL, Elkin EP, Pasta DJ, Konstan MW, Morgan WJ; Investigators of Coordinators of the Epidemiologic Study of Cystic Fibrosis.

Pediatr Pulmonol. 2015 Sep;50(9):856-62. doi: 10.1002/ppul.23227. Epub 2015 Jun 18.


Lumacaftor-Ivacaftor in Patients with Cystic Fibrosis Homozygous for Phe508del CFTR.

Wainwright CE, Elborn JS, Ramsey BW, Marigowda G, Huang X, Cipolli M, Colombo C, Davies JC, De Boeck K, Flume PA, Konstan MW, McColley SA, McCoy K, McKone EF, Munck A, Ratjen F, Rowe SM, Waltz D, Boyle MP; TRAFFIC Study Group; TRANSPORT Study Group.

N Engl J Med. 2015 Jul 16;373(3):220-31. doi: 10.1056/NEJMoa1409547. Epub 2015 May 17.


Behavioral and nutritional treatment for preschool-aged children with cystic fibrosis: a randomized clinical trial.

Powers SW, Stark LJ, Chamberlin LA, Filigno SS, Sullivan SM, Lemanek KL, Butcher JL, Driscoll KA, Daines CL, Brody AS, Schindler T, Konstan MW, McCoy KS, Nasr SZ, Castile RG, Acton JD, Wooldridge JL, Ksenich RA, Szczesniak RD, Rausch JR, Stallings VA, Zemel BS, Clancy JP.

JAMA Pediatr. 2015 May;169(5):e150636. doi: 10.1001/jamapediatrics.2015.0636. Epub 2015 May 4.


Randomized trial of efficacy and safety of dornase alfa delivered by eRapid nebulizer in cystic fibrosis patients.

Sawicki GS, Chou W, Raimundo K, Trzaskoma B, Konstan MW.

J Cyst Fibros. 2015 Nov;14(6):777-83. doi: 10.1016/j.jcf.2015.04.003. Epub 2015 Apr 25.


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.


Inflammation in cystic fibrosis lung disease: Pathogenesis and therapy.

Cantin AM, Hartl D, Konstan MW, Chmiel JF.

J Cyst Fibros. 2015 Jul;14(4):419-30. doi: 10.1016/j.jcf.2015.03.003. Epub 2015 Mar 23. Review.


Efficacy response in CF patients treated with ivacaftor: post-hoc analysis.

Konstan MW, Plant BJ, Elborn JS, Rodriguez S, Munck A, Ahrens R, Johnson C.

Pediatr Pulmonol. 2015 May;50(5):447-55. doi: 10.1002/ppul.23173. Epub 2015 Mar 9.


Treatment and demographic factors affecting time to next pulmonary exacerbation in cystic fibrosis.

VanDevanter DR, Pasta DJ, Konstan MW.

J Cyst Fibros. 2015 Nov;14(6):763-9. doi: 10.1016/j.jcf.2015.02.007. Epub 2015 Mar 6.


Antibiotic treatment of signs and symptoms of pulmonary exacerbations: a comparison by care site.

Schechter MS, Regelmann WE, Sawicki GS, Rasouliyan L, VanDevanter DR, Rosenfeld M, Pasta D, Morgan W, Konstan MW.

Pediatr Pulmonol. 2015 May;50(5):431-40. doi: 10.1002/ppul.23147. Epub 2014 Dec 19.


Pulmonary function outcomes for assessing cystic fibrosis care.

Wagener JS, Elkin EP, Pasta DJ, Schechter MS, Konstan MW, Morgan WJ; Investigators and Coordinators of the Epidemiologic Study of Cystic Fibrosis.

J Cyst Fibros. 2015 May;14(3):376-83. doi: 10.1016/j.jcf.2014.11.008. Epub 2014 Dec 9.


Association between the introduction of a new cystic fibrosis inhaled antibiotic class and change in prevalence of patients receiving multiple inhaled antibiotic classes.

Dasenbrook EC, Konstan MW, VanDevanter DR.

J Cyst Fibros. 2015 May;14(3):370-5. doi: 10.1016/j.jcf.2014.11.005. Epub 2014 Dec 11.


Safety, tolerability, and plasma exposure of tiotropium Respimat® in children and adults with cystic fibrosis.

Konstan MW, Sharma A, Moroni-Zentgraf P, Wang F, Koker P.

J Aerosol Med Pulm Drug Deliv. 2015 Apr;28(2):137-44. doi: 10.1089/jamp.2013.1115. Epub 2014 Nov 7.


Long-term safety and efficacy of ivacaftor in patients with cystic fibrosis who have the Gly551Asp-CFTR mutation: a phase 3, open-label extension study (PERSIST).

McKone EF, Borowitz D, Drevinek P, Griese M, Konstan MW, Wainwright C, Ratjen F, Sermet-Gaudelus I, Plant B, Munck A, Jiang Y, Gilmartin G, Davies JC; VX08-770-105 (PERSIST) Study Group.

Lancet Respir Med. 2014 Nov;2(11):902-910. doi: 10.1016/S2213-2600(14)70218-8. Epub 2014 Oct 9.


Pooled analysis of tiotropium Respimat(®) pharmacokinetics in cystic fibrosis.

Sharma A, Geller DE, Moroni-Zentgraf P, Kattenbeck S, Schmid M, Boland K, Rapp B, Konstan MW, Ratjen F, Elborn JS, Koker P.

Pulm Pharmacol Ther. 2014 Dec;29(2):217-23. doi: 10.1016/j.pupt.2014.08.004. Epub 2014 Aug 24.


Improvements in lung function and height among cohorts of 6-year-olds with cystic fibrosis from 1994 to 2012.

VanDevanter DR, Pasta DJ, Konstan MW.

J Pediatr. 2014 Dec;165(6):1091-1097.e2. doi: 10.1016/j.jpeds.2014.06.061. Epub 2014 Aug 16.


Study design considerations for evaluating the efficacy and safety of pancreatic enzyme replacement therapy in patients with cystic fibrosis.

Konstan MW, Borowitz D, Mayer-Hamblett N, Milla C, Hendeles L, Murray S, Kronmal RA, Casey S, Rose LM, Morgan WJ, Ramsey BW.

Clin Investig (Lond). 2013 Aug;3(8):731-741.


A CFTR corrector (lumacaftor) and a CFTR potentiator (ivacaftor) for treatment of patients with cystic fibrosis who have a phe508del CFTR mutation: a phase 2 randomised controlled trial.

Boyle MP, Bell SC, Konstan MW, McColley SA, Rowe SM, Rietschel E, Huang X, Waltz D, Patel NR, Rodman D; VX09-809-102 study group.

Lancet Respir Med. 2014 Jul;2(7):527-38. doi: 10.1016/S2213-2600(14)70132-8. Epub 2014 Jun 24.


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