M
Michael W. Konstan
Researcher at Case Western Reserve University
Publications - 244
Citations - 21189
Michael W. Konstan is an academic researcher from Case Western Reserve University. The author has contributed to research in topics: Cystic fibrosis & Ivacaftor. The author has an hindex of 50, co-authored 236 publications receiving 19099 citations. Previous affiliations of Michael W. Konstan include Boston Children's Hospital.
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Journal ArticleDOI
A CFTR potentiator in patients with cystic fibrosis and the G551D mutation
Bonnie W. Ramsey,Jane C. Davies,N. Gerard McElvaney,Elizabeth Tullis,Scott C. Bell,Pavel Dř evínek,Matthias Griese,Edward F. McKone,Claire E. Wainwright,Michael W. Konstan,Richard B. Moss,Felix Ratjen,Isabelle Sermet-Gaudelus,Steven M. Rowe,Qunming Dong,Sally Rodriguez,Karl Yen,Claudia L. Ordoñez,J. Stuart Elborn +18 more
TL;DR: Ivacaftor was associated with improvements in lung function at 2 weeks that were sustained through 48 weeks and substantial improvements were also observed in the risk of pulmonary exacerbations, patient-reported respiratory symptoms, weight, and concentration of sweat chloride.
Journal ArticleDOI
Effect of VX-770 in Persons with Cystic Fibrosis and the G551D-CFTR Mutation
Frank J. Accurso,Steven M. Rowe,John P. Clancy,Michael P. Boyle,Jordan M. Dunitz,Peter R. Durie,Scott D. Sagel,Douglas B. Hornick,Michael W. Konstan,Scott H. Donaldson,Richard B. Moss,Joseph M. Pilewski,Ronald C. Rubenstein,Ahmet Uluer,Moira L. Aitken,Steven D. Freedman,Lynn M. Rose,Nicole Mayer-Hamblett,Qunming Dong,Jiuhong Zha,Anne Stone,Eric R. Olson,Claudia L. Ordoñez,Preston W. Campbell,Melissa A. Ashlock,Bonnie W. Ramsey +25 more
TL;DR: This study showed that VX-770 was associated with within-subject improvements in CFTR and lung function and provides support for further studies of pharmacologic potentiation of CFTR as a means to treat cystic fibrosis.
Journal ArticleDOI
Effect of high-dose ibuprofen in patients with cystic fibrosis.
TL;DR: In patients with cystic fibrosis and mild lung disease, high-dose ibuprofen, taken consistently for four years, significantly slows the progression of the lung disease without serious adverse effects.
Journal ArticleDOI
Inflammatory cytokines in cystic fibrosis lungs.
Tracey L. Bonfield,J R Panuska,Michael W. Konstan,Kathleen A. Hilliard,Jay B. Hilliard,H Ghnaim,Melvin Berger +6 more
TL;DR: Enhanced macrophage production of proinflammatory cytokines and decreased production of the regulatory molecule IL-10 may have important roles in the pathogenesis of CF lung disease.
Journal ArticleDOI
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
John P. Clancy,Steven M. Rowe,Frank J. Accurso,Moira L. Aitken,Raouf S. Amin,Melissa A. Ashlock,Manfred Ballmann,Michael P. Boyle,Inez Bronsveld,Preston W. Campbell,Kris De Boeck,Scott H. Donaldson,Henry L. Dorkin,Jordan M. Dunitz,Peter R. Durie,Manu Jain,Anissa Leonard,Karen McCoy,Richard B. Moss,Joseph M. Pilewski,Daniel B. Rosenbluth,Ronald C. Rubenstein,Michael S. Schechter,Martyn Botfield,Claudia L. Ordoñez,George T. Spencer-Green,Laurent Vernillet,Steve Wisseh,Karl Yen,Michael W. Konstan +29 more
TL;DR: In this study, VX-809 had a similar adverse event profile to placebo for 28 days in F508del-CFTR homozygous patients, and demonstrated biological activity with positive impact on CFTR function in the sweat gland.