Literature DB >> 29256624

Ivacaftor-treated Patients with Cystic Fibrosis Derive Long-Term Benefit Despite No Short-Term Clinical Improvement.

Sonya L Heltshe1,2, Steven M Rowe3, Michelle Skalland2, Arthur Baines2, Manu Jain4.   

Abstract

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Year:  2018        PMID： 29256624      PMCID： PMC6005553          DOI： 10.1164/rccm.201710-2046LE

Source DB:  PubMed          Journal:  Am J Respir Crit Care Med        ISSN： 1073-449X            Impact factor:   21.405


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  11 in total

1.  Retrospective observational study of French patients with cystic fibrosis and a Gly551Asp-CFTR mutation after 1 and 2years of treatment with ivacaftor in a real-world setting.

Authors:  Dominique Hubert; Clémence Dehillotte; Anne Munck; Valérie David; Jinmi Baek; Laurent Mely; Stéphane Dominique; Sophie Ramel; Isabelle Danner Boucher; Sylvaine Lefeuvre; Quitterie Reynaud; Virginie Colomb-Jung; Prissile Bakouboula; Lydie Lemonnier
Journal:  J Cyst Fibros       Date:  2017-07-12       Impact factor: 5.482

2.  Efficacy and safety of ivacaftor in patients with cystic fibrosis and a non-G551D gating mutation.

Authors:  Kris De Boeck; Anne Munck; Seth Walker; Albert Faro; Peter Hiatt; Geoffrey Gilmartin; Mark Higgins
Journal:  J Cyst Fibros       Date:  2014-09-26       Impact factor: 5.482

3.  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).

Authors:  Edward F McKone; Drucy Borowitz; Pavel Drevinek; Matthias Griese; Michael W Konstan; Claire Wainwright; Felix Ratjen; Isabelle Sermet-Gaudelus; Barry Plant; Anne Munck; Ying Jiang; Geoffrey Gilmartin; Jane C Davies
Journal:  Lancet Respir Med       Date:  2014-10-09       Impact factor: 30.700

4.  Clinical mechanism of the cystic fibrosis transmembrane conductance regulator potentiator ivacaftor in G551D-mediated cystic fibrosis.

Authors:  Steven M Rowe; Sonya L Heltshe; Tanja Gonska; Scott H Donaldson; Drucy Borowitz; Daniel Gelfond; Scott D Sagel; Umer Khan; Nicole Mayer-Hamblett; Jill M Van Dalfsen; Elizabeth Joseloff; Bonnie W Ramsey
Journal:  Am J Respir Crit Care Med       Date:  2014-07-15       Impact factor: 21.405

5.  A CFTR potentiator in patients with cystic fibrosis and the G551D mutation.

Authors:  Bonnie W Ramsey; Jane Davies; N Gerard McElvaney; Elizabeth Tullis; Scott C Bell; Pavel Dřevínek; Matthias Griese; Edward F McKone; Claire E Wainwright; Michael W Konstan; Richard Moss; Felix Ratjen; Isabelle Sermet-Gaudelus; Steven M Rowe; Qunming Dong; Sally Rodriguez; Karl Yen; Claudia Ordoñez; J Stuart Elborn
Journal:  N Engl J Med       Date:  2011-11-03       Impact factor: 91.245

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

Authors:  Gregory S Sawicki; Edward F McKone; David J Pasta; Stefanie J Millar; Jeffrey S Wagener; Charles A Johnson; Michael W Konstan
Journal:  Am J Respir Crit Care Med       Date:  2015-10-01       Impact factor: 21.405

7.  Pseudomonas aeruginosa in cystic fibrosis patients with G551D-CFTR treated with ivacaftor.

Authors:  Sonya L Heltshe; Nicole Mayer-Hamblett; Jane L Burns; Umer Khan; Arthur Baines; Bonnie W Ramsey; Steven M Rowe
Journal:  Clin Infect Dis       Date:  2014-11-25       Impact factor: 9.079

8.  Multi-ethnic reference values for spirometry for the 3-95-yr age range: the global lung function 2012 equations.

Authors:  Philip H Quanjer; Sanja Stanojevic; Tim J Cole; Xaver Baur; Graham L Hall; Bruce H Culver; Paul L Enright; John L Hankinson; Mary S M Ip; Jinping Zheng; Janet Stocks
Journal:  Eur Respir J       Date:  2012-06-27       Impact factor: 16.671

9.  The Cystic Fibrosis Foundation Patient Registry. Design and Methods of a National Observational Disease Registry.

Authors:  Emily A Knapp; Aliza K Fink; Christopher H Goss; Ase Sewall; Josh Ostrenga; Christopher Dowd; Alexander Elbert; Kristofer M Petren; Bruce C Marshall
Journal:  Ann Am Thorac Soc       Date:  2016-07

10.  Defining the disease liability of variants in the cystic fibrosis transmembrane conductance regulator gene.

Authors:  Patrick R Sosnay; Karen R Siklosi; Fredrick Van Goor; Kyle Kaniecki; Haihui Yu; Neeraj Sharma; Anabela S Ramalho; Margarida D Amaral; Ruslan Dorfman; Julian Zielenski; David L Masica; Rachel Karchin; Linda Millen; Philip J Thomas; George P Patrinos; Mary Corey; Michelle H Lewis; Johanna M Rommens; Carlo Castellani; Christopher M Penland; Garry R Cutting
Journal:  Nat Genet       Date:  2013-08-25       Impact factor: 38.330
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  9 in total

Review 1.  Update in Cystic Fibrosis 2018.

Authors:  Bonnie W Ramsey; Gregory P Downey; Christopher H Goss
Journal:  Am J Respir Crit Care Med       Date:  2019-05-15       Impact factor: 21.405

2.  Treatment With LAU-7b Complements CFTR Modulator Therapy by Improving Lung Physiology and Normalizing Lipid Imbalance Associated With CF Lung Disease.

Authors:  Amanda Centorame; Daciana Catalina Dumut; Mina Youssef; Martin Ondra; Irenej Kianicka; Juhi Shah; Radu Alexandru Paun; Tomas Ozdian; John W Hanrahan; Ekaterina Gusev; Basil Petrof; Marian Hajduch; Radu Pislariu; Juan Bautista De Sanctis; Danuta Radzioch
Journal:  Front Pharmacol       Date:  2022-05-20       Impact factor: 5.988

Review 3.  Cystic Fibrosis: Emergence of Highly Effective Targeted Therapeutics and Potential Clinical Implications.

Authors:  Marcus A Mall; Nicole Mayer-Hamblett; Steven M Rowe
Journal:  Am J Respir Crit Care Med       Date:  2020-05-15       Impact factor: 21.405

4.  Rapid cystic fibrosis lung-function decline and in-vitro CFTR modulation.

Authors:  Emrah Gecili; Weiji Su; Cole Brokamp; Eleni-Rosalina Andrinopoulou; Francis J LaRosa Iii; Teresa Pestian; John P Clancy; George M Solomon; John J Brewington; Rhonda D Szczesniak
Journal:  J Cyst Fibros       Date:  2021-05-03       Impact factor: 5.527

5.  Ivacaftor partially corrects airway inflammation in a humanized G551D rat.

Authors:  Morgan Green; Natalie Lindgren; Alexander Henderson; Johnathan D Keith; Ashley M Oden; Susan E Birket
Journal:  Am J Physiol Lung Cell Mol Physiol       Date:  2021-04-07       Impact factor: 6.011

6.  Cystic Fibrosis: A Disease in Transformation, yet More Work to Be Done!

Authors:  Bonnie W Ramsey; Scott C Bell
Journal:  Am J Respir Crit Care Med       Date:  2022-03-01       Impact factor: 30.528

7.  Clinical Effectiveness of Lumacaftor/Ivacaftor in Patients with Cystic Fibrosis Homozygous for F508del-CFTR. A Clinical Trial.

Authors:  Scott D Sagel; Umer Khan; Sonya L Heltshe; John P Clancy; Drucy Borowitz; Daniel Gelfond; Scott H Donaldson; Antoinette Moran; Felix Ratjen; Jill M VanDalfsen; Steven M Rowe
Journal:  Ann Am Thorac Soc       Date:  2021-01

8.  Long term clinical effectiveness of ivacaftor in people with the G551D CFTR mutation.

Authors:  J S Guimbellot; A Baines; A Paynter; S L Heltshe; J VanDalfsen; M Jain; S M Rowe; S D Sagel
Journal:  J Cyst Fibros       Date:  2020-11-25       Impact factor: 5.482

Review 9.  A new era for people with cystic fibrosis.

Authors:  Marlou C Bierlaagh; Danya Muilwijk; Jeffrey M Beekman; Cornelis K van der Ent
Journal:  Eur J Pediatr       Date:  2021-07-02       Impact factor: 3.183
  9 in total

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