Literature DB >> 29614238

Changes in Lung Clearance Index in Preschool-aged Patients with Cystic Fibrosis Treated with Ivacaftor (GOAL): A Clinical Trial.

Felix Ratjen1, Michelle Klingel1, Philip Black2, Michael R Powers3, Hartmut Grasemann1, Melinda Solomon1, Scott D Sagel4, Scott H Donaldson5, Steven M Rowe6, Margaret Rosenfeld7.   

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Year:  2018        PMID: 29614238      PMCID: PMC6835087          DOI: 10.1164/rccm.201802-0243LE

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.  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

2.  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

3.  Lung clearance index as an outcome measure for clinical trials in young children with cystic fibrosis. A pilot study using inhaled hypertonic saline.

Authors:  Padmaja Subbarao; Sanja Stanojevic; Meghan Brown; Renee Jensen; Margaret Rosenfeld; Stephanie Davis; Lyndia Brumback; Per Gustafsson; Felix Ratjen
Journal:  Am J Respir Crit Care Med       Date:  2013-08-15       Impact factor: 21.405

Review 4.  Early Lung Disease in Infants and Preschool Children with Cystic Fibrosis. What Have We Learned and What Should We Do about It?

Authors:  Sarath C Ranganathan; Graham L Hall; Peter D Sly; Stephen M Stick; Tonia A Douglas
Journal:  Am J Respir Crit Care Med       Date:  2017-06-15       Impact factor: 21.405

5.  Assessment of clinical response to ivacaftor with lung clearance index in cystic fibrosis patients with a G551D-CFTR mutation and preserved spirometry: a randomised controlled trial.

Authors:  Jane Davies; Helen Sheridan; Nicholas Bell; Steve Cunningham; Stephanie D Davis; J Stuart Elborn; Carlos E Milla; Timothy D Starner; Daniel J Weiner; Po-Shun Lee; Felix Ratjen
Journal:  Lancet Respir Med       Date:  2013-09-10       Impact factor: 30.700

6.  Progression of Lung Disease in Preschool Patients with Cystic Fibrosis.

Authors:  Sanja Stanojevic; Stephanie D Davis; George Retsch-Bogart; Hailey Webster; Miriam Davis; Robin C Johnson; Renee Jensen; Maria Ester Pizarro; Mica Kane; Charles C Clem; Leah Schornick; Padmaja Subbarao; Felix A Ratjen
Journal:  Am J Respir Crit Care Med       Date:  2017-05-01       Impact factor: 21.405

7.  Efficacy and safety of ivacaftor in patients aged 6 to 11 years with cystic fibrosis with a G551D mutation.

Authors:  Jane C Davies; Claire E Wainwright; Gerard J Canny; Mark A Chilvers; Michelle S Howenstine; Anne Munck; Jochen G Mainz; Sally Rodriguez; Haihong Li; Karl Yen; Claudia L Ordoñez; Richard Ahrens
Journal:  Am J Respir Crit Care Med       Date:  2013-06-01       Impact factor: 21.405

8.  Safety, pharmacokinetics, and pharmacodynamics of ivacaftor in patients aged 2-5 years with cystic fibrosis and a CFTR gating mutation (KIWI): an open-label, single-arm study.

Authors:  Jane C Davies; Steve Cunningham; William T Harris; Allen Lapey; Warren E Regelmann; Gregory S Sawicki; Kevin W Southern; Sarah Robertson; Yulia Green; Jon Cooke; Margaret Rosenfeld
Journal:  Lancet Respir Med       Date:  2016-01-21       Impact factor: 30.700

9.  Preschool Multiple-Breath Washout Testing. An Official American Thoracic Society Technical Statement.

Authors:  Paul D Robinson; Philipp Latzin; Kathryn A Ramsey; Sanja Stanojevic; Paul Aurora; Stephanie D Davis; Monika Gappa; Graham L Hall; Alex Horsley; Renee Jensen; Sooky Lum; Carlos Milla; Kim G Nielsen; Jessica E Pittman; Margaret Rosenfeld; Florian Singer; Padmaja Subbarao; Per M Gustafsson; Felix Ratjen
Journal:  Am J Respir Crit Care Med       Date:  2018-03-01       Impact factor: 21.405

10.  Inter-test reproducibility of the lung clearance index measured by multiple breath washout.

Authors:  Esther Oude Engberink; Felix Ratjen; Stephanie D Davis; George Retsch-Bogart; Reshma Amin; Sanja Stanojevic
Journal:  Eur Respir J       Date:  2017-10-05       Impact factor: 16.671

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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.  Ivacaftor in Omani children with cystic fibrosis caused by p.Ser549Arg CFTR mutation.

Authors:  Sumaya Al Oraimi; Hussain Mohsin; Zainab Al Musawi; Younis Al Balushi; Khoula Al Shidhani; Qasem Al Salmi
Journal:  Int J Pediatr Adolesc Med       Date:  2021-11-29

3.  A screening tool to identify risk for bronchiectasis progression in children with cystic fibrosis.

Authors:  Daan Caudri; Lidija Turkovic; Nicholas H de Klerk; Tim Rosenow; Conor P Murray; Ewout W Steyerberg; Sarath C Ranganathan; Peter Sly; Stephen M Stick; Oded Breuer
Journal:  Pediatr Pulmonol       Date:  2021-10-12

Review 4.  Progression of Cystic Fibrosis Lung Disease from Childhood to Adulthood: Neutrophils, Neutrophil Extracellular Trap (NET) Formation, and NET Degradation.

Authors:  Meraj A Khan; Zubair Sabz Ali; Neil Sweezey; Hartmut Grasemann; Nades Palaniyar
Journal:  Genes (Basel)       Date:  2019-02-26       Impact factor: 4.096

Review 5.  Early Diagnosis and Intervention in Cystic Fibrosis: Imagining the Unimaginable.

Authors:  Andrea M Coverstone; Thomas W Ferkol
Journal:  Front Pediatr       Date:  2021-01-11       Impact factor: 3.418

6.  Clinical Effectiveness of Elexacaftor/Tezacaftor/Ivacaftor in People with Cystic Fibrosis: A Clinical Trial.

Authors:  David P Nichols; Alex C Paynter; Sonya L Heltshe; Scott H Donaldson; Carla A Frederick; Steven D Freedman; Daniel Gelfond; Lucas R Hoffman; Andrea Kelly; Michael R Narkewicz; Jessica E Pittman; Felix Ratjen; Margaret Rosenfeld; Scott D Sagel; Sarah Jane Schwarzenberg; Pradeep K Singh; George M Solomon; Michael S Stalvey; John P Clancy; Shannon Kirby; Jill M Van Dalfsen; Margaret H Kloster; Steven M Rowe
Journal:  Am J Respir Crit Care Med       Date:  2022-03-01       Impact factor: 30.528

Review 7.  Dornase alfa in Cystic Fibrosis: indications, comparative studies and effects on lung clearance index.

Authors:  Vito Terlizzi; Chiara Castellani; Giovanni Taccetti; Beatrice Ferrari
Journal:  Ital J Pediatr       Date:  2022-08-04       Impact factor: 3.288

8.  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

Review 9.  New Therapies to Correct the Cystic Fibrosis Basic Defect.

Authors:  Christelle Bergeron; André M Cantin
Journal:  Int J Mol Sci       Date:  2021-06-08       Impact factor: 5.923

  9 in total

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