Literature DB >> 22675793

Latest in cystic fibrosis.

Ravi P Nayak1.   

Abstract

Cystic Fibrosis (CF) is a genetic disease affecting multiple organs. There are about 30,000 patients with CF in the United States, resulting from mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene, as well as its protein product. The life expectancy of CF patients has increased steadily over recent years, with the current expectation being for them to live into their late 30s. This is due to increased understanding, and therapeutic advances in the CF treatment armamentarium.

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Year:  2012        PMID: 22675793      PMCID: PMC6181745     

Source DB:  PubMed          Journal:  Mo Med        ISSN: 0026-6620


  24 in total

1.  The Registry of the International Society for Heart and Lung Transplantation: Twenty-eighth Adult Lung and Heart-Lung Transplant Report--2011.

Authors:  Jason D Christie; Leah B Edwards; Anna Y Kucheryavaya; Christian Benden; Fabienne Dobbels; Richard Kirk; Axel O Rahmel; Josef Stehlik; Marshall I Hertz
Journal:  J Heart Lung Transplant       Date:  2011-10       Impact factor: 10.247

2.  Genetic modifiers of lung disease in cystic fibrosis.

Authors:  Mitchell L Drumm; Michael W Konstan; Mark D Schluchter; Allison Handler; Rhonda Pace; Fei Zou; Maimoona Zariwala; David Fargo; Airong Xu; John M Dunn; Rebecca J Darrah; Ruslan Dorfman; Andrew J Sandford; Mary Corey; Julian Zielenski; Peter Durie; Katrina Goddard; James R Yankaskas; Fred A Wright; Michael R Knowles
Journal:  N Engl J Med       Date:  2005-10-06       Impact factor: 91.245

3.  A controlled trial of long-term inhaled hypertonic saline in patients with cystic fibrosis.

Authors:  Mark R Elkins; Michael Robinson; Barbara R Rose; Colin Harbour; Carmel P Moriarty; Guy B Marks; Elena G Belousova; Wei Xuan; Peter T P Bye
Journal:  N Engl J Med       Date:  2006-01-19       Impact factor: 91.245

4.  Failure to recover to baseline pulmonary function after cystic fibrosis pulmonary exacerbation.

Authors:  Don B Sanders; Rachel C L Bittner; Margaret Rosenfeld; Lucas R Hoffman; Gregory J Redding; Christopher H Goss
Journal:  Am J Respir Crit Care Med       Date:  2010-05-12       Impact factor: 21.405

5.  Cystic fibrosis pulmonary guidelines: airway clearance therapies.

Authors:  Patrick A Flume; Karen A Robinson; Brian P O'Sullivan; Jonathan D Finder; Robert L Vender; Donna-Beth Willey-Courand; Terry B White; Bruce C Marshall
Journal:  Respir Care       Date:  2009-04       Impact factor: 2.258

6.  Inhaled aztreonam lysine for chronic airway Pseudomonas aeruginosa in cystic fibrosis.

Authors:  Karen S McCoy; Alexandra L Quittner; Christopher M Oermann; Ronald L Gibson; George Z Retsch-Bogart; A Bruce Montgomery
Journal:  Am J Respir Crit Care Med       Date:  2008-07-24       Impact factor: 21.405

Review 7.  Cystic fibrosis pulmonary guidelines: treatment of pulmonary exacerbations.

Authors:  Patrick A Flume; Peter J Mogayzel; Karen A Robinson; Christopher H Goss; Randall L Rosenblatt; Robert J Kuhn; Bruce C Marshall
Journal:  Am J Respir Crit Care Med       Date:  2009-09-03       Impact factor: 21.405

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

Authors:  Frank J Accurso; Steven M Rowe; J 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 Z Uluer; Moira L Aitken; Steven D Freedman; Lynn M Rose; Nicole Mayer-Hamblett; Qunming Dong; Jiuhong Zha; Anne J Stone; Eric R Olson; Claudia L Ordoñez; Preston W Campbell; Melissa A Ashlock; Bonnie W Ramsey
Journal:  N Engl J Med       Date:  2010-11-18       Impact factor: 176.079

9.  Genome-wide association and linkage identify modifier loci of lung disease severity in cystic fibrosis at 11p13 and 20q13.2.

Authors:  Fred A Wright; Lisa J Strug; Vishal K Doshi; Clayton W Commander; Scott M Blackman; Lei Sun; Yves Berthiaume; David Cutler; Andreea Cojocaru; J Michael Collaco; Mary Corey; Ruslan Dorfman; Katrina Goddard; Deanna Green; Jack W Kent; Ethan M Lange; Seunggeun Lee; Weili Li; Jingchun Luo; Gregory M Mayhew; Kathleen M Naughton; Rhonda G Pace; Peter Paré; Johanna M Rommens; Andrew Sandford; Jaclyn R Stonebraker; Wei Sun; Chelsea Taylor; Lori L Vanscoy; Fei Zou; John Blangero; Julian Zielenski; Wanda K O'Neal; Mitchell L Drumm; Peter R Durie; Michael R Knowles; Garry R Cutting
Journal:  Nat Genet       Date:  2011-05-22       Impact factor: 38.330

Review 10.  Evidence-based practice recommendations for nutrition-related management of children and adults with cystic fibrosis and pancreatic insufficiency: results of a systematic review.

Authors:  Virginia A Stallings; Lori J Stark; Karen A Robinson; Andrew P Feranchak; Hebe Quinton
Journal:  J Am Diet Assoc       Date:  2008-05
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