Literature DB >> 34672432

Increasing life expectancy in cystic fibrosis: Advances and challenges.

Kimberly A McBennett1,2, Pamela B Davis1, Michael W Konstan1,2.   

Abstract

Since the first description of cystic fibrosis in 1938, there have been significant advances in both quality of life and longevity for people living with this disease. In this article we describe the milestones of the last 80 years and what we perceive to be the remaining barriers to normalcy for this population.
© 2021 Wiley Periodicals LLC.

Entities:  

Keywords:  CFTR modulators; cystic fibrosis; lung function; survival

Mesh：

Substances：

Year:  2021        PMID： 34672432      PMCID： PMC9004282          DOI： 10.1002/ppul.25733

Source DB:  PubMed          Journal:  Pediatr Pulmonol        ISSN： 1099-0496


  76 in total

1.  Complications associated with symptomatic diagnosis in infants with cystic fibrosis.

Authors:  Frank J Accurso; Marci K Sontag; Jeffrey S Wagener
Journal:  J Pediatr       Date:  2005-09       Impact factor: 4.406

2.  Cystic Fibrosis Diagnostic Challenges over 4 Decades: Historical Perspectives and Lessons Learned.

Authors:  Philip M Farrell; Terry B White; Nico Derichs; Carlo Castellani; Beryl J Rosenstein
Journal:  J Pediatr       Date:  2017-02       Impact factor: 4.406

Review 3.  Liver disease in patients with cystic fibrosis.

Authors:  Natasha Kamal; Pallavi Surana; Christopher Koh
Journal:  Curr Opin Gastroenterol       Date:  2018-05       Impact factor: 3.287

4.  Long-term safety and efficacy of tezacaftor-ivacaftor in individuals with cystic fibrosis aged 12 years or older who are homozygous or heterozygous for Phe508del CFTR (EXTEND): an open-label extension study.

Authors:  Patrick A Flume; Reta Fischer Biner; Damian G Downey; Cynthia Brown; Manu Jain; Rainald Fischer; Kris De Boeck; Gregory S Sawicki; Philip Chang; Hildegarde Paz-Diaz; Jaime L Rubin; Yoojung Yang; Xingdi Hu; David J Pasta; Stefanie J Millar; Daniel Campbell; Xin Wang; Neil Ahluwalia; Caroline A Owen; Claire E Wainwright
Journal:  Lancet Respir Med       Date:  2021-02-10       Impact factor: 30.700

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

Authors:  Claire E Wainwright; J Stuart Elborn; Bonnie W Ramsey; Gautham Marigowda; Xiaohong Huang; Marco Cipolli; Carla Colombo; Jane C Davies; Kris De Boeck; Patrick A Flume; Michael W Konstan; Susanna A McColley; Karen McCoy; Edward F McKone; Anne Munck; Felix Ratjen; Steven M Rowe; David Waltz; Michael P Boyle
Journal:  N Engl J Med       Date:  2015-05-17       Impact factor: 91.245

6.  Outcomes of intensive care unit care in adults with cystic fibrosis.

Authors:  N Sood; L J Paradowski; J R Yankaskas
Journal:  Am J Respir Crit Care Med       Date:  2001-02       Impact factor: 21.405

Review 7.  Update on fat-soluble vitamins in cystic fibrosis.

Authors:  Asim Maqbool; Virginia A Stallings
Journal:  Curr Opin Pulm Med       Date:  2008-11       Impact factor: 3.155

8.  Lung transplantation for cystic fibrosis.

Authors:  Jonathan C Yeung; Tiago N Machuca; Cecilia Chaparro; Marcelo Cypel; Anne L Stephenson; Melinda Solomon; Tomohito Saito; Matthew Binnie; Chung-Wai Chow; Hartmut Grasemann; Andrew F Pierre; Kazuhiro Yasufuku; Marc de Perrot; Laura L Donahoe; Jussi Tikkanen; Tereza Martinu; Thomas K Waddell; Elizabeth Tullis; Lianne G Singer; Shaf Keshavjee
Journal:  J Heart Lung Transplant       Date:  2020-02-20       Impact factor: 10.247

9.  Disparities in Mortality of Hispanic Patients with Cystic Fibrosis in the United States. A National and Regional Cohort Study.

Authors:  Jason Rho; Chul Ahn; Ang Gao; Gregory S Sawicki; Ashley Keller; Raksha Jain
Journal:  Am J Respir Crit Care Med       Date:  2018-10-15       Impact factor: 21.405

10.  Critical illness among adults with cystic fibrosis in Texas, 2004-2013: Patterns of ICU utilization, characteristics, and outcomes.

Authors:  Lavi Oud
Journal:  PLoS One       Date:  2017-10-24       Impact factor: 3.240
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  1 in total

1.  Treatment of Polarized Cystic Fibrosis Airway Cells With HGF Prevents VX-661-Rescued F508del-CFTR Destabilization Caused by Prolonged Co-exposure to VX-770.

Authors:  Ana M Matos; Peter Jordan; Paulo Matos
Journal:  Front Mol Biosci       Date:  2021-12-22
  1 in total

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