2014
DOI: 10.1159/000357646
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Update on Key Emerging Challenges in Cystic Fibrosis

Abstract: Cystic fibrosis (CF) is a multisystem disease causing severe chronic sinopulmonary disease and loss of pancreatic exocrine function, which affects approximately 70,000 individuals worldwide. New therapeutic developments over the last few decades have resulted in a significant increase in survival, with the median predicted survival now reaching the late thirties and more and more CF patients living well into adulthood. However, with this advent of new therapies and the associated increase in survival, new chal… Show more

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Cited by 2,681 publications
(24 citation statements)
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References 89 publications
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“…In this meta-analysis, we found that the prevalence of MRSA infection has climbed up over years. This increase is in line with previous reviews done by LiPuma, Kahl and Jennings et al [3,10,43]. They reported that MRSA infection among CF population has demonstrated a notable increase in prevalence over the last decades.…”
Section: Resultssupporting
confidence: 91%
“…In this meta-analysis, we found that the prevalence of MRSA infection has climbed up over years. This increase is in line with previous reviews done by LiPuma, Kahl and Jennings et al [3,10,43]. They reported that MRSA infection among CF population has demonstrated a notable increase in prevalence over the last decades.…”
Section: Resultssupporting
confidence: 91%
“…CF is the most lethal inherited disease in the Caucasian population characterized by chronic airway inflammation (Jennings et al, 2014). The disease is caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene (Kerem et al, 1989), which encodes for a protein that, among different functions, includes the cAMPdependent chloride channel.…”
Section: Introductionmentioning
confidence: 99%
“…7,8 Complicated and time-consuming treatment regimens, which may result in low adherence to medications, are a challenge in the management of patients with CF. 9 Low adherence to pulmonary medications in patients with CF is associated with more frequent pulmonary exacerbations 10 and more CF-related hospitalizations and higher health-care costs, 11 all of which may result in reduced health care-related quality of life. 12 In addition to pulmonary medications, more than 80% of patients with CF younger than 40 years are prescribed pancreatic enzyme replacement therapy (PERT) to treat EPI.…”
Section: Introductionmentioning
confidence: 99%