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Lumacaftor-Ivacaftor in Patients with Cystic Fibrosis Homozygous for Phe508del CFTR.
BACKGROUND Cystic fibrosis is a life-limiting disease that is caused by defective or deficient cystic fibrosis transmembrane conductance regulator (CFTR) protein activity. Phe508del is the mostExpand
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Cystic fibrosis pulmonary guidelines: chronic medications for maintenance of lung health.
RATIONALE Cystic fibrosis is a recessive genetic disease characterized by dehydration of the airway surface liquid and impaired mucociliary clearance. As a result, individuals with the disease haveExpand
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Safety, efficacy and convenience of tobramycin inhalation powder in cystic fibrosis patients: The EAGER trial.
BACKGROUND A light-porous-particle, dry-powder formulation of tobramycin was developed, using PulmoSphere® technology, to improve airway delivery efficiency, substantially reduce delivery time, andExpand
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European Cystic Fibrosis Society Standards of Care: Best Practice guidelines.
Specialised CF care has led to a dramatic improvement in survival in CF: in the last four decades, well above what was seen in the general population over the same period. With the implementation ofExpand
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Cystic fibrosis pulmonary guidelines: treatment of pulmonary exacerbations.
The natural history of cystic fibrosis lung disease is one of chronic progression with intermittent episodes of acute worsening of symptoms frequently called acute pulmonary exacerbations TheseExpand
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Treatment of lung infection in patients with cystic fibrosis: current and future strategies.
In patients with cystic fibrosis (CF) lung damage secondary to chronic infection is the main cause of death. Treatment of lung disease to reduce the impact of infection, inflammation and subsequentExpand
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ECFS best practice guidelines: the 2018 revision.
Developments in managing CF continue to drive dramatic improvements in survival. As newborn screening rolls-out across Europe, CF centres are increasingly caring for cohorts of patients who haveExpand
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Pulmonary complications after esophagectomy.
BACKGROUND Pulmonary complications are common in patients who have undergone esophagectomy. There are no good predictive variables for these complications. In addition, the role that preoperativeExpand
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Pharmacokinetics and Safety of MP-376 (Levofloxacin Inhalation Solution) in Cystic Fibrosis Subjects
ABSTRACT The pharmacokinetics and tolerability of nebulized MP-376 (levofloxacin inhalation solution [Aeroquin]) were determined in cystic fibrosis (CF) subjects. Ten CF subjects received singleExpand
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