Managing cystic fibrosis: strategies that increase life expectancy and improve quality of life

M Cohen-Cymberknoh, D Shoseyov… - American journal of …, 2011 - atsjournals.org
The survival of patients with cystic fibrosis (CF) continues to improve. The discovery and
cloning of the CFTR gene more than 21 years ago led to the identification of the structure …

[HTML][HTML] European cystic fibrosis society standards of care: framework for the cystic fibrosis centre

S Conway, IM Balfour-Lynn, K De Rijcke… - Journal of Cystic …, 2014 - Elsevier
A significant increase in life expectancy in successive birth cohorts of people with cystic
fibrosis (CF) is a result of more effective treatment for the disease. It is also now widely …

[HTML][HTML] Mepolizumab for eosinophilic chronic obstructive pulmonary disease

ID Pavord, P Chanez, GJ Criner… - … England Journal of …, 2017 - Mass Medical Soc
Background Patients with chronic obstructive pulmonary disease (COPD) with an
eosinophilic phenotype may benefit from treatment with mepolizumab, a monoclonal …

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

DB Sanders, RCL Bittner, M Rosenfeld… - American journal of …, 2010 - atsjournals.org
Rationale: Patients with cystic fibrosis periodically experience pulmonary exacerbations.
Previous studies have noted that some patients' lung function (FEV1) does not improve with …

[HTML][HTML] Standards of care for patients with cystic fibrosis: a European consensus

E Kerem, S Conway, S Elborn, H Heijerman… - Journal of cystic …, 2005 - Elsevier
Cystic fibrosis (CF) is caused by mutations in the cystic fibrosis transmembrane conductance
regulator (CFTR) gene [1, 2]. This results in dysfunction of the apical membrane CFTR …

Exacerbations in cystic fibrosis· 1: epidemiology and pathogenesis

CH Goss, JL Burns - Thorax, 2007 - thorax.bmj.com
With the improving survival of patients with cystic fibrosis (CF), the clinical spectrum of this
complex multisystem disease continues to evolve. One of the most important clinical events …

New pharmacological approaches for cystic fibrosis: promises, progress, pitfalls

SC Bell, K De Boeck, MD Amaral - Pharmacology & therapeutics, 2015 - Elsevier
With the discovery of the CFTR gene in 1989, the search for therapies to improve the basic
defects of cystic fibrosis (CF) commenced. Pharmacological manipulation provides the …

Newborn screening for cystic fibrosis

C Castellani, J Massie, M Sontag… - The Lancet Respiratory …, 2016 - thelancet.com
Since the late 1970s when the potential of the immunoreactive trypsinogen assay for early
identification of infants with cystic fibrosis was first recognised, the performance of newborn …

Infection control recommendations for patients with cystic fibrosis: microbiology, important pathogens, and infection control practices to prevent patient-to-patient …

L Saiman, J Siegel - Infection Control & Hospital Epidemiology, 2003 - cambridge.org
Infection Control Recommendations for Patients With Cystic Fibrosis: Microbiology,
Important Pathogens, and Infection Control Practices to Prevent Patient-to-Patient …

Treatment of pulmonary exacerbations in cystic fibrosis

JM Bhatt - European Respiratory Review, 2013 - Eur Respiratory Soc
Pulmonary exacerbations have very important consequences in cystic fibrosis (CF), both in
terms of current morbidity as well as implications for long term morbidity and mortality. Even …