Update in cystic fibrosis 2011

F Ratjen, SA McColley - American journal of respiratory and critical …, 2012 - atsjournals.org
Major advances in our understanding of cystic fibrosis (CF) lung disease and its treatment
have materialized over the last decades. Targeting therapies to the major pathophysiologic …

Cystic fibrosis: the dawn of a new therapeutic era

SL Heltshe, J Cogen, KJ Ramos… - American Journal of …, 2017 - atsjournals.org
Clinical outcomes and management of cystic fibrosis (CF) have undergone startling and
dramatic advances in the last two decades. These improvements can be linked to the …

Update in cystic fibrosis 2012

CH Goss, F Ratjen - American journal of respiratory and critical care …, 2013 - atsjournals.org
Although cystic fibrosis (CF) remains a life-limiting disease, we have witnessed major
advances in the care and clinical outcome of persons with CF (1). Animal models are …

Update in cystic fibrosis 2005

FJ Accurso - American journal of respiratory and critical care …, 2006 - atsjournals.org
Cystic fibrosis (CF) is a complex, hereditary disease involving several organ systems (1). CF
is caused by mutations in the gene that codes for the CF transmembrane conductance …

Update in cystic fibrosis 2013

M Jain, CH Goss - American journal of respiratory and critical care …, 2014 - atsjournals.org
Although cystic fibrosis (CF) continues to be the most common life-shortening genetic
disease in whites, median predicted life expectancy has reached an all-time high in the CF …

Outcome measures for development of new therapies in cystic fibrosis: are we making progress and what are the next steps?

BW Ramsey - Proceedings of the American Thoracic Society, 2007 - atsjournals.org
Since the discovery of the gene encoding the cystic fibrosis transmembrane regulator
protein (CFTR) in 1989, rapid expansion in our understanding of the basic defect has …

Update in cystic fibrosis 2014

T Ong, BW Ramsey - American journal of respiratory and critical …, 2015 - atsjournals.org
The clinical face of cystic fibrosis (CF) has significantly changed over the 25 years since its
basic genetic defect was discovered (1). Increasing numbers of patients are diagnosed at …

Update in cystic fibrosis 2007

FJ Accurso - American Journal of Respiratory and Critical Care …, 2008 - atsjournals.org
Cystic fibrosis (CF) is a chronic condition involving several organ systems that results in life-
long morbidity and premature mortality (1). CF is caused by abnormalities in the gene that …

Cystic fibrosis: from tragedy to triumph

B Ramsey, A Bush - American Journal of Respiratory and Critical …, 2023 - atsjournals.org
Less than a century ago, children with cystic fibrosis (CF) could not be distinguished from a
multitude of malnourished children with chronic wet cough and respiratory infection from …

Update on pathogenesis of cystic fibrosis lung disease

SH Donaldson, RC Boucher - Current opinion in pulmonary …, 2003 - journals.lww.com
Update on pathogenesis of cystic fibrosis lung disease : Current Opinion in Pulmonary
Medicine Update on pathogenesis of cystic fibrosis lung disease : Current Opinion in …