Unraveling the CFTR function–phenotype connection for precision treatment in cystic fibrosis

ET Zemanick, D Polineni - … journal of respiratory and critical care …, 2019 - atsjournals.org
The clinical phenotype of cystic fibrosis (CF), chronic infection, and inflammation leading to
progressive obstructive lung disease and pancreatic insufficiency is caused by absence or …

Phoenix from the Ashes: Celebrating the 2023 North American Cystic Fibrosis Conference

A Bush, B Ramsey, M Shteinberg… - American Journal of …, 2023 - atsjournals.org
November 2–4 sees the 2023 North American Cystic Fibrosis Conference hosted by the
Cystic Fibrosis Foundation in Phoenix, Arizona. No respiratory disease has so dramatically …

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 …

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 …

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 2008

F Ratjen - American journal of respiratory and critical care …, 2009 - atsjournals.org
Cystic fibrosis (CF) is caused by mutations in the cystic fibrosis transmembrane regulator
(CFTR) gene, and more than 1,500 distinct mutations with different functional consequences …

Depths of Dysfunctional Epithelial and Immune Crosstalk in Cystic Fibrosis Revealed

LM Yonker, TB Kinane - … Journal of Respiratory and Critical Care …, 2024 - atsjournals.org
Highly effective cystic fibrosis transmembrane conductance regulator (CFTR) modulatory
therapy has revolutionized the lives of people with cystic fibrosis (CF) with at least one …

Update in cystic fibrosis 2006

FJ Accurso - American journal of respiratory and critical care …, 2007 - atsjournals.org
Cystic fibrosis (CF) is a complex inherited condition resulting from abnormalities in the gene
that codes for the cystic fibrosis transmembrane conductance regulator (CFTR). CFTR, a …

Update in cystic fibrosis 2009

PJ Mogayzel Jr, PA Flume - … journal of respiratory and critical care …, 2010 - atsjournals.org
The discovery of disease-causing mutations in the cystic fibrosis transmembrane
conductance regulator (CFTR) gene 20 years ago ushered in a revolution in our …

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 …