Cystic fibrosis is associated with increased inflammatory responses to pathogen challenge. Here we revisited the role of IL-1β in lung pathology using the experimental F508del-CFTR murine model on C57BL/6 genetic background (Cftr(tm1eur) or d/d), on double deficient for d/d and type 1 interleukin-1 receptor (d/d X IL-1R1-/-), and antibody neutralization. At steady state, young adult d/d mice di...

BACKGROUND In recent years, patients with cystic fibrosis (CF) have tended to experience a longer life expectancy and higher quality of life. In this context, the aim of the present study was to evaluate and compare the demographic, clinical, and laboratory markers of patients with CF during the last two decades at a CF referral center. METHODS A retrospective study of the demographic, clinic...


 CADTH reimbursement reviews are comprehensive assessments of the clinical effectiveness and cost-effectiveness, as well patient clinician perspectives, a drug or class.
 The inform non-binding recommendations that help guide decisions Canada's federal, provincial, territorial governments, with exception Quebec.
 This review assesses ELX-TEZ-IVA (Trikafta) ELX 100 mg, TEZ 50 IVA...

Woods PS, Tazi MF, Chesarino NM, Amer AO, Davis IC. TGF-induced IL-6 prevents development of acute lung injury in influenza A virus-infected F508del CFTR-heterozygous mice. Am J Physiol Lung Cell Mol Physiol 308: L1136–L1144, 2015. First published April 3, 2015; doi:10.1152/ajplung.00078.2015.—As the eighth leading cause of annual mortality in the USA, influenza A viruses are a major public hea...

BACKGROUND Microarray studies related to cystic fibrosis (CF) airway gene expression have gone some way in clarifying the complex molecular background of CF lung diseases, but have made little progress in defining a robust "molecular signature" associated with mutant CFTR expression. Disparate methodological and statistical analyses complicate comparisons between independent studies of the CF t...

Cystic fibrosis is the most common severe autosomal recessive disorder in Caucasians, with a frequency of 1 in 2000 live births and a carrier frequency of approximately 5% (1). It is a multi-system disorder that leads to chronic pulmonary and exocrine pancreatic disease, associated with elevated sweat electrolytes and male infertility due to bilateral absence of the vas deferens (2–4). Mutation...

BACKGROUND In vitro systems of primary cystic fibrosis (CF) airway epithelial cells are an important tool to study molecular and functional features of the native respiratory epithelium. However, undifferentiated CF airway cell cultures grown under submerged conditions do not appropriately represent the physiological situation. A more advanced CF cell culture system based on airway epithelial c...

Cystic fibrosis (CF) is an autosomal recessive inherited life-threatening disorder that causes severe damage to the lungs and the digestive system. In Palestine, mutations in the Cystic Fibrosis Transmembrane Conductance Regulator gene (CFTR) that contributes to the clinical presentation of CF are ill defined. A cohort of thirty three clinically diagnosed CF patients from twenty one different P...

Cystic fibrosis (CF) is a lethal genetic disorder most commonly caused by the F508del mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. It is not readily amenable to gene therapy because of its systemic nature and challenges including in vivo gene delivery and transient gene expression. Here, we use triplex-forming PNA molecules and donor DNA in biodegradable poly...