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 …

A systematic review of factors associated with health-related quality of life in adolescents and adults with cystic fibrosis

ARR Habib, J Manji, PG Wilcox, AR Javer… - Annals of the …, 2015 - atsjournals.org
Rationale: As the life expectancy for individuals with cystic fibrosis (CF) continues to
improve, an emphasis on optimizing health-related quality of life (HRQoL) has become …

The cystic fibrosis lung microbiome

MG Surette - Annals of the American Thoracic Society, 2014 - atsjournals.org
The chronic colonization of the lower airways by bacterial pathogens is the leading cause of
morbidity and mortality in patients with cystic fibrosis (CF). Pseudomonas aeruginosa is the …

The emerging burden of liver disease in cystic fibrosis patients: A UK nationwide study

MB Toledano, SK Mukherjee, J Howell, D Westaby… - PLoS …, 2019 - journals.plos.org
Objective Cystic fibrosis associated liver disease (CFLD) is the third largest cause of
mortality in CF. Our aim was to define the burden of CFLD in the UK using national registry …

Medication beliefs mediate between depressive symptoms and medication adherence in cystic fibrosis.

ME Hilliard, MN Eakin, B Borrelli, A Green… - Health …, 2015 - psycnet.apa.org
Objective: Depression is a known barrier to regimen adherence for chronic conditions.
Despite elevated depression rates and complex regimens for people with cystic fibrosis …

The epithelial barrier theory and its associated diseases

N Sun, I Ogulur, Y Mitamura, D Yazici, Y Pat, X Bu… - Allergy, 2024 - Wiley Online Library
The prevalence of many chronic noncommunicable diseases has been steadily rising over
the past six decades. During this time, over 350,000 new chemical substances have been …

Lung transplantation for cystic fibrosis: results, indications, complications, and controversies

JP Lynch III, DM Sayah, JA Belperio… - … in respiratory and …, 2015 - thieme-connect.com
Survival in patients with cystic fibrosis (CF) has improved dramatically over the past 30 to 40
years, with mean survival now approximately 40 years. Nonetheless, progressive respiratory …

Bisphosphonates for osteoporosis in people with cystic fibrosis

LS Conwell, AB Chang - Cochrane Database of Systematic …, 2014 - cochranelibrary.com
Background Osteoporosis is a bone mineralisation disorder occurring in about one third of
adults with cystic fibrosis. Bisphosphonates can increase bone mineral density and …

Inflammation, oxidative stress, and cardiovascular disease risk factors in adults with cystic fibrosis

EJ Reverri, BM Morrissey, CE Cross… - Free Radical Biology and …, 2014 - Elsevier
Cystic fibrosis (CF) represents one of a number of localized lung and non-lung diseases with
an intense chronic inflammatory component associated with evidence of systemic oxidative …

The clinical significance of the gut microbiota in cystic fibrosis and the potential for dietary therapies

L Li, S Somerset - Clinical Nutrition, 2014 - Elsevier
Cystic fibrosis (CF) is characterised by many comorbidities related to aberrant mucosa and
chronic inflammation in the respiratory and digestive systems. The intestinal mucosa serves …