Progress in therapies for cystic fibrosis

K De Boeck, MD Amaral - The Lancet Respiratory Medicine, 2016 - thelancet.com
Standard follow-up and symptomatic treatment have allowed most patients with cystic
fibrosis to live to young adulthood. However, many patients still die prematurely from …

Respiratory infection and inflammation in cystic fibrosis: a dynamic interplay among the host, microbes, and environment for the ages

C Yu, T Kotsimbos - International Journal of Molecular Sciences, 2023 - mdpi.com
The interplay between airway inflammation and infection is now recognized as a major
factor in the pathobiology in cystic fibrosis (CF). A proinflammatory environment is seen …

Impact of Coexistence Phenotype Between Staphylococcus aureus and Pseudomonas aeruginosa Isolates on Clinical Outcomes Among Cystic Fibrosis Patients

P Briaud, S Bastien, L Camus, M Boyadjian… - Frontiers in Cellular …, 2020 - frontiersin.org
Staphylococcus aureus (SA) is the major colonizer of the lungs of cystic fibrosis (CF) patients
during childhood and adolescence. As patients age, the prevalence of SA decreases and …

Unveiling Co-Infection in Cystic Fibrosis Airways: Transcriptomic Analysis of Pseudomonas aeruginosa and Staphylococcus aureus Dual-Species Biofilms

AP Magalhães, A França, MO Pereira, N Cerca - Frontiers in Genetics, 2022 - frontiersin.org
Cystic fibrosis (CF) is a common heritable genetic disorder caused by a defect in the cystic
fibrosis conductance regulator gene, resulting in several complications in the human body …

Viable but non‐cultivable state: a strategy for Staphylococcus aureus survivable in dual‐species biofilms with Pseudomonas aeruginosa?

AP Magalhães, T Grainha, AM Sousa… - Environmental …, 2021 - Wiley Online Library
Pseudomonas aeruginosa and Staphylococcus aureus are two of the most prevalent
respiratory pathogens in cystic fibrosis patients. Both organisms often cause chronic and …

The short-term effects of ORKAMBI (lumacaftor/ivacaftor) on regional and distal lung structures using functional respiratory imaging

E Lauwers, D Belmans, B Mignot… - Therapeutic …, 2021 - journals.sagepub.com
Background: Lumacaftor/ivacaftor (LUM/IVA) has shown modest benefits in previous
research, but the exact effects in the cystic fibrosis (CF) lung remain unclear. This study aims …

How do cystic fibrosis patients experience parenthood? A systematic review

A Jacob, J Journiac, L Fischer… - Journal of Health …, 2021 - journals.sagepub.com
Improved treatments for cystic fibrosis allow more patients to become parents. This article
presents a systematic review of literature on cystic fibrosis patients' experience of …

Long-Term Coexistence of Pseudomonas Aeruginosa and Staphylococcus Aureus Using an In Vitro Cystic Fibrosis Model

R Monteiro, AP Magalhaes, MO Pereira… - Future …, 2021 - Taylor & Francis
Aim: To investigate the role of pre-established Staphylococcus aureus on Pseudomonas
aeruginosa adaptation and antibiotic tolerance. Materials & methods: Bacteria were cultured …

Re-imagining cystic fibrosis care: next generation thinking

C Rang, D Keating, J Wilson… - European Respiratory …, 2020 - Eur Respiratory Soc
Cystic fibrosis (CF) is a common multi-system genetically inherited condition, predominately
found in individuals of Caucasian decent. Since the identification of the cystic fibrosis (CF) …

Systems biology and bile acid Signalling in microbiome-host interactions in the cystic fibrosis lung

DF Woods, S Flynn, JA Caparrós-Martín, SM Stick… - Antibiotics, 2021 - mdpi.com
The study of the respiratory microbiota has revealed that the lungs of healthy and diseased
individuals harbour distinct microbial communities. Imbalances in these communities can …