Cystic fibrosis: a review
T Ong, BW Ramsey - Jama, 2023 - jamanetwork.com
Importance Cystic fibrosis, a genetic disorder defined by variants in the cystic fibrosis
transmembrane conductance regulator (CFTR) gene, affects more than 30 000 individuals in …
transmembrane conductance regulator (CFTR) gene, affects more than 30 000 individuals in …
[HTML][HTML] 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 …
factor in the pathobiology in cystic fibrosis (CF). A proinflammatory environment is seen …
[HTML][HTML] Disease Course, Treatments, and Outcomes of Children With Systemic Juvenile Idiopathic Arthritis–Associated Lung Disease
Y Huang, L Sompii-Montgomery, J Patti… - Arthritis care & …, 2024 - ncbi.nlm.nih.gov
Objective. Systemic juvenile idiopathic arthritis–associated lung disease (SJIA-LD) is a life-
threatening disease complication. Key questions remain regarding clinical course and …
threatening disease complication. Key questions remain regarding clinical course and …
2022 update on clinical practice guidelines for idiopathic pulmonary fibrosis and progressive pulmonary fibrosis
V Tzilas, A Tzouvelekis, JH Ryu… - The Lancet Respiratory …, 2022 - thelancet.com
The 2022 update on guidelines for idiopathic pulmonary fibrosis (IPF) by
ATS/ERS/JRS/ALAT1 consists of two parts. The first part is an update on the diagnosis and …
ATS/ERS/JRS/ALAT1 consists of two parts. The first part is an update on the diagnosis and …
Diagnosis and management of the systemic juvenile idiopathic arthritis patient with emerging lung disease
C Towe, AA Grom, GS Schulert - Pediatric Drugs, 2023 - Springer
Chronic lung disease in children with systemic juvenile idiopathic arthritis (SJIA-LD) is an
emerging and potentially life-threatening disease complication. Despite recent descriptions …
emerging and potentially life-threatening disease complication. Despite recent descriptions …
[HTML][HTML] Seasonal Azithromycin use in paediatric protracted bacterial bronchitis does not promote antimicrobial resistance but does modulate the nasopharyngeal …
SJ Hardman, FM Shackley, K Ugonna… - International Journal of …, 2023 - mdpi.com
Protracted bacterial bronchitis (PBB) causes chronic wet cough for which seasonal
azithromycin is increasingly used to reduce exacerbations. We investigated the impact of …
azithromycin is increasingly used to reduce exacerbations. We investigated the impact of …
[HTML][HTML] Macrolide therapy in Pseudomonas aeruginosa infections causes uL4 ribosomal protein mutations leading to high-level resistance
L Goltermann, KL Andersen, HK Johansen… - Clinical Microbiology …, 2022 - Elsevier
Objectives Pseudomonas aeruginosa colonizes the cystic fibrosis (CF) airways causing
chronic bacterial lung infections. CF patients are routinely treated with macrolides, however …
chronic bacterial lung infections. CF patients are routinely treated with macrolides, however …
[HTML][HTML] Detection of bile acids in bronchoalveolar lavage fluid defines the inflammatory and microbial landscape of the lower airways in infants with cystic fibrosis
JA Caparrós-Martín, M Saladie, SP Agudelo-Romero… - Microbiome, 2023 - Springer
Abstract Background Cystic Fibrosis (CF) is a genetic condition characterized by neutrophilic
inflammation and recurrent infection of the airways. How these processes are initiated and …
inflammation and recurrent infection of the airways. How these processes are initiated and …
[HTML][HTML] Key inflammatory markers in bronchoalveolar lavage predict bronchiectasis progression in young children with CF
H Horati, C Margaroli, JD Chandler, MB Kilgore… - Journal of Cystic …, 2024 - Elsevier
Introduction Inflammation appears early in cystic fibrosis (CF) pathogenesis, with specific
elevated inflammatory markers in bronchoalveolar lavage fluid (BALF) correlating with …
elevated inflammatory markers in bronchoalveolar lavage fluid (BALF) correlating with …
[HTML][HTML] Azithromycin Augments Bacterial Uptake and Anti-Inflammatory Macrophage Polarization in Cystic Fibrosis
AA Tarique, N Tuladhar, D Kelk, N Begum, RM Lucas… - Cells, 2024 - mdpi.com
Background: Azithromycin (AZM) is widely being used for treating patients with cystic fibrosis
(pwCF) following clinical trials demonstrating improved lung function and fewer incidents of …
(pwCF) following clinical trials demonstrating improved lung function and fewer incidents of …