Cystic Fibrosis newborn screening (CFNBS) is the optimal method to diagnose the disease during the asymptomatic period. The aim of the study was to determine how CFNBS affects long term clin...
Trimeric Autotransporter Adhesins (TAA) found in Gram-negative bacteria play a key role in virulence. This is the case of Burkholderia cepacia complex (Bcc), a group of related bacteria able...
A homeostatic balance exists between the resident microbiota in the oral cavity and the host. Perturbations of the oral microbiota under particular conditions can contribute to the growth of...
Transfer from paediatric to adult services could lead to clinical deterioration; few studies have examined this. We sought to examine the clinical impact of a structured individualised trans...
Patients with cystic fibrosis (CF) harboring the P67L variant in the CF transmembrane conductance regulator (CFTR) often exhibit a typical CF phenotype, including severe respiratory compromi...
There are limited data regarding whether mortality is higher in patients with non cystic fibrosis bronchiectasis (bronchiectasis) than in those without bronchiectasis. Using 2005-2015 data f...
Hypergammaglobulinemia (hyper-IgG) and hypogammaglobulinemia (hypo-IgG) have been reported in patients with cystic fibrosis (CF). Although the clinical respiratory course is paradoxically di...
ABC-transporters transport a wealth of molecules across membranes and consist of transmembrane and cytosolic domains. Their activity cycle involves a tightly regulated and concerted domain c...
Dietary fiber functions as a prebiotic to determine the gut microbe composition. The gut microbiota influences the metabolic functions and immune responses in human health. The gut microbiot...
Complications from liver cirrhosis are a leading cause of death in children with cystic fibrosis. Identifying children at risk for developing liver cirrhosis and halting its progression are ...
Cystic Fibrosis (CF) is primarily a progressive lung disease, characterised by chronic pulmonary infections with opportunistic pathogens. Such infections typically commence early in life, pr...
Radiologic evidence of air trapping (AT) on expiratory computed tomography (CT) scans is associated with early pulmonary dysfunction in patients with cystic fibrosis (CF). However, standard ...
This was an observational cross-sectional study which was done to assess the expression profile of STATs and SOCS genes in cystic fibrosis. The mRNA was isolated from peripheral blood mononu...
The demand for Hospital in the Home has increased, especially as an avenue for treatment of respiratory exacerbations. However, a limiting factor of Hospital in the Home efficiency is excess...
Culture-independent studies have revealed that chronic lung infections in persons with cystic fibrosis (pwCF) are rarely limited to one microbial species. Interactions among bacterial member...
The purpose of this study was to pilot a home-based pulmonary rehabilitation (PR) program administered via a telemedicine approach using a combination of fitness application and self-selecte...
Pulmonary infections associated with Pseudomonas aeruginosa can be life-threatening for patients suffering from chronic lung diseases such as cystic fibrosis. In this scenario, the formation...
New drugs that target the basic defect in cystic fibrosis (CF) patients may now be used in a large number of patients carrying responsive mutations. Nevertheless, further research is needed ...
The benefits of unsupervised exercise programmes in obstructive lung disease are unclear. The aim of this systematic review was to synthesise evidence regarding the efficacy of unsupervised ...
The CFTR modulator ivacaftor has been variably effective in treating individuals with cystic fibrosis (CF) who harbor CFTR gating variants such as G551D, as well as other classes of CFTR var...
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