Chronic Lung Allograft Dysfunction (CLAD) limits long-term survival following lung transplantation. Colonization of the allograft by Pseudomonas aeruginosa is associated with an increased ri...
Antisense oligonucleotide (ASO)-based drugs for splicing modulation were recently approved for various genetic diseases with unmet need. Here we aimed to develop an ASO-based splicing modula...
Cystic fibrosis is the most prevalent inherited disease caused by a defect in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. The impaired electrolyte homeostasis caused...
We are writing this letter to provide an update of published information on antibiotics for cystic fibrosis (CF) pulmonary exacerbations to the State of the Art articles by Zobell et al. Inf...
Childhood lung infection is often associated with prominent neutrophilic airway inflammation and excess production of proteases such as neutrophil elastase (NE). The mechanisms responsible f...
Systemic pseudohypoaldosteronism type 1 (PHA1) is a rare genetic syndrome of tissue unresponsiveness to aldosterone caused by mutations affecting the epithelial Na channel (ENaC). The classi...
Staphylococcus aureus is one of the most common pathogens isolated from the airways of cystic fibrosis (CF) patients and often persists for extended periods. There is limited knowledge about...
Microbe-microbe interactions can strongly influence growth and biofilm formation kinetics. For Pseudomonas aeruginosa and Candida albicans, which are found together in diverse clinical sites...
Pseudomonas aeruginosa (PA) is an opportunistic pathogen that causes diverse human infections including chronic airway infection in patients with cystic fibrosis (CF). Comparing the genomes ...
To explore primary care providers' (PCPs') role in result notification for newborn screening (NBS) for cystic fibrosis (CF), given that expanded NBS has increased the number of positive scre...
To explore primary care providers' (PCPs') preferred roles and confidence in caring for infants receiving a positive cystic fibrosis (CF) newborn screening (NBS) result, as well as managemen...
Membrane voltage (Vm) plays a critical role in the regulation of several cellular behaviors, including proliferation, apoptosis, and phenotypic plasticity. Many of these same behaviors are a...
In this present study, the authors evaluated the predictive factors for adverse maternal-fetal outcomes in pregnancies of women with cystic fibrosis (CF). Patients were followed up by a refe...
Cystic fibrosis (CF) is a disease caused by mutations in the CF transmembrane conductance regulator. The resulting chloride and bicarbonate imbalance produces a thick static lung mucus. This...
The epididymis is composed of a pseudostratified epithelium comprised of various cell types. Studies have shown that rat basal cells share common properties with adult stem cells and begin t...
The use of pulmonary MRI in a clinical setting has historically been limited. Whilst CT remains the gold-standard for lung imaging in many clinical indications, technical developments in ul...
Pulmonary diseases offer many targets for oligonucleotide therapeutics. However, effective delivery of oligonucleotides to the lung is challenging. For example, splicing mutations in the cys...
Cystic Fibrosis Related Diabetes (CFRD) is a frequent comorbidity of patients with Cystic Fibrosis (CF). A worsening of clinical conditions appears before CFRD. It has been demonstrated a de...
Recurrent events are commonly encountered in longitudinal studies. The observation of recurrent events is often stopped by a dependent terminal event in practice. For this data scenario, we ...
Cystic Fibrosis (CF) is a life-long genetic disease, causing increased energy needs and a healthy diet with a specific nutrient distribution. Nutritional status is an indicator of disease pr...
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