The Lexiebean Foundation, Current News

Giving those affected by Cystic Fibrosis reason to Believe

Current News

Molecular characterization of gene regulatory networks in primary human tracheal and bronchial epithelial cells

Robust methods to culture primary airway epithelial cells were developed several decades ago and these cells provide the model of choice to investigate many diseases of the human lung. However, the molecular signature of cells from different regions of the airway epithelium has not been well characterized.

Improvement in Exophiala dermatitidis airway persistence and respiratory decline in response to interferon-gamma therapy in a patient with cystic fibrosis

Airflow obstruction infection and inflammation are key components of respiratory morbidity in patients with cystic fibrosis (CF). Chronic respiratory fungal infections, most commonly with moulds of the genera Aspergillus and Exophiala, are common in this patient cohort. An increased susceptibility to fungal infections may be observed in individuals with defects in IL-17RA signalling pathways, a situation characterised by excessive mucus production, increased Th2 cytokine, IL-17F, IL-33 and reduced IFN-γ secretion in patients without CF [1].

Mast cell tryptase changes with Aspergillus fumigatus – Host crosstalk in cystic fibrosis patients

Pulmonary and systemic antifungal immunity influences quality of life and survival of people with cystic fibrosis. Aspergillus fumigatus (Af) induces specific IgG and IgE. Mast cells respond to IgE, IgG and direct interactions with Af. Mast cells are the source of the protease tryptase. We aimed at evaluating serum baseline tryptase as a potential biomarker of the Af-host interaction in cystic fibrosis patients. Serum baseline tryptase, IgE and IgG directed to Af extract and Af molecular allergens were measured in 76 cystic fibrosis patients.

A comorbidity of CF in need of our attention and activity: Attention Deficit Hyperactivity Disorder!

Attention Deficit Hyperactivity Disorder (ADHD) has never received much attention in CF, even though patients with CF and ADHD demand plenty of our attention in the clinic! We all know patients showing hyperactive, fidgety, impulsive or distracted behaviors. We see children climbing our desks, running around the consultation room or not lying still on our examination tables. Their parents often seem helpless or so used to their child's behaviors they do not even attempt to regulate. In adult care, we see adults impulsively answer our carefully phrased questions, but who are often struggling to organize their complex CF treatment.

Risk factors for persistent Aspergillus respiratory isolation in cystic fibrosis

Aspergillus species are increasingly detected in the respiratory tracts of individuals with cystic fibrosis (CF), and chronic Aspergillus fumigatus is associated with more frequent hospitalizations for pulmonary exacerbations. However, patient and clinical factors that may contribute to the acquisition of persistent Aspergillus infection have yet to be identified. The objective of this study was to identify risk factors for development of Aspergillus respiratory isolation in CF.

Chronic infection sustained by a Pseudomonas aeruginosa High-Risk clone producing the VIM-1 metallo-β-lactamase in a cystic fibrosis patient after lung transplantation

The significance of chronic lung infection by multidrug-resistant (MDR) pathogens in Cystic Fibrosis (CF) transplanted patients remains controversial, and the available information is overall limited. Here we describe the case of a chronic infection, sustained by a metallo-β-lactamase (MBL)-producing P. aeruginosa strain, in a CF patient following lung transplantation.


A research session co-hosted by JCF and The Lancet Respiratory Medicine is scheduled for 7th June 2018 at the European Cystic Fibrosis Society conference in Belgrade, Serbia. The session will describe how these journals operate and disseminate the latest findings.