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The effect of azithromycin on structural lung disease in infants with cystic fibrosis (COMBAT CF): a phase 3, randomised, double-blind, placebo-controlled clinical trial

Structural lung disease and neutrophil-dominated airway inflammation is present from 3 months of age in children diagnosed with cystic fibrosis after newborn screening. We hypothesised that azithromycin, given three times weekly to infants with cystic fibrosis from diagnosis until age 36 months, would reduce the extent of structural lung disease as captured on chest CT scans.

A pilot study of disease related education and psychotherapeutic support for unresolved grief in parents of children with CF

Diagnosis of chronic disease in a child can result in unresolved grief (UG) in parents. This study aimed to evaluate the efficacy of psychological insight-oriented therapy (IOT) as a treatment for UG compared to disease related education in parents of children with cystic fibrosis. Sequence of delivery, first IOT then disease related education (or vice versa) was also examined, to let all participants experience both interventions.

A differentiated nasal epithelial cell model derived from children with acute wheeze and asthma

The airway epithelium is the primary structural and functional airway barrier and orchestrates innate immunity. Some children may have underlying epithelial vulnerabilities that contribute to the pathogenesis of acute wheeze and asthma.

Allergic diseases through precision medicine

Allergic diseases are rising worldwide, especially in childhood, and their clinical diversity increasingly exposes the limits of traditional phenotype-based classifications. Genetic susceptibility, environmental exposures, epithelial barrier biology, and immune pathways interact to shape highly variable disease trajectories and treatment responses. In this context, precision medicine is no longer only an aspirational concept, but a practical effort to define meaningful endotypes, identify clinically useful biomarkers, and connect biological insight to prevention and care.

Directing immune development to curb sky-rocketing disease

Once upon a time it was infectious diseases like polio, measles or tuberculosis that most worried parents. With these threats now largely under control, parents face a new challenge – sky-rocketing rates of non-infectious diseases such as asthma, allergies and autism.

Disruption of β-catenin/CBP signaling inhibits human airway epithelial-mesenchymal transition and repair.

The airway epithelium of both children and adults with asthma is relatively undifferentiated characterized by a significantly increased proportion of...

Productive infection of human embryonic stem cell-derived nkx2.1+ respiratory progenitors with human rhinovirus.

Our experiments provide proof of principle for the use of PSC-derived respiratory epithelial cells in the study of cell-virus interactions.

PRAGMA-CF. A Quantitative Structural Lung Disease Computed Tomography Outcome in Young Children with Cystic Fibrosis

Chest computed tomography (CT) is the gold standard for demonstrating cystic fibrosis (CF) airway disease. However, there are no standardized outcome...

Early pulmonary inflammation and lung damage in children with cystic fibrosis

Airway inflammation and infection are present from early in life, often before children are symptomatic.

Biomarkers in paediatric Cystic Fibrosis lung disease

Biomarkers in cystic fibrosis are used for the measurement of cystic fibrosis transmembrane regulator function in order to diagnose cystic fibrosis,...