The first 2 years of life : implications of recent findings

Purpose of reviewThis review highlights important observations published in the past 2 years that provideinsight regarding the early pathophysiology of cystic fibrosis lung disease and thatindicate potentially useful clinical endpoints for disease management and clinical trials.Recent findingsLung disease is evident soon after diagnosis even in children diagnosed with cysticfibrosis following newborn screening. Neutrophilic airway inflammation and lower airwayinfection with Pseudomonas aeruginosa appear to be important factors associated withlung disease including bronchiectasis detected using low-dose computed tomography.Children with cystic fibrosis managed in specialist centres can develop bronchiectasisin the first year of life and computed tomography reveals that bronchiectasis is present inmore than 40% of children with cystic fibrosis by 4 years of age.SummaryCurrent management of newly diagnosed children with cystic fibrosis in specialistcentres fails to prevent lung damage. In order to better define relations betweeninfection, inflammation and lung damage, simple, sensitive and specific markers of lowerairway infection are required. Data emerging from systematic early surveillanceprograms indicate that measures of lung damage, lung function, airway inflammationand endobronchial infection could be used as outcome measures for early interventionstudies to prevent the respiratory sequelae of cystic fibrosis.