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ABSTRACT
Introduction: Lung disease in cystic fibrosis begins in early life with neutrophil-dominated inflammation and infection, is progressive and results in structural lung damage characterised by bronchial dilation and bronchiectasis. Preventative strategies must be employed in early life but require a better understanding of how  bronchiectasis develops.

Areas covered: In this review we have addressed the diagnosis and early life risk factors for bronchiectasis in young children with cystic fibrosis. A systematic review was not performed and the literature reviewed was known to the authors.

Expert commentary: Bronchiectasis represents a process of progressive dilatation and damage of airway walls and is traditionally considered to be irreversible. Diagnosis is primarily by detecting a bronchial:arterial ratio of >1 on chest CT scan. Lung volume has a greater influence on airway diameter than on arterial making control of lung volume during scanning critical. Early life risk factors for the
onset and progression bronchiectasis include: severe cystic fibrosis genotype; neutrophilic inflammation with free neutrophil elastase activity in the lung; and pulmonary infection. Bronchiectasis develops in the majority of children before they reach school age despite the best current therapy. To prevent bronchiectasis novel therapies are going to have to be given to infants. 

 

Authors: Peter D. Sly and Claire E. Wainwright

Published in the Expert Review of Respiratory Medicine in September 2016.