Extract We were extremely pleased to read the interesting perspective on our manuscript 1 from J. Ding and co-workers, and are grateful for the opportunity to respond. We agree with the authors’ comments on the unknown trajectory that has ended in the “snapshot” of adult patients with paediatric-onset bronchiectasis (POBE) in our study. Indeed, this grim snapshot of overall substantial disease burden of these young individuals probably reflects life-long insults during periods of poor bronchiectasis care. While we do not have longitudinal data, no-one is born with bronchiectasis and so the more extensive lung damage and lower lung function in POBE can only reflect either the progressive nature of the disease in these patients or, as has been described in COPD, early life insults leading to a failure to achieve full pulmonary function. These lifelong trajectories are impossible to capture fully without longitudinal follow up of multiple clinical, radiological, physiological and biomarker data, but the key message that patients with POBE have a higher burden of disease remains clear.
Khalaili et al. (Sun,) studied this question.