Abstract

Background 
Early diagnosis and optimal management in paediatric non-CF bronchiectasis are essential to achieve better clinical outcomes. 

Aims objectives 
Our aim was firstly to explore the characteristics of a cohort of children with non-CF bronchiectasis, and secondly to analyse the burden of illness experienced over one year. 

Methods 
We performed a cross-sectional study on children aged under 18 with non-CF bronchiectasis in Leicestershire, England. We analysed their presenting features, and collated data about their clinical course over a year from November 2021 to November 2022.

Results 
 40 children with non-CF bronchiectasis were included, 23 being female (57%). They mostly presented initially with a chronic wet cough, and had persistent x-ray changes. The median age at diagnosis following a CT chest was 5 years (range 3-7). Thirty-four patients (85%) underwent a bronchoscopy, eight of whom had airway malacia. 
The median age range of the current cohort was 10.5 years (range 7.0 to 14.2 years). In the last year, eight patients had positive virology (20%), the main virus isolated being entero/rhinovirus.  Eight patients (20%) had positive bacteriology, the main agent being haemophilus influenzae. Three children had isolated pseudomonas aeruginosa. Thirty- five patients (85%) are on prophylactic antibiotics.  Median Z-score of FEV1 of the patients was -1.745 (range -4.98 to 1.03).  
Eight patients (20%) were admitted for intravenous antibiotics in the last year, with one child having 5 admissions in a year.

Conclusions 
Our data shows that paediatric non-CF bronchiectasis is associated with significant burden.  It reaffirms the importance of international collaboration on its management to improve patient outcomes.