Paediatric Bronchiectasis, a UK-wide cohort study

<bold>Background:</bold> Paediatric bronchiectasis (PB) is poorly understood and under-researched. There are few large-scale studies and no registries available to interrogate current health or treatment strategies. To improve care, we established PBNetUK, a UK network of 26 paediatric centres. This study reports our national audit estimating prevalence, exploring causes, treatments and health of this population. <bold>Methods:</bold> PBnetUK agreed a standardised protocol. Centres reported total numbers of children with CT confirmed bronchiectasis excluding CF. Data from clinical visits was captured over 3 months with local audit office approval. <bold>Results:</bold> 844 cases were identified from 22 centres (85%) with patient data available for 474 (56%). Reported median age was 12.2 y (IQR 8.5-15), 231 (49%) were female and 320 (68%) were Caucasian. Age at diagnosis was 6 years (IQR 3-9 years) with 33% being post-infective, 28% idiopathic and 15% due to PCD. Median FEV1 was 79.6% (IQR 63-92) and FVC 85.9% (IQR 73-97). Comorbidities (58%) included asthma/atopy (19%). Regular treatment included 60% on prophylactic oral antibiotics, 42% on inhaled corticosteroids and 41% mucolytics. 31% received iv antibiotics in last year. Of the 81% who had microbiology done in past year, 42% were positive (H. influenzae 59%, Pseudomonas aeruginosa (PA) 10%). <bold>Conclusion:</bold> This is the largest study to report clinical status of PB. Treatment burden is high with one third having intravenous antibiotics. 1 in 5 had no identifiable cause. Incidence of PA is low compared to adults. Better microbiological monitoring is needed. Importantly, establishment of the PBnetUK will aid future research, the next step is a national registry linking to the fledgling ERS Child-BEAR Registry.