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Factors associated with "Frequent Exacerbator" phenotype in children with bronchiectasis: The first report on
Nitin Kapur1, Enna Stroil-Salama2, Lucy Morgan3
1Department of Respiratory & Sleep Medicine, Queensland Children's Hospital and Faculty of Medicine, University of Queensland, QLD, Australia.
Insights
A frequent exacerbator (FE) phenotype in childhood bronchiectasis is identified, characterized by younger age, recent diagnosis, Pseudomonas aeruginosa infection, and hospitalization. Indigenous children were less likely to exhibit this FE phenotype.
Area of Science:
- Pediatric Pulmonology
- Respiratory Medicine
- Clinical Phenotyping
Background:
- Adult bronchiectasis research has identified phenotypes like the frequent exacerbator (FE), but pediatric cohorts lack similar scientifically derived classifications.
- Existing pediatric bronchiectasis data is primarily from single centers, limiting comprehensive characterization and phenotype identification.
Purpose of the Study:
- To characterize the clinical features of pediatric bronchiectasis using data from the Australian Bronchiectasis Registry (ABR).
- To compare clinical characteristics between Indigenous and non-Indigenous children with bronchiectasis.
- To identify and characterize a pediatric frequent exacerbator (FE) phenotype and its associated factors.
Main Methods:
- Retrieved data from 540 children (<18 years) with radiologically confirmed bronchiectasis enrolled between March 2016 and March 2020 across five Australian sites.
- Analyzed baseline characteristics, including underlying etiology, bronchiectasis type, spirometry, environmental exposures, and birth weight.
- Identified the FE phenotype (≥3 exacerbations/year) and assessed its association with demographic, clinical, and microbiological factors using adjusted odds ratios.
Main Results:
- The study included 540 children (median age 8 years), with past infection/idiopathic etiology being most common (70%).
- Indigenous children showed significantly higher environmental tobacco smoke exposure (84% vs 32%) and lower birth weight (2797g vs 3260g) compared to non-Indigenous children.
- The FE phenotype, present in 30% (162) of children, was associated with younger age, more recent diagnosis, recent hospitalization, and Pseudomonas aeruginosa infection, and was less common in Indigenous children.
Conclusions:
- Pediatric bronchiectasis characteristics vary even within a single country's cohort.
- A distinct pediatric FE phenotype exists, characterized by younger age, recent diagnosis, Pseudomonas aeruginosa infection, and prior hospitalization.
- Further prospective studies are needed to validate these findings and refine the characterization of childhood bronchiectasis phenotypes.
Introduction:
In adults with bronchiectasis, multicentre data advanced the field including disease characterisation and derivation of phenotypes such as 'frequent exacerbator (FE)' (≥3 exacerbations/year). However, paediatric cohorts are largely limited to single centres and no scientifically derived phenotypes of paediatric bronchiectasis yet exists. Using paediatric data from the Australian Bronchiectasis Registry (ABR), we aimed to: (a) describe the clinical characteristics and compare Indigenous with non-Indigenous children, and (b) determine if a FE phenotype can be identified and if so, its associated factors.
Methods:
We retrieved data of children (aged <18-years) with radiologically confirmed bronchiectasis, enrolled between March 2016-March 2020.
Results:
Across five sites, 540 children [288 Indigenous; median age = 8-years (IQR 6-11)] were included. Baseline characteristics revealed past infection/idiopathic was the commonest (70%) underlying aetiology, most had cylindrical bronchiectasis and normal spirometry. Indigenous children (vs. non-Indigenous) had significantly more environmental tobacco smoke exposure (84% vs 32%, p < 0.0001) and lower birth weight (2797 g vs 3260 g, p < 0.0001). FE phenotype present in 162 (30%) children, was associated with being younger (ORadjusted = 0.85, 95%CI 0.81-0.90), more recent diagnosis of bronchiectasis (ORadjusted = 0.67; 95%CI 0.60-0.75), recent hospitalization (ORadj = 4.51; 95%CI 2.45-8.54) and Pseudomonas aeruginosa (PsA) infection (ORadjusted = 2.43; 95%CI 1.01-5.78). The FE phenotype were less likely to be Indigenous (ORadjusted = 0.14; 95%CI 0.03-0.65).
Conclusion:
Even within a single country, the characteristics of children with bronchiectasis differ among cohorts. A paediatric FE phenotype exists and is characterised by being younger with a more recent diagnosis, PsA infection and previous hospitalization. Prospective data to consolidate our findings characterising childhood bronchiectasis phenotypes are required.
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