Factors associated with "Frequent Exacerbator" phenotype in children with bronchiectasis: The first report on children from the Australian Bronchiectasis Registry

Nitin Kapur; Enna Stroil-Salama; Lucy Morgan; Stephanie Yerkovich; Chien-Li Holmes-Liew; Paul King; Peter Middleton; Graeme Maguire; Daniel Smith; Rachel Thomson; Gabrielle McCallum; Louisa Owens; Anne B Chang

doi:10.1016/j.rmed.2021.106627

Factors associated with "Frequent Exacerbator" phenotype in children with bronchiectasis: The first report on children from the Australian Bronchiectasis Registry

Respir Med. 2021 Nov:188:106627. doi: 10.1016/j.rmed.2021.106627. Epub 2021 Sep 25.

Authors

Affiliations

¹ Department of Respiratory & Sleep Medicine, Queensland Children's Hospital and Faculty of Medicine, University of Queensland, QLD, Australia. Electronic address: nitin.kapur@health.qld.gov.au.
² Lung Foundation Australia, Milton, QLD, Australia.
³ Concord Clinical School, Faculty of Medicine and Health, University of Sydney, NSW, Australia.
⁴ Child Health Division, Menzies School of Health Research, Charles Darwin University, Darwin, NT, Australia; Australian Centre for Health Services Innovation, Queensland University of Technology, Australia.
⁵ Department of Thoracic Medicine, Royal Adelaide Hospital, South Australia, Australia.
⁶ Monash Respiratory and Sleep Medicine, Monash Medical Centre, VIC, Australia.
⁷ Department of Respiratory & Sleep Medicine, Westmead Hospital, Westmead, NSW, Australia.
⁸ Western Clinical School, University of Melbourne, Melbourne, VIC, Australia.
⁹ Thoracic Medicine, The Prince Charles Hospital, Brisbane, QLD, Australia.
¹⁰ Department of Respiratory Medicine, Greenslopes Private Hospital and Gallipoli Medical Research Institute, University of Queensland, Greenslopes, QLD, Australia.
¹¹ Child Health Division, Menzies School of Health Research, Charles Darwin University, Darwin, NT, Australia.
¹² Department of Respiratory Medicine, Sydney Children's Hospital, NSW, Australia.
¹³ Department of Respiratory & Sleep Medicine, Queensland Children's Hospital and Faculty of Medicine, University of Queensland, QLD, Australia; Child Health Division, Menzies School of Health Research, Charles Darwin University, Darwin, NT, Australia; Australian Centre for Health Services Innovation, Queensland University of Technology, Australia.

PMID: 34592538
DOI: 10.1016/j.rmed.2021.106627

Abstract

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 (OR_adjusted = 0.85, 95%CI 0.81-0.90), more recent diagnosis of bronchiectasis (OR_adjusted = 0.67; 95%CI 0.60-0.75), recent hospitalization (OR_adj = 4.51; 95%CI 2.45-8.54) and Pseudomonas aeruginosa (PsA) infection (OR_adjusted = 2.43; 95%CI 1.01-5.78). The FE phenotype were less likely to be Indigenous (OR_adjusted = 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.

Keywords: Bronchiectasis; Children; Frequent exacerbator; Phenotypes; Registry.

Publication types

Multicenter Study

MeSH terms

Adolescent
Australia
Bronchiectasis / physiopathology*
Child
Female
Humans
Male
Phenotype
Registries
Risk Factors
Spirometry
Symptom Flare Up*