Scripties UMCG - Rijksuniversiteit Groningen
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Bronchoscopic Findings in Children with Chronic Suppurative Lung Disease

(2017) Vries, J.J.V. de

Background Chronic suppurative lung disease (CSLD) in children describes a wide variety of respiratory diseases including protracted bacterial bronchitis (PBB), bronchiectasis and cystic fibrosis (CF). Diagnostic flexible bronchoscopy and bronchoalveolar lavage (BAL) are frequently performed in children with CSLD to determine airway inflammation and infection. To date no studies comparing bronchoscopy and BAL findings across these three forms of CSLD in children have been conducted. Therefore, the aim of this study was to compare and contrast the bronchoscopic findings across children with CSLD, in particular PBB, bronchiectasis and CF, from a single tertiary pediatric centre.
Methods All bronchoscopy reports, including BAL findings, from the Royal Children’s Hospital (relocated and renamed Lady Cilento Children’s Hospital) in Brisbane for bronchoscopies performed between March 2010 and November 2016 were retrospectively reviewed. Bronchoscopic data, including BAL results, and radiological data regarding the presence of bronchiectasis from children (<18 years, post-hoc reduced to <6 years) meeting a-priori defined definitions of PBB (n=126), bronchiectasis (n=138) and CF (n=71) were collected. Our primary outcome was the airway microbiology of the three diagnostic cohorts, measured as proportion of children within a cohort in which specific pathogenic microorganisms were present. Secondary outcomes included airway cytology and aberrant macroscopic findings.
Results Bronchoscopic results from 335 children (median age: 25 months; interquartile range 15-42 months) were recorded. Children with PBB (87%) and bronchiectasis (80%) had significantly more frequent lower airway bacterial infection compared to children with CF (62%) (P < 0.0001). Children with PBB and bronchiectasis had more frequently positive bacterial cultures of Haemophilus influenzae, Moraxella catarrhalis and Streptococcus pneumoniae, whereas children with CF more commonly grew Pseudomonas aeruginosa and Staphylococcus aureus. Airway neutrophilia was higher in children with CF (P < 0.0001), and amongst all diagnostic groups primarily determined by pathogenic bacterial presence. Malacia had no influence on rates of airway infection or inflammation in PBB and bronchiectasis. Other novel findings included the high prevalence rates of viral pathogens cytomegalovirus (±26%) and Epstein-Barr virus (±18%) in children with PBB and bronchiectasis.
Conclusion This first single-centre study reviewing the bronchoscopic findings in children with PBB, bronchiectasis and CF revealed that, already during early childhood (<6 years), a distinct microbiological pattern in airway infection exists. Airways of young children with CSLD are frequently infected by microorganisms that are traditionally associated with their underlying disease. This knowledge of diverging early childhood bacterial pathogens, and the observed association between bacterial infection and neutrophilic inflammation, may help guide adequate treatment strategies in young children unable to provide sputum samples

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