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Aspergillus in Children and Young People with Cystic Fibrosis: A Narrative Review

Summary

Cystic fibrosis is a serious genetic disease affecting children that damages the lungs through chronic infections. Aspergillus, a common mold in the environment, colonizes the airways of CF patients and can cause additional lung damage. While new CFTR modulator medications have improved outcomes and reduced Aspergillus infections, more research is needed to better diagnose and treat fungal infections in children with CF.

Background

Cystic fibrosis is a severe inherited disorder affecting over half children, characterized by persistent airway infection and progressive lung function decline. Aspergillus colonization and infection are common complications in people with CF, evolving into various disease phenotypes including Aspergillus bronchitis and allergic bronchopulmonary aspergillosis (ABPA). Management strategies primarily target bacterial infections, while the role of Aspergillus infections remains less understood in childhood.

Objective

This narrative review summarizes current understanding of Aspergillus impact on airways of children and young people with cystic fibrosis, addressing the gaps in evidence-based diagnostic and treatment guidelines for fungal infections in this pediatric population.

Results

The review documents variable Aspergillus colonization rates (9-13.2% in BAL studies, 11-32% in sputum studies) depending on sampling methods and detection techniques. Aspergillus positive BAL samples are associated with structural lung changes, lower lung function, and increased respiratory admissions. CFTR modulator therapies have reduced Aspergillus colonization prevalence from 9.4% to 3.1% in UK children with CF.

Conclusion

Aspergillus infections remain important complications in children with CF despite CFTR modulator advances. Current evidence supports that persistent Aspergillus colonization associates with lung function decline and structural damage, but standardized diagnostic definitions and treatment guidelines are needed for optimal pediatric management.
  • Published in:Journal of Fungi (Basel),
  • Study Type:Narrative Review,
  • Source: 10.3390/jof11030210; PMID: 40137248
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