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Aspergillus in Children and Young People with Cystic Fibrosis: A Narrative Review
Emily Chesshyre1,2, Eva Wooding1,2, Emily Sey1
1MRC Centre for Medical Mycology, Department of Biosciences, Faculty of Health and Life Sciences, University of Exeter, Exeter EX4 4QD, UK.
Insights
Cystic fibrosis (CF) affects many children, often leading to lung infections. This review examines the impact of Aspergillus infections in children with CF, highlighting knowledge gaps in diagnosis and treatment.
Area of Science:
- Pediatric Pulmonology
- Infectious Diseases
- Genetics
Background:
- Cystic fibrosis (CF) is a severe inherited disorder impacting over half a million children globally.
- Airway infections and inflammation leading to progressive lung function decline are hallmarks of CF.
- Aspergillus colonization is a known complication in CF patients, manifesting as various disease phenotypes.
Purpose of the Study:
- To review the current understanding of Aspergillus's impact on the airways of children and young people with CF.
- To highlight the lack of evidence-based diagnostic and treatment guidelines for Aspergillus infections in pediatric CF patients.
Main Methods:
- This study is a narrative review.
- It synthesizes existing literature on Aspergillus infections in pediatric CF patients.
Main Results:
- Aspergillus colonization and infection present diverse phenotypes in CF, including bronchitis, sensitization, and allergic bronchopulmonary aspergillosis (ABPA).
- Current CF management primarily focuses on bacterial infections, with less understanding of Aspergillus's role in childhood.
Conclusions:
- There is a significant knowledge gap regarding the diagnosis and management of Aspergillus infections in children with CF.
- Further research is needed to develop evidence-based guidelines for addressing Aspergillus-related complications in this population.
Abstract:
Cystic fibrosis is a severe, inherited, life-limiting disorder, and over half of those living with CF are children. Persistent airway infection and inflammation, resulting in progressive lung function decline, is the hallmark of this disorder. Aspergillus colonization and infection is a well-known complication in people with CF and can evolve in a range of Aspergillus disease phenotypes, including Aspergillus bronchitis, fungal sensitization, and allergic bronchopulmonary aspergillosis (ABPA). Management strategies for children with CF are primarily aimed at preventing lung damage and lung function decline caused by bacterial infections. The role of Aspergillus infections is less understood, especially during childhood, and therefore evidence-based diagnostic and treatment guidelines are lacking. This narrative review summarizes our current understanding of the impact of Aspergillus on the airways of children and young people with CF.
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