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Allergic bronchopulmonary aspergillosis complicating cystic fibrosis in childhood
Insights
Allergic bronchopulmonary aspergillosis (ABPA) can affect young children with cystic fibrosis (CF). Early diagnosis is key, as ABPA presents with asthma symptoms and lung changes, even in non-advanced CF cases.
Area of Science:
- Pediatric Pulmonology
- Allergy and Immunology
- Medical Mycology
Background:
- Allergic bronchopulmonary aspergillosis (ABPA) is typically seen in adults with cystic fibrosis (CF).
- This study investigates ABPA in young, atopic children with CF.
Observation:
- Seven young atopic children with CF presented with new or worsening asthma symptoms.
- Radiographic findings included lobar collapse or extensive, changing consolidation.
- All patients exhibited blood eosinophilia, positive Aspergillus fumigatus skin tests, and reversible airway obstruction.
Findings:
- Most patients had positive type III skin tests, precipitins to A. fumigatus, and elevated IgE with specific antibodies (RAST).
- None had advanced CF lung disease.
- Two children experienced complete lung collapse; five had recurrent pulmonary shadowing without significant CF progression.
Implications:
- Increased awareness of ABPA is crucial in atopic children with CF, especially with significant lung collapse or consolidation.
- Prompt diagnosis and management can prevent severe lung complications.
- This highlights the importance of considering ABPA in pediatric CF patients presenting with respiratory exacerbations.
Abstract:
Allergic bronchopulmonary aspergillosis, known to be associated with cystic fibrosis in older patients, occurred in 7 young atopic children with cystic fibrosis. The diagnosis was suggested by the onset of, or the increase in, asthmatic symptoms accompanied by major chest x-ray changes ranging from total collapse of a lung or lobe to extensive but changing areas of consolidation. Each of the children had a blood eosinophilia, positive type I skin tests to Aspergillus fumigatus, and reversible airways obstruction. Most had a positive type III skin test and circulating precipitins to A. fumigatus, with raised IgE levels which contained specific antibodies to the fungus on radioallergosorbent (RAST) test. None had advanced suppurative chest disease of cystic fibrosis. None was given specific antifungal agents; two received systemic treatment with corticosteroids, the other received additional drugs for their asthma. Two developed total collapse of one lung, one child being only 2 years old. Five have had recurrences of pulmonary shadowing typical of allergic aspergillosis but are not showing significant progression of their cystic fibrosis lung disease. Our experience suggests that there should be an increased awareness of this condition, particularly its association with extensive pulmonary collapse or consolidation in children with cystic fibrosis who are atopic.