Cystic fibrosis complicated by allergic bronchopulmonary aspergillosis
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Pediatric patient with known cystic fibrosis presents with worsening cough. On examination, there was reduced air entry in the right upper lobe.
Patient Data

There is nodular dense opacification in a finger-in-glove configuration overlying the right hilum and extending into the right upper lobe. This is likely to represent a combination of hilar lymphadenopathy and infective consolidation/bronchial plugging. In addition, there is perihilar consolidation on the left.
4 case questions available
Q: What are the two main underlying chronic lung pathologies that predispose to ABPA? show answer
A: Asthma and cystic fibrosis.
Q: Which laboratory tests are indicated if ABPA is clinically suspected? show answer
A: 1. Aspergillus serology; 2. Total serum IgE level; 3. Peripheral eosinophil count;
Q: What are the classic radiographic features of ABPA? show answer
A: 1. Transient, bilateral alveolar or subsegmental infiltrates with upper lobe predominance; 2. Central ring shadows representing bronchiectasis and bronchial wall thickening; 3. Branching tubular opacities representing mucus plugging, so-called 'finger-in-glove'
Q: What is the treatment for ABPA? show answer
A: Oral corticosteroids (plus oral antifungals if infection is recurrent/chronic).
Case Discussion
Atopic patients are predisposed to allergic bronchopulmonary aspergillosis (ABPA) through defective immuno-regulation. Both asthma and cystic fibrosis are strongly associated with atopy. Presenting symptoms raising the possibility of ABPA in these patient groups include worsening respiratory symptoms, low-grade fever, weight loss and malaise.
References
- 1. http://emedicine.medscape.com/article/353200-overview#showall
- 2. http://emedicine.medscape.com/article/296052-workup
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