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Fibrosing alveolitis in an infant
J Riedler1, A Golser, I Huttegger
1Dept of Paediatric Pulmonology, Kinderspital der Landeskrankenanstalten, Salzburg, Austria.
Insights
A rare infant lung disease caused interstitial fibrosis, initially unresponsive to standard treatments. Combination therapy with prednisone and azathioprine led to clinical improvement and normalized blood gases.
Area of Science:
- Pediatric Pulmonology
- Rare Pediatric Diseases
- Interstitial Lung Disease
Background:
- Infantile interstitial lung diseases present diagnostic challenges.
- Early symptoms like cough and tachypnea can be non-specific.
- Failure to thrive necessitates thorough investigation.
Observation:
- A 3-month-old infant presented with persistent respiratory distress and failure to thrive.
- Initial treatments with beta 2-agonists and antibiotics were ineffective.
- Chest radiography revealed infiltrates, but physical findings of diffuse crackles were unexplained.
Findings:
- Open lung biopsy demonstrated patchy interstitial fibrosis and thickened alveolar septa.
- Initial prednisone therapy showed limited clinical benefit.
- Combination therapy with azathioprine resulted in significant clinical improvement.
Implications:
- This case highlights the importance of considering rare interstitial lung diseases in infants with persistent respiratory symptoms.
- Prompt diagnosis and aggressive immunosuppressive therapy may be crucial for favorable outcomes.
- The successful use of azathioprine suggests its potential role in managing similar pediatric interstitial lung diseases.
Abstract:
A three month old female infant presented with unproductive cough, diffuse bilateral fine crackles, tachypnoea and failure to thrive despite a four month therapy with beta 2-agonists and antibiotics. A chest radiograph showing bilateral periphilar infiltrates and a patchy infiltrate in the right upper lobe and lingula did not explain the physical examination with diffuse bilateral fine crackles. As the condition did not improve and arterial oxygen tension (PaO2) and oxygen saturation decreased during the following two months, an open lung biopsy was performed. The surgeon described the lungs as rubbery in consistency and histological findings showed patchy mild interstitial fibrosis and thickened alveolar septa. A therapy with prednisone daily was started and given over a period of four months, but did not show sufficient improvement. Only after addition of azathioprine was clinical improvement and normalization of blood gases noted.