Abstract
We present the clinical, radiological, and pathological findings of open lung biopsies from monozygotic prematurely born male twins with respiratory distress at ages 6 and 8 weeks postnatally. Radiological examination showed a reticular nodular interstititial pattern on chest radiography. High-resolution computed tomography (HRCT) revealed ground-glass opacification and thickened interstitial septae in both infants. Lung biopsies showed a similar histology. There was diffuse interstitial thickening of the alveolar septa by mesenchymal cells, without prominent hyperplasia of type 2 pneumocytes, and without airspace exudates. Sections were periodic acid-Schiff (PAS)-positive within the cytoplasm of interstitial cells, indicating the presence of glycogen. Thus the diagnosis of pulmonary interstitial glycogenosis was made. Both infants were treated with glucocorticoids and had a favorable outcome. We speculate that pulmonary interstitial glycogenosis could be a histopathological form of chronic lung disease (CLD) of infancy.
| Original language | English |
|---|---|
| Pages (from-to) | 362-366 |
| Number of pages | 5 |
| Journal | Pediatric Pulmonology |
| Volume | 40 |
| Issue number | 4 |
| DOIs | |
| Publication status | Published - Oct 2005 |
| Externally published | Yes |
Keywords
- Interstitial lung disease
- Monozygotic twins
- Open lung biopsy
- Pulmonary interstitial glycogenosis