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Updated: Aug 14, 2026

Biochemical Titration of Glycogen In vitro
Published on: November 24, 2013
Pulmonary interstitial glycogenosis in identical twins
W Onland1, J J Molenaar, R J Leguit
1Department of Pediatrics, Emma Children's Hospital, Academic Medical Center, Amsterdam, The Netherlands.
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 interstitial 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.
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