Pulmonary interstitial glycogenosis in identical twins

W. Onland, J. J. Molenaar, R. J. Leguit, J. C. Van Nierop, L. A. Noorduyn, R. R. van Rijn, V. G. M. Geukers

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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 languageEnglish
Pages (from-to)362-366
JournalPediatric pulmonology
Issue number4
Publication statusPublished - 2005

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