1. Pulmonary interstitial glycogenosis in identical twins
- Author
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W. Onland, R.J. Leguit, R.R. van Rijn, J. C. Van Nierop, L. A. Noorduyn, Jan J. Molenaar, V.G.M. Geukers, Neonatology, CCA -Cancer Center Amsterdam, General Paediatrics, Paediatric Pulmonology, Other Research, Radiology and Nuclear Medicine, and Paediatric Intensive Care
- Subjects
Lung Diseases ,Male ,Pulmonary and Respiratory Medicine ,Pathology ,medicine.medical_specialty ,Monozygotic twin ,Biopsy ,Diseases in Twins ,Humans ,Medicine ,Lung ,medicine.diagnostic_test ,Respiratory distress ,business.industry ,Respiratory disease ,Interstitial lung disease ,Infant ,Twins, Monozygotic ,Hyperplasia ,Glycogen Storage Disease ,medicine.disease ,Radiography ,medicine.anatomical_structure ,Pediatrics, Perinatology and Child Health ,Reticular connective tissue ,business - Abstract
Summary. 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 acidSchiff (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. Pediatr
- Published
- 2005