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Updated: Aug 14, 2026
07:16
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.
Pediatric Pulmonology
|August 6, 2005
Summary
Pulmonary interstitial glycogenosis, a rare lung condition, was diagnosed in premature twins with respiratory distress. Treatment with glucocorticoids led to a favorable outcome, suggesting it may be a form of chronic lung disease in infancy.
Area of Science:
- Neonatal Medicine
- Pediatric Pulmonology
- Pathology
Background:
- Respiratory distress is a common concern in premature infants.
- Chronic lung disease (CLD) of infancy presents diagnostic challenges.
- Monozygotic twins offer a unique model for studying genetic and environmental influences on disease.
Observation:
- Clinical presentation of respiratory distress in 6- and 8-week-old premature male twins.
- Radiological findings included reticular nodular interstitial patterns on chest radiography.
- High-resolution computed tomography (HRCT) revealed ground-glass opacification and thickened interstitial septae.
Findings:
- Open lung biopsies demonstrated diffuse interstitial thickening of alveolar septa by mesenchymal cells.
- Absence of significant type 2 pneumocyte hyperplasia and airspace exudates.
- Periodic acid-Schiff (PAS)-positive staining indicated cytoplasmic glycogen within interstitial cells, confirming pulmonary interstitial glycogenosis.
Implications:
- Pulmonary interstitial glycogenosis is a newly described histopathological entity.
- Glucocorticoid therapy resulted in a favorable clinical outcome for affected infants.
- This condition is hypothesized to be a histopathological subtype of chronic lung disease of infancy.
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