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Updated: Jun 18, 2026

Detecting Glycogen in Peripheral Blood Mononuclear Cells with Periodic Acid Schiff Staining
Published on: December 23, 2014
Pulmonary interstitial glycogenosis
Michael Lanfranchi1, Sandra M Allbery, Lisa Wheelock
1Children's Hospital & Medical Center, Creighton University Medical School, Omaha, NE 68114, USA. MichaelLanfranchi@creighton.edu
Pulmonary interstitial glycogenosis is a rare lung disease in nonintubated infants. This condition, mimicking bronchopulmonary dysplasia, can be treated with corticosteroids.
Area of Science:
- Pediatric Pulmonology
- Neonatology
- Pediatric Radiology
Background:
- Interstitial lung disease (ILD) in neonates is often associated with bronchopulmonary dysplasia (BPD) in chronically intubated infants.
- ILD in nonintubated infants is uncommon, presenting diagnostic challenges.
Observation:
- A case of a 31-week gestation infant, not requiring intubation, who developed respiratory distress at 18 days of life.
- Initial radiographs were normal, but subsequent CT and radiographic findings revealed severe ILD, resembling BPD.
- Infectious etiologies were systematically excluded.
Findings:
- Lung biopsy confirmed the diagnosis of pulmonary interstitial glycogenosis (PIG).
- PIG is infrequently described in pediatric radiology literature.
- Radiographic and CT findings mimicked BPD, highlighting a potential diagnostic pitfall.
Implications:
- Pulmonary interstitial glycogenosis should be considered in the differential diagnosis of ILD in nonintubated infants.
- Early recognition and diagnosis are crucial.
- The condition has shown responsiveness to corticosteroid therapy, offering a potential treatment avenue.
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