Journal
PEDIATRIC RADIOLOGY
Volume 40, Issue 9, Pages 1562-1565Publisher
SPRINGER
DOI: 10.1007/s00247-010-1670-2
Keywords
Pulmonary interstitial glycogenosis; Cellular interstitial pneumonitis; Infants; Pediatric interstitial lung disease; Chest radiograph; High-resolution CT
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Pulmonary interstitial glycogenosis (PIG) is a rare pediatric interstitial lung disease. We report a case of a term boy presenting with tachypnea at birth requiring supplemental oxygen. Chest radiographs followed by high-resolution CT (HRCT) demonstrated hyperinflation and diffuse interstitial markings interspersed with multiple cystic spaces. An open lung biopsy demonstrated a minor component of PIG superimposed upon poor alveolarization. PIG in the setting of lung growth abnormality might be more common than previously described. Additionally, radiographic findings associated with most pediatric interstitial lung diseases are nonspecific, and histopathologic correlation is essential for diagnosis.
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