Pulmonary interstitial glycogenosis

Pulmonary interstitial glycogenosis (PIG) is a rare pediatric interstitial lung disease associated with alveolar growth abnormalities.

Clinical presentation

Patients may present in the neonatal period with disproportionate respiratory distress (neonatal respiratory distress).

Pathology

It is histologically characterized by abundant glycogen-laden mesenchymal cells within the pulmonary interstitium .

Associations

Many cases many show concurrent extrapulmonary comorbidities with congenital cardiac disease considered the most common of these .

Radiographic features

CT chest

Features are nonspecific, including ground-glass opacification (considered most common), consolidation, linear opacities, and mosaic attenuation without a uniform pattern.