Pulmonary interstitial glycogenosis

Last revised by David Luong on 27 Jul 2021

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

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

It is histologically characterized by abundant glycogen-laden mesenchymal cells within the pulmonary interstitium 1.

Many cases show concurrent extrapulmonary comorbidities with congenital cardiac disease considered the most common of these 3.

Features are nonspecific, including ground-glass opacification (considered most common 1), consolidation, linear opacities, and mosaic attenuation without a uniform pattern.


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