Cutz E, et al. - The intent of this trial was to shed light on the pulmonary interstitial glycogenosis (PIG) related to a spectrum of neonatal pulmonary disorders. All of the cases reported an affirmed PIG via positive periodic acid–Schiff (PAS) staining, immunopositivity for vimentin, and electron microscopy (EM). Few patients improved with age. However, 7 among them died of respiratory failure or complications of congenital heart disease (CHD), indicating that PIG could be clinically prominent when related to other severe disorders. The link between PIG with a spectrum of mostly congenital lung disorders supported its origin as a developmental abnormality of interstitial fibroblast differentiation instead of a nonspecific reactive process.