Pulmonary Interstitial Glycogenosis Cells Express Mesenchymal Stem Cell Markers

Pulmonary interstitial glycogenosis (PIG) was first defined as a distinct neonatal interstitial lung disease of unknown etiology that presents in neonates and young infants with mild to severe hypoxic lung disease [1]. Characterised clinically by unexplained respiratory distress and cyanosis with an onset during early infancy, PIG was primarily defined by the presence of distinct and unusual-appearing cells contained within the interstitium that were characterised by a widened interstitium containing variable numbers of immature-appearing, polygonal-to-spindle shaped cells, which may contribute to impaired gas exchange. The most unique feature of PIG cells is the widespread presence of non-membrane bound, periodic acid-Schiff (PAS) stain-positive, mono-particulate glycogen in the cytoplasm, for which the disease was named (“glycogenosis”) [1].\n\nFootnotes \n\nThis manuscript has recently been accepted for publication in the European Respiratory Journal . It is published here in its accepted form prior to copyediting and typesetting by our production team. After these production processes are complete and the authors have approved the resulting proofs, the article will move to the latest issue of the ERJ online. Please open or download the PDF to view this article.\n\nConflict of interest: Dr. Galambos has nothing to disclose.\n\nConflict of interest: Mr. Wartchow has nothing to disclose.\n\nConflict of interest: Dr. Weinman has nothing to disclose.\n\nConflict of interest: Dr. Abman has nothing to disclose.