Pulmonary interstitial glycogenosis cells express mesenchymal stem cell markers. Issue 4 (22nd October 2020)

• Record Type: Journal Article
• Authors: Galambos, Csaba; Wartchow, Eric; Weinman, Jason P.; Abman, Steven H.
• Is Part Of: European respiratory journal. Volume 56:Issue 4(2020)
• Publication Date: 2020-10-22
• Subjects: Respiratory organs -- Diseases -- Periodicals; Respiration -- Periodicals; 616.2
• DOI: 10.1183/13993003.00853-2020
• Languages: English
• ISSNs: 0903-1936

Abstract

Pulmonary interstitial glycogenosis (PIG) was first defined as a distinct neonatal interstitial lung disease of unknown aetiology 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 stain-positive, mono-particulate glycogen in the cytoplasm, for which the disease was named ("glycogenosis") [1]. By ultrastructure, PIG cells are considered primitive due to the presence of only sparse organelles and a lack of specific features that indicate differentiation towards any well-characterised pulmonary cell line, including lymphocytes or macrophages [1]. Pulmonary interstitial glycogenesis (PIG) is characterised by a unique but poorly characterised cell population in developmental lung disease. This study reports that PIG cells express cellular markers, suggesting a mesenchymal stem cell lineage. https://bit.ly/2YNdDIY