Jan C Kamp, Lavinia Neubert, Jonas C Schupp, Peter Braubach, Christoph Wrede, Florian Laenger, Tim Salditt, Jakob Reichmann, Tobias Welte, Arjang Ruhparwar, Fabio Ius, Nicolaus Schwerk, Anke K Bergmann, Sandra von Hardenberg, Matthias Griese, Christina Rapp, Karen M Olsson, Jan Fuge, Da-Hee Park, Marius M Hoeper, Danny D Jonigk, Lars Knudsen, Mark P Kuehnel
A minimal diffusion barrier is key to the pulmonary gas exchange. In alveolar capillary dysplasia (ACD), a rare genetically driven disease of early infancy, this crucial fibrovascular interface is compromised while the underlying pathophysiology is insufficiently understood. Recent in-depth analyses of vascular alterations in adult lung disease encouraged to extend these studies to ACD and compare the changes of the microvasculature. Lung tissue samples of children with ACD (n=12), adults with non-specific interstitial pneumonia (n=12), and controls (n=20) were studied using single gene sequencing, immunostaining, exome sequencing, and broad transcriptome profiling in addition to analysis of the vasculature by transmission electron microscopy...
November 27, 2023: American Journal of Pathology