AI Networking Cookbook: Practical recipes for AI-assisted network automation and development
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In routine care, CTD-ILD showed similar TFS but better PFS and less FVC decline than non-CTD ILD, whereas antifibrotic use was similar between groups.
BACKGROUND Pleuroparenchymal fibroelastosis (PPFE) is a rare, most often idiopathic fibrotic lung disease with poor prognosis, mainly affecting adults. Biallelic pathogenic variants in the deoxyguanosine kinase gene (DGUOK) cause a mitochondrial DNA (mtDNA) depletion syndrome, predominantly affecting infants with sever...
BOS engages structural-cell circuits largely shared with RAS and fibrotic lung diseases, but along an airway-centered rather than parenchyma-centered axis, and shared structural programs may therefore be targetable across CLAD phenotypes.
A potential involvement of vascular remodelling in CLAD pathophysiology is suggested, with changes consistent with pro-inflammatory and fibrogenic processes in CD31+ regions.
Considering the multifaceted differences of RAS and idiopathic pulmonary fibrosis, a surprising general principle of an entity-spanning composition of the fibrotic niche by aberrant basaloid cells localized at the fibrotic edge, ectopic COL15A1+ pVECs and CTHRC1+ fibrotic fibroblasts is revealed.
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