Article
Ground glass and fibrotic change in children with surfactant protein C dysfunction mutations.
Pediatric pulmonology - 1 Jul 2021
DeBoer Emily M, Liptzin Deborah R, Humphries Stephen M, Lynch David A, Robison Kyle, Galambos Csaba, Dishop Megan K, Deterding Robin R, Weinman Jason P
Abstract excerpt
INTRODUCTION: Therapeutics exist to treat fibrotic lung disease in adults, but these have not been investigated in children. Defining biomarkers for pediatric fibrotic lung disease in children is crucial for clinical trials. Children with surfactant protein C (SFTPC) dysfunction mutations develop fibrotic lung disease over time. We evaluated chest computed tomography (CT) changes over time in children with SFTPC...
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