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Updated: Jun 27, 2026

Refined Murine Model of Idiopathic Pulmonary Fibrosis
Published on: June 17, 2025
Pleural fibrosis mediates shrinking lungs syndrome in children
Esther H Krych1, Philip R Fischer, Mark E Wylam
1Department of Pediatric and Adolescent Medicine, Mayo Clinic, Rochester, MN 55905, USA.
Abstract:
The etiology of restrictive lung physiology in SLS is not well-defined, and has been hypothesized to be due to defects in lung recoil, phrenic nerve function and diaphragmatic strength. We present a case of SLS in an adolescent in whom imaging and electrophysiology studies demonstrate pleural fibrosis as the fundamental defect accounting for the restrictive lung physiology.
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