Pleural fibrosis mediates shrinking lungs syndrome in children.
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.
Krych
Esther H
EH
Department of Pediatric and Adolescent Medicine, Mayo Clinic, Rochester, MN 55905, USA.
Fischer
Philip R
PR
Wylam
Mark E
ME
D002648:Child; D005260:Female; D005355:Fibrosis; D006801:Humans; D008171:Lung Diseases / Q000000981:diagnostic imaging / Q000473:pathology*; D010995:Pleural Diseases / Q000000981:diagnostic imaging / Q000473:pathology*; D011859:Radiography; D013577:Syndrome