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 fibros...

Ausführliche Beschreibung

Gespeichert in:

Bibliographische Detailangaben
Veröffentlicht in:	Pediatric pulmonology 2009-01, Vol.44 (1), p.90-92
Hauptverfasser:	Krych, Esther H., Fischer, Philip R., Wylam, Mark E.
Format:	Artikel
Sprache:	eng
Schlagworte:	Biological and medical sciences Child Dermatology Female Fibrosis General aspects Humans Lung Diseases - diagnostic imaging Lung Diseases - pathology Medical sciences Pleural Diseases - diagnostic imaging Pleural Diseases - pathology pleural fibrosis Pneumology Radiography Respiratory system : syndromes and miscellaneous diseases shrinking lungs syndrome Skin involvement in other diseases. Miscellaneous. General aspects SLE/lupus Syndrome vanishing lungs syndrome zone of apposition
Online-Zugang:	Volltext
Tags:	Tag hinzufügen Keine Tags, Fügen Sie den ersten Tag hinzu!

Beschreibung
Zusammenfassung:	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. Pediatr Pulmonol. 2009; 44:90–92. © 2008 Wiley‐Liss, Inc.
ISSN:	8755-6863 1099-0496
DOI:	10.1002/ppul.20946