Pleural fibrosis mediates shrinking lungs syndrome in children

Esther H. Krych; Philip R. Fischer; Mark E. Wylam

doi:10.1002/ppul.20946

Pleural fibrosis mediates shrinking lungs syndrome in children

Esther H. Krych, Philip R. Fischer, Mark E. Wylam

Pulmonary and Critical Care Medicine

Research output: Contribution to journal › Article › peer-review

10 Scopus citations

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.

Original language	English (US)
Pages (from-to)	90-92
Number of pages	3
Journal	Pediatric pulmonology
Volume	44
Issue number	1
DOIs	https://doi.org/10.1002/ppul.20946
State	Published - Jan 2009

Keywords

Pleural fibrosis
SLE/lupus
Shrinking lungs syndrome
Vanishing lungs syndrome
Zone of apposition

ASJC Scopus subject areas

Pediatrics, Perinatology, and Child Health
Pulmonary and Respiratory Medicine

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10.1002/ppul.20946

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title = "Pleural fibrosis mediates shrinking lungs syndrome in children",

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.",

keywords = "Pleural fibrosis, SLE/lupus, Shrinking lungs syndrome, Vanishing lungs syndrome, Zone of apposition",

author = "Krych, {Esther H.} and Fischer, {Philip R.} and Wylam, {Mark E.}",

year = "2009",

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AU - Krych, Esther H.

AU - Fischer, Philip R.

AU - Wylam, Mark E.

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N2 - 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.

AB - 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.

KW - Pleural fibrosis

KW - SLE/lupus

KW - Shrinking lungs syndrome

KW - Vanishing lungs syndrome

KW - Zone of apposition

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Pleural fibrosis mediates shrinking lungs syndrome in children

Abstract

Keywords

ASJC Scopus subject areas

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