Pleural fibrosis mediates shrinking lungs syndrome in children
Identifieur interne : 001B80 ( Istex/Corpus ); précédent : 001B79; suivant : 001B81Pleural fibrosis mediates shrinking lungs syndrome in children
Auteurs : Esther H. Krych ; Philip R. Fischer ; Mark E. WylamSource :
- Pediatric Pulmonology [ 8755-6863 ] ; 2009-01.
English descriptors
- Teeft :
- Adolescent medicine, American college, Apposition, Arthritis rheum, Brosis, Case report, Chest pain, Diaphragm strength, Diaphragmatic, Electrophysiology studies, Erythematosus, Fibrosis mediates, Fundamental defect accounting, Lung syndrome, Lung volumes, Lungs syndrome, Lupus, Lupus erythematosus, Mayo clinic, Pediatr pulmonol, Pediatric pulmonology, Phrenic, Phrenic nerve function, Reference value, Restrictive lung physiology, Rheum, Syndrome, Systemic, Systemic lupus erythematosus.
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. Pediatr Pulmonol. 2009; 44:90–92. © 2008 Wiley‐Liss, Inc.
Url:
DOI: 10.1002/ppul.20946
Links to Exploration step
ISTEX:EAF7A2D0A9C93FBC7FFD6ACD3EFDDF950812705DLe document en format XML
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Additional Supporting Information may be found in the online version of this article.
</p><supportingInfoItem><mediaResource alt="supporting information" href="urn-x:wiley:87556863:media:ppul20946:ppul_20946_sm_suppFig1"></mediaResource><caption>Supplementary Figure 1. Chest CT reveals pleural thickening. Solid arrows indicate thickening of interlobar fissures. Dashed arrows indicate thickening of visceral pleura.</caption></supportingInfoItem><supportingInfoItem><mediaResource alt="supporting information" href="urn-x:wiley:87556863:media:ppul20946:ppul_20946_sm_suppFig2"></mediaResource><caption>Supplementary Figure 2. MRI (arrows) demonstrates thickening of anterior Zone of Apposition.</caption></supportingInfoItem></supportingInformation><abstractGroup><abstract type="main" xml:lang="en"><title type="main">Abstract</title><p>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.</p></abstract></abstractGroup></contentMeta></header></component></istex:document></istex:metadataXml><mods version="3.6"><titleInfo lang="en"><title>Pleural fibrosis mediates shrinking lungs syndrome in children</title></titleInfo><titleInfo type="abbreviated" lang="en"><title>Pleural Fibrosis Mediates Shrinking Lungs Syndrome</title></titleInfo><titleInfo type="alternative" contentType="CDATA" lang="en"><title>Pleural fibrosis mediates shrinking lungs syndrome in children</title></titleInfo><name type="personal"><namePart type="given">Esther H.</namePart><namePart type="family">Krych</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Department of Pediatric and Adolescent Medicine, Mayo Clinic, Rochester, Minnesota</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Philip R.</namePart><namePart type="family">Fischer</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Department of Pediatric and Adolescent Medicine, Mayo Clinic, Rochester, Minnesota</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Mark E.</namePart><namePart type="family">Wylam</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Department of Pediatric and Adolescent Medicine, Mayo Clinic, Rochester, Minnesota</affiliation><affiliation>E-mail: wylam.mark@mayo.edu</affiliation><affiliation>Correspondence address: Department of Pediatrics and Adolescent Medicine, Mayo Clinic, 200 First Street, Gonda 18, Rochester, MN 55905.</affiliation><role><roleTerm type="text">author</roleTerm></role></name><typeOfResource>text</typeOfResource><genre type="case-report" displayLabel="caseStudy" authority="ISTEX" authorityURI="https://content-type.data.istex.fr" valueURI="https://content-type.data.istex.fr/ark:/67375/XTP-29919SZJ-6">case-report</genre><originInfo><publisher>Wiley Subscription Services, Inc., A Wiley Company</publisher><place><placeTerm type="text">Hoboken</placeTerm></place><dateIssued encoding="w3cdtf">2009-01</dateIssued><dateCaptured encoding="w3cdtf">2008-06-30</dateCaptured><dateValid encoding="w3cdtf">2008-08-22</dateValid><copyrightDate encoding="w3cdtf">2009</copyrightDate></originInfo><language><languageTerm type="code" authority="rfc3066">en</languageTerm><languageTerm type="code" authority="iso639-2b">eng</languageTerm></language><physicalDescription><extent unit="figures">1</extent><extent unit="tables">0</extent><extent unit="references">27</extent><extent unit="words">671</extent></physicalDescription><abstract lang="en">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.</abstract><subject lang="en"><genre>keywords</genre><topic>shrinking lungs syndrome</topic><topic>vanishing lungs syndrome</topic><topic>SLE/lupus</topic><topic>pleural fibrosis</topic><topic>zone of apposition</topic></subject><relatedItem type="host"><titleInfo><title>Pediatric Pulmonology</title></titleInfo><titleInfo type="abbreviated"><title>Pediatr. Pulmonol.</title></titleInfo><genre type="journal" authority="ISTEX" authorityURI="https://publication-type.data.istex.fr" valueURI="https://publication-type.data.istex.fr/ark:/67375/JMC-0GLKJH51-B">journal</genre><note type="content"> Additional Supporting Information may be found in the online version of this article.Supporting Info Item: Supplementary Figure 1. Chest CT reveals pleural thickening. Solid arrows indicate thickening of interlobar fissures. Dashed arrows indicate thickening of visceral pleura. - Supplementary Figure 2. 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