Hyperlucent lung secondary to homocystinuria
Identifieur interne : 002A40 ( Main/Exploration ); précédent : 002A39; suivant : 002A41Hyperlucent lung secondary to homocystinuria
Auteurs : J. Herman [États-Unis] ; J. H. Miller [États-Unis] ; F. Wang [États-Unis]Source :
- Pediatric Radiology [ 0301-0449 ] ; 1996-09-01.
English descriptors
- Teeft :
- Abnormality, Bronchiolitis obliterans, Cystathionine, Homocystine, Homocystinuria, Hyperlucent, Hyperlucent lung, Irvine, Lung fields, Lung scanning, Nephrotic syndrome, Pediatr radiol, Perfusion, Perfusion scintiphoto, Pulmonary arteries, Right lung, Right side, Skeletal abnormalities, Syndrome, Thrombosis, Uniform ventilation.
Abstract
Abstract: At 23 months of age, one of a pair of monozygotic twins with radiographic unilateral hyperlucent lung was evaluated by radionuclide ventilation/perfusion pulmonary studies, which revealed a ventilation/perfusion mismatch of an entire lung. This twin died, and autopsy revealed pulmonary arterial thrombosis and histological changes compatible with homocystinuria, which was subsequently shown to be present in the surviving twin as well. A ventilation/perfusion lung scan of the surviving twin revealed multiple ventilation/perfusion mismatched defects, suggestive of pulmonary embolism. The presenting manifestation of homocystinuria in these patients was the pulmonary thrombotic disease. Neither twin had any other stigmata of homocystinuria at the time of initial presentation.
Url:
DOI: 10.1007/BF01356832
Affiliations:
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Le document en format XML
<record><TEI wicri:istexFullTextTei="biblStruct"><teiHeader><fileDesc><titleStmt><title xml:lang="en">Hyperlucent lung secondary to homocystinuria</title><author><name sortKey="Herman, J" sort="Herman, J" uniqKey="Herman J" first="J." last="Herman">J. Herman</name></author><author><name sortKey="Miller, J H" sort="Miller, J H" uniqKey="Miller J" first="J. H." last="Miller">J. H. Miller</name></author><author><name sortKey="Wang, F" sort="Wang, F" uniqKey="Wang F" first="F." last="Wang">F. Wang</name></author></titleStmt><publicationStmt><idno type="wicri:source">ISTEX</idno><idno type="RBID">ISTEX:769EF9E6E3F7D9E3EC4EC808FE37D3BBC1294405</idno><date when="1996" year="1996">1996</date><idno type="doi">10.1007/BF01356832</idno><idno type="url">https://api.istex.fr/ark:/67375/1BB-51J2N1S8-T/fulltext.pdf</idno><idno type="wicri:Area/Istex/Corpus">001762</idno><idno type="wicri:explorRef" wicri:stream="Istex" wicri:step="Corpus" wicri:corpus="ISTEX">001762</idno><idno type="wicri:Area/Istex/Curation">001762</idno><idno type="wicri:Area/Istex/Checkpoint">001830</idno><idno type="wicri:explorRef" wicri:stream="Istex" wicri:step="Checkpoint">001830</idno><idno type="wicri:doubleKey">0301-0449:1996:Herman J:hyperlucent:lung:secondary</idno><idno type="wicri:Area/Main/Merge">002A92</idno><idno type="wicri:Area/Main/Curation">002A40</idno><idno type="wicri:Area/Main/Exploration">002A40</idno></publicationStmt><sourceDesc><biblStruct><analytic><title level="a" type="main" xml:lang="en">Hyperlucent lung secondary to homocystinuria</title><author><name sortKey="Herman, J" sort="Herman, J" uniqKey="Herman J" first="J." last="Herman">J. Herman</name><affiliation wicri:level="2"><country xml:lang="fr">États-Unis</country><wicri:regionArea>Irvine Medical Center, University of California, Irvine, California</wicri:regionArea><placeName><region type="state">Californie</region></placeName></affiliation></author><author><name sortKey="Miller, J H" sort="Miller, J H" uniqKey="Miller J" first="J. H." last="Miller">J. H. Miller</name><affiliation wicri:level="2"><country xml:lang="fr">États-Unis</country><wicri:regionArea>Division of Nuclear Radiology, No. 49, Childrens Hospital Los Angeles, 4650 Sunset Boulevard, 90027, Los Angeles, CA</wicri:regionArea><placeName><region type="state">Californie</region></placeName></affiliation></author><author><name sortKey="Wang, F" sort="Wang, F" uniqKey="Wang F" first="F." last="Wang">F. Wang</name><affiliation wicri:level="2"><country xml:lang="fr">États-Unis</country><wicri:regionArea>Department of Radiology, University of California, Irvine Medical Center, Irvine, California</wicri:regionArea><placeName><region type="state">Californie</region></placeName></affiliation></author></analytic><monogr></monogr><series><title level="j">Pediatric Radiology</title><title level="j" type="sub">Roentgenology, Nuclear Medicine, Ultrasound, CT, MRI</title><title level="j" type="abbrev">Pediatr Radiol</title><idno type="ISSN">0301-0449</idno><idno type="eISSN">1432-1998</idno><imprint><publisher>Springer-Verlag</publisher><pubPlace>Berlin/Heidelberg</pubPlace><date type="published" when="1996-09-01">1996-09-01</date><biblScope unit="volume">26</biblScope><biblScope unit="issue">9</biblScope><biblScope unit="page" from="672">672</biblScope><biblScope unit="page" to="674">674</biblScope></imprint><idno type="ISSN">0301-0449</idno></series></biblStruct></sourceDesc><seriesStmt><idno type="ISSN">0301-0449</idno></seriesStmt></fileDesc><profileDesc><textClass><keywords scheme="Teeft" xml:lang="en"><term>Abnormality</term><term>Bronchiolitis obliterans</term><term>Cystathionine</term><term>Homocystine</term><term>Homocystinuria</term><term>Hyperlucent</term><term>Hyperlucent lung</term><term>Irvine</term><term>Lung fields</term><term>Lung scanning</term><term>Nephrotic syndrome</term><term>Pediatr radiol</term><term>Perfusion</term><term>Perfusion scintiphoto</term><term>Pulmonary arteries</term><term>Right lung</term><term>Right side</term><term>Skeletal abnormalities</term><term>Syndrome</term><term>Thrombosis</term><term>Uniform ventilation</term></keywords></textClass><langUsage><language ident="en">en</language></langUsage></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">Abstract: At 23 months of age, one of a pair of monozygotic twins with radiographic unilateral hyperlucent lung was evaluated by radionuclide ventilation/perfusion pulmonary studies, which revealed a ventilation/perfusion mismatch of an entire lung. This twin died, and autopsy revealed pulmonary arterial thrombosis and histological changes compatible with homocystinuria, which was subsequently shown to be present in the surviving twin as well. A ventilation/perfusion lung scan of the surviving twin revealed multiple ventilation/perfusion mismatched defects, suggestive of pulmonary embolism. The presenting manifestation of homocystinuria in these patients was the pulmonary thrombotic disease. Neither twin had any other stigmata of homocystinuria at the time of initial presentation.</div></front></TEI><affiliations><list><country><li>États-Unis</li></country><region><li>Californie</li></region></list><tree><country name="États-Unis"><region name="Californie"><name sortKey="Herman, J" sort="Herman, J" uniqKey="Herman J" first="J." last="Herman">J. Herman</name></region><name sortKey="Miller, J H" sort="Miller, J H" uniqKey="Miller J" first="J. H." last="Miller">J. H. Miller</name><name sortKey="Wang, F" sort="Wang, F" uniqKey="Wang F" first="F." last="Wang">F. Wang</name></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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