Severe lymphatic microangiopathy in Fabry disease.
Identifieur interne : 003E15 ( PubMed/Curation ); précédent : 003E14; suivant : 003E16Severe lymphatic microangiopathy in Fabry disease.
Auteurs : Beatrice R. Amann-Vesti [Suisse] ; Gabriele Gitzelmann ; Urs Widmer ; Nils U. Bosshard ; Beat Steinmann ; Renate KoppensteinerSource :
- Lymphatic research and biology [ 1539-6851 ] ; 2003.
Descripteurs français
- KwdFr :
- Adolescent, Adulte, Adulte d'âge moyen, Endothélium lymphatique (anatomopathologie), Facteurs sexuels, Femelle, Humains, Hétérozygote, Lymphe, Lymphoedème (anatomopathologie), Lymphographie (), Maladie de Fabry (anatomopathologie), Maladie de Fabry (diagnostic), Maladie de Fabry (génétique), Microcirculation, Microscopie de fluorescence, Mâle, Peau (anatomopathologie), Pression, Vaisseaux capillaires (anatomopathologie), Vaisseaux lymphatiques (anatomopathologie).
- MESH :
- anatomopathologie : Endothélium lymphatique, Lymphoedème, Maladie de Fabry, Peau, Vaisseaux capillaires, Vaisseaux lymphatiques.
- diagnostic : Maladie de Fabry.
- génétique : Maladie de Fabry.
- Adolescent, Adulte, Adulte d'âge moyen, Facteurs sexuels, Femelle, Humains, Hétérozygote, Lymphe, Lymphographie, Microcirculation, Microscopie de fluorescence, Mâle, Pression.
English descriptors
- KwdEn :
- Adolescent, Adult, Capillaries (pathology), Endothelium, Lymphatic (pathology), Fabry Disease (diagnosis), Fabry Disease (genetics), Fabry Disease (pathology), Female, Heterozygote, Humans, Lymph, Lymphatic Vessels (pathology), Lymphedema (pathology), Lymphography (methods), Male, Microcirculation, Microscopy, Fluorescence, Middle Aged, Pressure, Sex Factors, Skin (pathology).
- MESH :
- diagnosis : Fabry Disease.
- genetics : Fabry Disease.
- methods : Lymphography.
- pathology : Capillaries, Endothelium, Lymphatic, Fabry Disease, Lymphatic Vessels, Lymphedema, Skin.
- Adolescent, Adult, Female, Heterozygote, Humans, Lymph, Male, Microcirculation, Microscopy, Fluorescence, Middle Aged, Pressure, Sex Factors.
Abstract
Lymphedema has been described in a few cases of Fabry disease. The etiology of lymphedema in Fabry disease is unknown. The aim of the study was to evaluate morphology and function of lymphatic microvessels in this disease.
DOI: 10.1089/153968503768330229
PubMed: 15624436
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Bosshard</name></author><author><name sortKey="Steinmann, Beat" sort="Steinmann, Beat" uniqKey="Steinmann B" first="Beat" last="Steinmann">Beat Steinmann</name></author><author><name sortKey="Koppensteiner, Renate" sort="Koppensteiner, Renate" uniqKey="Koppensteiner R" first="Renate" last="Koppensteiner">Renate Koppensteiner</name></author></titleStmt><publicationStmt><idno type="wicri:source">PubMed</idno><date when="2003">2003</date><idno type="RBID">pubmed:15624436</idno><idno type="pmid">15624436</idno><idno type="doi">10.1089/153968503768330229</idno><idno type="wicri:Area/PubMed/Corpus">003E15</idno><idno type="wicri:explorRef" wicri:stream="PubMed" wicri:step="Corpus" wicri:corpus="PubMed">003E15</idno><idno type="wicri:Area/PubMed/Curation">003E15</idno><idno type="wicri:explorRef" wicri:stream="PubMed" wicri:step="Curation">003E15</idno></publicationStmt><sourceDesc><biblStruct><analytic><title xml:lang="en">Severe lymphatic microangiopathy in Fabry disease.</title><author><name sortKey="Amann Vesti, Beatrice R" sort="Amann Vesti, Beatrice R" uniqKey="Amann Vesti B" first="Beatrice R" last="Amann-Vesti">Beatrice R. Amann-Vesti</name><affiliation wicri:level="1"><nlm:affiliation>University Hospital of Zurich, Zurich, Switzerland. beatrice.amann@usz.ch</nlm:affiliation><country xml:lang="fr">Suisse</country><wicri:regionArea>University Hospital of Zurich, Zurich</wicri:regionArea></affiliation></author><author><name sortKey="Gitzelmann, Gabriele" sort="Gitzelmann, Gabriele" uniqKey="Gitzelmann G" first="Gabriele" last="Gitzelmann">Gabriele Gitzelmann</name></author><author><name sortKey="Widmer, Urs" sort="Widmer, Urs" uniqKey="Widmer U" first="Urs" last="Widmer">Urs Widmer</name></author><author><name sortKey="Bosshard, Nils U" sort="Bosshard, Nils U" uniqKey="Bosshard N" first="Nils U" last="Bosshard">Nils U. Bosshard</name></author><author><name sortKey="Steinmann, Beat" sort="Steinmann, Beat" uniqKey="Steinmann B" first="Beat" last="Steinmann">Beat Steinmann</name></author><author><name sortKey="Koppensteiner, Renate" sort="Koppensteiner, Renate" uniqKey="Koppensteiner R" first="Renate" last="Koppensteiner">Renate Koppensteiner</name></author></analytic><series><title level="j">Lymphatic research and biology</title><idno type="ISSN">1539-6851</idno><imprint><date when="2003" type="published">2003</date></imprint></series></biblStruct></sourceDesc></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Adolescent</term><term>Adult</term><term>Capillaries (pathology)</term><term>Endothelium, Lymphatic (pathology)</term><term>Fabry Disease (diagnosis)</term><term>Fabry Disease (genetics)</term><term>Fabry Disease (pathology)</term><term>Female</term><term>Heterozygote</term><term>Humans</term><term>Lymph</term><term>Lymphatic Vessels (pathology)</term><term>Lymphedema (pathology)</term><term>Lymphography (methods)</term><term>Male</term><term>Microcirculation</term><term>Microscopy, Fluorescence</term><term>Middle Aged</term><term>Pressure</term><term>Sex Factors</term><term>Skin (pathology)</term></keywords><keywords scheme="KwdFr" xml:lang="fr"><term>Adolescent</term><term>Adulte</term><term>Adulte d'âge moyen</term><term>Endothélium lymphatique (anatomopathologie)</term><term>Facteurs sexuels</term><term>Femelle</term><term>Humains</term><term>Hétérozygote</term><term>Lymphe</term><term>Lymphoedème (anatomopathologie)</term><term>Lymphographie ()</term><term>Maladie de Fabry (anatomopathologie)</term><term>Maladie de Fabry (diagnostic)</term><term>Maladie de Fabry (génétique)</term><term>Microcirculation</term><term>Microscopie de fluorescence</term><term>Mâle</term><term>Peau (anatomopathologie)</term><term>Pression</term><term>Vaisseaux capillaires (anatomopathologie)</term><term>Vaisseaux lymphatiques (anatomopathologie)</term></keywords><keywords scheme="MESH" qualifier="anatomopathologie" xml:lang="fr"><term>Endothélium lymphatique</term><term>Lymphoedème</term><term>Maladie de Fabry</term><term>Peau</term><term>Vaisseaux capillaires</term><term>Vaisseaux lymphatiques</term></keywords><keywords scheme="MESH" qualifier="diagnosis" xml:lang="en"><term>Fabry Disease</term></keywords><keywords scheme="MESH" qualifier="diagnostic" xml:lang="fr"><term>Maladie de Fabry</term></keywords><keywords scheme="MESH" qualifier="genetics" xml:lang="en"><term>Fabry Disease</term></keywords><keywords scheme="MESH" qualifier="génétique" xml:lang="fr"><term>Maladie de Fabry</term></keywords><keywords scheme="MESH" qualifier="methods" xml:lang="en"><term>Lymphography</term></keywords><keywords scheme="MESH" qualifier="pathology" xml:lang="en"><term>Capillaries</term><term>Endothelium, Lymphatic</term><term>Fabry Disease</term><term>Lymphatic Vessels</term><term>Lymphedema</term><term>Skin</term></keywords><keywords scheme="MESH" xml:lang="en"><term>Adolescent</term><term>Adult</term><term>Female</term><term>Heterozygote</term><term>Humans</term><term>Lymph</term><term>Male</term><term>Microcirculation</term><term>Microscopy, Fluorescence</term><term>Middle Aged</term><term>Pressure</term><term>Sex Factors</term></keywords><keywords scheme="MESH" xml:lang="fr"><term>Adolescent</term><term>Adulte</term><term>Adulte d'âge moyen</term><term>Facteurs sexuels</term><term>Femelle</term><term>Humains</term><term>Hétérozygote</term><term>Lymphe</term><term>Lymphographie</term><term>Microcirculation</term><term>Microscopie de fluorescence</term><term>Mâle</term><term>Pression</term></keywords></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">Lymphedema has been described in a few cases of Fabry disease. The etiology of lymphedema in Fabry disease is unknown. The aim of the study was to evaluate morphology and function of lymphatic microvessels in this disease.</div></front></TEI><pubmed><MedlineCitation Status="MEDLINE" Owner="NLM"><PMID Version="1">15624436</PMID><DateCreated><Year>2004</Year><Month>12</Month><Day>30</Day></DateCreated><DateCompleted><Year>2005</Year><Month>02</Month><Day>01</Day></DateCompleted><DateRevised><Year>2006</Year><Month>11</Month><Day>15</Day></DateRevised><Article PubModel="Print"><Journal><ISSN IssnType="Print">1539-6851</ISSN><JournalIssue CitedMedium="Print"><Volume>1</Volume><Issue>3</Issue><PubDate><Year>2003</Year></PubDate></JournalIssue><Title>Lymphatic research and biology</Title><ISOAbbreviation>Lymphat Res Biol</ISOAbbreviation></Journal><ArticleTitle>Severe lymphatic microangiopathy in Fabry disease.</ArticleTitle><Pagination><MedlinePgn>185-9</MedlinePgn></Pagination><Abstract><AbstractText Label="BACKGROUND" NlmCategory="BACKGROUND">Lymphedema has been described in a few cases of Fabry disease. The etiology of lymphedema in Fabry disease is unknown. The aim of the study was to evaluate morphology and function of lymphatic microvessels in this disease.</AbstractText><AbstractText Label="METHODS AND RESULTS" NlmCategory="RESULTS">In five male patients with Fabry disease, the initial lymphatic microvessels of the skin were studied in vivo, using a nearly atraumatic technique of fluorescence microlymphography and measurement of lymph capillary pressure. In addition, five female patients heterozygous for Fabry disease and 12 healthy controls were studied. The maximum spread of the fluorescent macromolecular dye into the network of superficial skin lymphatics was increased in the three male patients presenting with lymphedema (25, 26, and 45 mm, respectively). In the two male patients without swollen legs, the maximum spread of the dye was 3 and 7 mm, respectively, and in the female patients 8.8 mm (range, 4-17 mm), whereas in the healthy controls it reached only 4.3 mm (range, 1-7 mm). Fragmentation of the microlymphatic network was found in all patients, but not in controls. In controls, the diameter of the microvessels varied in a very narrow range (45-75 microm); in patients, the range was 15-150 microm. In patients with lymphedema, microlymphatic hypertension was present.</AbstractText><AbstractText Label="CONCLUSION" NlmCategory="CONCLUSIONS">In patients with Fabry disease severe structural and functional changes of the initial lymphatics of the skin are present.</AbstractText></Abstract><AuthorList CompleteYN="Y"><Author ValidYN="Y"><LastName>Amann-Vesti</LastName><ForeName>Beatrice R</ForeName><Initials>BR</Initials><AffiliationInfo><Affiliation>University Hospital of Zurich, Zurich, Switzerland. beatrice.amann@usz.ch</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Gitzelmann</LastName><ForeName>Gabriele</ForeName><Initials>G</Initials></Author><Author ValidYN="Y"><LastName>Widmer</LastName><ForeName>Urs</ForeName><Initials>U</Initials></Author><Author ValidYN="Y"><LastName>Bosshard</LastName><ForeName>Nils U</ForeName><Initials>NU</Initials></Author><Author ValidYN="Y"><LastName>Steinmann</LastName><ForeName>Beat</ForeName><Initials>B</Initials></Author><Author ValidYN="Y"><LastName>Koppensteiner</LastName><ForeName>Renate</ForeName><Initials>R</Initials></Author></AuthorList><Language>eng</Language><PublicationTypeList><PublicationType UI="D016428">Journal Article</PublicationType><PublicationType UI="D013485">Research Support, Non-U.S. Gov't</PublicationType></PublicationTypeList></Article><MedlineJournalInfo><Country>United States</Country><MedlineTA>Lymphat Res Biol</MedlineTA><NlmUniqueID>101163587</NlmUniqueID><ISSNLinking>1539-6851</ISSNLinking></MedlineJournalInfo><CitationSubset>IM</CitationSubset><MeshHeadingList><MeshHeading><DescriptorName UI="D000293" MajorTopicYN="N">Adolescent</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D000328" MajorTopicYN="N">Adult</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D002196" MajorTopicYN="N">Capillaries</DescriptorName><QualifierName UI="Q000473" MajorTopicYN="N">pathology</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D004729" MajorTopicYN="N">Endothelium, Lymphatic</DescriptorName><QualifierName UI="Q000473" MajorTopicYN="N">pathology</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D000795" MajorTopicYN="N">Fabry Disease</DescriptorName><QualifierName UI="Q000175" MajorTopicYN="Y">diagnosis</QualifierName><QualifierName UI="Q000235" MajorTopicYN="Y">genetics</QualifierName><QualifierName UI="Q000473" MajorTopicYN="Y">pathology</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D005260" MajorTopicYN="N">Female</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D006579" MajorTopicYN="N">Heterozygote</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D006801" MajorTopicYN="N">Humans</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D008196" MajorTopicYN="N">Lymph</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D042601" MajorTopicYN="N">Lymphatic Vessels</DescriptorName><QualifierName UI="Q000473" MajorTopicYN="Y">pathology</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D008209" MajorTopicYN="N">Lymphedema</DescriptorName><QualifierName UI="Q000473" MajorTopicYN="Y">pathology</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D008220" MajorTopicYN="N">Lymphography</DescriptorName><QualifierName UI="Q000379" MajorTopicYN="N">methods</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D008297" MajorTopicYN="N">Male</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D008833" MajorTopicYN="N">Microcirculation</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D008856" MajorTopicYN="N">Microscopy, Fluorescence</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D008875" MajorTopicYN="N">Middle Aged</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D011312" MajorTopicYN="N">Pressure</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D012737" MajorTopicYN="N">Sex Factors</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D012867" MajorTopicYN="N">Skin</DescriptorName><QualifierName UI="Q000473" MajorTopicYN="N">pathology</QualifierName></MeshHeading></MeshHeadingList></MedlineCitation><PubmedData><History><PubMedPubDate PubStatus="pubmed"><Year>2004</Year><Month>12</Month><Day>31</Day><Hour>9</Hour><Minute>0</Minute></PubMedPubDate><PubMedPubDate PubStatus="medline"><Year>2005</Year><Month>2</Month><Day>3</Day><Hour>9</Hour><Minute>0</Minute></PubMedPubDate><PubMedPubDate PubStatus="entrez"><Year>2004</Year><Month>12</Month><Day>31</Day><Hour>9</Hour><Minute>0</Minute></PubMedPubDate></History><PublicationStatus>ppublish</PublicationStatus><ArticleIdList><ArticleId IdType="pubmed">15624436</ArticleId><ArticleId IdType="doi">10.1089/153968503768330229</ArticleId></ArticleIdList></PubmedData></pubmed></record>Pour manipuler ce document sous Unix (Dilib)
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