Hereditary lymphedema, characteristics, and variations in 17 adult patients with lymphedema cholestasis syndrome 1/Aagenaes syndrome.
Identifieur interne : 001256 ( PubMed/Curation ); précédent : 001255; suivant : 001257Hereditary lymphedema, characteristics, and variations in 17 adult patients with lymphedema cholestasis syndrome 1/Aagenaes syndrome.
Auteurs : Monica Drivdal [Norvège] ; Carl-Erik Slagsvold ; Oystein Aagenaes ; Bengt Frode KaseSource :
- Lymphatic research and biology [ 1557-8585 ] ; 2014.
Descripteurs français
- KwdFr :
- Adulte, Adulte d'âge moyen, Cholestase (), Eau corporelle, Femelle, Humains, Indice de masse corporelle, Jeune adulte, Lymphoedème (), Lymphoedème (diagnostic), Lymphoedème (métabolisme), Lymphoedème (étiologie), Marqueurs biologiques (métabolisme), Mâle, Pronostic, Études cas-témoins, Études de suivi, Études transversales.
- MESH :
- diagnostic : Lymphoedème.
- métabolisme : Lymphoedème, Marqueurs biologiques.
- étiologie : Lymphoedème.
- Adulte, Adulte d'âge moyen, Cholestase, Eau corporelle, Femelle, Humains, Indice de masse corporelle, Jeune adulte, Lymphoedème, Mâle, Pronostic, Études cas-témoins, Études de suivi, Études transversales.
English descriptors
- KwdEn :
- Adult, Biomarkers (metabolism), Body Mass Index, Body Water, Case-Control Studies, Cholestasis (complications), Cross-Sectional Studies, Female, Follow-Up Studies, Humans, Lymphedema (complications), Lymphedema (diagnosis), Lymphedema (etiology), Lymphedema (metabolism), Male, Middle Aged, Prognosis, Young Adult.
- MESH :
- chemical , metabolism : Biomarkers.
- complications : Cholestasis, Lymphedema.
- diagnosis : Lymphedema.
- etiology : Lymphedema.
- metabolism : Lymphedema.
- Adult, Body Mass Index, Body Water, Case-Control Studies, Cross-Sectional Studies, Female, Follow-Up Studies, Humans, Male, Middle Aged, Prognosis, Young Adult.
Abstract
The characterizations of primary lymphedemas in different hereditary diseases are often published as case reports. In this study, 17 out of 20 Norweigian adult patients with lymphedema cholestasis syndrome 1 (LCS1)/Aagenaes syndrome were examined. The patients exhibited lymphedema and sporadic cholestasis. Individual clinical variations are described.
DOI: 10.1089/lrb.2014.0003
PubMed: 25317502
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sort="Kase, Bengt Frode" uniqKey="Kase B" first="Bengt Frode" last="Kase">Bengt Frode Kase</name></author></titleStmt><publicationStmt><idno type="wicri:source">PubMed</idno><date when="2014">2014</date><idno type="RBID">pubmed:25317502</idno><idno type="pmid">25317502</idno><idno type="doi">10.1089/lrb.2014.0003</idno><idno type="wicri:Area/PubMed/Corpus">001256</idno><idno type="wicri:explorRef" wicri:stream="PubMed" wicri:step="Corpus" wicri:corpus="PubMed">001256</idno><idno type="wicri:Area/PubMed/Curation">001256</idno><idno type="wicri:explorRef" wicri:stream="PubMed" wicri:step="Curation">001256</idno></publicationStmt><sourceDesc><biblStruct><analytic><title xml:lang="en">Hereditary lymphedema, characteristics, and variations in 17 adult patients with lymphedema cholestasis syndrome 1/Aagenaes syndrome.</title><author><name sortKey="Drivdal, Monica" sort="Drivdal, Monica" uniqKey="Drivdal M" first="Monica" last="Drivdal">Monica Drivdal</name><affiliation wicri:level="1"><nlm:affiliation>1 Centre for Rare Disorders, Oslo University Hospital , Oslo, Norway .</nlm:affiliation><country xml:lang="fr">Norvège</country><wicri:regionArea>1 Centre for Rare Disorders, Oslo University Hospital , Oslo</wicri:regionArea></affiliation></author><author><name sortKey="Slagsvold, Carl Erik" sort="Slagsvold, Carl Erik" uniqKey="Slagsvold C" first="Carl-Erik" last="Slagsvold">Carl-Erik Slagsvold</name></author><author><name sortKey="Aagenaes, Oystein" sort="Aagenaes, Oystein" uniqKey="Aagenaes O" first="Oystein" last="Aagenaes">Oystein Aagenaes</name></author><author><name sortKey="Kase, Bengt Frode" sort="Kase, Bengt Frode" uniqKey="Kase B" first="Bengt Frode" last="Kase">Bengt Frode Kase</name></author></analytic><series><title level="j">Lymphatic research and biology</title><idno type="eISSN">1557-8585</idno><imprint><date when="2014" type="published">2014</date></imprint></series></biblStruct></sourceDesc></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Adult</term><term>Biomarkers (metabolism)</term><term>Body Mass Index</term><term>Body Water</term><term>Case-Control Studies</term><term>Cholestasis (complications)</term><term>Cross-Sectional Studies</term><term>Female</term><term>Follow-Up Studies</term><term>Humans</term><term>Lymphedema (complications)</term><term>Lymphedema (diagnosis)</term><term>Lymphedema (etiology)</term><term>Lymphedema (metabolism)</term><term>Male</term><term>Middle Aged</term><term>Prognosis</term><term>Young Adult</term></keywords><keywords scheme="KwdFr" xml:lang="fr"><term>Adulte</term><term>Adulte d'âge moyen</term><term>Cholestase ()</term><term>Eau corporelle</term><term>Femelle</term><term>Humains</term><term>Indice de masse corporelle</term><term>Jeune adulte</term><term>Lymphoedème ()</term><term>Lymphoedème (diagnostic)</term><term>Lymphoedème (métabolisme)</term><term>Lymphoedème (étiologie)</term><term>Marqueurs biologiques (métabolisme)</term><term>Mâle</term><term>Pronostic</term><term>Études cas-témoins</term><term>Études de suivi</term><term>Études transversales</term></keywords><keywords scheme="MESH" type="chemical" qualifier="metabolism" xml:lang="en"><term>Biomarkers</term></keywords><keywords scheme="MESH" qualifier="complications" xml:lang="en"><term>Cholestasis</term><term>Lymphedema</term></keywords><keywords scheme="MESH" qualifier="diagnosis" xml:lang="en"><term>Lymphedema</term></keywords><keywords scheme="MESH" qualifier="diagnostic" xml:lang="fr"><term>Lymphoedème</term></keywords><keywords scheme="MESH" qualifier="etiology" xml:lang="en"><term>Lymphedema</term></keywords><keywords scheme="MESH" qualifier="metabolism" xml:lang="en"><term>Lymphedema</term></keywords><keywords scheme="MESH" qualifier="métabolisme" xml:lang="fr"><term>Lymphoedème</term><term>Marqueurs biologiques</term></keywords><keywords scheme="MESH" qualifier="étiologie" xml:lang="fr"><term>Lymphoedème</term></keywords><keywords scheme="MESH" xml:lang="en"><term>Adult</term><term>Body Mass Index</term><term>Body Water</term><term>Case-Control Studies</term><term>Cross-Sectional Studies</term><term>Female</term><term>Follow-Up Studies</term><term>Humans</term><term>Male</term><term>Middle Aged</term><term>Prognosis</term><term>Young Adult</term></keywords><keywords scheme="MESH" xml:lang="fr"><term>Adulte</term><term>Adulte d'âge moyen</term><term>Cholestase</term><term>Eau corporelle</term><term>Femelle</term><term>Humains</term><term>Indice de masse corporelle</term><term>Jeune adulte</term><term>Lymphoedème</term><term>Mâle</term><term>Pronostic</term><term>Études cas-témoins</term><term>Études de suivi</term><term>Études transversales</term></keywords></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">The characterizations of primary lymphedemas in different hereditary diseases are often published as case reports. In this study, 17 out of 20 Norweigian adult patients with lymphedema cholestasis syndrome 1 (LCS1)/Aagenaes syndrome were examined. The patients exhibited lymphedema and sporadic cholestasis. Individual clinical variations are described.</div></front></TEI><pubmed><MedlineCitation Status="MEDLINE" Owner="NLM"><PMID Version="1">25317502</PMID><DateCreated><Year>2014</Year><Month>12</Month><Day>16</Day></DateCreated><DateCompleted><Year>2015</Year><Month>08</Month><Day>18</Day></DateCompleted><DateRevised><Year>2015</Year><Month>11</Month><Day>19</Day></DateRevised><Article PubModel="Print"><Journal><ISSN IssnType="Electronic">1557-8585</ISSN><JournalIssue CitedMedium="Internet"><Volume>12</Volume><Issue>4</Issue><PubDate><Year>2014</Year><Month>Dec</Month></PubDate></JournalIssue><Title>Lymphatic research and biology</Title><ISOAbbreviation>Lymphat Res Biol</ISOAbbreviation></Journal><ArticleTitle>Hereditary lymphedema, characteristics, and variations in 17 adult patients with lymphedema cholestasis syndrome 1/Aagenaes syndrome.</ArticleTitle><Pagination><MedlinePgn>251-7</MedlinePgn></Pagination><ELocationID EIdType="doi" ValidYN="Y">10.1089/lrb.2014.0003</ELocationID><Abstract><AbstractText Label="BACKGROUND" NlmCategory="BACKGROUND">The characterizations of primary lymphedemas in different hereditary diseases are often published as case reports. In this study, 17 out of 20 Norweigian adult patients with lymphedema cholestasis syndrome 1 (LCS1)/Aagenaes syndrome were examined. The patients exhibited lymphedema and sporadic cholestasis. Individual clinical variations are described.</AbstractText><AbstractText Label="METHODS AND RESULTS" NlmCategory="RESULTS">Lymphedema was classified from Grade I to IV by clinical examinations and ultrasound B-mode scanning. To support the clinical findings, direct segmental multifrequency bioelectrical impedance analysis (DSM-BIA) was included and was compared to healthy matched controls. The lymphedema was similar to other hereditary lymphedemas, with more pronounced fluid retention in the lower extremities. It was generally more extensive, as it also included lymphedema in the arms, face, and trunk. Limited tissue fibrosis was observed, even after long-standing lymphedema.</AbstractText><AbstractText Label="CONCLUSIONS" NlmCategory="CONCLUSIONS">Approximately one-third of the patients had severe forms of lymphedema in the limbs (grades III and IV) and their conditions required close followup. A more frequent use of compression in the upper extremities is advised.</AbstractText></Abstract><AuthorList CompleteYN="Y"><Author ValidYN="Y"><LastName>Drivdal</LastName><ForeName>Monica</ForeName><Initials>M</Initials><AffiliationInfo><Affiliation>1 Centre for Rare Disorders, Oslo University Hospital , Oslo, Norway .</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Slagsvold</LastName><ForeName>Carl-Erik</ForeName><Initials>CE</Initials></Author><Author ValidYN="Y"><LastName>Aagenaes</LastName><ForeName>Oystein</ForeName><Initials>O</Initials></Author><Author ValidYN="Y"><LastName>Kase</LastName><ForeName>Bengt Frode</ForeName><Initials>BF</Initials></Author></AuthorList><Language>eng</Language><PublicationTypeList><PublicationType UI="D003160">Comparative Study</PublicationType><PublicationType UI="D016428">Journal Article</PublicationType></PublicationTypeList></Article><MedlineJournalInfo><Country>United States</Country><MedlineTA>Lymphat Res Biol</MedlineTA><NlmUniqueID>101163587</NlmUniqueID><ISSNLinking>1539-6851</ISSNLinking></MedlineJournalInfo><ChemicalList><Chemical><RegistryNumber>0</RegistryNumber><NameOfSubstance UI="D015415">Biomarkers</NameOfSubstance></Chemical></ChemicalList><SupplMeshList><SupplMeshName Type="Disease" UI="C535330">Aagenaes syndrome</SupplMeshName></SupplMeshList><CitationSubset>IM</CitationSubset><MeshHeadingList><MeshHeading><DescriptorName UI="D000328" MajorTopicYN="N">Adult</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D015415" MajorTopicYN="N">Biomarkers</DescriptorName><QualifierName UI="Q000378" MajorTopicYN="Y">metabolism</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D015992" MajorTopicYN="N">Body Mass Index</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D001834" MajorTopicYN="Y">Body Water</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D016022" MajorTopicYN="N">Case-Control Studies</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D002779" MajorTopicYN="N">Cholestasis</DescriptorName><QualifierName UI="Q000150" MajorTopicYN="Y">complications</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D003430" MajorTopicYN="N">Cross-Sectional Studies</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D005260" MajorTopicYN="N">Female</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D005500" MajorTopicYN="N">Follow-Up Studies</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D006801" MajorTopicYN="N">Humans</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D008209" MajorTopicYN="N">Lymphedema</DescriptorName><QualifierName UI="Q000150" MajorTopicYN="N">complications</QualifierName><QualifierName UI="Q000175" MajorTopicYN="N">diagnosis</QualifierName><QualifierName UI="Q000209" MajorTopicYN="Y">etiology</QualifierName><QualifierName UI="Q000378" MajorTopicYN="N">metabolism</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D008297" MajorTopicYN="N">Male</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D008875" MajorTopicYN="N">Middle Aged</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D011379" MajorTopicYN="N">Prognosis</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D055815" MajorTopicYN="N">Young Adult</DescriptorName></MeshHeading></MeshHeadingList></MedlineCitation><PubmedData><History><PubMedPubDate PubStatus="entrez"><Year>2014</Year><Month>10</Month><Day>16</Day><Hour>6</Hour><Minute>0</Minute></PubMedPubDate><PubMedPubDate PubStatus="pubmed"><Year>2014</Year><Month>10</Month><Day>16</Day><Hour>6</Hour><Minute>0</Minute></PubMedPubDate><PubMedPubDate PubStatus="medline"><Year>2015</Year><Month>8</Month><Day>19</Day><Hour>6</Hour><Minute>0</Minute></PubMedPubDate></History><PublicationStatus>ppublish</PublicationStatus><ArticleIdList><ArticleId IdType="pubmed">25317502</ArticleId><ArticleId IdType="doi">10.1089/lrb.2014.0003</ArticleId></ArticleIdList></PubmedData></pubmed></record>Pour manipuler ce document sous Unix (Dilib)
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