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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pubmed:25317502Le document en format XML
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<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>
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<author><name sortKey="Slagsvold, Carl Erik" sort="Slagsvold, Carl Erik" uniqKey="Slagsvold C" first="Carl-Erik" last="Slagsvold">Carl-Erik Slagsvold</name>
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<author><name sortKey="Aagenaes, Oystein" sort="Aagenaes, Oystein" uniqKey="Aagenaes O" first="Oystein" last="Aagenaes">Oystein Aagenaes</name>
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<term>Body Water</term>
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<term>Femelle</term>
<term>Humains</term>
<term>Indice de masse corporelle</term>
<term>Jeune adulte</term>
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<term>Études cas-témoins</term>
<term>Études de suivi</term>
<term>Études transversales</term>
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<keywords scheme="MESH" type="chemical" qualifier="metabolism" xml:lang="en"><term>Biomarkers</term>
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<keywords scheme="MESH" qualifier="complications" xml:lang="en"><term>Cholestasis</term>
<term>Lymphedema</term>
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<keywords scheme="MESH" qualifier="diagnosis" xml:lang="en"><term>Lymphedema</term>
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<keywords scheme="MESH" qualifier="diagnostic" xml:lang="fr"><term>Lymphoedème</term>
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<keywords scheme="MESH" qualifier="etiology" xml:lang="en"><term>Lymphedema</term>
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<term>Body Water</term>
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<term>Cross-Sectional Studies</term>
<term>Female</term>
<term>Follow-Up Studies</term>
<term>Humans</term>
<term>Male</term>
<term>Middle Aged</term>
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<term>Young Adult</term>
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<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>
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<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>
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<Issue>4</Issue>
<PubDate><Year>2014</Year>
<Month>Dec</Month>
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<Title>Lymphatic research and biology</Title>
<ISOAbbreviation>Lymphat Res Biol</ISOAbbreviation>
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<ArticleTitle>Hereditary lymphedema, characteristics, and variations in 17 adult patients with lymphedema cholestasis syndrome 1/Aagenaes syndrome.</ArticleTitle>
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<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>
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