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dc.contributor.authorBøe, Ole Wilhelm
dc.contributor.authorSveen, Kjell
dc.contributor.authorBørset, Magne
dc.contributor.authorDruey, Kirk M.
dc.identifier.citationThe American journal of case reports. 2018, 19 176-182.nb_NO
dc.description.abstractBACKGROUND: Systemic capillary leak syndrome (SCLS) (Clarkson’s disease) is a rare disorder of unknown etiology, characterized by transient episodes of hypotension, and the microvascular leak of fluids into the peripheral tissues, resulting in edema. Between 80–90% of patients with SCLS have a concomitant monoclonal gammopathy. Although translational in vitro studies have implicated vascular endothelial barrier dysfunction in the etiology of SCLS, the etiology and disease associations in clinical cases remain unknown. CASE REPORT: We report a case of SCLS in a 49-year-old woman who initially presented with an upper respiratory tract infection, which was complicated by edema and compartment syndromes in the extremities that required fasciotomies. Serum levels of the cell surface heparan sulfate proteoglycan, syndecan-1 (CD138), a measure of endothelial surface glycocalyx (ESG) damage, were measured by enzyme-linked immunoassay (ELISA), peaked at up to 500 ng/mL (reference range, 50–100 ng/mL) and normalized on disease remission. CONCLUSIONS: This case report supports the view that damage to the microvascular endothelium, has a role in the pathogenesis of acute SCLS. This case also indicated that monitoring serum levels of syndecan-1 (CD138) might be used to monitor the progression and resolution of episodes of SCLS.nb_NO
dc.publisherInternational Scientific Informationnb_NO
dc.rightsAttribution-NonCommercial-NoDerivatives 4.0 Internasjonal*
dc.titleRaised serum levels of syndecan-1 (CD138), in a case of acute idiopathic systemic capillary leak syndrome (SCLS) (Clarkson's disease)nb_NO
dc.typeJournal articlenb_NO
dc.typePeer reviewednb_NO
dc.source.journalThe American journal of case reportsnb_NO
dc.description.localcodeOpen Access. This paper has been published under Creative Common Attribution-NonCommercial-NoDerivatives 4.0 International (CC BY-NC-ND 4.0) allowing to download articles and share them with others as long as they credit the authors and the publisher, but without permission to change them in any way or use them commercially.nb_NO
cristin.unitnameInstitutt for klinisk og molekylær medisin

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