Publications by authors named "Kjell Sveen"
Article Synopsis
- Clarkson disease is a rare condition characterized by episodes of severe fluid leakage from blood vessels, leading to hypotensive shock and other symptoms.
- Researchers studied markers related to the endothelial glycocalyx (eGCX) in patients with this disease, finding elevated levels of certain components during acute flare-ups and recovery.
- The findings suggest that dysfunction of the eGCX, rather than direct damage to endothelial cells, may play a key role in the symptoms of Clarkson disease, indicating potential ways to monitor and treat the condition.
BACKGROUND 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.
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