A case report of Clarkson's disease: If you don't know it, you'll miss it |
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Authors: | Bonadies Nicolas Baud Patricia Peter Hans-Jakob Buergi Ulrich Mueller Beatrice U |
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Affiliation: | Department of Internal Medicine, University Hospital Bern, 3010 Bern, Switzerland. |
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Abstract: | The systemic capillary leak syndrome (SCLS), also known as Clarkson's disease, is a rare disorder characterized by paroxysmal capillary hyperpermeability with a shift of plasma fluid from the intravascular to the interstitial space. A 35-year-old previously healthy woman was admitted with rapidly developing hypovolemic shock syndrome, rhabdomyolysis, and diffuse edema. Laboratory analysis revealed a severe hemoconcentration, renal insufficiency, and paraproteinemia. After exclusion of infection, allergy, burning or drug-induced conditions, the clinical presentation was consistent with the diagnosis of SCLS. Though this is a rare entity, the substantial morbidity and mortality associated with it necessitate the physician's awareness in order to provide timely therapy. This report is meant to enhance awareness of SCLS. |
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